Expert group recommendation on inhaled mucoactive drugs in pediatric respiratory diseases: an Indian perspective.

Background: Currently, there are no guidelines or consensus statements about the usage of inhaled mucoactive drugs in pediatric respiratory disease conditions from an Indian perspective. Objective: To develop a practical consensus document to help pediatricians in clinical decision-making when choosing an appropriate mucoactive drug for the management of specific respiratory disease conditions. Methods: A committee of nine experts with significant experience in pediatric respiratory disease conditions and a microbiological expert constituted the panel. An electronic search of the PubMed/MEDLINE, Cochrane Library, Scopus, and Embase databases was undertaken to identify relevant articles. Various combinations of keywords such as inhaled, nebulized, mucoactive, mucolytic, mucokinetic, expectorants, mucoregulators, mucociliary clearance, respiratory disorders, pediatric, cystic fibrosis (CF), non-CF bronchiectasis, acute wheezing, asthma, primary ciliary dyskinesia (PCD), critically ill, mechanical ventilation, tracheomalacia, tracheobronchomalacia, esophageal atresia (EA), tracheoesophageal fistula (TEF), acute bronchiolitis, sputum induction, guideline, and management were used. Twelve questions were drafted for discussion. A roundtable meeting of experts was conducted to arrive at a consensus. The level of evidence and class of recommendation were weighed and graded. Conclusions: Inhaled mucoactive drugs (hypertonic saline, dry powder mannitol, and dornase alfa) can enhance mucociliary clearance in children with CF. Experts opined that hypertonic saline could be beneficial in non-CF bronchiectasis, acute bronchiolitis, and PCD. The current state of evidence is inadequate to support the use of inhaled mucoactive drugs in asthma, acute wheezing, tracheomalacia, tracheobronchomalacia, and EA with TEF.

Pancreatic, hepatobiliary, and gastrointestinal manifestations of children with cystic fibrosis: A 10-year experience from a tertiary care center in southern India.

OBJECTIVES: To describe the demography and spectrum of pancreatic, hepatobiliary, and gastrointestinal (GI) manifestations in children with cystic fibrosis (CF) from the Indian subcontinent. METHODS: In this retrospective study, relevant information from the database of all children with CF below 18 years of age was collected and analyzed. RESULTS: Among the total 109 children, 58 (53%) were from the southern states of India. The most common manifestation was pancreatic insufficiency (PI) in 85 (83%) children. Those with PI presented at an earlier age (1.8 vs. 6.9 years). Cirrhosis with portal hypertension was documented in only one patient and meconium ileus in three (2.8%). There was significant malnutrition in the PI cohort with a mean weight-for-age Z-score of - 3.17 ± 1.79 at diagnosis. Twenty-one (19%) patients had died during the follow-up and 18 (90%) of them had PI. There was no difference in the prevalence of selected pulmonary manifestations in the PI and pancreatic sufficient (PS) groups. Among children with PI, 78 were screened for ΔF508 mutation, 16 (21%) were homozygous, and 17 (22%) were heterozygous. In the PS group, only 2 (14%) were heterozygous for ΔF508 mutation. The median duration of follow-up of the patients was 1.8 (1.5) years. CONCLUSION: PI is the most common GI manifestation of children with CF and is associated with severe malnutrition and poor outcome. Timely identification and management of the comorbidities involving the digestive system are essential for better growth and quality of life in these children.

Effect of malnutrition in infants with cystic fibrosis in India: An underestimated danger.

AIMS: To assess the clinical profile and nutritional status of infants with cystic fibrosis (CF) and track their nutritional outcomes with treatment. MATERIALS AND METHODS: This retrospective study was conducted in a tertiary-care institute in South India. Demographic and clinical information were collected. The nutritional status and treatment outcome was assessed by Z-scores for weight-for-age (WAZ), length-for-age (LAZ), and weight-for-length (WLZ) at diagnosis and follow-up. RESULTS: Nineteen infants with CF had mean follow-up duration of 9.7 ± 8.7 months. There was a mean delay of 2.9 ± 2.1 months from symptom onset to diagnosis, by which time infants were severely malnourished (mean WAZ -4.68 ± 1.8). Pneumonia, summer dehydration with electrolyte abnormalities (42.1%), and a combination of anemia, hypoalbuminemia, and malnutrition (42.1%) were the predominant features. Significant weight loss had been recorded in undiagnosed infants by second month of life before symptom onset. At follow-up, there was a remarkable improvement in WAZ (P 0.001), but not LAZ and WLZ. There was a high mortality rate of 37% in these infants. CONCLUSIONS: Malnutrition is a significant morbidity in infants with CF in India. There was significant improvement of WAZ with treatment, but it lagged behind the recommended targets. There is an opportunity for identification of CF infants at the time of vaccination at six and ten weeks of age, by the primary care physician and pediatrician. Screening of young infants having failure to thrive in the immunization clinic may be a strategy for early diagnosis of infants with severe CF phenotype.

Isolated pulmonary Langerhans cell histiocytosis.

Isolated pulmonary involvement in Langerhans Cell Histiocytosis (LCH) is rare in childhood. The authors report a 6-y- old boy presenting with recurrent pneumothorax, whose CT thorax showed diffuse pulmonary cystic lucencies bilaterally. Biopsy of the lesions confirmed pulmonary LCH with Cd1a and S 100 positivity.

Rapunzel syndrome: a diagnosis overlooked.

UNLABELLED: We report two children who presented with a painless abdominal mass masquerading as a splenic enlargement. The paucity of symptoms resulted in delayed diagnosis. At laparotomy the trichobezoars were found to have a tail extending into the small intestine. CONCLUSION: This condition eludes diagnosis unless sought for particularly in the paediatric age group. Hence we present our experience of two consecutive cases of paediatric Rapunzel syndrome, along with the relevant literature and in retrospect, how an early diagnosis could have been made.

Neurologic deterioration in a child with Wilson's disease on penicillamine therapy.

Penicillamine is the standard therapy for Wilson's disease in children. We report an 8-year-old-girl with liver disease due to Wilson's disease who developed extrapyramidal symptoms following administration of penicillamine. Symptoms resolved within 20 hours of stopping the drug but recurred within 24 hours when gradually increasing small doses were recommenced.

Effect of Vitamin A supplementation on the immune response to measles vaccination.

A randomized controlled trial was conducted in 395 infants aged 9-12 months to determine the effect of Vitamin A supplementation on concurrently administered measles vaccine. Antibody response was measured using the plaque reduction neutralization assay. No statistically significant differences were demonstrated between the immune response in Vitamin A supplemented and unsupplemented children. Unlike some recent studies, we were unable to demonstrate an immune enhancing effect of Vitamin A supplementation. On the contrary, among children who were given Vitamin A, a lower, but statistically non-significant, proportion had protective antibody levels 6 months after vaccination.