Erythropoietic protoporphyria (EPP) is an autosomal dominant condition due to decreased activity of ferrochelatase. The disease is characterized by a wide range of photocutaneous changes and occasionally by liver disease. The level of protoporphyin is raised in erythkocytes and it may also be increased in the feces. We report herein a case of EPP present in a family which was diagnosed by a high free erythrocyte protoporphyrin (FEP) count.
Erythrocytes ; Feces ; Ferrochelatase ; Humans ; Liver Diseases ; Protoporphyria, Erythropoietic*

Erythropoietic protoporphyria (EPP) is an autosomal dominant condition due to decreased activity of ferrochelatase. The disease is characterized by a wide range of photocutaneous changes and occasionally by liver disease. The level of protoporphyin is raised in erythkocytes and it may also be increased in the feces. We report herein a case of EPP present in a family which was diagnosed by a high free erythrocyte protoporphyrin (FEP) count.
Erythrocytes ; Feces ; Ferrochelatase ; Humans ; Liver Diseases ; Protoporphyria, Erythropoietic*

Five medical students in the fourth-year took anatomy as their elective courses for 1 month. They dissected one cadaver, and investigated Digital Report, under the course subjects as follows; (1) the shape of thyroid gland and the location of its isthmus, (2) the branches of left and right coronary arteries, (3) the number of blood vessels and bronchi on the hilum of lung, (4) topographical relationship of the renal vessels and ureter, and the shape of the renal pelvis, (5) the location and attachment of the appendix, (6) the penetration of median nerve through the pronator teres, (7) the sensory nerves and the extensor tendons on the dorsum of hand, (8) the branches of deep femoral artery. The pancreatic and live samples were processed and stained with H&E, for LM observation, since the individual had suffered from pancreatic cancer and got a Pylorus preserving pancreatico-duodenectomy (PPPD). At the last step of the elective course, students wrote small articles following the conventional method for writing manuscript. From the viewpoint of professor, the anatomy course for the fourth-year students were definitely different from that for first-year students, and had many positive effects in terms of anatomy education.
Appendix ; Blood Vessels ; Bronchi ; Cadaver ; Coronary Vessels ; Education ; Femoral Artery ; Hand ; Humans ; Kidney Pelvis ; Lung ; Median Nerve ; Pancreatic Neoplasms ; Pylorus ; Students, Medical* ; Tendons ; Thyroid Gland ; Ureter ; Writing

STUDY DESIGN: Retrospective study. PURPOSE: To evaluate the radiological and clinical results of three different methods in the deformity correction of a degenerative flat back. OVERVIEW OF LITERATURE: There are no comparative studies about different procedures in the treatment of degenerative flat back. METHODS: Sixty-four patients who consecutively underwent corrective surgery for degenerative flat back were reviewed. The operations were performed by three different methods: posterior-only (group P, n=20), one-stage anterior-posterior (group AP, n=12), and two-stage anterior-posterior with iliac screw fixation (group AP-I, n=32). Medical and surgical complications were examined and radiological and clinical results were compared. RESULTS: The majority of medical and surgical complications were found in group AP (5/12) and group P (7/20). The sagittal vertical axes were within normal range immediately postoperatively in all groups, but only group AP-I showed normal sagittal alignment at the final follow-up. Postoperative lumbar lordosis was also significantly higher in group AP-I than in group P or group AP and the finding did not change through the last follow-up. The Oswestry disability index was significantly lower in groups AP and AP-I than in group P at the final follow-up. Meanwhile, the operating time was the longest in group AP-I, and total amount of blood loss was larger in group AP-I and group AP than in group P. CONCLUSIONS: Anterior-posterior correction showed better clinical results than posterior-only correction. Two-staged anterior-posterior correction with iliac screw fixation showed better radiological results than posterior-only or one-staged anterior-posterior correction. Two-staged anterior-posterior correction with iliac screw fixation also showed a lower complication rate than one-staged anterior-posterior correction.
Animals ; Congenital Abnormalities* ; Follow-Up Studies ; Humans ; Kyphosis ; Lordosis ; Lumbar Vertebrae ; Reference Values ; Retrospective Studies ; Treatment Outcome

