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*

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

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

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

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

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*

Recently the use of I-PSS (International prostate symptom score) is highly recommended for the evaluation of symptomatic benign prostatic hyperplasia (BPH) and many linguistic translations have been made. Regardless the validity and reliability of linguistic translation, there might be several social and cultural factors which affect to the symptom severity in BPH. We tried to show the prevalence of prostatism and the effect of social and cultural background which could affect to the results I-PSS questionnaire in Korea. A total of 841 men (40~79 years) who visited 4 health care center were included. Because of 107 men had medico-surgical history relating to voiding, final eligible subjects were 734. Total symptom scores increased by age decades (40~49, 50~59, 60~ 69, 70~79; median 7, 8, 11, 12 respectively). The rate of mild (0~7); moderate (8~19); and severe (20~35) symptom in 40th, 50th, 60th and 70th were 55.5%; 39.3%; 1.2%, 45.4%; 46.5%; 8.1%, 30.4%; 53.9%; 15.7% and 28.1%; 43.8%; 28.1% respectively. One cause of these high prevalence of prostatism in Korea is relatively very small proportion of readily treated BPH patients in general population (0.5%). Another factor of the high prevalence of prostatism is thought to be the different social and cultural concept to voiding status. These factors limit comparability of I-PSS questionnaire between different countries.
Delivery of Health Care ; Humans ; Korea* ; Linguistics ; Male ; Prevalence* ; Prostate ; Prostatic Hyperplasia ; Prostatism* ; Surveys and Questionnaires ; Reproducibility of Results ; Translations

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