Inspiratory muscle training in Morquio's syndrome: A case study

Savcı, Sema; Öztürk, Melda; Inal-Ince, Deni̇z; Gültekin, Zuhal; Sivri, Hatice Serap

doi:10.1002/ppul.20500

Cited by 8 publications

(8 citation statements)

References 16 publications

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“…In these cases, hypoxic and hypercapnic (type 2) respiratory failure and respiratory muscle weakness may occur. Savcı et al (6) reported that respiratory muscle weakness in patients with Morquio syndrome is caused by metabolic abnormalities, wall deformities, and the muscles' not being used. In our case, hypoxemia and hypercapnia caused by kyphoscoliosis, pectus carinatum deformity, and respiratory muscle weakness were also present.…”

Section: Discussionmentioning

confidence: 99%

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Type 2 Respiratory Insufficiency, Pulmonary Hypertension, and Obstructive Sleep Apnea Syndrome that Developed in Morquio Syndrome

Gülhan¹,

Ekici²,

Ekici³

et al. 2015

Eurasian J Pulmonol

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Mucopolysaccharidosis type IVA (Morquio syndrome) is a multisystemic autosomal recessive transitive disease induced by an N-acetylgalactosamine 6 sulfatase (GALNS) gene defect, progressing with lysosomal stock failure. The accumulation of chondroitin 6 sulfate and keratin sulfate is observed in the tissues due to GALNS deficiency. We presented a case in which Morquio syndrome was successfully treated with noninvasive mechanical ventilation in accordance with the literature. The case had obstructive sleep apnea syndrome (OSAS) induced by an obstruction that emerged as a result of type 2 respiratory insufficiency resulting from throcal deformity, inspiratory muscle weakness, pulmonary hypertension, and the accumulation of diffuse mucopolysaccharide in the upper respiratory tract.

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Section: Discussionmentioning

confidence: 99%

“…The respiratory problems observed in Morquio syndrome contain restrictive-type respiratory failures caused by thoracic deformities and respiratory muscle weakness. Another reason for the respiratory problems in these patients is obstructive sleep apnea syndrome (OSAS) (4)(5)(6).…”

Section: Introductionmentioning

confidence: 99%

Type 2 Respiratory Insufficiency, Pulmonary Hypertension, and Obstructive Sleep Apnea Syndrome that Developed in Morquio Syndrome

Gülhan¹,

Ekici²,

Ekici³

et al. 2015

Eurasian J Pulmonol

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“…1.097 üniversite öğrencisi üze-rinde yaptıkları çalışmada, BKİ değeri 25 kg/m 2 'nin üzerinde ve altında olan bireylerin fiziksel aktivite düzeyleri arasında anlamlı fark olmadığını belirtmişlerdir. 4 Jimenez-Pavon ve ark., şiddetli fiziksel aktivite ile yağ kütlesi indekslerinin negatif, kas kütlesinin ise pozitif ilişkili olduğunu göstermiş-lerdir. 26 Çalışmamızda da fiziksel aktivite ile BKİ arasında ilişki saptanmamıştır.…”

Section: Gereç Ve Yöntemlerunclassified

“…Savcı ve ark., 2006 yılında Türkiye'de yaptıkları çalışmada, üniversite öğrencilerinin sadece %18'inin yeterli fiziksel aktiviteye, %68'inin ise düşük fiziksel aktivite düzeyine sahip olduğunu belirtmişlerdir. 4 Ayrıca çalışmalarda; düzenli fiziksel aktivitenin, özellikle kardiyovasküler hastalık-lar ve metabolik sendrom gibi sağlığı etkileyen risk faktörlerini önemli derecede azalttığı ve ortalama yaşam süresini yaklaşık 2-4 yıl artırdığı rapor edilmiştir. 1,5 Denge, destek yüzeyi içerisinde vücut kütle merkezinin kontrol edilebilme becerisi olarak adlandırılmaktadır.…”

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The Relationship Between Body Composition and Physical Activity, Balance and Supporting Factors in University Students

