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 calf into which a biolized, total artificial heart (TAH) had been implanted survived for 145 days. All measured physiological parameters except central venous pressure (CVP) were back to normal one month after implantation, and thereafter the animal's physiological development was similar to that of a normal calf. The intimal weight, which was 96 kilograms at implantation, reached 190 kilogram at the end of experiment, with a daily gain rate of 0.9 kilogram per day. After the nineteenth postoperative week, signs of congestive heart failure appeared, such as high venous pressure, ascites, and enlarged liver although the calf outwardly appeared well. On postoperative day 146, the animal started foaming at the mouth, and a convulsion occurred; then, the experiment was terminated after 3,494 hours of pumping. At autopsy, there were acute bilateral bronchopneumonia involving mostly both upper lobes, pulmonary edema, slight chronic pneumonitis, and hepatomegaly. There were no serious thrombotic deposits inside the cardiac prosthesis.
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PMID:Survival for 145 days with a total artificial heart. 83 53

The clinical and hematological features of 100 patients with sickle cell anemia are reviewed. The heart was enlarged and a murmur was heard in nearly 80 percent of patients. Pneumonia and pulmonary infarction occurred in 43 percent and 12 percent of patients, respectively. Musculoskeletal involvement included the hand-foot syndrome (15 percent), leg ulcers (55 percent), aseptic necrosis ofbone (11 percent), and osteomyelitis (4 percent). Symptoms and signs related to the gastrointestinal system included jaundice (55 percent), hepatomegaly (50 percent), splenomegaly (23 percent), hepatitis (11 percent) and gallstones (9 percent). Three patients underwent cholecystectomy and three patients had their spleens removed. Pyelonephritis occurred in 17 patients, priapism in five and hematuria in seven. Nineteen women had 39 pregnancies, of which 35 resulted in the birth of healthy infants. At least 328 painful crises occurred in 73 patients. There were also 13 hemolytic crises, eight sequestration crises, and five aplastic crises. A trail of alkali therapy in 33 crises in children failed to produce beneficial effects greater than hydration and analgesics alone as used in the control group. Laboratory findings in the 100 patients were comparable to those previously reported in the literature. The renal concentrating defect in most patients was confirmed. There were six deaths: hepatic coma secondary to post-transfusion hepatitis, thrombosis of inferior vena cava, congestive heart failure, exsanguination from erosion of the pancreaticoduodenal artery, extensive bronchopneumonia, and pulmonary infarction.
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PMID:Sickle cell anemia- clinical manifestations in 100 patients and review of the literature. 113 Apr 36

Six hundred and fifty seven hospitalized patients under five in Child Health Department Hospital Tembakau Deli Medan, from January 1988 until December 1988 had been investigated retrospectively. Severe PCM are found 12 (1.8%), consisting of 7 (58.3%) boys and 5 (41.7%) girls. Most cases were found at the age of 1-2 years (33.3%). Marasmus were found in 5 cases (41.7%), Marasmic kwashiorkor in 4 (54%), while Kwashiorkor in 3 cases (25%). Clinical features of the patients are as follow: hepatomegaly 7 (58.3%), anorexia 6 (50%), old man face 5 (41.7%), subcutaneous fat decrease 5 (41.7%), thinsparse easily pick hairs 5 (41.7%), muscle hypotrophy 5 (41.7%), edema of the lower extremity 4 (33.3%), crazy pavement dermatosis 2 (16.7%). All patients were hospitalized combined with other diseases as chronic diarrhea 6 (50%), bronchopneumonia 5 (41.7%) and ascariasis 4 (33.3%). Mortality in 1 patient.
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PMID:Clinical features of severe malnutrition at the pediatric ward of Tembakau Deli Hospital, Medan. 207 60

