Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 33-yr-old Puerto Rican women was hospitalized for chemotherapy and multiple antibiotic treatment for relapse of acute myelomonocytic leukemia. While she was already receiving amphotericin for suspected Aspergillus infection, she developed hepatomegaly and abnormal liver enzymes with high serum bilirubin. The blood cultures were negative. Percutaneous liver biopsy revealed granulomatous fungal hepatitis identified by cultures as Trichosporon cutaneum. In spite of the continued administration of amphotericin, with the addition of 5-fluorocytosine, Trichosporon was later cultured from her blood, and she succumbed to fungemia and polymicrobial sepsis.
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PMID:Trichosporon hepatitis. 657 26

The purpose of this study has been to provide information on the mortality and morbidity rates for operation on nonbleeding cirrhotic patients and to identify factors that portend a grave prognosis. A review of 102 cirrhotic patients who underwent a variety of major therapeutic operations revealed a mortality rate of 19.6 percent. Mortality rates were significantly increased (p less than 0.05) by emergency operation (45.8 percent), gastrointestinal related operation (27.6 percent), ascites (37.5 percent), a bilirubin concentration greater than 3.5 mg (44.4 percent), a prothrombin time increase greater than 2 seconds (36.1 percent), a partial thromboplastin time increase greater than 2 seconds (50 percent), an alkaline phosphatase concentration greater than 70 units (40.9 percent), an operative blood loss greater than 1,000 ml (33.3 percent), and the presence of one or more postoperative complications (39.6 percent). Mortality rates were not increased after extremity, genitourinary, or gynecologic operations, an albumin concentration less than 3 g, a serum glutamic oxalacetic transaminase concentration greater than 40 units, hepatomegaly, and a history of previous gastrointestinal bleeding. When significant risk factors were added, mortality rates were significantly associated (p less than 0.001): zero to one factors 5.1 percent, two to three factors 19.4 percent, four to five factors 33.3 percent, and more than six factors 66.7 percent. The complication rate was 47.1 percent and included liver failure (42.2 percent), sepsis (18.6 percent), and bleeding (8.8 percent). Thus, in cirrhotic patients a clear need for operation must exist, liver function must be optimized preoperatively, and the most simple and expeditious procedure must be performed to avoid excessive blood loss and postoperative complications.
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PMID:Morbidity and mortality after operation in nonbleeding cirrhotic patients. 660 65

The records of 52 patients with amyloidosis admitted to Groote Schuur Hospital, Cape Town, between January 1969 and August 1982 were analysed. The male: female ratio was 1,3:1 and the mean age was 49,3 years. Forty-eight per cent of the patients had secondary amyloidosis, 21% had primary amyloidosis, 11,5% had localized amyloidosis and 11,5% had amyloidosis associated with multiple myeloma. Tuberculosis, chronic pulmonary sepsis and other chronic infections were present in 88% and rheumatoid arthritis in 16% of the patients with secondary amyloidosis. The commonest features at diagnosis were proteinuria (70%), oedema (52%) and hepatomegaly (39%). The diagnosis of amyloidosis was established by renal, liver and rectal biopsy (either singly or in combination) in 82% of cases. The prevalence of amyloidosis at autopsy was 0,28%.
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PMID:Amyloidosis at Groote Schuur Hospital, Cape Town. 674 Apr 24

Six infants with disseminated HSV had no mucocutaneous lesions at any time during the course of the illness. These infants presented with lethargy, poor feeding, apnea, acidosis, and hepatomegaly. The diagnosis of HSV was made by culturing the infant's oropharynx and blood, and the maternal cervix. Eight infants with HSV encephalitis had no skin, eye, or mucous membrane lesions. These infants presented with lethargy and low-grade fever, followed within 24 hours by the onset of focal partial motor seizures. The seizures were refractory to anticonvulsant therapy. The mean CSF white cell count was 131 cells/mm3;the glucose and protein concentrations were in the normal range. Brain biopsy was required for the early diagnosis of HSV encephalitis. These 14 cases presented 70% (14/20) of all infants with neonatal HSV diagnosed during the study period. HSV infection should be considered in infants with no mucocutaneous lesions who have signs usually associated with bacterial sepsis or who develop focal seizures during the first three weeks of life.
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PMID:Neonatal herpes simplex infection in the absence of mucocutaneous lesions. 706 32