Taşpınar¹,

Seyyar²,

Kurt³

et al. 2017

Turkiye Klinikleri J Health Sci

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Ö ÖZ ZE ET T A Am ma aç ç: : Üniversite öğrencileri son yıllarda artan sedanter yaşam tarzları nedeni ile vücut kompozisyonlarına yeteri kadar özen göstermemektedirler. Bu nedenle çalışmamız, üniversite öğrenci-lerinde vücut kompozisyonu ile fiziksel aktivite, denge ve destekleyici faktörler arasındaki ilişkiyi incelemek amacıyla planlandı. G Ge er re eç ç v ve e Y Yö ön nt te em ml le er r: : Çalışmaya, yaş aralığı 18-25 yıl olan 132 (74 kadın, 58 erkek) genç erişkin öğrenci dâhil edildi. Vücut kompozisyonu ölçümü Tanita BC-418 vücut analiz cihazı ile yapıldı. Fiziksel aktivite düzeyinin değerlendirilmesi için Uluslararası Fiziksel Aktivite Anketi (UFAA)'nin kısa formu, dinamik denge için Y denge testi ve destekleyici faktörler için Depresyon, Anksiyete, Stres Ölçeği kullanıldı. Elde edilen veriler uygun istatistiksel yöntemlerle karşılaştırıldı. B Bu ul lg gu ul la ar r: : Beden kitle indeksi (BKİ) değerleri ile fiziksel aktivite ve Y denge testi parametreleri arasında istatistiksel olarak anlamlı ilişki olmadığı saptandı. Katılımcıla-rın toplam ve alt ekstremite kas oranı ile fiziksel aktivite düzeyleri ve Y denge testi parametreleri arasında pozitif yönde anlamlı ilişki belirlenirken, yağ oranı arasında negatif yönde anlamlı ilişki olduğu saptandı. BKİ ile anksiyete arasında önemsiz derecede zayıf bir ilişki belirlenirken, diğer destekleyici faktörler arasında anlamlı bir ilişki gözlenmedi. S So on nu uç ç: : Vücutta kas oranındaki artış fiziksel aktivite ve denge üzerinde olumlu sonuçlar gösterirken, yağ oranındaki artış olumsuz sonuçlar meydana getirmiştir. Bu yüzden, üniversite öğrencileri ideal vücut kompozisyonlarını kazanmalıdırlar. Bunun için fiziksel aktivite, düzenli ve ömür boyu sürdürülen bir alışkanlık haline getirilmelidir.A An na ah ht ta ar r K Ke el li im me el le er r: : Vücut bileşimi; motor aktivite; postural denge; öğrenciler A AB BS ST TR RA AC CT T O Ob bj je ec ct ti iv ve e: : University students have not taken care sufficiently to their body composition because of sedentary lifestyles in recent years. Thus, our study was planned out to investigate the relationship between body composition and physical activity, balance and supporting factors in university students. M Ma at te er ri ia al l a an nd d M Me et th ho od ds s: : One hundred thirty two young adult university students (74 female, 58 male) aged between 18-25 years were included in this study. Body composition measurement was performed with Tanita BC-418 body analyzer device. The short form of International Physical Activity Questionnaire (IPAQ) for assessing the level of physical activity, Y balance test for dynamic balance, and Depression, Anxiety, Stress Scale for supportive factors were used. The obtained data were compared with appropriate statistical methods. R Re es su ul lt ts s: : It was determined that there was no statistically significant relationship between body mass index (BMI) values and physical activity and Y balance test parameters. A significant positive relationship was detected between participants' tota...

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“…Health related behaviors acquired in the early period of life effect risks of life style related disorders which can occur in the later period. In this respect, research on health behaviors of young people is important [7,8].…”

Section: Introductionmentioning

confidence: 99%

Untitled

2019

IJAEP

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This study was conducted to examine the effect of daily lifestyle habits on physical activity level in adolescents. The research was carried out in Konya province, Meram Vocational and Technical Anatolian high school. The mean age of the participants was 16.44±1.89, the mean height was 167.45±8.56 and the mean body weight was 58.95±13.43. The physical activity levels of the subjects were monitored 7 days a week with a pedometer. The questionnaire developed by the researchers to determine daily life habits was used. In this study, <5000 steps were considered to be "very weak", steps between 5001-7500 were "weak", 7501-1000 were "normal", 1001-12500 were "good", and >12500 were "very good". According to the findings of the study, the physical activity levels of the subjects were 9207.83 ± 4295.30. Physical activity levels of children who went to school by school bus were determined as 9023,25 ± 4122,33 and 9373,42±4452,18 of the subjects who went on foot. The results displayed no significant differences in their time spent on television and computer, dietary habits, how they go to school and intenseness of physical activities according to level of physical activity. As a result, in the adolescent age which development is the fastest, malnutrition and the excess of time spent on television and computer, negatively affect the level of physical activity. It can be said that the physical activity levels of adolescents who have made inactive life and malnutrition as a lifestyle are "somewhat active".

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Inspiratory muscle training in Morquio's syndrome: A case study

Cited by 8 publications

References 16 publications

Type 2 Respiratory Insufficiency, Pulmonary Hypertension, and Obstructive Sleep Apnea Syndrome that Developed in Morquio Syndrome

Type 2 Respiratory Insufficiency, Pulmonary Hypertension, and Obstructive Sleep Apnea Syndrome that Developed in Morquio Syndrome

The Relationship Between Body Composition and Physical Activity, Balance and Supporting Factors in University Students

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