Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

A follow-up study of 179 cases of human immunodeficiency virus (HIV) seropositive neonates born from HIV seropositive mothers is reported. At the time of the present study, HIV infection resulting from maternofetal transmission was found in 50 cases, while 108 infants were not infected; HIV infection remained uncertain in 16 cases; 5 infants were lost for follow-up. Out of the 50 infected cases, 20 were less than two-year old, 17 were 2-5 year old and 13 were older than 5 years. Very few remained asymptomatic after the age of 6 months, the most common symptoms being adenopathies and/or hepatomegaly and/or splenomegaly. Twenty-six had an acquired immunodeficiency syndrome (AIDS). Six died, from pneumocystosis (3), cytomegalovirus infection (1) and septicemia (2). Virus culture and polymerase chain reaction were the most efficient laboratory methods for early diagnosis of HIV infection, both being positive in more than 95% of the infected cases after the age of 3 months. A close clinical and biological supervision is recommended in these infants and children because of the permanent threat of infectious diseases in relation to their immunodeficiency. Treatment associates: 1) antiviral therapy with AZT as soon as the HIV infection is diagnosed; 2) primary prophylaxis against pneumocystosis with trimethoprim-sulfamethoxazol; 3) IV immunoglobulins in the case of repeated bacterial infection; 4) regular evaluation of the nutritional status and psychological assistance.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Management of HIV-seropositive newborn infants. Personal experience apropos of 179 cases]. 839 76

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

Intra hepatic inferior venacaval (IVC) obstruction at the site or just above hepatic vein opening in the IVC, is a common hepatic problem in Nepal. These patients either present as a chronic IVC obstruction with insidious onset or may present with rapid onset disease (acute/subacute disease). The former type may be asymptomatic (20%) or may have features of chronic liver dysfunction. The latter presents with rapid onset ascites. Presence of hepatomegaly with flank/back veins are characteristic clinical features of the disease. The disease is invariably encountered amongst rural poor, alcoholic and in the peripartum period. Ultrasonography, IVC graphy hepatogram and liver biopsies are the usual diagnostic modalities. Twenty four percent of the 126 chronic hepatic IVC disease had associated liver cell cancer at our centre. The aetiology of the disease is unclear. Presently it is believed that local thrombophlebitis in IVC causes such obstruction. Despite, surgical and radiological intervention, the ideal management in such patients is yet to be established. We believe conservative therapy, particularly prevention and early therapy of sepsis in such patients prolongs survival.
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PMID:Hepatic venous outflow obstruction in Nepal. 898 5

A 27-year-old woman with chronic renal failure, who had been treated with chronic ambulatory peritoneal dialysis and had developed sclerosing peritonitis, was admitted to the hospital with intra-abdominal sepsis. In spite of antibiotic therapy, sepsis recurred and was associated with intrahepatic cholestasis. In addition, over a period of about 4.5 weeks she developed hepatomegaly and portal hypertension unassociated with occlusion of the portal vein or one of its main extrahepatic branches. A wedge biopsy of the liver revealed extensive thick fibrosis of the liver capsule, intrahepatic cholestasis, diffuse swelling of hepatocytes, central veins that were difficult to visualize and small portal tracts. It is suggested that the sepsis was responsible for the intrahepatic cholestasis, swelling of hepatocytes and hepatomegaly. It is also suggested that the rigidity of the fibrotic liver capsule provided resistance to the development of hepatomegaly, with the result that intrahepatic pressure increased (compressing intrahepatic branches of the portal vein as well as portal tracts and central veins) and portal hypertension developed.
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PMID:Sclerosing peritonitis complicated by sepsis: a potential cause of portal hypertension. 951 60

Mother-to-child rates of HIV transmission are high in Africa. Findings are presented on 62 HIV-positive infants admitted to the Missionaries of Charity Orphanage, Addis Ababa, who were followed from July 25, 1991, to July 30, 1995. The infants were provided with regular clinical examination and treatment by a physician, as well as the monitoring of their HIV serostatus every 3 months until age 18 months and every year thereafter. Among infants over age 18 months, 14 were HIV seropositive and alive, and 4 were HIV positive, but died. 11 children were HIV positive and died before age 18 months and 33 seroreverted to HIV seronegative status. The level of mother-to-child HIV transmission was 29-47%. Among the clinical signs presented, generalized lymphadenopathy, hepatomegaly, splenomegaly, wasting, stunting, and delayed motor development were more often found in the definitely HIV-positive children. Upper respiratory tract infections, acute diarrhea, pneumonia, pyogenic skin infections, sepsis, and candidal infections were the most commonly seen illnesses.
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PMID:A four-year cohort study of HIV seropositive Ethiopian infants and children: clinical course and disease patterns. 957 11


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