Abstract:
:To elucidate the basis for the recurrent infections in patients with glycogen storage disease (GSD) Ib we tested polymorphonuclear leukocyte (PMN) function in one patient. Bactericidal capacity and phagocytosis-induced O2 consumption were reduced. Also, phorbol myristate acetate-stimulated superoxide production and glucose oxidation through the hexose monophosphate shunt were diminished compared to control subjects. Therefore it could be speculated that in PMN of patients with GSD Ib, glucose-6-phosphate has no access to the enzymes of the hexose monophosphate shunt due to a transport-related defect as shown for glucogenesis in hepatocytes.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Gahr M,Heyne Kdoi
10.1007/BF00442674subject
Has Abstractpub_date
1983-09-01 00:00:00pages
329-30issue
4eissn
0340-6199issn
1432-1076journal_volume
140pub_type
杂志文章abstract::To determine the relationship between total serum bilirubin (TSB) during the first 2 days of life and subsequent neonatal morbidity in very low birth weight (VLBW, less than 1500 g) infants. We performed a prospective study of 582 VLBW infants born between July 1, 2005 and December 31, 2009. TSB was measured in umbili...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-011-1634-z
更新日期:2012-04-01 00:00:00
abstract::An adolescent girl with severe thrombotic thrombocytopenic purpura (TTP) remained in a critical condition after 3 weeks of combined treatment with antiplatelet drugs, plasma infusions and plasma exchange. The introduction of vincristine resulted in gradual improvement and eventual complete remission which lasted for 2...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01957008
更新日期:1994-07-01 00:00:00
abstract::A 17-year-old patient suffering from Crohn disease (CD) and liver cirrhosis is presented. At an advanced stage of the disease, he died of a concomitant urosepsis. Autopsy showed that the liver cirrhosis was caused by sclerosing cholangitis. This very rare complication of CD in adolescence is discussed. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02072206
更新日期:1991-06-01 00:00:00
abstract::An 8-month-old boy and a 7-month-old girl presented with an acute, Coombs-positive auto-immune haemolytic anaemia and severe hepatitis. The clinical manifestations were pallor, jaundice and hepatomegaly. The liver histology revealed diffuse giant cell transformation and extensive necrosis with central-portal bridging....
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01955929
更新日期:1991-03-01 00:00:00
abstract::The tendency of non-operative management of appendicitis let us explore the natural history of appendiceal carcinoids, compare them with appendicitis patients, and determine the possibility of deciding the extent of the surgery and post-operative follow-up on behalf of the intraoperative findings. A retrospective revi...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-3258-z
更新日期:2018-12-01 00:00:00
abstract::In order to determine the hepatotoxicity of rifampicin in children treated for tuberculosis, a survey was performed of 18 children receiving this medicine in combination with isoniazid. Fifteen of the 18 children (83%) showed a rise in ASAT values and 11 (61%) in ALAT values exceeding 29U/L. Seven children with maxima...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441477
更新日期:1980-09-01 00:00:00
abstract::An infant girl three weeks of age with the leading symptom of skin haemorrhages is presented. On further investigation, the signs of severe hepatic damage with hypofibrinogenaemia and prothrombin complex impairment, and renal tubular dysfunction were disclosed. All these pathological symptoms, which were reversed on f...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00465567
更新日期:1977-12-30 00:00:00
abstract::This study was conducted to assess the clinical spectrum, management, and outcome of SARS-CoV-2-related multisystem inflammatory syndrome in children (MIS-C). We reviewed medical records of children with MIS-C diagnosis seen at the Children's Hospital of Michigan in Detroit between April and June 2020. Thirty-three ch...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-021-03935-1
更新日期:2021-01-16 00:00:00
abstract::We present clinical and biochemical data on a further patient with hyperargininaemia and the results of neurophysiological tests both before and during dietary treatment with an essential amino acid mixture. With normalisation of plasma arginine concentrations, neurological functions improved and brain stem auditory e...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02171562
更新日期:1990-02-01 00:00:00
abstract::It is known that the 5%-10% of adults infected with hepatitis B virus (HBV) develop a chronic infection and that HBV infection acquired at birth by an hepatitis B surface antigen (HBsAg)/hepatitis B "e" antigen (HBeAg)-positive mother almost invariably leads to chronic infection. Little information is, however, availa...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01954381
更新日期:1992-03-01 00:00:00
abstract::Two patients developed clinical features of intracranial bleeding--which were confirmed by computerized axial tomograms--during their induction therapy for acute lymphocytic leukemia. Coagulation studies showed clotting abnormalities including severe hypofibrinogenemia. These findings most probably were related to the...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00443266
更新日期:1981-11-01 00:00:00
abstract:UNLABELLED:Smith-Lemli-Opitz syndrome (SLOS), a multiple congenital anomaly with severe mental retardation, is caused by decreased activity of 7-dehydrocholesterol reductase. Fifteen Hungarian patients were diagnosed with SLOS on the basis of clinical symptoms, serum cholesterol, 7-dehydrocholesterol, and molecular gen...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1925-z
更新日期:2013-05-01 00:00:00
abstract:UNLABELLED:Growth retardation is a consistent finding in Williams-Beuren syndrome. The cause of short stature in this syndrome is unknown. Endocrine studies have failed to reveal abnormalities in the growth hormone-insulin-like growth factor I axis. We report a boy with confirmed Williams-Beuren syndrome, who was found...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051118
更新日期:1999-06-01 00:00:00
abstract:UNLABELLED:Intensive multimodality treatment has led to a remarkable improvement of prognosis in paediatric cancer patients, however, a great number of long-term survivors suffer from considerable tumour- or treatment-related late effects. Between January 1990 and December 1998, 223 consecutive survivors of childhood m...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/pl00008340
更新日期:2000-10-01 00:00:00
abstract::To compare the efficacy of low dose fentanyl infusion and 24% oral sucrose in providing optimal pain relief during laser for retinopathy of prematurity (ROP), we enrolled fifty-eight spontaneously breathing preterm infants undergoing laser. The preterm infants were randomized to either fentanyl infusion (1 mcg/kg/hr) ...
