[Thyroid lymphoma and Gougerot-Sjögren syndrome].
Explore the source record for details and available documents.
Aged↗
Biomedical subjects
R Jean
Publications and source records attributed to R Jean.
[Late manifestation of GM1 gangliosidosis in an adult. An unusual case of multiple sclerosis].
Explore the source record for details and available documents.
Age of Onset↗
Growth hormone therapy with three dosage regimens in children with idiopathic short stature. European Study Group Participating Investigators.
OBJECTIVE: In children with idiopathic short stature (ISS) we studied the growth-promoting effect at 4 years of recombinant human growth hormone (rhGH) therapy in three dose regimens and evaluated whether increasing the dosage after the first year could prevent a decline in height velocity (HV). DESIGN: Included were 223 patients who were treated with subcutaneous administrations of rhGH 6 days per week. They were randomized to three groups: 3 IU/m2 body surface/day, 4.5 IU/m2/day, and 3 IU/m2/day during the first year and 4.5 IU/m2/day thereafter, corresponding with dosages of 0.2 and 0.3 mg/kg body weight/week, respectively. Growth was compared with a standard of 229 untreated children with ISS [ISS standard]. RESULTS: During the first year of treatment HV almost doubled and was higher with 4.5 IU/m2 than with 3 IU/m2. In the second year HV no longer differed among the groups, but increasing the dosage slowed the rate of the fall of HV. During 4 years of therapy the height SD score for age increased by a mean (SD) of 2.5 (1.0) [ISS standards], or 1.2 (0.7) (British standards), bone age increased by 4.8 (1.3) years, and predicted adult height SD score increased by 1.5 (0.7). After 4 years the results of the group with 4.5 IU/m2 were slightly better than those of the other groups. When dropouts were included in the analysis (assuming a stable height SD score after discontinuation of rhGH therapy), height gain was still significant. CONCLUSIONS: During 4 years of rhGH therapy, growth and final height prognosis improved, slightly more with 4.5 IU/m2 than with 3 IU/m2 or 3 to 4.5 IU/m2. However, bone age advanced on average 4.8 years during this period; therefore, any effect on final height will probably be modest.
Body Height↗
Diagnostic value of nasal provocation challenge with allergens in children.
A total of 106 children suffering from perennial rhinitis and/or asthma, and all allergic to Dermatophagoides pteronyssinus (DP), underwent nasal provocation challenge (NPC) with DP to determine the best method of diagnosis. Posterior rhinomanometry was uninterpretable in 17 patients and gave negative results in 31. Clinical scores for sneezing and rhinorrhea were more effective but did not diagnose the disorder in 11 children. However, nine of the 11 had significant increases in eosinophil count in the late phase. Clinical scores and cytology were also useful for assessing whether NPC with allergens was positive in children. The feasibility and safety of NPC with DP are high for rhinitic and stable asthmatic patients, but mild reactions may occur during the late phase.
Adolescent↗
Nasal cytology in rhinitis children: comparison between brushing and blowing the nose.
Allergic rhinitis is a common disease in childhood, but nasal cytology is rarely used by pediatricians. We compared two techniques of cell sampling, brushing and blowing the nose, among 77 children suffering from chronic rhinitis, of whom 59 were allergic. Staining by the May-Grunwald-Giemsa method enabled the evaluation of the density of cells and especially differential counting of the inflammatory cells. Staining by the Luna method was used as a control for the eosinophils. For the eosinophil count, we found a strong correlation between the two methods of collecting the nasal secretions (r = 0.96). Because blowing the nose is painless and easy to perform, it is more appropriate than brushing in routine use for the diagnosis of allergic rhinitis in children and in nasal challenge with allergens.
Adolescent↗
[Spa treatment in pediatric pneumo-allergology and ENT].
