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

The clinical and hematological features of 100 patients with sickle cell anemia are reviewed. The heart was enlarged and a murmur was heard in nearly 80 percent of patients. Pneumonia and pulmonary infarction occurred in 43 percent and 12 percent of patients, respectively. Musculoskeletal involvement included the hand-foot syndrome (15 percent), leg ulcers (55 percent), aseptic necrosis ofbone (11 percent), and osteomyelitis (4 percent). Symptoms and signs related to the gastrointestinal system included jaundice (55 percent), hepatomegaly (50 percent), splenomegaly (23 percent), hepatitis (11 percent) and gallstones (9 percent). Three patients underwent cholecystectomy and three patients had their spleens removed. Pyelonephritis occurred in 17 patients, priapism in five and hematuria in seven. Nineteen women had 39 pregnancies, of which 35 resulted in the birth of healthy infants. At least 328 painful crises occurred in 73 patients. There were also 13 hemolytic crises, eight sequestration crises, and five aplastic crises. A trail of alkali therapy in 33 crises in children failed to produce beneficial effects greater than hydration and analgesics alone as used in the control group. Laboratory findings in the 100 patients were comparable to those previously reported in the literature. The renal concentrating defect in most patients was confirmed. There were six deaths: hepatic coma secondary to post-transfusion hepatitis, thrombosis of inferior vena cava, congestive heart failure, exsanguination from erosion of the pancreaticoduodenal artery, extensive bronchopneumonia, and pulmonary infarction.
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PMID:Sickle cell anemia- clinical manifestations in 100 patients and review of the literature. 113 Apr 36

The relationship of foetal haemoglobin (HbF) to several clinical and haematological parameters in 86 children aged 5 to 16 years who had sickle cell anaemia (SCA) has been examined. The mean HbF level was 6.8 +/- 3.6%. Higher HbF values were significantly associated with fewer blood transfusions, fewer hospital admissions and higher rate of hepatomegaly. A trend for children with higher HbF levels to have better physical development and a more normal haemogram was also noted though this was not statistically significant.
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PMID:Foetal haemoglobin in Nigerian children with sickle cell anaemia. Effect on haematological parameters and clinical severity. 128 Aug 70

Using a prospective and retrospective approach, the features of sickle cell disease (SCD) were investigated in 137 SCD children from the south-western region of Saudi Arabia. The patients were followed for a period of 2-5 years, during which period the severity of the disease was assessed and a 'severity index', was calculated for individual patients. The patients with SCD were classified into five groups based on the absence of thalassaemia (sickle cell anaemia, SCA), presence of beta zero-thalassaemia (HbS/beta zero-thalassaemia), SCA with alpha-thalassaemia.2 [heterozygotes (-alpha/alpha alpha) or homozygotes (-alpha/alpha)] and S/beta zero-thalassaemia with alpha-thalassaemia. The results showed a high prevalence of associated alpha-thalassaemia and variable levels of HbF in these patients. SCA patients with associated alpha-thalassaemia (-alpha/-alpha) and S/beta zero-thalassaemia patients with one alpha-gene deletion had the highest values for haematological parameters and lowest values of red cell indices. No specific difference could be identified in the clinical manifestations in the different groups with the exception that long bone crisis and hand-foot syndrome were not encountered in patients with associated alpha-thalassaemia. The frequency of hepatomegaly and splenomegaly was also lower in this group.
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PMID:The features of sickle cell disease in Saudi children. 221 76

Generally, individuals who are heterozygous to haemoglobin S (Hb AS) are asymptomatic and do not present any haematological or clinical manifestations. However, other associated genetic abnormalities may influence the presentation in Hb AS cases. This study was conducted on twenty children heterozygous for HB S who presented clinical manifestations similar to those of sickle cell anaemia. All these children had anaemia associated with several red cell morphological abnormalities. The white blood cell counts were elevated in all patients and differential count studies showed a substantial increase in lymphocytes and polymorphonuclear leucocytes in the majority of the cases. Forty-five per cent of the patients had associated alpha-thalassaemia, 60 per cent had beta-thalassaemia, 30 per cent had G-6-PD deficiency and 10 per cent had partial glutathione reductase deficiency. Pyruvate kinase activity was normal in all cases. Riboflavin deficiency was encountered in 30 per cent of the patients and iron deficiency in 15 per cent of these Hb S heterozygotes. The major clinical findings were splenomegaly, hepatomegaly, and vaso-occlusive crisis. The majority of the patients had received blood transfusions. The hand and foot syndrome was identified in three (15 per cent) of the patients. The haematological and clinical findings in these twenty Hb S heterozygotes are presented in this paper and the possible causes for these abnormalities are discussed.
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PMID:Case studies on haemoglobin S heterozygotes with severe clinical manifestations. 228 93

Tow age-matched groups of patients with sickle cell anaemia (SS), one with persistent gross splenomegaly (PGS) and the other without palpable spleens, were studied to determine the clinical and laboratory features associated with PGS. The group with PGS had significantly larger mean liver size, higher serum indirect bilirubin levels, but lower mean haematocrit, blood cell counts and serum albumin: globulin ratio when compared to the control group without splenomegaly. Large palpable livers (greater than 6 cm below the coastal margin) among the PGS group tended to be associated with digital clubbing. A retrospective analysis of their case files showed that patients with PGS had had predominantly anaemic crises while those without splenomegaly had had predominantly vaso-occlusive crises over a 2-year period. No difference was found between the 2 groups, in respect of anthropometric data, prevalence of hepatomegaly or frequency of hospital admissions and blood transfusions.
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PMID:Clinical and laboratory features associated with persistent gross splenomegaly in Nigerian children with sickle cell anaemia. 238 63

