A Osteogênese Imperfeita (OI) é caracterizada por fragilidade óssea e susceptibilidade a fraturas. As complicações da patologia podem afetar o desenvolvimento físico e motor, comprometendo as habilidades funcionais, o nível da marcha e a independência do indivíduo. Objetivo: Avaliar as características clínico-funcionais de crianças e adolescentes com Osteogênese Imperfeita (OI). Métodos: Estudo transversal no qual foram avaliados sujeitos de ambos os gêneros com idade entre 0 e 18 anos e diagnóstico de OI, em tratamento no CROI-RS. Os dados clínicos e funcionais foram coletados através de fichas específicas e avaliação funcional. Foram avaliados aspectos relacionados à presença de fraturas e deformidades ósseas, habilidade da marcha, força muscular e amplitude de movimento articular. A densidade mineral óssea (DMO) foi mensurada através do Dual Energy X-Ray Absoptometry (DEXA). O nível de significância adotado foi de 5% (p≤0,05). Resultados: Encontramos diferença significativa na ocorrência de fraturas, presença de deformidades ósseas, uso de haste intramedular, baixa densidade mineral óssea, tratamento medicamentoso e aspectos relacionados à marcha comparando OI tipo I, III e IV. As formas mais graves de OI (tipo III e IV) apresentaram fraturas nos primeiros meses de vida, maior deformidade óssea. Associação inversa entre amplitude de movimento articular geral e o nível da marcha e uma associação direta com idade de início de marcha, o número total de fraturas e a presença de deformidades ósseas. Encontrou-se uma associação direta entre a força muscular geral e o nível da marcha e uma associação inversa com a idade de início de marcha e presença de deformidades ósseas. Conclusão: Estes dados sugerem que as características clínico-funcionais variam de acordo com os tipos de OI. Nas formas moderada e grave de OI há maior limitação funcional influenciada pelo número de fraturas e presença de deformidades ósseas afetando negativamente o nível da marcha. / Osteogenesis Imperfecta (OI) is characterized by bone fragility and susceptibility to fractures. Complications of the disease can affect the physical and motor development, compromising the functional skills, level of gait and independence of the individual. Objective: To evaluate the clinical and functional features of children and adolescents with OI. Methods: A cross-sectional study which evaluated subjects of both genders aged between 0 and 18 years attended in the Reference Center for Treatment of OI of Rio Grande do Sul (CROI-RS). Clinical and functional data were evaluated through specific tokens and functional assessment. We evaluated aspects related to the presence of fractures and bone deformities, gait ability, muscle strength, joint range of motion and use of intramedullary rod. Bone mineral density (BMD) was measured by Dual Energy X-Ray Absoptometry (DEXA). The level of significance was set at 5% (p ≤ 0.05). Results: We found significant differences in the occurrence of fractures, presence of bone deformities, use of intramedullary rod, bone mineral density, drug therapy and aspects related to gait comparing OI types I, III and IV. The age of gait acquisition showed a direct association with overall joint range of motion and an inverse relationship with overall muscle strength. The level of ambulation was directly associated with overall muscle strength and inversely associated with overall joint range of motion. Conclusion: Our findings confirm that clinical and functional features vary according to OI type. Moderate and severe forms of OI are associated with greater functional limitation, influenced by fractures in early life, number of fractures and the presence of bone deformities, which negatively affect the acquisition and level of ambulation.

