Lymphangioleiomyomatosis(LAM) is a rare disease of women of child-bearing age in which there is progressive hyperplasia of atypical smooth mucle along lymphatics in the lung, and/or axial lymphatics in the thorax and abdomen, resulting in honeycombing of lung. Interestingly there has been a speculation that it represents a forme furste or incomplete expression of tuberous sclerosis complex. This is based on the observation that patients with tuberous sclerosis can manifest pulmonary lesions indistinguishable from LAM. We report a case of LAM occuring in a 39-year-old female, who complained of recurrent pneumothorax, chest pain and shortness of breath. Three years ago, the patient had right nephrectomy under the diagnosis of ruptured angiomyolipoma. A X-ray film of the chest showed honeycombing with a diffusely reticulonodular pattern and cyst-like spaces. She had a characteristic facial appearance of adenoma sebaceum, which her father and uncle had. Microscopically, the lung showed a marked smooth muscle proliferation around the slit-like lymphatic spaces and also some respiratory bronchioles.
Female ; Humans ; Adenoma

Eosinophilic gastroenteritis is a rare disease of unknown etiology. It is characterized by the infiltration of the gastrointestinal tract by mature eosinophils, and increased peripheral eosinophil count and associated with various food allergies. Clinical manifestations were related to the site of histologic infiltration in the wall and the segment of the involved gastrointestinal tract. Recently, the authors experienced one case of eosinophilic gastroenteritis of the small bowel with spontaneous rupture that was managed by segmental resection of ileum and end to end anastomosis. Four years previous, he has undergone segmental resection of the ileum due to ileum perforation of unknown etiology. The peripheral blood eosinophil count of this case was normal but the IgE level was elevated. Microscopically, there was a dense infiltration of eosinophils throughout the entire thickness of the ileal wall, particularly in the muscle layer. The patient recovered well, but had one episode of abdominal pain with diarrhea and those symptoms were improved following the administration of corticosteroids. A brief review of the etiology, pathology, clinical features, diagnosis and management of this disease is presented.
Abdominal Pain ; Adrenal Cortex Hormones ; Diagnosis ; Diarrhea ; Eosinophils* ; Food Hypersensitivity ; Gastroenteritis* ; Gastrointestinal Tract ; Humans ; Ileum ; Immunoglobulin E ; Pathology, Clinical ; Rare Diseases ; Rupture* ; Rupture, Spontaneous

OBJECTIVE AND METHODS: To determine correlations among ambulatory renin activity, ambulatory blood pressure and microalbumin excretion rate, 66 Korean essential hypertensives were studied after 4 week wash-out period. The ambulatory blood pressure was monitored every 30 minutes and mean BP were calculated automatically. Urinary microalbumin excretion rate(UAER) and ambulatory plasma renin activity(aPRA) collected at mid-day were measured by radioimmunoassays. Subjectives were divided into 2 groups by aPRA value(2ng/ml/hr). RESULT: 14 cases were high renin group and 52 cases low renin group. The mean BP were 148.83/94.69mmHg in low renin group, and 146.57/98.07mmHg in high-renin group without difference. UAER were not different also between both groups. 23.07%(4/14) of non-dippers were included in high renin group and 25.58%(12/52) in low renin group without statistical difference. The aPRA was significantly related to UAER and systolic and diastolic mean blood pressure. Also UAER was related significantly to day mean blood pressures. CONCLUSION: Thus aPRA is thought to be a meaningful indicator to predict hypertensive renal target organ damage as well as blood pressure measured with 24-hr ABPM.
Blood Pressure* ; Hypertension* ; Plasma* ; Radioimmunoassay ; Renin*

Eosinophilic granuloma of the lung, first described by Farrinaci et al. in 1951, is rare. A 35-year-old male smoker presented with recurrent pneumothorax. Open thoracotomy with bleb resection and biopsy was performed. Microscopically there was histological changes consistent with typical eosinophilic granuloma and intertitial fibrosis. The Langerhans cells showed positive reaction for S-100 protein and typical Birbeck granules in their cytoplasm. A brief summary of histopathological aspect of this disease and a review of literature are presented.
Male ; Humans ; Biopsy

