Variations in the course of the blood vessels are often incidental findings during clinical examination. Persistent left superior vena cava (PLSVC) is an uncommon anomaly, estimated to be present in about 0.3-0.5% of healthy individuals and in about 3-10% of patients with congenital heart disease. It results from the failure of the left anterior cardinal vein to degenerate during embryological development. Serious complications such as shock, angina and cardiac arrest have been described during catheterization in adults with a PLSVC. Since it frequently goes undiagnosed because of lack of symptoms when not accompanied by other anomalies, variations of the superior vena cava should be considered, especially when central venous catheterization via the subclavian or internal jugular vein is difficult. The embryological development, diagnosis, and clinical implications of a PLSVC are therefore reviewed in this article.

Left renal vein (LRV) entrapment, also known as nutcracker phenomenon if it is asymptomatic, is characterized by abnormality of outflow from the LRV into the inferior vena cava (IVC) due to extrinsic LRV compression, often accompanied by demonstrable lateral (hilar) dilatation and medial (mesoaortic) stenosis. Nutcracker syndrome, on the other hand, includes a well-defined set of symptoms, and the severity of these clinical manifestations is related to the severity of anatomic and hemodynamic findings. With the aim of providing practical guidance for nephrologists and radiologists, we performed a review of the literature through the PubMed database, and we commented on the definition, the main clinical features, and imaging pattern of this syndrome; we also researched the main therapeutic approaches validated in the literature. Finally, from the electronic database of our institute, we have selected some characteristic cases and we have commented on the imaging pattern of this disease.

Background Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent genetically determined renal disease. In affected patients, renal function may progressively decline up to end-stage renal disease (ESRD), and approximately 10% of those with ESRD are affected by ADPKD. The somatostatin analog octreotide long-acting release (octreotide-LAR) slows renal function deterioration in patients in early stages of the disease. We evaluated the renoprotective effect of octreotide-LAR in ADPKD patients at high risk of ESRD because of later-stage ADPKD. Methods and findings We did an internally funded, parallel-group, double-blind, placebo-controlled phase III trial to assess octreotide-LAR in adults with ADPKD with glomerular filtration rate (GFR) 15–40 ml/min/1.73 m 2 . Participants were randomized to receive 2 intramuscular injections of 20 mg octreotide-LAR ( n = 51) or 0.9% sodium chloride solution (placebo; n = 49) every 28 days for 3 years. Central randomization was 1:1 using a computerized list stratified by center and presence or absence of diabetes or proteinuria. Co-primary short- and long-term outcomes were 1-year total kidney volume (TKV) (computed tomography scan) growth and 3-year GFR (iohexol plasma clearance) decline. Analyses were by modified intention-to-treat. Patients were recruited from 4 Italian nephrology units between October 11, 2011, and March 20, 2014, and followed up to April 14, 2017. Baseline characteristics were similar between groups. Compared to placebo, octreotide-LAR reduced median (95% CI) TKV growth from baseline by 96.8 (10.8 to 182.7) ml at 1 year ( p = 0.027) and 422.6 (150.3 to 695.0) ml at 3 years ( p = 0.002). Reduction in the median (95% CI) rate of GFR decline (0.56 [−0.63 to 1.75] ml/min/1.73 m 2 per year) was not significant ( p = 0.295). TKV analyses were adjusted for age, sex, and baseline TKV. Over a median (IQR) 36 (24 to 37) months of follow-up, 9 patients on octreotide-LAR and 21 patients on placebo progressed to a doubling of serum creatinine or ESRD (composite endpoint) (hazard ratio [HR] [95% CI] adjusted for age, sex, baseline serum creatinine, and baseline TKV: 0.307 [0.127 to 0.742], p = 0.009). One composite endpoint was prevented for every 4 treated patients. Among 63 patients with chronic kidney disease (CKD) stage 4, 3 on octreotide-LAR and 8 on placebo progressed to ESRD (adjusted HR [95% CI]: 0.121 [0.017 to 0.866], p = 0.036). Three patients on placebo had a serious renal cyst rupture/infection and 1 patient had a serious urinary tract infection/obstruction, versus 1 patient on octreotide-LAR with a serious renal cyst infection. The main study limitation was the small sample size. Conclusions In this study we observed that in la...

Our results suggest, for the first time, the feasibility of CEUS, a low-cost and low-risk diagnostic procedure, in the diagnosis of APN in kidney transplant patients.