• Title/Summary/Keyword: FD

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McCune-Albright syndrome with acromegaly: A case report with characteristic radiographic features of fibrous dysplasia

  • Han-Gyeol, Yeom;Byung-Do, Lee
    • Imaging Science in Dentistry
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    • v.52 no.4
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    • pp.421-427
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    • 2022
  • McCune-Albright syndrome (MAS) is a rare multisystem disorder characterized by a clinical triad of polyostotic fibrous dysplasia (FD), skin pigmentation, and hyperfunctioning endocrinopathies. A 42-year-old man visited our medical hospital for the treatment of intermittent headaches and was diagnosed with MAS with acromegaly. This patient showed various clinical features of MAS, including pituitary adenoma, polyostotic FD, and hypogonadotropic hypogonadism. The FD lesions showed characteristic radiographic features, such as widespread, sclerotic bony lesions in the cranial bones, mixed radiolucent-radiopaque multilocular lesions in the mandible, and radiolucent lesions in the axial and appendicular skeleton. Over the years, the patient had been hospitalized multiple times due to accidental bony fractures associated with the fragile bony state of FD. This report presents a retrospective description of a case of MAS, with a review of the relevant literature.

Conservative Surgical Management of Fibrous Dysplasia with Osteomyelitis

  • Sung Bin Youn;Jeong Joon Han;Hoon Myoung
    • Journal of Korean Dental Science
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    • v.16 no.2
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    • pp.211-217
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    • 2023
  • Fibrous dysplasia (FD) accompanying osteomyelitis (OM) has been reported to result in recurrent, refractory pain and swelling. Although radical resection of the lesions has been suggested, effective surgical treatment strategies have not yet been established due to the limited number of studies on this pathological condition. In this report, we present the conservative surgical management of FD accompanying OM in two patients who exhibited recurrent signs and symptoms. The present report suggests that OM occurring in patients with FD can be successfully managed with conservative surgical treatment and following removal of the odontogenic origin although bone defect in which inflammatory fibro-osseous lesions is removed through decompression surgery may exhibit regeneration of dysplastic bone. In addition, for the prevention of OM in patients with FD, careful clinical examination and thorough management of dental-related pathologies are necessary with regular follow-up examinations to screen for the possibility of malignant changes.

Study of Ryodoraku Parameters for Diagnosing Gastric Dysmotility in Functional Dyspepsia (기능성 소화불량증에서 위운동성 장애 진단을 위한 양도락 지표 연구)

  • Kim, So-Yeon;Yoon, Sang-Hyup;Kim, Yoon-Bum;Jung, Sung-Ki
    • The Journal of Internal Korean Medicine
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    • v.29 no.2
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    • pp.401-412
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    • 2008
  • Objectives : The aim of this study was to investigate the characteristics of Ryodoraku and association of Ryodoraku with gastric dysmotility in functional dyspepsia(FD). Methods : Subjects were 154 patients with FD and 18 patients with asthma. We calculated the average Ryodoraku score(RS, ${\mu}A$) and each variation from physiologic range of 12 Ryodoraku points, and investigated the incidence when left and right points were simultaneously below(bilateral deficiency) or above(bilateral excess) physiologic range. Postprandial regularity of normal slow waves, power ratio, and postprandial % of bowel sound were obtained by electrogastrography and enterotachography, and were used as gastric dysmotility index. Results : 1. Bilateral excess of H4, H5, F1, and F4 and bilateral deficiency of H4, H5, and H6 were characteristic in FD compared with asthma patients. 2. Incidence of gastric dysmotility in bilateral deficiency of H4, H5, and H6 was 100%, and was higher than in total FD patients(88.3%). 3. There was a positive correlation between the variation of H6 and % postprandial bowel sound. 4. Deficient tendency of H4, H5, and H6 was more evident when RS was above $40{\mu}A$ in FD. Conclusions : These findings suggest that gastric dysmotility in FD can be diagnosed when a pattern of H4, H5, H6 bilateral deficiency and F1, F4 bilateral excess is shown at the same time. We think this phenomenon is related to low activity of the vagus nerve rather than meridian pathway with result based on positive correlation between variation of H6 and postprandial % of bowel sound.

