[Acquired Fanconi-de Toni-Debre syndrome due to therapy for Ewing's sarcoma in 5-years old boy]. / Nabyty zespól Fanconi-de Toni-Debre w przebiegu terapii miesaka Ewinga u piecioletniego chlopca.

We present a 5-years old boy with acquired Fanconi-de Toni-Debre syndrome being a effect of therapy for Ewing's sarcoma. At the age of 3 years, this boy was diagnosed as suffering from Ewing sarcoma of his right femur. The boy received a course of 8-month pre-surgery (6 VIDE--Vincristine, Ifosfamide, Doxorubicin, Etoposide cycles and 2 VAI--Vincristine, Actinomycin, Ifosfamide cycles) and 6-month post-surgery (6 VAI--Vincristine, Actinomycin, Ifosfamide cycles) cytostatic therapies according to EWING, EURO 99 protocol. In forth month of post-surgery cytostatic therapy, progressive malaise, polyuria, polydypsia, and recurrent vomiting occurred. The association between those symptoms and malignancy was excluded. Laboratory studies revealed hypokaliemia, hypophosphatemia, proximal tubular acidosis, proteinuria, glucosuria, aminoaciduria, hyperkaliuria and hyperphosphaturia. Acquired Fanconi-de Toni-Debre syndrome due to toxic effect of cytostatic therapy on renal proximal tubules was diagnosed. At present, two years after the time the diagnosis was made, despite constant substitution of potassium, phosphates and bicarbonates, deficit of body mass and height, and bone mineral density abnormalities are observed.

Acute renal failure due to bilateral xanthine urolithiasis in a boy with Lesch-Nyhan syndrome.

Lesch-Nyhan syndrome is a very rare X-linked recessive disorder caused by mutation in the gene encoding enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT). A complete deficiency of HPRT leads to severe purine overproduction and to uric acid renal lithiasis as a consequence. This may be effectively prevented by administration of allopurinol; however, its overdosage may result in xanthinuria and xanthine urolithiasis. We report on a 9-year-old boy with Lesch-Nyhan syndrome who developed acute renal failure due to bilateral staghorn xanthine urolithiasis resulting from long-term treatment with excessive doses of allopurinol. To the best of our knowledge, the presented case is the first one in the literature.

[Passive smoking and patterns of 24-hour ambulatory blood pressure in healthy children]. / Bierne palenie tytoniu a wyniki 24-godzinnego pomiaru cisnienia tqtniczego u zdrowych dzieci.

The aim of this study was to compare patterns of 24-hour ambulatory blood pressure between passive-smoking and non-smoking children. Subjects were 100 healthy children divided in 2 groups: passive smokers (72 children) and non-smokers (28 children). Children underwent 24-hour ambulatory blood pressure monitoring (ABPM). On the basis of ABPM results following daytime and night-time parameters were calculated for systolic blood pressure (SBP) and diastolic blood pressure (DBP): mean blood pressure (BP) value, BP load, BP variability magnitude, 24-hour blood pressure profile. The results were compared between passive smokers and non-smokers with t-Student test. Results with p < 0.05 were considered statistically significant. Smokers did not differ from non-smokers in mean daytime and night-time BP values. Daytime BP load, BP variability and 24-hour SBP profile were statistically higher in passive smokers, while night-time values of these parameters and DBP profile did not differ significantly. ABPM may be useful in detecting differences in BP characteristics of passive smoking and non-smoking children. Higher BP load and BP variability in smoking children may be early manifestations of BP regulation disorder, which might be helpful in early screening. Passive smoking may be a risk factor of hypertension.