Recombinant human interferon-gamma treatment in severe leucocyte adhesion deficiency.

Abstract:

:We describe a patient with leucocyte adhesion deficiency (LAD). Clinically, the patient had delayed umbilical cord detachment, omphalitis, impaired wound healing and persistent leucocytosis. The patient had the severe form of LAD, with a total absence of leucocyte cell adhesion molecules (LeuCAMs) and undetectable mRNA for the beta chain, the common subunit of the LeuCAMs. In vitro neutrophil chemotaxis, aggregation and oxygen consumption were severely impaired. In vitro incubation of neutrophils with recombinant human interferon-gamma (rIFN-gamma) showed an increase in oxygen consumption, but no effect on the expression of the LeuCAMs, or the beta chain mRNA. In vivo treatment with IFN-gamma was started. The Fc gamma RI receptor appeared on the neutrophils, the LeuCAMs remained undetectable, while the neutrophil functions remained disturbed. The patient died of surgical complications after 10 weeks of rIFN-gamma treatment. No new infections or side-effects due to rIFN-gamma were observed.

journal_name

Eur J Pediatr

authors

Weening RS,Bredius RG,Vomberg PP,van der Schoot CE,Hoogerwerf M,Roos D

doi

10.1007/BF01958952

keywords:

subject

Has Abstract

pub_date

1992-02-01 00:00:00

pages

103-7

issue

2

eissn

0340-6199

issn

1432-1076

journal_volume

151

pub_type

杂志文章
  • Mastoiditis in children: a prospective, observational study comparing clinical presentation, microbiology, computed tomography, surgical findings and histology.

    abstract:UNLABELLED:The aim of this study was to obtain comprehensive data on clinical presentation, microbiology, computed tomography, surgical findings and histology in acute, sub-acute and chronic mastoiditis. We performed a prospective, observational study in children under 16 years of age presenting to our institution duri...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-007-0549-1

    authors: Stähelin-Massik J,Podvinec M,Jakscha J,Rüst ON,Greisser J,Moschopulos M,Gnehm HE

    更新日期:2008-05-01 00:00:00

  • Total serum bilirubin levels during the first 2 days of life and subsequent neonatal morbidity in very low birth weight infants: a retrospective review.

    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

    authors: Zhu J,Xu Y,Zhang G,Bao Y,Wu M,Du L

    更新日期:2012-04-01 00:00:00

  • Phagocytic activities in neonatal monocytes.

    abstract::Monocytes play an essential role in cellular host defense as circulating phagocytes, as well as precursors of macrophages. We investigated the principal phagocytic activities in monocytes from cord blood of term infants by analysing adherence, random migration, chemotaxis, bactericidal activity, phagocytosis-associate...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00439252

    authors: Speer CP,Wieland M,Ulbrich R,Gahr M

    更新日期:1986-10-01 00:00:00

  • A new method for screening for hyperammonemia.

    abstract::A new method for the detection of hyperammonemia, using a kit based on the principle of microdiffusion of ammonia, is described. The method requires only one drop of blood and takes only 15 min to complete. Experiments for recovery and reproducibility were satisfactory, and good correlation was obtained when compared ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00442347

    authors: Tada K,Okuda K,Watanabe K,Iimura Y,Yamada S

    更新日期:1979-02-08 00:00:00

  • Rotavirus and not age determines gastroenteritis severity in children: a hospital-based study.

    abstract:BACKGROUND:The severity of childhood gastroenteritis is generally believed to be age-related rather than aetiology-related. Rotavirus-induced gastroenteritis is more severe than gastroenteritis caused by other enteric pathogens and is also age-related. We thus addressed the question of whether the increased severity of...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,多中心研究

    doi:10.1007/s00431-006-0237-6

    authors: Albano F,Bruzzese E,Bella A,Cascio A,Titone L,Arista S,Izzi G,Virdis R,Pecco P,Principi N,Fontana M,Guarino A

    更新日期:2007-03-01 00:00:00

  • Unexpected side-effects of inhaled steroids: a case report.

    abstract::An asthmatic child is presented who developed a cushingoid appearance with evidence of adrenal suppression and growth impairment while on low dose inhaled topical steroids. When the inhaled steroids were replaced by inhaled sodium cromoglycate his adrenal function recovered while his appearance and growth returned to ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF02093730

    authors: Priftis K,Everard ML,Milner AD

    更新日期:1991-04-01 00:00:00

  • Bone status of young adults with periodic avoidance of dairy products since childhood.

