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A rare case of disseminated rhinosporidiosis is reported in a young male patient. The pathophysiology, clinical feature, diagnosis and management of this condition are reviewed.
Key words: Rhinosporidiosis, Rhinosporidium Seeberi
Rhinospordiosis is a chronic granulomatous disease characterized by production of polyps or other manifestations of hyperplasia on mucous membrane surfaces, the etiologic agent being Rhinosporidium seeberi. The disease was First describe d by seeber (1900) in Argentina. This noval pathogen commonly affects mucosa of nose, eye and upper aero-digestive tract of men and animals. Isolated deep seated Rhinosporidiosis is rare. Diagnosis is mainly based on clinical suspicion and histopathological confirmation. At present, no existing medical treatment cures the disease and excision of the mass with cauterization of the base is considered as treatment of choice.
Case Report
A 35 year old male patient came to us with history of multiple swellings all over the body (Fig-1) for last one year, distension of the abdomen for last 2 months and loss of appetite for last 20 days.
The patient came to us for loss of appetite. He was not a known case of DM, HTN, SCD, Pul TB. On examination, the patient was chchectic with mild pallor, noi icterus, with a pulse rate of 84/minute, regular, BP-100/80 mmHg, CVS-NAD, Chest:-NAD, Abdomen: No hepatosplenomegaly or engorged abdominal veins. There were multiple subcutaneous swellings ranging from size 1 1cm to 5 7cm.
DC:-N-82%, L-17%, E-01%
FBS:-106 mg%
ESR:- 5mm in 1st hr,
Sr. Urea –30 mg%
Sr. Creatinine :- 1.43 mg%
Urine – NAD
HbsAg –Negative
Anti-HCV:- Negative
ELISA for HIV- Negative
S.Bilirubin – 0.6 mg%
SGPT :-51 IV/It.
SGOT :-41 lU/Lt
Sr.Alk. phosphatase :-221 lU/Lt
Sr. Albumin: 2.9 gm%
USG of Abdomen:- Normal
Chest X-Ray PA view –Normal
Biopsy study of subcutaneous swelling: Large numbers of spherules (size –10-200 um) in a stroma of connective tissue and capillaries. The spherules contain thousands of endospores.
The patient was diagnosed as a case of disseminated rhinosporidiosis. The patient was stared with tab Dapsone 100 mg/day & Vit-B complex. On regular follow up since last 6 months, the size of subcutaneous swelling were regressing.
Discussion :
RHINOSPORIDIOSIS is a chronic granulomatous disease characterized by production of polyps or other manifestations of hyperplasia on mucous membrane surfaces. The etiologic agent is Rhinosporidium seeberi.
Historical review:
Most of the early studies of rhinosporidiosis were made in India and Ceylon where the disease occurs frequently. Sporadic case have been detected and studied in many parts of the world. The systematic position of R. seeberi is still uncertain. Most investigators consider it has not been isolated in culture.
Clinical Types:
Friable, highly vascular, sessile or pedunculated polyps may appear on almost any mucosal surface, and rarely secondary lesions are found on skin, probably as aresult of autoinoculation by scratching. Lesions of the mucosae often spread by extension beyond the mucocutaneous border.
Primary lesions appear most often on the nasal mucosa and are accompanied by painless itching and a profuse mucoid discharge. The lesion is at first flat and sessile. Later hyperplastic growth greatly exceeds lateral extension of the lesion so that a polypoid mass much larger than the peduncle develops. The polyp may extend from the neres into the pharynx or externally over the lip and may reach weight of 20grams. It is friable and bleeds freely after trauma. Its surface is mucoid and papillate or so lobulate that its surface suggests that of a cauliflower. The color varies from pink to purplish red, and close examination of thesurface mayh reveal minute white sports which are the mature sporangia of the fungus. Lesions are found also on the larynx, Penis vagina, rectum and skin.
Lesions of the eye may cause symptoms similar to those produced by a foreign body, lacrimation or photophobia. Growth of the polyp may cause eversion of the lid. Lesions on th eskin being as papillomas and become warty with inclusions of myxomatous material. They are relatively painless except when on the sole of the foot and when they become so large as to be uncomfortably heavy. Dissemination to visceral organs is rare.
Differential diagnosis:
Typical lesions of rhinospordiosis can be recognized usually by the pink to purple colour, friable consistency and the presence of barely visible white sporangia within the polyp. Atypical lesions or those in unusual anatomical sites must be differential from warts, condylomata and hemorrhoids.
Little is known about the immunology of rhinosporidiosis.
On the examination of the gross tissue, unless rhinosporidiosis has been suggested by the clinician, or by the history of the patient’s geographic residence, the pathologist may consider the specimen an ordinary nasal polyp. The correct diagnosis can usually be made without difficulty on examination of routine H and E stained slide. Under the scanning lens of the microscope, although the polypoid structure may be evident, the histopathologic pattern differs greatly from tht of the common nasal polyp. The most striking feature is the presence in the stroma or epidermis of numerous sharply defined globular cysts which usually vary from 10 to 200  in diameter. Some of the cysts may be partly collapsed, assuming a semilunar shape. In contrast to the loose, edematous, myxomatous stroma of the ordinary nasal polyp, the stroma in rhinospordiosis is rather dense. There is a chronic inflammatory reaction in which neutrophils, plasma cells and lymphocytes are prominent. In contrast to the usual nasal polyp. Eosinophils are inconspicuous. Occasionally purulent microabscesses occur.
The cysts of all size have a sharply defined chitinous appearing wall. In a large maturing cyst the wall alone may be 5  thick. Histologically, rhinosporidiosis should be differentiated, specially in immuno-suppressed persons with other fungal infection like Coccidioides immitis.
Differential incidence: Although rhinosporidiosis is seen most often in children and young adults, it occurs at any age. No racial difference in susceptibility are recognized. The disease is seen much more frequently in men than in women, but the extent to which this difference is related to greater frequency or severity of exposure is difficult to evaluate. Infections are seen most often in labourers and in those with frequent exposure to water of streams and pools.
Geographic distribution:
Rhinosporidiosis is found must often in India and Ceylon, but it is reported also from the East Indies, the Malay, States, the Philippines, Iran, South Africa, Italy, England, Scotland, Southern United States, Mexico, Cuba, Argentina, Brazil and Paraguay.
Source of infection:
The disease is not contagious, and sources of infection are exogenous. The frequent history of prior extended to water of pools and rivers and the occurance of multiple cases among those members of a group of workmen most intimately and repeatedly exposed to water source suggest the R. seeberi has a natural habitat in water. Rhinosporidiosis was observed in workmen who dived under water to bring up san din buckets, but not in their associates who carried the sand from the water’s edge. It has been suggested that water insects or fish may be hosts of the fungus.
Laboratory diagnosis:
Direct examination of the surface of the polyp may reveal the subsurface position of sporangia which are white and so large (up to 350  in diameter) that thery can be seen with naked eye. Dissection of sporangia or excision and microscopic examination of tissue confirms the diagnosis. Culture is not successful, and the inability of R. seeberi to grow on artificial media, as well as some peculiarties about its reproductive cycle in tissues, have raised the question whether it is actually a fungus.
It is resembles in general appearance and in manner of sporulation some species of synchytrium, which are obligate parasites of plants, and which produce characteristic galls on the host plant. Animal inocultion is not helpful in diagnosis. Although R.seeberi is found in natural infections of horses, mules and cows, experimental infections usually do not succeed. Recently molecular methods like polymerase chain reaction are being developed for diagnosis.
Rhinosporidiosis should be suspected or considered in all cases of swellings of nose, nasopharynx and skin. Although disseminated Rhinosporidiosis is very rare, still remains a possibility and requires a different mode of treatment. Presently the medical treatment of Rhinosporidiosis is not satisfactory and requires further study and research.
1. Caldwell, G.T. and Roberts, J.D.: Rhinosporidiosis in the United States J.A.M.A. 1938; 110,1964.
2. Karunaratne W.A.E.: Rhinosporidiosis in Man, London, Athlone Press, 1964.
3. Weller, C.V. and Riker, A.D.: Rhinosporidiosis in Man, London, Athlone Press, 1964.
4. Weller, C.V. and Riker, A.D.: Rhinosporidiosis seeberi Am.J.Path, 1930,6,721-732.
5. Baron, E.J., Peterson, L.R. Finegold, S.M. New, Controversial difficult-to-cultivate or non-cultivate etiological agents of disease in Bailey and Scott’s Diagnostic Microbiology, 9th Edition, Mosby, st.Louis, Baltimore, Boston, 1994; p-585.
6. Gori, S. Scasso, A.: Cytologic and differential diagnosis of rhinosporidiosis: Acta Cytologica, 1994: 38(3):361-366.
7. Saha, S.NB., Mondal, A.R. Bera, S.P., Das S., and banerjee, A.R.: Rhinosporidial infection in West Bengal – Calcutta based hospital study. Indian Journal of Optolaryngology & Head Neck Surgery – 2001:53(2):100-104

