Disibling pansclerotic morphea, a severe form of generalized morphea, has a relentless clisabling course, producing marked contract,ion and joint deformity due to sclerosis of the skin and the deeper structures. We report herein an unusual case of morphea in a 25 year old male who had suffered from chronic recurrent ulceration with scar like sclerotic changes overlying limb folds, ankles, nape and abdomen since 4 years of age. The indurated lesions on the chest and abdomen were ivory colored, sclerotic guttae and plaques having a, violac ous border. At l4 years of age, the skin lesions were diagnostically ambiguous, wherea.s the immunologic results revealed a selective IgA deficiency with partial T lymphacyte inactivation. About 10 years later, the follow up visit showed this severe form of morphea over generalized area and disabling contractures of knees, elbows and alkles, but IgA value returned nearly to the nomal level.
Abdomen ; Adult ; Ankle ; Cicatrix ; Congenital Abnormalities ; Contracture ; Elbow ; Extremities ; Follow-Up Studies ; Humans ; IgA Deficiency ; Immunoglobulin A ; Joints ; Knee ; Male ; Scleroderma, Localized* ; Sclerosis ; Skin ; Thorax ; Ulcer

No abstract available.
Embryonic Stem Cells*

No abstract available.
Constriction, Pathologic*

We report herein a case of elephantiasis neuromatosa seen in neurofibromatosis patient, who was a 33-year-old male and had been diagnosed at the age of 13. Since that time on, small nodule present on the mid-back area led to develop grotesquely large, dark-brown colored, flabby mass with thick verucoid skin change, measuring about 30 * 20cm in size. Histopathologic examination of excised mass demonstrated moderate hyperplasia of epidermis and typical neurofibroma findings with soft tissue and fibrous tissue overgrowth, but no atypical cells. The electron microscopic study showed many perineurial cells surrounded by the continuous bssal lamina of fibroblsst in the dermal lesion and numerous giant pigmented granules in melanocyte and basal keratinocyte in the cafe-au-lait spot.
Adult ; Cafe-au-Lait Spots ; Elephantiasis* ; Epidermis ; Humans ; Hyperplasia ; Keratinocytes ; Male ; Melanocytes ; Neurofibroma ; Neurofibromatoses ; Skin

BACKGROUND: The purpose of this study is to evaluate the functional outcomes of reverse total shoulder arthroplasty (RTSA) and to assess factors affecting the patients' subjective satisfaction after RTSA. METHODS: Forty-three patients (mean age, 75.0 ± 5.2 years) who underwent RTSA for cuff tear arthropathy or irreparable cuff tears with preoperative magnetic resonance imaging and pre- and postoperative radiographs at 1 year, and whose various functional outcomes including pain visual analogue scale (VAS), simple shoulder test, Constant score, American Shoulder and Elbow Surgeons score, and active range of motion were evaluated preoperatively and at the last follow-up (>12 months) were enrolled. The outcome parameter was set as a satisfaction scale. Various clinical and radiographic factors were analyzed, and their correlations with postoperative satisfaction were evaluated. RESULTS: All functional scores, VAS pain score, and active forward flexion showed significant improvement after surgery (all p<0.001). Twenty-nine patients were satisfied with the results and 14 were dissatisfied. The presence of pseudoparalysis (p=0.028) and worse preoperative function (all p<0.05) were related with higher satisfaction. Any radiologic parameters did not affect patients' postoperative satisfaction. CONCLUSIONS: All patients showed a good functional outcome after RTSA, however the patients' subjective postoperative satisfaction was affected by preoperative functional status (higher satisfaction in poor preoperative function), not by radiological findings.
Arthroplasty* ; Elbow ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Range of Motion, Articular ; Shoulder* ; Surgeons ; Tears

No abstract available.
Acneiform Eruptions ; Bevacizumab

No abstract available.
Baculoviridae* ; Bombyx* ; Coinfection*

BACKGROUND: The combination of Philadelphia chromosome (Ph) and monosomy 7(-7) was rarely observed in acute lymphoblastic leukemia (ALL). With the results from immunophenotyplc and molecular analysis, Philadelphia chromosome positive ALL with monosomy 7[Ph(+)/-7] has been considered that it may be derived from neoplastic transformation at the pluripotent stem cell level. We compared the clini-cal, laboratory, and hematological findings between 5 cases of Ph(+)/-7 and 5 cases of Ph(+) without monosomy 7 [Ph (+) /N7]. METHODS: During the period from January, 1995 to December, 1996, total 72 cases of ALL were confirmed among 259 cases of hematologic malignancy with bone marrow cytogenetic analysis. Among 72 ALL cases, 5 cases of Ph(+)/-7(monosomy 7 or 7q abnormalities) were compared with Ph only or Ph without monosomy 7(ph(+)/N7] on the hematological, immunophenotypic, other laboratory, clinical findings and event ree survival (EFS) The karyotyping of the bone marrow specimens was analysed byshort-term unsynchronized culture methods such as overnight colcemid treatment and 24 hours incubation following ethidium bromide treatment. RESULTS: The mean age of Ph(+)/-7 was 30.6+/-12.8 years, and it was significantly different from that of Ph(+)/N7 (p=0.009), Four cases of Ph(+)/-7 were classified as ALL L2 subtype, and 2 cases revealed CNS involvements. Immunophenotyping was positive in CD10, CDl9, CD2O, CD22 and HLA-DR. But one case revealed e-B-lymphoid lineage with positivity in CD34, CDl3, and CD33. The response to chemotherapy and EFS was very poor in Ph(+)/-7 group, and the mean EFS was 3.2+/-1.9 months(p=0.014). All of cases showed induction on failure in chemotherapy, relapsed with bone marrow, CNS and extramedullary involvements, and expired due to sepsis. CONCLUSIONS: Ph(+)/-7 ALL had very Poor clinical course with being resistant to chemotherapy and unfavorable prognosis, revealed L2 subtype by FAB classification, and was slightly older in ages compared with Ph(+)/N7 ALL.
Bone Marrow ; Classification ; Cytogenetic Analysis ; Demecolcine ; Drug Therapy ; Ethidium ; Hematologic Neoplasms ; HLA-DR Antigens ; Hydrogen-Ion Concentration ; Immunophenotyping ; Karyotyping ; Monosomy* ; Philadelphia Chromosome* ; Pluripotent Stem Cells ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Sepsis

No abstract available.
Calcium* ; Estrogens* ; Female ; Humans ; Hypogonadism* ; Metabolism* ; Ovulation Induction* ; Ovulation*

No abstract available.
Hand* ; Wrist*