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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Several episodes of acute hepatic enlargement associated with a dramatic fall in haemoglobin concentration were observed in two patients with sickle cell anaemia. No appreciable disturbances of liver function or signs of cardiac failure were evident. The most likely mechanism was sequestration of sickled erythrocytes in the liver. This complication, which may have a basis similar to that of splenic sequestration and the sickle lung syndrome, may be easily overlooked unless the size of the liver is regularly monitored in patients with sickle cell crisis.
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PMID:Hepatic sequestration in sickle cell anaemia. 391 37

Between January 1976 and December 1978, the Microbiology Department of University College Hospital (UCH) Ibadan, isolated Salmonella typhi from the blood cultures of 93 children aged 0-14 years, who were admitted to the paediatric wards. Clinical case notes were retrieved and reviewed in 64 (68.8%) of them. Fifteen (23%) of the 64 children were less than one year of age while 22 (34%) were under the age of five years. The commonest presenting symptoms were fever, anorexia, diarrhoea and vomiting. A febrile convulsion was the presenting symptom in 13 (20%) of the patients, all of whom were under the age of five years. Hepatomegaly was almost twice as frequently observed as splenomegaly. Intestinal perforation was present in five of the patients. There was a high proportion of SS children who presented with fever, pallor, jaundice, generalized aches and pains and other clinical features of sickle cell disease and it is possible that such children are specially susceptible to typhoid fever. A clinical diagnosis of typhoid fever on admission was made in only 14 of the 64 children. Reasons are given for the low index of suspicion and it is suggested that any child with unremitting fever after adequate anti-malarial chemotherapy should be treated for enteric fever.
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PMID:Problems in the clinical diagnosis of typhoid fever in children in the tropics. 618 69

The spleen was assessed in 10 patients with sickle cell disease studied with computed tomography (CT) for abdominal pain and/or unexplained fever. Patients with homozygous sickle cell anemia were found to have small, densely calcified spleens with occasional low-density infarcts. Five of six had hepatomegaly, and there was one case each of hepatic abscess, infarcts, and hemochromatosis. All patients with heterozygous sickle cell disease were found to have splenomegaly, with a variety of findings including acute hemorrhage, acute and chronic infarcts, rupture, and possible sequestration. It was concluded that CT is useful for evaluating the status of the spleen and liver in symptomatic patients with sickle cell disease.
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PMID:Computed tomography of the spleen and liver in sickle cell disease. 661 Oct 49

Forty-five children with sickle cell anemia were studied with meticulous cholecystosonograms using a 5-MHz thyroid transducer. Good-quality images were obtained. The most informative and useful view was the left-side-down decubitus study with the ultrasound gantry angled 45% to the anteroposterior axis. Rotating the patient rapidly through 360 degrees did not increase the information content of the examination. We found that one third of a random group fo children with sickle cell anemia will have gallstones and one fifth will have"sludge". A kinked deformity of the gallbladder may simulate a gallstone. Neither age, sex, weight, physical findings (except for hepatomegaly), nor a variety of biochemical measurements of the blood will be of much value in predicting gallbladder disease in any given patient.
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PMID:Cholecystosonography in children with sickle cell disease: technical approach and clinical results. 678 89

Twenty-seven patients were seen and followed at our Sickle Cell Center over a period of seven years. Their clinical, hematological, and biochemical features were determined and compared to those of patients with sickle cell anemia who were concurrently investigated. The data indicate that the mild anemia of hemoglobin (Hb) SC disease is slightly microcytic and hyperchromatic. Parameters of hemolysis and the complications of chronic hemolytic anemia (cholelithiasis, leg ulcers, hepatomegaly, and cardiomegaly) are milder in Hb SC disease than in sickle cell anemia. Asplenia and its sequelae (increased platelet count and reduced serum IgM levels) are less frequent in Hb SC disease. Cerebrovascular accidents and the decreased leukocyte alkaline phosphatase scores are similar in both diseases. Thromboembolic complications, retinopathy, and renal papillary necrosis are more frequent in Hb SC disease.
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PMID:Clinical, hematological, and biochemical features of Hb SC disease. 713 65

Sickle cell intrahepatic cholestasis is a rare but potentially fatal complication of sickle cell disease. Its characteristic features include hepatomegaly, extreme total hyperbilirubinemia, coagulopathy, and acute liver failure. Although the pathophysiology is uncertain, most reports in the medical literature indicate that the prognosis is grim. The only effective therapy that has been reported in this setting is exchange transfusion. We describe two hemoglobin SS patients with sickle cell intrahepatic cholestasis. We conclude that exchange transfusion and supportive care aimed at correction of coagulopathy, stabilization of the acute liver disease, and perhaps most important, avoidance of surgical intervention are the keys to a successful outcome.
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PMID:Sickle cell intrahepatic cholestasis: approach to a difficult problem. 748 22