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The incidence of venomous snakebites is high in rural areas where people are engaged in agricultural activities & are in the habit of sleeping on the floor. About 40,000 people die each year from venomous snake bites (worldwide).Death can be prevented if diagnosis & management is made early in the course. Thus a high degree of clinical suspicion, a timely diagnosis & management is life saving in these cases as it happened in this case report.
Acute onset of pain abdomen in previously healthy young male adults who were sleeping on the floor, without any history of bite, were successfully treated & cured based on high index of clinical suspicion with appropriate antivenom therapy.
Key words : Venomous snake bite , Clinical suspicion, Acute abdomen, Timely diagnosis & management
Snakebite is common in rural areas. Venomous snakenites of the families like Elapidae (Cobra, Krait ) & Viperidae (Viper) are verycommon. Clinical symptoms & signs vary with the species of snake & the amount of envenocation. Fang marks , local swelling , echymosis, blistering , necrosis , are seen at the site of bite in viper bites. Bleeding & clotting disturbances, DICC, ARF , shock are also seen inm veperbites. (Krait ) local signs are absent & the neurotoxic symptoms mostly appear within 1-2hrs after the bite (15min.-15hrs). Blurring of vision , ptosis , dysphagia, Opthalmoplegia, paralysis of muscles of respiration, alteration of consciousness, coma, convulsion occur.
But a patient with a venomous snake bite presenting with features of Acute abdomen has not been reported in neurotoxic bites except in cases of viper & colubridae bites so far. Therefore acute pain abdomen with neurotoxic signs & symptoms should prompt one to suspect snake bite & to exclude other causes of Acute pain abdomen with neurological signs , so that appropriate therapy (ASV, Neostigmine) & supportive measures can be life sving.
Case report
(1) A young male adult about 26 years tushed in to the casualty O.P.D. in early morning with complaints of acute pain abdomen, vomiting , difficulty in deglutition for 6 hrs. Family History : Similar symptoms in his mother who died 1 month back. No other specific complaint was reported by his relatives except for some weakness & frothing from mouth. For this he was treated at Sundergarh district hospitas as a case of acute pancreatitis & was referred to V.S.S Medical College, Burla as patient deteriorated,. For further evaluation & management.
Examination revealed:
Average Body built, drowsy ptosis, frothing from mouth, Restlessness because of severe pin abdomen.
Pulse – 82/min, regular,
BP – 140/90 mmHg
R.R – 20/min, Thoracoabdominal type , No pallor, Icterus, Cyanosis, Lymphadenopathy
Chest – Clinically NAD
P/A – Revealed diffuse tenderness & rigid abdomen , No organomegaly.
Bowel sounds absent – No evidence of freefluid.
CNS – Conscious, drowsy, difficulty in speech & deglutition ( both liquied + solid ) bilateral ptosis was present
No sensory, motor deficit
No meningeal sign
Plantar ( B/L Flexor )
After about 5 min. of admission in the casualty O.P.D., Surgery consultation was done who excluded any surgical, cause of acute abdomen. Necessary investigations were done immediately , which revealed DC – N80 ,E1,L18,M1, TLC- 10,200/Cumm, HB % - 11.6gm %ESR-20mm,1st hr, BI.Urea-40 mg %Sr.Cr.-0.8mg%,Sr.Na+ -137m mol/L,Sr.K+=2.6M MOL/l,Sr.LIPASE- 180 I.U./L, Whole Blood Clotting test neg. , PT – N Sr. Amylase –27UL,St. X-ray Abnormal, U.S.G. Abd. – NAD , ECG – Normal except for the presence of U. waves due to hypokalemia, chest X- ray – NAD.
The patient was finally diagnosed provisionally basing on minimal Neurotoxic sogns & symptoms as a suspected case of neurotoxic snake bite & was treated with (150ml) i.e. 15 vials of Antisnake Venom (ASV), Inj. Atropine 0.6mg I.M. Followed by Inj. Neostigmine (0.5mg) I.V. every 30 minutes for 5 doses, and repeated at 6 hrly. Other supportive care included Nil orally. Throat suction, Broad spectrum antibiotics, I.V. fluids, Hydrocortisone, Symptomatic treatment of pain & vomiting. Hypokalemia was corrected by I.V. kc! Infusion.
With the above treatment after about 2 hours, the patient’s condition started improving gradually with dimunution of pain abdomen, disappearance of ptosis, dysphagia & the patient was symptom free after 2 days & was discharged from the hospital.
(2) Interestingly another young male of 21 years with definite history of Krait bite also presented after one month with the chief complaint of pain abdomen & on examination had features of neurotoxic signs & symptoms & improved with ASV, atropine, Neostigmine. This substantiates further the fact that acute pain abdomen may be a presenting symptoms in Neurotoxic (krait) bites.
Snake bite is common in rural areas. Agricultural workers, hunters, & people sleeping on the floor are at risk as are evident in these cases. History of snake bite may not be present in some cases especially those bitten by krait (Neurotoxic venomous snakes) & fang marks & local signs may not be present. Signs such as early morning ptosis, dysphagia & pain abdomen should prompt one to highly suspect snake bite (Neurotoxic) & to start treatment as early as possible for a good outcome.
A delay in clinical suspicion & diagnosis & thus institution of proper therapy would have proved fatal in the case like his mother who died one month back with similar symptoms without h/o snake bite & without antivemin therapy: could be due to same environment of snakebite.
Acute abdomen like presentation in neurotoxic snake bites is not yet reported in literature. However this case report suggests that Acute abdomen can be a presentation of snake bite & high clinical suspicion & appropriate therapy may save life.
1. Manson’s Tropical diseases – 20th Edition.
2. Hodge III D, Snake bites. In Emergency medicine Eds. May HL, Little Brown Company, Boston, 2nd Edition 1992, 925-928.
3. Reid H.A. Snake bite in tropics British Medical Journal, 1968 3 (614): 359-362.

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It has found that serum cholesterol is lowered in cases of depressive-psychosis and other disorders. In many circumstances psychosis often leads to suicidal death. In the present study total serum cholesterol has been estimated in Cases of suicidal death and compared with that of Control. It showed that Cases of suicidal death with history of psychosis register a significantly lower level of serum cholesterol when compared to that of Control.

Total serum cholesterol, Suicidal death, Depressive psychosis, Mental disorders.


Recent statistics reveal that about 100 persons commit suicide in India daily. The rate of suicidal death is increasing every day due to interplay of multiple factors like psychological, socio-economic, ill health, failures in the examinations, maladjustment in the social and so on. According to a French sociologist Durkeim "Suicide is death resulting directly or indirectly from a positive or negative act of the victim himself which he knows will produce this result". Suicide from existential point of view reflects a behaviour that seeks and finds a solution to an existential problem by making an on the life of the subject. Hence it is a behaviour on the part of the victim which triggers to end his life. There are some biochemical parameters that predict such behaviour which ultimately lead to attempted suicide, deliberate self-harm (Parasuicide) and finally suicide. Some prospective retrospective studies in suicidal victims have pointed out certain biochemical predictors associated with the suicidal behaviour. The present paper attempts to explore the extent of association of one of these predictors in victims of suicidal death.

In this study 25 Cases of suicidal having history of psychotic disorders like depressive psychosis, schizophrenia, schizo-affective and personality disorders were chosen and the blood sample was drawn from their heart chambers. The total serum cholesterol estimated in each Case by enzymatic cleavage of cholesterol ester by cholesterol esterase. In all cases the time since death was within six hours to avoid autolysis and decomposition. The collected in these test Cases were arranged in a tabular form taking into account age, sex, method of suicide and average total serum cholesterol. In 30 Control Cases i.e. victims of non-suicidal death chosen randomly, the were collected and worked out similarly fro comparison.

15-30 6 141 4 120
31 -45 5 130 4 110
46-60 4 110 1 105
>60 1 100 — —

Cases of suicidal death having history of psychiatric disorders.

Method of Suicide Number of Cases Mean Serum Cholesterol
(in mg%)
Railway Track 4 120
Poisoning 12 125
Burning 2 122
Hanging 5 105
Drowning 1 135
Drugs 1 167

Psychiatric Disorders Number of Cases Mean Serum Cholesterol
(in mg%)
Depressive Psychosis 20 135
Schizophrenia 4 140
Personality disorders 1 170

Cause of Death Number of Cases Mean Serum Cholesterol
(in mg%)
Road Accidents 15 170
Homicides 5 165
Other Accidents 7 190
Natural Death 3 200
* Cases other than suicidal death

The above tables clearly show that the serum cholesterol is decreased in Cases of suicidal death having history of various mental disorders. Among the suicidal death Cases, the Hanging Cases register comparatively low cholesterol level whereas Cases having history of depressive psychosis register a very low level when compared with that of Cases having other mental disorders. Non suicidal death Cases show more or loss normal level of serum cholesterol whereas Cases of death from natural causes show a moderate increase in the level of serum cholesterol probably due to cardiovascular causes. From various studies, it has been proved that serotonin ( 5 - hydroxyl tryptamine ) - a polypeptide in the brain modifies the mood of a person. Low serotonin is associated with various mental disorders including depressive psychosis. Research studies by Roy, Nuttirk and Linoiia (1988) have shown low serotonin level in C.S.F. of the victims of suicidal death. In another study, brains of suicidal victims reflect a moderately decreased level of serotonin and its metabolite 5 - HIAA i.e. 5 -hydroxyl indole acetic acid (Stenely, Man, Coheir (1988)). Further, serotonin is low in persons having low cholesterol level (BMJ-UCP (M.N.): APR 1996). It is because cholesterol helps in transport of serotonin to the brain (Lawrence, Palinkis,.UCP (M.N.): 1996),
If we correlate the results of the above studies, it is apparently clear that low cholesterol level is often associated with low serotonin level which accounts many a times for the characteristic self-destructing violent behaviour of the victims of suicidal death.

While lowering of cholesterol is beneficial to the heart, too low cholesterol level could make a person depressive leading to suicidal death. From the above study it is well understood that low cholesterol level is associated with many instances of suicidal death. Psychiatric patients having low serum-cholesterol level should be carefully observed for development of any suicidal behaviour and timely managed with treatment and life-preserving measures so that many suicides could be avoided.

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endocrinopathies or may a pointer towards a group of disorders. So, these skin manifestations are important in differential diagnosis of many disorders.

Diabetes mellitus, hyperthyroidism, hypothroidism, disorders of adrenals and gonads are the main endocrinopathies having skin manifestations and at time these signs help in reaching a definite diagnosis.

Certain skin lesions such as hyperpigmentation,sclerederma, xanthomas, lipomas, increased muscle mass , vesico bullous eruptions, yellow or red paules, fungal or bacterial infection, alopecia etc need the exclusion of endocrinopathies.

Skin is a very good reflector of internal disease in general and endocrine diseases in particular. Specific skin lesions may reflect a variety of endocrine diseases as well as manifest secondary conditions underlying metabolic disturbances. A knowledge of the same makes the diagnosis of endocrine diseases easier, which avoids delay and difficulty in diagnosis.

A timely diagnosis and early treatment spares future complications and saves the patients from their avoidable suffering. It is important that an attempt should be made to review the available literature to familiarize the treating physicians about the importance of skin markers of endocrine diseases.

These disorders are diabetes mellitus and endocrine disturbances of thyroid , adrenals , gonads and others.

