Congenital smooth musele hamartomas appear at birth as hypertrichotic patches or plaques with or without hyperpigmentation. Histologic characteristic is hyperplasia of dermal smooth muscle bundles. We report a case of congenital smooth muscle harnartoma in 5-year-old female, who showed localized excessive-hairy, skin-colored patch on the lateral surface of left. elbow, which were found at birth. Biopsy specimen showed hyperpigmentation of the basal layers of epidermis, melanophages in upper dermis, and hyperplasia of smooth muscle bundles in lower dermis.
Biopsy ; Child, Preschool ; Dermis ; Elbow ; Epidermis ; Female ; Hamartoma* ; Humans ; Hyperpigmentation ; Hyperplasia ; Hypertrichosis ; Muscle, Smooth* ; Parturition

No abstract available.
Pregnancy* ; Pyelonephritis*

No abstract available.
Humans ; Inpatients* ; Pediatrics ; Retrospective Studies*

PURPOSE: The purpose is to evaluate the CT or MRI findings to help in the differentiation between benign and malignant meningiomas. MATERIAL AND METHODS: The CT and MRI findings of 8 patients with surgically proven malignant meningioma were reviewed. Four cases of 8 malignant meningiomas and 18 cases of 27 benign lesions were studied with a 1.5T or 0.5T MRI. Radiologic analysis was focused on the heterogeneity of tumor, grade of peritumoral edema, presence of cystic degeneration and calcification, tumoral enhancement pattern, dural enhancement, and tumoral border. RESULT: Histologic types of malignant meningioma were meningotheliomatous (n=4), papillary(n=1), fibroblastic(n=l), angioblastic(n=l), and sarcomatous(n=l). Tumoral Heterogeneity was seen in 8 of 8 malignant lesions(100%) and 8 of 27 benign ones(30%). (p<0.01) Marked peritumoral edema was seen in 8 of 8 malignant lesions(100%) and 2 of 27 benign ones. (7.3%)(p<0.01) Most of malignant lesions(6 of 8 cases, 75%) showed ill defined border. Calcification was not present in malignant lesions in contrast with benign ones (14 of 27 cases, 52%). (p<0.05) No statistical significance was noted in cystic degeneration, dural tail like enhancement or tumoral enhancement pattern between malignant and benign meningiomas. MRI signal intensity of malignant meningiomas was homogeneously or heterogeneously hypointense on T1WI, heterogeneously isointense or hypointense on T2WI and heterogeneous or mixed on Gd-DTPA enhancement study. CONCLUSION: The CT or MRI findings such as heterogeneity, ill defined tumoral border, marked peritumoral edema, and absence of calcification may suggest the possibility of malignancy in meningioma.
Edema ; Gadolinium DTPA ; Humans ; Magnetic Resonance Imaging* ; Meningioma* ; Population Characteristics

OBJECTIVE: A few years ago it was shown that uncooked cornstarch feeding(UCS) could correct the biochemical abnormalities resulting from a deficiency of glucose-6-phosphatase in children with type ment. So far, little experience is available with UCS in Korea. We studied to compare the effects on biochemical control and growth of therapy with portacaval shunt and therapy with UCS. METHODS: Enghteen patients with GSD-I, ranging in age from 2 to 17 years, were included in these studies. They were thirteen male and five female children, and were diagnosed as GSD-I in Seoul National University from 1982 to 1994. Six patients (age 6.75+/-3.06, range 4.33~12.75 years) received portacaval shunt after preoperative intravenous hyperalimentation, and fifteen patients(age 7.08+/-4.09, rnage 2.00~17.00years) received UCS(1.75~2g/kg, four times daily). They included three patients who have had hepatic adenoma during follow-up after portacaval shunt. Height standard deviation score(SDS), liver size, blood glucose, serum cholesterol, serum uric acid, and serum transaminase were measured. RESULTS: After UCS, the changes of height SDS, liver size, blood glucose, and serum uric acid were significant but the changes of cholesterol, SGOT, and SGPT were not significant. The SDS of height increased significantly form -2.61+/-1.01 at onset of UCS to -1.93+/-0.86 after UCS. The liver size decreased significantly form 10.1+/-2.7cm at onset of UCS to 7.9+/-3.0cm after UCS. Blood glucose levels increased significantly form 50.6+/-14.3mg/dl at onset of UCS to 90.1+/-17.0mg/dl after UCS. Serum uric acid levels decreased significantly form 8.03+/-2.12mg/dl at onset of UCS to 5.67+/-2.34mg/dl after UCS. Statistically, significant difference were not found in those values after portacaval shunt. CONCLUSIONS: UCS was effective to maintain blood glucose, to minimize biochemical abnormalities and to optimize clinical outcome in patients with GSD. Individuals with GSD-I commonly developed hepatic adenoma and malignant transformation of these ademonas has occurred. So in any case, it is advisable to screen all GSD-I patients periodically by imaging techniques for tumor formation, and once tumors have been detected, to adopt strict dietary measures and observe them closely for evidence of suggestive malignant transformation.
Adenoma ; Alanine Transaminase ; Aspartate Aminotransferases ; Blood Glucose ; Child ; Cholesterol ; Female ; Follow-Up Studies ; Glucose-6-Phosphatase ; Glycogen Storage Disease ; Humans ; Korea ; Liver ; Male ; Parenteral Nutrition, Total ; Portacaval Shunt, Surgical ; Seoul ; Starch* ; Uric Acid

