Neurological autoimmune disorders with prominent gastrointestinal manifestations: A review of presentation, evaluation, and treatment.

BACKGROUND: The identification of autoantibodies directed against neuronal antigens has led to the recognition of a wide spectrum of neurological autoimmune disorders (NAD). With timely recognition and treatment, many patients with NAD see rapid improvement. Symptoms associated with NAD can be diverse and are determined by the regions of the nervous system affected. In addition to neurological symptoms, a number of these disorders present with prominent gastrointestinal (GI) manifestations such as nausea, diarrhea, weight loss, and gastroparesis prompting an initial evaluation by gastroenterologists. PURPOSE: This review provides a general overview of autoantibodies within the nervous system, focusing on three scenarios in which nervous system autoimmunity may initially present with gut symptoms. A general approach to evaluation and treatment, including antibody testing, will be reviewed.

ACTG2-Associated Visceral Myopathy With Chronic Intestinal Pseudoobstruction, Intestinal Malrotation, Hypertrophic Pyloric Stenosis, Choledochal Cyst, and a Novel Missense Mutation.

Primary visceral myopathy caused by a pathogenic mutation in the gene encoding the enteric smooth muscle actin gamma 2 ( ACTG2) affects gastrointestinal and genitourinary tracts and often presents as chronic intestinal pseudoobstruction. We present a case of pediatric onset chronic intestinal pseudoobstruction associated with a novel missense ACTG2 mutation c.439G>T/p.G147C. In addition to the known disease manifestations of feeding intolerance and intestinal malrotation, our patient had a late-onset hypertrophic pyloric stenosis and a late-onset choledochal cyst, the former of which has not previously been described in patients with ACTG2-associated visceral myopathy.

Hyaline-Vascular Type Castleman's Disease, Sarcoidosis, and Crohns Disease.

Sarcoidosis and Crohns disease have been associated with increased long term risk of lymphoproliferative disorders, including lymphomas. Newly developed lymphadenopathy in a patient with these disorders should prompt pathological evaluation. Castleman's disease is a lymphoproliferative disorder characterized by enlarged hyperplastic lymph nodes with regressed follicles surrounded by expanded mantle zones of small lymphocytes, and interfollicular vascular proliferation in the hyaline-vascular type. Similar to sarcoidosis and Crohns disease, its etiology is incompletely understood, although immune dysregulation, genetic factors and infectious and environmental factors are thought to play a role in all three diseases. Interleukin-6 is a possible pathological common factor between these three disease processed. Unicentric, hyaline-vascular type Castleman's disease can be treated successfully with complete surgical resection. We report a patient with long history of sarcoidosis and Crohns disease with newly developed lymphadenopathy which was found to be due to Castleman's disease.

The Need for Cultural Awareness Training: A Medical Student Perspective.

Inpatient training is a fundamental aspect of undergraduate and graduate medi- cal education. Physicians in training spend considerable time interacting with patients in a hospital setting during their time in medical school, residency, and fellowship. This time is crucial not only for the trainees to develop their techni- cal and diagnostic abilities, but also to improve their interpersonal communica- tion skills. The need to improve the nontechnical skills of trainees has already been recognized at the graduate medical education level. Our study aimed to examine medical student perspectives on certain nontechnical aspects of the patient-physician interaction and the effect of student demographics and cul- tural and religious preferences on their opinions. The study found that two-thirds of students had never had their teachers discuss how posture could be used to facilitate the medical interview, and 85% of the students agreed that their cur- riculum should include discussions on the appropriate posture for patient com- munication in an inpatient setting. We believe integrating some form of cultural awareness and competency training into the curriculum at the undergraduate medical education level would help prepare future physicians for an increasingly diverse patient population.

To Sit or Stand During the Medical Interview: A Poll of Caucasian Patients.

Nonverbal communication is an important component of the interaction between physician and patient. Multiple studies have revealed that patients perceive physicians who are seated during the medical interview as more compassionate and caring and as spending more time with patients. However, sitting on patient beds without permission may be culturally inappropriate and also may enhance nosocomial infection transmission. We interviewed 127 patients using a 14-item questionnaire soliciting patient demographics and their opinions regarding physician's nonverbal behaviors. One fifth of those surveyed reported having had a physician sit on their bed without permission, and more than 80% of patients preferred their physician adopt a position other than on the patient's bed during the medical interview. Asking patients about their preferences regarding physician posture demonstrates respect, and honoring patient's wishes could improve physician-patient communication. Although a sitting posture alone is unlikely to compensate for poor communication skills, asking patients about their preference regarding physician posture, and then following that preference, can be a simple, practical, and inexpensive way of improving communication, and, as a result, patient outcomes. This strategy should be taught in the medical curriculum.

