Congenital lobar emphysema: a rare cause of respiratory distress in a neonate

Congenital lobar emphysema (CLE) is a congenital condition characterized by distension and air trapping of the affected lobe of the lung. It is one of the causes of infantile respiratory distress, which may require surgical resection of affected lobe. Case characteristics: 3-day-old neonate with ventilation refractory respiratory distress. Imaging was suggestive of decreased lung tissue on the right side with ipsilateral mediastinal shift. Intervention/ outcome: Early surgical lobectomy was done to improve lung functions and the child improved dur to early intervention. Message: An early diagnosis with high index of suspicion helps patients with this rare congenital anomaly. Early intervention is the key to good long-term outcome. More awareness about the entity and treatment options available would greatly help improving the outcome and disease burden.

Pediatric hospitalizations associated with 2009 pandemic influenza A (H1N1): An experience from a tertiary care center in north India.

Objectives To describe our experience in children hospitalized with the pandemic Influenza A (H1N1) from Northern India. Methods The retrospective case study was conducted at the Pediatric ward and Pediatric Intensive Care Unit (PICU) dedicated to the children (aged 18 years or younger) with influenza-like illness (ILI) with positive laboratory test results for pandemic H1N1 by reverse-transcriptase polymerasechain- reaction assay. Results Between August 2009 and January 2010, a total of 100 children were hospitalized with suspected 2009 H1N1 influenza with Category “C” as described by the Government of India. Twenty five patients were positive for H1N1 and 9 for seasonal influenza A. The most common presentation (H1N1 positive) was with fever (100%), cough (100%), coryza (52%), respiratory distress (88%), vomiting (28%) and diarrhea (16%). One child presented with hypernatremic dehydration and seizures (Serum sodium 174 meq/l). Of the H1N1 positive hospitalized children, 7 (28%) had respiratory failure and required PICU admission, 4 (16%) required mechanical ventilation, and 3 (12%) died. The major radiological findings were bilateral pulmonary infiltrates and consolidation. All patients were treated with oral Oseltamivir suspension or capsule as per appropriate weigh band and supportive care as required. Two deaths were caused by refractory hypoxemia and one by refractory shock. Conclusions The exact incidence of Pandemic 2009 H1N1 influenza on morbidity and mortality is difficult to calculate since only Category “C” patients were screened.

Lipodystrophy and metabolic complications of highly active antiretroviral therapy.

Objective. To assess the metabolic drug toxicities of first-line, World Health Organization (WHO)-recommended generic highly active antiretroviral therapy (HAART) regimens, to estimate the prevalence of body fat redistribution and to identify associated risk factors. Methods. Cross- sectional observational study. During 3 month period, 52 HIV infected children (25 on HAART; 27 not on HAART) were assessed. Their sociodemographic, clinical, and immunological data was recorded. Children were examined or the signs of fat redistribution (peripheral lipoatrophy and central lipohypertrophy). Liver function tests, fasting blood sugar, lipid profile, serum amylase, serum lactate, blood pH and bicarbonate levels were done in all patients. Results. Twenty-two patients were on stavudine and three on zidovudine based HAART. None of the patients ever received any protease inhibitor. There were no cases of clinical or immunological failure. Children on HAART had significantly lower weight for age and body mass index but the mean height for age was similar between study groups. Only two cases of peripheral lipoatrophy were observed. Hypercholesterolemia was observed in four children on HAART but none without therapy. Hypertriglyceridemia was observed in three children on HAART and seven without therapy. Four cases of asymptomatic mild hyperlactatemia were observed. No case of any hyperglycemia or liver impairment was observed. Conclusion. Metabolic abnormalities and lipodystrophy are emerging complications of HAART in Indian children and needs very close follow up. Future studies with larger sample size and longitudinal model are recommended.

Cystic lymphangiomatous hamartoma masquerading as massive ascites.

We report a 4-year-old boy presenting with a tense massive ascites and large hydrocele. History and physical examination were unremarkable. Routine laboratory studies were normal. Abdominal ultrasonography revealed massive ascites. Contrast CT was suggestive of a large cyst covering the entire peritoneal cavity. At laparotomy, a large cystic tumor was found extending into the scrotum through the left inguinal ring. Histopathologic examination diagnosed the tumor as a cystic lymphangiomatous hemartoma. Although abdominal lymphangiomas are seen in children, but presenting as massive ascites with hydrocele is very rare.

Caspofungin.

Caspofungin is a new antifungal drug meant for intravenous use. It has been shown to be comparable to other antifungal agents such as amphotericin B and fluconazole for empirical therapy in febrile neutropenic patients, oropharyngeal/esophageal candidiasis and invasive aspergillosis. Its efficacy has also been documented in children in small uncontrolled trials. The biggest assets of caspofungin are its excellent tolerability/safety profile and minimal drug interactions.

Deferasirox: the new oral iron chelator.

Deferasirox is a new tridentate oral iron chelator developed by computer remodeling recently approved by FDA for children above 2 years. Phase II/III trials have demonstrated similar efficacy to desferrioxamine and better chelation efficiency. Adverse events were minor and growth remained unaffected. Data on cardiac iron chelation is limited although some studies have shown it comparable to deferiprone. The benefit to risk profile of deferasirox is favorable. This promising new drug might decrease the burden of subcutaneous or intravenous infusion improving compliance and hence the life expectation in thalassemic patients.

Insulin therapy.

Optimal glycemic control in type 1 diabetes mellitus (T1DM) requires Intensive Insulin Therapy. Implementation of intensive therapy should be early and prolonged as suggested by the results of Diabetes control and complications trial and Epidemiology of Diabetes Interventions and Complications (EDIC) study. Proper implementation of intensive therapy requires a course teaching flexible intensive insulin treatment combining dietary freedom and insulin adjustment as shown by the Dose adjustment for normal eating (DAFNE) randomized controlled trial. Pen injectors appear to be feasible for routine use although pumps may be required in special situations. Various types of insulin are available in the market, including newer analogs (Iispro, aspart, glargine). Although insulin analogs seem to be more physiological, controlled studies suggested either similar efficacy to regular insulin or only a minor benefit in favor of insulin analogs. The primary concern in developing countries like India is the cost-benefit ratio of short acting insulin analogs in the treatment of diabetic children but this still remains unclear. It would be premature to recommend switching patients to newer analogs especially those who are well controlled, especially when the long-term data is still awaited. The choice of post-meal short acting insulin in toddlers may be decided by the care provider if deemed appropriate. Noninvasive insulin deliveries are now in development. It does appear that the most clinically viable non-invasive system to date may be pulmonary delivery.

Peripheral gangrene: an uncommon manifestation of disseminated tuberculosis.

Peripheral gangrene as a manifestation of tuberculosis is very uncommon. An 8 year old male child presented with non-healing ulcer over the sole of left foot, left inguinal tubercular lymphadenopathy with scrofloderma, tubercular pleural effusion and gangrene of the right fore foot. The child improved on antitubercular treatment.