Postoperative Acute Kidney Injury in Williams Syndrome Compared With Matched Controls.

OBJECTIVES: Cardiovascular manifestations occur in over 80% of Williams syndrome (WS) patients and are the leading cause of morbidity and mortality. One-third of patients require cardiovascular surgery. Renal artery stenosis (RAS) is common in WS. No studies have assessed postoperative cardiac surgery-related acute kidney injury (CS-AKI) in WS. Our objectives were to assess if WS patients have higher risk of CS-AKI postoperatively than matched controls and if RAS could contribute to CS-AKI. DESIGN: This was a retrospective study of all patients with WS who underwent cardiac surgery at our center from 2010 to 2020. The WS study cohort was compared with a group of controls matched for age, sex, weight, and surgical procedure. SETTING: Patients underwent cardiac surgery and postoperative care at Lucile Packard Children's Hospital Stanford. PATIENTS: There were 27 WS patients and 43 controls (31% vs 42% female; p = 0.36). Median age was 1.8 years (interquartile range [IQR], 0.7-3.8 yr) for WS and 1.7 years (IQR, 0.8-3.1 yr) for controls. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Postoperative hemodynamics, vasopressor, total volume input, diuretic administration, and urine output were collected in the first 72 hours. Laboratory studies were collected at 8-hour intervals. Multivariable analysis identified predictors of CS-AKI.Controlled for renal perfusion pressure (RPP) and vasoactive inotrope score (VIS), compared with controls, the odds ratio (OR) of CS-AKI in WS was 4.2 (95% CI, 1.1-16; p = 0.034). Higher RPP at postoperative hours 9-16 was associated with decreased OR of CS-AKI (0.88 [0.8-0.96]; p = 0.004). Increased VIS at hour 6 was associated with an increased OR of CS-AKI (1.47 [1.14-1.9]; p = 0.003). Younger age was associated with an increased OR of CS-AKI (1.9 [1.13-3.17]; p = 0.015). CONCLUSIONS: The OR of CS-AKI is increased in pediatric patients with WS compared with controls. CS-AKI was associated with VIS at the sixth postoperative hour. Increases in RPP and mean arterial pressure were associated with decreased odds of CS-AKI.

The initial process of enamel prism arrangement and its relation to the Hunter-Schreger bands in dog teeth.

The three-dimensional architecture of enamel prisms at early stages of enamel formation and its spatial relationship to the Hunter-Schreger bands were examined in canine tooth germs by light and electron microscopy. In serial semithin sections of demineralized tooth germs tangential to the enamel-dentin junction, a straight row of enamel prisms was depicted along the longitudinal tooth axis at the level of the enamel-dentin junction and then their three-dimensional arrangement was reconstructed using computer software. The spatial arrangement of the groups of enamel rods oriented in specific sideward directions was also reconstructed in deep layers of the enamel. Initially, all enamel prisms were parallel to perpendicular toward the enamel-dentin junction, but at 10µm from the enamel-dentin junction, some small specks, or groups of enamel prisms--tilting to the right or the left--emerged as small islands. In each speck of enamel prism, the inclined prisms were uniformly oriented in a sideward direction and gradually expanded their boundary until merging with the neighboring specks inclined in the same direction. Consequently, at 50µm from the enamel-dentin junction, the group of enamel prisms oriented either to the right or the left formed alternately arranged horizontal belt-like zones, corresponding to the parazone or the diazone of the Hunter-Schreger bands. Reversed images of scanning electron-micrographs of the exposed surfaces of the developing enamel revealed round and bulb-like profiles of Tomes' processes at early amelogenesis and its changes into a characteristic structure combined with flat secretory and enclosing nonsecretory faces that dictated the orientation of corresponding enamel prisms. The results suggest that the groups of enamel prisms oriented in sideward directions first appear as small island-like specks near the enamel-dentin junction, which later merge and form alternating horizontal belt-like zones as a consequence of morphological changes of the Tomes' processes. However, the mechanisms whereby the functional grouping of secretory ameloblasts with similarly oriented Tomes' processes is induced are yet to be determined.