The incidence rate of hospitalized lysosomal storage diseases in Poland in 2013-2015 based on data from the National Health Fund.

INTRODUCTION: The reported data address the incidence of lysosomal storage diseases, obtained from the public health service databases in Poland. Data are given by subtypes from the National Health Fund between 2013 and 2015. MATERIAL AND METHODS: Patients with lysosomal storage diseases were identified in the National Health Fund database (2013-2015). In order to ensure that the reported incidence data included only new patients. The geographic area of residence in 2013-2015 was divided into 6 parts. RESULTS: The incidence rate of lysosomal storage diseases in Poland is about 1.84/1 million/person/years. Other sphingolipidosis was the largest disease category, with 127 patients (incidence 1.1 patients/million habitants), follow by GM2 gangliosidosis - 29 patients (incidence 0.25 patients/million habitants). Men had a somewhat higher incidence than women (respectively IR = 2.53, IR = 1.84). The number of deaths with lysosomal storage disease patients hospitalized between the years 2013 through 2015 is higher in young people (0-9 years old). CONCLUSIONS: The incidence rate of lysosomal storage diseases in Poland is about 1.84 per million person-years. Other sphingolipidosis was the largest disease category, followed by GM2 gangliosidosis. The hospitalization rate of lysosomal storage diseases was higher in men.

Treatment of classic phenylketonuria in Poland in the years 2009-2015 based on the database of the Polish National Health Fund.

INTRODUCTION: To avoid the risk of intellectual disabilities, newborns in Poland are screened for phenylketonuria and are recommended to start a life-long phenylalanine-restricted diet shortly after birth. The aim of this paper is to evaluate the health care for patients with classical phenylketonuria in Poland. MATERIAL AND METHODS: We reviewed the National Health Fund's reporting data concerning information on healthcare services for patients with classical phenylketonuria (PKU), which were reported to the payer by the healthcare service providers between 2009 and 2015. The analysis was prepared within the framework of mapping the health care needs of patients with metabolic diseases published in December 2016 (http://www.mapypotrzebzdrowotnych.mz.gov.pl/). RESULTS: A total of 2706 patients with PKU (including 1180 children) were registered in the healthcare system in the period covered. The estimated national prevalence of PKU was 1 per 7758 live births. Paediatric patients up to 12 moths of age accounted for over 40% of all visits to outpatient clinics. Patients over 28 years of age accounted for only 1% of all PKU patients receiving specialist outpatient care. There were twice as many clinics providing health care to children than to adults. The majority of adult patients received healthcare from the same providers as children. Sixty-nine percent of adults and 64% of children were treated in the two largest outpatient centres. There were 12 deaths, with a median age of 63 years. The working-age adults accounted for 50% of the deaths. CONCLUSIONS: Adult patients with PKU do not receive sufficient healthcare. The discontinuation of healthcare by adults with PKU can result from the lack of an adequate transition process from paediatric to adult care.

Analysis of hospital management of chronic respiratory diseases in light of the "Maps of Health Needs" project in Poland.

INTRODUCTION: The "Maps of Health Needs" project has been carried out in Poland since 2016 and its purpose is to implement quality-promoting and organisational solutions in the Polish healthcare system. This paper is the analysis of hospitalisations for chronic respiratory diseases recorded in Polish National Health Fund databases in 2014. MATERIAL AND METHODS: The study included 122,000 hospitalisations of adults and 22,000 hospitalisations of children. Epidemio-logical parameters (incidence and prevalence) and major hospitalisation parameters were determined through statistical analysis. RESULTS: The highest registered incidence was observed in asthma patients (548 per 100,000 inhabitants) followed by COPD patients (233 per 100,000 inhabitants). Asthma patients were also characterised by the highest prevalence, with lower values being observed in COPD patients. In the group of adults, patients aged 65 years or older and 80 years or older accounted for 44% and 14% of hospitalised adults respectively. The analysis also revealed that 66% of hospitalisations of adults included patients with asthma, COPD and respiratory failure. The development of respiratory failure prolongs hospitalisation and increases both in-hospital and post-discharge mortality. In children, 90% of the identified hospitalisations were for asthma, chronic inflammatory lung diseases and cystic fibrosis. CONCLUSIONS: The results of the study demonstrate that pulmonary obstructive diseases are associated with a considerable burden. Therefore, corrective actions within the Polish healthcare system are required to decrease the number of hospitalisations for these diseases.

