• Archives of Plastic Surgery
• /
• 제37권3호
• /
• pp.281-284
• /
• 2010

Purpose: Pseudohypoparathyroidism is a hereditary disorder characterized by symptoms and signs of hypoparathyroidism, typically in association with distinctive skeletal and developmental defects. Hypoparathyroidism is caused by a insufficient end-organ response to PTH (parathyroid hormone). Hypoparathyroidism consists of four types in which the most common form, pseudohypoparathyroidism-Ia, accompany with Albright's hereditary osteodystrophy. We experienced a case of a woman who had been suffering from calcified mass on left foot, diagnosed Albright's hereditary osteodystrophy. Methods: We present a case of a 24-year-old Korean female who visited plastic surgery department with a painful mass on dorsum of the left foot. On the physical exam, bony hard and painful mass, fixed to dermis, was noted. Plain X-ray films demonstrate suspicious calcification on subcutaneous tissue of dorsum of the left foot. The patient was diagnosed pseudohypoparathyroidism 2 years ago at the plastic surgery department. At the visiting time, the laboratory results were within normal range even though the patient actually had a disease. The reason is because the patient has been treated with Vit.D, calcium replacement therapy and thyroid hormone therapy. Moreover, the patient has been treated with anticonvulsant agents due to epilepsy. On the brain computer tomography (CT), calcification was noted on the basal ganglia and dentate nucleus. So we decided the total excision of entire mass from the left foot. Results: We excised main mass with numerous pinhead sized masses which were scattered around the main mass. The $6.0{\times}4.0{\times}0.5\;cm$ sized main mass was bony hard, and its surface was flat and margin was irregular. The permanent biopsy was confirmed that the main mass and all the scattered tiny masses were heterotopic calcification. The patient did not suffer from the pain after the mass excision. The wound has been healed without any problem. Conclusions: Heterotrophic calcification is often accompanied with pseudohypoparathyroidism, but such a huge one is uncommon. We report a case of pseudohypoparathyroidism with heterotrophic calcification developed in dorsum of left foot who was diagnosed by excisional biopsy.

• The Korean Journal of Physiology and Pharmacology
• /
• 제27권1호
• /
• pp.1-8
• /
• 2023

Hypothyroidism alone can lead to myocardial fibrosis and result in heart failure, but traditional hormone replacement therapy does not improve the fibrotic situation. Hydrogen sulfide (H₂S), a new gas signaling molecule, possesses anti-inflammatory, antioxidant, and anti-fibrotic capabilities. Whether H₂S could improve hypothyroidism-induced myocardial fibrosis are not yet studied. In our study, H₂S could decrease collagen deposition in the myocardial tissue of rats caused by hypothyroidism. Furthermore, in hypothyroidism-induced rats, we found that H₂S could enhance cystathionine-gamma-lyase (CSE), not cystathionine β-synthase (CBS), protein expressions. Finally, we noticed that H₂S could elevate autophagy levels and inhibit the transforming growth factor-β1 (TGF-β1) signal transduction pathway. In conclusion, our experiments not only suggest that H₂S could alleviate hypothyroidism-induced myocardial fibrosis by activating autophagy and suppressing TGF-β1/SMAD family member 2 (Smad 2) signal transduction pathway, but also show that it can be used as a complementary treatment to conventional hormone therapy.

• Clinical and Experimental Pediatrics
• /
• 제57권10호
• /
• pp.425-433
• /
• 2014

Adrenal and thyroid hormones are essential for the regulation of intrauterine homeostasis, and for the timely differentiation and maturation of fetal organs. These hormones play complex roles during fetal life, and are believed to underlie the cellular communication that coordinates maternal-fetal interactions. They serve to modulate the functional adaptation for extrauterine life during the perinatal period. The pathophysiology of systemic vasopressor-resistant hypotension is associated with low levels of circulating cortisol, a result of immaturity of hypothalamic-pituitary-adrenal axis in preterm infants under stress. Over the past few decades, studies in preterm infants have shown abnormal clinical findings that suggest adrenal or thyroid dysfunction, yet the criteria used to diagnose adrenal insufficiency in preterm infants continue to be arbitrary. In addition, although hypothyroidism is frequently observed in extremely low gestational age infants, the benefits of thyroid hormone replacement therapy remain controversial. Screening methods for congenital hypothyroidism or congenital adrenal hyperplasia in the preterm neonate are inconclusive. Thus, further understanding of fetal and perinatal adrenal and thyroid function will provide an insight into the management of adrenal and thyroid function in the preterm infant.

