Danuta Chlebna-Sokół

Clinical evaluation of patients with idiopathic juvenile osteoporosis.

The aim of this study is to evaluate the occurrence of idiopathic juvenile osteoporosis by clinical, radiographic and biochemical methods in 7 patients aged 10 years to 18 years. The prevailing clinical symptoms included pain in the lower limbs and spine, gait disturbances and weak muscles; confirmed fractures were seen in four out of seven patients. In all patients, bone mineral density was determined by dual-energy X-ray absorptiometry and calcium-phosphorus metabolism assays were performed. Clinical evaluation of our patients revealed a wide variety of classic clinical symptoms, and also radiographic and biochemical features. In this complex evaluation, we can distinguish two types of disease occurrence, mild and acute.

Immunogenicity and safety of heptavalent conjugate vaccine against Streptococcus pneumoniae in pre-term Polish infants

The purpose of the study was to assess post-vaccination immune response and occurrence of adverse events in the group of prematurely born infants. The study included 40 pre-term infants. Each child was vaccined four times (2, 4, 6 and 16 months) with the heptavalent conjugated pneumococcal vaccine (PCV7). Assessing of the level of antibodies was performed before vaccination, 4 weeks after primary series, before and 4 weeks after the booster dose. The research participants were qualified into 2 groups: group I - 19 children born before 30th gestational week, group II - 21 children born between the 30th and 34th gestational week. After the basic vaccination, an increase in the average antibody concentration in the area of all serotypes in most of the children tested was registered, with no significant differences observed between the groups. However, differences between individual serotypes were observed. The lowest values were found for serotype 6B. Before administering the booster dose, a significant drop in antibody titre in all of the children tested was noted. The last vaccination caused another significant increase in antibody concentration in both groups and the results obtained were markedly higher than those obtained after administering three vaccine doses. The majority of the children tested (with the exception of three from group II) achieved the preventive antibody level ≥ 0.35 μg/ml. In all of the children, no serious adverse events were observed. Our research showed, that heptavalent pneumococcal conjugate vaccine is immunogenic in children born before the completion of the 34th week of pregnancy. A booster dose of vaccine must be given at the right time to optimal response to the vaccine for all serotypes. Finally, any serious adverse events were observed.

Choroby cywilizacyjne w wieku rozwojowym – zespół metaboliczny u dzieci łódzkich w okresie dojrzewania☆

Streszczenie Wstep Zespol metaboliczny do niedawna uwazano za zaburzenie wystepujące wylącznie u osob doroslych, stale jednak rosnąca czestośc otylości wśrod mlodziezy wiąze sie z czestszym rozpoznawaniem zaburzen gospodarki lipidowej, weglowodanowej i podwyzszonego ciśnienia tetniczego w tym wieku. Cel Celem pracy bylo uzyskanie odpowiedzi na pytanie, czy w badanej grupie dzieci z nadmiarem masy ciala wystepuje zespol metaboliczny lub jego skladowe. Material i metody Badaniami objeto 132 dzieci w wieku 10–15 lat z nadmiarem masy ciala. Przeprowadzono u nich pomiary antropometryczne oraz ciśnienia tetniczego krwi. Oznaczono stezenie wszystkich frakcji lipidow i glukozy w surowicy. Wyniki Otylośc prostą rozpoznano u 94 dzieci, natomiast u pozostalych 38 stwierdzono nadwage; u 111 spośrod nich wystepowal centralny typ otluszczenia. U 48/132 pacjentow odnotowano hipertriglicerydemie, a u 22/132 obnizone stezenie HDL-cholesterolu, natomiast u zadnego pacjenta nie stwierdzono nieprawidlowej glikemii na czczo. Wzrost wartości ciśnienia tetniczego powyzej 90pc wykazano u 78/132 dzieci. Wedlug przyjetych kryteriow CEP/ATP III zmodyfikowanych na podstawie NHANES III, u 42 (31,81%) pacjentow rozpoznano zespol metaboliczny, natomiast u kolejnych 46 stwierdzono wystepowanie jednocześnie 2 cech tego zespolu. Wnioski Zespol metaboliczny rozpoznano u okolo 1/3 (32%) badanych dzieci z nadmiarem masy ciala. Pojedyncze skladowe zespolu metabolicznego, stwierdzone u wiekszości pozostalych dzieci z nadwagą i otylością, mogą stanowic wczesny objaw nieprawidlowości metabolicznych sprzyjających inicjacji tego zespolu. Wyniki badan wskazują na pilną potrzebe wprowadzenia wczesnej profilaktyki zespolu metabolicznego, w tym przede wszystkim nadmiaru masy ciala, przez powszechną edukacje, dotyczącą glownie wlaściwego odzywiania i odpowiedniej aktywności fizycznej.

