Biomedical subjects
Publications and source records attributed to R Mariani.
We report on a case of Sharp syndrome which was diagnosed late in on 11-year old boy. After 5 years of corticosteroid treatment, he presented with myopericardic aggravation, glomerular disease, and seizures with delirium, which responded to high-dose steroid therapy with early switch using synthetic antimalarial drugs. Six years later the results are still satisfactory.
Magnetic resonance imaging (MRI) of the sellar and juxta sellar area was performed in 40 children with idiopathic hypopituitarism. MRI was normal in the 17 patients with partial growth hormone (GH) deficiency and in the 5 patients with GH neurosecretory dysfunction. Among the 18 patients with complete GH deficiency, the 10 with pituitary stalk transection presented more often with perinatal abnormalities, had the most severe GH deficiencies and more frequently multiple hormone deficiencies, in particular TSH deficiency. Pituitary gland size studies showed that the small size of the residual glandular tissue was responsible for these particularities. If MRI is of no great interest for functional GH deficiency, in half of the primary complete GH deficiencies, it enables to visualize a pituitary stalk transection with regeneration of neurohypophysis.
During the last fifteen years, inhaled corticosteroids were increasingly used for prophylaxis in childhood asthma. The development of inhaled corticosteroids with a high ratio of topical vs systemic potency, now makes possible their use in large doses which are needed to control the most severe forms of the disease, in order to avoid long term oral corticosteroid treatment for these patients. The efficacy of inhaled corticosteroids depends on an optimal pulmonary deposition of the drug which is obtained with children by inhaling the drug via a large volume spacer. Beyond their beneficial effects on the clinical condition of the patients and on pulmonary function, inhaled corticosteroids should improve the long term prognosis of asthma, by reducing chronic inflammation in the airways and the associated bronchial hyperreactivity. At usual dosages, inhaled corticosteroids are free of harmful side effects. However, caution and close observation of adrenal function and growth development are required when prescribing inhaled corticosteroids at high dosages for a long period. Nevertheless, inhaled corticosteroids present very few serious side effects compared with those induced by long term oral corticosteroid treatment.
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Fifteen children and adolescents with muscular dystrophy underwent investigation by Doppler echocardiography and high amplification electrocardiography to evaluate the quality of left ventricular function. High amplification ECG showed minor intra-atrial conduction defects in 3 cases and abnormal late micro potentials without arrhythmogenic late potentials in 3 other cases. Significant abnormalities were observed in 40% of children examined. Doppler echocardiography showed relative conservation of systolic left ventricular function but a decrease in indices of contractility was observed in 46% of cases, especially in patients with Duchenne muscular dystrophy. Abnormalities of left ventricular distensibility were observed earlier and were more common (53% of cases). A significant decrease in cardiac output was found in 50% of patients, especially among the older and more severely affected children irrespective of the type of muscular dystrophy. These two investigations provide complementary information for the evaluation of left ventricular function of children with muscular dystrophy and they are proposed routinely for the follow-up of these patients.
The variability of blood pressure measured by oscillometry and the validity of this technique were studied in premature infants. Thirty premature babies whose postnatal age was six days or more had their blood pressure measured 11 times over a twenty-minute period for three consecutive days. Movements were found to have a very substantial influence on results that could not be reliably compared with values obtained invasively. No consistent pattern of variations of oscillometric results obtained during the eleven measurements of use for recommending a specific measurement time was found. Diastolic blood pressure fell gradually over the three-day study period. The most reliable of the eleven daily values was the lowest value for which good correlations were found between the three study days; this lowest value was not influenced by neonatal or postnatal factors.
The various ways in which infants may be contaminated with HIV are described. Transmission by blood perfusion persists in only some countries where HIV detection in blood products is not performed and sterilization is doubtful. Transplacental contamination is well documented by the finding of HIV in various foetal tissues, amniotic fluid, placenta and foetal blood; it seems to be predominant. Transmission during delivery and breast-feeding plays a lesser role, but it cannot be excluded. Horizontal transmission is extremely rare. The frequency of vertical transmission varies from one series to another, but the most often quoted figure is approximately 30 p. 100. An Atlanta C.D.C. stage III or IV and a low percentage of T4 lymphocytes are thought to facilitate the mother-to-foetus transmission. Many elements are still imperfectly known; their elucidation requires further studies on large series of subjects.
