Physical activity and bone mass density in ?-thalasemia subjects (A Review)
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Physical activity and bone mass density in |3-thalasemia subjects (A Review)
MSc. Genti Pano, PhD Student at Sport Sciences Research Institute, Department of Physical Activity Health and Recreation Research,
Sports University of Tirana, Albania E-mail: genti. pano@outlook. com Qna Robert,
Prof. Assoc. Dr., Deparment of Sport Medicine, Faculty of Movement Sciences, Sports University of Tirana, Albania
Physical activity and bone mass density in p-thalasemia subjects (A Review)
Abstract: Beta thalassemia is a blood disorder that reduces the production of hemoglobin. Patients with thalassemia show a variety of bone disorders that include bone deformities, osteopenia and osteoporosis, growth failure, and spinal deformities Karimi .
Main objective of this paper was to review the recent literature regarding physical activity influence on bone mass density in-thalassemic subjects.
The search was made mainly focusing in PubMed, ResearchGate, Hinari, for studies focusing on PA intervention in в-thalassemia subjects. Only 6 papers fulfilled the inclusion criterias.
There is a lack of studies focusing in PA influence on bone mass density in these patients category. Further research is needed to identify risk factors, means of prevention and also to establish specific PA engagment guidelines and recomandations for this social category.
Keywords: Thalassemia, Bone mass density, Physical Activity.
Beta thalassemia is a blood disorder that reduces the production of hemoglobin. Patients with thalassemia show a variety of bone disorders that include bone deformities, osteopenia and osteoporosis, growth failure, and spinal deformities . Beta (в-) thalassemia syndromes of which в-thalassemia major, also known as Mediterranean anemia or Cooley’s Anemia, is the clinically most severe one.
The major beta (в-) thalassaemia syndromes are:
• в- thalassemia-
• в-thalassemia intermedia-
• Other rare thalassemia’s.
Approximately 7% of the global population is a carrier for Hemoglobin disorders. A carrier of a pathological Hb gene encounters no health problems. Between
300. 000- 500,000 children are born annually with a severe hemoglobin disorder. About 80% of affected children are born in middle and low income countries. About 70% are born with sickle cell and the rest with thalassemia disorders. 50−80% of children with sickle cell anemia and
50. 000- 100,000 children with в-thalassemia major die
each year in low and middle income countries World Bank 2006 , (report of a joint WHO-March of Dimes meeting 2006). Patients with в-thalassemia major, the most severe form of thalassemia, cannot make adult hemoglobin, and as a consequence cannot produce normal red blood cells. In these individuals, each red blood cell contains much less hemoglobin, and there are far fewer red cells than the normal range. This causes anemia, which is severe in these patients. A child with в-thalassemia major does not, however, develop severe anemia until 3 months to one year of age. If left untreated, affected children have a very poor quality of life and most will die at a very young age. The basic components of management in these patients are blood transfusion and iron chelation.
Main objective of this paper was to review the recent literature regarding physical activity influence on bone mass density in thalassemic subjects (ft-thalassemic specifically).
The search was made mainly focusing in: PubMed, ResearchGate, Hinari, for studies focusing on PA intervention in в-thalassemia subjects.
Section 8. Medical science
Results 3. Fung E. B. ., 2012-
Only 6 papers fulfilled the inclusion criterias: 4. Hamidieh A. A. ., 2013-
1. Vincent L. - 5. Valizadeh N. ., 2014-
2. Fung E. B. ., 2010- 6. Schundeln M. M. ., 2014.
Author’s Name Year of publication Type of study Number of subjects Type of intervention
Vincent L. et al. 2010 Not specified 5 subjects with a-t (a-t), 6 SCT carriers (SCT) and 9 SCT carriers with a-t (SCT/a-t). Skeletal muscle histomorphological and energetic characteristics. Subjects underwent a muscle biopsy and also performed an incremental maximal exercise and a time to exhaustion test.
Fung E. B. et al. 2010 Cross-sectional, multicenter study 386 257 transfused thalassemia patients compared with 113 non-transfused patients. Fat, lean, and bone mineral density (BMD) were assessed by DXA. Medical history, food frequency and physical activity questionnaires.