Analysis of the incidence of acute respiratory diseases in the paediatric population in Poland in the light of the "Health Needs Map".

INTRODUCTION: Statistical data on the structure of acute respiratory diseases incidence in the paediatric population are still scarce. The demand for such data results mainly from the need to constantly implement new systemic and economic solutions. The aim of the study was to attempt to use reported data for an assessment of the incidence of acute respiratory diseases in various age groups. MATERIAL AND METHODS: An analysis of selected acute respiratory diseases was conducted in relation to diagnoses reported from 1 January to 31 December 2014 to the National Health Fund (NFZ, Narodowy Fundusz Zdrowia) in accordance with the codes of the International Statistical Classification of Diseases and Related Health Problems, 10th Revision. The study was conducted under the Knowledge Education Development operational programme co-funded by the European Social Fund. RESULTS: A total of 101,000 children were hospitalised due to acute respiratory diseases, which amounted to 1,554 hospitalisa-tions per 100.000. The most common causes of hospitalisation were pneumonia and bronchitis/bronchiolitis. Boys were hospital-ised more often in each age group. The shortest average length of stay (ALOS) was 5.21 days and concerned hospitalisation due to bronchitis. The longest length of stay for children was due to tuberculosis (14.3 days). The highest age average of a child was recorded in pleural diseases (10.51 years) and the lowest in bronchitis (2.93 years). Rehospitalisation was necessary in children in whom tuberculosis or pleural diseases were diagnosed (1.43 vs 1.34). A total of 67 inpatient deaths were recorded, of which 19 were due to pneumonia or its complications. CONCLUSIONS: Epidemiological data reported to the National Health Fund (NFZ) seem quite reliable and do not differ significantly from those reported in other European countries. The analysed data may be useful in estimating health needs in paediatrics.

Medical care of patients with disorders of aromatic amino acid metabolism: a report based on the Polish National Health Fund data records.

INTRODUCTION: Patients with disorders of aromatic amino acid metabolism are a heterogeneous group. They vary in morbidity and medical care requirements. Polish newborn screening program allows for quick diagnosis of some inborn errors of metabolism (such as classical phenylketonuria, mild hyperphenylalaninemias, tyrosinemia type 1 and tyrosinemia type 2) and subsequent immediate treatment. THE AIM OF THE STUDY: To evaluate the effect of the Polish public healthcare system in terms of management and access to health care services for children and adults with disorders of aromatic amino acid metabolism. MATERIAL AND METHODS: The analysis was based on the National Health Fund (NFZ) reporting data for 2009-2015. The analysis included patients with disorders of aromatic amino acid metabolism converting ICD-10 coding according to the International Classification of Diseases. The analysis covered patients with codes E70, E70.0, E70.1, E70.2, E70.3, E70.8, E70.9. The analysis was prepared as part of the mapping of health needs in metabolic diseases, http://www.mapypotrzebzdrowotnych.mz.gov.pl/. RESULTS: In 2009-2015, 4090 patients with disorders of aromatic amino acid metabolism were registered in the NFZ system. The largest number of patients were hospitalized and registered in outpatient specialistic care (AOS) in the first year of life. After the second year of life, the number of hospitalized patients was almost zero, and the number of children (< 18 years) with AOS according to age was stable. After the 18 years of age the number of patients in the AOS gradually decreased. The population of patients aged 0-28 years accounted for 99% of all cases, after 28 years of age were only one percent of the total population. There were 95 deaths, the average age of death was 77 years. In the whole study group the highest number of deaths was recorded after 70 years of age, 21% of all deaths were reported in both working-age patients children (2 deaths). Patients with classical phenylketonuria were the most commonly reported in the AOS. 22% of patients were coded with ICD-10 as E70 without extension. CONCLUSIONS: Children aged 0-18 years with disorders of amino acid metabolism had full access to a well-organized specialized medical care system in Poland. In contrast, care for adult patients with the disorders was limited. It is necessary to properly code the disease using ICD-10 extension codes in order to avoid inconsistency in data reporting or misdiagnosis.