[Hereditary fructose intolerance (HFI) as cause of isolated gamma GT rise in a 5-year old boy with hepatomegaly].

abstract：:A case of true hermaphroditism was described. The patient had an uterus, a fallopian tube, a testide on the left in the position of the ovary and an ovary on the right side. The chromosome picture corresponded to a 47, XXY/46, XX/46 XXY mosaic. The phenotype of the patient was male so the decision for male sex seemed ...

journal_title：Klinische Padiatrie

authors： Barta L,Konyar E,Regöly-Merei A,Toth J

更新日期：1976-09-01 00:00:00

abstract：:This is a report of the neonatal form of nonketotic hyperglycinemia with rapid progressing neurological symptoms, respiratory distress and seizures. The EEG pattern is characteristic, the ratio of cerebrospinal fluid to plasma glycine abnormally high. The result of cranial computerized tomography demonstrates also in ...

journal_title：Klinische Padiatrie

authors： Plöchl E,Rittinger O,Doringer E,Laubichler W

更新日期：1991-11-01 00:00:00

abstract：:While many aspects related to the etiology and pathogenesis of sudden infant death remain unclear, the ultimate trigger event appears to be an acute regulatory disturbance of the cardiorespiratory neurons of the reticular formation of the brain stem. Retrospective studies have demonstrated that SIDS often occurs after...

journal_title：Klinische Padiatrie

authors： Sterniste W,Bock W

更新日期：1992-03-01 00:00:00

abstract：:Mycobacterial infections are rarely reported in Cystic Fibrosis patients although they quite often develop predisposing risk factors such as underweight, secondary diabetes mellitus and chronic inflammatory pulmonary disease. Furthermore glucocorticoid therapy is mandatory in some patients. CF heterozygotes are said t...

journal_title：Klinische Padiatrie

authors： Friedrichs F,Kusenbach G,Skopnik H

更新日期：1991-09-01 00:00:00

abstract：:The pros and cons of home monitoring especially for premature infants with continuing apneic episodes and/or chronic lung disease are an ongoing discussion. The controversy spans socio-economic requirements, medical indication as well as patient and family needs. Here, the costs of home monitoring and follow-up care o...

journal_title：Klinische Padiatrie

authors： Förster KM,Immler S,Ensslen M,Flemmer A,Schulze A,von Poblotzki M,Genzel-Boroviczény O,Heinen F,Hilgendorff A

更新日期：2015-03-01 00:00:00

abstract：BACKGROUND:Lupus vulgaris (LV) is the most common form of cutaneous tuberculosis (TB) in Europe, nevertheless the overall incidence is low. It constitutes about 1.5% of all extra-pulmonary cases worldwide. A slight raise in TB incidence rates among children was recently registered in Germany, which can be explained by ...

journal_title：Klinische Padiatrie

authors： Striegel AK,Nazzal R,Fabri M,Rietschel E,Dötsch J,Mauch C,Tantcheva-Poor I

更新日期：2014-01-01 00:00:00

abstract：:Neurofibromatosis type 1 is the most common of the phakomatoses and the clinical follow-up is an interdisciplinary challenge. The data of 27 patients with NF1 were systematically reviewed and compared to data from the literature. All of our patients had clinical signs of NF1. Besides the classic criteria café-au-lait ...

journal_title：Klinische Padiatrie

authors： Syrbe S,Eberle K,Strenge S,Bernhard MK,Herbertz S,Bierbach U,Hirsch W,Froster UG,Kiess W,Merkenschlager A

更新日期：2007-11-01 00:00:00

abstract：:Newly diagnosed immune thrombocytopenia occurs in 3-5/100 000 children < 14 y per year. Bleeding symptoms do not correlate with platelet count. Diagnostic approach includes history, clinical examination and analysis of blood count with blood smear by experienced hematologist. Additional investigations are only necessa...

journal_title：Klinische Padiatrie

authors： Eberl W,Dickerhoff R,Pediatric Committee of Society of Thrombosis and Hemostasis Research.

更新日期：2012-04-01 00:00:00

abstract：:A report was made of three patients who exhibited pronounced heptosplenomegaly at the time of hospital admission: a six-year-old girl and two brothers, 1 year 4 months and 4 years 4 months of age. As is typical for Niemann-Pick disease, foam cells were found in the bone marrow and the liver biopsy specimens for the th...

journal_title：Klinische Padiatrie

authors： Priscu R,Pancu L,Balan A,Petrescu TC,Iacob C

更新日期：1977-11-01 00:00:00

abstract：OBJECTIVE:To investigate the change of psychological symptoms and quality of life in children and adolescents with congenital heart disease, cancer, or cystic fibrosis after a family-oriented inpatient rehabilitation program. METHODS:302 participants in the intervention (aged 4-17 years, mean=8.7) were assessed using ...

