Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytosis X describes a disease characterized by histiocytic infiltration of the reticuloendothelial system, skin, bones, and pituitary gland. The disseminated form frequently occurs in infants and children. Chemotherapy has significantly improved the prognosis in this disorder. Sixty-three per cent of survivors, however, have some residual disability related to fibrosis of tissues previously infiltrated by histiocytes. In instances of liver involvement, healing by fibrosis may result in cirrhosis with portal hypertension and bleeding esophageal varices. Clinical findings include hepatosplenomegaly, jaundice, ascites, hypoalbuminemia, prolonged prothrombin time, and Bromsulphalein retention. Histologic examination of the liver shows a characteristic dense "macronodular" periportal cirrhotic pattern. Three children with portal hypertension and bleeding varices due to healed histiocytosis X were sucessfully managed by portosystemic shunt procedures. Portacaval, mesocaval, and central splenorenal shunts were equally effective in relieving poral hypertension. These children had neither recurrence of bleeding nor evidence of encephalopathy. Two children remain well whereas in one patient a primary hepatoma developed fourteen years posthung and he died of pulmonary metastases. Portosystemic shunt procedures effectively relieve the threat of potentially fatal variceal hemorrhage and improve the opportunity for long-term survival in children with cirrhosis and portal hypertension due to healed histiocytosis X.
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PMID:Portal hypertension in infants and children with histiocytosis X. 108 50

A 29-year-old male was diagnosed as having essential thrombocythemia (ET) in 1975. From that time, his platelet count gradually increased to more than 2 X 10(6)/microliter until 1979. However, his platelet count gradually decreased to less than 6 X 10(5)/microliter in 1985. Also, in 1982, erythroblasts and immature myeloid cells began to appear in the peripheral blood, and the liver and spleen became palpable in 1985. Bone marrow then revealed osteomyelosclerosis. These findings suggested that ET had transformed to myelofibrosis with myeloid metaplasia. Increased hepatosplenomegaly was accompanied by the appearance of ascites in June, 1988, and an esophageal varix ruptured in December of the same year. The varix was resected and the spleen was removed. After the operation, ascites did not recur and his condition became stable. Portal hypertension in this patient was considered to be due mainly to increased blood flow from the enlarged spleen.
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PMID:[Essential thrombocythemia transformed to myelofibrosis with myeloid metaplasia after seven years]. 204 Nov 65

We present a report of a pregnancy complicated by chronic schistosomiasis. A.H. is a 22 year old G1 Filipina, referred at 34 weeks gestational age for evaluation of hypersplenism and progressive thrombocytopenia. Physical exam was remarkable for hepatosplenomegaly. Laboratory evaluation of hepatic, renal, hematologic, autoimmune, or infectious disease etiology or abnormality was negative. Ultrasound revealed a normal fetus, an enlarged spleen, dilated splenic and hepatic vasculature and an enlarged liver with periportal fibrosis. Abdominal computed tomography revealed subcapsular calcifications, dilated ducts, and periportal fibrosis. Esophagogastrojejeunoscopy showed no varices. The presumptive diagnosis of hypersplenism secondary to chronic hepatosplenic schistosomiasis was made. Based on pathophysiology and literature review conservative management was elected. Liver functions, clotting functions, and platelet counts were monitored closely. Intense antepartum fetal monitoring was performed. Platelet counts remained between 30 and 40K. The patient went into labor at 38 3/7 weeks and delivered a 3148 g female Apgars 8/9 by spontaneous vaginal delivery. Evaluation of the placenta was negative for ova. By 2 weeks postpartum platelet counts had increased to the 60-70 k range, and a rectal biopsy, positive for S. japonicum ova was performed. Treatment with three doses of Praziquantel 20 mg/kg q8h was completed prior to her return to the Philippines.
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PMID:Hepatosplenic schistosomiasis in pregnancy: report of a case and review of the literature. 212 Nov 52