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Trends of Tongue Features in Functional Dyspepsia Patients (기능성 소화불량 환자에서 설 지표의 경향성 파악)

  • Kim, Jihye;Ko, Seok-jae;Park, Jae-woo;Kim, Keun Ho
    • The Journal of Internal Korean Medicine
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    • v.39 no.4
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    • pp.637-644
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    • 2018
  • Objectives: In this study, the tongue features of patients with functional dyspepsia (FD) were compared with those of healthy controls. Methods: This prospective, case-control study was conducted on patients with FD and controls recruited at a single center. After screening, the subjects were allocated to the patient or control groups (patients=42, controls=40). Tongue images were acquired using a computerized tongue image acquisition system (CTIS). An independent t-test was conducted to compare the measurements from patients and controls. Binary logistic regression was performed to determine significant differences between the two groups after adjusting for age and sex. Results: The CIE $a^*$ color value in the tongue coating area was significantly lower in the patients with FD than in the controls (p=0.001). The tongue coating ratios were also significantly higher in the FD group than in the control group (p=0.003). We found that the CIE $a^*$ color value in the tongue coating area and the tongue coating ratios were significant predictive factors in both groups, based on binary regression analysis (p=0.016, 0.044, respectively). Conclusions: This study found that FD was significantly associated with CIE $a^*$ color value in the tongue coating area and tongue coating ratios. We suggest that these factors could be used as objective indicators of FD.

The Effects of 5-benzylacyclouridine on the Cytotoxicities of Fluorinated Pyrimidine Antimetabolic Agents in L5178Y Cells (L5187Y 세포에 대한 불화피리미딘 대사억제제 독성에 관한 Benzylacyclouridine의 영향)

  • Lee, Kang-Hyun;Cha, Sung-Man
    • The Korean Journal of Pharmacology
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    • v.26 no.1
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    • pp.91-100
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    • 1990
  • The benzylacycoluridines (BAU and BBAU) are potent and specific inhibitors of uridine phosphorylase (UrdPase). In contrast to the report that benzylacyclouridines potentiated 5-fluoro-2'-deoxyuridine (FdUrd) cytotoxicity against human solid tumor cells (Cancer Res., 44:1852, 1984), continuous exposure of mouse lymphoma L5178Y cells, to FdURd, 5-fluorouridine (FUrd), 5'-deoxy-5-fluorouridine (5'-dFUrd), or 5-fluorouracil (FUra) showed no potentiation of cytotoxicity by benzylacyclouridines. In fact, under the conditions employed, benzylacycoluridines protected the cells from the cytotoxicity of FdUrd, FUrd, or 5'-dFUrd, but not FUra in a dose dependent manner. Intraperitoneal coadministration of BAU or BBAU and a 5-fluorinated pyrimidine (i.e., FdUrd, FUrd, or FUra), to mice bearing L5178Y cells also did not significantly increase the life span compared to those treated with the antimetabolites alone. Anabolism of these nucleosides through the sequential action of UrdPase and orotate phosphoribosyltransferase (OPRTase), inhibition of nucleoside transport by benzylacyclouridines, or both could be responsible for the ineffectiveness of UrdPase inhibitors to potentiate the antineoplastic activity of fluoropvrimidines in L5178Y cells.