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During 1989, a nosocomial infection rate of 3.15% was observed among the 412 neonates hospitalised for more than 2 days in the paediatric intensive care unit and a special care baby unit in the medical centre of Nice. Certain factors only partially explain the above, and it is probable that the non-invasive methods of monitoring and care and the experienced nursing staff contribute to a great extent to this low frequency of infection.
Sixteen bronchoalveolar lavages (BAL) were performed in 15 children with HIV1 seropositivity, 12 of them being infected by HIV1. BAL was performed during episodes of acute pneumonitis with respiratory distress (group I: three cases) or without severity (group II: five cases), or in the presence of asymptomatic radiological pulmonary abnormalities (group III: seven cases). A specific diagnosis of infection was obtained in five cases of acute pneumonitis and 12 micro-organisms were identified by BAL: three cytomegaloviruses, three respiratory syncytial viruses, two Pneumocytis carinii, one Haemophilus influenzae, one Herpes simplex virus type 1, one Escherichia coli and one group A streptococcus. In three cases two micro-organisms were simultaneously identified. Cytological examination showed a high proportion of polymorphonuclear leukocytes in cases of acute pneumonitis (group II) and alveolar lymphocytosis in clinically asymptomatic children with radiological pulmonary abnormalities (group III). BAL appears to be a reliable tool for the investigation of pulmonary infections in children with HIV1 seropositivity. In addition it has the advantage of revealing latent cytological abnormalities in these patients.
The outcome of infants born to HIV1 seropositive (HIV1+) mothers is still uncertain is spite of current progress in techniques for viral detection. Using the almost elective tropism of HIV1 virus for CD4 lymphocytes and the central role of these cells in the immune regulation, we studied blood lymphocyte populations with 7 monoclonal antibodies during the first month of life in 28 newborns of HIV1 seropositive mothers. Our data are compared to an age-matched population of 35 control infants. According to our results, the coexistence of a low CD8 lymphocyte percentage together with a high CD4/CD8 ratio seems to be correlated with an unfavourable outcome.
The authors report on the case of a newborn infant without pathological history suffering from severe and lasting myoclonus occurring during deep sleep and associated with normal electroencephalogram. Deep sleep myoclonus is a benign syndrome which disappears spontaneously within a few months. The long-term prognosis is excellent, but the etiology remains unknown.
Ovulation induction and in vitro fecundation have increased the incidence of multiple pregnancies. We report the neonatal aspects of 29 triplet pregnancies. The presence of neonatologists at every delivery and during all the transfers to the neonatal unit probably accounts for the low morbidity and the absence of an early and late neonatal mortality of our study.
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Peak expiratory flow (PEF) variation was measured in 18 healthy children (Group I) aged 9.25 to 13.3 years (mean, 11 years) and in 17 asymptomatic asthmatic children (Group II) aged 8.75 to 14.25 years (mean, 11.9 years) receiving no bronchodilators. PEF was measured four times a day for a period of 10-14 days. There were no differences between Group I and Group II regarding age, height, weight, sex, duration of the study, and overall mean PEF values. Mean daily PEF variation amplitude (difference between the highest and the lowest daily values as a percent of the mean) was significantly higher in Group II than in Group I subjects (15.2 vs. 9.9%), and so was cosinor amplitude (9.6 vs. 5.9%). Significant circadian rhythm in PEF was detected by cosinor analysis in nine subjects of Group I and in eight subjects of Group II. Mean PEF bathyphase calculated by cosinor analysis (trough time in a 24 h scale) was similar in the two groups (0503 h, in Group I and 0434 h in Group II). The results of this study show that the amplitude of circadian variation in PEF tends to be higher in asymptomatic asthmatic children than in normal children, but there is too much overlap to use PEF monitoring to identify an individual asymptomatic asthmatic child. Therefore, diurnal variation of PEF is not suitable for diagnosing mild asthmatics.
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The usual method of measuring arterial blood pressure cannot be used in children less than 4 years of age because of lack of cooperation. We investigated variations of blood pressure measured during 20 minutes by an oscillometric method (Dinamap) in 100 children aged 4 to 48 months and tried to come up with some recommendations for measuring and interpreting blood pressure. Serial blood pressure recordings showed poor reproducibility due to movements which are impossible to avoid and which account for wide variations. Because of this variability, a single measurement is inadequate and must be repeated. The average of repeated recordings does not seem to be a reliable parameter. We suggest that the lower measured value (minima) as the only parameter allowing us to track the progressive increase in blood pressure with age. The data probably explain the large discrepancies between published studies taking into account the use of different methods.