A Osteogênese Imperfeita (OI) é caracterizada por fragilidade óssea e susceptibilidade a fraturas. As complicações da patologia podem afetar o desenvolvimento físico e motor, comprometendo as habilidades funcionais, o nível da marcha e a independência do indivíduo. Objetivo: Avaliar as características clínico-funcionais de crianças e adolescentes com Osteogênese Imperfeita (OI). Métodos: Estudo transversal no qual foram avaliados sujeitos de ambos os gêneros com idade entre 0 e 18 anos e diagnóstico de OI, em tratamento no CROI-RS. Os dados clínicos e funcionais foram coletados através de fichas específicas e avaliação funcional. Foram avaliados aspectos relacionados à presença de fraturas e deformidades ósseas, habilidade da marcha, força muscular e amplitude de movimento articular. A densidade mineral óssea (DMO) foi mensurada através do Dual Energy X-Ray Absoptometry (DEXA). O nível de significância adotado foi de 5% (p≤0,05). Resultados: Encontramos diferença significativa na ocorrência de fraturas, presença de deformidades ósseas, uso de haste intramedular, baixa densidade mineral óssea, tratamento medicamentoso e aspectos relacionados à marcha comparando OI tipo I, III e IV. As formas mais graves de OI (tipo III e IV) apresentaram fraturas nos primeiros meses de vida, maior deformidade óssea. Associação inversa entre amplitude de movimento articular geral e o nível da marcha e uma associação direta com idade de início de marcha, o número total de fraturas e a presença de deformidades ósseas. Encontrou-se uma associação direta entre a força muscular geral e o nível da marcha e uma associação inversa com a idade de início de marcha e presença de deformidades ósseas. Conclusão: Estes dados sugerem que as características clínico-funcionais variam de acordo com os tipos de OI. Nas formas moderada e grave de OI há maior limitação funcional influenciada pelo número de fraturas e presença de deformidades ósseas afetando negativamente o nível da marcha. / Osteogenesis Imperfecta (OI) is characterized by bone fragility and susceptibility to fractures. Complications of the disease can affect the physical and motor development, compromising the functional skills, level of gait and independence of the individual. Objective: To evaluate the clinical and functional features of children and adolescents with OI. Methods: A cross-sectional study which evaluated subjects of both genders aged between 0 and 18 years attended in the Reference Center for Treatment of OI of Rio Grande do Sul (CROI-RS). Clinical and functional data were evaluated through specific tokens and functional assessment. We evaluated aspects related to the presence of fractures and bone deformities, gait ability, muscle strength, joint range of motion and use of intramedullary rod. Bone mineral density (BMD) was measured by Dual Energy X-Ray Absoptometry (DEXA). The level of significance was set at 5% (p ≤ 0.05). Results: We found significant differences in the occurrence of fractures, presence of bone deformities, use of intramedullary rod, bone mineral density, drug therapy and aspects related to gait comparing OI types I, III and IV. The age of gait acquisition showed a direct association with overall joint range of motion and an inverse relationship with overall muscle strength. The level of ambulation was directly associated with overall muscle strength and inversely associated with overall joint range of motion. Conclusion: Our findings confirm that clinical and functional features vary according to OI type. Moderate and severe forms of OI are associated with greater functional limitation, influenced by fractures in early life, number of fractures and the presence of bone deformities, which negatively affect the acquisition and level of ambulation.

A Osteogênese Imperfeita (OI) é caracterizada por fragilidade óssea e susceptibilidade a fraturas. As complicações da patologia podem afetar o desenvolvimento físico e motor, comprometendo as habilidades funcionais, o nível da marcha e a independência do indivíduo. Objetivo: Avaliar as características clínico-funcionais de crianças e adolescentes com Osteogênese Imperfeita (OI). Métodos: Estudo transversal no qual foram avaliados sujeitos de ambos os gêneros com idade entre 0 e 18 anos e diagnóstico de OI, em tratamento no CROI-RS. Os dados clínicos e funcionais foram coletados através de fichas específicas e avaliação funcional. Foram avaliados aspectos relacionados à presença de fraturas e deformidades ósseas, habilidade da marcha, força muscular e amplitude de movimento articular. A densidade mineral óssea (DMO) foi mensurada através do Dual Energy X-Ray Absoptometry (DEXA). O nível de significância adotado foi de 5% (p≤0,05). Resultados: Encontramos diferença significativa na ocorrência de fraturas, presença de deformidades ósseas, uso de haste intramedular, baixa densidade mineral óssea, tratamento medicamentoso e aspectos relacionados à marcha comparando OI tipo I, III e IV. As formas mais graves de OI (tipo III e IV) apresentaram fraturas nos primeiros meses de vida, maior deformidade óssea. Associação inversa entre amplitude de movimento articular geral e o nível da marcha e uma associação direta com idade de início de marcha, o número total de fraturas e a presença de deformidades ósseas. Encontrou-se uma associação direta entre a força muscular geral e o nível da marcha e uma associação inversa com a idade de início de marcha e presença de deformidades ósseas. Conclusão: Estes dados sugerem que as características clínico-funcionais variam de acordo com os tipos de OI. Nas formas moderada e grave de OI há maior limitação funcional influenciada pelo número de fraturas e presença de deformidades ósseas afetando negativamente o nível da marcha. / Osteogenesis Imperfecta (OI) is characterized by bone fragility and susceptibility to fractures. Complications of the disease can affect the physical and motor development, compromising the functional skills, level of gait and independence of the individual. Objective: To evaluate the clinical and functional features of children and adolescents with OI. Methods: A cross-sectional study which evaluated subjects of both genders aged between 0 and 18 years attended in the Reference Center for Treatment of OI of Rio Grande do Sul (CROI-RS). Clinical and functional data were evaluated through specific tokens and functional assessment. We evaluated aspects related to the presence of fractures and bone deformities, gait ability, muscle strength, joint range of motion and use of intramedullary rod. Bone mineral density (BMD) was measured by Dual Energy X-Ray Absoptometry (DEXA). The level of significance was set at 5% (p ≤ 0.05). Results: We found significant differences in the occurrence of fractures, presence of bone deformities, use of intramedullary rod, bone mineral density, drug therapy and aspects related to gait comparing OI types I, III and IV. The age of gait acquisition showed a direct association with overall joint range of motion and an inverse relationship with overall muscle strength. The level of ambulation was directly associated with overall muscle strength and inversely associated with overall joint range of motion. Conclusion: Our findings confirm that clinical and functional features vary according to OI type. Moderate and severe forms of OI are associated with greater functional limitation, influenced by fractures in early life, number of fractures and the presence of bone deformities, which negatively affect the acquisition and level of ambulation.

