Neutropenia During Tocilizumab Treatment Is Not Associated with Infection Risk in Systemic or Polyarticular-course Juvenile Idiopathic Arthritis.

OBJECTIVE: To determine whether neutropenia is associated with increased risk for infection in patients with systemic juvenile idiopathic arthritis (sJIA) and polyarticular-course juvenile idiopathic arthritis (pcJIA) treated with tocilizumab (TCZ). METHODS: Data up to Week 104 from 2 phase III trials of intravenous TCZ in sJIA (n = 112; ClinicalTrials.gov, NCT00642460) and pcJIA (n = 188; ClinicalTrials.gov, NCT00988221) were pooled. Worst common toxicity criteria grade and lowest observed absolute neutrophil count (ANC) were identified for each patient. Associations between patient characteristics and lowest observed ANC were tested using univariate regression analysis. Infection and serious infection rates per 100 patient-years (PY) in periods associated with grades 1/2 and 3/4 neutrophil counts were compared with rates associated with normal neutrophil counts. RESULTS: ANC decreased to grade ≥ 3 in 25.0% and 5.9% of sJIA and pcJIA patients, respectively, and decreases were transient. Young age (p = 0.047) and methotrexate use (p = 0.012) were positively associated with neutropenia in patients with sJIA but not in patients with pcJIA. The rate of serious infections in patients with sJIA (10.9/100 PY; 95% CI 6.8-16.5) tended to be higher than in patients with pcJIA (5.2/100 PY; 95% CI 3-8.5). No increase in rates of serious or nonserious infections was observed during periods of neutropenia in either trial. CONCLUSION: Patients with JIA treated with TCZ experienced transient neutropenia that was not associated with an increased number of infections.

Telogen effluvium as the first symptom of Crohn's disease in a child.

Crohn's disease usually manifests gastrointestinal symptoms, however in some cases the patient presents with prominent or even exclusive extraintestinal involvement. Alopecia has been reported as a complication of therapeutic agents used in the treatment of inflammatory bowel disease, and, in a few cases of adult patients, prior to the appearance of gastrointestinal symptoms. We present a 10 year-old-child with telogen effluvium that appeared one year before the diagnosis of Crohn's disease, as the first and only symptom at that time. Other systemic causes of hair loss such as micronutrient deficiencies, endocrine imbalance or chemical exposure were excluded. Eight months later the patient presented with mild iron deficiency and signs of social retraction, while two months before the final diagnosis of Crohn's disease other more characteristic alarming symptoms (mild fever, oral apthous ulcers, weight loss) were added to the clinical picture. Alopecia improved after remission of Crohn's disease, reappeared when the patient relapsed, and finally resolved gradually when complete remission of Crohn's disease was achieved. Telogen effluvium was the first symptom of Crohn's disease in a child, and, although this is a rare association, it should be considered as an extraintestinal manifestation of Crohn's disease.

Comparative study of the health-related quality of life of children with epilepsy and their parents.

PURPOSE: We aimed to evaluate the health-related quality of life (HRQoL) of schoolchildren with epilepsy and its determinants and the HRQoL of their parents in comparison with those of healthy children and their parents. MATERIALS AND METHODS: The study sample comprised 100 children with epilepsy (58 males), 8-16 years of age, diagnosed at least 6 months earlier. The children with epilepsy were divided into two subgroups: A, with well controlled idiopathic epilepsy, and B, with drug-resistant or symptomatic epilepsy and with concomitant neurodevelopmental problems. A control group consisted of 100 healthy age- and gender-matched children. One parent in each family completed two questionnaires standardized for use in Greece: KIDSCREEN-27 (version for parents) to assess the HRQoL of the children and SF-12 to assess the parental HRQoL. For each of the five dimensions of KIDSCREEN-27 and for the physical and mental component scales of the SF-12 tool, the standardized mean difference (SMD) was used for comparison between the various groups and subgroups. Linear regression analysis was used to explore the effect of specific illness-related factors on the five dimensions of KIDSCREEN-27 in the children with epilepsy. RESULTS: The parent-reported scores on KIDSCREEN-27 of the children with epilepsy were worse overall than those of healthy children, but the difference reached statistical significance only for the dimensions of "physical well-being" (p = 0.001) and "school environment" (p < 0.001). The differences were greater in adolescents (age group: 13.5-16years). The worst scores were recorded in subgroup B, the children with severe epilepsy, in the dimensions "physical well-being" (p < 0.001), "school environment" (p < 0.0001), and "peers and social support" (p = 0.044). The factors found to have a significant effect on all dimensions were mental retardation, physical disability, abnormal brain imaging findings, learning problems, and, to a lesser degree, administration of a large number of antiepileptic drugs and prolonged treatment. The parents of children with resistant epilepsy and accompanying neurodevelopmental problems scored significantly worse on the SF-12 mental health scale than those of healthy children (p < 0.001). CONCLUSIONS: Epilepsy, particularly severe epilepsy with concomitant neurodevelopmental problems, adversely affects the HRQoL of both schoolchildren and their parents.

