Correlation between criteria of diagnosis of low bone mineral density in adult and pediatric thalassemic patients: a prospective study.

BACKGROUND: Finding the correlation between criteria for low bone mineral density (BMD) in adulthood and childhood is a matter of interest. This study aimed to find how many of thalassemic patients with Z-Score ≤-2 during childhood, will be found with T-Score ≤-2.5 or Z-Score ≤-2.0 in adulthood. METHODS: The results of BMDs (one in childhood and one in adulthood) of 30 patients with beta thalassemia major (12 males and 18 females) with mean age of 17.63 y/o in childhood and 20.67 y/o in adulthood were studied, retrospectively. A Norland XR-46 device was used for measuring BMD. RESULTS: Z-Score ≤-2 was found in 33% of children and T-Score ≤-2.5 and Z-Score ≤-2.0 was found in 43 and 66% of adults, respectively. Correlation of pediatric Z-scores was good with adult T-scores and Z-scores (0.699-0.833). Relation of low BMD diagnosis in pediatric and adults was significant, and Kappa showed a moderate to good correlation between criteria for low BMD in childhood and adulthood, both with special significance in femoral region (8.10-14.47 and 0.258-0.703, respectively). Z-score ≤-2 significantly increased risk of low BMD in adulthood only when T-Score ≤-2.5 was used as low BMD criteria in adulthood (6.2-15.7). More patients dropped to poorer diagnosis group when Z-Score ≤-2.0 was used as low BMD criteria in adulthood. CONCLUSIONS: There is a moderate to good agreement of these criteria, at least in femoral region but using T-Score ≤-2.5 as low BMD criteria in adulthood caused a better prediction power for Z-Score ≤-2 of childhood.

Reliability of ultrasonography to measure cervical multifidus, semispinalis cervicis and longus colli muscles dimensions in patients with unilateral cervical disc herniation: An observational cross-sectional test-retest study.

BACKGROUND: Ultrasonography (US) has been suggested to assess the morphology and function of cervical muscles; but little is known about the reliability of the US measures in patients with cervical disc herniation (CDH). The purpose of this study was to evaluate within-day inter and intra-rater and between-day intra-rater reliability of US to measure dimensions of deep cervical muscles in patients with unilateral CDH. METHODS: Thirty patients with unilateral CDH participated. Anterior-posterior and lateral dimension of longus colli (LC), multifidus (MF) and semispinalis cervicis (SC) were measured using B-mode ultrasound. The measurements were repeated by rater A 1 h (for within-day reliability) and one week (for between-day reliability) later. For inter-rater reliability, rater B performed all muscles measurements like rater A. RESULTS: Within-day reliability measurement for all muscles was good to excellent with IntraClass Correlation Coefficients (ICC) ranging from 0.82 to 0.96, standard error of measurement (SEM) from 0.18 to 0.46 and minimal detectable changes (MDC) from 0.43 to 1.09. Between-day reliability was good for all muscle dimensions with ICC ranging from 0.75 to 0.89, SEM from 0.30 to 0.64 and MDC from 0.71 to 1.52. Inter-rater reliability was also good with ICC ranging from 0.75 to 0.89, SEM from 0.34 to 0.65 and MDC from 0.81 to 1.55. CONCLUSIONS: US was demonstrated to have high within-day inter and intra-rater and between-day intra-rater reliability to measure muscles dimensions in patients with unilateral CDH. It can be used to assess deep cervical muscles or to monitor the effects of interventions.

How Age, Sex and Transfusion Affects the Incidence of Endocrine and Bone Density Disorders in Major Thalassemic Patients.

Background: Beta-thalassemia major patients frequently have endocrinopathies. We tried to determine relation between demographic and transfusion factor and endocrinopathies. Methods: Major beta-thalassemia patients (n=114 cases), 3-38 yr of age, entered this study. Female to male ratio was 51/63. Children (less than 20 yr) formed 57% of participants. Information about bone mineral density (BMD) and hormonal and biochemistry blood evaluation including fasting blood sugar (FBS), ferritin, triiodothyronine (T3), thyroxine (T4) and thyroid-stimulating hormone (TSH), luteinizing hormone (LH) and follicle-stimulating hormone (FSH), testosterone (males), and estradiol (females) entered data sheet. Results: Sex and ferritin level showed no significant correlation with above disorders. Age significantly correlated to short stature, diabetes, low BMD at femur and neck (P, 0.031, 0.008, 0.009 and <0.001, respectively) . The risk of short stature had increased in 12 yr and older patients 7.71 times than younger patients (P= 0.008). The risk of diabetes had increased in 35 yr and older patients 26.25 times than younger patients (P= 0.03). The risk of Z-score ≤ -2 in femoral region has increased in 19 yr and older patients 5.84 times than younger patients (P= 0.002). The risk of Z-score ≤ -2 in spinal region has increased in 14 yr and older patients 17 times than younger patients (P= 0.007). Conclusion: The main factor related with endocrinopathies was age. The correlation between age and short stature, diabetes and low BMD was positive. Therefore, we recommend early monitoring of thalassemia patients (in their late childhood and early teenage) for these complications.

