Opsoclonus–myoclonus in children associated or not with neuroblastoma

ObjectiveTo compare the clinical data at diagnosis, treatment and neurological outcome in 34 children with opsoclonus–myoclonus syndrome (OMS) associated with a detected neuroblastoma or not.Study designThis is a multicentric retrospective study of 34 children presenting with OMS from four pediatric centers diagnosed between 1988 and 2008.ResultsTwenty-two patients had OMS associated with a neuroblastoma. These patients all had neuroblastomas with favourable prognostic features; all underwent surgery, six received chemotherapy. Twelve children had OMS without a detected neuroblastoma. For OMS, the main treatment in all children was corticotherapy (n = 33), but immunoglobulins (n = 13), cyclophosphamide (n = 4) and rituximab (n = 4) were also given. In the 27 OMS patients with or without neuroblastoma whose follow up was greater than two years, the neurological outcome was evaluated: 59.3% had neurological sequelae, including motor, praxic and/or language sequelae (n = 9), persistent ataxia (n = 6) and moderate motor deficit (n = 3). No significant difference in neurological outcome was noted between the two patient groups.ConclusionOur retrospective study provides further evidence that OMS with or without a detected neuroblastoma is the same disease, whose major challenges are the neurological sequelae. An international collaboration is required to improve the knowledge about OMS, the treatment and the outcome in this rare disorder.     

Is albuminuria associated with obesity in school children?

Hirschler V, Molinari C, Maccallini G, Aranda C. Is albuminuria associated with obesity in school children? Background: Different studies in adults have shown that obesity is an independent risk factor for end‐stage renal disease. Objective: It was to (i) determine the association between albuminuria and age, gender, BMI, waist circumference (WC), and blood pressure (ii) relate albuminuria to gender and age in healthy school children. Methods: Over 1564 students (806 males) aged 9.35 ± 2.00 yr from 9 elementary schools in Buenos Aires, were examined between April and September 2005. BMI, WC, blood pressure, urinary albumin excretion and albumin/creatinine ratio (ACR) were determined. Results: Over 220 (14.1%) children were obese (OB), and 300 (19.2%) overweight (OW) using centers for disease control (CDC) norms. Median ACR and urinary albumin levels were higher in normal weight children than in OW/OB children (p < 0.01). Median values for ACR were higher in girls than in boys (p < 0.001). OW/OB proved to be a protective factor against ACR [OR, 0.77 (95% CI 0.64–0.92)], whereas female gender [OR, 2.72 (95% CI 2.13–3.47)] was associated with a higher ACR adjusted for age systolic and diastolic blood pressure in the multiple logistic regression analysis. Therefore, children with OW/OB had 23% lower odds of having ACR ≥ III quartile and girls had more than two and half times the risk of having high ACR. Conclusions: Our results suggest that OW/OB is inversely associated with childhood albuminuria while female gender is positively associated. Whether the results can be explained by the increased physical activity in lean children should be the subject of future investigations. These results suggest that micro‐albuminuria in children may not be a useful tool in the early identification of children at risk for future renal and cardiovascular disease (CVD).     

Is obesity associated with asthma in young children?

OBJECTIVE: The aim of this study was to evaluate the association between obesity and asthma in a population-based sample of Canadian children. STUDY DESIGN: Baseline data from the National Longitudinal Survey of Children and Youth were used in this cross-sectional study. We included 11199 children age 4 to 11 years whose biological mother reported data on asthma, height, and weight. Body mass index was categorized, and obesity was defined as body mass index >or=85th percentile. Children with asthma had parents who reported the diagnosis, and they took prescribed inhalants, had wheezing or an attack in the previous year, or had their activities limited by asthma. Multiple logistic regression was used. RESULTS: The prevalence of asthma was 9.9%. Maternal history of asthma was a risk factor for asthma among all children. Single child status and maternal depression were risk factors for girls. The odds ratio for asthma, comparing highest and lowest body mass index categories, was 1.02 (99% confidence interval, 0.70-1.46) for boys and 1.06 (99% confidence interval, 0.67-1.69) for girls. CONCLUSION: This study suggests that there is no statistical association between obesity and asthma among Canadian children age 4 to 11 years.     

