Encephalocraniocutaneous lipomatosis (ECCL) is a rare sporadic neurocutaneous syndrome characterized by presence of central nervous system, ocular and cutaneous anomalies. The exact pathogenesis is still not known. We present the third case from the Indian subcontinent, who is a five year old girl with history of right sided seizures. Dermatological examination showed alopecia on right side of the scalp and ipsilateral limbal dermoid and nodular skin tags over the upper eyelid. The computerized tomography scan of the brain revealed porencephalic cyst, cerebral calcifications and atrophy of right brain. The histopathology of the skin lesions showed lipomatous hamartoma and features of non scarring alopecia. The constellation of these findings and in adherence to the diagnostic criteria of ECCL proposed in 2009, we consider this report as a definite case of ECCL.
Hepatitis A virus (HAV) is a benign self-limiting gastro intestinal infection of the children belonging to developing countries. Haematological complications like immune thrombocytopenic purpura (ITP) is rarely reported as a manifestation of acute hepatis A infection. We report a 6 year old female child with bleeding manifestations diagnosed to have ITP associated with acute hepatitis A infection.
Background & Objectives: To compare serum zinc levels in children suffering from acute gastroenteritis, pneumonia (malnourished and normal) with a control group. To study the effects of zinc in reducing the frequency and duration of diarrhea and pneumonia & its impact on the further recurrence of these infections. Methods: One hundred thirty (130) children in the age group of 6 months to 5 years were included. The Blood samples were drawn for zinc assay from all children at initial presentation and supplement with adjuvant oral zinc for 14 days. The duration of hospital stay and symptom resolution and the effect of supplementation over the next six months on these children was studied. Results: The mean serum zinc levels were significantly lower in children with AGE and Pneumonia as compared to that of the children in the control group which was very highly significant (71.39, 69.45, 81.65 mcg/dl respectively). Also, the malnourished children in both the AGE & LRTI groups had a mean zinc level that was significantly lesser than the adequately nourished children (67.77:73.85 in the AGE group & 57.52: 75.62 in the LRTI group). In the follow-up period for six months, children with undernourishment and those who had low zinc levels at initial presentation had significantly more recurrences Conclusions: Children with AGE and LRTI have a reduced serum zinc level when compared to controls. Zinc supplementation reduces the duration of symptoms in both AGE and LRTI children with low zinc levels and who were undernourished had frequent recurrences.
Short stature due to Growth hormone deficiency could be due to multiple etiologies. One such rare cause is the Pituitary stalk transection syndrome which is characterized by a triad of thin or absent pituitary stalk, aplasia or hypoplasia of the anterior pituitary and absent or ectopic posterior pituitary seen on magnetic resonance imaging (MRI). The early identification of growth hormone deficiency through growth hormone stimulation tests, evaluation of the hypothalamicpituitary anatomy by performing MRI brain and the early initiation of growth hormone replacement therapy may salvage the child from pathological short stature.
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