Multicentric Reticulohistiocytosis: a Rare Yet Challenging Disease

Multicentric reticulohistiocytosis (MRH) is a rare systemic inflammatory granulomatous disease that primarily manifests clinically with severe erosive arthritis and widespread papulonodular skin lesions but can involve multiple other organ systems. Despite the fact that this condition can become aggressive, debilitating as well as deforming with significant detrimental consequences, the etiology of this disease remains poorly understood. Moreover, the fact that MRH is such an uncommon disease has created an obstacle in the path of adequate clinical trials that are needed for better understanding of this phenomenon and for the development of treatment options for this patient population. In this review, we will attempt to discuss the epidemiology, pathophysiology, clinical features, associated conditions, differential diagnoses, diagnostic workup, and available treatments of MRH with the hope of creating a better understanding of this very challenging yet elusive disease process.     

Economic Evaluation of Urgent-Start Peritoneal Dialysis Versus Urgent-Start Hemodialysis in the United States

Patients presenting late in the course of kidney disease who require urgent initiation of dialysis have traditionally received temporary vascular catheters followed by hemodialysis. Recent changes in Medicare payment policy for dialysis in the USA incentivized the use of peritoneal dialysis (PD). Consequently, the use of more expeditious PD for late-presenting patients (urgent-start PD) has received new attention. Urgent-start PD has been shown to be safe and effective, and offers a mechanism for increasing PD utilization. However, there has been no assessment of the dialysis-related costs over the first 90 days of care.The objective of this study was to characterize the costs associated with urgent-start PD, urgent-start hemodialysis (HD), or a dual approach (urgent-start HD followed by urgent-start PD) over the first 90 days of treatment from a provider perspective.A survey of practitioners from 5 clinics known to use urgent-start PD was conducted to provide inputs for a cost model representing typical patients. Model inputs were obtained from the survey, literature review, and available cost data. Sensitivity analyses were also conducted.The estimated per patient cost over the first 90 days for urgent-start PD was $16,398. Dialysis access represented 15% of total costs, dialysis services 48%, and initial hospitalization 37%. For urgent-start HD, total per patient costs were $19,352, and dialysis access accounted for 27%, dialysis services 42%, and initial hospitalization 31%. The estimated cost for dual patients was $19,400.Urgent-start PD may offer a cost saving approach for the initiation of dialysis in eligible patients requiring an urgent-start to dialysis.     

The impact of pharmacologic sympathetic and parasympathetic blockade on atrial electrogram characteristics in patients with atrial fibrillation

Background: Ablation of atrial autonomic inputs exerts antifibrillatory effects. However, because ablation destroys both myocardium and nerve cells, the effect of autonomic withdrawal alone remains unclear. We therefore examined the effects of pharmacologic autonomic blockade (PAB) on frequency and fractionation in patients with atrial fibrillation (AF). Methods: Esmolol and atropine were administered and electrograms were recorded simultaneously from both atria and the coronary sinus. In 17 patients, AF was recorded for 5 minutes and dominant frequency (DF) and continuous activity (CA) were compared before and during PAB. Results: Examination of the pooled data (537 sites, 17 patients) revealed a statistically significant decrease in mean DF (5.61–5.43Hz, P < 0.001) during PAB. Site‐by‐site analysis showed that 67% of sites slowed (0.45 ± 0.59 Hz), whereas 32% accelerated (0.49 ± 0.59Hz). Fractionation was reduced: median CA decreased from 31% to 26% (P < 0.001). In patient‐by‐patient analysis, mean DF/median CA decreased in 13 of 17 patients and increased in four. The spatial heterogeneity of DF decreased in nine of 17 patients (spatial coefficient of variation of DF at “nondriver sites” decreased by a mean of 2%). Conclusion: PAB decreases DF and CA in the majority of sites. Given the complexity of interactions between atrial cells during AF, the effects of PAB on DF and fractionation are more heterogeneous than the effects of PAB on isolated cells. (PACE 2011; 34:1460–1467)     

Consanguineous Iranian kindreds with severe Tourette syndrome

The search for vulnerability genes for Tourette syndrome has been ongoing for nearly three decades. The contribution of recessive loci with reduced penetrance is one possibility that has been difficult to explore. Homozygosity mapping has been successfully used to detect recessive loci within populations with high rates of consanguinity. Using this technique, even quite small inbred families can be informative due to autozygosity in which the two alleles at an autosomal locus are identical by descent (i.e., copies of a single ancestral gene). To explore the utility of this approach, we identified 12 consanguineous Iranian families. Remarkably, these families were seen with an unusual natural history characterized by the early onset of vocal tics and coprolalia and frequent comorbidity with obsessive‐compulsive disorder. Genotyping the affected and unaffected members of these pedigrees has the potential to identify rare recessive contributions to this disorder. © 2008 Movement Disorder Society     

Duration of untreated psychosis and pathways to care in patients with first-episode psychosis in Iran

Aim: This is the first study on the duration of untreated psychosis and pathways to care among patients with first‐episode psychosis in Iran as a developing country. Methods: Ninety‐one patients with a first episode of non‐organic psychosis admitted to a university‐affiliated psychiatric hospital in Iran were assessed for duration of untreated psychosis (DUP), pathways to care and mode of onset. Results: Median DUP was 11 weeks (mean = 52.3 weeks). Following the onset of psychosis, most patients were first seen by a psychiatrist (n = 23, 25.3%), a traditional healer (n = 21, 23.1%) or a general practitioner (n = 16, 17.6%). Most referrals to the psychiatric hospital were made by the family (n = 30, 33.1%), or health professionals (n = 29, 31.9%). Acute onset and rural place of residence were associated with shorter DUP in multivariate analysis. Conclusions: Median DUP was not long in an inpatient sample with first‐episode psychosis, which may be due to the preponderance of affective and acute psychoses in this sample and some help‐seeking or service variables.