Trichorhinophalangeal syndiome, a rare genetic disease, is characterzed by the triad of slow growing, brittle hair with early loss, a pear-shaped nose with bulbous tip and long philtrum, and coneshaped phalangeal epiphyses wiith resultant shortening and. deformity of hands and feet. A 24-year-old female visited our department for the evaluation of lalopecia. She had had sparse, thin, and brittle hair since birth. She also complained of short fingers ar d a pear-shaped nose with bulbous tip. The X-ray findings of her hands and feet showed cone-shaped epiphyses with shortening of the phalangeal bones. There was no family history of hair, nasal, or palnkoplantar phalangeal abnormality. Chromosomal study did not reveel any abnormal finding. Shortened phalangeal bones of her index and middle fingers were slightly improved by lengthening procedure with iIlizarov apparatus.
Congenital Abnormalities ; Epiphyses ; Female ; Fingers ; Foot ; Hair ; Hand ; Humans ; Lip ; Nose ; Parturition ; Young Adult

No abstract available.
Immunoglobulin M* ; Lymphoma* ; Paraproteinemias*

PURPOSE: This report outlines the incidence and the clinical features of patients with a neuroendocrine (NE) neoplasm of the colon and rectum and describes, in detail, their histologic and immunohistochemical findings. Also, we attempted to determine the impact of several clinical variables, including tumor stage, tumor location, NE pattern, and cellular subtype on survival. METHOD: Of 690 colorectal cancers operated on from April 1990 to November 1998 at Chungnam National University Hospital, 41 cases were originally diagnosed as poorly differentiated adenocarcinoms on the basis of conventional light microscopy. Paraffin blocks from the aforementioned cases were retrieved, and sections were im munostained with antibodies to human chromogranin A, neuron specific enolase, and synaptophysin. RESULTS: Of the 690 cases of colorectal caner, 35 cases (5.1%) of NE neoplasm were identified retrospectively: 28 males and 7 females. About 90% of the tumors were located at the cecum and the rectosigmoid. Pathologic stages were as follows: modified Dukes stage B2, 6; stage C1, 8; stage D1, 12; and stage D2, 9. The most common metastatic site at the time of diagnosis was the liver (8/9). Four NE patterns were identified: pure NE (n=4), and predominantly NE (n=8), equal NE and exocrine (n=4), and predominantly exocrine (n=19). Two cellular subtypes were identified: well-differentiated (n=3) and intermediate (n=32) cells. Survival statistically correlated with stage (p=0.03), but not with age, sex, tumor location, NE pattern, or cellular subtype. CONCLUSION: This study suggests that NE neoplasms of the colon and rectum are more frequent than previously believed. Since a NE neoplasm is believed to be an extremely biologically aggressive tumor, recognition of a NE neoplasm is very important because of its evident clinical and therapeutic implications.
Antibodies ; Cecum ; Chromogranin A ; Chungcheongnam-do ; Colon* ; Colorectal Neoplasms ; Diagnosis ; Female ; Humans ; Immunohistochemistry ; Incidence ; Liver ; Male ; Microscopy ; Paraffin ; Phosphopyruvate Hydratase ; Rectum* ; Retrospective Studies ; Synaptophysin

Isolated ACTH deficiency is a rare disorder, and usually characterized by its chronic course. The 59 year-old woman patient who had been healthy until 2 months ago, admitted because of abdomial pain, general weakness and loss of weight about 12kg for 2 months. She looked a little pallor but color of skin was not remarkable. Blood pressure, serum electrolyte, and glucose on admission were within normal range. Serum calcium was elevated with the value of 12.6mg/dL, which was normalized after hydration. Cortisol response to RI induced hypoglycemia did not show any response. Anterior pituitary hormone except ACTH showed normal response during combined pituitary hormone stimulation test. These clinical and laboratory finding reveals that isolated ACTH deficiency was developed in a short term period. There were no abnormalities in sellar MRI except pineal cyst. Her complaints were disappeared dramatically after hydroccetisone replacement at 4th. hospital day. Here we report a case of isolated ACTH deficiency, which was rapidly developed, with hypercalcemia, abdominal pain, and loss of weight about 12kg for 2 months.
Abdominal Pain ; Adrenocorticotropic Hormone* ; Blood Pressure ; Calcium ; Female ; Glucose ; Humans ; Hydrocortisone ; Hypercalcemia ; Hypoglycemia ; Magnetic Resonance Imaging ; Middle Aged ; Pallor ; Reference Values ; Skin ; Weight Loss