A retrospective study on severe malnutrition concerning children hospitalized at the Pediatric ward of Dr. Pirngadi Hospital, Medan from January 1 to December 31, 1988 was conducted. Patients less than five years old were included in this study. The purpose of this study was to know the incidence of severe malnutrition, its symptoms and signs, the immunization status, feeding pattern and socio-economic factors. Out of the 3370 hospitalized patients, 2453 (72.78%) were children under five years old. Of these, 312 (12%) suffered from severe malnutrition. It consisted of marasmus 131 (41.9%), marasmic kwashiorkor 94 (30.1%) and kwashiorkor 87 (27.8%). The highest incidence was found in the age group of 0-2 years (58%). Clinical manifestation of marasmus were old man face (131 or 100%), muscular hypotrophy (118 or 71.9%) and decreased subcutaneous fat (116 or 71.1%) in marasmic kwashiorkor children 46 or 50% had their hair easily picked out, 45 or 46.3% showed hyperpigmentation and 48 or 52% had pretibial edema in the kwashiorkor group 29 or 63% had moon face, 52 or 60.4% showed crazy pavement dermatosis, 77 or 51.3% had hepatomegaly and 87 or 48% pretebial edema. Moon face was seen in 29 (63%), crazy Pavement Dermatosis in 52 (60.4%), hepatomegaly in 77 (51.3%), and pretebial edema in 87 (48%) of kwashiorkor cases. The accompanying diseases were mostly diarrhea (95%) and bronchopneumonia (22%). Immunization status showed that BCG comprised 50.6%, while DPT III and OPV III in 13.7% and 10.5% respectively and measles only 0.64%. More than half (59.6%) of them were breast-fed up to 6 months.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Clinical features of severe malnutrition at the pediatric ward of Dr. Pirngadi Hospital Medan. 207 61

A juvenile California sea lion (Zalophus californianus) died 28 days after admission to the California Marine Mammal Center. Necropsy revealed hemorrhagic fluid in the stomach and hepatomegaly. Histologically, there was evidence of multifocal necrotizing hepatitis and acute suppurative bronchopneumonia. Amphophilic intranuclear inclusions were found, and electron microscopy revealed virions morphologically classifiable as adenovirus.
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PMID:Viral hepatitis (adenovirus) in a California sea lion. 627 45

A female newborn, the second child of healthy non consanguineous parents, exhibited muscular hypotonia, areflexia, apathy, seizures, hepatomegaly and failure to thrive since birth. The peculiar skull shape was lacking. In the urine pipecolic acid and trihydroxycoprostanoic acid were excreted. At the age of seven weeks she died of bronchopneumonia. Lightmicroscopy revealed malformations and deficiency of myelinisation in the brain, renal cysts and fatty metamorphosis in the enlarged liver, which showed only minimal siderosis. Ultrastructurally no peroxisomes could be found in liver and kidney. No peroxisomes were detected by histochemical demonstration of catalase in frozen liver tissue which was taken immediately after death and stored for three months. Absence of peroxisomes is pathognomonic for the cerebro-hepato-renal syndrome of Zellweger and occurs in the liver irrespective of duration and degree of liver damage. It is best demonstrated by enzymehistochemical electron microscopy. With this method peroxisomes can be visualized even 30 h post mortem. In deep frozen normal liver tissue the activity of catalase remains very stable and enables the identification of peroxisomes even after a 12 months period of storage. In the cerebro-hepato-renal syndrome of Zellweger, frozen liver tissue should be stored for biochemical and diagnostic enzymehistochemical studies.
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PMID:[Morphology and diagnosis of Zellweger syndrome. A contribution to combined cytochemical-finestructural identification of peroxisomes in autopsy material and frozen liver tissue with case report]. 734 41

Fifty cases of visceral leishmaniasis were admitted in Children's Hospital, Islamabad. Common clinical features were fever (100%), splenomegaly (100%), hepatomegaly (100%), anaemia (96%), abdominal distension (40%), bronchopneumonia (26%) and bleeding diathesis (22%). Hb was below 7.0 G/dl in 80%, white cell count below 4 x 10(9)/cmm in 88% and platelet count below 100 x 10(9)/c4mm in 86%. All the patients showed leishmania donovani bodies in the marrow smears. Fourteen patients were treated with aminosidine (15 mg/kg), intramuscularly daily for 4 weeks. All responded dramatically and none of them went into relapse in a year's follow-up. No side-effects were observed. Aminosidine can therefore, be recommended as a treatment of choice for visceral leishmaniasis in children.
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PMID:A new breakthrough in treatment of visceral leishmaniasis in children. 747 90

