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The strategy for morphological investigations in children with acute pyelonephritis (APN) remains debatable. We studied 70 children (median age 2.0 years) admitted with a first episode of pyelonephritis using a high-resolution ultrasound technique (RUS) and compared the results with 99m technetium-dimercaptosuccinic acid (DMSA) renal scintigraphy. The DMSA scan was abnormal in 62 children (89%). However, using a high-frequency transducer we found abnormal sonogram changes in 61 children (87%), consisting of an increased kidney volume in 42, and/or a thickening of the wall of the renal pelvis in 42, and/or a focal hyper- or hypoechogenicity in 36, and/or a diffuse hyperechogenicity in 31 children. Micturating cystourethrography was performed in all children, revealing vesicoureteral reflux (VUR) in 22 (31%). Among those children with VUR, 4 had a normal DMSA scan, 2 an abnormal RUS, and 2 a normal DMSA scan and RUS. Our data suggest that B-mode RUS performed with a high-frequency transducer by a trained radiologist is nearly as sensitive as the DMSA scan in diagnosing renal involvement in children with unobstructed APN and in predicting VUR.

One hundred and four children suffering from Haemophilus influenzae meningitis were studied in a multi-centre study in France between October 1991 and June 1993. The clinical and bacteriological findings were similar to those usually found in this condition. Fifty three per cent of the H. influenzae strains cultured produced beta-lactamase and 98% were of type b. Forty-six children who had been prescribed oral antibiotics before hospitalisation were analysed in this study. This analysis took into account the nature of the antibiotic and whether it was prescribed as the treatment of first or second intention. Treatment failures, defined according to pre-established criteria, were found to be independent of whether or not the incriminated strain was beta-lactamase producing and whether or not it was sensitive in vitro to the antibiotic prescribed. None of the 46 children who had received pre-hospital oral antibiotics died while three of the 58 who had received pre-hospital oral antibiotics died. However, it is difficult to establish in our study a clear relationship between the reduction of mortality and previous oral antibiotic treatment in bacteriologically proven cases of H. influenzae meningitis.

To assess the ability of baseline MRI characteristics to predict the early development of clinically definite MS (CDMS) and combined CDMS/MRI outcomes in 190 patients with a positive MRI at the time of their first demyelinating event.Based on individual and sets of baseline MRI characteristics, the authors evaluated the percentage of patients meeting outcomes of CDMS and various combined CDMS/MRI outcomes by 18 months. They also optimized a cutpoint for dichotomizing each baseline MRI characteristic and evaluated these variables using logistic regression to determine which MRI characteristics best predicted CDMS by 18 months.The presence of two or more gadolinium-enhancing lesions better predicted the development of CDMS and combined CDMS/MRI outcomes by 18 months than any other individual MRI characteristic or set of MRI characteristics. Among patients with two or more gadolinium-enhancing lesions, 52% developed CDMS compared with 24% of patients with fewer than two lesions. For those meeting the criteria of Barkhof et al., 32% of patients developed CDMS compared with 16% of those not meeting these criteria. Irrespective of individual or sets of criteria, however, the majority of patients developed either CDMS or demonstrated disease activity on brain MRI by 18 months.For patients with positive MRI at the time of their initial neurologic event, both gadolinium-enhancing lesions and the Barkhof criteria are predictors for development of CDMS over a short interval. However, these results, based on a combined CDMS/MRI outcome, suggest that the majority of these patients are already in the earliest stages of MS, regardless of whether any further MRI criteria are met.

We evaluated 190 patients in the placebo group of the CHAMPS trial in order to assess factors associated with short-term clinical and brain magnetic resonance imaging (MRI) outcomes in patients with a first clinical demyelinating event involving the optic nerve, spinal cord, or brainstem/cerebellum, and subclinical demyelination on brain MRI. The two study outcomes were 1) development of clinically definite multiple sclerosis (CDMS) and 2) development of CDMS or two or more new or enlarging brain MRI T2 lesions. The presence of gadolinium (Gd)- enhancing lesions on the baseline scan was the only MRI characteristic associated with a higher risk of both the clinical and combined outcomes (p=0.003 and

Group B III streptococcus (GBS) is a predominant pathogen in neonates in France. Relapse is rare and two successive relapses are exceptional: only three cases have been yet reported.A newborn infant of 18 days of age presented a first episode of invasive group B III streptococcal infection with meningitis and ventriculitis. At 53 days of age, a second episode with bacteriemia and parotidis appeared. At 63 days of age, she presented a third episode with meningitis. Genome analysis of the three bacterial strains isolated during the three episodes showed the same clonal origin.We discuss the incidence of the treatment, the eventual presence of a penicillin-tolerant GBS, the possible relapse or recurrence of the pathogen and the role of the relative immunodeficiency in infant.

