[Progressive combined immunity defect with ectomesodermal dysplasia (author's transl)].

Abstract:

:A boy with dysplastic features had to be treated for recurrent diarrhoea and bronchitis since his tenth month of life. Defects in humoral and cellular immunity were found. The ecto-mesodermal abnormalities are so characteristic that in connection with the immunological changes it seems to be justified to assume a yet undescribed syndrome.

journal_name

Klin Padiatr

journal_title

Klinische Padiatrie

authors

Tympner KD,Belohradsky B,Eife R,Klose PK

subject

Has Abstract

pub_date

1978-11-01 00:00:00

pages

610-3

issue

6

eissn

0300-8630

issn

1439-3824

journal_volume

190

pub_type

杂志文章
  • [Achalasia, alacrimia and cortisol deficiency--Allgrove syndrome].

    abstract::Review of a rare multisystem disorder with the main symptoms achalasia of the esophagus, alacrimia and glucocorticoid deficiency as well as multiple other symptoms especially of the nervous system. Report of one case diagnosed in the age of 11.5 years with a case history of about 7 years. Description of the danger to ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2008-1046527

    authors: Hübschmann K

    更新日期:1995-05-01 00:00:00

  • [Wiedemann-Beckwith syndrome: clinical characteristics, constitutional chromosome abnormalities and tumor incidence].

    abstract::The observation of constitutional chromosome abnormalities in a one and half year old boy with Wiedemann-Beckwith syndrome (WBS) and hepatoblastoma prompted us to review the currently available literature dealing with this subject. In approximately 500 published cases with complete and incomplete forms of WBS, 42 mali...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1026804

    authors: Haas OA,Zoubek A,Grümayer ER,Ferstl G,Gadner H

    更新日期:1987-07-01 00:00:00

  • Extending the Concept of Advance Care Planning to the Perinatal Period.

    abstract::Easier access to prenatal diagnostic procedures led to its widespread use as a screening measure. Hence, today it is more common for life-limiting illnesses to be diagnosed during fetal life. The concept of Advance Care Planning (ACP) provides a framework for caregivers, families and their multidisciplinary teams to a...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/a-1179-0530

    authors: Garten L,von der Hude K,Strahleck T,Krones T

    更新日期:2020-09-01 00:00:00

  • [Left flank tumor of fluctuating size in a 5-week-old boy: perirenal urinoma caused by urethral valves].

    abstract:BACKGROUND:Flank swelling and pseudotumors of the kidney are unusual manifestations of obstructive uropathies in small children. Our case illustrates typical problems and briefly reviews management options. CASE REPORT:A 5-week-old boy presented with a large, palpable urinoma due to posterior urethral valves. Sonograp...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2006-933545

    authors: von Schnakenburg C,Dorn A,Jacobi C,Fisch D,Pohl M,Frankenschmidt A

    更新日期:2006-09-01 00:00:00

  • Early and long term outcome in children with esophageal atresia treated over the last 22 years.

    abstract:BACKGROUND:The survival of infants born with esophageal atresia (EA) is > 90% at present. The purpose of this study was to evaluate early complications and long term outcome in children with EA treated at our institution. METHODS AND PATIENTS:Retrospective analysis of 111 children with EA undergoing repair of EA or tr...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0030-1249610

    authors: Lacher M,Froehlich S,von Schweinitz D,Dietz HG

    更新日期:2010-09-01 00:00:00

  • [Diagnostic and differential-diagnostic considerations regarding the assessment of children's reactions within the scope of psychosocial care in paediatric oncology and haematology].

    abstract::Oncological diseases in children and adolescents represent a considerable burden for the children themselves and for their families. In order to position such concomitants of a life-threatening disease appropriately among the diagnostic options of psychosocial cancer care one is repeatedly confronted with the issue of...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2007-985872

    authors: Schreiber-Gollwitzer B,Gollwitzer K

    更新日期:2007-11-01 00:00:00

  • [Antacids for postoperative prevention of stress ulcer in infants: a dose finding study].