Rituximab-based therapy and long-term control of autoimmune autonomic ganglionopathy.

We present a patient with autoimmune autonomic ganglionopathy (AAG) who had persistently positive ganglionic nicotinic acetylcholine receptor antibody levels despite immunosuppressive therapy. Rituximab-based therapy for an incidental lymphoma was associated with prolonged symptomatic and serological control of AAG.

The impact of physician posture during oncology patient encounters.

Non-verbal communication is an important component of the physician-patient interaction. Oncology patients face specific emotional and psychological issues requiring additional physician emotional support. Multiple studies in oncology patients have revealed that patients perceive physicians seated during the medical interview to be more compassionate, caring, and likely to spend more time with the patients. These are all associated with improved patient outcomes. Barriers to sitting may be due to those imposed by time, space, and reduced perceived benefit of sitting by the physician. Although a sitting posture alone is unlikely to compensate for poor communication skills, assessing patient preference to physician posture, and following their preference, can be a simple way of improving communication, and thus patient outcomes, especially in oncology patients. The widespread introduction of the electronic medical record (EMR) system over the last decade has added a "third wheel" to the original dyadic physician-patient relationship. Physician posture and eye gaze towards to the EMR and its components has a deleterious effect on communication. Appropriate training and sensitization in this regard should be provided for physicians.

Physician Posture at the Bedside: A Study of African-American and Hispanic Patient Preferences at a Teaching Hospital.

The physician-patient interaction is central to any clinical encounter. Although the technical components of the interaction are conveyed by verbal communication, the nonverbal cues are instrumental in setting up the physician-patient relationship and carrying it forward. Often, patient preferences in terms of non-verbal communication cues depend on the ethnicity of the patient. In this article, we present a study of Hispanic and African-American patients regarding their preference of physician posture. Together, these groups represent almost one third of the U.S. population. In our study, a majority of patients preferred their physicians to be seated during the medical interview. Although these results were similar across cultural groups, subtle differences were observed in patient preference regarding other nonverbal communication methods by physicians. It is important for physicians to be aware of these differences. Including course-work that highlights cultural sensitivities in the medical school curriculum is a good way to create awareness among the next generation of physicians.

Splanchnic vein thrombosis in acute pancreatitis: a single-center experience.

OBJECTIVES: This study aimed to estimate outcomes of splanchnic vein thrombosis (SVT) in hospitalized patients with acute pancreatitis (AP). METHODS: This was a retrospective study (January 1996 to December 2006) via chart review. RESULTS: Over 10 years, 1.8% (45/2454) of patients with AP with a mean (SD) age of 58 (15) years were diagnosed with SVT. Splenic vein thrombosis was the most common form of SVT (30/45 patients, 67%). Seventeen patients were anticoagulated with heparin, when the SVT was diagnosed in the acute stage followed by oral anticoagulation (AC). The thrombosis that was most commonly anticoagulated was portal vein thrombosis in 11 (65%) of 17 patients. Of 17 patients in the AC group, 2 (12%) showed recanalization as compared with 3 (11%) of 28 patients in the non-AC group (P > 0.05). The mortality was 3 (7%) of 45 (2 from the AC group versus 1 in the non-AC group, P > 0.05). Two of these died of multiorgan failure, and the other, from septic shock. None of the deaths were due to bleeding complications. CONCLUSIONS: Splanchnic vein thrombosis occurred in 1.8% patients of AP. The use of AC was reasonably safe with no fatal bleeding complications. However, there was no significant difference in the recanalization rates in those with and without AC.

Long-term follow-up of patients with monoclonal gammopathy of undetermined significance after kidney transplantation.

