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This is the first report from Argentina of liver transplantation in patients with transthyretin related familial amyloidotic polyneuropathy. The aims of the study were to analyze the clinical characteristics of this new focus and evaluate the postoperative complications and long term follow up. Five of ten patients evaluated underwent liver transplantation. During the waiting period the polyneuropathy disability score in each patient progressed one or two stages. Pretransplant modified body mass index was 723. The procedure was done with full size grafts in four cases and a split right graft in one. All patients presented postoperative complications related to disease: severe edema of the legs, recurrent choledochal lithiasis, postoperative hernia, necrotizing fasciitis and ischemic rectosigmoidal perforation. Assessment of three patients after 20 months of transplantation showed improvement in somatic and mental symptoms. No improvement was noted in cardiac denervation and gastric stasis. Liver transplantation is a rational therapeutic option for transthyretin familial amyloidotic polyneuropathy in Argentina and should be indicated in earlier stages of the symptomatic disease to reduce the postoperative morbidity and mortality. Family studies and follow up of asymptomatic carriers will define the epidemiological behavior in this country and facilitate early therapeutic intervention.
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PMID:Liver transplantation in transthyretin familial amyloid polyneuropathy: first report from Argentina. 1061 53

Botulinic toxin (BT) is a new method of lowering intraurethral pressure in symptoms of obstructive voiding in patients with neurogenic dysfunction of the lower urinary tracts (LUT). Transperineal introduction of 100 units of BT type A (botox, Allergan) was used under electromyographic control into the external urethral sphincter of 9 patients (6 males and 3 females) with LUT neurogenic dysfunction aged 17 to 68 years (mean age 37.2 years). Two patients had subnormal detrusor contractility due to myelodysplasia and diabetic polyneuropathy, two other patients--non-incontinent striated urethral sphincter after hemorrhagic stroke and spinal contusion, five patients suffered from detrusor-sphincteral dyssynergia (DSD) resultant from Schmorl's hernia, multiple sclerosis, Charcot-Marie disease and ischemic stroke of the spinal cord. Three patients had cystostomic drainage. The rest of the patients complained of dysuria, three patients performed self-catheterization, mean volume of the residual urine was 170 ml (180-240 ml). In 10 days residual urine was not found in 2 patients with subnormal detrusor contractility and in 4 patients with DSD. Abdominal pressure fell from 75 to 39 cm, on the average. In DSD patients maximal detrusor pressure fell from 59 to 29 cm, on the average. Mean maximal urinary flow rate rose from 4.3 to 9.6 ml/s. In 20 days, on the average, suprapubic fistula healed in all the patients. In a month, therapeutic effect persisted in all the patients. Complications, side effects were not registered. BT treatment to induce adequate urine evacuation in neurological patients is a promising approach in neurourology. Further studies should find answers to questions about regimen of BT introduction, loss of sensitivity, new indications in urology.
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PMID:[Botulinic toxin in patients with neurogenic dysfunction of the lower urinary tracts]. 1545 54

Open abdomen is the final result of a variety of diseases and their treatment strategies. The aim of this article is to present systematically late complications after open abdominal therapy and our own treatment results from 2003 to 2005. The main diagnoses for open abdomen are persistent peritonitis, abdominal compartment syndrome, and abdominal injuries. A perioperative mortality rate of 10-56%, long stays at the ICU, and a mean of 3-5 reoperations are characteristic for the severity of such diseases. Late complications may include incisional hernia (47-78%), gastrointestinal and pancreatic fistulas (8-41%), postoperative delayed abscess (10-21%), polyneuropathy (21%), psychic disorders (24%), indigestion (12%), and ossification (17%). These postoperative disorders may range in severity from clinically less significant to therapy-relevant with surgical consequences. Despite the high morbidity, approximately 75% of surviving patients achieve good quality of life. In our opinion, this justifies the extensive treatment concepts and associated high costs.
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PMID:[Late complications of open abdomen]. 1673 81

A total of 198 neurological patients on physiotherapeutic rehabilitation participated in a questionnaire survey on their quality of life. The patients had diabetic polyneuropathy (n = 86), disorders in spinal blood circulation (n = 65), 47 patients were operated for discal hernia of the lumbar spine. It was found that all the responders suffer from physical, psychological, emotional and social sequelae of their diseases which deteriorate their quality of life. The severity of this deterioration depends on the form and stage of the disease, motor and sensitive disturbances. Rehabilitation improved subjective response, social, psychological and emotional parameters. Thus, the proposed questionnaires proved valid for assessment of physiotherapy efficacy in neurological patients.
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PMID:[Quality of life of neurological patients during therapy and rehabilitation]. 1675 37