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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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. Cirrhosis is relatively common and often the terminal event in SS disease. Choledocholithiasis and cholecystitis are infrequent complications despite the prevalence of gallstones in SS anemia.
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PMID:Sickle cell hepatopathy. 87 Sep 77

The treatment of patients with sickle cell disease and cholelithiasis is controversial. This retrospective study assesses the outcome of preoperative transfusion and timely cholecystectomy in symptomatic sickle cell disease patients. Fourteen patients who had undergone cholecystectomy were determined to have sickle cell disease. The patients' mean age was 17.9 years. Eleven patients were female. Thirteen patients had complained of abdominal pain. Ultrasound confirmed the diagnosis of cholelithiasis in 12 of 13 patients tested. Hemoglobin before treatment averaged 7.7 g/dL. Transfusion or exchange transfusion was given to 12 patients, raising the average hemoglobin to 10.3 g/dL. Postoperative morbidity was 14%: one patient had a urinary tract infection and another a left-lower-lobe pneumonia. No sickle cell crises or deaths occurred. Postoperative hospital stay averaged 4.4 days. With judicious use of preoperative transfusion, early cholecystectomy for symptomatic gallstones was well tolerated by sickle cell disease patients and is advisable to avoid the morbid sequelae of acute cholecystitis and peroperative sickle cell crisis.
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PMID:Cholecystectomy in patients with sickle cell disease: experience at a regional hospital in southeast Georgia. 150 60

This paper reviews the literature reports concerning sickle cell disease and the hepatobiliary system. Sickle cell disease can cause progressive injury to the liver with significant fibrosis, often cirrhosis, and decreased liver function by adulthood. Asymptomatic patients commonly have hepatomegaly and elevated liver enzyme levels. The presence of sickle cell disease obscures features otherwise useful in differential diagnosis. Acute episodes of the disease selectively affect the liver in 10% of patients, causing hepatic crisis with abdominal pain, nausea, fever, jaundice, and transaminase elevation. Viral hepatitis is often clinically indistinguishable from hepatic crisis, but in viral hepatitis the abdominal pain is usually less, the jaundice tends to be more severe, and the transaminase elevation more prolonged. The two can be distinguished by serology and liver biopsy. Furthermore, acute cholecystitis or choledocholithiasis may have clinical and laboratory features similar to sickle cell hepatic crisis or viral hepatitis. By adulthood, 50%-70% of sickle cell patients have gallstones. Elective cholecystectomy is indicated for those who are symptomatic, but, because of operative mortality, there is disagreement concerning surgery for asymptomatic patients. The literature contains nine well-documented cases of acute hepatic failure related to sickle cell disease. The mechanism is unclear; however, as the necrosis is often not severe, a metabolic problem is suggested.
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PMID:Hepatobiliary system in sickle cell disease. 351 88

Experience with elective cholecystectomy in ten patients with sickle cell disease and one patient with sickle-thalassemia from 1977-1984 was reviewed. In contrast to an earlier review from our institution, the current series had a low morbidity. This improvement is attributed to careful perioperative care, especially preoperative transfusion, hydration, and oxygenation. Because of the increasing longevity of sickle cell patients, because of the incidence of eventual significant complications of even asymptomatic cholelithiasis, and in order to simplify medical management by eliminating the diagnostic confusion between acute cholecystitis and sickle cell hepatobiliary crisis, the authors believe that elective cholecystectomy is to be recommended for the sickle cell patient with asymptomatic gall stones.
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PMID:Elective cholecystectomy for the patient with sickle cell disease and asymptomatic cholelithiasis. 395 72

The patterns of radionuclide hepatobiliary scans in nine children with sickle cell disease and acute right upper quadrant abdominal pain were reviewed. The most common pattern observed was delayed gall bladder visualization, consistent with chronic cholecystitis. The value of hepatobiliary imaging in distinguishing acute cholecystitis from crisis is presented.
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PMID:Biliary scintigraphy in children with sickle cell anemia and acute abdominal pain. 405 65

Acute abdominal pain frequently accompanies sickle cell crisis. The character of this pain may be difficult to discriminate from acute surgical processes such as acute cholecystitis or appendicitis. Seven patients with sickle cell disease presenting with abdominal pain underwent surgery. Review of the medical records demonstrated a characteristic pattern of presentation consistent from crisis to crisis. When patients with known sickle cell disease present with symptoms of abdominal pain, (1) the character of the symptoms, (2) precipitating events, (3) white blood cell count, (4) bilirubin, and (5) fever should be compared with those characteristics in previous crises. Deviation from previous patterns suggests an illness caused by problems other than sickel cell crisis.
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PMID:Acute surgical illness in patients with sickle cell anemia. 725 5

Cholelithasis was diagnosed in 40 of 100 consecutive patients with sickle cell anemia treated in the emergency room. Incidence apparently was not related to sex or degree of reticulocytosis, but did increase with age and perhaps was greater in patients with lower percentage of hemoglobin F and more complications of their hemoglobinopathy. Cholelithiasis was not unusual in children less than 11 years old. No major surgical complications were noted during 29 elective cholecystectomies, but urgent cholecystectomy, performed for real or suspected acute cholecystitis in seven patients, resulted in multiple major postoperative problems. With the low morbidity of elective procedures, as well as simplification of subsequent abdominal pain crisis management, elective cholecystectomy in patients with stable sickle cell conditions is recommended. Surgery during sickle cell crises or acute cholecystitis, with the attendant increased morbidity, should be avoided.
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PMID:Cholelithiasis in sickle cell anemia: surgical or medical management. 739 90

