Abstract:
:Familial glucocortivoid insufficiency--also called hereditary lack of response of the adrenals to ACTH-has not yet been described in Europe. Isolated glucocorticoid insufficiency combined with intact secretion of aldosterone and high plasma level of ACTH are characteristic. The diagnostic difficulties are demonstrated by the deaths before diagnosis of 13 siblings of 21 patients from 9 families. One such family is described. The first of 3 children died at the age of 3-1/2 years after a two day illness interpreted as encephalitis. Another boy, aged 4-1/2 years, had a hypoglycemic attack. 3 months later he died in status epilepticus after a short feverish illness. At autopsy the adrenals were very small. Histologically only the glomerulous zone was developed. An the hypophysis there was hyperplasia of the R cells producing ACTH. The 6 year old sister had been pronouncedly pigmented from the age of a few months onwards. Age in terms of height and bone development corresponded to 8-1/2 and 7-1/2 years. After intravenous insulin and synacthen plasmacorticoids could not be found. With normal and low supply of salt, the following data were found: Renin-activity 2.9 and 5.6 ng/ml p.h. respectively; rate of aldosterone secretion 62.6 and 151.5 mug/24 hrs.; average aldosterone plasma concentration 9.39 and 27.7 ng/100 ml respectively; MCR 666 and 5471 p/24 hrs. Plasma ACTH in this patient (5086--7200 pg/ml) and, post mortem, in her brother (8100 pg/ml), were extremely reaised.
journal_name
Klin Padiatrjournal_title
Klinische Padiatrieauthors
Petrykowski W,Burmeister P,Böhm Nsubject
Has Abstractpub_date
1975-05-01 00:00:00pages
198-215issue
3eissn
0300-8630issn
1439-3824journal_volume
187pub_type
杂志文章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...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/a-1110-7303
更新日期:2020-02-25 00:00:00
abstract::According to the theories of Schachter concerning the external response of obese adults, which have been confirmed by Jung, Pudel and Nisbett, we tested these theories in obese adolescents. Eating behaviour of 16 grossly obese adolescents (8 males, 8 females, mean age 13.2 +/- 0.3 years, mean relative body weight 124 ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-1025512
更新日期:1990-05-01 00:00:00
abstract:AIM:Reports describing coronavirus disease 2019 (Covid-19) in children are fewer than adult studies due to milder clinical picture. We aimed to share our experience at a single center with an emphasis on collective decision making. MATERIALS AND METHODS:A suspected case was defined as the presence of symptoms suggesti...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/a-1263-1222
更新日期:2021-01-01 00:00:00
abstract:BACKGROUND:Due to rising survival rates in cancer and autoimmune diseases fertility preservation before gonadotoxic therapies has become increasingly important. Although fertility can be significantly affected by gonadotoxic therapies, the possibility of fertility preservation during childhood has not been sufficiently...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0044-100396
更新日期:2018-02-08 00:00:00
abstract::In a retrospective study we evaluated clinical features and diagnostic impact of sonography, white blood cell count, C-reactive protein as well as bacterial etiology of children treated surgically for bacterial cervical lymphadenitis. We report on 38 children at the age between 2.5 months and 14 years who were admitte...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2003-41403
更新日期:2003-07-01 00:00:00
abstract:BACKGROUND:Most recent studies analyzing candidate biological prognostic factors in childhood medulloblastoma (MB) are limited by small patient numbers due to dependence on fresh-frozen tumor material. By contrast, large archives of formalin-fixed, paraffin-embedded MB samples exist from homogeneously treated patients....
