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

Majid, Hafsa; Jafri, Lena; Ahmed, Sibtain; Talati, Jamsheer; Moiz, Bushra; Khan, Aysha Habib

doi:10.1016/j.amsu.2019.07.016

Cited by 3 publications

(2 citation statements)

References 16 publications

(16 reference statements)

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“…Hypoparathyroidism is reported in 3.6 to 22.5% of patients with BTM (30). If unrecognized or untreated, it may cause decreased bone density, fractures and bone pain, which are often detected in thalassemia, as well as severe hypocalcemia findings (31). In our study, hypoparathyroidism was found 1.6%, lower rate than the literature.…”

Section: Discussioncontrasting

confidence: 52%

Pediatrik Beta Talasemi Major Hastalarında Endokrin Komplikasyonlar: Tek Merkez Deneyimi

Yenigürbüz

Akıncı

ÜSTYOL³

et al. 2022

Harran Üniversitesi Tıp Fakültesi Dergisi

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Background: Our aim was to identify risk factors and clinical correlates of endocrine complications in β-thalassemia major (BTM) patients.Materials and methods: This was a retrospective study carried out in the pediatric hematology and pediatric endocrinology departments of a tertiary healthcare institution with the medical data of 249 children with BTM (108 females, 43.4%; 141 males, 56.6%) with a median age of 7.17 (2 -17.8) years. Baseline descriptive, clinical features including endocrine complications and laboratory data were noted. Correlation between the presence of endocrine complications and demographic, clinical, and laboratory variables were sought. The effects of age, gender, race, height, weight, and splenectomy on endocrine complications were evaluated separately in each complication group. Results: Vitamin D deficiency/insufficiency is the most common endocrine complication (41.7%). According to Turkish children, Syrian children had also significantly lower vitamin D concentration (p=0.001). At least one endocrinopathy was reported in the majority of BTM patients (67.9%). Accordingly, pubertal status (p=0.014) and Syrian nationality (p=0.007) had significant impacts on TSH levels. Syrian children and those with delayed puber-ty had greater likelihood for subclinical or evident hypothyroidism. The likelihood of suffering from at least one endocrine complication was higher in older children (p=0.042) and those with Syrian nationality (p=0.025)Conclusion: Disorders of endocrine and metabolic nature are common in children with BTM. Early detection and protocol-based multidisciplinary management of these disorders constitute the most suitable strategies to in-crease patients' quality of life. Surveillance, early detection and treatment, and collaborative follow-up with a multidisciplinary team are the key points in the reduction of the severity and frequency of endocrine complica-tions as well as optimization of therapeutic outcomes.

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Section: Discussioncontrasting

confidence: 52%

Pediatrik Beta Talasemi Major Hastalarında Endokrin Komplikasyonlar: Tek Merkez Deneyimi

Yenigürbüz

Akıncı

ÜSTYOL³

et al. 2022

Harran Üniversitesi Tıp Fakültesi Dergisi

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“…В исследовании, прицельно изучавшем нарушения кальций-фосфорного обмена у детей с БТМ, первичный гипопаратиреоз был выявлен у 3,4%, субклинический — у 52,3%, а вторичный — у 34% [ 15 ]. Считается, что причиной гипопаратиреоза может быть как перегрузка паращитовидных желез железом, так и подавление секреции паратгормона вследствие повышенной резорбции костной ткани при активном гемопоэзе на фоне хронической анемии [ 1 ].…”

Section: гипопаратиреозunclassified

Endocrine disorders in patients with transfusion-dependent hereditary anemias

Vitebskaya

Bugakova

Писарева

et al. 2022

Probl Endokrinol (Mosk)

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Often transfusions red blood cells in patients with hereditary anemias lead to iron overload, that can cause endocrine complications, such as growth retardation, hypothyroidism, hypogonadism, and disorders of carbohydrate metabolism.Clinical case 1. A boy with transfusion-dependent (TD) Diamond-Blackfan anemia at 16.3 years presented with impaired fasting glucose, growth hormone (GH) deficiency, hypogonadotropic hypogonadism; GH therapy was initiated. At the age of 16.8 years old secondary hypothyroidism, secondary hypocorticism and diabetes mellitus were diagnosed. At 17.2 years continuous glucose monitoring (CGM) detected glucose elevations up to 11.7 mmol/l. Therapy with GH and testosterone ethers was continued; levothyroxine and cortef were stopped by patient. At 17.9 years height was 163 cm; no data supporting hypothyroidism nor hypocorticism; glycaemia within goal range.Clinical case 2. A girl with TD beta-thalassemia major at the age of 11.5 years presented with GH deficiency; GH therapy has been conducted from 12.8 to 15.3 years of age. At 13.8 years retardation of pubertal development was diagnosed. At 15.0 hyperglycemia 7.2 mmol/l was detected; normal results of oral glucose tolerance test (OGTT) were observed; glycemia elevations were up to 9.5 mmol/l according to CGM data. At 16.0 height was 152 cm; because of pubertal development arrest hormone replacement therapy was prescribed.CONCLUSION. Growth, pubertal and carbohydrate metabolism disorders were diagnosed in patients with TD hereditary anemias, that confirms the necessity of regularly endocrine investigation. To detect impairment of carbohydrate metabolism investigation of fasting blood glucose, OGTT, and CGM is recommended; glycated hemoglobin measurement is not considered reasonable.

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