1. Diabetes demopathy (pigmented pretibial patches, skin spots): Lesion of diabetic demopathy are less than 1 cm and are red brown , atrophic macules on the anterior shins which may begin as papules . Capillary basement membranes appear to be thickened.
2. Necrobiosis lipoidica diabeticorum (NLD): MLD lesions also characteristically appear on the anterior shins and are oval, atrophic plaques that can reach several centimeters in size. They have a raised , erythematous border and a yellow – brown, waxy central atrophic area through which telangiectatic subcutaneous vessels are easily visible. These lesions may also ulcerate.
3. Disseminated granuloma annulare (GA): Multiple small, grouped erythematous to flesh-coloured papules, which coalesce into small plaques covering the entire body surface have been associated with diabetes mellitus. However, patients with GA, even if it is extensive, do not necessarily have diabetes mellitus.
4. Lipodystrophy.:
a. The Siep-Lawrence syndrome (and related disorders with lipodystrophy) may be cogential and include insulin- dependent diabetes mellitus, hypertricosis, hyperpigmentation, increased muscle mass and somatic growth elevated trglycerides and cirrhosis of the liver.
b. Local lipoatrophy or lipohypertropy may appear at sites of insulin injection. The mechanism is unclear.
5. Insulin resistance and acanthosis nigricans: May be associated with polycystic ovaries in young women (Type A) or with antibodies to insulin receptors and other autoimmune phenomena (Type B).
6. Bullous diabeticorum: Tense blisters that appear suddenly on apparently normal skin usually on the extremities may occur in diabetes. Histologically the blister may be intraepidermla . Lesions may tend to ulcerate.
7. Eruptivexanthomas are accumulations of lipid in macriphages to form widespread yellow to red papules, which may occur in a linear fashion in response to trauma (the Koebner Phenomenon). These lesions appear in patients with poorly controlled diabetes mellitus who have massive hypertriglyceridemia.
8. Sclerederma (adultorum of Buschke) is a rare condition seen in severe insulin- dependent diabetes in which there is thickening of the collagen in the dermis and deposition of acid mucopolysaccarides between collagen bundles. The lesions are broad areas of non-pitting edema and hardening of the skin, especially on the face, neck, and upper trunk.
9. Idiopathic hemochromatosis , a disorder of excessive iron storage, consists of a triad of hepatic cirrhosis, cardiac insufficiency and diabetes. There is striking hyperpigmentation due to increased melanin deposition in the basal layer of the epidermis.

1.) Candida infection of the skin and mucous membranes occur in similar locations as described in glucocoriticoid excess.
2.) Cutaneous bacterial infections, especially staphylococcal infection such as folliculitis furinculocis and frank abscess formation may occur in poorly controlled diabetes patients.
Opportunistic infection by fungal organisms such as Mucor and Rhizopus (Phycomycosis) are rare but are seen in diabetics. Such lesions may be fatal.

1. General features of hyperthyroidism include warmth, increased sweating flushing and a smooth velvety texture of the skin. In sever cases there may be generalized. hyperpigmentation.
2. Hair and nails: Hair may becomethin, fine, limp and oily. There is distal onycholysis (separation of the nail plate from the nail bed). characteristically starting on the fourth finger (plummer nail).
3. Pretibial myxedema (PTM) :Localised accumulations of acid mucopolysccharides (hyaluronic acid) appear as red to brown to yellow plaque, usually on the anterior shins. There is a “peau f orange” pebbly surface with dilated hair follicles and coarse hairs. Lesions are non-pitting, cool, non-tender and may be pruritic. PTM is seen with active Graves Diseaseas well as or euthyroid “burnt out” or treated patients. High long-acting thyroid stmultor )LATS) levels have been associated with PTM.
4. Thyroid acropachy: Diffuse thickening of the distal extremities, clubbing and periosteal new bone formation with characteristic perpendicular bone spicules are seen associated with Graves Disease, often with opthalmopathy and pretibial myxedema.
1. Congential signs of hypothyrodism are shubtle and should be vanishing with the advent of newly started screening programs for hypothrroidsm. General features includes cool, pale, translucent, “alabaster” skin with a yellow hue. This colour and texture are due to decreased blood flow anaeia. infiltration with mucopolysaccharides, prolonged jaundice and / or cartoenmia. Sweating is reduced. Thermal instability and frank hypothermiaare accompanied by persistent cuits marmorata (purplish discoloration), a violaceous mottling in a vascular pattern. Umbilical hernia, short proximal limbs depressed nasal bridge and large tongue complete the picture of congential hypothyroidism.
2. Acquired hypothyroidism has many of the same general feature as seen in the congential disease. Children and adults, in addition have more prominent dry and scaling skin resembling ichthyosis. Sweating is minimal as the eccrine ducts become atrophic, Generalised myxydema make the skin puffy, pale and doughy with a non-pitting edema, espically noticeable around the face and eyelids due to the accumulation of acid mucopoly saccharides.
3. Hair:Scalp hair is lost in a generalized pattern. The remain scalp hair is coarse and dry. Loss of the lateral one third of the eyebrows. (St. Ann Sign) is characteristic. Pubic and axillary hair is spares. Children may get a paradoxical hypertrichosis of arms. back and legs which is reversible with treatment.

A. HYPOADRENALISM (Adrenocortical insufficiency. Addition’s disease)
1. Patients gradually develop generalized hyperpigmentation, there is darkening of the aerolae, genital skin preexistent nevi, palmar and plantar creases and recent scars. Blue black pigment deposit are seen on the gum at the toot margin and on the hard palate. The etiology of hyperpigmentationis probably due to excessive secretion of melanocyte –stimulating hormone (MSH).

2. 2). Hair in pubic and axillary areas becomes sparse and nails may develop hyperpigmented and linear streaks.

3. 3). Vitiligo rarely occurs in patients with Addison’s disease.

Pituitary cushing syndrome, adrenal tumors, ad autonomous nodlar adrenal hyperplasia may have features of androgen excess as well as of the glucocorticold excess.
Iatrogenic Cushing Syndrome, resulting from systemic or topical glucocorticosteroids, does not have the androgenic features.
a). Primary features of glucocorticoid excess
1. General features include plethora, telangiectasias and increased growth of fine, downy vellus hair, especially on the sides of the face. With prolonged exposure, there is generalized atrophy of the skin and easy bruisability.
2. Striae represents both dermal and epidermal atrophy and occur in areas of skin tension. They are pigmented and red to blue because the dermal and subcutaneous blood vessels show through the translucent and atrophic skin. In some cases, they may become hyperpigmented with melanin.
3. Steriod acne appears on the upper arem, chest and back as well as on the face. The lesions are actually perifolliclar, hyperkeratotic papules, althroug in late stages these may become pustular and comedonal. The erupt at the same stage of development.
4. Redistribution of subcutaneous fat to the cheeks, upper back, buttocks and abdomen is characteristic.
5. In pituitary disease of in syndromes of ectopic adrenocorticotropic hormone (ACTH) production, there may be hyperpigmentaton, such as seen in Addition’s disease.
6. The androgenic effects from weak adrenal androgens such as dehydropiandrosterone (which may be converted to more potent androgens such as testosterone and dihydrotestosterone) produce classic acne vulgaris as well as hirsutism and male pattern hair loss in women.
h. Secondary features of glucocorticoid excess
1. Tinea versicolor appear as scaly, oval macules and patches on the upper trunk, back and arms wit variable hyperpigmentation or hypopigmentation. it is due to a superficial colonization with the fungus. Malassezia furfur.
2. Candida (Monilia) infections of the skin and mucous membranes include buccal mucosa (thrush) vaginal mucosa, nails and surrounding tissues (onychomycosis and paronychia). Interdigital webs, corners of the mouth (perleche) and intertriginous folds (intertigo).
3. There is an increased occurrence of cutaneous bacterial infections with pustules, furuncles and abscesses especially caused by Staphylococcus aureus.

1. Infancy, severe forms of CAH are caused by enzyme defects in the to the synthesis of cortisol and may have only mild cutaneous signs, primarily hyperpigmentation of genital skin from excessive pituitary production of ACTH or MSH or both.
2. In later childhood, early onset of pubic and auxiliary hair and acne are signs of androgen excess.
3. In postpubertal adult women and perhaps in some men “mild” (or “partial” or late onset”) forms of CAH are now being recognized primarily from their skin manifestation of severe late onset or persistent acne, hair loss and hirsutism in women.
Hirustism is defined as excessive hair in androgen-dependent areas , such as upper lip, chin, areolae, linea albha and upper inner thighs. Male pattern hair loss (androgenic alopecia) is thinning of the hair over the crown of the scalp with relative sparing of the sides and back. Affected hair are of smaller diameter and grow to shoter length before entering the resting phase and falling out.
The diagnosis of CAH is made by finding elevation of the hormone that the sustrate for the deficient enzyme (e.g 17-alpha-hydroxyprogesterone in 21 hydroxylase deficiency, II-deoxycortisolin II-hydroxylase deficiency and 17-alpha- hydroxypregnenolone in 3-beta-hydroxysteroid dehydrogenase deficiency). If levels are not elevated in basal blood samples, these may be found as abnormal responses to ACTH stimulation.

A. Androgen excess: from the ovary will cause the same skin manifestations of acne, hair loss, and/or hirsutism as described above. Rarely, ovarial tumors are the cause; most grequently, polycystic ovarian disease (PCOD) is the source of hyperandrogenemia from the ovary.
B. In gonadal dysgenesis: (Turner Syndrome), there may be a few special cutaneous features such as multiple brown –black acquired nevi, a double row of eyelashes and low W-shaped posterior hairline, short fourth (and often fifth) digits on the hands and feet with dysplasia or absence of nails hyperconvexity of finger nails and toenails.

