Two cases of Kartageneranjx syndrome were presented. They were sisters in the same family and were 15 years and 13 years old respectively. Chief complaints of the first case were chronic cough and expectoration and of the second case were chronic cough, expectoration and nasal stuffiness. Situs inversus, bronchiectasis and paranasal sinusitis were identified in both cases. EKG showed typical findings of mirror image compatible with dextrocardia in all two cases. Administration of antibiotics and postural drainage were performed with good resultin the first case but no remarkable improvement in the second case. A brief review of literatures was made.
Adolescent ; Anti-Bacterial Agents ; Bronchiectasis ; Cough ; Dextrocardia ; Drainage, Postural ; Electrocardiography ; Humans ; Siblings* ; Sinusitis ; Situs Inversus

No abstract available.
Urinary Bladder*

A heterotopic or ectopic pancreas is defined as the presence of pancreatic tissue that lacks anatomic and vascular continuity with the main body of the pancreas. Pancreatic heterotopia is characterized by the presence of pancreatic acinar, islet, and ductular elements. Most examples are incidentally encountered during surgery, and, on rare occasions, epigastric pain, weight loss, hemorrhage, gastric outlet obstruction, and intussusception have been directly attributable to the presence of the heterotopic pancreas. A malignant transformation in an ectopic pancreas is exceptional and intestinal obstruction of the small bowel by an adenocarcinoma arising in an ectopic pancreas has not been reported yet. We present the case of a ductal adenocarcinoma originating in the heterotopic pancreas in the jejunum of a 73-year-old man. The tumor was found to obstruct the jejunum at a distance of about 25 cm distal to the ligament of Treitz. The resected jejunum histologically showed a ductal adenocarcinoma that originated from the ectopic pancreas. To our knowledge, this would be the first case in Korea, which the patient presented with a small bowel obstruction due to a malignant transformation of an ectopic pancreas.
Adenocarcinoma* ; Aged ; Gastric Outlet Obstruction ; Hemorrhage ; Humans ; Intestinal Obstruction ; Intussusception ; Jejunum* ; Korea ; Ligaments ; Pancreas* ; Weight Loss

No abstract available.
Antibodies, Antineutrophil Cytoplasmic* ; Wegener Granulomatosis*

Neurofibromatosis, first clearly described by von Recklinghausen in 1882, is a dominantly inherited mesodermal and ectodermal dysplasia with a broad spectrum of clinical findings. Most common is the classical neurofibromatosis, which has three major features: (1) multiple neural tumors dispersed anywhere on or in the body; (2) numerous pigmented skin lesions, some of which are "cafe au lait" spots; and (3) pigmented iris hamartomas also called Lisch nodules. Other lesions sometimes seen in patients with von Recklinghausen's disease include congenital malformations of various types, vascular lesions, neurilemoma meningioma and other intracranial neoplasms, pheochromocytoma, medullary carcinoma of thyroid gland, neuroblastoma, ganglioneuroma and Wilms' tumor. Approximately 10% of the patients with neurofibromatosis have the gastrointestinal tract tumors as neurofibroma, ganglioneuroma and leiomyoma. Some of them show evidence of malignancy. Increased activity of the protein "nerve growth factor" in the sera of the patients with disseminated neurofibromatosis has been reported. We would like to report here two cases of von Recklinghausen's neurofibromatosis with involvement of the gastrointestinal tract.
Hamartoma ; Meningioma

