Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome following allogeneic HPC transplantation: a diagnostic dilemma.

Background: Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) has been described as a specific sequela of allogeneic HPC transplantation (HPCT). Nevertheless, because multiple transplant-related sequela can cause the characteristic clinical features of TTP-HUS, the diagnosis is difficult.

Study Design And Methods: All English-language articles describing patients with TTP-HUS following HPCT were identified.

Severe deficiency of VWF-cleaving protease (ADAMTS13) activity defines a distinct population of thrombotic microangiopathy patients.

Background: Severe deficiency of ADAMTS13 activity is a biologic risk factor for thrombotic microangiopathy (TMA). It was hypothesized that severe ADAMTS13 deficiency is associated with a distinct TMA subpopulation.

Study Design And Methods: ADAMTS13 activity before treatment was determined retrospectively in 107 adult TMA patients treated with plasma exchange.

Management of adult patients with persistent idiopathic thrombocytopenic purpura following splenectomy: a systematic review.

Background: Treatment of chronic refractory idiopathic thrombocytopenic purpura is a dilemma because many patients have minimal symptoms, response to treatment is uncertain, and treatments may have serious adverse effects.

Purpose: To determine the effectiveness of treatments for adult patients with idiopathic thrombocytopenic purpura who have not responded to splenectomy.

Data Sources: English-language reports from 1966 through 2003 that were retrieved from MEDLINE and Reference Update and bibliographies of retrieved articles.

The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry: a community perspective of patients with clinically diagnosed TTP-HUS.

The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry enrolls all consecutive patients for whom plasma exchange treatment is requested for clinically diagnosed TTP-HUS within a defined geographic region. During 14.5 years, from January 1, 1989 until June 30, 2003, 301 patients have been enrolled; follow-up is complete on 300 patients.

Initial management of immune thrombocytopenic purpura in adults: a randomized controlled trial comparing intermittent anti-D with routine care.

We conducted a randomized clinical trial in adults with a new diagnosis of ITP and a platelet count <30000/muL to test the hypothesis that initial intermittent treatment with anti-D may avoid or defer the need for splenectomy when compared to current routine care (glucocorticoid treatment, followed by splenectomy). Splenectomy was to be performed in the anti-D group if patients failed to respond to three consecutive anti-D treatments given within 10 days. The incidences of splenectomy were 14 of 37 (38%) in the routine care group and 14 of 33 (42%) in the anti-D group (absolute risk reduction = 4.

Relationship between age, prostate volume, prostate-specific antigen, symptom score and uroflowmetry in men with lower urinary tract symptoms.

Objectives: To estimate the relationship between age and the values of different diagnostic tests in men with lower urinary tract symptoms (LUTS) suggestive of benign prostatic enlargement (BPE) and to compare prostate growth curves between the normal population sample, healthy men and men with LUTS.

Material And Methods: A series of 354 men (mean age 70.2 years; range 45-91 years) with LUTS due to BPE were stratified into seven age groups and reviewed retrospectively.

Clinical diagnosis of bladder outlet obstruction in men with lower urinary tract symptoms: reliability of commonly measured parameters and the role of idiopathic detrusor overactivity.

Aims: There is no generally accepted consensus how to evaluate patients with lower urinary tract symptoms (LUTS) suggestive of bladder outlet obstruction (BOO). We have tried to determine whether the most frequently used objective variables as prostate volume, IPS-score, maximum flow rate, residual urine volume, functional bladder capacity, and pressure-flow study are reliable for diagnosis of BOO and we investigated the influence of idiopathic detrusor overactivity (IDO) on this condition.

Methods: A total of 153 men with LUTS and suspected BOO were systematically examined with routine investigation including digital rectal examination, transrectal ultrasound (TRUS), post-void residual urine volume measurement, uroflowmetry, and pressure-flow study.

A youth development approach to profiling sexual abstinence.

Objective: To use youth and parent demographic factors, youth assets (strengths), and community constructs to develop age-group-specific profiles for youth who abstain from sexual activity.

Methods: Data were collected using in-person interviews of youth (N = 1253) residing in randomly selected, inner-city neighborhood households. Data were analyzed to create classification trees.

A support group for patients who have recovered from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS): The six-year experience of the Oklahoma TTP-HUS Study Group.