A. Mucocutaneous candidiasis has been associated with muliple endocrinopathy syndrome of autoimmune disorders, including hypoparathyroidism, Addison’s disease, premature ovarian failure, diabetes and Hashimoto’s thyroiditis. Vitiligo complete loss of pigment in patches and alopecia areata patchy hair loss which may progress to total loss of scalp hair (alopecia totails) or of al body hair (alopecia universails) have also been seen in association with these endocrinopathies.
B. Alopecia has been reported in association with vitamin –D resistant rickets in a few well-studied families.
C. Multiple mucosal neuromas are found in the multiple endocrine neoplasia (MEN) syndrome in association with a marfanoid habitus, thick lips hig calcitonin levels, medullary carcinoma of the thyroid and phenochromocytoma.
D. The glucagonoma syndrome consists of a diffuse, scaly brightly erythematous rash made up of gyrate, serpiginous plaques with clear centers and superficial scales. The lesions tend to expand, thus the name necrolytic migratory erythema. Such lesion are seen in association with malignant neoplasms of the alpha cells of the pancreas and have regressed when the tumor has been surgically removed.
E. Acanthosis nigricans (AN) has been associated with a wide variety of endocrine disorders including. Cushing syndrome, pituitary tumours, acromegaly, Addisons’s disease and polycystic ovarian disease. Most cases of AN have underlying insulin resistance.

• Many endocrinopathies involve skin as primary or secondary complication.
• Skin is a very good reflector of systemic disorders including the endocrinal diseases.
• Diabetes mellitus is commonly associated with varied dermatological lesions.
• Thyroid disorder (hyper or hypo) involve skin through various mechanisms.
• Adrenal pathology is associated with various manifestation of skin and its appendages.
• Other uncommon endocrinopathies are also someway associated with dermatopathies.

Endocrinipathies are associated with a large number of skin markers. A perfect knowledge about the pointers is mandatory for an endocrinologist or a physician.

The services of a dermatologist will be of immense help in such a situation and hence ideally in case of doubt in any cases the opinion of the dermatologist should be obtained in the greater interest of proper, diagnosis and ideal patient care.


Dr. B.K. Barik, Dr. Dinabandhu Sahu, Dr. Abhiram Behera, Dr. Pusparani Das


"Super Vasmol" a commonly used hair dye containing various chemical and herbal ingradients may produce severe local and systemic manifestation when ingested. The fatality is low, the incidence is rare. Here we are reporting a case of vasmol poisoning who was thoroughly investigated and followed up till complete recovery.

Super Vasmol, PPD, Resorcinol, Gastritis, Angioneurotic oedema.


`"SUPER VASMOL 33 WITH AYURPRASH" is an emulsion hair dye containing Paraphenyl Diamine (PPD), Liquid Paraffin, Cetostearyl Alcohol, Sodium Lauryl Sulphate, EDTA Disodium, Resorcinol, Propylene glycol, Herbal extracts, preservatives and perfume.

Severe angioneurotic oedema, acute renal failure (PPD); oedema, haemolysis, methemoglobinemia in infants, blue black pigmentation (Resorcinol); Acute haemolysis (Propylene glycol); headache, vomiting, gastritis (EDTA) are the serious systemic manifestations when the dye is ingested besides the local allergic reactions observed when in contact with skin in few cases.


A 20 year female alleged to consume 'Super Vasmol 33' on previous night at 10 PM presented with vomiting , dyspnoea, dysphagia, difficulty of speech and gross swelling of neck and face below the chin and mandible (both sides) and dark urination.

The Patient was admitted to the local hospital (after 8 hours of ingestion) with worsening of above mentioned symptoms and treated with injection hydrocortisone (200 mg), injection Chlorpheniramine maleate (1 Amp) injection Cefotaxim (1gm-2 vials), injection Deriphylline (1 Amp) , injection Ranitidine (1 Amp) and oxygen inhalation. As the patient did not improve he was referred to this hospital.

At the time of admission (12 hours after ingestion) the patient was severely dyspnoeic with semi opened mouth having gross swelling of tongue and oral mucosa with salivation. The lip and gums were swollen with black pigmentation, ulceration and bleeding. There was difficulty in speech and swallowing. The urine colour was greenish black and volume was 400 ml since 12 hours. There was swelling efface (submandibular, submental and parotid region) extending to neck.

On examination the temperature was 98.8°F, Pulse 88/minute, regular , BP 126/80 mm Hg, without any localized or generalized lymphadenopathy. There was no pallor, cyanosis or icterus except black pigmentation of lip and gum. Respiration rate was 24/min, regular with vesicular breath sound without any added sound. Heart sounds, were normal without any murmur. There was no neurological deficit. The pharynx could not be examined due to oedema and ulceration of tongue & oral cavity. P/A examination revealed soft abdomen without any organomegaly. The urine was greenish black in color with suppressed volume (400 ml -12 hours). Other systemic examination were normal.

Patient was treated with gastric lavage by normal saline, O2 inhalation, injection Adrenaline - 0.6 mg (starting dose), Injection Avil, Injection Hydrocortisone (100mg) 8 hourly, injection Pantoprazole OD, injection Frusemide, injection Deriphylline 8 hourly and Cefriaxone 1 gm I/V twice daily and Tiniba infusion 500 mg daily and intravenous fluid. Patient gradually improved 3rd day onwards with decrease in mucosal swelling and face & neck swelling. There was slow improvement of the voice, dysphagia, dyspnoea and throat pain. Pharyngeal examination revealed oral mucositis with inflamed postcricoid area and presence of slough in the pyriform fossa. Gradually the colour of urine changed from greenish black to normal with increased volume. The patient was able to take orally (from liquid to solid) with supportive treatment of Xylocaine viscus, antacids.

Early investigation report shows Hb- 10.2gm%, ESR - 28mm/1st hr, TLC-13,000, DC - N 92, EO, BO, L8, MO, RBS - 148mg%, B. Urea 58mg%, S. Creatinine - 2.3mg%, S. Sodium -138 mmol/L, S.Potassium - 3.8 mmol/L, S. Bilirubin - 2.8 mg/dl (Total) 1.6 mg/dl (Direct), SCOT - 852 IU/L, SGPT - 1100 ILJ/L, Alkaline Phosphatase 290IU/L. Urine examination shows proteinurea (+), Puscells 0-3 /HPF with granular and hyaline cast. Upper Gl endoscopy (on 4th day of admission) shows erosive gastritis.

On 10th day, the patient recovered fully. The investigation parameters were normal.


The commonly used hair dye having multiple chemical ingredients cause various complications. The fatality is low and incidence of poisoning is rare.

PPD, one of the intermediate dye used in hair colour is associated with systemic toxicities like severe angioneurotic oedema, acute renal failure and rhabdomyolysis. Liquid paraffin, a saturated hydrocarbon can produce extremely rare hypersensitivity reaction and dermatitis. Cetostearyl alcohol, the combination of aliphatic alcohol and esters act as non-ionic surfactant can produce allergic and urticaria! reaction. Sodium lauryl sulphate, an anionic surfactant used as detergent and foaming agent act as direct irritant at high concentration to the skin. 1% may cause contact dermatitis with burning, redness, tightening of skin with painful fissure and lamellar exfoliation. 2% may produce painful oral desquamation. Resorcinol can produce oedema, haemolysis and methemoglobinaemia in infants with blue-black pigmentary chages.

EDTA (0.3gm%) in excess produce headache, vomiting and gastritis. Propylene glycol is relatively nontoxic, may cause acute hemolysis. In animal when injected causes haemoglobinuric acute renal failure. It has low oral toxicity. Absorption through intact skin is minimal but the application of Propylene glycol containing compounds to infants with large areas of desquamation (e.g. Silver Sulphadizine Therapy in Toxic Epidermal necrolysis) has resulted in cardio-respiratory arrest. The kidney excretes 45% of absorbed dose unchanged and the remainder is metabolized by hepatic alcohol dehydrogenase to lactate, acetate and pyruvate. Liver metabolism produces lactic acids, which enters the glycolytic pathway and after large exposure causes an anion gap and metabolic acidosis. Used as a vehicle in oral, injectable and topical preparations, signs of alcohol intoxication seen after intake of vitamins suspended in Propylene glycol. The compound may be absorbed and produce a rise in serum osmolarity. Contact dermatitis, erythematous oedematous plaque and hypersensitivity occurs. Primarily Propylene glycol is a CMS depressant in large doses and such doses may cause hypoglycaemia, lactic acidosis and seizure in susceptible patients.

Our patient who was attended within hours of ingestion presented with some degree of renal, hepatic and haemolytic features. Local reaction, orophayngeal involvement were profound along with erosive gastritis.


1. Goldfrank's Toxicologic Emergencies - 6th Edition, Year 1998, Page Nos. 476-r, 477T, 913-915, 1057-1058.
2. Medical Toxicology Diagnosis and Treatment of Human Poisoning - Mathew J. Ellenhorn, Donald G, Barceloux, Year 1998, Page Nos. 31,32, 36, 442, 528-530,809-810,906,964.
3. Clinical Management of poisoning and drug overdose, Haddad, Shannon & Winchester, 3rd edition, Year 1998, Page No. 1170.

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Takayasu arteritis is a chronic, non-specific, idiopathic inflammatory disease that primarily affects large vessels. The clinical features reflect limb or organ ischamia due to gradual stenosis of involved arteries. It often occurs in females in their reproductive years.

Takayasu’s arteries (TA) is synonymous with non-specific aortoarteritis, aortic arch syndrome, pulseless disease, Martorell Syndrome.

Early reports suggested that the disease was confined to females from Eastern Asia, but it has been recognized world wide in both sexes but among populations, manifestations vary. There are several cohort studies in different parts of both Eastern and Western hemispheres. The results of different studies are dealt with the etiological factors, various markers, clinical features diagnostic criteria and management plans.

The study in India resulted in modification of diagnostic criteria for Indian patients and these criteria are more sensitive and specific than diagnostic criteria used earlier.

Non specific aortorarteritis was introduced to the medical fraternity in 1827 by R. Adams who was the first physician to note the absence of pulses in all four extremities. In 1856, Savroy reported a case of a young female with absence of pulses. In 1908 , Mikito Takayasu a Japanese opthamologist described peculiar peculiar wreath like appearance of retinal blood vessels with absence of radial pulse.

Takayasu’s arteritis is synonymous with non-specific aortoarteritis aortic arch syndrome, pulseless disease, Martorell Syndrome.

