The main goal of the study was to assess the effect of exclusive breastfeeding on the neurodevelopment of children over a seven-year follow-up period and to test the hypothesis that the observed cognitive gain in breastfed children in the first years of life is a strong predictor of their cognitive development trajectory, which may be continued in later life.
The analysis is based on data from the seven-year follow-up of 468 term babies (>36 weeks of gestation) born to non-smoking mothers participating in an ongoing prospective cohort study. The cognitive function of children was assessed by psychometric tests performed 5 times at regular intervals from infancy through the preschool age. The study included valid neurodevelopmental assessment of the children – 443 participants were evaluated least twice, 425 – three times and 307 five times in the follow-up period. The association between the cognitive achievements of preschool age children and exclusive breastfeeding of various duration was performed using the GEE (General Estimation Equation) longitudinal model, adjusted for major confounders such as maternal education, gender, parity, and weight gain in pregnancy.
Children breastfed exclusively for up to 3 months had IQs that were on average 2.1 points higher compared to the others (95%CI: 0.24 – 3.9); children breastfed for 4 – 6 months scored higher by 2.6 points (95%CI: 0.87 – 4.27); and the benefit for children breastfed even longer (>6 months) increased by 3.8 points (95%CI: 2.11 – 5.45). Other predictors were maternal education, gender of the child, having an older sibling, and weight gain during pregnancy.
The results of the study support the WHO expert recommendations on exclusive breastfeeding for six months; moreover, they provide evidence that even a shorter duration of exclusive breastfeeding in early infancy produces beneficial effects on the cognitive development of children. The breastfeeding-related IQ gain observed already at the age of 1 was sustained through preschool age and the difference in terms of IQ score between breastfed children and the reference group (mixed breastfeeding) held constant over the whole preschool period.
breastfeeding; cognitive function in early childhood; prospective birth cohort study
The aim of this study was to investigate the changes in the peripheral blood of newborns of hypertensive mothers. The umbilical cord blood from newborns of 31 hypertensive mothers and 32 healthy mothers were examined. In all subjects, complete blood count, peripheral blood smear, reticulocyte count, vitamin B12, folate, ferritin levels and hemoglobin electrophoresis were performed. The subjects were followed up on for 1 year in terms of infections. RBC, hemoglobin, reticulocyte count and normoblast count were higher in the newborns of hypertensive mothers compared to the control group, and total leukocytes, neutrophil, lymphocyte, monocyte, eosinophil, and thrombocyte counts were lower. The number of neutropenic and thrombocytopenic subjects in newborns of hypertensive mothers was higher compared to the control group. On peripheral smears, dysplastic changes in neutrophils and erythrocytes were observed with a higher rate in newborns of hypertensive mothers compared to the control group. HbF levels were found to be higher in newborns of hypertensive mothers compared to the control group. During the follow-up period of 1 year, the number of infections in newborns of hypertensive mothers was found to be higher than the control group. Conclusion: Newborns of hypertensive mothers should be carefully evaluated and monitored in terms of hematologic abnormalities. Complete blood counts and peripheral blood smears can be used as significant parameters for early diagnosis of possible complications.
Pregnancy; Hypertension; Newborn; Blood parameters
Primary nocturnal enuresis is a prevalent childhood condition that can persist into adulthood. Desmopressin is an antidiuretic available as orally disintegrating lyophilisate (melt) or solid tablet. Recent findings suggesting different food interactions and clinical characteristics, including compliance, between desmopressin melt and tablet motivated a post hoc analysis of a previously reported randomised, crossover study. The efficacy of desmopressin melt compared with tablet was evaluated using the International Children’s Continence Society (ICCS) responder definitions. Compliance was further analysed using detailed criteria, and the association between efficacy and compliance was examined. In total, 221 patients aged 5–15 years, already receiving desmopressin tablets were randomised to the treatment sequence melt (120/240 μg)/tablet (0.2/0.4 mg) or tablet/melt in two consecutive 3-week periods. The probability of being a responder (partial or full) during either period was significantly more likely with desmopressin melt compared with tablet (odds ratio, 2.0; confidence intervals, 1.07–3.73; p = 0.03). There was no period effect on compliance in the tablet/melt sequence and no difference in the number of completely compliant patients in each formulation group; however, more patients were >75 % compliant in period 1 compared with period 2 in the melt/tablet sequence. Increased compliance was associated with greater reductions in the number of wet nights for both formulations. Conclusions: Desmopressin melt, compared with tablet, improves the probability of being a responder. Switching from tablet to melt formulation increased patient compliance. Increased compliance was associated with increased efficacy. Switching to desmopressin melt may benefit patients with suboptimal responses to desmopressin tablet.