Fung E. B. et al. 2012 A longitudinal cross-over pilot trial 18 Subjects were asked to stand on a vibrating platform (30 Hz, 0.3 g) for 20 min/day for 6 months.
Hamidieh A. A. et al. 2013 Not specified 20 Bone mineral density (BMD) of 20 patients from three thalassemia classes whose mean (SD) age was 7.4 (3. 8) years were tested with a Norland XR-46 device at baseline (before transplantation), 6 and 12 months after transplantation.
Valizadeh N. et al. 2014 Cross sectional descriptive study 10 (younger than 18 years old) scanned for Bone Mineral Density (BMD).
Schundeln M. M. et al. 2014 cross-sectional study 45 Biochemical, radiographic and anamnestic parameters of bone health were assessed.
Results and Discussion
In Vincent L. et al., 2010 study, the focus were the measurements of skeletal muscle histomorphological and energetic characteristics. 10 control HbAA subjects ©, 5 subjects with a-t (a-t), 6 SCT carriers (SCT) and 9 SCT carriers with a-t (SCT/a-t) were enrolled and became part of study. Subj ects have underwent a muscle biopsy and also performed an incremental maximal exercise and a time to exhaustion test. In Fung E. B. , 2010 study, the subjects were assessed by DXA for: Fat, lean, and bone mineral density (BMD). Also- medical history, food frequency and physical activity questionnaires were conducted. The main objective of Fung E. B. , 2012 study was to evaluate the effectiveness of low magnitude whole body vibration (WBV) therapy on bone. Subjects were asked to stand on a vibrating platform (30 Hz, 0.3 g.) for 20 min/day for 6 months. Areal bone mineral density (a BMD) by DXA and volumetric BMD by peripheral quantitative computed tomography (pQCT) was assessed at baseline, 6 and 12 months.
Hamidieh A. A. , 2013 study assesses the adverse effects of transplantation on growing bones of pediatric thalas-semic patients. Bone mineral density (BMD) of 20 patients from three thalassemia classes were tested at baseline (before transplantation), 6 and 12 months after transplantation.
Transfusion duration and chelation therapy had shown positive significant relationships to BMD (g/cm2), but no significant relation with the BMD Z-score. The aim of Valizadeh N. , 2014 study was to assess the frequency of bone loss in patients with thalassemia major and intermedia in Urmia City ofWest Azerbaijan, Iran 10 patients (younger than 18 years old) with transfusion dependent thalassemia attending to Motahari and Emam Khomeini hospitals in Urmia city of Iran were enrolled and scanned for Bone Mineral Density (BMD). Among them, 8 patients had low BMD and 2 of them had normal BMD in lumbar spine. 6. The Schundeln M. M. , 2014 study focus was to assess bone health in pediatric patients with chronic hemolytic anemia. A cross-sectional study was conducted involving 45 patients with different forms of hemolytic anemia (i. e., 17 homozygous sickle cell disease and 14 hereditary spherocytosis patients). Biochemical, radiographic and anamnestic parameters of bone health were assessed. Patients with homozygous sickle cell anemia were more frequently and more severely affected by impaired bone health than patients with hereditary spherocytosis.
Conclusions & amp- Recommendation’s Based on the reviewed studies we can conclude that: There is a lack of studies focusing in PA influence on bone mass density in these patients category. According
Reproductive health and hormonal status of women with polycystic ovary syndrome
to Fung E. B. , 2010, the maj ority of adult patients with thalassemia had healthy body composition with rare obesity, young, non-transfused patients appear at risk for being underweight. Findings of Fung E. B. , 2012 study suggest that vibration therapy may be an effective non-pharmacologic intervention in Thal. According to Valizadeh N. , 2014 patients with all type thalassemia and hemoglobin H disease in age of higher than 8 year old should: Perform annual BMD and Treat low BMD with different medications (administration ofbisphosphonate, calcium and vitamin D supplements). Medical consultation
with a rheumatologist and/or an endocrinologist should be performed in these patients. Adequate calcium containing foods, avoiding heavy activities, stop smoking, iron chelation therapy in adequate dosage, early diagnosis and treatment of endocrine insufficiency and regular blood transfusions can help to achieve an optimal bone density in these patients. Changing lifestyle with mild daily exercise,
Further research is needed to identify: Risk factors, means of prevention and also to establish specific PA engagment guidelines and recomandations for this social category.