We performed L1 posterior vertebral columnar resection and posterior correction for Andersson's lesion and thoracolumbar kyphosis in an ankylosing spondylitis patient during motor evoked potential (MEP) monitoring. We checked MEP intra-operatively, whenever a dangerous procedure for neural elements was performed, and no abnormal findings were seen during surgery. After the operation, we examined neurologic function in the recovery room; the patient showed a progressive neurologic deficit and no response to MEP. After emergency neural exploration and decompression surgery, the neurologic deficit was recovered. We questioned whether to acknowledge the results of this case as a false negative. We think the possible reason for this result may be delayed development of paralysis. So, we recommend that MEP monitoring should be performed not only after important operative steps but also after all steps, including skin suturing, for final confirmation.
Decompression ; Emergencies ; Evoked Potentials, Motor ; Humans ; Kyphosis ; Neurologic Manifestations ; Paralysis ; Skin ; Spondylitis, Ankylosing

A retrogpective review of hematologic rnonitoring involving aggressive chemotherapy was careiyd out ta assese whetber there ia a predictable relatiorship between the white blood ce11 count end the platelet count as a refleetion of bone marrow toxicity and when maximum myeloauppression occur during a treatment program. This data revealed that the white blood cell and granulocyte levels are closely related and that myeloeuppression can oceur during any course of CAP(cyclophosphamide, adriamycin, and cisplatin), VBP(vinblastine, bleomycin, and cisplatin) chemotherspy in gynecological cancer. Thus, for these treatment regimena in gynecoldgical malignancies, the white blood cell and granulocyte count is sufficient for momtoing toxicity and adjusting future courses of chemotherapy. There are no bone merrow depresaions by the treatment regirnens for the gestational trophoblastic disease.
Bleomycin ; Bone Marrow ; Doxorubicin ; Drug Therapy* ; Equidae ; Gestational Trophoblastic Disease ; Granulocytes ; Humans ; Leukocytes ; Platelet Count

PURPOSE:BK virus associated nephropathy (BKVAN) affects 1-10% of kidney transplant (KT) patients and it produces a progressive destruction of allograft. Reducing immunosuppression is the only way to save the graft, while it needs tight monitoring of the graft rejection and graft survival is poorer in advanced case. Leflunomide has immunosuppressive effect and also antiviral activity. Addition of leflunomide may improve BK virus clearance and graft survival. METHODS:6 KT patients with biopsy proven BKVAN (Histological pattern B) were treated with leflunomide and reduced immunosuppression. All patients were monitored with serial determination of viral load in blood and graft function. RESULTS:BKVAN was diagnosed at 14 months (7-28) post transplant, at that time median serum creatinine concentration was 2.8 mg/dL (1.8-3.6). 12.5 months (6-16) later of leflunomide treatment, median serum creatinine was 2.3 mg/dL and no graft loss was found. CONCLUSION:Leflunomide therapy with reduced immunosuppression may be effective in the treatment for BKVAN.
Biopsy ; BK Virus ; Creatinine ; Graft Rejection ; Graft Survival ; Humans ; Immunosuppression ; Isoxazoles ; Kidney ; Kidney Transplantation ; Transplantation, Homologous ; Transplants ; Viral Load ; Viruses

