How I diagnose and treat thrombocytopenia in geriatric patients.

ABSTRACT: Thrombocytopenia in older individuals is a common but diagnostically challenging condition that has variable clinical impact to those who are affected. Diagnostic approach requires evaluation of the preexisting clinical conditions, detailed review of medications, and assessment for disorders that warrant urgent treatment. In this article, we describe a systematic approach to diagnosis of thrombocytopenia and present a schematic review for management strategies. Three clinical scenarios are presented that are relevant for their prevalence and management challenges in an older adult population. The first scenario addresses primary immune thrombocytopenia (ITP) and reviews different treatment options. The second one addresses complications of thrombocytopenia in management of the myelodysplastic syndrome. The last one reviews diagnostic challenges of drug-induced ITP.

Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia.

DESCRIPTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications. METHODS: The guidelines were developed using the AGREE II (Appraisal of Guidelines for Research and Evaluation II) framework and GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. The guidelines expert panel included expert physicians (clinical and genetic) in HHT from 15 countries, guidelines methodologists, health care workers, health care administrators, patient advocacy representatives, and persons with HHT. During the preconference process, the expert panel generated clinically relevant questions in 6 priority topic areas. A systematic literature search was done in June 2019, and articles meeting a priori criteria were included to generate evidence tables, which were used as the basis for recommendation development. The expert panel subsequently convened during a guidelines conference to conduct a structured consensus process, during which recommendations reaching at least 80% consensus were discussed and approved. RECOMMENDATIONS: The expert panel generated and approved 6 new recommendations for each of the following 6 priority topic areas: epistaxis, gastrointestinal bleeding, anemia and iron deficiency, liver VMs, pediatric care, and pregnancy and delivery (36 total). The recommendations highlight new evidence in existing topics from the first International HHT Guidelines and provide guidance in 3 new areas: anemia, pediatrics, and pregnancy and delivery. These recommendations should facilitate implementation of key components of HHT care into clinical practice.

Difficult to swallow: warm autoimmune hemolytic anemia in a Jehovah's Witness treated with hemoglobin concentrate complicated by achalasia.

BACKGROUND: The acute treatment of severe warm autoimmune hemolytic anemia (wAIHA) is focused on maximizing the oxygen delivery capacity of the patient's circulation and reversal of the underlying autoimmune process. The most effective means of preventing ischemic injury acutely is replacement of red blood cells (RBCs) via allogeneic RBC transfusion. However, in cases where this is not an option, other strategies must be considered including the use of hemoglobin-based oxygen carriers (HBOCs). CASE REPORT: Herein we present a case of a 70-year-old Jehovah's Witness with wAIHA who required emergent HBOC-201 to prevent life-threatening decompensation. The treatment was complicated by hypertension and achalasia likely related to the nitric oxide scavenging effects of HBOC-201. These side effects were managed appropriately, and the patient ultimately recovered. CONCLUSION: Early recognition of the need and ready familiarity with its properties on the part of the physician are critical to the utilization of HBOC-201 in a safe and timely fashion.

Using novel PF4-dependent P-selectin expression assay to diagnose heparin-induced thrombocytopaenia postliver transplantation.

Heparin-induced thrombocytopaenia (HIT) is a well-known adverse event associated with the use of heparin products. HIT may be difficult to diagnose in patients following liver transplantation as patients routinely require massive transfusion support and immunosuppression. As an alternative or adjunctive to the serotonin release assay, the PF4-dependent P-selectin expression assay (PEA) may be a useful diagnostic test in the determination of HIT in this patient population. In this case, we describe a 63-year-old man who had an orthotopic liver transplant that was complicated by HIT that was diagnosed using the PEA.

Change in stated clinical practice associated with participation in the Dental Practice-Based Research Network.

