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)

Varying degrees of respiratory distress developed in 3 dogs in which hyperadrenocorticism was diagnosed. The respiratory distress was attributed to pulmonary artery thrombosis. Radiography revealed pleural effusion, increased diameter and blunting of the pulmonary arteries, lack of perfusion of the obstructed pulmonary vasculature, and overperfusion of the unobstructed pulmonary vasculature. Thrombosis was confirmed by nonselective angiocardiography in each case. In 1 case, selective angiocardiography showed marked reduction of the transit time of contrast medium from the right atrium to the aorta. Hypertension proximal to the site of thrombosis was confirmed in 2 cases by showing increases in the right ventricular systolic pressures (80 mm of Hg in one case and 54 mm of Hg in the other case). In 3 cases, there was moderate hypoxemia with hypocapnia, suggesting a ventilation-perfusion mismatch. Clinical findings other than respiratory distress included hepatomegaly, ventral edema, orthopnea, and a jugular pulse. Pulmonary artery thrombosis, as it occurred in these 3 cases, was compared with the disease in man. It was concluded that pulmonary artery thrombosis should be suspected in cases of intractable dyspnea, right-sided heart failure of unexplained origin, and acute unexplainable death.
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PMID:Pulmonary artery thrombosis in three dogs with hyperadrenocorticism. 723 99

A cross-sectional echocardiographic study of 50 black Zimbabwean children with clinical human immunodeficiency virus (HIV) infection was carried out. The median age was 9 months. Seventy per cent had chronic cough, 60% respiratory distress and 40% cyanosis. Sixty per cent had pericardial effusion and 48% right ventricular hypertrophy (RVH) and dilation. However, the clinical diagnosis of heart failure was difficult as most of these children (80%) had hepatomegaly. These findings suggest that respiratory disease plays a role in the causation of RVH in these children. As cardiac causes of RVH were absent, this was presumed to be due to cor pulmonale. HIV-infected children presenting with respiratory distress may have clinically unapparent cor pulmonale. Early and appropriate management may by beneficial.
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PMID:Cor pulmonale in children with human immunodeficiency virus infection. 767 13

Thirteen patients with symptomatic polycystic liver disease who were selected for fenestration were reviewed. The main preoperative complaints that related to polycystic liver disease were severe abdominal pain, respiratory distress, clinical ascites, or leg edema. All symptoms disappeared after operation, and the number and size of the cysts were smaller. Five patients developed transient but massive ascites during the postoperative period, and long-term follow-up demonstrated a moderate recurrence of hepatomegaly in two patients. These postoperative complications and failures appeared to be at least partly related to a more extensive evolution of the disease in the patients in whom it developed.
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PMID:Fenestration in the management of polycystic liver disease. 774 Aug 6

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

The clinical characteristics of 16 neonates with malaria parasitaemia diagnosed on Giemsa stained smears were documented during a 3-month rainy season period in a tropical African city. The prevalence of neonatal malaria was 8 per cent. Seventy-five per cent of these neonates had congenital malaria, 13 per cent transfusional malaria, and 13 per cent had acquired malaria. Plasmodium falciparum was found in all positive smears. Bacterial cultures of blood, urine, and cerebrospinal fluid were sterile. The predominant clinical features were those of fever (88 per cent), respiratory distress (57 per cent), pallor and anaemia (38 per cent each), hepatomegaly (31 per cent), and jaundice and diarrhoea (25 per cent each). Twenty-five per cent of the neonates were resistant to chloroquine sulphate; 19 per cent of the chloroquine resistant babies were also resistant to quinine sulphate 13 per cent of whom responded to halofantrine hydrochloride. One died a day after completing a full course of quinine, with a post-mortem blood smear showing no change in the density of parasitaemia.
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PMID:Clinical characteristics of neonatal malaria. 860 38

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

Progressive disseminated histoplasmosis (PDH), a recognized defining illness of AIDS, is an opportunistic fungal infection caused by Histoplasma capsulatum. The authors report a case of PDH in a HIV-infected African child from a Histoplasma capsulatum non-endemic area. An 8-year-old girl from Kwazulu/Natal, South Africa, was admitted to King Edward VIII hospital with pyrexia and respiratory distress. Pale with generalized lymphadenopathy, she had been sick with general malaise and fever for 3 weeks. A punched-out painless ulcer was present on the child's lower left leg and she had ulcerative lesions on the tip of her tongue and the angle of her mouth. There was a tender hepatomegaly and clinical signs of pneumonia, while a chest roentgenogram showed right upper lobe consolidation with early cavitation. The purified protein derivative tuberculin skin test was negative and no acid-fast bacilli were detected on three sputum samples taken on different days. A Western blot test conducted for antibodies to HIV was positive. Additional laboratory tests were conducted. The patient was treated with parenteral acyclovir for herpesvirus infection, ceftriaxone for severe community-acquired pneumonia, and trimethoprim-sulfamethoxazole because Pneumocystis carinii infection was part of the clinical differential diagnosis. Bone marrow aspirate and trephine biopsy revealed yeast forms of H. capsulatum. The girl died on the second day of hospital admission, before antifungal therapy could be commenced.
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PMID:Disseminated histoplasmosis in a human immunodeficiency virus-infected African child. 910 50