Seventy-one Saudi and Yemeni Arabs with sickle cell anaemia from western Saudi Arabia aged between 1 1/2 and 42 years were studied. The mean steady state haemoglobin concentration of 8.1 g/dl was lower than that of 10.7 g/dl reported previously for sickle cell anaemia in eastern Saudi Arabia. The patients were divided into an SSLF group with fetal haemoglobin (HbF) of 10.0% or below (44 patients) and an SSHF group having HbF above 10.0% (27 patients). No significant differences were found in the haemoglobin concentrations, haematological indices and incidences of bone changes of the two groups. SSLF patients were significantly more prone to infections (P less than 0.01), however. Also, there was an overall high incidence of hepatomegaly (69.0%) and splenomegaly (54.9%) and hepatomegaly was significantly more common in the SSLF group (P less than 0.02). Many of the patients, even with HbF levels over 10.0%, did not follow a benign course and suffered from severe anaemia, infections of the respiratory and urinary tracts, bone pains and infarcts, or bossing of the skull. Rarer complications included hepatic crisis, chest syndrome, retinal haemorrhage, epistaxis and hemiplegia. It is therefore apparent that Saudi Arabian sickle cell anaemia, even in patients with raised haemoglobin F levels, may be as clinically severe as in African patients.
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PMID:Non-benign sickle cell anaemia in western Saudi Arabia. 240 55

The pattern of illness in 60 consecutive children with homozygous sickle cell disease who attended the Paediatric Emergency Room of a busy Lagos hospital with acute illness was studied prospectively. Their ages ranged from 3 months to 13 years with a peak in the 2nd year. There were twice as many boys as girls. The commonest symptoms were fever, limb or abdominal pain and cough, and the commonest signs were pallor and hepatomegaly. Painful crises occurred in 27, anaemic crises in 11, and a combination of these in 12 children. Infection was detected in 76% of subjects in crises. Infection was found in 82% of all the children and was mainly bacterial. The commonest infections were pneumonia (35%), bacteraemia (32%), tonsillitis/pharyngitis (17%) and osteomyelitis (8%). The predominant bacteria isolated were Klebsiella spp (38%), E. coli (23%), Staph. aureus (23%), Staph. albus (23%) and Pseudomonas spp (23%). Some children had multiple isolates. Bacterial infection was a major cause of morbidity in very young children and merits appropriate control and preventive measures in this age group. The spectrum of bacteria isolated makes it unlikely that the specific anti-pneumococcal measures widely advocated in Europe and America for young children with SCA would be appropriate in Nigeria.
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PMID:Acute illness in Nigerian children with sickle cell anaemia. 244 66

The clinical, haematological and biochemical features of 50 Sudanese patients with sickle cell disease (SCD) were determined. Of 23 patients with complete family data, 21 had sickle cell anaemia (homozygous HbSS), 2 had sickle-cell/beta+thalassaemia but none had sickle cell/beta Othalassaemia. The remaining 27 patients had HbSS phenotype. 84% of patients were from the Baggara tribe in western Sudan, where HbS is a natural extension of the west African HbS belt. 21 patients were children under 2 years old; 19 were 3-10 years old; and the remaining 10 were over 10 years old. Young patients presented mainly with painful vaso-occlusive crisis, severe anaemia, hand and foot syndrome, fever, underweight, malnutrition and various infectious diseases. All patients had mild to moderate cardiac enlargment; 42% had a moderately enlarged spleen but only 10% had an enlarged liver; 20% had infarctive lesions of long bones and another 8% had Salmonella osteomyelitis. Leg ulcers, priapism, enuresis and cholelithiasis were not observed. Patients had a mean haemoglobin concentration of 7.3 g/dl; reticulocyte count of 15.1%; serum bilirubin of 2.1 mg/dl; HbA2 level of 2.8% and HbF of 7%. Thus, the observed pattern of SCD in Sudan is comparable to the severe type described for Africans and not comparable to the benign form found in Shiite Moslem Arabs of Saudi Arabia. 6 adults with mild SCD had HbF levels below 5%. Amelioration of the disease, therefore, does not seem to be related to HbF levels; nor was it possible to relate it to high levels of erythrocyte 2,3-diphosphoglycerate.
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PMID:Sickle cell disease in Sudan. 245 19

Episodes of hepatic sequestration were seen in four patients with sickle cell anaemia (SCA). One case presented a severe and fatal sequestration crisis whereas the others showed mild episodes associated with less striking haematological and clinical changes. These clinical pictures are similar to those seen in the splenic sequestration crises of children with SCA. Different causes of liver enlargement in SCA suggest that the clinical spectrum representing intrahepatic trapping of blood could range from the acute sequestration crisis to chronic sequestration events which very probably should play any role in the pathogenesis of the hepatomegaly frequently found in these patients.
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PMID:Clinical features of hepatic sequestration in sickle cell anaemia. 258 96

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


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