Abstract:
:Hemophagocytic lymphohistiocytosis (HLH) is a systemic inflammatory disorder characterized by uncontrolled histiocytic proliferation, hemophagocytosis, macrophage activation, and up-regulation of inflammatory cytokines (Grom AA., Current opinion in rheumatology 2003; 15: 587-590). HLH is usually divided into two types: primary (familial) HLH and secondary (reactive) HLH. Primary HLH is associated with primary immune deficiencies in which specific gene mutations play an important role, such as perforin defects.
journal_name
Klin Padiatrjournal_title
Klinische Padiatrieauthors
Li Y,Wang Y,Li S,Liu M,Wang D,Xu C,Zhang Ldoi
10.1055/a-1110-7303subject
Has Abstractpub_date
2020-02-25 00:00:00eissn
0300-8630issn
1439-3824pub_type
杂志文章abstract:UNLABELLED:After corrective surgery for congenital heart defects (CHD) many patients suffer from residual defects, some with reduced cardiorespiratory capacity and possible impairment of their health related quality of life (HRQoL). The aim of our study is to evaluate, how children after surgery for CHD rate their HRQo...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0034-1382019
更新日期:2014-09-01 00:00:00
abstract::From 1975-1986 6 boys with congenital nephrogenic diabetes insipidus were diagnosed at the age of 3 months to 10 years. Symptoms appeared within the first few weeks of life. The diagnosis was confirmed by polyuria, low urinary osmolality (97-225 mosm/kg H20), hypernatraemia (max. 171 mmol/l) and the missing response t...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1026741
更新日期:1989-11-01 00:00:00
abstract::Longitudinal growth was assessed in 10 children aged 0.8 to 11.8 years with adrenocortical tumors. All children had signs of androgen excess. In addition, 5 children had glucocorticoid excess, 1 child had estrogen excess. Multiple height recordings, expressed as height standard deviation score for chronological age, w...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025405
更新日期:1991-03-01 00:00:00
abstract::A HPLC-method is described to determine the Pseudouridine/creatinine ratio in spontaneous urine samples in infancy and childhood. The urines of 74 healthy children between 1 and 18 years of age and of 231 children with different diseases were examined for this ratio, making 1097 measurements. 157 children suffered fro...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025295
更新日期:1989-05-01 00:00:00
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
更新日期:1995-11-01 00:00:00
abstract::Low birth weight babies and sick full-term babies, who require total parenteral nutrition and repeated intravenous applications of drugs, which irritate peripheral veins, very often need a reliable central venous catheter. The aim of our paper was to study prospectively the efficiency and the complications of peripher...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1046574
更新日期:1994-01-01 00:00:00
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
更新日期:2005-11-01 00:00:00
abstract:BACKGROUND:The medical care of pediatric cancer patients in the German health care system relies on special structures. All children and adolescents with a diagnosis of cancer receive uniform treatment within clinical studies or registers and exclusively at centers which can ensure interdisciplinary care by a multiprof...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/a-1158-9341
更新日期:2020-11-01 00:00:00
abstract::The aetiology and pathogenesis of the Duchenne-type muscular dystrophy (pseudohypertrophic muscular dystrophy) are still largely unknown. The possibilities of treating the disease are rather limited. Treatment is the more successful, the earlier diagnosis was possible, and the earlier treatment is initiated. The CK-Sc...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:
更新日期:1978-11-01 00:00:00
abstract::In children with primary immunodeficiencies, the onset of symptoms precedes the diagnosis and the initiation of appropriate treatment by months or years. This delay in diagnosis is due to the fact that while these disorders are rare, some of the infections seen in immunodeficient patients are common. Defective antibod...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0033-1354415
更新日期:2013-11-01 00:00:00
abstract::Five patients with arteriovenous angiomas in the head (2 galea angiomas, 1 mixed form with extracranial and intracranial localization of the arteriovenous fistulas and 2 intracranial arteriovenous fistulas) were described. Four patients were examined as newborns or infants. Oximetric data, intracardial pressure and ci...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1976-05-01 00:00:00
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
更新日期:1990-11-01 00:00:00
abstract:BACKGROUND:Essential Thrombocythemia (ET) is an acquired myeloproliferative disorder (MPD) characterized by excessive production of platelets. The disorder is usually affecting adults and is rarely diagnosed in children. PATIENTS AND METHODS:In this retrospective study we describe 12 children aged 5-16 years in whom E...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2004-832356
更新日期:2004-11-01 00:00:00
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
更新日期:2003-09-01 00:00:00
abstract::48 patients between one and seventeen years of age were stimulated with GRF (1-29) NH2. Seventeen of them showed a constitutional delay in growth and puberty, twenty-three suffered from growth-hormone deficiency (GHD) and eight had a suspected GHD as a result of pharmacological tests. Although the results demonstrate ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025510
更新日期:1990-05-01 00:00:00
abstract::We describe two patients with neuraligic amyotrophia of the shoulder: a 15 year old boy and a girl which was 6 years old at the time of primary manifestation. Mostly adolescent patients are concerned. Beginning is acute, the etiology unclear. The patients initially feel intensive pain in one shoulder, which typically ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1046558
