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Biomedical subjects

G Touati

Publications and source records attributed to G Touati.

69 records · Page 4Linked to original sources
PubMed1990

[Tumors of the heart in newborn infants and infants. Apropos of 5 cases].

Between February 1985 and March 1987, 5 children underwent resection of primary cardiac neoplasms, 3 of them in the first days of life and 2 before the age of 6 months. Routine echocardiographic follow-up of pregnancies allowed detection of cardiac tumors in 2 foetuses 30 and 36 weeks old. In 3 children the diagnosis was suspected by the discovery of cardiac murmur or congestive heart failure. The first case was a hemangioma, originating from outside the left ventricular wall, and was resected without cardiopulmonary bypass (CPBP). The other case was a pseudomyxoma, spreading extensively in to the right atrium. The third case was a rhabdomyoma arising from the pulmonary infundibulum with clinical manifestations of tuberous sclerosis. The last two patients had intraseptal lesions, just above the aortic valve; complete resection was therefore impossible, particularly in one patient with multiple tumors. There was one death related to congestive heart failure. The remaining four survivors were followed up for an average of 18.4 months (+/- 12.9) and all were in functional class I. Echocardiographic follow-up showed evidence of a residual subaortic lesion in one asymptomatic patient.

Female↗
PubMed1989

Heart- and heart-lung transplantation in children.

Since January 1987, 16 prepubertal children have undergone heart (13) or heart-lung (3) transplantation. Immunosuppression included cyclosporine and azathioprine and excluded steroids except in case of rejection. The indications for heart transplantation were hypoplastic left heart syndrome (4 infants, mean age = 2 months), congenital heart disease (4 patients, mean age = 5.7 years) and cardiomyopathy (5 patients, mean age = 2.8 years). There were 4 early deaths (acute graft failure in 2, pulmonary hypertension in 1, infection in 1) and 1 late death (heart failure at 3 months). The 8 survivors had a mean follow-up of 12 months (range 1-19 months). Late complications were minimal. There were 4 episodes of rejection in 2 patients. There was no infection, normal somatic growth and no systemic hypertension. Renal function remained within normal limits although mild-to-moderate tubulointerstitial lesions were found in 4 renal biopsies. Three children (9-11 years old) underwent heart-lung transplantation. The early postoperative course was difficult with 6 episodes of rejection and 5 infections. One patient died at 3 months from infectious complications. One child has a complete rehabilitation 8 months posttransplantation. The last patient is clinically well at 7 months but has a residual tracheal stenosis. The long-term fate of these children, and particularly the long-term effects of cyclosporine therapy are unknown. Heart and heart-lung transplantation remain under investigation but may be reasonable approaches for infants and children with end-stage cardiac and/or pulmonary disease.

Acute Kidney Injury↗
PubMed1988

Coarctation of the aorta in infants: which operation?

In this series, 178 infants (age, less than or equal to 3 months old) underwent repair of aortic coarctation. Pure coarctation was present in 63 patients (Group 1), 47 infants had additional ventricular septal defects (Group 2), and 68 patients had associated complex heart disease (Group 3). Subclavian flap angioplasty was used in 26 patients, limited resection and end-to-end anastomosis in 45 patients, extended resection and end-to-end anastomosis in 99 patients, and miscellaneous procedures in 8 infants. The early mortality was 8% for the first group, 11% for the second group, and 37% for the third group (p less than 0.001). Mean follow-up was 32 months and included 97% of patients. Actuarial survival at five years was 90% for the first group, 84% for the second group, and 40% for the third group. Recoarctation occurred in 15 operative survivors (11%); 7 necessitated reoperation. Freedom from recoarctation at five years was 89% after subclavian flap angioplasty, 81% after end-to-end anastomosis, and 86% following extended resection and end-to-end anastomosis. Early mortality and late results were not influenced by the type of coarctation repair but were determined by the clinical status and the presence of associated major cardiac anomalies. These results suggest that the surgical procedure should be individualized for each infant to optimize the aortic anatomy.

Anastomosis, Surgical↗
PubMed1988

[Cardiac transplantation in the infant and young child. Preliminary results].

Between January and December, 1987, a programme of heart transplantation in paediatrics was designed and carried out in 9 children by the medical and surgical teams of the Necker/Enfants Malades-Laënnec hospitals group, Paris. Six of the patients were infants of less than 2 years (4 were under one year), and the oldest child was 10 years old. All patients seemed to be condemned to an early death either because their congenital heart disease was beyond the resources of conventional surgery (6 cases) or because their dilated cardiomyopathy was refractory to all medical treatments. Three children died at the end of the operation or a few days afterwards, due to poor quality graft (1 case), fulminating bacterial superinfection (1 case) or intractable pulmonary hypertension (1 case). The remaining 6 children are now living as normally as possible in their respective families. The long-term immunosuppressive treatment consists of cyclosporine and azathrioprine; corticosteroids are only used at the very beginning of treatment or in case of graft rejection. Only two episodes of rejection, confirmed by endomyocardial biopsy, were observed in the same patient during the first postoperative month. Biopsy was never performed systematically in order to spare the patient's vein, and the diagnosis of rejection was suspected on clinical grounds.(ABSTRACT TRUNCATED AT 250 WORDS)

Biopsy↗
PubMed1988

[Heart and heart-lung transplantation in the child. Indications, methods and results].

