Pseudohypoaldosteronism type 1 is a genetic renal tubular disease of salt wasting, presenting in young infants. Tubular unresponsiveness to elevated endogenous and exogenous aldosterone is the suggested pathogenetic mechanism. Oral sodium chloride supplementation relieve the clinical symptoms and electrolyte distrubances. We experienced 2 cases of PHA type 1 in 38-day and 45-day old male infants who were presented with failure to thrive, vomiting and/or dehydration. Laboratory data showed hyponatremia, hyperkalemia, hypochloremia and metabolic acidosis. Renal and adrenal functions were normal. Plasma renin activity and plasma aldosterone concentration were markedly elevated. Under the diagnosis of pseudohypoaldosteronism type 1, oral supplementation of NaCl and/or kayexalate improved the clinical states of the patients.
Acidosis ; Aldosterone ; Dehydration ; Diagnosis ; Failure to Thrive ; Humans ; Hyperkalemia ; Hyponatremia ; Infant ; Male ; Plasma ; Pseudohypoaldosteronism* ; Renin ; Sodium Chloride ; Vomiting

Clinical observations were made on 338 low birth weight infants, who were delivered at Pusan Wallace Memorial Baptist Hospital, during the 3 year periods from 1989 to 1991. The results were as follows; The incidence of LBWI was 5.6% and sex ratio was 1:1.09. Among them AGA was 62.7%. 2) Of all infants, 47.3% were in the birth weight range of 2251-2500 gm and 25.1% were in the gestational age group of 37-38 week. 3) LBWI were most prevalent among mothers under 20 years old and over 41 years old. 4) The Incidence of LBWI was higher in multiparity than primiparity and in multiple pregnancy than single pregnancy. 5) Vaginal delivery was 63.9% and c-section delivery was 36.1%. 6) The order or frequency of the etiology of LBWI was multiple pregnancy, premature rupture of membrane and toxemia. 7) The mortality rate of LBWI was 12.7% and mortality rate by weight-gestational age was lower in AGA group than SGA and LGA group. 8) A Higher mortality rate was noted in the group of lower birth weight infant, and in the group of more shortened gestational age. 9) 48.8% of death occured within ist day of birth. The common causes of death were respiratory distress syndrome, asphyxia, and apnea.
Adult ; Apnea ; Asphyxia ; Birth Weight ; Busan ; Cause of Death ; Female ; Gestational Age ; Humans ; Incidence ; Infant* ; Infant, Low Birth Weight* ; Infant, Newborn ; Membranes ; Mortality ; Mothers ; Parity ; Parturition ; Pregnancy ; Pregnancy, Multiple ; Protestantism ; Rupture ; Sex Ratio ; Toxemia ; Young Adult

Parosteal lipoma is a rare and generally asymptomatic benign tumor of mature adipose tissue that is located in direct apposition to the external surface of the bone. These tumors are occasionally associated with reactive changes in the underlying bone. The reactive bone formation is generally restricted to the base of the tumor, that is, near the periosteum. We recently experienced a case of parosteal lipoma in the proximal femur, which displayed exuberant bone and cartilage formation, and this led us to a misdiagnosis of chondroblastic osteosarcoma on the initial biopsy. We report here on this case with a special emphasis on making the differential diagnosis from osteosarcoma.
Adipose Tissue ; Biopsy ; Cartilage ; Chondrocytes ; Diagnosis, Differential ; Diagnostic Errors ; Femur* ; Lipoma* ; Osteogenesis ; Osteosarcoma ; Osteosarcoma, Juxtacortical ; Periosteum