It is an inflammatory vascular disease involving large size arteries resulting in occlusive and ecstatic changes mainly in the aorta and its branches. It is a panarteritis. In the first phase, there is acute florid inflammation of all three coats of a large artery (advantitia, medic, intima). In healed fibrotic phase, there is cuffing of vasa vasorum, destruction of elastic tissue and media, followed by fibrosis. There is hyalinization, thickening plaque and patch formation. All these give rise to ‘tree bark’’ appearance of inner wall of large arteries. Adventitia shows most prominent inflammatory reaction. Skipped areas of aortic involvement are quite characteristic of aortorarteritis.

Till date, no definite etiological factor has been established. Infection has been considered to play an important role in the pathogenesis of Takayasu arteritis. Tuberculosis has been particularly implicated in view of the high prevalence of infection in affected patients in endemic zones. Seko et al have reported that CD4, CD8 and natural respond) is expressed strongly in arterial wall. Takayasu arteritis has been associated with different HLA alleles in different populations. Sequence analysis has shown that some of the alleles have specific epitopes which strengthen the argument in favour of an auto-immune pathogenesis. The associated HLA are HLA-DR2, MB1, BW52, DR12, DQW1, HLA-DR4, HLAB5, B21 and others.

It is predominatly a disease of young females in their second or third decade. It occurs worldwide, but commonly seen is Japan, East Asia, India and Mexico. Age of onset may range from infancy to middle age. The disease has predilection for fermales with wide geographical variations. The natural history of this disease has to phases. In the first Pre-pulseless phase, there are non-specific sysmptoms like night sweats, malaise, weight loss, arthralgia, mild anemia etc. It may remit spontaneously in 3 months or progress to pulseless chronic phas.e The symptoms are due to stenotic lesions and are dyspnoea, headahe, diminished urination, intermittent claudication (upper link>lower limb), visual disturbances, Raynaud’s phenomenon, light headedness, leg ulcer etc.

1. Diminished or absent pulses associated with CURRENT MEDICAL JOURNAL OF INDIA: VOL.X, NO. 8 NOVEMBER’ 2004 limb claudication and BP discrepancy (84-96% of patients).
2. Vascular bruit, often at multiple sites such as carotids, subclavians, abdominal vessels etc. (80-94% of patients.)
3. Hypertension generally due to RA stenosis (33-83%), atypical coarctation, diminished aortic capacity and diminished baroreceptor activity.
4. Takayasu’s retinopathy (37%).
5. Aortic regurgitition resulting from ring dilatation of aorta, separation of valve cusps, valve thickening (20-24)% etc.
6. Congestive heart failure associated with hypertension AR, DCM etc.
7. Neurological features secondary to hypertension and /or ischaemia including postural dizziness seizures, amaurosis (complete loss of vision).
8. Pulmonary artery involvement (14-100%) evidenced by oligemic lung fields in chest X-ray. Usually involves right upper lobe artery.
9. Coronary artery involvement is usually limited to ostium and proximal part may cause angina AMI, CCF and sudden death.
10. Erythema nodosum of skin.

Inflammatory aortoarteritis : which may be seen in syphilis, tuberculosis, SLE rheumatoid arthritis, spondyloarthropathy, Beheet’s disease. Kawasaki disease, Gaint cell arteritis coarctation of aorta, Marfan’s syndrome. Elher Danlos Syndrome etc.

i) High ESR:
• Ishikawah found patients with equal distribution in all categories and higher value in younger.
• In a study by Hall et al, 3/4th of patients reported high ESR.
• In a study by Keer et al, 72% of patients with active disease had high ESR, 36% on remission had high ESR.
ii) Other markers:
CRP, vWF, thrombomodulin, tissue factor tPA, various adhesion molecules, IL-IB, IL-6 RANTES etc.

Etiological studies in India and surrogate markers:
• Strong association with HLAB5 (B51, B52).
• No association with any one of five alleles of MICA gene.
• ICMA-I VCAM-I, E- selection, PECAM-I.
• Total T cell in TA patients were significantly higher i.e., high CD4: ratio.
• Increased basal activity of protein kinase C and increase basal level of intracellular Ca+ in T cell.
• Anti-aorta antibody.
• Anti-endothelial Ab.
• Anti-cardio-lipin Ab (IgG)
B. Doppler USG is a very useful tool for early diagnosis and treatment . it is not –invasive and can assess vascular wall inflammation narrowing and blood flow.
C. Angiography as an investigation is the gold standard.
It includes management of active disease, regular follow-up and management of different complications.

1. Systemic features
2. High ESR.
3. Clinical features of vascular ischamia or inflammation
4. Typical angiographic features.

Drugs used presently are the glucocorticoids (Prednisolone), Cytotoxic agents (Cylophosphamide, azathioprine, methotrexate) and mycophenolate mofetil. Plan suggested by Kerr et al is shown in Flow chart on the next page.

Failure of Cytotoxic Agents.
After starting cytotoxic agent, if it is not possible to taper glucocorticoid to alternate day regimen in 6 months or to discontinue completely within 12 months, then it is failure of cytotoxic agents.
Patients with cytotoxic failure should be treated with minimum does of glucocorticoids.
Anti-hypertensive agents to be started in patients with hypertension but hypertension is worsened by steroids. ACE inhibitors requires careful monitoring in renovascular hypertension.

Major advancement in the treatment of this otherwise morbid condition has been brought by PTCA. Lesions in aortoarteritis are purely stenitic in 85% of patients, purely dilative in 2% and mixed in 13%. Stenotic lesions in aorta, renal artery, subclavian, carotid, iliac/ saphernofemoral arteries have been dilated by ballon angioplasty with or without stenting in several patients in India with excellent immediate and long –term follow-up results. After successful PTCA, hypertension is controlled in 87% of patient and claudication improved in 86% patients. Five year mortality rate decreased to 9% in successful angioplasty from 42% in failed angioplasty.

• Hypertension associated with critical stenosis of RA.
• Extremity ischaemia limiting activities of daily living.
• Clinical features of cerebro –vascular ischaemia or critical stenosis (70% narrowing) or both of at least three cerebral vessels.
• Cardiac ischaemia in the setting of proven coronary artery disease.

• Bypass grafting using synthetic (Dacron graft or autologus vessel (saphenous) graft.
• Rescretion and replacement of inter-position graft.
• Patch aortoplasty, endarterectomy and repair of aneurysm.
• Aortic valve replacement in AR.

Several studies have been reported from India an Japan on takayasu’s artertis. Some of the finding are as follows:

The age of onset in Indian patient is either second or third decade whereas in Japan, the median age of onset is 29 yrs and in Europe, it is 41 yrs. There is a gross geographical variation of predilection for females. In japan, females:male ration is 8:1 Mexico 5:1, Israel 1:2:1 , In India 6:4:1 (by Panja et al), 1:5:1 (by Jain et al), 1:2:1 (by Sharma et al). In Japan, the disease mainly involves proximal aorta with features of reversed coarctation where as in India decending thoracic aorta and abdominal aorta are mainly involved and is known as Middle Aortic syndrome. Association with other disease like SLE, rheumatoid arthritis, polymyalgia rheumatica have been reported in India and other countries.

In India, Jain reported anemia in 63.3% cases high ESR in 56 patients out of 93 patients, high creatinine (>2mg%) in 12.3% cases, LVH (by ECG) in 59.4% cases, cardiomegaly in 32% cases and abnormal urogram in 52 out of 77 cases (table-1)

The angiographic finding reported by Jain differ from the findings by Kerr et al in North America (Studyof 60 patients)

This classification is based on natural history complications and prognosis of the disease in an individual. The four most important complications are the retinopathy secondary hypertension, aortic regurgiation and aneurusm formation. Each complication is graded as mild, moderate and severe.
Table No-I
Sign Percentage
• Hypertension
• Anaemia
• Abdominal bruit
• Extra-abdominal bruit
• Valvular lesions
• Congestive heart failure
• Hemiparesis
• Takayasu’s retinopathy
• Hypertensive retinopathy 77.4

The classification is as follows:
• Group-I Uncomplicated with or without pulmonary artery involvement.
• Group-II A- Mild/ Moderate single complication with uncomplicated disease.
• Group-II B Severe single complication with uncomplicated disease.
• Group-III Two or more than two complications or any patients with arteries.

There are various angiographic classification for TA. The first one is proposed by Ueno et al (1967) . In 1975 Lupi Herrera et al proposed another classification. In Japan, few physicians are still following Nasu’s classification. But previously used angiographic classification are susperseded by new angiographic classification proposed in Takayasu conference in 1994 and are as follows:
Type –I Involvement of branches from aortic arch
Type –II a Involvement of ascending aortaarch and its braches.
Vessels Percentages
• Ascending aorta
• Arch of aorta
• Descending thoracic aorta
• Abdominal aorta
• Subclavian artery (R.L)
• Carotid artery (R.L)
• Celiac artery
• Superior mesenteric artery
• Renal artery (R.L)
• Inferior mesenteric artery
• Iliac artery (R.L)
• Pulmonary artery 12.6
Type-II b Involvement of ascending aorta and its branches and thoracic aorta
Type –III Involvement of descending and abdominal aorta and / or renal artery.
Type-IV Involvement of abdominal aorta and/ or renal artery.
Type-V Combined II b +IV
Note:- Involvement of coronary artery is designated as c (+) and involvement of pulmonary artery is designated as p(+).
This classification is helpful in comparing the patients groups according to vessels involved planning surgery . But is of little value in prognosis.
Based on the analysis of 96 patients Ishikawa proposed a criteria for clinical diagnosis in 1988 and is shown in Table-3.


Age <40 years at diagnosis or onset of characteristic signs / symptoms of 1 month duration.

Table No-3
A comparative study of vascular lesions of the patients from Japan (total No of patients 396) and India (total no of patients 510) by angiography:
Japan India
• Narrowing
• Stenosis
• Occlusion
• Dilatation
• Aneurysm 23%
3% 56%

1. Left mid-subclavian artery lesion by angiography.
2. Right mid-subclavian artery lesion by angiography.
1. High ESR> 20mm first hour (Westergren)
2. Carotid artery tenderness.
3. Hypertension 140/90mm Hg in brachial or > 160/90mmHg in popliteal at age <40 years.
4. Aortic regurgitation or annuloarotic ectasia.
5. Pulmonary artery lesion
6. Left mild –common carotid artery lesion.
7. Distal brachio-cephalic aorta lesion.
8. Decending thoracic aorta lesion.
9. Abdominal aorta lesion, with absence of lesion in aorta-iliac region consisting of 2 cm of terminal aorta and bilateral common iliac arteries determined by angiography.