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Skin Color Measurement of LU10; Comparison between Functional Dyspepsia Patients and Healthy Controls (기능성 소화불량증 환자와 건강인의 어제혈 색택 비교 연구)

  • Kim, Min-ji;Ko, Seok-Jae;Park, Jae-Woo
    • The Journal of Korean Medicine
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    • v.37 no.3
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    • pp.27-38
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    • 2016
  • Objectives: According to Korean Medicine theory, the skin color of LU10 serves as a diagnostic clue to dyspeptic symptoms. The aims of this study were (1) to find the difference of skin color in LU10 region between functional dyspepsia (FD) and healthy control (HC) and (2) to examine the relationship between LU10 skin color parameters and dyspeptic symptoms. Methods: 39 participants (29 FD and 10 HC) have participated in this study. They were asked to complete gastrointestinal scale (GIS), gastrointestinal symptom rating scale (GSRS), Nepean dyspepsia index (NDI), functional dyspepsia-related quality of life (FD-QoL), visual analogue scale (VAS) for dyspeptic symptoms, food retention questionnaire (FRQ) and cold heat questionnaire (CHQ). $L^*$ (luminance), $a^*$ (red-green balance) and $b^*$ (yellow-blue balance) values of LU10 region were calculated through digital images of the participant's hand. Then we evaluated test-retest reliability of $L^*$, $a^*$ and $b^*$ values of LU10 region. Additionally, we compared $L^*$, $a^*$ and $b^*$ values of LU10 between FD and HC, and examined the relationship between LU10 color parameters and seven questionnaires scores. Results: Only $L^*$ values in LU10 region were significantly higher in FD compared with HC. GIS scores and the subset scores of NDI had a positive correlation with $L^*$ values significantly. Correlation coefficients of test-retest reliability of skin color measurement of LU10 ranged from 0.871 to 0.936 representing very strongly statistically significant (P<0.001). Conclusions: We confirmed the difference of skin color in LU10 region between FD and HC, and relationship between LU10 skin color parameters and dyspeptic symptoms.

Anatomical variations of trabecular bone structure in intraoral radiographs using fractal and particles count analyses

  • Amer, Maha Eshak;Heo, Min-Suk;Brooks, Sharon L.;Benavides, Erika
    • Imaging Science in Dentistry
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    • v.42 no.1
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    • pp.5-12
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    • 2012
  • Purpose : This study was performed to evaluate possible variations in maxillary and mandibular bone texture of normal population using the fractal analysis, particles count, and area fraction in intraoral radiographs. Materials and Methods : Periapical radiographs of patients who had full mouth intraoral radiographs were collected. Regions of interest ($100{\times}100$ pixels) were located between the teeth of the maxillary anterior, premolar, and molar area, as well as the mandibular anterior, premolar, and molar areas. The fractal dimension (FD) was calculated by using the box counting method. The particle count (PC) and area fraction (AF) analyses were also performed. Results : There was no significant difference in the FD values among the different groups of age, gender, upper, and lower jaws. The mean FD value was $1.49{\pm}0.01$. The mean PC ranged from 44 to 54, and the mean AF ranged from 10.92 to 11.85. The values of FD, PC, and AF were significantly correlated with each other except for the upper molar area. Conclusion : According to the results, patients with normal trabecular pattern showed a FD of approximately 1.5. Based on these results, further investigation would be recommended if the FD value of patient significantly differenct from this number, since the alteration of this value indicates microstructural modification of trabecular pattern of the jaws. Additionally, with periapical radiographs, simple and cost-effective, PC and AF could be used to assess the deviation from the normal.

Functional Evaluation of Lotus Root on Serum Lipid Profile and Health Improvement (고지방 식이에 있어 체내 지질농도 및 건강 개선에 미치는 연근 추출물의 기능성 평가)

  • Park Sung-Hye;Hyun Joong-Soon;Shin Eon-Hwan;Han Jong-Hyun
    • Journal of the East Asian Society of Dietary Life
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    • v.15 no.3
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    • pp.257-263
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    • 2005
  • In this study, lotus root(Nelumbo nucifera G.) which has been used in oriental medicine and folks remedy, was studied to apply to functional foods. We investigated the effects of the lotus root extracts(LTE) with hot water on the reduction of serum lipid and improvement of blood parameters in rats fed high fat diet for 5 weeks. Sprague-Dawley rats weigh $150g\pm15g$, were randomly assigned to 4 groups such as basal diet only(BDG), high fat diet without LTE(FDCG), high fat diet with $6\%$ LTE(FD6L), high fat diet with $12\%$ LTE(FD12L). The results of this study were as follows. Hematological data were not significantly different among 4 groups. Serum transferrin concentration and GOT activity were reasonable levels in FD6L and FD12L groups compared with FDCG group. Total cholesterol, LDL-cholesterol, triglyceride in serum and atherogenic index were remarkably reduced in LTE supplemented groups as compared with the control group. But HDL-cholesterol contents in FD6L and FD12L groups were significantly increased compared with that in control group. These results imply that lotus root extracts could be used as a potent food resources for decrease of serum lipid concentration.

