Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

According studies run during four years (1992-1995) in the Pediatric Unit of Libreville Hospital, to determine importance and features of sickle-cell disease in children in Gabon, profit and mode of regular follow-up, data of hospitalisation and management of children with sickle-cell disease were found as follow: Sickle-cell disease is third rank of admission motivations (13% of total admissions), and first after 4 years. Mortality is 8.4% of total mortality. Main causes of hospitalisation were acute anemia, painful crisis, and bacterial infections. Half of children had no medical follow-up, third was regularly checked, the remaining very irregularly, 80% of died children had no medical follow-up. A survey with an ambulatory taking-care which concerned 210 kids regularly checked, shows theses outcomes: the social families background was either medium or disavow. More than half of children were detected before one year, mainly by complication. Third had splenomegaly, 70% hepatomegaly and 40% were icteric. Based on 103 children tested for HIV, only 2 were found positive, but 22% were positive for HBs Ag. Growth, puberty and school retardation is a supplementary social handicap. A comparative with other cohorts must headlight genetic and personal environing matters. Unit of management of sickle-cell disease, annexed to a Pediatric Unit, provided that official acknowledgement, seems to be the best solution, as regards cost/efficiency, in the local context.
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PMID:[The importance of sickle cell anemia in a pediatric environment in Gabon]. 943 15

Clinical, hematological and molecular features of 116 unrelated sickle-cell anemia patients from Brazzaville were investigated. The mean age of the patients was 9.4 +/- 5.3 years. 232 beta(s)-chromosomes were haplotyped and almost all sickle cell anemia patients (91%) were homozygous for the Bantu haplotype. All hematological indices were similar for males and females. Mean hemoglobin (Hb) concentration was 6.6 +/- 1.4 g/dl. Fetal Hb (HbF) levels were from 1 to 28%, with a mean of 8.8 +/- 5.8%. There was a curvilinear relationship between %HbF and age suggesting that HbF level had a selective effect on the survival of patients. This effect was sex dependent. The (-alpha(3.7)) gene frequency was 0.45 and was not affected by stratification for age. Hematological characteristics of patients with (-alpha/alphaalpha, -alpha/-alpha) and without (alphaalpha/alphaalpha) the -alpha(3.7) alpha-thal-2 deletion showed trends similar to those reported in Jamaican and US sickle cell anemia patients. Hyperbilirubinemia (>38 micromol/l) was common and high lactic dehydrogenase (LDH) concentrations were recorded. Bilirubin concentrations for males and females were similar whereas those for LDH concentration were not. Hepatomegaly and splenomegaly were common. Splenomegaly was strongly associated with the -alpha(3.7) alpha-thal-2 deletion. These clinical and hematological observations indicate a more severe form of hemolytic disease in Congolese SS patients.
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PMID:Sickle-cell disease in Brazzaville, Congo: genetical, hematological, biochemical and clinical aspects. 1083 58

The pattern of acute illness was determined in 102 adolescents and adults with sickle cell anaemia who presented to the emergency unit of a Lagos hospital. The patients had a mean age of 20.5 years (SD 13.1) and a male-female ratio of 1.5. The symptoms included fever (72%), fatigue and weakness (59%), anorexia (59%) and pain (57.5%) while major clinical signs were pallor (100%), jaundice (71%) and hepatomegaly (68%). Sixty-eight per cent of patients had sickle cell crises, including one with hemiplegic stroke, 10% with combined anaemia and pain crises, 33% with anaemia crises only and 23.5% with pain crises only. Sixty-three per cent had infection which was malaria in 24.5%, bacterial in 17% and viral in 6%. Of 16 patients with pyrexia of unknown origin, seven responded to treatment with chloroquine and eight to antibiotics. Infection was detected in 50% of the patients with sickle cell crises. The association between anaemia crises and malaria was significant (P < 0.05). Of the eight deaths, seven (88%) had anaemia crises. In contrast to studies conducted two decades ago in the same hospital, the prevalence of anaemia crises now exceeds that of pain crises and malaria now exceeds that of bacterial infection. Severe symptomatic anaemia (anaemia crisis) was more frequently associated with infection (mostly malaria) than was bone pain crisis. The Girdle pain crisis more frequently resulted in a fatal outcome than the uncomplicated bone pain crisis.
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PMID:Acute sickle cell syndromes in Nigerian adults. 1093 Nov 63