Enuresis; Desmopressin; Compliance; Formulation
We tested the hypothesis that the use of supplemental oxygen (sO2) at discharge from the NICU in extremely preterm neonates is associated with a greater risk of neurodevelopmental impairment (NDI) at 18 months corrected gestational age (CGA) than the risk of NDI of those neonates discharged in room air. Four hundred twenty-four charts were retrospectively reviewed from infants born at <27 weeks and transferred to Nationwide Children’s Hospital from December 1, 2004 to June 14, 2010. Use of sO2 was evaluated on day of life (dol) 28, at 36 weeks post-menstrual age (PMA), and at discharge. Logistic regression was used to identify postnatal risk factors associated with sO2 at discharge and NDI. At dol 28, 96 % of surviving patients received sO2, and therefore had bronchopulmonary dysplasia (BPD) by definition from a National Institutes of Child Health and Human Development workshop. At 36 weeks PMA, 89 % continued on sO2 (moderate/severe BPD), and at discharge, 74 % continued on sO2. When factors associated with NDI were examined, the need for mechanical ventilation ≥28 days (adjOR = 3.21, p = 0.01), grade III–IV intraventricular hemorrhage (IVH) (adjOR = 4.61, p < 0.01), and discharge at >43 weeks PMA (adjOR = 2.12, p = 0.04) were the strongest predictors of NDI at 18 months CGA. There was no difference in Bayley Scales of Infant Development, third edition composite scores between patients with no/mild BPD and patients with moderate/severe BPD (cognitive p = 0.60, communication p = 0.53, motor p = 0.19) or those scores between patients on and off oxygen at discharge (cognitive p = 0.58, communication p = 0.70, motor p = 0.62). Conclusions: The need for sO2 at discharge is not associated with an increased risk of NDI in these patients. The strongest predictors of poor neurodevelopmental outcome in this population were prolonged positive pressure support, grade III–IV IVH, and discharge at >43 weeks PMA.
Mechanical ventilation; Supplemental oxygen; Very low birth weight; Intraventricular hemorrhage; Necrotizing enterocolitis
Infantile autism is a serious comprehensive developmental disorder. The diagnosis of hearing loss or its exclusion, which often suggests suspected autism, is very important for early ENT, psychotherapy, and psychiatric treatment. One hundred children diagnosed with autism aged from 3 to 18 years, with a median age of 5 years, were evaluated. The control group of healthy children consisted of 100 children, aged from 3 to 18 years, with a median age of 6 years. Anamnesis and physical examination, including pediatric assessment and otoscopic examination, were carried out on children in both groups. Each child underwent bilateral otoacoustic emission examination in the 0.7, 1, 2, and 4 kHz bands and impedance audiometry examination. The data obtained were subjected to a basic statistical assessment. Chi2 Pearson's test was used to compare results of tests in both groups. The absence of otoacoustic emission for the 1 and 2 kHz bands was significantly more frequent in the group of autistic children than in the control group. Furthermore, types B and C2 tympanometric curves were significantly more common in the group of autistic children than in the group of healthy children.