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Correlation Analysis between Gastric Emptying Measured by Ultrasonography and Spleen Qi Deficiency Pattern in Patients with Functional Dyspepsia (기능성 소화불량 환자에서 초음파로 측정한 위 배출능과 비기허증(脾氣虛證)간의 상관성 분석)

  • Baek, Seung-hwan;Kim, Jin-sung
    • The Journal of Internal Korean Medicine
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    • v.36 no.4
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    • pp.527-546
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    • 2015
  • Objectives The purpose of this study was to investigate the correlation between gastric emptying measured by ultrasonography and Korean medical instruments of diagnosis and assessment in functional dyspepsia (FD) patients. Among the subgroups of FD, postprandial distress syndrome (PDS) is related to gastric motility disorder.Methods Ten patients with FD and particularly with PDS as well as 10 healthy controls were enrolled in the study from September to November 2015. The gastric emptying shown as the half-life of gastric volume (T1/2) was measured by ultrasonography. The severities of spleen qi deficiency and dyspepsia symptoms were assessed by a spleen qi deficiency questionnaire (SQDQ) and the Nepean Dyspepsia Index-Korean version (NDI-K), respectively. In addition, a food retention questionnaire (FRQ), a damum questionnaire (DQ), a cold and heat questionnaire (CHQ), a deficiency and excess questionnaire (DEQ), and a visual analogue scale (VAS) of distention and fullness were completed by every participant.Results In comparison with the control group, the FD group showed significantly higher scores for the SQDQ, NDI-K, FRQ, DQ, DEQ, and VAS of distention and fullness. T1/2 was also significantly higher in the FD group than in the control group. There were significant correlations between T1/2 and the SQDQ score. However, there were no significant correlations between T1/2 and other questionnaire scores except for one item of the NDI-K.Conclusions According to these findings, it was determined that measuring gastric emptying using ultrasonography could be a quantitative indicator to diagnose spleen qi deficiency in FD patients.

A novel GLA mutation in a Korean boy with an early cardiac manifestation of Fabry disease

  • Kwon, Soonhak;Park, Jin-Sung;Jung, Jae Hun;Hwang, Su Kyeong;Kim, Yeo Hyang;Lee, Yun Jeong
    • Journal of Genetic Medicine
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    • v.15 no.1
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    • pp.28-33
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    • 2018
  • Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by the deficiency of ${\alpha}$-galactosidase A. Patients with classical FD present acroparesthesia, hypohidrosis, cornea verticillata, disseminated angiokeratoma, and microalbuminuria in childhood, and develop life-threatening renal, cardiac, and cerebrovascular complications typically after the fourth decade of life. To date, more than 700 mutations responsible for FD have been identified in the human GLA gene. Herein, we report a novel GLA mutation, c.1117_1141del25 (p.Gly373Profs*10), identified in an 11-year-old Korean boy with FD presenting early cardiac and neurologic manifestation and in other affected family members. The boy had acroparesthesia, hypohidrosis, cornea verticillata, and left ventricular hypertrophy. His mother and sister also had acroparesthesia. Two males on the mother's side had similar pain and died of unknown causes. The plasma ${\alpha}$-galactosidase A activity (4.1 nmol/hr/mg protein) of the patient was markedly lower than the mean value of the controls. The plasma level of globotriaosylsphingosine was elevated in the patient and all the carriers. We concluded the novel GLA mutation c.1117_1141del25 is a pathogenic mutation for FD, probably related to the early cardiac manifestation of FD.