Benign prostatic hyperplasia and urethral stricture are common causes of urethral obstruction over 50 years of age. To evaluate the usefulness of the Gianturco expandable stent for dilatation of the urethra, 14 single stents were placed in the posterior urethra (2 in prostatic urethra, 12 in membranous urethra), 20 in the anterior urethra. And 4 six-eight connected stents ere positioned in the posterior urethra to straddle at the internal sphincter or external sphincter and followed up for 2 weeks (one dog), 1 month (two dog), 2 months (one dog), 3 months (one dog), 5 months (one dog), 9 months (three dogs), and 14 months (two dogs). Seven of 18 stents in the posterior urethra and 5 to 20 in the anterior urethra were migrated during the follow-up. By 2 weeks after placement. 100% expansion was achieved in the posterior urethra, but by 1 month in the anterior urethra. Partial or complete epithelial covering of the stents was observed 1 month after stent placement in the anterior urethra, but mucosal folds were observed in the anterior and posterior urethra. Urinary incontinence was not observed in all cases. Our experience suggests that Gianturco expandable stent can be used in dilating and maintaining the lumen of the prostatic urethra and urethral stricture.
Animals ; Dilatation ; Dogs* ; Follow-Up Studies ; Prostatic Hyperplasia ; Stents* ; Urethra* ; Urethral Obstruction ; Urethral Stricture ; Urinary Incontinence

Preoperative uterine artery embolization and cervical evacuation as conservative management of cervical pregnancy has been tried in recent years. However, cervical suturing, vasoconstrictor injection, or cervical ballooning was frequently used as an ancillary measures in those procedures in most of the previous studies. We report two cases of cervical pregnancy that were successfully treated with preoperative uterine artery embolization and removal of gestational material without ancillary procedures. Our therapeutic modality seems to be safe and effective for conservative management of cervical pregnancy.
Adult ; Case Report ; Cervix Uteri ; Dilatation and Curettage ; *Embolization, Therapeutic ; Female ; Human ; Pregnancy ; Pregnancy, Ectopic/*therapy/ultrasonography ; Preoperative Care ; Uterine Hemorrhage/prevention & control ; Uterus/*blood supply

Anaphylaxis is a life-threatening, systemic reaction manifested by urticaria, angioedema, bronchospasm, syncope and hypotension, which occurs after the exposure to specific antigen such as antibiotics, aspirin, nonsteroidal antiinflammatory drugs, foods, bee sting, or exercise. A 33-year-old woman suffering from anaphylactoid attack after intramuscular administration of ribostamycin at private clinic, was visited to our emergency room and admitted. She had allergic rhinitis and her 8-year-old daughter had atopic dermatitis. She had three previous intramuscular administrations of ribostamycin at every three weeks due to recurrent pelvic inflammatory disease. At third time of ribostamycin administration, she had experienced generalized pruritus, flushing and shortness of breath but these symptoms were resolved spontaneously without medication. Therefore, she didn't notice to attending physician. During the fourth administration of ribostamycin, she developed generalized urticaria and angioedema with dyspnea, dizziness, and severe hypotension. We experienced a case of ribostamycin-induced anaphylaxis, which is rarely mentioned in published articles. We performed a skin prick test and an intradermal test to ribostamycin, which were positive, and report a case of ribostamycin-induced anaphyalxis.
Adult ; Anaphylaxis* ; Angioedema ; Anti-Bacterial Agents ; Aspirin ; Bees ; Bites and Stings ; Bronchial Spasm ; Child ; Dermatitis, Atopic ; Dizziness ; Dyspnea ; Emergency Service, Hospital ; Female ; Flushing ; Humans ; Hypotension ; Intradermal Tests ; Nuclear Family ; Pelvic Inflammatory Disease ; Pruritus ; Rhinitis ; Ribostamycin ; Skin ; Syncope ; Urticaria

Herpes simplex virus esophagitis and candidal esophagitis can be found in immunocompromised patients. But it rarely occurs in immunocompetent patients. Diagnosis is usually established with an upper gastrointestinal endoscopy. The lesions of herpes simplex virus esophagitis involves the squamous mucosa, where the earlist manifestation is a vesicle, rarely. We report a case of herpes simplex virus esophagitis who, being immunologically healthy, presented with substernal pain and dysphagia and showed vesicle formation on endoscopy. The patient also showed whitish patch and spot on esophageal mucosae. The patient showed loss of symptom and improvement of endoscopic finding after conservative management and antifungal therapy.
Deglutition Disorders ; Diagnosis ; Endoscopy ; Endoscopy, Gastrointestinal ; Esophagitis* ; Herpes Simplex* ; Humans ; Immunocompromised Host ; Mucous Membrane ; Simplexvirus*

