Case Report: Extensive Gangrene: A Rare Presentation of Multisystem Inflammatory Syndrome in Children.

A wide spectrum of cutaneous manifestations are reported in multisystem inflammatory syndrome in children (MIS-C). However, gangrenous changes are less frequently reported. A 3-year old boy, with a known case of unoperated tetralogy of Fallot, presented with a short history of fever, rash, and melena. The rash was black and diffuse, with a rapid progression. The patient was stable hemodynamically at admission, with pallor, grade II clubbing, edema, and oral ulcers. Inflammatory markers were raised. He developed gangrenous changes over the ears and acral areas. He had very high levels of antibodies to severe acute respiratory syndrome coronavirus2 (SARS-CoV-2) infection and was diagnosed as having MIS-C. Skin biopsy revealed near total epidermal necrosis with dermal vascular thrombi and negative immunofluorescence. Skin biopsy was positive for IgG antibodies to SARS-CoV-2. He was treated with antibiotics, immunomodulation with steroids, intravenous immunoglobulin, and plasmapheresis. He had features of both micro- and macroangiopathy. Gradually the child improved, with residual deformity.

Meningitis related ventriculitis--experience from a tertiary care centre in northern India.

OBJECTIVE: To assess clinical profile and short term outcome of patients with meningitis related ventriculitis. METHODS: Case records of 47 patients admitted with a diagnosis of ventriculitis (complicating meningitis) over 3 y were analysed retrospectively. RESULTS: The mean age of patients was 4.96 mo (20 d - 42 mo). The common symptoms were fever (27/47), enlarging head (23/47) and seizures (12/47) with signs of raised intracranial pressure (29/47). Ventricular cerebro-spinal fluid (CSF) showed mean cells - 237 cells/µL, sugar - 36 mg/dL and protein - 568 mg/dL. Gram stain and/or culture were positive in 5 patients. Ultrasonography of the head (done in 32 of 47 patients) showed hydrocephalus in 29 and internal echoes, debris or septations in ventricles in 5 patients. Computed tomography (CT) of brain (done in 42 of 47 patients) showed hydrocephalus in 36, abscesses in 7, ependymal enhancement in 4, infarcts in 4 and subdural empyema in 3 patients. MRI brain (done in 13 patients) showed hydrocephalus in all, 6 had cysts and 3 had ependymal enhancement. Thirty seven patients were started on ceftriaxone combined with vancomycin/cloxacillin with/without amikacin, and 10 patients received vancomycin and meropenem. Mean duration of antibiotics treatment was 4.17 wk (range; 0.8-12 wk). External ventricular drainage (EVD) was done in 22/47 patients. Cure rate (improvement in clinical and CSF parameters) in patients with external ventricular drainage was 59% as against 50% in patients who received antibiotics alone. At least 19 patients required ventriculoperitoneal (VP) shunt for unresolved significant hydrocephalus by discharge. There were 10 deaths, four of them occurred within first 2 wk of admission, one in 3rd wk and 5 within few months of discharge from hospital. CONCLUSIONS: Ventriculitis associated with meningitis is a difficult to treat infection with significant mortality and morbidity. It requires prolonged administration of antibiotics. External ventricular drainage may improve cure rate and hasten clearing of CSF infection.

Management of acute diarrhea in emergency room.

Acute diarrhea is the second leading cause of under-five mortality in India. It is defined as the passage of frequent watery stools (>3/24 h). Recent change in consistency of stools is more important than frequency. Acute diarrhea is caused by variety of viral, bacterial and parasitic agents. The common ones are: Rotavirus, E. coli, Shigella, Cholera, and Salmonella. Campylobacter jejuni, Giardia and E. histolytica are also not uncommon. The most important concern in management of acute diarrhea in Emergency room (ER) is fluid and electrolyte imbalances and treatment of underlying infection, wherever applicable. It includes, initial stabilization (identification and treatment of shock), assessment of hydration and rehydration therapy, recognition and treatment of electrolyte imbalance, and use of appropriate antimicrobials wherever indicated. For assessment of hydration clinical signs are generally reliable; however, in severely malnourished children sunken eyes and skin turgor are unreliable. Oral Rehydration Therapy is the cornerstone of management of dehydration. Intravenous fluids are not routinely recommended except in cases of persistent vomiting and/or shock. Majority of cases can be managed in ER and at home. Hospitalization is indicated in infants <3 mo, children with severe dehydration, severe malnutrition, toxic look, persistent vomiting and suspected surgical abdomen. Supplementations with zinc and probiotics have been shown to reduce severity and duration of diarrhea; however evidence does not support the use of antisecretary, antimotility and binding agents. Education of parents about hand hygiene, safe weaning and safe drinking water etc., can help in reducing incidence of this important health problem in the country.

An Indian girl with Fanconi-Bickel syndrome without SLC2A2 gene mutation.

Fanconi-Bickel syndrome is a rare autosomal-recessive disorder caused by defects in the facilitative glucose transporter 2 (GLUT2) gene. It is characterized by hepatorenal glycogen accumulation, tubular nephropathy and impaired utilization of glucose and galactose. In this communication, we present the case of a 5-year-old girl who presented with deforming rickets and massive hepatomegaly. Liver biopsy confirmed the diagnosis of glycogen storage disorder. However, the mutation of the SLC2A2 (GLUT2) gene was not found. Mutation negative patients with characteristic Fanconi-Bickel syndrome phenotype suggest additional underlying mechanisms that need exploration.