Abstract:
BACKGROUND:Clotting factor replacement forms the pillar of treatment for children with hemophilia. Most children can be treated using peripheral venipuncture, but very young children and children with poor venous access might require a central venous catheter. Short-term and long-term complications of implantable venous access device placement (also known as port placement) can result in important morbidity and mortality in children with hemophilia. OBJECTIVE:The purpose of this study is to describe our experience with port placement in children and adolescents with severe hemophilia (<1% of the Factors VIII or IX). MATERIALS AND METHODS:We performed a retrospective review over a 10-year period to identify port placement in pediatric patients with severe hemophilia. We reviewed demographic and procedural information, access frequency, mechanical complications, and central-line-associated bloodstream infections (CLABSI). Eighteen males were included, with median age at insertion of 3.9 years (0.7-22.7 years). Fifteen of the 18 patients had hemophilia Type A and 3/18 had Type B. Thirteen had high neutralizing inhibitor titers. RESULTS:Technical success in port placement was achieved in 26/27 (96.3%) patients, with 1 port failure caused by venous occlusion from prior catheter placement. Port catheter size ranged from 5 French (Fr) to 7.5 Fr. All were single-lumen and placed via right (76.9%) or left (23.1%) internal jugular vein; 59.3% were placed during general anesthesia, and all had factor replacement prophylaxis. A peripherally inserted central catheter (PICC) was placed concurrently in 69.2% of the cases; per hospital policy, the port was only accessed 15 days post-placement to reduce the risk of site hematoma. Two patients were lost to follow-up. The total catheter days was 15,893. Ports were removed in 14/24 cases, most commonly because of CLABSI (7/24; 29.2%) and transition to peripheral infusion (3/24; 12.5%). Bleeding was the most common complication in the first 30 days after placement. There were nine CLABSI events (0.57 per 1,000 catheter days), all in patients with high neutralizing inhibitor titers. A higher frequency of port access (more or equal to daily vs. less than daily) correlated with higher infection rates (P=0.02). Median time from port insertion to first infection was 348 days (range 167-1,055 days). There were four fibrin-sheath-related catheter occlusions (0.25 per 1,000 catheter days): three catheters were salvaged with intra-catheter tissue plasminogen activator (tPA) instillation resulting in a salvage of an additional 1,214 catheter days, and one catheter was removed after tPA failure (0.06 per 1,000 catheter days). CONCLUSION:Port maintenance in boys with severe hemophilia is challenging given the need for long-term frequent device access that is associated with catheter-related infections. The rate of bleeding or infection did not differ in patients whether the device was accessed immediately or 15 days post placement. With appropriate pre- and post-procedural factor replacement, immediate and early term severe complications are not common.
journal_name
Pediatr Radioljournal_title
Pediatric radiologyauthors
Bedoya MA,Raffini L,Durand R,Acord MR,Srinivasan A,Krishnamurthy G,Vatsky S,Escobar F,Cahill AMdoi
10.1007/s00247-020-04668-3subject
Has Abstractpub_date
2020-07-01 00:00:00pages
1148-1155issue
8eissn
0301-0449issn
1432-1998pii
10.1007/s00247-020-04668-3journal_volume
50pub_type
杂志文章abstract::Sonographic studies on 52 children with suspected peripheral vascular disease examined between January 1988 and December 1989 were retrospectively reviewed. A technique for examining the jugular, subclavian veins, and superior vena cava (SVC) was developed and is described. The technique for examining the extremities ...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/BF02011718
更新日期:1991-01-01 00:00:00
abstract::Mass-like renal lesions in children occur in a diverse spectrum of conditions including benign and malignant neoplasm, infection, infarction, lymphatic malformation, and traumatic injury. Although mass-like renal lesions can sometimes be suspected on plain radiographs and evaluated with US in children, subsequent CT i...
journal_title:Pediatric radiology
pub_type: 杂志文章,评审
doi:10.1007/s00247-007-0548-4
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abstract::In 200 unselected upper abdominal sonographies in children the hepatic venous anatomy was examined with special reference to caudal hepatic veins. In 42% of patients caudal hepatic veins could be demonstrated sonographically, while large caudal hepatic veins could be shown in 12.5% of patients. The large caudal hepati...
journal_title:Pediatric radiology
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abstract::The anatomical anomalies that characterize septo-optic-dysplasia (deMorsier Syndrome) are variable and often subtle. We report imaging studies of nine patients with septo-optic-dysplasia which provide radiologic and ultrasonographic clues to this disorder. In addition, we propose that cerebral schizencephaly may be a ...
journal_title:Pediatric radiology
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doi:10.1007/BF02388642
更新日期:1989-01-01 00:00:00
abstract::Clavicle fractures are the most common bony injury that occurs during the delivery process. We present a case of medial clavicular physeal fracture mimicking sternoclavicular dislocation diagnosed by ultrasound (US) in a neonate. The infant presented to our clinic at 12 days old with improving left upper extremity pse...
journal_title:Pediatric radiology
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journal_title:Pediatric radiology
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更新日期:1988-01-01 00:00:00
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journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-013-2691-4
更新日期:2013-10-01 00:00:00
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journal_title:Pediatric radiology
pub_type: 杂志文章,评审
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journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s002470000331
更新日期:2000-11-01 00:00:00
abstract::The CT findings in 120 cerebral palsied children are analysed. The 72.5% positive findings are correlated with the clinical types, as well as the aetiological basis for the cerebral palsy. The spastic type, 83.3% of the total number of children, had the highest positive findings. The yield was increased in children wi...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/BF02010628
更新日期:1989-01-01 00:00:00
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journal_title:Pediatric radiology
pub_type: 杂志文章,已发布勘误
doi:10.1007/s00247-017-4064-x
更新日期:2018-07-01 00:00:00
abstract::Four patients between the ages of 2 and 20 years with malignant brain tumors were given several sonographic examinations, using an operative calvarial defect or sutural diastasis as an acoustic window. Three of our cases and 86% of cases with malignant brain tumors reported in the literature are echogenic, making poss...
journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/BF02388756
更新日期:1985-01-01 00:00:00
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journal_title:Pediatric radiology
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doi:10.1007/s00247-003-0999-1
更新日期:2003-10-01 00:00:00
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pub_type: 杂志文章,多中心研究
doi:10.1007/s00247-020-04747-5
更新日期:2020-09-01 00:00:00
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更新日期:1982-01-01 00:00:00
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journal_title:Pediatric radiology
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更新日期:2020-05-01 00:00:00
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journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-008-1003-x
更新日期:2008-12-01 00:00:00
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journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/s00247-013-2634-0
更新日期:2013-08-01 00:00:00
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更新日期:2001-03-01 00:00:00
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journal_title:Pediatric radiology
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journal_title:Pediatric radiology
pub_type: 杂志文章,评审
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更新日期:2019-09-01 00:00:00
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更新日期:2009-04-01 00:00:00
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journal_title:Pediatric radiology
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更新日期:1999-11-01 00:00:00
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journal_title:Pediatric radiology
pub_type: 杂志文章
doi:10.1007/BF02018618
更新日期:1991-01-01 00:00:00
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journal_title:Pediatric radiology
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