Christian Cable

Eculizumab safely reverses neurologic impairment and eliminates need for dialysis in severe atypical hemolytic uremic syndrome.

This case report describes how eculizumab reversed neurologic impairment and improved renal damage in severe atypical hemolytic uremic syndrome. A 50-year-old female, after presenting with diarrhea and abdominal pain, developed pancolitis, acute renal failure, and thrombocytopenia. The patient underwent total abdominal colectomy. Pathology confirmed ischemic colitis with scattered mesenteric microthrombi. Due to mental and respiratory decline, she remained intubated. Continuous venovenous hemodialysis was initiated. Renal failure, neurologic changes, hemolysis, thrombotic microangiopathy, and low complement levels all suggested atypical hemolytic uremic syndrome. Eculizumab 900 mg was administered intravenously on hospital day 6 and continued weekly for four doses followed by maintenance therapy. She recovered neurologically and renally after the third dose, and hematologically by the sixth dose. Her recovery has been sustained on long-term eculizumab treatment. In severe atypical hemolytic uremic syndrome, eculizumab safely reverses neurologic impairment and eliminates the need for dialysis. The optimal duration of treatment with eculizumab remains to be determined.

Reduced dose maintenance eculizumab in atypical hemolytic uremic syndrome (aHUS): an update on a previous case report

Objective To describe how maintenance eculizumab sustains improved renal function in severe atypical hemolytic uremic syndrome (aHUS). Case report A previously described 50-year-old woman with aHUS had a remarkable recovery with eculizumab, which safely reversed profound neurologic damage and eliminated the need for dialysis. Her recovery has been sustained on long-term eculizumab treatment. She initially received eculizumab 900 mg weekly for four doses. On week 5 she commenced maintenance therapy starting at 1200 mg every 2 weeks. Due to nausea and vomiting at that dose, the maintenance dosing was reduced to 600 mg weekly, beginning on dose seven. After receiving 600 mg weekly for nine doses, eculizumab was then reduced to 600 mg every 2 weeks, with continued improvement in renal function. This dosing is lower than the usual 1200 mg every 2 weeks described in the adult literature and used in current clinical trials of aHUS. Conclusion Six months after the initial diagnosis, our patient continues to have improved renal function on maintenance doses of eculizumab as low as 600 mg every 2 weeks.

Prevalence of anemia after Roux-en-Y gastric bypass surgery: what is the right number?

BACKGROUND The reported prevalence of anemia after malabsorptive bariatric surgery has varied from 5% to 64% in the post-Roux-en-Y gastric bypass (RYGB) population, owing to the small study sample sizes and generally poor follow-up in surgical referral practices. The present study estimated the prevalence of anemia in the post-RYGB population and determined whether an integrated healthcare system with a shared electronic medical record could improve postoperative follow-up. METHODS The medical records from 1009 sequential patients who had undergone RYGB from 2000 to 2005 were retrospectively analyzed. All anemia data within the healthcare systems electronic medical record were accessible. RESULTS Of the 1009 medical records, 720 contained ≥1 year of follow-up data concerning anemia. Anemia had developed or worsened in 259 patients, for a prevalence of 36% in the present sample. The anemia was moderate to severe (hemoglobin <10 g/dL) in 88 patients (12.2%.), many of whom subsequently required parenteral iron. Of the 1009 patients, the follow-up data for ≥3 years concerning anemia were available for only 357 patients (35.4%). CONCLUSION The results of the present study have shown that in a large population of patients undergoing RYGB, the prevalence of anemia was great enough to justify more intensive long-term screening. We hope the findings from the present report influence the national screening standards for this patient population.

The patient panel conference experience: what patients can teach our residents about competency issues.

Purpose In 2007, the Scott & White/Texas A&M HSC College of Medicine began requiring all internal medicine residents to attend quarterly patient panel conferences, during which former Scott & White patients speak frankly about their inpatient and outpatient experiences. The main purpose of this mixed-methods pilot study was to determine whether residents’ competency education could be enhanced via the conferences. Method Of the 54 internal medicine residents in the residency program, 31, 39, and 41 participated in three patient panel conferences, respectively, between December 2007 and August 2008. Each resident completed an assessment that included a reflection on his or her own practice and the identification of competency issues highlighted by patients’ oral narratives. Content analyses of responses to open-ended questions were performed. Consensus on themes was reached. Descriptive statistics were run on quantitative data. Results Six themes were identified: improve communication with patients/families, improve patient care, improve professional behaviors, empathize with patients/families, display sensitivity to patients’/families’ needs/concerns, and recognize system issues. When asked if the conference highlighted competency problems, residents answered “agree” or “strongly agree” as follows: 82% for professionalism, 82.9% for systems-based practice, 85.2% for interpersonal and communication skills, and 84.4% for patient care. The majority were able to provide examples of competency issues. Conclusions The patient panel conference experience was a powerful mechanism for enhancing competency education. The conferences were an effective means of presenting real-life examples of systems issues in the context of a hospital system.

