Progressive Supranuclear Palsy (PSP) or Steele- Richardson � Olszewski Syndrome is a neurodegenerative condition affecting brainstem and basal ganglia. Often misdiagnosed as Parkinson�s Disease. It is characterized by supranuclear ophthalmoplegia, pseudobulbar palsy, axial rigidity and mild dementia. Diagnosis is usually clinical and treatment is usually supportive.

Hydatid disease is a parasitic infection caused by cystode, EG which is a tape worm causing endemic disease in many parts of world mainly in India3,4. The disease in man is caused by infection of larvae of the tape worm which reside in digestive system of dog (definitive host) after reaching the duodenum of man (intermediate host), the chitinous layer of the larvae is digested and released oncospheres pass through portal circulation, spreads to the liver, lungs and other organs and form cystic lesions3,4,5,6. The life cycle of the parasite is completed when intermediate host dies and body is consumed by definitive host.

Dyselectrolytemia (specially related to Ca++, Mg++) is a very common problem we face in our day-today practice. It can present with various symptoms and signs mimicking a major neurological disease. Clinical suspicion and prompt supplementation is required to reduce the morbidity and mortality of the patients.

Proteins are our only source of amino acids - compounds that form every aspect of the human body.The structure of protein is very complex. The proteins we eat in foods (such as meat, fish, poultry, eggs, cheese, and soy) must be broken down by a number of protein enzymes, or proteases, that are made in the stomach and pancreas. These enzymes break down proteins into smaller molecules and finally into individual amino acids that are ready for absorption. However if we are stressed, eating unhealthy, or are deficient in the pancreatic enzyme, protease, the protein we consume will be poorly digested and not broken down into small enough units for absorption.Oversized protein molecules in the intestine can trigger the release of histamine and other inflammatory compounds.This can result in gas, bloating, belching,constipation, diarrhea, nausea, feeling of fullness for a long time after eating and INFLAMMATION.More problems arise if these oversized proteins are accidentally absorbed into the body, often called "leaky gut syndrome ".If poor protein digestion happens with every meal, inflammation can be triggered throughout the day, never allowing the inflammatory process to die down.Inflammed intestine allow poorly digested protein to pass into the bloodstream.Antibodies in the bloodstream identify these proteins as foreign invaders and alert the immune system to initiate an unneeded and unnecessary immune response against the body itself that has the potential to cause great harm.

Topic: - �Overview of AIDS in Children�

Author:-
Dr. Niyaz Ahmad Buchh.
Associate Professor (Pediatrics)
SKIMS Medical College,
Bemina, Srinagar.

Address for correspondence:-
Dr. Niyaz Ahmad Buchh,
Children�s Clinic Rainawari,
Srinagar Kashmir 190003.
Mobile:-9419478800
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A).Defining HIV & AIDS:-
HIV: - Humane immunodeficiency virus is a virus that destroys part of the body�s immune system.
AIDS:-Acquired immunodeficiency syndrome is the final stage of the disease caused by HIV.
B).Epidemiology of AIDS:-
1. First case of childhood AIDS was detected in an infant in 1983.
Seropositivity of HIV in blood was noticed in 0.1-1.5% babies (ICMR Report 1988).Whereas same was noted in 8.9-9.3% multitranfused babies of thalasemia, hemophilia etc in a Delhi study 1993.
2. WHO had estimated > 42 cases of AIDS including 4 million in India by 2002.
3. 2.7 million Children are < 15 years of age.
4. 5 million new cases are to be added annually including 0.8 million children.
5. 3 million die including 58,000 children.
6. 10 million children are orphaned and estimated 20 million by 2010.
7. >90% live in developing nations and sub-Saharan areas.
It is worth to highlight pediatric AIDS because it has got unique mode of transmission, diagnostic difficulties, nonspecific clinical features and of course having high mortality because of its rapid progression and most of them die within 1st two years of life due to high viral load and depletion of infected CD4 lymphocytes in infants than adults. I n childhood AIDS usually mother is the source (symptomatic or asymptomatic) and father the cause and the child suffers due to none of his own fault. The disease seems to be disease of whole family.
C).Transmission of AIDS in Children:-
1. Vertical Transmission: Almost 90-100% children <13 years in USA and 74-86% in India acquire AIDS through vertical transmission from their mothers, also called as parent-to-child transmission (PTCT). The infection is transmitted during,
a) Antenatal period during pregnancy through placental circulation (30-40%).Virus has been detected as early as 10 weeks gestation in an aborted fetus by culture and polymerase chain reaction (PCR) within 48 hours.
b) Intrapartum period during delivery through contaminated secretions and blood in birth passage (60-70%).It is detected by culture and PCR within 4-6 weeks.
c) Postnatal period through breast feeding and have increased chances of transmission from HIV +ve mothers by 14%.It is detected by 3-6 months by culture and PCR.Chances of transmission through breast milk are high because early breast milk is moir� cellular, lacks specific HIV Ig A antibodies and rate of transmission is reduced by half on stopping breast milk.
2. Transfusion of blood products in 3-6% cases, which was more common earlier before routine screening of blood and its products was done.
3. Others like syringes and needles etc which is very rare in children.
4. Sexual Abuse:-Very rare in children, however, fast growing cause of transmission in USA in adolescent group (13-19 years).
D).Factors influencing PTCT transmission of AIDS in children:-
1. Recent infection of HIV infection.
2. Severity of HIV infection.
3. Infection with other sexually transmitted disease.
4. Obstretic procedures like vacuum extraction or forcef delivery leading to injury to body.
5. Duration of breast feeding increases risk of transmission by 14-29% if given for first 5 months of life.
6. Exclusive breast feeding or mixed feeding, since chances of intestinal epithelial damage more in mixed feeding, thus transmission of HIV infection more in mixed feeding.
7. Condition of breasts like cracked nipples, breast abscess etc.
8. Condition of baby�s mouth like abrasions, ulcers etc.