journal_title:European journal of pediatrics
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00431-019-03514-5
更新日期:2020-02-01 00:00:00
abstract:UNLABELLED:Recent evidence has demonstrated the prenatal initiation of childhood obesity as epidemiological studies and animal studies have illustrated the effect of the intrauterine milieu for subsequent development of childhood obesity. This study investigates the relationship between severe childhood obesity and the...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-013-1949-z
更新日期:2013-06-01 00:00:00
abstract::This study aimed to estimate the number of infants who died of unrecognized congenital adrenal hyperplasia (CAH) in Austria and the Czech Republic within the past 13 years, before the introduction of adequate neonatal screening. The study was based on retrospective analysis of neonatal screening cards of 242 infants w...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0154-8
更新日期:2007-01-01 00:00:00
abstract::A girl, 13 months of age, presented with generalised granulomatous skin, hair and mucosal candidiasis. Her lymphocytes failed to respond in vitro to Candida antigen (CA); the intradermal test with CA was also negative. Serum immunoglobulins, complement components, granulocyte functions (phagocytic and fungicidal), T-c...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442747
更新日期:1984-11-01 00:00:00
abstract:UNLABELLED:Scorpion envenomation is a health problem in children in tropical and subtropical regions. The aim of this study was to evaluate demographic and clinical characteristics as well as outcomes in referred children to Assiut University Children Hospital during the year 2012 with a history of scorpion sting. The ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-013-2244-8
更新日期:2014-06-01 00:00:00
abstract::Most drugs that are currently prescribed in pediatrics have not been tested in children. Pediatric drug studies are stimulated in the USA by the pediatric exclusivity provision under the Food and Drug Administration Modernization Act (FDAMA) that grants patent extensions when pediatric labeling is provided. We investi...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0381-z
更新日期:2007-08-01 00:00:00
abstract::Iodine deficiency is well documented in Belgium in adults including pregnant women, adolescents, schoolchildren, and neonates, but no data are available in the age group 6 months-3 years. We investigated the status of iodine nutrition in 111 healthy subjects in this age group in an attempt to evaluate the risk of brai...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310000707
更新日期:2001-04-01 00:00:00
abstract::An Arab Moslem kindred is reported in which six abnormal male fetuses were born in three closely related sibships. They had amelia, malformed head and other severe skeletal and visceral malformations. The similarities and differences between the developmental and inherent characteristics found in our patients and in f...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441792
更新日期:1985-11-01 00:00:00
abstract::A series of information processing tasks was administered to 22 PKU children aged 8.5 years who had been under dietary treatment since birth as well as to 20 controls of the same age. This contribution presents the results of two tasks from this series: a continuous performance task and a calculation task. The continu...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02126298
更新日期:1990-01-01 00:00:00
abstract:UNLABELLED:Reports on schistosomiasis in children growing up in Europe are rare despite increased travel activity. We report on eight male and three female German children aged 50 months to 15 years with schistosomiasis. Six children were asymptomatic, whereas two presented with typical signs of Katayama fever. Persist...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310051326
更新日期:2000-07-01 00:00:00
abstract::A desquamative, interstitial pneumonitis was diagnosed histologically in a 9-month-old boy who first became ill at the age of 5 weeks. The desquamative interstitial pneumonitis was associated with an acquired cytomegalovirus (CMV) infection. Despite treatment with corticoids, acyclovir and artificial ventilation, the ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02429072
更新日期:1986-12-01 00:00:00
abstract::Children with symptomatic hypoglycemia and asymptomatic hypoglycemia-prone children were shown to differ in the degree of ketosis after a 20 h fast. In the latter children the close negative correlation between ketone body levels and glucose levels yielded a regression line against which the former children's data cou...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442403
更新日期:1980-06-01 00:00:00
abstract::A combination of humoral immunodeficiency and isolated growth hormone deficiency was observed in a girl with Mulibrey nanism. The humoral immunodeficiency consisted of subnormal concentration of serum IgG, in particular IgG2 and IgG4, and low concentration of serum IgM. Serum IgA and IgD were elevated, IgE was absent....
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01955061
更新日期:1993-06-01 00:00:00
abstract::We report a case of an infected subgaleal hematoma caused by an unusual micro-organism in a previously healthy 11-month-old girl. Our patient presented at the emergency department with an increasing scalp swelling for 2 weeks, and culture of the evacuated fluid yielded Streptococcus pneumoniae. Although she was born a...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-009-0922-3
更新日期:2009-06-01 00:00:00
abstract::This paper describes the design of a study to follow the development of boys and girls in secondary schools from the age of 12 through 17 on an annual basis, in order to acquire more information concerning the growth and development of teenagers. In this study, both physical and psychological characteristics are measu...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442158
更新日期:1978-10-12 00:00:00
abstract::A pure growth of Branhamella catarrhalis was obtained from a purulent bronchial exudate in a 28-month-old Rwandese girl, hospitalized for acute inspiratory dyspnoea with fever. The outcome was favourable under treatment with ampicillin, although the isolate was shown to produce a beta-lactamase in vitro. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442307
更新日期:1985-03-01 00:00:00