Objective: To observe the effect of heat shock on tropoelastin mRNA and protein expression in cultured human dermal fibroblasts and to elucidate the possible role of heat shock in the development of solar elastosis in human skin. Methods: Primary cultured human dermal fibroblasts were incubated in 43 ℃ water bath for 30 minutes, then returned to 37 ℃, 5%(volume fraction) CO 2 atmosphere normal culture condition. The cells were harvested at hour 24 and 48 after heat treatment respectively. Total RNA was extracted from cell lysis and tropoelastin mRNA level was measured by Northern blot assay. Tropoelastin protein level in cultured supernatant was measured by Western blot assay. Results: At hour 24 after heat treatment, the level of tropoelastin mRNA was enhanced to (163?12)% of control group (P

Telomerase is an RNA-dependent DNA polymerase that synthesizes TTAGGG telomeric DNA onto chromosomal ends to compensate for sequence loss during replication. It has been detected in a variety of human malignancies, suggesting that such activity may play a role in the tumorigenic process. To determine whether telomerase is reactivated in malignant fibrous histiocytoma, 12 tissue samples with this tumor were analyzed for the telomerase activity by a radioactive PCR-based TRAP (telomeric repeat amplification protocol) assay. All of the tumors were further investigated for the expression of human telomerase RNA (hTR) by an in situ hybridization (ISH). Telomerase activity was detected in one (8.3%) sample. Expression of hTR was demonstrated in 7 (58.3%): one telomerase-positive and six telomerase-negatives. These data indicate that the reactivation of telomerase is an uncommon event and not an important factor involved in tumorigenesis in malignant fibrous histiocytoma. It is noteworthy that 50% of the patients with grade 2 tumors expressed hTR, suggesting that telomerase RNA may be useful as a marker for identifying tumor aggressiveness earlier than the conventional histopathologic grading scale.
Carcinogenesis ; DNA ; Histiocytoma, Malignant Fibrous* ; Humans ; In Situ Hybridization ; RNA* ; RNA-Directed DNA Polymerase ; Telomerase*

We experienced a case of a 4-year-old girl presenting with vomiting, abdominal pain and mobile mass in the periumbilical area. On physical examination, the abdomen was soft and a 10x10 cm sized, painless firm mobile round mass was palpated in the periumbilical area. An abdominopelvic computed tomography scan showed a huge hypodense mass with encapsulation and multiple septation in the left lower quadrant of the abdomen, compressing the intestinal loops. At operative exploration, she was found to have a soft, yellowish round mass originating from the ileal mesentery, situated 10 cm proximal to the ileocecal valve. The mass was completely excised together with the adjoining ileum, and bowel continuity was restored. Histopathologic diagnosis revealed a mesenteric lipoma, composed of mature adipocytes with no evidence of necrosis or malignancy. The postoperative course was uneventful.
Abdomen ; Abdominal Pain ; Adipocytes ; Child ; Humans ; Ileocecal Valve ; Ileum ; Lipoma ; Mesentery ; Necrosis ; Physical Examination ; Preschool Child ; Vomiting

PURPOSE: To compare the efficacy of a 20-gauge and an 18-gauge needle in sono-guided percutaneous automated gun biopsy for establishing the specific diagnosis of renal parenchymal disease in pediatric kidneys. MATERIALS AND METHODS: In 60 pediatric patients with renal parenchymal diseases, percutaneous sono-guided gun biopsy was performed by an experienced radiologist. In two groups of 30 patients, regardless of their age, two needle passes were performed, using alternately an 18-gauge or a 20-gauge biopsy needle. The core of renal tissue thus obtained was examined with light, immunofluorescent or electron microscopy by the renal pathologist. The mean number of intact glomeruli of whole tissue core per biopsy, as seen on the light microscopy, and post-bioptic complications were compared between the two different needle size groups. RESULTS: The number (mean+/-1 standard deviation) ofglomeruli obtained per biopsy was 17+/-8 in the 18-gauge needle group, and 14+/-5 in the 20-gauge group. Between two groups, there was no major post-bioptic complication requiring specific treatment, nor a statistically significant difference in the frequency of minor complications. CONCLUSION: Even though more glomeruli were obtained with an 18-gauge needle, the number obtained with a 20-gauge needle also permitted adequate pathologic examination. Both an 18-gauge and a 20-gauge needle may thus be suitable for renal biopsy in pediatric patients.
Biopsy* ; Diagnosis ; Humans ; Kidney ; Microscopy ; Microscopy, Electron ; Needles*

Transcatheter closure of atrial septal defects has become a popular procedure. The availability of a preprocedural imaging study is crucial for a safe and successful closure. Both the anatomy and morphology of the defect should be precisely evaluated before the procedure. Three-dimensional (3D) echocardiography and cardiac computed tomography are helpful for understanding the morphology of a defect, which is important because different defect morphologies could variously impact the results. During the procedure, real-time 3D echocardiography can be used to guide an accurate closure. The safety and efficiency of transcatheter closures of atrial septal defects could be improved through the use of detailed imaging studies.
Echocardiography ; Echocardiography, Three-Dimensional ; Heart Septal Defects, Atrial* ; Tomography Scanners, X-Ray Computed ; Vascular Access Devices