In addition to the obligatory criteria, the presence of two major criteria or one major and two or more minor criteria or four more minor criteria suggest a high probability of the presence of Takayasku’s disease.

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We report a case of Acute pancreatitis in a case of falciparum malaria. The incidence of other complications are common. The mechanism of this rare complication and its pathophysiology is reviewed. ( The Ind. Pract. 2005; 58(10): 649-651).

Malaria (Plasmodium Falciparum Malaria), Complications, Acute Pancreatitis, Serum Amylase, Serum Lipase.

Malaria, a burning problem in tropical countries and a disease of global concern has a major preponderance in India affecting life of all ages and races. It complications are responsible for taking away a major fraction of patient death in our country. The acute and chronic complications are many. As far as falciparum malaria is concerned it has diverse life threatening complications. Acute pancreatitis is very rare but devastating complication of falciparum malaria which we have put as a case report.

A 41 year old male presented in general medicine ward in October 2004 with history of fever of 5 days which was associated with chill and rigor. There was yellow coloration of urine and conjunctiva for 3 days and gradual decrease of urination for 2 days. There was 5-6 episodes of loose motions and vomiting and pain abdomen (confined to epigastic region) for 10 hours before admission. The patient was not an alcoholic. There was no previous such history of pain abdomen. There was no history of trauma to abdomen. He is not a known case of DM, HTN, SCD APD. There was no history of taking any drug.

On examination the patient was average built, conscious with moderate pallor moderate icterus with mild oedema (bilateral pedal) no lymphadenopathy with a pulse rare 92 per minutes, regular, BP 100/70 mm of HG, without any signs of dehydration. Abdominal examination revealed mild distension and diffuse tenderness maximum at epigastric area, smooth tender hepatomegaly ( 5 cm below the costal line) moderate non tender spenomegaly (3 cm below the left costal margin), mild ascites and diminished bowel sound. Cardiovascular system, respiratory system and other systemic examination revealed no abnormality.

HB- 9.8gm%
TLC – 10,200/mm2
DC – N69%, E-1%, L30%, B0%, M0%
Widal test – Negative
Microfilaria – Negative
Serum Bilirubin – Total 13.3mg%, Direct 10.4%
RBS – 172mg%
SGPT – 138IU/L
Serum Alkaline Phosphatase – 110 IU/L
S. Urea – 106mg%
S.Creatinine – 4.2mg%
Serum Amylase – 2050 IU/L
Serum Lipase – 3050IU/L
Serum Ca++ - 7.2gm%
HBs Ag – Negative
HCV Ag – Negative
Malaria Parasite – Slide +ve
QBC – pfr (++)
ICT – pfr (+)

Ultrasonogram revealed hepatosplenomegaly and features of acute pancreatitis and mild ascites. CT scan of abdomen also revealed hepatosplenomegaly ascites and acute pancreatitis.

On the basis of blood report patient was treated along the line of complicated malaria with artesunate, injection cefipime, metronidazole injection, inject able pantoprazole, intravenous fluid and one unit of blood transfusion. The patient was conservatively managed for acute pancreatitis and nasogastric tube aspiration and nil orally for 5 days. The patient recovered within a span of 7 days after which the MP was negative. Serum amylase, serum lipase, Urea, Creatinine and bilirubin came down to 100IU/L, 230 IU/L, 28mg %, 0.8 mg%, 4.2 mg% respectively on 7th day. Repeat USG of abdomen revealed only mild hepatomegaly. The patients was relieved on 14th day of admission.

Pancreatitis clinically presents with upper abdominal pain accompanied by elevated levels of pancreatic enzymes i.e amylase and lipase. The type such as acute chronic haemorrhagic, necrotic are distinguished by history, clinical , biochemical and radiological findings.

Acute pancreatitis presents as neusea, vomiting , anorexia, abdominal pain. Out of numerous causes of acute pancreatitis malaria is rare. But it must be looked for in tropical country like India with high prevalence of malaria.

Falciparum malaria is the deadest among all other types of malaria with varied complications, multiorgan involvement and diverse sequlae. The parasite may affect pancrease causing acute pancreatitis or acute haemorrhagic pancreatitis. The clinical and laboratory findings follow similar pattern of other causes of acute pancreatitis.

The pathogenesis may be initiated by sequestration of parasite in the organ, blood vessels or ducts, damaging acinar cells by premature activation of digestive enzymes with cells. The damaged acinar cells initiate inflammation, activation of platelets and compliment system, which leads to release to cytokines (e.g TNF - , IL – 1, NO, PAF), free radicals and other vasoactive substances. These further directly damage the gland and may cause pancreatic oedema, necrosis, ischaemia and capillary leak syndrome. Tehse lead to a vicious inflammatory cycle damaging further pancreatic tissue. Association of sepsis further leads to organ damage and complications.

Malaria presenting with classical symptoms and signs of acute pancratitis along with other organ involvement may be difficult to correlate. The abdominal pain may be sharp, sudden or constant may be localized to epigastric,periumbilical, block or lower chest. But common presentation in malaria is fever, icterus with distended abdomen, diffuse or localized tenderness. Grey Turners signs or Cullen sign may be noted in advanced cases.

Blood count and chemistry panel usually distinguishes pancreatitis from other acute abdominal causes, Test of malaria (MP, Slide , QBC, ICT) determines malaria aetiology, Increased TLC, Hyperglycaemia, decreased Ca++, Increased alkaline phsophatase, S. Amylase, S. Lipase reflects pancreatic damage.

Serum amylase has low sensitivity (75-92%)and Specificity (20-60%) but is used to confirm acute pancreatitis , when upper limit is raised 3-6 times of normal specificity increases. Serum amylase level is raised 2-12 hours after pancreatic insult and peaks 12-71 hours after.

Serum lipase having sensitivity 86-100% and specificity of 50-99%. Serum lipase raised 4-8 hours after signs and symptoms and peaks at 24 hour and decrease over 8-14 days.

Other recent markers are immunoreactive cationic trypsin, pancreatic elastase – 1 and phospholipase.

Although USG and Ct scan of abdomen makes the diagnosis easy, cholangio-pancreatography may be accompolished bu ERCP or MRCP.

The primary treatment of acute pancreatitis with malaria is to treat the cause i.,e malaria. The treatment of acute pancreatitis is primarily supportive providing adequate hydration, pain relief and pancreatic rest by nasogastric suction and nil orally. Antibiotics are added to prevent sepsis or necrotic pancreatitis with setting of multiorgan involvement.

The prognosis of acute pancreatitis with malaria depends on the stage of presentation, haemorrhagic pancreatitis bears poor prognosis.

Falciparum malaria which has various complications may present as an acute pancreatitis, along with other organ affection. This is a rare condition and must be in mind of clinicians when patient presenting with clinical features of malaria, with pain abdomen , vomiting and localized abdominal tenderness.

A high index of suspicion and thorough clinical examination and investigation are the ways to diagnose the complication i.e, acute pancreatitis. Specific antimalarial drugs are the primary modalities of treatment, along with the conservative management for acute pancreatitis.

1. Von Sonnenberg F, Los Cher T, Nothdurgt HD, Prufer L @ Pubmed . PMID : 3519146.
2. Michelle M, Piet Zak MD (1) Dan W Thomas MD (2).
3. Parenti DM, Steingberg W, Kang P – Infectious causes of ac pancreatitis , 1996; 13(4) – 356-71.

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Herpes simplex virus (HSV) encephalitis usually caused by HSV1 is gravest and commonest form of acute encephalitis. It is usually present with fever, headache, seizure, confusion, stupor and coma. Psychosis may be associated with these manifestations. Here we are reporting a case of clinico-radiologically diagnosed case of HSV encephalitis that present predominantly with psychosis and make diagnostic dilemma. (The Ind. Pract 2005; 58 (1):38-39.

Herpes Simplex Virus, Encephalitis, Psychosis

Among all acute encephalitis 10% of cases are due to HSV. It is usually present like other acute encephalitis. Psychosis is a distinct feature due to involvement of temporal lobe, medial and orbital part of frontal lobe. CT scan imaging may identify the cerebral lesion at temporal and frontal lobe. Fatality rate varies between 30-70%. Early presumptic diagnosis with the help of clinical examination, C.S.F. examination and radio imaging and early therapeutic intervention with acyclovir may save the life of the patient. C.S.F. PCR is the diagnostic approach now for herpes simplex encephalitis.

A 38 year old male presented with psychotic behavior or last 20 days. He was febrile for about 6 days 2 weeks back. Fever was typically low grade, contunuosu. With the onset of fever he starts incoherent talk.

He was also unable to recognize the known persons. His speech frequency was very much decreased and he was reluctant to take food. He was admitted to local private hospital on days of his illness where his CS examination was done. At that time his CSF cell count was 296/cumm, mostly lymphocytes, protein 72mg% and sugar 48mg%. His treatment was started there in the line of TB meningitis but patient gradually deteriorated. Due to his uncontrolled psychotic behavior, psychatric consultation was done, treated with haloperidol. But patient’s psychosis did not improve. Rather his bladder and bowel control was lost.

On the day of our hospital admission patient was febrile, drowsy, disoriented, illusion and auditory hallucination were present. Sensory aphasia, inhoherent talks were associated with those features. His plantar reflex was B/L extensor and gait unsteady. Investigation revealed WBC 11100/cumm. ESR 15mm and RBS 127mg% DC – N 51% and L 49%. Repeat C.S.F. examination was done which revealed total WBC count 76/cumm mostly lymphocytes, protein 28 mg% and sugar 78 mg%. CSF contained plenty of RBC. CT scan of brain showed hypodense area at B/L temporal and fronto medial lobe (Fig. 1 and Fig 2) suggestive of HS encephalitis. Treatment was started with acyclovir 500 mg IV 8 hourly with monitoring pf S. Creatinine level. On 10th day of therapy patient’s psychosis decreased, was able to recognize his wife and son and plantar become flexor, though auditory and gustatory hallucination still persisted.

