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sickle cell hepatopathy

Lydia H Pecker, Nidhi Patel, Susan Creary, Anil Darbari, Emily Riehm Meier, Deepika S Darbari, Ross M Fasano
The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non-SCD cause of hepatopathy in a patient with viral hepatitis...
April 18, 2018: Pediatric Blood & Cancer
Rushikesh Shah, Cesar Taborda, Saurabh Chawla
Sickle cell disease (SCD) is a common hemoglobinopathy which can affect multiple organ systems in the body. Within the digestive tract, the hepatobiliary system is most commonly affected in SCD. The manifestations range from benign hyperbilirubinemia to overt liver failure, with the spectrum of acute clinical presentations often referred to as "sickle cell hepatopathy". This is an umbrella term referring to liver dysfunction and hyperbilirubinemia due to intrahepatic sickling process during SCD crisis leading to ischemia, sequestration and cholestasis...
August 15, 2017: World Journal of Gastrointestinal Pathophysiology
Aamir Malik, Chandni Merchant, Mana Rao, Rosemary P Fiore
BACKGROUND: Sickle cell disease can affect the liver by way of the disease process, including sickling in hepatic sinusoids, as well as its treatment, including repeated blood transfusions leading to hemosiderosis and hepatitis. Sickle cell intrahepatic cholestasis (SCIC) is an extreme variant of sickle cell hepatopathy, and is associated with high fatality. CASE REPORT: We present the case of a 31-year-old man with past medical history of sickle cell disease and cholecystectomy who was admitted with uncomplicated vaso occlusive crisis and during the hospital stay developed fever, upper abdominal pain, and jaundice...
November 28, 2015: American Journal of Case Reports
Kate Gardner, Abid Suddle, Pauline Kane, John O'Grady, Nigel Heaton, Adrian Bomford, Swee Lay Thein
Sickle cell disease (SCD) has evolved into a debilitating disorder with emerging end-organ damage. One of the organs affected is the liver, causing "sickle hepatopathy," an umbrella term for a variety of acute and chronic pathologies. Prevalence of liver dysfunction in SCD is unknown, with estimates of 10%. Dominant etiologies include gallstones, hepatic sequestration, viral hepatitis, and sickle cell intrahepatic cholestasis (SCIC). In addition, causes of liver disease outside SCD must be identified and managed...
April 10, 2014: Blood
Angela Siciliano, Giorgio Malpeli, Orah S Platt, Christophe Lebouef, Anne Janin, Aldo Scarpa, Oliviero Olivieri, Eliana Amato, Roberto Corrocher, Yves Beuzard, Lucia De Franceschi
BACKGROUND: Sickle cell disease, a genetic red cell disorder inherited in an autosomal recessive manner, occurs throughout the world. Hepatic dysfunction and liver damage may be present in sickle cell disease, but the pathogenesis of these conditions is only partially understood. DESIGN AND METHODS: Transgenic mice with sickle cell disease (SAD mice) and wild-type mice were exposed to an ischemic/reperfusion stress. The following parameters were evaluated: hematologic profile, transaminase and bilirubin levels, liver histopathology, and mRNA levels of nuclear factor-κB p65, endothelial nitric oxide synthase, inducible nitric oxide synthase, heme oxygenase-1 and phosphodiesterase-1, -2, -3, and -4 genes in hepatocytes obtained by laser-capture microdissection...
January 2011: Haematologica
Aglaia Zellos, John K Boitnott, Kathleen B Schwarz
Autoimmune hepatitis (AIH) is thought to be a primary liver disease, occurring in the absence of any known etiology. We present three unusual cases of new-onset AIH in young female patients with longstanding preexisting liver disease (Alagille's syndrome, cystic fibrosis liver disease and sickle cell hepatopathy). All patients developed an insidious onset of abdominal pain, fatigue, jaundice and hepatitis after many years of their primary diagnosis and had negative serology for hepatitis A, B, C, cytomegalovirus and Epstein-Barr virus...
September 2010: Digestive and Liver Disease
Ranjana Bandyopadhyay, Sanjay K Bandyopadhyay, Anita Dutta
Sickle cell hepatopathy is a well-documented entity that ranges from the self-limiting hepatic right upper quadrant syndrome to the potentially lethal intrahepatic cholestasis and acute hepatic sequestration syndromes. We describe a 26-year-male with homozygous sickle cell disease who had this unique hepatic presentation and was documented to have characteristic findings of cholestasis, portal inflammation and sinusoidal dilatation on histopathology.
April 2008: Indian Journal of Pathology & Microbiology
Timothy J S Cross, Philip A Berry, Nuzhat Akbar, Julia Wendon, S L Thein, Phillip M Harrison
A 38-year-old Ghanaian man presented with a 6-month history of worsening pruritus, jaundice, and ascites. He was previously fit and well and rarely drank alcohol. Screening tests for chronic liver disease including viral, autoimmune, and other metabolic causes including iron overload were unremarkable. A liver biopsy performed at the referring hospital demonstrated intralobular cholestasis and cirrhosis. He was listed for liver transplantation but subsequently developed sepsis with multiple organ failure and died...
