Vesicocervical fistula: an unusual complication of vesicocaginal fistula repair

A rare case of vesicocervical fistula following repair of a vesicovaginal fistula is presented. The patient complained of cyclical menouria since the first repair done 15 years ago and gradually worsening urinary incontinence. A laparoscopic assisted repair of the fistula was performed and the patient is fully continent at 12-months follow up.An erratum to this article can be found at     

Limited capacity of the nuclear matrix to bind telomere repeat binding factor TRF1 may restrict the proliferation of mortal human fibroblasts

The maintenance of telomere integrity is essential for prolonged cell proliferation, and failure in this mechanism is a most consistent manifestation of cellular senescence. In this study, we investigated the role of telomere repeat binding factor (TRF1) in the proliferation of human fibroblasts. TRF1 expression is upregulated in a large variety of immortal human cells and supports de novo telomere formation in a dose-dependent manner. These observations suggest that the suppression of TRF1 might limit telomere maintenance and thus the life span of mortal cells. However, primary fibroblasts ectopically overexpressing TRF1 were unable to avoid senescence. On the other hand, exogenously expressed TRF1 in primary fibroblasts neither supported de novo telomere formation nor bound to the nuclear matrix as tightly as observed in immortal cells that show upregulated TRF1 expression. We present evidence suggesting that mortal human cells lack specific ligand(s) that anchor TRF1 to the nuclear matrix and that this contributes to their limited lifespan.     

Down syndrome: still a social stigma

Down Syndrome (DS) is a commonly occurring chromosomal abnormality. The incidence increases with advancing maternal age over 35 years. Over the last three decades, tremendous progress has been made in the medical and surgical treatment of these infants. Nationally, a great deal of resources are allocated to DS infants to improve their growth and development. Yet, the perception remains that the DS infant is still not openly accepted by parents and society, as illustrated by the presented cases. This lack of acceptance creates many complex ethical challenges in treating such babies, starting with fetal diagnosis of the disorder in the womb and moving through early stages after the birth of the baby. We argue that health professionals have the responsibility to help make public attitudes more accepting of Down Syndrome. Professionals should encourage social and community involvement of these children. The National Association for Down Syndrome should be contacted periodically to promote activities to enhance public awareness. To the end of greater acceptance, we suggest a ritual at birth that might improve the acceptance of the DS child into the family and the community and hence help improve social attitudes toward Down Syndrome.     

A mouse model of episodic ataxia type-1

Episodic ataxia type-1 (EA1) is a dominant human neurological disorder characterized by stress-induced attacks of ataxia. EA1 is caused by mutations in the voltage-gated potassium channel Kv1.1, and affected individuals are heterozygous. Here we introduced the V408A EA1 mutation into mice using homologous recombination. In contrast to Kv1.1 null mice, homozygous V408A/V408A mice died after embryonic day 3 (E3). V408A/+ mice showed stress-induced loss of motor coordination that was ameliorated by acetazolamide, a carbonic anhydrase inhibitor that minimizes EA1 symptoms in human patients. We made electrophysiological recordings from cerebellar Purkinje cells in both V408A/+ mice and their wild-type littermates. V408A/+ mice showed a greater frequency and amplitude of spontaneous GABAergic inhibitory postsynaptic currents (IPSCs) than did wild type; however, the amplitude or frequency of miniature IPSCs and the basket cell firing frequency did not differ between groups. The stress-induced motor dysfunction in V408A mice is similar to that of family members harboring the EA1 allele, and our findings suggest that these behavioral changes are linked to changes in GABA release.     

Pathologic evaluation of a spherical polyvinyl alcohol embolic agent in a porcine renal model

