[Hereditary pheochromocytoma--a family affected by von Hippel-Lindau disease]. / Hereditární feochromocytom--rodina postizená von Hippel-Lindauovou chorobou.

The authors present a case of a 37 year old male (proband) with a 13 year history of progressive sight impairment leading to blindness and a 4 year history of a mild hypertension. He was incidentally found to have large adrenal tumors after an ultrasound kidney examination. The tumors were confirmed with CT scan and magnetic resonance imaging. A bilateral pheochromocytoma was biochemically demonstrated and successfully removed. The eye diagnosis of angiomatosis retinae von Hippel-Lindau was ascertained after a search of the patient files in other medical departments, which led to a family screening. Proband's brother, having hypertension and a history of a cerebellar astrocytoma operation, was also diagnosed with CT scan to have a bilateral pheochromocytoma. Unfortunately, at the same time he was found to have a large irremovable neuroendocrine pancreatic carcinoma, which caused complications and his eventual death. Both proband and his brother were affected by the same CGG(Arg167)->CAG(Gln) mutation in the exon 3 of the VHL gene. Other living and examined family members were not affected, which was confirmed by negative genetic testing. One year after the pheochromocytoma operation, proband was diagnosed to have a retroperitoneal tumor left to the aorta, clinically silent, with slightly and non-constantly elevated urine norepinephrine and normetanephrine. Metaiodobenzylguanidine scintigraphy showed that it was a paraganglioma. The old CT and magnetic resonance picture review demonstrated that the tumor had already been present at the time of the operation. It was surgically removed and histologically verified. It is a pity that proband had not been sent by his ophthalmologist for an endocrine examination when the eye diagnosis was determined. Affection of the family would have been discovered earlier, and proband's brother might have possibly been saved.

[Results of the United Kingdom Prospective Diabetes Study and its importance in the treatment of hypertension in diabetics]. / Výsledky studie United Kingdom Prospective Diabetes Study a její význam pro lécbu hypertenze u diabetiku.

In 1997 the United Kingdom Prospective Diabetes Study (UKPDS), the largest and longest study in type 2 diabetes so far, was finished. Answers to fundamental questions of non-insulin dependent diabetes mellitus therapy were expected: 1. What is the benefit from a good metabolic control of diabetes? 2. Has any particular antidiabetic therapy advantages over the other ones? 3. What is the importance of concurrent antihypertensive therapy? The evidence was given that intensive therapy (with antidiabetics or insulin) substantially and significantly reduced the risk of microvascular complications, but the reductions of mortality and macrovascular complications were not significant. An adverse cardiovascular effect of sulfonylureas or insulin were not confirmed. All intensive therapeutic approaches reached comparable results only metformin appeared to be advantageous first-line therapy of choice in obese diabetics. The intensive control of hypertension proved to be even more effective in prevention both the microvascular and namely the macrovascular complications as well as in reducing diabetes related mortality.

[Clinical experience with changing type I diabetics from animal to human insulin administered by the NovoPen 3 applicator]. / Klinické zkusenosti z prevodu diabetiku I. typu ze zvírecích inzulinu na humánní inzuliny podávané aplikátory NovoPen 3.

The objective of the study was to assess the safety of changing ambulatory patients from animal insulin produced in the Czech Republic administered by classical insulin syringes to human insulins of the Danish firm Novo Nordisk, using a NovoPen 3 applicator. Furthermore antibody levels against hog, bovine and human insulin were assessed. Forty-seven patients with diabetes type I stabilized on an intensified insulin regime were after a four-day preparatory period divided at random into two groups. Patients in group A (n = 22) were after randomization changed to human insulin, patients in group B (n = 25) eight weeks later. From the onset of treatment with human insulins up to the end of the study the mean daily dose of insulin in both groups increased (in group A by 1.51 IU/day, in group 1.35 IU/day). This is not statistically or clinically significant. During the same period a statistically significant decline of the mean value of the daily 8-point glycaemic profile was recorded (in group A by 0.85 mmol/l, in group B by 0.51 mmol/l). Glycosylated haemoglobin declined also significantly in the course of the study (in group A by 1.64%, p = 0.00004, in group B by 1.02%, p = 0.0077). The greatest drop occurred during the preparatory period. Despite the increased daily insulin dose and improved compensation the number of hypoglycaemic events declined significantly in both groups (in group A by 0.78%, p = 0.0102, in group B by 0.74%, p = 0.0134). Hypoglycaemic coma was not recorded in either group. A significant drop of insulin antibodies was found in both group after the onset of treatment with human insulins. From the results of the study ensues that metabolically compensated type I diabetics with a mean daily insulin dose of 0.6 IU/kg body weight can be changed without any complications, in the ambulatory department, to human insulins with the same dosage. Concurrently a gradual decline of antibodies can be expected.