Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032285 (pneumonia)
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This paper gives, in detail, the causes of either liver disease or hepatomegaly in 100 patients, mostly adults, admitted to the medical wards of Angau Memorial Hospital, Lae, during 1968 and 1969. The major findings included liver cell carcinoma, cirrhosis (often with chronic active hepatitis), tropical splenomegaly, pericholangitis and hepatitis. There were 27 with miscellaneous findings including ten with normal, or almost normal, livers despite the definite enlargement. Patients with liver cell carcinoma presented late in the course of their illness and had a poor prognosis. Others, with pericholangitis, had clinical features of portal hypertension indistinguishable from that complicated cirrhosis. There was an unexpected number with chronic active hepatitis and a liver biopsy is essential for such a diagnosis. Hepatic sinusoidal lymphocytosis is almost invariably found in patients with TS but may occasionally be found in those with a non-palpable spleen. Patients with right heart failure of chronic respiratory disease, and jaundice of acute pneumonia were excluded from the study.
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PMID:Liver disease in Papua New Guinea. 19 19

Budd-Chiari syndrome is an unusual form of portal hypertension, resulting from partial or total obstruction of the major hepatic veins or of inferior vena cava. This is illustrated by the clinical history of a two year old girl admitted by anemia, hepatomegaly, thrombocytopenia, pleural effusion, ascites and ultrasonographic, scintigraphic and angiographic evidence os suprahepatic veins and inferior vena cava obstruction. Liver biopsy showed signs of chronic congestion and fibrosis. Initial manifestation of illness may be acute or, as in this patient, with an insidious onset of symptoms. Medical therapy gives usually poor results. Several surgical approaches have been tried to alleviate liver congestion, which in this case was intended by a 6 mm wide Goretex ringed tube prosthesis extending from superior mesenteric vein throughout right atrium. Patency of the artificial shunt was proven after several days by Doppler ultrasound but the patient died of pneumonia two months after operation.
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PMID:[Budd-Chiari syndrome]. 184 28

A 24-year-old Japanese man presented with dyskeratosis congenita (DC, Zinsser-Cole-Engman syndrome) complicated by non-cirrhotic portal hypertension, signet ring carcinoma of the rectum and Pneumocystis carinii pneumonia. At the age of 9 years, he was diagnosed as having DC on the basis of typical clinical manifestations including atrophic lingual papillae, hyperpigmentation of the skin, thrombocytopenia, and ophthalmological abnormalities. A few years later pancytopenia and splenomegaly developed. At 24 years, signet ring carcinoma of the rectum was detected but could not be resected because of the severity of the pancytopenia. Death was due to respiratory failure from P. carinii pneumonia. At autopsy the case illustrated several unique findings for DC, including non-cirrhotic portal hypertension, atrophy of frontal lobe and markedly slender folia of the cerebellum and superimposed infections with herpes zoster virus and P. carinii. Striking lymphocyte depletion and atrophy of lymphoid parenchyma in lymph nodes, tonsils, spleen, gastrointestinal tract, or thymus were seen histologically. The morphological picture supports the suggestion that there is a defect in the cell-mediated immune system in patients with DC, although immunoglobulin levels in the blood are normal. The cell-immune deficiency is a major factor in the poor prognosis.
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PMID:Dyskeratosis congenita (Zinsser-Cole-Engman syndrome). An autopsy case presenting with rectal carcinoma, non-cirrhotic portal hypertension, and Pneumocystis carinii pneumonia. 216 77

Endoscopic variceal sclerotherapy (EVS) is an effective means of controlling variceal hemorrhage, which develops as a consequence of portal hypertension. While esophageal perforation, ulceration, strictures, and mediastinitis are potential complications associated with this procedure, it is not clear whether isolated pleuropulmonary events such as pleuritis, pneumonitis, and adult respiratory distress syndrome are causally related to the EVS. Endoscopy and sedation with the attendant risk of aspiration, particularly in the background of hepatic encephalopathy, may account for some of these events. Recent controlled studies of respiratory function demonstrate that EVS as such results in minor changes in gas exchange, lung volumes, and pulmonary and systemic hemodynamics. Most pulmonary complications have been reported with the use of sodium morrhuate sclerosant. Comparative studies among different sclerosants are necessary to evaluate relative safety. Finally, there have been rare reports of myocardial ischemia and pericarditis reported in association with EVS, but these are of a transient nature. Chest symptoms, roentgenographic pleuropulmonary changes, pulmonary hemodynamics, and cardiac perturbations are transient and should not preclude offering EVS to patients with variceal hemorrhage.
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PMID:Cardiorespiratory effects of endoscopic esophageal variceal sclerotherapy. 222 Aug 81

Splenectomy for massive splenomegaly and hypersplenism carries a significant morbidity and mortality. We have used partial splenic embolization (PSE) as an effective alternative to splenectomy. Ten PSE procedures were performed on nine patients without mortality and with minimal morbidity. The age of the patients ranged from 8 months to 32 years (mean 14 years). The causes of splenomegaly and hypersplenism included cystic fibrosis with cirrhosis (2), tyrosinemia and cirrhosis (1); thalassemia (1), hemophilia with Human Immune Deficiency Virus infection (2), chronic hepatitis with portal hypertension (1), malignant histiocytosis (1), and Wiskott-Aldrich Syndrome (1). All procedures were performed under local anesthesia with sedation. A percutaneous femoral artery approach to the splenic artery was used to deliver Ivalon sponge particles (280-800 microns) into the spleen. Splenic infarction was assessed by postembolization angiograms. All of the patients except one demonstrated improvement of hematologic parameters. In one patient, however, cytopenia improved only after a second embolization. In the total series, there was an early mean rise of 8,600/mm3 in the leukocyte count (range 2,900-14,900) and 212,000/mm3 in the platelet count (range 30,000-718,000). Follow-up ranged from 4 months to 7 years. Improvement of the blood picture has been persistent in seven of the eight patients who showed initial improvement. Transient procedural complications included fever (5), pleural effusion (2), pneumonia (1), and splenic abscess (1). One patient had paralytic ileus lasting for 10 days and one patient developed a streptococcal peritonitis 3 weeks after embolization. No patient developed pancreatitis or vascular compromise of other abdominal viscera.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Partial splenic embolization. An effective alternative to splenectomy for hypersplenism. 226 5

