[Juvenile chronic arthritis].
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Adolescent↗
Biomedical subjects
W Stögmann
Publications and source records attributed to W Stögmann.
[The history of the Gottfried von Preyer pediatric hospital: from a military hospital to a modern medical center for children].
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Austria↗
[Recommendations for the treatment and prevention of atopic dermatitis in children].
Atopic dermatitis (AD) is a chronically-relapsing disease of the skin caused by hereditary hypersensitivity to a lot of environmental substances. Disturbances of humoral and cellular immunity, abnormal vascular reactions and abnormal skin functions play a role in pathogenesis. AD shows different forms in different age-groups with pruritus as a commune feature. The treatment of AD is unsatisfactory and effective only by consequent cure. The principles are avoiding of pruritus and scratching, avoiding of chemical and mechanical irritants of the skin and avoiding of bacterial superinfections. Topic steroid-treatment is very effective, dose and application however have to be strongly controlled. The nutrition of children with AD should be as normal as possible, extreme regimes of diet have to be avoided. An effective prophylaxis of atopic disease is not known till now.
Child↗
[Iodine deficiency in childhood despite prevention. Current data from the Vienna area].
Since 1963 iodized table salt prophylaxis (10 mg KI/kg table salt) in Austria has led to a decrease in endemic goitre in children. Nevertheless, goitre frequency is rising again after the first two decades of life and iodine deficiency is found in 50% of the patients. In order to evaluate the adequacy of the iodine supply in childhood in the Vienna area the urinary iodine excretion was determined in 124 healthy non-goiterous children from 4 weeks to 16 years of age. Our investigation demonstrates a decrease in urinary iodine excretion in the older children (iodine excretion 400 micrograms/per g creatinine in the first year of life versus 96 micrograms I/per g creatinine after the age of 10, p less than 0.001). At the same time the number of children showing grade I iodine deficiency (according to WHO criteria) increases with age, so that more than 50% of children older than 10 years in the Vienna area have an insufficient iodine supply (p less than 0.0001). Thus, the iodine supply should be improved by increasing the level of iodization of table salt to 20 mg KI/kg and by the more frequent intake of food naturally containing a higher iodine concentration.
Adolescent↗
[The current treatment concept of Turner syndrome].
Turner's Syndrome (XO Karyotype or XO/XX mosaicism) affects 1 in 2500 females. It results in short stature (mean adult height is about 146 cm), infertility and the lack of secondary sexual characteristics. Hormone replacement therapy to develop secondary sexual characteristics has been used for years whereas several growth promoting agents undergo clinical trials at the moment. Our current management for patients with Turner's Syndrome includes the induction of puberty at a bone age of 11 to 12 years with low dose oestradiol and the application of biosynthetic human growth hormone (hGH) when growth velocity falls below the normal range. In our group of previously untreated patients (4.6-14 years) one year results of hGH application (12-18 IU/m2/week 7 equal doses sc/week) show a clear improvement of growth velocity (3.43 + 0.44 cm/year before therapy versus 6.2 + 1.14 cm/year after the first year of treatment, p less than 0.001). The growth promoting effect of hGH therapy might further be improved by the combination with anabolic steroids like oxandrolon.
Adolescent↗
[The radiation accident].
The reactor accident of Chernobyl in April 1986 has shown us all the dangers which are inherent ever in the peaceful use of atomic energy. The effects of exposure to ionizing radiation are dependent on biological effectiveness, on dose, on duration of exposure and on the age of the exposed person (the younger the graver). Acute ionizing radiation of the whole body leads to radiation disease or radiation syndrome of different stages of severity according to dosage. If the patient survives other consequences of ionizing radiation may arise: non-stochastic effects such as cataracts, keloid formation, fibrosis of the lungs and infertility) and stochastic effects (oncogenesis and mutagenesis). The sensitivity to ionizing radiation is especially high in childhood because of the high velocity of cell metabolism and cell growth, the large body-surface area and because their repair mechanism following radiation damage is not yet.
Accidents↗
[You make the diagnosis. Caudal regression syndrome].
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Abnormalities, Multiple↗
[Dwarfism in the Alagille syndrome caused by somatomedin C deficiency?].