PURPOSE: To evaluate the effects of a spherical embolic agent consisting of polyvinyl alcohol (PVA) and to compare this agent with commercially available embolization agents. MATERIALS AND METHODS: Eleven miniature pigs were included in the study population. The upper poles of both kidneys were selected as the target organs for embolization. PVA spheres (700-900 micro m) were used in nine kidneys, PVA particles (500-710 micro m) were used in six kidneys, gelatin spheres (700-900 micro m) were used in five kidneys, and gold-colored gelatin spheres (700-900 micro m) were used in three kidneys. Two animals were killed immediately after embolization. In the remaining animals, angiography was performed before sacrifice 7 days (in five pigs) or 28 days (in four pigs) after embolization. Pathologic and histologic evaluation of the kidneys was performed. RESULTS: All agents resulted in target vessel occlusion and end-organ infarction. All arteries embolized with spherical agents were recanalized at follow-up angiography. In vessels embolized with PVA particles, the occluding plug consisted of thrombus and PVA. In vessels embolized with spherical agents, the occluding plug consisted mostly of the embolic agent. PVA spheres were associated with the mildest inflammatory responses at 7 and 28 days when compared with PVA particles and gelatin-based microspheres. Arterial wall destruction was seen to a greater extent in kidneys embolized with gelatin-based microspheres than in those embolized with PVA-based agents. CONCLUSIONS: The spherical, PVA-based embolization agent resulted in target organ infarction and temporary arterial occlusion. The inflammatory response to PVA spheres was significantly less aggressive than the response to other agents tested. Further study with clinical and long-term pathologic follow-up is suggested to determine if these findings may have favorable clinical implications for patients undergoing embolization procedures.     

The absence of chronic rejection in pediatric primary liver transplant patients who are maintained on tacrolimus-based immunosuppression: a long-term analysis

BACKGROUND: Although the outcome of liver transplantation has improved significantly during the past two decades, graft loss caused by chronic rejection after liver transplantation still occurs in 2% to 20% of recipients. The overall incidence of chronic rejection is also reported to be low in adult recipients, and risk factors have been identified. Chronic rejection is associated with the inability to maintain baseline immunosuppression. Additionally, the diagnoses of primary biliary cirrhosis, primary sclerosing cholangitis, autoimmune hepatitis, hepatitis B virus, and hepatitis C virus, common indications for liver transplantation in adults, are associated with a higher incidence of chronic rejection. Fortunately, these diagnoses are rarely seen in children. Little is known about chronic rejection in long-term pediatric liver transplant survivors. The purpose of this longitudinal study was to examine the incidence of biopsy-proven chronic rejection in long-term survivors of primary pediatric liver transplantation under tacrolimus-based immunosuppression. METHODS: From October 1989 to December 1992, 166 children (boys=95, girls=71; mean age=5.0+/-2.9 years) received a primary liver transplant. These patients were followed until March 2000 with a mean follow-up of 9+/-0.8 (range, 7.4-10.4) years. All liver biopsy specimens and explanted grafts were evaluated for evidence of chronic rejection using the International Banff Criteria. RESULTS: The mortality rate during the follow-up period was 15% (n=25). Retransplantation was required in 11% (n=18) of recipients. Actuarial patient and graft survival rates at 10 years were 84.9% and 80.1%, respectively. There were 535 liver biopsy samples available for evaluation, including the 18 explanted allografts. Biopsy specimens of three other functioning allografts showed evidence of chronic rejection. Immunosuppression had been discontinued or drastically reduced in these recipients because of life-threatening infections, noncompliance, or both. On restoring baseline immunosuppression, all three children had normalized liver function and the allografts were maintained; the liver transplant patients who are alive currently have normal liver functions. CONCLUSION: The findings of this study suggest that chronic rejection does not occur in pediatric liver transplant recipients receiving tacrolimus-based immunosuppression, provided baseline immunosuppression is maintained.     

Spontaneous coronary artery dissection in a patient with systemic lupus erythematosis

Spontaneous coronary artery dissection (SCAD) is an uncommon condition that may lead to sudden coronary artery occlusion resulting in a fatal acute myocardial infarction. It usually affects young to middle age women. A Medline search from 1966 to 2001 (using keywords: coronary artery dissection and systemic lupus erythematosis) revealed no prior reports of coronary dissection in a patient with systemic lupus erythematosis (SLE). We describe a 48-year old woman with SLE who sustained a fatal spontaneous left main coronary artery dissection. Coronary angiogram was notable for marked variability in the size of coronary lumen from systole to diastole. This case demonstrates the need to consider SCAD in the evaluation of chest pain and myocardial infarction in patients with SLE. Furthermore, in the absence of classical angiographic findings of coronary dissection, a detailed review of phasic changes in coronary lumen during a cardiac cycle could help reach this diagnosis.