A Hemoglobinopathy That Produces an Array of Different Hemoglobin A1c Values.

Hemoglobin A1c (HbA1c) refers to non-enzymatically glycated hemoglobin and reflects the patient's glycemic status over approximately 3 months. An elevated HbA1c over 6.5% National Glycohemoglobin Standardization Program (NGSP) (48 mmol/mol the International Federation of Clinical Chemistry and Laboratory Medicine (IFCC)) can be used to diagnose diabetes mellitus. In our laboratory, HbA1c is determined by ion-exchange chromatography which has the advantage of detecting common Hb variants such as Hb S, C, E and D without adversely affecting the HbA1c determination. Certain homozygous or compound heterozygous hemoglobinopathies such as homozygous sickle disease and Hb SC disease can significantly lower the HbA1c by reducing red cell lifespan. Occasionally however, rare and mostly benign hemoglobinopathies can interfere with this technique resulting in an apparent elevation of HbA1c in an otherwise non-diabetic patient. In this report, we describe such a hemoglobinopathy termed Hb Wayne that resulted in a significant HbA1c elevation in a normoglycemic individual. HbA1c was determined by multiple methods including immunoassay, a modified capillary electrophoresis and an alternative ion-exchange system. These techniques yielded significantly lower A1c results, more in keeping with the patient's clinical background. The alternative ion-exchange system resulted in a low A1c that was qualified by warning flags on the chromatogram that indicated the result was not reportable. The hemoglobinopathy in question, Hb Wayne, is a frameshift mutation in the alpha globin gene that results in an extended alpha globin polypeptide that can form two variants Hb Wayne I and Wayne II. Hb Wayne is a clinically silent asymptomatic disorder with no hematologic consequences. The artifactual elevation of HbA1c is, in contrast, very significant because it may result in a misdiagnosis of diabetes mellitus leading to unnecessary treatment. In this report, we compare our findings with other descriptions of Hb Wayne in the literature and corroborate a number of previous observations and conclusions.

Variability in the relationship between the distal femoral mechanical and anatomical axes in patients undergoing primary total knee arthroplasty.

Currently, an intramedullary (IM) guide is often used for performing the distal femoral resection in total knee arthroplasty (TKA). However, this method assumes that in most patients, the distal femoral mechanical-anatomical angle (FMAA) is 5°. Preoperative, standing, AP hip-to-ankle radiographs were reviewed in 493 patients undergoing primary TKA, and the FMAA was digitally measured. Correlation coefficients relative to several radiographic measurements, along with demographic variables, were performed. A significant number of patients (28.6%) had an FMAA outside the range of 5° ± 2° (range 2.0°-9.6°). The only measurement demonstrating a fair/moderate correlation with the FMAA was the neck-shaft angle (r = -0.55). Using an IM resection guide, without obtaining AP hip-to-ankle radiographs to determine each patient's true FMAA, may lead to malalignment of the femoral component.

Patient specific cutting guides versus an imageless, computer-assisted surgery system in total knee arthroplasty.

BACKGROUND: Patient specific cutting guides (PSC) in total knee arthroplasty (TKA) have recently been introduced, in which preoperative 3-dimensional imaging is used to manufacture disposable cutting blocks specific to a patient's anatomy. The purpose of this study was to compare the alignment accuracy of PSC to an imageless CAS system in TKA. METHODS: Thirty-seven patients (41 knees), received a TKA using an imageless CAS system. Subsequently, 38 patients (41 knees), received a TKA using a MRI-based, PSC system. Postoperatively, standing AP hip-to-ankle radiographs were obtained, from which the lower extremity mechanical axis, tibial component varus/valgus, and femoral component varus/valgus mechanical alignment were digitally measured. Each measurement was performed by two blinded, independent observers, and interclass correlations were calculated. A student's two-tailed t test was used to compare the two cohorts (p-value<0.05=significant). RESULTS: In the PSC cohort, 70.7% of patients had an overall alignment within 3° of a neutral mechanical axis (vs. 92.7% with CAS, p=0.02), 87.8% had a tibial component alignment within 2° of perpendicular to the tibial mechanical axis (vs. 100% with CAS, p=0.04), and 90.2% had a femoral component alignment within 2° of perpendicular to the femoral mechanical axis (vs. 100% with CAS, p=0.2). Interclass correlation coefficients were good to excellent for all radiographic measurements. CONCLUSION: While PSC techniques appear sound in principle, this study did not demonstrate patient specific cutting guides to obtain the same degree of overall mechanical and tibial component alignment accuracy as a CAS technique. LEVEL OF EVIDENCE: III: Retrospective cohort study.