    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

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1055/s-2008-1043985

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

    更新日期:1996-01-01 00:00:00

  • Calcium and phosphor intake in preterm infants: sensitivity and specifity of 6-hour urine samples to detect deficiency.

    abstract::Aim of the present study was to test whether six-hour (6 h) urine specimens predict the 24-hour (24 h) mineral homeostasis in individual infants born preterm. Urinary Calcium (Ca) and Phosphate (P) concentrations were studied in 60 stable infants; gestational age 34 (25-42) weeks. In 58 infants four 6 h urine specimen...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0031-1301361

    authors: Mihatsch W,Trotter A,Pohlandt F

    更新日期:2012-03-01 00:00:00

  • [Anaerobic microflora of praepubertal children (author's transl)].

    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

    pub_type: 杂志文章

    doi:10.1055/s-2008-1033769

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

    更新日期:1982-01-01 00:00:00

  • [Undetected Cases of Non-Accidental Burns in Children - Preventive Strategies].

    abstract:BACKGROUND:Thermal injuries are a frequent cause of accidents within the pediatric population that may result in long periods of hospitalization as well as severe physical and mental impairment and lifelong consequences. Especially in infants, burns caused by scalding are the most common cause of injury. Beside acciden...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0043-119416

    authors: Klinke M,Schmidt CM,Tegtmeyer L,Reinshagen K,Boettcher M,Koenigs I

    更新日期:2018-03-01 00:00:00

  • [Non-immunologic hydrops fetalis (NIHF)--case report of double partial trisomy 15q and 17q resulting from familial translocation 15/17 and cytogenetic findings in 50 cases with hydrops fetalis].

    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

    pub_type: 杂志文章

    doi:10.1055/s-2008-1026810

    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

  • [The Value of Conventional Radiographs of the Pelvis in Detection of Perthes Disease 3 Months After an Episode of Acute Transient Synovitis].

    abstract::Background: Transient synovitis is the most common hip joint disorder in children. Perthes disease occurs around the same age and may not be distinguishable to an episode of transient synovitis in the early stage. Therefore all children in our clinic with transient synovitis underwent a follow-up X-ray 3 months later ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0042-116150

    authors: Lenoir U,Slongo T,Aghayev E,Joeris A

    更新日期:2017-03-01 00:00:00

  • Protein supplementation of human milk for the nutrition of VLBW-infants: human milk protein vs. meat protein hydrolysate.

    abstract::In 25 very low birth weight infants appropriate for gestational age the influences of different human milk (HM) preparations on weight gain, gross indices of nitrogen metabolism and energy balance were studied during the second month of postnatal life. HM was fortified either by HM-protein (HMP) or by an enzymatic mea...

    journal_title:Klinische Padiatrie

    pub_type: 临床试验,杂志文章

    doi:10.1055/s-2007-1025538

    authors: Boehm G,Senger H,Friedrich M,Müller DM,Beyreiss K

    更新日期:1990-09-01 00:00:00

  • [Therapy of choroid plexus carcinoma in childhood. Case report and review of the literature].

    abstract::Carcinomas of the plexus choroideus (PCC) represent the rare malignant variety of plexus choroideus papillomas and take a particularly unfavourable course. Tumors of the plexus choroideus account for about 2-4% of all primary brain tumors in children and 0.5% of those in adults. The PCC is more frequent in children th...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2008-1046511

    authors: Peschgens T,Stollbrink-Peschgens C,Mertens R,Völker A,Thron A,Heimann G

    更新日期:1995-03-01 00:00:00

  • [Cognitive and motor abilities and behavioural outcome in children after neonatal operation with cardiopulmonary bypass].

    abstract:BACKGROUND:Empirical results about the cognitive, motor and psychic development in patients with d-transposition of the great arteries after arterial switch operation remain unclear. Potential reasons are the patients' initial status, improved perinatal care or the quality of surgery. To exclude quality of surgery as t...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2007-985863

    authors: Vahsen N,Kavsek M,Toussaint-Götz N,Schneider K,Urban AE,Schneider M

    更新日期:2009-01-01 00:00:00

  • [Treatment of chronic myeloid leukemia in children and adolescents--concept of the multicenter pilot study CML-ped].