Bibliographical identification Title of Diplom work: Degree of the relationship between the level endomorfie and neuromotor age for selected soccer players U12 category of the highest competition in the Czech Republic Place of work: UK FTVS Author: Jakub Šlitr Field of study: Physical education and sport Head of work: Mgr. Jakub Kokštejn Ph.D. Defence year: 2016 Generalization Title: Degree of the relationship between the level endomorfie and neuromotor age for selected soccer players U12 category of the highest competition in the Czech Republic Objectives: The aim of this thesis is to find differences in body composition parameters selected and neuromotor development at two football teams in the U12 age category. At the same time the aim of the thesis to determine possible relationships between the studied indicators of body composition and psychomotor development Methods: To detect psychomotor development we used a battery of tests BOT-2, we used the short form of the test battery BOT-second Anthropometric data were obtained using the method to determine the somatotype Health-Carter. Values were measured using anthropometric instruments: weight, caliper, measuring tape, caliper and stadiometer. Results: Among the teams of Slavia and Sparta were found factually or statistically significant differences in...

Bibliographical identification Title of Diplom work: Degree of the relationship between the level endomorfie and neuromotor age for selected soccer players U12 category of the highest competition in the Czech Republic Place of work: UK FTVS Author: Jakub Šlitr Field of study: Physical education and sport Head of work: Mgr. Jakub Kokštejn Ph.D. Defence year: 2015 Generalization Title: Degree of the relationship between the level endomorfie and neuromotor age for selected soccer players U12 category of the highest competition in the Czech Republic Objectives: The aim of this thesis is to find differences in body composition parameters selected and neuromotor development at two football teams in the U12 age category. At the same time the aim of the thesis to determine possible relationships between the studied indicators of body composition and psychomotor development Methods: To detect psychomotor development we used a battery of tests BOT-2, we used the short form of the test battery BOT-second Anthropometric data were obtained using the method to determine the somatotype Health-Carter. Values were measured using anthropometric instruments: weight, caliper, measuring tape, caliper and stadiometer. Results: Among the teams of Slavia and Sparta were found factually or statistically significant differences in...

