Anthropometric measurements in children having transfusion-dependent beta thalassemia

Moiz, Bushra; Habib, Aysha; Sawani, Sobiya; Raheem, Ahmed; Bilal, Hasan; Gangwani, Manesh Kumar

doi:10.1080/10245332.2017.1396044

Cited by 20 publications

(25 citation statements)

References 14 publications

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“…This comes in accordance with Moiz et al [38] who found significant negative correlation between height for age z-score and serum ferritin levels and Rathaur et al [39] who found (65.71%) of studied patients had short stature, 77% were underweight and rest had normal BMI and they found also statistically significant negative correlation between serum ferritin and short stature .…”

Section: Discussionsupporting

confidence: 91%

Magnetic Resonance Imaging Versus Serum Iron Status as Diagnostic Tools for Pituitary Iron Overload in Children with Beta Thalassemia

Morad

El-Shanshory

Hablas

et al. 2021

JAMMR

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Background: Thalassemia is a common genetic disorder associated with endocrine disorders. Iron deposition may start in the anterior pituitary gland, but clinical signs are usually not evident until puberty. Aim of the study was to evaluate pituitary iron overload in children with β thalassemia using MRI T2* and correlate MRI T2* and biochemical markers of iron overload with pituitary hormones. Patients and Methods: This study was carried out on 30 children with β-thalassemia major (19 females and 11 males ) with their age ranging from 10 - 18 years and mean age value of 12.8 ± 2.4 and 30 healthy children of matched age and sex as controls in the period from September 2018 to September 2019. For all patients the following were done: complete clinical evaluation including anthropometric data and Tanner staging, laboratory investigations including serum iron status, thyroid function, basal and growth hormone provocation test by clonidine, Follicle stimulating hormone (FSH) , Luteinizing hormone (LH) , and pituitary MRI T2*. Results: Weight, Z score of weight, height, Z score of height and Body mass index (BMI) were found significantly lower in patients compared with controls. Patients had delayed puberty compared with controls. Pituitary MRI T2* was found significantly lower in patients compared with controls (P=0.001). FSH, LH, and provocative growth hormone levels were found significantly lower in patients compared to controls (p <0.001). Serum ferritin & iron were found significantly higher in patients than controls (P<0.001). Significant negative correlations were found between serum ferritin and Weight, Height, Z score of Weight, Z score of Height, BMI and pituitary hormones. Significant negative correlations were found between Pituitary MRI T2* and serum ferritin. Significant positive correlations were found between Pituitary MRI T2* and Weight, Height, Z score of Weight, Z score of Height and pituitary hormons. The pituitary T2* carried the sensitivity of 80% ,100% and 100% and specificity of 70% ,83.3% and 100% for predicting GH hormone abnormality, LH and FSH respecetively. Conclusions: There were a significant positive correlations between pituitary MRI T2*and anthropometric measurement and pituitary hormones of studied patients. There was a significant negative correlation between Pituitary T2* and serum ferritin. Pituitary iron overload can be detected by MRI T2* which is a diagnostic tool in detecting pituitary iron overload with subsequent effect on hormonal secretion especially growth hormone. Recommendations: Children with thalassemia should undergo meticulous follow up regarding regular blood transfusion, regular iron chelators in a proper manner and doses and routine use of MRI T2* in the evaluation of pituitary iron overload before irreversible damage occur in the pituitary gland.

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Section: Discussionsupporting

confidence: 91%

Magnetic Resonance Imaging Versus Serum Iron Status as Diagnostic Tools for Pituitary Iron Overload in Children with Beta Thalassemia

Morad

El-Shanshory

Hablas

et al. 2021

JAMMR

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“…Study setting and patient recruitment has been described previously [6]. Briefly, 367 diagnosed cases of transfusion dependent β-TM between 5 and 17 years of age were recruited from the outpatient clinics of a non-profit organization providing free of cost blood components to the patients with various chronic blood disorder.…”

Section: Methodsmentioning

confidence: 99%

“…In a cross sectional survey of patients with transfusion dependent β-TM, our group reported iron over load and delayed physical growth [6]. In addition, in the same group of patients altered bone and mineral homeostasis (as indicated by hypocalcemia, hyperphosphatemia, vitamin D deficiency and altered parathyroid hormone activity) was identified with high frequency of pain and fractures in 12.5% of patients (submitted for publication).…”

Section: Introductionmentioning

confidence: 99%

“…The aim of this study was to determine the prevalence of different types of parathyroid dysfunction in transfusion dependent β-TM using biochemical markers and assess the difference between measured and maxPTH using nomogram by Harvey et al for its application in clinical practice. The anthropometric measurements and bone and mineral abnormalities have been described previously in these patients [6].…”

Section: Introductionmentioning

confidence: 99%

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Unique classification of parathyroid dysfunction in patients with transfusion dependent thalassemia major using Nomogram-A cross sectional study

