The evolving management of blunt hepatic trauma in a rural setting

Over the past decade, a nonoperative approach toward the management of blunt hepatic trauma has become prevalent at most major urban trauma centers. To determine the applicability of the nonoperative approach in a rural setting, a 10-year retrospective review was conducted at a level I rural university-based trauma center. The Census Bureau defines ruralized areas to provide a better separation of urban and rural territory and population. A ruralized area is composed of one or more places and the adjacent surrounding territory that together have a maximum of 50,000 persons. West Virginia University is classified as a rural academic medical center and is situated in Morgantown, whose permanent population does not exceed 35,000. All patients with documented blunt hepatic trauma between July 1990 and June 2000 were identified and reviewed. To evaluate evolving trends, the patients were divided into two groups: group A (July 1, 1990-June 30, 1995) and group B (July 1, 1995-June 30, 2000). There were 236 patients with documented blunt hepatic trauma identified between July 1, 1990, and June 30, 2000). Overall, 70 per cent of patients were managed conservatively. When comparing the two groups, statistical significance was obtained in mean hospital length of stay (LOS) [19.8 days A vs. 9.1 days B (P < 0.0001)]; mean intensive care unit (ICU) days [15.2 days A vs. 5.3 days B (P < 0.0001)], blood transfusion [10 units A vs. 4.2 units B (P < 0.0016)], number of patients requiring surgery [52 (46%) A vs. 37 (30%) B (P < 0.022)]. There was only one death associated with nonoperative management. We have shown a definite trend toward nonoperative management of blunt hepatic trauma in a rural setting over the past decade. More than 70 per cent of our liver injury patients over the past 5 years have been managed nonoperatively, with statistically significant reductions in hospital LOS, ICU LOS, and transfusion requirements. We have found a definite trend over the past decade toward nonoperative management of blunt hepatic trauma in a rural setting. The rural setting with a delay in transport time to level I trauma center also did not significantly affect the outcome of the patients with nonoperative management of liver injuries. Approximately 78 per cent of our liver injury patients over the past 5 years have been managed nonoperatively and are associated with statistically significant reductions in hospital and ICU LOS and transfusion requirements.     

Prevalence of WT1 mutations in a large cohort of patients with steroid-resistant and steroid-sensitive nephrotic syndrome

BACKGROUND: Nephrotic syndrome (NS) represents the association of proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Steroid-resistant nephrotic syndrome (SRNS) is defined by primary resistance to standard steroid therapy. It remains one of the most intractable causes for end-stage renal disease (ESRD) in the first two decades of life. Sporadic mutations in the Wilms' tumor suppressor gene WT1 have been found to be present in patients with SRNS in association with Wilms' tumor (WT) and urinary or genital malformations, as well as in patients with isolated SRNS. METHODS: To further evaluate the incidence of WT1 mutations in patients with NS we performed mutational analysis in 115 sporadic cases of SRNS and in 110 sporadic cases of steroid-sensitive nephrotic syndrome (SSNS) as a control group. Sixty out of 115 (52%) patients with sporadic SRNS were male, 55/115 (48%) were female. Sex genotype was verified by haplotype analysis. Mutational analysis was performed by direct sequencing and by denaturing high-performance liquid chromatography (DHPLC). RESULTS: Mutations in WT1 were found in 3/60 (5%) male (sex genotype) cases and 5/55 (9%) female (sex genotype) cases of sporadic SRNS, and 0/110 (0%) sporadic cases of SSNS. One out of five female patients with mutations in WT1 developed a WT, 2/3 male patients presented with the association of urinary and genital malformations, 1/3 male patients presented with sexual reversal (female phenotype) and bilateral gonadoblastoma, and 4/5 female patients presented with isolated SRNS. CONCLUSION: According to the data acquired in this study, patients presenting with a female phenotype and SRNS and male patients presenting with genital abnormalities should especially be screened to take advantage of the important genetic information on potential Wilms' tumor risk and differential therapy. This will also help to provide more data on the phenotype/genotype correlation in this patient population.     

