Phenylketonuria Patients' and Their Caregivers' Perception of the Pandemic Lockdown: The Results of a National Online Survey.

The first pandemic lockdown dramatically impacted many aspects of everyday life, including healthcare systems. The purpose of this study was to identify problems of patients with phenylketonuria (PKU) and their parents/caregivers during that time. We aimed to analyse potential differences in the self-reported compliance and characteristics of contacts with a doctor/dietitian before and during the pandemic lockdown and the perception of access to special food and opinions on remote contacts between a particular group of respondents.

The Impact of the COVID-19 Pandemic on the Perception of Health and Treatment-Related Issues among Patients with Phenylketonuria in Poland-The Results of a National Online Survey.

There is agreement that the pandemic has affected the healthcare system and behaviour of patients. This study aims to identify problems encountered by patients with phenylketonuria (PKU) and their parents/caregivers during the six-week pandemic lockdown in Poland (15 March to 30 April 2020). To determine the factors that influenced health and treatment-related issues, as well as the respondents' perception of the impact of the pandemic, study participants were asked to complete a non-validated online questionnaire comprising 31 questions (including 27 single-choice, two multiple-choice and two open-ended ones).

Free of salt high-pressure deliming of animal hides.

The wastewater pollution of tanneries is of high concern. The investigation of technologies to minimize the consumption of chemicals in the leather production process can reduce the environmental burden. We focus on the reduction of ammonium salts in the leather production process.

Weaning practices in phenylketonuria vary between health professionals in Europe.

Background: In phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula.

Early feeding practices in infants with phenylketonuria across Europe.

Background: In infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe.

Methods: We sent a cross sectional, survey monkey® questionnaire to European health professionals working in IMD.

Phenylketonuria patients' and their parents' knowledge and attitudes to the daily diet - multi-centre study.

Background: The aim of the study was to assess both patients' and their parents' knowledge of phenylketonuria (PKU) treatment and compliance with PKU diet.

Methods: The study included 173 PKU patients aged 10-19 and 110 parents of PKU children who were enrolled in the study on the basis of questionnaire data. The study also included 45 patients aged ≥20.

Phenylketonuria patients' and their parents' acceptance of the disease: multi-centre study.

Purpose: Phenylketonuria (PKU) still poses a therapeutic challenge for patients and medical professionals. The aim of the study was to assess both patients' and their parents' acceptance of the disease.

Methods: The study included 218 PKU patients and 178 parents of PKU children who were enrolled in the study on the basis of questionnaire data.

Molecular genetics of PKU in Poland and potential impact of mutations on BH4 responsiveness.

Tetrahydrobiopterin (BH4) has been recently approved as a treatment of patients with phenylketonuria. However, as a confirmation of BH4-responsiveness, it might require a very expensive trial treatment with BH4 or prolonged BH4-loading procedures. The selection of patients eligible for BH4-therapy by means of genotyping of the PAH gene mutations may be recommended as a complementary approach.

The 10-valent pneumococcal non-typeable Haemophilus influenzae protein D conjugate vaccine (PHiD-CV) coadministered with DTPw-HBV/Hib and poliovirus vaccines: assessment of immunogenicity.

Background: Immunogenicity of the 10-valent pneumococcal non-typeable Haemophilus influenzae protein D-conjugate vaccine (PHiD-CV) was evaluated when coadministered with DTPw-HBV/Hib and OPV at 6, 10, and 14 weeks of age in the Philippines, or with DTPw-HBV/Hib and IPV at 2, 4, and 6 months of age in Poland.

Methods: In this double-blind, controlled study (107007/NCT00344318), 400 Filipino and 406 Polish infants 6 to 12 weeks of age were randomized (3:1) to receive either PHiD-CV or the 7-valent pneumococcal conjugate vaccine (7vCRM). Immune responses were assessed 1 month post-dose III.

Quality of life in noncompliant adults with phenylketonuria after resumption of the diet.

Background: Discontinuation of dietary therapy in adults with phenylketonuria can lead to neuropsychological abnormalities and emotional problems. The aim of our study was to assess the change in quality of life in adult patients returning to the diet and to define the reasons for failure in diet resumption.