journal_title：Klinische Padiatrie

authors： Goldbeck L,Hölling I,Schlack R,West C,Besier T

更新日期：2011-03-01 00:00:00

abstract：:In 15 patients with cystic fibrosis 18 blood samples were investigated for signs of infection including full white blood count, c-reactive protein (CRP) and tumour-necrosis-factor alpha (TNF). Ten patients were hospitalized for pulmonary exacerbation, one for orthostatic collapse and one for equivalent of meconium ile...

journal_title：Klinische Padiatrie

authors： Heininger U,Böwing B,Forster D,Berthold M

更新日期：1991-05-01 00:00:00

abstract：:Positive anti-DNA-antibodies and lowered C3 and C4 levels were found in serum of a 13 year old girl presenting with edema, microscopic hematuria and proteinuria. Renal biopsy revealed diffuse endo- and extracapillary glomerulonephritis with mesangial deposits of IgA, IgG, IgM, C1q, C3 and fibrinogen. In spite of treat...

journal_title：Klinische Padiatrie

authors： Michalk D,Scharf J,Waldherr R

更新日期：1983-11-01 00:00:00

abstract：:Oral administration of zinc sulfate did not improve the clinical conditions in two patients with Ehlers-Danlos-Syndrome type I. Zinc levels in serum and hair were normal before and during treatment. ...

journal_title：Klinische Padiatrie

authors： Lenard HG,Lombeck I

更新日期：1979-11-01 00:00:00

abstract：:In a randomised study the efficacy of a cytomegalic hyperimmune globulin preparation (CMV-HIGP) which had been treated with beta-propiolactone was analysed. The study included 85 patients with acute lymphoblastic leukemia (ALL) and Non-B-Non-Hodgkin-lymphoma (NHL) who were treated initially or underwent a relapse ther...

journal_title：Klinische Padiatrie

authors： Gerein V,Kropp H,Schwabe D,Gussetis E,Langer B,Kornhuber B

更新日期：1989-07-01 00:00:00

abstract：:During the last 6 years 73 previously untreated children and adolescents with acute lymphoblastic leukemia were enrolled on a non-randomized therapy protocol. In this longitudinal study the specific accent was put on the intensified induction treatment of 8 weeks' duration which was thought to achieve a higher remissi...

journal_title：Klinische Padiatrie

authors： Riehm H,Gadner H,Welte K

更新日期：2013-05-01 00:00:00

abstract：UNLABELLED:In the prophylaxis of stress ulcers with antacids in young infants there are no recommendations of dosages that consider the physiologic maturation of gastric acid secretion. During the first six month of life the amount of gastric acid secretion in relation to body weight and body surface area increases exp...

journal_title：Klinische Padiatrie

authors： Kentrup H,Skopnik H,Wolter L,Engelhart W,Nauert C,Heimann G

更新日期：1996-01-01 00:00:00

abstract：:Severe combined immunodeficiency (SCID) is a heterogeneous group of congenital diseases characterized by their presentation with life threatening infections in the first months of life. The clinical presentation and the therapeutic outcome is influenced by multiple factors: the genetic defect, infectious complications...

journal_title：Klinische Padiatrie

authors： Hönig M,Schulz A,Friedrich W

更新日期：2011-11-01 00:00:00

abstract：:Immunological responses are divided into those mediated by humoral antibody (B-Cells) and those by cells (T-Cells). Both depend upon the activity of small lymphocytes which become thymus or bursa analogue dependent during embryonic development. Primary immundeficiencies are therefor defects of lymphocytes attributed i...

journal_title：Klinische Padiatrie

authors： Haas RJ,Niethammer D,Dietrich M,Kleihauer E

更新日期：1975-11-01 00:00:00

abstract：:Objective: Toilet refusal syndrome (TRS) is a common disorder in toddlers, defined by use of diapers and refusal of toilet for defecation, while toilet phobia (TP) is a rare disorder in which toilets are avoided completely. Both disorders have not been described systematically in children, yet. Therefore, the aim was ...

journal_title：Klinische Padiatrie

authors： Wagner C,Niemczyk J,von Gontard A

更新日期：2017-01-01 00:00:00

abstract：:An increasing number of children suffering from cancer has been treated successfully during the last 25 years using therapy protocols of the Gesellschaft für Pädiatrische Onkologie und Hämatologie. The patients had to undergo an interdisciplinary treatment modality and a mostly intensive chemotherapy. Late effects cou...

journal_title：Klinische Padiatrie

authors： Beck JD,Winkler K,Niethammer D,Brandis M,Hertzberg H,von der Hardt K,Greil J,Uberall MA,Rossi R,Lamprecht-Dinnesen A

