Prioritizing lean management practices in public and private hospitals.

Purpose - The purpose of this paper is to prioritize 21 healthcare wastes in public and private hospitals of United Arab Emirates (UAE). Design/methodology/approach - Seven healthcare wastes linked with lean management are further decomposed in to sub-criteria and to deal with this complexity of multi criteria decision-making process, analytical hierarchical process (AHP) method is used in this research. Findings - AHP framework for this study resulted in a ranking of 21 healthcare wastes in public and private hospitals of UAE. It has been found that management in private healthcare systems of UAE is putting more emphasis on the inventory waste. On the other hand, over processing waste has got highest weight in public hospitals of UAE. Research limitations/implications - The future directions of this research would be to apply a lean set of tools for the value stream optimization of the prioritized key improvement areas. Practical implications - This is a contribution to the continuing research into lean management, giving practitioners and designers a practical way for measuring and implementing lean practices across health organizations. Originality/value - The contribution of this research, through successive stages of data collection, measurement analysis and refinement, is a set of reliable and valid framework that can be subsequently used in conceptualization, prioritization of the waste reduction strategies in healthcare management.

Extracorporeal photopheresis for chronic graft-versus-host disease: a systematic review and meta-analysis.

BACKGROUND: The safety of extracorporeal photopheresis (ECP) in steroid-refractory chronic graft-versus-host disease (SR-cGVHD) has been explored in multiple studies but reported response rates (RR) vary significantly across studies. METHODS: We conducted a meta-analysis to assess the efficacy of ECP for SR-cGVHD. A search of electronic databases for studies published between 1984 and 2012 was conducted. End points included RR: complete response (CR), overall response rates (ORR), and organ-specific RR. The initial search generated 312 studies, of which 18 met the selection criteria (N=595). A random effects model was used for pooled rates. RESULTS: Pooled CR rates and ORR were 29% (confidence interval [CI], 19-42%) and 64% (CI, 65-82%), respectively. One-year overall survival was available for 4 studies only and was 49% (CI, 29-70%). The pooled RR for skin, liver, ocular, oral, lung, gastrointestinal and musculoskeletal SR-cGVHD was 74%, 68%, 60%, 72%, 48%, 53%, and 64%, respectively. There was a significant heterogeneity among studies due to differences in ECP schedules and duration. No significant differences in responses to ECP for pediatric and adult populations were found. Sensitivity analysis could not be undertaken due to a limited number of prospective studies. CONCLUSION: ECP is an effective therapy for oral, skin, and liver SR-cGVHD, with modest activity in lung and gastrointestinal SR-cGVHD.

Early-onset cerebellar atrophy associated with mutation in the CACNA1A gene.

Mutations in the CACNA1A gene were described in familial hemiplegic migraine, episodic ataxia type 2, and spinocerebellar ataxia type 6. Familial hemiplegic migraine and episodic ataxia type 2 are caused by point mutations in the CACNA1A gene, and spinocerebellar ataxia type 6 develops as a result of a CAG triple expansion in exon 1 of the gene. Phenotypic variability and clinical overlap are well recognized. We describe a 3-year-old child with clinical and radiologic signs of early-onset cerebellar atrophy. The family history was significant for migraine, and in some members of the family, a diagnosis of hemiplegic migraine was established. The combination of cerebellar atrophy in our patient and the family history suggested involvement of the CACNA1A gene. The sequence analysis of genomic DNA from the proband identified heterozygosity for a mutation (Thr666Met) in the CACNA1A gene. Subsequently, his father, who was mildly affected, and two other relatives were demonstrated to carry the same mutation. Therefore, CACNA1A gene mutations should be considered in the differential diagnosis of congenital cerebellar atrophy.

Dermatologic conditions seen in end-stage renal disease.

