A 4-day-old male infant presented with complaints of jaundice on the third day of life. He was full-term and appropriate for gestational age and born to unrelated parents. All laboratory investigation tests were normal except total serum bilirubin of 27.4 mg/dl with a direct bilirubin 0.29 mg/dl. Abdominal and cranial ultrasonography (US) was performed on sixth day of life because of severe hyperbilirubinemia. Abdominal US revealed adrenal hematoma. Enclosed hematomas may cause significant unconjugated hyperbilirubinemia in absence of other high-risk conditions.
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http://dx.doi.org/10.1038/sj.jp.7211394 | DOI Listing |
Expert Rev Gastroenterol Hepatol
January 2025
Department of Hepatology, Institute of Liver & Biliary Sciences, New Delhi.
Introduction: Acute kidney injury (AKI) in patients with acute-on-chronic liver failure (ACLF) is driven by the severity of systemic inflammation, acute portal hypertension driving circulatory dysfunction, hyperbilirubinemia, and toxicity of bile acids. The spectrum is mostly structural, associated with reduced response to vasoconstrictors. The progression is rapid and need of renal replacement therapy and extracorporeal therapies may be required for the management.
View Article and Find Full Text PDFHeliyon
January 2025
Department of Hematology and Immunohematology, School of Biomedical and Laboratory Sciences, College of Medicine and Health Sciences, University of Gondar, Gondar, Ethiopia.
Background: Blood biomarkers offers an independent insight for the pathophysiology of hyperbilirubinemia. However, they are not practically used for the differential diagnosis of the hyperbilirubinemia severity. Therefore, the current study aimed to assess the differential diagnostic value of peripheral blood biomarkers with disease severity as an alternative.
View Article and Find Full Text PDFCureus
December 2024
Obstetrics and Gynecology, Latifa Hospital, Dubai, ARE.
Glanzmann thrombasthenia (GT) is an autosomal recessive platelet functional bleeding disorder caused by mutations in the ITGA2B or ITGB3 genes, often presenting as mucocutaneous bleeding. GT typically presents in infancy, but this study reports a rare case of neonatal presentation in a female infant born to consanguineous parents. The mother, a 27-year-old woman with a family history of GT, presented at 36 weeks gestation for an elective cesarean due to a breech presentation.
View Article and Find Full Text PDFGenes (Basel)
December 2024
Department of Paediatrics Hemato-Oncology and Paediatric Gastroenterology, Pomeranian Medical University, 70-204 Szczecin, Poland.
Background: Hexokinase (HK) deficiency is a rare autosomal recessively inherited disease manifested by chronic nonspherocytic hemolytic anemia. Most patients present with a mild to severe course of the disease (fetal hydrocephalus, neonatal hyperbilirubinemia, severe anemia). We reviewed 37 cases of patients with hexokinase deficiency described so far, focusing on the severity of the disease, clinical presentation, treatment applied, and genetic test results.
View Article and Find Full Text PDFInt J Hematol
January 2025
Department of Hematology, Nagaoka Red Cross Hospital, 2-297-1, Senshu, Nagaoka, 940-2085, Japan.
Sinusoidal obstruction syndrome (SOS), also known as hepatic veno-occlusive disease (VOD), is a life-threatening complication of hematopoietic stem cell transplantation. In severe cases, SOS/VOD progresses to multiple organ failure with a mortality rate higher than 80%. Early diagnosis and treatment based on severity assessment improve the prognosis of severe SOS/VOD, but conventional diagnostic criteria may be insufficient for an early diagnosis.
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