Wiener klinische Wochenschrift
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Wien. Klin. Wochenschr. · Jan 1992
Review[Differential diagnosis and therapy of various forms of hyperphenylalaninemia: facts and fiction].
The various conditions which can lead to elevated blood phenylalanine (PHE) levels must be differentiated promptly in the neonatal period so that the correct treatment can be implemented as soon as possible. In order to exclude the rare tetrahydrobiopterin (BH4) deficiency, it is advisable to perform a BH4 loading test and to determine the renal excretion of pterins, as well as the dihydropteridine reductase activity in erythrocytes. The practical consequence of differentiating the various types of PHE hydroxylase deficiency is that with both phenylketonuria (PKU; PHE greater than 20 mg/dl) and hyperphenylalaninemia (HPA) with PHE levels above 15 mg/dl a diet restricted in PHE is initiated, whereas HPA infants with PHE levels below 8 mg/dl are fed normally. ⋯ Psychometric investigations of PKU patients after diet discontinuation at different ages, as well as animal studies are in favour of a diet-for-life. The diet in PKU patients is known to lead to some side effects such as bony changes and amino acid imbalance, as well as deficiency of selenium and carnitine. Finally, great efforts have to be made in order to avoid the increasing danger of PHE embryofetopathy in the offspring of PKU mothers (maternal PKU).
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Wien. Klin. Wochenschr. · Jan 1992
Randomized Controlled Trial Multicenter Study Clinical Trial[The Hypertension Optimal Treatment Study. Background information].
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Wien. Klin. Wochenschr. · Jan 1992
Case Reports[Alcoholic ketoacidosis--3 episodes in one patient].
3 episodes of alcoholic ketoacidosis were observed in one female patient over a period of 19 months. The clinical picture consisted of vomiting, dehydration, hyperventilation and abdominal pain. Predominant laboratory findings were acidosis (pH less than 7) and hyperglycaemia, with blood glucose values of 354, 330 and 147 mg/dl. ⋯ The picture of ketoacidosis in mostly chronically malnourished alcoholics reflects not only the complex abnormalities of acid-base balance caused by excessive cumulation of ketoacids, but also the related severe depletion of electrolytes and extracellular volume. Adequate acute therapy (as for diabetic ketoacidosis) and thorough follow-up treatment of any concurrent conditions result in rapid reversal of the syndrome in most cases. Since there are few reports of repeated episodes in one patient, an overview of this disorder is presented concerning management and differential diagnosis of the basis of our case report.
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Wien. Klin. Wochenschr. · Jan 1992
The diagnostic impact of magnetic resonance imaging on the evaluation of suspected spinal cord disease.
The data from 262 patients studied consecutively for suspected spinal cord disease were analysed to determine the utility of magnetic resonance imaging (MRI) in this clinical setting. Damage to the spinal cord was detected in a total of 188 (72%) patients and was caused by myelocompression in two thirds and by intramedullary lesions in one third of the patients. ⋯ It is concluded that MRI is the procedure of choice for assessing patients with medullary symptoms. Irrespective of localizing neurologic findings the evaluation of the entire spine and even of the brain may be necessary to obtain maximum diagnostic information.
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Wien. Klin. Wochenschr. · Jan 1992
[New perspectives of avalanche disasters. Phase classification using pathophysiologic considerations].
This study comprises an analysis of the data on 332 persons totally buried by avalanches in Switzerland between 1981 and 1989. The survival rate was calculated with the aid of a computer-assisted estimation procedure according to Turnbull. The curve pattern was interpreted according to pathophysiological considerations, on the basis of which the time course of the battle for survival was divided into 4 phases: 1) Survival phase: until 15 minutes after burial under the snow masses. ⋯ With sufficient oxygen reserves and freedom of thoracic movement a "phase of relative safety" occurs, whereby the survival probability diminishes further only slowly. The first deaths due to hypothermia arise after 90 minutes. 4) Rescue phase: from the time of extrication from the snow until arrival in hospital. There is an increased risk of a fatal outcome during the rescue procedure and immediately afterwards through augmented hypothermia.(ABSTRACT TRUNCATED AT 250 WORDS)