Five cases of hepatic haemangioma are described, and a sixth (previously reported) is reviewed. Clinical features, investigation, and management are described to show the great variability of the complications and prognosis. Five children presented in the first 10 weeks of life with hepatomegaly; 4 developed congestive cardiac failure; 3 had cutaneous haemangiomata. One child presented at age 4 years with hepatomegaly and anaemia, and on investigation had features of chronic disseminated intravascular coagulation. Focal decrease or patchiness in hepatic uptake of technetium-99m colloid, and abnormal intrahepatic circulation was shown in all cases. In 3 children liver biopsy was performed to exclude malignant disease. In one patient there was spontaneous regression of the tumour by age 3 years. In 3 cases hepatic artery ligation was necessary to control congestive cardiac failure which had persisted despite treatment with digoxin, diuretics, and oral corticosteroids, a procedure which was without complications after up to 8 years. One infant with intractable portal hypertension, hepatic vein obstruction, and severe cholestasis died with persisting alimentary haemorrhage and intra-abdominal sepsis. One child aged 4 years showed no immediate response to hepatic artery ligation but the size of her tumour got smaller and the clinical features diminished after irradiation. These tumours cause considerable morbidity and have a high reported mortality. If congestive cardiac failure is not rapidly controlled, hepatic artery ligation should be performed.
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PMID:Hepatic haemangiomata: diagnosis and management. 746 56

Peliosis is an uncommon condition characterized by multiple-blood-filled cavities mostly involving the liver. Although the etiology is unknown the condition may be associated with several disease states and medications. We report the MR findings of peliosis hepatis in a patient with Fanconi anemia who had been treated with anabolic androgenic steroids for 3 years. The MR examination of the upper abdomen was performed on a 0.5 T system. The signal intensity of the right lobe of the liver was diffusely increased in all sequences. Within the enlarged liver, multiple foci of brighter signal were seen involving both right and left lobes. The lesions showed contrast enhancement. A cystic cavity with an enhancing rim was seen representing a haematoma cavity. The spleen was spared the patient died of sepsis and the postmortem examination confirmed the diagnosis of peliosis hepatis.
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PMID:MR findings in peliosis hepatis. 776 Nov 58

Life-threatening portal hypertension (PHN) in patients with chronic myeloproliferative disorders may result from increased portal flow caused by marked splenomegaly or an increased resistance to portal flow from either a large vein thrombosis or an intrahepatic obstruction usually associated with agnogenic myeloid metaplasia (AMM). The former cause is correctable by splenectomy alone, whereas the latter requires portal-systemic shunt surgery. Few data exist regarding the outcome of portal-systemic shunt surgery in patients with AMM and intrahepatic obstruction. During the past 25 years, 13 patients with chronic myeloproliferative disorders underwent portal-systemic shunt surgery at our institution. The cause of PHN was intrahepatic obstruction in ten patients and hepatic vein thrombosis in three. Ten of the thirteen patients had AMM as initial diagnosis. Only one patient had intraoperative complications, and four patients had either sepsis or thrombosis during the postoperative period. Twelve patients survived the postoperative period and had a median postsurgical survival of 3 years (range, 0.25 to 19 years). The long-term complications of the operation were very few and included hepatic encephalopathy (one patient), portal vein thrombosis (one patient), and shunt occlusion (one patient). The procedure was successful in alleviating complications of PHN in all but one patient. Deterioration of hepatic function and subsequent hepatomegaly were unusual. Portal-systemic shunt surgery seems to be a useful option in patients with AMM and life-threatening PHN from intrahepatic obstruction.
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PMID:Outcome of portal-systemic shunt surgery for portal hypertension associated with intrahepatic obstruction in patients with agnogenic myeloid metaplasia. 803 84

