Journal of the Pediatrics Association of India

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Thalassemia patients require multiple transfusions. Iron overload and hepatitis C are known complications due to transfusions. Iron overload is treated with iron chelators. Hepatitis C treatment is rapidly evolving in children. This review discusses the management of these patients in light of newly available direct-acting antiviral agents. Early diagnosis and treatment remains the cornerstone in the management of this triple disease.

Nail is rightly regarded as a mirror to systemic diseases. Nails serve as a valuable diagnostic tool as it is very easy to examine all 20 of them quickly. A careful examination of nails may give vital clues to underlying sinister diseases evidenced by alteration of their shape, size, color, or texture. This review may help pediatricians to suspect an underlying serious systemic disease just by a careful examination of nails.

Introduction: Gallstone disease is being increasingly diagnosed as a cause of pediatric pain abdomen, requiring cholecystectomy. Most cases are idiopathic, while uncommon etiologic associations nowadays include obesity and hemolytic anemias/hemoglobinopathies; prolonged disease and high fat diet are predisposing factors. Management aspect is similar to adults. Aim: This study aims to compare the profile, interventions, and outcomes of pediatric gallstone disease patients, having undergone cholecystectomy, between two surgeons working at separate centers in India. Materials and Methods: Case records of all pediatric patients (≤15 years' age) having undergone cholecystectomy at both the centers by the respective surgeons (AP = 43 and KA = 17; total = 60 cases), between August 2015 and November 2019, were examined. Data were tabulated and inferences were drawn. Results: Male patients outnumbered female patients in both the studies (AP – M: F = 24:19, KA – M: F = 12:5). Anemia/hypoproteinemia and obesity were prevalent among the operated children (AP = 6 and KA = 3). Biliary pancreatitis (AP = 2 and KA = 2)/choledocholithiasis (AP = 7 and KA = 3) was the uncommon presentation; common bile duct obstruction with cholangitis/sepsis was rarer still. Laparoscopic cholecystectomy was the commonly performed procedure (AP lap: open = 41:2 and KA lap: open = 10:7). Choledocholithiasis patients underwent prior endoscopic retrograde cholangio-pancreatography (AP = 7 and KA = 3). Most patients had cholesterol/mixed gallstones. Complications were minimal, comparable with adult procedures. Conclusion: Pediatric gallstone disease is understated in literature and underestimated in clinical practice. Early diagnosis and timely referral to a surgeon for appropriate treatment can help prevent complications and reduce time–work–loss to child/parent.

Introduction: Immunization coverage rate is the percentage of the target population that has received the last recommended dose for each vaccine recommended in the national schedule and is one of the best indicators of public health outcome and service and vaccines are important tools in preventing infectious diseases. Aim and Objective: The aim and objective were to assess the immunization compliance and adverse event following immunization (AEFI). Methodology: The present retroprospective observational study was carried out by collecting response of prevalidated immunization compliance and adverse event questionnaire from parents who brought their children for vaccination up to the age of 5 years. This survey asked about immunization compliance and AEFI. Results: A total of 188 randomly selected parents were interviewed about their child's immunization compliance and AEFI. Out of the selected parents, 86.17% agreed for the necessity of vaccines against vaccine preventable disease (VPD) and 13.83% were unaware of the necessity of vaccination. Knowledge about severity of VPDs was known to only 56.38% of parents and 13.82% of parents did not have knowledge of severity or harmfulness of VPDs. Approximately 71.8% of parents were good compliant, while 28.2% were some compliant with immunization schedule during the 6 months of the study period. Discussion: Missed opportunities resulted in subsequent late immunization. The most common errors occurring in this sample were missed opportunities for Bacillus Calmette–Guérin (BCG) vaccination, as most of the children did not get BCG vaccination at birth and superfluous administration of oral polio vaccine observed due to the National Pulse Polio Immunization Program. A consequence of not adhering to the recommended childhood immunization schedule was that doses were given too late. Conclusion: Efforts to improve compliance with immunization schedule are recommended to optimize VPDs.

Neurocutaneous syndromes (NCSs) are a group of genetic disorders characterized by developmental lesions of the neuroectoderm. We present five cases of NCSs, all of which were associated with seizures presenting in the pediatric age group with classical skin lesions.

Epidermolysis bullosa (EB) is a congenital blistering disorder, classified into three types based on the level of split in the skin as simplex, junctional and dystrophic. Here, the authors reports a mother who had 13 weeks of amenorrhea and brought an 8-month-old male infant presenting with multiple blisters and erosion all over the body, associated with nail changes since birth. On further investigations, histopathological data showed the changes of EB along with Sanger sequencing and next-generation sequencing (NGS) technique showing mutation in COL7A1 gene which is confirmatory for EB. Based on clinical, histopathological, and molecular genetics data, the diagnosis of dystrophic EB with less severe mitis variant was made. Screening for the unborn child showed similar mutation, and parents were counseled about the termination of pregnancy but due to social restrains, they refused and an affected child was born with similar features as that of index case.

Background: Neonatal septicemia, also called neonatal sepsis, encompasses various systemic infections of the newborn such as septicemia, meningitis, pneumonia, arthritis, osteomyelitis, and urinary tract infection. Neonatal sepsis is one of the important causes of neonatal mortality, worldwide. It is estimated that 33% of the neonatal deaths are due to sepsis-related causes. As Vitamin D has a role in strengthening immunity and hence controlling infection, it would be pertinent to study its role in the prevention of neonatal sepsis. Deficiency in Vitamin D contributes to this deficit in immune system. Methods: This was a pilot randomized, double-blind, placebo-controlled trial, enrolling 112 antenatal mothers at the beginning of the third trimester of pregnancy. The blood sample for Vitamin D level was collected from mothers at the time of enrollment. Study group received sachet containing 1.2 lac units of oral Vitamin D once a month for three doses. Control group received glucose powder only, once a month for three doses. Allocation was done using opaque envelope method. At the time of delivery, umbilical cord blood sample for Vitamin D levels was collected. Those clinically symptomatic at birth or within 72 h of birth were admitted in the neonatal intensive care unit and evaluated. All infants were followed up in the neonatal follow-up clinic on a weekly basis till 1 month of age. Results: In the babies born to supplemented mothers, 1% of the newborns were Vitamin D deficient, 1% were insufficient, and 48% had sufficient Vitamin D as compared to the unsupplemented group where 4% were deficient, 16% were insufficient, and 30% had sufficient Vitamin D levels (P < 0.001). 96% of the neonates had sufficient Vitamin D levels in the supplemented group compared to only 60% in the unsupplemented group (P < 0.001). Vitamin D supplementation of mothers ensured adequate levels in 96% of their infants compared to 60% in the unsupplemented group. There were three preterm deliveries and two cases of probable early-onset sepsis (EOS) in unsupplemented mothers. There was one case of probable late-onset sepsis in newborn in both groups. All four neonates with sepsis had Vitamin D levels in the deficient or insufficient range. Cord blood levels were deficient in five neonates and one of them (20%) developed EOS; cord blood levels were insufficient in 17 neonates and one (5.88%) developed EOS. None of the newborns in the sufficient range developed EOS. EOS was significantly higher in Vitamin D-deficient/insufficient group compared to that in Vitamin D-sufficient group (P < 0.003).

Online since 30^thJuly 2020