Dagmar l'Allemand-Jander

Ostschweizer Kinderspital (KISPI)

Nerve conduction velocity (NCV) abnormalities are the forerunners of diabetic peripheral neuropathy (DPN). Therefore, this study aimed to analyze the effect of glucose profile quality on NCV in children and young adults with type 1 diabetes. Fifty-three children age 5 to 23 years with type 1 diabetes were recruited to participate in the study, whic...

One-sixth of Swiss children are affected by overweight, and despite the implementation of an evidence-based multiprofessional approach, there has only been moderate therapeutic success. An unfavourable home environment and psychosocial stresses on the family may impede lifestyle changes. This longitudinal observational study included children with...

Background/Aim: Nerve conduction speed (NCS) abnormalities are considered to be early signs of diabetic peripheral neuropathy. We investigated which determinants impact the NCS and how it is related to markers of metabolic control in children and young adults with diabetes mellitus. Method: Fifty-four children aged five to 23 years suffering from t...

Effective interventions for the prevention and treatment of child and adolescent obesity play an important role in reducing the global health and economic burden of non-communicable diseases. Although multi-component interventions targeting various health behaviors are deemed promising, evidence for their effectiveness is still limited. Self-regula...

Background: Less than 2% of overweight children and adolescents in Switzerland can participate in multi-component behaviour changing interventions (BCI), due to costs and lack of time. Stress often hinders positive health outcomes in youth with obesity. Digital health interventions, with fewer on-site visits, promise health care access in remote re...

Childhood obesity is one of the major disease patterns of the twenty-first century. Due to the need for multi-professional therapies requiring intensive personnel and financial resources, IT-supported interventions promise help. Meta analyses, however, show their limited impact on health outcomes up till now. The current work aims therefore to desi...

Obesity is a global issue and has a direct impact on the public and private healthcare system. In this paper we describe the design and evaluation of a novel mobile health application that supports obese children and their parents to improve their nutrition intake. First results from quantitative app usage logs, questionnaires and interviews indica...

Background: Weight status in children and adolescents is commonly defined using age- and gender-corrected standard deviation scores for body mass index (BMI-SDS, also called z-scores). Values are not reliable for the extremely obese however. Moreover, paediatricians and parents may have difficulties understanding z-scores, and while percentiles ar...

Childhood obesity is becoming an alarming issu with implications affecting the society and the healthcare sector. In response, multi-professional programs with physical activity, nutritional and psychological components have been proposed. Still, du to limited resources only small number of patients can be included in these programs. Health informa...

The globally increasing prevalence of childhood obesity is one of the most serious public health challenges of the twenty-first century. Du to the need for multi-professional therapies that require a high amount of personnel and financial resources, IT-supported interventions promise help. So far, meta-studies show their limited impact on health ou...

The general practitioner or pediatrician mostly is the first point of contact for overweight children and may recognize adiposity early enough in order to start therapy of obesity or comorbidity or to initiate measures of prevention. Interventions against overweight are most efficient before age 7 in terms of short-and long-term results and should...

Context: The predictive value of body mass index (BMI), waist circumference (WC), and waist-to-height ratio (WtHR) to define cardiometabolic risk is unclear in childhood obesity. Objective: [corrected] The associations between BMI, WtHR, or WC and cardiometabolic risk markers were analyzed in a multicenter data collection of obese youth. Design...

Objective: The aim was to analyze the effectiveness of treatment concerning obesity-associated comorbidities in clinical practice. Methods: A total of 11,681 overweight children with ≥ 6-month follow-up treated at 175 centers specialized in pediatric obesity care in Central Europe were included in this analysis (mean body mass index (BMI) 29.0 ±...

Objective: Although there is evidence of increasing prevalence of impaired glucose metabolism in obese children from smaller single cohorts, data are lacking on the progression of glucose metabolism in this patient group.We aimed to assess the prevalence and the longitudinal course of impaired glucose metabolism assessed by oral glucose tolerance...

Background. In 1992 in Berlin a screening for congenital adrenal hyperplasia (CAH) was introduced. Diagnosis. Since then nearly 250 000 newborns were screened and 26 newborns with classical CAH due to 21-hydroxylase deficiency were detected. The diagnosis was ascertained by specific determination of serum 17-OHP and molecular genetic diagnosis. The...

Overweight and obese youth represent a challenge for the affected individual, the healthcare system as well as society as a whole. Increased long-term cardiovascular risk is one of the major consequences of early-onset obesity, affecting both life expectancy and quality of life. The aim of this report is to study the effects of age, gender and obes...

