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Biomedical subjects

C Rey

Publications and source records attributed to C Rey.

At least 217 records · Page 12Linked to original sources
PubMed1993

Acquired sideroblastic anaemia during treatment of Wilson's disease with triethylene tetramine dihydrochloride.

We report one case of acquired sideroblastic anaemia in a patient treated for Wilson's disease with triethylene tetramine dihydrochloride (TTH). No other cause of acquired sideroblastic anaemia was found, and neither iron nor pyridoxine therapy could correct this anaemia. In contrast, decreasing the dose of TTH led to disappearance of ringed sideroblasts. Thus TTH should be added as a further cause of secondary acquired sideroblastic anaemia. The pathophysiology of this finding, probably linked to an abnormality of mitochondrial iron metabolism, is briefly discussed.

Adolescent↗
PubMed1993

Transcatheter occlusion of patent ductus arteriosus with adjustable buttoned device. Initial clinical experience.

BACKGROUND: Several devices are available for transcatheter occlusion of patent ductus arteriosus. Most of these devices either require complicated intracardiac maneuvering, have not been tried in humans, or need a large-sized sheath for implantation of the device. METHODS AND RESULTS: During a 26-month period ending November 1992, 14 patients underwent transcatheter closure of patent ductus arteriosus with an adjustable buttoned device delivered via a 7F sheath under an institutional review board-approved custom-made device protocol. The children were 15 months to 8 years of age with weight range of 7.2 to 19 kg. The patent ductus arteriosus measured from 2 to 7.5 mm (median, 3 mm) at the narrowest diameter and was conical, short, or tubular. They were occluded with devices measuring 15 to 20 mm. The ratio of pulmonary to systemic flow decreased (P < .01) from 1.9 +/- 0.6 (mean +/- SD; range, 1.3 to 3.2) to 1.05 +/- 0.1 (1 to 1.3). Continuous murmur of patent ductus arteriosus disappeared in all except 1 patient. Small residual shunts were detected by color Doppler studies in 4 of 14 patients (29%). All patients were followed for 1 to 24 months (mean, 6 +/- 7 months). The device was intact in all patients, and no breakage of the wires was noted. No shunts were seen in 12 of 14 patients (86%), and minute residual shunts were seen in 2 children. No major complications were encountered. CONCLUSIONS: It is concluded that transcatheter closure of patent ductus arteriosus with the adjustable buttoned device is feasible and effective and can be accomplished via small 7F sheaths; a 7F sheath is adequate for all ductal sizes; and the adjustable length of the loop accommodated all types of patent ductus arteriosus. Clinical trials on a larger number of patients are warranted.

Cardiac Catheterization↗
PubMed1993

[Severe complications and mortality in mental eating disorders in adolescence. On 99 hospitalized patients].

BACKGROUND: Subclinical medical complications frequently occur during the follow-up of anorexia nervosa and bulimia. This paper describes some of these. POPULATION AND METHODS: Charts of 99 adolescent patients (89 girls and 10 boys), aged 11.8 to 22 years (mean: 16.6 +/- 2.1 years), admitted for anorexia nervosa (N:92) or bulimia (N:7), were analyzed retrospectively. All severe or potentially severe, clinical and non-clinical, findings at admission were included in the study. RESULTS: Anorexic patients had a mean weight loss of 31.5% (22 of them were also vomiters or laxative abusers). Initial nasogastric tube feeding was necessary in 19 patients and parenteral nutrition in 2. Bradycardia and hypotension were common. A variety of ECG abnormalities were seen in 86% of the patients. Mitral valve prolapse was present in 14 of the 43 patients examined by echocardiography. Electrolyte imbalance was also common: hyponatremia in 7 patients, hypokalemia in 21, hypochloremia in 10 of the 12 vomiters, hypophosphatemia in 7, hyperazotemia in 24 and hypoglycemia in 22. Bone marrow hypoplasia was frequent, with leukopenia in 29 patients, anemia in 21 and thrombocytopenia in 5. No patient developed infectious complications. One patient presented with an acute gastric dilatation and another with spontaneous pneumomediastinum. One patient, 14 year-old, died 3 years after the onset of anorexia from acute water intoxication. CONCLUSION: These well-known complications are more common in anorexic than in bulimic patients. Their prevention requires rigorous and continuous medical supervision.

