Sozialpadiatrisches Zentrum

Dortmund, Germany

Sozialpadiatrisches Zentrum

Dortmund, Germany
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Mendes U.,Sozialpadiatrisches Zentrum | Piller G.,Praxis fur Kinder und Jugendlichenpsychotherapie | Girschick H.,Sozialpadiatrisches Zentrum
Gynakologische Praxis | Year: 2016

The encounter of potential neglect and abuse in childhood is a particular demanding situation for all involved professionals. With the article presented we try to describe options for the modes of action predominantly in the outpatient, but also inpatient setting. Differences between the criminal and social law will be explained, in addition to a proceeding based on the German «Bundeskinderschutzgesetz» (German child protection law).

Neubauer A.-P.,Childrens Hospital Auf der Bult | Voss W.,Sozialpadiatrisches Zentrum | Wachtendorf M.,Sozialpadiatrisches Zentrum | Jungmann T.,Leibniz University of Hanover
Annals of Neurology | Year: 2010

Objective: Erythropoietin has been reported to possess neuroprotective properties in animal studies. No previous studies have investigated the neurodevelopmental outcome of extremely low birth weight (ELBW) infants treated with recombinant human erythropoietin (rEpo) and evaluated it at school age. Methods: Of 200 ELBW infants treated from 1993 to 1998, 171 (86%) survived, and 148 (87%) were followed up to the age of 10 to 13 years. The neurodevelopmental and school outcome of the ELBW infants receiving rEpo treatment for stimulation of erythropoiesis in the first weeks of life (n = 89) was compared to that of untreated children (n = 57). To test for a neuroprotective effect of erythropoietin therapy, analyses of variance (ANOVAs) were conducted with erythropoietin treatment and intraventricular hemorrhage (IVH) as independent variables and Hamburg-Wechsler Intelligence Test for Children-III (HAWIK-III) intelligence quotient (IQ) scores as dependent variables. Results: The rEpo group scored significantly better than untreated children in the overall developmental assessment (55% vs 39% normally developed, p < 0.05) as well as in the psychological examination (mean composite HAWIK-III IQ score, 90.8 vs 81.3, p < 0.005). The results of ANOVAs show that these differences were ascribable to children with IVH. Whereas those children with IVH treated with rEpo scored significantly better than untreated children (52% vs 6% normally developed, composite HAWIK-III IQ score, 90.3 vs 67.0), treated and untreated children without IVH did not differ in their outcome. The treatment and control groups were comparable in perinatal parameters relevant to prognosis. Interpretation: The results of our observational study confirm the hypothesis of a neuroprotective effect of rEpo in ELBW infants with IVH. This offers a promising preventative therapeutic option for the treatment of these high-risk infants. © 2010 American Neurological Association.

PubMed | Biochimie Metabolique Hopital Bichat Claude Bernard, University of Amsterdam, University Hospitals Leuven, Kinderklinik Villingen and 18 more.
Type: Journal Article | Journal: Journal of inherited metabolic disease | Year: 2016

Alpha-1,3-glucosyltransferase congenital disorder of glycosylation (ALG6-CDG) is a congenital disorder of glycosylation. The original patients were described with hypotonia, developmental disability, epilepsy, and increased bleeding tendency.Based on Euroglycan database registration, we approached referring clinicians and collected comprehensive data on 41 patients.We found hypotonia and developmental delay in all ALG6-CDG patients and epilepsy, ataxia, proximal muscle weakness, and, in the majority of cases, failure to thrive. Nine patients developed intractable seizures. Coagulation anomalies were present in <50% of cases, without spontaneous bleedings. Facial dysmorphism was rare, but seven patients showed missing phalanges and brachydactyly. Cyclic behavioral change, with autistic features and depressive episodes, was one of the most significant complaints. Eleven children died before the age of 4years due to protein losing enteropathy (PLE), sepsis, or seizures. The oldest patient was a 40year-old Dutch woman. The most common pathogenic protein alterations were p.A333V and p.I299Del, without any clear genotype-phenotype correlation.ALG6-CDG has been now described in 89 patients, making it the second most common type of CDG. It has a recognizable phenotype and a primary neurologic presentation.

