• Clinical and Experimental Reproductive Medicine
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• v.41 no.3
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• pp.108-114
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• 2014

Male factors account for 20%-50% of cases of infertility and in 25% of cases, the etiology of male infertility is unknown. Effective treatments are well-established for hypogonadotropic hypogonadism, male accessory gland infection, retrograde ejaculation, and positive antisperm antibody. However, the appropriate treatment for idiopathic male infertility is unclear. Empirical medical treatment (EMT) has been used in men with idiopathic infertility and can be divided into two categories based on the mode of action: hormonal treatment and antioxidant supplementation. Hormonal medications consist of gonadotropins, androgens, estrogen receptor blockers, and aromatase inhibitors. Antioxidants such as vitamins, zinc, and carnitines have also been widely used to reduce oxidative stress-induced spermatozoa damage. Although scientifically acceptable evidence of EMT is limited because of the lack of large, randomized, controlled studies, recent systematic reviews with meta-analyses have shown that the administration of gonadotropins, anti-estrogens, and oral antioxidants results in a significant increase in the live birth rate compared with control treatments. Therefore, all physicians who treat infertility should bear in mind that EMT can improve semen parameters and subsequent fertility potential through natural intercourse.

• Korean Journal of Clinical Laboratory Science
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• v.49 no.4
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• pp.495-497
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• 2017

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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.26 no.5
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• pp.284-289
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• 2023

A 14-year-old girl was admitted to the emergency department for excessive bile-containing vomiting and severe abdominal pain. She had been healthy until she intentionally lost 25 kg over a 6-month period. Thick, bloody bile-mixed food particles were drained from the stomach through a nasogastric tube. Abdominal computed tomography revealed huge stomach dilatation with extensive gastric pneumatosis, possible near rupture, acute pancreatitis, and a very narrow third of the duodenum, indicating superior mesenteric syndrome. Gastrofibroscopy revealed multiple hemorrhagic ulcers and numerous beadlike cystic lesions in the stomach. Laboratory examination results were notable for severe deficiencies in critical nutrients, including iron, zinc, proteins, and prealbumin, as well as undernutrition-associated endocrine complications such as hypothyroidism and hypogonadotropic hypogonadism. Excessive vomiting ceased after the endoscopic removal of stagnant gastric contents. Gastric pneumatosis improved after 3 days of supportive care.

• Development and Reproduction
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• v.20 no.1
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• pp.51-61
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• 2016

Neurokinin B (NKB) and neurokinin B related peptide (NKBRP) belong to tachykinin peptide family. They act as a neurotransmitter and/or neuromodulator. Mutation of NKB and/or its cognate receptor, NK3R resulted in hypogonadotropic hypogonadism in mammals, implying a strong involvement of NKB/NK3R system in controlling mammalian reproduction. Teleosts possess NKBRP as well as NKB, but their roles in fish reproduction need to be clarified. In this study, NKB and NKBRP coding gene (tac3) was cloned from Nile tilapia and sequenced. Based on the sequence, Nile tilapia NKB and NKBRP peptide were synthesized and their biological potencies were tested in vitro pituitary culture. The synthetic NKBRP showed direct inhibitory effect on the expression of GTH subunits at the pituitary level. This inhibitory effect was confirmed in vivo by means of intraperitoneal (ip) injection of synthetic NKB and NKBRP to mature female tilapia (20 pmol/g body weight [BW]). Both NKB and NKBRP had no effect on the plasma level of sex steroids, E2 and 11-KT. However, NKBRP caused declines of expression level of GnRH I, Kiss2 and tac3 mRNAs in the brain while NKB seemed to have no distinct effect. These results indicate some inhibitory roles of NKBRP in reproduction of mature female Nile tilapia, although their exact functions are not clear at the moment.

• Clinical and Experimental Reproductive Medicine
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• v.25 no.3
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• pp.293-297
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• 1998

Hypogonadotropic hypogonadism, or the lack of function of the testis or ovary secondary to the lack of pituitary and or hypothalamic trophic hormones, is also sometimes generally termed Kallmann's syndrome. Whether such deficiencies arise from an inborn error of hypothalamic organization and pituitary connection or damage to the hypothalamic pituitary system in prepubertal life, the manifestations of a eunuchoid or apubertal individual with potentially competent pituitary and gonadal function will result. Beyond the achievement of puberty, a similar situation can be recreated by the administration of a long-acting GnRH analog or by conditions of secondary hypothalamic dysfunction such as anorexia nervosa where shutdown of GnRH and its resultant effects cause cessation of gonadal function and even a regression of secondary sexual characteristics. Technically, these conditions are not Kallmann's syndrome but one must recognize the similarities. We have experienced a case of isolated gonadotropin deficiency which showed a negative KALIG-1 gene in infertile patient with primary amenorrhea. So we report this case with a brief review of literatures.