Made available in DSpace on 2014-12-17T14:13:47Z (GMT). No. of bitstreams: 1 CarlaISA_DISSERT.pdf: 627930 bytes, checksum: 7c426688b937c0d47c15acbf76266f27 (MD5) Previous issue date: 2010-06-11 / This study compared the evolution of posture, tone and neonatal reflexes in preterm infants without clinical or neurological complications before and after the age of term using the scale of Saint-Anne Dargassies. To reach the age of the term, was applied Scales Amiel-Barrier-Shnider changed and the Prechtl, traditionally used in the evaluation of term newborns, looking for possible changes to the term, while also evaluating the sensitivity of these scales. We studied 20 non-complicated preterm infants, both sexes aged 32-36 weeks, born in Janu?rio Cicco Maternity School, from August 2006 to August 2007. Was applied to the scale of Saint-Anne Dargassies every two weeks until reach the term, and the range of Amiel-Barrier-Shnider changed and the Prechtl, after reaching 39 and 41 weeks. The evaluation result of articular angles was subjected to the test of Friedman ANOVA, significant differences between the three measurements of the scale of Saint-Anne Dargassies only for angles heel-to-ear to term. Neonatal reflexes changed in the period of prematurity were the cardinal points reflexes, Moro reflexes, cross extensions reflex and the automatic walking reflexes. The posture was the parameter which remained unchanged in the three scales. Considering a significance level of 5% by applying Cochran Q Test, it was found that the scale of Saint-Anne Dargassies is more sensitive to detect suspects. With this methodology and the results it was possible to prepare a manuscript: The neurological examination of non-complicated preterm newborns using the Sanit-Anne Dargssies Scale from birth to term: normal or altered? In which we describe that despite the good clinical condition, the RNP show changes in tone and neonatal reflexes. These data are important because though non-complicated RNP need further attention its maturation process, enabling us to detect and intervene early. With these results we can build a scale simplified neurological assessment made with items found most altered during the application of three scales. The development of this project has a multidisciplinary approach, because it involved Paediatric Neurologist, Physiotherapist and Neonatologist, as recommended by PPGCSA / Esse estudo comparou a evolu??o da postura, reflexos neonatais e t?nus nos prematuros sem complica??es cl?nicas ou neurol?gicas antes e ap?s a idade de termo utilizando a escala de Saint-Anne Dargassies. Ao atingir a idade de termo, foi aplicado as Escalas de Amiel-Barrier-Shnider modificada e a de Prechtl, tradicionalmente utilizadas na avalia??o do RN de termo, a procura de poss?veis altera??es ao termo e ao mesmo tempo avaliando tamb?m a sensibilidade dessas escalas aplicadas. Foram estudados 20 prematuros n?o complicados, ambos os sexos com idade gestacional variando de 32 a 36 semanas, nascidos na Maternidade Escola Janu?rio Cicco, no per?odo de agosto de 2006 a agosto de 2007. Foi aplicada a escala de Saint-Anne Dargassies a cada 2 semanas at? atingir o termo, e a escala de Amiel-Barrier-Shnider modificada e a de Prechtl, ap?s atingirem 39 e 41 semanas. O resultado da avalia??o dos ?ngulos articulares foi submetido ao teste de Friedman ANOVA, observando-se diferen?as significativas entre as 3 medi??es da escala de Saint-Anne Dargassies, apenas para os ?ngulos calcanhar-orelha at? o termo. Os reflexos neonatais alterados no per?odo de prematuridade foram os dos pontos cardinais, reflexo de Moro, reflexo de alargamento cruzado e o de marcha autom?tica. A postura foi o par?metro que se manteve sem altera??es nas 3 escalas. Considerando um n?vel de signific?ncia de 5% ao aplicar o Cochran Q Test, constatou-se que a escala da Saint-Anne Dargassies ? mais sens?vel para detectar indiv?duos suspeitos. Com esta metodologia e com os resultados obtidos foi poss?vel elaborarmos um manuscrito: The neurological examination of non-complicated preterm newborns using the Sanit-Anne Dargssies Scale from birth to term: normal or altered? No qual descrevemos que, apesar da boa condi??o cl?nica, os RNP apresentam altera??es de t?nus e reflexos neonatais. Esses dados s?o importantes porque apesar de RNP n?o complicados necessitam de uma maior aten??o em todo seu processo maturacional, o que nos permitiria detectar e fazer uma interven??o precoce. Com os resultados obtidos conseguimos construir uma escala simplificada de avalia??o neurol?gica constitu?da com os itens mais alterados encontrados durante a aplica??o das 3 escalas. O desenvolvimento deste projeto tem um enfoque multidisciplinar, pois envolveu Neurologista Pediatra, Fisioterapeuta e Neonatologista, como preconizado pela PPGCSA