更新日期：1995-07-01 00:00:00

abstract：:The oscillatory resistance to respiration (Ros) was measured in 218 children and 260 adults using the Siregnost FD5 (Siemens, Erlangen), and compared with whole body plethysmography (Raw) and with spirometry. There was a markedly better correlation between Ros and Raw (r=0.82;n=88) in children than in adults (r=0.53;n...

journal_title：Klinische Padiatrie

authors： Berdel D,Holle JP,Hartmann V,Magnussen H

更新日期：1981-03-01 00:00:00

abstract：:16 term and preterm neonates received intravenous nutriton for at least three days. The dosage was 1--2 g amino acids, 1 g of fat, and 8--10 g glucose per kg body weight and day. Compatibility was investigated by daily determinations of the amino acid pattern in the serum and measurement of free fatty acids and trigly...

journal_title：Klinische Padiatrie

authors： Petrich C,Stephan U,Remy R,von Voss H,Göbel U,Herpertz LM

更新日期：1979-07-01 00:00:00

abstract：:Bacteriologic cultures of the vagina and the cervix of 38 praepubertal children, aged 3 months to 16 years (average age 9 years) from the outpatient department for infants and juveniles of the 1st Department of Obstetrics and Gynecology of the University of Vienna were obtained vaginascopically using anaerobic transpo...

journal_title：Klinische Padiatrie

authors： Gerstner G,Boschitsch E,Grünberger W,Rotter M

更新日期：1982-01-01 00:00:00

abstract：:Standards for supine length/height velocity from birth to 16 years (percentiles, mean and standard deviation) are presented, based on the longitudinal growth studies from Bonn and Dortmund. During the first two years of life growth velocity is twice to three times higher as in the following years. Many things point to...

journal_title：Klinische Padiatrie

authors： Brandt I,Reinken L

更新日期：1988-11-01 00:00:00

abstract：:In approximately 10% of the children suffering from juvenile chronic arthritis (JCA), rheumatic iridocyclitis was also diagnosed. In 90 JCA and iridocyclitis patients we studied relative risk, clinical course and prognosis by means of several clinical and immunological parameters. Antinuclear antibodies (ANA), selecti...

journal_title：Klinische Padiatrie

authors： Michels H,Schuchmann L,Truckenbrodt H,Renz K

更新日期：1982-03-01 00:00:00

abstract：:In a retrospective study we evaluated clinical features and diagnostic impact of sonography, white blood cell count, C-reactive protein as well as bacterial etiology of children treated surgically for bacterial cervical lymphadenitis. We report on 38 children at the age between 2.5 months and 14 years who were admitte...

journal_title：Klinische Padiatrie

authors： Tröbs RB,Gräfe G,Müller P,Handrick W

更新日期：2003-07-01 00:00:00

abstract：:Background: Diabetes mellitus is a common endocrinopathy in patients with thalassemia major, but the occurrence of hemoglobinopathies is rare in Germany and Western Europe. The longitudinal German-Austrian DPV (Diabetes Patienten Verlaufsdokumentation) registry allows a comprehensive characterization of this group of ...

journal_title：Klinische Padiatrie

authors： Warncke K,Konrad K,Kohne E,Hammer E,Ohlenschläger U,Herrlinger S,Jäger A,Holl RW

更新日期：2016-11-01 00:00:00

abstract：:We report the first case of non-immune hydrops fetalis (NIHF) with trisomy 15q11/17q22.5 resulting from a familial translocation 15/17. Furthermore the cytogenetic findings of 50 cases with hydrops fetalis are presented. Of the 30 cytogenetic analyzable cases 7 showed chromosomal abnormalities (4x45,X; 2x47,XY,+21; 1x...

journal_title：Klinische Padiatrie

authors： Schmid G,Haverkamp F,Rechmann J,Schwanitz G,Zerres K,Hansmann M,Kowalewski S

更新日期：1987-07-01 00:00:00

abstract：:Aim of this study was to describe the course of perinatal factors in neonates with meconium aspiration syndrome (MAS) from 1990 to 2010 and to determine risk factors for a severe course of the disease.All neonates with MAS hospitalized in our level III neonatal intensive care unit from 1990 to 2010.Retrospective analy...

journal_title：Klinische Padiatrie

authors： Hofer N,Jank K,Resch E,Urlesberger B,Reiterer F,Resch B

更新日期：2013-12-01 00:00:00

abstract：:Meconium ileus equivalent (MIE) complicating cystic fibrosis of the pancreas (CF) increases in frequently with increasing age of patients. In the present paper the course of 11 children and adolescents with MIE diagnosed and treated at the University Dept. of Paediatrics in Zürich during the last 15 years, i.e. 9% of ...

journal_title：Klinische Padiatrie

authors： Rosenberg E,Shmerling DH

更新日期：1983-09-01 00:00:00