Chronic hepatomegaly (CH) is an index of severe disease in children with sickle cell anaemia (HbS). We studied 14HbS children with CH and two groups of controls made up of age and sex-matched HbA and HbS children without CH. The objective was to determine and compare hepatic perfusions in the children and also determine which clinical and ultrasonographic features are associated with CH. Hepatic perfusion was assessed using abdominal duplex ultrasonography which made possible the measurement of portal vein diameter (PVD) and the velocity of portal blood flow. The mean weights, blood pressure profiles and ultrasonographic splenic span and common bile duct diameters (BDD) did not differ significantly among the three groups. Similarly, the man PVDs of the three groups were not significantly different despite the large livers of the CH group. The CH group however showed linear growth faltering, a significantly lower mean haematocrit, faster pulse rate but paradoxically reduced hepatic portal blood flow velocity than HbA controls. Because of the disproportion of PVD relative to liver size in HbS patients with CH and the reduced portal blood flow velocity, hepatic perfusion is probably poorer in these patients. HbS children with CH therefore need careful management of hepatic and anaemic crises to prevent severe hypoxic liver damage.
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PMID:Chronic hepatomegaly in steady state haemoglobin S children: some clinical and abdominal duplex ultrasonographic observations. 1192 52

Hepatic sequestration is an uncommon complication in patients with homozygous sickle cell disease. Although transfusion therapy has been effective for the acute condition, no definitive treatment of chronic hepatic sequestration has been identified. We describe a 17-year-old male patient with hemoglobin SS and chronic hepatic sequestration who was treated with long-term (60 months) hydroxyurea. After 36 months of HU therapy, the patient had both an excellent hematologic response and a resolution of hepatic sequestration, as evidenced by disappearance of clinical hepatomegaly, normalization of liver volume on serial computed tomography scans, as well as decreased sinusoidal dilatation and congestion and red blood cell sickling on liver biopsy. The findings in this case suggest that hydroxyurea may benefit patients who have unusual complications of sickle cell disease, such as chronic erythrocyte sickling in the liver.
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PMID:Resolution of chronic hepatic sequestration in a patient with homozygous sickle cell disease receiving hydroxyurea. 1262 Dec 48

Hepatomegaly and alterations in hepatic function are common to all patients with sickle-cell disease. In these patients, hepatic sickling is a manifestation of severe intrahepatic vaso-oclusive crises, even at levels of 25 % HbS and hematocrits of more than 45-50 %, which in 10 % of cases can lead to acute hepatic failure (AHF). AHF can be due to a variety of causes, including hematologic malignancies, but T cell lymphoma, which is usually secondary to diffuse hepatic infiltration and ischemia, is an exceptional cause, although other mechanisms can be involved. Cytokines released by lymphomas have recently been implicated as a cause of AHF.We describe a black girl with sickle cell disease, who developed AHF due to T cell lymphoma without lymphomatous infiltration of the liver. The only mechanism found to explain the clinical findings was release of cytokines by lymphoma. In patients with AHF of unknown etiology we propose early liver biopsy, because prognosis depends on the presence or absence of hepatic tumour infiltration. If AHF develops in a patient with diagnosed malignant disease, cytokine release may be the cause of AHF. Consequently, early diagnosis of the underlying disease and provision of liver support, as well as direct removal of inflammatory mediators from the circulation by exchange transfusion or other methods, should be the main priorities.
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PMID:[Acute liver failure due to T cell lymphoma without hepatic infiltration]. 1262 21

