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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifty infants with obstructive jaundice were reviewed. Other than jaundice, hepatomegaly and splenomegaly were the other common presentations. The mean age of referral was 9.4 weeks. This was unsatisfactory as the prognosis depends on the age of corrective surgery. Majority of the non surgical cholestasis were due to idiopathic hepatitis. These patients did well as all of them were asymptomic and had no evidence of liver disease at 2 years of age.
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PMID:A clinical study of obstructive jaundice among Singapore infants. 130 62

Seven women, mean age 47.7 years, with primary biliary cirrhosis (6 patients in the II-III stage and I patient in IV stage of the disease) were treated in the course of 16 months with ursodeoxycholic acid (Ursofalk) 500 mg daily. At the end of the 3-d month of treatment the itching had passed in 3 of the patients and in the remaining 4 patients it had substantially decreased. In all patients the subjective complaints, dyspeptic syndrome, appetite and sleep improved. The serum concentrations of bilirubin, copper and cholesterol started to decrease and the serum activity of the enzymes alkaline phosphatase, ALAT and ASAT also decreased. In one patient the treatment was discontinued in the 6-th month because of allergic reaction. After 16 month treatment in the 6 patients who completed the treatment the itching passed and the working capacity improved. The serum concentrations of bilirubin, cholesterol, copper and IgG significantly fell (p less than 0.01), the serum activity of alkaline phosphatase, gamma glutamyl transpeptidase, ALAT and ASAT fell near the upper normal range. The hepatomegaly, splenomegaly, McLagan's flocculation test, serum concentration of IgM and the titer of the specific antimitochondrial antibodies (M2) did not change in spite of the treatment. The results show the ursodeoxycholic acid as a perspective therapeutic means for primary biliary cirrhosis which lowers or overcomes the syndrome of intrahepatic cholestasis and limits the activity of the cirrhotic process in the liver. Ursodeoxycholic acid is well tolerated.
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PMID:[The treatment of primary biliary liver cirrhosis with ursodeoxycholic acid (preliminary report)]. 177 66

Although rather uncommon and multifactorial in etiology, liver cirrhosis is a severe and often rapidly fatal disease in pediatrics. In our institution, during the last 15 years, 22 children with liver cirrhosis have been followed. The underlying predisposing condition was HBV infection (8 cases), CMV perinatal infection (2 cases), Wilson's disease (4 cases), chronic cholestasis (2 cases) and alcohol abuse (2 cases); in 4 cases no predisposing condition was evident. In all cases the histological examination of the liver was the diagnostic cornerstone. The mean age at diagnosis was 6 years and 8 months, with an early onset especially in the posthepatitis cirrhosis. In 10 out of 22 patients, cirrhosis was not preceded by an history of chronic liver disease. Poor subjective symptomatology was present in 13 of the cases, hepatomegaly in all, splenomegaly in 18 cases, signs of hepatic failure in 13 cases. In all patients various impairments of hepatocellular synthesis were detectable, especially during the period preceding the development of hepatic insufficiency. The mean time to cirrhosis was 5 years. The average duration of the follow up was 3 years and 4 months: during the follow up 6 patients improved, 5 patients showed no clinical or functional modifications of their hepatic disease, 3 patients worsened and 8 died. In order to perform suitable treatment of liver cirrhosis the need of early diagnosis and etiological definition should be emphasized.
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PMID:[Liver cirrhosis in childhood. Considerations on 22 cases with different etiology]. 217 33