Relationship between endocrine changes and bone markers in pediatric thalassemic patients after hematopoietic stem cell transplantation.

BACKGROUND: Beta thalassemia major and its treatment by hematopoietic stem cell transplantation can have deleterious effects on bone integrity and a main part of such effects is due to their deleterious effects on endocrine systems. So, we assessed the effects of endocrine changes during HSCT (Hematopoietic Stem Cell Transplantation) on growing bones of pediatric thalassemic patients. METHODS: Bone-specific alkaline phosphatase and osteocalcin (bone formation markers), N-terminal telopeptide (NTX, bone resorption marker), calcium (Ca), phosphorus (P), alkaline phosphatase (Alk ph), parathyroid hormone (PTH), vitamin D (vit D), prolactin, luteinizing hormone (LH), follicle-stimulating hormone (FSH), thyroxine (T4), triiodothyronine (T3), thyroid-stimulating hormone (TSH), insulin-like growth factor 1 (IGF-1), testosterone (in males) or estradiol (in females), measured in 20 major thalassemic patients with mean age of 10.8±3.9 years. Parameters at the baseline (before HSCT), and 1 month and 3 months after HSCT. RESULTS: After stem cell transplantation, changes of mean serum levels of NTX, osteocalcin, prolactin, LH, T4, IGF-1, testosterone (in males), Ca, Alk ph, PTH, and vit D were not significant, but bone specific Alk ph, P, T3, TSH, FSH and estradiol changed significantly (P=0.013, P=0.001, P=0.48, P=0.02, P=0.04 and P=0.001, respectively). After one month, there was a significant positive relationship between osteocalcine and T3 (p= 0.009). After 3 months, also, there was a significant positive relationship between osteocalcine and T3 and T4 as well as a negative one with IGF-1 (P<0.001, P<0.02 and P<0.03, respectively). CONCLUSIONS: Endocrine disorders do not appear to have an overall positive or negative effect on bone metabolism (anabolism or catabolism) in HSCT pediatric thalassemic patients in short term (three months).

The role of using different reference population in the prevalence of low BMD in the thalassemia patients.

PURPOSE: Prevalence of osteoporosis reported as high as 50% in thalassemia major patients. We compared bone density (BMD) of our patients with results of bone densitometry of participants of a national study, to find if BMD diagnosis is ethnicity sensitive or not. METHODS: In 177 adult beta thalassemia major patients and 490 normal subjects of 20-39 y/o range, dual-energy X-ray absorptiometry of the spine and femur performed. Normal subjects participated in Iranian Multi-center Osteoporosis Study (IMOS). Mean and standard deviation of normal ones achieved and Z-score of patients re-calculated based on normal Iranians. As BMD of normal participants and patients performed by devices of different brands, analyses done based on calculated standard BMD of all participants. RESULTS: BMD of patients was significantly lower than normal participants (P value <0.001). Frequency of Z-score ≤-2 found in 52% and 56% of patients in femur and spine regions, respectively. New Z-score of patients according to Iranian normal populations calculated. Frequency of this new Z-score ≤-2 found in 16% and 72% of patients in femur and spine regions, respectively. CONCLUSIONS: We think in secondary osteoporosis, may be other cut-off points, especially according to BMD of normal population of that geographical region are needed.

Correction to: The role of using different reference population in the prevalence of low BMD in the thalassemia patients.

[This corrects the article DOI: 10.1007/s40200-019-00455-6.].

Short-term Assessment of HSCT Effects on the Hypothalamus-Pituitary Axis in Pediatric Thalassemic Patients.

BACKGROUND: Beta thalassemia major (BTM) and its treatment by hematopoietic stem cell transplantation (HSCT) may have deleterious effects on the endocrine systems. We assessed endocrine complications of HSCT in pediatric patients for 3 months. METHODS: In 20 (6 female) pediatric major thalassemic patients (mean age of 10.8 ± 3.9 years old), prolactin, luteinizing hormone (LH), follicle-stimulating hormone (FSH), T4, T3, thyroid-stimulating hormone (TSH), IGF-1, testosterone (in males) or estradiol (in females) were measured as a batch at the Endocrinology and Metabolism Research Center (EMRC) of Tehran University of Medical Sciences (TUMS) laboratories before HSCT and 1 and 3 months afterwards. The cosyntropin test for all and the clonidine test for short stature patients was conducted before HSCT. RESULTS: Before HSCT, delayed puberty and hypogonadotropic hypogonadism was found in 10% and 20% of patients, respectively. GH deficiency, low IGF1 and short stature was found in 25%, 55% and 40% of patients, respectively. Hypocortisolism, hypothyroidism and panhypopituitarism was found in 15%, 10% and 15% of patients, respectively. Prevalence of hypogonadotropic hypogonadism, low IGF1, hypothyroidism and panhypopituitarism was found in 20%, 40%, 10% and 10% of patients after 3 months, respectively (delayed puberty and short stature prevalence do not change after 3 months). HSCT caused lower T3 and estradiol and higher TSH. Corticosteroid users (15) had higher GH and lower T3 and testosterone or estradiol. Ferritin had a significant (negative) correlation with (before) prolactin and a significant correlation with T3 and T4 after HSCT. Age and acute graft-versus-host disease (GVHD) had no significant effect. CONCLUSION: Considering the small sample size and short duration of the study, it is difficult to reach any conclusion however it seems HSCT does not appear to have an overall positive or negative effect on prevalence of pituitary- hypothalamus axis disorders in pediatric thalassemic patients in 3 months.

