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Dr. Rajesh Jain's Profile
Huge bilateral ovarian cysts in adulthood as the presenting feature of Van Wyk Grumbach syndrome due to chronic uncontrolled juvenile hypothyroidism
Cases of multicystic ovaries with primary hypothyroidism
have been reported in girls as a cause of pseudoprecocious
puberty,[1,2] and in adult females with abdominal pain.[3] The
ovarian cysts are thought to be due to increased ovarian
sensitivity to gonadotropins[4] or more likely an action
of increased circulatory levels of thyroid stimulating
hormone (TSH) (secondary to chronic untreated primary
hypothyroidism) on follicle stimulating hormone (FSH)
receptor (FSHR) causing gonadal stimulation.[5,6] Here
we report a case of juvenile primary hypothyroidism
presenting in adulthood as abdominal mass due to huge
bilateral ovarian cysts.

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Solitary median maxillary central incisor, a clinical predictor of hypoplastic anterior pituitary, ectopic neurohypophysis and growth hormone deficiency
Clinical evaluation of a 5-year-old boy seeking medical
advice for micropenis was detected to have solitary
median maxillary central incisor (SMMCI), short stature[height: 95.2 cm; < 3rd percentile; standard deviation score
(SDS): – 2.57; target height SDS: – 0.63], small face, low set
ears, depressed nasal bridge, prominent forehead and
stretched penile length of 2 cm . He was born
at term of breech delivery with an uneventful perinatal
history. Investigations were significant for delayed bone
age (3.5 years; Greulich-Pyle), low insulin like growth
factor-1 (IGF-1) (41 ng/mL; normal, 50 – 286), GH deficiency
(post clonidine 100 μ g peak growth hormone: 1.2 ng/mL;
normal > 10 ng/mL), hypoplastic anterior pituitary (white
arrow; partial empty sella), stalk agenesis and ectopic
neurohypophysis (EN) located in tuber cinereum (black
arrow) on MRI. Optic nerve, olfactory bulbs,
corpus callosum and septum pellucidum were normal. GH
replacement resulted in an 11 cm height gain in 1 year.

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A Bearded Indian Female: A Rare Presentation of Cushing's Syndrome
A 16-year-old Indian girl presented with increased facial
hair growth, weight gain, amenorrhea and generalized
weakness for the last 3 months. On examination she was
found to have severe hirsutism, her modified Ferriman-
Gallwey score was 24/36, she had broad purple striae on
abdomen, hypertension and proximal myopathy. On
investigations, the patient was found to have ACTH
dependent Cushing's syndrome.

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Mauriac syndrome: A rare complication of type 1 diabetes mellitus
Mauriac syndrome is characterized by dwarfi sm, obesity
and hepatomegaly in patients with insulin-dependent
diabetes mellitus. It is associated with poor control of
type 1 diabetes mellitus (T1DM) in adolescents, and may
present as obesity, hepatomegaly, cushingoid facies and
elevated transaminases.[1] It is typically associated with
growth failure and delayed pubertal maturation, which
should alert the physician over insuffi cient management of
diabetes mellitus and the related development of Mauriac
syndrome, although these can be reversed with good
glycemic control.[2]

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Restoration of sinus rhythm following levothyroxine treatment in a case of primary hypothyroidism presenting with atrial fi brillation and pericardial effusion
A 72-year-old man presented with palpitation, dyspnea, and chest discomfort. Initial investigations revealed atrial fi brillation (AF)
and pericardial effusion, further investigations unraveled primary hypothyroidism (thyroid stimulating hormone) of 34.7 IU/ml and
total thyroxine (T4) of 5.57 g/dl). Treatment with levothyroxine led to resolution of symptoms, AF, and pericardial effusion.

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