Seema Alam
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View article: Condition-Specific Growth Charts for Children With Alagille Syndrome
Condition-Specific Growth Charts for Children With Alagille Syndrome Open
Importance Different degrees of growth delay have been reported in children with Alagille syndrome (ALGS), yet these patients are routinely evaluated using standard growth charts. Objective To develop condition-specific growth charts for A…
View article: Elevated Serum Bile Acids Predict Poor Liver Outcomes in Children With Alagille Syndrome: Results From the <scp>GALA</scp> Study Group
Elevated Serum Bile Acids Predict Poor Liver Outcomes in Children With Alagille Syndrome: Results From the <span>GALA</span> Study Group Open
Background and Aim Alagille syndrome (ALGS) is a rare disorder characterised by cholestasis and extrahepatic manifestations. Given the current era of ileal bile acid transporter (IBAT) inhibitor therapies that reduce serum bile acid (SBA) …
View article: Phenotypic Divergence of <i>JAG1</i> ‐ and <i>NOTCH2</i> ‐Associated Alagille Syndrome & Disease‐Specific <i>NOTCH2</i> Variant Classification Guidelines
Phenotypic Divergence of <i>JAG1</i> ‐ and <i>NOTCH2</i> ‐Associated Alagille Syndrome & Disease‐Specific <i>NOTCH2</i> Variant Classification Guidelines Open
Background & Aims Alagille syndrome (ALGS) is a rare, autosomal dominant disorder with high phenotypic heterogeneity. Disease‐causing variants are primarily identified in Jagged1 ( JAG1 ), with fewer reported in NOTCH2 . JAG1 variants caus…
View article: Clinico‐Pathological Spectrum of Hepatitis A Virus‐Induced Autoimmune‐Like Hepatitis in Children
Clinico‐Pathological Spectrum of Hepatitis A Virus‐Induced Autoimmune‐Like Hepatitis in Children Open
There is limited evidence that hepatitis A virus (HAV) infection can trigger hepatic autoimmunity, but this area remains largely unexplored. This study was thus planned with the aim to compare HAV‐induced autoimmune‐like hepatitis (HAV‐ALH…
View article: Natural course and outcomes of children with ubiquitin‐specific protease 53 (USP53)‐related genetic chronic cholestasis
Natural course and outcomes of children with ubiquitin‐specific protease 53 (USP53)‐related genetic chronic cholestasis Open
Ubiquitin‐specific protease 53 (USP53) is essential for formation of cellular tight junctions and variations in this gene disrupt the tight junctions, resulting in cholestasis. We describe the clinical manifestations and outcomes of patien…
View article: Circulating bacterial peptides and linked metabolomic signatures are indicative of early mortality in pediatric cirrhosis
Circulating bacterial peptides and linked metabolomic signatures are indicative of early mortality in pediatric cirrhosis Open
Background: Patients with pediatric cirrhosis-sepsis (PC-S) attain early mortality. Plasma bacterial composition, the cognate metabolites, and their contribution to the deterioration of patients with PC-S to early mortality are unknown. We…
View article: Role of Splenic Hepatic elastography ratio in differentiating Non Cirrhotic Portal Fibrosis and Chronic Liver Disease in children
Role of Splenic Hepatic elastography ratio in differentiating Non Cirrhotic Portal Fibrosis and Chronic Liver Disease in children Open
Background: Differentiation of Non Cirrhotic Portal Fibrosis(NCPF) from chronic liver disease(CLD) in children and adolescents with portal hypertension(PHT) is challenging especially in cases where liver stiffness measurement(LSM) and Hepa…
View article: Prevalence and Short-Term Outcomes of Electrolyte Disorders in patients with Heart Failure
Prevalence and Short-Term Outcomes of Electrolyte Disorders in patients with Heart Failure Open
Background: Electrolyte abnormalities (EAs) are frequently observed in heart failure (HF) patients and are associated with worse short-term outcomes. This study aimed to assess the prevalence and short-term outcomes of electrolyte disorder…
View article: Event-free survival of maralixibat-treated patients with Alagille syndrome compared to a real-world cohort from GALA
Event-free survival of maralixibat-treated patients with Alagille syndrome compared to a real-world cohort from GALA Open
Background and Aims: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is an approved pharmacologic therapy for chol…
View article: Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study
Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study Open
Background and Aims: Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers, and real‐world data are lacking. This study aimed to elucidate the natu…
View article: Adequate Chelation and Cupriuresis in Hepatic Wilson disease patients under Combination (Chelator + Zinc) therapy at 2 years of follow up
Adequate Chelation and Cupriuresis in Hepatic Wilson disease patients under Combination (Chelator + Zinc) therapy at 2 years of follow up Open
Purpose Role of 24-hour urinary copper excretion (UCE) in treatment monitoring of Wilson disease (WD) is not well studied especially in pediatric population. Hence, present study is conducted with aim to evaluate UCE and its role in decidi…
View article: Newer Markers and Calculated Acute Kidney Injury Score: Better Diagnostic Accuracy for AKI in Children with Chronic Liver Disease (CLD) and Portal Hypertension (PHTN)
Newer Markers and Calculated Acute Kidney Injury Score: Better Diagnostic Accuracy for AKI in Children with Chronic Liver Disease (CLD) and Portal Hypertension (PHTN) Open
For diagnosing Acute Kidney Injury ( AKI ) newer biomolecules such as serum cystatin C and urinary neutrophil gelatinase-associated lipocalin ( NGAL ) are more accurate in estimating GFR.Renal resistive index [RRI] is early marker of AKI i…
View article: Clinicopathological Correlation-Infant with Hepatosplenomegaly
Clinicopathological Correlation-Infant with Hepatosplenomegaly Open
Clinicopathological correlation-Infant with hepatosplenomegalyClinical protocol History Six-and-a-half-month-old girl presented with upper abdominal distension and growth failure since the age of 3 months.Abdominal distention gradually pro…
View article: Recent updates on progressive familial intrahepatic cholestasis types 1, 2 and 3: Outcome and therapeutic strategies
Recent updates on progressive familial intrahepatic cholestasis types 1, 2 and 3: Outcome and therapeutic strategies Open
Recent evidence points towards the role of genotype to understand the phenotype, predict the natural course and long term outcome of patients with progressive familial intrahepatic cholestasis (PFIC). Expanded role of the heterozygous tran…