Chromosome abnormalities, including syndromes, are prevalent in newborns with congenital heart disease. Further research is needed to evaluate the utility of cytogenetic screening in all children with congenital heart disease.
Background: Current guidelines recommended aortic measurements during diastole in adults and during systole in children. Recent studies in adults have demonstrated noteworthy differences in aortic measurements during systole and diastole in the same subjects. In the present study, we aimed to characterize systolic and diastolic differences in aortic measurements in healthy children. Materials and Methods : This retrospective study included 272 children who had a complete echocardiogram and no heart disease. Aortic measurements at the annulus (ANN), aortic root (AOR), sinotubular junction (STJ), and ascending aorta (AAO) were performed. Systolic and diastolic values were compared by calculating the mean systolic to diastolic (SD) percent difference for each segment; if the SD difference was >5%, it was considered clinically important. Similar measurements were conducted by another observer in 18% of the subjects. Results: Systolic measurements were larger than diastolic measurements with mean SD percent differences >5% ( P < 0.001) for the AOR (7.3% ± 5.5%), STJ (10.24% ± 7.1%), and AAO (9.8% ± 7.4%). There was no clinically significant SD difference for the ANN. There was an excellent intraclass correlation coefficient between observers (0.982–0.995). Conclusions: Systolic measurements for the AOR, STJ, and AAO were larger than diastolic measurements. Normal reference values are utilized to design treatment for patients with abnormal aortic sizes, and the timing in the cardiovascular cycle used to decide the reference values should be equivalent to the timing used to make measurements in clinical practice. This is particularly imperative as patients transition their care from a pediatric to an adult cardiologist.
Low‐density lipoprotein (LDL) apheresis has been shown to improve remission in patients with steroid‐resistant nephrotic syndrome (SRNS). Here, we report a case study of two patients who failed apheresis treatment for SRNS and required transplant with subsequent recurrence of nephrotic syndrome and response to apheresis treatment. Two patients were treated with 12 sessions of LDL apheresis for SRNS without improvement and subsequently required renal transplantation. The first patient received an ABO‐incompatible kidney transplant requiring plasma exchange (PE) with subsequent recurrence of focal segmental glomerulosclerosis. The second patient also received a renal transplant after treatment failure and subsequently developed recurrence of nephrotic syndrome in the transplanted kidney. Both patients underwent repeat therapy with lipoprotein apheresis. The first patient underwent lipoprotein apheresis after completing PE with significant improvement in serum creatinine and urine protein creatinine ratio. Three years later, he continued to do well and remains in remission. The second patient also responded well to repeat therapy with lipoprotein apheresis and had significant improvement with a urine protein creatinine ratio of 0.8 and a serum creatinine of 0.9 mg/dL 6 months after transplant. Lipoprotein apheresis was able to result in remission of nephrotic syndrome in these patients with posttransplant recurrence of disease. This is the first report of patients not responding to treatment pretransplant but responding posttransplant. Lipoprotein apheresis should be considered in patients with recurrence of nephrotic syndrome after renal transplantation even with a history of treatment failure prior to transplantation.
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