No abstract available.
Lip* ; Ulcer*

No abstract available.
Lip* ; Ulcer*

We report cases of two patients with acute generalized exanthematous pustulosis(AGEP). One patient had localized cutaneous infection and the other rhinoplasty. Both were being treated with ampicillin and developed intense erythemas followed by generalized subcorneal pustulation associated with fever and a neutrophilic leukocytosis. Histopathological findings were subcorneal spongiform pustules showing preponderance of polymorphonuclear leukocytes. Generalized pustular psoriasis, subcorneal pustular dermatosis, impetigo and pemphigus foliaceus should be differentiated from AGEP. The causative drug in both of our cases was ampicillin and fast resolution of pustules was observed with a low dosage of systemic steroid within 5 days.
Acute Generalized Exanthematous Pustulosis* ; Ampicillin* ; Erythema ; Fever ; Humans ; Impetigo ; Leukocytosis ; Neutrophils ; Pemphigus ; Psoriasis ; Rhinoplasty ; Skin Diseases, Vesiculobullous

Electron mlactimpic studies were performed on three primary and two early secondary syphilitic lesions from three patients. AZ of these patients had both secondary syphilitic lesions and chancres. There was no difference in the morphology and location of treponemes in these patients as compared to those who had only a chancre or only a syphilid reported earlier. This study revealed that Treponema pallida were principally located in the intercellular space of keratinocytes and exhacellular space of the dermis. Furthermore, these organisms were observed within histiocytes, endothelial cells, fibroblasts, lymphocytes and even plasma cells. The plasma cells had well developed endoplasmic reticulum, and in some, dilatation of the endoplasmic reticulum was noted. Many t mponemes were demonstrated in and around blood vessels. The vascular endothelial cells were proliferating and the basement membranes were thick.
Basement Membrane ; Blood Vessels ; Chancre* ; Dermis ; Dilatation ; Endoplasmic Reticulum ; Endothelial Cells ; Extracellular Space ; Fibroblasts ; Histiocytes ; Humans ; Keratinocytes ; Lymphocytes ; Microscopy, Electron ; Plasma Cells ; Syphilis ; Syphilis, Cutaneous* ; Treponema pallidum* ; Treponema*

BACKGROUND: Zinc which is widely used to treat Behcet's disease, is known to be an important modulator in various aspects of immunity including cell mediated immunity (CMI). CMI is suspected of playing a major role in the pathogenesis of Behçet's disease. OBJECTIVE: This study was done to clarify the relationship of CMI and zinc in Behçet's disease. METHODS: Serum zinc level, NK cell activity, and ADCC were measured in 83 patients with Behçet's diseade. The results were analyzed using multiple regression analysis. RESULTS: ADCC and serum zinc level were found to be two significant variables that affect NK cell activity positively and negatively, respectively. CONCLUSION: Serum zinc is presumed to exert inhibitory effect on NK cell activity but does not affect ADCC in Behçet's disease patients.
Antibody-Dependent Cell Cytotoxicity ; Humans ; Immunity, Cellular ; Killer Cells, Natural ; Zinc*

