Aplastic anaemia in pregnancy - a single centre, North American series.

Aplastic anaemia (AA) is infrequently observed in pregnancy. We describe 19 pregnancies in 9 women at a tertiary care centre over a period of 30 years. Spontaneous resolution of AA did not occur postpartum in the five pregnancies where AA was first diagnosed in pregnancy. In the remaining pregnancies, although haematological indices declined and transfusion support was needed in 35% of pregnancies, relapses were not observed. There were no deaths, but complications occurred in 79% of pregnancies. Preterm delivery and postpartum haemorrhage were observed in 21% and 26% of pregnancies, respectively.

Reducing the hospital burden of heparin-induced thrombocytopenia: impact of an avoid-heparin program.

Heparin-induced thrombocytopenia (HIT) is an adverse drug reaction occurring in up to 5% of patients exposed to unfractionated heparin (UFH). We examined the impact of a hospital-wide strategy for avoiding heparin on the incidence of HIT, HIT with thrombosis (HITT), and HIT-related costs. The Avoid-Heparin Initiative, implemented at a tertiary care hospital in Toronto, Ontario, Canada, since 2006, involved replacing UFH with low-molecular-weight heparin (LMWH) for prophylactic and therapeutic indications. Consecutive cases with suspected HIT from 2003 through 2012 were reviewed. Rates of suspected HIT, adjudicated HIT, and HITT, along with HIT-related expenditures were compared in the pre-intervention (2003-2005) and the avoid-heparin (2007-2012) phases. The annual rate of suspected HIT decreased 42%, from 85.5 per 10 000 admissions in the pre-intervention phase to 49.0 per 10 000 admissions in the avoid-heparin phase ( ITALIC! P< .001). The annual rate of patients with a positive HIT assay decreased 63% from 16.5 to 6.1 per 10 000 admissions ( ITALIC! P< .001), adjudicated HIT decreased 79% from 10.7 to 2.2 per 10 000 admissions ( ITALIC! P< .001), and HITT decreased 91% from 4.6 to 0.4 per 10 000 admissions ( ITALIC! P< .001). Hospital HIT-related expenditures decreased by $266 938 per year in the avoid-heparin phase. To the best of our knowledge, this is the first study demonstrating the success and feasibility of a hospital-wide HIT prevention strategy.

The changing face of childhood celiac disease in north america: impact of serological testing.

OBJECTIVE: The goal was to evaluate the impact of immunoglobulin A endomysial antibody testing on the incidence and clinical presentation of childhood celiac disease. METHODS: The incidence and clinical presentation of celiac disease in patients <18 years of age in 1990-1996 (pretesting group) versus 2000-2006 (testing group) were compared. RESULTS: The median age at diagnosis was 2 years (95% confidence interval: 2-4 years) in the pretesting group (N = 36), compared with 9 years (95% confidence interval: 8-10 years) in the testing group (N = 199; P < .001); the female/male ratios (1.6:1) were similar (P = .982). The incidence of celiac disease increased from 2.0 cases per 100000 children (pretesting group) to 7.3 cases per 100000 children (testing group; P = .0256). The frequency of classic celiac disease presentations decreased from 67% (pretesting group) to 19% (testing group; P < .001), but the incidence of classic celiac disease did not differ (0.8 vs 1.6 cases per 100000; P = .154). In the testing group, 13 previously unrecognized clinical presentations were observed in 98 children, including 35 with family history, 18 with abdominal pain, and 14 with type 1 diabetes mellitus. The frequency of Marsh IIIc lesions decreased from 64% (pretesting group) to 44% (testing group; P = .0403). In the testing group, classic celiac disease remained predominant (67%) in young children (<3 years), whereas atypical gastrointestinal and silent presentations predominated in older children. CONCLUSIONS: Antibody testing for celiac disease tripled the incidence of celiac disease and quadrupled the median age at diagnosis.

Celiac disease and IgA deficiency: complications of serological testing approaches encountered in the clinic.

BACKGROUND: IgA deficiency causes false-negative IgA-based celiac serology results in patients with celiac disease. Using a case-finding strategy, we examined the prevalence of IgA deficiency, physician evaluation, and management of IgA deficiency during serological testing for celiac disease. METHODS: We reviewed consecutive IgA-endomysial antibody (EMA) and serum IgA results from the laboratory database over 17 months. We cross-referenced seronegative patients with IgA deficiency (IgA <0.06 g/L) to the pathology database to evaluate intestinal biopsy results. Ordering physicians received a questionnaire regarding the management of seronegative patients with IgA deficiency who had no biopsy record. RESULTS: Among the 9533 patients tested for IgA-EMA, 4698 (49%) were tested for IgA deficiency. IgA deficiency occurred in 35 of 4698 (0.75%) patients screened for IgA deficiency. Only 19 of 35 (54%) IgA-deficient patients were diagnosed appropriately with either intestinal biopsy (17 patients) or measurement of IgG-tissue transglutaminase (2 patients). Thirteen (76%) of the 17 IgA-deficient patients who underwent upper endoscopy with or without colonoscopy displayed gastrointestinal pathology on biopsies, including 3 (18%) with celiac disease. No further evaluation to exclude celiac disease was performed for the remaining 16 of 35 (46%) IgA-deficient, EMA-negative patients because of inappropriate management (6 patients), administrative error (7 patients), or patient/physician refusal (3 patients). CONCLUSIONS: IgA deficiency occurred in 1:131 patients tested for celiac disease, and celiac disease occurred in 1:6 of those properly evaluated. Inadequate evaluation of IgA deficiency while testing for celiac disease occurred frequently and resulted in the underdiagnosis of both. Changes in testing algorithms and reporting of results were made to improve testing for celiac disease and IgA deficiency.

Celiac disease: are endomysial antibody test results being used appropriately?

BACKGROUND: The aim of this study was to retrospectively examine how positive IgA-endomysial antibody (EMA) test results for celiac disease were being interpreted and acted on by physicians in the Calgary Health Region. METHODS: We reviewed consecutive EMA test results, with or without a serum IgA, obtained during a 17-month period. Seropositive tests were cross-referenced to the surgical database to determine the number of patients who underwent intestinal biopsy and the results of the biopsy. We sent questionnaires to the ordering physicians of positive tests with no record of intestinal biopsy. RESULTS: Among 11,716 EMA tests in 9533 patients, 349 results were positive in 313 patients (3%). Intestinal biopsies were performed in 218 (70%) of the seropositive patients; 194 of them were diagnostic of celiac disease. Celiac disease was also found in 10 EMA-negative patients. Of the 109 positive tests performed in 95 patients with no subsequent biopsy, 28 had appropriate indications to not perform a biopsy; the most common reason being that the test had been ordered to follow up on a previous biopsy-proven diagnosis of celiac disease (n = 21). For 33 other positive test results without a subsequent biopsy, management appeared to be inappropriate, most commonly (n = 21) because of a recommendation to follow a gluten-free diet despite lack of a tissue diagnosis of celiac disease. For the remaining 48 positive EMA results, administrative issues prevented evaluation (n = 19), the patients refused further evaluation (n = 11), or physician surveys were not returned (n = 18). CONCLUSIONS: Celiac disease affected 2% of patients, with a similar prevalence in male and female patients. Most positive EMA tests (77%) were appropriately managed by physicians. Beginning a gluten-free diet without biopsy or failing to follow up on a positive EMA test remain common errors of management.