INTRODUCTION: Long-term data regarding kidney transplantation (KTx) patients with monoclonal gammopathy of undetermined significance (MGUS) are scarce. We evaluated the long-term outcomes of these patients in a single-center retrospective study from the Mayo Clinic, Rochester, Minn., USA. METHODS: Patients who had an MGUS before transplant or developed one after KTx were selected. Monoclonal protein was screened as part of the KTx evaluation by serum protein electrophoresis. Screening for posttransplant lymphoproliferative disorder (PTLD) or MGUS after transplant was not required by protocol. Patients with multiple myeloma, dysproteinemia-related kidney disease or no pretransplant serum protein electrophoresis were excluded. RESULTS: Between 1963 and 2006, 3,518 patients underwent KTx. MGUS was identified in 42 patients, with 23 before transplant and 19 after transplant. Median follow-up for these patients was 8.5 years (range 0.3-37). Four (17.4%) pretransplant MGUS patients developed a hematologic malignancy: 2 smoldering multiple myeloma and 2 PTLD - an Epstein-Barr virus-positive diffuse large cell lymphoma and a Hodgkin lymphoma. None of the 19 patients who developed an MGUS after transplant progressed to multiple myeloma, but 2 (10.5%) developed Epstein-Barr virus-negative T cell lymphoproliferative disorders at 16 and 26 years after transplant. Median survival was 26.1 and 28.0 years for the pretransplant and posttransplant MGUS groups, respectively. CONCLUSION: Progression from true MGUS to multiple myeloma is rare after KTx. KTx appears safe in true MGUS patients if the monoclonal gammopathy was not the cause of the kidney disease. None of the patients progressed to multiple myeloma, but 2 developed smoldering multiple myeloma and several developed PTLD. Further studies are needed to explain the relationship between MGUS and PTLD.

Platelet and red blood cell indices in Harris platelet syndrome.

Inherited thrombocytopenias, including inherited giant platelet disorders (IGPD) or macro thrombocytopenias are relatively rare, but their prevalence is likely underestimated from complexities of diagnosis and a spectrum of subclinical phenotypes. Harris platelet syndrome (HPS) is the most common IGPD reported from the Indian subcontinent. Of note there are an increased number of hemoglobinopathies reported from the geographic location. We analysed red blood cell and platelet indices of blood donors with HPS from the north eastern part of India and compared them with blood indices of blood donors of south India. We found a statistically significant lower platelet count in blood donors with HPS (median, range) 132 (71-267) vs. 252 (160-478) as compared to donors from south India (P < 0.001). Mean platelet volume (MPV) was higher in donors with HPS 13.1, (range 12-21.9 fl) as compared to donors from south India 7.35 (range 6-9.2 fl) (P < 0.001). This study showed that blood donors with HPS had a low median platelet bio-mass 0.17 (0.10-0.38%) vs. 0.19 (0.13-0.28%) in donors from south India. The platelet distribution width (PDW) was 17.4 (14.9-19.6) in donors with HPS vs. 16.38 (15.2-18.5) in south Indian blood donors (P < 0.001). Thirty-three donors with HPS had a normal platelet count with MPV more than 12 fL. Only donors with HPS had giant platelets and thrombocytopenia on peripheral blood smear examination. None of these donors had Dohle body inclusion in their leukocytes. Compared to donors from south India, donors with HPS had a significantly lower hemoglobin 13.8 (12-16.3 gm/dL) vs. 14.8 (12-18) respectively (P < 0.001) while red distribution width (RDW) was higher in HPS 13.6 (11.5-16.7) vs. 12.8 (11.4-15.1). However we did not find any statistically significant difference in MCV, MCH, MCHC between the two groups. Peripheral blood smear did not show any obvious abnormal red blood cell morphology. In the blood donors with HPS we found a statistically higher MPV, RDW and a lower platelet count and platelet biomass. A population-based study will be helpful in determining the existence of any hemoglobinopathies among subjects with HPS.

An innovative model for tuberculosis control: an academic medical center-public health department partnership.

Between 1996 and 1999, the incidence rate of active tuberculosis (TB) in Olmsted County, Minnesota, increased by 365%--from 3.4 cases per 100,000 population to 15.8 per 100,000 people. The need for early detection and treatment of TB, efficient care delivery, and cost containment led to the establishment in 2001 of an innovative centralized TB clinic. The clinic was established through a collaboration between Mayo Clinic and the Olmsted County Public Health Department. Following its inception, conversion rates for sputum-positive culture increased from 69.2% to 92%, and the percentage of patients taking part in directly observed therapy increased from 20.8% to 94.6%. Because of successful medical outcomes and acceptance by patients, providers, and the community, the clinic model lends itself to replication elsewhere in the United States.