Portal hypertension in chronic lymphocytic leukemia (CLL) is rare. A 64-year-old woman with CLL for 5 years and increasing hepatosplenomegaly developed portal hypertension and bleeding gastric varices. There was no portal vein thrombus by abdominal echography and angiography. Following splenectomy and devascularization of the fornix, the gastric varices disappeared. The liver biopsy showed dense leukemic cell infiltration in portal triads, but no fibrosis. The portal hypertension in this case may be mainly due to increased portal flow from the enlarged spleen and leukemic cell infiltration in the liver. Previously reported cases are summarized.
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PMID:[Portal hypertension in chronic lymphocytic leukemia]. 269 62

Twenty-seven children with congenital hepatic fibrosis were followed for three months to 12 years. Hepatosplenomegaly, normal liver function tests, and kidney abnormalities were present in most patients, indicating that a correct diagnosis of congenital hepatic fibrosis could be made using simple clinical, biologic, and radiologic criteria. Esophageal endoscopy showed varices in 21 patients. Sixteen children underwent portal-systemic shunt surgery. Follow-up examinations did not show any impairment of liver function or any sign of hepatic encephalopathy. Cholangitis was present in only three children.
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PMID:Congenital hepatic fibrosis in children. 726 89

Presentation of autosomal recessive polycystic kidney disease (ARPKD) ranges from severe renal impairment and a high mortality rate in infancy to older children and adolescents with minimal renal disease and complications of congenital hepatic fibrosis (CHF), cholangitis and portal hypertension. Renal transplantation improves prognosis but it is unclear whether CHF in transplanted children follows the same clinical course as in older children with less severe renal disease. The aim of this study was to evaluate morbidity from CHF in ARPKD post renal transplantation. Data were analyzed for six males and eight females, transplanted for ARPKD (mean age 8.3 years, range 1-22.3 years) at the University of Minnesota between 1972 and 1998. Follow-up was for a mean of 14.5 years (range 3.1-33.6 years). One and 5 years patient survival rates were 93% and 86%, respectively. Overall five patients (36%) died; 4/5 deaths were related to CHF. Causes of death were hepatic failure immediately post transplant (n = 1), septicemia related to bile duct dilatation (n = 3) and multiorgan failure (n = 1). One and 5years graft survival rates were 87% and 70%, respectively. One patient had a combined liver-kidney transplant and two were re-transplanted. Initial signs of CHF were splenomegaly (n = 5), hepatosplenomegaly (n = 4) and gastrointestinal bleed (n = 2). Progression of CHF through childhood included hypersplenism (n = 7), esophageal varices with gastrointestinal bleeding (n = 5) and bile duct dilatation (n = 5). Portal hypertension was treated with portosystemic shunt (n = 3), sclerotherapy (n = 2), banding of varices (n = 1) and transjugular intrahepatic portosystemic shunt (n = 1). Of the nine survivors (mean age 12.8 years) 78% have functioning grafts (one liver-kidney transplant), 63% have portal hypertension and 22% have asymptomatic biliary dilatation. Complications of CHF developed in 79% of children who received a renal transplant for ARPKD. Mortality related to CHF occurred in 29% and accounted for 80% (4/5) of the deaths.
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PMID:Morbidity from congenital hepatic fibrosis after renal transplantation for autosomal recessive polycystic kidney disease. 1211 59