1. Fung Ellen B., Xu Yan, Kwiatkowski Janet, Vogiatzi Maria G., Neufeld Ellis, Olivieri Nancy, Vichinsky Elliott P., Giardina Patricia J., and Thalassemia Clinical Research Network. //J Pediatr. — 2010, October. — 157 (4): 641−647. — e2. doi: 10. 1016/j. jpeds. 2010. 04. 064.
2. Fung Ellen B., Gariepy Catherine A., Sawyer Aenor J., Higa Annie, and Vichinsky Elliott P. The effect of whole body vibration therapy on bone density in patients with thalassemia: A pilot study. //American Journal of Hematology. — Published online 28 June 2012 in Wiley Online Library//[Electronic resource]. — Available from: htpp: //wileyonlinelibrary. com. -DOI: 10. 1002/ajh. 23 305
3. Valizadeh N. M., Farrokhi F., Alinej ad V., Said Mardani S. M., Valizadeh N., Hej azi S., Noroozi M. Bone density in transfusion dependent thalassemia patients in Urmia, Iran. //Iranian Journal of Pediatric Hematology Oncology. — Vol 4. — No 2. — 2014.
4. Vincent L., Feasson L., Oyono-Enguelle S., Banimbek V., Monchanin G., Dohbobga M., Wouassi D., Martin C., Gozal D., Geyssant A., Thiriet P., Denis C., Messonnier L. Skeletal muscle structural and energetic characteristics in subjects with sickle cell trait, -thalassemia, or dual hemoglobinopathy. //J Appl Physiol. — 2010. — 109: 728−734.
5. Hamidieh A. A., Hamidi Z., Nedaeifard L., Heshmat R., Alimoghaddam K., Larijani B., Ghavamzadeh A., Mohajeri-Teh-rani M. R. Changes of Bone Density in Pediatric Patients with-thalassemia Major after Allogenic Hematopoietic Stem Cell Transplantation. //Arch Iran Med. — 2013. — 16 (2): 88−92.
6. Karimi M., Fotouhi Ghiam A., Hashemi A., Alinejad S., Soweid M., Kashef S. Bone mineral density in betathalassemia major and intermedia. //Indian Pediatr. — 2007. — 44 (1): 29−32.
7. Schundeln M. M., Goretzki S. C., Hauffa P. K., Wieland R., Bauer J., et al. Impairment of Bone Health in Pediatric Patients with Hemolytic Anemia. — 2014. — PLoS ONE 9 (10): e108400. doi: 10. 1371/journal. pone. 108 400.
8. Trikas A., Tentolouris K., Katsimaklis G., Antoniou J., Stefanadis C., Toutouzas P. Exercise capacity in patients with Bthal-assemia major: relation to left ventricular and atrial size and function. //Am Heart J. — 1998. — 136: 988−990.
9. World Bank 2006, report of a joint WHO-March of Dimes meeting. — 2006.
Gulammakhmudova Dilobar Valijanovna, Republican specialized scientific-practice medical center of obstetrics and gynecology, Tashkent, Uzbekistan, scientific explorer E-mail: dilobar. gulommahmudova@mail. ru
Reproductive health and hormonal status of women with polycystic ovary syndrome
Abstract: In this work there have studied reproductive health, also features of reproductive and hormonal disturbances at polycystosys ovarian syndrome (POS) among of women at the active reproductive age, which belongs to the uzbek population.
Keywords: ovarian polycystosys, reproductive health, hyperandrogenia.
Actuality. Nowadays problem of population’s reproductive health protection takes one of the first places in the laws and politics of modern progressive society and is one of the priority directions in the Republic of Uzbekistan. One of the most frequent causes of reproductive function
violations of women of fertile age is polycystic ovary syndrome (POS) [1- 5- 6- 7]. Therefore, this pathology is under close attention of doctors in various countries and in our Republic as well. Along with that clinical practice dictates the need to assist the patients suffering from POS and help them