• Title/Summary/Keyword: Connective Tissue Disease

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DENTAL MANAGEMENT OF A CHILD WITH WILLIAMS SYNDROME (윌리엄스 증후군 환아의 치과적 치험례)

  • Shun, Ye-Kyung
    • Journal of the korean academy of Pediatric Dentistry
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    • v.34 no.4
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    • pp.666-671
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    • 2007
  • Williams syndrome is a rare congenital disorder characterized by multiple anomalies involving the cardiovascular system, connective tissue, and the central nervous system resulting in mental retardation, distinctive facial features, and cardiovascular disease. It is also known to present typical oral manifestation including dental malformations, agenesis of teeth, and malocclusion. Impaction of a permanent tooth due to its deviant eruption path was observed in this case. The aim of this article is to report oral manifestation of a girl with Williams syndrome and the following dental treatment procedure.

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Pathological studies on Expired Guinea Pigs due to Epizootic Streptococal Infection (유행성 연쇄상구균 감염에 의해 폐사된 기니픽의 병리학적 연구)

  • Son, Woo-Chan;Lee, Sang-Koo
    • Korean Journal of Veterinary Pathology
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    • v.1 no.1
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    • pp.61-65
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    • 1997
  • Epizootic streptococal infction is one of the common disease in guinea pig. We have confirmed 54 case of epizootic streptococal infection in guinea pig by histopathology and epidemiology. Grossly infection is characterized by the presense one to several enlarged lymph nodes particularly of the mandible cervical lymph nodes and accompanied by pleuropneumonia. Histologically there were lymphadenitis with abscesses surrounded by loose connective tissue fibrinopurulent pneumonia pleuritis myocarditis pericarditis and peritonitis. Epidemiological survey revealed that mortality was high in young adult female(15.1%) and changeable weather season such as spring and fall. From findings it was suggested that epizootic streptococal infection of guinea pig begin to infect in breeding colony around weaning period and died from that infection at animal facility during the quarantine period.

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Valve Sparing Aortic Root Replacement in Children with Loeys-Dietz Syndrome

  • Sim, Hyung-Tae;Seo, Dong Ju;Yu, Jeong Jin;Baek, Jae Suk;Goo, Hyn Woo;Park, Jeong-Jun
    • Journal of Chest Surgery
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    • v.48 no.4
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    • pp.272-276
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    • 2015
  • Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.

Solitary Plasmacytoma of the Rib

  • Lee, Hae-Young;Kim, Jong-In;Kim, Ki-Nyun
    • Journal of Chest Surgery
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    • v.45 no.4
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    • pp.269-271
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    • 2012
  • Solitary plasmacytoma of the bone, and especially of a single rib, is a rare disease. Here we report a 73-year old male patient complaining of continuous chest wall pain around the right 5th rib shaft who underwent a wide excision of the rib tumor with surrounding connective tissue. He was diagnosed with solitary plasmacytoma and will undergo radiation therapy. We report this case with a review of the literature.

Desquamative Diseases of Gingiva (임상가를 위한 특집 3 - 잇몸이 벗겨지는 질환)

  • Jang, Hyun-Seon
    • The Journal of the Korean dental association
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    • v.52 no.12
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    • pp.726-733
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    • 2014
  • The gingiva consists of an epithelial layer and an underlying connective tissue layer. The oral epithelium is a keratinized, stratified, squamous epithelium. The epithelium can be divided into the following cell layer: basal layer, prickle cell layer, granular cell layer and keratinized cell layer. The desquamative disease of gingiva means exfoliative diseases of epithelial layer on the gingiva. The chronic desqumative gingivitis is usually related to the dematologic disorders that produce cutaneous and mucous membrane blisters. The cicatricial pemphigoid and lichen planus are representative diseases of the dermatologic cases. Patients may be asymptomatic or symptomatic. When symptomatic, their complaints range from a mild burning sentation to an severe pain. The clinical examination must be considered with a thorough history, and routine histologic and immunofluorescence studies. A systemic approach needs to achieve accurate diagnosis and treatment of the gingival desquamative diseases.

