[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 marker of disease progression, later on integrated in research studies for patients survival, the scoring systems loose their power due to the possibility of computerized data storage of unlimited variables. Computerized networking allows immediate access to all measured data of an individual or of groups of patients with detailed criteria. Analysis of such data are less vulnerable for mistakes than individually scored patients data; scoring systems in cystic fibrosis will probably become an historical measure.

journal_name

Klin Padiatr

journal_title

Klinische Padiatrie

authors

Schöni MH

doi

10.1055/s-2007-1025188

subject

Has Abstract

pub_date

1993-01-01 00:00:00

pages

3-8

issue

1

eissn

0300-8630

issn

1439-3824

journal_volume

205

pub_type

杂志文章,评审
  • Evaluation of desmopressin effect on primary haemostasis in pediatric patients with aspirin-like defect as hereditary thrombocytopathy.

    abstract:OBJECTIVES:Despite about 3 decades of clinical experience with the therapy of inherited thrombocytopathies (HTP) with desmopressin (DDAVP) the mechanisms of haemostatic effects of DDAVP in these diseases remain unclear. Therefore platelet function diagnostics was carried out in whole blood (WB) from children with aspir...

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    doi:10.1055/s-0031-1275347

    authors: Tauer JT,Gneuss A,Lohse JE,Jürgens T,Knöfler R

    更新日期:2011-05-01 00:00:00

  • [Wegener's granulomatosis as a rare cause of hemoptysis in childhood].

    abstract::Wegener's granulomatosis is together with Churg-Strauss syndrome and microscopic polyangiitis a very rare cause of hemoptysis in childhood. We report on a 14 years old girl who developed arthralgia, purpura, glomerulonephritis and a diffuse alveolar haemorrhage with hemoptysis after a short time period of unspecific s...

    journal_title:Klinische Padiatrie

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    doi:10.1055/s-2005-836847

    authors: Heckmann M,Weber K,Reineck P,Schüler D,Geidel C,Steiss JO

    更新日期:2006-01-01 00:00:00

  • [Familial chromosome translocation (1;5;15) as a cause of partial trisomy 1p].

    abstract::An unbalanced structural chromosome aberration was found in a 6-year-old girl. Mother and maternal grandmother were shown to be carriers of a complex balanced translocation involving chromosomes 1, 5 and 15 and are of normal phenotype. The unbalanced status in the proband was defined as a partial trisomy 1p21-1p31. Pa...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1026754

    authors: Schürmann M,Wethling H,Niemeyer ML,Schwinger E

    更新日期:1987-01-01 00:00:00

  • [Toxocariasis--a neglected ubiquitous helminthiasis in children and adolescents].

    abstract::More than 40 years ago, Toxocara ssp. was identified as the cause of larva migrans visceralis and ocular larva migrans, which mainly affect infants and children. Although widespread in most parts of the world, the parasitic disease is rarely diagnosed in Germany. Focusing on clinical and pathophysiological similaritie...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2008-1043867

    authors: Hohenschild S

    更新日期:1998-05-01 00:00:00

  • [Thyroglossal duct cysts in paediatric patients: early operative intervention reduces rate of complications].

    abstract:BACKGROUND:Thyroglossal duct cysts arise from mucus production in an incomplete regressed thyroglossal duct. Often they are only noticed in case of infection or secondary fistulation. The operative management is a Sistrunk procedure. METHOD AND PATIENTS:In a retrospective study we analyse 26 patients aged 0,3 - 10 yea...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2001-17223

    authors: von Bismarck S,Höllwarth ME

    更新日期:2001-09-01 00:00:00

  • [Congenital syphilis after reactivation of "healed" maternal primary infection].

