Volume of the spleen in children as measured on CT scans: normal standards as a function of body weight.

Schlesinger, Alan E.; Edgar, Kenneth; Boxer, L

doi:10.2214/ajr.160.5.8470587

Cited by 66 publications

(37 citation statements)

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“…10 Splenomegaly was defined as a spleen volume greater than the 95th percentile of the normal distribution in children. 11 For the goniometry measurements, an increase of Ն10°range of motion was considered clinically important for the majority of joint motions, except for hip abduction and internal and external rotation, knee flexion and extension, and ankle dorsiflexion, where an increase of Ն5°was considered clinically important. AEs were recorded continuously throughout the study.…”

Section: Methodsmentioning

confidence: 99%

Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome

Muenzer

Beck

Eng

et al. 2011

Genetics in Medicine

200

218

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Purpose: This study evaluated the safety and effectiveness of long-term enzyme replacement therapy with idursulfase (recombinant human iduronate-2-sulfatase) in patients with Hunter syndrome. Methods: All 94 patients who completed a 53-week double-blinded study of idursulfase enrolled in this open-labeled extension study and received intravenous idursulfase at a dose of 0.5 mg/kg weekly for 2 years, and clinical outcomes and safety were assessed. Results: No change in percent predicted forced vital capacity was seen, but absolute forced vital capacity demonstrated sustained improvement and was increased 25.1% at the end of the study. Statistically significant increases in 6-minute walking test distance were observed at most time points. Mean liver and spleen volumes remained reduced throughout the 2-year extension study. Mean joint range of motion improved for the shoulder and remained stable in other joints. Both the parent-and child-assessed Child Health Assessment Questionnaire Disability Index Score demonstrated significant improvement. Infusion-related adverse events occurred in 53% of patients and peaked at Month 3 of treatment and declined thereafter. Neutralizing IgG antibodies were detected in 23% of patients and seemed to attenuate the improvement in pulmonary function. Conclusions: Weekly infusions of idursulfase result in sustained clinical improvement during 3 years of treatment. Genet Med 2011:13(2):95-101.

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Section: Methodsmentioning

confidence: 99%

Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome

Muenzer

Beck

Eng

et al. 2011

Genetics in Medicine

200

218

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show abstract

“…20 Splenomegaly was defined as having a splenic volume greater than the 95 th percentile of the normal distribution in children. 21 Liver and spleen volumes were determined by magnetic resonance imaging (MRI) using the same imaging protocol at all sites. MRI images were sent to Bio-Imaging Technologies, Inc, Newtown, PA for determination of liver and spleen volumes.…”

Section: Enzyme Replacement Therapy In Mucopolysaccharidosis Type IImentioning

confidence: 99%

A phase II/III clinical study of enzyme replacement therapy with idursulfase in mucopolysaccharidosis II (Hunter syndrome)

Muenzer

Wraith

Beck³

et al. 2006

Genetics in Medicine

521

509

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Purpose: To evaluate the safety and efficacy of recombinant human iduronate-2-sulfatase (idursulfase) in the treatment of mucopolysaccharidosis II. Methods: Ninety-six mucopolysaccharidosis II patients between 5 and 31 years of age were enrolled in a double-blind, placebo-controlled trial. Patients were randomized to placebo infusions, weekly idursulfase (0.5 mg/kg) infusions or every-other-week infusions of idursulfase (0.5 mg/kg).Efficacy was evaluated using a composite endpoint consisting of distance walked in 6 minutes and the percentage of predicted forced vital capacity based on the sum of the ranks of change from baseline. Results: Patients in the weekly and every-other-week idursulfase groups exhibited significant improvement in the composite endpoint compared to placebo (P ϭ 0.0049 for weekly and P ϭ 0.0416 for every-other-week) after one year. The weekly dosing group experienced a 37-m increase in the 6-minute-walk distance (P ϭ 0.013), a 2.7% increase in percentage of predicted forced vital capacity (P ϭ 0.065), and a 160 mL increase in absolute forced vital capacity (P ϭ 0.001) compared to placebo group at 53 weeks. Idursulfase was generally well tolerated, but infusion reactions did occur.

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“…In most other studies, sizes between the 5 th and 95 th percentiles were the accepted normal limits 17 . However, this practice results in approximately 10 % of children with normal visceral organs falling outside these limits 18 .…”

Section: Discussionmentioning

confidence: 91%

Sonographic biometry of spleen among school age children in Nsukka, Southeast, Nigeria

Eze

Agwu²,

Dn³

et al. 2013

Afr H. Sci.

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Volume of the spleen in children as measured on CT scans: normal standards as a function of body weight.

Cited by 66 publications

References 0 publications

Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome

Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome

A phase II/III clinical study of enzyme replacement therapy with idursulfase in mucopolysaccharidosis II (Hunter syndrome)

Sonographic biometry of spleen among school age children in Nsukka, Southeast, Nigeria

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