A 30-year-old HBsAg-positive woman was admitted to the hospital because of 6 days of progressive shortness of breath. She was in severe respiratory distress with circulatory collapse. She had an enlarged liver but no stigmata of chronic liver disease or signs of cirrhosis. She had rapidly developed respiratory arrest and was transferred to intensive care unit. Heart ultrasonography and Doppler scan showed right heart straining and high pulmonary artery pressure. Despite cardiovascular and respiratory support she died a few hours after admission. Autopsy revealed combined hepatocellular-cholangiocarcinoma infiltrating the entire liver, metastatic invasion of lung blood vessels and absence of right ventricular hypertrophy. The incidence of hepatocellular-cholangiocarcinoma, a variant of hepatocellular carcinoma, is roughly 2-3% and the presenting symptoms are abdominal pain, weight loss, jaundice, fever or decompensation of liver disease. Associated HBsAg positivity and cirrhosis are reported in 20-30% and 60% of patients, respectively. Metastases to lungs are relatively frequent but this is the first report of hepatocellular-cholangiocarcinoma presented with acute respiratory distress due to massive pulmonary embolism.
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PMID:Combined hepatocellular-cholangiocarcinoma presented with massive pulmonary embolism. 1102 Aug 95

We report a Turkish family with parental consanguinity and at risk for sialidosis type II, an inherited autosomal recessive disorder caused by lysosomal alpha-N-acetyl-neuraminidase (sialidase, NEU1) deficiency. The proband was a premature male infant that presented with hydrops, hepatomegaly, respiratory distress syndrome, and anemia and that died of respiratory insufficiency 2 months after birth despite intensive care. An abnormally increased [14C]methylamine incorporation and an isolated deficiency of lysosomal alpha-N-acetyl-neuraminidase were found in cultured skin fibroblasts. A previous pregnancy of the mother terminated in a spontaneous abortion in the 13th week of gestation. A successive pregnancy showed hydrops fetalis, and an enzymatic assay of cultured amniotic fluid cells indicated a deficiency of alpha-N-acetyl-neuraminidase. Following pregnancy termination at 20 weeks gestation, light microscopy of fetal tissues revealed classic vacuolation not only in liver, bone marrow, brain, and kidney, but also in endocrine organs such as the thyroid gland, adrenal gland, hypophysis, and testes, and in the thymus. DNA analysis of the family showed that both the proband and the third sibling had a novel homozygous nonsense point mutation at nucleotide 87 in exon 1 of the alpha-N-acetyl-neuraminidase (neu1) gene causing a substitution of tryptophan at codon 29 by a termination codon (W29X). DNA sequencing of polymerase chain reaction products identified the parents as heterozygous carriers. To detect neu1 mRNA expression, a real-time reverse transcription/polymerase chain reaction was performed, and similar rates of neu1 mRNA expression were found in the fibroblasts of the fetus, the 2nd sibling, and in controls. The very early termination codon with complete loss of neuraminidase activity is probably the molecular basis of the unusually severe vacuolation pattern in this form of congenital sialidosis.
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PMID:Prenatal diagnosis and fetal pathology in a Turkish family harboring a novel nonsense mutation in the lysosomal alpha-N-acetyl-neuraminidase (sialidase) gene. 1170 24

Neuroblastoma is a common solid tumor of childhood that can involve the abdomen, thorax, pelvis, or the head and neck. The clinical manifestations are dependent on the widespread distribution of neural crest tissue and the length of the sympathetic chain involvement. Abdominal pain and hypertension may occur as a result of renal vasculature compression; respiratory distress may be evident in thoracic tumors; and Homer's syndrome or heterochromia of the iris may manifest from neuroblastoma of the head and neck. In addition, symptoms of cord compression and back pain may result from spinal cord compromise due to epidural invasion. Metastatic involvement of the liver, skin, periorbital regions, or bone may cause hepatomegaly, skin nodules, proptosis, or bone marrow failure, respectively. Clinical findings along with tumor metastasis may be studied by various imaging modalities to assess the nature and extent of the tumor. Diagnostic tests include plain radiography, ultrasonography, CT scanning, and MR imaging. Bone marrow studies, bone scans, and scintigraphy with 131I-metaiodobenzylmandelic may be utilized for metastatic evaluation. By using these imaging studies to detect the nature and behavior of neuroblastoma, early intervention may indeed improve patient survival.
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PMID:Imaging neuroblastoma in children. 1262 83


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