• Childhood Kidney Diseases
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• v.22 no.2
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• pp.64-66
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• 2018

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent hereditary renal disease and causes terminal chronic renal failure. ADPKD is characterized by bilateral multiple renal cysts, which are produced by mutations of the PKD1 and PKD2 genes. PKD1 is located on chromosome 16 and encodes a protein that is involved in cell cycle regulation and intracellular calcium transport in epithelial cells and is responsible for 85% of ADPKD cases. Although nine cases of unilateral ADPKD with contralateral kidney agenesis have been reported, there have been no reports of early childhood ADPKD. Here, we report the only case of unilateral ADPKD with contralateral kidney dysplasia in the world in a four year-old girl who was intrauterinely diagnosed since she was 20 weeks old and followed for four years until present.

• Korean Journal of Head & Neck Oncology
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• v.30 no.1
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• pp.35-38
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• 2014

Sclerosing polycystic adenosis(SPA) is a pathology of salivary gland which occurs infrequently. It has a controversial etiology. It is a recently described and extremely rare disease entity. Its reactive, sclerosing and inflammatory process is similar to fibrocystic changes and adenosis tumor of breast. Most cases has been reported to be occurred within major salivary glands. This disease is frequently confused with other salivary gland carcinomas, especially because of its histomorphological similarity. Here, we report a case of SPA within the submandibular gland in a 62-year-old woman.

• Journal of Genetic Medicine
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• v.17 no.1
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• pp.1-10
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• 2020

Polycystic ovarian syndrome (PCOS) is the most common endocrine disorder in women, which is characterized by the oligo/anovulation, hyperandrogenism (HA) and polycystic ovarian morphology which are diagnostic criteria. PCOS has diverse clinical aspects in addition to those diagnostic criteria including increased risk for cardiovascular diseases, metabolic syndrome, dyslipidemia, type 2 diabetes and impaired fertility. Because of the heterogeneity of the disease, the pathogenesis of the disease has not been elucidated yet. Therefore, there is no cure for the endocrinopathy. HA and insulin resistance (IR) has been considered two major pillars of the pathogenesis of PCOS. Recent advances in animal studies revealed the critical role of neuroendocrine abnormalities in developing PCOS. Several pathways related to neuroendocrine origin have been investigated such as hypothalamus pituitary ovarian axis, hypothalamus pituitary adrenal axis and hypothalamus pituitary adipose axis. This review summarizes the current knowledge about the role of HA and IR in developing PCOS. In addition, we review the results of recent genome wide association studies for PCOS. This new perspective improves our understanding of the role of neuroendocrine origins in PCOS and suggest a novel potential therapeutic target for the treatment of PCOS.

• The Journal of Korean Obstetrics and Gynecology
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• v.21 no.2
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• pp.284-291
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• 2008

Purpose: The purpose of this study is to report the effect of oriental treatments for PCOD(Polycystic Ovarian Disease) patient with amenorrhoea. Methods: A 24 years old women, who diagnosed PCOD(Polycystic Ovarian Disease), was enrolled in this study. She received oriental treatments such as herbal medicine, acupuncture for 5 months. And we proceeded to checkup female hormone regularly, such as Estrogen, Progesteron, LH, FSH, Prolactin, testosterone. We observed the menstruation period and figure out LH/FSH ratio. Results: 1. We treated PCOD patient with oriental herbal medicine(Changbudodamtang) and acupuncture. 2. PCOD patient restart menstruation regularly. 3. PCOD patient's LH/FSH ratio decreased. 4. PCOD patient's testosterone was within normal limit. Conclusion: 1. We treated PCOD patient with oriental herbal medicine(Changbudodamtang) and acupuncture, and then PCOD patient restart menstruation regularly. 2. We treated PCOD patient with oriental herbal medicine(Changbudodamtang) and acupuncture, and then we observed that LH/FSH ratio decreased.

• Biomolecules & Therapeutics
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• v.32 no.3
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• pp.291-300
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• 2024

Autosomal dominant polycystic kidney disease (ADPKD), a congenital genetic disorder, is a notable contributor to the prevalence of chronic kidney disease worldwide. Despite the absence of a complete cure, ongoing research aims for early diagnosis and treatment. Although agents such as tolvaptan and mTOR inhibitors have been utilized, their effectiveness in managing the disease during its initial phase has certain limitations. This review aimed to explore new targets for the early diagnosis and treatment of ADPKD, considering ongoing developments. We particularly focus on cell polarity, which is a key factor that influences the process and pace of cyst formation. In addition, we aimed to identify agents or treatments that can prevent or impede the progression of renal fibrosis, ultimately slowing its trajectory toward end-stage renal disease. Recent advances in slowing ADPKD progression have been examined, and potential therapeutic approaches targeting multiple pathways have been introduced. This comprehensive review discusses innovative strategies to address the challenges of ADPKD and provides valuable insights into potential avenues for its prevention and treatment.