Each year in France, 42,000 children receive spa therapy, which is covered by the national health care insurance system. In over three cases out of four, the treatment is ordered by the child's physician for respiratory tract disease which fails to respond adequately to conventional therapy. Asthma, recurrent bronchitis, and spasmodic cough are the main indications in pneumo-allergology; seromucous otitis media, naso-sinusitis and refractory pharyngitis are the most common pediatric ENT diseases treated in spa centers. The two main types of mineral water used are sulfur-rich waters in patients with prominent infection and chloride and bicarbonate-rich waters when allergy is the main problem. Experimental studies point to the fact that these waters have immunomodulating effects. However, other therapeutic interventions in spa centers, including rehabilitation and health education, also play a role. Evaluations of spa therapy for respiratory tract diseases carried out by government agencies have demonstrated decreases in school absenteeism and above all in the use of drugs in treated patients. The future of pediatric spa therapy will likely depend on the development of preventive interventions in spa centers.
Asthma↗
Ventilatory control during exercise in children with mild or moderate asthma.
The aim of this study was to specify whether during exercise the neural response to increased resistive load in asthmatic children corresponds to a modification of the neuromuscular inspiratory drive, to a modification of the breathing pattern, or to both. Thus, nine children with mild or moderate asthma (aged 10-15 yr) and nine normal children (aged 11-16 yr) were studied during an incremental load exercise with a cyclic ergometer, the load of which was increased by steps of 30 W.3 min-1. During the 3rd min of each workload, we measured the following parameters: O2 consumption (VO2), CO2 production (VCO2), ventilation (VE), tidal volume (VT), respiratory frequency (f), ratio of inspiratory to total time of respiratory cycle (T1/TTOT), mean inspiratory flow (VT/T1) as well as mouth occlusion pressure measured at 100 ms (P0.1), and inspiratory power for breathing (W). At maximum level, the two groups showed identical values for heart rate, ventilation divided by weight (VEBW), T1/TTOT), VT/T1, P0.1, and W. However, asthmatic children had lower maximal power (P less than 0.02), higher tidal volume divided by weight (VTBW) (P less than 0.05), and lower f (P less than 0.01). At a same level of exercise (60, 90, or 120 W), in both groups, we found identical values for P0.1, VEBW, VO2, T1/TTOT, and VTBW/T1. However, asthmatic patients exhibited higher VTBW and lower f(limit of significance). This resulted from higher inspiratory and total time durations. Furthermore, they showed a higher inspiratory power for breathing. It was the same for f and VTBW if the results were expressed in relation to the VO2 in ml.kg-1.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Extracardiac malformations in tetralogy of Fallot].
The purpose of our study was to determine the incidence and type of malformations associated with tetralogy of Fallot (TF). Among 133 patients followed up for 12 years, 30 who had either complete TF (n = 26) or pulmonary atresia and interventricular septal defect (n = 4) presented with another, extracardiac malformation. These malformations were part of a malformative syndrome in 21/30 patients, including 4 trisomies 21, 6 embryofoetopathies unquestionably (antiepileptics n = 2, alcohol n = 1) or possibly (hormonal treatment n = 1) of environmental origin, 6 branchial arch pathologies and 5 miscellaneous syndromes. The extracardiac malformation was isolated in the remaining 9 patients, including 5 cases of skeletal anomaly and one case each of omphalocele and microcephaly. Altogether, in these 30 patients skeletal and neurosensory anomalies were largely predominant. Skeletal anomalies involved the vertebral column in 8 cases (cervical 3, thoracic 4, lumbar 1) and the limbs in 5 cases (2 of which were phocomelias). The 4 patients with pulmonary atresia and interventricular septal defect also presented with an early embryopathy: Shprinzen's velocardiofacial syndrome (n = 2), DiGeorge syndrome (n = 1) and situs inversus (n = 1). We compared TF with other congenital heart diseases in our population and found that the incidence of associated malformations was about average. The various associations are discussed. In genetic syndromes, trisomy 21 predominates and TF is less frequent than atrioventricular canal. In syndromes of environmental origin, the role of antiepileptic drugs (chiefly phenytoin and trimethadione) is well-known, alcohol is less often responsible and the TF-phocomelia association is suggestive of progesterone. In branchial arch syndromes, TF is the usual cardiopathy.(ABSTRACT TRUNCATED AT 250 WORDS)
Abnormalities, Drug-Induced↗
[Isolated cerebral calcifications after prophylactic treatment of cerebromeningeal involvement of acute lymphoblastic leukemia: relation of psycho-intellectual sequelae].
