The Flexner Report--100 years later.

The Flexner Report of 1910 transformed the nature and process of medical education in America with a resulting elimination of proprietary schools and the establishment of the biomedical model as the gold standard of medical training. This transformation occurred in the aftermath of the report, which embraced scientific knowledge and its advancement as the defining ethos of a modern physician. Such an orientation had its origins in the enchantment with German medical education that was spurred by the exposure of American educators and physicians at the turn of the century to the university medical schools of Europe. American medicine profited immeasurably from the scientific advances that this system allowed, but the hyper-rational system of German science created an imbalance in the art and science of medicine. A catching-up is under way to realign the professional commitment of the physician with a revision of medical education to achieve that purpose.

Portraits of an illness.

Access to patients' inner lives can be expanded and enriched by incorporating the arts and humanities into the clinical encounter. A series of self-portraits created by an artist undergoing induction chemotherapy for leukemia afforded a unique opportunity to concentrate one's gaze upon the patient as a stimulus for reflection on suffering and isolation of patients. Poetry and theater were also invaluable in expanding the physician's awareness of the shared experience of illness. The process highlights the central role of the "New Humanities" in modern medicine, where science informs the arts and the arts inform science and medicine.

Guidelines for safe surgery in patients with systemic mastocytosis.

Systemic mastocytosis (SM) is a rare disorder with important perioperative implications. The physiological stress of operative procedures and a variety of anesthetic and analgesic medications can be triggers of acute exacerbation of this condition. We present two patients with systemic mastocytosis. One underwent open left inguinal hernia and umbilical hernia repair and the other laparoscopic ventral hernia repair. The literature for perioperative management of patients with SM is extensively reviewed. Both patients were treated preoperatively with intravenous antihistamines and steroids to minimize SM reactions. The first patient underwent uneventful open left inguinal hernia repair and umbilical hernia repair under spinal anesthesia. The second patient underwent general anesthesia. A composite mesh was used to repair a 9 x 12-cm Swiss cheese incisional hernia at the site of previous surgery. After the administration of neostigmine and glycopyrrolate, she developed a generalized rash without any hemodynamic instability. The patient was treated with intravenous Solu-Medrol and Benadryl and was extubated successfully and had an unremarkable postoperative course. Patients with SM require careful perioperative management for surgery under spinal and general anesthesia. These patients can undergo surgical procedures safely and effectively without compromising the standard of care.

Well rounded.

Team ward rounding is a time-honored practice. Trainees learn not only clinical aspects but also ethical values of patient care. Despite current emphases on the principles of professionalism and humanism in medicine, there remains little discussion about applying these principles to ward rounds. We believe that rounds can become classrooms for the intentional inculcation of professionalism and humanism as counterweights to unintentionally promulgated brusqueness, ambivalence, cynicism, and frustration. We gathered examples of "humanistic rounding" from various institutions that should stimulate discussion and illustrate that no specialized training is required to "humanize" rounds; rather, willingness and creativity are key ingredients.

A clinician's approach to clinical ethical reasoning.

We offer a systematic strategy that situates clinical ethical reasoning within the paradigm of clinical reasoning. The trajectory of this strategy parallels clinical reasoning: a plain statement of the initial problem, careful gathering of data, a differential diagnostic assessment, and articulation and confirmation of a justified plan. This approach pays special attention to the goals of medical care, because so much depends on whether or not physician and patient share the same goals. This approach also addresses the heterogeneity of clinical problems that at first appear ethical and acknowledges the ethical pluralism that pervades clinical ethics.

Oxaliplatin induces a delayed immune-mediated hemolytic anemia: a case report and review of the literature.

We report a case of a 59-year-old woman with metastatic carcinoma of the ileocecal region who received FOLFOX(oxaliplatin/leucovorin/5-fluorouracil) and bevacizumab therapy and exhibited a partial remission with minimal side effects. She developed a mild self-limited episode of immune-mediated hemolytic anemia during her 16th cycle of chemotherapy, which precluded her from receiving further oxaliplatin. We review the literature on oxaliplatin-induced immune-mediated hemolysis, including its mechanism, presenting symptoms, laboratory features, management, and implications for future therapy.

Should disagreement about physician-assisted suicide discourage professional societies from engaging in public debate? A survey of 677 Connecticut internists.

OBJECTIVE: To determine whether physicians within a state-level professional association who disagree about the ethics of physician-assisted suicide (PAS) also disagree about organized professional engagement in public discussions regarding PAS. DESIGN: Anonymous mail questionnaire. PARTICIPANTS: Connecticut members of the American College of Physicians-American Society of Internal Medicine as of February 1999. MEASUREMENTS: Attitudes toward the legalization of PAS and organized participation in public discussions about PAS, and demographic and religious characteristics. MAIN RESULTS: The mean age of 677 respondents was 51 years and 20% were women. The legalization of PAS was favored by 28% of respondents, and opposed by 53%, but 84% favored organized participation in appropriate public discussions regarding PAS. Differences in religious affiliation were strongly associated with disagreements about PAS: physicians with Jewish affiliation or no affiliation were more likely to favor legalizing PAS than those with Catholic or other Christian affiliations (45%, 38%, 14%, and 19%, respectively, P < .001). Religious affiliation was not associated with differences in attitude toward organized participation in public discussions about PAS. Statistically significant differences in attitude toward public participation were found between some subgroups according to differing attitudes toward PAS, but in each case at least 80% of physicians still supported participation. CONCLUSIONS: Internists in this state-level organization widely supported organized participation in public discussions regarding PAS despite disagreements about its legalization, and support for organized participation was not affected by differences in religious affiliation nor substantially attenuated by differences in attitudes toward PAS.

