Clinical Profile and Outcome of Clinical BCG Disease in Infants.

Objective: To describe the clinical profile, immunological status and outcome of BCG disease in infants. Methods: All infants with a diagnosis of BCG disease in a period of 17 months were followed up. Results: Among 25 infants with BCG disease; 19 had local/ regional involvement and 6 had suspected or confirmed distant/disseminated disease, Mean (range) age of presentation was 3.6 (1.5-9) months. Two of 6 infants with disseminated disease required second-line anti-tubercular treatment. One infant with confirmed disseminated disease had INFγ R1 receptor deficiency. There was no mortality. Conclusion: Most infants with BCG- related disease have local or regional disease.

Severe lipoid pneumonia following aspiration of machine oil: Successful treatment with steroids.

Lipoid pneumonia in children follows mineral oil aspiration and may result in acute respiratory failure. Majority of the patients recover without long-term morbidity, though a few may be left with residual damage to the lungs. We report a case of a two-and-a-half-year-old child with persistent lipoid pneumonia following accidental inhalation of machine oil, who was successfully treated with steroids.

Juvenile paget?s disease.

Juvenile Pagets disease (JPD), a rare genetic disorder characterized by markedly accelerated bone turnover, presents in early childhood. We report a child with typical features of JPD who remained undiagnosed till 15 years of age. Rarity of this disease in Indian literature and need for early diagnosis to prevent progression of disease prompted us to report this case.

Primary hypothyroidism with precocious puberty and bilateral cystic ovaries.

It is a general observation that children with hypothyroidism have delayed sexual maturation and delayed puberty. However, longstanding untreated hypothyroidism could be associated with isosexual precocious puberty. Here we report a nine year old girl with longstanding untreated hypothyroidism presenting with precocious puberty and bilateral multicystic ovaries, which promptly responded to thyroid hormone replacement.

Do the length-based (Broselow) Tape, APLS, Argall and Nelson's formulae accurately estimate weight of Indian children?

This study was aimed at validating the usefulness of a length based pediatric emergency tape (Broselow) in an Indian population. The secondary objective was to validate age based weight estimation formulae (Nelson, Argalls, APLS) for emergency needs (doses, sizes). This cross sectional study was done at a tertiary teaching hospital on a sample of 500 children attending outpatient clinic. Inclusion criteria was age between 1 month to 12 years. Children who were unstable, uncooperative or critically ill requiring emergency care and those measuring more than 145 cm in length or weighing more than 35 kg weight were excluded from the study. Measurement of actual weights, calculation of weight, adrenaline dose, fluid bolus and endotracheal tube size was done by all four methods. Results indicated good positive correlation between actual measured weights and weights estimated using Broselow Tape (r = 0.974), APLS (r = 0.902), Argalls modification (r = 0.902), and combined Nelson formulae (0.935). However, specific Nelson formulas for 7-12 yr and 3-12 mo were especially poor in correlation. Bland-Altman Plots comparing actual weight showed least mean bias for Broselow Tape estimations in < 15 kg group (0.080 +/- 0.96 kg) and maximum bias with Nelsons formula for 7 to12 yr (5.204 +/- 4.272 kg). For adrenaline doses and fluid bolus calculations, Broselow estimations were valid estimates. Broselow tape did underestimate endotracheal tube size (mean bias -0.53 +/- 0.18). To conclude, length based pediatric emergency tape (Broselow) correlates well with overall emergency decision making process in our setting. This is especially validated in the age group 0.1 to 6.7 yr weighing less than 15 kg.

Congenital Nephrotic Syndrome with adrenal calcification and cardiac malformation.

Congenital Nephrotic Syndrome (CNS) with adrenal calcification and CNS with congenital heart disease (CHD) have rarely been reported. However, CNS with both these rare associations has never been previously reported. Here we report a case of CNS with both rare associations, perhaps the first report from India to the best of our knowledge.