• Clinical and Experimental Reproductive Medicine
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• v.41 no.1
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• pp.29-32
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• 2014

Objective: To investigate the causes of amenorrhea in Korean women. Methods: Medical records from 1,212 women with amenorrhea who visited the Department of Obstetrics and Gynecology, Asan Medical Center, between January 1989 and December 2011 were retrospectively reviewed. Amenorrhea was categorized as either primary or secondary. Results: Primary amenorrhea was identified in 132 of the patients (10.9%) and secondary amenorrhea in 1,080 (89.1%). The most frequent causes of primary amenorrhea were gonadal dysgenesis (28.0%, 37/132); Mayer-Rokitansky-K$\ddot{u}$ster-Hauser syndrome (20.0%, 27/132); and constitutional delay and androgen insensitivity syndrome (8.3%, 11/132; 8.3%, 11/132, respectively). Secondary amenorrhea was due to polycystic ovary syndrome (48.4%, 523/1,080); premature ovarian insufficiency (14.0%, 151/1,080); and nutrition-related hypogonadotropic hypogonadism (8.3%, 90/1,080). Conclusion: In this retrospective study, gonadal dysgenesis was the most common cause of primary amenorrhea and polycystic ovary syndrome was the most common cause of secondary amenorrhea in Korean women.

• Clinical and Experimental Reproductive Medicine
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• v.37 no.1
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• pp.49-56
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• 2010

Objective: To investigate assisted reproductive technology (ART) outcomes in women with WHO class I anovulation compared with control group. Design: Retrospective case-control study. Methods: Twenty-three infertile women with hypogonadotropic hypogonadism (H-H) who undertook ART procedure from August 2003 to January 2009 were enrolled in this study. A total of 59 cycles (H-H group) were included; Intra-uterine insemination with super-ovulation (SO-IUI, 32 cycles), in vitro fertilization with fresh embryo transfer (IVF-ET, 18 cycles) and subsequent frozenthawed embryo transfer (FET, 9 cycles). Age and BMI matched 146 cycles of infertile women were collected as control group; 64 cycles of unexplained infertile women for SO-IUI and 54 cycles of IVF-ET and 28 cycles of FET with tubal factor. We compared ART and pregnancy outcomes such as clinical pregnancy rate (CPR), clinical abortion rate (CAR), and live birth rate (LBR) between the two groups. Results: There was no difference in the mean age ($32.7{\pm}3.3$ vs. $32.6{\pm}2.7$ yrs) and BMI ($21.0{\pm}3.1$ vs. $20.8{\pm}3.1kg/m^2$) between two groups. Mean levels of basal LH, FSH, and $E_2$ in H-H group were $0.62{\pm}0.35$ mIU/ml, $2.60{\pm}2.30$ mIU/ml and $10.1{\pm}8.2$ pg/ml, respectively. For ovarian stimulation, H-H group needed higher total amount of gonadotropin injected and longer duration for ovarian stimulation (p<0.001). In SO-IUI cycles, there was no significant difference of CPR, CAR, and LBR between the two groups. In IVF-ET treatment, H-H group presented higher mean $E_2$ level on hCG day ($3104.8{\pm}1020.2$ pg/ml vs. $1878.3{\pm}1197.7$ pg/ml, p<0.001) with lower CPR (16.7 vs. 37.0%, p=0.11) and LBR (5.6 vs. 33.3%, p=0.02) and higher CAR (66.7 vs. 10.0%, p=0.02) compared with the control group. However, subsequent FET cycles showed no significant difference of CPR, CAR, and LBR between the two groups. Conclusion: H-H patients need higher dosage of gonadotropin and longer duration for ovarian stimulation compared with the control groups. Significantly poor pregnancy outcomes in IVF-ET cycles of H-H group may be due to detrimental endometrial factors caused by higher $E_2$ level and the absence of previous hormonal exposure on endometrium.