Clinical researchers have attempted many methods to translate scientific evidence into routine clinical practice, with varying success. Practice-based research networks (PBRNs) provide an important, practitioner-friendly venue to test these methods. Dentist practitioner-investigators from the Dental Practice-Based Research Network (DPBRN) completed a detailed questionnaire about how they diagnose and treat dental caries. Next, they received a customized report that compared their answers to those from all other practitioner-investigators. Then, 126 of them attended the DPBRN's first network-wide meeting of practitioner-investigators from all five of its regions. During that meeting, certain questions were repeated and new ones were asked about the dentist's intention to change the way that he or she diagnosed or treated dental caries. Less than one-third of practitioner-investigators intended to change how they diagnosed or treated caries as a result of receiving the customized report. However, as a result of the meeting, the majority of these same practitioner-investigators stated an intention to change toward a more conservative, less surgically invasive approach. These findings are consistent with the idea that a highly interactive meeting with fellow practitioner-investigators may be an effective means to translate scientific findings into clinical practice. Practitioner-investigators are open to changing how they treat patients as a result of engaging fellow practitioner-investigators in the scientific process.

Endocrine and neuroanatomic features associated with weight gain and obesity in adult patients with hypothalamic damage.

PURPOSE: Obesity is a common consequence in patients with tumors of the hypothalamic region and of related treatment in children. Much less information is available on adult patients and long-term survivors. The aims of this study were to estimate the prevalence of obesity in adult patients with acquired structural hypothalamic damage and to define the characteristics of patients at greatest risk of obesity. METHODS: A retrospective study was conducted of 52 patients (25 women; median age at diagnosis, 44 years; range, 17 to 78 years) with tumors involving the hypothalamic region. These included 22 craniopharyngiomas, 24 pituitary adenomas, and six other hypothalamic tumors. Changes in body mass index were determined, magnetic resonance imaging scans were scored by a radiologist for tumor size and the extent of involvement of the hypothalamus, and current hormone replacement therapy was recorded, to identify possible features associated with new or worsened obesity (defined as a body mass index > or =30 kg/m(2) at the latest follow-up, which had increased by at least 2 kg/m(2) since diagnosis of the tumor). RESULTS: Serial body mass index data from diagnosis to the latest follow-up were available for 42 patients. After a median of 5 years (range, 1 to 19 years) of follow-up, most patients with hypothalamic damage were obese (52% [n = 22] vs. 24% [n = 10] at the time of diagnosis, P < 0.0001). In a multivariate model, use of desmopressin (odds ratio [OR] = 13; 95% confidence interval [CI]: 2.0 to 86; P = 0.007) and growth hormone replacement (OR = 7.6; 95% CI: 1.1 to 51; P = 0.04) were associated with new or worsened obesity during follow-up. No correlation was found between the initial size or location of the tumor and subsequent weight gain. CONCLUSION: Obesity is highly prevalent in adult survivors of hypothalamic tumors. Use of desmopressin and growth hormone therapy, but not size or location of the tumor, were associated with weight gain and obesity following diagnosis. These findings may be helpful in identifying patients at increased risk of obesity, to whom earlier intervention could be offered.

An unusual occurrence: a case of venous thromboembolism in pregnancy associated with heterotaxy syndrome.

BACKGROUND: Heterotaxy is a relatively uncommon congenital anomaly that is usually diagnosed incidentally on imaging studies in adults. We present an unusual case of venous thromboembolism in a 26 year old pregnant female with Heterotaxy syndrome. CASE PRESENTATION: A 26 year-old pregnant female at 13 weeks gestation suffered cardiac arrest with successful cardiac resuscitation and return of spontaneous circulation. The cardiac arrest was secondary to massive pulmonary embolism requiring thrombolytic therapy and stabilization of hemodynamics. She had extensive evaluation to determine the etiology for the pulmonary embolism and was noted to have an anatomic variation consistent with heterotaxy syndrome on imaging studies. After thrombolysis the patient was treated with UFH and then switched to enoxaparin without complication until 25 weeks of gestation when she experienced worsening abdominal pain with associated headaches, lightheadedness and elevated blood pressures needing elective induction of labor. The infant died shortly after delivery. The anticoagulation was continued for additional 3 months and she was subsequently placed on low dose aspirin to prevent recurrent venous thromboembolic episodes. She is currently stable on low dose aspirin and is into her third year after the venous thromboembolism without any recurrence. CONCLUSION: To our knowledge, this is the first reported case of venous thromboembolism in pregnancy associated with heterotaxy syndrome. A discussion on pathophysiology of venous thromboembolism in pregnancy and heterotaxy syndrome has been undertaken along with treatment approach in such situations.

