No abstract available.
Sepsis*

No abstract available.
Choanal Atresia*

No abstract available.
Diagnosis* ; Pyloric Stenosis, Hypertrophic*

Maffucci's syndrome is a very rare, congenital and non-hereditary mesodermal dysplasia manifested by multiple enchondromas and soft tissue hemangiomas. Since Maffucci had reported this sysdrome in 1881, there have been more than 100 cases reported, and also there has been reported that Maffuddi's syndrome has various interstitial tumor. However there hasn't been any report about Maffucci's syndrome with testicular teratoma. Here we report in this paper that the patient was diagnosed as Maffucci's syndrome of enchondroma and liver hamangioma and also had testicular teratoma. He was admitted for the evaluation of gynecomatia and diagnosed as enchondroma by bone biopsy of the right rib and tibia. Liver hemangioma was also found through abdominal ultrasonogram, CT scan and liver biopsy. And the testicular teratoma was confirmed through testicular biopsy. He is finally diagnosed as Maffucci's syndrome with testicular teratoma and literatures were reviewed.
Biopsy ; Chondroma ; Enchondromatosis ; Hemangioma ; Humans ; Liver ; Mesoderm ; Ribs ; Teratoma* ; Tibia ; Tomography, X-Ray Computed ; Ultrasonography

In 1947, Ota et al. first reported a case of phakomatosis pigmentovascularis and defined a subgroup of this disease having a congenital generalized hemangioma and pigmented lesions including a mongolian spot-like lesion, nevus pigmentosus and nevus of Ota. We experienced a case of phakomatosis pigmentovasularis type IIb in a 10-day old male baby, who since birth, had generalized nevus flammeus and blue spots, syndactyly between 2nd and 3rd toes in both feet, and dilated collecting system in left kidney.
Foot ; Hemangioma ; Humans ; Kidney ; Male ; Neurocutaneous Syndromes* ; Nevus ; Nevus of Ota ; Parturition ; Port-Wine Stain ; Syndactyly ; Toes

Objective: We evaluated the protective effect of dexamethasone (DX) administration on brain damage produced in a perinatal model of cerebral hypoxia-ischemia in the rat. Since hyperglycemia has been shown to reduce hypoxic-ischemic brain injury (HI) in immature tar, we investigated the role of glucocorticoid-induced hyperglycemia in the neuroprotective mechanism of DX. Methods: Hypoxic-ischemic brain injury in 7-day-old rats was induced by right common carotid artery occlusion and 2 hours of 8% oxygen. Pups received 3 doses of DX (0.5mg/kg/d intraperitoneally) 48 hours, 24 hours and immediately before HI (Dx1)(n=12), a single dose of DX 24 hours(DX2)(n=16), 3 hours (DX3)(N=10)or immediately before HI (DX4)(n=14), a single dose of DX immediately after HI (DX5) (n=9), 3 doses of DX immediately, 24 hours and 48 hours after HI (DX6) (n=14) and a single dose of DX 24 hours before HI with insulin (0.5U/kg, subcutaneously, 1.5 hours before HI)(IN)(n=8). Control pups (n=15) received a single dose of normal saline 24 hours before HI. Blood glucose was estimated before hypoxia, 1 hour and 2 hours after hypoxia using glucometer in DX 1~4. IN and control rats. Pups were killed at 14 days of age for determination of mortality during HI, gross cerebral infarction and right cerebral hemisphere atrophy. We measured the diameter of each cerebral hemisphere and cortical thickness from a coronal section at the dorsal hippocampus level, and expressed the % atrophy from the change in the right vs left hemisphere diameter. Results: The mortality that occurred during and after HI was similar in all groups. The incidence of gross cerebral infarction was 0.0%, 0.0%, 75.0%, 83.3%, 87.5%, and 90.0% in DX 1~6, respectively, 0.0%in IN, and 100.0% in control group. There was a significant difference (p<0.001)in the incidence of gross cerebral infarction of DX1, DX2, IN vs control group. The mean % atrophy was 5.4 +/- 2.2, 4.9 +/- 1.8, 21.7 +/- 8.1, 29.7 +/- 5.0, 37.4 +/- 5.5, 33.4 +/- 9.3 in DX 1~6, respectively, 1.5 +/- 1.1 in IN, and 29.1 +/- 3.4 (mean+/-SEM) in control group. There was a significant difference in % atrophy of DX1, DX2, IN vs control group. Before hypoxia, there was no significant difference in blood glucose between saline, all DX, and DX with insulin treated groups. But after hypoxia, pups in DX1 and Dx2 were more hyperglycemic compared to DX 3~4, IN, or saline treated groups. Conclusions: Dexamethasone administration in the neonatal period protects the brain during the subsequent periods of hypoxia-ischemia in rats and glucocorticoid-induced hyperglycemia does not explain the neuroprotective effects dexamethasone.
Animals ; Anoxia ; Atrophy ; Blood Glucose ; Brain Injuries ; Brain* ; Carotid Artery, Common ; Cerebral Infarction ; Cerebrum ; Dexamethasone* ; Hippocampus ; Hyperglycemia* ; Hypoxia-Ischemia, Brain ; Incidence ; Insulin ; Mortality ; Neuroprotective Agents ; Oxygen ; Rats*

