FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2912593532> ?p ?o ?g. }

• W2912593532 endingPage "1710" @default.
• W2912593532 startingPage "1710" @default.
• W2912593532 abstract "I enjoyed reading the comprehensive review on sickle cell disease by Graham Serjeant (September 6, p 725).1Serjeant GR Sickle-cell disease.Lancet. 1997; 350: 725-730Summary Full Text Full Text PDF PubMed Scopus (247) Google Scholar However there is no mention of hepatic crisis or sickle-cell hepatopathy in Graham Serjeant's seminar.Hepatic crisis is characterised by nausea, abdominal pain, low-grade fever, raised bilirubin, slight increase in alkaline phosphatase, and intact coagulation functions. In his description of hepatic crisis, Diggs2Diggs LW Sickle cell crises.Am J Clin Path. 1965; 44: 1-19Crossref Google Scholar claimed that 10% of patients with sickle-cell disease who seek medical attention have jaundice secondary to intrahepatic “regurgitant jaundice”. This is attributed to the sudden trapping of sickled red cells in the liver, somewhat similar to acute splenic sequestration. The course is variable but there is usually resolution in 1-2 weeks.Sickle cell intrahepatic cholestasis is characterised by severe right upper-quadrant pain, encephalopathy, extreme hyperbilirubinaemia, and coagulopathy associated with an enlarging liver and a precipitous drop in the haematocrit. Sickle-cell hepatopathy was first described by Green3Green TW Conley CL Berthrong M The liver in sickle cell anemia.Bull Johns Hopkins Hosp. 1953; 92: 99-127PubMed Google Scholar and co-workers in 1953. Death from liver failure has been reported. In sickle-cell hepatopathy, there is sequestration of sickled red cells in the hepatic sinusoids with subsequent sinusoidal obstruction and dilation. Sinusoidal dilation and Kupffer cell erythrophagocytosis are common pathological findings unique to sickle-cell hepatopathy.3Green TW Conley CL Berthrong M The liver in sickle cell anemia.Bull Johns Hopkins Hosp. 1953; 92: 99-127PubMed Google Scholar Although the finding of sickled red cells in hepatic sinusoids indicates an underlying sickling disorder, it may not always substantiate the diagnosis of sickle-cell hepatopathy, especially when liver tissue is preserved in formalin, which may potentiate sickling.4Omata M Johnson CS Tong M Tatter D Pathological spectrum of liver diseases in sickle cell disease.Dig Dis Sci. 1986; 31: 247-256Crossref PubMed Scopus (71) Google Scholar The only effective therapy for sickle-cell hepatopathy is exchange transfusion with both packed red cells and fresh plasma that can correct the coagulopathy and reverse the process of intrahepatic cholestasis. Avoidance of surgical intervention is also key to a successful outcome.5Shao SH Orringer EP Sickle cell intrahepatic cholestasis: approach to a difficult problem.Am J Gastroenterol. 1995; 90: 2048-2050PubMed Google ScholarDifferential diagnosis of both hepatic crisis and sickle-cell hepatopathy should include other diseases such as viral hepatitis, autoimmune liver disease, sclerosing cholangitis, choledocholithiasis, and cholecystitis, all of which can be seen in patients with sickle-cell disease. Regular and scrupulous examination of hepatic size, particularly when there is a precipitous fall in haemoglobin concentration, should be a part of the management of patients with sickle-cell disease who are in crisis. Liver biopsy is important in establishing the correct diagnosis and in instituting appropriate managment, though it should be borne in mind that the patient may have another distinct liver disease co-existing with sickle-cell disease.These two clinical entities of hepatic crisis and sickle hepatopathy could be placed either under “vasoocclusion” or under “other” manifestations given in panel 2 in the seminar by Graham Serjeant. I enjoyed reading the comprehensive review on sickle cell disease by Graham Serjeant (September 6, p 725).1Serjeant GR Sickle-cell disease.Lancet. 1997; 350: 725-730Summary Full Text Full Text PDF PubMed Scopus (247) Google Scholar However there is no mention of hepatic crisis or sickle-cell hepatopathy in Graham Serjeant's seminar. Hepatic crisis is characterised by nausea, abdominal pain, low-grade fever, raised bilirubin, slight increase in alkaline phosphatase, and intact coagulation functions. In his description of hepatic crisis, Diggs2Diggs LW Sickle cell crises.Am J Clin Path. 1965; 44: 1-19Crossref Google Scholar claimed that 10% of patients with sickle-cell disease who seek medical attention have jaundice secondary to intrahepatic “regurgitant jaundice”. This is attributed to the sudden trapping of sickled red cells in the liver, somewhat similar to acute splenic sequestration. The course is variable but there is usually resolution in 1-2 weeks. Sickle cell intrahepatic cholestasis is characterised by severe right upper-quadrant pain, encephalopathy, extreme hyperbilirubinaemia, and coagulopathy associated with an enlarging liver