Different presentation of autoimmune hepatitis in children: Case series.

Autoimmune hepatitis (AIH) is a disorder causing chronic hepatic inflammation. Its clinical presentation is highly variable from the affected child might have only biochemical evidence of liver dysfunction or present in hepatic failure. It is important to distinguish AIH from other forms of chronic hepatitis because a high percentage of cases respond to immunosuppressive therapy. In this article, we describe the clinical presentation, biochemical and histological findings, treatment, and clinical outcome of the four children with AIH in a tertiary care center in Mumbai. Most patients with AIH have high levels of immunoglobulin. All four cases showed high serum Ig G levels and responded to oral prednisolone only. AIH should be excluded for all patients with symptoms or signs of prolonged, relapsing, or severe liver disease so that treatment can be promptly initiated and morbidity can be reduced.

Effectiveness of Supramaximal Angiotensin-converting Enzyme Inhibition in Controlling Proteinuria in Congenital Nephrotic Syndrome with Cytomegalovirus Infection and an NPHS1 Mutation.

Congenital nephrotic syndrome (NS) is characterized by early-onset heavy proteinuria. Most cases of congenital NS are associated with genetic mutations in the podocyte proteins. The causal relationship of perinatal infections with congenital NS has not yet been proven. Inadequate response to the treatment of such infections should prompt us to conduct genetic testing for congenital NS. The heavy proteinuria associated with congenital NS is usually difficult to control with conventional treatment. It often results in progressive kidney disease with a high risk of mortality in early life. Here, we describe an infant who developed congenital NS and was found to have a coexisting Cytomegalovirus infection and an underlying NPSH1 mutation. Proteinuria did not respond to a standard dose of enalapril. A supramaximal dose of enalapril was tried and was effective and safe in controlling the proteinuria. It was associated with improved growth, complete resolution of edema, normal serum albumin, and normal renal function beyond 2 years of age.

A study to assess the impact of medical nutrition therapy compared to standard nutrition therapy in children with severe thinness in the age group of 5 to 10 years.

Aim: The aim of our study was to study and compare the impact and efficacy of medical nutrition therapy (MNT) with that of the standard nutrition therapy(SNT) in children diagnosed with Severe thiness in the age group of 5-10 years and diagnosed as severely thin. Study Design: It was a prospective comparative study, conducted over a period of 18 months at the Nutrition Rehabilitation, Research and Training Centre (NRRTC) associated with a tertiary care hospital in India. Methods and Materials: A total of 113 children in the age group of 5 to 10 years with severe thinness were enrolled and divided into 2 groups-58 were placed in the MNT group and 55 in the SNT group for a period of 8 weeks. The children in both groups received MNT and SNT diet over the said period of 8 weeks. Primary outcome variables were weight gain and body mass index (BMI) (i.e., change in nutritional status). Results: At the end of 8 weeks, the rate of weight gain (gm/kg/day) was significantly greater in the MNT group, that is, 2.35 gm/kg/day as compared with the SNT group, that is, 0.73 gm/kg/day, and the change in nutritional status was significantly better in the MNT group (p < 0.001) with 62.1% achieving normal nutritional status, 32.8% remaining thin, and 5.2% remaining severely thin as compared to the SNT group in which 9.1% achieved normal nutritional status, 65.5% remained thin, and 25.5% remained severely thin. Conclusion: To conclude, MNT is superior to SNT for the treatment of severe thinness (ST).

Surgical Management of an Infant with Nonresolving Pneumonia.

We describe a 3-month-old baby who presented with a nonresolving pneumonia which failed to respond to antibiotic therapy. An underlying congenital pulmonary adenomatous malformation was suspected. On thoracotomy, she was found to have a tuberculous mediastinal abscess which was drained. Mediastinal abscess is a rare occurrence in childhood tuberculosis.

One-Stage Bilateral Lobectomy in an Infant with Bilateral Congenital Lobar Emphysema.

We report a 4-month-old baby presenting with bilateral congenital lobar emphysema. A two-staged bilateral lobectomy was planned, but bilateral lobectomy had to be performed as a single-staged procedure. Data are scarce on the appropriate approach to children with bilateral involvement. Both single-staged and two-staged procedures have shown variable success.

A Rare Case of Salmonella meningitis and Hemophagocytic Lymphohistiocytosis.

Enteric fever is a common infectious disease of the tropical world. Common age group involved is children aged between 5 and 10 years. In addition to diarrhea, it may lead to extraintestinal infections including aseptic meningitis, hepatitis, cholecystitis, acute abdomen, intestinal perforation, pneumonia, psychosis, and ataxia. Hematologic complications leading to hemophagocytosis have a prevalence of < 1%. Salmonella meningitis has an incidence of 6% with poor prognosis neurological sequelae. We report a rare case of enteric fever that presented with hemophagocytic syndrome and S. meningitis. Response to third-generation cephalosporins is dramatic, eventually giving good prognosis.

Clinical Characteristics, Management, and Short-Term Outcome of Neonates Born to Mothers with COVID-19 in a Tertiary Care Hospital in India.

