No abstract available.
Child* ; Humans ; Infant* ; Intussusception*

PURPOSE:Noonan syndrome(NS) is characterized by short stature, congenital heart disease, and typical facies. Recombinant human growth hormone(GH) has been reported to improve growth rate in a similar fashion to that seen in Turner syndrome. We investigated the clinical characteristics and growth reponses to GH therapy in children with NS. METHODS:The cases of sixty seven patients with NS were reviewed retrospectively. Ten of the 65 patients were assessed height, weight and pubertal stage every 3 months during GH therapy. RESULTS:Webbed neck(70%), delayed development(59.7%), low set posterior hairline(56.7%), eye abnormalities(56.7%) and mental retardation(55.2%) were the leading clinical characteristics. Short stature below the 3rd percentile was presented in 73.8 %. Growth patterns in NS children were variable and the evaluation of their growth must be individualized. The increments of height SDS were significant in children with GH therapy(height SDS:from -2.8+/-.6 to -2.3+/-.9, growth velocity:from 4.4+/-.8 cm to 9.2+/-.9 cm during first year, and 6.1+/-.1 cm during second year) (P<0.05). CONCLUSION: This study characterized the clinical profiles in Korean children with NS, which should be further extended with more children with NS. Additionally, the significant increase in final adult height after GH therapy in children with NS should be observed.
Adult ; Child* ; Facies ; Heart Defects, Congenital ; Humans ; Noonan Syndrome* ; Retrospective Studies ; Turner Syndrome

PURPOSE:Noonan syndrome(NS) is characterized by short stature, congenital heart disease, and typical facies. Recombinant human growth hormone(GH) has been reported to improve growth rate in a similar fashion to that seen in Turner syndrome. We investigated the clinical characteristics and growth reponses to GH therapy in children with NS. METHODS:The cases of sixty seven patients with NS were reviewed retrospectively. Ten of the 65 patients were assessed height, weight and pubertal stage every 3 months during GH therapy. RESULTS:Webbed neck(70%), delayed development(59.7%), low set posterior hairline(56.7%), eye abnormalities(56.7%) and mental retardation(55.2%) were the leading clinical characteristics. Short stature below the 3rd percentile was presented in 73.8 %. Growth patterns in NS children were variable and the evaluation of their growth must be individualized. The increments of height SDS were significant in children with GH therapy(height SDS:from -2.8+/-.6 to -2.3+/-.9, growth velocity:from 4.4+/-.8 cm to 9.2+/-.9 cm during first year, and 6.1+/-.1 cm during second year) (P<0.05). CONCLUSION: This study characterized the clinical profiles in Korean children with NS, which should be further extended with more children with NS. Additionally, the significant increase in final adult height after GH therapy in children with NS should be observed.
Adult ; Child* ; Facies ; Heart Defects, Congenital ; Humans ; Noonan Syndrome* ; Retrospective Studies ; Turner Syndrome

Hypereosinophilic syndrome is characterized by marked eosinohia and eosinopbilic infiltrition of various organ-systems without any identifiable underlyin sathologic conditions such as hypersensitivity, drug eruption, parasitic infestat,ion, malignant tuinc, and autoimrnune and infectious d iseases. A 31 year-old male had weight loss, abnormal peripheral cirrultiction both extremities and two distinct skin lesions. One showed rice sized, erythematous follicular popules on both extremit.ies and trunk and the other showed a walnut sized, gangrenous ulcer on the left 2nd finger tip. The patients skin lesions and circulation defect. improved after retrnent with steroid and the blood eosinophilia returned to normal.
Adult ; Blood Circulation* ; Drug Hypersensitivity ; Eosinophilia ; Extremities* ; Fingers ; Humans ; Hypereosinophilic Syndrome* ; Juglans ; Male ; Skin ; Ulcer ; Weight Loss

The involvement of mucosal mast cells (MMC) in the pathophysiology of irritable bowel syndrome (IBS) is still controversial. We aimed to re-evaluate the role of MMC in visceral hypersensitivity associated with IBS using a rat IBS model that develops the IBS symptom after a subsidence of acetic acid-induced colitis. No significant difference in the number of MMC was observed between normal rat colon and IBS rat colon. (61.7 +/-2.9/mm 2 in normal vs. 88.7 +/-13.3/mm 2 in IBS, p >0.29). However, the degranulation rate of MMC was significantly higher in IBS rat colon (49.5 +/-2.4% in normal vs. 68.8 +/-3.4% in IBS, p >0.05). Pretreatment of a mast cell stabilizer, doxantrazole (5 mg/kg, i.p.), reduced the degranulation rate of MMC and significantly attenuated visceral hypersensitivity to rectal distension in IBS rat, whereas it had no effect on the visceral sensory responses in normal rat. These results suggest that, although the number of MMC is not significantly changed in IBS rat colon, the higher degranulation rate of MMC is responsible for visceral hypersensitivity in this model IBS.
Acetic Acid/toxicity ; Animals ; Cell Count ; Colitis/chemically induced/*pathology ; Hypersensitivity/*pathology ; Image Processing, Computer-Assisted ; Intestinal Mucosa/*pathology ; Irritable Bowel Syndrome/*pathology ; Male ; Mast Cells/drug effects/*pathology ; Models, Theoretical ; Phosphodiesterase Inhibitors/pharmacology ; Rats ; Rats, Sprague-Dawley ; Thioxanthenes/pharmacology ; Viscera/immunology ; Xanthones/pharmacology