and a precipitous drop in the haematocrit. Sickle-cell hepatopathy was first described by Green3Green TW Conley CL Berthrong M The liver in sickle cell anemia.Bull Johns Hopkins Hosp. 1953; 92: 99-127PubMed Google Scholar and co-workers in 1953. Death from liver failure has been reported. In sickle-cell hepatopathy, there is sequestration of sickled red cells in the hepatic sinusoids with subsequent sinusoidal obstruction and dilation. Sinusoidal dilation and Kupffer cell erythrophagocytosis are common pathological findings unique to sickle-cell hepatopathy.3Green TW Conley CL Berthrong M The liver in sickle cell anemia.Bull Johns Hopkins Hosp. 1953; 92: 99-127PubMed Google Scholar Although the finding of sickled red cells in hepatic sinusoids indicates an underlying sickling disorder, it may not always substantiate the diagnosis of sickle-cell hepatopathy, especially when liver tissue is preserved in formalin, which may potentiate sickling.4Omata M Johnson CS Tong M Tatter D Pathological spectrum of liver diseases in sickle cell disease.Dig Dis Sci. 1986; 31: 247-256Crossref PubMed Scopus (71) Google Scholar The only effective therapy for sickle-cell hepatopathy is exchange transfusion with both packed red cells and fresh plasma that can correct the coagulopathy and reverse the process of intrahepatic cholestasis. Avoidance of surgical intervention is also key to a successful outcome.5Shao SH Orringer EP Sickle cell intrahepatic cholestasis: approach to a difficult problem.Am J Gastroenterol. 1995; 90: 2048-2050PubMed Google Scholar Differential diagnosis of both hepatic crisis and sickle-cell hepatopathy should include other diseases such as viral hepatitis, autoimmune liver disease, sclerosing cholangitis, choledocholithiasis, and cholecystitis, all of which can be seen in patients with sickle-cell disease. Regular and scrupulous examination of hepatic size, particularly when there is a precipitous fall in haemoglobin concentration, should be a part of the management of patients with sickle-cell disease who are in crisis. Liver biopsy is important in establishing the correct diagnosis and in instituting appropriate managment, though it should be borne in mind that the patient may have another distinct liver disease co-existing with sickle-cell disease. These two clinical entities of hepatic crisis and sickle hepatopathy could be placed either under “vasoocclusion” or under “other” manifestations given in panel 2 in the seminar by Graham Serjeant." @default.
• W2912593532 created "2019-02-21" @default.
• W2912593532 creator A5073573938 @default.
• W2912593532 date "1997-12-01" @default.
• W2912593532 modified "2023-09-23" @default.
• W2912593532 title "Sickle-cell disease" @default.
• W2912593532 cites W185399268 @default.
• W2912593532 cites W1979875948 @default.
• W2912593532 cites W2332915075 @default.
• W2912593532 cites W2364888002 @default.
• W2912593532 cites W2913013323 @default.
• W2912593532 doi "https://doi.org/10.1016/s0140-6736(05)64318-8" @default.
• W2912593532 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/9400543" @default.
• W2912593532 hasPublicationYear "1997" @default.
• W2912593532 type Work @default.
• W2912593532 sameAs 2912593532 @default.
• W2912593532 citedByCount "1" @default.
• W2912593532 crossrefType "journal-article" @default.
• W2912593532 hasAuthorship W2912593532A5073573938 @default.
• W2912593532 hasConcept C126322002 @default.
• W2912593532 hasConcept C1491633281 @default.
• W2912593532 hasConcept C2779134260 @default.
• W2912593532 hasConcept C54355233 @default.
• W2912593532 hasConcept C71924100 @default.
• W2912593532 hasConcept C86803240 @default.
• W2912593532 hasConceptScore W2912593532C126322002 @default.
• W2912593532 hasConceptScore W2912593532C1491633281 @default.
• W2912593532 hasConceptScore W2912593532C2779134260 @default.
• W2912593532 hasConceptScore W2912593532C54355233 @default.
• W2912593532 hasConceptScore W2912593532C71924100 @default.
• W2912593532 hasConceptScore W2912593532C86803240 @default.
• W2912593532 hasIssue "9092" @default.
• W2912593532 hasLocation W29125935321 @default.
• W2912593532 hasLocation W29125935322 @default.
• W2912593532 hasOpenAccess W2912593532 @default.
• W2912593532 hasPrimaryLocation W29125935321 @default.
• W2912593532 hasRelatedWork W2063989134 @default.
• W2912593532 hasRelatedWork W2084059607 @default.
• W2912593532 hasRelatedWork W2138421187 @default.
• W2912593532 hasRelatedWork W2473885471 @default.
• W2912593532 hasRelatedWork W2748952813 @default.
• W2912593532 hasRelatedWork W2899084033 @default.
• W2912593532 hasRelatedWork W3031052312 @default.
• W2912593532 hasRelatedWork W4283512960 @default.
• W2912593532 hasRelatedWork W583169958 @default.
• W2912593532 hasRelatedWork W75613116 @default.
• W2912593532 hasVolume "350" @default.
• W2912593532 isParatext "false" @default.
• W2912593532 isRetracted "false" @default.
• W2912593532 magId "2912593532" @default.
• W2912593532 workType "article" @default.