Albert Faye

Clinical outcome in children with chronic recurrent multifocal osteomyelitis

OBJECTIVE To determine the clinical outcome of children with chronic recurrent multifocal osteomyelitis (CRMO). METHODS We retrospectively reviewed clinical, biological and radiological data of children with CRMO at five French paediatric centres. Outcome data were obtained through review of hospital charts and questionnaires sent to all patients to assess disease activity and educational and vocational achievement. RESULTS Forty patients were assessed (34 females and 6 males) with a median age at diagnosis of 11.5 yrs (range 2-17). Median number of initial bony lesions was 2 at onset, and 3.5 over disease course. Median time since diagnosis was 3.5 yrs (range 0.5-15) and median duration of active disease 2.7 yrs (range 0.5-13.5). Nine (22.5%) patients had psychological or physical sequelae. Twenty-nine children (72.5%) responded to the questionnaire. Twenty-six had no physical disability as judged by the HAQ 0-1, two had moderate disability (HAQ: 1-2) and one had severe disability (HAQ: 2-3). Seventeen patients (58.6%) had active disease at follow-up (after 6 months to 15 yrs since diagnosis) and continued to have pain (median value of visual analogue scale: 10/100). CRMO had interfered with patients education in two cases. CONCLUSIONS Clinical outcome of children with CRMO is generally good, but a sizeable proportion of patients have active disease at follow-up, and a minority of patients can have a severe and prolonged disease course despite intensive treatments. Further studies are required to determine predictive factors for severe disease.

Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet’s disease: an international cohort study of 110 patients. One-year follow-up data

OBJECTIVE To set-up an international cohort of patients suspected with Behçets disease (BD). The cohort is aimed at defining an algorithm for definition of the disease in children. METHODS International experts have defined the inclusion criteria as follows: recurrent oral aphthosis (ROA) plus one of following-genital ulceration, erythema nodosum, folliculitis, pustulous/acneiform lesions, positive pathergy test, uveitis, venous/arterial thrombosis and family history of BD. Onset of disease is <16 years, disease duration is ≤3 years, future follow-up duration is ≥4 years and informed consent is obtained. The expert committee has classified the included patients into: definite paediatric BD (PED-BD), probable PED-BD and no PED-BD. Statistical analysis is performed to compare the three groups of patients. Centres document their patients into a single database. RESULTS At January 2010, 110 patients (56 males/54 females) have been included. Mean age at first symptom: 8.1 years (median 8.2 years). At inclusion, 38% had only one symptom associated with ROA, 31% had two and 31% had three or more symptoms. A total of 106 first evaluations have been done. Seventeen patients underwent the first-year evaluation, and 36 had no new symptoms, 12 had one and 9 had two. Experts have examined 48 files and classified 30 as definite and 18 as probable. Twenty-six patients classified as definite fulfilled the International Study Group criteria. Seventeen patients classified as probable did not meet the international criteria. CONCLUSION The expert committee has classified the majority of patients in the BD group although they presented with few symptoms independently of BD classification criteria.

Low Birth Weight in Perinatally HIV-Exposed Uninfected Infants: Observations in Urban Settings in Cameroon