Autism; Children; Conductive hearing loss
The Hunter Outcome Survey (HOS), an international, long-term observational registry of patients with Hunter syndrome, was used to develop a simple mnemonic screening tool (HUNTER) to aid in the diagnosis of Hunter syndrome. Data regarding the prediagnosis prevalence of ten specific signs and symptoms present in individual patients enrolled in the HOS were used to develop the HUNTER mnemonic screening tool. A total score of 6 or greater using a weighting scheme in which certain manifestations were assigned a weight of 2 (facial dysmorphism, nasal obstruction or rhinorrhea, enlarged tongue, enlarged liver, enlarged spleen, joint stiffness) and others assigned a weight of 1 (hernia, hearing impairment, enlarged tonsils, airway obstruction or sleep apnea) correctly identified 95 % of patients who had no family history of Hunter syndrome or who were not diagnosed prenatally. No association between age at diagnosis and HUNTER score was found. Conclusion: The HUNTER mnemonic appears to be a useful screening tool. Further validation in the clinical setting will be necessary to confirm its utility.
Mucopolysaccharidosis type II; Lysosomal storage disease; Hunter syndrome; Diagnosis
This study was a retrospective chart review performed to examine and describe physician practice patterns in managing attention deficit/hyperactivity disorder (ADHD) across Europe. Physicians treating ADHD in the UK, France, Germany, Italy, the Netherlands and Spain were recruited. Each physician abstracted medical records of five patients (aged 6–17 years at time of review) with a documented diagnosis of ADHD made between January 2004 and June 2007. Data provided by the physician via the abstraction included (a) physician characteristics, (b) patient characteristics, (c) ADHD diagnosis and (d) ADHD outcomes (adherence, symptom control and satisfaction). A total of 779 patients met study inclusion criteria. In the overall population, patients’ mean (SD) age at time of diagnosis was 8.9 (2.6) years. The predominant treatment choice was long-acting methylphenidate, which was prescribed to more than 56 % of patients. According to physicians, only 30.8 % of patients showed ‘complete symptom control’ on current treatment and only 31.8 % of physicians reported being ‘very satisfied’ with their patients’ current treatment. Physicians’ assessments of complete symptom control and physician satisfaction with treatment were low, indicating unmet needs with current ADHD management in Europe.
Attention deficit/hyperactivity disorder; Methylphenidate; Amphetamine; Atomoxetine
Myeloid and plasmacytoid dendritic cells (MDC/PDC) play crucial roles in bridging adaptive and innate immunity by affecting development of both cellular and humoral immunity. The immune system evolves after birth as reflected in dynamic changes in numbers and functions of various immune cells with age. However, age-associated changes in DC subsets in children have not been elucidated despite the fact that such normative data are crucial for evaluating alternations of DC subsets in various pediatric diseases. This study addressed age-associated changes in DC subsets and CD40/86 expression on PDC (markers of maturation/activation) in 50 healthy children in comparison with 25 children with specific polysaccharide antibody deficiency (SPAD). Our results revealed age-dependent decrease of PDC numbers (p<0.0001), although there was no age-associated changes in CD40/CD86 expression. MDC1/MDC2 numbers did not reveal such linear age-dependent changes and MDC1/PDC ratio reached around 2 as typically seen in young adults after 10 years of age. In contrast, SPAD patients did not reveal such age-associated changes and showed decreased fluorescence intensity of CD86 in PDC cells. These results indicate lineage specific, age-dependent changes in DC subsets in normal children and possible altered development of these cells in SPAD children, emphasizing the importance of age-appropriate controls.
Dendritic cells (DCs); Plasmacytoid DC; Myeloid DC; Specific polysaccharide antibody deficiency (SPAD); CD86
Growth references are useful in monitoring a child's growth, which is an essential part of child care. The aim of this paper is to provide updated growth references for Polish preschool children and to assess how well children in Poland match or diverge from the World Health Organization (WHO) growth standards/references and recent German height-for-age references. The height-, weight-, body mass index-for-age, and weight-for-height references were constructed with the LMS method using data from a recent, large, population-representative sample of 4,941 preschool children aged 3 to 6 years (the OLA study). In the case of boys, the third, 50th, and 97th height percentiles of new Polish and German references overlap almost completely, whereas the WHO growth standards/references percentiles are systematically lower. In the case of girls, comparison between the new Polish and German height references showed conformity on the third and 50th percentile, whereas body height values of the WHO standards/references are shorter. Polish children aged 3 to 6 years from for the nation representative sample, had significantly greater than zero mean z scores of height-, weight-, and BMI-for-age and weight-for-height, relative to the WHO growth standards/references. The number of children in the sample with height-for-age below −2 SD was significantly lower than expected and number of children with height-for-age above +2 SD was significantly higher than expected. Conclusion: The OLA study growth references can be recommended as national references for preschool children in Poland.