Acute transverse myelitis is a rare disorder in childhood. It usually occurs as a post-infectious disease, but a precise infectious agent is identified in only 20% of cases.The diagnosis of acute transverse myelitis was made in a 5.5-year-old girl who initially presented with left Claude-Bernard-Horner syndrome and meningitis. A few days later, motor and sensory tetraparesia with bladder dysfunction was observed. Magnetic resonance imaging showed a diffuse lesion in the medulla, with a hypersignal in the T2 and a hyposignal in the T1 sequences. Serum analysis showed the presence of a viral infection due to the lymphocytic choriomeningitis (LCM) virus. The outcome was marked by complete recovery of the sensorimotor deficit, but a persistence of the left Claude-Bernard-Horner syndrome.In rare cases, the LCM virus is responsible for myelitis. In the present case, the Claude-Bernard-Horner syndrome was secondary to the cervico-medullary lesion. Recent reports in the literature have been discussed, in particular as regards the use of immunomodulatory therapy, which clearly improves patient prognosis.

Sinus histiocytosis with massive cervical lymphadenopathy (Rosai-Dorfman disease) is a non-neoplastic lymphoproliferative disorder. Extranodal involvement, especially of the nervous system, is unusual. We report a case revealed by neurological symptoms.A 10-year-old girl presented with paraparesis due to a dural extramedullary mass on magnetic resonance imaging. Massive cervical lymphadenopathy appeared secondarily. Radiological investigations showed mediastinal, paranasal sinus and lower eyelid involvement. The diagnosis of Rosai-Dorfman disease was established histologically and by immunohistochemical studies of nodal lesions by the demonstration of characteristic sinus histiocytosis with sheets of S-100 protein and CD-68 positive large histiocytes displaying lymphocyte phagocytosis. A dramatic response occurred with complete resolution of all clinical findings after treatment with corticosteroids and etoposide, although neurological lesions were unchanged on magnetic resonance imaging.Despite its rarity, this case underlines the unknown pathogenesis of this disease (immune dysfunction?) and the difficulties of treatment (choice of chemotherapeutic agents, duration).

In two multicentre, non-comparative studies, a total of 477 children, aged 2 months to 15 years, suffering from respiratory tract infections or skin and soft tissue infections were treated with roxithromycin (50 mg sachets). The mean duration of treatment was 9 days and the mean daily dose was 6 mg/kg/day administered b.i.d. The overall safety of roxithromycin was assessed by analysing the adverse events reported by the patients or investigators, the discontinuation of treatment because of adverse events, and the laboratory data. Adverse events reported by 20 (4%) children were considered to be possibly drug related. These adverse events were mainly digestive in nature and mild or moderate in severity. Treatment was discontinued because of adverse events in six (1%) children. Analysis of laboratory data revealed a few variations without any clinical significance.

The duration of antibiotic treatment of bacterial meningitis is always a topical issue. In our study (58 children), 21 of 24 meningococcal meningitis were treated for 4 or 5 days, 16 of 22 Haemophilus influenzae and 4 of 6 pneumococcal meningitis were treated for 7 days without increase in neurologic sequelae. A return of blood CRP levels to normal values was observed in all these patients simultaneously. Thus, CRP seems to be a good biological parameter for discussing treatment discontinuation. Furthermore, in some complications such as subdural effusion, a new increase of CRP levels was observed after the 5th day. A sequential follow-up of CRP levels at days J0, 5, 7, 10, seems a very useful tool for management of bacterial meningitis.

In three patients with muscular dystrophy, the unexpected occurrence of spinal stiffness suggested a diagnosis of rigid spine syndrome. One case belongs to the benign form of the congenital muscular dystrophies; in the other cases however, the severity and the distribution of the muscular process, associated with neurological abnormalities, seems to correspond to a unique variant of congenital muscular dystrophy. These observations underline the heterogeneity of the rigid spine syndrome.

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)

A retrospective study carried out from January 1981 through August 1988 identified nine cases of materno-fetal infections (Staphylococcus aureus, 1 case; Klebsiella pneumoniae, 3 cases; enterococcus, 3 cases; Hemophilus influenzae, 1 case; and Pneumococcus, 1 case) in six girls and three boys; four premature infants developed respiratory distress requiring mechanical ventilation. In four cases, maternal history found risk factors for infection. All patients developed circulatory failure and one had purulent meningitis. Diagnosis was confirmed upon recovery of the microorganism from a central sample in six cases; in three cases, only the peripheral cultures were positive but diagnosis was supported by the positive maternal history and by clinical findings. Bacterial species were not associated with specific clinical or biological features, but infections due to Hemophilus influenzae, Klebsiella pneumoniae and pneumococcus were particularly severe (two deaths and two infants with neurologic sequelae). In some studies, unusual microorganisms account for 46% of septicemias and 36% of purulent meningitis. Group D streptococci are the most common bacteria in this category and may account for 10% to 15% of early neonatal septicemias. Organism-specific features are analyzed on the basis of data from the literature. Concerning therapy, this epidemiologic aspect of materno-fetal infections complicates the choice of the initial antimicrobial agents; because third-generation cephalosporins are not effective on listeria and group D streptococci, we advocate more widespread use of the ureidopenicillins instead of ampicillin.

The association of the Willi-Prader syndrome and a t(15q15q) is reported. This, in conjunction with an earlier report of this association, suggests that a gene related to the Willi-Prader syndrome may be present on chromosome 15.