The evaluate the diagnostic findings of salivary gland tumors, we reexamined aspiration cytology smears of 7 cases of pleomorphic adenoma, 3 cases of adenoid cystic carcinoma, and 3 cases of mucoepidermoid carcinoma, performed during April 1986 to March 1990, which were comfurmed by surgical excision and histologic diagnosis. The results obtained are summarized as follows : 1. All cases of pleomorphic adenoma showed branching cellular clusters of epithelial and myoepithelial cells. Acellular elements including myxomatous and chondroid components were observed. There were no cellular pleomorphism and nucleoli. Keratinizing squamous epithelial cells and keratin pearls were noted. 2. The smears of adenoid cystic carcinoma showed cell balls or cell cords containing a central hyaline core. Nuclear atypism and the nucleoli were frequently observed. There were no keratinizing squamous epithelial cells. 3. The smears of mucoepidermoid carcinoma showed mainly sheets or clusters of intermediate cells and some mucin-producing cells. Some nuclear pleomorphism was observed. Mucinous material and many inflammatory cells were present in the background.
Adenoids* ; Adenoma, Pleomorphic* ; Biopsy, Fine-Needle* ; Carcinoma, Adenoid Cystic* ; Carcinoma, Mucoepidermoid* ; Diagnosis ; Epithelial Cells ; Hyalin ; Mucins ; Salivary Glands*

PURPOSE: Transabdominal sonography has been widely used in the diagnosis of suspected urinary calculi, assisted by secondary findings from urinary tract obstruction, but this method shows low specificity and relatively high incidence of false-positives. Recently, detection of stone itself with new Doppler finding and bladder distension on sonography has demonstrated high sensitivity. METHODS: We performed prospective transabdominal ultrasonography for emergency department patients with acute urinary colic pain over a 3 month period. With adequate bladder distension by intravenous hydration and initial spontaneous voiding drive, stones itself could be tracked along the entire length of urinary tract by emergency physician who received short-term education course. RESULTS: One hundred and thirty one patients enrolled; 86 received a sonographic exam and 57 showed urinary calculi. The detection rate of urinary calculi was 71.3%. The most common sonographic feature used in the diagnosis of urinary calculi was a distinct echogenic stone appearance within the dilated urinary tract with posterior acoustic shadowing. CONCLUSION: We concluded that detection of urinary calculi by transabdominal ultrasonography could be very useful in mbination with secondary sonographic finding from urinary tract obstruction in the evaluation of suspected urinary colic.
Acoustics ; Colic ; Diagnosis ; Education ; Emergencies ; Emergency Service, Hospital ; Humans ; Incidence ; Prospective Studies ; Sensitivity and Specificity ; Shadowing (Histology) ; Ultrasonography* ; Urinary Bladder ; Urinary Calculi* ; Urinary Tract

PURPOSE: This study estimated the prevalence of lower urinary tract symptoms (LUTS) in Korean men and the conditions for being diagnosed with or treated for LUTS/benign prostatic hyperplasia (BPH). METHODS: We analyzed cross-sectional data collected from 69,851 Korean men who were 40 years of age or older and had participated in the Korean Community Health Survey performed in 2011. Interviewers performed face-to-face surveys that included sociodemographic questions, the International Prostate Symptom Score (IPSS), and questions regarding whether the subjects had been diagnosed with or treated for LUTS/BPH. We estimated the prevalence of LUTS and assessed whether the subjects had been diagnosed with or treated for LUTS/BPH. RESULTS: Moderate to severe LUTS, storage symptoms, and voiding symptoms increased with age. The IPSS quality of life score was 1.5±0.004 in the mild LUTS group (n=57,701), 3.3±0.01 in the moderate LUTS group (n=9,203), and 4.3±0.02 in the severe LUTS group (n=2,947) (P<0.0001). The prevalence of moderate to severe LUTS in those who had not been diagnosed with LUTS/BPH was 64.5% (7,847 of 12,150), and the prevalence of moderate to severe LUTS in those who had been diagnosed with LUTS/BPH but had not been treated was 23.5% (2,853 of 12,150). CONCLUSIONS: The severity of LUTS in Korean men increased with age, and the IPSS quality of life score increased with the severity of LUTS. Many Korean men with moderate to severe LUTS had not been diagnosed or treated for LUTS/BPH. Socioeconomic conditions played an important role in health-seeking behavior among patients with LUTS/BPH.
Health Surveys* ; Humans ; Lower Urinary Tract Symptoms* ; Male ; Prevalence ; Prostate ; Prostatic Hyperplasia ; Quality of Life

