Defective B-cell and regulatory T-cell function in Wiskott-Aldrich syndrome.

abstract：:Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by tender, red inflammatory nodules or papules that occur in association with infection, malignancy, connective tissue disease, or following exposure to certain drugs. Although drug-induced Sweet syndrome is rare, granulocyte c...

journal_title：European journal of pediatrics

authors： Kim MJ,Choe YH

更新日期：2010-12-01 00:00:00

abstract：UNLABELLED:A newborn boy was referred to our hospital because of hemolytic anemia and severe hyperbilirubinemia. Extensive investigations aimed at determining the cause of hemolysis was initiated at the time of admission and 3 months after blood transfusion. Notably, no intrinsic erythrocyte abnormalities could be dete...

journal_title：European journal of pediatrics

authors： Vos MJ,Martens D,van de Leur SJ,van Wijk R

更新日期：2014-12-01 00:00:00

abstract：:Two unique associations of disease with Escherichia coli O157:H7 are presented. These cases broaden the spectrum of disease associated with this organism which currently includes bloody and non-bloody diarrhoea, haemolytic-uraemic syndrome, and thrombotic thrombocytopenic purpura. ...

journal_title：European journal of pediatrics

authors： Cimolai N,Anderson JD,Bhanji NM,Chen L,Blair GK

更新日期：1990-01-01 00:00:00

abstract：:Nineteen (12 male, 7 female) children, who have received craniospinal irradiation for the treatment of a brain tumour distant from the hypothalamic-pituitary axis, resulting in growth hormone (GH) deficiency (CS-PRGHD), have been treated with GH. Eight have completed growth. Comparison has been made with the growth of...

journal_title：European journal of pediatrics

authors： Clayton PE,Shalet SM,Price DA

更新日期：1988-08-01 00:00:00

abstract：UNLABELLED:We studied the clinical and biochemical factors associated with surfactant dysfunction and factors affecting the responsiveness to exogenous surfactant among 27 neonates with haemorrhagic pulmonary oedema (HPE). HPE was defined as the presence of a large amount of blood-stained lung effluent and respiratory ...

journal_title：European journal of pediatrics

authors： Amizuka T,Shimizu H,Niida Y,Ogawa Y

更新日期：2003-10-01 00:00:00

abstract：:Factor VIII procoagulant activity and factor VIII related antigen were examined in 20 full-term and preterm newborn infants during the first days of life. The control group involved 15 adults volunteers. Factor VIII activity was estimated by a one-stage test and factor VIII related antigen was determined by immunelect...

journal_title：European journal of pediatrics

authors： Maak B,Scheidt B,Frenzel J

更新日期：1978-07-19 00:00:00

abstract：:A 10-year-old boy presented with priapism of 10 h duration which after unsuccessful conservative measures, was relieved by a saphenocorporeal shunt. A 4-year history of intermittent vague aching of fingers and toes accompanied by low-grade fever was reported. Fabry disease was confirmed by the lack alpha-galactosidase...

journal_title：European journal of pediatrics

authors： García-Consuegra J,Padrón M,Jaureguizar E,Carrascosa C,Ramos J

更新日期：1990-04-01 00:00:00

abstract：:Retrospective analysis was performed at an affiliated university children's hospital with consecutive patients receiving a venoarterial extracorporeal membrane oxygenation (VA-ECMO) for refractory cardiogenic shock from July 2007 to May 2018. Fifty-six patients underwent VA-ECMO for refractory cardiogenic shock with t...

journal_title：European journal of pediatrics

authors： Yang L,Ye L,Fan Y,He W,Zong Q,Zhao W,Lin R

更新日期：2019-06-01 00:00:00

abstract：:In 238 boys with cryptorchidism, between the ages of 4 and 48 months, luteinizing hormone releasing hormone (LHRH) was administered as nasal spray 1.2 mg/day for 4 weeks. The nonresponders received human chorionic gonadotropin (HCG) 500 I.U. i.m. three times a week for 3 weeks. With the combined treatment 37.8% of tes...

journal_title：European journal of pediatrics

authors： Lala R,Matarazzo P,Chiabotto P,de Sanctis C,Canavese F,Hadziselimovic F

更新日期：1993-01-01 00:00:00

abstract：UNLABELLED:The aim was to assess the incidence of complications related to skin-to-skin contact (SSC) in newborns with an umbilical venous catheter (UVC). We carried out a prospective follow-up study of all UVCs in a level 3 unit where SSC is systematic. A total of 333 babies were included (mean gestational age of 31.3...