Eighty sickle cell anaemia patients, 29 with persistent hepatomegaly and 51 without, were evaluated with respect to clinical severity score. Haemoglobin F (HbF) was also determined in 52 patients, 20 with persistent hepatomegaly and 32 without. The severity scores were computed from the steady state haematocrit, number of transfusions per year, and number of crises per year. The HbF level was determined by the alkali denaturation method. The patients with persistent hepatomegaly had significantly higher clinical severity scores (P < 0.001) and significantly lower levels of HbF (P < 0.0001). The implication of the finding is that patients with persistent hepatomegaly may have a more severe clinical course than those without, and clinicians managing them may have to pay more particular attention to them. In effect, persistent hepatomegaly may be a useful indicator of severity in sickle cell anaemia.
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PMID:Persistent hepatomegaly: an index of severity in sickle cell anaemia. 753 25

This report describes the case of a 63-year-old obese and alcoholic butcher who suffered two episodes of distal oligoarthritis. These episodes were attributed to gout on the basis of response to colchicine and demonstration of hyperuricemia. Given the presence of hepatomegaly, splenomegaly and hyperleukocytosis, a sternal puncture and myelogram were performed and led to diagnosis of chronic myelogenous leukemia. In addition to the classic predisposing factors for gout, sickle cell anemia could be implicated in Black Africa. Longer life expectancy for patients with sickle cell anemia could increase the incidence of gout in Black Africa.
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PMID:[Chronic myeloid leukemia manifesting as gout. Reflections on secondary gout in Black Africa]. 756 98

Seven hundred and three Nigerian village children in their first six years of life were subjected to anthropometric measurements and physical examination in early 1988. The heights of 66.9% and weights of 60.5% of them fell below the third percentile of a Nigerian equivalent for international reference population standard. Mid upper arm circumference values indicated moderate to severe malnutrition in over 25% of all 1-5 year old children surveyed. Fever, cough, headache and diarrhoea were the commonest symptoms encountered in the children. Mild pallor of the conjunctival mucosa and physical signs of protein energy malnutrition were commonly seen. Fungal and septic skin lesions were present in 11.45 and 11.1% of the children respectively, whilst rhinorrhoea was seen in 4.7%, otitis media in 6% and pharyngotonsillitis in 3.3%. Thirty four (4.8%) of the children had haemic whereas five had pathological murmurs. Dental calculi were present in 15.8%, umbilical herniae in 18.2%, hepatomegaly in 48.2% and splenomegaly in 23% of the children. Seven (1%) had cerebral palsy. The implication is that malnutrition, sickle cell disease, malaria and other infections are the prevailing causes of morbidity in the preschool aged children surveyed. Desirable improvements include upgrading socio-economic and living conditions and instituting appropriate control measures.
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PMID:Anthropometric measurement in children aged 0-6 years in a Nigerian village. 758 49

Admission records of children with sickle cell anemia (SS), in the two main teaching hospitals in Kuwait, were reviewed for a 1-year period. The haplotypes of 92 beta s chromosomes (from 39 SS, 11 AS, 2 S beta-thalassemia [S beta-thal] and 1 SD individuals) were determined using an allele-specific oligonucleotide (ASO) hybridization technique, while the alpha-globin gene status of 27 SS and 33 AS individuals, i.e. 120 chromosomes, was determined with a combination of polymerase chain reaction and AS techniques. A vasooclusive crisis was the most common (60.0%) cause of hospitalization, followed by infections (20%). Hospital admissions were most common during the hottest month of the year (July). Few complications of the disease were seen among patients on follow-up; however, splenomegaly was present in 24.0%, hepatomegaly in 15.2%, gallstones in 15.2% and aseptic necrosis of the femoral head in 6.1%. Haplotype 31 (Saudi Arabia/India) is the most frequent in this community, being present in 80.4% of the chromosomes tested; Benin haplotype 19 was found in 12.0% and Bantu haplotype 20 in 6.5%. Hb F in the haplotype 31 homozygotes and heterozygotes ranged from 11.4 to 35.1% (mean 22.5 +/- 5.2%). The frequency of alpha-thal determinants in the study was 40.0%, the commonest being the -alpha 3.7-kb deletion (27.5%), the alpha 2 polyadenylation signal (AATAAA-> AATAAG) mutation (10.2%) and the IVS-I 5' end GAGGT-GAGG->GAGG pentanucleotide (5 nt) deletion (3.3%). SS patients with coexistent alpha-thal trait did not have severe recurrent infections and none had gallstones. The high frequencies of the Saudi Arabia/India beta s haplotype and alpha-thalassemia trait contribute to the mild nature of SS disease among Kuwaiti Arabs comparable to that in eastern Saudi Arabia.
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PMID:Morbidity, beta S haplotype and alpha-globin gene patterns among sickle cell anemia patients in Kuwait. 887 12


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