The heterotopic and tissues may be divided into two categories: those that are found in the head and neck region, and those that arise elsewhere. The latter type is rare and most cases are found in the lungs of patients with neural tube defect, particularly anencephaly. Our report descrives anencephalic male infant with heterotopic glial nodules in both lungs. The largest nodule is 2x1.5x1 cm, locates in the lower lobe of the left lung, and has a round gray-white cut surface with cystic spaces. Microscopically, the nodules consist of irregularly arranged astrocytes and glial fibers, in which are embedded gland-like or cystically dilated bronchioles. The astrocytes and glial fibers are strongly positive for glial fibrillary acidic protein and show astrocytic filaments on electron microscopy. This will be an additional case supporting the amniotic fluid aspiration/implantation theory of pathogenetic mechanism of distal heerotopic glial tissue.
Infant ; Male ; Female ; Humans

No abstract available.
Nephrotic Syndrome*

Bronchogenic cyst is an uncommon congenital lesion which is derived from the primitive foregut. Most bronchogenic cyst may develope at the tracheal bifurcation, both main bronchi, the lung parenchymeand the mediastinum. A 40-year old male was evaluated for dyspnea and chest tightness. Computed tomography revealed a well dermarcated, 7.2 x 7.9 cm sized, homogeneous mass compressing the left atrium. 2D-echo showed grade III mitral regurgitation. We completely removed the cystic mass and then confirmed the bronchogenic cyst in the pathological diagnosis. During the follow up period, the patient progressed well without any symptoms and showed grade I mitral regurgitation on the 2D-Echo. Therefore, we report a case of the bronchogenic cyst causing grade III mitral regurgitation.
Adult ; Bronchi ; Bronchogenic Cyst* ; Diagnosis ; Dyspnea ; Follow-Up Studies ; Heart Atria ; Humans ; Lung ; Male ; Mediastinum ; Mitral Valve Insufficiency* ; Thorax

Following results were obtained from the light microscopic and stereomicroscopic observations of the breasts of rats treated with 9, 10-Dimethyl-1,2-Benzanthracene(DMBA). 1) Adenocarcinomas developed in 17 rats (24%) among 70 DMBA-treated rats. 2) Terminal and buds (TEB) were observed longer in DMBA-treated rats than in control group, but they finally disppeared 4 monthes after treatment. 3) Many hyperplastic alveolar nodules (HAN) developed in DMBA-treated rats. 4) There were no transitional lesions between TEB and adenocarcinoma or HAN and adenocarcinoma. 5) The number of lobules was decreased in DMBA-treated rats. On the other hand, terminal ducts were increased in number. These findings suggest that DMBA stimulate the regression of lobules and induce to form terminal ducts from which adenocarcinomas and HAN develop independently.
Rats ; Animals ; Adenocarcinoma ; Breast Neoplasms

Forty eight female Sprague-Dawley rats received a subcutaneous implant containing 12.5 mg estradiol ant the age of 3 weeks. Three rats were killed in 1, 2, 3, 4, 6 weeks and in every month during 2~12 months after implantation, and the breasts were examined by light microscope. In all rats, enlargement of terminal end buds was obseved in 1~2 weeks, maximum development of hyperplastic alveolar nodules in 3 weeks, and marked dilatation and secretion of alveoli or ducts in 1~12 months after implantation. Ductal epithelial hyperplasia was observed in 27 rats and carcinomas developed in 23 rats in 2~12 months after implantation. It was thought that the changes induced by estradiol are more similar to the human breast lesions, compared with changes induced by chemical carcinogens such as dimethylbenzanthracene(DMBA), because breast carcinomas developed in close relationship with ductal epithelial hyperplasia in both estradiol-treated rats and humans, but not in DMBA-treated rats.
Female ; Humans ; Rats ; Animals ; Carcinogens

Purpose: Hematuria and proteinuria have various causes and consequential outcomes in children. Immunosuppressants are needed in some children with biopsy-proven glomerulonephropathy but have many adverse effects. Since the clinical practice patterns of Korean pediatric nephrologists are diverse, we surveyed their opinions. Methods: Using a clinical vignette, the survey was emailed to all Korean Society of Pediatric Nephrology members. The questionnaires included diagnosis, examination, medications, and dietary recommendations for patients with hematuria and proteinuria. Results: A total of 32 clinicians (5.48%, 22 pediatric certificated nephrologists) responded to the survey. Most responders (87.5%) suspected immunoglobulin A nephropathy, and 68.8% replied that kidney biopsies were a diagnostic tool. Renin-angiotensin system inhibition (62.5%) or steroids (18.8%) were selected as the treatment. Salt and protein intakes were usually encouraged as dietary reference intakes (34.4% and 65.6%, respectively). Conclusions Children with abnormal urinalysis have various causes, treatments, and prognoses. As treatments such as immunosuppressants can have many adverse effects, it is necessary to confirm an accurate diagnosis and indications of treatments before starting the treatment. Recommendations for a diet should not hinder growth.

