Infantile nephropathic cystinosis.

INTRODUCTION: Infantile nephropathic cystinosis (INC) is a metabolic disorder due to impaired carrier-mediated transport of cystine out of cellular lysosomes. OBJECTIVE: To examine the prevalence and clinical characteristics of INC in paediatric patients with endstage renal disease (ESRD) in Serbia and give a recent statement of the disease. METHODS: ESRD database of the Centre for Paediatric Renal Replacement Therapy (RRT) in Serbia was used to identify all patients with INC who started RRT before age of 19 years during the period January 1980 - December 2008; their records concerning clinical characteristics, therapy and outcome were evaluated. RESULTS: Only three of 298 paediatric patients with ESRD had INC. The first signs of the illness were recognised during infancy. Fancony syndrome was diagnosed in the second year, but the diagnosis of cystinosis was delayed at mean 6 years. ESRD occurred in the first decade of life. All patients underwent cadaver kidney transplantation. At the end of the study period all patients were alive. A 31-year-old female patient was on maintenance chemodialysis due to graft failure after functioning for 11 years. She was growth retarded, single, unemployed, with severe signs of renal dystrophy. Two male patients (14.3 and 14.7 years old) had normal graft function, normal education, and good quality of life, although they were also severe growth retarded. CONCLUSION: The prevalence of infantile nephropathic cystinosis is low in Serbia. The diagnosis of cystinosis was delayed in all patients, although they exhibited the typical course of the disease.

A case of primary peripheral T-cell type non-Hodgkin lymphoma originating in the iris--clinicopathological findings.

BACKGROUND: The ocular adnexal region is the primary localization of extranodal lymphoma in 5% to 15% of all Non-Hodgkin lymphoma. Intraocular lymphoma of T-cell origin is extremely rare and such sites of infiltration have been rarely observed in clinical examination. CASE REPORT: We presented a 56-year-old man with iris infiltration by primary intraocular peripheral T-cell lymphoma. The patient was in clinical stage I BE and the treatment was initiated according to cyclophosphamide, hydroxydaunorubicin, oncovin, prednisone (CHOP) regimen. When the second course of the therapy was scheduled, the patient developed central nervous system lymphoma infiltration. Although De Angelis regimen was used, 3 months after the diagnosis was established, lethal outcome ensued due to disease progression. CONCLUSION: According to our experience we can conclude that further therapeutical approach to patients with primary intraocular T-cell lymphoma requires modification of conventional treatment regimens. The lower median survival in these patients suggests that the disease may be of more aggressive course.

Removal of atrazine, lindane and diazinone from water by organo-zeolites.

Systematic adsorption tests were carried out to determine the efficiency of organo-zeolite (OZ) for removal of atrazine, lindane and diazinone from water. The hydrophobic character of OZ-pesticide interactions was confirmed by measuring the amount of pesticides sorbed on zeolite samples modified with 25, 50, 75 and 150 mmol of stearyldimethylbenzylammoniumchloride (SDBAC)/kg of zeolite. The effects of adsorbent particle size, solid content in the suspension and the initial pesticide concentration in the solutions were also investigated. For effective adsorption of diazinone onto an OZ, it is necessary for the SDBAC/diazinon ratio to be higher than 25. The adsorption capacities, calculated by fitting the experimental data to the Langmuir-Freundlich equation, were 2.0 micromol/g (atrazine), 4.4 micromol/g (diazinone) and 3.4 micromol/g (lindan). At lower initial concentrations of pesticide solution, a linear dependence existed between the amount adsorbed and the equilibrium concentration of pesticide. Column experiments showed that at volumetric flow of 6 cm3/ min, the breakthrough points (at C/C0 = 0.1) were 560 bed volume (BV) for lindane and 620 for diazinone.