Preschool children; Growth references; LMS method
This study had two objectives. First, to determine the prevalence of hollow (high-arched) and flat foot among primary school children in Cracow (Poland). Second, to evaluate the relationship between the type of medial longitudinal arch (MLA; determined by the Clarke’s angle) and degree of fatness. The prevalence of underweight, overweight, and obesity was determined by means of IOTF cut-offs with respect to age and gender. A sample of 1,115 children (564 boys and 551 girls) aged between 3 and 13 years was analyzed. In all age groups, regardless of gender, high-arched foot was diagnosed in the majority of children. A distinct increase in the number of children with high-arched foot was observed between 7- and 8-year olds. Regardless of the gender, high-arched foot was more common among underweight children. In the group of obese children, the biggest differences were attributed to gender. High-arched foot was the most frequently observed among boys. In all gender and obesity level groups, the flat foot was more common among boys than among girls. Conclusions: High-arched foot is the most common foot defect among children 3–13 years old regardless of gender. Flat foot is least frequently observed in children 3–13 years old. A statistic correlation between MLA and adiposity is observed. Stronger correlation is observed among girls.
Flat foot; High-arched foot; MLA; Adiposity; Children
Introduction and purpose of the study
With this study we aimed to describe a “true world” picture of severe paediatric ‘community-acquired’ septic shock and establish the feasibility of a future prospective trial on early goal-directed therapy in children. During a 6-month to 1-year retrospective screening period in 16 emergency departments (ED) in 12 different countries, all children with severe sepsis and signs of decreased perfusion were included.
A 270,461 paediatric ED consultations were screened, and 176 cases were identified. Significant comorbidity was present in 35.8 % of these cases. Intensive care admission was deemed necessary in 65.7 %, mechanical ventilation in 25.9 % and vasoactive medications in 42.9 %. The median amount of fluid given in the first 6 h was 30 ml/kg. The overall mortality in this sample was 4.5 %. Only 1.2 % of the survivors showed a substantial decrease in Paediatric Overall Performance Category (POPC). ‘Severe’ outcome (death or a decrease ≥2 in POPC) was significantly related (p < 0.01) to: any desaturation below 90 %, the amount of fluid given in the first 6 h, the need for and length of mechanical ventilation or vasoactive support, the use of dobutamine and a higher lactate or lower base excess but not to any variables of predisposition, infection or host response (as in the PIRO (Predisposition, Infection, Response, Organ dysfunction) concept).
The outcome in our sample was very good. Many children received treatment early in their disease course, so avoiding subsequent intensive care. While certain variables predispose children to become septic and shocked, in our sample, only measures of organ dysfunction and concomitant treatment proved to be significantly related with outcome. We argue why future studies should rather be large multinational prospective observational trials and not necessarily randomised controlled trials.
