Clinical experience with two-point mDixon turbo spin echo as an alternative to conventional turbo spin echo for magnetic resonance imaging of the pediatric knee.

BACKGROUND: Two-point modified Dixon (mDixon) turbo spin-echo (TSE) sequence provides an efficient, robust method of fat suppression. In one mDixon acquisition, four image types can be generated: water-only, fat-only, in-phase and opposed-phase images. OBJECTIVE: To determine whether PD mDixon TSE water-only and, by proxy, PD in-phase images generated by one acquisition can replace two conventional PD TSE sequences with and without fat suppression in routine clinical MR examination of the knee. MATERIALS AND METHODS: This is a retrospective study of 50 consecutive pediatric knee MR examinations. PD mDixon TSE water-only and PD fat-saturated TSE sequences (acquired in the sagittal plane with identical spatial resolution) were reviewed independently by two pediatric radiologists for homogeneity of fat suppression and detection of intra-articular pathology. Thirteen of the 50 patients underwent arthroscopy, and we used the arthroscopic results as a reference standard for the proton-density fat-saturated and proton-density mDixon results. We used the Kruskal-Wallis rank test to assess difference in fat suppression between the proton-density mDixon and proton-density fat-saturated techniques. We used kappa statistics to compare the agreement of detection of intra-articular pathology between readers and techniques. We also calculated sensitivity, specificity and accuracy between arthroscopy and MR interpretations. RESULTS: Proton-density mDixon water-only imaging showed significant improvement with the fat suppression compared with proton-density fat-saturated sequence (P=0.02). Each observer demonstrated near-perfect agreement between both techniques for detecting meniscal and ligamentous pathology and fair to substantial agreement for bone contusions, and chondral and osteochondral lesions. CONCLUSION: Two-point mDixon water-only imaging can replace conventional proton-density fat-saturated sequence. When same-plane proton-density fat-saturated and non-fat-saturated sequences are required, proton-density water-only and proton-density in-phase image types acquired in the same acquisition shorten the overall examination time while maintaining excellent intra-articular lesion conspicuity.

Nitric oxide for inhalation in the acute treatment of sickle cell pain crisis: a randomized controlled trial.

CONTEXT: Inhaled nitric oxide has shown evidence of efficacy in mouse models of sickle cell disease (SCD), case series of patients with acute chest syndrome, and 2 small placebo-controlled trials for treatment of vaso-occlusive pain crisis (VOC). OBJECTIVE: To determine whether inhaled nitric oxide gas reduces the duration of painful crisis in patients with SCD who present to the emergency department or hospital for care. DESIGN, SETTING, AND PARTICIPANTS: Prospective, multicenter, double-blind, randomized, placebo-controlled clinical trial for up to 72 hours of inhaled nitric oxide gas vs inhaled nitrogen placebo in 150 participants presenting with VOC of SCD at 11 centers between October 5, 2004, and December 22, 2008. Intervention Inhaled nitric oxide gas vs inhaled nitrogen placebo. MAIN OUTCOME MEASURES: The primary end point was the time to resolution of painful crisis, defined by (1) freedom from parenteral opioid use for 5 hours; (2) pain relief as assessed by visual analog pain scale scores of 6 cm or lower (on 0-10 scale); (3) ability to walk; and (4) patient's and family's decision, with physician consensus, that the remaining pain could be managed at home. RESULTS: There was no significant change in the primary end point between the nitric oxide and placebo groups, with a median time to resolution of crisis of 73.0 hours (95% confidence interval [CI], 46.0-91.0) and 65.5 hours (95% CI, 48.1-84.0), respectively (P = .87). There were no significant differences in secondary outcome measures, including length of hospitalization, visual analog pain scale scores, cumulative opioid usage, and rate of acute chest syndrome. Inhaled nitric oxide was well tolerated, with no increase in serious adverse events. Increases in venous methemoglobin concentration confirmed adherence and randomization but did not exceed 5% in any study participant. Significant increases in plasma nitrate occurred in the treatment group, but there were no observed increases in plasma or whole blood nitrite. CONCLUSION: Among patients with SCD hospitalized with VOC, the use of inhaled nitric oxide compared with placebo did not improve time to crisis resolution. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00094887.

Clinical differences between children and adults with pulmonary hypertension and sickle cell disease.

Pulmonary hypertension (PHT) is an important co-morbidity in sickle cell disease (SCD). Despite increasing research in adults, the prevalence and implication of this condition in children is unknown. Charts of 362 SCD patients followed at the Children's Hospital & Research Center Oakland were reviewed to determine clinical variables associated with obtaining echocardiographic screening for PHT, clinical associations of PHT, and associated mortality following diagnosis in adults and children with SCD. In this cohort, patients with underlying lung abnormalities or those on chronic transfusions were more likely to have echocardiograms, however the diagnosis of PHT was often unrecognized. A different clinical phenotype for PHT in adults versus children was identified. Associations with PHT for adults included age, renal and lung disease, hepatitis C, chronic transfusions, and a history of acute chest syndrome (ACS), with ACS being protective. Surprisingly, for children, a history of sepsis, along with a history of ACS, or obstructive lung disease were associated with PHT. Survival analysis found significant mortality for PHT, with a hazard ratio of 17.3 (95% confidence interval 4.9-60.4). The divergent clinical spectrum for PHT between adults and children may point to different age-specific mechanisms or biological expression of PHT.