Factors associated with community-acquired pneumonia in hospitalised children and adolescents aged 6 months to 13 years old

According to the World Health Organisation, community-acquired pneumonia is the main cause of paediatric death, accounting for 20 % of deaths in children younger than 5 years old, and 90 % of these deaths occur in non-industrialised countries. This study has as objective to evaluate the influence of socio-economic, environmental and breastfeeding factors on the occurrence of pneumonia. An unmatched case–control study was conducted in children aged 6 months to 13 years old at a children’s hospital in Brazil. Multivariate analysis by logistic regression was performed to determine the variables used to predict pneumonia. A total of 252 children were selected. In the adjusted (by age) multivariate analysis, the following variables were associated with community-acquired pneumonia: (a) protective factors: breastfeeding >3 months, absence of other unrelated comorbidities, non-smoking mother, being the only child, child’s age >5 years and mother’s age >19 years old; (b) risk factors: maternal education <8 years and child’s birth order [≥second]. In the multivariate analysis, considering only children from 6 months to 5 years old, the following variables were associated with community-acquired pneumonia: (a) protective factors: breastfeeding >3 months, non-smoking mother and no smokers in the child’s bedroom; (b) risk factors: maternal education <8 years and prenatal complications. Conclusion: These findings contribute favourably to effectively minimising the risk factors related to the disease process and natural history of community-acquired pneumonia.     

Are signs and symptoms associated with persistent corneal abrasions in children?

BACKGROUND: Corneal abrasions are common eye injuries in children. Most are treated with antibiotic drops or ointment, patching of the affected eye, and follow-up within 24 hours to confirm resolution by fluorescein examination. OBJECTIVE: To determine if signs and symptoms at follow-up were associated with the presence of a persistent corneal abrasion or abnormal visual acuity. DESIGN: Retrospective case series. SETTING: A children's hospital. PATIENTS: Children who were aged 4 years or older with the diagnosis of corneal abrasion between May 1992 and December 1996 and who had a follow-up examination. RESULTS: Seventy-seven patients (57% male) were enrolled (median age, 7 years). The respective sensitivities, specificities, positive predictive values, and negative predictive values of selective signs and symptoms for persistent abrasions were as follows: for pain, 53%, 93%, 80%, and 80%; for photophobia, 57%, 100%, 100%, and 80%; for redness, 100%, 46%, 44%, and 100%; for pain and redness, 40%, 96%, 80%, and 80%; and for at least 1 sign or symptom, 95%, 48%, 47%, and 95%. Twenty-six patients had persistent corneal abrasions at follow-up. Six of these 26 patients were symptom free at follow-up, and 15 patients had only redness as a persistent sign. Five patients had abnormal visual acuity, one of whom was asymptomatic. All 3 patients with complications were symptomatic. CONCLUSIONS: Signs and symptoms are inconsistently associated with persistent corneal abrasions. Asymptomatic patients may have persistent corneal abrasions, suggesting the need for selective follow-ups.     

Off–label prescribing patterns of antiemetics in children: a multicenter study in Italy

Acute gastroenteritis (AG) represents both the main cause of acute vomiting in children under 3 years old and a major cause of access to the emergency department. Even if several drugs may be able to reduce the emesis, the pharmacological treatment of vomiting in children remains a controversial issue, and several drugs are prescribed outside their authorized drug label with respect dosage, age, indication, or route of administration and are named as off-label. The aim of present study was to assess the off-label use of antiemetic drugs in patients less than 18 years with vomiting related to AG. This study was carried out in eight pediatric emergency departments in Italy. The following data were obtained crossing the pharmacy distribution records with emergency departments’ patient data: sex and age of the patients and detailed information for each drug used (indication, dose, frequency, and route of administration). We recorded that antiemetic drugs were prescribed in every year, particularly in children up to 2 years old, and compared with both literature data and data sheet; 30 % of the administered antiemetics were used off-label. In particular, domperidone was the only antiemetic used labeled for AG treatment in pediatric patients, while metoclopramide and ondansetron have been off-label for both age and indications (i.e., AG treatment). Conclusions: In conclusion, we documented an off-label use of antiemetics in children, and this could represents a problem of safety for the patient and a legal risk for the prescribing physician if patients have an unwanted or bad outcome from treatment.     