Association of dietary pattern with biochemical blood profiles and bodyweight among adults with Type 2 diabetes mellitus in Tehran, Iran.

BACKGROUND: This study was conducted to identify dietary patterns and evaluated their association with biochemical blood profiles and body weight among adults with type 2 diabetes mellitus. METHODS: This was a cross sectional study conducted among 400 patients with type 2 diabetes mellitus in Tehran from March to August 2013. Biochemical blood profiles, socio-demographic, lifestyle, anthropometric measurements, and dietary data were obtained. Dietary data from food frequency questionnaire were used to derive dietary patterns. Factor analysis was conducted to ascertain the dietary patterns, and analysis of covariance was fitted to assess the relation between blood profiles, body weight and adherence to dietary patterns. RESULTS: Three dietary patterns by factor analysis were identified, Vegetable & Poultry, Western and Semi-healthy. After control for potential confounders, body mass index (b = -0/03, p < 0.05) were negatively associated with vegetable and poultry dietary pattern. Conversely, total cholesterol (b = 0.004, p < 0.01) and fasting blood glucose (b = 0.014, p < 0.05) were positively associated with western dietary pattern. A dietary pattern labeled as semi-healthy pattern was found to be positively related to HDL-cholesterol (b = 0.006 p < 0.01). Associations between semi-healthy pattern, LDL-cholesterol (b = -0.120 p < 0.05) and waist circumference (b = -0.020, p < 0.05) were negative. CONCLUSION: Adherence to Vegetable & Poultry dietary pattern was favorably related to body weight, semi healthy related to lower LDL and higher HDL cholesterol whereas western related to higher fasting blood glucose and total cholesterol. Further studies are necessary to confirm the benefits of the dietary patterns for diabetes.

Comparison of bone mineral density changes in pediatric thalassemic patients with and without hematopoietic stem cell transplant.

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/cm2 and 0.048 ± 0.045 g/cm2 in transplant patients and 0.045 ± 0.072 g/cm2 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.

Relation between BMD and biochemical, transfusion and endocrinological parameters in pediatric thalassemic patients.

UNLABELLED: Low BMDs, short stature, hypogonadism, subclinical hypothyroidism, and IFG are found in 3.3, 10, 33, 16.6, 6.6, and 26.6 % of 30 pediatric ß thalassemia major patients, respectively. Age is related with low Z-scores. Short stature and hypogonadism patients were older. These patients' monitoring in late childhood and early teenage for these complications is recommended. BACKGROUND: Beta-thalassemia major patients frequently have low BMD and increased fracture risk. We tried to determine the relation between BMD and biochemical, transfusion, and endocrinological parameters in pediatric patients. METHODS: Thirty beta-thalassemia major patients entered the study. Male to female ratio was 14/16. Physicians collected demographic; anthropometric; menstrual; transfusion and treatments histories; and serum levels of calcium, phosphorus, alkalin phosphatase, FBS, Hb, Ferritin, T3, T4, TSH, LH, FSH, testosterone (for boys), and estradiol (for girls). BMD of the spine and femur was measured using a DXA Norland XR-46 device. RESULTS: Prevalence of "low bone density" (Z-score < -2) in the spine and femur was 3.3 and 10 %, respectively. Short stature (Z-score < -2.5) and hypogonadism was seen in 33 and 16.6 % of patients, respectively. Hypogonadism was found only in boys. Subclinical hypothyroidism and impaired fasting glucose were found in 6.6 and 26.6 % of our patients, respectively. Age and transfusion duration was positively related with spinal BMD (P values, <0.001 and <0.001, respectively), and both related negatively with spinal Z-score (P values, 0.014 and 0.043, respectively). Age and transfusion duration related positively with femoral BMD (P values 0.030 and 0.017, respectively), and both related negatively with femoral Z-score (P values, 0.015 and 0.041, respectively). Mean age of short stature and hypogonadism patients were significantly higher than those who were normal (P values, 0.007 and 0.044, respectively). CONCLUSION: The main factor related with lower Z-scores of the femur and spine was age. Short stature and hypogonadism patients were significantly older. So, we recommend early monitoring of thalassemia patients (in their late childhood and early teenage) for these complications.