    abstract::CML is a rare malignancy in childhood not yet being treated by an established protocol of the Society of Pediatric Oncology and Hematology (GPOH). Up to now only allogeneic bone marrow transplantation (BMT) offers a curative treatment option. After being enrolled into the study "CML-päd" patients with a matched siblin...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,多中心研究

    doi:10.1055/s-2008-1046480

    authors: Suttorp M,Lion T,Creutzig U,Klingebiel T,Gadner H

    更新日期:1996-07-01 00:00:00

  • [Analysis of a Family-centred Care Programme with Follow-up Home-visits in Neonatology - In Times of the Directive from G-BA].

    abstract:BACKGROUND:Marked progress in neonatology changed care of very preterm infants (VLBW) over the last decades - but also the attitude towards family-centred care (FCC). With the directive of the German Federal Joined Committee (G-BA), politicians recognize the necessity of neonatal FCC. AIM:To evaluate time and personne...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0042-102787

    authors: Hüning BM,Reimann M,Sahlmen S,Leibold S,Nabring JC,Felderhoff-Müser U

    更新日期:2016-07-01 00:00:00

  • Drug handling in a paediatric intensive care unit--can errors be prevented by a three-step intervention?

    abstract:BACKGROUND:Drug handling in paediatric intensive care units (PICU) is prone to medication errors. We aimed to identify type and prevalence of those errors and to assess preventative interventions. METHODS:Prospective intervention study investigating a 3-step intervention for preventing errors in drug handling in a 10-...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0033-1364030

    authors: Niemann D,Bertsche A,Meyrath D,Oelsner S,Ewen AL,Pickardt B,Henhapl T,Hoffmann G,Meyburg J,Bertsche T

    更新日期:2014-04-01 00:00:00

  • [Predictors of follow-up course of asthma complaints in early childhood--results of a follow-up study].

    abstract::A follow-up study was undertaken to determine the predictors of the course of recurrent wheezing episodes in children (between 0 to 8 years). In 1991, 600 children with wheezing episodes had been recorded by physicians who participated in a sentinel practice network. On each consultation with the same physicians from ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1043843

    authors: Seidler A,Schlaud M,Raum E,Schwartz FW

    更新日期:1998-01-01 00:00:00

  • [Visceral leishmaniasis (Kala-Azar) in a 3-year-old German infant (author's transl)].

    abstract::The article reports on a case of visceral leishmaniasis in a 3-year-old child of German residents in Rome who had passed two vacations on the isle of Ischia. Initial signs were intermittent temperatures, marked anorexia, hepatosplenomegaly and pancytopenia, with spontaneous recovery after three weeks. At that time, le...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:

    authors: Wittermann C,Bienzle U,Betke K

    更新日期:1979-05-01 00:00:00

  • Megestrol acetate to correct the nutritional status in an adolescent with growth hormone deficiency: Increase of appetite and body weight but only by increase of body water and fat mass followed by profound cortisol and testosterone depletion.

    abstract::Megestrol acetate (MA) is a synthetic, orally active derivative of the naturally occurring hormone progesterone. MA is increasingly used to correct loss of appetite and improve the nutritional status. We used MA in an adolescent with growth hormone (GH) deficiency due to former irradiation therapy in order to evaluate...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2002-25268

    authors: Schmid I,Stachel DK,Freudenberg S,Schmitt M,Schuster F,Haas RJ

    更新日期:2002-03-01 00:00:00

  • [D-penicillamine-induced IgA deficiency in the therapy of Wilson's disease].

    abstract::Serum IgA deficiency was first noted in a 10 year old boy 8 months after the onset of D-penicillamine therapy. Special immunological examinations revealed a deficiency of the secretory component of IgA while cellular functions of T- and B-lymphocytes were normal. The patient showed discrete clinical signs compatible w...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2007-1025560

    authors: Ibel H,Feist D,Endres W,Belohradsky BH

    更新日期:1990-11-01 00:00:00

  • [Kleinschmidt's syndrome (author's transl)].