• Journal of Korean Neurosurgical Society
• /
• 제42권3호
• /
• pp.216-219
• /
• 2007

Lymphocytic hypophysitis is a clinically rare disease, and it has been known to be an autoimmune disease which mainly affects pregnant women at the end of gestation or right after delivery. The authors experienced a case of lymphocytic hypophysitis in a 29-year-old pregnant woman with rapid progressing visual disturbance. Sella MRI showed a mass-like lesion of hypophysis and hypertrophy of pituitary stalk with evidences of hypopituitarism. Cesarean section was done and then TSA was performed. The pathologic diagnosis was lymphocytic hypophysitis. After TSA, visual acuity was improved and visual field defect was recovered. She was given thyroid hormone replacement therapy because of transient partial hypopituitarism for 6 months after surgery. One must consider the probability of lymphocytic hypophysitis, if there are alteration of visual acuity and visual field defect which aggravate rapidly during pregnancy due to mass effect, decreased serum hormonal levels shown in hypopituitarism and sella MRI findings of hypertrophy of pituitary stalk and enlargement of pituitary gland.

• 동아시아식생활학회:학술대회논문집
• /
• 동아시아식생활학회 2010년도 추계학술대회
• /
• pp.47-47
• /
• 2010

Chilliness is a complaint among menopausal women in Japan, but increasing evidence indicates that young women also suffer from chilliness. However, neither diagnostic criteria nor drug therapies exist for treating chilliness. We noticed it and recently elucidated methodological issues related to identifying women with chills. Using a discriminant analysis, we demonstrated that four parameters were important determinants of chilliness. On the other hand, after repeated reports of serious adverse effects with hormone replacement therapy, much attention has been given to the development of new remedies to alleviate menopausal depressive state in women, but methods for their preclinical evaluation have not been clarified. We previously developed a procedure to predict the effect of candidate substance on the menopausal depressive-like state in female mice. Since either chilliness or mood disorder is known to disturb a quality of life in women, in this symposium, the efficacy of Korean red ginseng on chilliness in women and depressive-like state will be discussed.

• 고신대학교 의과대학 학술지
• /
• 제33권2호
• /
• pp.141-149
• /
• 2018

Hot flush (hot flush or facial flush) is the most frequent symptom experienced by women of peri-menopausal age. It may appear on women or even men after surgery or chemotherapy. Hot flush is one of the biggest reason for women to undergo hormone replacement therapy (HRT). It also crucially affects various aspects of life quality such as occupation, social life, daily routine and health awareness. The most effective and fundamental remedy for hot flush is HRT. However, a few women is not responsive to HRT and investigation to elucidate other causes of hot flush is warranted, especially in elderly. The contraindications such as breast cancer mandates other modality of treatment. Variety of treatment for hot flush other than HRT will be discussed herein.

• Clinical and Experimental Pediatrics
• /
• 제52권10호
• /
• pp.1061-1068
• /
• 2009

