• Journal of Oral Medicine and Pain
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• v.37 no.3
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• pp.135-139
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• 2012

Hereditary hemorrhagic telangiectasia is a rare autosomal dorminant disease that features abnormal and fragile vascular dilations of terminal vessels in skin and mucous membranes, as well as arteriovenous malformations of internal organs, particularly lungs, brain, and liver. Often patients have not been diagnosed with HHT for a long time, and undiagnosed HHT patients unnecessarily develop serious complications such as severe life-threatening hemorrhage, stroke or brain abscess. Therefore, early detection and appropriate screening is very important. Early detection of HHT allows the appropriate screening for the presence of silent disease such as AVMs in the lungs, liver, or brain, and preventive treatment in the patient and their affected family members. Dentists should be familiar with HHT because the telangiectases on skin and oral mucosa are often the most dramatic and most easily identified component of HHT. Recently, we experienced a case of HHT. We present the case with a review of the literature.

• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.1018-1023
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• 2007

Hereditary hemorrhagic telagiectasia (HHT), which is characterized by the classic triad of mucocutaneous telangiectases, arteriovenous malformations (AVMs) and inheritance, is an autosomal dominant disorder. The characteristic manifestations of HHT are all due to abnormalities of the vascular structure. This report deals with the case of a 14-year-old girl with typical features of HHT that include recurrent epistaxis, mucocutanous telangiectases, pulmonary and cerebral AVMs and a familial occurrence.

• Journal of Veterinary Clinics
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• v.24 no.3
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• pp.437-440
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• 2007

A six-year-old, intact female Indian ring-necked parakeet (Psittacula krameri manillensis) was presented due to chronic weight loss and anorexia. The bird was tentatively diagnosed as proventricular dilation disease (PDD) by radiographic evidences of dilated crop and proventriculus. The patient was symptomatically treated, however the bird was die two months after the first presentation. At necropsy finding, distinctive signs of PDD were noted, and PDD concurrent with ingluvitis was finally diagnosed according to histopatholgic examination.

• Tuberculosis and Respiratory Diseases
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• v.46 no.4
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• pp.489-499
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• 1999

Background: The patient with bronchiectasis may have obstructive ventilatory impairment combined with mild restrictive ventilatory impairment due to fibrosis of surrounding lung parenchyme and pleural adhesions caused by chronic recurrent pulmonary infections. Since hyperinflation or emphysematous change can be occured in bronchiectasis, pulmonary functions such as lung volumes and diffusing capacity may also vary with associated emphysema. Methods: For the evaluation of lung volumes and diffusing capacity in bronchiectasis with respect to the anatomic types and severity of bronchiectasis, a total of 40 cases comprising 24 cases of tubular, and 16 cystic type of bronchiectasis were analyzed retrospectively. Correlation between lung functions and extent of bronchiectasis or associated emphysema detected in HRCT were also evaluated. Results: Vital capacity(VC) tended to decrease in cystic type than in tubular type. As the severity of bronchiectasis became serious, the VC were significantly reduced, whereas the total lung capacity(TLC), residual volume(RV) and its ratio to the total lung capacity(RV/TLC) had no significant difference. Lung clearance index(LCI) was significantly increased in cystic type than in tubular type, whereas the slope of phase III in single breath nitrogen curve($\triangle$N2/L) was not significantly changed regard to the type and severity of bronchiectasis. DLCO and DLCO/VA reflecting diffusing capacity were significantly decreased in cystic type and also as the severity of bronchiectasis became serious. The correlation coefficient of VC, DLCO and LCI with the extent of bronchiectasis were -0.322, -0.339 and 0.487, respectively, whereas other parameters were not significantly correlated with the extent of bronchiectasis. VC and DLCO correlated negatively with the extent of emphysema while RV, RV/TLC, LCI and $\triangle$N2/L correlated positively. Conclusion: These findings suggest that the reduction of VC and diffusing capacity or uneven distribution of inspired gas in bronchiectasis are related to both the extent of bronchiectasis and associated emphysema while increased residual volume be related to the extent of associated emphysema alone.

• Journal of the korean veterinary medical association
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• v.23 no.1
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• pp.33-35
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• 1987

• Tuberculosis and Respiratory Diseases
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• v.40 no.4
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• pp.390-394
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• 1993

Background: Bronchiectasis is characterized by chronic sputum production and complications such as hemoptysis and repeated infections. Even though some patients are cured by surgical resection of bronchiectatic lesions, most bronchiectasis patients can not be treated surgically because of multiple site involvement, and they are treated by medical measures such as postura1 drainage and antibiotics when indicated. Recently there have been some reports that low-dose longterm erythromycin treatment is effective on bronchiectsis, and it is well known that low-dose longterm erythromycin treatment is the treatment of choice in diffuse panbronchiolitis which is characterized by chronic sputum production and dyspnea. To evaluate the efficacy of erythromycin, we tried erythromycin on twenty five stable bronchiectasis patients for more than six months. Methods: We tried erythromycin 250 mg b.i.d. for more than 6 months. We checked respiratory symptoms, chest PA, spirometry, and side effects before treatment and after 3 and 6 months of treatment. Results: 1) 32% of the patients showed marked improvement of symptoms and PFT. 32% showed slight improvement of symptoms with little change of PFT, and 36% showed no change of symptoms and signs. 2) Analysis of the patients showing marked improvement revealed that most of them had diffuse bronchiectasis and paranasal sinus involvement. Conclusion: These results suggest that low-dose longterm erythromycin treatment can be tried on diffuse bronchiectasis patients with sinus involvement. And further studies will be followed on the mechanism of erythromycin in bronchiectasis.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1043-1047
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• 2002

Phlebectasia is an abnormal dilatation of an isolated vein and a rare venous anomaly and is usually asymptomatic. Clinically internal jugular phlebectasia is a self limited benign condition and usually no treatment is required after initial diagnosis. So suspection of this disease and appropriate diagnostic approaches are essential to avoid unnecessary surgical intervention. We present three cases of internal jugular phlebectasia of which diagnosis was made by neck sonography and CT.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.1
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• pp.93-98
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• 1999

We experienced a case of primary intestinal lymphangiectasia in a 20-month-old boy, which was confirmed gastrofiberscopically and histologically only after a high-fat meal before the procedure. A characteristic endoscopic finding was the appearance of tiny white dots scattered in the duodenal mucosa, which were proved dilated lymph vessels in the lamina propria on histological examination. Treatment with low-fat diet and medium chain triglycerides supplementation was satisfactory.

• 보건세계
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• v.39 no.2 s.426
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• pp.4-5
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• 1992

• 보건세계
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• v.48 no.2 s.534
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• pp.10-13
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• 2001