Haemophilus influenzae type b and pertussis vaccinations in preterm infants

Abstract.Introduction: The aim of the study was to evaluate the serological efficacy of Hiberix and Infanrix-DTPa vaccines in preterm infants.Materials and Methods: The results of the investigation of 61 preterm infants immunized three times (primary vaccination) with Hiberix and Infanrix-DTPa at 6-week intervals are presented. Of the 61 children, 17 were additionally immunized with a booster dose of these vaccines. Postvaccinal response to these immunizations was evaluated by means of an immunoenzymatic method.Results: We observed a significant increase in protective postvaccinal antibody titers against Haemophilus influenzae type b (Hib) and Bordetella pertussis after the primary vaccination compared with the initial antibody levels (p<0.05). A significant increase (p<0.0002) in protective antibody titers after the booster dose of Hiberix compared with the primary vaccination was also noted. No correlations between birth weight, gestational age, and the achieved levels of postvaccinal anti-polyribosylribitol phosphate of Hib and of anti-pertussis toxin and anti-filamentous hemagglutinin of B. pertussis antibodies after the primary vaccination or booster dose were found. After the booster dose, all the preterm infants responded with the production of protective postvaccinal antibody titers against Hib and B. pertussis. Conclusions:Due to the very good immunogenicity of the vaccines against Hib studied, inclusion of this immunization should be proposed in the obligatory vaccination schedule in Poland, especially in preterm infants. An additional immunization (i.e. a second booster dose) of Polish children with acellular pertussis (DTPa) vaccine is necessary to protect them from decreasing protective anti-pertussis antibody titers in early childhood.

Is it possible to predict a risk of osteoporosis in patients with juvenile idiopathic arthritis? A study of serum levels of bone turnover markers

BACKGROUND Low bone mineral density is a common finding in children with systemic connective tissue diseases, including juvenile idiopathic arthritis (JIA). The influence of the ongoing process of bone remodeling on the disease course merits further investigation. The aim of this study was to assess the clinical relevance of markers of bone turnover and their potential role as predictors of higher fracture risk and, by extension, risk of osteoporosis. MATERIALS AND METHODS Blood samples were collected from 59 patients diagnosed with JIA in order to determine serum levels of the following markers of bone turnover: Beta-Crosslaps, osteocalcin, bone alkaline phosphatase, osteoprotegerin and receptor activator for nuclear factor kappa-B ligand. The values were analyzed with laboratory parameters and results of dual X-ray absorptiometry (DXA). RESULTS Osteoprotegerin and bone alkaline phosphatase levels were age-dependent. Beta-Crosslaps values were significantly higher in patients with positive JADAS27 score (p=0.0410). Osteoprotegerin levels were higher in patients treated with biological agents than only with disease-modifying anti-rheumatic drugs (p=0.0273). There was no relation between markers of bone turnover and sex, DXA results, dosage of glucocorticosteroids and disease duration. CONCLUSIONS The authors postulate performing DXA measurements every 6 months in patients with higher disease activity. The potential lower fracture risk in children with JIA within biological treatment needs further assessment. Age- and sex-adjusted reference rates of bone turnover markers need to be developed for Central European patients in order to assess individual values properly.

Vitamin D receptor gene (BsmI and FokI) polymorphism and calcium-phoshporus indices in children from Lodz region

Introduction. Maintenance of calcium-phosphorus indices, including liver and renal metabolites of vitamin D, is regulated extensively hormonally and genetically. One of many possible genetic factors is vitamin D receptor (VDR) gene. The study aimed at evaluating the relationship between calcium-phosphorus indices and selected VDR polymorphism in children. Patients and methods. The study group consisted of 395 children, aged 6 to 18. All of them underwent gene typing using the PCR-RFLP method within polymorphic loci BsmI (rs1544410) and FokI (rs2228570) of the VDR receptor gene. Comparative group for incidence of particular genotypes consisted of 294 children, while 161 patients were evaluated for calcium-phoshporus indices and bone turnover markers (osteocalcin and N-terminal telopeptide of type I collagen) using standard methods. Skeletal densitometry (Total body and Spine programmes) examination using DXA method was carried out within study group. Statistica 8.0 PL was used for statistical analysis of relationship between evaluated results. Results. Average test results of serum calcium-phosphorus indices wthin the whole study group (n=161) fell within reference limits, only 12/161 results with concentration of 25OHD fell under 20mg/ ml. The lowest, but nevertheless within reference limits, concentration of osteocalcin was observed in 1Klinika Propedeutyki Pediatrii i Chorób Metabolicznych Kości 2 Klinika Pediatrii, Onkologii, Hematologii i Diabetologii Vitamin D receptor gene (BsmI and FokI) polymorphism and calciumphoshporus indices in children from Lodz region 1Department of Paediatric Propedeutics and Bone Metabolism Diseases, Medical University, Lodz 2Department of Pediatrics, Oncology, Hematology and Diabetology Medical University of Lodz

Evaluation of vitamin D levels in children hospitalized with symptoms suggesting metabolism disorders in skeleton system