journal_title：European journal of pediatrics

authors： Catherine ZG,Béatrice P,Fabrice L,Claire H,Alain D

更新日期：2016-04-01 00:00:00

abstract：:From time to time, paediatricians are confronted with children who might suffer from a primary immunodeficiency disease. For practical purposes, these children can be divided into four main clinical categories: (1) a relatively large group of children with recurrent ear-nose and throat and lower respiratory tract infe...

journal_title：European journal of pediatrics

authors： de Vries E,Noordzij JG,Kuijpers TW,van Dongen JJ

更新日期：2001-10-01 00:00:00

abstract：:In this study, we compare the prevalence of alcohol and cigarette use among Polish adolescents with type 1 diabetes mellitus (T1DM) (n = 209), aged 15-18 years, with that of a large cohort of their healthy peers, using standardized questionnaire used in the European School Survey Project on Alcohol and Drugs (ESPAD). ...

journal_title：European journal of pediatrics

authors： Hogendorf AM,Fendler W,Sierosławski J,Bobeff K,Węgrewicz K,Malewska KI,Przudzik MW,Szmigiero-Kawko M,Sztangierska B,Myśliwiec M,Szadkowska A,Młynarski WM

更新日期：2017-06-01 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

authors： Keller KM,Bürgin-Wolff A,Lippold R,Wirth S,Lentze MJ

更新日期：1996-04-01 00:00:00

abstract：:A patient with a cerebro-hepato-renal syndrome was investigated. The visceral manifestations were those of the Zellweger syndrome (ZS); however, the child exhibited muscular hypertonia and survived into the 2nd year of life. Ultramicroscopically, hepatocytes were lacking peroxisomes, but, contrary to findings in one p...

journal_title：European journal of pediatrics

authors： Versmold HT,Bremer HJ,Herzog V,Siegel G,Bassewitz DB,Irle U,Voss H,Lombeck I,Brauser B

更新日期：1977-03-18 00:00:00

abstract：UNLABELLED:Implementation of guidelines for group B streptococcal (GBS) prepartum screening (PS) rarely has been prospectively evaluated. To assess PS at 35-37 weeks of gestation and compare its predictive value to that of an intrapartum screening (IS) within 7 days of delivery, a surveillance cohort study was conducte...

journal_title：European journal of pediatrics

authors： Kunze M,Zumstein K,Markfeld-Erol F,Elling R,Lander F,Prömpeler H,Berner R,Hufnagel M

更新日期：2015-06-01 00:00:00

abstract：UNLABELLED:A postal survey of 31 European centres was conducted to document current practices regarding screening and management of hepatitis C virus (HCV)-infected pregnant women and their children. Antenatal HCV prevalence was low. Universal antenatal screening programmes were in place in ten centres, selective scree...

journal_title：European journal of pediatrics

authors： Pembrey L,Newell ML,Tovo PA

更新日期：1999-10-01 00:00:00

abstract：:Two siblings were found to be affected by long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, one of which died suddenly and unexpectedly on the 3rd day of life suffering from extreme hypoketotic hypoglycaemia. The younger sibling started to have feeding problems, lowered consciousness, and liver dysfunction at the...

journal_title：European journal of pediatrics

authors： Duran M,Wanders RJ,de Jager JP,Dorland L,Bruinvis L,Ketting D,Ijlst L,van Sprang FJ

更新日期：1991-01-01 00:00:00

abstract：:In this study messenger ribonucleic acid (mRNA) and DNA of five Wilms tumours were investigated. As expected, the level of insulin-like growth factor (IGF) II-mRNA was elevated up to 50 times in tumour tissue as compared to normal adjacent kidney tissue. In addition, genomic DNA was isolated and digested with appropri...

journal_title：European journal of pediatrics

authors： Irminger JC,Schoenle EJ,Briner J,Humbel RE

更新日期：1989-06-01 00:00:00

abstract：:A 2-month-old infant was admitted to hospital because of recurrent vomiting for 1 week, progressive apathy, and focal seizures. The cranial MRI showed a noticeable result. ...

journal_title：European journal of pediatrics

authors： Larsen A,Martin C,Meyer S,Rohrer T,Papanagiotou P,van der Knaap M,Gortner L

更新日期：2012-06-01 00:00:00

abstract：:We report on a 10-year-old girl who was presented to the Emergency Department with a 3-day history of "vaginal bleeding", the cause of which was determined to be urethral prolapse. Urethral prolapse occurs when the urethra mucosa evaginates beyond the urethral meatus. This uncommon condition in prepubescent girls shou...