The cataract extraction in traumatic cataract with corneal laceration is likely to be different from the ordinary cataract extraction in several points. We tried to know the problems of the operation, the visual outcomes and the postoperative complications in these eyes. In 25 traumatic cataract with corneal laceration, we performed cataract extraction and corneal suture at the same time in 11 eyes and at different time in 14 eyes. The intraoperative problems were difficult anterior capsulotomy, corneal opacity, difficult lens delivery etc.The major postoperative complications were anterior chamber inflammation, corneal edema, posterior capsular opacity and exudative pupillary membrane. In 23 eyes[92%], the visual acuity was increased more than 2 lines. Cataract extraction in traumatic cataract with corneal laceration was very effective surgery, and cataract extraction after primary corneal suture has done had good results in severe corneal laceration with suspicious posterior capsular rupture and non-fitted preoperative preparation.
Anterior Chamber ; Cataract Extraction ; Cataract* ; Corneal Edema ; Corneal Opacity ; Inflammation ; Lacerations* ; Membranes ; Postoperative Complications ; Rupture ; Sutures ; Visual Acuity

We describe a 23-year-old man showing typically clinical and histological features of targetoid hemosiderotic hemangioma. About 35 cases of this newly-described entity have been reported since the first description by Santa Cruz and Aronherg in 1988. It is important to distinguish these tumors from patch stage Kaposi's sarcoma, retiform hemangioendothelioma and progressive lymphangioma.
Hemangioendothelioma ; Hemangioma* ; Humans ; Lymphangioma ; Sarcoma, Kaposi ; Young Adult

PURPOSE: This study was made to evaluate the clinical characteristics and the treatment of the gangrene of the male genitalia manifested with progressive life threatening disease. MATERIALS AND METHODS: Between June 1992 and December 1998, medical records for patients with Fournier's gangrene were reviewed with respect to age at presentation, associated disease, and clinical course and the treatment. RESULTS: The age of patients ranged from 29 to 92 years. The prodromal sign noted were usually low abdominal and inguinal pain. There were no differences in the causative pathogens compared to those of previous reports. The common underlying diseases were diabetes and chronic liver disease. Hypoalbuminemia and leukocytosis were found in all. Early radical debridement and aggressive antibiotics therapy were performed in all cases. Reconstructive surgery of defected skin was performed 7 to 45 days(mean 33.6) after debridement and period of hospital stay ranged from 14 to 123 days(mean 35.8). The motality rate was 13.3%. CONCLUSIONS: It is thought that the motality rate of Fournier`s gangrene is reduced by early aggresseve debridement of the wound and empirical triple antibiotic therapy, and the comprehension of clinical course is important as well.
Anti-Bacterial Agents ; Comprehension ; Debridement ; Fournier Gangrene ; Gangrene* ; Genitalia, Male ; Humans ; Hypoalbuminemia ; Length of Stay ; Leukocytosis ; Liver Diseases ; Male ; Medical Records ; Prodromal Symptoms ; Skin ; Wounds and Injuries

The present study was performed in order to investigate the correlation among the number of T zone histiocytes, the clinicopathologic parameters and the patient's survival. The subjects in this study were 131 advanced gastric carcinomas including 86 follow-up cases and 41 early gastric carcinomas. The infiltration of T zone histiocytes into tumor tissues was investigated using the immunohistochemical method with andti s-100 protein, nd the data obtained were evaluated statistically. T zone histiocytes were scattered among the cancer cells and in areas formed clusters within the cancer stroma. These cells were abundantly present in tumor tissues and around lymphoid follicles but were rarely seen in surrounding normal stomach tissue. Analysis of the number of T zone histiocytes showed no correlation between the number of T zone histiocytes and the clinicopathologic features except the degree of lymphocytic infiltration within the advanced and the early gastric carcinomas. Survival of the patients with a marked infiltration of T zone histiocytes in stage III was longer than that with mild infiltration of T zone histiocytes, but there was no statistical significance(P value=0.084). Multivariate regressio analysis revealed that the depth of invasion(P value=0.0074) and the lymphocytic infiltration(P value=0.0152) were the important prognostic factors. The results indicate that good prognosis is expected in cases with less deep invasion and high lymphocytic infiltration, and that the number of T zone histiocytes is in proportio to the degree of lymphocyte infiltration within the tumor, although not directly correlated with the patient's survival.

No abstract available.
Bronchitis, Chronic* ; Humans