Majid

Jafri

Ahmed

et al. 2019

Annals of Medicine and Surgery

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Introduction Hypoparathyroidism is a rare complication of iron overload in patients with transfusion dependent β thalassemia major (β-TM). We aim to determine the prevalence of parathyroid dysfunction in patients with β-TM. Methods Diagnosed cases of transfusion dependent β-TM between 5 and 17 years of age were recruited from outpatient clinics of a non-profit organization in Karachi, Pakistan. Blood and urine samples were collected in fasting to determine Ca, P, Alb, Mg, Cr 25OHD and iPTH. Patients were grouped on the basis of upper and lower levels of Ca, 25OHD and iPTH for assessing parathyroid dysfunction into primary hypoparathyroidism [ low calcium (Ca) & intact parathyroid hormone (iPTH) ], sub-clinical hypoparathyroidism [ low iPTH and 25 hydroxy vitamin D (25OHD), low/normal Ca], normal functioning parathyroid gland [ Normal Ca, iPTH and 25OHD ] and secondary hyperparathyroidism [ high iPTH, low/normal Ca and/or 25OHD ]. Using PTH nomogram subject specific expected PTH (maxPTH) was calculated. Difference between maxPTH and measured iPTH was determined to assess the utility of nomogram in identifying parathyroid gland dysfunction. The statistical analysis was performed using the Statistical Package of Social Sciences (SPSS) version 20. Results Median age of patients was 11 years (13–7) with males being 54.2% (n = 205). Based on Ca, 25OHD and iPTH, primary hypoparathyroidism was identified in 3.4% (n = 13) [median iPTH 11.3 pg/ml (12.6–7)], 52.3% (n = 192) had subclinical hypoparathyroidism [iPTH 40.4 pg/ml (52.7–28.7)], and 34% (n = 125) were identified as secondary hyperparathyroidism [iPTH 88.6 pg/ml (116–74.7)]. Normal response to Ca & 25OHD was seen in 10.6% (n = 39) [iPTH 44.2 pg/ml (53.8–33.4)] patients. High phosphorous was present in all groups. Difference between maxPTH & iPTH was highest in primary hypoparathyroidism, followed by subclinical and secondary hyperparathyroidism. Conclusion Nomogram by Harvey et al. identify low secretion capacity of parathyroid gland that correlated with biochemical classification of patients. It requires clinical validation before using in clinical practice for assessing parathyroid dysfunction.

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“…[5][6][7][8] Patients with ß thalassemia commonly have skeletal abnormalities including limbs,vertebral column,skull and bones of the face, commonly called squirrel -like face [9] . Theorofacial bone changes are the results of ineffective erythropoiesis, the bones become thinner with pathological fractures may occur due to overexpansion of the bone marrow and extra medullary region and this is frequently noticed in patients who receive insufficient or irregular blood transfusions [10,11] . Patient survival depends on regular blood transfusions and iron chelating therapy,In spite of being an effective cure but there are many serious associated long-term problems.…”

Section: Introductionmentioning

confidence: 99%

Effect of Blood Groups and Complications on Orofacial Measurements of B Thalassemia Adult Patients

Mohammed¹,

Abdullah²,

Al-Taee³

2021

MLU

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Background: β-thalassaemia is a common hereditary disorders in Iraq and worldwide .with different treatment schemes,, including blood transfusion, iron chelation and splenectomy, patients with B thalassemia may develop different skeletal changes. Limited studies link these complications with orofacial changes, This study aimed to evaluate the orofacial dimensions in β-thalassemia patients with age, gender, blood groups, splenectomy and cholecystectomy and to assist surgeons for planning their future intervention.Method: This study was conducted on 130 ß-thalassemia patients of both gender,,and two age categories (20-30 yrs), & (21-40 yrs), five orofacial measurements (face,eyes,nose,mouth and ears) and sixteen parameters were measured and calculated for differences,P value is estimated,data analysis were done by using Chi square test and SPSS version 20 Results: Significant findings were found for mandibular width and mouth width at older age group (31-40 years), the nasal width and ear height are more in male, patients with splenectomy have significance for lower 1\3 face and ear width. Cholecystectomy increase eye length measurments and ear height but for blood groups the P ˃ 0.05 Conclusions: Orofacial bone changes are pronounced in β-thalassaemia with distinct findings regarding sex dimorphism, older are more prone to have variations, earlier splenectomy is advisable,cholecystectomy is better choice for symptomatic patients, blood groups have no significance.

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Anthropometric measurements in children having transfusion-dependent beta thalassemia

Abstract: The study showed that children with beta thalassemia major had delayed physical growth possibly secondary to iron overload. Effective and early iron chelation is needed for preventing growth failure in transfusion-dependent beta thalassemia.

Cited by 20 publications

References 14 publications

Magnetic Resonance Imaging Versus Serum Iron Status as Diagnostic Tools for Pituitary Iron Overload in Children with Beta Thalassemia

Magnetic Resonance Imaging Versus Serum Iron Status as Diagnostic Tools for Pituitary Iron Overload in Children with Beta Thalassemia

Unique classification of parathyroid dysfunction in patients with transfusion dependent thalassemia major using Nomogram-A cross sectional study

Effect of Blood Groups and Complications on Orofacial Measurements of B Thalassemia Adult Patients

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