Adolescent↗
PubMed1993

[Prospective study on post-acute myocardial infarction patients].

Long-term prognosis in acute myocardial infarction (AMI) can be related to a number of risk factors, including the electrocardiographic stress test (ECG Test). This study analyzes its contribution to the prognosis and management of such patients. 60 individuals discharged from a coronary care unit, without or with persisting complications underwent an ECG test 4-6 weeks following AMI, and were followed for a period of 12-39 months. 40 cases had a negative ECG test. During follow-up they continued to be generally asymptomatic and their survival was 100%. Coronary angiography in patients younger than 45 years revealed mainly single vessel disease. 20 cases (33%) had a positive ECG test. During follow-up 50% of these had anginal manifestations, and coronary angiograms revealed mainly 2-3 vessel disease. 6 patients underwent surgical revascularization. One patient died awaiting surgery. Overall survival in this group was 95%. It is concluded that stress testing performed during the convalescence period of patients with an AMI allows recognition of patients with a potentially poorer prognosis, which can be modified by appropriate study and revascularization in selected cases, bringing survival expectations to the same level of more benign cases.

Adult↗
PubMed1993

[Dilatation of critical aortic value stenosis in infants under 3 months of age. Our experience from 15 cases].

Between April 1988 and October 1992, 15 consecutive infants aged less than 3 months (average 17 days) with critical valvular stenosis underwent balloon dilatation. Thirteen were less than 1 month old and all had low output syndromes. The diagnosis and follow-up assessments were made by Doppler echocardiography. Before valvuloplasty the maximum instantaneous gradient was 75 +/- 34 mmHg. The average diameter of the aortic ring was 6.8 +/- 1.3 mm. Seven patients had aortic rings with diameters of less than 7 mm. Ten infants had a right-to-left shunt via a patent ductus arteriosus and pulmonary hypertension. Dilatation was performed after surgical denudation of the left carotid artery: a balloon catheter with a diameter 0.85 times that of the aortic ring was used. After dilatation, the gradient was 26 +/- 18 mmHg (p < 0.001). Grade I aortic regurgitation was observed in 4 cases and Grade II in 2 cases. Eight patients died 1 to 100 days after dilatation (seven of low output and one sudden death). Of these patients, 6 had aortic rings < 7 mm diameter. The average follow-up was 20.6 +/- 15.5 months (range 2 to 48 months). None of the patients had a precordial or carotid diastolic murmur. The gradient increased with improvement in left ventricular function. Two patients had a stable Grade I aortic regurgitation. Carotid Doppler echocardiographic control examinations performed in 4 patients were normal. Poor prognostic factors were: aortic ring diameter < 7 mm (p < 0.025) and a low gradient after dilatation (p < 0.012).(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Valve Stenosis↗
PubMed1993

[Atresia or congenital stenosis of the left coronary ostium. Myocardial revascularization in 5 children].

Five patients, 3 months to 13 year old with atresia (4) or stenosis (1) of the left coronary artery underwent myocardial revascularisation at Marie Lannelongue Hospital. The preoperative symptoms were dominated in one case by cardiac failure due to myocardial infarction and in the other cases by effort angina with syncope. The operative technique of revascularisation in one case was angioplasty with enlargement of the left main coronary artery with a patch and, in the others, bypass graft of the left coronary artery with the left internal mammary artery. All 5 patients survived surgery and are asymptomatic with a follow-up of 6 months to 7 years. Control coronary angiography confirmed the patency of the different procedures of myocardial revascularisation. In 3 children, exercise electrocardiography was normal. Myocardial revascularisation of a child with a congenital stenotic abnormality of the left coronary network is possible, the technique of which depends on the diameter of the left main stenosis when the vessel is absent or atresic, left internal mammary artery bypass graft is the only solution. When the left main coronary is stenosed but patent, direct reconstructive surgery is an interesting alternative.

Adolescent↗
PubMed1993

[Percutaneous implantation of endoprosthesis for stenosis of branches of the pulmonary artery. Apropos of a case].

The authors report the case of a young man operated for tetralogy of Fallot and in whom surgical treatment of severe stenosis of the origins of the right and left pulmonary arteries had failed. Correction of these stenoses was obtained by implanting percutaneously an endoprosthesis in the right pulmonary artery with a good result: control angiography showed that the stenosis had disappeared and an increase in the right pulmonary artery diameter from: 5 to 11 mm, and the left pulmonary artery diameter from 7 to 14 mm. Perfusion pulmonary scintigraphy showed equal flow in the two lungs.

