Noncompliance to gluten-free diet was noted in 24 % of children with celiac disease.

Data on the clinical profile, echocardiographic findings, and outcome of acute fulminant myocarditis (AFM) in children from resource limited countries are limited. To study the clinical profile and short-term outcomes of children aged 2 months to 17 years with AFM managed with only supportive care. We enrolled all children admitted with AFM in our hospital from January 2009 to October 2010. Although the information on patients admitted from January 2009 to March 2010 were retrieved from the case records, data of children admitted from April 2010 were recorded prospectively. AFM was diagnosed based on clinical and echocardiographic criteria. We collected information regarding clinical course, treatment details, and echocardiography findings using a structured performa. All of the children, including those for whom baseline information was collected from the records, were followed-up prospectively to determine short-term outcomes. A total of 10 children, of whom 6 were male, presented with AFM. Their median age was 7.5 (interquartile range [IQR] 2 to 13) years, and the mean left-ventricular ejection fraction (LVEF) was 26% (SD 11.5). Of the 10 children, 9 were discharged, and 1 child died. At discharge, all children showed improvement in the symptoms, but only 4 had improvement in LV function on echocardiography. Factors associated with poor recovery of LV function at discharge were anasarca, low LVEF, and increased serum glutamate pyruvate transaminase levels at admission. One child had died at 2-month follow-up, and another child developed dilated cardiomyopathy at 15 months after discharge. Children with AFM had good immediate- and short-term outcomes even without the use of mechanical assist devices. Decreased LVEF at admission was found to be one of the most important determinants of poor immediate outcomes in these children.

No abstract

Hepatobiliary dysfunction is common in Gram-negative neonatal septicaemia. The onset of abnormalities is early in most cases but ultimately resolve within 2 to 3 months after sepsis. The presence of conjugated hyperbilirubinemia in neonatal sepsis may carry a better prognosis in terms of survival and has no significant effect on growth during early infancy.

No abstract

The involved faculty and residents had high satisfaction levels with mini-CEX evaluation. Mini-CEX has a potential to be incorporated in the formative evaluation of postgraduate pediatric students as part of the workplace-based assessment.

Inflammatory myofibroblastic tumor (IMT) is a rare entity of neoplastic origin. It usually occurs in children and adolescents and most commonly involves pulmonary and gastrointestinal sites. Here, the authors present two cases; one is the nine months old boy with a subcutaneous IMT in the left temporal region that was treated successfully with surgical resection. To the best of our knowledge, this is the first reported case of a subcutaneous IMT in this particular region. The second is an eight years old girl with an IMT of the right hemi-pelvis. The mass had complete surgical excision with clear margins and no residual disease. She was kept on regular follow-up with ultrasound abdomen. However, her disease relapsed with the appearance of lesions in right iliac fossa, right ovary, and liver. Biopsy of the relapsed abdominal mass confirmed ALK-positive IMT. She was treated with ALK inhibitor Crizotinib. She was monitored with regular blood complete picture, hepatic and renal function test, and ultrasound abdomen. Her lesions started regressing within one month, and she achieved complete remission after 6 months of treatment.

Background: In the developed world, 5-years survival of childhood acute myeloid leukaemia (AML) has improved to 70%. However, the survival rates in the developing world are below 40%. The main contributing factors to these reduced survival rates are a late presentation, malnutrition and high treatment-related mortality. Aim: To document the factors affecting treatment outcome of childhood AML at a tertiary care facility of Pakistan. Methods and Results: All newly registered cases of AML under 18 years of age from January 1, 2012 onwards who completed their treatment before November 30, 2019 were included. Data of 219 cases of AML containing 140 (63.9%) males and 79 (36.1%) females was analyzed. The mean age was 6.30 ± 3.66 years. Pallor was the commonest presenting features in 180 (82.2%) and M2 was the commonest French American-British (FAB) subtype in 103 (47.0%) cases. In univariate analysis, high white blood cells (WBC) count at presentation (P = .006), poor nutritional status (P = .005), unfavourable cytogenetics (P = .019), certain types of FAB AML subtype (P = .005), and use of etoposide in induction chemotherapy (P = .042) significantly adversely affected overall survival (OS). Neutropenic sepsis and bleeding were the major causes of treatment-related mortality. Response to induction chemotherapy was the most significant prognostic factor in the multivariate analysis (P = <.001). After a median follow-up of 40.96 ± 26.23 months, 5-year OS and DFS of the cohort were 40.6% and 38.3% respectively. Conclusions: In this largest cohort of childhood AML from Pakistan, high WBC count at presentation, malnutrition, unfavourable cytogenetics and use of etoposide during induction chemotherapy were associated with decreased OS and DFS rates. Response to the induction chemotherapy was the most significant prognostic factor.