Patients' attention to and understanding of adverse drug reaction warnings

Correspondence: Tresa Muir McNeal Scott & white Hospital, Dept of Internal Medicine, 2401 South 31st St, Temple, TX 76508, USA email tmcneal@swmail.sw.org Introduction: Medications are critical to the management of patient conditions, and they can have significant effects on the success or failure of medical interventions. Patient perceptions of drug warnings play an important role in medication compliance and ultimately disease management. Several factors may affect patients’ understanding of drug warnings and drug labeling, including health literacy and interactions with physicians and pharmacists. Purpose: The purpose of this article is to provide a review of the literature related to patient perceptions of drug warnings and drug labeling. Descriptive articles and studies regarding patient perceptions and knowledge of adverse drug reaction warnings were reviewed. Methods: The following databases were utilized to search the literature related to patient perceptions of drug warnings: PubMed, Academic Search Premiere, CINAHL, Medline, Psych Info, Business Source Complete, Alternative Healthwatch, Health Source (both Nursing/Academic and Consumer additions), JSTOR, and Master File Premiere. For the purpose of this review, any peer-reviewed article was eligible. Exclusionary criteria included: articles published in languages other than English, articles/studies on patient perceptions of vaccines and chemotherapy, and articles related to perceptions of medications administered in the inpatient setting. Forty-six articles were included in the review. Results: Health literacy has been shown to have a major impact on patients’ ability to understand potential adverse reactions and instructions on correct dosing of medications. Direct communication with physicians and pharmacists is one of the most important and effective variables in promoting understanding of drug warnings. Appropriateness of written medical information that is informative and timely can improve patients’ perceptions of drug warnings and hopefully disease management. Conclusion: As patients increasingly assume more personal responsibility as informed consumers of health care, it is even more important to address patient perceptions of drug warnings considering how this fits in the context of their overall care.

Newly diagnosed congenital factor VII deficiency and utilization of recombinant activated factor VII (NovoSeven(

This case report presents a newly diagnosed congenital factor VII deficiency treated with recombinant activated factor VII (rFVIIa). Congenital factor VII deficiency is a rare autosomal-recessive bleeding disorder that occurs in fewer than 1/500,000 persons. Its presentation can vary from epistaxis to hemarthroses and severe central nervous system bleeding, and correlates poorly with factor VII levels. Our patient had not had a significant hemostatic challenge prior to his presentation and therefore never had any symptomatology suggestive of this disease. He was treated with rFVIIa, and was able to undergo repair of his fractures without bleeding. Case report A 19-year-old African-American male presented to the emergency room after an altercation that resulted in significant trauma. He sustained bilateral mandibular angle fractures and orbital floor fractures, requiring urgent surgical correction. On initial evaluation, he was noted to have a prolonged prothrombin time of 40.1 seconds, with an International Normalized Ratio of 4.0, a normal activated partial thromboplastin time of 29.9 seconds, and a platelet count of 241. After receiving vitamin K and fresh frozen plasma, he was taken to the operating room for a temporary rigid maxillomandibular fixation. A 1:1 mixing study with normal plasma corrected the prothrombin time (decreasing from 40.7 to 14.7 seconds) and a factor VII assay revealed 5% of the normal factor VII level. The patient was diagnosed with congenital factor VII deficiency. Due to his coagulopathy and the extensive surgical correction needed, rFVIIa was administered and surgery was accomplished without hemorrhagic sequelae. Conclusion This case report and review describes a rare congenital disease, the history of rFVIIa use, and its mechanism. rFVIIA use in our patient provided a treatment option that allowed the necessary surgical correction, but further prospective studies on dose optimization would ensure adequate dosing with minimal risk of severe side effects.

