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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a retrospective study, the authors examined the occurrence of hydrops in foetal and neonatal cases in a five-year period. During this time, the clinical and pathologic diagnosis of hydrops was established in 28 cases. In three cases, the hydrops was caused by Rh incompatibility, and in 25 cases non-immune hydrops was discovered by clinical and pathologic examination. The cause of hydrops was recognised in 25 cases and no underlying disease was discernible in three. In 12 cases, the pregnancy was spontaneously terminated. Artificial abortion was performed in four cases. In this series, four hydropic newborns died on the first week of their life. The average age in artificial abortion was 16-20 gestational week and 24 in spontaneous abortion or stillbirth. Pathologic examination revealed increased weight, oedema, ascites, and hydrothorax in all cases. In half of the cases, there was also hydropericardium. Hepatosplenomegaly, cardiomegaly, pulmonary hypoplasia, increased extramedullary hemopoiesis, and placenta oedema were seen in all the cases. Causes of the non-immune hydrops were cardiac malformation in 4 cases, chromosome anomaly in 3, cystic hygroma in 2, skeletal anomaly in 1, foeto-foetal transfusion in 3, infection in 7 and sacrococcygeal teratoma in 1 case. In two cases, the underlying disease was reported to be of maternal origin. Comparing the clinical and pathologic findings there were only three cases with absolute agreement. The underlying disease revealed by pathologic examination was regarded to be significant from respect of inheritance, therapeutic approach, and further management in 10 cases. The authors emphasise the importance of pathologic examinations in foetal hydrops and point out the significance of clinico-pathological collaboration in the decision about further management of hydropic foetuses.
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PMID:[Hydrops fetalis--a retrospective study]. 1735 Sep 12

Spontaneous bacterial empyema (SBEM) is infection of a preexisting pleural effusion without evidence of pneumonia. It has been reported mostly in patients with hepatic hydrothorax. Only 1 case of SBEM in a noncirrhotic patient has been reported. We present an unusual case of bilateral SBEM from Streptococcus pneumoniae bacteremia in a noncirrhotic patient. A 52-year-old man presented with bilateral pleuritic chest pain and dyspnea for 2 days. His medical history included congestive heart failure, hemodialysis-dependent renal failure and known bilateral pleural effusions. No ascites or hepatosplenomegaly was noticed. Bilateral pleural effusions were again present on physical examination and confirmed by a chest computed tomography scan. Cardiac medical treatment and hemodialysis failed to improve his condition. Bilateral thoracentesis revealed purulent pleural fluid that was culture-positive for Streptococcus pneumonia as were blood cultures. There was no clinical or radiographic evidence of pneumonia. The detailed clinical course, treatment and highlighted points are described.
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PMID:Spontaneous bacterial empyema in a noncirrhotic patient: an unusual scenario. 2182 62

Cholesterol ester storage disease (CESD) is a rare autosomal recessive lipid storage disorder associated with mutations of the gene encoding lysosomal acid lipase, manifestations of which include chronic liver disease and early atherosclerosis. Although normally presenting in childhood, severity is variable and the condition can occasionally remain undetected until middle age. Typical presentation is with asymptomatic hepatosplenomegaly and hyperlipidaemia, though the condition is probably underdiagnosed. Treatment is supportive and may include attention to cardiovascular risk factors. Phase I/II trials of enzyme replacement therapy are ongoing, but this approach remains experimental. We present the case of a 42-year-old woman diagnosed with CESD in childhood who ran an indolent course until re-presentation with cirrhotic hydrothorax. She underwent orthotopic liver transplantation but required re-transplantation for hepatic artery thrombosis. She remains well with excellent graft function 2 years later. Although atherosclerosis was apparent at assessment, and may have contributed to hepatic artery thrombosis, partial correction of the metabolic defect and restoration of liver function by transplantation together with ongoing medical therapy should permit reasonable survival over the longer term from both a liver and a vascular perspective. This is the first reported case of orthotopic liver transplantation for CESD in an adult, which was the only available option to improve survival. The case highlights the importance of monitoring patients with CESD through adulthood and suggests that liver replacement at a later stage may yet be indicated and remain of benefit.
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PMID:Orthotopic liver transplantation in an adult with cholesterol ester storage disease. 2343 May 18

Hepatic hydrothorax, a rare and debilitating complication of cirrhosis, carries high morbidity and mortality. First-line treatment consists of dietary sodium restriction and diuretic therapy. Some patients, mainly those who are refractory to medical management, will require invasive pleural drainage. The authors report the case of a 76-year-old man in a late cirrhotic stage of alcoholic chronic liver disease, presenting with recurrent right-sided hepatic hydrothorax, portal hypertension, hepatosplenomegaly and thrombocytopaenia. After recurrent admissions and complications, the potential for adjusting diuretic therapy was limited. After unsuccessful talc pleurodesis, an indwelling tunnelled pleural catheter was placed with effective symptomatic control. One month later, the patient was readmitted with empyema due to Acinetobacter radioresistens Despite optimised medical and surgical treatment, the patient died 4 weeks later.
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PMID:Hepatic hydrothorax: indwelling catheter-related Acinetobacter radioresistens infection. 3087 55