Antiphospholipid Antibody Syndrome In The Thrombophilia Pilot Project

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/106074/1/jth01382.pd

Thrombosis And Hemostasis Centers Pilot Sites Registry: Thrombophilia Screening In Children

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/106147/1/jth03026.pd

Renal structure and hypertension in autosomal dominant polycystic kidney disease

Renal structure and hypertension in autosomal dominant polycystic kidney disease. Hypertension has been reported to occur in 50 to 75 percent of subjects with autosomal dominant polycystic kidney disease (ADPKD) prior to the onset of marked renal insufficiency but concurrent with cystic deformation of the renal parenchyma. The present study was undertaken to examine whether the renal structural abnormalities are greater in hypertensive (HBP) versus normotensive (NBP) male and female patients with ADPKD who were matched within gender groups for age, body surface area, serum creatinine concentration (males HBP 1.2 ± 0.02 vs. NBP 1.1 ± 0.03 mg/dl, NS; females HBP 0.9 ± 0.03 vs. NBP 0.9 ± 0.02 mg/dl, NS) and creatinine clearance (males HBP 100 ± 3 vs. NBP 108 ± 3 ml/min/1.73 m2, NS; females HBP 97 ± 3 vs. NBP 96 ± 2 ml/min/1.73 m2, NS). Renal volume was significantly greater in the HBP compared to the NBP group (males HBP 624 ± 47 vs. NBP 390 ± 43 cm3, P < 0.0005; females HBP 466 ± 32 vs. NBP 338 ± 24 cm3, P < 0.002). Since increased renal volume is due to increased cysts, the results indicate that the early high incidence of hypertension in ADPKD correlates with the renal structural abnormalities in this disorder

Diagnosis of autosomal dominant polycystic kidney disease in utero and in the young infant.

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/135563/1/jum198765249.pd

Risk factors and management of deep venous thrombosis in children following post-surgical hypopituitarism in craniopharyngioma

Post-operative hypopituitarism following craniopharyngioma (CP) surgery is treated by replacement of various hormones. The risk of deep venous thrombosis (DVT) following CP surgery and initiation of hormones has not been well studied and recognized. We present three patients with DVT who had family history of DVT and procoagulant risk factors such as inherited thrombophilia and elevated Von Willebrand factor levels due to treatment with desmopressin. We discuss the individualized management of anticoagulation and the dilemma of starting estrogen and progesterone replacement therapy in them. Pediatr Blood Cancer 2011;57:175–177. © 2011 Wiley-Liss, Inc.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/84403/1/22937_ftp.pd

Elevated maternal lipoprotein (a) and neonatal renal vein thrombosis: a case report

<p>Abstract</p> <p>Introduction</p> <p>Renal vein thrombosis, although rare in adults, is well recognized in neonates and is one of the most common manifestations of neonatal thromboembolic events. The etiology of renal vein thrombosis remains unidentified in the majority of cases. We report a case of renal vein thrombosis in a neonate associated with elevated maternal lipoprotein (a).</p> <p>Case presentation</p> <p>A full-term female infant, appropriate for gestational age, was born via spontaneous vaginal delivery to an 18-year-old primigravida. The infant's birth weight was 3680 g and the Apgar scores were eight and nine at 1 and 5 minutes respectively. Evaluation of the infant in the newborn nursery revealed a palpable mass in the right lumbar area. Tests revealed hematuria and a high serum creatinine level of 1.5 mg/dl. An abdominal ultrasound Doppler flow study demonstrated an enlarged right kidney, right renal vein thrombosis, and progression of the thrombosis to the inferior vena cava. There was no evidence of saggital sinus thrombosis. An extensive work-up of parents for hypercoagulable conditions was remarkable for a higher plasma lipoprotein (a) level of 73 mg/dl and an elevated fibrinogen level of 512 mg/dl in the mother. All paternal levels were normal. The plasma lipoprotein (a) level in the neonate was also normal. The neonate was treated with low molecular weight heparin (enoxaparin) at 1.5 mg/kg/day every 12 hours for 2 months, at which time a follow-up ultrasound Doppler flow study showed resolution of the thrombosis in both the renal vein and the inferior vena cava.</p> <p>Conclusion</p> <p>There have been no studies to date that have explored the effect of abnormal maternal risk factors on fetal hemostasis. A case-control study is required to investigate whether elevated levels of maternal lipoprotein (a) may be a risk factor for neonatal thrombotic processes. Although infants with this presentation are typically treated with anticoagulation, there is a lack of evidence-based guidelines. Treatment modalities vary between study and treatment centers which warrants the establishment of a national registry.</p

Current prescription of prophylactic factor infusions and perceived adherence for children and adolescents with haemophilia: a survey of haemophilia healthcare professionals in the United States

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/92069/1/hae2756_sm_DataS1.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/92069/2/hae2756.pd

Proceedings of the International Haemophilia Prophylaxis Study Group Meeting, November 2003, Montreal, PQ, Canada

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/72691/1/j.1365-2516.2005.01053.x.pd

Establishment of embryonic stem cells secreting human factor VIII for cell-based treatment of hemophilia A

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/72012/1/j.1538-7836.2008.03022.x.pd

Healthcare resource utilization among haemophilia A patients in the United States

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/91210/1/j.1365-2516.2011.02677.x.pd