만성 신질환 환아들의 치료는 신대체 요법과 더불어 적절한 식이 요법, 수분, 전해질 및 산-염기의 관리, 신장 골형성 장애의 예방 및 치료, 빈혈과 고혈압, 성장 지연의 치료 등으로 이루어진다. 식이 요법은 환아들의 성장이 충분히 이루어질 수 있도록 탄수화물, 지방, 단백질 및 열량의 공급이 적절하게 이루어져야 한다. 수분, 전해질 및 산-염기의 상태를 확인하고 적절하게 관리해야 한다. 혈청 칼슘, 인, 부갑상선 호르몬 농도가 사구체 여과율에 따른 적절한 목표치 안에서 유지될 수 있도록 인 결합 제제와 비타민 D 제제를 투여하여 신장 골형성 장애를 예방하여야 한다. 적혈구 생산 촉진제와 철분 제제를 투여하여 혈색소 값을 11-12 g/dl로 유지하여야 한다. 수분 및 염분의 균형과 적절한 항고혈압제의 투여로 고혈압을 조절해야 한다. 사람 재조합 성장 호르몬을 투여하여 최종 성인 키를 호전시키도록 한다.

• Journal of Genetic Medicine
• /
• 제20권2호
• /
• pp.60-69
• /
• 2023

Purpose: Despite enzyme replacement therapy (ERT) and/or allogeneic hematopoietic stem cell transplantation, individuals with mucopolysaccharidosis (MPS) I or II often experience significant growth deficiencies. This study aimed to assess the safety and efficacy of recombinant human growth hormone (hGH) treatment in children diagnosed with MPS I or II. Materials and Methods: A total of nine pediatric patients-four with MPS I and five with MPS II-underwent treatment with ERT and hGH at Samsung Medical Center. Results: The mean hGH dose administered was 0.26±0.03 mg/kg/week. In the MPS I group, three patients showed an increase in height Z-score from -4.09±0.83 to -3.68±0.43 after 1 year of hGH treatment, and to -3.10±0.72 by the end of the hGH regimen. In the MPS II group, while the height Z-score of four patients decreased according to standard growth charts, it improved from 1.61±1.79 to 2.71±1.68 based on the disease-specific growth chart through hGH treatment. Two patients discontinued hGH treatment due to lack of efficacy after 22 and 6 months each of treatment, respectively. No new-onset neurological symptoms or necessity for prosthetic or orthopedic surgery were reported during hGH treatment. Conclusion: This study provides insights into the impact of hGH on MPS patients, demonstrating its potential to reverse growth deceleration in some cases. Further research is needed to explore the long-term effects of hGH on changes in body composition, muscle strength, and bone health in this population.

• Clinical and Experimental Pediatrics
• /
• 제60권2호
• /
• pp.31-37
• /
• 2017

Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers. Psychosocial issues frequently affect adherence to glucocorticoid treatment. Therefore, the safe transition of adolescents to adult care requires regular follow-up of patients by a multidisciplinary team including pediatric and adult endocrinologists. The major goals for management of adults with 21-hydroxylase deficiency are to minimize the long-term complications of glucocorticoid therapy, reduce hyperandrogenism, prevent adrenal or testicular adrenal rest tumors, maintain fertility, and improve quality of life. Optimized medical or surgical treatment strategies should be developed through coordinated care, both during transition periods and throughout patients' lifetimes. This review will summarize current knowledge on the management of adults with CAH, and suggested appropriate approaches to the transition from pediatric to adult care.

• Journal of Yeungnam Medical Science
• /
• 제33권2호
• /
• pp.125-129
• /
• 2016

Stress induced cardiomyopathy (SC) is characterized by transient left ventricular (LV) dysfunction in the absence of coronary artery disease. We report on a patient with panhypopituitarism who developed SC resulting from withdrawal of hormonal replacement therapy (HRT). A 52-year-old male visited our hospital for progressively worsening dyspnea. The patient had discontinued HRT 7 days ago, which had been administered for 18 months after transsphenoidal adenomectomy for pituitary macroadenoma. Initial electrocardiogram showed marked sinus bradycardia. Transthoracic echocardiography showed apical ballooning with an LV ejection fraction of 25%. No significant obstructive lesions were observed on coronary angiography. With a clinical diagnosis of SC associated with panhypopituitarism, HRT was restarted, including glucocorticoid and thyroxine, along with standard heart failure management. His LV function had normalized at 2-month follow-up. He remains asymptomatic and administration of beta-blocker and angiotensin converting enzyme inhibitor were discontinued He currently only requires HRT.