No abstract available.
Decompression* ; Facial Nerve* ; Melkersson-Rosenthal Syndrome*

No abstract available.
Child* ; Cyclophosphamide* ; Humans ; Nephrotic Syndrome* ; Retrospective Studies*

No abstract available.
Fetus ; Humans* ; Thymus Gland*

A case of osteomalacia is presented. A 46-year-old woman had had complaints of pain in low back and both knee joints for four years. A bell shaped thorax with kyphoscoliosis was noted. She was diagnosed as an osteomalacia by roentgenograph and laboratory examinations. Treatment by medication was followed with good results.
Female ; Humans ; Knee Joint ; Middle Aged ; Osteomalacia ; Thorax

There had been total 20 patients with early onset(4 months~2 years) primary nephrotic syndrome in the Department of Pediatrics, Seoul National University Children's Hospital, during the period from March 1987 to February 1993. We analysed clinical courses, response to treatment, pathological findings and prognosis of the patients And the results were as follows; 1) The initial responders to steroid treatment were 10(50%), of whom 3 became late nonresponders. Of the 10 initial nonresponders, 8 revealed continuing nonresponsiveness. 2) Incidence of hypertension was significantly higher in the intial nonresponders than in the initial responders. 3) Renal biopsies were performed in 9 initial nonresponders and 2 late nonresponders. And the results were 6 with minimal change lesion, 4 with focal segmental glomerulosclerosis and 1 with mesangiocapillary glomerulonephritis. 4) Six continuing or late nonresponders received methylprednisolone pulse therapy or cyclophosphamide. And partial remission was induced in 4 of them. 5) The overall 3 year maintenance rate of normal renal function was 81%(100% in the initial responders and 64% in the initial nonresponders). In conclusion, the initial steroid responsiveness and prognosis were poorer in patients with early onset nephrotic syndrome than in patients with usual childhood nephrotic syndrome.
Biopsy ; Cyclophosphamide ; Glomerulonephritis, Membranoproliferative ; Glomerulosclerosis, Focal Segmental ; Humans ; Hypertension ; Incidence ; Methylprednisolone ; Nephrotic Syndrome* ; Pediatrics ; Prognosis ; Seoul

BACKGROUND: Recently, the molecular pathologic investigation for clonality in lymphomas has been introduced and has gained a role in the diagnosis of lymphomas. In fact, the clonality test using TCRGR phenomenon has been done by Southern blot analysis (SBA) and polymerase chain reaction (PCR) for molecular pathologic diagnosis of T cell lymphomas. However, it is difficult to perform SBA with paraffin embedded specimens or with samples of small skin biopsies. OBJECTIVE: We investigated the efficacy of PCR amplification of TCR gene in paraffin em-bedded cutaneous T cell lymphomas. METHODS: Iii this study, the clonality was assessed by polymerase chain reaction (PCR) analysis of T cell receptor gamma (TCR) gene from the DNA extracts obtained from paraffin em-bedded tissues (PET) of malignant T cells, B cell lymphomas, and benign cutaneous T cell proliferative disorders. Heteroduple-x-analyses were also performed to rule out the false positives. RESULTS: Among the total of 62 cases analyzed, monoclonality was observed in 4 out of 10 mycosis fungoides, 7 out of 9 cutaneous T cell lymphomas excluding mycosis fungoides, 1 out of 3 angiocentric lymphomas, 2 out of 2 lymphomatosis papulosis, 1 out of 7 large plaque parapsoriasis, and 1 out of 2 T cell lymphomas in other organs. No monoclonality was observed in 9 inflammatory cutaneous diseases, 5 small plaque parapsoriasis, 4 cutaneous B cell lymphomas, and 11 B cell lymphomas in lymph nodes. CONCLUSION: The results suggest that the PCR method and heteroduplex analysis used in this study were not only practical but also efficacious for the diagnosis of cutaneous T cell lymphomas using tissues embedded in paraffins.
Biopsy ; Blotting, Southern ; Diagnosis ; DNA ; Gene Rearrangement* ; Genes, T-Cell Receptor ; Heteroduplex Analysis* ; Lymph Nodes ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Cutaneous ; Mycosis Fungoides ; Paraffin ; Parapsoriasis ; Polymerase Chain Reaction* ; Receptors, Antigen, T-Cell* ; Skin ; T-Lymphocytes