Introduction. Commonly occurring vitamin D deficiency in children can significantly influence the development of their skeleton. Objectives. Aim of the study is to evaluate the relationship between serum vitamin D levels and the occurrence of the skeleton system disorders in children at different developmental periods. Material and methods. The study included 1007 children aged 0-18 years (471 girls and 538 boys) who were hospitalized in the Department of PropedeuticsPaediatrics and Metabolic Bone Diseases, Medical University of Lodz in 2011–2015. Patients were divided into 6 groups: group I – 0–12 months, group II – 1–3 years, group III – 4–6 years, group IV – 7–10 years, group V – 11–14 years and group VI – 15–18 years. For each patient, the concentration of hydroksycholekacyferolu (25OHD) in serum was measured with chemiluminiscent immunoassay technology. Vitamin D deficiency was diagnosed at a concentration 25OHD ≤ 30 ng/ml. Results. 721 of children (71.59%) had vitamin D deficiency, more often boys. There was an increased incidence of vitamin D deficiency with age. Average concentrations of 25OHD reaches ≥ 30 ng/ml only in August Klinika Propedeutyki Pediatrii i Chorób Metabolicznych Kości Uniwersytetu Medycznego w Łodzi 1Centralny Szpital Kliniczny Uniwersytetu Medycznego w Łodzi, Ośrodek Pediatryczny im. M. Konopnickiej Evaluation of vitamin D levels in children hospitalized with symptoms suggesting metabolism disorders in skeleton system Department of Paediatric Propedeutics and Bone Metabolic Diseases Medical University of Lodz 1M. Konopnicka Pediatric Centre, Central Hospital of the Medical University od Lodz

Effect of vitamin D receptor gene (VDR) polymorphism on body height in children – own experience

UNLABELLED Genetic and environmental factors have an influence on the process of growth and development of the body. One of numerous genetic factors can be the vitamin D receptor gene (VDR). The study aimed at evaluating the relationship between VDR polymorphism and somatic parameters in children. PATIENTS AND METHODS The study group consisted of 395 children, aged 6-18 years. All the patients underwent gene typing using the PCR-RFLP method within polymorphic loci BsmI (rs1544410), FokI (rs2228570), ApaI (rs7975232) and TaqI (rs731236) of the VDR receptor gene. 294 children made up the control group in the study on the incidence of particular genotypes; in 161 patients somatic measurements of body weight and height were made with standard methods and skeletal densitometry (total body and spine programmes) examination was performed. Statistica 10.0 PL was used for statistical analysis. RESULTS In patients with low bone mass a relationship between body height and FokI VDR polymorphism was noted. The p-value was statistically significantly different in group I (p=0.002) and borderline significant in group III (p=0.09). None of the polymorphisms of the VDR receptor gene demonstrated any statistically significant differences in anthropometric values in the control group and in children with osteoporosis. SUMMARY The presence of the F allele of FokI polymorphism of the VDR receptor gene results in increased height, which is best observed in children with low bone mass. The FF genotype favours increased height in the study group of children from Łódź.

Recurrent fractures as a new skeletal problem in the course of Angelman syndrome

Angelman syndrome is a genetically inherited syndrome with severe retardation of psychomotor development and speech disturbances, usually accompanied by epilepsy, typical dysmorphic features, and some skeletal symptoms. The aim of the current report is to present new skeletal symptoms which may occur in the course of AS, based on a case report of an 8-year-old girl with confirmed 15q11;12 microdeletion and recurrent low-trauma bone fractures. According to our knowledge it is the first report of such skeletal symptoms in patient with a diagnosis of AS.

Selected risk factors of fractures in children — own observation

Bone fractures may depend on Vitamin D Receptor Gene (VDR), bone mineral density, bone turnover markers. Patients and methods. 161 patients were recruited and underwent: skeletal densitometry (DXA) method and bone turnover studies (Osteocalcin and Ntx).The study group was evaluated using restriction enzyme digestion at BsmI (rs1544410), FokI (rs2228570), ApaI (rs7975232) and TaqI (rs731236), polymorphic sites of the VDR gene. Multivariate logistic regression was used to assess factor significance. The model included variables with sex- and age-standardized parameters, VDR genotypes, and bone metabolism marker levels. Results. Factors associated with fractures were: osteocalcin concentration and Z-score BMDt. Odds Ratio (OR) values equaled: 1.01 (95%Confidence Interval (95%CI) 1.00–1.02) for osteocalcin (p=0.006), and 0.66 (95%CI 0.42-1.03; p=0.07) for Z-score BMDt. In patients with reduced bone mass, factors related to fractures were: osteocalcin (0.04) and carriage of BsmI b (0.07) or ApaI a alleles (0.08). ORs were 1.01 (95%CI 1.00–1.02) for OC, 0.29 (95%CI 0.07–1.14) for BsmI, and 2.13 (95%CI 0.91–4.99) for ApaI polymorphic allele carriage. Conclusions. Carriage of BsmI b allele reduces, while carriage of ApaI a allele and heightened osteoclacin level increase the risk of fractures in study children with reduced bone mass. VDR polymorphism, bone mineral density and bone formation’s marker — osteocalcin maybe considered as risk factor for fracure in children from Lodz region.