Childhood overweight (body mass index (BMI)>90th centile) poses a major public health problem in so far as adult diseases manifest themselves already during childhood. In this review, after examining the prevalence of metabolic and cardiovascular diseases, the issue of whether BMI or other clinical parameters are valid tools to predict co-morbiditi...

Comorbidities of childhood obesity challenge health-care systems in Europe. Further, there is a lack of population-specific prevalence data and diagnostic strategies available, especially for obesity-related disturbances of liver function. Therefore, the prevalence of elevated liver enzymes and their relationship to biological parameters were studi...

To study whether metformin reduces obesity, homeostasis model assessment for insulin resistance index (HOMA-IR), and the metabolic syndrome (MtS) in obese European adolescents in addition to previous unsuccessful lifestyle intervention. After 6 months of multiprofessional lifestyle intervention, 70 out of 86 adolescents without improvement in body...

Activating mutations in the TSHR gene were found in patients suffering from nonautoimmune hyperthyroidism. In the past, it was assumed that thyroid hyperplasia is due to constitutive activation of the Gs/adenylyl cyclase signaling pathway; however, the physiological role of the Gq/11 pathway in this context remains unclear. In this study, we invest...

An impressive discrepancy between reported and measured parental height is often observed. The aims of this study were: (a) to assess whether there is a significant difference between the reported and measured parental height; (b) to focus on the reported and, thereafter, measured height of the partner; (c) to analyse its impact on the calculated t...

P450c17 has two distinct activities: 17alpha-hydroxylase activity and 17,20-lyase activity. Combined 17alpha-hydroxylase/17,20-lyase deficiency leads to a severe defect in the production of cortisol and sex steroids. In affected males this results in impaired masculinization with ambiguous or female external genitalia. Female patients have normal g...

Although randomized controlled trials demonstrated the long-term efficacy of lifestyle interventions in overweight children, the effects of these interventions in clinical practice under real-life conditions are largely unknown. One hundred twenty-nine centers specialized in outpatient pediatric obesity care participated in this quality assessment....

Insulin resistance (IR) and hypertension are common in overweight children, and the adipocyte-derived hormones resistin, adiponectin, and leptin may modulate IR and blood pressure (BP). Few data exist in children on dietary determinants of IR, BP, or leptin, and no data exist on dietary determinants of resistin and adiponectin. Therefore, the objec...

To explore how foot growth relates to musculoskeletal loading in children with Prader-Willi syndrome (PWS). In 37 children with PWS, foot length (FL) before and after 6 years of growth hormone therapy (GHT) was retrospectively evaluated with parental and sibling's FL, height, and factors reflecting musculoskeletal loading, such as weight for height...

Muscle hypotonia and failure to thrive are key symptoms of Prader-Willi syndrome (PWS) allowing diagnosis during infancy already. Improved general care as well as Coenzyme Q(10) (CoQ(10)) and growth hormone (GH) are administered to improve PWS children's outcome. This study aims to investigate psychomotor development of young PWS children in relati...

We report on a 20-year-old man in whom endocrinological investigation owing to dysmorphic signs characteristic for Turner syndrome revealed mixed gonadal dysgenesis. The patient was referred to us for further diagnostic investigations on a right intrascrotal tumour. Both testes were intrascrotal and hypotrophic with normal testosterone production....

Although retinol-binding protein (RBP)-4 concentrations are elevated in animal models of obesity and insulin resistance (IR), the link between RBP4 and IR in humans is less clear. There are few published data on RBP4 levels in overweight children, and most previous studies did not control for vitamin A (VA) status and/or subclinical inflammation. T...

High amounts of dietary fructose may contribute to dyslipidemia in adults, but there are few data in children. Childhood adiposity is associated with smaller LDL particle size, but the dietary predictors of LDL size in overweight children have not been studied. We aimed to determine whether LDL particle size is associated with dietary factors and s...

In boys with Prader-Labhart-Willi syndrome (PWS), hypogonadism causes pubertal arrest and reduces pubertal muscle growth. Formerly, it was assumed that therapy with gonadal hormones accentuates behaviour abnormalities in PWS. Our aim was to assess the clinical effects of human chorionic gonadotropin (hCG) therapy on pubertal development, muscle mas...

In obese children, subclinical inflammation is often present and is correlated with the metabolic syndrome. Dietary factors, such as fatty acids and antioxidants, potentially modulate the association between adiposity and subclinical inflammation, but few data are available in children. The aim of the study was to determine whether dietary fat or a...

The specific form of hypogonadism in Prader-Labhart-Willi syndrome (PWS), central or peripheral, remains unexplained. The objectives of this study were to investigate the cause of hypogonadism in PWS and determine whether human chorionic gonadotropin (hCG) treatment can restore pubertal development. This was a clinical follow-up study, divided into...