Over a 20 month period, 19 children aged 13 days to 15 years (10 under 3 years), received a heart transplantation (isolated: 16 cases, heart-lungs: 3 cases). There were 5 early deaths and 2 during the 3rd postoperative month (total: 44%). Postoperative period is currently over for 8 of the 9 survivors, who lead a normal life, with rather simple constraints: oral cyclosporin-azathioprine immunosuppressive therapy, monthly evaluation in the out-patient department. The method is relatively simple when it concerns heart transplantation. It is quite heavier and complex when it concerns heart-lung transplantation. On the whole, subject to a rigorous selection of the transplant (whose later dysfunction was the major cause of failure, with bacterial or viral infections), these methods find reasonable indications in a number of heart and/or lung malformations or dysfunctions inaccessible to conventional treatments or which used up their resources, making short-term death likely.

Adolescent↗
PubMed1988

Aortic coarctation with hypoplastic aortic arch. Results of extended end-to-end aortic arch anastomosis.

Between 1980 and 1986, 80 infants (less than or equal to 3 months old) with symptomatic aortic coarctation and associated severe tubular hypoplasia of the transverse aortic arch underwent surgical treatment. Extended end-to-end aortic arch anastomosis was used in an attempt to correct both the isthmic stenosis and the hypoplasia of the transverse arch. After complete excision of the coarctation tissue, a long incision was made in the inferior aspect of the aortic arch, which was then anastomosed to the obliquely trimmed distal aorta. Pure coarctation was present in 17 patients (group I); 24 infants had an additional ventricular septal defect (group II), and 39 patients had associated complex heart disease (group III). The overall early mortality rate was 26% (confidence limits 21% to 32%) (18% in group I, 17% in group II, and 36% in group III). The early risk declined with time and was 18% (confidence limits 12% to 26%) for the last 2 years (seven deaths in 39 patients). Follow-up was 100% for a mean of 19 months. Actuarial survival rate at 3 years was 82% for group I, 78% for group II, and 32% for group III. Recurrent coarctation (gradient greater than or equal to 20 mm Hg) occurred in six operative survivors (10%, confidence limits 6% to 16%) and necessitated reoperation in three. Freedom from recoarctation at 4 years was 88%. Because extended end-to-end aortic arch anastomosis provides adequate correction of the aortic obstruction and entails a low risk of restenosis, it is our procedure of choice in infants with coarctation and severe hypoplasia of the aortic arch.

Actuarial Analysis↗
PubMed1987

[Valvuloplasties for acquired mitral insufficiency in children (Carpentier's technic). Long-term results in 87 cases].

Between March, 1969 and March, 1984, 89 children aged from 2 to 12 years (mean: 8.3 +/- 2.5 years) and presenting with mitral valve regurgitation underwent valvuloplasty by the Carpentier technique. The cause of the regurgitation was rheumatic fever in 84 cases (94 p. 100), bacterial endocarditis in 4 cases and Barlow's disease in 1 case. Mitral valve regurgitation was divided into 3 types, namely: type I, normal valve motion (5 cases), type II, valve prolapse (74 cases) and type III, restricted valve motion due to fibrosis of the leaflets or chordae (20 cases). The hospital mortality rate was 2.3 p. 100 (2 deaths). The cumulative follow-up was 546 patients/years, and the actuarial survival rate at 10 years was 89.96 +/- 8.5 p. 100. At 10 years the actuarial thromboembolic complication rate was 2 p. 100, or 0.3 +/- 0.2 p. 100 per patient year, and the actuarial valvuloplasty deterioration rate was 27 +/- 8.5 p. 100. The risk of re-operation was 2.2 +/- 0.6 p. 100 per patient year. At 10 years 78.4 +/- 7.2 p. 100 of the children were free of all re-operation, and 69 p. 100 had no complication. Thus, whenever possible (i.e. in 90 p. 100 of the cases, according to our experience) and considering the satisfactory long-term results, all children with acquired mitral valve regurgitation should undergo mitral valvuloplasty as first-line treatment.

Child↗
PubMed1986

Long-term results of valve repair in children with acquired mitral valve incompetence.