Paediatric; Child; Emergency medicine; Sepsis; Shock; Outcome
In Europe, the introduction of monovalent meningococcal serogroup C (MenC) conjugate vaccines has resulted in a significant decline in MenC invasive disease. However, given the potential for strain evolution and increasing travel to areas of high endemicity, protection against additional serogroups is needed. In this study, the immunogenicity, measured by a serum bactericidal activity assay using rabbit complement (rSBA), and the safety of a quadrivalent meningococcal serogroups A, C, W-135 and Y tetanus toxoid conjugate vaccine (MenACWY-TT) were compared to that of a licensed monovalent MenC conjugate vaccine (MenC-CRM197) in children 2–10 years of age. Children were randomised (3:1) to receive a single dose of either MenACWY-TT or MenC-CRM197. Non-inferiority of the immunogenicity of MenACWY-TT versus MenC-CRM197 in terms of rSBA-MenC vaccine response was demonstrated. Exploratory analyses suggested that rSBA-MenC geometric mean titres adjusted for pre-vaccination titres were lower in children vaccinated with MenACWY-TT compared to MenC-CRM197. Nevertheless, at 1 month post-vaccination, ≥99.3 % of the children who received MenACWY-TT had rSBA titres ≥1:128 for each of the four vaccine serogroups, which is the more conservative correlate of protection. The reactogenicity and safety profile of MenACWY-TT was clinically acceptable and no serious adverse events considered related to vaccination were reported throughout the study. Conclusion: When administered to European school-age children, MenACWY-TT has a clinically acceptable safety profile and, when compared with MenC-CRM197, the potential to broaden protection against meningococcal disease caused by serogroups A, W-135 and Y while maintaining protection against MenC. This study has been registered at www.clinicaltrials.gov NCT00674583.
Quadrivalent meningococcal vaccine; Conjugate vaccine; Tetanus toxoid; Child; Bactericidal activity; Randomised trial
The New Vaccinations of Infants in Practice online survey in seven countries evaluated vaccination-related attitudes and concerns of parents of infants and health care providers (HCPs) who provide pediatric medical care. The survey showed that HCPs and parents were open to adding new vaccines to the immunization schedule, even if it requires co-administration with current vaccines or introduction of new office visits. Parental disease awareness campaigns would be helpful to achieve widespread acceptance of changes to vaccination schedules. In addition, HCPs would ideally provide disease education to parents to accompany recommendations for a new vaccine.
Electronic supplementary material
The online version of this article (doi:10.1007/s00431-012-1904-4) contains supplementary material, which is available to authorized users.
Survey; Health care providers; Parents; Attitude; Infant; Immunization; Concomitant; Vaccinations; Vaccine; Meningococcal; Provider practices
Diarrhea treatment with either Lactobacillus GG (LGG) or smectite as an adjuvant to standard rehydration therapy has proven efficacy. In countries where both LGG and smectite are available, concomitant use is frequently practiced. We investigated whether LGG plus smectite is superior to LGG alone in the management of children with acute gastroenteritis (AGE). A double-blind, placebo-controlled, randomized trial was performed. Children aged 4 to 60 months with AGE received LGG 6 × 109 colony forming units/day plus randomly either smectite (3 g) or placebo as an adjuvant to the standard rehydration therapy. Of the 88 children randomized, 81 (92 %) were available for intention-to-treat analysis. The duration of diarrhea in the LGG/smectite group (n = 44) compared with the LGG/placebo group (n = 37) was similar (P = 0.43). There were no significant differences between the study groups for the secondary outcomes, with three exceptions. On day 4, in the LGG/placebo group compared to the LGG/smectite group, there was significantly reduced stool frequency (P = 0.03). While there was a significant (P = 0.05) difference in stool consistency on the Bristol Stool Form Scale on day 4, it was not of clinical relevance. Finally, in the LGG/smectite group compared to the LGG/placebo group, there was a significantly shorter duration of intravenous therapy after randomization (P = 0.02). No adverse events were observed in the study groups. Conclusion: LGG plus smectite and LGG alone are equally effective for treating young children with AGE. Combined use of the two interventions is not justified.