Adolescent↗
PubMed1993

[Taussig-Bing malformation with straddling of both atrioventricular valves. Echocardiographic description of a case].

The authors report the case of an infant with the Taussig-Bing malformation and straddling of the two atrioventricular valves, the diagnosis of which was made by echocardiography and confirmed at surgery. Anatomic repair of the transposed great arteries was preceded by reconstruction of the interventricular septum which left the abnormal tricuspid subvalvular apparatus in the right ventricle and the abnormal mitral subvalvular apparatus in the left ventricle. Postoperative Doppler echocardiography confirmed the absence of left ventricular outflow obstruction.

Double Outlet Right Ventricle↗
PubMed1993

[Familial scimitar syndrome].

The authors report a new case of a familial form of the scimitar syndrome: father and son. Both presented an adult form which was well tolerated. Another familial form has been reported in the medical literature: father and daughter. The authors discuss the possible genetic mode of transmission in the light of these two cases.

Adult↗
PubMed1993

[Neural sarcoidosis in children. A case report].

A case of cerebral sarcoidosis of fortuitous discovery is reported in an adolescent. Neurosarcoidosis is exceedingly rare in the pediatric age groups. Clinical manifestations are widely variable and nonspecific, making diagnosis difficult in patients without extraneurologic involvement. The mainstay of management is early, prolonged corticosteroid therapy. Outcome varies with location of the lesions.

Adolescent↗
PubMed1993

[Sudden crib death. Experience in a referral center 1986-1991].

BACKGROUND: Sudden infant death syndrome (SIDS) may have several etiologies, all of which must be identified in order to recognize those infants believed to be at risk. One of the best ways to do this is by prospective studies on a large population of infants who died of SIDS. METHODS: A total of 171 infants who died from SIDS between January 1, 1986 and June 30, 1991, were studied. 154 infants were completely investigated, including a post mortem examination. They were assigned to one of 4 groups, according to whether death was due to diseases of poor prognosis (group A), diseases that are occasionally fatal but potentially treatable (group B), minor diseases not normally fatal (group C), or was essentially unexplained (group D). RESULTS: The classical risk factors for SIDS were found in this population: incidence peaked in males (sex-ratio 1.5), during the cold seasons (62%), between 1 and 6 months of age (94%), mainly between 1 and 4 months of age (84%). Symptoms were definitely present during the 2 days before death in 50%. 20% of cases had clinical histories of congenital disease, complicated or recurrent postnatal disease, or fulminant recent disease. Group A included 107 infants (69% of the 154 completely investigated patients). Premature birth (17.5%) and low birth weight for gestational age (10.5%) were more frequent in our series than in the normal population. CONCLUSION: The cause of death was identified in about 75% of cases. This possibility improves management of further siblings of SIDS victims even though the variety of risk factors makes prevention of SIDS difficult.

Autopsy↗
PubMed1992

Age-related changes in arachidonic acid peroxidation and glutathione-peroxidase activity in human platelets.

Lipid peroxidation, vitamin E level and glutathione-peroxidase activity were determined in platelets from elderly (greater than 68 years) and young (21-43 years) people. To further assess the platelet lipid peroxidation, the metabolism of endogenous arachidonic acid in unstimulated platelets as well as that of the exogenous one were measured in the two groups. The oxygenated metabolites of arachidonic acid were enhanced in the elder population under both conditions tested. In addition, the platelet malondialdehyde content, a marker of the overall lipid peroxidation, was also found significantly increased in platelets from aged subjects. On the other hand, the platelet vitamin E level and the glutathione-peroxidase activity were significantly depressed in the elder group compared to the young one. These results suggest that the increased platelet activation observed with age could be linked to the accumulation of lipoxygenase-dependent peroxides associated with the decreased antioxidative defence of the cells, especially glutathione-peroxidase activity.

Adult↗
PubMed1992

The effect of fluoride treatment on bone mineral crystals in the rat.