Hampel O.A.,Sozialpadiatrisches Zentrum | Schaadt A.K.,Sozialpadiatrisches Zentrum | Hasmann S.E.,Sozialpadiatrisches Zentrum | Petermann F.,University of Bremen | And 2 more authors.
Klinische Padiatrie | Year: 2010

Stepping Stones Triple P is a behavioural parenting training for families with a handicapped child, which has already been successfully evaluated in its country of origin, Australia. Within a multicentric study of several Social Pediatric Centers (SPCs) and further clinics/institutions in Germany, it has been established as a group parenting training (37 families per training). Statistical analysis proved significant effects concerning dysfunctional parenting, parental stress and child behaviour problems as well as little side effects and a positive acceptance of stepping stones by the families. © Georg Thieme Verlag KG Stuttgart · New York.

Hampel O.A.,Sozialpadiatrisches Zentrum | Hasmann S.E.,Sozialpadiatrisches Zentrum | Schaadt A.K.,Sozialpadiatrisches Zentrum | Holl R.,University of Ulm | And 2 more authors.
Kindheit und Entwicklung | Year: 2010

Stepping Stones Triple P is a behavioral parenting training for families with a handicapped child which has already been successfully evaluated in its country of origin, Australia.Within amulticenter study of several social pediatric centers (SPCs) and further clinics/institutions, it has been established as a parenting group training program (3-7 families per training) and proved its effectiveness concerning dysfunctional parenting behavior, parental stress, and child behavior problems. The present study indicates that adverse psychosocial contexts influence disadvantageously the therapy's effectiveness. Otherwise parental cooperation correlates less with the therapy outcome. © Hogrefe Verlag, Göttingen 2010.

Zacharias R.,Campus Management | Schmidt M.,Campus Management | Kny J.,Campus Management | Sifringer M.,Campus Management | And 5 more authors.
Brain Research | Year: 2010

Exposure to Gamma-aminobutyric-acid (GABA)A-receptor agonists and N-Methyl-d-Aspartate (NMDA)-antagonists has been demonstrated to induce neurodegeneration in newborn rats. Exogenous erythropoietin (EPO) protects against NMDA antagonist-mediated neuronal death. In this study we evaluated whether EPO is also effective in limiting neurodegeneration of the GABAA-mimetic agent propofol in newborn rats. 6 day old rats were randomized to one of four groups and treated with intraperitoneal applications of 3 × 30 mg/kg propofol at 0, 90 and 180 min, propofol in combination with 5000 IU/kg rEPO, propofol in combination with 20,000 IU/kg rEPO or sham injections of PÄD II solution as controls. After 24 h, brains of the animals were histopathologically examined and a summation score of degenerated cells was calculated for every brain. Propofol increased neuronal degeneration scores from 16,090 ± 4336 to 28,860 ± 6569 (p < 0.01). This effect was completely abolished by low-dose rEPO (14,270 ± 4542, p < 0.001 versus propofol only; p > 0.05 versus controls). In contrast, high-dose rEPO was not protective (23 930 ± 8896, p > 0.05 versus propofol only). Propofol may cause neuronal death in newborn rat brains, which is prevented by low-dose rEPO but not high-dose rEPO. © 2010 Elsevier B.V. All rights reserved.

Episodes that resemble seizures but are not epileptic are mostly psychogenic seizures. They are often misdiagnosed for far too long. Many characteristics raise the suspicion of psychogenic seizures such as situational beginning, firm closing of the eyelids, asynchronous movements, shaking of the head, closed mouth, lack of cyanosis, rapid reorientation and a change of seizure symptoms during time. EEG recording during a seizure enables to reliably distinguish between epileptic and psychogenic seizures. Interictal EEG recordings however may give false-positive findings (epileptiform discharges, although no epilepsy is present) or give false-negative findings (normal EEG despite epilepsy). There is good evidence that the way in which a patient spontaneously reports his attacks, provides crucial evidence for the distinction psychogenic vs. epileptic seizure. Patients with epileptic seizures try to describe the attack as accurate as possible. They want to understand how it came about and want to reconstruct the seizure onset. Psychogenic seizures are caused by repressed subconscious conflicts and fears. The patient therefore provides descriptions of surrounding factors but not the seizure itself. Mainly external circumstances are reported, negations are frequent. Activities with which is tried to stop the seizure are hardly ever spontaneously mentioned. To make these differences in the spontaneous description evident it is essential to conduct the history taking in a way, which instead of leading the patient, offers him sufficient space to describe what is important to him.