更新日期:1995-09-01 00:00:00
abstract:BACKGROUND:Up to now, reliable data has not been available on the actual costs of treating oncological patients. However, such data material is urgently required in view of the institution of the health service reform with the concomitant introduction of the G-DRG remuneration system. PATIENTS:The medical services and...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2003-39379
更新日期:2003-05-01 00:00:00
abstract:UNLABELLED:We present updated results of stem cell transplantation with highly purified stem cells from haploidentical parental donors and infection with human adenovirus (HAdV) post stem cell transplantation (SCT). Survival post SCT is primarily determined by relapse, infections and far less by GvHD or other transplan...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2005-872530
更新日期:2005-11-01 00:00:00
abstract:HINTERGRUND: Die zeitliche Verzögerung zwischen Symptombeginn und Diagnose ist eine Herausforderung in der Behandlung von Kindern mit arteriell ischämischem Schlaganfall. Frühere Studien zur klinischen Präsentation beschäftigten sich v. a. mit kumulativen Symptomen. ZIELSETZUNG: Ziel dieser Studie ist es, mögliche Sym...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/a-0684-9794
更新日期:2018-10-01 00:00:00
abstract::In 55 high-risk neonates of different birth-weight and gestational age, transcutaneous pO2 (tcpO2) was continuously monitored during transports from the Obstetric Units to the Neonatal Intensive Care Units. We were able to demonstrate that the need for oxygen was overestimated in most of the children and thus FiO2 cou...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033907
更新日期:1986-11-01 00:00:00
abstract::An eight-year old girl showing a X/X-translocation (45,X/46,Xt(X;X)(q26;p11)) is presented. Besides short stature and congenital heart-anomaly no other phenotypical abnormalities were present. In all cells containing the abnormal X-chromosome, the latter was late labeling as shown by autoradiography and BrdU-labeling....
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1975-11-01 00:00:00
abstract::Severe anaphylactic reactions were observed in 2 children, aged 2 and 14 years, with acute lymphocytic leukemia and a 12 month old infant with a functional T-cell defect after intravenous administration of a polyethyleneglycol (PEG)-treated immunoglobulin preparation. All 3 children suffered additionally from hypogamm...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1026802
更新日期:1987-07-01 00:00:00
abstract:BACKGROUND:The prognosis of children with multisystem Langerhans cell histiocytosis (LCH) has improved with the application of chemotherapy. However, treatment strategies used varied from conservative approach with treatment only during disease exacerbation to intensive chemotherapy starting immediately after diagnosis...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2000-9667
更新日期:2000-07-01 00:00:00
abstract::The selective uptake and accumulation of 131J-Metaiodobenzylguanidine in neuroblastoma cells in vivo may be utilized for targeted irradiation. The experience with 32 neuroblastoma patients refractory to conventional high dose chemotherapy is reported. At diagnosis 8 patients had Evans stage III and 22 stage IV. 11/32 ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033713
更新日期:1988-05-01 00:00:00
abstract::Mucoid impaction and plastic bronchitis are relatively rare disorders affecting patients of any age. Here we report the case of mucoid impaction developing in a four-year-old child as a complication of pneumonic infection treated four weeks prior. p.a. chest X-ray and fiberoptic bronchoscopy showed atelectasis and the...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2005-836608
更新日期:2006-03-01 00:00:00
abstract:BACKGROUND:Intracellular antibodies (intrabodies) have been used for the generation of phenotypic knockouts in vivo by surface depletion of extracellular or transmembrane proteins. Intrabodies present an alternative to methods of gene inactivation that target genomic DNA or m-RNA, such as RNA interference. Several stud...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,收录出版
doi:10.1055/s-2006-933415
更新日期:2006-05-01 00:00:00
abstract::The long term follow up of 55 children with allergy treated by specific hyposensitization therapy is reported. Hyposensitization therapy had been terminated from 2--7 years previously. The success of treatment was subjectively judged to be satisfactory of good in 62% of the cases. In the group of asthmatic children, a...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1975-11-01 00:00:00
abstract::Glutaracidemia/glutaraciduria type I is an acute or subacute neuropathic disorder of infancy or early childhood. The following symptoms characterize the clinical course: macrocephalus present at birth, cerebral atrophy revealed by CT or MRI scans, most striking in the frontal and temporal lobes, choreoathetosis and dy...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025211
更新日期:1993-03-01 00:00:00
abstract::The progression of alimentary fatty liver to liver cirrhosis is a very rare observation. During the year after surgical extirpation of a suprasellar craniopharyngioma in a seven years old boy developed severe obesity and again six years later at autopsy a complete liver cirrhosis with fatty liver was established. Inju...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1046584
更新日期:1994-01-01 00:00:00
abstract:BACKGROUND:Epidemiology and resistance patterns of bacterial pathogens in pediatric UTI show large interregional variability and rates of bacterial resistances are changing due to different antibiotic treatment. We intended to evaluate data from northern Germany. PATIENTS AND METHODS:In 100 children (53 female, 47 mal...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2004-823143
更新日期:2004-03-01 00:00:00