Made available in DSpace on 2016-12-06T17:07:18Z (GMT). No. of bitstreams: 1 Joyce Ribeiro.pdf: 4347798 bytes, checksum: 9df5743fbbbc2c7e90ea438ed800f2b5 (MD5) Previous issue date: 2009-03-06 / Coordenação de Aperfeiçoamento de Pessoal de Nível Superior / The technological advances have allowed a greater survival these newborns at risk. However, the biological risk factors to which these children were exposed make it have a greater chance to present delay in their growth and development. The aim of this study was evaluate the repercussions of the biological risk factors in the neuromotor development of the nurslings from birth to two months of life. The population was represented by all children with age from birth to two months of life that during the data collection period presented born register in the health services from Fraiburgo SC. The sample was constituted to 80 nurslings, being 47 (58,8%) of male sex and 33 (41,3%) of female sex. The children valued were divided in two groups (control and experimental) and subdivided in four subgroups according to the age in days that were. The experimental group was constituted to 25 nurslings with biological risk history. The control group was compost to 55 nurslings without risk history. The instruments used for the data collection were: M-flex, the Neonatal Behavioral Assesment Scale, biopsychosocial form and the child health card. The data analysis was accomplished by SPSS system, version 13.0. The statistic tests used were: t test to independent sample, U Mann, Witnney and Spearman tests. Was found prevalence of married mothers, complete high school or higher education and practiced remunerated activity. The maternal mean age during the parturition was 28 years old, being that a significant number of mothers presented between 34 and 40 years old in either groups. There were events of anemia, hypertension and/or hypotension, diabetes, syphilis and bleeding between the diseases that affected the mothers during the gestational period. The type of parturition more common was Cesarean in either groups. The mean of gestational age was 37,92 weeks, being that 12 nurslings presented prematurity. The nurslings of the risk group presented Apgar index in the first and fifth minute, cephalic perimeter, weight and length at the birth under at the nurslings group without risk history, also demonstrated neuromotor performance under at the nurslings group without risk history with respect to reflexes. Nurslings with biological risk presents grip pattern of the right and the left hands under at the group without risk history. This study showed that the biological risk factors them will can carry repercussions with respect to neuromotor development, due to the fact that the children of the risk group presented differentiated patterns compared the children without risk history as the motor development. / Os avanços tecnológicos têm permitido uma maior sobrevivência de recém nascidos de risco. No entanto, os fatores de risco biológicos aos quais essas crianças foram expostas fazem com que tenham maior chance de apresentar atraso no seu crescimento e desenvolvimento. O objetivo deste estudo foi avaliar as repercussões de fatores de risco biológicos no desenvolvimento neuromotor de lactentes do nascimento aos dois meses de vida. A população deste estudo foi representada por todas as crianças com idade compreendida do nascimento aos dois meses de vida, que durante o período de coleta de dados apresentou registro de nascimento junto as unidades de Saúde do município de Fraiburgo-SC. A amostra foi composta de 80 lactentes, sendo que 47 (58,8%) do sexo masculino e 33 (41,3%) do sexo feminino. As crianças avaliadas foram separadas em dois grupos (controle e experimental) e subdivididas em quatro subgrupos de acordo com a idade em dias que se encontravam. O Grupo Experimental foi constituído por 25 lactentes que apresentaram fatores de risco biológicos. O Grupo controle foi composto por 55 lactentes sem histórico de risco. Os instrumentos utilizados para coleta dos dados foram: M-flex, Neonatal Behavioral Assessment Scale, formulário biopsicossocial e cartão de saúde da criança. Os dados foram tratados através do programa SPSS versão 13.0. Os testes estatísticos utilizados foram: teste t para amostra independentes, teste U Mann Witnney e Spearman. Constatou-se prevalência de mães casadas, com ensino médio ou superior completo e que exerciam atividade remunerada. A idade média das mães durante o parto foi de 28 anos sendo que um número significativo de mães apresentou idade entre 34 e 40 anos em ambos os grupos. Constatou-se casos de anemia, alterações pressóricas, diabetes, sífilis e hemorragias entre as patologias que acometeram as mães durante o período gestacional. O tipo de parto mais freqüente foi o parto Cesário em ambos os grupos. A média de idade gestacional foi de 37,92 semanas, sendo que 12 lactentes apresentaram prematuridade. Os lactentes do grupo de risco apresentaram índices de Apgar no primeiro e quinto minuto, perímetros cefálicos, pesos e comprimentos ao nascimento inferiores aos do grupo sem histórico de risco, também demonstraram desempenho neuromotor inferior aos lactentes sem histórico de risco em relação aos reflexos. Lactentes de risco biológico apresentaram padrões de força da mão direita e esquerda inferiores ao grupo sem histórico de risco. Este estudo demonstrou que os fatores de risco biológico poderão trazer repercussões em relação ao desenvolvimento neuromotor, visto que as crianças do grupo de risco apresentaram padrões diferenciados em relação às crianças sem histórico de risco quanto à motricidade.