The cardiovascular risk factors hypertension and dyslipidemia clearly predict morbidity and mortality.Data on the prevalence before, during and after puberty, and on the rela- tion to gender and degree of overweight, pro-vide a basis for early intervention. 65 cen- ters specialised in obesity care contributed standardized, anonymized data documente...

Unlabelled: The reduction of spontaneous physical activity (PA) and of muscle tissue are thought to be major causes of fat accretion and metabolic deterioration in Prader-Labhart-Willi syndrome (PWS). We investigated whether a generalized physical training programme in a home setting improves these parameters. The prospective study included 11 pre...

Dosage recommendations for the initial therapy of congenital hypothyroidism (CH) in newborns vary between 8 microg/kg/d and 10-15 microg/kg/d. To evaluate the practicability of LT4 in liquid form and to define the initial dosage for optimal treatment. Liquid LT4 solution was administered to 28 consecutive newborns with primary CH. We measured TSH,...

To assess body composition of infants with Prader-Willi syndrome (PWS) by using deuterium dilution and investigating the efficacy of early institution of growth hormone (GH) therapy in increasing lean mass (LM) and preventing massive obesity. One group of 11 children with PWS <2 years before and during 30-month GH therapy (GH group) was compared wi...

In children with Prader-Labhart-Willi syndrome (PWS), the insulin secretion is reduced, despite obesity, being ascribed to the growth hormone (GH) deficiency of hypothalamic origin. Besides, an increased prevalence of diabetes mellitus was described in this syndrome. Hence, we addressed the questions of how body composition and insulin secretion ar...

To determine if muscle training in Prader-Willi Syndrome (PWS) can improve local body composition, physical capacity, and activity. Seventeen children and adolescents with PWS and 18 control children were enrolled in a daily short calf muscle training program for 3 months. Before (t(0)) and after 3 months of training (t(3m)), spontaneous physical a...

Berlin, Humboldt-Univ., Habil.-Schr., 2003. Computerdatei im Fernzugriff.

Only in the recent past has Prader-Willi syndrome (PWS) also been diagnosed in newborns and infants, and the first descriptions of respiratory disturbances in young, not yet obese patients have been published only recently. We report an infant with PWS and respiratory problems, who suffered sudden death. To our knowledge, this is the first such cas...

To explain why adrenal androgens rise with increasing adiposity during childhood, the role of body mass index (BMI), leptin and IGF-I was studied. We also tested whether these parameters contribute to inducing premature adrenarche (PA). In a cross-sectional study, 26 prepubertal obese children were compared with a group of 26 prepubertal children o...

The case of a boy with Prader-Willi syndrome (PWS) who suffered from respiratory problems since birth and suddenly died at the age of 6.5 years, 4 months after initiation of GH therapy, is presented. This case indicates the possibility of fatal courses in infants and children with PWS as a consequence of respiratory problems and raises the question...

Since hyperandrogenism in simple obesity is assumed to arise from hyperinsulinism and/or increased insulin-like growth factor I (IGF-I) or leptin levels, we examined how in patients with Prader-Willi syndrome (PWS), the most frequent form of syndromal obesity, the accelerated adrenarche can be explained despite hypothalamic-pituitary insufficiency...

Mutations of the Wilms tumor suppressor gene (WT1 ) have been described only in patients with syndromes associated with urogenital malformation and Wilms tumor or nephropathy. We present a male patient with an isolated genital malformation caused by a WT1 mutation.

Pediatricians and neonatologists now understand the clinical picture of Prader-Willi syndrome (PWS) in infants as genetic tools are available to confirm this diagnosis. Hence, an increasing number of very young, still underweight children are being diagnosed with PWS. Some features, such as low prenatal weight and below-average height, subsequent p...

The adrenal cortex encloses the neuroendocrine medulla and is itself subdivided into three distinct zones, each having a specific function and regulation. While the glomerulosa and the fasciculata control vital systems of mineral and energy supply, which are stringently regulated by higher control factors, the function of the reticularis is less cl...

A case of nonclassic (NC) 21-hydroxylase deficiency, with a moderately elevated 17-hydroxyprogesterone level (145 nmol/L in filter paper blood spot), was detected in newborn screening. The newborn's phenotype was female, with no sign of virilization. Confirmatory diagnosis revealed elevated serum levels of 17-hydroxyprogesterone and of 21-desoxycor...

Unlabelled: Cardiovascular risk factors in Prader-Willi syndrome (PWS, OMIM 176270) may be independently caused by overweight or hypothalamic growth hormone (GH) deficiency. The present observational study in 23 children with PWS, aged 0.3-14.6 years, focuses on the specific pattern, age-dependency and interrelation of cardiovascular risk factors,...

