Osteoimmunology in mucopolysaccharidoses type I, II, VI and VII. Immunological regulation of the osteoarticular system in the course of metabolic inflammation.

BACKGROUND: Mucopolysaccharidoses (MPSs) are rare genetic diseases caused by a deficient activity of one of the lysosomal enzymes involved in the glycosaminoglycan (GAG) breakdown pathway. These metabolic blocks lead to the accumulation of GAGs in various organs and tissues, resulting in a multisystemic clinical picture. The pathological GAG accumulation begins a cascade of interrelated responses: metabolic, inflammatory and immunological with systemic effects. Metabolic inflammation, secondary to GAG storage, is a significant cause of osteoarticular symptoms in MPS disorders. OBJECTIVE AND METHOD: The aim of this review is to present recent progress in the understanding of the role of inflammatory and immune processes in the pathophysiology of osteoarticular symptoms in MPS disorders and potential therapeutic interventions based on published reports in MPS patients and studies in animal models. RESULTS AND CONCLUSIONS: The immune and skeletal systems have a number of shared regulatory molecules and many relationships between bone disorders and aberrant immune responses in MPS can be explained by osteoimmunology. The treatment options currently available are not sufficiently effective in the prevention, inhibition and treatment of osteoarticular symptoms in MPS disease. A lot can be learnt from interactions between skeletal and immune systems in autoimmune diseases such as rheumatoid arthritis (RA) and similarities between RA and MPS point to the possibility of using the experience with RA in the treatment of MPS in the future. The use of different anti-inflammatory drugs requires further study, but it seems to be an important direction for new therapeutic options for MPS patients.

[Selected parameters of the oxidative-antioxidative balance in children with chronic severe bronchial asthma]. / Ocena wybranych parametrów równowagi oksydacyjno-antyoksydacyjnej u dzieci chorych na przewlekla ciezka astme oskrzelowa.

The aim of this study was to evaluate the involvement of selected parameters of oxidative-antioxidative system in children with severe bronchial asthma. In our study the intensity of peroxide lipid oxidation was calculated by the contents of malonic dialdehyde in plasma. At the same time antioxidant activity was evaluated. The plasma contents of vitamin E and total antioxidant status were measured, as were activity of glutathione peroxidase and superoxide dismutase in blood. Our study was performed on 24 children treated for severe bronchial asthma. The control group consisted of 27 healthy children. The study has revealed that children suffering from bronchial asthma exhibit disturbances in the oxidative/antioxidative balance, manifested by increased oxidative activity and by changes in certain parameters of the antioxidative system. Our results support the theory of free radicals involvement in pathophysiology of severe bronchial asthma.

[Usefulness of peak expiratory flow measurement for selection of dry powder inhalers in asthmatic children]. / Przydatnosc pomiaru szczytowego przeplywu wdechowego w doborze inhalatora suchego proszku u dzieci chorych na astme oskrzelowa.

Peak inspiratory flow rate (PIFR) in 64 asthmatic children aged 6-12 yrs was investigated. In-Check set (peak inspiratory flow meter) which can mimic the internal resistance of some dry powder inhalers, e.g. Discus, Aerolizer and Turbuhaler, was used. All children achieved the minimum and optimum PIFR independently to bronchial obstruction when using Discus resistance. Minimum and optimum values of PIFR adequate for Turbuhaler resistance attained 100% and 77% of analysed patients. The worst results were observed in regard to Aerolizer resistance when 97% of children achieved minimum and only 30% optimum PIFR for this inhaler. Checking actually PIFR may be useful before prescribing dry pressure inhaler for children, especially in cases with bronchial obstruction.

[Correlation between peak expiratory flow and selected indices of bronchial potency in children between 4 and 18 years of age]. / Korelacja miedzy szczytowym przeplywem wydechowym a wybranymi wskaznikami droznosci oskrzeli u dzieci w wieku od 4 do 18 roku zycia.

The study was conducted on 474 children diagnosed at the Lung Function Laboratory of the Children' Hospital in Lublin in the year 1992. 55% of the analyzed children were males. The children's initial diagnosis were: bronchial asthma, allergic rhinitis, chronic respiratory disorders, diabetes mellitus, abnormalities of the thorax. In 28 children no abnormalities were detected. PEF was measured with a Peak Flow Meter produced by Vitalograph. Children in the age group 4-6 years had PEF measured with a mini meter, reading values between 0 to 300 L/min. A standard meter was used for older children detecting values between 0 to 700 L/min. Spirometry, MEFV curves, resistance were analyzed with the abcPNEUMO 2000 spirometer (abcMED). Correlation was calculated between PEF and FEV1, MEF25, MEF50, MEF75, PEF, PIF, FEV1%FVC, Raw. Multiple regression analysis was performed to identify the best subpopulation of independent variants. In all children a correlation was found between PEF and the major parameters of airways potency. The weakest correlation was found in children younger than 6 years. PEF correlated best with FEV1 and MEF75.

[Minutiae of skin ridges in Down's syndrome]. / Minucje listewek skórnych w zespole Downa.

Minutiae of the epidermal ridges were examined in 16 children with Down's syndrome and 50 children without genetic and familial abnormalities. Minutiae in standard areas on the hand palms (according to Grzeszyk's concept) were examined. Comparative analysis confirmed by the statistical analysis showed significant differences in the incidence of particular minutiae types on the hand palms of children with Down's syndrome and control group.

[Reactivity of periodontal vessels in children with insulin-dependent diabetes]. / Reaktywnosc naczyn przyzebia u dzieci z cukrzyca insulinozalezna.

The morphological changes of capillaries and small vessels in diabetes are preceded by functional disturbances. The purpose of the work was assessment of the reactivity of parodontal vessels in children with insulin-dependent diabetes. The study was done in 50 children with this diabetes aged 7 to 17 years, and 45 healthy controls (without parodontal changes). The photoplethysmographic method was used. Nitroglycerine test of Böhme was carried out estimating the pattern of the so called inclination time before and after sublingual nitroglycerine administration. The capillaries of the diabetic children reacted more strongly to nitroglycerin than those of controls. It is concluded that children with diabetes of recent onset have functional vascular disturbances in the parodontium. Photoplethysmography is a simple non-invasive method useful in the assessment of early vascular disturbances in the parodontium of children.

[Use of ceftriaxone in urinary and respiratory tract infections]. / Zastosowanie ceftriaksonu w zakazeniach ukladu moczowego i oddechowego.

Efficiency of ceftriaxone (Rocephin Hoffman Laroche) was assessed in 16 children aged between 3 and 14 years and in 4 adults aged between 17 and 70 years with severe infections of the urinary and respiratory tracts caused by E. coli. S. pneumoniae, P. aeruginosa, P. mirabilis or enterococci. Pyelonephritis as a sole pathology was diagnosed in 10 patients whereas in further 8 patients it complicated other diseases (nephrotic syndrome, hepatitis, cholangitis, leukemia). Pneumonia complicated nephritis leukemia or lymphoma in 8 children. Peritonitis was diagnosed in 1 adult patient. Ceftriaxone was given in a single daily dose of 50 mg/kg to all children and 2.0 g to adult patients for 7-10 days. No adverse reactions were noted. Clinical improvement was achieved in all treated patients. Cultures became negative in 17 cases after the treatment. Significant bacteremia caused by P. aeruginosa persisted in 2 patients and by E. coli in 1 patient. No toxic effects on liver, renal, pancreatic and bone marrow functioning were seen. Ceftriaxone may be safely and efficiently used for the treatment of the urinary and respiratory infections.