    abstract:UNLABELLED:The congenital syphilis is an infectious fetopathy which is able to affect the whole organism. In most cases symptoms are not obvious before week fourth to twelve of life. The infection of the mother is the precondition for the child's disease. The placental transfer takes place after the fifth to sixth mont...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2000-9612

    authors: Vieker S,Siefert S,Lemke J,Püst B

    更新日期:2000-11-01 00:00:00

  • [Non-invasive determination of oxygen saturation with the oxygenmet pulse wave oximeter. Comparison with the invasive reflectometric method (author's transl)].

    abstract::Oxygen saturation was determined non-invasively with the oxygenmet pulse wave oximeter 1471 and at the same time with the invasive reflectometric method. The determinations were carried out on 49 children (males = 24, females = 25) during 52 catherizations of the heart. Among these children aged between one week and 1...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1034484

    authors: Krenkel R,Liappis N,Redel D,Hildenbrand G

    更新日期:1981-07-01 00:00:00

  • Seroprevalence and significance of antibodies to hepatitis C virus in pediatric patients with malignant diseases.

    abstract::The prevalence of antibodies to hepatitis C virus recombinant antigen c100-3 was determined in 82 pediatric patients with malignant diseases who received blood transfusions as support during their chemotherapy. By prescreening, 12 of 82 patients were repeatedly positive. No positive correlation was established neither...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2007-1025441

    authors: Schneppenheim R,Rautenberg P,Behnke H,Vietor K

    更新日期:1991-07-01 00:00:00

  • [Lead poisoning in childhood: a silent epidemic in the slums of the United States (author's transl)].

    abstract::While leadpoisoning has been with mankind since the dawn of civilization, only in recent years this disease was recognized as a serious public health problem. It has been estimated that more than half a million children are suffering from lead intoxication in the U.S.A. In most instances this disease is caused by eati...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:

    authors: Püschel SM

    更新日期:1975-09-01 00:00:00

  • [Robin-Silverman-Smith (fetal face) syndrome].

    abstract::Report of a case of the fetal face syndrome associated with severe oligophrenia and septum pellucidum cyste in a male patient aged 30 years. The case demonstrates the good prognosis of the hypogenitalism resp. small genitalia; the correct diagnosis in this syndrome can the patients save a plastic of the penis. ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1046568

    authors: Fehlow P,Walther F,Pfeifer T,Schuchard UM

    更新日期:1995-11-01 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

  • [Effects of self-adapting G-DRG system 2004 to 2006 on in-patient services payment in pediatric hematology and oncology patients of a university hospital].

    abstract:BACKGROUND:Reimbursement of inpatient treatment by daily constant charges is replaced by diagnosis- and procedure-related group system (G-DRG) in German acute care hospitals excerpt for psychiatry since 2004. Re-designs of G-DRG system were undertaken in 2005 and 2006. Parallel to implementation requirement- and resour...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2006-942274

    authors: Christaras A,Schaper J,Strelow H,Laws HJ,Göbel U

    更新日期:2006-11-01 00:00:00

  • [Progress and change in nuclear magnetic resonance diagnosis of congenital and acquired heart defects].

    abstract::Since 1987, the authors have examined 186 patients (76 girls, 110 boys) with a variety of congenital and acquired heart diseases by means of magnetic resonance imaging (MRI). The patients' ages ranged from 2 days to 20 years (mean age 3.97 years). During the study new techniques were developed, which extend the applic...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2007-1025369

    authors: Sieverding L,Jung WI,Fleiter TH,Klose U,Steil E,Hassberg D,Rosendahl W

    更新日期:1992-09-01 00:00:00

  • [Therapy-related pamphlet for patients in pediatric oncology].

    abstract::This notebook which is of postcard size contains important information in case of emergency and details about the current illness and treatment. It contains follow-up information about the pattern of growth, endocrinological development and assessment of renal tubular function. The necessary follow-up investigations a...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1046621

    authors: Maass E

    更新日期:1994-07-01 00:00:00

  • [Liposomal daunorubicine combined with cytarabine in the treatment of relapsed/refractory acute myeloid leukemia in children].