Alagille syndrome (= arterio-hepatic dysplasia) is a rare congenital syndrome consisting of cholestasis with paucity of intrahepatic bile ducts, pulmonary artery stenosis, skeletal anomalies and typical facies. Growth retardation, which is not correlated with vertebral anomalies or the degree of cholestasis, occurs in about two thirds of patients. We report on hormonal aspects of growth retardation in an 8 year old Austrian boy with the typical features of arteriohepatic dysplasia. Thyroid function and a cortisol profile were normal and we found normal HGH response to insulin stimulation. The Somatomedin C-activity was well below the age-adjusted normal range. Even after test-induced HGH peaks no increase in Somatomedin C-activity could be observed. A six month course of phenobarbital-, cholestyramine- and D-penicillamine-therapy led to significant improvement of cholestasis, however Somatomedin C values and growth velocity remained unchanged. Results in our patients show that Somatomedin C-deficiency might be an important cause of growth retardation in children with chronic liver disease, at least in arteriohepatic dysplasia.
Bile Ducts↗
[Epileptic seizures in childhood: classification, diagnosis, therapy].
This is a review about epilepsies of childhood dealing with the elementary principles in classification, diagnosis and treatment. In each case of a patient suffering with epilepsy 3 diagnoses have to be made: the diagnosis of the seizure-type, the diagnosis of the epilepsy-type, the diagnosis of the etiology. The seizure-type has to be diagnosed as generalized (e.g. grand mal, absence seizure) or partial (simple or complex) which can be obtained from the clinical picture and EEG. The classification of seizures is different from the classification of epilepsies: recurrent seizures, only a single type or more types in the same patient, make the diagnosis of the epilepsy-type. The etiologic diagnosis is often not possible to make and requires careful judgment in the use of special procedures; however, the diagnosis of the seizure-type includes their etiology in as much as, for example, awake-grand mal and absence seizures can be classified as idiopathic resp. sleep-grand mal and partial seizures as symptomatic. The most essential principle for the treatment of epilepsies is: the seizures diagnosis determines the appropriate therapy. The drugs of choice for partial seizures and most cases with grand mal are phenytoin and/or carbamazepine; absence seizures and benign myoclonic seizures highly respond to valproate; atonic seizures are most resistant to therapy. To make an effective treatment you have to know the dose, the therapeutic plasma drug level, the drug's half life and the side-effects. The aim of epilepsy-treatment has to be: avoidance of seizures without side-effects.
Anticonvulsants↗
[Epidemiology and clinical course of perinatally acquired hepatitis B infection].
A report is presented on 10 children with perinatally-acquired hepatitis B virus infection. In 9 cases the mother was most probably the source of infection (vertical transmission of hepatitis B), whilst one child was infected via repeated blood transfusion. 7 mothers were of south-eastern European and/or Anatolian origin. Only 2 mothers suffered from clinically apparent liver disease (one had chronic-aggressive hepatitis with positive HBs-antigen and the other had acute hepatitis B). All the others were healthy hepatitis B carriers. Of 7 mothers examined 2 were HBe-antigen positive, and 5 were anti-HBe positive. The clinical course of infection in children varied: 2 children developed subclinical infection, 2 developed acute hepatitis B, which was fulminant with a fatal outcome in one. 6 children showed antigen persistence (HBs-antigen carriers) over the whole period of observation. In one child this antigen persistence is associated with cirrhosis of the liver, 3 children suffer from chronic-persistent hepatitis B, 2 children are healthy carriers; all children are HBe-antigen positive and, thus, seem to be highly infectious. Apart from the risk to the individual, perinatal hepatitis B infection in a certain population. Hence, preventive measures are indicated and should be carried out in form of HBs-antigen screening of pregnant women - at least of high-risk groups - and active-passive immunization of the newborns at risk. The indication for immunization should not depend on the HBe-antigen status of HBs-antigen positive mothers.
Austria↗
Fructose-1,6-diphosphatase deficiency: glycerol excretion during fasting test.
A Turkish boy had suffered since the age of 10 months from recurrent attacks of severe metabolic acidosis and hypoglycaemia precipitated by moderate respiratory tract infections. A liver biopsy showed lack of fructose 1,6-diphosphatase and absence of phosphorylase. The patient died in shock following fructose ingestion. Upon fasting, acidosis with increased lactate and glycerol excretion was found. Findings indicate that, in this inherited disorder of gluconeogenesis, lactic acidosis combined with increased glycerol excretion upon fasting are of diagnostic importance.
Acidosis↗
[Vitamin D deficiency rickets: single-dose therapy versus continuous therapy].