From March 1969 to March 1984, 89 children aged 2 to 12 years (mean 8.3 +/- 2.5) with acquired mitral valve incompetence underwent mitral repair using Carpentier's techniques. There were 84 cases of rheumatic valve disease, four cases of endocarditis, and one case of Barlow's syndrome. Valve dysfunction was classified into three types: type I (normal leaflet motion), five patients; type II (prolapsed leaflet), 74 patients; and type III (restricted leaflet motion), 10 patients. Cumulative follow-up was 546 patient/years. At 10 years, 90% of the patients were still alive, with an incidence of valve-related death of 5.5%; 98% of the patients were free of thromboembolism, 78% were free of reoperation, and 69% of the patients were free of any complications related to valve repair. We conclude that whenever feasible (92% of the cases in our experience), mitral valve repair using valvuloplasty techniques is the preferred procedure in the surgical treatment of acquired mitral valve incompetence in children.

Child↗
PubMedDate not supplied

Methylmalonic and propionic acidurias: management without or with a few supplements of specific amino acid mixture.

In a series of 137 patients with methylmalonic acidaemia (MMA) and propionic acidaemia (PA) diagnosed since the early 1970s, we report in more detail 81 patients (51 MMA and 30 PA) diagnosed between 1988 and 2005. In this series, 14% of patients died at initial access revealing the disease before or despite treatment, 18% died later, and the remainder (68%) are still alive. All patients were treated with the same protocol of enteral feeds with a low-protein diet adjusted to individual tolerance, carnitine, antibiotics, and only occasional use of an amino acid (AA) mixture. There was intensive follow-up and monitoring using measurements of urinary urea. Thirty-nine patients with severe forms, followed for more than 3 years, are analysed in particular detail. Of the 17 PA patients, 6 had moderate disability (all neonatal-onset forms), whereas 11 were normal or slightly delayed in their mental development. Four presented with cardiomyopathy, of whom 2 died. Of the 22 MMA patients, 13 presented in the neonatal period, of whom 3 died later, 2 are in renal failure and only 5 are still alive and have a normal or slightly delayed mental development. In the 9 patients with late-onset forms, there were no deaths and all patients but one have normal mental development. Among the 39 patients, only 40% were given an AA supplement at 3 years, and 50% between 6 and 11 years. The actual intake of natural protein was 0.92, 0.78 and 0.77 g/kg per day at 3, 6 and 11 years, respectively, in patients without AA supplementation, whereas it was 0.75, 0.74 and 0.54 g/kg per day in the group who received small quantities of AA (0.4-0.6 g/kg per day). In both groups, feeding disorders were frequent: 55% at 3 years, 35% at 6 years and 12% at 11 years. Many patients were given a food supplement by tube overnight or were even exclusively tube fed: 60% at 3 years, 48% at 6 years and still 27% at 11 years. Growth velocity was near the normal values. Plasma valine and isoleucine were low to very low, as were leucine and phenylalanine but to a lesser extent. Albumin, vitamins, trace elements and markers of bone metabolism were within the normal values. IGF1, 24-hour urine calcium and body mass density were low. Body composition showed a normal to low lean mass and a normal to high fat mass.

Amino Acid Metabolism, Inborn Errors↗
PubMedDate not supplied

[Congenital hypothyroidism. Therapeutic strategy at the early stage of treatment].

The aim of this work was to determine the optimum dosage of L-thyroxine (L-T4) given to infants with congenital hypothyroidism (CH). Thirty seven hypothyroid infants diagnosed through the French screening programme for CH have been treated in our clinic. The study analysed the biochemical parameters (TSH, FT4, FT3) and the L-T4 doses during the first year of life. Treatment was started at 23 days of age (range: 13 to 37). A dose of 7.5 micrograms/kg/d of L-T4 was given at diagnosis. After 2 weeks of treatment, FT3 was normal and FT4 at the upper limit of the normal values. At that time, TSH plasma levels were normal (less than 6 micro UI/ml) in 47% of cases. After 1.7 month of treatment, 22% of patients had TSH levels greater than 10 micro UI/ml despite normal FT4 and FT3. This group of patients, despite being given an identical L-T4 dose, had a significantly lower FT4. They were not different from those who normalized TSH levels in terms of etiology, delayed bone maturation and levels of FT4 or FT3 at diagnosis. In conclusion, an initial dose of 7.5 micrograms/kg of L-T4 normalized FT4, FT3 and TSH in 80% of our patients. Twenty percent of patients seem to need more L-T4 to bring TSH levels back to normal at the end of the second month of treatment.

Congenital Hypothyroidism↗
PubMedDate not supplied

[Gammacisternography and fronto-basal head injuries (author's transl)].

The authors give the results of 65 radioisotopic cisternographies for suspected rhinorrheas in frontal basal head injuries. Indium 111-DTPA lombar injection allows the investigation at least for 48 hours. After localisating the CSF fistulae, the gammacisternography permits us to study associated CSF circulation anomalies. Results and discussion give the correlation between positive tests and surgical findings. The best successful conditions of exploration are enough activity and a fluent clinical leakage of CSF. Nasal coton pledgets improve the success of the exploration.

Cerebrospinal Fluid Rhinorrhea↗