The objective of this studies is to determine the frequency of antibodies to Hepatitis C virus (anti-HCV), (2) assess the role of blood transfusion in transmission of infection, and (3) evaluate the clinical implication of anti-HCV sero-positivity in patients with Sickle cell anaemia (SCA). Two hundred and seventy-eight (278) patients with SCA were evaluated by questionnaire interviews for risk factors, clinical examination and serum testing for anti-HCV using a third generation ELISA kit. The overall anti-HCV prevalence was 5.0% (14/278). Anti-HCV was positive in 7% (5/76) of never transfused compared with 5% (9/202) of previously transfused sicklers. (p = 0.5). Clinically, splenomegaly alone or in combination with hepatomegaly was associated with a positive anti-HCV (p = 0.04 and 0.01 respectively). Anti-HCV was detected in 5% of adult patients with Sickle cell anaemia, especially in patients with persistent splenomegaly alone or in combination with hepatomegaly. Blood transfusion did not appear to be the major route of HCV transmission.
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PMID:Hepatitis C virus infection in patients with sickle cell anaemia at the Lagos University Hospital. 1456 40

The major pathology in sickle cell anaemia (SCA) is sickling of red cells due to the precipitation of reduced haemoglobin. We report our experience with extract of Cajanus cajan as a possible antisickling agent by determining changes, if any, in clinical and laboratory features of the disease in patients given the extract in a single-blind placebo-controlled study. One hundred patients with steady-state SCA were randomized into treatment and placebo arms. The extract/placebo were administered twice daily to the subjects. Weight, hepatosplenomegaly, blood levels of biliurubin, urea, creatinine, and packed cell volume (PCV) were monitored over a 6-month period. Recall episodes of pain 6 months before enrolment were compared with episodes of pains recorded during the treatment period. Twenty-six cases (55.3 per cent) had hepatomegaly on enrolment. This significantly reduced to 33.3 per cent at 6 months (p = 0.03); but increased in the placebo arm (p > 0.05). The total number of recall painful episodes in cases was 207 (mean 4.4 +/- 10.3 (SD), range 0-60) and fell to 191 (mean 4.2 +/- 4.4 (SD), range 0-16); p = 0.03. Episodes of pain increased from 109 in controls (mean 2.6 +/- 5.0 (SD), range 0-26) to 164 (mean 3.9 +/- 4.3 (SD), range 0-22); p = 0.01. Mean PCV in the cases showed no appreciable changes (p = 0.1) but there was a significant increase in the controls (p = 0.02). In conclusion, the extract may cause a reduction of painful crises and may ameliorate the adverse effects of sickle cell anaemia on the liver. The mechanism of action remains to be determined.
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PMID:Clinical evaluation of extract of Cajanus cajan (Ciklavit) in sickle cell anaemia. 1591 66

Sickle cell intrahepatic cholestasis (SCIC) is a rare complication seen in sickle cell patients who present with sudden onset of RUQ pain, progressive hepatomegaly, mild elevation of transaminases, coagulopathy, and extreme hyperbilirubinemia. Early recognition of this entity is essential to avoid life-threatening complications. Diagnosis can be challenging given the overlap in clinical presentation with other conditions affecting the hepatobiliary biliary system in sickle cell anemia such as hepatitis, cholecystitis, and hepatic crisis. Treatment is currently limited to exchange transfusion. The authors present two patients with SCIC and cholelithiasis; the clinical picture of one is complicated by choledocholithiasis.
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PMID:Sickle cell intrahepatic cholestasis with cholelithiasis. 1665 Dec 37

Liver pathology occurring in patients with sickle cell disease is commonly related to viral hepatitis or hepatic iron deposition due to repeated transfusions; cholestasis and cirrhosis may also occur. Consequently, the differential diagnosis of abnormal liver tests in patients with sickle cell anemia is often complicated. We report the case of a patient presenting with jaundice and abnormal liver biochemistries, without typical evidence of the liver diseases associated with sickle cell anemia. Biochemical markers for viral hepatitis were negative. CT scan only showed hepatomegaly. The liver biopsy revealed marked sinusoidal congestion with red blood cells without significant steatosis or increased iron deposition. The patient's medical history corroborated with biochemical tests and histological examination of the liver suggested that worsening hemolysis related to increased sickling of erythrocytes intrahepatically led to sinusoidal dilatation and probably caused the abnormal liver tests.
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PMID:Exacerbation of sickle cell disease itself as a cause of abnormal liver chemistry tests. 1717 33


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