PURPOSE: Adrenocortical tumors are uncommon in children and comprise only a small proportion of primary adrenal neoplasms. The biologic behavior of these tumors may be very difficult to predict, and their rarity has hindered identification of clinical characteristics. Patients with functioning tumors have excessive steroid hormone production, and the clinical manifestation depends on the predominant hormone produced. The detection of nonfunctioning tumors is not easy and the diagnosis may be delayed. Benign tumors can be cured by complete surgical excision, but malignant cases have poor response to treatment and worse prognosis. Early diagnosis and proper management are very important because of the large proportion of functioning malignant tumors in children. We report clinical features of adrenocortical tumors in children that may be of help in the early detection, proper management, and assessment of prognosis of patients. METHODS: We reviewed the clinical characteristics of 14 cases of adrenocortical tumors, among 85 children diagnosed with adrenal tumors, who visited the Severance Hospital, College of Medicine, Yonsei University, from January 1970 to July 1996. RESULTS: 85 Patients were diagnosed with adrenal tumors. Among them, 71 cases 83.5%) were tumors of the adrenal medulla, neuroblastoma and pheochromocytoma, and 14 cases (16.5%) were adrenocortical tumors, consisting of 5 cases of adenoma, 7 cases of carcinoma, and 2 cases unspecified. The age distribution ranged from 16 months to 14 years of age, and the mean was 5 years & 11 months (median 4 years & 2 months). Sex distribution revealed a male to female ratio of 1:1.33. The left to right ratio was 3.7:1, showing a left side predominance. 13 Cases (92.9%) were functioning tumors: 12 cases (92.3%) had clinical evidence of androgen excess, among which 6 cases (46.2%) were associated with Cushing's syndrome, and 1 case was compatible with primary aldosteronism. Serum cortisol, urinary 17-ketosteroids and 17-hydroxycorticosteroids concentrations were measured in 11 cases and urinary concentrations of 17-ketosteroids were elevated in all 11 cases (100%), while 17-hydroxycorticosteroids were elevated in 4 cases (36.4%). Abnormalities of serum cortisol were found in all cases except 1: serum cortisol concentrations were abnormally elevated in 5 cases (45.5%), and the remainder (5 cases, 45.5%) showed loss of diurnal variation. Dexamethasone suppression test was performed in 9 cases, and all (100%) showed no suppression. Preoperative radiologic studies included abdominal sonograms, CT or MRI scans, and angiography. Histology showed carcinomas to be bigger and heavier than adenomas, and microscopically carcinomas had necrosis, calcifications, and invasions of vessels. Distant metastases were found in 4 cases (12.7%). Adrenalectomy with complete surgical excision was performed in 12 cases. Long-term follow-up was possible in 10 patients after operation: 3 patients initially diagnosed with adenoma survived without tumor recurrence over a year, and among 6 carcinoma patients, 4 expired within a year, and 2 survived, with one patient currently undergoing postoperative chemotherapy. Of the 10 patients currently under going follow-up, one patient was initially diagnosed with a histologically unspecified tumor, and has survived 4 years after operation. CONCLUSIONS: When adrenocortical neoplasms are suspected by clinical symptoms and laboratory findings, abdominal ultrasonogram, CT or MRI scans must be performed immediately. Early detection and proper management are important for better prognosis, but are often delayed in the majority of cases. Ultimately, pediatricians need to be familiar with clinical characteristics and laboratory findings of adrenocortical tumors, bearing in mind the possibility of diagnosis in children.
17-Hydroxycorticosteroids ; 17-Ketosteroids ; Adenoma ; Adrenal Gland Neoplasms ; Adrenal Medulla ; Adrenalectomy ; Age Distribution ; Angiography ; Child ; Cushing Syndrome ; Dexamethasone ; Diagnosis ; Drug Therapy ; Early Diagnosis ; Female ; Follow-Up Studies ; Humans ; Hydrocortisone ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Male ; Necrosis ; Neoplasm Metastasis ; Neuroblastoma ; Pheochromocytoma ; Prognosis ; Recurrence ; Sex Distribution ; Ultrasonography

Melkersson-Rosenthal (M-R) syndrome consists of a triad of (1) recurrent peripheral facial nerve paralysis which develops alternatively on both sides of face, (2) non-inflammatory facial edema, and (3) furrowed tongue. Since the cause of M-R syndrome is unknown, various forms of therapy have been tried, but there were no conclusive evidence that they altered the course of the disease. A 27-year-old female and a 44-year-old male patient with recurrent facial nerve paralysis were diagnosed with M-R syndrome. We report the two cases of M-R syndrome with the brief review of literatures.
Adult ; Edema ; Facial Nerve ; Female ; Humans ; Male ; Melkersson-Rosenthal Syndrome* ; Paralysis ; Tongue, Fissured