    abstract::Proper nutrition throughout childhood and adolescence is crucial for normal bone development. We investigated whether adherence to Christian Orthodox Church fasting is characterized by periodic avoidance of animal foods (including dairy products), since childhood affects stature or bone health in young adults. This cr...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-019-03542-1

    authors: Rodopaios NE,Mougios V,Koulouri AA,Vasara E,Papadopoulou SK,Skepastianos P,Dermitzakis E,Hassapidou M,Kafatos AG

    更新日期:2020-04-01 00:00:00

  • Congenital self-healing non-Langerhans cell histiocytosis.

    abstract::Clinical, morphological, ultrastructural and immunological studies were performed in a case of congenital self-healing non-Langerhans cell histiocytosis. The patient showed several aspects that have not been published before: a large nodule in the vulvar region, vesiculobullous elements and pneumonia (asymptomatic). T...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00441808

    authors: Oranje AP,Vuzevski VD,de Groot R,Prins ME

    更新日期:1988-10-01 00:00:00

  • Correlation between serum antibody-levels against group B streptococci and gestational age in newborns.

    abstract::Sera from 33 newborn infants with gestational ages ranging from 27 to 41 weeks were tested by radioimmunoassay for IgG antibodies to surface antigens of group B streptococci (GBS) types Ia, Ib, II and III. Antibody levels to GBS antigens were positively correlated to gestational age and birthweight. However, only the ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00445584

    authors: Christensen KK,Christensen P,Duc G,Höger P,Kind C,Matsunaga T,Müller B,Seger RA

    更新日期:1984-06-01 00:00:00

  • Intestinal IgA deposition in Henoch-Schönlein purpura with severe gastro-intestinal manifestations.

    abstract:UNLABELLED:In patients with Henoch-Schönlein purpura (HSP) presenting with severe gastro-intestinal (GI) symptoms, IgA deposition was studied in endoscopically obtained mucosal biopsies. A total number of 11 patients (male, 7; female, 4) were enrolled in this study; 7 patients underwent upper GI endoscopy and biopsy 1 ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF02075757

    authors: Kato S,Ebina K,Naganuma H,Sato S,Maisawa S,Nakagawa H

    更新日期:1996-02-01 00:00:00

  • Bunk beds--a still underestimated risk for accidents in childhood?

    abstract:UNLABELLED:A retrospective analysis of 218 bunk-bed accidents and a random sample survey with 991 family interviews were performed in order to establish guidelines for bunk-bed accident prevention. Falls from the top bed during sleep (35.1%) or while playing (34.4%) and falling off the ladder (23.2%) are the leading ca...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s004310051303

    authors: Mayr JM,Seebacher U,Lawrenz K,Pesendorfer P,Berghold A,Baradaran S

    更新日期:2000-06-01 00:00:00

  • Performance of PRISM III and PELOD-2 scores in a pediatric intensive care unit.

    abstract:UNLABELLED:The study aims were to compare two models (The Pediatric Risk of Mortality III (PRISM III) and Pediatric Logistic Organ Dysfunction (PELOD-2)) for prediction of mortality in a pediatric intensive care unit (PICU) and recalibrate PELOD-2 in a Portuguese population. To achieve the previous goal, a prospective ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-015-2533-5

    authors: Gonçalves JP,Severo M,Rocha C,Jardim J,Mota T,Ribeiro A

    更新日期:2015-10-01 00:00:00

  • Anatomy of the naso-lacrimal duct in new-borns: therapeutic considerations.

    abstract::The topography and morphology of the lacrimal ducts in newborn infants was demonstrated by means of post mortem dacryocystograms and microphotograms of histologic slides. Because of bends in the course of the lower tear duct, a percentage of probings will fail to effect satisfactory perforation of the Hasner membrane....