Over a year (October 1992-September 1993), 30 patients (23 males and 7 females) with sickle cell anaemia and symptomatic gallstones underwent laparoscopic cholecystectomy (LC). The mean age was 26 years (range 15-44 years). The indications were: acute cholecystitis in four patients and long-standing biliary colic in the other 26. Twenty-four patients (80%) had a previous history of abdominal sickle cell crises. The preoperative HbF and HbS levels ranged from 18 to 33% and from 66 to 77.2%, respectively. The haemoglobin level ranged from 8.6 to 12 g% (mean 9.7 g%). Blood transfusion was given preoperatively to 19 patients (63%) and three patients needed perioperative transfusion. The mean operative time was 75 min (range 60-100 min). One of the four emergency cases was converted to open cholecystectomy due to difficult anatomy and inability to grasp a thick-walled, distended gallbladder. Two patients developed minor chest infections and one had an acute vaso-occlusive crisis on the fifth postoperative day and died despite the appropriate treatment (morbidity 6.6%, mortality 3.3%). The median hospital stay was 2 days (range 1-5 days). We believe that LC can be conducted safely in sickle cell patients with gallstones with minimal morbidity and mortality.
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PMID:Laparoscopic cholecystectomy in patients with sickle cell disease. 858 41

Sickle cell disease is characterized by chronic hemolytic anemia and vaso-occlusive painful crises. The vascular occlusion in sickle cell disease is a complex process and accounts for the majority of the clinical manifestation of the disease. Abdominal pain is an important component of vaso-occlusive painful crises. It often represents a substantial diagnostic challenge in this population of patients. These episodes are often attributed to micro-vessel occlusion and infarcts of mesentery and abdominal viscera. Abdominal pain due to sickle cell vaso-occlusive crisis is often indistinguishable from an acute intra-abdominal disease process such as acute cholecystitis, acute pancreatitis, hepatic infarction, ischemic colitis and acute appendicitis. In the majority of cases, however, no specific cause is identified and spontaneous resolution occurs. This chapter will focus on etiologies, pathophysiology and management of abdominal pain in patients with sickle cell disease.
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PMID:Unusual causes of abdominal pain: sickle cell anemia. 1583 95

The role and value of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric age group is not well established, because pancreatic and biliary diseases are less common in children. This however is not the case in areas like the Eastern Province of Saudi Arabia where sickle cell disease (SCD) and other hemoglobinopathies are common, with increased frequency of cholelithiasis and choledocholithiasis. The purpose of this study was to evaluate the indications, findings, safety and therapies of ERCP in children. One hundred and twenty five children had diagnostic and/or therapeutic ERCP as part of their management at our hospital. Their medical records were reviewed for: age at diagnosis, sex, Hb electrophoresis, indication for ERCP, findings, therapy and complications. There were 77 males and 48 females. Their age at presentation ranged from 5-18 year (mean 13.25 year). The majority of them had sickle cell disease (77.6%). The indications for ERCP were: obstructive jaundice (67.2%), recurrent biliary colic with or without jaundice (10.4%), acute and chronic pancreatitis (7.2%), postoperative bile leak (2.4%), cholangitis with obstructive jaundice (2.4%), hepatitis of unknown etiology (3.2%), cirrhosis of unknown etiology (4%), thalassemia with jaundice (0.8%), hemobilia (0.8%), acute cholecystitis with jaundice (0.8%), and sickle cell disease with ulcerative colitis and obstructive jaundice (0.8%). In six children, ERCP was done following laparoscopic cholecystectomy. ERCP was carried out under sedation in 91 (72.8%) children and under general anesthesia in 34. It was successful in 121 (96.8%) children while cannulation of the Ampulla failed in four. ERCP was normal in 43 children, but eight of them showed evidence of recent stone passage and in six, there were gallstones. In the remaining children, ERCP revealed: normal CBD with stones (18 patients), dilated CBD with stones (17 patients), dilated CBD without stones (19 patients), dilated biliary tree with stones (10 patients), dilated biliary tree without stones (six patients), bile leak (two patients), dilated biliary tree with stones and choledocho-duodenal fistula (one patient), choledochal cyst (two patients), septate gallbladder (one patient), normal ERCP with multiple pancreatic cysts (one patient) and biliary stricture (one patient). The following procedures were carried out: 35 had endoscopic sphincterotomy and stone extraction, 20 had endoscopic sphincterotomy, four had CBD stenting, one underwent removal of a stent, two had insertion of a nasobiliary tube and one had biliary endoprosethesis. There was no mortality. One had bleeding from the site of sphincterotomy which stopped after adrenaline injection. Four patients (3.2%) developed transient mild pancreatitis which settled conservatively. ERCP in the pediatric age group is safe both as a diagnostic and therapeutic procedure. ERCP can provide valuable information which aid in the diagnosis of biliary and pancreatic diseases in children as well as therapy with the technical feasibility of endoscopic sphincterotomy. This is specially so in the era of laparoscopic cholecystectomy, where ERCP should be the treatment of choice in children with CBD stones who are going or have previously undergone laparoscopic cholecystectomy.
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PMID:Diagnostic and therapeutic ERCP in the pediatric age group. 1714 28


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