LGG; Diosmectite; Dioctahedral smectite; Diarrhea; Infants; RCT
Waist circumference, a proxy measure of abdominal obesity, is associated with cardio-metabolic risk factors in childhood and adolescence. Although there are numerous studies about waist circumference percentiles in children, only a few studies cover preschool children. The aim of this study was to develop age- and gender-specific waist circumference smoothed reference curves in Turkish preschool children to determine abdominal obesity prevalence and to compare them with reference curves obtained from different countries. The design of the study was cross-sectional. A total of 2,947 children (1,471 boys and 1,476 girls) aged 0–6 years were included in the study. The subjects were divided according to their gender. Waist circumference was measured by using a standardized procedure. The age- and gender-specific waist circumference reference curves were constructed and smoothed with LMS method. The reference values of waist circumference, including 3rd, 10th 25th, 50th, 75th, 90th, and 97th percentiles, and standard deviations were given for preschool children. Waist circumference values increased with age, and there were differences between genders. The prevalence of abdominal obesity was calculated as 10.1 % for boys and 10.7 % for girls. Having compared our data with two other countries’ data, we found that our waist circumference data were significantly lower. This is the first cross-sectional study for age- and gender-specific references of 0- to 6-year-old Turkish children. The gender- and age-specific waist circumference percentiles can be used to determine the risk of central obesity.
Waist circumference reference values; Preschool children
Approximately one-third of boys with X-linked adrenoleukodystophy (X-ALD) develop an acute, progressive inflammatory process of the central nervous system, resulting in rapid neurologic deterioration and death. Hematopoietic cell transplantation (HCT) can halt the progression of neurologic disease if performed early in the course of the cerebral form of X-ALD. We describe a retrospective cohort study of 90 boys with X-ALD evaluated at our institution between 2000 and 2009, to determine if early diagnosis of X-ALD following the diagnosis of unexplained adrenal insufficiency (AI) improves outcomes. We describe 7 cases with a delay in the diagnosis of X-ALD, and compare their outcomes to 10 controls with the diagnosis of ALD made within 12 months following diagnosis of AI. At the time of evaluation for HCT, boys with a delay in the diagnosis of X-ALD had more extensive cerebral involvement and more limited functioning. These boys also were 3.9 times more likely to die, and had significant advancement of cerebral disease after HCT, compared to boys with a timely diagnosis of X-ALD.
Early diagnosis of cerebral X-ALD following the diagnosis of unexplained AI, and subsequent treatment with HCT, improves both neurological outcomes and survival in boys with cerebral X-ALD.
adrenoleukodystrophy; adrenal insufficiency; Addison’s disease; hematopoietic cell transplantation
Heritable connective tissue diseases comprise a heterogeneous group of multisystemic disorders that are characterized by significant morbidity and mortality. These disorders do not merely result from defects in the amount or structure of one of the components of the extracellular matrix, as the extracellular matrix also serves other functions, including sequestration of cytokines, such as transforming growth factor beta (TGFβ). Indeed, disturbed TGFβ signaling was demonstrated in several heritable connective tissue diseases, including syndromic forms such as Marfan or Loeys-Dietz syndrome and non-syndromic presentations of thoracic aortic aneurysm/dissection. Because of these findings, new therapeutic targets have been unveiled, leading to the initiation of large clinical trials with angiotensin II type 1 receptor antagonists that also have an inhibiting effect on TGFβ signaling. Here, we present an overview of the clinical characteristics, the molecular findings, and the therapeutic strategies for the currently known syndromic and non-syndromic forms of thoracic aortic aneurysm/dissection.
Heritable connective tissue disease; Thoracic aortic aneurysm; Marfan syndrome; Loeys-Dietz syndrome; TGFβ signaling
The varying clinical manifestations of Lyme borreliosis, transmitted by Ixodes ricinus and caused by Borrelia burgdorferi, frequently pose diagnostic problems. Diagnostic strategies vary between early and late disease manifestations and usually include serological methods. Erythema migrans is pathognomonic and does not require any further laboratory investigations. In contrast, the diagnosis of neuroborreliosis requires the assessment of serum and cerebrospinal fluid. Lyme arthritis is diagnosed in the presence of newly recognized arthritis and high-titer serum IgG antibodies against B. burgdorferi. The committee concludes the following recommendations: Borrelial serology should only be ordered in case of well-founded clinical suspicion for Lyme borreliosis, i.e., manifestations compatible with the diagnosis. Tests for borrelial genomic sequences in ticks or lymphocyte proliferation assays should not be ordered. When results of such tests or of serological investigations that were not indicated are available, they should not influence therapeutic decisions. Laboratories should be cautious when interpreting results of serological tests and abstain from giving therapeutic recommendations and from proposing retesting after some time without intimate knowledge of patient's history and disease manifestations.