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2007-985843
更新日期:2007-11-01 00:00:00
abstract::Cytological examination of cerebrospinal fluid needs special techniques of cell collecting and staining. On condition of realizing the methodical requirements cytology of the cerebrospinal fluid brings more advancement in diagnostics than other laboratory examinations. The advantages of this procedure are: 1. Meningit...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1975-03-01 00:00:00
abstract:BACKGROUND:Lupus vulgaris (LV) is the most common form of cutaneous tuberculosis (TB) in Europe, nevertheless the overall incidence is low. It constitutes about 1.5% of all extra-pulmonary cases worldwide. A slight raise in TB incidence rates among children was recently registered in Germany, which can be explained by ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,评审
doi:10.1055/s-0033-1343412
更新日期:2014-01-01 00:00:00
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
更新日期:1981-07-01 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::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::With the advent of prenatal diagnosis and the feasibility of intrauterine treatment, non-immune hydrops fetalis (NHIF) is gaining increasing importance. 14 patients are reported 10 of whom having been diagnosed prenatally. 13 were born prematurely severe asphyxia being a frequent occurrence. 7 out of 14 infants surviv...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033984
更新日期:1985-07-01 00:00:00
abstract::A case of an infant with a mediastinal neurenteric cyst is used to review this rare entity. By definition a neurenteric cyst is the combination of an entodermal cyst with a vertebral dysplasia. Neurenteric cysts are located in the posterior mediastinum, preferentially on the right side. In a third of the patients thes...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1026749
更新日期:1987-01-01 00:00:00
abstract::Problems of terminology, differential diagnosis, etiology and therapy of language development disorders are demonstrated by a casuistic example. Besides the importance of clinical date special attention was laid on neuropsychological diagnostic procedures and their therapeutic implications. Therapeutic interventions c...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:
更新日期:1978-09-01 00:00:00
abstract::Hypotension is diagnosed in a high percentage of very low birth weight infants, particularly in the first 24 hours after birth and the resultant clinical approach is to support the blood pressure vigorously. However, the research base is not yet available to give a definite answer to the question, which blood pressure...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2003-36890
更新日期:2003-01-01 00:00:00
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
更新日期:2001-09-01 00:00:00
abstract::An increasing number of children suffering from cancer has been treated successfully during the last 25 years using therapy protocols of the Gesellschaft für Pädiatrische Onkologie und Hämatologie. The patients had to undergo an interdisciplinary treatment modality and a mostly intensive chemotherapy. Late effects cou...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1046537
更新日期:1995-07-01 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::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
更新日期:1987-09-01 00:00:00
abstract::We identified betulinic acid (BetA) as a new cytotoxic agent active against neuroectodermal tumor cells including neuroblastoma, medulloblastoma, glioblastoma and Ewing's sarcoma cells representing the most common solid tumors of childhood. BetA induced apoptosis independent of wild-type p53 protein and accumulation o...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1043808
更新日期:1999-07-01 00:00:00
abstract::The secretory IgA system is common to all mucosal membranes and is presumably of great importance for their defense. In addition to the secretory IgA antibodies produced in a mucosa in response to a local antigenic stimulus there is a spread of this type of IgA response via committed lymphocytes. They originate from c...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1033997
更新日期:1985-07-01 00:00:00
abstract::Members of the histone deacetylase (HDAC) family exhibit great promise as potential drug targets in pediatric tumors including neuroblastoma, medulloblastoma, ependymoma and Ewing's sarcoma. HDAC inhibitors of various structural classes have shown anti-tumoral effects in pre-clinical pediatric tumor models as single a...
journal_title:Klinische Padiatrie
pub_type: 杂志文章,多中心研究
doi:10.1055/s-0032-1323692
更新日期:2012-10-01 00:00:00
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
更新日期:1985-03-01 00:00:00
abstract::A two month old boy with Fournier's Gangrene of the scrotum as a result of unnecessary needle puncture of the hydrocele is being reported. The importance of appropriate diagnosis operation and drug-therapy is also described. ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1034104
更新日期:1984-11-01 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::In a prospective investigation, a rapid latex test for Helicobacter pylori in the serum (Pyloriset) was carried out in 39 patients with recurrent abdominal pain aged from six to 15 years. The test was positive in 19 patients. All of these children were subjected to gastroduodenoscopy. Seven cases showed a Helicobacter...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1046500
更新日期:1995-01-01 00:00:00
abstract::A severe hyperammonemia is the characteristic finding in patients with enzyme defects in urea cycle and one of the main causes of the acute metabolic crisis dsuring the newborn period and infancy. A case report is given about two male infants, who died in the age of one and of seven months respectively. In the second ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2008-1035594
更新日期:1980-05-01 00:00:00
abstract:QUESTIONS:Psychosocial variables like stress or a high achievement motivation are discussed as a possible contributory factor to an attack in migraine. The aim of our study was to examine, whether children with migraine may be at risk for impaired achievement motivation. PATIENTS/METHODS:37 children with migraine and ...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-2004-822810
更新日期:2005-07-01 00:00:00
abstract:BACKGROUND:Vaccination with 2 doses of > 95% of the population is necessary to eliminate measles. In Switzerland and especially in the central part, vaccine coverage is low (2006: 65%). This led 2006-2009 to a measles epidemic with thousands of cases and high costs. One death was noted in a formerly healthy 12 year old...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0033-1363254
更新日期:2014-01-01 00:00:00
abstract::Inherited disorders of platelets constitute a group of rare diseases that give rise to bleeding syndromes of variety severity, with more severe cases being first diagnosed during infancy and childhood. To appropriate diagnose a platelet function disorder during early childhood the knowledge of the physiological charac...
journal_title:Klinische Padiatrie
pub_type: 杂志文章
doi:10.1055/s-0030-1249065
更新日期:2010-05-01 00:00:00