Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The paper describes one case of the mixed form of mucoviscidosis, with involvement of the lung (chronic obstructive bronchitis, bronchiectasis, emphysema, pneumosclerosis), liver (cirrhosis), pancreas (lipomatosis with islet atrophy), ileum (enteritis). There were the following complications: cor pulmonale, esophageal varicose veins, ascitis, splenomegaly, secondary diffuse renal amyloidosis of the kidney, adrenals, and spleen, and brain edema. Due to modern therapy the patient reached the age of 25 years, which is a rarity.
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PMID:[Pathomorphology of the mixed form of mycoviscidosis in an adult patient]. 1683 Jun 24

Spontaneous splenorenal shunt is a rare condition, sometimes causing complications in cirrhotic patients. We report a 30-year old man with liver cirrhosis, hypertrophic caudal lobe and spontaneous splenorenal shunt. Real-time and color Doppler ultrasonography evidenced enlarged caudal lobe (130 x 95 mm) with direct veins draining into dilated inferior cava vein (diameter 25 mm, flow 52 cm/sec). In the left renal hilus a large vein with a flow typical for portal vein system was found, velocity 25-37 cm/sec. Indirect splenoportography noticed splenomegaly, dilated lienal and portal vein with hepatopetal blood flow, perisplenic varices, and large spontaneous splenorenal shunt. Whole inferior caval vein was dilated, while hepatic veins were intact. Hemodynamic consequences of this large shunt were dilation of inferior cava vein with hyperkinetic systemic flow, and secondary hypertrophy of liver caudal lobe.
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PMID:Spontaneous splenorenal shunt in a patient with liver cirrhosis and hypertrophic caudal lobe. 1741 Mar

We describe ultrasonographic and computed tomographic features of hepatic lesions in two cases of disseminated Langerhans' cell histiocytosis affecting children. In the first case, hyperechoic band like periportal lesions were observed at ultrasonography, which on computed tomography was found to be hypodense admixed with fatty attenuation (HU@23 to - 57) at places. In addition, the caudate lobe was very prominent. In the second case, the hepatic parenchyma showed predominantly hyperechoic diffusely heterogeneous echogenicity. There were features of cirrhosis of liver with portal hypertension in the form of atrophy of right lobe with hypertrophy of left lobe of liver with lobulated outline, prominent main portal vein and splenoportal axis, splenomegaly and gastroesophageal varices. Both the patients were put on chemotherapy as per schedule (Protocol: DAL HX - 83) and are on follow up.
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PMID:Imaging findings in hepatic Langerhans' cell histiocytosis. 1712 88

In this article, we present the CT and MR imaging characteristics of the cirrhotic liver. We describe the altered liver morphology in different forms of viral, alcoholic and autoimmune end-stage liver disease. We present the spectrum of imaging findings in portal hypertension, such as splenomegaly, ascites and varices. We describe the patchy and lacelike patterns of fibrosis, along with the focal confluent form. The process of hepatocarcinogenesis is detailed, from regenerative to dysplastic nodules to overt hepatocellular carcinoma. Different types of non-neoplastic focal liver lesions occurring in the cirrhotic liver are discussed, including arterially enhancing nodules, hemangiomas and peribiliary cysts. We show different conditions causing liver morphology changes that can mimic cirrhosis, such as congenital hepatic fibrosis, "pseudo-cirrhosis" due to breast metastases treated with chemotherapy, Budd-Chiari syndrome, sarcoidosis and cavernous transformation of the portal vein.
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PMID:Cirrhosis: CT and MR imaging evaluation. 1714 54

Congenital hepatic fibrosis (CHF) is a rare congenital multisystemic disorder, mostly inherited in autosomal recessive fashion, primarily affecting renal and hepatobiliary systems. Main underlying process of the disease is the malformation of the ductal plate, the embryological precursor of the biliary system, and secondary biliary strictures and periportal fibrosis ultimately leading to portal hypertension. The natural course of the disease is highly variable ranging from minimally symptomatic disease to true cirrhosis of the liver. However, in most patients the most common manifestations of the diseases that are related to portal hypertension, particularly splenomegaly and bleeding varices. Many other disease processes may co-exist with the disease including Caroli's disease, choledochal cysts and autosomal recessive polycystic kidney disease (ARPKD) reflecting the mulstisystemic nature of the disease. The associating biliary ductal disease led the authors to think that all these entities are a continuum and different reflections of the same underlying pathophysiological process. Although, conventional method of diagnosis of CHF is the liver biopsy the advent of imaging technologies and modalities, today, may permit the correct diagnosis in a non-invasive manner. Characteristic imaging features are generally present and recognition of these findings may obviate liver biopsy while preserving the diagnostic accuracy. In this article, it is aimed to increase the awareness of the practising radiologists to the imaging findings of this uncommon clinical disorder and trail the blaze for future articles relating to this issue.
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PMID:Imaging findings in congenital hepatic fibrosis. 1716 79

