Intention to discontinue care among primary care patients

BACKGROUND: Specific elements of health care process and physician behavior have been shown to influence disenrollment decisions in HMOs, but not in outpatient settings caring for patients with diverse types of insurance coverage. OBJECTIVE: To examine whether physician behavior and process of care affect patients' intention to return to their usual health care practice. DESIGN: Cross-sectional patient survey and medical record review. SETTING: Eleven academically affiliated primary care medicine practices in the Boston area. PATIENTS: 2,782 patients with at least one visit in the preceding year. MEASUREMENT: Unwillingness to return to the usual health care practice. RESULTS: Of the 2,782 patients interviewed, 160 (5.8%) indicated they would not be willing to return. Two variables correlated significantly with unwillingness to return after adjustment for demographics, health status, health care utilization, satisfaction with physician's technical skill, site of care, and clustering of patients by provider: dissatisfaction with visit duration (odds ratio [OR], 3.2; 95% confidence interval [CI], 1.4 to 7.4) and patient reports that the physician did not listen to what the patient had to say (OR, 8.8; 95% CI, 2.5 to 30.7). In subgroup analysis, patients who were prescribed medications at their last visit but who did not receive an explanation of the purpose of the medication were more likely to be unwilling to return (OR, 4.9; 95% CI, 1.8 to 13.3). CONCLUSION: Failure of physicians to acknowledge patient concerns, provide explanations of care, and spend sufficient time with patients may contribute to patients' decisions to discontinue care at their usual site of care.     

Concomitant diagnosis of primary Sjögren's syndrome and systemic AL amyloidosis

A 48 year old woman was referred to hospital for buccal discomfort. Physical examination showed a macroglossia and features of xerostomia. She was diagnosed as having primary Sjögren''s syndrome according to the criteria proposed by the European Community study group in 1993. Furthermore, a lower lip salivary gland biopsy showed amyloid deposits that were also seen in the stomach and in the bone marrow. Echocardiography was consistent with cardiac amyloidosis. Serum immunofixation identified a monoclonal IgGλ. As far as is known, this is the first report of systemic primary amyloidosis associated with Sjögren''s syndrome. The relation between these two disorders is discussed.

     

Drug-associated lymphoma and pseudolymphoma: recognition and management

This article discusses ways to recognize and manage lymphomas and pseudolymphomas associated with drug exposure. Over the last 30 years, the classification of pseudolymphomas and lymphomas has undergone significant change, especially following the application of sophisticated immunostaining and gene rearrangement analysis. The term cutaneous pseudolymphomas (CPL) is a nonspecific term for a heterogeneous group of benign reactive T- or B-cell lymphoproliferative processes that simulate cutaneous lymphomas clinically or histologically. While pseudolymphomas are relatively rare diseases, their clinical and histological heterogeneity has led to multiple systems of categorization based on immunological factors, causative agents, presentation, and clinical course.     

Interleukin‐6 receptor shedding is enhanced by interleukin‐1β and tumor necrosis factor α and is partially mediated by tumor necrosis factor α–converting enzyme in osteoblast‐like cells

Objective

Interleukin‐6 (IL‐6) and soluble IL‐6 receptor (sIL‐6R) activation of gp130 represents an alternative pathway for osteoclast development in inflammatory conditions. The goal of the present study was to investigate changes in sIL‐6R levels in response to the inflammatory cytokines IL‐1β and tumor necrosis factor α (TNFα) and to determine the role of TNFα‐converting enzyme (TACE) in this process.

Methods

Levels of sIL‐6R in the culture media of MG63 and SAOS‐2 osteoblast‐like cell lines after exposure to various agents were determined by immunoassay. TACE protein levels were measured by Western immunoblotting. Cells were transfected with small interfering RNA (siRNA) or with an expression plasmid for IL‐6R and TACE to determine the potential involvement of TACE in IL‐6R shedding.

