Glucose-6-phosphate Dehydrogenase Deficiency in a Male Child with Acute Liver Failure Secondary to Hemophagocytosis Lymphohistiocytosis Triggered by Enteric Fever: A Rare Tetrad

particularly in endemic regions of South Asia. 3 ALF in children may present with jaundice, coagulopathy, or encephalopathy. IntroductIonHemophagocytic lymphohistiocytosis (HLH) is a rare but severe hyperinflammatory syndrome characterized by uncontrolled immune activation, a cytokine storm, and multiorgan dysfunction.HLH, often triggered by infection, is termed secondary HLH.Familial forms of primary HLH result from genetic defects in natural killer (NK) cells and cytotoxic T cells, typically affecting perforin and intracellular vesicles.In children, HLH often manifests with prolonged fever, hepatosplenomegaly, and cytopenias and can sometimes mimic or precipitate acute liver failure (ALF).Liver involvement is common in HLH, and fulminant liver failure has been documented as a serious presenting feature, especially in neonates with familial forms, complicating diagnosis and management. 1 Infections are among the most common causes of secondary HLH.Viral agents like Epstein-Barr virus (EBV), cytomegalovirus (CMV), and influenza virus are wellknown triggers.2 Enteric fever caused by Salmonella Typhi is an unusual but reported precipitant of HLH in pediatric patients, 1-

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Journal
Annals of Pediatric Gastroenterology and Hepatology ISPGHAN
Published
2026-10-28
DOI
https://doi.org/10.5005/jp-journals-11009-0197
Primary Topic
Autoimmune and Inflammatory Disorders Research
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article
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article

Glucose-6-phosphate Dehydrogenase Deficiency in a Male Child with Acute Liver Failure Secondary to Hemophagocytosis Lymphohistiocytosis Triggered by Enteric Fever: A Rare Tetrad

Indar Kumar Sharawat, Sarthak Chakrabarti, Juhi Tiwari, Prateek Kumar Panda et al.
Annals of Pediatric Gastroenterology and Hepatology ISPGHAN
Autoimmune and Inflammatory Disorders Research
article

Glucose-6-phosphate Dehydrogenase Deficiency in a Male Child with Acute Liver Failure Secondary to Hemophagocytosis Lymphohistiocytosis Triggered by Enteric Fever: A Rare Tetrad

Indar Kumar Sharawat, Sarthak Chakrabarti, Juhi Tiwari, Prateek Kumar Panda, Anukriti Agnihotry, Hitesh Poonia, Ashish Jha
article en

Abstract

particularly in endemic regions of South Asia. 3 ALF in children may present with jaundice, coagulopathy, or encephalopathy. IntroductIonHemophagocytic lymphohistiocytosis (HLH) is a rare but severe hyperinflammatory syndrome characterized by uncontrolled immune activation, a cytokine storm, and multiorgan dysfunction.HLH, often triggered by infection, is termed secondary HLH.Familial forms of primary HLH result from genetic defects in natural killer (NK) cells and cytotoxic T cells, typically affecting perforin and intracellular vesicles.In children, HLH often manifests with prolonged fever, hepatosplenomegaly, and cytopenias and can sometimes mimic or precipitate acute liver failure (ALF).Liver involvement is common in HLH, and fulminant liver failure has been documented as a serious presenting feature, especially in neonates with familial forms, complicating diagnosis and management. 1 Infections are among the most common causes of secondary HLH.Viral agents like Epstein-Barr virus (EBV), cytomegalovirus (CMV), and influenza virus are wellknown triggers.2 Enteric fever caused by Salmonella Typhi is an unusual but reported precipitant of HLH in pediatric patients, 1-

Annals of Pediatric Gastroenterology and Hepatology ISPGHANVol. 7(3)
All India Institute of Medical Sciences Rishikesh (IN)
Openalex Percentile: Top 12%
Autoimmune and Inflammatory Disorders Research
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Glucose-6-phosphate Dehydrogenase Deficiency in a Male Child with Acute Liver Failure Secondary to Hemophagocytosis Lymphohistiocytosis Triggered by Enteric Fever: A Rare Tetrad — Indar Kumar Sharawat, Sarthak Chakrabarti, et al. · Annals of Pediatric Gastroenterology and Hepatology ISPGHAN (2026) | TGRS Research Map | TGRS