Lyme borreliosis; Borrelia burgdorferi; Neuroborreliosis; Lyme arthritis; Diagnosis; Children; Recommendations
Hereditary angioedema due to C1 inhibitor (C1 esterase inhibitor) deficiency (types I and II HAE-C1-INH) is a rare disease that usually presents during childhood or adolescence with intermittent episodes of potentially life-threatening angioedema. Diagnosis as early as possible is important to avoid ineffective therapies and to properly treat swelling attacks. At a consensus meeting in June 2011, pediatricians and dermatologists from Germany, Austria, and Switzerland reviewed the currently available literature, including published international consensus recommendations for HAE therapy across all age groups. Published recommendations cannot be unconditionally adopted for pediatric patients in German-speaking countries given the current approval status of HAE drugs. This article provides an overview and discusses drugs available for HAE therapy, their approval status, and study results obtained in adult and pediatric patients. Recommendations for developing appropriate treatment strategies in the management of HAE in pediatric patients in German-speaking countries are provided.Conclusion Currently, plasma-derived C1 inhibitor concentrate is considered the best available option for the treatment of acute HAE-C1-INH attacks in pediatric patients in German-speaking countries, as well as for short-term and long-term prophylaxis.
C1-INH (C1 inhibitor, C1-esterase inhibitor); Hereditary angioedema; Consensus; Pediatric
Skinfold thicknesses are used as valid anthropometric indicators of regional body fatness. Actual population-based values for skinfold thicknesses for Polish children are not available. The purpose of this study was to provide population-based values for triceps, subscapular, and abdominal skinfold thicknesses in healthy children and adolescents. A total number of 17,416 boys and girls aged 6.5–18.5 years, randomly selected from whole Polish population of children and adolescents, were enrolled in the study. Skinfold thicknesses (triceps, subscapular, and abdominal) were measured using Harpenden skinfold caliper. All measurements were taken after the training of participating investigators. The LMS method was used to fit percentile curves across age for each skinfold. Q tests for fit were used to assess the global goodness of fit of our final models. The study shows for the first time smoothed population-based values of body fat distribution indices for Polish children and adolescents 7–18 years of age. Reported skinfold centiles are higher compared to previously established for Warsaw children and very close to the actual US data. Conclusion Our study provided for the first time population-based values for skinfold thicknesses evaluation in a way allowing to calculate reliable Z scores. The early detection of abnormal fat stores, using our population-based values and respective Z scores, may be now implemented for practice.
Triceps; Subscapular; Abdominal skinfold thickness; Population-based values; Children; Polish
Coeliac disease (CD) is an immune-mediated systemic condition elicited by gluten and related prolamines in genetically predisposed individuals and characterised by gluten-induced symptoms and signs, specific antibodies, a specific human leukocyte antigen (HLA) type and enteropathy. The risk of coeliac disease is increased in first-degree relatives, certain syndromes including Down syndrome and autoimmune disorders. It is thought to occur in 1 in 100–200 individuals, but still only one in four cases is diagnosed. Small-bowel biopsy is no longer deemed necessary in a subgroup of patients, i.e. when all of the following are present: typical symptoms or signs, high titres of and transglutaminase antibodies, endomysial antibodies, and HLA-type DQ2 or DQ8. In all other cases, small-bowel biopsy remains mandatory for a correct diagnosis. Therapy consists of a strictly gluten-free diet. This should result in complete disappearance of symptoms and of serological markers. Adequate follow-up is considered essential. Conclusion: Although small-bowel biopsy may be omitted in a minority of patients, small-bowel biopsy is essential for a correct diagnosis of CD in all other cases. Diagnostic work-up should be completed before treatment with gluten-free diet instituted.
