Self-Mutilation in Neurodegeneration with Brain Iron Accumulation

Neurodegeneration with brain iron accumulation (NBIA) is the term applied to a heterogeneous group of disorders resulting in iron deposition in the basal ganglia. Well-known phenotypic features are progressive regression with extra pyramidal involvement and a variable course. A 10-year-old child born to consanguineous parents presented with progressive generalized opisthotonic dystonia, retrocollis, oromandibular dyskinesias, apraxia for swallowing, optic atrophy and severe self-mutilation of lips. MR imaging showed brain iron accumulation. Other causes of self-mutilation were excluded. Early infantile onset, ophisthotonic dystonia with oromandibular dyskinesias and characteristic MR images are suggestive of NBIA. There is only one case reported in the literature of self-mutilation in this condition.     

Effect of local mupirocin application on exit-site infection and peritonitis in an Indian peritoneal dialysis population.

BACKGROUND: Staphylococcus aureus-associated peritonitis and catheter exit-site infections (ESIs) are important causes of hospitalization and catheter loss in patients undergoing chronic peritoneal dialysis. Intranasal and topical use of mupirocin has been found to be an effective strategy in decreasing S. aureus-related infectious complications in persons who are carriers of S. aureus; however, there is no consensus regarding the prophylactic use of mupirocin irrespective of carrier status. We aimed to determine the potential effectiveness of application of mupirocin cream at the catheter exit site in preventing ESI and peritonitis irrespective of carrier status in a tropical country such as India. METHODS: This prospective historically controlled study was done in a total of 40 patients. From August 2003, all patients, incident and prevalent, were instructed to apply 2% mupirocin cream daily to the exit site instead of the older practice of povidone-iodine and gauze dressing. Patients were not screened to determine whether they were S. aureus carriers. The infection-related data for 1 year, until July 2004, were compared with the historical control, which was infection-related data for the year preceding the year of mupirocin application. RESULTS: Mean age of the study population was 62 years, with 61.8% being male and 64.3% being diabetic. Local application of mupirocin led to a significant reduction in the incidence density per patient-month of both ESI and peritonitis compared to controls (0.15 vs 0.37 and 0.37 vs 0.67, p = 0.01 for both). This amounted to a relative reduction of 60.5% and 55% respectively. ESI and peritonitis due to S. aureus were also significantly lower in the study group compared to controls (incidence density per patient-month 0.05 vs 0.13 and zero vs 0.17 respectively, p < 0.01 for both). There occurred no catheter removal due to infection-related complications during the study period compared to two during the control period. None of the patients reported a mupirocin-related adverse effect. CONCLUSIONS: Daily application of mupirocin at the exit site is a well-tolerated and effective strategy in reducing the incidence of ESI and peritonitis in a tropical country such as India. It can thus significantly reduce morbidity, catheter loss, and transfer to hemodialysis in peritoneal dialysis patients.     

Complex urethral disruptions: In pursuit of a successful reconstruction

OBJECTIVES: We analyzed the methods and outcomes of urethroplasty in men with complex urethral disruptions. METHODS: The medical records of 40 men with complex urethral disruptions were analyzed. Surgical methods were individualized according to stricture location, severity and length of the stricture, bladder neck characteristics and presence of complicating factors. Patients were divided into four groups based on the above characteristics. RESULTS: End-to-end urethroplasty performed in six patients with short bulbar strictures (<3 cm) was successful in all. Elaborated perineal repair was performed in 10 patients with intermediate (3-6 cm) strictures with or without complicating factors. Elaborated perineal repair with urethral substitution was performed in nine patients with long segment stricture (>6 cm). Abdominal transpubic repair was successfully applied to patients with rectourethral fistula or lacerated bladder neck. Success rate of anastomotic urethroplasty was 95% while over all success rate was 85%. CONCLUSION: Guidelines for urethral reconstruction of complex urethral disruptions are predicated on stricture length, location, bladder neck characteristics and associated complicating factors. End-to-end urethroplasty with stricture excision is highly reliable for short strictures for which previous operative repair have failed. Elaborated perineal repair is extremely versatile for intermediate and longer strictures with associated complicating factors. Abdominal transpubic urethroplasty is effective for patients with rectourethral fistula or lacerated bladder neck.     

