To determine the prevalence of high blood pressure (BP) in healthy school Pakistani children and its association with high body mass index (BMI), asymptomatic hematuria and proteinuria, we studied 661 public school children and measured their body weight, height and BP and urine dipstick for hematuria performed on a single occasion. Hypertension (BP >95 th centile) and pre-hypertension (BP >90 th centile) were defined based on the US normative BP tables. Over-weight and obesity were defined according to the World Health Organization (WHO) classification of BMI. The mean age of the children was 14 ± 1.3 years. The mean BMI was 18.5 ± 4.3 kg/m 2 . The majority (81.8%) of the children were found to be normotensive (BP <90 th centile). Pre-hypertension was observed in 15% and hypertension in 3% of the children. Overweight was observed in 7.7% and obesity in 1% of the children. The independent risk factors for hypertension and pre-hypertension were age of the child (RR 1.2 95% CI 1-1.4), gender (RR 2.0 for being female 95% CI 1-4.4), BMI >25 (RR for BMI b/w 25-30 = 2.6, RR for BMI >30 = 4.3), positive urine dipstick for proteinuria (RR = 2.3 95% CI 0.7-7.7) and positive urine dipstick for hematuria (RR 1.0 95% CI 0.2-8.3). Hypertension in children is strongly correlated with obesity, asymptomatic proteinuria and hematuria. Community based screening programs for children should include BP recording, BMI assessment and urine dipsticks analysis and approach high-risk groups for early detection and lifestyle modifications.

A total of 124 patients were enrolled in the study. Seven neonates (5.6%) developed EONS and positive cultures were seen in four neonates (3.2%) with a maternal history of PROM. The organisms identified in cultures were Klebsiella pneumonia, group B streptococcus, Staphylococcus aureus, and Streptococcus species in EONS caused by prolonged PROM. Conclusions Early recognition of risk factors, recognition of clinical conditions with prompt laboratory screening for infection, and early establishment of empirical antibiotic treatment are effective preventive measures. Such approaches would be a secure and efficient strategy, particularly in developing countries.

Dengue fever (DF) is an acute febrile illness that follows a self-limiting course. However, some patients suffer from complications, including myocarditis, due to the involvement of other organs. A child presented at the Aga Khan University Hospital in Karachi, Pakistan, in June 2013 with a high-grade fever, malaise and epigastric pain radiating to the chest. Positive DF antigen and immunoglobulin M assays confirmed the diagnosis of DF. Persistent bradycardia with low blood pressure led to further cardiac investigations which showed a decreased ejection fraction and raised serum cardiac enzymes, indicating myocardial damage. With supportive care and use of inotropes, the spontaneous normalisation of cardiac enzyme levels and ejection fraction was observed. The child was discharged five days after admission. This case highlights the importance of identifying myocarditis in DF patients suffering from cardiac symptoms that are not explained by other potential aetiologies. Awareness, early suspicion and supportive care are essential to ensure favourable outcomes.

A 9 year old girl presented to us with complaints of fever and pain in burn wounds with deteriorating health for one month. According to Lund and Broder's chart, burns spanned the posterior trunk (13%), right arm (1.5%), left arm (1.5%), and buttocks (2.5%). The wounds showed improper healing. She had previously underwent split-thickness skin grafting, using skin harvests from thighs and antimicrobial therapy with vancomycin, fluconazole and colomycin with limited clinical improvement. Analgesia was administered. Blood cultures and tissue cultures from the burns indicated polymicrobial wound infection and sepsis, including methicillin resistant Staphylococcus aureus, Klebsiella pneumoniae and Pseudomonas aeruginosa. Despite broad-spectrum antibiotics, fever persisted and condition deteriorated. Antifungals were also administered with no clinical improvement. Eventually another split-thickness skin grafting was done to provide fresh grafts. In due course, ultraviolet light exposure, of wavelength 32-40 nm/W/cm 2 , was considered for treatment. In prone position, the wounds were exposed to ultraviolet phototherapy 6-8 h daily for 8 days. Eventually, wound healing and sepsis improved. Antibiotics were optimized and high protein diet was started. Eventually the wounds showed fresh margins and visible signs of healing. With remarkable clinical improvement and no further fever spikes, the patient was eventually discharged. She was advised to shower regularly, apply bandages with acetic acid. On her last outpatient follow up, 2 weeks after discharge, she was doing well, with no complaints of pain or fever. Examination of burns showed clean wounds, with clear margins and good graft uptake. She did not require any further grafting or surgical procedures thereafter.

Brown-Vialetto-Van Laere syndrome (BVVLS) or riboflavin transporter deficiency (OMIM 211530) is a rare treatable autosomal recessive neurodegenerative disorder. This condition is associated with progressive pontobulbar palsy. We describe the clinical course of a 16-month-old boy with BVVLS and a novel homozygous mutation from Pakistan. Our patient presented with stridor and respiratory insufficiency. Hearing loss which is the most common sign of this condition was absent, making it an unusual presentation of BVVLS. His examination revealed ptosis and tongue fasciculation. His riboflavin receptor mutational analysis showed the homozygous mutation in the gene. Per oral riboflavin was administered, and subsequently, he was able to be weaned off the ventilator. Now the child is improving and attaining developmental milestones.

Background Tumor lysis syndrome (TLS) is serious complication of anticancer chemotherapy, leading to substantial morbidity and mortality in adults and pediatric patients. Objective To report the incidence and outcomes of TLS in pediatric patients with hematologic malignancies at a center in Pakistan. Methods Retrospective chart review of 317 pediatric patients with hematologic malignancies during January 2008-December 2013. Demographic features and clinical and laboratory parameters of TLS, with immediate and 6-month outcomes were determined using a semi-structured questionnaire. Results Median age at diagnosis was 9 years, with the 79.2% patients being male. Laboratory TLS was present in 36 patients (11.4%), with 27 (8.5%) developing clinical TLS and 13 (4.1%) requiring intensive care support. Hyperphosphatemia was the most frequent metabolic abnormality (14.2%), followed by hypocalcemia (13.9%), hyperuricemia (12.6%), and hyperkalemia (1.3%). 45 patients (14.2%) developed acute kidney injury (AKI). Patients who developed TLS had a signi cantly higher white blood cell count at initiation of chemotherapy (142.0 x 10 9 /L [SD, 173.1] vs 31.5 x 10 9 /L [SD, 58.0]; P = .01) and a higher incidence of AKI (58.3% vs 8.5% of patients; P < .001). Limitations Retrospective design of study, high rate of loss to follow-up, and unavailability of lactate dehydrogenase levels in a majority of patients. Conclusion The incidence of TLS pediatric hematologic malignancies was 11.4% at our center. The main cause of death was sepsis. Hyperphosphatemia was the common metabolic derangement and hyperkalemia was the least common. TLS warrants intensive supportive care to prevent further morbidity and decrease mortality.

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