Immunoglobulin A (IgA) nephropathy is the most prevalent form of glomerulonephritis worldwide. A renal biopsy is required for an accurate diagnosis, as no convenient biomarker is currently available. We developed a serological test based upon the observation that this nephropathy is characterized by undergalactosylated IgA1 in the circulation and in mesangial immune deposits. In the absence of galactose, the terminal saccharide of O-linked chains in the hinge region of IgA1 is terminal or sialylated N-acetylgalactosamine. A lectin from Helix aspersa, recognizing N-acetylgalactosamine, was used to develop an enzyme-linked immunosorbent assay that measures galactose-deficient IgA1 in serum. The median serum lectin-binding IgA1 level was significantly higher for 153 Caucasian adult patients with IgA nephropathy without progression to end-stage renal disease as compared with that for 150 healthy Caucasian adult controls. As the lectin-binding IgA1 levels for the controls were not normally distributed, the 90th percentile was used for determination of significant elevation. Using a value of 1076 U/ml as the upper limit of normal, 117 of the 153 patients with IgA nephropathy had an elevated serum lectin-binding IgA1 level. The sensitivity as a diagnostic test was 76.5%, with specificity 94%; the positive predictive value was 88.6% and the negative predictive value was 78.9%. We conclude that this lectin-binding assay may have potential as a noninvasive diagnostic test for IgA nephropathy.

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We present a case of a 27-year-old female with severe mitral regurgitation caused by a single long aberrant chorda tendinea. This chorda extended from the base of the right coronary cusp of the aortic valve, through the A2 scallop of the mitral valve, and attached to the dome of the left atrium. Initial transthoracic echocardiogram (TTE) demonstrated a mildly redundant anterior mitral leaflet with thickened leaflet tip and moderate eccentric, posteriorly directed mitral regurgitation. Repeat TTE revealed a chord-like structure attached to the midportion of the anterior mitral leaflet and extending to the left ventricular outflow tract. Transesophageal echocardiography (TEE) suggested two aberrant chordae tendineae tethering the A2 scallop on both the left atrial and left ventricular side. Patient underwent surgical resection of the aberrant chorda. During the excision of the chorda the structural integrity of the A2 scallop was compromised, necessitating mitral valve repair with excellent results.

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A 56 year-old male with past medical history significant for diabetes and obesity presented for an elective diagnostic cardiac catheterization. The catheterization films are shown below (Figures 1 and 2). DiscussionAdult coronary anomalies are not very common and are usually casual findings of diagnostic angiographic studies. The incidence of these anomalies has been reported to be between 0.29-1.34%. Although coronary artery anomalies are far less common than acquired coronary artery disease, their impact on premature cardiac morbidity and mortality among young adults is not trivial. In a study of 126 nontraumatic sudden deaths in young adults, cardiac abnormality was found in 64 cases(51%), with coronary artery abnormalities being the most common cardiac abnormality(61%).Coronary artery anomalies can be classified as hemodynamically significant or insignificant. Hemodynamically significant anomalies of the coronary arteries are characterized by abnormalities of myocardial perfusion, which lead to an increased risk of myocardial ischemia or sudden death. These anomalies include an anomalous origin of either the LCA or the RCA from the pulmonary artery, an anomalous course between the pulmonary artery and the aorta (interarterial) of either the RCA arising from the left sinus of valsalva or the LCA arising from the right sinus of valsalva, occasional myocardial bridging, and congenital coronary artery fisula.Anomalous origin of the coronary artery from the pulmonary artery (ALCAPA) is one of the most serious congenital coronary artery anomalies. It has an estimated prevalence of one in 300,000 live births. Most affected patients show symptoms in infancy and early childhood. Approximately 90%of untreated infants die in the 1st year of life, and only a few patients survive to adulthood. In the most common form of this disease, the LCA arises from the pulmonary artery and the RCA arises normally from the aorta (Bland-White-Garland syndrome).The four recognized patterns of an anomalous origin of a coronary artery from the opposite or noncoronary sinus are (a) the RCA arising from the left coronary sinus, (b) the LCA arising from the right coronary sinus, (c) the LCx or LAD artery arising from the right coronary sinus, and (d) the LCA or RCA (or a branch of either artery) arising from the noncoronary sinus. A coronary artery arising from the opposite or noncoronary sinus can take any of four common courses, depending on the anatomic relationship of the anomalous vessel to the aorta and the pulmonary trunk: (a)interarterial (ie, between the aorta and the pulmonary artery), (b) retroaortic, (c) prepulmonic, or (d) septal (subpulmonic). It is of great clinical importance which course is taken, an interarterial course carries a high risk for sudden cardiac death. The most common course of an anomalous RCA arising from the left sinus of Valsalva is interarterial; this variant can be associated with sudden cardiac death in up to 30% of patients. It has been postulated that, when dilation of the aorta occurs during exercise...