Objective: This study assesses the prevalence of hypoglycemia among patients presenting at the University of Benin Teaching Hospital, Benin City, Nigeria with cholestasis of infancy.
Methods: During a period of five years, forty patients aged between 15 days and 12 months who presented with cholestasis of infancy, were admitted and screened for hypoglycemia, using Accutrend glucometer. For patients with low blood glucose values, blood samples were further analyzed, using the standard glucose-oxidase method.
Results: Of the 2,835 patients admitted over a five-year period, 40 (1.4%) had cholestasis of infancy, giving an incidence of 14 cases per 1000 admissions, with a sex ratio of 2.1: 1 in favour of males. Nine (22.5%) of the 40 infants with cholestasis had at least one blood glucose concentration less than 2.6 mmol/L (hypoglycemia). Of the nine hypoglycemic infants, three (33.3%) had one blood glucose concentration less than 1.6 mmol/L (severe hypoglycemia). Seven (77.8%) of the nine hypoglycemic infants were diagnosed in the first 36 hours of admission. Lethargy and poor feeding were observed in three infants with severe hypoglycemia and three of them died. Six (66.7%) of the hypoglycemic infants were below 3 months of age.
Conclusions: Hypoglycemia was observed among patients with cholestasis of infancy and the prevalence was higher among infants below 3 months of age.
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http://dx.doi.org/10.5001/omj.2012.82 | DOI Listing |
JPGN Rep
November 2024
The Department of Pediatrics III, Unit of Gastroenterology and Hepatology Pediatric, Abderrahim Harrouchi Children Hospital Ibn Rochd University Hospital Casablanca Morocco.
A recent nonsyndromic phenotype, newly linked to mutations in the ZFYVE19 gene, is characterized by the appearance of cholestasis accompanied by an increase in serum gamma-glutamyltranspeptidase (GGT) from infancy or early childhood. Affected individuals generally present with hepatosplenomegaly and may develop portal hypertension. The disease is thought to be the result of cholangiocyte-specific ciliary dysfunction, indicating a ciliopathy that appears to be limited to the liver.
View Article and Find Full Text PDFClin Exp Pediatr
November 2024
Department of Medical Genetics, National Taiwan University Hospital, Taipei, Taiwan.
Background: Citrin deficiency is a rare metabolic disorder prevalent in East and Southeast Asia that affects liver or neurological function throughout various life stages. While early diagnosis and dietary management can improve prognosis for infant onset disease, data on long-term neurocognitive outcomes is scarce.
Purpose: This study aimed to clarify whether transient metabolic disturbances during early childhood have a lasting effect on the neurocognitive function of individuals with citrin deficiency.
Radiol Case Rep
January 2025
Department of Radiology, Faculty of Medicine Padjadjaran University, Dr. Hasan Sadikin Hospital Bandung, West Java, Indonesia.
A choledochal cyst (CC) or biliary cyst is a congenital or acquired anomaly affecting the biliary tree. It involves the dilation of the biliary tree that could affect the extrahepatic and/or the intrahepatic segments. A choledochal cyst (CC) has traditionally been considered as a cystic dilation of the extrahepatic bile duct.
View Article and Find Full Text PDFPediatr Cardiol
November 2024
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Background: Matrix metalloproteinase 7 (MMP-7) is a novel biomarker for diagnosis of biliary atresia (BA), the most common cholestatic liver disease in infancy. There is a pressing need to determine the utility of MMP-7 levels in infants with congenital heart disease (CHD) to avoid unnecessary invasive diagnostic procedures in this high-risk population. We investigated the utility of MMP-7 in discriminating BA from non-BA cholestasis in infants with CHD and whether MMP-7 elevation was present in infants requiring treatment for clinically significant PH.
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