Based on the reported favourable effects of growth hormone (GH) treatment on growth and body composition in Prader-Labhart-Willi syndrome, we studied age dependency and the long-term effects on growth dynamics to elucidate the assumed hypothalamic GH deficiency. We examined 23 children treated with hGH (24 U/m(2)/week) during a median of 4 (range 1...

Obesity and hypothalamic GH deficiency contribute in different ways to the disturbances of body composition in Prader-Willi syndrome (PWS); while both increase the fat compartment, the reduction of lean tissue mass has been attributed mainly to GH deficiency. Therefore, body composition measured by dual-energy X-ray absorptiometry was prospectively...

Prader-Labhart-Willi syndrome (PWS) is the most frequent form of syndromal obesity. Its main features are associated with hypothalamic dysfunction, which has not yet been comprehensively described. The aim of this review is to present arguments to define the presence of genuine growth hormone (GH) deficiency (GHD) in these patients. Decreasing grow...

The aim of this retrospective study was to investigate the frequency of thyroid dysfunction as assessed by TSH, T3 and T4 in a large cohort of 290 obese and 280 healthy children. In addition, thyroid autoantibodies were measured in random subgroups of 123 obese and 80 control children, iodine excretion in 50 and thyroid volume in 23 of the obese ch...

Mutations in the WT1 gene causing Wilms tumors were first reported in WAGR syndrome (Wilms tumor, Aniridia, Genitourinary malformation, mental Retardation) and Denys Drash syndrome (pseudohermaphroditism, Wilms tumor, nephropathy), but only in a few patients with hypospadias and cryptorchidism without other signs of Denys Drash (DDS) or WAGR syndro...

There is increasing evidence that in the fetal and postnatal development of the adrenal gland, trophic and differentiating effects of ACTH are locally modulated by a species-specific pattern of growth factors. As we have shown previously in human adult adrenocortical cells (HAC) in culture, IGF-I and, even more, IGF-II enhance the steroidogenesis a...

There is increasing evidence that in the fetal and postnatal development of the adrenal gland, trophic and differentiating effects of ACTH are locally modulated by a species-specific pattern of growth factors. As we have shown previously in human adult adrenocortical cells (HAC) in culture, IGF-I and, even more, IGF-II enhance the steroidogenesis a...

8 patients of 7 families with nonclassical adrenal hyperplasia (NCAH) were analysed for defects of the 21-hydroxylase-B-gene. As the defects were small or rare, complete molecular genetic diagnostic up to sequencing of this gene was necessary to detect the genotype, which then was associated with the phenotype. However, mutations in 4 alleles from...

In several species, including the human fetus, insulin-like growth factors (IGF-I and IGF-II) have been reported to modulate adrenal steroidogenesis, thus contributing to adrenal cortical differentiation. In the present study, we examined the long term effects of IGF-I and -II on human adult adrenal fasciculata-reticularis cells cultured in a chemi...

15N-nitrogen balances before and on human growth hormone (hGH) were studied in 13 girls with Turner syndrome (TS) aged 4.4-16 (median 13.2) years (45,X0 or equivalent, no X0/XX mosaicism, no estrogen replacement). The results were compared with those reported from 9 patients with growth hormone deficiency (GHD). The TS patients received subcutaneou...

Inasmuch as recombinant human growth hormone is now more generally available for the treatment of different types of short stature, there is a need for better short-term indicators of treatment success. In healthy children, serum concentrations of antigens related to the aminoterminal propeptide of type III procollagen (P-III-NP) closely follow the...

In 51 sick newborns the influence of two different nonionic, iodine-containing contrast agents, Amipaque (group 1) and Omnipaque (group 2) and of long-term treatment with polyvinylpyrrolidone-iodine (PVP-I) (group 3) on thyroid function was studied. In the dose given, freshly dissolved Amipaque releases roughly 100 micrograms 'free' iodide/kg body...

Transient hyperthyrotropinemia and/or hypothyroidism have been found in many newborn infants during thyroid screening programs. In Europe the most likely causes are iodine deficiency and iodine overload. Because of the high incidence of transient hyperthyrotropinemia in Berlin we measured iodine concentrations in casual urine samples of newborns wi...

In July 1978 a neonatal screening program for congenital hypothyroidism was introduced in Berlin (West) covering more than 98% of the neonates born in the city area. Up to July 1982 TSH was determined on the fifth day of life in 74,350 newborns using a radioimmunoassay for TSH determination in dried blood spots. With a cut-off limit at 20 microU/ml...