What We Learn from Bone Complications in Congenital Diseases? Thalassemia, an Example

Hamidi, Z

doi:10.5772/31270

Cited by 5 publications

(5 citation statements)

References 68 publications

(176 reference statements)

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“…Other studies showed also no significant difference in sex ratios between controls and HHD (29). In an important study, thalassemia affected males and females equally and occurs in approximately 44/100,000 live births (23,30). A non-statistically significant difference was observed between male (53.5%) and female (46.5%) thalassemia patients (31).…”

Section: Discussionmentioning

confidence: 99%

“…β-thalassemia is the most frequent disease, followed by intermediate thalassemia and thalassemia with the sickle, whereas other types of HHD have a lower prevalence in Najaf province. In other studies, sex differences in hemoglobinopathies have been detected, where β-thalassemia heterozygous (trait) was the most frequently encountered hemoglobinopathy (17.64%) and β-thalassemia homozygous (major and intermediate) was 2.92% (22).Thalassemia occurs in approximately 44 of every 100,000 live births worldwide(23).…”

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confidence: 94%

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Hereditary Hematologic Disorders in Najaf Province-Iraq

Al‐Hakeim¹,

Abdulla²,

Almulla³

et al. 2020

Preprint

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Background: Hereditary hematologic disorders (HHDs) originate from genetic factors that cause disruptions of blood homeostasis. The Thalassemia Unit, Najaf province, Iraq, keeps all medical records of all patients with HHD from the Najaf province. Objectives: We aimed to conduct an epidemiological study to obtain a comprehensive epidemiological picture with regard to sex distribution, and effects of blood groups and consanguinity. Patients and Methods: All registered HHD patients (until May 2019) in the Thalassemia Unit (1122 patients, aged 0.5-65 years old) were included in the study. Results: The prevalence of HHD in Najaf city is 75.6 per 100,000 population. The number of patients registered as new patients is increasing over the years. There are significantly more male than female HHD patients (Male=54.37%, Female=45.63%, p=0.023). The top 3 prevalent HHD are Major thalassemia (33.15%), intermediate thalassemia (31.91%), and Thalassemia+Sickle (12.65%). The highest percentage of patients were in the center of Najaf (56.42%) followed by Kufa, Mishkhab, Haidariah, Manathera, and Abbasiya. The distribution of blood groups and Rh factors of HHD patients in the Najaf population was not significantly different from that in the normal population. The consanguinity rate observed in our patients' parents (78.24%) was significantly higher (p<0.0001) as compared with the consanguinity rate in the population (45.8%). Conclusions: During the last few years, there was an increase in HHD cases in Najaf city and, therefore, there is an urgent need to increase awareness about the effects of consanguinity marriages on HHD in order to limit the incidence of HHD.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 94%

Hereditary Hematologic Disorders in Najaf Province-Iraq

Al‐Hakeim¹,

Abdulla²,

Almulla³

et al. 2020

Preprint

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“…10 It appears that vitamin D deficiency is another contributing factor to low bone mass associated with thalassemia; however, the prevalence of this problem as reported in different studies varies. 5 Gradual liver iron overloading (and deficiency in liver hydroxylation of vitamin D) and a defect in vitamin D absorption in older thalassemic patients, have been reported as disease mechanisms. 6 As suggested previously, thalassemic patients need frequent blood transfusions during their life.…”

Section: Introductionmentioning

confidence: 99%

“…11 These problems also are risk factors for low BMD. Even the intensive use of chelating agents used to remove the excess iron 12 may cause growth failure 5 and low BMD.…”

Section: Introductionmentioning

confidence: 99%

“…2 The common finding of low BMD has been reported, based on different bone densitometry methods, 3 some of which are not generally in good agreement. 4 Several factors are addressed in the reduction of bone mass in thalassemic patients, including endocrinopathies, growth delay, [5][6] and liver disease. 7 On a molecular basis, an increase in the ratio of sRANKL/OPG is reported in thalassemic patients, 8 indicating enhanced osteoclastic bone resorption and bone loss.…”

Section: Introductionmentioning

confidence: 99%

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Untitled

2015

Exp Clin Transplant

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Objectives: Beta thalassemia major is a genetic hemoglobin disorder that affects bone density. The disease leads to deteriorating bone structure but can be treated with hematopoietic stem cell transplant. We aimed to assess bone mineral density changes in pediatric beta thalassemia major patients who had undergone a hematopoietic stem cell transplant compared with similarly affected patients who had not undergone a hematopoietic stem cell transplant. Materials and Methods: Forty beta thalassemia major patients, 20 transplant and 20 nontransplant, younger than 16 years of age were enrolled. The mean age of transplant patients was 8.15 years and nontransplant patients was 9.5 years (P = .242). The female:male ratio was 1:1 in both groups. None of the patients reached puberty during this study. Bone mineral density was evaluated in transplant patients before and 1 year after hematopoietic stem cell transplant. Bone mineral density of nontransplant patients also was evaluated 1 year after their initial bone mineral density test. A Norland XR-46 densitometer was used to make all bone mineral density measurements. None of the patients had a z score < -2. Results: Mean bone mineral density changes in the femur and spine during this study were 0.008 ± 0.075 g/cm 2 and 0.048 ± 0.045 g/cm 2 in transplant patients and 0.045 ± 0.072 g/cm 2 and 0.036 ± 0.058 g/cm2 in nontransplant patients. No significant differences between bone mineral density changes in transplant and nontransplant patients were detected during the study. Conclusions: No significant effects on bone mineral density were detected in hematopoietic stem cell transplant pediatric beta thalassemia major patients compared with similarly affected nontransplant patients. Studies of longer duration may be required to identify significant changes in bone mineral density in hematopoietic stem cell transplant patients.

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