The Osler-Cushing covenant.

In 1900 William Osler established a friendship with Harvey Cushing that encompassed the personal and professional aspects of their lives for over two decades. Their shared participation in the covenant of medicine shaped an intense friendship and mentoring relationship that profited both individuals immeasurably. The choice of Cushing as the recipient of Osler's mentoring had its origins in their rearing, avocations, and in the way of life that they shared. In Cushing, Osler identified a surrogate son who joined with him in defining the course of medicine and surgery over the next century.

Lymphomas of mucosal-associated lymphoid tissue in common variable immunodeficiency.

Common variable immunodeficiency (CVID) is a primary immunodeficiency disease characterized by low serum immunoglobulins IgG, IgA, and usually IgM. The central immune deficiency is impaired secretion of immunoglobulins and lack of antibody production; however, T cell dysfunction and a variety of inflammatory complications suggest global immune dysregulation. A number of reports have documented the association of primary immunodeficiency diseases with the development of non-Hodgkin's lymphoma (NHL). In CVID, the risk has been estimated to lie between 1.4% and 7%. As for NHL arising in other immunodeficiency states, the lymphomas in CVID are extranodal and are usually B cell in type. Of 22 B cell lymphomas that have appeared over a period of 25 years in a cohort of subjects with CVID, five lymphomas, appearing in more recently studied subjects, that arose in mucosal sites would be classified as mucosa-associated lymphoid tissue (MALT) lymphomas. MALT lymphomas are low-grade B cell lymphomas that result from a proliferation of neoplastic marginal-zone related cells of lymphoid tissue and tend to occur in organs that have acquired lymphoid tissue due to long-term infectious or autoimmune stimulation. Lymphomas of this kind have not been described in patients with congenital immunodeficiency, although chronic mucosal antigen stimulation is an integral part of these immune deficiency states.

Diabetes prevalence among Puerto Rican adults in New York City, NY, 2000.

This study assessed the prevalence of diagnosed diabetes and associated characteristics among Puerto Rican adults in New York City, NY, with a random-digit-dialed telephone survey with a dual-frame sampling design. Overall, 11.3% (95% confidence interval = 8.7%, 14.0%) had diagnosed diabetes; diabetes was significantly related to age, obesity, and family history; and the prevalence was high among those with the least education. This study showed the ability to obtain critically needed diabetes information from ethnic minorities at the local level.

Pulmonary hypertension in type 1 Gaucher's disease: genetic and epigenetic determinants of phenotype and response to therapy.

Type 1 Gaucher's disease (GD) is recognized for striking but unexplained phenotypic diversity. Rarely, severe pulmonary hypertension (PH) may occur in GD but its clinical spectrum, determinants or its response to enzyme replacement therapy (ERT)+/-vasodilators is not known. One hundred and thirty-four consecutive patients with Type 1 GD were screened to estimate right ventricular systolic pressure (RVSP) by Doppler echocardiography. Ninety-four patients were on ERT and 40 were untreated. Eight additional GD patients were studied that represented consecutive tertiary referrals with severe PH. Angiotensin converting enzyme (ACE) gene polymorphisms and acid beta-glucosidase gene (GBA) mutations were determined by DNA analysis. Mild, asymptomatic PH (RVSP>35<50 mmHg) was prevalent in Type 1 GD: 30% in untreated patients and 7.4% among patients receiving ERT (P<0.001). Splenectomy was strongly associated with severe, life-threatening PH: all patients with severe PH (RVSP 50-130 mmHg) were asplenic compared to only 31% of patients with RVSP<50 mmHg (Odds ratio [OR] 28.8, 95% CI 1.6-531.6, P<0.001). Other characteristics of patients presenting with severe PH were poor compliance to ERT (4/9 patients) or no ERT (5/9 patients), a family history of a sib with GD and PH (2/2 patients), an excess of ACE I allele (OR 2.3, 95% CI 1.1-4.9, P=0.034) and an excess of non-N370S GBA mutation (OR 6.0, 95% CI 1.1-33, P=0.003). Severe PH was ameliorated by ERT+/-vasodilators during 4.6+/-4.0 yr (range 1-12 yr) follow-up; three patients were initially considered for lung transplantation but improved such that they are no longer active transplant candidates. Our study reveals a remarkable predisposition for PH in type 1 GD. Progression to severe, life-threatening PH occurs in the presence of additional genetic factors (non-N370S GBA mutation, positive family history, and ACE I gene polymorphism) and epigenetic modifiers (i.e., asplenia and female sex). Splenectomy should be avoided and in high-risk patients, ERT+/-vasodilators/coumadin should be initiated.

The mast cell and mast cell disease

Mast cell disease or mastocytosis is a heterogeneous group of clinical disorders characterized by the proliferation and accumulation of mast cells in a variety of tissues, most often the skin. The signs and symptoms of mast cell disease are varied, dependent on the localization of mast cells in different organs and the local and systemic effects of mediators released from these cells. Although mast cell disease is most commonly identified in the skin, involvement of the skeletal, hematopoietic, gastrointestinal, cardiopulmonary, and central nervous systems may be seen. Clinical management of mastocytosis depends most heavily on knowledge of the diverse effects of mast cell mediators on various tissues and organs, the stimuli that can cause their release, and the different methods available for blocking the effects of these mediators.