The skin changes reported in patients with end-stage renal disease (ESRD) are diverse and manifold. In this article we focus on a collection of specific cutaneous entities seen most frequently in the setting of ESRD, each presenting with distinctive and unique morphology. These include perforating disorders, porphyria cutanea tarda, pseudoporphyria, calcinosis cutis, calciphylaxis, and nephrogenic systemic fibrosis. The clinical features, histopathology, pathophysiology, differential diagnosis, and management of each entity are reviewed.

Characteristics of Mohs practices in the United States: a recall survey of ACMS surgeons.

OBJECTIVE: The purpose of this study is to better characterize the typical Mohs practice in the United States and to generate data that may be useful in future practice models. METHODS: A survey was mailed in 2004 to all 599 members of the American College of Mohs Surgery with United States addresses listed in the 2003 directory. RESULTS: Most respondents were part of a single speciality group, in a suburban or urban setting, performed between 501-1,000 cases per year, and had been in practice from 0-5 years. The vast majority of Mohs excisions are for basal cell cancers and squamous cell cancers, followed by melanoma. Primary closure is the most common method of repair, followed by the use of flaps. Only 6% of cases were referred to other specialties for closure. Many surgeons augment their practice with non-Mohs cosmetic procedures. CONCLUSION: The characteristics of current Mohs surgery practices in the United States provides useful data for training programs, potential trainees, workforce issues, statistical modeling systems, and Mohs surgeons in the evaluation of their own practices.

Mutations in RNF135, a gene within the NF1 microdeletion region, cause phenotypic abnormalities including overgrowth.

17q11 microdeletions that encompass NF1 cause 5%-10% of cases of neurofibromatosis type 1, and individuals with microdeletions are typically taller than individuals with intragenic NF1 mutations, suggesting that deletion of a neighboring gene might promote human growth. We identified mutations in RNF135, which is within the NF1 microdeletion region, in six families characterized by overgrowth, learning disability, dysmorphic features and variable additional features. These data identify RNF135 as causative of a new overgrowth syndrome and demonstrate that RNF135 haploinsufficiency contributes to the phenotype of NF1 microdeletion cases.

Sodium metabisulfite--a marker for cosmetic allergy?

A 45-year-old woman developed dermatitis of the face after she applied a cosmetic package comprising day and night creams. Patch tests were performed with the British Contact Dermatitis Society (BCDS) standard, bases + preservatives, and cosmetic series in addition to samples of both creams and the individual constituents. She had positive tests to both cosmetic creams, sodium sulfite from the manufacturer's samples (5% white soft paraffin (WSP)), and sodium metabisulfite (1% pet) in our bases + preservatives battery. Sodium sulfite is a constituent of both cosmetic creams. We assume that the positive test to sodium metabisulfite is a cross-reaction. We hypothesize that a reaction to sodium metabisulfite may be a marker for sulfite allergy in cosmetics and might account for some of the unexplained positives in previous reports.

Concurrence of systemic lupus erythematosus and pemphigus: coincidence or correlation?

BACKGROUND: Pemphigus and systemic lupus erythematosus (SLE) have previously been reported to coexist in the same patient. However, the relationship between the 2 diseases has not been elucidated. OBJECTIVE: This review was conducted to examine the relationship between pemphigus and SLE when they occur together in the same patient. METHODS: We conducted a retrospective review of the literature to identify previously reported cases of pemphigus and SLE coexisting in the same patient. The temporal relationship, clinical course, response to therapy and effects of 1 disease on the other were examined. RESULTS: Eight patients with a dual diagnosis of pemphigus and SLE have been previously reported. Most were female and non-Caucasian, with a mean age of 41 years. In the 8 patients reviewed here clinical outcomes, organ system involvement and demographic profiles are more typical of SLE. Seven of these 8 patients had pemphigus vulgaris, and 1 had pemphigus erythematosus. The limited follow-up did not permit studying issues of disease interaction. An additional 17 patients with pemphigus have been reported who have features suggestive of SLE. Organ system involvement in these patients was less typical of SLE. CONCLUSION: It appears that a true dual diagnosis of pemphigus and SLE is less common than suggested by the literature. Comparing patients with only pemphigus or only SLE to those with both may provide insights into genetic predisposition and pathogenesis, and provide an opportunity to study the effects of drugs that influence their course.