A support group for patients who have recovered from thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS), named The Oklahoma TTP-HUS Study Group, has been a successful program for 6 years. This group has met 3 times each year with an average attendance of 16 former patients; in addition, an average of 14 family members and friends have attended each meeting. Eighty-four percent of patients who attended a meeting were women as compared to 68% women among those who did not attend (P = 0.

ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients.

Initial management of patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is difficult because of lack of specific diagnostic criteria, high mortality without plasma exchange treatment, and risks of plasma exchange. Although severe ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 repeats) deficiency may be specific for TTP, the role of ADAMTS13 activity measurements for initial management decisions is unknown. ADAMTS13 was measured before beginning plasma exchange treatment in 142 (88%) of 161 consecutive patients with clinically diagnosed TTP-HUS with assignment to 1 of 4 categories: less than 5% (severe deficiency), 5% to 9%, 10% to 25%, and more than 25%.

Self-reported initial management of childhood idiopathic thrombocytopenic purpura: results of a survey of members of the American Society of Pediatric Hematology/Oncology, 2001.

The purpose of this study was to update physicians' self-reported initial management practices for childhood idiopathic thrombocytopenic purpura (ITP) from an initial survey in 1997. A questionnaire was sent by e-mail in October 2001 to 753 members of the American Society of Pediatric Hematology/Oncology (ASPH/O). The questionnaire had 14 questions, based on the clinical presentation of a 5-year-old boy with ITP, a platelet count of 7,000/microL, scattered petechiae, and no mucous membrane bleeding.

Correlates of thrombin generation in patients with advanced prostate cancer.

Thrombin generation is increased in men with advanced prostate cancer. Thrombin has the ability to interact with, and affect the biology of, a variety of cell types including prostate cancer cell lines. We therefore looked for correlations between thrombin generation and other markers of disease activity in spot urine samples obtained from men with advanced prostate cancer.

Reliability and validity of the youth asset survey (YAS).

Purpose: To describe the construction of a survey that was developed to measure youth assets. The paper details the ten youth developmental assets measures within the survey, including the psychometric properties of those measures derived from factor analysis and reliability testing.

Methods: Three studies were conducted, including a study of 1,350 randomly selected youth (mean age = 15.

Cytokine signaling and hematopoietic homeostasis are disrupted in Lnk-deficient mice.

The adaptor protein Lnk, and the closely related proteins APS and SH2B, form a subfamily of SH2 domain-containing proteins implicated in growth factor, cytokine, and immunoreceptor signaling. To elucidate the physiological function of Lnk, we derived Lnk-deficient mice. Lnk(-/-) mice are viable, but display marked changes in the hematopoietic compartment, including splenomegaly and abnormal lymphoid and myeloid homeostasis.

Adolescent tobacco use: the protective effects of developmental assets.

Purpose: To examine the effects of 10 youth developmental assets on adolescent tobacco use.

Design: Survey of a randomly selected sample using in-home interviewing methodology.

Setting: Inner-city areas of two midsized Midwestern cities.

An adolescent age group approach to examining youth risk behaviors.

Purpose: To investigate relationships among youth risk behaviors and demographic factors using an adolescent age group approach.

Design: Cross-sectional data from a randomly selected population. Risk behaviors were compared within specific demographic factors and by adolescent age groups.

Factor V Leiden: a genetic risk factor for thrombotic microangiopathy in patients with normal von Willebrand factor-cleaving protease activity.

Thrombotic microangiopathy (TM) is associated with abnormalities of von Willebrand factor-cleaving protease (VWCP) and other hemostatic factors. This study hypothesized that TM patients might have genetically determined thrombotic risk factors that predispose them to aberrant microvascular thrombosis. DNA samples from 30 white and 12 African American adult TM patients were analyzed for genetic alleles associated with vascular thrombosis, and plasma samples were analyzed for levels of VWCP activity.

Quinine-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: frequency, clinical features, and long-term outcomes.

Background: Quinine-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is thought to be uncommon and to have a good prognosis.

Objective: To describe the frequency, clinical features, and long-term outcomes of quinine-associated TTP-HUS.

Design: Case series.

Unintentional platelet removal by plasmapheresis.

Therapeutic plasmapheresis may remove platelets as well as plasma. Unintentional platelet loss, if not recognized, may lead to inappropriate patient assessment and treatment. A patient with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is reported in whom persistent thrombocytopenia was interpreted as continuing active disease; thrombocytopenia resolved only after plasma exchange treatments were stopped.