Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Heart transplants in pediatric patients have moved from an experimental, end-stage treatment to a valuable therapy for inoperable congenital heart disease and dilated cardiomyopathies. Cardiac rejection is a frequent problem in children with heart transplants. The technical difficulties of obtaining biopsies in small children have encouraged the use of noninvasive methods of detection although endomyocardial biopsy continues to be the most reliable method of surveillance for cardiac rejection. We examined our experience over 7 years with 4 patients (ages 8 months to 10 years) who had a total of 88 endomyocardial biopsies using echocardiographic guidance. The biopsies were done using a Caves-Schultz biotome via the right internal jugular vein. Biopsies were performed in the procedure room, 61%; pediatric intensive care unit, 18%; hospital private room, 18%; and catheterization laboratory, 3% of the time. At 5.0, 6.5, or 8.5 French biotome was used depending on the size of the patient. Twelve episodes of rejection were detected in these patients. The time for venous access was 17 +/- 15 min, and the biopsies took 25 +/- 11 min using this approach. Biopsies were obtained from the right ventricular free wall, apex, and septum to detect focal rejection for a total of 4-6 tissue samples per biopsy. Neither general anesthesia nor fluoroscopy was needed for these biopsies. Costs ranged from $1120 in the patient room to $1590 in the pediatric intensive care unit. Five different biopsies using a femoral approach with fluoroscopic guidance averaged $2250 and did not include the functional assessment obtained using echocardiography. Using the echocardiogram one can see the ventricular muscle and completely avoid perforation. Thus, this technique is technically feasible in children with few complications at a reduced cost, compared to fluoroscopically guided biopsies.
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PMID:Endomyocardial biopsies in pediatric patients with no irradiation. Use of internal jugular venous approach and echocardiographic guidance. 199 21

Chagas' disease (American trypanosomiasis) is an endemic parasitic disease in some areas of Latin America. About 16-18 million persons are infected with the aetiological agent of the disease, Trypanosoma cruzi, and more than 100 million are living at risk of infection. There are different modes of infection: (1) via blood sucking vector insects infected with T. cruzi, accounting for 80-90% of transmission of the disease; (2) via blood transfusion or congenital transmission, accounting for 0.5-8% of transmission; (3) other less common forms of infection, eg, from infected food or drinks or via infected organs used in transplants. The acute phase of the disease can last from weeks to months and typically is asymptomatic or associated with fever and other mild nonspecific manifestations. However, life-threatening myocarditis or meningoencephalitis can occur during the acute phase. The death rate for persons in this phase is about 10%. Approximately 10-50% of the survivors develop chronic Chagas' disease, which is characterized by potentially lethal cardiopathy and megacolon or megaoesophagus. There are two drugs available for the aetiological treatment of Chagas' disease: nifurtimox (Nfx) and benznidazole (Bz). Nfx is a nitrofurane and Bz is a nitroimidazole compound. The use of these drugs to treat the acute phase of the disease is widely accepted. However, their use in the treatment of the chronic phase is controversial. The undesirable side effects of both drugs are a major drawback in their use, frequently forcing the physician to stop treatment. The most frequent adverse effects observed in the use of Nfx are: anorexia, loss of weight, psychic alterations, excitability, sleepiness, digestive manifestations such as nausea or vomiting, and occasionally intestinal colic and diarrhoea. In the case of Bz, skin manifestations are the most notorious (e.g., hypersensitivity, dermatitis with cutaneous eruptions, generalized oedema, fever, lymphoadenopathy, articular and muscular pain), with depression of bone marrow, thrombocytopenic purpura and agranulocytosis being the more severe manifestations. Experimental toxicity studies with Nfx evidenced neurotoxicity, testicular damage, ovarian toxicity, and deleterious effects in adrenal, colon, oesophageal and mammary tissue. In the case of Bz, deleterious effects were observed in adrenals, colon and oesophagus. Bz also inhibits the metabolism of several xenobiotics biotransformed by the cytochrome P450 system and its reactive metabolites react with fetal components in vivo. Both drugs exhibited significant mutagenic effects and were shown to be tumorigenic or carcinogenic in some studies. The toxic side effects of both nitroheterocyclic derivatives require enzymatic reduction of their nitro group. Those processes are fundamentally mediated by cytochrome P450 reductase and cytochrome P450. Other enzymes such as xanthine oxidoreductase or aldehyde oxidase may also be involved.
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PMID:Toxic side effects of drugs used to treat Chagas' disease (American trypanosomiasis). 1693 19

The purpose of this study was to evaluate certain clinical aspects of hyperthyroidism in Albania, which is an iodine deficient country, as it is known that iodine intake may influence the type of thyroid hyperfunction. The files of sixty-six patients with thyrotoxicosis who were hospitalised for their disease were retrospectively analysed. 59.1% of these patients suffered from toxic multinodular goiter, 27.3% from Grave's disease (toxic diffuse goiter), 10.6% from toxic adenoma, 1.5% from iodide-induced hyperthyroidism and 1.5% from transient hyperthyroidism due to subacute thyroiditis. There was an increased female to male ratio (83.3% vs 16.7%, respectively, p<0.001). 83.9% of all hyperthyroid patients lived in cities, while 16.1% lived in villages. Ophthalmopathy was found in 11.1% of patients with Graves' disease, and thyrotoxic heart disease was found in 14% of patients with thyrotoxicosis. 71.9% of all patients with hyperthyroidism were treated with propylthiouracil (PTU), while 28.1% of them were treated with methimazole; 67.2% of all these patients also received propranolol hydrochloride, while 32.8% were prescribed atenolol. Compliance was lower than that reported in other studies as only 41% of all patients received their treatment regularly. Side effects from treatment with antithyroid drugs were as follows: 4.1% (2/48) of patients treated with propylthiouracil presented leukopenia with agranulocytosis, and 6.1% of them toxic hepatitis, while 11.1% (2/18) of patients treated with methimazole presented agranulocytosis. In conclusion, the mode of presentation and side effects of hyperthyroidism appears to be different in Albania when compared with other countries, probably as a result of iodine deficiency and/or possibly nutritional status. Compliance with treatment is lower than that reported in other series, while antithyroid drug side effects seem to be more frequent. The latter observation may be due to the fact that only hospitalised patients were analysed in this study.
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PMID:Clinical aspects of hyperthyroidism in hospitalised patients in Albania. 1698 78

Thalassemia major is a rare inherited anemia, and affected children require blood transfusions every 2-4 weeks to survive. Repeated blood transfusions lead to a build-up of toxic levels of iron in the body, causing organ damage and premature death, primarily due to iron-induced heart disease. Deferiprone is one of a few drugs that are routinely used in medicine for the treatment of iron overload in thalassemic patients. This drug is usually administered daily at high doses (50-100 mg/kg) with a very low toxicity. Agranulocytosis is the most serious side effect of deferiprone, with a reported incidence of 0.6 per 100 patient-years. We document an illustrated case report of necrotizing gingivostomatitis, an oral manifestation of agranulocytosis secondary to deferiprone use involving the gingiva and palatal mucosa of a thalassemia major patient. Various causes of precipitation of agranulocytosis in these patients and a possible relationship of necrotizing gingivostomatitis with deferiprone are highlighted in this case report.
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PMID:Necrotizing stomatitis: a possible periodontal manifestation of deferiprone-induced agranulocytosis. 1977 31