Advances in the management of CSF rhinorrhoea.

Cerebrospinal fluid fistulae are under-diagnosed, difficult to locate and often clinically silent. They are potentially lethal and carry a long-term cumulative risk of meningitis. They should be fully investigated and treated aggressively. Current endoscopic techniques combined with intrathecal fluorescein dye enable most defects to be located and sealed with minimal morbidity.

Orofacial paraesthesia: an unusual presentation of acoustic neuroma.

Patients with acoustic neuromas commonly present with unilateral hearing loss, tinnitus and unsteadiness. An uncommon initial feature is involvement of the trigeminal nerve. The unusual feature in the case reported here was orofacial paraesthesia, which preceded hearing loss by 3-4 years. Diagnosis was made following a referral by the patient's dentist.

Neurologic aspects of lymphoma and leukemias.

The lymphomas and leukemias are a heterogenous group of hematologic malignancies with protean manifestations. Neurologic sequelae of the diseases have been recognized since the time the conditions were first described in the mid-1800s. Although our understanding of the various presentations of these blood disorders evolved along with our knowledge of malignancies, accurate diagnosis can still be difficult. It is critical for neurologists to have a high index of clinical suspicion to appropriately recognize their heralding features. This review's focus is the relevant clinical neurologic features and diagnostic studies that identify leukemias and lymphomas affecting the nervous system.

Supplementing online surveys with a mailed option to reduce bias and improve response rate: the National Dental Practice-Based Research Network.

OBJECTIVE: Dentists in the National Dental Practice-Based Research Network are offered online and mail options for most questionnaire studies. We sought to quantify differences a) in characteristics of dentists who completed a questionnaire online as compared with those who completed a mail option offered to online nonresponders and b) in prevalence estimates for certain practice characteristics. METHODS: Invitation letters to participants provided an identification number and log-in code with which to complete the online survey. Nonrespondents received a reminder letter after the fourth week, and after an additional 4-week period, a final reminder was sent, along with a paper questionnaire version, allowing completion online or by paper. RESULTS: Of 632 US dentists who completed the survey, 84 (13 percent) used the paper version. Completion by paper was more common among males, older dentists, and those in general practice (P<0.05). The proportions of dentists who used electronic dental records, who consistently used a rubber dam when performing root canals, and who either worked with or employed expanded-function auxiliaries were lower among dentists who completed the survey using the paper-mail version than among those who completed it online; these differences remained significant in models adjusted for gender, age, and practice type. CONCLUSION: Even in an era of increasingly electronic communication by dentists, not including a paper option when conducting surveys can result in overestimation of the prevalence of key dental practice characteristics.

Purpose, structure, and function of the United States National Dental Practice-Based Research Network.

OBJECTIVE: Following a successful 2005-2012 phase with three regional practice-based research networks (PBRNs), a single, unified national network called "The National Dental PBRN" was created in 2012 in the United States to improve oral health by conducting practice-based research and serving dental professionals through education and collegiality. METHODS: Central administration is based in Alabama. Regional centres are based in Alabama, Florida, Minnesota, Oregon, New York and Texas, with a Coordinating Centre in Maryland. Ideas for studies are prioritized by the Executive Committee, comprised mostly of full-time clinicians. RESULTS: To date, 2763 persons have enrolled, from all six network regions; enrollment continues to expand. They represent a broad range of practitioners, practice types, and patient populations. Practitioners are actively improving every step of the research process, from idea generation, to study development, field testing, data collection, and presentation and publication. CONCLUSIONS: Practitioners from diverse settings are partnering with fellow practitioners and academics to improve clinical practice and meet the needs of clinicians and their patients. CLINICAL SIGNIFICANCE: This "nation's network" aims to serve as a precious national resource to improve the scientific basis for clinical decision-making and foster movement of the latest evidence into routine practice.