Relationship between cancer and oral pemphigoid patients with antibodies to alpha6-integrin.

BACKGROUND: Mucous membrane pemphigoid is an autoimmune mucocutaneous blistering disease. A subset, known as anti-epiligrin cicatricial pemphigoid is associated with a high risk for malignancy. Oral pemphigoid (OP) is limited to the oral cavity. The purpose of this study was to determine the association between malignancy and patients with OP with antibodies to alpha6-integrin subunit. METHODS: We determined the incidence of cancer in 72 patients with OP and compared it to the expected incidence using age and sex-specific rates of malignancy in the National Cancer Institute's Surveillance, Epidemiology, and End Results (NCI SEER) Registry. RESULTS: During a mean observation period of 9.1 years (range: 2.8-40), for 70, three OP patients developed malignancies. The expected number of cancers based on the NCI SEER Registry was 8.83. The relative risk for cancer in OP patients, with autoantibodies to alpha6-integrin, was 0.34 (95% CI, 0.07-0.99, P < 0.05). CONCLUSION: It appears that patients with OP, with antibodies to alpha6, may have a possible reduced relative risk for developing cancer.

Multicentric reticulohistiocytosis.

Multicentric reticulohistiocytosis is a rare granulomatous disease of unknown etiology, characterized by cutaneous nodules and destructive arthritis. Skin lesions can cause significant deformity, and approximately half of affected patients develop a severe disabling arthritis. The disease is often associated with malignancy; however, the paraneoplastic nature of multicentric reticulohistiocytosis is not established. The diagnosis is confirmed by the presence of oncocytic ("ground-glass") histiocytes and multinucleated giant cells on histopathology of the cutaneous nodules and the synovial membrane.

Dual diagnosis of pemphigus vulgaris and connective tissue disease.

This is a retrospective analysis of patients with pemphigus vulgaris and connective tissue disease (CTD) present as systemic lupus erythematosus, mixed CTD, or both. Pemphigus vulgaris was severe, difficult to treat, but eventually responded to therapy, resulting in a remission. In 6 patients, the CTD was stable and controlled with symptomatic therapy, and in 7 patients required systemic therapy. Life-threatening systemic involvement in systemic lupus erythematosus or mixed CTD-such as renal, cardiac, and neurologic-were absent in these patients on 8 years (range 3-18 years) of follow-up. There are two limitations to this study. First, it is a retrospective study. Second, no other disease control groups were used for comparison purposes.

The use of mole-mapping diagrams to increase skin self-examination accuracy.

BACKGROUND: Monthly skin self-examination (SSE) is associated with reduced incidence of advanced melanoma, but SSE is prone to error in detecting early changes of melanoma. OBJECTIVE: We sought to improve SSE accuracy by requiring participants to complete a mole-mapping diagram. METHODS: After completing a baseline survey, participants received SSE instruction, had their backs digitally photographed, and half were randomized to complete a mole-mapping diagram. Some photographs were altered by adding an image of a 5-mm pigmented lesion. At follow-up, participants were asked to identify any changes introduced to their photographs. RESULTS: A total of 88 participants completed the study. In all, 33% (n = 15) of the control group (no diagram) and 52% (n = 22) of the intervention group (mole-mapping diagram) (P = .06) gave accurate assessments. Analysis of only altered images indicated that the intervention group gave more accurate assessments (60% vs 33%, P = .01). LIMITATIONS: This study was limited by sample size, only addressed lesions on the back, and did not involve actual melanomas in study participants. CONCLUSIONS: Mole-mapping diagrams may improve SSE accuracy, and may be useful as a simple, cost-effective intervention in reducing melanoma mortality.

Anal involvement in pemphigus vulgaris.