Intracranial calcifications were demonstrated by CT scan in 5 children after complete remission of acute lymphoblastic leukemia (ALL). Initial treatment included prophylactic irradiation of central nervous system and intrathecal methotrexate. Behavioral abnormalities or learning difficulties were clinically apparent in all children at the time of the radiologic examination. Isolated intracranial calcifications represent one of possible cerebral sequelae that are to be found in 53% of children receiving treatment for ALL. No fine correlation between direct toxic effect of methotrexate or, post-radiation lesion, and neuro-psychological sequelae can be done. In an attempt to avoid some of this sequelae, suppression of cranial irradiation from ALL treatment protocols is now studied.
Brain Diseases↗
Nasal eosinophilia in children: its use in the nasal allergen provocation test.
This pediatric cytological and clinical study aimed at assessing the value of nasal eosinophilia during nasal provocation tests for identifying an offending allergen. The population studied comprised 50 children aged from 4 to 18 yr; 39 of these had well-characterized allergic rhinitis, which in 21 cases was combined with asthma, and the remaining 11 had nonatopic chronic rhinitis. Nasal secretions, collected by nose blowing, were stained with May-Grünwald-Giemsa or Wright stain. The percentage of nasal eosinophils was obtained by examining the cells in the whole slides. Counts were carried out on secretions collected before challenge, after insufflation of saline solution (for verification), and 40 min after insufflation into each nostril of an allergen (housedust mite extract). The nasal provocation test was considered positive when insufflation of the allergen increased nasal eosinophilia by more than 10%, provided that the prechallenge proportion of eosinophils was less than 50%. No increase in specific bronchial resistance was noted. These results indicate that nasal provocation tests are safe, even in asthmatic children.
Adolescent↗
[Congenital heart diseases and urinary malformations].
The purpose of this study was to evaluate the advisability of a systematic search for uropathy in patients with malformative heart disease. Thirty-three cases of urinary tract malformation associated with congenital cardiopathy are reported. These cases represent 2.8 p. 100 of all cardiac patients seen during the same period. The congenital heart diseases were varied, with a predominance of ventricular septal defect (48 p. 100) followed by dextrocardia, single ventricle and coarctation of the aorta. UT abnormalities included vesico-ureteral reflux (36 p. 100), renal agenesis (5 cases), renal dysplasia or hypoplasia (4 cases), upper UT obstruction (4 cases), hypospadias (3 cases), renal ectopia (3 cases), lower UT obstruction (2 cases), polycystic kidney, megaloureter, horseshoe kidney and supernumerary kidney (1 case each). A study of the literature showed that the two uropathies with a risk of associated cardiopathy are renal agenesis and horseshoe kidney. The cardiopathy-uropathy association was found in isolation (11 cases) or combined with cryptorchidism (2 cases) or with multiple malformations (20 cases). The malformations were diverse, the most frequent being neurosensorial malformations (70 p. 100), followed by skeletal malformations (55 p. 100) equally divided between apine and limbs, genital malformations (35 p. 100) and digestive tract malformations (25 p. 100). A study of the cardiopathy-uropathy concordances failed to elicit any predominant association; in the literature, ventricular septal defect is usually associated with horseshoe kidney.(ABSTRACT TRUNCATED AT 250 WORDS)
Abnormalities, Multiple↗
[Fatal cerebral toxoplasmosis in a leukemia child in remission].
We report the case of a 4 year-old boy who died from cerebral toxoplasmosis while in remission from acute lymphoblastic leukemia. The clinical picture was remarkably insidious and non specific: diagnosis is particularly difficult in immunodepressed patients. Diagnosis was made on seroconversion. No autopsy was performed. An association of sulfadiazine-pyrimethamine is often effective, but the prognosis is poor in the absence of a specific and precocious treatment.
Brain Diseases↗
[Bronchiolitis in infants. Respiratory functional exploration in the acute phase].