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00442367

    authors: Müller KM,Busse H,Osmers F

    更新日期:1978-09-08 00:00:00

  • Partial anomalous pulmonary vein connection: an underestimated cardiovascular defect in Ullrich-Turner syndrome.

    abstract:UNLABELLED:Congenital cardiovascular defects, commonly affecting the aortic valve or the aortic arch (50% to 70%), are seen in ca. 17%-44% of patients with Ullrich-Turner syndrome (UTS). However, there are only 36 case reports worldwide on the coincidental finding of partial anomalous pulmonary vein connection (PAPVC) ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,评审

    doi:10.1007/s00431-003-1384-7

    authors: Bechtold SM,Dalla Pozza R,Becker A,Meidert A,Döhlemann C,Schwarz HP

    更新日期:2004-03-01 00:00:00

  • The accuracy of voiding urosonography in detecting vesico-ureteral reflux: a summary of existing data.

    abstract:UNLABELLED:The primary objective of this review was to assess the diagnostic accuracy of voiding urosonography (VUS) in detecting reflux (VUR). As a secondary objective, the reported technical suggestions and diagnostic mistakes were shown to improve the examination protocol and provide the most accurate results. Using...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-002-0954-4

    authors: Valentini AL,De Gaetano AM,Destito C,Marino V,Minordi LM,Marano P

    更新日期:2002-07-01 00:00:00

  • Influence of blood pressure level and age on within-visit blood pressure variability in children and adolescents.

    abstract::Blood pressure (BP) is variable in children and this could affect BP assessment, but the magnitude of within-visit BP variability (BPV) over consecutive measurements has never been investigated. This study aimed to determine the direction and magnitude of, and factors affecting, within-visit BPV in children and adoles...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-017-3049-y

    authors: Veloudi P,Blizzard CL,Srikanth VK,Schultz MG,Sharman JE

    更新日期:2018-02-01 00:00:00

  • A child with acute pancreatitis and recurrent hypoglycemia due to 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.

    abstract::A five-year-old-girl with a history of recurrent hypoglycemia presented with acidosis, intractable vomiting, and abdominal tenderness; the diagnosis of acute pancreatitis was made by abdominal ultrasonography and supportive biochemical studies. Urinary organic acid analysis revealed metabolites suggestive of HMG-CoA l...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00540255

    authors: Wilson WG,Cass MB,Søvik O,Gibson KM,Sweetman L

    更新日期:1984-09-01 00:00:00

  • Molecular characterisation and neuropsychological outcome of 21 patients with profound biotinidase deficiency detected by newborn screening and family studies.

    abstract:UNLABELLED:Early recognition by newborn screening and oral biotin supplementation may prevent clinical and neurological deficits in profound biotinidase deficiency (residual plasma biotinidase activity <10%). In order to evaluate possible correlations of molecular characteristics, onset and continuation of treatment an...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-003-1351-3

    authors: Möslinger D,Mühl A,Suormala T,Baumgartner R,Stöckler-Ipsiroglu S

    更新日期:2003-12-01 00:00:00

  • Somatic symptom and related disorders in a tertiary paediatric hospital: prevalence, reach and complexity.

    abstract::Specialist paediatric services manage a variety of presentations of functional somatic symptoms. We aimed to describe the presentation and management of children and adolescents with somatic symptom and related disorders (SSRDs) requiring admission to a tertiary children's hospital with the objective of informing the ...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-020-03867-2

    authors: Wiggins A,Court A,Sawyer SM

    更新日期:2020-11-13 00:00:00

  • Triple A syndrome: 32 years experience of a single centre (1977-2008).

    abstract::Triple A syndrome is an autosomal recessive disorder characterized by alacrima, achalasia, ACTH-resistant adrenal insufficiency, autonomic dysfunction, and neurodegeneration. Mutations in the AAAS gene on chromosome 12q13 encoding the nuclear pore protein ALADIN have been reported in these patients. Over the period 19...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-010-1222-7

    authors: Milenkovic T,Zdravkovic D,Savic N,Todorovic S,Mitrovic K,Koehler K,Huebner A

    更新日期:2010-11-01 00:00:00

  • Follow up study on children with dyslipidaemia detected by mass screening at 18 months of age: effect of 12 months dietary treatment.

    abstract::The present study was done to evaluate the effect of short-term dietary therapy on 148 dyslipidaemic children (24 familial hypercholesterolaemia, 105 non-familial hypercholesterolaemia and 19 hypertriglyceridaemia), detected by mass screening in children at 18 months of age. In the model diet used for treatment, 15% o...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF01957537

    authors: Ohta T,Nakamura R,Ikeda Y,Hattori S,Matsuda I

    更新日期:1993-11-01 00:00:00

  • Correction to: Cost-effectiveness of rule-based immunoprophylaxis against respiratory syncytial virus infections in preterm infants.

    abstract::The name of the co-author Wendy J. Ungar was inadvertently omitted on the original published article. Her name and affiliation have now been added to the author list. ...

    journal_title:European journal of pediatrics

    pub_type: 已发布勘误

    doi:10.1007/s00431-019-03526-1

    authors: Blanken MO,Frederix GW,Ungar WJ,Nibbelke EE,Koffijberg H,Sanders EAM,Rovers MM,Bont L,Dutch RSV Neonatal Network.