INTRODUCTION: Anal involvement in patients with pemphigus vulgaris has rarely been reported. We report 16 pemphigus vulgaris patients with anal involvement. METHODS: We retrospectively reviewed the clinical data on 16 patients treated at a tertiary dermatology referral center. RESULTS: Of 16 patients with anal involvement of pemphigus vulgaris, ten were female and six were male. The mean age of onset was 56 (range, 37-82) years. All patients had involvement of pemphigus vulgaris at multiple sites, including oral involvement. Recurrent episodes of anal pemphigus vulgaris were noted in nine (56 percent) patients, with a mean of 2.4 recurrences (range, 1-11). In all patients, pemphigus vulgaris was controlled with systemic and local therapy. Long-term follow-up for a mean of 53 (range, 4-188) months indicated that no long-term sequela occurred because of anal involvement. CONCLUSIONS: Anal involvement in pemphigus vulgaris is not very common and generally occurs in patients with severe disease. With appropriate topical and systemic therapy, patients have full recovery with no sequelae.

Incidence of and risk factors for medical malpractice lawsuits among Mohs surgeons.

BACKGROUND: Despite rising medical malpractice costs, little is known about the factors associated with claims filed against Mohs surgeons. OBJECTIVE: We sought to define the scope of medical malpractice claims filed against Mohs surgeons and to identify salient factors associated with the filing and disposition of those claims. METHODS: A comprehensive survey was mailed to 599 physicians with US addresses listed in the 2003 directory of the American College of Mohs Micrographic Surgery and Cutaneous Oncology. RESULTS: Of the 300 completed surveys returned, 33 (11%) reported ever having been sued. Physicians who practiced Mohs surgery for a longer period of time were more likely to have been sued for malpractice. Physicians reported the wrong site and functional outcome as the most frequent causes of malpractice lawsuits.

Proliferating multicentric reticulohistiocytosis associated with papillary serous carcinoma of the endometrium.

Multicentric reticulohistiocytosis (MRH) is a rare histocytic disease characterized by destructive arthritis in association with classic skin findings. Although MRH is not strictly a paraneoplastic disease, one quarter of cases are malignancy related. We report a case of MRH with an initial remission followed by an acute exacerbation several years later heralding the clinical presentation of endometrial carcinoma. During this flareup a skin biopsy specimen revealed a diffuse dermal infiltrate composed of histiocytes with ground-glass cytoplasm and multiple atypical mitoses. Approximately 40% of the cells stained with the proliferation marker Ki-67. Treatment of endometrial carcinoma resulted in improvement of skin and joint symptoms, and a repeat biopsy specimen no longer demonstrated mitotic figures. These findings support a reactive and proliferative cause of MRH.

Involvement of the female genital tract in pemphigus vulgaris.

BACKGROUND: Involvement of the genital tract in women with pemphigus vulgaris has rarely been reported. We report 34 patients with pemphigus vulgaris who have involvement of the vulva, vagina, or both. METHODS: We reviewed the clinical data on 34 women treated at a tertiary dermatology referral center from July 1988 to June 2005. RESULTS: Of 34 patients, 21 had labial involvement, 3 had vaginal involvement, and 10 had both labial and vaginal involvement. The mean age of onset of pemphigus vulgaris was 49 years. All patients had pemphigus vulgaris involvement at multiple other sites, and all had other mucous membrane involvement, most commonly oral (97%). Recurrent episodes of genital pemphigus vulgaris were noted in 35%, with a mean of 6 recurrences (range 1-11). In all the patients the pemphigus vulgaris was controlled with systemic and local therapy. Long-term follow-up for a mean of 76 months (range 9-204) indicated that no long-term sequela occurred due to genital involvement. CONCLUSION: Involvement of the female genital tract in pemphigus vulgaris is rare. Vulvar lesions occur more commonly than vaginal lesions. With appropriate topical and systemic therapy, patients can have full recovery with no sequelae. LEVEL OF EVIDENCE: II-3.