Methods: Quality of life was assessed by means of the Psychological General Well-Being Index before study entry and subsequently after 3 and 9 months.

[The influence of hepatitis G virus infection on the course of chronic virus hepatitis B or C in children].

Hepatitis G virus (HGV) infection is often observed in patients with hepatitis B or C virus (HBV or HCV) infections. The aim of this study was an evaluation of the influence of HGV infection on the course of virus hepatitis B and C in children. 113 children aged 2-15 years old, with hepatitis B or C were enrolled to the study.

[The incidence rate of HGV infection in children with selected liver diseases].

Hepatitis G virus (HGV) was detected and described five years ago and up till now it presented unexplained problem for clinicians. Although reports on the pathogenicity of the virus are inconsistent, most researchers admit that the infection is very common. The aim of the study was an evaluation of HGV infection incidence rate among children with selected liver diseases.

[Antithrombin III, protein C and protein S in children with chronic viral hepatitis B or C].

Abnormalities of blood coagulation are a major part of acute and chronic viral hepatitis. In this study antithrombin III, protein C and protein S were determined in 100 children with chronic hepatitis B or C infection (range age 3-17) according to degree of hepatic histological activity and fibrosis. There was no statistically significant correlation between levels of examined parameters in children with chronic viral hepatitis compared with those in control group and between the degree of hepatic histological activity and fibrosis and coagulation parameters.

Assessment of iron metabolism in children with chronic hepatitis B -- prognostic factor in interferon alpha therapy.

Background: The aim of the study was to assess iron metabolism in children with chronic hepatitis B and to establish whether it had any influence on the results of interferon alpha therapy.

Material/methods: The study was carried out in a group of 38 children aged from 2 to 16 years with chronic hepatitis B diagnosed according to serological, biochemical and histopathological criteria. All the patients were treated according to the commonly adopted schedule: interferon alpha administered subcutaneously three times a week at 3 MIU doses for 20 weeks.

Studies of the effectiveness of interferon alpha treatment for chronic hepatitis C in children.

Introduction: The significance of hepatitis C infection in Poland, particularly in a pathology of the developmental age still increased. The aim of the study was the analysis of interferon alpha therapy efficacy in children with chronic C hepatitis.

Material And Methods: 30 children (aged from 3 years to 15 years, 16 females, 14 males) were included in the study.

Antioxidant defense in children with chronic viral hepatitis B and C.

Introduction: The aim of the study was estimation of antioxidant defence state in children with chronic viral hepatitis.

Material And Methods: We examined 100 children with chronic viral hepatitis who have serological and molecular markers of HBV or HCV infection. In all patients catalase (CAT), superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) activities in erythrocytes were assayed.

[Chronic hepatitis C in a 12-year-old girl coinfected with HGV].

In this study we present a 12-year-old girl with chronic C hepatitis coinfected with HGV, in which severe life-threatening side effects from alfa interferon therapy occurred after 3 months of injection and required definite IFN withdrawal. It seems, that infection HGV may predispose patients with chronic C hepatitis treated with alpha interferon to this severe side effect.

[Clinical and morphological picture of hepatitis C in children with genotype 4c4d].

Actually, HCV genotype 4 is frequent in central Africa and in the Middle East. In contrast, in Europe genotype 4 is uncommon (1-4%). In this study we present 12 children with HCV genotype 4c4d.

[Holter EKG method of monitoring during gastroscopic examination in children].

Assessing the results of ECG monitoring by the Holter method during gastroscopic examination in children a more frequent incidence was found of isolated ventricular ectopic beats than in the control group. No complex forms of arrhythmia were found in either group.

[An attempt to estimate epidemiologic prevalence of HBs associated antigen among hospitalized children].

We have observed the problem of the more frequent occurrence of the hepatitis-associated antigen (HBsAg) among multiple hospitalized children and we have analysed the medical documentation of children who were treated in the Department of Gastroenterology and Pediatrics of the Medical Academy of Bydgoszcz in 1989-1992 years. In this period of time we have recorded 39 children with HBsAg. In 80% (31 patients) there are children, who were hospitalized two or more times.