INTRODUCTION: We describe the clinical characteristics, management, and short-term outcomes of SARS-CoV-2 neonates born to mothers with COVID-19 in a tertiary care hospital in Mumbai, India. METHODS: The study is a retrospective analysis of 524 neonates born to mothers with COVID-19 admitted from 14th April 2020 to 31st July 2020. RESULTS: SARS-CoV-2 infection was detected in 6.3% of the newborns of the mothers with COVID-19. No significant differences were observed between maturity at gestation, birth weight and sex of SARS-CoV-2 infected and noninfected newborns. The risk of sepsis was 4.09 [95% confidence interval (95% CI) 1.28-13.00] fold higher in the neonates with SARS-CoV-2 as compared to the noninfected group (p = 0.031). Poor feeding was significantly more common among SARS-CoV-2 infected neonates (12.1%) as compared to the noninfected neonates (2.7%) (p = 0.017). There was a total of 13 neonatal deaths, of which 3 deaths occurred in SARS-CoV-2 infected neonates (9%) while 10 (2.04%) in the SAR-CoV-2 negative group. The risk of neonatal death was higher in SARS-CoV-2 infected newborns [odds ratio (OR) 4.8; 95% CI 1.25-18.36]. CONCLUSION: Neonatal SARS-CoV-2 infection is observed in almost 6% of neonates born to mothers with perinatal COVID-19. There is a higher risk of adverse outcomes such as neonatal sepsis and death in the SARS-CoV-2 infected as compared to the noninfected neonates.

The current pandemic of COVID-19 has affected all the countries globally. However, the adverse impact of the pandemic is more seen in the low-income and middle-income countries (LMICs). Although there is evidence on the adverse impact of the SARS-CoV-2 on the health of mothers and neonates, the evidence is mainly from high-income countries. For reducing the mortality and morbidity due to COVID-19 in LMICs, there is a need to generate evidence from the LMICs. The present study is a part of the National Registry of pregnant women with COVID-19 in India (PregCovid registry). Our study demonstrates a higher risk of adverse outcomes such as neonatal sepsis and death in the SARS-CoV-2 infected as compared to the noninfected neonates. The study also showed the risk of SARS-CoV-2 infection in 6.3% of neonates born to mothers with COVID-19.

Psychogenic Belching: A Case Report.

Conversion symptoms usually appear with emotional conflicts of the patient. Belching's psychogenic aetiology is not unheard of and can be a manifestation of conversion disorder though rare. Current case is of a middle aged woman presenting with belching unresponsive to medical management. The patient improved after supportive psychotherapy sessions and placebo medication.

Study of prodromal and residual symptoms of depression.

BACKGROUND: The definitive diagnosis of depression calls for fulfillment of certain criteria in terms of symptoms, severity, and duration, but subthreshold cases are not uncommon. These may evolve to become clinically diagnosable depression preceded by prodrome. The current study was conducted to study prodromal and residual symptoms in depression. MATERIALS AND METHODS: Eighty follow-up patients of depressive episode (F32, International Classification of Diseases-10) in remission defined by Hamilton Depression Rating Scale score <8 were interviewed. A symptom was identified as prodromal if it appeared at any time before the period of onset of symptoms sufficient to fulfill the criteria to make a diagnosis of depressive episode. Clinical Interview for Depression and Related Syndromes was used to identify the presence of symptoms. Statistical analysis was done with McNemar test and Pearson's Chi-square test using SPSS software version 20.0. RESULTS: The mean age of patients was 41.25 (±8.58) years and the sample was predominately female patients (80%). All the eighty patients had at least one prodromal symptom. The mean duration of prodrome was 115 (±64.46) days. Irritability (45%), insomnia (45%), and reduced energy (43.8%) were the most frequent prodromal symptoms. Frequency of irritability was comparable in prodromal and residual phases of depression (P = 0.074) and significantly associated with a positive family history of depression (P = 0.004). CONCLUSION: Prodrome is present in most cases of depression lasting from weeks to months. Prodrome is frequented by irritability, anxiety, sleep problems, and fatigability. Irritability is associated with genetic loading of depression and likely to present as residual symptom if it is present in prodromal phase.

Omphalocele, exstrophy of cloaca, imperforate anus and spinal defect (OEIS Complex) with overlapping features of body stalk anomaly (limb body wall complex).

OEIS is an extremely rare constellation of malformations, which includes omphalocele, exstrophy of cloaca, imperforate anus, and spinal defect. We report here autopsy findings in a case of OEIS complex, which apart from the major anomalies of the complex had bilateral club foot that is, congenital talipes equinovarus, right hydroureter, and body stalk anomaly. The umbilical cord was absent, and the umbilical vessels were embedded in an amniotic sheet, which connected the skin margin of the anterior body wall defect to the placenta, this feature being the hallmark of limb body wall complex (LBWC). This case further supports the view that OEIS and LBWC represent a continuous spectrum of abnormalities rather than separate conditions and may share a common etiology and pathogenetic mechanism as proposed by some authors.

A late presenting congenital diaphragmatic hernia misdiagnosed as spontaneous pneumothorax.

Congenital diaphragmatic hernia (CDH) is described as (1) failure of diaphragmatic closure at development, (2) presence of herniated abdominal contents into chest and (3) pulmonary hypoplasia. Usually, pleural space is drained urgently when there is respiratory distress and radiological appearance of mediastinal shift. We present a case of a 5-month-old baby, diagnosed as tension pneumothorax and treated with chest drain insertion. CDH was the intraoperative diagnosis.