journal_title：European journal of pediatrics

authors： Shavit I,Solt I

更新日期：2008-05-01 00:00:00

abstract：:To nine cystic fibrosis patients with chronic bronchopulmonary infection of severely damaged lungs invaded by Pseudomonas aeruginosa, eleven courses of prolonged tobramycin treatment (5 mg/kg/day) for four to 16 weeks were administered. Pulmonary symptoms improved and a better quality of life was achieved in all but o...

journal_title：European journal of pediatrics

authors： Paporisz U,Posselt HG,Wönne R,Ristow W,Röser D,Knothe H,Bender SW

更新日期：1979-04-03 00:00:00

abstract：:A male patient with fucosidosis exhibited the following characteristics: 1. Early onset and rapid progression of neurological symptoms. 2.Skin changes compatible with angiokeratoma corporis diffusum. 3. Complete or nearly complete deficiency of alpha-fucosidase. 4. Survival to adult age (20 years). The deficiency of a...

journal_title：European journal of pediatrics

authors： Søvik O,Lie SO,Fluge G,Van Hoof F

更新日期：1980-12-01 00:00:00

abstract：:Acid-base disturbances have been usually evaluated with the traditional Henderson-Hasselbach method and Stewart's physiochemical approach by quantifying anions of tissue acids (TA). It is hypothesized that an increase in tissue acids during metabolic acidosis would cause a compensatory decrease in the plasma chloride ...

journal_title：European journal of pediatrics

authors： Kurt A,Ecevit A,Ozkiraz S,Ince DA,Akcan AB,Tarcan A

更新日期：2012-06-01 00:00:00

abstract：:Fourteen children aged 9 months-4 years with moderate to severe mental retardation and varying neurologic lesions were referred for severe and continuous nocturnal sleep disturbances and very abnormal day/night schedules. All children had previously been given hypnotic medications and behavioral treatments which had l...

journal_title：European journal of pediatrics

authors： Guilleminault C,McCann CC,Quera-Salva M,Cetel M

更新日期：1993-09-01 00:00:00

abstract：:Changes of the complement components in the sera of 13 children with treated coeliac disease were studied after gluten challenge. The levels of C 1 and C3-activator (factor B) were significantly decreased at 4 h after the challenge, as were the levels of total complement (CH 50) and the components C 1, C 4 and C 1-ina...

journal_title：European journal of pediatrics

authors： Bosch C,Becker M,Rotthauwe HW,Opferkuch W

更新日期：1980-08-01 00:00:00

abstract：:As simple and nonsurgical means of differentiating biliary atresia (BA) from intrahepatic cholestasis of unknown origin (IC), liver function tests including serum lipoprotein-X (LP-X) and gamma-glutamyltranspeptidase (GGTP) were done and evaluated for their usefulness in the diagnosis of 27 cholestatic Japanese young ...

journal_title：European journal of pediatrics

authors： Tazawa Y,Yamada M,Nakagawa M,Tada K,Konno T,Ohi R,Kasai M

更新日期：1986-04-01 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

authors： Boots I,Sukhai RN,Klein RH,Holl RA,Wit JM,Cohen AF,Burggraaf J

更新日期：2007-08-01 00:00:00

abstract：UNLABELLED:Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked recessive disorder in which haemolytic anaemia is the major symptom. The Beutler spot test employed in mass-screening for galactosaemia in newborns requires several intrinsic erythrocyte enzymes such as G6PD for its reaction and can theoretic...

journal_title：European journal of pediatrics

authors： Okano Y,Fujimoto A,Miyagi T,Hirono A,Miwa S,Niihira S,Hirokawa H,Yamano Y

更新日期：2001-02-01 00:00:00

abstract：:We report the case of 5-week-old male infant who presented as a 'near miss cot death'. He had the immunodeficient syndrome of defective neutrophil mobility and delayed umbilical cord separation. He was shown to have staphylococcal endocarditis with a large vegetation on the mitral valve, and acute obstruction of the m...

journal_title：European journal of pediatrics

authors： Walters MD,Deanfield JE,Robinson PJ,Matthew DJ

更新日期：1986-12-01 00:00:00

abstract：UNLABELLED:We correlated arachidonic acid (AA) and docosahexaenoic acid (DHA) status with anthropometric measures and growth rates in a group of low birth weight infants (< or = 2500 g; gestational ages 30-41 weeks; n = 143). AA and DHA status were measured in erythrocytes (RBC) and plasma cholesterol esters (CE) durin...

journal_title：European journal of pediatrics

authors： Woltil HA,van Beusekom CM,Schaafsma A,Muskiet FA,Okken A

更新日期：1998-02-01 00:00:00