A casts of Barrett's esophagus complicated with stricture is reported. A 82 years old male was undergone distal esophagectomy and esophagogastrostomy on suspicion of malignancy. The microscopic examination of specimen taken from the lesion revealed the "tall columnar cells" which were indicative of Barrett's esophagus, hence the diagnosis. Barrett's esophagus is a rare disease in Korea. Because of the malignant potential of the disease, surgery involving esophagectomy is often required.
Aged, 80 and over ; Barrett Esophagus* ; Constriction, Pathologic ; Diagnosis ; Esophageal Stenosis* ; Esophagectomy ; Humans ; Korea ; Male ; Rare Diseases

No abstract available.
Female ; Humans ; Hypertension, Pregnancy-Induced* ; Pregnancy ; Pregnancy Trimester, Second*

Sotos syndrome (SS, OMIM 117550) is characterized by prenatal and postnatal overgrowth with multiple congenital anomalies. However, there have been few cases of growth retardation caused by renal failure from infancy. We report a case of dysplasia of the bilateral kidneys with renal failure and poor postnatal growth. A 2-month-old boy visited the emergency room owing to poor oral intake and abdominal distension. He was born at the gestational age of 38 weeks with a birth weight of 4,180 g. After birth, he had feeding difficulty and abdominal distension. Upon physical examination, his height and weight were in less than the 3rd percentile, while his head circumference was in the 50th percentile on the growth curve. He also showed a broad and protruding forehead and high hairline. Blood laboratory tests showed severe azotemia; emergent hemodialysis was needed. Abdominal ultrasonography revealed bilateral renal dysplasia with multiple cysts and diffuse bladder wall thickening. A posterior urethral valve was suggested based on vesicoureterography and abdominal magnetic resonance findings. Results of a colon study to rule out congenital megacolon did not reveal any specific findings. The conventional karyotype of the patient was 46, XY. Array comparative genomic hybridization study revealed a chromosome 5q35 microdeletion including the NSD1 gene, based on which SS was diagnosed. We describe a case of SS presenting with end stage renal disease due to posterior urethral valve. The typical somatic overgrowth of SS in the postnatal period was not observed due to chronic renal failure that started in the neonatal period.
Azotemia ; Birth Weight ; Colon ; Comparative Genomic Hybridization ; Databases, Genetic ; Emergency Service, Hospital ; Forehead ; Gestational Age ; Head ; Hirschsprung Disease ; Humans ; Infant ; Karyotype ; Kidney ; Kidney Failure, Chronic* ; Male ; Parturition ; Physical Examination ; Renal Dialysis ; Renal Insufficiency ; Sotos Syndrome* ; Ultrasonography ; Urinary Bladder

PURPOSE: The use of a 12-week steroid regimen (long-term therapy, LT) for the first episode of idiopathic nephrotic syndrome (NS) reportedly induces a more sustained remission and lower relapse rate than previous regimens, including an 8-week steroid regimen (short-term therapy, ST). Here, we assessed the potential for selective application of 2 steroid regimens (LT vs. ST) based on the days to remission (early responders [ER] vs. late responders [LR]) for the first idiopathic NS episode in children. METHODS: Patients were divided into 4 subgroups (ST+ER, ST+LR, LT+ER, and LT+LR) according to the initial steroid regimen used and rapidity of response; the baseline characteristics, relapse rates, and cumulative percentage of children with sustained remission were then compared among the 4 subgroups. RESULTS: Fifty-four children received ST, and the remaining 45 children received LT. As observed in previous studies, children receiving LT showed significantly lower relapse rates during the first year after the first NS episode than those receiving ST. The ST+ER group showed significantly lower relapse rates during the first one year and two years after the first NS episode than the the ST+LR group, whereas there were no significant differences of the relapse rates and duration to the first relapse between the ST+ER and LT+ER groups. CONCLUSION: We suggest that the initial steroid regimen in idiopathic NS patients can be shortened according to the duration to remission i.e., LT in patients achieving remission after the first week of steroid therapy, and ST in those achieving remission within the first week of steroid therapy.
Child ; Humans ; Nephrotic Syndrome* ; Recurrence ; Steroids