BACKGROUND: The catheter ablation usin radiofrequency(RF) energy in patients with atrioventricular nodal reentrant tachycardia(AVNRT) and atrioventricular reentrant tachycardia(AVRT) ahs been proved as a safe and effective nonpharmacologic therapeutic modality. The purpose of our study was to evaluate the success rate and complications of the initial experience and to determine the extent of myocardial damage of RF catheter ablation. METHODS: Electrophysiologic study was performed with the standard technique. Twenty five patinents(M:F=16:9, mean age:42 years old) underwent RF catheter ablation in St. May's Hospital from April to December in 1994. The RF generator in this study was RFG-3D model and catheters were 6F or 7F steerable catheters with 4mm kistal tip. In order to evaluate the extent of myocardial damage the WBC count, LDH, CK, and CK-MB fraction were checked before and after RF catheter ablation and the Tc99m myocardial scintigraphy was performed within 72 hours of the porcedure. RESULTS: Six of twenty five patients had AVNRT, in which the success rate of selective ablation of the slow pathway was 83.8%. Nineteen patients with AVRT had one accessory pathway. The ablation success rate of 14 accessory pathways in left free wall location was 85.7%, and that of 3 in left posteroseptal location was 66.6%. Two right sided accessory pathways were not ablated successfully. The level of CK-MB fraction after ablation was within normal limit. Only one case revealed grade 2 of hot spot in Tc99m myocardial scintigraphy. CONCLUSION: The RF catheter ablation in patients with AVNRT and AVRT is a safe and effective nonpharmacologic therapeutic modality. But right sided accessory pathways are more difficult to ablate than left sided accessory pathways, requiring more experiences and better technique. The extent of myocardial damage after RF catheter ablation reveals relatively minimal by cardiac enzyme study and Tc99mmyocardial scintigraphy.
Catheter Ablation* ; Catheters ; Humans ; Myocardial Perfusion Imaging ; Radionuclide Imaging ; Tachycardia, Supraventricular*

OBJECTIVE: To estimate the prevalence of congenital heart disease from the 1998 student heart disease screening program. METHODS: The heart disease screening program for elementary students was conducted in Kyonggi-do, in 1998. The subjects of the present study comprised the 40,402 students who attended the schools in the catchment area of a collaborative university hospital and who participated in the primary examination. The congenital heart disease (CHD) patients were initially identified through a questionnaire about prior medical history, and further through diagnostic tests & medical examinations in the secondary & the tertiary examinations. Certain assumptions were used in the estimation of the number of CHD cases among non-participants of the secondary & tertiary examinations. The overall prevalence of CHD was estimated by adding the CHD detection rates of the participants and the estimated prevalence of the non-participants. RESULTS: Among the 40,402 primary participants, 1,655 were referred further, of whom 79.1% (1,309) participated in the secondary examination. Of these, 121 were referred to the tertiary examination, with a participation rate at this last stage of 80.2%. The positive predictive value (PPV) of the screening tools was the highest when the results of both EKG and the questionnaire were positive. Because 85.9% of the detected cases had a past history of CHD, PPV was higher when the selection criteria in the questionnaire included past CHD history than when it didnt. The CHD detection rate among the participants was 1.76 cases/1,000 and the presumed number of cases among the non-participants was 31; giving an estimated final CHD prevalence of 2.52 cases/1,000 (95% CI : 2.06-3.06). Among the identified cases of CHD, VSD (52.8%) was the most common, followed by PDA (9.7%), TOF (9.7%) & PS (9.7%). CONCLUSION: Because the characteristics of the non-participants differed from those of the participants, the estimation of prevalence was influenced by the participation rate. Of the detected cases, 85.9% had a past history of diagnosis or operation for CHD. These findings suggested that the prevalence estimated in this study may be an underestimation of the actual condition. Therefore, a birth cohort study is required in order to more accurately estimate the prevalence and the effects of the program.
Cohort Studies ; Diagnosis ; Diagnostic Tests, Routine ; Electrocardiography ; Gyeonggi-do ; Heart Defects, Congenital* ; Heart Diseases* ; Heart* ; Humans ; Mass Screening* ; Parturition ; Patient Selection ; Prevalence* ; Questionnaires

BACKGROUND: The autonomic nervous system plays a critical role in triggering ventricular arrhyhmia and sudden death early after acute myocardial infarction. This study was designed to determine whether or not vagal and sympathetic responses are impaired after myocardial infarction and to evaluate the utility of physiologic stress tests for assessing autonomic dysfunction after myocardial infarction. METHODS: 8 male patients with acute myocardial infarction(Group A) and old myocardial infarction(Group B) were studied with 8 control subjects of coinciding age and sex. 5 physiologic stress tests(deep breathing, sudden standing, Valsalva maneuver, ice bag application on the face, 70degrees headd-up tilt test) were performed. RESULTS: 1) Variation in heart rate during deep breathing, Standing-up, Valsalva maneuver, and ice bag application on the face was less in Group A than in Group B or Group C. 2) There was no significant difference in variation of heart rate between Group B and Group C. 3) Variation in heart rate caused by 70degrees tilt was not significantly different among the three groups. CONCLUSION: Early after myocardial infarction, parasympathetic responses were significantly impaired, whereas sympathetic responses remained intact. Heart rate variability using physiologic stress test may be provide a means of detecting autonomic dysfunction after acute myocardial infarction.
Autonomic Nervous System* ; Death, Sudden ; Exercise Test ; Heart Rate ; Humans ; Ice ; Male ; Myocardial Infarction* ; Respiration ; Valsalva Maneuver