Patients with mutations in NPHS2 (podocin) do not respond to standard steroid treatment of nephrotic syndrome

Nephrotic syndrome (NS) represents the association of proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Steroid-resistant NS (SRNS) is defined by primary resistance to standard steroid therapy. It remains one of the most intractable causes of ESRD in the first two decades of life. Mutations in the NPHS2 gene represent a frequent cause of SRNS, occurring in approximately 20 to 30% of sporadic cases of SRNS. On the basis of a very small number of patients, it was suspected that children with homozygous or compound heterozygous mutations in NPHS2 might exhibit primary steroid resistance and a decreased risk of FSGS recurrence after kidney transplantation. To test this hypothesis, NPHS2 mutational analysis was performed with direct sequencing for 190 patients with SRNS from 165 different families and, as a control sample, 124 patients with steroid-sensitive NS from 120 families. Homozygous or compound heterozygous mutations in NPHS2 were detected for 43 of 165 SRNS families (26%). Conversely, no homozygous or compound heterozygous mutations in NPHS2 were observed for the 120 steroid-sensitive NS families. Recurrence of FSGS in a renal transplant was noted for seven of 20 patients with SRNS (35%) without NPHS2 mutations, whereas it occurred for only two of 24 patients with SRNS (8%) with homozygous or compound heterozygous mutations in NPHS2. None of 29 patients with homozygous or compound heterozygous mutations in NPHS2 who were treated with cyclosporine A or cyclophosphamide demonstrated complete remission of NS. It was concluded that patients with SRNS with homozygous or compound heterozygous mutations in NPHS2 do not respond to standard steroid treatment and have a reduced risk for recurrence of FSGS in a renal transplant. Because these findings might affect the treatment plan for childhood SRNS, it might be advisable to perform mutational analysis of NPHS2, if the patient consents, in parallel with the start of the first course of standard steroid therapy.     

Acute liver transplant rejection: incidence and the role of high-doses steroids

The aim of this study was to assess the incidence of acute rejection (AR), and the efficacy of high doses of steroids during induction of immunosuppression for AR treatment. Fifty-five patients (33.5%) experienced AR episodes in our series; but, there were no deaths or retransplantations related to AR. The median time from liver transplantation to AR was 18.5 days (range, 2-351 days). In the group with the initial dose of methylprednisolone (MP) 0.05). After 1-year observation, liver function tests were similar in both AR and non-AR groups. The only biochemical parameter that was significantly lower in the non-AR group was the aspartate aminotransferase (AST). Liver function tests determined after 1-year follow-up were not significantly different between the groups with AR treated with doses of MP lower versus higher than 1.25 g. However, liver function tests in the group treated for AR with higher doses of MP were slightly better than in the remaining subjects. Recurrence of AR occurred in 5 cases in the group with lower doses of MP (1.25 g). A relatively low dose of MP was effective to treat AR. The tendency of AR patients treated with higher dose of MP to display better liver function needs further investigation. However, AR does not seem to affect later liver function.     

Comparison of 44/107L one-step immunocapture enzyme-immunoassay and time-resolved fluoroimmunoassay for influenza A diagnosis

One-step immunocapture enzyme-immunoassay (EIA) was compared with time-resolved fluoroimmunoassay (TR-FIA) for rapid diagnosis of influenza A infection by antigen detection. The high-affinity monoclonal antibodies (MAbs) recognising two independent epitopes on the conservative nucleoprotein were used for capture (MAb 44) and detection (MAb 107L) of antigen by both assays. The detection limit for purified recombinant influenza A virus nucleoprotein was approximately 10 pg by EIA and 5 pg by TR-FIA. The performance of the methods was evaluated by testing 43 known positive and 50 negative clinical specimens (nasopharyngeal washes and aspirates). The sensitivity and specificity was 93% and 92% for EIA and 100% and 98% for TR-FIA, respectively, in comparison to the reference A3/A1 TR-FIA. The relationship of 44/107L immunoassays has been evaluated: in comparison to 44/107L TR-FIA (100%), EIA confirmed 93% of positive and 94% of negative samples. In conclusion, the capture-detector pair of MAbs 44 and 107L can be used for the sensitive detection of influenza A viral antigen in clinical samples by both immunocapture methods. Despite the slightly lower accuracy of the EIA, widespread availability and economy of the EIA methodology makes it an advantageous alternative for the laboratory diagnosis of influenza A virus infections.     

Über einen der Parakeratosis variegata (Unna) bzw. Pityriasis lichenoides chronica (Neisser-Juliusberg) nahestehenden eigentümlichen Fall

Ohne ZusammenfassungHiezu Taf. XXX u. XXXI.