Familial gigantiform cementoma (가족성 거대 백악종)

  • Han Won-Jeong;Kim Eun-Kyung
    • Imaging Science in Dentistry
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    • v.36 no.3
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    • pp.157-162
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    • 2006
  • Familial gigantiform cementoma is a rare fibre-cemento-osseous disease of the jaws which appears to be transmitted as an autosomal dominant trait with variable expressivity of the phenotype. A 7-year-old girl visited DKUDH complaining of the painless facial deformity. Clinically, significant facio-lingual expansion was observed at the left maxilla, left mandibular body and symphysis portion. Malposition of lower anterior teeth was found. Panoramic radiograph and CT scan showed the extensive expansile mixed lesion at maxilla and mandible. Bone scan revealed hot spot at the maxilla and left side of mandible. Histologic examination revealed moderately dense fibrous connective tissue with scattered masses resembling cementum. The patient's mother had a history of the mandibular resection due to benign tumor. Her younger brother had buccal expansion of right mandible. We report our finding of a family that has exhibited clinical, radiographic and histologic findings consistent with the familial gigantiform cementoma.

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Overlap myositis associated with Sjögren's syndrome

  • Eun Ji Lee;Rae On Kim;Yon Hee Kim;Hakjae Roh
    • Annals of Clinical Neurophysiology
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    • v.25 no.1
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    • pp.41-44
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    • 2023
  • Overlap myositis (OM) associated with Sjögrens syndrome (SS) is a relatively uncommon disorder. It is more difficult to detect OM when a patient had not been previously diagnosed with SS. We report a case of OM associated SS that initially presented with dysphagia followed by proximal weakness. If proximal muscle weakness is preceded by dysphagia, the possibility of OM associated with connective-tissue disease should be considered.

Therapeutic Effects in the RIP-treated liver Fibrosis Rat Model (천연식물추출물(RIP)이 쥐의 간섬유화 치료에 미치는 영향)

  • Cho, Soo-Hyun
    • Journal of Korean Biological Nursing Science
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    • v.8 no.2
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    • pp.41-59
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    • 2006
  • Chronic liver diseases and hepatic cancer have been reported as 10% of cause of death in Koreans. Regardless of various causes, chronic liver disease accompanies commonly hepatic fibrosis. But still the mechanism of hepatic fibrosis remains poorly understood. Using the dimethylnitrosamine(DMN)-induced hepatic fibrosis rat model, We performed to evaluate the possible therapeutic effect of RIP(extracts of Phellodendron amurense and Patrinia scabiosaefolia) and to investigate the changes in referential connective tissue proteins($TGF-{\beta}_1$, ${\alpha}$-smooth muscle actin, and vimentin) as a marker of fibrogenesis. For these purposes, liver tissues were stained with H & E, and Azan staining for estimation of developing fibrosis. In the DMN-treated rat liver tissue, fibrosis were developed forming incomplete septal fibrosis. Whereas, in the RIP-treated rat liver tissues, the fibrosis were decreased recovering to normal morphology. The expressions of $TGF-{\beta}_1$, ${\alpha}$-smooth muscle actin($\alpha-SMA$), and vimetin were increased in the DMN-treated rat liver tissues, but decreased in the various areas of RIP-treated rat liver tissues. According to these results, RIP could be a possible therapeutic agent to reduce hepatic fibrosis, and the $TGF-{\beta}_1$, ${\alpha}$-SMA, and vimentin could be possible indicative markers of hepatic fibrosis development and recovery.

