[Pneumatosis intestinalis in pediatric heart transplantation].
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Biomedical subjects
Publications and source records attributed to M Camino López.
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A girl with Down's syndrome, moyamoya disease and sagittal sinus thrombosis is described. She was diagnosed after acute neurological deterioration by MRI and angiography. Recombinant tissue plasminogen activator (r-TPA) was injected locally to recanalise the thrombus. The patient's condition significantly improved and she was discharged. After 2 years of follow-up the child remains asymptomatic. Moyamoya syndrome and cerebral venous thrombosis should not be overlooked as a cause of acute neurological deterioration in a child with Down's syndrome. MRA appears to be a safe and accurate alternative to traditional angiography for the diagnosis of moyamoya disease. Local fibrinolysis with r-TPA is the treatment of choice for cerebral venous thrombosis due to its safety and efficacy.
The use of diagnostic and therapeutic techniques is very important to ensure optimum, effective treatment in patients with heart disease to thereby obtain an adequate cost-benefit relationship. The aim of establishing guidelines for the evaluation and management is to achieve this relationship, but these guidelines are difficult to establish in pediatric cardiology despite 50 years of experience in this field. The physiopathologic peculiarities of newborns, in addition to the complex anatomical features of many heart diseases and their clinical presentation, diagnostic and therapeutic difficulties and the presence of residue, sequela and complications of the surgical technique make the elaboration of such guidelines at this age complex. Guidelines for some heart diseases are presented together with a descriptive analysis and a therapeutic schedule of other congenital heart diseases.
A 14-year-old boy manifested acute abdominal pain, vomiting, high temperature and diarrhea. He also underwent increasing hyponatremia and hyperkalemia after appendectomy. Further testing confirmed Addison disease. The serum adrenal antibody test was positive, and other autoimmune diseases were excluded.
Background percutaneous PDA occlusion has been proposed as a valid therapeutic alternative to surgical closure. Between April 1990 and July 1994, 44 patients underwent percutaneous PDA occlusion. Thirteen patients were less than 18 months old and weighed 8.7 +/- 3.2 Kg (Group I). Thirty-one patients, older than 18 months of age, had a mean weight of 36.8 +/- 20.6 Kg (Group II). Four patients in group I and two in group II had associated cardiac anomalies Residual shunt after percutaneous occlusion was studied by color Doppler. Fifty-one occluding devices were used. Forty-nine were implanted and there were two device embolizations. Twelve devices were implanted in group I. One device needed surgical removal from the right ventricle where it had embolized. In group II, 31 devices were implanted and one embolized to the descending aorta and was percutaneously removed through the femoral artery sheath. In two patients of group I and four of group II, a second device was implanted. The total occlusion rate immediately after implantation at 24 hours and at 6 months was 75%, 83% and 83% in group I and 64.5%, 77% and 84% in group II. After implantation of a second device the total occlusion rate without residual shunt reached 100% in group I (after 16 +/- 12 months follow-up) and 97% in group II (after 23 +/- 16 months of follow-up). We conclude that percutaneous ductal occlusion with a Rashkind device is a valid therapeutic alternative to surgical closure in all age groups.