[Diagnosis and therapy of primary defects of immunity (author's transl)].

Abstract:

:Immunological responses are divided into those mediated by humoral antibody (B-Cells) and those by cells (T-Cells). Both depend upon the activity of small lymphocytes which become thymus or bursa analogue dependent during embryonic development. Primary immundeficiencies are therefor defects of lymphocytes attributed in most cases to gene defects. Early diagnosis is the necessary prerequisit for possible treatment. Tests for assessing the cellular immune status are: skin testes and in vitro tests. Clinical syndroms are: lymphocytopenia, hypoplasia, deficiency of lymphocytes in lymphatic tissues normal cortical germinal centres. Recurrent infections with viruses, candida or pneumocystis carinii are common. Tests for assesing the humoral immune status are: measurement of immunglobulin levels in serum, isohaemagglutinins, tests for antibody formation following active immunization, in vitro tests. Clinical syndroms are: no germinal centres in lymphnodes, no plasma cells. Recurrent bacterial infections. Treatment of B-cell deficiencies: immunglobulin replacement therapy. Treatment of T-cell deficiencies by grafting of thymus transplants. Combined immune deficiencies are treated with bone marrow transplants from matched donors. For bone marrow transplantation germfree state seems to be the ideal situation to protect the patient against infection before the therapeutic maneuver and for prevention of graft versus host reaction in the posttransplantation period.

journal_name

Klin Padiatr

journal_title

Klinische Padiatrie

authors

Haas RJ,Niethammer D,Dietrich M,Kleihauer E

subject

Has Abstract

pub_date

1975-11-01 00:00:00

pages

484-95

issue

6

eissn

0300-8630

issn

1439-3824

journal_volume

187

pub_type

杂志文章,评审
  • [Between heaven and hell - children's emotional perception of stemcell transplantation].

    abstract::The following paper is the manuscript of an oral presentation during the annual GPOH meeting on May 22nd, 2000 in Berlin. Following her experiences as a member of the psychosocial care team of a children's cancer unit the author discusses the emotional impact of stemcell or bone marrow transplantation in children. Psy...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2001-16857

    authors: Griessmeier B

    更新日期:2001-07-01 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

  • [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

  • [Infectious complications in the child undergoing hematopoietic stem cell transplantation].

    abstract::Infectious complications are a major cause of morbidity and mortality in pediatric and adult patients undergoing hematopoietic stem cell transplantation. The incidence and the severity code of infections depend on the function of the host's immune system. This function is strongly correlated to the application of immu...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2005-872508

    authors: Schuster FR,Laws HJ

    更新日期:2005-11-01 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

  • [Integration of psychosocial services into the management of the child with cancer].

    abstract::The integration of colleagues from psychosocial occupations into the traditional team of doctors and nurses demands a holistic treatment concept, the general desire for interdisciplinary action, and readiness for patience in the process of mutual learning. The acceptance of the psychosocial services by the patient and...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1026726

    authors: Janssen F

    更新日期:1989-07-01 00:00:00

  • [Lumbar cisternography in the evaluation of hydrocephalus in premature infants].

    abstract::Radionuclide lumbar cisternography with 111In-DTPA was utilized as a bedside procedure to evaluate CSF dynamics and the patency of the ventricular system in 29 preterm infants with hydrocephalus. Serial images of the brain were obtained at 0, 1, 2, 6, 24 and 48 hours after instillation of the isotope in the lumbar sub...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1033955

    authors: Roloff DW

    更新日期:1985-03-01 00:00:00

  • [Giant lymph nodes in combined autoimmune neutro- and thrombocytopenia].

    abstract::A boy aged 15 years is described in whom a combined autoimmune neutro- and thrombocytopenia developed since the age of 11. Cell-membrane bound IgG antibodies were detected on neutrophils and platelets. A therapy with prednisone and/or immunoglobulins showed only a transient normalization of the peripheral blood values...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1033676

    authors: Schneider H,Jobke A,Böhm N

    更新日期:1988-01-01 00:00:00

  • [Heroin withdrawal syndrome in newborns (author's transl].

    abstract::A case of a severe heroin withdrawal syndrom in a newborn infant is reported. Diagnostic and therapeutic aspects of the disease are reviewed. ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:

    authors: Petrich C,Voss HV,Bretschneider A,Göbel U

    更新日期:1976-11-01 00:00:00

  • Congenital Central Hypoventilation Syndrome due to PHOX2b gene defects: inheritance from asymptomatic parents.