Factors affecting the use of peritoneal dialysis among the ESRD population in India: a single-center study.

OBJECTIVES: Factors such as limited health-care budget allotment and poor accessibility of the majority of the population to hemodialysis (HD) facilities should favor the use of peritoneal dialysis (PD) in India. However, only 6% of end-stage renal disease patients undergoing dialysis in India are on PD. We undertook this prospective study to evaluate various factors that could contribute to this low rate of use of PD at a tertiary-care state-run hospital in Northern India. METHODS: All the patients who entered our HD or PD program from August 2001 to December 2003 were interviewed using a preset questionnaire. The questionnaire recorded their basic disease and comorbidity, social and demographic characteristics, awareness of the various modalities of renal replacement therapy (RRT), and the reasons for choosing their present modality of therapy. Treating nephrologists were also interviewed with respect to the factors that, in their opinion, were responsible for the limited use of PD at our institute. RESULTS: In total, 342 patients on HD, 66 patients on PD, and 24 nephrologists were interviewed. The rate of PD use was 16.2%. Mean age of patients on HD and PD was 34.6 +/- 11.8 years and 62.9 +/- 10.3 years respectively (p < 0.0001). The incidence of diabetes mellitus and coronary artery disease in the HD and PD populations was 2.5% and 62.5%, and 9.1% and 46.7% respectively (p < 0.0001 for both). Only 30.4% of patients on HD were aware of PD as a modality of RRT and 83.6% of them found PD to be expensive, 65.4% had low enthusiasm toward a domiciliary therapy such as PD, and 61.5% were not recommended PD by their nephrologist. Only 5 (7.6%) patients were initiated on PD directly, the remaining 61 patients were shifted from HD after a mean duration on HD of 185.3 +/- 15.4 days: 67.1% were shifted due to poor tolerance of HD, 29.4% were advised to shift to PD because of comorbidity and vascular access problems, and only 3.3% took up PD because of the independent lifestyle it offered. None of the interviewed nephrologists routinely discussed PD in predialysis counseling. They found financial constraints (100%), lack of patient enthusiasm (100%), doubtful patient compliance (83.2%), and lack of an organized PD program (79.2%) to be the main factors limiting more widespread use of PD at our institute. CONCLUSIONS: Peritoneal dialysis is an underused modality of RRT at our institute. The patients who are taken up for PD at our institute are elderly and have a higher incidence of other comorbid conditions, such as diabetes mellitus and coronary artery disease. Also, most patients who switch to PD do so due to their unsuitability for HD rather than by their own choice. The factors contributing to this low rate of use of PD are ignorance of PD, increased cost of therapy, low enthusiasm toward domiciliary therapy, and lack of adequate infrastructure for PD at our institute. Effective predialysis counseling, reduction in the cost of the therapy, and development of an adequate infrastructure can increase the rate of use of PD.     

Interactions of new plasmid-mediated beta-lactamases with third-generation cephalosporins

The kinetic constants of three recently identified plasmid-mediated beta-lactamases--SHV-2, CTX-1, and CAZ-1--markedly active against third-generation cephalosporins were analyzed in comparison with three better-characterized beta-lactamases--two plasmid-mediated enzymes, TEM-2 and PIT-2/SHV-1, and R-30, a beta-lactamase from Klebsiella oxytoca that has few similarities to the newer enzymes. All of these enzymes are synthesized constitutively, demonstrate efficient hydrolysis of penicillins, are highly susceptible to the action of clavulanic acid and sulbactam, and have no detectable activity against the cephamycins and imipenem. With the methoxyimino cephalosporins, including those of the third generation, the rates of hydrolysis observed for the SHV-2, CTX-1, and CAZ-1 enzymes are high and show no relation to those observed for the other presently known beta-lactamases. Structure-activity relations suggest that the oxime substituent of these cephalosporins is a major structural factor in the catalytic process observed with the three new beta-lactamases.     

Candida septicaemia and abscess formation following multiple injuries with blunt rupture of the stomach

A case of multiple injuries including blunt rupture of the stomach is reported. It demonstrates that Candida needs to be considered as a possible cause of continued sepsis following a ruptured viscus in the presence of multiple trauma. Surgical drainage is required in this situation, in the same way that abscesses caused by more conventional organisms require aggressive surgical treatment.