General Paediatrics -
Prof. Dr. Hansjosef Böhles

>

Expertise

The Department of General Paediatrics (Head of Department Prof. Dr. H. Böhles) consists of the following subdivisions: metabolic diseases, endocrinology, gastroenterology (including Mukoviszidosis), neurology and pulmonology/allergology.

The main areas of interest are inherited and iatrogenic metabolic diseases, endocrinology and nutritional aspects of disease. The centre has outpatient clinics for both metabolic and neurometabolic diseases together with a metabolic ward and a specialized metabolic laboratory offering a wide range of analyses necessary for the diagnosis and monitoring of patients with suspected inherited metabolic disease. Among the tests offered, analysis of neurotransmitter in CSF, metabolites of creatine synthesis, purines and pyrimidines and fatty acids are available for research purposes.

Major research interests are:

• Oxidative stress in children
  
• Neurology of inflammatory bowel disease
  
• Disorders of neurotransmission
  
• Influence of feeding ω-3-fatty acids on systemic inflammation
  
• Oxidative stress in patients with various forms of ataxia
  
• Effect of unsaturated fatty acids on allergic inflammation
  
• Effects of feeding docosahexaenoic acid in patients with cystic fibrosis
  
• The effect of ω-3-fatty acids on the course of bronchial asthma
  
  

>

Resources and Abilities

• Aminoacid analysis (Analyser)
  
• Organic acid analysis by gas-chromatography/mass spectrometry (GCMS)
  
• Fatty acid analysis (C12---C26) by GC and GCMS
  
• Neurotransmitters in CSF by HPLC with electrochemical detection (CoulArray)
  
• Purines/Pyrimidines (HPLC with DiodeArray detection)
  
• Creatine/guanidinoacetate (HPLC)
  
• Direct and indirect calorimetry
  
• Analysis of DNA-Oxidation (8-hydroxy-29-deoxyguanosine)
  
  

>

Staff

• Prof. Dr. S. Zielen [Paediatric Pneumologie, Allergologie und Mucoviszidosis)
  
• Priv. Doz. Dr. M. Kieslich (Paediatric Neurology)
  
• Dr. H.-G. Posselt (Paediatric Gastroenterology und Mucoviszidosis)
  
• Dr. J. Herwig (Diabetes mellitus)
  
• Frau Dr. F. Asskali (aminoacids and fatty acids)
  
• Dr. A.C. Sewell (organic acids)
  
• K. Müller (neurotransmitters, purines/pyrimidines, calorimetry)

>

Publications

• Wilhelm M, Tobias R, Asskali F, Kraehner R, Kuly S, Klinghammer L, Boehles H, Daniel WG. Red blood cell omega-3 fatty acids and the risk of ventricular arrhythmias in patients with heart failure. Am Heart J (2008) 155: 971-977.
  
• Koletzko B, Lien E, Agostoni C, Böhles H, Campoy C, Cetin I, Decsi T, Dudenhausen JW, Dupont C, Forsyth S, Hoesli I, Holzgreve W, Lapillonne A, Putet G, Secher NJ, Symonds M, Szajewska H, Willatts P, Uauy R; World Association of Perinatal Medicine Dietary Guidelines Working Group. The roles of long-chain polyunsaturated fatty acids in pregnancy, lactation and infancy: review of current knowledge and consensus recommendations. J Perinat Med (2008) 36: 5-14.
  
• Schubert R, Kitz R, Beermann C, Rose MA, Baer PC, Zielen S, Boehles H. Influence of low-dose polyunsaturated fatty acids supplementation on the inflammatory response of healthy adults. Nutrition (2007) 23: 724-730.
  
• Parbel S, Vlaho S, Gebhardt B, Porto L, Hattingen E, Klingebiel T, Böhles H, Kieslich M. Diagnostic difficulties in encephalitis and glioma. Klin Padiatr (2007) 219: 222-224.
  
• Herwig J, Scholl-Schilling G, Böhles H. Glycaemic control and hypoglycaemia in children, adolescents and young adults with unstable type 1 diabetes mellitus treated with insulin glargine or intermediate-acting insulin. J Pediatr Endocrinol Metab (2007) 20: 517-525.
  
• Kitz R, Rose MA, Schönborn H, Zielen S, Böhles HJ. Impact of early dietary gamma-linolenic acid supplementation on atopic eczema in infancy. Pediatr Allergy Immunol (2006) 17: 112-117.
  
• Arndt T, Gressner A, Herwig J, Meier U, Sewell AC. Argininosuccinate lyase deficiency (ASL) and carbohydrate-deficient transferrin (CDT): experience with four independent CDT analysis methods--misleading results given by the %CDT TIA assay. CLIN CHIM ACTA (2006) 373: 117-120
  
• Gebhardt B, Dittrich S, Parbel S, Vlaho S, Matsika O, Bohles H. N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia. J Inherit Metab Dis (2005) 28: 241-244.