    abstract:BACKGROUND:Congenital Central Hypoventilation Syndrome (CCHS, Ondine's curse) is a rare syndrome of dysfunction of the autonomic nervous system characterized by a decreased response to hypercarbia requiring mechanical ventilation in most cases. CCHS is an autosomal-dominant disease associated with tumors of neural cres...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0029-1220941

    authors: Hammel M,Klein M,Trips T,Priessmann H,Ankermann T,Holzinger A

    更新日期:2009-09-01 00:00:00

  • Clinical outcome of patients with childhood acute lymphoblastic leukaemia and an initial leukaemic blood blast count of less than 1000 per microliter.

    abstract:BACKGROUND:One of the strongest predictive factors for therapy outcome in childhood acute lymphoblastic leukaemia (ALL), treated according to ALL-BFM protocols, is the response to initial prednisone treatment. Prednisone response is characterized by the peripheral leukaemic blast count. The threshold value for the char...

    journal_title:Klinische Padiatrie

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1055/s-2001-16848

    authors: Lauten M,Stanulla M,Zimmermann M,Welte K,Riehm H,Schrappe M

    更新日期:2001-07-01 00:00:00

  • [Reference values for the concentration of free thyroxine and free triiodothyronine in the serum of euthyroid children].

    abstract::Concentrations of free thyroxine (n = 341, male = 205, female = 136) and free triiodothyronine (n = 318, male = 198, female = 120) were determined using the radioimmunoassay method in blood serum of euthyroid children. Beyond the 30. day of life no significant differences were found in different age groups for the fre...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1026821

    authors: Liappis N,Starke A

    更新日期:1987-09-01 00:00:00

  • Diagnostic value of immature myeloid information in early-onset bacterial infection in term and preterm neonates.

    abstract:BACKGROUND:For quick detection of neonatal early-onset bacterial infection (EOBI) pro-inflammatory cytokines like Interleukin-6 (IL-6) and Interleukin-8 (IL-8) in combiantion with C-reactive Protein (CRP) have been used. Automated determination of immature myeloid information (IMI) seems to be an additional useful tool...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0034-1395552

    authors: Neunhoeffer F,Dabek MT,Renk H,Rimmele P,Poets C,Goelz R,Orlikowsky T

    更新日期:2015-03-01 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

  • [Echocardiographic diagnosis of congenital diverticuli of the right and left ventricle].

    abstract::A congenital diverticulum of the ventricle is a very rare cardiac lesion which is almost always associated with additional cardiovascular anomalies. In the majority of cases a diverticulum of the heart has been diagnosed during cardiac catheterization for evaluation of additional cardiovascular anomalies. We report no...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2007-1025221

    authors: Wild F,Hofbeck M,Singer H,Hammersen G

    更新日期:1993-05-01 00:00:00

  • [A familial brain malformation syndrome with symptomatic hypernatremia].

    abstract::A familial cerebral malformation combined with chronic hypernatremia is reported. We found three children of a Moroccan family with microcephaly and hypoplasia of the frontal brain. The frontal parts of the lateral ventricles were absent. Two of the children had a defect of the midline structures with fusion of the la...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1033901

    authors: Franek A,Böcker H,Kahlen T

    更新日期:1986-09-01 00:00:00

  • [Neuropsychological sequelae in children with AML treated with or without prophylactic CNS-irradiation].

    abstract:BACKGROUND:In study AML-BFM 87 the relapse rate was lower in patients receiving cranial irradiation (CRT). However, CRT has always been associated with adverse cognitive side effects. Therefore, the impact of CRT on neuropsychological function in children with AML was retrospectively evaluated. PATIENTS:We tested 53 c...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2002-19861

    authors: Reinhardt D,Thiele C,Creutzig U,AML-BFM-Studiengruppe.

    更新日期:2002-01-01 00:00:00

  • [Arthogryposis multiplex congenita with neurogenic atrophy and brachydactyly (author's transl)].

    abstract::Case report of arthrogryposis multiplex congenita with histologically proved neutrogene atrophy of muscles. The also existing reduction deformities of the limbs, showing aplasia and hypoplasia of phalanges may hypothetically be of neuropathic origin. ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:

    authors: Eckel U,Spranger J,Klein H

    更新日期:1979-05-01 00:00:00

  • [The West-Berlin therapy study of acute lymphoblastic leukemia in childhood--report after 6 years].

    abstract::During the last 6 years 73 previously untreated children and adolescents with acute lymphoblastic leukemia were enrolled on a non-randomized therapy protocol. In this longitudinal study the specific accent was put on the intensified induction treatment of 8 weeks' duration which was thought to achieve a higher remissi...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-0033-1337959

    authors: Riehm H,Gadner H,Welte K

    更新日期:2013-05-01 00:00:00

  • [Invagination: diagnosis and conservative therapy].