HSV 1 and 2 infection can manifest a spectrum of illness from stomatitis and progenitor lesion to facial nerve palsy and encephalitis. Encephalitis is due to HSV1. The unique localization of the disease in the temporal lobe may be explained by the virus route of entry into the CNS1. The lesion takes the form of haemorrhagic necrosis of temporal and medial part of frontal lobe2. Lesion of these areas in imaging study with CT or MRI is highly suggestive3. Patients with Japanese encephalitis display abnormalities of thalamus, basal ganglia and midbrain. Other forms of viral encephalitis imaging studies are non- specific.

Manifestations like hallucination, anosmia, personality changes, bizarre behavior or delirium, aphasia usually observed in H.S. encephalitis. Temporal lobe seizure usually seen in ECG. Affection of memory can be recognized in the convalescent state.

CSF often cloudy due to RBC as well as WBC protein may be raised but sugar usually normal. In minority of cases, low sugar in CSF may confuse with TB or fungal meningitis. Test for detection of HSV in the CSF by PCR are useful in diagnosis while virus is replicating in first few days of illness.

Our patient presented at OPD after 3 weeks of illness when the absolute ways to establish the diagnosis is fluorescent antibody study and viral culture of brain tissue obtained by biopsy. Avoiding this hazardous procedure, we treated the patient with empirical antiviral agents based on compatible clinical, radiological and CSF findings. Treatment option for HSE is acyclovir in the dose of 30 mg /kg/day for 10-14 days5. Our patient was responding well with above mentioned treatment. After one month follow up , patient improved with mild psychotic behaviour, gaustatory hallucination and depression.

1. Davis L.E., Joshson R.T.: An explanation for localization of herpes simplex encephalitis. Ann Neural 1979; 5: 2.
2. Adams H. Miller D: Herpes Simplex encephalitis: A clinical and pathological analysis of twenty two cases. Post Grad M.D. J 1973; 49:93.
3. Davis J.M. et al : CT of herpes simplex encephalitis with clinico pathological correlation. Radiology 1978; 129:409 – 17.
4. Lakeman F.D., Whitley R.J. et al : Diagnosis of Herpes simplex encephalitis: Application of PCR to CSF from brain biopsied patient correlation with disease. J. infect disease 1995; 171: 857.

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Sickle cell disease is an autosomal dominant disorder, sickle haemiglobin is the abnormal haemoglobin  chain glutamin acid is replaced by valine. Sickle haemoglobin has the unique property of forming polymersdeoxygenated state. These polymers deform the RBCS to sickle cell. Formation of polymers depends on the concentration of HbS inside the cell. So small reductions in HbS concentration inside the cell might result in significant clinical benefits. That is the reason why sickle cell trait is clinically silent were the HbS concentration is low. With better understanding of pathophysiology of sickle cell disease and its complications, it treatment has also progressed.

Among the haemolytic anaemias, vasoocclusive features are unique in SCD. Now it is well understood that vasoocclusion and tissue ischaemia in SCD involve not only the polymerization of HbS but also interaction between RBCs, endothelium, leucocytes, platelets and plasma factors. Intracellular polymerization of HbS is decreased by a rise in foetal haemoglobin and there increasing HbF is the most clinically studied approach against sickling. Infections, brain injury, renal disease , pain priapism can now be prevented. Complications from lung injury, surgery and transfusion can be minimized.

Management of SCD
Apart from general measures and treatment for symptomatic relief , now the newer therapeutic agents are directed towards prevent of complications.

Sickling can be interrupted at several key pathways:
1. HbF Augmentation
Most promising agent in hydroxyurea, a ribonucleotide reductase inhibitor, causes myelosuppressive – induced HbF synthesis, resulting in improved red cell survival and decreased sickling. Hydroxyurea is orally active, effective , safe in short term and beneficial in most patients, in the dose of 10-15mg/kg to a maximum of 35 mg/kg. Before starting hydroxyurea, base line evaluation like blood counts, MCV, HbF concentration and serum chemical values and test for pregnancy ( a contraindication) to be done. Blood counts should be performed every 4-6 weeks intervals, granulocyte count should be at least 2000/mm3 and platelet counts at least 80,000/mm3 before or during treatment. An initial Hb concentration below 5.5 gm% is not a contraindication to treatment with hydroxyurea. Adults with higher TLC and reticulocyte counts and larger treatment associated decreases in these counts tends to have greater increases in HbF production and hence better response to hydroxyurea. Although hydroxyurea lowers pain episodes, pulmonary events and hospitalizations, 40% of treated patients do not respond or have progressive organ failure, the reason for this is yet to be settled.
Clinical advances in treatment of SCD
Clinical Features Interventions
1. Pain • Prevention with hydroxyurea
• Patients controlled analgesic devices
• Newer NSAIDS
2. Infection vaccine • Prophylactic penicillin and pneumococcal
3. Anaemia • Phenotypically matched RBCs
4. Lung injury prevention • Hydroxyurea
• Antibiotics (Macrolides)
• Transfusions
• Screening for pulmonary hypertension
5. Brain Injury prevention • Screening with transcranial Doppler , MRI, neurocognitive testing
6. Renal • ACE inhibitors for preteinuria
• Improved renal transplantation
7. Call bladder disease • Laparoscopic cholecystectomy
8. Surgery / anaesthesia safety • Preoperative transfusion
9. Priapism • Adrenergic agonist
• Antiandrogen therapy
10. Avascular necrosis of hip • Decompression coring procedures
11. Severe disease (recurrent acute chest syndrome, pain crises or CNS disease) • Allogenic BMT (< 16 years)
• Chronic transfusion and /or hydroxyurea
12. Neonatal screening
13. Family counseling

Other drugs which can increase HBF concentration are short chain fatty acids like valproic acid, 2-deoxy- 5 azacytidine , erythropoitein.

Other emerging therapeutic agents
Drug Mechanism Benefits
1. Clotrimazole Inhibits red cell Gardos channel Red-cell rehydration
2. Sulphasalazine Endothelial Activation Antiadhesion therapy
3. Deferiprone Chelete membrane iron Antiadhesion therapy
4. Acenocumarol, heparin Decrease thrombin Antiadhesion therapy
5. Pheresis Decrease HbS Transfusion therapy
6. Allogenic – Haematopoietic stem cell transplantation
7. Genetherapy –
a. Direct gene replacement – Direct delivery of - globin gene.
b. Indirect gene therapy – Srythropoitin delivery

2. Antisickling agent (through multiple pathway)
Nitric oxide (NO) is a critical factor in the pathphysiology of SCD and hence a potential treatment option. NO regulates vessel tone , endothelial adhesion, leucocytes and platelet activity, an important factor in ischaemia reperfusion injury and sickle cell induced ischaemia. In SCD, more adhesion molecules are produced due to decreased availability of NO. Oral arginine supplementation induces NO production, reduces red cell sickling by inhibiting the Gardos channel (Calcium activiated K = Channel). Treatment of sickle – cell patients with NO or its precursor L arginine have shown promising antisickling activity with vasodilator properties. NO or arginine supplementation may be synergistic with hydroxyurea and seems to further increase. No release and decrease adhesive molecules.

The need to study new applications of current treatment and to devise new treatments direct at disrupting multiple factors of the pathophysiology of the disease remains most important. New therapeutic options like hydroxyurea. No or L-Arginine, BMT, gene therapy appears promising. While awaiting the new treatments for the underlying disease, several partial problems remains unsolved. For example, how should the acute chest syndrome be managed, which patients should undergo exchange transfusion? How aggressively should be blood pressure be lower to decrease the risk of stroke? Can we better understand the cause of striking variability in sickle cell disease, so that hazardous treatment can be directed to the patients who are most likely to have the worst disease complications?

1. Bunn H.F. Pathogenesis and treatment of sickle cell disease. N. Engl J Med 1997; 337:762-9.
2. Steinberg M.H. Management of sickle cell disease. N. Engl j Med 1999;340:1021 – 30.
3. Morris C.R., Kuypers A., Larkin S et al : Arginine therapy : a novel strategy to induce nitric oxide production in sickle cell disease. Br J Haematol 2000; 111-498-500.
4. Vichinsky E . New therapies in sickle cell disease. Lancet 2002; 360: 629-31.

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Sickle cell haemoglobinopathy, a common haemolytic disease predominant in Western part of Orissa is a multisytemic disease, with varied manifestation. Through some case of nephropathy has been reported, earlier here we report a case of sickle cell disease (SED who had primary hypogonadism, hypertension and sickle cell Nephropathy. The pathogenesis and management are reviewed,.

Sickle cell disease, primary hypogonadism, hypertension and sickle cell Nephropathy.

Sickle cell haemoglobinopathy occurs due to genetic defect where due to mutation of  globin the 6th amino acid is changed from Glutamic acid to Valine. Due to dexoygenation there occurs structural changes in RBC Clinically the disorder manifests as chronic haemolysis repeated infection growth retardation and sickle cell crisis. The crisis includes alpastic crisis haemolytic crisis sequestration crisis and vasoocculsive crisis. Renal complications are encountered in some cases where the patient presents with haematuria proteinuria, renal insufficiency.

A 21 years male was admitted to this hospital in July 2005 with history of fever and jaundice for 5 days generalized pruritus for 4 days and decreased uriniation for 4 days. He had gradual diminished of vision of right eye since 5 years. He had taken Blod transfusion 2 months back at local hospital. On enquiry it was found that another brother who had sickle cell disease died 3 years back, due to vasoocculsive crisis. Both parents were found to be sickle cell trait. The patient had normal milestones of development but there was retarded development of secondary sexual characters and gyaecomastia since last 4 years (Fig.1.2)

On examination the patient had severe pallor and icterus, Blood pressure was 160/90 mmHg in both upper limbs. Pulse rate –110/min regular Temperature 100.20F respiration rate 18/minute. Per abdominal examination revealed moderate hepatomegaly and moderate spelenomegaly. He had bilateral gynaecomastia with sparse public and axillay hairs and facial hair. Both the testis volume were reduced (2.5 to 3 cm) soft with normal testicular sensations. Other systemic examinations including higher intellectual function were normal.

