Pathological laughing and crying(PLC) is a condition that is characterized by episodic, brief, contextually inappropriate, uncontrollable outbursts of laughing and/or crying. It can be observed in patients with various neurological disorders. PLC often causes distress in interpersonal functioning and activities for patients and their families. PLC can be recognized easily with proper understanding of the condition and its nature. Also it generally shows good response to various pharmacological treatments. This review aims to encourage the diagnosis and treatment of PLC by providing definition and clinical presentation of PLC, analysis of its pathophysiology and various current treatment options.
Crying* ; Diagnosis ; Humans ; Nervous System Diseases

The complement system is known to be involved in the pathogenesis of the skin lesions in pernphigus vulgaris, bullous pemphigoid, dermatitis herpetiformis, epidermolysis bullosa acquisita, and systemic lupus erythematosus. Authors examined the skin specimens of each disease cases, who did not show any evidence of complement deficiency, to determine the deposition of complement components(C4, C3, Chb-9) and their inhibitors(C4bp, Factor H, S-protein) by modified direct immunofluorescence. We also looked at the staining pattern and localization, for further insights of their pathobiologic contributions in each disease. The findings of deposits of complement components up to C9, as well as inhibitor proteins at the primary histopathologic sites, in the majority of those cases, may indicate that the complement system, to certain extent, involves the inflamrnatory reactions in these diseases. The co-localization of C5b-9 and S-protein could be regarded as the consequence of in situ formation of SC5b-9 complexs or as the result of non-lytic adsorbed complexes of fluid phase SC5b-9. The pathologic role of the complement seems to depend mostly on the complement-fixing biologic property and the amount of the tissue bound immune complexes, which are often heterogeneous to different diseases and among different patients.
Antigen-Antibody Complex ; Complement Factor H ; Complement Membrane Attack Complex ; Complement System Proteins* ; Dermatitis Herpetiformis ; Epidermolysis Bullosa Acquisita ; Fluorescent Antibody Technique, Direct ; Humans ; Lupus Erythematosus, Systemic ; Pemphigoid, Bullous ; Skin ; Skin Diseases*

The purpose of this study w-s to predict the respose to the chincap therapy from the initial cephalometric measurements and to obtain the indication of chincap therapy. 40 patients selected for this study were classified into two groups by the occlusal stability after completion of permanent dentition and the iiu4n ovement of facial profile, after chincap therapy. One was g response group which consisted of 25 children and the other was poor response group with 15 patients. Various measurements of the craniofacial structure in the initial lateral cephalogram were calculated and analyzed by t-test and discriminant analysis. The results were, as follows 1. Good response group had more horizontal growth pattern in initial stage of treatment, and the contributing measurements were Bjork sum, anterior-posterior facial height ratio, gonial angle, lower gonial angle and occlusal plane to AB plane angle. 2. The critical points and predictive values of the influential skeletal measurements were calculated. 3. The discriminant function was obtained from three. major influential measurements; Bjork sum, gonial angle and occliml plane to AB plane angle, and this function could discreminate correctly in 85% of this samples.
Child ; Dental Occlusion ; Dentition, Permanent ; Humans

PURPOSE: The purpose of this study was to describe the clinical characteristics of neonatal urinary tract infection (UTI) caused by Klebsiella pneumoniae and non- Klebsiella pneumoniae UTI. METHODS: We compared clinical characteristics of 84 neonatal patients with UTI caused by Klebsiella pneumoniae who were hospitalized at the Department of Pediatricsat Han Dong University, Sunlin Hospital during the period between May, 1994 and August, 1998. The cases were divided into two groups depending upon causative organisms' Klebsiella pneumoniae UTI vs non-Klebsiella pneumoniae UTI, and the clinical characteristics of these groups were compared. RESULTS: Escherichia coli was the most common bacterial pathogen causing neonatal UTI, followed by Klebsiella pneumoniae. There was no significant difference in the sex distribution of Klebsiella pneumoniae UTI, but non-Klebsiella pneumoniae UTI showed male predominence. There were no significant differences in the incidences of hematologic, urologic, radiologic findings and perinatal complications in between these 2 groups. CONCLUSION: Klebsiella pneumoniae is the second most common pathogen causing neonatal UTI. There were no specific differences in the laboratory, symptomatologic, and radiologic findings in these two groups.
Escherichia coli ; Humans ; Incidence ; Klebsiella pneumoniae* ; Klebsiella* ; Male ; Pneumonia ; Sex Distribution ; Urinary Tract Infections* ; Urinary Tract*