BACKGROUND: It is widely believed that Graves' disease is an autoimmune disorder characterized by the presence of the circulating TSH receptor antibody (TRAb). The majority of the activity of TRAb is of the immunoglobulin G (IgG) class. However, other immunoglobulin such as immunoglobulin E (IgE), may play a role in the activity. IgE accumulation has been reported to occur in the thyroid gland and ocular muscles of subjects with Graves' disease. Furthermore, it has been noted that recurrence of Graves' disease can be induced by an allergy to pollen. Because an allergy to pollen is commonly associated with IgE, IgE might play a role in the induction of Graves' disease. Therefore, investigated whether IgE was elevated in Graves' disease, and evaluated the potential relationship between the levels of TRAb and IgE Graves' disease. METHODS: Forty-six patients with Graves' disease, and 6 with chronic thyroiditis, diagnosed at the Kosin Medical Center between April, 2000 and July, 2000 were included in this study. Thirty-five persons without thyroid disease or a history of allergic rhinitis were used as normal controls. The level of TRAb was measured using thyrotropin binding inhibitory immunoglobulin (TBII). Serum total IgE was measured using an enzymeimmunoassay method. Test for thyroid function, TBII and total IgE were performed in all cases, and the results statistically analyzed. RESULTS: TBII, as IgG, and the serum IgE level were higher in the patients with Graves' disease, and the levels of the latter were 598.1+/-1112.9U/mL, 98.5+/-79.7U/mL and controls 161.7+/-194.4U/mL in the Graves' patients, those with thyroiditis and the controls, respectively (p<0.05). The prevalence of allergic rhinitis in Graves' disease was 10.9%. The serum IgE level in Graves' disease with, and without, allergic rhinitis were 903.1+/-1152.2U/mL and 560.8+/-1117.0U/mL, respectively, although there was no significancant difference between the two groups. According to the clinical stage, the serum TBII level was higher in the untreated Graves', and relapsed patients 49.9+/-23.9% and 21.1+/-3.1%, respectively, than in the treated group, 7.4+/-18.6% (p<0.05). The serum IgE level was higher in the untreated Graves' and relapsed patients 758.6+/-1250.2U/mL and 1198.5+/-1952.1U/mL, respectively, than in the treated group 233.8+/-432.7U/mL, although this was not significant. According to the duration of treatment, the serum TBII levels were higher in the untreated Graves' patients, and those treated for less than 1 year, than in those treated for more than 1 year, with values of 49.9+/-23.9, 24.8+/-3.8 and 2.22+/-1.97%, respectively (p<0.05). The serum IgE level was higher in the untreated Graves' disease (758.6+/-1250.2U/mL) than in the groups treated for less than 12 months (158.3+/-91.5U/mL) and more than 12 months (252.7+/-483.4U/mL), but the differences were not significant. CONCLUSIONS: The concentration of IgE was high in Graves' patients, and although not statistically significant, the serum IgE level in Graves' patients with allergic rhinitis was higher than those without. With regard to the clinical stage of Graves' disease, the change in the IgE level tended to follow that of the TBII. Further study will be required to define the possible role of IgE in the pathogenesis in Graves' disease.
Graves Disease* ; Humans ; Hypersensitivity ; Immunoglobulin E ; Immunoglobulin G ; Immunoglobulins ; Muscles ; Pollen ; Prevalence ; Receptors, Thyrotropin ; Recurrence ; Rhinitis ; Thyroid Diseases ; Thyroid Gland ; Thyroiditis ; Thyrotropin

The incidence of a pulmonary leiomyosarcoma as a primary lung tumor is quite rare. We report a case of primary leiomyosarcoma with a cardiac invasion in a 76 year old man. He was admitted due to left anterior chest wall pain for one month. Chest computed tomography showed a 9x8x10cm sized , large round mass in the left upper and lower lobes, and an amorphous low density lesion within the left atrium. Chest magnetic resonance imaging showed a large round mass in the left upper and lower lobes with growth into the left atrium. A diagnosis of leiomyosarcoma with prominent osteoclast-like giant cells was made based on the microscopic and immunohistochemical findings of a permanent specimen by explothoracotomy. The pathologic features of the tumor showed round mononuclear hyperchromatic cells and multinucleated giant cells that resembled osteoclasts. The immunohistochemical staining showed that the giant cells are positive for CD68 but negative for the muscle markers while the round cells were positive for the muscle marker. The patient refused further treatment and died after two months.
Aged ; Diagnosis ; Giant Cells* ; Heart Atria ; Humans ; Incidence ; Leiomyosarcoma* ; Lung* ; Magnetic Resonance Imaging ; Osteoclasts ; Thoracic Wall ; Thorax