    abstract::The article reports on the case history of a 8 month old baby who fell ill of a ileocolic intussusception. The barium enema demonstrated the typical cupping in the head of the barium. The successful reposition by hydrostatic pressure was manifest by barium reflux in the small intestine. ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1034383

    authors: Klingmüller V

    更新日期:1983-07-01 00:00:00

  • [Supplementary intravenous nutrition in term and preterm neonates (author's transl)].

    abstract::16 term and preterm neonates received intravenous nutriton for at least three days. The dosage was 1--2 g amino acids, 1 g of fat, and 8--10 g glucose per kg body weight and day. Compatibility was investigated by daily determinations of the amino acid pattern in the serum and measurement of free fatty acids and trigly...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:

    authors: Petrich C,Stephan U,Remy R,von Voss H,Göbel U,Herpertz LM

    更新日期:1979-07-01 00:00:00

  • [Intensive care in pediatrics (author's transl)].

    abstract::Centralized treatment of critically ill pediatric patients and related problems are presented. Pediatric intensive care allows to overbridge deranged or interrupted organ functions while simultaneously treating the disease causing the disturbance. Its efficiency depends on continuity of medical care. Medical and nursi...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章,评审

    doi:10.1055/s-2008-1035616

    authors: Wille L

    更新日期:1980-09-01 00:00:00

  • [Septicaemia in the neonatal and infant period (author's transl)].

    abstract::The paper reported about septicaemia in the neonatal and infant period. Continuing former investigations by Kienitz in the same pediatric clinic we have got a survey from 1933 to 1974. In the last years we saw increasingly more infections with gram-negative organisms, especially by Pyocyaneus with a severe prognosis. ...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:

    authors: Diekmann L,Kotzur J

    更新日期:1976-03-01 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

  • [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

  • [Therapy of iodine deficiency goiter in adolescents with iodine or a combination of iodine and levothyroxine with special reference to lipid parameters].

    abstract::The are various opinions about the optimal therapy of endemic goiter in adolescents. From 106 patients (75 girls) suffering from endemic goiter 50 patients (group A) were treated with 300 micrograms iodine per day and 56 patients (group B) with 100 micrograms iodine plus 100 micrograms levothyroxine per day. Before th...

    journal_title:Klinische Padiatrie

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1055/s-2008-1046460

    authors: Rönnefarth G,Kauf E,Deschner F,Forberger M

    更新日期:1996-05-01 00:00:00

  • [Cerebral AV fistula with cardiac insufficiency in newborns (author's transl)].

    abstract::A case of arteriovenous aneurysm of the great vein of Galen with congestive heart failure in a newborn infant is presented. Efficiancy of medical management and stop of growth of the head circumference point towards a good prognosis. Patients with this malformation have been divided into four clinical groups presentin...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:

    authors: Beitzke A,Zach M,Gypser G,Schreyer H

    更新日期:1977-09-01 00:00:00

  • [Acute erythromelalgia with hypertension in a 5-year old boy].

    abstract:BACKGROUND:Burning pain of red and warm hands and/or feet are the classical symptoms of erythromelalgia. CASE REPORT:We describe the symptoms of acute idiopathic erythromelalgia and arterial hypertension in a five-year-old boy. Five days after a gastroenteritis the patient developed burning hands and feet in combinati...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1043837

    authors: Zenz W,Zöhrer B,Zobel G,Schulze-Bauer C

    更新日期:1999-11-01 00:00:00

  • [Longitudinal data of physical growth of healthy children. III. Circumferences of thorax, upper arm, thigh and calf of children aged 2 to 15 years (author's transl)].

    abstract::From 1968--1978 a longitudinal study was performed concerning development of thorax, upper arm, thigh and calf circumferences in 709 boys and 711 girls 2--15 years old. In boys the mean increase in thoracic circumference amounted to 32.9 cm, in upper are circumference to 8.3 cm, in thigh circumference to 20.6 cm and i...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1033856

    authors: Reinken L,Stolley H,Droese W,van Oost G

    更新日期:1980-01-01 00:00:00

  • [No motivation, no time, no money--does oncologic graduate and continuing education still have a future? Presentation at the 50th Scientific Session of the Society of Pediatric Oncology and Hematology within the scope of the KOK and PSAPOH general session

    abstract::For many years the nursing profession has been considered a dead-end street without any prospects of career advancement or continued qualification. This situation has thoroughly changed. Nursing as an academic specialty has been established in 24 colleges. Furthermore, on-the-job specialty training has become availabl...

    journal_title:Klinische Padiatrie

    pub_type: 杂志文章

    doi:10.1055/s-2008-1043896

    authors: Voss H

    更新日期:1998-07-01 00:00:00