Respiratory function tests were performed in infants hospitalized for bronchiolitis immediately after the acute stage, in order to appreciate the function of the respiratory muscles. A study of the ventilatory mechanics, of the transdiaphragmatic pressure, of the ventilatory rate and of the blood gases was performed. Results were compared with those obtained in 10 "healthy" infants. They showed: a decrease in the dynamic compliance (DYN.C) (p less than 0.001) and an increase in the total pulmonary resistances (TPR) (p less than 0.05) providing evidence for the intensity of bronchial and bronchiolar obstruction; an increase in the abdominal and intrathoracic pressures difference (Pdi) (p less than 0.01) which may reach 3 times the normal value and which is proportional to the decrease in DYN.C. (r = 0.74; p less than 0.001), providing evidence for the intensification of the inspiratory diaphragmatic effort and of its adjustment to the degree of obstruction; a reverse of the abdominal pressure curve with respect to that in normal subjects, with an increased pressure level, providing evidence for abdominal distension and increased pressure during all the expiration, suggesting the active participation of the abdominal muscle to expiration; a change in the ventilatory rate, characterized by a high respiratory frequency, a decreased tidal volume, a shortened inspiratory time and a normal inspiratory time/total time ratio. this rate is opposed to that observed in older children with bronchial obstruction. It might optimize the respiratory muscles output, as at this age, they have not yet reached all their contractile capacity.
Acute Disease↗
[Intestinal malformations and congenital heart diseases].
A series of 21 patients with both congenital heart disease and intestinal malformation seen over a 12-year period is reported. The intestinal malformations were: anorectal malformations (11 cases), duodenal atresia (5), omphalocele (4) and common mesentery (1). Congenital heart diseases consisted of: atrial septal defect (ASD) (10 cases), ventricular septal defect (VSD) (2), tetralogy of Fallot (2) and miscellaneous cardiopathies. In patients with anorectal malformations ASD and VSD predominated (6/11 cases) and multiple malformations syndromes were present in 8 cases, including trisomy 13, Vater syndrome, skeletal (4), neurological (3) and renal abnormalities (3); 3 children died. Duodenal atresia was always associated with left-to-right shunt: VSD (3), ductus arteriosus (2), complete atrioventricular canal (1) and trisomy 21 (2); one child died. Omphalocele coexisted with VSD (2), tetralogy of Fallot (1), dual outlet right ventricle (1), trisomy 21 (1) and multiple malformations syndromes (3); 2 children died. The patient with common mesentery had left-to-right shunt. Comparison of this series with data from the literature showed that children with congenital heart disease have a much higher incidence of intestinal malformations than those with normal heart and that they frequently present with multiple malformations (chromosome aberrations or multiple organ lesions). This multiple malformation complex is particularly common in anorectal malformations where the incidence of congenital heart diseases is 9 to 14%, with predominance of VSD and tetralogy of Fallot. In patients with duodenal atresia trisomy 21 is extremely frequent, and the incidence of cardiopathies is 18%; in the absence of trisomy 21 the cardiopathy is complex.(ABSTRACT TRUNCATED AT 250 WORDS)
Abnormalities, Multiple↗
[GRF and nocturnal GH secretion in children].
Explore the source record for details and available documents.
Child↗
[Alveolar microlithiasis in children. Contribution of bronchoalveolar lavage].
This case of pulmonary alveolar microlithiasis emphasizes the rarity of the disease and its exceptional diagnosis in infants which relies on the pulmonary pathologic study. Besides allowing for diagnosis, the study of the alveolar brushing fluid permitted to describe for the first time pictures of post-necrotic cellular calcification which is probably responsible for the disease.
Calcinosis↗
[Oral occlusion pressure in scoliosis and neuromuscular syndromes].
Oral occlusion pressure, a neuro-muscular index of the activity of the respiratory centers, was measured in 2 groups of children: one of 43 "healthy" children, in order to establish a predictive equation according to age (Y = 1.23 + 8.30 X A-1) and another of children presenting with respiratory failure, either due to an impairment of the thoracic cavity (10 dorsal scoliosis) or to an impairment of the respiratory muscles (29 children with muscular dystrophy or spinal muscular atrophy). The occlusion pressure increases with the degree of the ventilation deficiency in children with scoliosis. It remains paradoxically normal in children with neuro-muscular disease, irrespective of the ventilation deficiency. In the latter, the respiratory centers activity being considered as normal, this situation would indicate their muscular incapacity to correctly express this activity. It would show a severe muscular deficiency with a high risk of occurrence of respiratory distress accidents.
Adolescent↗
[Idiopathic hirsutism in girls: in vitro physiopathologic studies].
Explore the source record for details and available documents.
Adolescent↗