Abstract:
BACKGROUND:For Thrombasthenia Glanzmann (GT) patients presenting with a severe clinical phenotype due to complete lack of thrombocyte function or increased titres of anti-platelet antibodies hematopoietic stem cell transplantation (SCT) is the only curative therapy. CASE REPORT:We report the case of a 13-month-old boy, presenting with a severe course of GT, who was successfully treated with an HLA-identical sibling bone marrow transplant. SCT was complicated by anti-platelet alloimmunization after platelet transfusion successfully treated with high dosage immunoglobulins (2 g/kg) and partial plasma exchange. CONCLUSION:SCT may be a viable option for selected patients with GT. However, SCT in GT carries its own significant risks, resulting from the development of anti-platelet antibodies. A critical risk-benefit analysis is mandatory prior to SCT.
journal_name
Klin Padiatrjournal_title
Klinische Padiatrieauthors
Wiegering V,Winkler B,Langhammer F,Wölfl M,Wirbelauer J,Sauer K,Kobsar A,Meyer T,Strauss A,Bakchoul T,Eyrich M,Schlegel PGdoi
10.1055/s-0031-1273726subject
Has Abstractpub_date
2011-05-01 00:00:00pages
173-5issue
3eissn
0300-8630issn
1439-3824journal_volume
223pub_type
杂志文章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::Prolactin levels were measured immediately after the seizure in some, and 15 to 20 minutes later in all of 67 children aged between 6 months and 17 years. Values were determined after grand mal, complex partial and petit mal seizures and psychogen seizures. A more than 2 to 3 fold prolactin increase over the baseline ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025341
更新日期:1992-05-01 00:00:00
abstract::The article describes the method of counter-current immunoelectrophoresis as a rapid and safe technique for the identification of bacterial antigens in body fluids. This technique has proved very useful especially in patients who had been pretreated with antibiotics, since the culture yields are usually negative in su...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034426
更新日期:1981-01-01 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::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
更新日期:1987-11-01 00:00:00
abstract::Analysis of the cellular DNA content was carried out in 162 children with ALL and 34 children with AML admitted to the university children's hospital Münster between 1979 and 1984. DNA aneuploidies were identified at a similar frequency in ALL (40%) and AML (44%). However, the degree of DNA aneuploidies (DNA-index) wa...
journal_title:Klinische Padiatrie
pub_type: 临床试验,杂志文章
doi:10.1055/s-2008-1033972
更新日期:1985-05-01 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: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
更新日期:2010-09-01 00:00:00
abstract:BACKGROUND:Gender differences in overall neonatal survival and in short term pulmonary outcome have been reported. Furthermore gender differences in childhood chronic lung disorders have been described all in favor of females. METHODS:A typical survey on published data regarding gender differences in lung development ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-0033-1333758
更新日期:2013-03-01 00:00:00
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
更新日期:1992-09-01 00:00:00
abstract::Internal hydrocephalus follows intraventricular hemorrhage in about 10%. Progression is directly related to the degree of hemorrhage. Several studies confirmed that cerebral damage may occur without an increase of intracranial pressure or head circumference. Assessment and therapeutic consequences depend entirely on s...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033725
更新日期:1988-07-01 00:00:00
abstract::15 parameters of coagulation and fibrinolysis were investigated in 38 children with type I diabetes mellitus without clinical signs of diabetic angiopathy. Compared to an age matched non diabetic control group spontaneous platelet aggregation was enhanced, plasma levels for factor VIII C, von Willebrand factor, antith...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025468
更新日期:1991-11-01 00:00:00
abstract::The synchronous occurrence of blasts of the lymphatic and myelogenous lineage in acute leukemia has been rarely observed. Reviewing individual case reports it is suggested that malignant transformation already starts at the level of the stem cell. We are reporting a case of a 13-year old boy who initially showed morph...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034453
更新日期:1981-05-01 00:00:00
abstract:BACKGROUND:The role for testis-sparing surgery in the treatment of primary intratesticular lesions in childhood is growing. The reliability of scrotal ultrasonograpy (US) in the management of these lesions is still controversial. PATIENTS:Between 1991 and 2007, 383 children and adolescents presented with testicular ab...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0031-1271813
更新日期:2011-05-01 00:00:00
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
更新日期:1995-05-01 00:00:00
abstract::Reference values for 327 children aged between 1 day and 15 years are given for the revised standard method for the determination of serum creatine kinase (CK) activity by activation with N-acetylcysteine (NAC). No sex-dependence could be observed, but CK activity was found to vary markedly with age. The following val...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1035606