We describe a case of fine needle aspiration cytology of metastatic nasopharyngeal carcinoma in the lymph node of the neck presenting a predominantly spindle cell pattern. A 36 year-old male patient complained of dysesthesia on the right face and a palpable neck mass. Fine needle aspiration was done on the neck mass. Tumor cells were present in syncytial groups or singly with mainly spindle shaped nuclei, vesicular chromatin, thin and regular nuclear membrane, occasional prominent nucleoli and a few foci of cellular cohesiveness. The cytoplasm was scant and pale with ill-defined borders. Mature lymphocytes were present in the background of aspirates and within the tumor cell clusters. Histologically, the tumor of nasopharynx showed several areas of spindle cell pattern. Because the tumor cells showed a predominantly spindle shape with vesicular nuclear chromatin, the differential diagnosis of spindle cell sarcoma or granuloma of epithelioid cells were considered, but the characteristic morphology of the nuclei with vesicular chromatin and prominent nucleoli, and cellular cohesiveness were important in making the diagnosis of nasopharyngeal carcinoma. The possibility of metastatic carcinoma should always be considered in fine needle aspiration cytology of the lymph node in the neck because the incidence of metastatic carcinoma, particularly of the nasopharyngeal carcinoma in the lymph nodes of the neck is relatively high.
Child ; Male ; Female ; Humans ; Diagnosis, Differential ; Incidence ; Neoplasm Metastasis

PURPOSE: To investigate the relation of the gastric epithelial cell proliferation, apoptosis and genotypes of H. pylori in children. METHODS: Histologic grading by updated Sydney system, PCNA immunostaining, TUNEL method and the genotypes (cagA, picB and iceA) by PCR were performed in H. pylori positive (N=20) and negative (N=20) gastric biopsy specimens. RESULTS: PCNA index was significantly different between H. pylori positive children (77.4+/-13.12) and H. pylori negative children (52.3+/-12.20) (p=0.000). There were positive correlations between PCNA index and H. pylori density (r=0.624, p=0.000), polymorphonuclear neutrophil activity (r=0.460, p=0.005) and chronic inflammation (r=0.433, p=0.009). Apoptosis index of H. pylori positive children (0.70+/-0.411) was significantly higher than of H. pylori negative children (0.14+/-0.201) (p=0.000). Positive correlations between apoptosis index and H. pylori density (r=0.691, p=0.000), polymorphonuclear neutrophil activity (r=0.585, p=0.000) and chronic inflammation (r=0.535, p=0.001) were noted. As PCNA index increased, apoptosis index significantly increased (r=0.527, p=0.001). The positive rates of genotypes were cagA 90%, picB 75%, iceA1 60% and iceA2 15%, respectively. There were no significant correlations between the status of the genotypes and PCNA index, apoptosis index, the endoscopic findings and the histologic findings. CONCLUSION: PCNA index and apoptosis index in H. pylori positive children were higher than in H. pylori negative children but were not related to H. pylori genotypes. This study suggested that correlatively increased gastric epithelial cell proliferation and apoptosis are important to pathogenesis of H. pylori infection in children.
Apoptosis* ; Biopsy ; Child* ; Epithelial Cells* ; Genotype* ; Helicobacter pylori* ; Helicobacter* ; Humans ; In Situ Nick-End Labeling ; Inflammation ; Neutrophils ; Polymerase Chain Reaction ; Proliferating Cell Nuclear Antigen

BACKGROUND: Germline mutations in the breast cancer-associated genes BRCA1 and BRCA2 confer susceptibility and a lifetime risk of breast. Several morphological and clinical features have been attributed to hereditary tumors. However, in sporadic breast cancer, the interrelationship between the loss of heterozygosity (LOH) of these loci and clinical features remains to be fully elucidated. METHODS: Microdissected paraffin-embedded tissue blocks of 48 cases of surgically resected breast carcinoma were investigated to identify the LOH of BRCA1 and BRCA2 using microsatellite markers. RESULTS: Of 48 cases, 22 (45.9%) exhibited LOH at BRCA1 locus while in 29 out of 48 (60.4%) cases LOH was observed for the BRCA2 region. There was no significant correlation between LOH at BRCA1/2 and the patient's age, tumor size, histologic grade or lymph node metastasis. When comparing the frequency of LOH with the expression of several prognostic factors, such as p53, c-erb B2 protein, estrogen and progesterone receptor using immunohistochemical stain, there was only correlation with LOH at BRCA2 and the progesterone receptor. CONCLUSIONS: Our results suggest that allelic deletion play a role to the development of sporadic breast cancers.
Breast Neoplasms* ; Breast* ; Estrogens ; Germ-Line Mutation ; Loss of Heterozygosity* ; Lymph Nodes ; Microsatellite Repeats ; Neoplasm Metastasis ; Paraffin* ; Receptors, Progesterone