BACKGROUND: The central physiological derangement of hemorrhagic fever with renal syndrome (HFRS) caused by hantaan virus (HTNV) is a vascular dysfunction, manifested by hemorrhage, impaired vascular tone and increased vascular permeability. Platelet activating factor (PAF), whose actions are mediated through a specific receptor, is a potent bioactive lipid. PAF has diverse biological functions in the vascular system, such as increasing vascular permeability, adhesion of leukocytes to the endothelium and reduction of cardiac output, which result in hypotension and shock. The goal of the present study was to investigate whether PAF is involved in the pathogenesis of HFRS. For this purpose, we evaluated the effect of HTNV on the expression of PAF receptor (PAF-R) and on the activity of PAF-acetylhydrolase (PAF-AH) instead of PAF because PAF is rapidly degraded by PAF-AH in vivo. MATERIALS AND METHODS: To evaluate the expression of PAF-R, we performed reverse-transcription PCR, western blot and FACS analyses using HTNV-infected human umbilical vein endothelial cells (HUVECs) and non-infected (control) HUVECs. In addition, we measured the activity of plasma PAF-AH in HFRS patients and normal healthy persons. RESULTS: The mRNA and protein expression of PAF-R was increased in HTNV-infected HUVECs compared with control HUVECs at 2 and 3 days post-infection (d.p.i.). FACS analysis showed that HTNV induced the surface expression of PAF-R in HUVECs from 2 d.p.i. The activity of plasma PAF-AH was 2.5-fold lower in HFRS patients than in normal healthy persons. CONCLUSION: Increased PAF-R expression by HTNV might increase the responsiveness to PAF in endothelial cells. Reduced PAF-AH activity in the blood of HFRS patients might delay PAF degradation. These results suggest that changes in PAF-R and PAF-AH by HTNV might influence to PAF activity and might be involved in the vascular dysfunction of HFRS.
Blood Platelets ; Blotting, Western ; Capillary Permeability ; Cardiac Output ; Endothelial Cells ; Endothelium ; Hantaan virus ; Hemorrhage ; Hemorrhagic Fever with Renal Syndrome ; Human Umbilical Vein Endothelial Cells ; Humans ; Hypotension ; Leukocytes ; Plasma ; Platelet Activating Factor ; Platelet Membrane Glycoproteins ; Polymerase Chain Reaction ; Receptors, G-Protein-Coupled ; RNA, Messenger ; Shock

No abstract available.
Carcinoma, Transitional Cell* ; Diagnosis* ; Kidney*

Endodermal sinus tumor of the mediastinum is a very rare malignant tumor, usually affecting young adolescents, and its histologic findings are characteristic as that of gonadal germ cell origin. We describe the cytologic finding of fine needle aspiration of mediastinal endodermal sinus tumor in a 19-year-old male patient, comparing with tissue section. The tumor cells in smears were usually in tight clusters with large overlapping cells, which were arranged in a papillary or microacinar pattern, suggesting carcinoma. The tumor cells were large, round or oval with a small amount of cytoplasm which was occasionally vacuolated and had indistinct border. The nuclei were large, pleomorphic, and vesicular with large promi- nent nucleoli. The tissue sections showed typical findings of endodermal sinus tumor. Careful correlation of cytological findings and the serum alpha-fetoprotein level would be helpful to confirm the diagnosis.
Adolescent ; alpha-Fetoproteins ; Biopsy, Fine-Needle ; Biopsy, Needle ; Carcinoma, Papillary ; Carcinoma, Squamous Cell ; Child* ; Clonorchiasis ; Cytoplasm ; Diagnosis ; Dietary Proteins* ; Endodermal Sinus Tumor ; Female ; Germ Cells ; Giant Cells ; Gonads ; Humans ; Kidney Failure, Chronic* ; Male ; Mediastinum ; Middle Aged ; Thyroid Gland ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux* ; Young Adult

Background: It has been reported that steroid and lidocaine reduce inflammatory responses to endotoxin. The aim of this study is to compare the effects of lidocaine with those of steroid on inflammatory responses to Escherichia coli endotoxemia in the rabbit lung. Methods: Twenty four animals were randomly assigned to one of four groups. Group I (endotoxemic group; control, n=6): They were received E. coli endotoxin (500 microg/kg) intravenously through the ear vein and followed by saline infusion at 20 ml/kg/hr for 4 hours. Group II (steroid treated group, n=6): They were treated with steroid (30 mg/kg) intravenously just after endotoxin injection and then saline was given at 20 ml/kg/hr for 4 hours. Group III (lidocaine treated group, n=6): They were received same as the endotoxemic group and lidocaine (3 mg/kg IV bolus, then continuous infusion at the rate of 2 mg/kg/hr) was administered with saline at 20 ml/kg/hr for 4 hours. Group IV (steroid & lidocaine treated group, n=6): They were received same as the endotoxemic group and after endotoxin infusion, steroid (30 mg/kg) and lidocaine (3 mg/kg IV bolus, then continuous infusion at the rate of 2 mg/kg/hr) was administered intravenously with saline. Numbers of neutrophils and monocytes in the samples of peripheral blood and lung tissue were counted and compared to evaluate the anti-inflammatory effect of drugs. Results: The group II, III, and IV showed significant attenuation of inflammatory responses compared to group I in the rabbit lung(P<0.05). But there were no significant differences among group II, III, and IV. There was no additive effect between steroid and lidocaine. Conclusion: These results suggest that lidocaine could be used as an alternative drug to steroid for its anti-inflammatory effects and cost-effectiveness.
Animals ; Ear ; Endotoxemia* ; Escherichia coli ; Lidocaine* ; Lung* ; Monocytes ; Neutrophils ; Veins

No abstract available.
Liver* ; Neoplasm Metastasis*