• The Journal of Internal Korean Medicine
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• v.41 no.2
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• pp.132-140
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• 2020

Objectives: This research study aimed to determine the effect of Korean medicine treatments on a patient with lumbar disc herniation accompanying polycystic kidney disease. Methods: Acupuncture, herbal medicine, pharmacopuncture, spine decompression therapy, Motion Style Acupuncture Treatment (MSAT), and Chuna were preceded for treatment. We checked the patient's Oswestry Disability Index (ODI), numeric rating scale (NRS), and straight leg raise test (SLRT) on admission and discharge; we also used the NRS and SLRT to evaluate the patient's symptoms on every third day during the hospital stay. Because it is important to manage blood urea nitrogen (BUN), serum creatinine, and blood pressure during the early stage of polycystic kidney disease, BUN and serum creatinine levels were checked weekly while blood pressure was checked every morning. Results: Twelve days after admission, the NRS for lower back pain and right leg pain decreased from 7 to 3 and from 7 to 2, respectively. The ODI value also decreased from 56 to 20 while the SLRT value increased from 30/70 to 60/70. The BUN and serum creatinine levels and the blood pressure readings were all within normal range every time they were checked. Conclusions: The use of Korean medicine treatments resulted in improvements in NRS, ODI, and SLRT on a patient with a herniated lumbar disc herniated who had a past history of polycystic kidney disease; thus, the patient was able to maintaining kidney functioning. Herbal medicine, an alternative method of analgesic anti-inflammatory drugs that has been evaluated as relatively safe on liver and kidney function, could be suggested on a patient with a past history of polycystic kidney disease to maintain kidney function when renal function and blood pressure are monitored.

• Electrolytes & blood pressure
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• v.16 no.2
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• pp.23-26
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• 2018

A 22-year-old male patient was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). He received conservative treatment with an angiotensin-converting enzyme inhibitor. Two years later, oral therapy, consisting of 60 mg tolvaptan per day, was initiated. Compared with height-adjusted total kidney volume, the rate of kidney growth reduced significantly from 7.33% to 0.66% annually, since commencement of the tolvaptan therapy. The liver enzyme profile and serum sodium level and osmolality were constantly within normal ranges. In Korea, this is the first reported case of a patient with ADPKD who received tolvaptan treatment for more than 1 year. This case demonstrates that long-term tolvaptan treatment appears to be safe, well tolerated, and effective for ADPKD.

• Clinical and Experimental Reproductive Medicine
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• v.38 no.1
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• pp.42-46
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• 2011

Objective: This study was aimed to investigate endometrial histology and to find predictable clinical factors for endometrial disease (hyperplasia or cancer) in women with polycystic ovary syndrome (PCOS). Methods: We investigated the endometrial histology and analyzed the relationship between endometrial histology and clinical parameters, such as LH, FSH, estradiol, testosterone, fasting and 2 hours postprandial glucose and insulin, insulin resistance, body mass index, endometrial thickness, menstrual status from 117 women with PCOS. Statistical analysis was performed with chi square and t-test, p-value<0.05 was considered as statistically significant. And receiver operating characteristic curve was used to find predictable clinical factors for endometrial disease and to decide the cuff off values. Results: In 117 women with PCOS, endometrial histologic profiles are as follows: proliferative phase in 90 women (76.9%), endometrial hyperplasia in 25 women (21.4%), and endometrial cancer in 2 women (1.7%). Of 25 women with endometrial hyperplasia, simple hyperplasia without atypia, complex hyperplasia without atypia and complex hyperplasia with atypia were diagnosed in 15 (12.8%), 6 (5.1%), 4 (3.4%) women, respectively. Age and endometrial thickness were significantly related with endometrial disease, p=0.013 and p=0.001, respectively. At the cut off level of 25.5 years in age, sensitivity and specificity predicting for endometrial disease were 70.4% and 55.6%, respectively (p=0.023). At the cut off level of 8.5 mm in endometrial thickness, sensitivity and specificity were 77.8% and 56.7%, respectively (p=0.000). Conclusion: In women with PCOS, the incidence of endometrial hyperplasia and cancer were 21.4% and 1.7%. The age and endometrial thickness may be used as clinical determining factors for endometrial biopsy.

• Journal of Veterinary Clinics
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• v.31 no.5
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• pp.466-468
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• 2014

Polycystic kidney disease (PKD) is characterized by multiple cysts within the renal parenchyma and is a common heritable disease in humans, dogs, and cats. However, a few cases of PKD have been described in captive pygmy hippopotamuses. Bilateral PKD was observed in a 33-year-old, 198-kg female pygmy hippopotamus during its necropsy in Seoul Zoo on 15 January 2013. The diagnosis of PKD was confirmed by gross findings and histopathological examination. One kidney was slightly enlarged, and the lower portion of other kidney contained a large cyst filled with light yellow, watery fluid. Both kidneys had numerous, variably sized fluid-filled cysts of 2 to 20 mm in diameter. Considerable portions of the renal cortex and medulla were replaced by cysts. Microscopic inspection showed that the cysts were lined with low cuboidal to flat epithelial cells. The present case report of PKD in a pygmy hippopotamus is the first in Korea.

• Animal cells and systems
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• v.10 no.2
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• pp.65-71
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• 2006

Autosomal Dominant Polycystic Kidney Disease (ADPKD) is one of the most common inherited renal disorders in the world. Mutations in PKD1 located on chromosome 16p13.3 are responsible for 85% of all the ADPKD patients whereas mutations in PKD2 on chromosome 4q21-23 are responsible for the rest of the cases. Genetic heterogeneity and the problems of mutation detection in PKD1 suggest that linkage analysis is an important approach to study the genetics of ADPKD. To evaluate the availability of six (CA)n microsatellite markers for the linkage analysis of ADPKD in the Korean population, we examined the allele frequencies and heterozygosities of the markers. With the exception of KG8, five markers were highly informative, with PIC values over 0.5, but the PIC value of KG8 marker was less informative than other five markers because of the low number of alleles. Therefore, this study will be useful in linkage analysis for ADPKD families in the Korean population.