Clinical Features and Genetic Sequencing of Children with Fanconi-Bickel Syndrome.

The present paper reports 10 patients (9 families) with Fanconi-Bickel syndrome managed during 2010-2021. Patients presented with polyuria, polydipsia, hepatomegaly, rickets, and stunting at a median of 5 (3, 7.3) mo; one had transient neonatal diabetes.

Management and outcomes in children with lupus nephritis in the developing countries.

Background: Lupus nephritis (LN) has variable prevalence, severity, and outcomes across the world.

Objectives: This review compares the outcomes of childhood LN in low- and middle-income countries (LMICs) and high-income countries (HICs) and aims to summarize long-term outcomes of pediatric LN from LMICs.

Data Sources: A systematic literature search, conducted in PubMed, EMBASE, and Cochrane database in the last 30-years from January 1992, published in the English language, identified 113 studies including 52 from lower (n = 1336) and upper MICs (n = 3014).

Anti-factor H antibody associated hemolytic uremic syndrome following SARS-CoV-2 infection.

Background: The pathogenesis of autoantibody generation in anti-factor H (FH) antibody associated atypical hemolytic uremic syndrome (aHUS) is unknown and is perhaps triggered by an infectious or environmental agent. We observed an unusual increase of patients with anti-FH antibody associated aHUS coinciding with the second pandemic wave in New Delhi and suspected that SARS-CoV-2 infection might be a potential trigger.

Methods: We screened for SARS-CoV-2 infection using reverse transcriptase polymerase chain reaction (RT-PCR) and serology in 13 consecutive patients with anti-FH antibody associated aHUS during the past year in New Delhi.

Anti-Compliment Factor H Antibody Associated Hemolytic Uremic Syndrome in Children with Abbreviated Plasma Exchanges: A 12-Month Follow-up Study.

Introduction: Anti-Compliment Factor H antibody hemolytic uremic syndrome (AFH-HUS) is a common cause of paediatric atypical HUS in India. We wanted to study the outcome of patients receiving less than recommended plasma exchange (PLEX) but adequate immunosuppression, with respect to hypertension, preservation of renal function and proteinuria.

Methods: A retrospective study was performed in 15 children admitted from 2016 to 2018 with AFH-HUS.

Pericardial effusion in anti-complement factor H antibody-associated atypical hemolytic uremic syndrome: two case reports.

Hemolytic uremic syndrome (HUS), a cause of pediatric acute kidney injury (AKI), has a spectrum of extra-renal manifestations. While neurological and gastrointestinal system involvement is common, cardiac involvement is rare. This is more so with pericardial involvement, though it has been reported in a handful of HUS cases associated with shiga toxin-producing Escherichia coli (STEC HUS).

Anti-complement factor I antibody associated atypical hemolytic uremic syndrome - A new insight for future perspective!

Atypical hemolytic uremic syndrome (aHUS) is caused mainly by complement dysregulation. Although various defects in the complement system explaining pathophysiology have been described in recent years, the etiology still remains unclear in about thirty percent of cases. In exploring other causes, similar to anti- complement factor H (anti-CFH) antibody associated HUS, we hypothesized that anti-complement factor I (anti-CFI) antibody could play a role in aHUS.

Usefulness of automated fragmented red blood cell percentage in the diagnosis of paediatric haemolytic uraemic syndrome.

Introduction: Presence of schistocytes in peripheral blood smear supporting haemolysis is important for diagnosis and decision-making in paediatric haemolytic uraemic syndrome (HUS). High observer dependency and requirement of expertise for peripheral smear evaluation propels us to think of other modalities to overcome these issues. We envisage that newer techniques like automated fragmented red blood cell percentage (FRC %), whose role has been described in transplant associated HUS and thrombotic thrombocytopenic purpura, can serve the purpose.