[DRESS syndrome]. / DRESS syndróm.

DRESS syndrome (Drug Rash with Eosinophilia and Systemic Symptoms) is severe drug-induced allergic-type reaction which occurs few days to weeks after taking a drug in a predisposed patient. Organ damage, eosinophilia and skin rash are typical at presentation. Corticotherapy is often necessary in severe cases. In this report we describe a case of 56-year old female with fever, elevated liver tests and skin rash. DRESS syndrome was diagnosed and allopurinol was indentified as a causative drug. Due to possible fatal outcome, DRESS syndrome should be considered in a differential diagnosis of all patients presenting with similar signs and symptoms.

[Von Hippel-Lindau syndrome - two sides of the same coin]. / Von Hippelov-Lindauov syndróm - dve strany jednej mince.

Von Hippel-Lindau syndrome (VHL) is a rare genetic disease. Its incidence is 1 : 36,000, there is the familial occurrence in 80 % of cases , the remaining cases are de novo mutations. The disease is caused by the highly penetrant mutations in the VHL gene (3p25.3) and is characterized by the occurrence of benign and malignant neoplasms. The most common VHL tumors are the tumors of the retina, brain and spinal hemangioblastomas, renal cell carcinoma, pheochromocytoma, endolymfatic sac tumors and pancreatic tumors and cysts. The mean age of the VHL patients during the diagnosis is 20-40 years. The diagnosis can be confirmed by a positive family history and the presence of one of the typical tumor. In case of no family history, the diagnosis has to be assessed by the presence of the multiple tumors. The clinical signs and prognosis of VHL depend on the location and extent of the tumors. The life expectancy is 50 years. The most common causes of death are complications of the renal cancer and the brain tumors. The treatment requires a multidisciplinary collaboration through the whole life of patients. This 2 cases report we demonstrate the differences among the patients with de novo mutations disease and the patient with familial incidence.Key words: pheochromocytoma - renal cell carcinoma - von Hippel-Lindau syndrome.

[The most recent aspects of diagnosis and therapy of hyponatraemia]. / Najnovsie aspekty diagnostiky a terapie hyponatriémie.

Hyponatraemia is the most common electrolyte disbalance in clinical practice, which is associated with increased patients morbidity and mortality. At present the pathophysiology of hyponatraemia is explored in more details, antidiuretic hormone and osmoregulation play the major roles. This article informs about relatively new classification of hyponatraemia for clinical practice based on the severity of clinical symptoms and based on the effective serum osmolality. It also offers diagnostic and treatment guidelines of hyponatraemia, which are based on current recommendations of the world experts and on the evidence based medicine.

Hyponatraemia is an independent predictor of in-hospital mortality.

BACKGROUND: Hyponatraemia increases morbidity and mortality, but the extent to which this condition influences mortality independently of other contributing factors is unclear. MATERIALS AND METHODS: All hyponatremic patients admitted to the internal medicine department during a six month period were included. Medical records were reviewed and patients' demographics, underlying disease, cause of hyponatremia and in-hospital deaths were noted. Control group consisted of patients with normonatremia admitted to the same department during the same period matched 1:1 by sex, age and underlying disease. Difference in in-hospital mortality rate between the study and control groups was tested by chi-square test. Baseline demographics, underlying diseases, cause of hyponatremia and state of hyponatremia correction as possible risk factors for mortality were tested in a multivariate analysis. RESULTS: The baseline cohort of all admitted patients consisted of 2,171 patients. Hyponatraemia was found in 278 (13%) patients (160 females and 118 males). The three most common causes of hyponatremia included gastrointestinal loss (52 patients), decreased oral intake (47 patients), and dilution hyponatremia (45 patients). The in-hospital mortality rate in the hyponatremic group was significantly higher compared with the control group (22% vs 7%, respectively; OR 3.75, 95% CI 2.17-6.48, p<0.0001). In a multivariate analysis age above 65 years, dilution hyponatremia, decreased oral intake as etiologic factors of hyponatremia, and unsuccessful hyponatremia correction were independent factors associated with increased mortality. CONCLUSION: Hyponatraemia represents independent factor associated with in-hospital mortality. Age above 65 years, failure to correct hyponatremia and some specific etiologic factors of hyponatremia are related to increased mortality.