The laboratory examination showed:-
Hb:- 6.6% TLC- 8400/mm3, D/C –N 68 E 2L 30 M0 B 0
Serum Billrubin – Total 21 mg/dl
Direct 19 mg/dl.
S.G. O.T - 58IU/L
S.G. P.T - 57 IU/L
S. Alk Phospatase- 338IU/L
• Hb electrophosis showed SS band.
• Urine analysis showed – Albumin (++) with granular cast and RB Ci 30- 40/HPF and 24 hour urine protein was 300mg/in 24 hour with urine output- 2 Litres.
• Serum Zn- 7.5 (11.5-18..5 ml/L)
• Untrasonogram of abdomen showed moderate hepatomegaly, spelnomegaly (18cm) with coarse echotexture with spotty calcification. Both kidneys were normal size(11.0cm x 4.5cm) with diminished corticomeduallry differentiation.
• Chest x-ray and skull X- ray were normal.
• Foundscopy revealed papilloedema (Rt eye) with Grade II hypertensive retinopathy. Visual acuity was 6/60 in right eye and 6/9 in Left eye.
• Fasting lipid profile showed TC-109mg%, HDL-25mg% , LDL-50mg% , TG-376mg%, VLDL-75.30mg% , 212.86ngm/dL (270-1070mg/dl)
• Hormonal assay showed Testosterone –FSH – 8.41 IU/ml, LH17.97IU/Ml, Prolactin –5.45ngm/ml, TSH-3.79IU/ML, (0.5-4.7U/ml) 2.72U/ml) (1.6-23.0 ngm/ml).
• Kidney biopsy showed features of focal segmental glomerulosclerosis (Fig-3, Fig-4)

Patients was treated with antimalarial (Artesunate) antibiotic (Taxim) Pentoxyphylline, folic acid arginine Antihypertensive- Imandipril-10mg (ACE inhibitor) was started and responded well with normalization of BP. After 15 days of treatment the BP was controlled at 120/80 mm Hg, with reduction of Proteinuria. His serum bilirubin came down to 4.6mg/dl (total) and 3.8mg/dl (direct). The patient was discharged.

On subsequent follow up (after 45 days of discharge) 24 hours urine protein –25mg/24 hours, Urine output 2.5 Litres, BP was 120/70.

The gonadal dysfunction as noted in sickle cell disease most often is attributed to primary gonadal failure. Levels of LH are usually increased in sickle cell disease. changes in FSH are usually inconsistent . Testosterone levels are almost always low in male adolescents and adults with sickle cell disease & these levels may respond poorly to GnRH stimulation.

Extreme delay in secondary sexual character is usually confined to males. Testicular size and volume are reduced and testicular histology showed immaturity of seminiferus tubules and replacement by hyaline materials secondary sexual character such as bearded growth, public and axilary hair are retarded.

The determinant of this type of growth may be chronic anemia patients with higher fetal hemoglobin have normal epiphyseal closures. Zinc level are lower in patient with sickle cell disease and studies have shown that zinc supplementation increased growth of body hair, increased testosterone levels and improved testosterone response to LHRH.

Sickle cell nephropathy in the form of glomerular disease occurs in 15 to 30% of sickle cell disease. Usually 15-30% patients develop proteinuria in the first 3 decades. The pathology is usually focal segmental glomerulosclerosis chronic renal failure can be predicted by presence of worsening anaemia, proteinuria, nephritic syndrome and hypertension. ACE inhibitors and ARB retard the progression of renal disease by lowering systemic & glomeruocapillary hypertension.

Hypoxia of renal medulla may also cause papillary necrosis. Coinheritance of microdeletion in  gene appear to protect against development of nephropathy. Cortical infarcts can cause persistent haematuria, pipiallry infects occurs in 50% of patients with sickle cell trait. Painless gross haematuria occurs with higher frequency in sickle cell trait than sickle cell disease.

Ultrasound examination may reversal a focal or diffuse increased echogenecity which is predominant in sickle cell disease. In the medulla renal tubules and vasarecta involvement can occur. Tubular changes results in atrophy dilatation, proteinacous cast and the so called thyroidization of renal medulla also occur.

In sickle cell disease the GFR is markedly elevated in young patients but decreases with age. As age increases the effective renal blood flow. effective plasma renal flow are decreased. Increased GFR, ERRF in sickle cell disease is usually due to increased synthesis of prostaglandins in early part which decrease in later life.

Sickle cell disease is a fairly common disease in this belt of India but usually the focus is directed towards managing vasoocclusive crisis.

But proteinuria, hypertension are the harbinger of sickle cell nephropathy and will respond to ACE inhibitors and ARB. The hypogonadism may be treated in early life by good nutrition, prevention of anaemia and zinc supplementation.

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We report a relatively uncommon association of massive pleural effusion (left) with pancreatic pseudocyst in an adult male patient. The pathophysiology, clinical features diagnosis and management of this conditions are reviewed. (The Ind. Pract 2004; 57(8): 553-554).

Key Words: Pancreatic Pseudocyst, Pleural Effusion

Pseudocysta are localized collections of panecreatic secretions that lack and epithelial lining and persist for more than 4 weeks. In the past pseudocysts were detected. indirectly by clinical suspicion, appearance of a palpable abdominal mass and from barium contrast studies that demonstrated a mass. The advent of pancreatic imaging by ultrasonography and CT Scan has lead to the realization that pseudocysts appear in 10% patients with acute pancreatitis.

A variety of clinical and radiographic finding have been associated wit pancreatitis and pleural effusion is one of them. pleural effusion may be bilateral, confined to left side or rarely right sided and may be massive.

A 50 year old male patient admitted with history of chest pain for 1 month, swelling of abdmen with upper abdominal pain for 1 month, loss of appetite with nausea for 15 days and dyspnoea for last 3 days. Six months prior to admission he was provisionally diagnosed to be a case of pulomonary tuberculosis with left sided pleural effusion and was on treatment with ATT (4 drugs) for 6 months without any clinical improvement. He is not a known case of DM, HTN, SCD. No history of intake of other drugs. He is a chronic alcoholic for last 20 years.

On examination the patients was of average built with mild pallor, no icterus, no lymphadenopathy, with a pulse rate of 76 per minute, regular BP=118/80 mmHg. Cardiavascular system examination revealed apex beat Rt. 5th ICS. Chest examination revealed trachea shifted to right side. on left side reduced movement , stony dull percussion note, vocal response absent, breath sound diminished. Per abdomen examination revealed mild hepatomegaly with a firm intra abdominal swelling palpable in the middle left upper abdominal region, non tender with irregular margin.

Hb = 8.6gm%
TLC = 8000/mm3
DC =N-60% , E-4%, L-36%
ESR =25mm 1st hr
FBS = 92mg%
Urine =NAD
S. urea = 38mg%
S. Creatinine = 1.1 mg%
Na+ = 135m.mol/L
K+ = 3.4 m.mol/L

Pleural fluid analysis revealed , coffee coloured, haemorrhage fluid with TLC could 700 mm3 mostly mestothelial cells mixed with cellular elements of blood. No malignant cells seen. Biochemical study revealed glucose- 60mg% protein – 1.6mg%. LDH- 2280IU/L Amylase- 30 IU/L.

Chest X- ray (PA view0 reveals massive pleural effusion (lt) with trachea shifted to rt.side. USG abdomen and pelvis reveals head of pancreas normally seen but the body and tail could not be visualized. A large cystic lesion of 15cm x 12cm with echogenicity seen with left sided pleural effusion.

The patient was diagnosed as a case of pancreatic pseudocyst with left sided pleural effusion due to acute pancreatitis probably alcohol induced. Pleural tapping was done and about 3000ml of haemorrhagic fluid was drawn out. He was put on of loxacin. valdecoxib, haematinics and other supportive treatment. The patient showed marked improvement during hospital stay and planned for surgical management for the existing pseudocyst.


Pancreatic pseudysts are collections of tissues, fluid debris enzymes and blood which develop over a period of 4 weeks after the onset of acute pancreatitis and constitute about 10% of patients of acute pancreatitis. It is encountered most frequently with alcoholic panaceatitis. Pseudocysts associated with pleural effusion are most common on the left but may be bilateral and rarely limited to right pleural space (Gumaste Singh, Dave et a, 1992) it is assumed to be a new prognotic parameters for acute pancreatitis (Lankisch Droge, Becher et al1994).

Psyeuodycysts lack epithelial lining and disruption of the pancreatic ductal system is common. Approximatelty 85% are located in the body or tail of pancreas and 15% in the head. If the pancreatic duct disruption is posterior, an internal fistula may be develop between the pancreatic duct and the pleural space producing a pleural effusion which is usually left sided and often massive. Conservative therapy is indicated if the pseudocyst is shrinking evidenced by serial ultrasound and minimal symptoms pseudocysts (every 3 to 6 months) Long acting somatostatin anagogic octreotide which inhibits pancreatic secretion is useful in cases of pancreatic ascetics with pleural effusion.

Pseudocyst with communicating duct if strictured require internal surgical or endoscopic drainage. Transgastric percutaneous approach is favoured. Associated massive left sided pleural effusion often required thoracentesis or chest tube drainage. A disrupted pancreatic duct can be treated by stenting.


Association of the left sided massive pleural effusion with pancreatic pseudocyst is relatively uncommon. However, additional studies are needed to substantiate these results.

1. Gumaste V., Singh, V., Dave P. Significance of pleural effusion in patients with acute pancreatitis. Am J. Gastroenterilogy 1992; 87: 871.
2. Lankisch P.,G. Droge M and Becher R. Pleural effusion : A new negative prognostic parameter for acute pancreatitis. Am J Gateroesterol 1994; 89: 1849.
3. Slesinger and Fordtran’s Gastrointestinal and liver disease (6th edn) vol1 Sleisenger Feldman Scharshmidt Klein (W.B Saunders Co) 815:826:836.
4. TB of Gastroenterology (Vol-II) 2nd Edn Year 1995 (T. Yamada. J.B. Lippincott Co. Philadelphia ) page 2078-84.
5. Harrisons principles of Int. Medicine 15th Edn Vol 2 year 2001: Page-1798.

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