OBJECTIVE: The main purposes of this study were to understand the correlations among Modified Barthel Index (MBI), Functional Independence Measure (FIM), and ESCROW (Environment, Social support, Cluster of family members, Resources, Outlook, Work or School status) Profile, and to establish the more appropriate assessment standards to check patient's conditions in the hospital and in their homes and society. METHOD: Thirty-four patients, who received the rehabilitation treatment and home visiting at the Presbyterian Medical Center, were evaluated for their functions by MBI, FIM, and ESCROW Profile. RESULTS: The mean scores of assessment measures by home visiting were all higher than in the hospital showing an improvement of the patients' functions after discharge from the hospital. The results of MBI and FIM in the hospital and home visiting showed a significant correlation, while the results of MBI and Cognitive FIM measure indicated a relatively low correlation coefficient. Although each result of MBI, FIM, and ESCROW provided a low correlation when the patients were in the hospital, the result for home visiting revealed very significant correlations. Especially, the items of environment, social support, outlook, and work status of ESCROW showed very significant correlations with MBI and FIM. CONCLUSION: The results showed that MBI and FIM measurements were very useful in observing and following up the functional conditions of the patients, while ESCROW profile was more appropriate to evaluate the familial and social rehabilitation status.
House Calls ; Humans ; Protestantism ; Rehabilitation* ; Social Environment

PURPOSE: To determine the clinical and mammographic characteristics of primary lymphoma of the breast. MATERIALS AND METHODS: The clinical and mammographic records of five patients (six breasts; bilateral involvement in one patient) with histologically-proven lymphoma of the breast were retrospectively reviewed. RESULTS: All patients had complained of a palpable tender mass with relatively rapid growth over a period of about 3 months. In five cases, the mass was located in the right breast and in one case in the left. Mammography revealed diffuse increased opacity of the entire breast in three cases, a homogeneous well-defined mass in one, multiple well-defined masses in one, and ill-defined lobulated opacity in one. Skin thickening and architectural distortion was seen in one case, and axillary lymphadenopathy in two. In no case was microcalcification and nipple retraction seen. CONCLUSION: In primary breast lymphoma, mammographic finding are non-specific. When a breast grows rapidly and is tender, however, and mammography shows a relatively large well defined mass and associated axillary lymphadenoapathy, the possibility of primary lymphoma should be considered.
Breast* ; Humans ; Lymphatic Diseases ; Lymphoma* ; Mammography ; Nipples ; Retrospective Studies ; Skin

PURPOSE: The objective of this study is to investige the clinical features, natural histories, and results of medical and surgical treatment of NMD in patients who were diagnosed during childhood. METHODS: We performed a retrospective analysis of medical records of 57 patients with NMD who were newly diagnosed by MRI or pathologically in epilepsy children since March 1993 to June 2000. RESULTS: These 57 patients with NMD consisted of 26 with cortical dysplasia, 9 with lissencephaly, 7 with polymicrogyria, 6 with schizencephaly, 4 with hemimegalencephaly, 3 with heterotopias, and 2 with double cortex. Clinically, 94.7% of these patients showed seizures, 33.3% with developmental delay, 21.1% mental retardation, 15.8% cerebral palsy, and 7.0% attention deficit hyperactivity disorder. Their response to antiepileptic drugs was good to 31 patients (75.6%), moderate to 3 (7.3%), and poor to 7 (17.1%). Twelve patients were completely seizure-free after receiving medication for at least 15 months. Seventeen patients tolerated with monotherapy with antiepileptic drugs. Fourteen patients underwent surgical resection. The results of operation were highly correlated with the complete removal of epileptic focus. Six patients who underwent complete resection were seizure-free after operation. On the other hand, Eight patients who had incomplete resection of the epileptic focus showed poor outcome. CONCLUSION: Most of previous reports suggested that NMD is associated with refractory to medical treatments, and early surgical operation has been recommended. Our study demonstrates remarkably good responses of NMD patients with medical treatment only.
Anticonvulsants ; Attention Deficit Disorder with Hyperactivity ; Cerebral Palsy ; Child* ; Epilepsy ; Hand ; Humans ; Intellectual Disability ; Lissencephaly ; Magnetic Resonance Imaging ; Malformations of Cortical Development ; Medical Records ; Neuronal Migration Disorders* ; Neurons* ; Retrospective Studies ; Seizures