The application of fine needle aspiration (FNA) cytology to the soft tissue tumors had been neglected. In recent years, however, FNA has been used increasingly in the preoperative diagnosis of these tumors due to its usefulness and accuracy. We present 3 cases of liposarcoma, myxoid, myxoid with round cell, and pleomorphic, diagnosed by FNA cytology with histologic confirmation. Good correlation between his- tologic and FNA cytologic findings was found. Although the cytologic appearances of liposarcomas varied with histologic type, the main criterion was the presence of atypical multivacuolated lipoblast with characteristically scalloped nuclei.
Biopsy, Fine-Needle ; Child* ; Diagnosis ; Humans ; Hypertension, Renovascular* ; Liposarcoma ; Liposarcoma, Myxoid ; Pectinidae

The clinical, laboratory, and renal biopsy finding in 22 chilren with recurrent hematuria are presented. 1.The mean age at oneset was 7 6/12 years, and male to female ratio was 1.8:1. The gross hematuric attack was preceded by upper respiratory infection in 15 cases(68%), while strenuo-us exercise in 10 cases(45%). Loin pain was accmpanied with gross hematuria in 4 cases(18%). 2. Casts were found on urinalysis in 12 cases(55%), and 7 cases(32%) revealed more than 0.5gm in 24 hour urinary protein excretion. Serum IgA level was elevated in 8 cases(50%) out of 16 tested. Persistent proteinuria and microscopic hematuria between gross hematuric attack-s were found in 5 cases(23%) and 11 cases(50%) respectively. 3. Light microscopy showed minimal change lesion in 7 cases(32%), mesangial proliferative glomerulonephritis in 7 cases(32%), mesangiopathic glomerulonephritis in 5 cases(23%), focal segmental glomerulosclerosis in 2 cases(9%), and membranoproliferative glomerulonephritis in 1 case. Immunofluorescent study, performed in 16 cases, revealed the mesangial deposition of IgA in 9 cases(56%), IgG in 7 case(44%), C3 in 11 cases(69%), and IgM in 2 cases(15%) out of 13 performed. Electron dense deposits were identified in 13 cases of 14 observed with electron microscopy. One or more findings of mesangial proliferation, focal segmental glomerulosclerosis, cresc-ent formation on light microscopy were regarded as severe pathological abnormalities and such findings were associated with more than 0.5gm of protein in 24hour urine, persistent proteinuria between attacks, and elevated serum IgA level. Statistically significant differences were also n-oted between the cases with severe pathological abnormalities and the cases without them in terms of mean serum IgA level and the amount of protein in 24 hour urine. No patient has developed complications, such as hypertension or chronic renal failure, duri-ng the follow-up periods ranging from 5 months to 9 6/12 years, being mean 2 8/12 years.
Biopsy ; Female ; Follow-Up Studies ; Glomerulonephritis ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulosclerosis, Focal Segmental ; Hematuria* ; Humans ; Hypertension ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Kidney Failure, Chronic ; Male ; Microscopy ; Microscopy, Electron ; Proteinuria ; Urinalysis

Forty eight skin biopsies obtained from 24 patients were reviewed, and clinical, histological and immunohistochemical findings were analyzed. Results obtained are as follows: 1) Skin manifestation was plaque, erythroderma, scale and hyperpigmentation in mycosis fungoides, and subcutaneous nodule, mass and ulcerated patch in cutaneous lymphoma. The skin of lymphomatoid papulosis revealed hemorrhagic ulcerated and erythematous papules which healed spontaneously. 2) Histologically, mycosis fungoides showed epidermotropism in most cases. Pautrier's micro-abscesses were present in one-fourth of the cases. Malignant lymphoma was different in histology from mycosis fungoides. As compared with mycosis fungoides, it showed less frequent epidermotropism, more compact and diffuse infiltration of atypical lymphocytes, more often association with ulcer and necrosis, and more frequent mitotic figures. Lymphomatoid papulosis showed striking hemorrhage and edema of the papillary dermis. 3) Based on the results of immunohistochemical study, mycosis fungoides and lymphomatoid papulosis were considered as a T cell proliferative disorder of the skin. According to these findings, lymphoproliferative disorders of the skin occurred predominantly in the elderly and males. Clinical and histopathologic findings overlapped and were similar each other. It was difficult to make a definite diagnosis in early lesions, and a sequential follow up biopsy was required. It is concluded that strict criteria such as marked atypia and clustering of atypical cells are necessary for a histologic diagnosis of malignant lymphoproliferative disorder of the skin.
Biopsy

Alveolar soft part sarcoma is a clinically and morphologically distinct soft tissue tumor that was first defined and named by Christopherson et al in 1952. Since 1953, alveolar soft part sarcoma invading bone have been reported sporadically. We experienced a case of alveolar soft part sarcoma with metastasis to femoral shaft, which was treated by wide resection & vascularized fibular strut graft.
Neoplasm Metastasis ; Sarcoma, Alveolar Soft Part ; Transplants