    abstract:BACKGROUND:First-line treatment in AML commonly included high cumulative doses of anthracyclines with an increasing risk of cardiotoxicity. Liposomal daunorubicin (L-DNR) is thought to be less cardiotoxic without impairment of efficacy. METHODS:The AML-BFM REZ 97 study included two reinduction blocks with L-DNR (2 x 6...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2002-33185

    authors: Reinhardt D,Hempel G,Fleischhack G,Schulz A,Boos J,Creutzig U

    更新日期:2002-07-01 00:00:00

  • [Early childhood intervention - access to risk families and support through actors from the health-care sector].

    abstract::Interdisciplinary cooperation and networking determine the success of activities for supporting families at risk for early childhood abuse. The integration of the healthcare sector might be important.The medical standard of perinatal care at the University hospital includes information exchange about family risk facto...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0034-1374603

    authors: Clauß D,Deutsch J,Krol I,Haase R,Willard P,Müller-Bahlke T,Mauz-Körholz C,Körholz D

    更新日期:2014-07-01 00:00:00

  • [Cow milk-specific humoral and cellular immune response in infants with high risk of atopy under feeding a whey hydrolysate infant formula].

    abstract:BACKGROUND:Hypoallergenic infant formulas (HAF) were developed for atopy prevention in infants with high risk of atopy if these cannot be breastfed. HAF mount an antigen-specific immune response in infants. The aim of the study was to analyse the immune response in infants fed with a new infant formula based on a whey ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2003-42664

    authors: Nentwich I,Pazdírková A,Koberská I,Pokojská E,Szépfalusi Z,Lokaj J

    更新日期:2003-09-01 00:00:00

  • [A study group on brain tumors in childhood. Introduction, establishment and aims].

    abstract::Tumors of the central nervous system account for 15.7% of all malignancies registered at the documentation center for childhood malignancies in Western Germany. This group constitutes the largest entity of solid tumors. With the exception of medulloblastoma the five-year survival probability of children with cns tumor...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1026789

    authors: Kühl J

    更新日期:1987-05-01 00:00:00

  • Does megatherapy contribute to survival in metastatic neuroblastoma? A retrospective analysis. German Cooperative Neuroblastoma Study Group.

    abstract::To evaluate the impact of megatherapy on the outcome of high risk neuroblastoma, 39 patients with stage IV neuroblastoma, who underwent megatherapy, were analyzed retrospectively and compared to 49 patients, who continued chemotherapy according to the German cooperative trial NB85. The groups were comparable concernin...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1043950

    authors: Hero B,Kremens B,Klingebiel T,Bender-Götze C,Burdach S,Schrappe M,Berthold F

    更新日期:1997-07-01 00:00:00

  • [Sweat testing practices in German cystic fibrosis centres].

    abstract:BACKGROUND:Surveys in USA and UK have shown inconsistencies between practice of sweat testing and guidelines of the National Committee for Clinical Laboratory Standards (NCCLS), which could increase the risk of false-positive and -negative results. To determine the current practice of sweat testing in German cystic fib...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2007-872458

    authors: Naehrlich L

    更新日期:2007-03-01 00:00:00

  • [Diagnosis of cerebral function disorders and subsequent brain damage in newborn infants under intensive care].

    abstract::Possibilities of recognizing cerebral function disturbances in newborns as early as the acute phase of the condition, and of prognosticating permanent brain damage are described. Clinical findings such as lethargy or spasms in the second and third weeks of life following hypoxia indicate severe function disturbances w...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2008-1034092

    authors: Karch D

    更新日期:1984-11-01 00:00:00

  • [Experiences with enteral nutrition of patients with cystic fibrosis (CF) via a percutaneous endoscopic gastrostomy (PEG)].