Are diagnostic magnetic resonance patterns life-saving in children with biotin-thiamine-responsive basal ganglia disease?

Background

Biotin-thiamine responsive basal ganglia disease (BTBGD) is an autosomal recessive disorder caused by mutations in the SLC19A3 gene and characterized by recurrent sub-acute episodes of encephalopathy that typically starts in early childhood. This study describes characteristic clinical and magnetic resonance imaging (MRI) findings of six cases of BTBGD diagnosed with newly identified mutations and genetically confirmed, with very early and different presentations compared to cases in the previous literature.

Congenital genitourinary abnormalities in children with Williams–Beuren syndrome

ObjectiveWilliams–Beuren syndrome (WBS) is an autosomal dominant disorder caused by a gene deletion on chromosome 7q11.23. Patients with WBS usually show a group of features such as developmental delay, cardiovascular anomalies, mental retardation, and characteristic facial appearance. Abdominal wall defects, external genitalia anomalies, and structural abnormalities of the urinary tract have been scarcely evaluated and were the focus of our study.Materials and methodsWe prospectively evaluated 41 boys and 38 girls with WBS, with a mean age of 8.8 ± 4.1 (range 3–19 years). All patients were examined for the evaluation of inguinal and umbilical hernias and genital anomalies. All patients were offered a radiological evaluation, including urinary tract ultrasound, voiding cystourethrogram, and dimercaptosuccinic acid renal scintigraphy (DMSA scan).ResultsOf the 41 boys, 30 (73.1%) had abnormalities on physical examination, including bilateral undescended testis in 13 (31.7%), retractile testis in four (9.7%), hypospadias in four (9.7%), and unilateral cryptorchidism in three (7.3%) patients. Of the 38 female subjects, 17 (44.7%) had at least one abnormality, including umbilical hernia in 11 (28.9%), unilateral inguinal hernia in four (10.5%), and bilateral inguinal hernia in three (7.8%) patients. Uroradiological abnormalities were found in 41 patients (51.9%). On sonography, six (7.6%) patients had unilateral hydronephrosis, three (3.8%) had a duplicated collecting system, and two (2.5%) had kidney stones. On DMSA, performed in 36 patients, four (11.1%) had unilateral renal scarring and two (5.5%) had bilateral renal scarring. Cystourethrography was obtained from 56 patients, of whom 27 (48.2%) had bladder diverticulum, 18 (32.1%) had bladder wall trabeculation, and three (5.3%) had vesicoureteral reflux. We found no association of urological abnormalities with cardiovascular defects.ConclusionsPatients with WBS have a high prevalence of abdominal wall, external genitalia, and urological abnormalities, emphasizing the importance of proper physical examination and radiological investigation in this population.     

Immunological patterns in young children with Down syndrome: is there a temporal trend?

Down syndrome is associated with an increased susceptibility to infections due to a deficiency of both specific and nonspecific immunity. AIM: The aim of the study was to analyze the temporal trends, if any, of some variables related to the immunological status of children affected by Down syndrome. METHODS: Heparinized blood samples were obtained by venipuncture in 30 children with Down syndrome, who were regularly followed in our department and analyzed for hematologic values, lymphocyte subpopulations, immunoglobulin dosage and zinc level. Results were compared with those of the normal population. RESULTS: In the first 5 years of life, we observed a progressive decrease in the medium values of lymphocytes, CD4(+) and plasma zinc levels, and an increase in CD8(+), immunoglobulin A, immunoglobulin G, immunoglobulin M and natural killer, but generally without exceeding the interval of normality. CONCLUSIONS: In Down syndrome children, the immune cellular status is similar to the normal population as far as white blood cell, lymphocyte, CD4(+), CD8(+), natural killer and immunoglobulins are concerned. Plasma level of zinc is normal from birth until 5 years but with a temporal trend of progressive reduction. This observation supports the hypothesis that a pharmacological supplementation may be necessary in Down syndrome children only after 5 years of age.     