Background The consequences of maternal HIV infection for fetal growth are controversial. Here, we estimated the frequency of small for gestational age and gender (SGAG) among neonates born to HIV-infected or uninfected mothers and assessed the contribution, if any, of maternal HIV to the risk of SGAG. Methods The data used were obtained from the ANRS-Pediacam cohort in Cameroon. Pairs of newborns, one to a HIV-infected mother and the other to an uninfected mother, were identified during the first week of life, and matched on gender and recruitment site from 2007–2010. SGAG was defined in line with international recommendations as a birth weight Z-score adjusted for gestational age at delivery and gender more than two standard deviations below the mean (−2SD). Considering the matched design, logistic regression modeling was adjusted on site and gender to explore the effect of perinatal HIV exposure on SGAG. Results Among the 4104 mother-infant pairs originally enrolled, no data on birth weight and/or gestational age were available for 108; also, 259 were twins and were excluded. Of the remaining 3737 mother-infant pairs, the frequency of SGAG was 5.3% (95%CI: 4.6–6.0), and was significantly higher among HIV-infected infants (22.4% vs. 6.3%; p<.001) and lower among HIV-unexposed uninfected infants (3.5% vs. 6.3%; p<.001) than among HIV-exposed uninfected infants. Similarly, SGAG was significantly more frequent among HIV-infected infants (aOR: 4.1; 2.0–8.1) and less frequent among HIV-unexposed uninfected infants (aOR: 0.5; 0.4–0.8) than among HIV-exposed uninfected infants. Primiparity (aOR: 1.9; 1.3–2.7) and the presence of any disease during pregnancy (aOR: 1.4; 1.0–2.0) were identified as other contributors to SGAG. Conclusion Maternal HIV infection was independently associated with SGAG for HIV-exposed uninfected infants. This provides further evidence of the need for adapted monitoring of pregnancy in HIV-infected women, especially if they are symptomatic, to minimize additional risk factors for SGAG.

Fusobacterium necrophorum multiple abscesses in children with sickle cell disease.

1. Schieve LA, Boulet SL, Boyle C, Rasmussen SA, Schendel D. Health of children 3 to 17 years of age with Down syndrome in the 1997-2005 national health interview survey. Pediatrics 2009; 123: e253–60. 2. Guazzarotti L, Trabattoni D, Castelletti E, Boldrighini B, Piacentini L, Duca P, et al. T lymphocyte maturation is impaired in healthy young individuals carrying trisomy 21 (Down syndrome). Am J Intellect Dev Disabil 2009; 114: 100–9. 3. Loh RK, Harth SC, Thong YH, Ferrante A. Immunoglobulin G subclass deficiency and predisposition to infection in Down’s syndrome. Pediatr Infect Dis J 1990; 9: 547–51.

Uncomplicated outcome after an accidental overdose of nevirapine in a newborn

We report the first case of a massive accidental overdose of nevirapine in a 1-week newborn, due to confusion between nevirapine (Viramune) and nelfinavir (Viracept). The drug was eliminated spontaneously and quickly. We only observed mild neutropenia and hyperlactatemia, which regressed on its own without any clinical complication. Despite the good evolution of this massive overdose, physicians should be aware of confusion risks between some antiretroviral drugs.

VIROLOGICAL RESPONSE TO EARLY COMBINED ANTIRETROVIRAL THERAPY IN HIV-INFECTED INFANTS: EVALUATION AFTER TWO YEARS OF TREATMENT IN THE PEDIACAM STUDY

Background Little is known about virological responses to early combined antiretroviral therapy (cART) in HIV-infected infants in limited-resource settings. We estimated the probability of achieving viral suppression within two years of cART initiation, and investigated the factors associated with success. Methods We analysed all 190 infants from the Cameroon PediaCAM study who began free cART before the age of 12 months. The main outcome measure was viral suppression (<1000 cp/mL) on at least one occasion. The other outcome measures considered were viral suppression (<400 copies/ mL) on at least one occasion and confirmed viral suppression (both thresholds) on two consecutive occasions. We used competing-risks regression for a time-to-event analysis to estimate the cumulative incidence of outcomes, and univariate and multivariate models to identify risk factors. Results During the first 24 months of cART, 20.0% (38) of the infants died, giving a mortality rate of 11.9 deaths per 100 infant-years [95% CI: 8.1–15.7]. The probability of achieving a viral load below 1000 or 400 copies/mL was 80.0% [69.0–81.0] and 78.0% [66.0–79.0], respectively. The probability of virological suppression (with these two thresholds) on two consecutive occasions was 67.0% [56.0–70.0] and 60.0% [49.0–64.0], respectively. Virological success was associated with not having missed any doses of treatment before the visit, but not with socioeconomic and living conditions. Conclusions The long-term daily administration of drugs to babies seems to be difficult. Mortality remained high despite early cART initiation. Future studies should focus on longer-term treatment outcomes in children still alive after two years of treatment.