Patterns of von Willebrand Disease Screening in Girls and Adolescents With Heavy Menstrual Bleeding.

OBJECTIVE: To estimate the frequency of von Willebrand disease screening and factors that affect screening frequency in a national sample of girls and adolescents with heavy menstrual bleeding. METHODS: In this retrospective cohort study, we used a national claims database for privately and publicly insured patients between 2011 and 2013 for girls aged 10-17 years. Diagnostic criteria of heavy menstrual bleeding were the presence of one inpatient or two outpatient International Classification of Diseases, 9th Revision codes for heavy menstrual bleeding. We defined severe heavy menstrual bleeding as heavy menstrual bleeding plus an inpatient stay for menstrual bleeding, iron deficiency anemia, or blood transfusion. To assess whether patient- or facility-level characteristics affected screening, we performed logistic regression analysis including patient age, health care provider type seen at first visit for menorrhagia, patient residence in a metropolitan statistical area (proxy for urban vs rural inhabitance), and approximate travel time to the nearest hemophilia treatment center. RESULTS: We identified 23,888 postpubertal girls and adolescents with heavy menstrual bleeding (986 with severe heavy menstrual bleeding). Von Willebrand disease screening was performed in 8% of females with heavy menstrual bleeding and 16% with severe heavy menstrual bleeding. Younger age at diagnosis, commercial insurance, and living within a metropolitan statistical area were associated with higher screening rates. Patients who underwent testing for iron deficiency anemia had the highest likelihood of undergoing screening (odds ratio 7.08, 95% CI 6.32-7.93). Among patients living in a metropolitan statistical area, those 60 minutes or more from a hemophilia treatment center were less likely to undergo screening. CONCLUSION: Despite recommendations by the American College of Obstetricians and Gynecologists for more than 15 years, fewer than 20% of postpubertal girls and adolescents with heavy menstrual bleeding underwent screening for von Willebrand disease in this cohort. Increased clinician awareness and adherence to recommended screening recommendations may increase diagnosis of von Willebrand disease.

Steroid-induced Hypertension During Induction Chemotherapy for Acute Lymphoblastic Leukemia in US Children's Hospitals.

Childhood acute lymphoblastic leukemia achieves excellent cure rates in part due to induction chemotherapy including high dose corticosteroids. Hypertension (HTN) is a known complication of corticosteroids, but incidence and risk factors for steroid-induced HTN are poorly understood. We sought to describe these using a large pediatric health database. Of the 5578 unique patients receiving induction chemotherapy, 14.7% received anti-HTN medications during their initial hospital admission. We found that age below 1 year, obesity, secondary diabetes mellitus, and abnormal glucose were associated with developing steroid-induced HTN. We also found that ICD-9 codes had poor sensitivity for detecting treatment of HTN, suggesting underreporting by physicians.

Hormonal Contraception and Risk of Thromboembolism in Women With Diabetes.

OBJECTIVE: To investigate safety of hormonal contraception with regard to thromboembolic events in women with type 1 or 2 diabetes. RESEARCH DESIGN AND METHODS: We used data from 2002-2011 in Clinformatics Data Mart to identify women in the U.S., 14-44 years of age, with an ICD-9-CM code for diabetes and a prescription for a diabetic medication or device. We examined contraceptive claims and compared time to thromboembolism (venous thrombosis, stroke, or myocardial infarction) among women with diabetes dispensed hormonal contraception using a modification of Cox regression to control for age, smoking, obesity, hypertension, hyperlipidemia, diabetic complications, and history of cancer; we excluded data for 3 months after women gave birth. RESULTS: We identified 146,080 women with diabetes who experienced 3,012 thromboembolic events. Only 28% of reproductive-aged women with diabetes had any claims for hormonal contraception, with the majority receiving estrogen-containing oral contraceptives. Rates of thromboembolism were highest among women who used the contraceptive patch (16 per 1,000 woman-years) and lowest among women who used intrauterine (3.4 per 1,000 woman-years) and subdermal (0 per 163 woman-years) contraceptives. Compared with use of intrauterine contraception, progestin-only injectable contraception was associated with increased risk of thromboembolism (12.5 per 1,000 woman-years; adjusted hazard ratio 4.69 [95% CI 2.51-8.77]). CONCLUSIONS: The absolute risk of thromboembolism among women with type 1 or 2 diabetes using hormonal contraception is low. Highly effective, intrauterine and subdermal contraceptives are excellent options for women with diabetes who hope to avoid the teratogenic effects of hyperglycemia by carefully planning their pregnancies.

Intravenous iron therapy in non-anemic iron-deficient menstruating adolescent females with fatigue.

