Oncogenous or tumor-induced rickets is recognized as Vitamin D-resistant hypophosphatemic rickets that heals after excision of a coexisting tumor in either bone or soft tissue. Its pathogenesis is not clarified, but it has been suggested that the tumor elaborates a homoral substance inhibiting tubular reabsorption of phosphate or that the tumor inhibits the synthetic process of 1, 25-dihydroxcholecalciferol. We present two cases of vitamin D-resistant rickets, one associated with intrasseous well-differentiated osteosarcoma in the pelvis and the other associated with fibrosarcoma of distal femur. The case with low grade osteosarcoma showed immediate improvement of rickets after excision of the tumor, but the case with fibrosarcoma showed no improvement after amputation and was fatally terminated 5 months later due to lung metastasis.
Amputation ; Femur ; Fibrosarcoma ; Lung ; Neoplasm Metastasis ; Osteosarcoma ; Pelvis ; Rickets ; Rickets, Hypophosphatemic ; Vitamins

No abstract available.
Cleft Lip* ; Congenital Abnormalities* ; Skin*

No abstract available.
Meningitis, Cryptococcal*

Avascular necrosis of the femoral head is generally localized in the anterosuperior aspect of the head. The posterior part of the head often retains a normal, smooth contour containing healthy cartilage even in much advanced cases. Because of this anatomical localization of the lesion, Y. Sugioka has devised a transtrochanteric rotational osteotomy in young and middle-aged person, which reduces the weight-bearing force on the necrotic area, and transfers shearing forces to the healthy posterior cartilage of the femoral head to prevent progressive collapse of the articular surface and improve alignment when articular collapse followed femoral head subluxiation. The authors reviewed 13 cases of Sugioka operation, performed at the Department of Orthopedic Surgery, Seoul National University Hospital during the period from December 1983 to Feburary 1986 and the following results were obtained. 1. There were 11 males and 1 female. The average age of the 13 cases was 33.3 years. 2. In all the cases of Grade I and Grade II hips, there was not progressiveness of necroticarea of femur heads. 3. By U.C.L.A. hip rating system, preoperative mean scores of pain, walking ability, function and activity were 2.5, 5.7, 4.8 and 4.3, respectively, while postoperative scores of these were increased to 7.7, 9.5, 8.4 and 7.8, respectively. 4. Average ranges of motion of hip joint were increased more postoperatively than preoperatively.
Cartilage ; Female ; Femur Head ; Head ; Hip ; Hip Joint ; Humans ; Male ; Necrosis ; Orthopedics ; Osteotomy ; Seoul ; Walking ; Weight-Bearing

We report a 51-year-old Vietnam War veteran with an unusual variant of macular amyloidosis presenting as poikilodermatous skin lesions. The extensive mottled brown pigmentation was checkered with small hypopigmented or normal skin-colored spots and intermingled with telangiectasia. Skin biopsy revealed subepidermal amyloid deposits. There was no evidence of extracutaneous involvements. This case could be easily confused with other true poikiloderma lesions.
Amyloidosis/pathology* ; Journal Article ; Human ; Male ; Middle Age ; Skin Diseases/pathology*

PURPOSE: Combined chemoradiotherapy is standard management for locally advanced non-small cell lung cancer (LA-NSCLC), but standard treatment for elderly patients with LA-NSCLC has not been confirmed yet. We evaluated the feasibility and efficacy of concurrent chemoradiotherapy (CCRT) for elderly patients with LA-NSCLC. MATERIALS AND METHODS: Among patients older than 65 years with LA-NSCLC, 36 patients, who underwent CCRT were retrospectively analyzed. Chemotherapy was administered 3-5 times with 4 weeks interval during radiotherapy. Thoracic radiotherapy was delivered to the primary mass and regional lymph nodes. Total dose of 54-59.4 Gy (median, 59.4 Gy) in daily 1.8 Gy fractions and 5 fractions per week. RESULTS: Regarding the response to treatment, complete response, partial response, and no response were shown in 16.7%, 66.7%, and 13.9%, respectively. The 1- and 2-year overall survival (OS) rates were 58.2% and 31.2%, respectively, and the median survival was 15 months. The 1- and 2-year progression-free survivals (PFS) were 41.2% and 19.5%, respectively, and the median PFS was 10 months. Regarding to the toxicity developed after CCRT, pneumonitis and esophagitis with grade 3 or higher were observed in 13.9% (5 patients) and 11.1% (4 patients), respectively. Treatment-related death was not observed. CONCLUSION: The treatment-related toxicity as esophagitis and pneumonitis were noticeably lower when was compared with the previously reported results, and the survival rate was higher than radiotherapy alone. The results indicate that CCRT is an effective in terms of survival and treatment related toxicity for elderly patients over 65 years old with LA-NSCLC.
Aged ; Carcinoma, Non-Small-Cell Lung ; Chemoradiotherapy ; Disease-Free Survival ; Esophagitis ; Humans ; Lymph Nodes ; Pneumonia ; Retrospective Studies ; Survival Rate

No abstract available.
Purpura, Schoenlein-Henoch*

Objective: Viral infections play an important role in the development of schizophrenia, inducing the faulty immunological responses and aberrant inflammation. IFN-γ-inducible protein 16 (IFI16) is an immunological DNA sensor against viral infections, triggering the inflammatory responses. In this study, we investigated an association between putative promoter single nucleotide polymorphisms (SNPs) and haplotypes of IFI16 and schizophrenia. Methods: A total of 280 schizophrenia patients and 427 control subjects were recruited in this study. We genotyped three promoter SNPs (rs1465175, rs3754464, rs1417806) using direct sequencing. Associations of SNPs and haplotypes of IFI16 with schizophrenia were analyzed. The promoter activities on the haplotypes of IFI16 were measured. Results: The T allele of rs1465175 and the C allele of rs1417806 were protectively associated with schizophrenia (p=0.021 on rs1465175; p=0.016 on rs1417806), whereas the G allele of rs3754464 was associated with an increased risk of schizophrenia (p=0.019). In haplotype analysis, a significant association between the GGA haplotype and schizophrenia was shown (p=0.013). Moreover, we found that the GGA haplotype elevated the promoter activity compared to the GAA haplotype, whereas the TAC haplotype reduced that. Conclusion The promoter SNPs and haplotypes of IFI16 may contribute to the susceptibility of schizophrenia, affecting the promoter activity of IFI16.

Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

No abstract available.
Child* ; Eosinophils* ; Humans ; Immunoglobulin E* ; Skin Tests* ; Skin*