Objective: To determine the prevalence of growth abnormality and endocrine dysfunction in a group of multi transfused thalassemic children and to correlate these with their body iron stores.
Methods: This cross sectional study included 35 Thalassemia Major patients, aged 13 to 24 years. Growth and puberty were assessed clinically and the laboratory values of various hormone levels were stratified with their age and sexual maturity.
Results: 57.14% patients were short, 60% had not attained puberty, and 87.5% of the girls had primary amenorrhea. 14.29% had low FSH and 2.86% low LH levels. 89.47% of the boys had low free testosterone and 43.75% of the girls had low estradiol levels. While 20% had high TSH levels, 40% had high PTH levels, of which 92.8% had low levels of Vitamin D. Low levels of IGF-1 were noted in 51.43%.
Conclusions: In this study analysis, short stature and hypogonadism were frequent findings. These results support the need for vigilant clinical evaluation of growth and puberty, as well as appropriate hormonal evaluation in poly transfused thalassemic children in order to detect and treat endocrine dysfunction early. The authors also recommend aggressive and adequate chelation from early life so that permanent damage to the endocrine glands can be prevented.
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http://dx.doi.org/10.1007/s12098-010-0351-3 | DOI Listing |
BMC Genomics
December 2024
College of Animal Science and Technology, Gansu Agricultural University, Lanzhou, 730030, China.
Background: The Hezuo (HZ) pig, a famous indigenous breed in China, is characterized by precocious puberty compared with foreign-introduced pig breeds. Sexual maturation is a complex physiological process, and in recent years, circular RNAs (circRNAs), a new class of noncoding RNAs with endogenous regulatory functions, have been shown to play important roles in regulating sexual maturation. However, the dynamic expression and regulatory mechanism of circRNAs during sexual maturation in HZ pigs remain unclear.
View Article and Find Full Text PDFBMC Pediatr
December 2024
Department of Endocrinology and Metabolism, Shanghai Children's Medical Center, School of Medicine, Shanghai Jiao Tong University, 1678 Dongfang Road, Shanghai, 200127, China.
The ARCN1 gene encodes the delta subunit of the coatomer protein complex I (COPI), which is essential for mediating protein transport from the Golgi complex to the endoplasmic reticulum. Variants in ARCN1 are associated with clinical features such as microcephaly, microretrognathia, intrauterine growth restriction, short rhizomelic stature, and developmental delays. We present a case of a patient exhibiting intrauterine growth restriction, preterm birth, microcephaly, micrognathia, and central precocious puberty.
View Article and Find Full Text PDFTomography
November 2024
Department of Orthopaedics and Traumatology, The University of Hong Kong, Hong Kong 999077.
Assessment of skeletal maturity is a common clinical practice to investigate adolescent growth and endocrine disorders. The distal radius and ulna (DRU) maturity classification is a practical and easy-to-use scheme that was designed for adolescent idiopathic scoliosis clinical management and presents high sensitivity in predicting the growth peak and cessation among adolescents. However, time-consuming and error-prone manual assessment limits DRU in clinical application.
View Article and Find Full Text PDFZhongguo Dang Dai Er Ke Za Zhi
December 2024
Department of Endocrine Genetics and Metabolism, National Children's Regional Medical Center/Xi'an Children's Hospital, Xi'an 710003, China.
Objectives: To establish an efficient and clinically applicable predictive model and scoring system for central precocious puberty (CPP) in girls, and to develop a diagnostic prediction application.
Methods: A total of 342 girls aged 4 to 9 years with precocious puberty were included, comprising 216 cases of CPP and 126 cases of isolated premature thelarche. Lasso regression was used to screen for predictive factors, and logistic regression was employed to establish the predictive model.
Aesthetic Plast Surg
December 2024
Private Practice, Kültür Mah., Plevne Bulvarı, Ocakoğlu Apt, No: 3, D: 6, 35220, Alsancak, İzmir, Turkey.
Background: Human ear growth continues beyond puberty, resulting in ears that are typically approximately 10 millimeters (mm) larger in older individuals compared to their younger counterparts. Not only is the overall growth of the ear an indicator of age, but also the disproportionate growth of its parts. The largest part, the concha, shows the slowest growth rate, while the smallest part, the earlobe, shows the greatest growth rate.
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