In order to investigate the effect of fluoride on bone mineral crystals, we gave groups of female rats 8 mM NaF/L water and distilled water to control groups. The rats were sacrificed at six weeks, three months, and six months. The fluor content of the bone was determined by neutron activation. X-ray diffraction showed no difference in bone crystal size/strain with fluoride treatment. Fourier transform Infrared Spectroscopy (FTIR) showed an increased crystallinity in fluoride-fed animals, which seems to be associated with a decrease of labile phosphate environment. Three carbonate bands have been found in fluoridated and normal bone samples. The distribution of carbonate ions on type A and B sites is strongly affected by fluoride. Type A carbonate is always present in bone, but decreases with increasing bone fluoride content. A carbonate band found at 866 cm-1 may correspond to a fluoride interaction with type B carbonate ions. Lastly, phosphate bands have been found to be shifted towards high wave number, which is probably related to the change in unit cell size induced by the fluoride ion. All these changes induced by fluoride reduce the solubility of bone crystals by direct incorporation of fluoride ions in the apatite lattice and by decreasing the labile phosphate environments.

Animals↗
PubMed1992

Branched-chain amino acid metabolism in isolated perfused liver of cirrhotic rats.

We examined the possible contribution of the liver to the alterations in branched-chain amino acid (BCAA) metabolism in cirrhosis. The livers of male Sprague-Dawley rats with CCl4-induced cirrhosis were removed and placed in a recirculating perfusion system. Net amino acid uptake and release were determined over 55 min. Results were compared with those obtained with control animals, which were either pair-fed or fed ad libitum. Intrahepatic amino acid concentrations were determined at the end of the perfusion. The release of isoleucine and leucine was significantly lower in the cirrhotic livers than in the controls fed ad libitum. There was no difference between the cirrhotic and pair-fed groups with regard to the fluxes of the three BCAA. Intrahepatic concentrations of BCAA were reduced only in pair-fed controls. These results suggest that both cirrhosis and a low protein/calorie diet alter hepatic BCAA flux, but via different mechanisms. In cirrhosis, alterations could be due both to low food intake and to BCAA metabolism in non-parenchymal cells.

Amino Acids, Branched-Chain↗
PubMed1992

Early palliation of univentricular hearts with subaortic stenosis and ventriculoarterial discordance. The arterial switch option.

The optimal Fontan-type operation greatly depends on appropriate initial palliation. Several surgical techniques have been used in infancy to palliate patients with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis. The two most common are pulmonary artery banding and Damus-Norwood procedures. Palliative arterial switch operation is another surgical option that was used in this early series of seven infants. The principle of this operation is to "switch" the subaortic obstruction into a subpulmonary obstruction; the coronary artery relocation on the large pulmonary trunk creates a harmonious aortic root and the connection of the rudimentary ventricular chamber to the pulmonary artery trunk creates a natural protection of the pulmonary vascular bed through the restrictive bulboventricular foramen. Seven infants with univentricular hearts, ventriculoarterial discordance, and subaortic stenosis underwent a palliative arterial switch operation. All infants had an associated aortic arch obstruction of various degrees, including one with interrupted aortic arch, five with coarctation with severe arch hypoplasia, and one with isolated arch hypoplasia. There were three with double-inlet left ventricle, three with tricuspid atresia, and one with transposition of the great arteries with ventricular septal defect and severe right ventricular hypoplasia. The subaortic obstruction was patent at birth in five patients who underwent a palliative switch operation in the first 2 months of life, and rapidly occurred following a previous neonatal pulmonary artery banding associated with arch repair in two patients who underwent a switch operation at 5 and 8 months of age, respectively. The operation includes aortic arch repair without prosthetic material, an atrial septectomy, and the arterial switch. An associated pulmonary shunt was required in five patients and a pulmonary artery banding in one. There was one early death in a patient with [S,L,L] anatomy and congenital atrioventricular block, leading to an early mortality of 14% (95% confidence limits: 1% to 28%). There was one late death. Four survivors are waiting for a Fontan-type procedure, and one survivor had satisfactory right ventricular growth. Early palliative arterial switch operation offers several advantages: reconstruction of a harmonious aortic root, natural protection of the pulmonary bed through the restrictive bulboventricular foramen, prevention of deleterious myocardial hypertrophy, and arch reconstruction without the introduction of a foreign material. This aggressive technique may provide a satisfactory palliation in infants with univentricular hearts and ventriculoarterial discordance, when the bulboventricular foramen/aortic anulus ratio is less than 0.8 or when the subaortic stenosis is severe enough to be associated with an arch obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)

Abnormalities, Multiple↗