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

BACKGROUND AND OBJECTIVES: Coronary artery stenting actually shows a high efficacy in the treatment of coronary heart disease, but has the major limitation of restenosis. The ethylene-vinyl acetate copolymer (EVA), a biocompatible nondegradable copolymer, has been employed as a rate-controlling membrane in several drug delivery systems. Herein, the feasibility of an EVA-coated coronary stent was evaluated as a possible route for localized drug delivery. MATERIALS AND METHODS: A total of 15 rabbits were employed in this study. An uncoated stent was implanted into the non-diseased iliac artery in six rabbits, and an EVA-coated stent into a further nine. On the 30th day following the stent implantations, stented segments of the iliac arteries were removed for histological processing and morphometric analysis. RESULTS: The mean neointimal area of the uncoated and coated groups were 1.009 and 1.011 mm2 (p=0.56), respectively. No inflammatory cells were found in coated group. There were no apparent differences between the two groups. CONCLUSION: The results from this study have demonstrated that an EVA-coated coronary stent might be an appropriate method for the controlled-release of a drug.
Biological Availability* ; Coronary Disease ; Coronary Vessels ; Drug Delivery Systems ; Iliac Artery* ; Membranes ; Polyvinyls ; Rabbits ; Stents*

Hydatidiform moles are generally separated into two classifications. Complete hydatidiform moles are characterized by cystic swelling of all villi, often pronounced trophoblastic hyperplasia, lack of fetal parts, all 46 chromosomes of paternal origin, and a major risk for persistent trophoblastic tumor. Partial hydatidiform moles appear to be a milder version of complete moles with both normal and cystic villi, focal trophoblastic hyperplsia, a fetus or indication of previous fetal existence, 69 chromosomes with a maternal contribution, and a malignant potential less than described for complete moles. Hydatidiform mole with coexistent fetus is a very rare phenomenon, with an estimated incidence of 0.005 to 0.01 percent of all pregnancies. Due to advances in cytogenetics and ultrasonography, now permit the diagnosis of this pregnancy antenatally. However this unusual pregnancy has the risks of malignant change and severe medical complications, so it is a dilemma to decide continuation or termination of pregnancy. We experienced a case of partial hydatidiform mole with coexistent live fetus, which was diagnosed by ultrasonography at 12 gestational weeks, and confirmed normal karyotype (diploid) of the coexistent fetus. A brief reviews of related literature was done.
Classification ; Cytogenetics ; Diagnosis ; Diploidy ; Female ; Fetus* ; Hydatidiform Mole* ; Hyperplasia ; Incidence ; Karyotype ; Pregnancy ; Trophoblastic Neoplasms ; Trophoblasts ; Ultrasonography

We have experienced a case of hepatoblastoma in 22 months old male infant who presented with abdominal distention and hepatomegaly. Diagnosis was confirmed by X-ray of abdomen, liver scan and histological findings. A brief review of literature was also presented.
Abdomen ; Diagnosis ; Hepatoblastoma* ; Hepatomegaly ; Humans ; Infant ; Liver ; Male

STUDY DESIGN: Retrospective study. PURPOSE: To investigate influence of bone mineral density (BMD) on the surgical correction of lumbar degenerative kyphosis (LDK). OVERVIEW OF LITERATURE: No studies so far have reported the influence of BMD on the surgical correction of LDK. METHODS: Forty LDK patients with more than 2 years follow-up were studied. Pelvic incidence (PI), pelvic tilt, sacral slope, sagittal vertical axis (SVA), lumbar lordosis (LL), and thoracic kyphosis were measured preoperatively, immediate postoperatively and at final follow-up. Adverse outcomes: proximal adjacent fractures, sagittal decompensation, pseudoarthrosis, and cage subsidence were documented. RESULTS: There were 37 females and 3 males. Average age was 65.1+/-4.5 years and mean follow-up was 34.2+/-16.7 months. 42.5% were Takemitsu type 3 curves, 27.5% type 2, 20.0% type 4 and 10.0% type 1. 37.5% had osteopenia, 40.0% osteoporosis and 22.5% had severe osteoporosis. SVA improved from 237.0+/-96.7 mm preoperatively to 45.3+/-41.8 mm postoperatively (p=0.000). LL improved from 10.5degrees+/-14.7degrees to -40.6degrees+/-10.9degrees postoperatively (p=0.000). At final follow-up SVA deteriorated to 89.8+/-72.2 mm and LL to 34.7degrees+/-15.8degrees (p=0.000). The association between late sagittal decompensation, pseudoarthrosis, or proximal adjacent fractures and osteoporosis was insignificant. The difference between immediate postoperative LL and PI (PIDiff) had a significant association with sagittal decompensation and pseudoarthrosis. CONCLUSIONS: Osteoporosis did not influence the degree of correction, late sagittal decompensation, proximal adjacent fractures, and pseudoarthrosis in LDK. PIDiff had a significant association with sagittal decompensation and pseudoarthrosis.
Animals ; Axis, Cervical Vertebra ; Bone Density* ; Bone Diseases, Metabolic ; Female ; Follow-Up Studies* ; Humans ; Incidence ; Kyphosis* ; Lordosis ; Male ; Osteoporosis ; Pseudarthrosis ; Retrospective Studies

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