Cardiac arrest associated with hyperkalemia during red blood cell transfusion is a rare but fatal complication. Herein, we report a case of transfusion-associated cardiac arrest following the initiation of extracorporeal membrane oxygenation support in a 9-month old infant. Her serum potassium level was increased to 9.0 mEq/L, soon after the newly primed circuit with pre-stored red blood cell (RBC) was started and followed by sudden cardiac arrest. Eventually, circulation was restored and the potassium level decreased to 5.1 mEq/L after 5 min. Extracorporeal membrane oxygenation (ECMO) priming is a relatively massive transfusion into a pediatric patient. Thus, to prevent cardiac arrest during blood-primed ECMO in neonates and infants, freshly irradiated and washed RBCs should be used when priming the ECMO circuit, to minimize the potassium concentration. Also, physicians should be aware of all possible complications associated with transfusions during ECMO.
Blood Transfusion ; Death, Sudden, Cardiac ; Erythrocyte Transfusion ; Erythrocytes ; Extracorporeal Membrane Oxygenation* ; Heart Arrest* ; Humans ; Hyperkalemia* ; Infant* ; Infant, Newborn ; Potassium

No abstract available.

BACKGROUND: The intrapleural hypofibrinolysis is caused by mainly excessive concentration of pleural plasminogen ogen activator inhibitor-1 antigen(PAI-1 Ag), which binds tissue type plasminogen activator. In pleural inflammation induced by sclerosing agents for pleurodesis, levels of pleural PAI-1 antigen increase in relation to decreing D-dimer levels. It has been known that the pleural mexothelial cells have the capability of secreting PAI-1 Ag in response to inflammation in vivo. Therefore, we estimated whether pleural inflammation changes the balance between fibrinolytic and coagulative properties in exudative pleural effusions. METHOD: The thirty cases was included in our study. We determined the pleural levels of glucose, lactic dehy-drogenase(LDH), pH and the counts of white blood cell(WBC), polymorpho leukocyte(PMN), lymphocyte as the parameters of pleural inflammation and cellular components of pleural fluid. The plasma level of fibrinogen in fluid and the neutrophil count in blood were determined. The levels of D-dimer, PAI-1 Ag and thrombin-antithrombin lll complex(TAT) were determined by ELISA(Behring, Marburg, Germany). RESULTS: The causes of pleural effusion were as following : tuberculous in 14 cases, malignant in 10 cases and parapneumonic in 6 cases. The levels of pleural D-dimer, PAI-1 Ag and TAT was significantly higher than that of plasma(p<0.001). The severity of pleural inflammation did not correlated with pleural D-dimer, PAI-1 Ag, TAT and their plaxma levels. But the level of pleural TAT correlated with pleural WBC and lymphocyte count. CONCLUSION: We found the the severity of pleural inflammations did not correlated with pleural D-dimer, PAI-1 Ag, TAT and the possibility of local production of PAI-1 antigen is present.
Fibrinogen ; Fibrinolysis* ; Glucose ; Hydrogen-Ion Concentration ; Inflammation ; Lymphocyte Count ; Lymphocytes ; Neutrophils ; Plasma ; Plasminogen ; Plasminogen Activator Inhibitor 1 ; Pleural Effusion ; Pleurodesis ; Sclerosing Solutions ; Tissue Plasminogen Activator

Diaper dermatitis is an infant dermatosis associated with the wearing of diapers, Despite of its high frequency in clinical pediatrics the clinical characteristics are not well described in the korean literatvre. We investigated the clinical and mycological characteritics of diaper dermatitis in 50 serial patients. Clinically diaper dermatitis was classified. into five categories,irritant contact derniatitis(ID), candidiasis(CD). combined rash of ID and CD(CR.), intertrigo, and miliari. Of 50 patients with diaper dermatitis, 18 had ID; 16, CI3; 10, CR; 5, intertrigo, and 1, miliaria, The mean age of patients with ID (11.8 months) was higher than that of patients with CD (4.0 months). Candida albicans(C. albicans) w is isolated from 25 patients with diaper dermatitis. The rate of positive cultures by clinical diagnosis was 130% in CD, 11.1% in ID, 60, 0% in CR, and 20.0% in intertrigo. C. albicons was isolated from 23 patients(82.1%) of patients with clinically suspected Ci") and 2 of 22 patients without the characteristic erythematous papulesThe presence of eryhematous papules was the most characteristic clinical feature of infection, Other candida species isolated from cut,aneous lesions were C. tropicalsis, C.'. parapsiolosis, and C. stellatoidea.
Candida ; Candidiasis ; Dermatitis* ; Diagnosis ; Exanthema ; Humans ; Infant ; Intertrigo ; Miliaria ; Pediatrics ; Skin Diseases

There is are d for more accurate prediction in surgical orthodontic treatment. Video imaging is an important technology in planning orthognathic surgery and educating Patients about the esthetic results after treatment. Preoperative and postoperative lateral cephalogram of 30 patients who had one piece Le Fort I osteotomy advancement and mandibular set back by bilateral intraoral vertical ramal osteotomy with or without genioplasty were used in this study. The computer generated soft tissue line drawing prediction were compared with the actual postoperative cephalograms. The results are as follows. 1. 14 variables showed Statistically significant differences from 24 variables between computer predicted profile and post operative profile 2. Most of the differences were found in the maxilla-related soft tissue landmarks. 3. The predicted results were more accurate in the groups who had small amount of mandibular set back. 4. The predicted results were more accurate in the groups who had no genioplasty. Most of these differences were within 2mm ranges. Therefore profile change prediction by video imaging could be considered clinically acceptable.
Genioplasty ; Humans ; Jaw* ; Orthognathic Surgery* ; Osteotomy