Abstract:
:Presentation of cystic fibrosis (CF) with an acrodermatitis enteropathica-like skin rash, anemia, and hypoproteinemia without pulmonary disease is rarely reported before. We describe an 11-month-old boy with rash and edema as the presenting signs of cystic fibrosis. The interesting additional finding in our patient was the graying hair after 3 months of age. A reversal of the gray hair was observed by pancreatic enzyme replacement therapy. In conclusion, acrodermatitis-like eruption and hypoproteinemia can be a presenting sign of CF. Graying hair has not been noticed so far as a sign of CF in these patients.
journal_name
Eur J Pediatrjournal_title
European journal of pediatricsauthors
Dalgic B,Egritas Odoi
10.1007/s00431-011-1447-0subject
Has Abstractpub_date
2011-10-01 00:00:00pages
1305-8issue
10eissn
0340-6199issn
1432-1076journal_volume
170pub_type
杂志文章abstract::The fatty acid status of an unselected group of 84 Congolese children was determined by estimating the fatty acid composition of the plasma phospholipid (PL) and cholesterolester (CE) fractions using capillary gas chromatography. In comparison with North American and European children a wide range of values and low me...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00445928
更新日期:1988-11-01 00:00:00
abstract::Child maltreatment is a major social problem with many adverse consequences, and a substantial number of maltreated children are not identified by health care professionals. In 2010, in order to improve the identification of maltreated children in hospitals, a new hospital-based policy was developed in Amsterdam, The ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1869-3
更新日期:2013-02-01 00:00:00
abstract::Vitamin D is important for calcium homeostasis, muscle, and bone health. It has also immunomodulatory capacities in vivo and in vitro. Regulatory T cells (Treg) have been found to suppress a number of T cell-mediated immune disorders, including allergic responses and autoimmune diseases. This study aimed to investigat...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1688-6
更新日期:2012-08-01 00:00:00
abstract::A 7-year-old girl was admitted with a severe abdominal pain. Abdominal ultrasound and CT revealed a large splenic infarction, leading to the diagnosis of chronic myeloid leukemia. ...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-012-1675-y
更新日期:2012-07-01 00:00:00
abstract::We present a case of Pott's disease, where the patient presented with neurological impairment due to vertebral granulomatous necrosis, needing immediate decompression and later stabilizing and reconstructive orthopaedic surgery, in order to create awareness for TB in general, especially this forgotten form of spinal t...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-006-0347-1
更新日期:2007-10-01 00:00:00
abstract:UNLABELLED:A prospective study was undertaken throughout Wales over the period from 1 January 1995 to 30 March 1997, to determine the incidence of childhood inflammatory bowel disease (IBD). Thirty-eight (24 male) new cases of IBD were reported. Twenty (16 male) of the reported cases had Crohn's disease, 11 (5 male) ha...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050066
更新日期:2000-04-01 00:00:00
abstract::In a prospective study elastase alpha 1-proteinase inhibitor (E alpha 1PI), polymorphonuclear (PMN) count, the immature to total neutrophil count ratio (I/T ratio), and C-reactive protein (CRP) were analysed in 74 patients (76 cases) with neonatal septicaemia at the time of initial clinical symptoms. At that early sta...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02113257
更新日期:1992-05-01 00:00:00
abstract::A developmental approach to the management of sleep disturbances was successful in 85% (44/52) of the children (aged 2-36 months) within 2-6 weeks. No drugs were used. The approach was based on the following two principles: taking into account the characteristics of sleep behavior in infants and young children by sett...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00442443
更新日期:1984-08-01 00:00:00
abstract::The main goal for the neonatologist is to facilitate the adaptation to extra-uterine life during initial transition, while minimizing lung injury opening and protecting the premature lung from the first breath onwards. An appropriate management from birth should lead to the achievement of an early functional residual ...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/s00431-017-2984-y
更新日期:2017-10-01 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:UNLABELLED:The effect of different feeding regimens, notably the use of hydrolysed cow's milk formulas, on the development of allergic reactions and the development of cow's milk protein-IgG antibodies is still disputed. We prospectively compared the development of allergic manifestations and cow's milk protein-IgG ant...
journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1007/BF02002723
更新日期:1996-04-01 00:00:00
abstract::The Kabuki (Niikawa-Kuroki) syndrome was reported in 1981 by Niikawa et al. and Kuroki et al. in a total of ten unrelated Japanese children with a characteristic array of multiple congenital anomalies and mental retardation. The syndrome is characterized by a distinct face, mild to moderate mental retardation, postnat...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01983409
更新日期:1994-06-01 00:00:00
abstract:UNLABELLED:Concentrations of galactose (Gal) in plasma and galactose metabolites in red blood cells (RBC) were determined in 18 normal neonates and 249 others with hypergalactosaemia according to the Paigen method. Normal neonatal values for plasma Gal, RBC galactose-1-phosphate (Gal-1-P), RBC uridine diphosphate gluco...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/pl00008352
更新日期:2000-11-01 00:00:00
abstract:UNLABELLED:Convincing evidence of moderate hyperhomocysteinaemia as a risk factor for vascular disease has accumulated within the last decade being independent of conventional risk factors and equally strong as hypercholesterolaemia and smoking. A synergistic interaction with hypertension and smoking in their joint art...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/pl00014292
更新日期:1998-04-01 00:00:00
abstract::Soon after birth a 36-week-gestational age, appropriate for dates, newborn infant developed hyperinsulinaemic hypoglycaemia which responded to diazoxide and glucose infusion. While normoglycaemic, he became increasingly ill with high lactate, pyruvate, and alanine levels. Provocation tests suggested a defect in lactat...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00496802
更新日期:1983-12-01 00:00:00
abstract::The clinical presentation of combined pituitary hormone deficiency (CPHD) is variable. Some patients present with hypoglycemia during the neonatal period or during the first few years of life. Others present later in childhood with growth failure. We report on 7 patients with very late diagnosed severe hypopituitarism...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-019-03489-3
更新日期:2020-01-01 00:00:00
abstract:UNLABELLED:The objective of this open study was to monitor the long-term effectiveness of the Lederle-Takeda diphtheria and tetanus toxoids and acellular pertussis antigen(s) (DTaP) vaccine and the Wyeth-Lederle diphtheria and tetanus toxoids and pertussis whole cell (DTP) vaccine in children who had received four dose...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-001-0893-5
更新日期:2002-03-01 00:00:00
abstract:UNLABELLED:Lemierre syndrome used to be a complication of severe oropharyngeal infection with regional thrombophlebitis, septicaemia and septic metastatic infections caused by Fusobacterium necrophorum in the pre-antibiotic era. A case of septic arthritis of the hip caused by F. necrophorum as a complication of tonsill...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s004310050730
更新日期:1997-11-01 00:00:00
abstract::A 4.5-year-old male patient is described with chorioretinopathy, minor facial anomalies, delayed closure of the fontanel, mental retardation, moderate hypotonia, epilepsy and hepatic fibrosis. Postural control, intentional vocalising and manual dexterity were superior to the performance of patients with classical Zell...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF00441774
更新日期:1985-11-01 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:UNLABELLED:Acid reflux and/or oesophagitis may be responsible for inconsolable crying in infants. We evaluated prospectively the presence of acid reflux disease, oesophagitis and the accuracy of pH monitoring in the prediction of oesophagitis in a population of irritable infants. A 24-h oesophageal pH monitoring with a...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-004-1403-3
更新日期:2004-06-01 00:00:00
abstract::Central line-associated bloodstream infection (CLABSI) is a significant cause of morbidity and mortality in neonatal intensive care units (NICUs). A "bundle" is defined as a combination of evidence-based interventions that provided they are followed collectively and reliably, are proven to improve patient outcomes. Th...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-020-03844-9
更新日期:2021-02-01 00:00:00
abstract::Osteoporosis-pseudoglioma syndrome (OPPG) is a rare autosomal-recessive disorder, characterized by severe osteoporosis and early-onset blindness. Loss of function mutations in the gene encoding low-density lipoprotein receptor-related protein 5 (LRP5) have been established as the genetic defect of the disease. We repo...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-018-3299-3
更新日期:2019-03-01 00:00:00
abstract::Prolonged emergency department (ED) length of stay (LOS) is used as a proxy for ED overcrowding and is associated with adverse outcomes of patients requiring therapy and reduced patient satisfaction. Our aim was to identify and quantify variables which affect ED-LOS. Patients admitted to the pediatric ED of a large re...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-017-2879-y
更新日期:2017-05-01 00:00:00
abstract::Cerebral blood flow velocities (CBFV) were measured by the pulsed Doppler method in 41 infants of smoking mothers and in 59 apparently healthy control infants. Although gestational age, birth weight, and systolic blood pressure were lower in infants exposed to tobacco smoke prenatally, systolic (65 +/- 11 vs. 47 +/- 1...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF01956151
更新日期:1993-03-01 00:00:00
abstract::The glycogen storage disorders (GSD)-I, -III, -VI and -VIII are associated with hypertriglyceridaemia or mixed hyperlipidaemia which poses the question whether these patients have an increased risk for atherosclerosis. The atherogenicity of triglycerides has remained controversial, while increased plasma cholesterol l...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02072094
更新日期:1993-01-01 00:00:00
abstract::Biochemical markers such as plasma and urinary metabolite concentrations and in vitro enzyme activity are of limited prognostic value in the most common disorders of propionate metabolism, methylmalonic acidaemia (MMA) and propionic acidaemia (PA). In vivo propionate oxidation was compared with conventional prognostic...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/BF02009661
更新日期:1990-03-01 00:00:00
abstract::Vitamin K prophylaxis in infancy aims to prevent life-threatening vitamin K deficiency bleeding (VKDB). The Dutch prophylactic oral daily regimen was increased sixfold from 25 to 150 μg because of a high failure rate. To evaluate the efficacy of this new regimen, incidences of intracranial VKDB under both regimens wer...
journal_title:European journal of pediatrics
pub_type: 杂志文章
doi:10.1007/s00431-019-03391-y
更新日期:2019-07-01 00:00:00
abstract::Two matched groups of term newborn infants with idiopathic jaundice were subjected to intensive double direction green or blue light phototherapy (PT). The efficacy of treatment was expressed as rate of decline of serum bilirubin concentration after 6, 12 and 24 h of light exposure. More rapid response was obtained us...
journal_title:European journal of pediatrics
pub_type: 临床试验,杂志文章
doi:10.1007/BF01955530
更新日期:1991-02-01 00:00:00
abstract::Several methods have been used for lung function testing in the ventilated newborn. The interest in the field has been stimulated by the recent appearance of commercially available equipment for assessment of mechanical parameters and of functional residual capacity in this group. Nevertheless, lung function testing i...
journal_title:European journal of pediatrics
pub_type: 杂志文章,评审
doi:10.1007/BF02179669
更新日期:1994-01-01 00:00:00