Is Helicobacter pylori infection associated with Henoch-Schonlein purpura in Chinese children? a meta-analysis

Background

The prevalence of Helicobacter pylori (H. pylori) infection is high in China. It not only causes the damage of gastric epithelium, but also plays a potential pathogenic role in several extraintestinal diseases. Henoch-Schonlein purpura (HSP) is one of the most common vasculitis syndromes affecting children. Although its cause is unclear, HSP is often considered to be associated with infectious agents. This metaanalysis of previously published studies was conducted using a predefined protocol to evaluate the underlying association between H. pylori infection and HSP in Chinese children.

Methods

Predefined search strategy and inclusion criteria were set up to select studies reporting the prevalence of H. pylori infection among HSP children and control groups. Included studies were subjected to quality assessment and data extraction by two independent reviewers. The pooled odds ratio (OR) was calculated as the effect size via both traditional and cumulative meta-analysis. Heterogeneity was investigated by subgroup analysis, and the nonparametric ??trim and fill?? method was performed to adjust the overall estimate for the existence of publication bias.

Results

Ten eligible studies covering 749 HSP children and 560 controls were included for metaanalysis. Observational epidemiology studies clearly aimed at detecting the potential association between H. pylori infection and HSP with retrospective data collection from the children enrolled consecutively. Overall, 49.27% (369/749) of HSP children had evidence of H. pylori infection compared with 23.39% (131/560) of children in the control group. The pooled OR of H. pylori infection in HSP children (10 studies with 749 HSP children) was 3.80 [95% confidence interval (CI): 2.54?C5.68, P<0.001], and the overall estimate from the cumulative meta-analysis confirmed the association with more narrow confidence interval (OR=3.35, 95% CI: 2.95?C3.81). In HSP children mainly with abdominal manifestations (8 studies with 337 HSP children), the pooled OR was 4.62 (95% CI: 2.66?C8.01, P<0.001). The adjusted pooled OR was 2.04 (95% CI: 1.48?C2.82, P<0.001), determined by the nonparametric ??trim-andfill?? method for eliminating the effect of publication bias. H. pylori eradication therapy (4 studies with 266 HSP children) was capable of reducing the recurrence of HSP (RR=0.38, 95% CI: 0.25?C0.58, P<0.001). Although the subgroup analysis for heterogeneity suggested that diagnostic methods and geographical diversity might be account for the heterogeneity, statistical analysis of differences revealed no differences between subgroups, indicating their limited impact on the overall estimates.

Conclusions

These results suggest the necessity of screening H. pylori infection in HSP children, particularly in those with gastrointestinal manifestations in China. Eradication therapy may reduce the recurrence of HSP in children with H. pylori infection. However, further mechanistic and more clinical studies in different populations and regions are needed to confirm this association and the effect of eradication of H. pylori in HSP children.     

Characteristics of Kikuchi–Fujimoto disease in children compared with adults

Kikuchi–Fujimoto disease (KFD) is a benign, self-limiting disease characterized by cervical lymphadenopathy. Although it was primarily thought to be a disease of young adults, it has been increasingly recognized in children. To define the characteristics of KFD in children, we reviewed the medical records of patients younger than 18 years of age who were diagnosed with KFD from 2001 to 2012 at Korea University Medical Center, as well as worldwide published reports of KFD. A total of 140 pediatric patients and 733 patients of all ages was analyzed. Compared to the female predominance found in adults (2:1), young boys were more commonly affected than young girls (1.4:1). Cervical lymphadenopathy was the most common clinical finding in children, as it was in adults. Lymphadenopathy was more likely to be tender (69 vs. 44 %, p?<?0.001) but less generalized (1 vs. 8 %, p?<?0.05) in children compared to adults. Fever (82 vs. 35 %, p?<?0.001) and rash (10 vs. 4 %, p?<?0.05) were observed in children more commonly than in adults. Leukopenia was observed in 50 and 38 % of children and adults, respectively. Rates of recurrence and association with autoimmune diseases in children were comparable to those of adults. Cervical lymphadenopathy was the most common clinical manifestation of KFD in all ages. While fever and rash were more common in children with KFD compared to adults, generalized lymphadenopathy was rarer.     