Classés parmi les Polluants Organiques Persistants, les Hydrocarbures Aromatiques Polycycliques (HAP) sont des composés ubiquitaires dans l'environnement, auxquels l'Homme est exposé principalement via l'ingestion d'aliments contaminés. Les HAP sont connus depuis les années 1990 comme pouvant être neurotoxiques tant chez l'Homme que chez l'animal. Le transfert des HAP entre la mère et le foetus via le placenta ainsi que la présence de ces composés dans le lait maternel ont été montrés à plusieurs reprises, posant ainsi la question du risque lié à une exposition survenant lors de phases précoces du développement de l'individu. Parce que ce risque n'a été que partiellement étudié, le travail de thèse présenté dans ce manuscrit a eu pour objectif d'évaluer la toxicité à court et à long terme pour le système nerveux en développement, d'un mélange de 16 HAP ingéré par la rate gestante et/ou allaitante. Les HAP ont été administrés via un aliment contaminé à deux doses, 2 et 200 µg/kg/jour La dose la plus faible correspond aux niveaux de contamination environnementale via l'alimentation de la mère pendant les périodes de gestation et/ou d'allaitement. Les résultats montrent que l'exposition périnatale au mélange de HAP n'a pas induit d'effets à court terme sur le développement neuromoteur et sensoriel des jeunes rats. En revanche, une augmentation à long terme des niveaux d'activité et d'anxiété a été relevée chez les animaux exposés. Par ailleurs, des modifications du métabolisme énergétique cérébral, évalué par l'activité enzymatique de la cytochrome oxydase sur des coupes de cerveaux prélevés à différents âges ont été observées, notamment au niveau du système limbique. La mesure des concentrations de HAP dans le compartiment cérébral des ratons a montré la présence de l'ensemble des molécules mères, y compris chez les animaux témoins, ce qui laisse supposer l'existence d'un bruit de fond environnemental non négligeable dans ce type d'étude. Finalement, plusieurs facteurs modulent la toxicité induite, parmi lesquelles la période d'exposition, et l'administration d'un mélange et non d'une molécule seule. En conclusion, ce travail a permis de mettre en évidence, chez le rat, une neurotoxicité retardée suite à une exposition précoce à un mélange de 16 HAP tant sur le plan comportemental que métabolique, ce qui pose la question du risque pour l'Homme, et en particulier pour l'individu en développement, d'une exposition à ce type de composés / Compounds in the environment to which human is exposed mainly through ingestion of contaminated food. PAHs are known since the 1990s as being neurotoxic both in humans and in animals. PAH transfer between mother and fetus through the placenta, and the presence of these compounds in breast milk have been shown, thus raising the question of risk exposure occurring during early stages of development of the individual. Because this risk was only partially studied, the thesis presented in this manuscript was designed to assess the short-term and long-term toxicity for the developing nervous system, of a mixture of 16 PAHs ingested by the pregnant and / or breastfeeding rat. PAHs were administered via a contaminated food at two doses, 2 and 200 µg/kg /day dose. The lowest one corresponds to the levels of environmental contamination via the mother's diet during gestation and/or lactation. The results show that perinatal exposure to the mixture of PAHs did not induce short-term effects on neuromotor and sensory development on pups. In contrast, a long-term increase in activity and anxiety levels was observed in the exposed animals. In addition, changes in cerebral energy metabolism, as assessed by the enzymatic activity of cytochrome oxidase on brain sections taken at different ages were observed, particularly in the limbic system. Measures of the concentrations of PAHs in the pup brain compartment showed the presence of all the parent compounds, including control animals, suggesting the existence of an environmental noise significant. Finally, several factors modulate PAH toxicity, including the exposure period, and the administration of a mixture instead of a single molecule. In conclusion, this work has highlighted, in rats, delayed neurotoxicity due to early exposure to a mixture of 16 PAHs, which raises the question of risk to humans, and in particular for the individual developing exposure to such compounds

Clefts of orofacial komplex are the most common birth development defects. One of them is Pierre Robin sequence, which is characterized by presence of micrognathia, glossoptosis and upper airway obstruction. Because of these symptoms, isolated cleft palate "U" or "V" shaped can be part of the diagnose. Other clinical manifestations are feeding difficulties, obstructive sleep apnea syndrome and / or gastroesophageal reflux disease. The aim of the treatment is to secure the airway and the child's thriving after birth by using conservative or invasive treatment methods. Choosing of methods and procedures should be established by multidisciplinary cooperation. The other part of diploma thesis is trying to find out, which clinical manifestation are the most common in patients from the Czech Republic compare to foreign patients and the differences in management of the treatment. Furthermore we are trying to investigate, whether there is higher percentage of patients with delayed neuromotor development among PRS patients, which are under physical therapy. The last part is trying to find out the relationship between the poor posture and PRS diagnose in children age 4 - 6 years.