Infected right atrial thrombosis is an uncommon but severe complication of the use of central indwelling catheters in children. Massive thrombi around a right atrial catheter were seen in two critically ill premature infants after 3 weeks of parenteral nutrition. A catheter-related sepsis had previously occurred and had been treated by antibiotics. Subsequently, protracted thrombocytopenia, fluctuating hepatomegaly, and increasing respiratory distress were found in both cases. A right atrial mass was detected by 2 dimensional echocardiography. Cardiotomies for removal of the infected thrombus were performed with success in both cases. In one infant, weighing only 900 gm, surgical removal was accomplished with the aid of inflow stasis. Premature infants with catheter-related sepsis appear at greater risk for intracardiac thrombosis and should undergo echocardiography as part of their evaluation. Infected atrial thrombi can be safely removed by cardiotomy even in the very small premature infant.
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PMID:Diagnosis and management of catheter-related infected intracardiac thrombosis in premature infants. 844 97

Classical galactosaemia, deficiency of galactose-1-phosphate uridyltransferase (GALT), is characterized by acute symptoms of hepatomegaly, jaundice, sepsis, cataracts and growth retardation. Treatment with dietary galactose restriction corrects these complications immediately; however, most of these children develop long-term complications of verbal dyspraxia, mental retardation and ovarian failure. Our previous molecular study showed that the most common mutation of the GALT gene is a missense mutation of Q188R (replacement of glutamine-188 by arginine) in approximately 60-65% of the German galactosaemic population. The coding region of GALT was amplified by the polymerase chain reaction from genomic DNA of classical galactosaemic individuals, who are negative or heterozygous for Q188R, and was further characterized by direct sequencing. Three new disease-causing mutations, two missense and a stop codon mutation, were identified in three patients from two families with mild galactosaemic variants: firstly R67C, replacement of arginine-67 by cysteine and W316X, the stop codon at tryptophan-316 in one male; secondly A330V, replacement of alanine-330 by valine in two female siblings. In the first family the patient was also heterozygous for the polymorphism N314D and in the second family both girls were compound heterozygotes for Q188R and A330V. All three galactosaemic individuals have a considerable amount of the residual GALT activity in RBC and the galactose-1-phosphate (GALP) level decreased much faster on treatment than that of other galactosaemic patients with missense mutations such as Q188R. The clinical and biochemical data of these patients were much more favourable in comparison with those of two female galactosaemic individuals, one homozygous for L195P and the other compound heterozygous for Q188R and L195P. These three missense mutations (R67C, L195P and A330V) also occur in highly conserved regions. These observations suggest that the phenotypic variation in galactosaemic individuals may be due to different molecular aetiologies.
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PMID:Mutations in the galactose-1-phosphate uridyltransferase gene of two families with mild galactosaemia variants. 859 37

Two children with double aortic arch and aortoesophageal fistula (AEF) are reported to warn of this lethal complication of double aortic arch and to stress important points in the diagnosis and management. A review of the records of 30 children with double aortic arch disclosed two patients who had AEF. The first patient had respiratory distress and repair of a vascular ring (double aortic arch) at 5 weeks of age. At 9 weeks of age, because of difficulty with tracheal extubation, aortopexy was performed. Ten days later, profuse upper gastrointestinal bleeding required control by a Sengstaken-Blakemore (SB) tube. Thoracotomy and repair AEF was accomplished successfully under cardiopulmonary bypass. The second patient had hepatomegaly and Pseudomonas sepsis. Endotracheal and nasogastric intubation was necessary, and subsequently the double aortic arch was demonstrated by magnetic resonance imaging (MRI). On the 48th day of hospitalization, life-threatening upper gastrointestinal hemorrhage required insertion of an SB tube. Cardiopulmonary bypass allowed successful repair of the AEF. Both children are alive, after 3 and 2 years (respectively). These patients demonstrate that AEF must be diagnosed clinically (no imaging technique is effective); its history and physical presentation are typical. The SB tube is effective for controlling the hemorrhage until cardiopulmonary bypass can be performed to allow repair.
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PMID:Aortoesophageal fistula and double aortic arch: two important points in management. 880 21


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