Bone health in children with long–term idiopathic subclinical hypothyroidism

Background

Previous Italian paediatric blood pressure (BP) tables overestimated the prevalence of hypertension in adolescents of specific geographic areas, such as Sardinia, an island in the Mediterranean Sea. This is probably due to a not very homogeneous distribution of the subjects studied, most from Middle and Northern Italy, and the long period from the survey.

Methods

BPs were repeatedly measured over a period of 3 years in 839 children (52.6% males. Age range: from 11 to 14 years during this period), using a standard mercury sphygmomanometer. For each gender, the specific percentile curves of systolic and diastolic BP were constructed.

Results (corrected by the 50^th percentile of height)

Males (11-14 years)

mean systolic BP (50^th centile): from 111 to 115 mmHg. Hypertensive systolic BP (> 95^th percentile): from 127 to 135 mmHg. Mean diastolic BP (50^th centile): from 65 to 69 mmHg. Hypertensive diastolic BP (> 95^th percentile): from 78 to 82 mmHg.

Females(11-14 years)

mean systolic BP (50^th centile): from 110 to 112 mmHg. Hypertensive systolic BP (> 95^th percentile): from 127 to 130 mmHg. Mean diastolic BP (50^th centile): from 65 to 67. Hypertensive diastolic BP (> 95^th percentile): from 78 to 80 mmHg.

Conclusions

Sardinian BP tables emphasizes the need to integrate the previous standards with more up-to-date and representative reports on Italian children, as periodically performed in the USA, in order to increase the number of subjects to be checked, and to obtain a national coverage better and more completely representative of every geographic area of our country.     

Hemiconvulsion–Hemiplegia–Epilepsy syndrome associated with inflammatory-degenerative hystopathological findings in child with congenital adrenal hyperplasia

Hemiconvulsion–Hemiplegia (HH) syndrome represents an uncommon consequence of prolonged unilateral clonic or hemiconvulsive status epilepticus in childhood, usually occurring during a febrile illness, followed by ipsilateral hemiplegia. The subsequent appearance of focal seizures configures the so called Hemiconvulsion–Hemiplegia–Epilepsy (HHE) syndrome. The pathogenesis of HH/HHE syndrome is still unclear. We describe the case of a 4 year-old girl with congenital adrenal hyperplasia (CAH) whom developed HH/HHE syndrome with drug resistant seizures at the age of 21 months and underwent left cerebral hemispherotomy at the age of 3 years and 6 months. Histopathological findings showed the presence of an underlying inflammatory-degenerative process. Disregulation of the inflammatory cascade has been proposed as one of the possible pathogenetic mechanisms underlying HH/HHE syndrome. To our knowledge however, this is the first report of an association with a histologically documented inflammatory process. The clinical and histopathological findings of our reported case lend support to the possible role of inflammation in the pathogenesis of HH/HHE syndrome.     

Motor and perceptual–motor competence in children with Down syndrome: variation in performance with age

The aim of this study was to determine whether perceptual–motor competence in school-age children with Down syndrome was generally delayed or varied as a function of type of action. Twenty-two children with Down syndrome (13 males, 9 females), aged between 4.5 and 14 years were assessed on two standardized tests, the Movement Assessment Battery for Children (Movement ABC), a test of motor competence assessing gross and fine motor coordination, and on the Developmental Test of Visual–Motor Integration, a test focusing on shape copying. In order to obtain a profile of each child's performance on the different items contained in the Movement ABC, the test was used in its extended form. This involves testing any child who failed an item appropriate for his/her level, at progressively lower levels until a base-line measure was obtained.All the children obtained scores below the 5th percentile for their age on both tests. However, superimposed on this delay, we found distinct variation as a function of task. Whereas some aspects of gross motor function showed age development with delayed but regular acquisitions, all the aspects of fine motor skills assessed were more severely impaired and showed little development with age. Accuracy and timing of tasks requiring bimanual coordination were most impaired in our sample while balance and ball skills showed more variability. These results suggest that intervention in the motor domain should be varied according to each child's particular profile of performance.