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9. Provision of antiretroviral drugs.
10. Advanced maternal age increases chances of transmission.
11. Low CD4 count in mother as well as in baby.
12. High maternal viral load. If mother has <50,000 copies /ml (1.6% fold risk) compared to if >50,000 copies/ml (3.7% fold risk).
13. No antiretroviral therapy given during pregnancy, delivery and breast feeding.
14. Low antiviral antibodies in mother.
15. Preterm and low birth weight babies have 3.7 fold increased risk, if born <34 weeks of pregnancy.
16.1st born of the twin babies has 2 fold increased transmission due to more trauma and exposure to contaminated secretions in birth passage.
17. Use of illicit drugs y mother during pregnancy.
18. Delivery by Caesarian section decreases transmission by 87%, if done along with Zidovudine therapy in both mother and baby as well.
E).Feeding of baby of HIV positive mother:-
It has been observed in a study from developing country that out of 25 babies, relative risk of death due to diarrhea increases if baby is given formula feeds during 1st year of life comparing to one who is exclusively breast feed. The risk increases to 23/25,if given formula feeds during 1st 2 months of life comparing to 1/25,if exclusively breast feed. Same is increased chances of deaths due to respiratory infections in formula feed babis.Because of these complications a policy statement on HIV and infant feeding has been developed collaboratively by UNAIDS,WHO & UNICEF(1997).which says,
�AS a general principle, in all populations, irrespective of HIV infection
rates, breast feeding should continue to be protected, promoted and
supported.�
This principle holds good particularly in developing countries like India where breast feeding as recommended by WHO, should continue despite mother being HIV+ve and chances of transmission being more in breast fed babies but simultaneously the mortality and morbidity being much higher in artificially fed babies in our social set up.
F).AFASS Criteria for replacement feeding for baby of HIV positive mother:-
a) Acceptability: - Will not breast feeding stigmatize and discriminate family/mother?
b).Feasibility:- Does mother/family have adequate time skills, resources and support for correct preparations and feeding.
c).Affordability:-Can family afford purchase, preparation, storage and associated cost of preparation and feeding?
d).Sustainability:-Is continuous uninterrupted and dependable system of distribution of all products for duration of replacement feed available?
e).Safety:-Would replacement feeds be correctly and hygienically stored and prepared and fed in clean cups and pots with clean hands?
G).Breast milk feeding options by HIV+ve mothers:-
1. Exclusive breast feeding for 6 months and continued breast feeding fat least for 2 years.
2. Modified breast feeding by exclusive breast feeding for shorter duration followed by early replacement feeding by home made commercial formulas.
3. Expressed breast milk and heating it by flash method for a longer period before feeding.
4. Breast feeding by HIV-ve mother (donor�s breast milk).
All these options are discussed with the mother and if possible with the family and proper feeding advised keeping in mind various social and economic factors of the family.


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H).Clinical features of childhood AIDS:-
Clinical features are nonspecific and vary from infants to older children, the latter behaving like adults. Infant may be normal at birth, or with lymphadnopathy, hepatosplenomegaly, rash, fever, recurrent diarrhea, oral thrush and chronic parotid swelling etc.
In older children almost all systems are involved, leading to progressive encephalopathy, Pneumonia, cardiomyopathy, malabsorption syndrome, renal involvement, dermatitis, anemia, Lymphoma and various opportunistic infections like tuberclosis, canadidiasis etc.
I).Diagnosis of AIDS:-
a) Clinical features but unfortunately these are nonspecific.
b) Screening of mother.
c) Immunological tests.
1. ELISA:-commonly practiced but less specific. Two successive ELISA with different proteins if +ve is suggestive of AIDS.
2. Western Blot Test:-detects antibodies to various structural proteins of virus like
Envelope:-gP160, 120, 41,
Gag:-P 55,40,24,17,
Pole:-P66, 51, 32.
WHO criteria is 2 out of 3 proteins if +ve is diagnostic, but the test is expensive and nonavailble everywhere.
3. Culture and PCR: - more helpful in perinatal AIDS and 100 % specific if done together in perinatal AIDS.
J).Prevention of childhood AIDS:-
1. Decrease maternal viral load by antiviral therapy.
a). Thai Trial advocates 300 mg of AZT from 36 weeks of pregnancy followed by 300 mg during delivery and stop breast feeding, has decreased transmission by 50%.
b). Ultra short Regimen in Uganda adopts single dose of oral Nevirapin 200 mg to mother at labour and 2 mg/kg to new born within 72 hrs of birth and relative risk is decreased by 42%. 100 countries have started this regimen.
2. Decrease exposure of fetus during delivery by performing elective Caesarian section.
3. Safer infant feeding practices as discussed above.
4. Primary prevention of AIDS by parents by avoiding extramarital sex, performing safe sex, safe syringe precautions and tested blood transfusions etc.
K).Summery of Childhood AIDS:-
a).AIDS is emerging as rapidly one of the major public health problems in India.
b).Approximately 30,000 newborn babies in India suffer due to PTCT.
c).Prevention of PTCT important strategies to decrease pediatric HIV infection.
d).Single dose Nevirapin to mother and baby at labour and within 723 hrs of birth effectively decrease vertical transmission.
e).Diagnosis of HIV very crucial with excellent laboratory back up.
f). Most rapid progression and fulminant course in infants and children.
g).Above all breast feeding to continue even by HIV+ve mothers in developing countries, however other feeding options to be adopted on individual basis.
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