Nephrotic syndrome due to focal segmental glomerulosclerosis occurring in early pregnancy.

Physiological proteinuria is common during pregnancy, but pathological proteinuria may also occur. Nephrotic range proteinuria most commonly occurs due to preeclampsia, but primary renal disease may also occur. We present a case of a woman who presented with nephrotic range proteinuria and nephrotic syndrome at an early stage of her second pregnancy. Due to proteinuria, haematuria, progressive symptoms and high need for medical treatment, renal biopsy was performed and she was noted to have focal segmental glomerulosclerosis (FSGS). She commenced immunosuppression but remained nephrotic during pregnancy. She delivered a healthy baby boy at 35 weeks gestation. Postpartum she remained nephrotic, and developed further complications requiring renal replacement therapy. Despite a successful pregnancy, this case of FSGS highlights that despite aggressive treatment FSGS in pregnancy is often associated with adverse renal outcome. We discuss investigations, including renal biopsy and empiric treatment options in this report.

Neuropsychiatric Outcome of an Adolescent Who Received Deep Brain Stimulation for Tourette's Syndrome.

This case study followed one adolescent patient who underwent bilateral deep brain stimulation of the centromedian parafascicular complex (CM-Pf) for debilitating, treatment refractory Tourette's syndrome for a period of 1.5 years. Neurocognitive testing showed no significant changes between baseline and follow-up assessments. Psychiatric assessment revealed positive outcomes in overall adaptive functioning and reduction in psychotropic medication load in this patient. Furthermore, despite significant baseline psychiatric comorbidity, this patient reported no suicidal ideation following electrode implantation. Deep brain stimulation is increasingly being used in children and adolescents. This case reports on the positive neurologic and neuropsychiatric outcome of an adolescent male with bilateral CM-Pf stimulation.

Clinical and biochemical outcome of hepatorenal transplantation for hereditary systemic amyloidosis associated with apolipoprotein AI Gly26Arg.

BACKGROUND: Treatment of systemic amyloidosis comprises measures to support failing organ function coupled with attempts to reduce the supply of the respective amyloid fibril precursor protein. Orthotopic hepatic transplantation is effective in familial amyloid polyneuropathy associated with variant transthyretin, because this protein is produced almost exclusively in the liver. Hepatic transplantation has not been performed in hereditary apolipoprotein AI (apoAI) amyloidosis, and the liver's contribution to plasma apoAI levels has not been determined in vivo. METHODS: A 57-year-old Irish man with hereditary systemic amyloidosis associated with apoAI Gly26Arg, which had led to end-stage renal failure and progressive liver dysfunction, underwent hepatorenal transplantation. His outcome was followed clinically and his amyloid deposits were monitored with serum amyloid P component scintigraphy. The proportion of variant apoAI in the plasma was estimated by quantitative isoelectric focusing before and after liver transplantation. RESULTS: Plasma levels of variant apoAI decreased by 50% after liver transplantation, and the patient was asymptomatic 2 years after surgery. Subclinical amyloid deposits that were present in his spleen and heart preoperatively have regressed and stabilized respectively. CONCLUSIONS: Orthotopic liver transplantation substantially reduces the supply of the amyloid fibril precursor protein in hereditary apoAI amyloidosis, and the excellent outcome in this patient probably reflects the balance between deposition and turnover of amyloid having been altered in favor of the latter. These findings support the use of liver transplantation in patients with hereditary apoAI amyloidosis who develop hepatic dysfunction.

Female rats exhibit greater susceptibility to early alcohol-induced liver injury than males.

It is known that women develop hepatic injury more rapidly and with exposure to less ethanol than men; however, mechanisms remain unclear. The purpose of this study was to determine if an enteral alcohol delivery model could be used to study susceptibility of females to alcohol-induced liver injury. Male and female Wistar rats (age- or weight-matched) were given ethanol (11-12 g.kg-1.day-1) continuously for up to 4 wk via intragastric feeding, and control rats received a high-fat diet without ethanol. There were no significant differences in body weight among the groups studied. Furthermore, mean ethanol concentrations, their cyclic pattern in urine, and rates of ethanol elimination were also not different between the genders under these conditions. Ethanol treatment elevated serum aspartate aminotransferase levels in male rats to 126 +/- 10 IU/l after 4 wk. In females, however, values increased more rapidly and reached significantly higher values at 4 wk (168 +/- 18 IU/l). Steatosis, inflammation, and necrosis assessed histologically also developed more rapidly and were more severe in females than males. Steatosis due to ethanol exposure, which was localized in centrilobular areas in males, was panlobular in the female. Moreover, endotoxin in plasma, intercellular adhesion molecule 1 expression in hepatic sinusoidal-lining cells, and the number of infiltrating inflammatory cells in the liver were 2-2.5-fold greater in females than males. These changes possibly account for increased hepatic injury due to ethanol in the female.

Impact of changing attitudes in carotid surgery on community hospital practice.

In 1985, institutional guidelines for the evaluation and performance of carotid surgery were established in our community hospital. During the 5-year period from 1985 through 1989, 159 carotid reconstructions were done. There were four major strokes (3%), one eventually resulting in death, with the second death in this series from a myocardial infarction (mortality 1%). The combined mortality/major stroke morbidity incidence was 3%. Three transient ischemic attacks (2%) postoperatively cleared promptly without residua. During the latter 1980s, an increasing number of vascular surgeons were doing less carotid surgery. Monitoring institutional quality assurance and individual surgeon performance within the community hospital is becoming a reality. Our experience with institutional guidelines for the evaluation and conduct of carotid surgery, together with an assessment of results and ongoing individual surgeon performance, is presented. Maintaining acceptable morbidity and mortality statistics can be enhanced by having a plan for assessment, management, and concurrent review.

Preliminary experience with a large scale vascular registry.

The preliminary study carried out on a voluntary basis, has illustrated the feasibility of a large scale vascular registry serving as a benchmark against which a standard of practice of vascular surgery can be assessed. An initial experience with 165 surgeons active in the clinical practice of surgery (contributing 8,800 major vascular reconstructive cases in a 9 month period) suggests access to an enormous data base of vascular surgery practice against which community standards may be measured.

Guidelines for performance of transluminal angioplasty in the hospital.

The expanding use of balloon catheter dilatation of arterial lesions has demonstrated its effectiveness as a relatively simple therapeutic method to improve the initial patency of focal, proximally located atherosclerotic stenoses. It is recommended that the vascular surgeon assume a leadership role in establishing guidelines within each institution for the assessment and conduct of transluminal angioplasty. The careful selection of patients with an appropriate indication for the procedure is a preferred alternative to its improper and indiscriminate use.

Transluminal angioplasty: adjunct or gimmick?

An experience with transluminal dilatation using the Gruntzig catheter in 94 patients during the past 2 years with follow-up studies of 1 month to 2 years has provided certain guidelines for applying this technique to the management of peripheral vascular atherosclerosis. While the immediate success rate for the use of transluminal dilatation as a method of improving blood flow is encouraging, further studies are necessary to determine the mechanism of healing and the duration of patency and compare them with present vascular reconstructive procedures. A cooperative effort between surgeons and their non-surgical colleagues is necessary for optimal assessment and use of this technique.