The records of 52 children with Niemann-Pick disease type C were reviewed to establish whether the disease process and outcome varied with the initial clinical pattern; 34 children (65%) had cholestatic liver disease and hepatosplenomegaly in infancy; 18 were seen at a mean age of 4 years with splenomegaly or neurologic disease or both. Of the 34 children with early cholestatic liver disease, three died in the neonatal period; cholestasis and hepatomegaly subsided in the remaining 31 children, although splenomegaly persisted. Of these 31 children, 15 had persistent liver disease with elevated aminotransferase values. Serial liver biopsy specimens showed that 3 of the 15 children had normal architecture and 12 had hepatic fibrosis, with progression to cirrhosis in 5. No other significant morbidity or additional deaths were associated with the liver disease. The clinical importance of persistent liver disease was overshadowed by the subsequent development of severe neurologic disease. There was no difference in the age at onset of the disease (mean, 4.5 years) or in the pattern of neurologic disease, including supranuclear ophthalmoplegia, whether or not the child had early liver disease. Overt neurologic disease has not yet developed in seven surviving children with liver disease at onset. Sixty-seven percent of children died during the study; the main cause of death was bronchopneumonia. We conclude that the diagnosis of Niemann-Pick disease type C should be considered in patients with unexplained neonatal hepatitis, especially if splenomegaly is a persistent feature. Because liver biopsy specimens may not demonstrate storage cells, bone marrow aspiration to detect the characteristic storage cells is recommended in such patients.
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PMID:Niemann-Pick disease type C: diagnosis and outcome in children, with particular reference to liver disease. 815 88

During a 16-month period children presenting to a pediatric outpatient facility from an area with a high tuberculosis incidence (> 400/100,000) and suspected of having respiratory tuberculosis (TB) were evaluated for close contact with adult pulmonary tuberculosis, weight loss, symptom duration, respiratory signs, lymphadenopathy and hepatosplenomegaly and by chest radiography and tuberculin testing (Mantoux or tine). Probable tuberculosis was diagnosed in 258 children and was confirmed in 109 (42%) patients with a mean age of 31 months by culture of Mycobacterium tuberculosis from gastric aspirate or another source. Eleven children with confirmed TB had a normal chest radiograph. After review of special investigations, clinical course and follow-up of the remaining 149 children, 86 children (58%) with a mean age of 32.4 months were considered to have probable TB and 63 (42%) with a mean age of 27 months not to have TB. Significantly fewer children in the "not TB" group than in the confirmed and probable TB groups had a close adult pulmonary tuberculosis contact (13 (21%) and 95 (49%), respectively; P < 0.01). There was no difference between the "not TB" group and the confirmed and probable TB groups in the proportion presenting with weight loss, cough or other respiratory symptoms, a symptom duration > 2 weeks, the presence of bronchial breathing, wheeze, hepatomegaly or splenomegaly or peripheral lymphadenopathy. Final diagnoses in the "not TB" group included bacterial or viral pneumonia or bronchopneumonia in 37, asthma often accompanied by segmental collapse in 9 and cavitating pneumonia in 3 children.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Respiratory tuberculosis in childhood: the diagnostic value of clinical features and special investigations. 776 Nov 83

100 cases of Kyasanur Forest Disease were studied clinically and autopsy or postmortem biopsies of various organs were done in 8 cases. 1. Our study showed that KFD passes through 4 stages each lasting for about a week. i.e. a prodromal stage with fever, hypotension hepatomegaly, a stage of complication characterized by haemorrhage, neurological manifestation or bronchopneumonia, a stage of recovery followed by a li stage of fever in some cases. 2. The exact of cause of haemorrhage could not be identified though Disseminated Intravascular Coagulation was suspected. 3. Hypotension in KFD could be of Myocardial origin. 4. Encephalopathy in KFD could be due to a metabolic cause probably of hepatic origin. 5. Lung signs could be due to intraalveolar haemorrhage and secondary infection.
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PMID:Clinical study of 100 cases of Kyasanur Forest disease with clinicopathological correlation. 822 55


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