STUDY DESIGN: Genetic screening of the estrogen receptor 2 (ESR2) genes in patients with ossification of the posterior longitudinal ligament (OPLL). OBJECTIVE: We studied the relationships between ESR2 gene polymorphisms and OPLL to understand the pathophysiology of OPLL. SUMMARY OF LITERATURE REVIEW: The OPLL has a strong genetic component. Several familial surveys and human leukocyte antigen (HLA) haplotype studies reveal that genetic background is an important component in the occurrence of OPLL and a large number of gene analysis studies were utilized to clarify the susceptible gene for OPLL, including COL11A2, BMP-2, TNF-alpha, NPPS, leptin receptor, transforming growth factor (TGF)-beta, Retinoic X receptor, ER, IL-1, PTH, and VDR have been performed. MATERIALS AND METHOD: Genomic deoxyribonucleic acid (DNA) samples obtained from 164 patients (93 men and 71 women) with OPLL and 219 control subjects, without the disease (105 men and 114 women) were amplified by polymerase chain reaction, and polymorphism genotypes were determined by the restriction endonuclease digestion. The distribution of genotypes was compared between the patients with the disease and the control subjects. RESULTS: The polymorphism of ESR2 [rs1256049, exon6, Val328Val, p=0.018, odd ratio (OR)=2.41, 95 confidence interval (CI)=1.15-5.02 in the recessive model] only showed statistically significant association between the control and the OPLL groups. The rest SNPs of ESR2 did not show any significant differences between the control and the OPLL groups. CONCLUSIONS: Estrogen receptor 2 (ESR2) gene polymorphisms (rs 1256049) was associated with OPLL. In future studies, we will perform target SNP chip between OPLL and candidate gene.
Digestion ; DNA ; DNA Restriction Enzymes ; Estrogen Receptor beta ; Estrogens ; Genetic Testing ; Genotype ; Haplotypes ; Humans ; Interleukin-1 ; Leukocytes ; Longitudinal Ligaments ; Male ; Ossification of Posterior Longitudinal Ligament ; Polymerase Chain Reaction ; Polymorphism, Single Nucleotide ; Receptors, Leptin ; Spine ; Succinimides ; Transforming Growth Factors ; Tumor Necrosis Factor-alpha

PURPOSE: To present our operative experiences with carpometacarpal (CMC) injuries, excluding thumb. MATERIALS AND METHODS: Thirty four fracture and dislocations of CMC joint excluding thumb were reviewed retrospectively. Emphases were placed on injury mechanisms, anatomical location, times between diagnosis and surgery, treatment and complications. RESULTS: The average age of patients was 31.5 years. 19 cases of axial loading by blow as an injury mechanism. The 5th CMC joint was found to be the most frequently involved single joint (18 cases of 34 cases). Dorsal dislocation of CMC joints was present in 12 cases. Comminution of the carpal or metacarpal bone was present in 18 cases. The average time to surgery was 6 days. Twenty-seven cases were operated upon by closed reduction and percutaneous pinning. Seven cases were treated by open reduction and internal fixation. In the last follow up period, a clinically full hand function was restored in 31 cases. Intermittent pain was present in 6 cases in which there was grip weakness in 4 cases and limitation of motion in 3 cases. However, all cases were able to activities of daily living. CONCLUSION: We obtained good outcomes in CMC joint injuries through the accurate diagnosis and proper operative treatment.
Activities of Daily Living ; Carpometacarpal Joints* ; Diagnosis ; Dislocations ; Follow-Up Studies ; Hand ; Hand Strength ; Humans ; Joints ; Retrospective Studies ; Thumb

PURPOSE: To analyze the results between PFN (Proximal Femoral Nail) and DHS (Dynamic Hip Screw) on the operative treatment of unstable intertrochanteric fractures retrospectively. MATERIALS AND METHODS: 35 cases of unstable intertrochanteric fractures (grouped 24 patients with DHS and 11 patients with PFN) who were taken the operations from Jan. 2001 to Mar. 2002 were analysed regarding to union state, union time, operation time, sliding length of lag screws, blood loss, postoperative complications and functional recovery scores by Sk?vron with ANOVA and multivariate linear regression. RESULTS: The means of union time were 17.9 weeks (DHS) and 17.0 weeks (PFN), sliding length of lag screws were 3.9 mm (DHS) and 2.1 mm (PFN), perioperative blood losses were 743 cc (DHS) and 736 cc (PFN), operation time were 93.4 minutes (DHS) and 102 minutes (PFN), and the functional recovery scores by Sk?vron were 71.8% (DHS) and 76.8% (PFN), respectively. The results of our study indicate that there were not statistically significant differences between PFN and DHS groups in treatment of unstable intertrochanteric fractures (p>0.05). But, there was less sliding of lag screws in PFN group in statistical significance (p<0.05). CONCLUSION: Authors think that PFN is one of the useful implants in treating unstable intertrochanteric fractures of the femur in regarding to sliding.
Femur* ; Hip Fractures ; Hip* ; Humans ; Linear Models ; Postoperative Hemorrhage ; Retrospective Studies