A 12 month old boy weighing 6.4kg with esophageal varices caused by congenital biliary hypoplasia was scheduled for emergency sclerotherapy under general anesthesia. Anesthesia was induced with thiamylal sodium 3mg.kg-1 i.v. and then maintained with nitrous oxide, oxygen and a low concentration of enflurane, paralysed with pancuronium bromide. As soon as a small dose of sclerosant (5% ethanolamine oleate) was injected, transient moderate bradycardia and hypotension occurred. As his spontaneous breathing was very weak and the movements of extremities convulsive and his consciousness drowsy, prophylactic respiratory care was carried out. He had pneumonia and manifestation of DIC 4 days after sclerotherapy. He died of a massive tracheal hemorrhage. The cause of the patient's death seemed largely due to the several toxicities of sclerosant itself. We stress that although this therapy is effective for the child with portal hypertension, the incidence of complications might be high in patient with severely damaged liver function. Therefore, anesthetic and postoperative management in injection sclerotherapy should be performed very carefully.
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PMID:[Death after delayed recovery and respiratory failure following injection sclerotherapy in a small infant under general anesthesia]. 261 9

In order to determine immediate criteria of prognosis for patients with portal hypertension hospitalized for digestive hemorrhage, in an intensive care unit, 18 variables were recorded during the 24 hours following admission in 65 patients. Data related to death were age, ascites, hepatic encephalopathy, shock, active hemorrhage, acute pneumonia, decrease in prothrombin time, use of esophageal balloon tamponade, use of mechanical ventilation, number of red blood cell units transfused. Discriminant analysis yielded a linear combination of 4 variables which best separated survivors from non survivors with the following equation: F = 0.330 X hepatic encephalopathy + 0.433 X shock + 0.226 X active hemorrhage + 0.0097 X age - 0.396. The threshold decision of the hemorrhage prognosis index (HPI) was F = 0.57; 80 p 100 of all patients were correctly classified. In order to be validated, HPI was compared with a general (SAPS) and specific (Pugh's classification) scoring system, in a prospective study of 57 episodes of digestive hemorrhage. In this study, sensitivity was better with HPI than with SAPS (0.70 versus 0.45), specificity was higher with HPI than with Pugh's classification (0.86 versus 0.70). Percentage of correctly classified patients was higher using HPI (81 p. 100) than SAPS (77 p. 100) and Pugh's classification (68 p. 100). We suggest that the HPI, determined with 4 easily defined and recorded variables should be used prospectively to compare efficacy of different treatments.
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PMID:[Analysis of factors related to early mortality in digestive hemorrhage caused by portal hypertension]. 349 65

From 1980 to 1984 fortyeight children with portal hypertension were treated, 37 out of these had a prehepatic bloc. In 19 children a sclerotherapy was performed. Seven times a bleeding recurrency occurred and an esophageal stenosis was seen in 2 cases. The distal splenorenal shunt (Warren) was performed 16 times. All children survived, one pneumonia and one slight pancreatitis were observed post-operatively. Four times a shunt obstruction was found at follow-up examinations. Bleeding recurrencies did not appear.
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PMID:[Therapy of portal hypertension in childhood]. 387 23

The Budd-Chiari syndrome is caused by an occlusion of the hepatic veins and is often associated with an obstruction in the hepatic portion of the inferior vena cava (IVC). Therefore, the various shunt operations done in an attempt to relieve the portal hypertension are often not effective. By using a radical surgical technique on six patients with Budd-Chiari syndrome, the liver was freed and a wide longitudinal incision was made in the hepatic portion of the IVC. The obstructed hepatic vein was reopened using a Fogarty catheter, and a pericardial patch reinforced with a Teflon prosthesis was secured over the incision. Two patients were still asymptomatic 3 and 4 years after operation. Two patients died postoperatively of pneumonia and hepatic insufficiency. The two other patients were discharged in fair condition, and one died 6 months postoperatively following hepatic failure.
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PMID:A radical operation for the Budd-Chiari syndrome associated with obstruction of the inferior vena cava. A report of six patients. 673 15

161 children followed up postoperatively following splenectomy, 29% had spherocytosis, 14% Hodgkin's disease, 12% traumatic rupture of the spleen, 11% portal hypertension and 7% idiopathic thrombocytopenia. Postoperatively a slight wound infection occurred in 5% of the children, while complications were seen in 2% which could be interpreted as directly caused by the operation; in 23 patients, however, (i.e. 15%), severely infections occurred such as pneumonia, meningitis and sepsis. The lethality rate of the infected children was 31.8%. Postoperatively we determined the leucocyte count, thrombocytes and erythrocyte count, the immunoglobulins IgG, IgA, IgM and IgE, the serum concentrations of the complement components C3, C4 and the serum proteins alpha 1-antitrypsin and transferrin. The data obtained were compared with the corresponding data reported in the literature.
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PMID:[Complications of splenectomy in childhood (author's transl)]. 704 92


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