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*

Ketotifen, a benzocycloheptathiophene, has an orally effective antiallergic as well as antihistaminic properties. In pervious studies, Ketotifen has shown encouraging results on patient with allergic rhinitis, either perennial or seasonal. 39 patients with allergic rhinitis had been treated with Ketotifen 1 mg twice daily for 8 weeks. And we obtained following results. 1) The efficacy rate in sneezing attack was 73.5%, in nasal discharge 71%, in nasal obstruction 58%. 2) Some improvements in at least one of three-major symptoms were noted within 1 week in 30.7%, within 2 weeks in 55.8%, within 3 weeks in 66.7%, within 8 weeks in 87.2%. 3) Physical findings such as colour, swelling of turbinate, character of rhinorrhea were not improved significantly. 4) Side effect was observed only in one patient with abdominal pain and diarrhea, which was subsided after interruption of administration. These results suggested that Ketotifen was effective in treatment of allergic rhinitis.
Abdominal Pain ; Diarrhea ; Humans ; Ketotifen* ; Nasal Obstruction ; Rhinitis, Allergic* ; Seasons ; Sneezing ; Turbinates

A case of invasive cribriform carcinoma of the breast is presented with fine need- le aspiration cytologic features. The aspiration was performed from a papable 2.0cm mass in the upper outer quadrant of the right breast in a 53 year-old woman. The aspirate showed cellular smear composed of larger three dimensional tight clusters, smaller monolayered loose clusters, and many individual cells on the clean background. In the clusters, the tumor cells were bordering central lumina, quite similar to the cribriform in histology. The tumor cells in the clusters and individual tumor cells had uniform, small and round nuclei. The chromatin was finely granular, and nuclear membrane was smooth. No discernible nucleoli were present (nuclear grade 1-2). Unless the abundance of individual cells and the cribriform growth pattern are recognized in the smear, the cytologic diagnosis of invasive cribriform carcinoma is difficult.
Female ; Humans ; Biopsy

So-called sclerosing hemangioma of the lung is a rare benign neoplasm which usually presents with a coin lesion detected through routine chest X-ray. We report two cases showing characteristic cytologic appearances which have been rarely reported. Both cases were young females with coin lesions in the lung. Fine needle aspiration of each case revealed unique but some different cytologic features. Case 1 showed bland-looking polygonal epithelial cells resembling bronchioloalveolar cells having pre- dominantly papillary configurations with loosely arranged solid sheets or isolated cells. Cytoplasms were plump, cyanophilic, and finely granular, with eccentric nuclei, The nuclei were usually monotonous, round-to-ovoid, and vesicular with a small but conspicuous nucleolus. In comparison to case 1, case 2 revealed largely loose pavement-like solid sheets or clusters rather than papillary patterns in the hemorrhagic background. The size of tumor cells were a little smaller than that of case 1. Bronchioloalveolar carcinoma and papillary adenocarcinoma of metastatic origin were considered to be one of the important differential diagnoses with these cytologic features. Histologically, both cases exhibited findings compatible with so-called sclerosing hemangioma of the lung.
Adenocarcinoma, Bronchiolo-Alveolar ; Adenocarcinoma, Papillary ; Biopsy, Fine-Needle ; Cytoplasm ; Diagnosis, Differential ; Epithelial Cells ; Female ; Humans ; Kidney* ; Lung ; Numismatics ; Pulmonary Sclerosing Hemangioma ; Renal Insufficiency* ; Thorax

Creutzfeldt-Jakob disease(CJD) is characterized clinically by rapidly progressive dementia with pyramidal, extrapyramidal, and cerebellar symptoms and signs, and histologically by spongiform change, neuronal loss and reactive gliosis. We have experienced 2 cases of CJD. Case 1 was a 36-year-old male who had suffered from myoclonus and cerebellar symptoms including sluggish speech, gait and balance disturbance. Case 2 was a 70-year-old female who had showed cognitive dysfunction, ataxic gait and disturbance of extraocular movement. Both patients, underwent brain biopsy. Case 1 revealed marked cortical atrophy, 2mm in thickness, with neuronal loss and astrocytic proliferation extending into white matter. The spongiform change, made up of many small, usually rounded or oval, vacuoles was noted mainly in the neuropil. Case 2 revealed remarkable spongiform change throughout the cortex and cytoplasmic vacuoles compressing the nuclei of neuronal cells were numerous. Neuronal loss and gliosis were also found without considerable change in the white matter. On double immunostaining against GFAP and PrP(Prion Protein), there was a weak positive reaction for PrP in the perinuclear cytoplasm in case 1, and a strongly positive reaction in case 2. The electron microscopic examination showed numerous membrane-bound vacuoles in neuropil and perikarya of neurons. The majority of the vacuoles were multiseptated by thin membranous structures. They demonstrated curled, or disrupted membrane, that had foldings and protrusions into the vacuolar clear spaces. There were neither identifiable virus-like particles nor amyloid deposition.
Female ; Male ; Humans

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