21 research outputs found
Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's) : an ARChiVe Cohort Study
OBJECTIVE:
To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA).
METHODS:
The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e-entry) cohort, with the data censored to November 2015. The EMA algorithm was used to uniquely distinguish children with MPA from children with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons.
RESULTS:
In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n\u2009=\u200948) or GPA (n\u2009=\u2009183). The median time to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA compared to those with GPA, pulmonary manifestations were less frequent (44% versus 74%) and less severe (primarily, hemorrhage, requirement for supplemental oxygen, and pulmonary failure). Renal pathologic features were frequently found in both groups (75% of patients with MPA versus 83% of patients with GPA) but tended toward greater severity in those with MPA (primarily, nephrotic-range proteinuria, requirement for dialysis, and end-stage renal disease). Airway/eye involvement was absent among patients with MPA, because these GPA-defining features preclude a diagnosis of MPA within the EMA algorithm. Similar proportions of patients with MPA and those with GPA received combination therapy with corticosteroids plus cyclophosphamide (69% and 78%, respectively) or both drugs in combination with plasmapheresis (19% and 22%, respectively). Other treatments administered, ranging in decreasing frequency from 13% to 3%, were rituximab, methotrexate, azathioprine, and mycophenolate mofetil.
CONCLUSION:
Younger age at disease onset and, perhaps, both gastrointestinal manifestations and more severe kidney disease seem to characterize the clinical profile in children with MPA compared to those with GPA. Delay in diagnosis suggests that recognition of these systemic vasculitides is suboptimal. Compared with adults, initial treatment regimens in children were comparable, but the complete reversal of female-to-male disease prevalence ratios is a provocative finding
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DECAY STUDIES OF NUCLEI NEAR THE PROTON DRIP LINE: [SUP]35 CO, [SUP]31 OR, [SUP]69 BR, AND [SUP]65 AS
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A FAST IN-BEAM RECOIL CATCHER WHEEL AND THE OBSERVATION OF BETA-DELAYED TWO-PROTON EMISSION FROM [SUP]31 AR
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Delayed proton activities produced in /sup 24/Mg + Ca and /sup 28/Si + Ca reactions
A search for new delayed proton emitters in /sup 24/Mg and /sup 28/Si bombardments of Ca targets has been conducted to extend knowledge about the proton drip line in the A = 60 to 65 mass region. Definitive evidence for the A = 4n + 1, T/sub z/ = -3/2 nuclide /sup 61/Ge is presented, but a search for the next member in this series, /sup 65/Se, proved fruitless. 6 refs., 3 figs
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Improvements to the helium-jet coupled on-line mass separator RAMA
Some general improvements to the on-line mass separator RAMA have yielded a factor of five increase in yield for most elements. By placing the ion source region at the full accelerating potential of 20 kV, the effective skimmer-plasma distance has been reduced from 12 cm to <2 cm. Changes in the helium-jet chamber and large volume pumping arrangement necessitated by placing the ion source region at high voltage are also given. Finally, details for a new highly shielded detector station are presented