Because growth failure is a frequent complication of chronic liver disease in childhood, we examined the growth hormone/insulin-like growth factor type I axis and its relationship to growth disturbances, nutritional status, and carbohydrate metabolism in nine children (2.1 to 18.6 years of age) with chronic cholestatic liver disease. Seven had cholestasis associated with splenomegaly and histologic findings of cirrhosis; two patients had Alagille syndrome. Stature was less than or equal to 15th percentile in all except the youngest subject and less than 5th percentile in five subjects. Ten-hour, nocturnal, integrated serum concentrations of growth hormone were considerably higher in patients with cholestasis than in control subjects (mean +/- SD) 9.7 +/- 3.8 vs 4.7 +/- 1.9 ng/ml; p less than 0.02). Serum concentrations of insulin-like growth hormone type I were less than 95th percentile confidence intervals for age- and sex-matched norms in five patients and at the lower limits of normal in the remaining four patients. Insulin sensitivity, determined with the minimal model intravenous glucose tolerance test, was not decreased in five patients despite elevated levels of circulating growth hormone. The estimated mean caloric and protein intake exceeded the recommended dietary allowance and the weight-for-height index was greater than 90% for six of nine patients. Triceps and subscapular skin-fold thicknesses, indicators of body fat stores, were greater than 25th percentile for five of nine and eight of nine patients, respectively, suggesting deficient lipolytic action of GH. We conclude that children with cholestatic liver disease have a resistance to the growth-promoting, diabetogenic, and lipolytic properties of growth hormone.
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PMID:Resistance to the growth-promoting and metabolic effects of growth hormone in children with chronic liver disease. 239 94

A case of secondary syphilitic hepatitis is reported. A 49-year old woman was admitted for weight-loss, fever, hepatomegaly and splenomegaly. Diagnosis of syphilitic hepatitis was based on cholestasis associated with positive serologic tests for syphilis without other immunological disturbances. Biopsy of the liver showed a moderate infiltration in and around the portal tracts. Immunofluorescence study for treponema was negative. Rapid improvement was obtained with penicillin initially associated with steroid therapy. Liver involvement in secondary syphilis is characterized by anicteric cholestasis, an inflammatory syndrome, and periportal infiltrate inconstantly associated with centrilobular necrosis, granulomatous reaction and presence of treponemas in the lesions. Due to the increasing frequency of sexually transmitted diseases, this diagnosis could become more frequent.
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PMID:[Hepatic involvement in secondary syphilis]. 355 64

The paper is concerned with the results showing high diagnostic potentialities of i.v. liposome lienography for the detection of the splenomegalic syndrome in acute cholestasis, cholestatic hepatitis and disturbances of the extrahepatic portal blood flow. The authors managed to establish the dependence of the value of secondary splenomegaly on the period of cholestasis and a degree of occlusion of the portal vein. In acute cholestasis splenomegaly was manifested in 5 days by a two-fold increase in the spleen with reverse development within 2.5 mos. A rapid 2-fold increase in the spleen for 24 h was noted in occlusion of the portal vein by 40%, and a three-fold increase was noted in 5 mos. with subsequent death of the animals. A decrease in the extrahepatic portal blood flow by 25% also resulted in splenomegaly but without death of the animals. The authors have assumed that the use of i.v. liposome lienography ensures timely diagnosis of the splenomegalic syndrome and provides an opportunity to assess function of the reticuloendothelial system of the spleen.
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PMID:[Secondary splenomegaly in cholestasis and disorders of portal blood flow in an experiment detectable by intravenous liposome splenography]. 356 Dec 15

A personal case series triggers an examination of surgical risk in patients with uncomplicated cirrhosis of the liver. After a general introduction the conditions that increase surgical risk in cirrhotic patients are analysed. These include generally poor resistance, altered haemostasis, a tendency towards cholestasis, water retention and hepatic encephalopathy. The conditions most often requiring surgical treatment are then considered. They include associated pathologies (cholelithiasis, hernias, tooth extractions, bleeding haemorrhoids etc) and complications of cirrhosis variceal bleeding, intractable ascites, splenomegaly, hepatocytoma).
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PMID:[Internal medicine evaluation of the surgical risk in cirrhosis patients]. 382 13