更新日期:1980-07-01 00:00:00
abstract::"Lack of space" in utero is considered to be a major factor in the aetiology of the congenital dislocation of the hip. This study tries to answer the question whether hypertrophy of a newborn has to be regarded as a risk factor on the basis of the principle mentioned above. The results of postnatal clinical and sonogr...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025256
更新日期:1993-11-01 00:00:00
abstract::Report on a case of acute childhood leukemia, who presents with the following exceptional features: During complete remission early bilateral leukemic infiltrations of the testes, followed--after an intervall of several months--by a serve, general relapse with ascites. New induction therapy resulted in a second comple...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1979-01-01 00:00:00
abstract::Positive anti-DNA-antibodies and lowered C3 and C4 levels were found in serum of a 13 year old girl presenting with edema, microscopic hematuria and proteinuria. Renal biopsy revealed diffuse endo- and extracapillary glomerulonephritis with mesangial deposits of IgA, IgG, IgM, C1q, C3 and fibrinogen. In spite of treat...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034411
更新日期:1983-11-01 00:00:00
abstract:BACKGROUND:The considerable increase of obesity in children and adolescents poses a major challenge to the health care system. METHODS:In an observation study of the Bundeszentrale für gesundheitliche Aufklärung (BZgA) somatic data of 1916 overweight and obese children and adolescents aged 8-17 years were compared to ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0031-1280766
更新日期:2011-12-01 00:00:00
abstract:BACKGROUND:Sonographic screening helps to discover pathological findings in asymptomatic patients. In these cases the decision about diagnostic and therapeutic consequences is difficult. CASE REPORT:We describe a girl with a congenital small solitary hepatic cyst with rapidly increasing size after the second year, whi...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2002-35364
更新日期:2002-11-01 00:00:00
abstract::Autoimmune hemolytic anaemia (AIHA) in childhood is associated with antibodies produced by the patient himself, which coat his red cells causing their hemolysis. Although in some cases no underlying disease could be found, in the majority of children a virus etiology is apparent. There are very few reports regarding t...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1977-05-01 00:00:00
abstract::The best nutrient for newborn babies is breast milk and preferably provided via breast feeding. Pooled human milk must be used when the babies' own mother's milk is not available. Very often pooled milk is pasteurised. Recent studies, however, suggest avoidance of any sterilisation procedures in view of the decreased ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033821
更新日期:1982-09-01 00:00:00
abstract:UNLABELLED:We present an unusual case of neonatal liver failure. Isolated ascites was diagnosed in a female fetus at week 34 gestational age upon routine ultrasound. In the 35th week of gestation a cesarean section was carried out after puncture of fetal ascites. After birth the patient showed symptoms and complication...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-970591
更新日期:2008-01-01 00:00:00
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
更新日期:2001-07-01 00:00:00
abstract:BACKGROUND:Current resuscitation guidelines recommend the use of simulation-based medical education (SBME) as an instructional methodology to improve patient safety and health. We sought to investigate the evidence-base for the effectiveness of SBME for neonatal and pediatric resuscitation training. METHOD:Therefore, ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-0034-1372621
更新日期:2014-09-01 00:00:00
abstract:BACKGROUND:Pachyonychia congenita is considered to be a genodermatosis of autosomal inheritance. It is characterized by nail hypertrophy, shortly present after birth. Later on follicular keratosis of the extremities and hyperkeratosis of palms and soles can be found. HISTORY AND CLINICAL FINDINGS:We report a child wit...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1043783
更新日期:1999-05-01 00:00:00
abstract::Hereditary Factor VII deficiency is one of the rare congenital coagulopathies. Prolonged prothrombin time (PT) with normal partial prothrombin time (PTT) may be an indicator for Factor VII deficiency. A family with hereditary heterozygous Factor VII deficiency is presented in whom no symptoms of a bleeding disorder we...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025388
更新日期:1992-11-01 00:00:00
abstract::Transfusion-induced hemochromatosis remains a major therapeutic complication in the management of patients with chronic hemolytic anemias. Red cell units harvested with blood cell separators have an average estimated mean cell age of 30 days, compared to 60 days for unfractionated blood. Our initial experience with tr...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033936
更新日期:1985-03-01 00:00:00
abstract::The Thorax-Tube according to Matthys and its successful application in 18 premature and mature newborns and older children is described. The advantages of this new device are discussed. ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1977-03-01 00:00:00