The abnormalities in Hypothalamic-pituitary-adrenal(HPA) axis are associated with many psychiatric symptoms including depression. We present a report of a 71 year old man who was admitted to the psychiatric department presenting symptoms of headache, avolition, loss of energy, psychomotor retardation, poor appetite, insomnia, anxiety resulting from adrenal insufficiency and hypopituitarism. Hypothyroidism and electrolyte disturbance were managed and headache, insomnia, anxiety, GI symptoms were improved. But he remained in anergic state. After discharge, he was readmitted to infection department with high fever and drowsy mentality. Adrenal insufficiency was recognized and he was treated with corticosteroid replacement therapy. Finally his diagnosis was made as panhypopituitarism and overall symptoms were resolved. In this case, we showed how the atypical symptoms resulting from hypopituitarism develop and progress. Hypothyroidism, adrenal insufficiency, and growth hormone deficiency resulting secondarily from panhypopituitarism were associated with various nonspecific symptoms such as loss of energy, fatigue, insomnia, weight loss, decreased appetite etc. In clinical situation, differential diagnosis with depression is needed when clinicians were met a patient with these nonspecific symptoms. It is important that laboratory tests and differential diagnosis with endocrine diseases should be conducted, especially in geriatric patients with nonspecific symptoms like anergia, fatigue, poor appetite and so on.
Adrenal Insufficiency ; Anxiety ; Appetite ; Axis ; Depression ; Diagnosis, Differential ; Endocrine System Diseases ; Fatigue ; Fever ; Growth Hormone ; Headache ; Humans ; Hypopituitarism ; Hypothyroidism ; Sleep Initiation and Maintenance Disorders ; Weight Loss

No abstract available.
Dyskeratosis Congenita*

A fine needle aspiration biopsy specimen of a retroperitoneal mass in a 26-year-old man who had had an orchiectomy for seminoma was submitted for cytologic evaluation. Cytologic features of the specimen included uniform neoplastic cells found singly or in groups of several cells intermingled with lymphocytes in a characteristic foamy, lacelike background. These cells varied from 10 to 20 m in diameter. The nuclei were round to ovoid with fine or reticular chromatin and one or more prominent nucleoli. The poorly defined cytoplasm stained pale-blue or blue with cytoplasmic vacuoles. The cytologic appearance was consistent with seminoma. Documented reports of the cytological appearance of seminoma are rare. The diagnosis of primary gonadal seminoma by fine needle aspiration biopsy is probably not indicated since the treatment of a primary gonadal tumor, regardless of its histogenesis, requires surgical resection. However, fine needle aspiration biopsy is extremely valuable in the diagnosis of extragonadal as well as metastic and recurrent seminoma.
Adult ; Biopsy ; Biopsy, Fine-Needle ; Chromatin ; Cyclosporine* ; Cytoplasm ; Diagnosis ; Endothelin-1* ; Gonads ; Humans ; Liver ; Lymphocytes ; Nephrosis, Lipoid* ; Orchiectomy ; Plasma* ; Seminoma ; Vacuoles

Ulcerative colitis, one of the inflammatory bowel disease, is primarily managed medically with combinations of 5-ASA and steroids. However, this chronic disease requires surgical management if symptoms persist or complications develop despite medical management. The clinical course, the indications and outcome for surgical management of pediatric ulcerative colitis patients were studied from medical records retrospectively. Twenty-one patients under the age of 15 who were endoscopically diagnosed with ulcerative colitis at the Seoul National University Children's Hospital between January, 1988 and January, 2003 were subjected to the study. Mean follow up period was 3 years and 10 months. The mean age was 10.3 years old. All patients received medical management primarily after diagnosis and 8 patients (38%) eventually required surgical management. Of 13 patients who received medical management only, 7 patients (53%) showed remission, 4 patients are still on medical management, and 2 patients expired due to congenital immune deficiency and hepatic failure owing to sclerosing cholangitis respectively. In 8 patients who received surgical management, 1 patient underwent surgery due to sigmoid colon perforation and 7 patients due to intractability of medical management. The perforated case received colon segmental resection and the other 7 patients recieved total protocolectomy with ileal pouch-anal anastomosis. One patient expired postoperatively due to pneumonia and sepsis. One patient is still on medical management because of mild persistent hematochezia after surgery. The other operated patients are showing good prognosis without any management. Pediatric ulcerative colitis patients can be surgically managed if the patient is intractable to medical management or if complications such as perforation is present. Total protocolectomy & ileal pouch-anal anastomosis is thought to be the adequate surgical method.
Child* ; Cholangitis, Sclerosing ; Chronic Disease ; Colitis, Ulcerative* ; Colon ; Colon, Sigmoid ; Diagnosis ; Follow-Up Studies ; Gastrointestinal Hemorrhage ; Humans ; Inflammatory Bowel Diseases ; Liver Failure ; Medical Records ; Pneumonia ; Prognosis ; Retrospective Studies ; Seoul ; Sepsis ; Steroids ; Ulcer*

Chronic granulomatous disease (CGD) is an inherited immunodeficient disease characterized by recurrent infections and granuloma formation. Granulomatous obstruction of esophagus is one of the rare complications of CGD. The use of steroids and antimicrobials for esophageal obstruction by granuloma in CGD patients has been controversial due to the possibility of concomitant inapparent infection. We report a case of esophageal obstruction in an 8-year-old CGD patient showing the poor response to antibiotics therapy. However, dramatic improvement of symptoms and radiologic findings of esophageal obstruction were achieved after steroid therapy. One month after discontinuation of steroid, esophageal obstruction recurred and the patient was re-treated with steroid. After that time, he experienced one more recurrence of esophageal obstruction. This symptom subsided after antibiotics therapy without steroid and he has been followed up to the present without further relapse.
Anti-Bacterial Agents ; Child ; Esophageal Stenosis ; Esophagus ; Granuloma ; Granulomatous Disease, Chronic* ; Humans ; Recurrence ; Steroids

No abstract available.
Gram-Positive Bacterial Infections* ; Teicoplanin*