Objective: To assess and compare the diagnostic accuracy of the Pediatric Risk of Mortality (PRISM) III score and Pediatric Sequential Organ Failure Assessment (p-SOFA) for the prediction of mortality in critically ill children.Methodology: This was a cross-validation study conducted at the Pediatric Intensive Care Unit (PICU) of the National Institute of Child Health Karachi from February 2021 to July 2021. Two hundred eighty-six critically ill children of age one month to 15 years of either gender staying in PICU for more than 24 hours were included. Within 24 hours of admission, the p-SOFA and PRISM III 24 scores were calculated for all eligible children. The outcome of the study was mortality within 30 days of PICU admitted children. Data were analyzed using Statistical Package for the Social Sciences (SPSS) version 23.Results: The median age was 24 months (range: 1-144 months). The 30-day mortality was estimated as 57%. The p-SOFA and PRISM scores were significantly greater in children who did not survive than survivors. The maximum p-SOFA score (area under the curve (AUC)=0.81, 95% CI=0.76-0.86, p=0.001) and PRISM III 24 score (AUC=0.75, 95% CI=0.69-0.81, p=0.001) had good discrimination for 30-day mortality. For the prediction of 30-day mortality at the cut-off value of p-SOFA>2, the sensitivity was 93.87%, specificity was 38.21%, and accuracy was 69.93%. Whereas at the cut-off value of PRISM III 24 score>8, the sensitivity was 55.83%, specificity was 77.24%, and accuracy was 65.03%. Conclusion:The p-SOFA score is a good predictor for 30-day mortality in critically ill children and had better accuracy than the PRISM III 24 score.

Congenital Adrenal Hypoplasia (AHC) first described in 1948, characterized by adrenal insufficiency, and hypo-gonadotropic hypogonadism. X linked AHC mainly affects males, it occur mainly due to mutation in NR0B1 gene. In this report, we present two cases of NR0B1 mutation from same family with dichotomous pubertal presentation. An infant diagnosed and being managed as case of adrenal insufficiency, developed precocious puberty at the age of 11 months. His work-up showed increased LH, FSH and Testosterone. Genetic Analysis revealed NR0B1 mutation. He was managed with Gn RH agonist and steroid replacement. His brother 15 years old, treated as primary adrenal insufficiency, presented with arrested puberty, his genetic analysis also showed NR0B1 mutation. Keywords: AHC; adrenal hypoplasia; precocious puberty; hypogonadotropic; hypogonadism.

We report a case of 6 years old girl with ovarian Steroid Cell Tumor, Not Otherwise Specific (SCT-NOS). She was admitted to the hospital with increased weight gain, hypertrichosis, and cushingoid facies in form of prominent cheecks, moon facies with dorsocervical fat pad, obese built, acanthosis nigricans on back of neck and marked hirsutism. Initial workup showed raised level if cortisol with loss of diurnal rhythm, and undetectable Plasma ACTH levels, increased testosterone levels. Ultrasound revealed left sided adnexal mass having no flow inside, which was later confirmed on CT as neoplastic lesion of left ovary measuring 6.9 X 8.1 X 4.9 cm. The patient underwent left oophorectomy and pathological examination showed steroid cell tumour, limited to ovary and negative for malignancy. She was kept on IV hydrocortisone preop and post operatively and antihypertensives. After tumor resection her testosterone and cortisol returned to normal levels. Keywords: steroid cell tumor; not otherwise specific; cushing syndrome; ovarian mass.