Coeliac disease; Gluten intolerance; HLA-DQ2; HLA-DQ8; Endomysium antibodies; Transglutaminase type 2 antibodies; Small-bowel biopsy; Marsh classification; Gluten-free diet
Mucopolysaccharidosis II (MPS II), or Hunter syndrome, is an X-linked lysosomal storage disorder caused by a deficiency in the enzyme iduronate-2-sulfatase. Affected patients suffer progressive damage to multiple organ systems and early mortality. Two thirds of patients also manifest cognitive impairment and developmental delays. MPS II can be extremely difficult to diagnose before irreversible organ and tissue damage has occurred because of an insidious onset and the overlap in signs and symptoms with common childhood complaints. This is particularly true of patients without cognitive impairment (attenuated phenotype). Although not curative, early treatment with enzyme replacement therapy before irreversible organ damage has occurred may result in the greatest clinical benefit. Here, the signs, symptoms, and surgical history that should trigger suspicion of MPS II are described, and the diagnostic process is reviewed with a focus on practical considerations and the avoidance of common diagnostic pitfalls. Once a diagnosis is made, multidisciplinary management with an extended team of pediatric specialists is essential and should involve the pediatrician or family practice physician as facilitator and medical home for the patient and family. Conclusion: Because routine newborn screening is not yet available for MPS II, the involvement and awareness of pediatricians, family practice physicians, and pediatric specialists is critical for early identification, diagnosis, and referral in order to help optimize patient outcomes.
Lysosomal storage disease; Mucopolysaccharidosis II; Hunter syndrome; Glycosaminoglycans; Iduronate-2-sulfatase; Diagnosis
Despite the high prevalence of enuresis, the professional training of doctors in the evaluation and management of this condition is often minimal and/or inconsistent. Therefore, patient care is neither optimal nor efficient, which can have a profound impact on affected children and their families. Once comprehensive history taking and evaluation has eliminated daytime symptoms or comorbidities, monosymptomatic enuresis can be managed efficaciously in the majority of patients. Non-monosymptomatic enuresis is often a more complex condition; these patients may benefit from referral to specialty care centers. We outline two alternative strategies to determine the most appropriate course of care. The first is a basic assessment covering only the essential components of diagnostic investigation which can be carried out in one office visit. The second strategy includes several additional evaluations including completion of a voiding diary, which requires extra time during the initial consultation and two office visits before treatment or specialty referral is provided. This should yield greater success than first-line treatment. Conclusion: This guideline, endorsed by major international pediatric urology and nephrology societies, aims to equip a general pediatric practice in both primary and secondary care with simple yet comprehensive guidelines and practical tools (i.e., checklists, diary templates, and quick-reference flowcharts) for complete evaluation and successful treatment of enuresis.
Enuresis; Monosymptomatic; Consensus guidelines; Primary care; Management
We report a 16-year-old girl in whom Takayasu arteritis (TA) was manifested mainly by severe arterial hypertension on her right arm, which was detected during a routine examination at school. Her systolic blood pressure on the right arm was significantly higher than that on the left one. There was also a pressure difference between the right arm and legs. The pulse of the left external carotid artery and that of the left radial artery was absent. Vascular bruits over interscapular and right supra- and subclavian areas were heard on auscultation. The diagnosis of TA was confirmed by a spiral computed tomography angiography, which showed a thickened thoracic aortic wall and narrowing of its lumen. In addition, complete occlusion of the left common carotid artery and the left subclavian artery was observed. Conclusion: The rarity of the disorder and the heterogeneous nature of its clinical manifestation predispose to a late diagnosis and delayed treatment. Our report highlights the fact that the condition can and does occur in a pediatric population in Europe and hence must be considered in patients presenting with suggestive symptoms and signs, especially in young patients with unexplained hypertension. Clinical suspicion and proper imaging are crucial for the correct diagnosis and management of patients with TA. A brief review of literature completes this report.
Takayasu arteritis; Arterial hypertension; Children