Results

IL‐1β and TNFα increased the levels of sIL‐6R in the culture media of MG63 osteoblast‐like cells. This effect was not influenced by cycloheximide or 5,6‐dichlorobenzimidazole riboside but was markedly inhibited by the calcium chelator EGTA and by the TACE and matrix metalloproteinase inhibitor hydroxamate (Ru36156). IL‐1β and TNFα had no influence on the alternatively spliced form of IL‐6R RNA. Levels of sIL‐6R were reduced when MG63 cells were transiently transfected with TACE siRNA. Transfection of SAOS‐2 cells with expression plasmids for IL‐6R and TACE produced a dose‐dependent increase in sIL‐6R levels.

Conclusion

IL‐1β‐ and TNFα‐mediated induction of IL‐6R shedding in osteoblast‐like cells is at least partly dependent on TACE activation.

     

Efficacy of interferon alpha in the treatment of refractory and sight threatening uveitis: a retrospective monocentric study of 45 patients

AIM: Severe uveitis is potentially associated with visual impairment or blindness in young patients. Therapeutic strategies remain controversial. The efficacy of interferon alpha-2a (IFN-alpha2a) in severe uveitis, refractory to steroids and conventional immunosuppressive agents, was evaluated. PATIENTS AND METHODS: Patients were included after a major relapse of uveitis following corticosteroids and immunosuppressants. IFN-alpha2a (3 million units three times a week) was administered subcutaneously. Efficacy was assessed by improvement in visual acuity, decrease in vitreous haze, resolution of retinal vasculitis and macular oedema, assessed by fundus examination and fluorescein angiography, and decrease in oral prednisone threshold. RESULTS: 45 patients were included. Median age was 32.3 years (range 8-58) and sex ratio (F/M) was 0.66. Uveitis was associated with Beh?et's disease in 23 cases (51.1%) and with other entities in 22 cases (48.9%). Median duration of uveitis before interferon therapy was 34.9 months (range 3.4-168.7) and an average of 3.26 relapses following corticosteroids and immunosuppressants was noted. Uveitis was controlled in 82.6% of patients with Beh?et's disease and 59% of patients with other types of uveitis (p = 0.07). During a mean follow-up of 29.6 months (range 14-55), median oral prednisone threshold decreased significantly from 23.6 mg/day (range 16-45) to 10 mg/d (range 4-14) (p<0.001). Interferon was discontinued in 10 patients (22.2%) with Beh?et's disease and in four patients without Beh?et's disease. Relapses occurred in four and one cases, respectively. CONCLUSIONS: Interferon therapy appears to be an efficient strategy in severe and relapsing forms of Beh?et's disease but also in other uveitic entities. However, it seems to act more to suspend rather than cure the disease. Therefore, IFN-alpha2a may be proposed as a secondline strategy after failure of conventional immunosuppressants.     

Neonatal maculopapular exanthema revealing septicemia due to Klebsiella pneumoniae via maternofetal infection

BACKGROUND: Neonatal exanthema of bacterial origin is caused very rarely by Gram-negative bacilli. We report a case of neonatal maculopapular exanthema evocative of Klebsiella pneumoniae septicemia resulting from maternofetal infection. OBSERVATION: On the fourth day of life, a newborn infant presented incipient morbilliform maculopapular exanthema on the face. During delivery, the mother had presented hyperthermia and meconium was found in the amniotic fluid. Clinical examination of the newborn was normal. No clinically obvious site of entry of infection was seen. Laboratory tests revealed major inflammatory syndrome. Blood cultures were positive for K. pneumoniae, which was also found in blood cultures from the mother. Screening for other causes of infection was negative. Parenteral antibiotics for 10 days yielded favorable results with simultaneous resolution of the exanthema, normalization of laboratory values and negative blood cultures. DISCUSSION: Exanthemas of infectious origin are not associated with any specific organism. The most common causative micro-organisms are Listeria monocytogenes, B streptococci, colibacilli, and more rarely, staphylococci. There have been reports of a number of cases of neonatal septicemia due to Gram-negative bacilli responsible for maculopapular exanthemas but the causative organism was not identified. To our knowledge, Klebsiella pneumoniae has never been incriminated in the appearance of this type of rash via maternofetal transmission. Consequently, the presence of neonatal exanthema should prompt screening for sepsis, even in the absence of other evocative signs, and in particular in settings of apyrexia.     