September 2007: American Journal of Hematology
Kristin L Mekeel, Max R Langham, Regino Gonzalez-Peralta, Shiro Fujita, Alan W Hemming
Severe liver disease is an unusual but potentially fatal complication of sickle-cell disease (SCD). Liver transplantation has been complicated by ongoing SCD and thrombosis. We reviewed 214 pediatric transplants done at our institution from 1990 to 2005. Three patients were transplanted for complications of SCD, including intrahepatic cholestasis and viral hepatitis. Overall patient and graft survival was 66%. One patient died after 6 years from a subdural hematoma. There were not any incidences of graft loss, primary nonfunction, or thrombosis...
April 2007: Liver Transplantation
Lawrence S Friedman
No abstract text is available yet for this article.
April 2007: Liver Transplantation
Hyunah Ahn, Chin-Shang Li, Winfred Wang
BACKGROUND: Standard diagnostic criteria and therapy are lacking for sickle cell hepatopathy, an uncommon complication of sickle cell disease. Here we propose diagnostic and therapeutic guidelines based on our experience and on reported cases. METHODS: We defined sickle hepatopathy by a total serum bilirubin concentration >13 mg/dl not explained by severe acute hemolysis, viral hepatitis, extrahepatic obstruction, or hepatic sequestration. We reviewed the records of all children with sickle hepatopathy seen at our institution during the past 20 years and the reported cases from the literature...
August 2005: Pediatric Blood & Cancer
S Sitalakshmi, Anuradha Srikrishna, Shanthala Devi, Prema Damodar, Theresiamma Mathew, Jessy Varghese
A 10 year study of malaria during 1989-98 recorded an increase in the incidence of malaria from 0.22 in 1989 to 1.3 in 1996 following which it has reached a plateau. The cases were chiefly from Karnataka, Andhra Pradesh and Tamil nadu. The P. falciparum infection and mixed infections (P. falciparum and vivax) were found to be on the rise. Peak of malaria cases were recorded in the months of June-July and in Oct-Nov coinciding with the rains showing a seasonal pattern. The common haematological findings were anemia, thrombocytopenia, pancytopenia and leucopenia...
July 2003: Indian Journal of Pathology & Microbiology
Hussain H Al-Saeed, Ahmed H Al-Salem
Sickle cell anemia (SCA) is one of the commonly inherited hemoglobinopathies in the Kingdom of Saudi Arabia. It is characterized by periods of remissions and exacerbations called crises as well as certain pathological phenomenon such as acute chest syndrome, priapism, hepatopathy, and cerebrovascular stroke. Blood transfusion (BT) as therapy and prophylaxis in SCA, although was advocated as early as the 1940's, there are still debates regarding its benefits and risks. This is a review of the value of BT in patients with SCA with emphasis on the risks and benefits as well as guidelines towards safe BT...
December 2002: Saudi Medical Journal
Nada Zakaria, Alex Knisely, Bernard Portmann, Giorgina Mieli-Vergani, Julia Wendon, Roopen Arya, John Devlin
After several complications following percutaneous liver biopsy in patients with sickle cell disease, we reviewed our experience. We examined 14 patients with sickle cell disease who underwent a percutaneous liver biopsy. Clinicopathologic findings were correlated with outcome. Of 14 patients, 5 (36%) suffered serious hemorrhage; 4 died (80%; 28% of all patients). None of the 9 patients without biopsy complications was in an acute sickling crisis at the time of biopsy; 4 of 5 patients with complications were in acute sickling crisis...
January 1, 2003: Blood
S Banerjee, C Owen, S Chopra
No abstract text is available yet for this article.
May 2001: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
J W Childs
In the United States, sickle cell disease primarily affects people of African descent, and the multisystemic complications caused by the resultant vaso-occlusive state create a multitude of diagnostic considerations. In the musculoskeletal system, likelihood is high for avascular necrosis of the femoral humeral head, as a consequence of skeletal infarcts, and also for leg ulceration and osteomyelitis; in the eyes, the incidence of proliferative retinopathy is high; in the urinary tract, dehydration is common, and causes for renal failure are many; in the pulmonary system, pneumonia is of prime concern, as are sickle cell chest syndrome (from occlusion within the microvasculature of the lung) and the deadly sickle cell chronic lung disease, for which pulmonary function tests are important in early asymptomatic stages...
October 1995: Journal of the American Osteopathic Association
A Horn, N Freed, A A Pecora
No abstract text is available yet for this article.
February 1987: Journal of the American Osteopathic Association
G R Buchanan, B E Glader
Since the approach to the management and outcome of extreme hyperbilirubinemia in patients with sickle cell anemia is not clearly defined, we reviewed our experience with marked hyperbilirubinemia in six children with sickle cell disease. Intrahepatic sickling (sickle hepatopathy) rather than hepatitis or biliary stones appeared primarily responsible for the extreme jaundice in at least four children and possibly in all six. Signs and symptoms were few, and laboratory abnormalities were not striking other than marked hyperbilirubinemia (total serum bilirubin concentrations ranging from 20...
July 1977: Journal of Pediatrics
T W Sheehy
Hepatic dysfunction is a common finding in patients with sickle cell disease but viral hepatitis appears to be an unusual complication in the adult SS patient. Only five cases of viral hepatitis were recorded in 378 admissions for SS crisis. In contrast, hepatic crisis occurred as a distinct event in 9% of 88 patients with sickle cell anemia. This entity must be differentiated from acute cholecystitis or viral hepatitis. Transiently abnormal results of hepatitic function tests were observed in another 26 patients with extrahepatic crisis...
May 1977: Southern Medical Journal
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