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Short-Term Lung Function Changes and Predictors of Progressive Systemic Sclerosis-Related Interstitial Lung Disease

  • Kaenmuang, Punchalee;Navasakulpong, Asma
    • Tuberculosis and Respiratory Diseases
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    • v.83 no.4
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    • pp.312-320
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    • 2020
  • Background: Systemic sclerosis (SSc) involves multiple organ systems and has the highest mortality among connective tissue diseases. Interstitial lung disease is the most common cause of death among SSc patients and requires closer studies and follow-ups. This study aimed to identify lung function changes and predictors of progressive disease in systemic sclerosis-related interstitial lung disease (SSc-ILD). Methods: A retrospective study extracted SSc patients from an electronic database January 2002-July 2019. Eligible cases were SSc patients >age 15 diagnosed with SSc-ILD. Factors associated with progressive disease were analyzed by univariate and multivariate logistic regression analyses. Results: Seventy-eight SSc-ILD cases were enrolled. Sixty-five patients (83.3%) were female, with mean age of 44.7±14.4, and 50 (64.1%) were diffuse type SSc-ILD. Most SSc-ILD patients had crackles (75.6%) and dyspnea on exertion (71.8%), and 19.2% of the SSc-ILD patients had no abnormal respiratory symptoms but had abnormal chest radiographic findings. The most common diagnosis of SSc-ILD patients was non-specific interstitial pneumonia (43.6%). The lung function values of diffusing capacity of the lung for carbon monoxide (DLCO) and DLCO per unit alveolar volume declined in progressive SSc-ILD during a 12-month follow-up. Male and no previous aspirin treatment were the two significant predictive factors of progressive SSc-ILD with adjusted odds ratios of 5.72 and 4.99, respectively. Conclusion: This present study showed that short-term lung function had declined during the 12-month follow-up in progressive SSc-ILD. The predictive factors in progressive SSc-ILD were male sex and no previous aspirin treatment. Close follow-up of the pulmonary function tests is necessary for early detection of progressive disease.

The comparative study between Dura mater and $Guidor^{(R)}$ in the healing of the classIII furcation defects in dogs (성견 3급 분지부 병소에서 Dura mater와 $Guidor^{(R)}$사용후 치주조직 치유의 비교 연구)

  • Choi, Seong-Ho;Koo, Hyun-Seo;Jung, Hyun-Chul;Cho, Kyoo-Sung;Chai, Jung-Kiu;Kim, Chong-Kwan
    • Journal of Periodontal and Implant Science
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    • v.27 no.3
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    • pp.479-493
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    • 1997
  • There are various treatment methods including barrier membranes in attaining periodontal regeneration and regaining the function of destructed periodontal tissues due to periodontal disease. Barrier membranes consist of non-Resorbable and resorbable types such as Dura mater and $Guidor^{(R)}$ used in the treatment of intrabony defects and classII furcation defects have been shown to be effectively increased the amount of new bone and cementum.In our study we used premolars with class III furcation defects created by removing the bone 4mm apically from CEJ in adult dogs and placed resorbable membrane Dura mater and $Guidor^{(R)}$ for the test group and flap operation was carried out for the control groups. The effect of membrane on junctional epithelium, alveloar bone, cementum, and gingival connective tisssue in the regeneration and healing potential of periodontal tissues was evaluated and healing results were evaluated histologically and histometrically 8 weeks following the surgical procedure. 1. In the clinical observation, there was no exposure of furcation defects in the control group, whereas slight membrane exposure was noted in the test group. 2. New bone was formed up to the level of the notch in the control group, whereas in the test group new bone formation was observed above the level of the notch. 3. New cementum was formed in both groups of the experiment. 4. The connective tissue observed between the new cementum and new bone in the test group were functionally orientated, compared to the irregular formation of connective tissues found in the control group. 5. Root resorption or ankylosis was not observed in any of the groups 6. The mean and median of the control group were 4.31% and 2.23% and for the Dura mater group were 27.85% and 15.57% respectively. There was no significant difference between Dura mater and the control group. 7. The mean and median of the control group were 4.31% and 2.23% and for the $Guidor^{(R)}$ group were 37.27% and 37.19% respectively. There was a significant difference in these two groups(P$Guidor^{(R)}$ were 37.27% and 37.19%. There was no significant difference between the two test groups. Thus, by using Dura mater and Guidor in classIII furcation defects, the predictable amount of periodontal ligament and alveolar bone regeneration may result.

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