Multiple Endocrinologic Complications in Thalassemia Major

( Siong Hu Wong ),( Julia Omar ),( Tuan Salwani Tuan Ismail ) 대한임상검사과학회 2017 대한임상검사과학회지(KJCLS) Vol.49 No.4

Thalassemia major is a genetic disorder with a defective synthesis of either the alpha or the beta chain of hemoglobin A. Blood transfusion is crucial for the survival in these patients. Unfortunately, endocrine dysfunction is a very common complication in these patients and is principally due to excessive iron overload as a result of frequent blood transfusions. Although regular blood transfusion may increase life expectancy, disturbances in growth and pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes, and disorderly bone growth are common side effects. We hereby present a case of a 23-year-old, unmarried woman with beta thalassemia major presenting with primary amenorrhea, poor development of secondary sexual character, and short stature. Thorough history, clinical examination, and laboratory investigation, including dynamic function test (insulin tolerance test) were conducted. These tests confirmed that she had multiple endocrinopathies, including hypogonadotropic hypogonadism, growth hormone deficiency, and subclinical adrenal insufficiency, which were caused by iron overload. She required hormone replacement therapy. Early recognition of possible deficiencies in hypothalamo-pituitary-end organ hormones caused by iron overload in thalassemia patients that undergo frequent blood transfusion procedures is essential. Appropriate treatments, including transfusion regimen and chelation therapy, as well as specific treatment of each complication are the crucial for the successful management and improvement of quality of life these patients.

Multiple Endocrinologic Complications in Thalassemia Major

Wong, Siong Hu,Omar, Julia,Ismail, Tuan Salwani Tuan Korean Society for Clinical Laboratory Science 2017 대한임상검사과학회지(KJCLS) Vol.49 No.4

Thalassemia major is a genetic disorder with a defective synthesis of either the alpha or the beta chain of hemoglobin A. Blood transfusion is crucial for the survival in these patients. Unfortunately, endocrine dysfunction is a very common complication in these patients and is principally due to excessive iron overload as a result of frequent blood transfusions. Although regular blood transfusion may increase life expectancy, disturbances in growth and pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes, and disorderly bone growth are common side effects. We hereby present a case of a 23-year-old, unmarried woman with beta thalassemia major presenting with primary amenorrhea, poor development of secondary sexual character, and short stature. Thorough history, clinical examination, and laboratory investigation, including dynamic function test (insulin tolerance test) were conducted. These tests confirmed that she had multiple endocrinopathies, including hypogonadotropic hypogonadism, growth hormone deficiency, and subclinical adrenal insufficiency, which were caused by iron overload. She required hormone replacement therapy. Early recognition of possible deficiencies in hypothalamo-pituitary-end organ hormones caused by iron overload in thalassemia patients that undergo frequent blood transfusion procedures is essential. Appropriate treatments, including transfusion regimen and chelation therapy, as well as specific treatment of each complication are the crucial for the successful management and improvement of quality of life these patients.

Gonadotropin-releasing hormone stimulation test and diagnostic cutoff in precocious puberty: a mini review

Siti Nadirah Ab Rahim,Julia Omar,Tuan Salwani Tuan Ismail 대한소아내분비학회 2020 Annals of Pediatirc Endocrinology & Metabolism Vol.25 No.3

The gonadotropin-releasing hormone (GnRH) stimulation test is a valuable tool in diagnosing and differentiating causes of early pubertal occurrences. Utility of the test can be limited in some instances, however, including the early phases of pubertal hypothalamic-pituitary-gonadal axis activation, in girls showing commonly overlapping pictures, and in obese children due to excess circulating estrogen that suppresses luteinizing hormone (LH). A lack of consistent baseline and stimulated gonadotropin cutoffs observed in different studies also contributes to limitations in testing. Nevertheless, early detection of true pathological causes for pubertal disorders is needed to allow prompt treatment and better prognosis. While basal LH can be beneficial as a good screening tool for detecting pubertal disorder, it does not preclude the need for GnRH testing. The aim of this review was to highlight the role of GnRH stimulation tests and varying testing cutoffs in diagnosis of precocious puberty and its classification.

Consumption of Nopal Powder in Adult Women

Mayra Elisa Sanchez-Murillo,Edwyn Omar Cruz-Lopez,Marıa Julia Verde-Star,Catalina Rivas-Morales,Marıa Eufemia Morales-Rubio,Aurora de Jesus Garza-Juarez,Jorge Martın Llaca-Dıaz,Ma de Jesus Ibarra-Sala 한국식품영양과학회 2020 Journal of medicinal food Vol.23 No.9

Osteoporosis is a chronic disease in adult women caused by menopause and some other factors, which entails deficiency of calcium in diet. Natural products are the best source of nutriments to reduce the risk of chronic diseases. Nopal (Opuntia ficus-indica) is a plant characterized by its nutritional components and benefits to health. Its calcium content increases with maturation process that could be beneficial for consumers. Nopal powder (NP) was elaborated from nopal harvested within 16–24 weeks of maturation, and the nutritional content was determined. An experimental clinical trial was performed to evaluate the effect of NP. A total of 69 women between 40 and 60 years old participated in the study. During 24 weeks, experimental group (n = 56) consumed a daily dose of 5 g of NP and control group (n = 13) continue with habitual diet. Changes in bone mineral density (BMD), body mass index (BMI), body fat percentage and serum calcium were assessed. Between baseline and after 24 weeks of consumption, no significant changes were found in BMD P = .885 experimental group and P = .970 control group, BMI P = .865 experimental group and P = .984 control group, body fat P = .744 experimental group and P = .740 control group and serum calcium P = .282 experimental group and P = .959 control group. These results indicate that advanced maturation NP does not have influence in bone health, BMI, and body composition in adult women.

A randomized, double-blind study comparing multiple doses of Channa striatus supplementation for knee osteoarthritis

Abdul Kadir Azidah,Abdul Kadir Arifah,A. H. Roslida,Abdul Manan Mat Jais,Julia Omar,Abdul Nawfar Sadagatullah,Azlina Ishak,Norhayati Mohd. Noor,Ahmad Tarmizi Musa 경희대학교 융합한의과학연구소 2017 Oriental Pharmacy and Experimental Medicine Vol.17 No.4

Knee osteoarthritis (OA) is the leading cause of chronic disability at older age. Channa striatus (CS) is a freshwater fish that is traditionally valued for its medicinal properties in promoting wound healing and reducing post-operative pain. This study evaluate the efficacy of different doses of oral Channa striatus extract on primary knee osteoarthritis patients. A randomized, double-blind, placebo-controlled 3-arm trial was conducted comparing oral CS extract 1000 mg/day or 500 mg/day and placebo among knee OA patients for a 6-month intervention period. The main outcome measures were Western Ontario and McMaster University Osteoarthritis Index (WOMAC), analgesic scores and serum cartilage oligomeric matric protein (COMP). Laboratory-based blood tests were used as safety measures. A total of 120 patients were randomized, and 112 patients were included in the intention-to-treat analysis. Significant reductions in WOMAC stiffness and function scores were achieved at month 6 in CS 1000 mg/day and CS 500 mg/day compared to placebo groups (p < 0.05). No significant differences were found between the groups in terms of analgesic scores, serum COMP and biochemical parameters. No serious adverse events were reported in the study. In conclusion, both doses of CS showed similar efficacy and were more effective than the placebo in treating the symptoms of knee OA.