Endothelial dysfunction, but not structural atherosclerosis, is evident early in children with heterozygous familial hypercholesterolemia.

Children with heterozygous familial hypercholesterolemia (heFH) are prone to premature atherosclerosis. Vascular endothelial dysfunction may predict increased cardiovascular risk in children with heFH. The aim of this study was to assess for early functional and structural vascular changes in children with heFH. This cross-sectional study included 30 children with heFH (mean age 12 years) and 30 age- and sex-matched controls. Brachial artery flow-mediated dilation (FMD), carotid intima-media thickness (cIMT), carotid-femoral pulse wave velocity, and large- and small vessel compliance were measured noninvasively. HeFH children exhibited significantly greater total and LDL cholesterol, apolipoprotein B, and lipoprotein (a) levels (p < 0.05 for all) and lower FMD (6.23 ± 3.88 vs. 9.46 ± 4.54 %, p < 0.004) compared with controls. When children were divided in age subgroups, FMD was found to be significantly decreased in heFH compared with control subjects only in ages >10 years (p < 0.05). However, FMD was found to be similarly impaired in heFH children in all age subgroups (two-way analysis of variance, p = 0.39). No differences in other vascular function indices were found. In heFH patients, but not in controls, FMD was inversely correlated with cIMT (r = -0.378, p = 0.036). In conclusion, endothelial dysfunction occurs early in heFH children indicating an increased risk for premature cardiovascular disease and reflecting probably the need for early initiation of anticholesterolemic treatment. Decreased FMD is detected before structural atherosclerotic changes occur.

Frequency, clinical characteristics, and genotype distribution of rotavirus gastroenteritis in Greece (2007-2008).

Rotavirus is the leading cause of acute gastroenteritis among young children worldwide. A prospective multi-center study was conducted (2007-2008) in five Pediatric Hospitals to determine the prevalence, the clinical characteristics, and genotype distribution of rotavirus infection in Greece. Faecal samples were examined for the presence of group A rotavirus antigen by immunochromatography. Rotavirus strains were subjected to G and P genotyping by reverse-transcriptase polymerase chain reaction (PCR) and sequencing. A total of 393 children (216 boys) of median age 23 months, participated in the study. Rotavirus was the cause of acute gastroenteritis in 166 children, 42.3% (CI 95%, 37.4-47.1%) of non-hospitalized and 47.8% (CI 95%, 41.7-53.9%) of hospitalized patients. Rotavirus gastroenteritis occurred between December and April in 78.6% of the cases. Most children with RVG (77.8%) were between 3 months and 3 years old. The mean value of Clark severity score was 12.9 ± 5.1 for RVG and 10.5 ± 4.9 for non-RVG (P < 0.01). Genotypes were determined in 117 strains and their distribution was as following: G1P[8], 49%; G2P[4], 31%; G4P[8], 10%; G9P[8], 9%; and G8P[14], 1%. In conclusion, rotavirus is a frequent cause of acute gastroenteritis in Greece. The genotypes circulating are similar with those of other European countries.