Determination of skeletal age is clinically relevant in patients being considered for epiphysiodesis to manage leg-length inequality. The multiplier method was the least accurate of the prediction methods in this patient population, which may have implications in calculating the appropriate timing of epiphysiodesis.
Background:
The prognosis of Legg-Calvé-Perthes disease (LCPD) is dependent upon several factors, with the length and severity of the fragmentation stage among the most important. Previous retrospectively collected data from a single center have suggested that early proximal femoral varus osteotomy (PFO) may shorten the length of fragmentation and allow 34% of patients to bypass fragmentation altogether resulting in less femoral head deformity. The purpose of this study was to validate these findings in a prospectively collected multicenter cohort.
Methods:
Patients with LCPD treated with early PFO (during Waldenström stage I) were prospectively followed with serial radiographs at 3-month intervals until a minimum of 2-year follow-up. Waldenström stages and lateral pillar class were determined by mode assessments from 3 pediatric orthopaedic surgeons. The duration of fragmentation was defined as the interval between the first radiographs demonstrating features of stage IIa and stage IIIa. “Complete” bypass was defined as the absence of stage IIa or IIb findings on sequential radiographs with no development of femoral head deformity or collapse. “Partial” bypass was defined as the absence of advanced features of fragmentation and femoral head collapse (stage IIb).
Results:
Forty-six patients (80% male individuals) with initial stage LCPD and a mean age of 8.2±1.2 years were identified. The weighted kappa statistics for Waldenström staging and lateral pillar classifications showed excellent (0.833) and substantial (0.707) agreement, respectively. Ninety-eight percent of patients (45/46) underwent some period of fragmentation lasting between 91 and 518 days; the median duration was 206 days (interquartile range, 181 to 280). One patient (2%) bypassed fragmentation completely; 8 patients (17%) demonstrated partial bypass. Patients who completely or partially bypassed fragmentation experienced significantly less severe lateral pillar collapse (P=0.016) and shorter fragmentation duration (P=0.001).
Conclusions:
In this prospective multicenter cohort, we found a lower rate of fragmentation bypass than previously reported. Nonetheless, our data support the previous contention that early PFO may shorten fragmentation and minimize collapse in LCPD compared with historical controls. Further study with larger cohorts and a more rigorous definition of what constitutes bypass is warranted to clarify the effect of early PFO on the reparative biology of LCPD.
Level of Evidence:
Level IV—therapeutic study.
Introduction: Juvenile dermatomyositis (JDM) can be classified into clinical serological subgroups by distinct myositis-specific antibodies (MSAs). It is incompletely understood whether different MSAs are associated with distinct pathological characteristics, clinical disease activities, or response to treatment. Methods: We retrospectively reviewed clinicopathological data from consecutive JDM patients followed in the pediatric rheumatology clinic at a single center between October 2016 and November 2018. Demographics, clinical data, and laboratory data were collected and analyzed. Detailed muscle biopsy evaluation of four domains (inflammation, myofiber, vessels, and connective tissue) was performed, followed by statistical analysis. Results: Of 43 subjects included in the study, 26 (60.5%) had a detectable MSA. The most common MSAs were anti-NXP-2 (13, 30.2%), anti-Mi-2 (7, 16.3%), and anti-MDA-5 (5, 11.6%). High titer anti-Mi-2 positively correlated with serum CK > 10,000 at initial visit (r = 0.96, p = 0.002). Muscle biopsied from subjects with high titer anti-Mi-2 had prominent perifascicular myofiber necrosis and perimysial connective tissue damage that resembled perifascicular necrotizing myopathy, but very little capillary C5b-9 deposition. Conversely, there was no positive correlation between the levels of the anti-NXP-2 titer and serum CK (r = − 0.21, p = 0.49). Muscle biopsies from patients with anti-NXP-2 showed prominent capillary C5b-9 deposition; but limited myofiber necrosis. Only one patient had anti-TIF1γ autoantibody, whose muscle pathology was similar as those with anti-NXP2. All patients with anti-MDA-5 had normal CK and near normal muscle histology. Conclusions: Muscle biopsy from JDM patients had MSA specific tissue injury patterns. These findings may help improve muscle biopsy diagnosis accuracy and inform personalized treatment of JDM.
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