Although stomal varices are a rare complication, bleeding stomal varices often need to be treated owing to symptoms of hypovolemic shock, recurrence of stomal bleeding, or deterioration in the quality of life. Various treatment strategies for the management of bleeding stomal varices have thus far been reported. We report the case of a 60-year-old woman with refractory recurrent bleeding from varices in a sigmoid stoma, along with nonalcoholic steatohepatitis and marked splenomegaly. A physical examination revealed that the skin was discolored and bluish around the circumference of the sigmoid stoma. The venous phase of a celiac arteriogram revealed an afferent vein from the splenic vein and another from the inferior mesenteric vein, and veins draining into the left superficial epigastric vein. A balloon-occluded retrograde transvenous obliteration (BRTO) procedure was performed. The skin around the stoma, initially discolored bluish, improved markedly. After 10 months of follow-up, the patient has remained well without further episodes of stomal bleeding. To our knowledge, this is the first case of recurrent hemorrhage from stomal varices that was successfully treated by BRTO in a patient with portal hypertension.
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PMID:Treatment of bleeding stomal varices by balloon-occluded retrograde transvenous obliteration. 1732

Left-sided portal hypertension is a rare clinical syndrome which may lead to bleeding from isolated gastric varices. Pancreatic disease is the most common etiology. Left-sided portal hypertension should be considered in the presence of gastrointestinal bleeding with normal liver function and unexplained splenomegaly. It may be difficult to diagnose this entity both endoscopically and radiologically. While splenectomy is the treatment of choice for cases complicated by variceal bleeding, there is no consensus on the treatment of asymptomatic patients. The prognosis of left-sided portal hypertension mainly depends on the underlying etiology.
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PMID:Left-sided portal hypertension. 1793 55

Congenital hepatic fibrosis (CHF) is an autosomal recessive disorder that belongs to the family of fibropolycystic liver diseases. This family includes a spectrum of disorders which are usually found in combination with each other and are usually inherited. Clinically fibropolycystic diseases have three effects being present in different proportions, those of a space occupying lesion, of portal hypertension and of cholangitis. In most patients, the first manifestations of CHF are signs and symptoms related to portal hypertension such as splenomegaly and varices. Portal hypertension in these patients has been attributed to the hypoplasia or compression of the portal vein radicles in the fibrous bands. Cavernous transformation of the portal vein (CTPV) is a relatively rare condition resulting from extrahepatic portal vein obstruction with recanalization or collateral vein formation to bypass the obstruction. It has been found that patients with CHF having an accompanying CTPV have relatively large splenomegaly and suffers more frequent episodes of bleeding from esophageal varices. We believe that CTPV is a congenital component of CHF and also one of the important causative factors of portal hypertension in these patients.
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PMID:Is portal vein cavernous transformation a component of congenital hepatic fibrosis? 1746 91

Splenomegaly was discovered by chance in a 9-year-old boy who had no further complaints. Apart from splenomegaly and mild thrombocytopenia, no other pathological condition was found in the first instance. Ultrasound revealed a spleen with a median length of 16.7 cm. Blood tests remained stable and a bone marrow biopsy showed no pathology either. Doppler ultrasound of the splenic vessels was normal; screening for a coagulation disorder was not performed. A wait-and-see policy was instituted. Later, the patient developed haematemesis because ofoesophageal varices. It turned out that he had portal hypertension caused by thrombosis of the portal vein. The underlying cause was a heterozygous protein-C deficiency. Treatment consisted of anticoagulant therapy and a mesocaval shunt procedure. Splenomegaly is an atypical clinical manifestation in childhood. Coagulation disorders should be considered in children with splenomegaly.
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PMID:[Clinical reasoning and decision making in practice. A 9-year-old boy with isolated splenomegaly]. 1826 5

Partial splenic embolization (PSE) is a non-surgical procedure developed to treat hypersplenism as a result of hepatic disease and thus avoid the disadvantages of splenectomy. A femoral artery approach is used for selective catheterization of the splenic artery. Generally, the catheter tip is placed as distally as possible in an intrasplenic artery. After an injection of antibiotics and steroids, embolization is achieved by injecting 2-mm gelatin sponge cubes suspended in a saline solution containing antibiotics. PSE can benefit patients with thrombocytopenia, esophagogastric varices, portal hypertensive gastropathy, encephalopathy, liver dysfunction, splenic aneurysm, and splenic trauma. The contraindications of PSE include secondary splenomegaly and hypersplenism in patients with terminal-stage underlying disease; pyrexia or severe infections are associated with a high risk of splenic abscess after PSE. Complications of PSE include daily intermittent fever, abdominal pain, nausea and vomiting, abdominal fullness, appetite loss, and postembolization syndrome. Decreased portal-vein flow and a rapid increase in the platelet count after excessive embolization may cause portal-vein or splenic-vein thrombosis.
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PMID:Partial splenic embolization. 1803 10


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