OBJECTIVES: The purpose of this study was to examine the effect of cognitive-behavioral group therapy on improving adolescents' behavioral problems, especially violent behavior. METHOD: The subjects were 27 middle school students referred by their teachers for behavioral problems. We devided them into 4 teams and treated them for 15 sessions of cognitive-behavioral group therapy. The control group were 20 middle school students who had no cognitive-behavioral therapy. We assessed their behaviors pre- and post intervention by two series of scale, ie. self ratings and teacher ratings. Self ratings included 'misbehavior scales' and 'violent behavior subscale'; teacher ratings included 'teacher-children rating scale' and 'detection of misbehavior scale'. RESULT: 1)In self-rating scales, the misbehavior of the subject group decreased more than the coltrol group, but not the violent scale. There were statistically significant group differences of improving effect in misbehavior scales. 2)In teacher rating scales, there was no significant decrease in the behavioral problems of the subject group. 3)By teams there were significant different intervention effects among 4 teams in self rating misbehavior scale. Team 2 improved on all scales except teacher-rating detection of misbehavior scale. 4)Determinant factors of intervention effect on behavioral problems were the degree of baseline behavioral problems, sex of subjects and therapist. CONCLUSION: The results from this study suggest that cognitive-behavioral group therapy was effective in decreasing the behavioral problems of adolescents. But the effect of therapy depends on sex, the degree of behavioral problems of subjects and the therapist.
Adolescent ; Humans ; Psychotherapy, Group* ; Violence ; Weights and Measures

Graft-versus-host disease is commonly observed after allogeneic bone marrow transplantation but rarely recognized after transfusion of solid-organ transplantation. Tansfusion-associated graftversus-host disease can occur in immunosuppressed recipients and immunocompetent transplant recipients. The clinical manifestations of gastrointestinal or hepatic dysfunction, rash and pancytopenia should heighten the physician's index of suspicion for GVHD. Among premature infants, only four cases have been reported to develop transfusion-associated graft-versus-host disease in the world, with a mortality rate of 100 percent. We recently experienced a preterm male infant who developed acute GVHD (erythematous maculopapular skin rash, hepatic dysfunction and pancytopenia) at two months of age and recovered with Dexamethasone and supportive treatment. A skin biopsy, which performed on the confluent erythematous maculopapular rashes during the active and healing stage of the skin rash, revealed characteristic features of GVHD. Prematurity may be considered as a risk factor for the development of GVHD possibly related to complicated prolonged illnesses which requires intensive care and multiple transfusions. To our knowledge, this is the first report of survival after transfusionassociated graft-versus-host disease in preterm infants.
Biopsy ; Bone Marrow Transplantation ; Dexamethasone ; Exanthema ; Graft vs Host Disease* ; Humans ; Infant ; Infant, Newborn ; Infant, Premature* ; Critical Care ; Male ; Mortality ; Pancytopenia ; Risk Factors ; Skin ; Transplantation

Myeloid-derived suppressor cells (MDSCs) are highly immunosuppressive myeloid cells that show increased expression in cancer patients; however, the molecular mechanisms underlying their generation and function are unclear. Whereas granulocytic-MDSCs correlate with poor overall survival in breast cancer (BC), the presence and relevance of monocytic (Mo)-MDSCs are unknown. Here, we report for the first time increased chemokine and chemokine receptor production by Mo-MDSCs in BC patients. A clear population of Mo-MDSCs with the typical cell surface phenotype (human leukocyte antigen-antigen D related [HLA-DR]low/− CD11b+ CD33+ CD14+) increased significantly during disease progression. In addition, the chemokine receptor expression level on Mo-MDSCs in patients with invasive BC was the highest. Furthermore, different chemokine receptor expression patterns were noted in Mo-MDSCs between healthy controls (HC) and BC patients. Additionally, CD4 T cells proliferations were significantly decreased in the invasive BC groups compared with the HC group. However, the ductal carcinoma in situ (DCIS) group had no significantly compared with the HC group. Our data suggest that monitoring chemokine and chemokine receptor production by Mo-MDSCs may represent a novel and simple biomarker for assessing disease progression in BC patients.
Breast Neoplasms ; Carcinoma, Intraductal, Noninfiltrating ; Chemokines* ; Disease Progression ; Humans ; Leukocytes ; Myeloid Cells ; Phenotype ; Receptors, Chemokine ; T-Lymphocytes

Gastrointestinal stromal tumors (GISTs) are not uncommon and often cause gastrointestinal bleeding. GISTs occurring in the small intestine are occasionally difficult to identify by endoscopy and CT. In this case, the patient underwent CT three times before surgery, and the lesion was found to be located in a different area of the abdominal cavity on each CT scan. Moreover, the lesion was missed in the first two CT images because it was difficult to distinguish it from the nearby collapsed small intestine. The lesion was eventually detected through angiography; however, the correct diagnosis and treatment were delayed for 3 years because it was mistaken for a vascular malformation, which is the most common cause of obscure GI bleeding in elderly patients. This report emphasizes the need for interventional radiologists to be updated and vigilant of the angiographic features of GISTs to make an accurate diagnosis and establish a management strategy.