    更新日期:2020-02-01 00:00:00

  • A fatal case of portal hypertension complicating systemic mastocytosis in an adolescent.

    abstract::Portal hypertension was observed in a 17-year-old girl with urticaria pigmentosa since 2 months of age. Liver biopsies showed portal and sinusoidal infiltration with mast cells although spleen biopsies showed only fibrosis. CONCLUSION. Portal hypertension is a complication of systemic mastocytosis that can lead to dea...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF01959789

    authors: Fonga-Djimi HS,Gottrand F,Bonnevalle M,Farriaux JP

    更新日期:1995-10-01 00:00:00

  • Treatment of status epilepticus with thiopentone sodium anaesthesia in a child.

    abstract::A 4-month-old infant was admitted to the Pediatric Intensive Care Unit with Pneumococcal meningitis. A few hours after admission he developed intractable convulsions that could not be stopped with phenytoin, phenobarbitone and a continuous drip of diazepam. Thiopentone sodium anaesthesia was induced for 24 h terminati...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF00441663

    authors: Goitein KJ,Mussaffi H,Melamed E

    更新日期:1983-04-01 00:00:00

  • Ethical issues in the daily medical care of children.

    abstract::The main ethical imperative of all paediatric actions is the demand to do everything "in the best interests of children". Relevant guidelines can be derived from the UN Declaration on the Rights of Children, whereupon every child has the fundamental right to life and dignity, and is entitled to optimal medical care. P...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-005-0002-2

    authors: Kurz R,Gill D,Mjones S,Ethics Working Group of Confederation of European Specialists in Paediatrics.

    更新日期:2006-02-01 00:00:00

  • Benign paroxysmal vertigo of childhood: a migraine equivalent.

    abstract::Eight patients with the syndrome of benign paroxysmal vertigo of childhood are presented. This symptom complex is characterized by attacks of vertigo in young children combined with nystagmus, ataxia and transiently decreased vestibular function, but without impaired consciousness. Diagnostic and follow-up studies rev...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/BF01846035

    authors: Koehler B

    更新日期:1980-08-01 00:00:00

  • Omalizumab in the treatment of eosinophilic esophagitis and food allergy.

    abstract::Omalizumab is currently used in severe asthma and has been tried in other allergic disorders. The authors report two patients with multiple food allergies and eosinophilic esophagitis on a very restrictive diet who have been treated with omalizumab, in order to improve food intolerance--the major distressing factor in...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章

    doi:10.1007/s00431-011-1540-4

    authors: Rocha R,Vitor AB,Trindade E,Lima R,Tavares M,Lopes J,Dias JA

    更新日期:2011-11-01 00:00:00

  • Clinical characteristics and outcome of children stung by scorpion.

    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

    authors: Mohamad IL,Elsayh KI,Mohammad HA,Saad K,Zahran AM,Abdallah AM,Tawfeek MS,Monazea EM

    更新日期:2014-06-01 00:00:00

  • Cord blood alpha-fetoprotein concentrations in term newborns of smoking mothers.

    abstract:UNLABELLED:To investigate the toxic effect of tobacco smoke on the fetus, we measured in cord blood the concentrations of alpha-fetoprotein (AFP), the principal serum protein in early ontogenic development, and erythropoietin (EPO), as an index of chronic fetal hypoxia. A total of 103 consecutively enrolled term newbor...

    journal_title:European journal of pediatrics

    pub_type: 临床试验,杂志文章

    doi:10.1007/s004310051152

    authors: Beratis NG,Varvarigou A,Christophidou M,Vassilakos P,Tsapanos V,Kourounis G

    更新日期:1999-07-01 00:00:00

  • Clinical practice treatment of HIV infection in children.

    abstract::Perinatal transmission remains the main cause of HIV infection in the pediatric population. Treatment of HIV-infected children has become less of a problem in resource-rich countries with a remarkable decrease of perinatal infections, resulting in an effective prevention of mother-to-child transmission and antiretrovi...

    journal_title:European journal of pediatrics

    pub_type: 杂志文章,评审

    doi:10.1007/s00431-008-0914-8

    authors: Brichard B,Van der Linden D

    更新日期:2009-04-01 00:00:00