Learner Perspectives of a Surgical Educators Faculty Development Program Regarding Value and Effectiveness: A Qualitative Study.

Background: The BaylorScott & White Health Division of Plastic Surgery began a faculty development program designed to train clinicians to be better educators. The program consisted of presession reading, 11 small group didactic sessions, and individually chosen educational projects. Cross-discipline collaboration was pursued by enrolling faculty and students from diverse departments. Department chair permission was required for participation. The purpose of this qualitative study was to explore learner perspectives of the Surgical Educators Program. Methods: Fourteen physicians completed the Surgical Educators Program. Focus groups were held with the learners who completed the 2011 and 2012 programs 6 months after completion. The groups were moderated by an educational faculty member who was not involved in any aspect of the course. Questions were designed to elicit the components of the course curriculum of significance to the learners. Narrative data were digitally recorded and transcribed verbatim, and the investigators performed independent content analyses to identify themes. The data were thematically coded and summarized using calculation of frequencies. Results: Thirteen learners participated in the focus groups. Three main themes were identified. First, the program increased the participants’ knowledge and practice of medical education. Second, the structure of the program was a key contributor to the outcomes. Third, the program produced a community of practice. Conclusions: This study suggests that a faculty development program comprising a diverse group of physicians consisting of readings, guided small group sessions, and a mandatory project can train plastic surgical faculty to become better surgical educators within the constraints of a busy clinical practice.

A 41-Year-Old Man With Massive Splenomegaly, Fevers and Night Sweats

CASE PRESENTATION, JEFFRY BLACKWELL, MD (SENIOR RESIDENT IN INTERNAL MEDICINE) A 41-year-old man presented to the emergency department complaining of weakness, fevers and night sweats for the past several months. He described a 6-month history of progressive generalized weakness, noting that, during the past 3 months, he had developed nonproductive cough and frequent drenching night sweats. The severity of his weakness confined him to his home in the 2 months before presentation. The patient’s medical history was significant for hepatitis C and was treated successfully with pegylated interferonalpha 3 years before. In addition, he had experienced constipation during the past 1 year and had undergone a Lord anal dilatation procedure because of severity of the complaint. At the time of the operation, approximately 6 months before his presentation, he had been told that his blood counts were “low.” The patient’s medications were Senna and a multivitamin. He smoked 1 pack of cigarettes monthly and imbibed mixed drinks twice weekly. Because of severe fatigue during the past year, he used occasional oral methamphetamines. He had never used intravenous drugs. The patient is homosexual and stated that he usually used condoms. His last sexual contact was 2 years before presentation. At the time of hepatitis C treatment, he was human immunodeficiency virus (HIV) negative per his report. Family history was noncontributory. Review of systems was additionally positive for a 10-pound weight loss, poor appetite, early satiety, depressive symptoms, easy bruising and diffuse myalgias. The patient was dyspneic on exertion. There was no history of hemoptysis. There was no orthopnea, paroxysmal nocturnal dyspnea, chest pain or palpitations. Physical examination revealed a thin, chronically ill-appearing Caucasian white man. Vital signs were as follows: temperature 100.9 F, pulse 87 beats per minute, respiratory rate 14 breaths per minute and blood pressure 119/71 mm Hg. The patient was breathing comfortably on room air with a recorded oxygen saturation of 100%. No scleral icterus was present. The oral cavity had good dentition without evidence of thrush. No neck masses or lymphadenopathies were palpable. Heart rhythm was regular with a slight systolic flow murmur noted. Lung examination was remarkable for right lower lobe expiratory wheezing that cleared after deeper effort. The left hemiabdomen was firm from the costal margin to pelvis, and no spleen tip was discernable. Hepatomegaly was not noted. Trace lower extremity edema was present. No skin rashes were present. Neurologic examination revealed normal cranial nerve function, reflexes, speech, mental status and gait. No focal weakness or sensory loss was present. Rectal examination was not performed because of the initial laboratory finding of neutropenia. On presentation, the complete blood count was remarkable for a white blood cell count of 600 10/L with 9% granulocytes, 71% lymphocytes, 11% monocytes and 9% eosinophils. Hemoglobin was 7.2 g/dL with mean cellular volume of 87, and the platelet count was 73,000. Review of the peripheral blood smear confirmed severe pancytopenia. No nucleated red blood cells or younger white blood cell forms were noted. Creatinine was 0.81 mg/dL, and serum electrolytes and liver enzymes were normal. Calcium was 7 mg/dL, and serum albumin was 2.3 mg/dL. Reticulocyte count was 5.8% (normal 1%), lactate dehydrogenase was 240 units/L and haptoglobin was detectable at 37 mg/dL. An acute viral hepatitis panel test was negative. Antibodies to HIV1/2 were negative. Hepatitis C quantitative polymerase chain reaction revealed no viral replication. Urine drug screen was negative, as was urine screen for heavy metal. Blood cultures were obtained and remained negative. Parvovirus, cytomegalovirus and Epstein-Barr virus testings were negative. A computed tomography scan of the chest/abdomen/ pelvis (Figure 1) demonstrated bibasilar ground glass opacities, interlobular septal thickening, nodular upper lobe densities, mediastinal and right hilar lymphadenopathy and a massively enlarged heterogeneous spleen (Figure 2). A bone marrow biopsy and aspiration was performed. The aspirate was free flowing and demonstrated normal morphology with mature granulocytic and lymphocytic cell lines. The biopsy showed 95% cellularity with hyperplasia of all the 3 cell lines. BacteFrom the Texas A&M HSC College of Medicine, College Station, Texas; and Scott and White Memorial Hospital, Temple, Texas. Submitted June 1, 2010; accepted in revised form July 9, 2010. Correspondence: Jules B. Puschett, MD, Neuroscience and Experimental Therapeutics, Texas A&M College of Medicine/Scott and White Memorial Hospital, 2401 S. 31st Street, Temple, TX 76508 (E-mail: jpuschett@ swmail.sw.org).