Pauli S.,University of Gottingen | Schmidt T.,University of Gottingen | Funke R.,Sozialpadiatrisches Zentrum | Zoll B.,University of Gottingen | And 4 more authors.
European Journal of Medical Genetics | Year: 2012

We report on monochorionic diamniotic male twins discordant for the trisomy 12p syndrome. Trisomy 12p mosaicism with a supernumerary der(12)(pter > q12) was detected in approximately 50% of lymphocytes in both children. Fluorescence in situ hybridisation (FISH) revealed a high grade mosaicism of approximately 77% trisomy 12p cells in buccal smear and 85% in hair follicles in the affected twin, while in the normal developing brother an additional 12p chromosome fragment could not be detected in those tissues. Instead, in 3% of buccal smear and hair follicle cells a minute supernumerary marker chromosome comprising central portions of chromosome 12 was observed. Trisomy 12p mosaicism, confined to the lymphocytes of the unaffected twin, may be due to prenatal twin-to-twin transfusion, explaining the conspicuously discordant clinical phenotype. We discuss the possible sequence of events leading to the cytogenetic findings and compare the clinical phenotype presented in the affected twin with other cases of trisomy 12p and tetrasomy 12p (Pallister-Killian syndrome). © 2012 Elsevier Masson SAS.

Borusiak P.,Sozialpadiatrisches Zentrum | Biedermann H.,Praxis fur Manualtherapie | Bosserhoff S.,Marien Hospital | Opp J.,Sozialpadiatrisches Zentrum
Headache | Year: 2010

Objective.-Clinical trials concerning cervical spine manipulation and mobilization in children and adolescents with cervicogenic headache are lacking. Methods.-We performed a multicenter, prospective, randomized, placebo-controlled, and blinded trial in 52 children and adolescents (21 boys, 31 girls) aged 7-15. After prospective baseline documentation for 2 months patients were either assigned to placebo or true manipulation with another 2-month follow-up. Main outcome measures were defined as: percentage of days with headache, total duration of headache, days with school absence due to headache, consume of analgesics, intensity of headache. Results.-We did not find a significant difference comparing the groups with placebo and true manipulation with respect to the defined main outcome measures. Conclusions.-We were not able to show an efficacy of cervical spine manipulation in 52 children and adolescents. © 2009 American Headache Society.

Schulte E.C.,TU Munich | Schulte E.C.,Helmholtz Center Munich | Claussen M.C.,TU Munich | Jochim A.,TU Munich | And 14 more authors.
Movement Disorders | Year: 2013

Background: Recently, mutations in an open-reading frame on chromosome 19 (C19orf12) were identified as a novel genetic factor in neurodegeneration with brain iron accumulation (NBIA). Because of the mitochondrial localization of the derived protein, this variant is referred to as mitochondrial membrane protein-associated neurodegeneration with brain iron accumulation (MPAN). Methods/Results: We describe the clinical phenotype and MRI of 3 newly identified individuals with MPAN due to either previously reported or novel homozygous or compound heterozygous genetic alterations in C19orf12. Conclusions: MPAN is characterized by a juvenile-onset, slowly progressive phenotype with predominant lower limb spasticity, generalized dystonia, and cognitive impairment. Typical additional features include axonal motor neuropathy and atrophy of the optic nerve. MRI showed iron deposition in the globus pallidus and substantia nigra without the eye-of-the-tiger sign, which is typical for PKAN, the most frequent form of NBIA. © 2012 Movement Disorder Society Copyright © 2012 Movement Disorders Society 28 2 February 2013 10.1002/mds.25256 Brief Report Brief Reports Copyright © 2012 Movement Disorders Society.

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