The treatment of choice, in the Northeast of Brazil, of patients with a history of upper GI bleeding from ruptured esophageal varices (EV) and with hepatosplenomegaly secondary to schistosomiasis (HSS), is splenectomy and left gastric vein ligation (SLGL). However, the effect of this procedure on the EV pressure, the parameter that best correlates to re-bleeding risk, has not yet been evaluated. With the introduction of a minimally invasive technique to measure the EV pressure, it has become possible to assess the effect of this surgery without an increased risk to the patient. SLGL was performed in twenty two patients with a history of HSS and upper GI Bleeding secondary to esophageal varices. The non-invasive endoscopic pneumatic balloon was used to measure the EV pressure before surgery and the results were then compared with measurements made between five and eight days post-operatively. The pre-operative EV pressure ranged from 20.0 mmHg to 28.7 mmHg (mean 24.35 +/- 2.36 mmHg), with no correlation between the pressure and the calibre of the varices. In the post-operative period, a significant decrease in EV pressure was observed, ranging from 14.6 mmHg to 21.5 mmHg (mean 17.29 +/- 1.75 mmHg, p < 0.001). These results support the use of SLGL in patients with HSS and a history of variceal bleeding. The operation results in, at least for the short term and in the majority of cases, a reduction in the EV pressure, and therefore a reduced risk of repeating upper GI Bleeding.
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PMID:Splenectomy and ligation of the left gastric vein in schistosomiasis mansoni: the effect on esophageal variceal pressure measured by a non-invasive technique. 1212 10

The long-term effect of single-dose praziquantel on morbidity and mortality from Schistosoma mansoni was investigated in surveys in 1987 and 1994 in central Sudan. Prevalence of infection dropped from 53% to 34%, and intensity of infection (> or = 400 eggs/g of faeces) from 31% to 18%. There was a reduction in hepatomegaly and hepatosplenomegaly, although splenomegaly alone was unchanged. Prevalence of periportal fibrosis decreased from 14% to 10%. Endoscopic investigation of patients with fibrosis showed a reduction in oesophageal varices from 47% to 30%. Mortality due to bleeding varices was high (community-wide, up to 11/100 infected patients with bleeding). Thus praziquantel mass treatment can be spaced to a much longer period, reducing the expense of treatment, delivery and distribution.
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PMID:Effects of single-dose praziquantel on morbidity and mortality resulting from intestinal schistosomiasis. 1219 50

Klippel - Trenaunay syndrome (KTS) is characterized by a cutaneous vascular nevus of the involved extremity, bone and soft tissue hypertrophy of the extremity and venous malformations. We present a case of KTS with splenic hemangiomas and rectal varices. A 29-year-old woman was referred for intermittent hematochezia for several years. She had history with a number of operations for cutaneous and soft tissue hamangiomas since the age of one year old and for increased circumference of her left thigh during the last few months. Abdominal CT revealed multiple hemangiomas in the spleen, fusiform aneurysmal dilatation of the deep veins and soft tissue hemangiomas. There was no evidence of hepatosplenomegaly or liver cirrhosis. Colonoscopy revealed hemangiomatous involvement in the rectum. There were rectal varices without evidence of active bleeding. Upon venography of the left leg, we also found infiltrative dilated superficial veins in the subcutaneous tissue and aneurysmal dilatation of the deep veins. The patient was finally diagnosed with KTS, and treated with oral iron supplementation only, which has been tolerable to date. Intervention or surgery is not required. When gastrointestinal varices or hemangiomatous mucosal changes are detected in a young patient without definite underlying cause, KTS should be considered.
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PMID:[Involvement of splenic hemangioma and rectal varices in a patient with klippel: trenaunay syndrome]. 2196 Jan 5

Management of a patient with portal hypertension and hypersplenism who is scheduled for elective surgery is a challenge for anesthesia providers. We report a case that was scheduled for elective surgery in which preoperative splenic artery embolization improved the hematologic parameters. A 32-year-old woman was scheduled for total abdominal hysterectomy for multiple fibroids and menorrhagia with pancytopenia. She also had diagnoses of chronic liver disease with cirrhotic changes, portal hypertension, and grade IV Manageesophageal varices with hepatosplenomegaly. In view of pancytopenia, splenic artery partial embolization was done preoperatively. Her hematologic parameters improved, and she underwent surgery under general anesthesia with intrathecal morphine analgesia, uneventfully. The condition of patients with portal hypertension with associated hypersplenism and pancytopenia who are scheduled for elective surgery can be optimized with preoperative partial splenic artery embolization.
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PMID:Role of splenic artery partial embolization in a patient with portal hypertension and pancytopenia undergoing hysterectomy under anesthesia. 2258 77


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