The wandering spleen: an unusual case of thrombocytopenia

Thrombocytopenia is a common laboratory finding in current medical practices. The workup of thrombocytopenia can be challenging with numerous causes that can be included in the differential diagnosis. Thrombocytopenia can be due to bone marrow hypoproliferation, peripheral destruction, or sequestration. This paper presents a case of isolated thrombocytopenia in a young female and discusses the causes of thrombocytopenia.

Leptomeningeal myelomatosis in previously treated high-risk kappa light chain multiple myeloma: case report and literature review

Correspondence: Maro Ohanian Department of Hematology/Oncology, scott and White Healthcare, 2401 south 31st street, Temple, TX 76508, UsA Tel +1 281 217 1974 Fax +1 254 771 4890 email ohanianus@yahoo.com Objective: To describe leptomeningeal myelomatosis (LM) in previously treated high-risk kappa light chain multiple myeloma (MM) and to review the literature. Case report: A 71-year-old female with previously treated kappa light chain myeloma presented with right lumbosacral discomfort. Magnetic resonance imaging (MRI) of spine revealed multiple intradural masses involving the cauda equina, with mass effect on adjacent nerve roots. Brain MRI was unremarkable. Cerebrospinal fluid flow cytometry confirmed an abnormal population of plasma cells with kappa restriction; CD38, CD138, and CD56 were positive. She was originally diagnosed with kappa light chain myeloma 10 months earlier while hospitalized for anemia, thrombocytopenia, renal failure, and hypercalcemia. Bone marrow revealed plasma cell myeloma approaching 100% cellularity, with 92% plasma cells, atypical plasmacytoid cells with prominent nucleoli, and significant cytogenetic abnormalities: deleted 13, c-myc rearrangements, −X, +1. Treatments consisted of seven cycles of bortezomib with weekly dexamethasone. Her last dose had been 4 months earlier. After treatment, bone marrow demonstrated a complete remission with normal cytogenetics. Her clinical course had otherwise been indolent with a good hematologic response. After diagnosis of LM, therapy included focal external beam radiation to the cauda equina, weekly bortezomib and dexamethasone, intrathecal (IT) cytarabine liposomal every 2 weeks for five doses, and monthly IT cytarabine liposomal thereafter. The cerebrospinal fluid gradually cleared on serial lumbar punctures and follow-up MRI demonstrated near complete resolution of the intradural masses. Five months after diagnosis the patient is essentially asymptomatic. Conclusion: The incidence of central nervous system (CNS) involvement in MM patients is 1%. LM is associated with cytogenetic abnormalities and plasmablastic morphology. It can occur with a seemingly low tumor burden. Novel agents such as bortezomib allow for prolonged survival in high-risk patients; however, with inadequate CNS penetration, complications such as LM may be inevitable.