Four children with solid and papillary epithelial neoplasm of the pancreas are reported. Three were girls. Mean age at operation was 12 years and 7 months (10-13 years). Clinical presentation included nausea, vomiting, and apalpable mass. One had hemoperitoneum due to tumor rupture. In two cases, tumors were in the body of the pancreas, and one the body and tail, and in one,the tail. Mean diameter of the tumors was 10.8 cm (8-15cm). Surgical procedures were distal pancreatectomy and splenectomy in 2 cases, distal pancreatectomy in one, and subtotal pancreatectomy and splenectomy in one. Mean follow-up period was 61 months (6-121 months). Three patients are still alive without any recurrence. However, in the one case of ruptured tumor, portal vein thrombosis and liver metastasis developed after subtotal pancreatectomy and splenectomy during the course of postoperative adjuvant chemotherapy.
Chemotherapy, Adjuvant ; Child* ; Drug Therapy ; Female ; Follow-Up Studies ; Hemoperitoneum ; Humans ; Liver ; Nausea ; Neoplasm Metastasis ; Neoplasms, Glandular and Epithelial* ; Pancreas* ; Pancreatectomy ; Recurrence ; Rupture ; Splenectomy ; Venous Thrombosis ; Vomiting

Four children with solid and papillary epithelial neoplasm of the pancreas are reported. Three were girls. Mean age at operation was 12 years and 7 months (10-13 years). Clinical presentation included nausea, vomiting, and apalpable mass. One had hemoperitoneum due to tumor rupture. In two cases, tumors were in the body of the pancreas, and one the body and tail, and in one,the tail. Mean diameter of the tumors was 10.8 cm (8-15cm). Surgical procedures were distal pancreatectomy and splenectomy in 2 cases, distal pancreatectomy in one, and subtotal pancreatectomy and splenectomy in one. Mean follow-up period was 61 months (6-121 months). Three patients are still alive without any recurrence. However, in the one case of ruptured tumor, portal vein thrombosis and liver metastasis developed after subtotal pancreatectomy and splenectomy during the course of postoperative adjuvant chemotherapy.
Chemotherapy, Adjuvant ; Child* ; Drug Therapy ; Female ; Follow-Up Studies ; Hemoperitoneum ; Humans ; Liver ; Nausea ; Neoplasm Metastasis ; Neoplasms, Glandular and Epithelial* ; Pancreas* ; Pancreatectomy ; Recurrence ; Rupture ; Splenectomy ; Venous Thrombosis ; Vomiting

Linear nevus sebaceous syndrome is an uncommon neurocutaneous disorder characterized by nevus sebaceus, seizures, and mental retardation, and is often accompanied by ocular anormalies. We recently experienced a case of conjunctival choristoma associated with linear nevus sebaceous syndrome in a 17-month-old boy. The child was the product of a full-term uncomplicated pregnancy and normal delivery with normal karyotype of 46, XY. He was noted at birth to have a conjunctival mass on the right eye and linear nevi over the midline facial region and right temporoparietal scalp. Ophthalmologic examination revealed optic atrophy, peripapillary staphyloma, macular hypogenesis, and osseous choristoma of the choroid. A computed tomography scan demonstrated widening of the subarachnoid space and moderate atrophy of the cerebral and cerebellar hemispheres. He has been followed by ophthalmology and completely free of seizures since birth. The patient underwent excision of the conjunctival mass and biopsies of the skin lesion at 17 months of age. Histologic examination of the skin biopsies was characteristic for the linear sebaceous nevus of Jadassohn. Histologic findings of the conjunctival lesion showed a complex choristoma that contained lacrimal gland tissue and cartilage.
Atrophy ; Biopsy ; Cartilage ; Child ; Choristoma* ; Choroid ; Humans ; Infant ; Intellectual Disability ; Karyotype ; Lacrimal Apparatus ; Male ; Neurocutaneous Syndromes ; Nevus* ; Nevus, Sebaceous of Jadassohn ; Ophthalmology ; Optic Atrophy ; Parturition ; Pregnancy ; Scalp ; Seizures ; Skin ; Subarachnoid Space

Spontaneous gastric perforation in the newborn is a rare disease that requires early diagnosis and prompt surgical treatment. Between 1988 and 2001 at the Department of Pediatric Surgery, Kyungpook National University Hospital, 9 cases of spontaneous gastric perforation were treated. Seven were males and two females. The mean gestational age and birth weight were 36.7 weeks and 2,455 grespectively. All patients presented with severe abdominal distention and pneumoperitoneum on cross table lateral film of the abdomen. Perforations were located on the anterior wall along the greater curvature of the stomach in six and on the posterior wall along the greater curvature in two. One case showed two sites of perforation on the anterior and posterior wall along the greater curvature. Six patients were managed with debridement and primary closure and the others with debridement and partial gastrectomy. Peritoneal drainage was not performed. There were four deaths; two from sepsis due to leakage from the anastomotic site, one as a result of acute renal failure, and the other by associated respiratory distress syndrome. Spontaneous gastric perforation in the newborn is usually located along the greater curvature. Elevated intragastric pressure is a possible cause of the perforation. Poor prognosis is related to associated diseases and prematurity.
Abdomen ; Acute Kidney Injury ; Birth Weight ; Debridement ; Drainage ; Early Diagnosis ; Female ; Gastrectomy ; Gestational Age ; Gyeongsangbuk-do ; Humans ; Infant, Newborn* ; Male ; Pneumoperitoneum ; Prognosis ; Rare Diseases ; Sepsis ; Stomach

There have been a few reports of familial anorectal malformations extending over more than one generation. We experienced a case of a family with 3 members spanning 2 generations affected with isolated low type anorectal malformations. They had same low type of anorectal malformations. In all 3 patients, a perianal anoplasty was performed.
Family Characteristics ; Humans