This is a comparative study of massive single dose treatment (400 000 I. U. vitamin D3, divided in 2 X 200 000 I. U. on day 1 and day 3, orally) versus continuous treatment (9600 I. U. vitamin D3-drops for 18 days) of vitamin D-deficiency rickets (VDR). To estimate the effectiveness of these two kinds of treatment the serum concentrations of calcium (Ca), phosphorus (P), alkaline phosphatase (aP), calcitriol (25-OH-CC), parathyroid hormone (PTH) and calcitonin (Ct) before and during treatment (on day 3, 7, 14, 21) were studied in 10 patients, 5 in each group, with VDR. There was no difference in the course of serum concentrations of Ca, P and aP in these two treatment-groups. The elevated PTH returned more quickly to normal range in the single dose study group. Ct-serum concentrations showed too different courses in the studied patients, therefore no comparison could be made. 25-OH-CC showed a very quick increase in the single dose-group and a slower one in the continuous treatment group the climax in both groups reached a value far over the tentative range. According to these results an unequivocal better effect of one of these two compared VDR-treatments could not be seen, what means that both treatments can be recommended.
Alkaline Phosphatase↗
[Beta-HCG-producing choriocarcinoma of the pineal area as a cause of precocious puberty].
We report the case of an eight-year-old boy with precocious puberty due to a beta-HCG producing choriocarcinoma of the pineal region. beta-HCG-levels were elevated initially (9659 mU/ml) and did not normalize completely after surgical removal of the brain tumor. A relapse of the tumor in the hypothalamic region led to a new increase of beta-HCG-level up to 41258 mU/ml. Histopathology of a subsequently removed metastasis in the right lung showed immunohistological evidence of beta-HCG production. Introduction of chemotherapy was followed by a normalization of the beta-HCG-level and of the previously elevated hormone values.
Brain Neoplasms↗
[Transient hypoplasia of the intrahepatic bile ducts. Clinical and morphologic studies].
Two boys were thriving normally up to the age of three and nine weeks respectively when obstructive cholestasis developed. Liver biopsy revealed paucity of intrahepatic bile ducts. Extrahepatic bile ducts were not occluded. In both cases cholestasis completely disappeared after five and nineteen weeks respectively. Control liver biopsis showed normal numbers of intrahepatic bile ducts. We conclude that paucity of intrahepatic bile ducts may be transient.
Bile Ducts, Intrahepatic↗
[Differential diagnosis of hypokalemia--Bartter's syndrome: clinical aspects and therapy].
The case histories of 2 children with the typical characteristics of Bartter's syndrome are reported. The differential diagnosis from other forms of hypokalaemia, as well as pathophysiology and clinical manifestations of this syndrome are presented. The therapeutic effect of indomethacin, an inhibitor of prostaglandin synthesis, is discussed; side effects of this treatment are pointed out.
Adolescent↗
[Recommendations for local therapy of common dermatoses in children].
Recommendations for topical dermatologic therapy of the most common skin diseases in infancy and childhood are given with tabular summaries. They are concerned with diaper dermatitis, seborrhoeic dermatitis, atopic dermatitis, scabies and pediculosis. Special attention is given to topical steroid therapy in seborrhoeic and atopic dermatitis and to the possible neurotoxic side-effects of Lindan, the most effective substance against scabies and pediculosis.
Administration, Topical↗
[Inhibition of prostaglandin synthesis during the therapy of nephrogenic diabetes insipidus].
A report is presented of a male infant with nephrogenic diabetes insipidus type I. A low-solute-load diet and large fluid intake were not able to prevent hypertonic dehydration and to control the disease. It responded within 3 days to indomethacin (3 mg/kg body weight), a prostaglandin synthetase inhibitor. The boy is doing well on this regimen and no side-effects have been observed after 2 years of treatment. Hitherto only a few case reports have been published on this therapeutic regimen, but all confirm the high efficacy of prostaglandin synthesis inhibition as the treatment of choice in nephrogenic diabetes insipidus.
Diabetes Insipidus↗
[Pseudohypoparathyroidism: a generalized and multiple disorder in the adenyl cyclase system].
Pseudohypoparathyroidism (PHP) is caused by non response of target organs (kidney and bone) to parathyroid hormone because of defects in adenylate cyclase-system. The often seen combination of other endocrine abnormalities with PHP speak for a generalized defect of hormonsensitive adenylate cyclases, but not for isolated resistance to parathyroid hormone. At least two defects are known: in Typ I-PHP the defect is before the formation of cyclic AMP (cAMP) because of a reduced activity of receptor-cyclase coupling protein. In Typ II-PHP the defect lies behind cAMP-formation (defect in the reception of cAMP-signal?).
Adenylyl Cyclases↗