Alpha-1-antitrypsin is a blood glycoprotein synthesized in the liver. It is a protease inhibitor of the serpine group and has a specific action for elastase. Alpha-1-antitrypsin electrophoresis shows about 20 phenotypes, the normal one being PiM. The allele PiZ is usually responsible for liver or lung disease in children or adults, respectively. Eleven per cent of PiZZ infants present with prolonged neonatal cholestasis. Twenty-five of 45 PiZZ infants with prolonged neonatal cholestasis presented with later cirrhosis. Persistence of jaundice beyond the sixth month of age, early development of splenomegaly, persistence of hard hepatomegaly and liver function abnormalities, and early portal fibrosis have a poor prognostic significance. The most severe course occurs in infants with an early histologic pattern of paucity of interlobular bile ducts. Portal hypertension was present in 19 of 25 children presenting with cirrhosis; 8 of 25 PiZZ children with cirrhosis died during childhood.
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PMID:[Alpha 1-antitrypsin deficiency in childhood]. 387 73

Two hundred and thirty six patients with amoebic liver abscess were investigated for cholestasis, its mechanism and the natural course of the disease. Cholestasis was seen in 29% of cases and it presented with some unusual features: it was frequently seen in young men (mean age 38.6 +/- 6.3 years) (87%) with acute onset (69%) and was associated with signs of peritonism, or peritonitis (28%), splenomegaly (12%) and hepatic encephalopathy (coma 13%). Raised diaphragm was seen only in 37% of cases. Alcoholism may have contributed to the cholestasis in 37% of cases. Multiple (43%) and single (32%) large liver abscesses, especially on the inferior surface of the liver (25%), were common in jaundiced patients with amoebic liver abscess, while size and number of abscesses were directly related to the raised serum bilirubin concentrations. Bromsulphalein excretion (BSP) was found to be significantly reduced (p less than 0.01) in patients with jaundice (60%). Retrograde injection of contrast media into the common bile duct during six necropsies showed compression by amoebic liver abscess on the hepatic ducts. The mortality (43%) and the complications were significantly higher (p less than 0.001) in patients with jaundice. The aspiration/surgical drainage of amoebic liver abscess together with a combination of metronidazole and di-iodohydroxyquinoline was more effective than either metronidazole alone, or dehydroemetine with chloroquine.
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PMID:Cholestasis in amoebic liver abscess. 396 31

Primary biliary cirrhosis (PBC) is a chronic nonsuppurative, destructive cholangitis, whose etiology is unknown. Morbidity arises early from pruritus and later from hypercholesterolemia with xanthoma formation. Therapy is supportive and directed at the complications of cholestasis. Plasmapheresis has been reported to benefit patients with hyperlipidemia and PBC; thus a pilot study of plasmapheresis utilizing the Haemonetics Model 30 with replacement by albumin and saline was conducted. Five patients (four female and one male) with a mean age of 43 (range 29-58) and a mean duration of illness of 9.5 years (range 6-21) with marked jaundice, xanthomas, xanthelasma, hepatomegaly, fatigability, anorexia, and pruritus, as well as mild nausea were studied. Peripheral neuropathy was present in two patients. Two patients had splenomegaly. Two patients had an associated Sjogren syndrome. All patients had high serum bilirubin, alkaline phosphatase, and cholesterol levels and mild elevations in aspartate amino transferase and alanine amino transferase activities. Immune complexes measured in four patients were present. Antimitochondrial antibody titers were significant in all patients. Patients underwent a mean of 63 plasmapheresis procedures over a mean of 112 weeks removing a mean of 94.7 liters of plasma. No serious toxicity was seen. All patients showed a reduction in pruritus, xanthomas, xanthelasmas, and serum cholesterol values. The two patients who had evidence of Sjogren syndrome noted subjective improvement. All patients who had fatigue, anorexia and nausea also noted moderate improvement. There was no change in hepatomegaly or splenomegaly in patients demonstrating such organomegaly. Liver function did not change significantly. Overall, four patients had improvement in their condition and one patient achieved stability.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The clinical effectiveness and safety of chronic plasmapheresis in patients with primary biliary cirrhosis. 403 Jul 9


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