Interstitial brachytherapy in management of primary cutaneous melanoma: 4 cases

BACKGROUND: Traditionally melanoma has been considered as a radioresistant tumor. However, recent observations regarding radiobiology and clinical response of melanoma have prompted physicians to re-evaluate the role of radiotherapy in the management of melanoma. Indeed, radiotherapy is frequently used in the treatment of metastatic melanoma but the role of this therapeutic approach in the primary tumor management is unclear. We report 4 cases of thick primary melanoma treated by interstitial brachytherapy. CASE REPORTS: Four patients (3 men, 1 woman; age: 73, 74, 79, 84 years), three with lentigo maligna melanoma and one with nodular melanoma, were treated exclusively by interstitial brachytherapy. The thickness of the 4 tumors was more than 3 mm. This treatment was chosen either because of the impossibility of conservative surgery or because of a contraindication for general anesthesia. Median follow-up was 48 months [18 to 65 months] and we did not observe any local tumor relapse. The cosmetic and functional results of this therapy were excellent. DISCUSSION: These four cases illustrate the possibility of obtaining good local control of thick primary melanoma by interstitial brachytherapy where surgery is impossible, although without challenging the standard surgical therapy for primary melanoma. Before our study, this treatment was used only in one study to treat melanoma patients. In this case however, interstitial brachytherapy was accompanied by surgery. Therefore, the findings reported here are the first to demonstrate the efficacy of this treatment when used as the sole treatment for primary melanoma. In conclusion, where surgery is difficult or impossible, interstitial brachytherapy may be used to treat primary melanoma with excellent local tumor control and a good cosmetic outcome.     

Superficial venous thrombophlebitis as the initial manifestation of hypereosinophilic syndrome: study of the first 3 cases

BACKGROUND: Superficial venous thrombophlebitis (SVT), often perceived as benign, can coexist with hypercoagulable states. Predisposing risk factors for SVT are similar to those observed for deep venous thrombosis. Association of eosinophilia with SVT is a rare situation that can reveal neoplasia, malignant blood disorders, or vasculitis, but it has never been described in hypereosinophilic syndrome (HES). We herein describe the clinical and biological features, outcome, and response to therapy of 3 patients with SVT associated with eosinophilia that revealed HES. OBSERVATIONS: Superficial venous thrombophlebitis was the initial manifestation of HES in all 3 patients. The mean eosinophil count at diagnosis was 2.4 x 10(3)/muL. All patients received corticosteroids and anticoagulants as the initial treatment, with marked improvement of SVT and return of the eosinophil count to reference limits. All patients experienced relapse and remained dependent on corticosteroid therapy. Two patients received interferon alfa with dramatic regression of SVT, allowing a decrease in the dose of corticosteroids. CONCLUSIONS: We report, to our knowledge, the first 3 cases of SVT related to HES. Superficial venous thrombophlebitis was difficult to treat, with dependence on corticosteroid therapy and partial efficacy of anticoagulant and antiplatelet therapy. Interferon alfa was effective in preventing relapse of SVT related to HES. Mechanisms implied in this thrombogenesis are multiple and remain speculative.     

Pulse methylprednisolone therapy for threatening periocular haemangiomas of infancy

Periocular haemangiomas of infancy can cause severe and rapid ocular damage. Oral corticosteroids remain the front-line treatment to minimize the consequences of these haemangiomas. The aim of this report is to summarize our experience with pulse intravenous methylprednisolone as an alternative therapy for periocular haemangioma when visual prognosis is engaged. Fifteen infants, who presented periocular haemangioma with functional impact on vision, received 2 mg/kg methylprednisolone intravenously twice a day for 2 days. Following pulse therapy, 2 mg/kg/day prednisolone was given orally with gradual tapering. No further visual impact was noticed following pulse therapy. Two patients relapsed, needing new pulses or, in one case, vincristine. No serious side-effects were recorded. Pulse methylprednisolone therapy permitted a particularly rapid shrinkage of haemangiomas and a complete disappearance of their visual impact within 2 days. Apparently more rapid than the usual oral corticosteroids, pulse intravenous methylprednisolone decreases the risk of ocular complications, which correlates with the duration of the influence of haemangiomas.