Autosomal dominant polycystic kidney disease (ADPKD) is the most common form of polycystic kidney disease (PKD) and is a typical adult-onset multisystem disorder. It is a progressive disease characterized by the disruption of renal tubular integrity, involving the modulation of cellular proliferation and apoptosis. Most ADPKD results from a mutation in either the PKD1 or PKD2 gene encoding polycystin-1 and polycystin-2, respectively. With the inconsistent disease course of ADPKD, biomarkers that can predict the treatment efficacy and rapid progression of the disease are needed. Studies have identified neutrophil gelatinase-associated lipocalin (NGAL) as a biomarker for predicting the progression of ADPKD patients. The NGAL protein is expressed at a low level in the kidneys, which helps to regulate iron transport and participates in epithelial differentiation, inflammation, and cell proliferation. NGAL level also increases in serum and urine during renal detrimental conditions such as ischemia and acute and chronic kidney diseases. On the other hand, some studies have also demonstrated that NGAL may act as a tubulogenic factor controlling cell growth and that the upregulation of the Ngal gene hinders tubular cell proliferation, resulting in significantly reduced cyst growth in cellular and murine models of ADPKD. This review attempts to correlate ADPKD and NGAL based on available research findings to evaluate the therapeutic potential of NGAL in ADPKD.

Background/Aims: Exacerbating factors of ulcerative colitis (UC) are multiple and complex with individual influence. We aimed to evaluate the efficacy of disease control by searching and restricting inflammation trigger factors of UC relapse individually in daily clinical practice. Methods: Both patients with UC history or new diagnosis were asked to avoid dairy products at first doctor visit. Individual-reported potential trigger factors were restricted when UC flared up (Mayo endoscopy score ≥1) from remission status. The remission rate, duration to remission and medication were analyzed between the groups of factor restriction complete, incomplete and unknown. Results: The total remission rate was 91.7% of 108 patients with complete restriction of dairy product. The duration to remission of UC history group was significantly longer than that of new diagnosis group (88.5 days vs. 43.4 days, P=0.006) in patients with initial endoscopic score 2–3, but no difference in patients with score 1. After first remission, the inflammation trigger factors in 161 relapse episodes of 72 patients were multiple and personal. Milk/dairy products, herb medicine/Chinese tonic food and dietary supplement were the common factors, followed by psychological issues, non-dietary factors (smoking cessation, cosmetic products) and discontinuation of medication by patients themselves. Factor unknown accounted for 14.1% of patients. The benefits of factor complete restriction included shorter duration to remission (P<0.001), less steroid and biological agent use (P=0.022) when compared to incomplete restriction or factor unknown group. Conclusions Restriction of dairy diet first then searching and restricting trigger factors personally if UC relapse can improve the disease control and downgrade the medication usage of UC patients in daily clinical practice.

OBJECTIVE: To establish diagnostic criteria for pulmonary arterial hypertension (PAH) in children by using parameters obtained through noninvasive cardiac computed tomography (CCT). MATERIALS AND METHODS: We retrospectively measured parameters from CCT images of children from a single institution in a multiple stepwise process. A total of 208 children with mean age of 10.5 years (range: 4 days–18.9 years) were assessed. The variables were classified into three groups: the great arteries; the ventricular walls; and the bilateral ventricular cavities. The relationship between the parameters obtained from the CCT images and mean pulmonary arterial pressure (mPAP) was tested and adjusted by the children's body size. Reference curves for the pulmonary trunk diameter (PTD) and ratio of diameter of pulmonary trunk to ascending aorta (rPTAo) of children with CCT images of normal hearts, adjusted for height, were plotted. Threshold lines were established on the reference curves. RESULTS: PTD and rPTAo on the CCT images were significantly positively correlated with mPAP (r > 0.85, p < 0.01). Height was the body size parameter most correlated with PTD (r = 0.91, p < 0.01) and rPTAo (r = −0.69, p < 0.01). On the basis of the threshold lines on the reference curves, PTD and rPTAo both showed 88.9% sensitivity for PAH diagnosis, with negative predictive values of 93.3% and 92.9%, respectively. CONCLUSION: PTD and rPTAo measured from CCT images were significantly correlated with mPAP in children. Reference curves and the formula of PTD and rPTAo adjusted for height could be practical for diagnosing PAH in children.
Aorta ; Arterial Pressure ; Arteries ; Body Size ; Child ; Diagnosis ; Heart ; Humans ; Hypertension ; Retrospective Studies