    abstract::This is a case-report about a 4 year old boy with "cherry red" epiglottis, purulent meningitis and pleuropneumonia. Purulent meningitis and pleuropneumonia are not complications of treatment of "cherry red" epiglottis but an entity caused by Hemophilus influenzae-infection and is called Kleinschmidt's syndrome (Hemoph...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:

    authors: Freudenberg V,Wolf H

    更新日期:1976-07-01 00:00:00

  • [Recurrent subacute endocarditis caused by Streptococcus mutans in a child].

    abstract::Bacterial endocarditis belongs to the rare diseases in childhood. It occurs usually as a single episode and almost exclusively in children with congenital heart disease. In recent years, however, an increased number of renewed endocarditis after the first episode were reported, especially in drug addicts. We present a...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2008-1046498

    authors: Hunkert F,Handrick W,Kinzel P,Schneider P,Spencker FB,Günther E

    更新日期:1995-01-01 00:00:00

  • [Doppler echocardiographic investigation of left ventricular diastolic function in preterm infants with and without a patent ductus arteriosus].

    abstract::We studied the influence of preload augmentation on Doppler-derived left ventricular diastolic function parameters in infants with a birth weight <1500 g. In 44 neonates with a closed duct and 29 neonates with an open ductus arteriosus a complete echocardiographic study including Doppler investigations of the mitral i...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2004-817684

    authors: Schmitz L,Stiller B,Koehne P,Koch H,Lange PE

    更新日期:2004-01-01 00:00:00

  • [Malignant testicular tumors in children and adolescents: concept of the MAHO 82 cooperative therapeutic study of the Society for Pediatric Oncology].

    abstract::The German Society of Pediatric Oncology (GPO) designed a cooperative study to improve the outlook of patients with malignant (testicular) germ cell tumors. According to stage and histology of the tumor different surgical approaches to retroperitoneal lymphadenectomy are suggested. Local radiotherapy is not recommende...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1034068

    authors: Haas RJ,Brämswig J,Göbel U,Harms D,Janka G,Weissbach L

    更新日期:1983-05-01 00:00:00

  • Tumorangiogenesis: a network of cytokines.

    abstract::During angiogenesis new blood vessels sprout from an existing vascular bed. This is a prerequisite for tumor growth beyond a certain size and for metastasis formation. Tumors produce a number of cytokines. The development of in vivo bio-assays for angiogenesis and in vitro analysis of endothelial cells permits charact...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2008-1043960

    authors: Hotfilder M,Nowak-Göttl U,Wolff JE

    更新日期:1997-07-01 00:00:00

  • [Persistent Lactobacillus casei subspecies rhamnosus bacteremia in a 14 year old girl with acute myeloid leukemia. A case report].

    abstract::The present clinical observation is related to a 14-year-old girl suffering from acute myeloid leukemia. The clinical course was complicated by episodes of severe enterocolitis, E. coli- septicemia, pancreatitis and pneumonia. In the course of continued cytostatic and antibiotic treatment a persistent asymptomatic Lac...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1043764

    authors: Majcher-Peszynska J,Heine W,Richter I,Eggers G,Mohr C

    更新日期:1999-03-01 00:00:00

  • [Introduction and critical comparison of clinical scores for characterizing the degree of morbidity of patients with cystic fibrosis].

    abstract::Scoring systems for evaluation of the degree of severity in patients with cystic fibrosis are critically evaluated. Cross-sectional comparison of Shwachman's score, Cooperman's score, Taussig-NIH score, Bernese score and Huang score reveals that advantages and disadvantages of each score exist. In early days used as a...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2007-1025188

    authors: Schöni MH

    更新日期:1993-01-01 00:00:00

  • [Interferons--biologic principles and clinical uses].

    abstract::Interferons are components of the nonspecific defense system. Their most prominent biological roles are the antiviral, the antiproliferative, and the immunoregulatory activities. However, their primary functions within the organism remains to be determined. Three types of IFNs have been described so far (IFN alpha, IF...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1033980

    authors: Jacobsen H,Kirchner H

    更新日期:1985-07-01 00:00:00