Wiener klinische Wochenschrift
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Wien. Klin. Wochenschr. · Dec 2012
Case ReportsClinically symptomatic heterozygous carnitine palmitoyltransferase II (CPT II) deficiency.
Two symptomatic patients with heterozygous carnitine palmitoyltransferase II (CPT II) deficiency are reported. Patient 1, a 21-year-old female professional tennis player, suffered from exercise-induced attacks of muscle pain, burning sensations and proximal weakness. Patient 2, a 30-year-old male amateur marathon runner developed muscle cramps and rhabdomyolysis upon extensive exercise and insolation-induced fever. ⋯ No second mutation could be found upon sequencing of all the exons of CPT2 gene including exon-intron boundaries. Biochemically, residual CPT activity in muscle homogenate upon inhibition by malonyl-CoA and Triton-X-100 was intermediate between controls and patients with mutations on both alleles. Although CPT II deficiency is an autosomal recessive disorder, the reported patients indicate that heterozygotes might also have typical attacks of myalgia, pareses or rhabdomyolysis.
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This position statement reflects the opinion of the Austrian Diabetes Association concerning the perioperative management of patients with diabetes mellitus based on the available scientific evidence. The paper covers necessary preoperative examinations from an internal/diabetological point of view as well as the perioperative metabolic control by means of oral antidiabetics and/or insulin therapy.
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Wien. Klin. Wochenschr. · Dec 2012
ReviewEuropean Competence Network on Mastocytosis (ECNM): 10-year jubilee, update, and future perspectives.
The European Competence Network on Mastocytosis (ECNM) was initiated in 2002 as a multidisciplinary and multinational cooperative approach to increase awareness and to improve diagnosis and therapy of mastocytosis. The network is composed of local centers, physicians, and scientists who have dedicated their work to patients with mastocytosis. A strategic goal of the ECNM is to provide the best available information about the disease to patients and physicians. ⋯ The ECNM also launched a mastocytosis registry that has been activated in 2012. Using the central database of this registry, cooperative multicenter studies, which should include sufficient numbers of patients and robust evaluations, will be conducted. These studies will increase our knowledge about optimal management and therapy of patients with mastocytosis in the future.
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Wien. Klin. Wochenschr. · Dec 2012
Therapeutic hypothermia for severe adult Herpes simplex virus encephalitis.
Despite antiviral treatment and advances in critical care, the Herpes simplex encephalitis (HSE) still has a poor outcome in a significant portion of patients. In severe cases of HSE, reduced carbon dioxide reactivity is usually present and these patients don't respond to the usual treatment of brain edema and intracranial hypertension. ⋯ In this case series, patients presented with severely impaired consciousness and very high predicted death rate as measured by Glasgow coma scale and Acute Physiology and Chronic Health Evaluation (APACHE II) score respectively. According to our findings, TH in carefully selected patients with HSE holds promise as an adjunctive to the antiviral treatment.
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Wien. Klin. Wochenschr. · Dec 2012
Comparative StudyPositive end-expiratory pressure lung recruitment: comparison between lower inflection point and ultrasound assessment.
Maintenance of the open lung alveoli in the expiration on mechanical ventilation in acute lung injury/acute respiratory distress syndrome (ALI/ARDS) remains challenging despite advances in lung imaging. The inspiratory lower inflection point (LIP) on the ventilator pressure-volume (P-V) curve estimates the required end-expiratory pressure for recruitment of alveolar consolidation. Alternatively, the end-expiratory pressure for recruitment of crater-like subpleural alveolar consolidation could be simply followed with ultrasound. These two methods for setting the ventilators positive end-expiratory pressure (PEEP) were compared. ⋯ In this study, positive end-expiratory pressures for recruitment of subpleural consolidations followed by ultrasound always exceed the pressures measured with LIP. Respecting this, ultrasound method could be the guide for PEEP lung recruitment.