Zdeněk Doležel

Intravitreal pegaptanib combined with diode laser therapy for stage 3+ retinopathy of prematurity in zone I and posterior zone II.

Purpose To investigate efficacy of intravitreal injection of pegaptanib and laser photocoagulation for treatment of stage 3+ retinopathy of prematurity (ROP) affecting zone I and posterior zone II, and to compare the results in terms of regression, development of peripheral retinal vessels, and final structural outcome with conventional laser photocoagulation or combined with cryotherapy. Methods In a prospective comparative study, 152 eyes with zone I, II posterior ROP 3+ (76 premature rabies), from 2009 to 2011, were included. Patients were randomly assigned to receive intravitreal pegaptanib (Macugen® 0.3 mg = 0.02 mL, Pfizer) with conventional diode laser photocoagulation in group 1 (68 eyes of 34 infants) or only laser therapy combined with cryotherapy in group 2 (84 eyes of 42 infants), bilaterally. The primary outcome of treatment success was defined as absence of recurrence of stage 3+ ROP. The mean follow-up after treatment was 19.3 months in group 1 and 21.5 months in group 2. Results Final favorable anatomic outcome and stable regression of ROP at last control examination was noted in 89.7% of eyes in group 1 and 60.8 % of eyes in group 2. Regression of plus disease and peripheral retinal vessels development appeared significantly more rapidly in group 1. No recurrence of neovascularization (stage 3+ ROP) was identified in 85.4% of patients in group 1 and 50% of patients in group 2. Conclusions Results of this study support the administration of intravitreal pegaptanib as useful therapy in the management of stage 3+ ROP.

Controlled diet in phenylketonuria and hyperphenylalaninemia may cause serum selenium deficiency in adult patients: the Czech experience

Phenylketonuria is an inherited disorder of metabolism of the amino acid phenylalanine caused by a deficit of the enzyme phenylalanine hydroxylase. It is treated with a low-protein diet containing a low content of phenylalanine to prevent mental affection of the patient. Because of the restricted intake of high-biologic-value protein, patients with phenylketonuria may have lower than normal serum concentrations of pre-albumin, selenium, zinc and iron. The objective of the present study was to assess the compliance of our phenylketonuric (PKU) and hyperphenylalaninemic (HPA) patients; to determine the concentration of serum pre-albumin, selenium, zinc and iron to discover the potential correlation between the amount of proteins in food and their metabolic control. We studied 174 patients of which 113 were children (age 1–18), 60 with PKU and 53 with HPA and 61 were adults (age 18–42), 51 with PKU and 10 with HPA. We did not prove a statistically significant difference in the concentration of serum pre-albumin, zinc and iron among the respective groups. We proved statistically significant difference in serum selenium concentrations of adult PKU and HPA patients (p = 0.006; Mann–Whitney U test). These results suggest that controlled low-protein diet in phenylketonuria and hyperphenylalaninemia may cause serum selenium deficiency in adult patients.

IS CENTRAL EUROPE SAFE FROM ENVIRONMENTAL LEAD INTOXICATIONS? A CASE SERIES

Preventive measures in Central Europe were successful in suppressing both occupational and environmental lead exposure so that they did not constitute a severe public health problem. However, rare lead intoxications still appear. We report on lead intoxication in four family members where the source was removed lead ceiling paint. The symptoms of the lead intoxication started several weeks after removal and the inhalational exposure to the minimum dust residues lasted for more than three months before the poisoning was diagnosed. Father developed anaemia and saturnine colics. He and his two daughters received antidotal treatment which had to be repeated in the children. Finally, all recovered completely.Lead intoxication may be easily overlooked due to the unspecific symptoms. It is necessary to think of this rare poisoning which may be caused by old paints, historical ceramics and lead shots, in addition to commercial products imported from abroad.

PO-0136 Increased Risk Of Vitamin B12 Nutritional Deficiency In Long-term Treated Patients With Phenylketonuria And Hyperfenylalaninemia

The aim of this study was to assess the prevalence of nutritional deficiency of vitamin B12 in long-term treated patients with phenylketonuria (PKU) and hyperfenylalaninemia (HPA), together with parameters of vitamin B12 and metabolic control. Methods In 51 patients aged 3–48 years (28 children, 23 adults) was examined levels of active vitamin B12 in serum, folate concentration in blood, plasma homocysteine and methylmalonic acid concentrations in urine. Results We found a statistically significant difference between the levels of folate in the blood among patients with PKU and HPA (p = 0.046, Mann Whitney U test). This difference was also statistically significant for adults with HPA and PKU (p = 0.004, Mann Whitney U test). There was a statistically significant difference in the proportion of normal homocysteine concentrations in plasma in the overall evaluation of both groups (p = 0.023, chi -square test). This difference was also statistically significant in adults with HPA and PKU (p = 0.032, chi -square test). In the group of adults we detected a statistically significant difference in the concentrations of active vitamin B12 in the blood as in the evaluation of the concentration and the proportion of patients with normal levels (p = 0.031, Mann Whitney U test, p = 0.006, chi -square test). Conclusions In the analysed group of patients we demonstrated that our patients are at risk of vitamin B12 nutritional deficiency and the risk increases with age.

1037 Determination of Prealbumin, Selenium, Zinc and Iron Concentration in Serum for Monitoring the Nutrition Status of Phenylketonuric and Hyperphenylalaninemic Patients

Background and Aims Phenylketonuria is an inherited disorder of metabolism of the amino acid phenylalanine caused by a deficit of the enzyme phenylalaninhydroxylase. It is treated with a low-protein diet containing a low content of phenylalanine to prevent mental affection of the patient. The objective of the present study was to assess the compliance of our phenylketonuric (PKU) and hyperphenylalaninemic (HPA) patients; to determine the concentration of serum pre-albumin and trace elements to discover the potential correlation between the amount of proteins in food and their metabolic control. Methods The prospective study contained altogether 174 patients, of which 113 were children, 60 with PKU and 53 with HPA and 61 were adults, 51 with PKU and 10 with HPA. Results We did not prove a statistically significant difference in the levels of serum pre-albumin, zinc and iron among the respective groups. We proved statistically significant difference in the level of serum selenium among PKU and HPA patients in adulthood (p=0.006, Mann-Whitney U test). Conclusion The therapeutic restrictive diet for PKU and HPA makes the patient liable to the risk of nutritional deficit.