    abstract:BACKGROUND:We wanted improve the nutritional status of patients with cystic fibrosis with enteral feeding via a percutaneous endoscopic gastrostomy (PEG). PATIENTS AND METHODS:In a period of 8 years 11 patients underwent a percutaneous endoscopic gastrostomy (PEG), 4 males and 7 females, in the median 14,9 years of ag...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2001-18460

    authors: Richter T,Meier C,Steppberger K,Knorrek G,Lietz T

    更新日期:2001-11-01 00:00:00

  • [Potential coulometry, a new methodology for the determination of iron concentration in serum. Comparison with the bathophenanthroline method in protein-free solution (author's transl)].

    abstract::Iron concentration was determined by the aid of the ESA Ferrochem 3050 analyzer and the bathophenanthroline method with deproteinization. Both methods showed a good precision and accuracy. The investigation was carried out in 505 sera with an iron concentration between 9 and 333 micrograms/dl. The comparison of the tw...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1033773

    authors: Liappis N

    更新日期:1982-01-01 00:00:00

  • [Importance of prevention of infectious complications in pediatric hematologic-oncologic patients].

    abstract::The control of severe infectious complications by preventive strategies, early diagnosis of infections and empiric broad-spectrum antibiotic therapy contributed to a marked improvement of survival in children with cancer over the last 20 years. This article overviews of the importance of prevention of infectious compl...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2001-17498

    authors: Fleischhack G,Simon A

    更新日期:2001-09-01 00:00:00

  • [Fat-emulsions and acid-base balance in neonates of low weight at birth (author's transl)].

    abstract::The influence of a single intravenous infusion of triglycerides (1 g triglyceride per kg body weight) on the acid base equilibrium was investigated in 16 newborn infants with low birth weight during the first days of age. No changes of pH, PCO2 and standard bicarbonate, base excess, buffer base and actuelle bicarbonat...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:

    authors: Melichar V,Wolf H,Jirsová V,Vondrácek J

    更新日期:1975-09-01 00:00:00

  • [The portal system and hepatic artery in children with biliary atresia. 1: Ultrasound and simple duplex ultrasound parameters].

    abstract::In 37 children with extrahepatic biliary atresia between 12 days in 9 years of age we performed 72 duplexsonographic examinations of the portal system and the hepatic artery. In the control group we investigated 61 healthy children. All investigations were performed with computer sonography (Acuson 128) with a 3.5 MHz...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2007-1025481

    authors: Grunert D,Stier B,Schöning M

    更新日期:1990-01-01 00:00:00

  • [Postoperative sonographic follow-up of brain tumors in childhood].

    abstract::Postoperative neurosonography was performed in 69 babies and children after osteoclastic neurosurgery for primary cerebral tumors. Astrocytoma of the posterior fossa (26 cases) and medulloblastoma (25 cases) were frequent histological findings. Early postoperative bed-side examinations revealed reliably complications ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1026831

    authors: Dittrich M,Gutjahr P,Dinkel E,Higer HP,Müller W

    更新日期:1987-11-01 00:00:00

  • Risk Factors Associated with Intraventricular Hemorrhage in Preterm Infants with ≤28 Weeks Gestational Age.

    abstract:OBJECTIVE:To identify obstetric and neonatal risk factors associated with the development of germinal matrix-intraventricular hemorrhage (GM-IVH) in high-risk preterm neonates. METHODS AND PATIENTS:Data from 279 preterm infants (246 mothers) with a gestational age≤28+0 weeks admitted to our NICU between January 2004 a...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0042-111689

    authors: Waitz M,Nusser S,Schmid MB,Dreyhaupt J,Reister F,Hummler H

    更新日期:2016-09-01 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

  • [The meconium ileus equivalent in mucoviscidosis].

    abstract::Meconium ileus equivalent (MIE) complicating cystic fibrosis of the pancreas (CF) increases in frequently with increasing age of patients. In the present paper the course of 11 children and adolescents with MIE diagnosed and treated at the University Dept. of Paediatrics in Zürich during the last 15 years, i.e. 9% of ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1034390

    authors: Rosenberg E,Shmerling DH

    更新日期:1983-09-01 00:00:00