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)

Orthotopic cardiac transplantation has become established for selected infants with severe forms of congenital heart disease. This study reviews the combined experience and intermediate term results of infants undergoing orthotopic cardiac transplantation from Children's Memorial Hospital, Chicago, and Kosair Children's Hospital, Louisville. From June 1986 through December 1989, 20 orthotopic cardiac transplantations were performed in 19 patients. Sixteen patients had variants of hypoplastic left heart syndrome. One infant had anomalous origin of the left coronary artery with severe ischemic cardiomyopathy. Two infants had aortic stenosis with endocardial fibroelastosis, and one had extracorporeal membrane oxygenation as a bridge to transplantation. Immunosuppression included cyclosporine, azathioprine (Imuran), and corticosteroids with an effort to wean the patients from steroids by 6 months to 2 years. Three early deaths resulted--from technical errors in two patients and from hyperacute rejection in one patient at 3 days. Four late deaths have occurred. Two patients died at 2 and 13 months of acute rejection. One patient died at 15 months of acute rejection after retransplantation. One patient died at 7 months of respiratory syncytial viral pneumonia. The remaining 12 patients are surviving 5 to 47 months (means 20 months) after orthotopic cardiac transplantation. Rejection surveillance in the first 6 months is by clinical signs supplemented by echocardiography, electrocardiography, and cell cycle analysis; endomyocardial biopsy is used after 6 months of age. For the cumulative series, 24 episodes of suspected rejection have been treated during 277 at-risk patient months with intravenous methylprednisolone (Solu-Medrol) (n = 18) and monoclonal antibody (OKT3) (n = 6), for an incidence of 1.04 episodes of rejection per patient per year. Serious posttransplantation infections including endocarditis, catheter sepsis, meningitis, and colonic perforation were successfully treated in four patients. Subjectively, their quality of life is excellent as shown by normal growth and developmental milestones and a low hospital readmission rate (1.4 episodes per patient per year). These encouraging intermediate term results warrant continued application of infant orthotopic cardiac transplantation for severe forms of congenital heart disease.
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PMID:Intermediate term results of infant orthotopic cardiac transplantation from two centers. 190 39

A 74-year-old woman was referred to our department in December 1999 for a pyoderma gangrenosum (PG) arising at the edges of chronic leg ulcer. The history was positive for benign monoclonal gammopathy, ischemic hypertensive cardiopathy, polyarthrosis and venous lower leg deficiency. Monoclonal gammopathy of IgA Kappa type was diagnosed 10 years before with a continued benign nature. In 1990 a post traumatic PG of the left leg was diagnosed and a therapy with Cyclosporine A was started with healing of the lesion. In June 1999, 6 months before the hospitalization, a typical venous ulcer of the right leg appeared and was treated with bed-rest, compression bandaging and topical desloughing therapy. In the last month, after a minor surgical debridement of the wound, the lesion developed pustules evolving into a painful, necrotic ulcer with ragged, purple-red, undetermined borders (Fig. 1). A relapse of PG was suspected. Histological examination was consistent with pyoderma gangrenosum and showed massive neutrophilic infiltration, hemorrhage and necrosis of the overlying epidermis. Wound culture was negative. Other laboratory examinations only showed IgA = 8.15 g/L. Investigation of other underlying medical conditions were normal or negative. The venous leg ulcer gradually healed with antiseptic and compression-bandage therapy. After a 4-month course of topical steroid therapy with good results, the PG recurred also involving the proximal area of the leg. Methylprednisolone 50 mg/day was started. Healing began 10 days later and 2 months later the wound healed and epithelialized. Steroid was reduce to 5 mg daily for 4 months. No recurrence was seen when the drug was stopped.
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PMID:Pathergic pyoderma gangrenosum in a venous ulcer. 1201 Mar 42