No abstract available.
Urinary Tract*

PURPOSE: The aim of this study was to examine the relationship between tooth loss and sub-clinical atherosclerosis in Korean adults. MATERIALS AND METHODS: The subjects were part of a cohort study conducted in Ansan city by the Korea University medical school as part of the Korean Genome project. 749 subjects over than 40 years old were evaluated. After taking panoramic radiography, the amount of tooth loss was calculated. The intima-media thickness (IMT) was assessed by using ultrasonography at the common carotid artery. Traditional cardiovascular risk factors for atherosclerosis were also evaluated. The relationship between tooth loss and the IMT was evaluated using ANOVA with Scheffe's multiple comparison method in univariate analysis. Multiple regression analysis was also performed to determine the significance between the IMT and tooth loss. RESULTS: With age, tooth loss increased, but there was no significant increase in other traditional cardiovascular risk factors. Univariate analysis revealed the IMT to be positively related with the amount of tooth loss. Regression analysis of the IMT in the anterior and posterior tooth loss revealed that only the posterior tooth loss was significantly related with the IMT at all sites of the common carotid artery (right far wall, P = .015; left far wall, P = .008; right near wall, P < .001; left near wall, P = .001). CONCLUSION: This study verified the positive relationship between the increased tooth loss at the posterior area and the accumulation of atheroma in arteries.
Adult ; Arteries ; Atherosclerosis ; Carotid Artery, Common ; Carotid Intima-Media Thickness ; Cohort Studies ; Genome ; Humans ; Korea ; Plaque, Atherosclerotic ; Radiography, Panoramic ; Risk Factors ; Schools, Medical ; Tooth ; Tooth Loss

BACKGROUND AND OBJECTIVES: Qualitative oculomotor abnormalities have been reported in parkinsonian patients for many years, but conflicting results have been obtained. This study was performed to evaluate the correlation between the severity of the disease and the abnormalities of the ocular movements in idiopathic parkinson's disease. METHODS: We gave the vestibular function tests in patients with idiopathic parkinson's disease and normal controls. Eye movement recordings were made with automated electronystagmography and rotation test was performed. A total of 46 patients (mean age : 61.2+/-6.7) and 24 controls (mean age : 60.5+/-4.3) were studied. The severity of the disease was divided into two groups by modified Hoehn & Yahr staging ; H-Y stage 1 and 2 as a mild group and stage 3 and 4 as a severe group. RESULTS: Saccadic latency and accuracy, pursuitic gain and velocity, vestibulo-ocular reflex (VOR) suppression by vision were significantly altered in patients, whereas mean velocity of optokinetic nystagmus (OKN) and VOR gain in darkness were normal. Alteration of saccadic latency and accuracy, pursuitic gain and velocity, VOR suppression by vision were profound in the severe group compared with a mild group and controls, but the above parameters did not differ between a mild group and controls. In a hemiparkinson's group, saccadic latency and accuracy, pursuitic gain, OKN mean velocity and gain was not different between the both sides. CONCLUSION: The results indicate that severe Parkinson's disease damages nigrostriatal or other specific pathways which were involved in the regulation of the saccadic, pursuitic and pursuitic-mediated visual fixation system. In a hemiparkinson's group asymmetric damage of dopaminergic innervation which was involved in the regulation of ocular movements was not found.
Darkness ; Electronystagmography ; Eye Movements ; Humans ; Nystagmus, Optokinetic ; Parkinson Disease* ; Reflex, Vestibulo-Ocular ; Vestibular Function Tests