Menstruating women, with or without underlying bleeding disorders, are at increased risk for developing iron deficiency-related fatigue, even in the absence of anemia. Oral iron therapy has limitations which include poor absorption and non-adherence due to gastrointestinal side effects. We performed a prospective clinical trial of post-menarchal adolescent females with iron-deficiency with or without mild anemia and fatigue who received a standardized regimen of intravenous iron sucrose. The baseline mean (SD) hemoglobin was 11.96 g dl(-1) (1.05) in 20 girls (ages 14-21 years); with a range of 10.3-14.1 g dl(-1) . In this cohort, intravenous iron was well tolerated and patients demonstrated a sustained increase in ferritin levels with means (SD) of 13.4 ng ml(-1) (13.1) at baseline to 141.5 ng ml(-1) (104.5) at 6 weeks and 85.2 ng ml(-1) (128.4) at 6 months after the infusions. We used a standardized (Peds QL(TM) Multidimensional) fatigue scale to objectively measure fatigue and proxy scores by parents with mean screening scores (SD) of 35.2 (16.8) and 31.9 (19.6), respectively. We demonstrated a clinically significant improvement both in patient as well as parent fatigue scores (in 19 out of 20 subjects) at 6 weeks (Mean (SD) 58.3 (21.3) [P < 0.0001] and 57 (24.4) [P < 0.0001], respectively); as well as 3 and 6 months after the iron infusions. In nonanemic patients, iron administration did not significantly influence hemoglobin concentration. Therefore, the fatigue-reducing effects of iron therapy reflect the nonhematological functions of iron. Am. J. Hematol. 91:973-977, 2016. © 2016 Wiley Periodicals, Inc.

The Effects of Hospital Length of Stay on Readmissions for Children With Newly Diagnosed Acute Lymphoblastic Leukemia.

Although regimens for induction therapy in children with acute lymphoblastic leukemia (ALL) are similar across the United States, typical practice with regard to inpatient length of stay (LOS) varies by institution. US children's hospitals were categorized by typical induction LOS; and readmissions, pediatric intensive care unit (PICU) admissions, and average adjusted charges were compared for the first 30 days from initial admission. Using Pediatric Health Information System data, we extracted ALL induction admissions from 2007 to 2013. We categorized hospitals into 3 categories based on median LOS: short (≤7 d), medium (8 to 15 d), or long (≥16 d). Median LOS varied from 5 to 31 days across hospitals. Thirty-day median inpatient costs per patient ranged from $32 K for short LOS, $40 K for medium LOS, and $47 K for long LOS. Compared with short LOS hospitals (n=14), medium LOS (n=8) and long LOS hospitals (n=8) had lower odds of PICU readmissions (odds ratio [OR], 0.68; P=0.0124 and OR, 0.31; P<0.001, respectively), and long LOS hospitals had lower odds of any readmission (OR, 0.44; P<0.0001). Average LOS for children with newly diagnosed ALL varies widely by institution. Children's hospitals that typically admit new ALL patients for >7 days have fewer PICU readmissions but substantial increase in total induction inpatient costs.

Hydroxyurea use in Children with Sickle Cell Disease: Do Severely Affected Patients Use It and Does It Impact Hospitalization Outcomes?

BACKGROUND: Expert guidelines recommend that hydroxyurea (HU) be offered to all children with hemoglobin SS and Sß(0) sickle cell disease (SCD) and be considered for children with clinically severe hemoglobin SC or Sß(+) . This study aims to determine the rate of HU use in hospitalized children, if HU is differentially used in children with clinically severe SCD, and if HU users have shorter length of stay (LOS), fewer intensive care unit (ICU) admissions, and fewer inpatient transfusions compared to nonusers. PROCEDURE: Using the Pediatric Health Information System, we performed a retrospective analysis of children ages 2-18 years with SCD discharged between January 1, 2011 and September 30, 2014. We defined patients as having clinically severe SCD if they had a recent ICU admission or ≥3 admissions in the preceding year. RESULTS: Of the 2,665 unique children identified, approximately 80% had an inpatient code indicating HU use. Significantly more (p < 0.001) nonusers (30.1%) had a recent ICU admission compared to HU users (18.7%). More nonusers (33.9%) had a history of ≥3 admissions compared to HU users (21.5%) (p < 0.001). After applying propensity score weighting, the groups did not differ in their LOS, prevalence of ICU admissions, or prevalence of transfusions. CONCLUSIONS: HU use is high among hospitalized children with SCD. However, HU is not utilized by many children with clinically severe SCD. These results support that HU be considered in children with SCD to prevent hospitalization rather than as a treatment to improve hospitalization outcomes.