Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of chronic kidney disease and the fourth leading cause of end-stage kidney disease, accounting for over 50% of prevalent cases requiring renal replacement therapy. There is a pressing need for improved therapy for ADPKD. Recent insights into the pathophysiology of ADPKD revealed that cyst cells undergo metabolic changes that up-regulate aerobic glycolysis in lieu of mitochondrial respiration for energy production, a process that ostensibly fuels their increased proliferation. The present work leverages this metabolic disruption as a way to selectively target cyst cells for apoptosis. This small-molecule therapeutic strategy utilizes 11beta-dichloro, a repurposed DNA-damaging anti-tumor agent that induces apoptosis by exacerbating mitochondrial oxidative stress. Here, we demonstrate that 11beta-dichloro is effective in delaying cyst growth and its associated inflammatory and fibrotic events, thus preserving kidney function in perinatal and adult mouse models of ADPKD. In both models, the cyst cells with homozygous inactivation of Pkd1 show enhanced oxidative stress following treatment with 11beta-dichloro and undergo apoptosis. Co-administration of the antioxidant vitamin E negated the therapeutic benefit of 11beta-dichloro in vivo, supporting the conclusion that oxidative stress is a key component of the mechanism of action. As a preclinical development primer, we also synthesized and tested an 11beta-dichloro derivative that cannot directly alkylate DNA, while retaining pro-oxidant features. This derivative nonetheless maintains excellent anti-cystic properties in vivo and emerges as the lead candidate for development.

Echinococcosis is caused by tapeworms belonging to the Echinococcus genus. The most common site of infection is the liver although it may involve almost any organ. Symptoms of pulmonary echinococcosis vary depending on the location and structure of the cyst. While uncomplicated cysts usually appear at imaging as well-defined homogeneous lesions with fluid content and smooth walls of variable thickness, complicated lesions may have a more heterogeneous content with higher density making more difficult the distinction from malignancies or other infections. Hereby we describe the case of a 61-year-old Northern African male admitted to our tertiary center for left upper chest pain who then underwent a chest computed tomography (CT) scan which demonstrated a large hypodense lesion, with smooth and thick walls, in the upper left lobe. The following magnetic resonance confirmed the homogeneous fluid content, and the 18 F- fluorodeoxyglucose-positron emission tomography/CT demonstrated a mild uptake of the walls. According to these findings, the main differential diagnoses at imaging included bronchogenic cyst, synovial sarcoma, and pulmonary hematoma although the patient denied any recent trauma. Given the large size and clinical symptoms he underwent surgery. Intra-operative frozen section, supported by imprint cytology, excluded the presence of malignancy while suggested an echinococcal laminar exocyst. The final pathological examination confirmed the diagnosis of echinococcosis (i.e., Echinococcus Granulosus protoscolex). After surgery he was treated with albendazole and at the six-month follow-up he was in good clinical conditions. Our case highlights the importance of considering rare infections, particularly in individuals from endemic areas. Frozen tissue analyses can be a diagnostic challenge and often require ancillary tools such as imprint cytology and serial sections for more sensitive and accurate diagnosis.

Renal cystic diseases are a clinically and genetically diverse group of renal diseases that can manifest in utero, infancy, or throughout childhood and adulthood. These diseases may be unilateral or bilateral with a single cyst or multiple cysts, or with increased echogenicity of the renal cortex without macroscopic cysts. Certain cystic renal diseases are life-threatening, with many developing chronic kidney and hepatic disease if not recognized early enough. Therefore, due to the prevalence and life-altering complications of this specific group of diseases in vulnerable populations, it is crucial for clinicians and healthcare providers to have an overall understanding of cystic diseases and how to pre-emptively detect and manage these conditions. In this review, we discuss in detail the epidemiology, genetics and pathophysiology, diagnosis, presentation, and management of numerous genetic and sporadic renal cystic diseases, such as polycystic kidney disease, multicystic dysplastic kidney, and calyceal diverticula, with an emphasis on prenatal care and pregnancy counseling.

Caroli disease is a rare congenital disorder resulting from the dilation of large intrahepatic bile ducts. Patients affected with Caroli disease are at increased risk of complications resulting from bile stasis and stone formation. We report the case of a 37-year-old woman with a past surgical history of cholecystectomy who presented to the emergency room with a chief complaint of abdominal pain and nausea. The pain was characteristic of acute pancreatitis but she was hemodynamically stable. Total bilirubin was 4.1 mg/dL with a direct fraction of 3.1 mg/dL, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were 850 IU/L and 1025 IU/L, respectively. Serum amylase and lipase were elevated at 581 IU/L and 1328 IU/L, respectively. CT scan of abdomen/pelvis without contrast showed common bile duct (CBD) measuring 1.6 cm with intrahepatic biliary system dilation and mild peripancreatic fat stranding. She was diagnosed with acute pancreatitis. On the second day, she developed a temperature of 99.6°F. Hepatitis immunity panel was negative for acute hepatitis. The patient was started on antibiotics (IV ciprofloxacin and metronidazole) for suspicion of acute cholangitis. Endoscopic retrograde cholangiopancreatography (ERCP) was done which showed mild dilated intrahepatic ducts and CBD dilation of 1.6 cm, and a choledochal cyst at CBD. Sphincterotomy was done and good bile drainage was reported. She was later discharged in a stable condition. Caroli disease affects males and females equally and most are diagnosed before the age of 30 years correlated with the onset of symptoms. By far, the most commonly reported symptom is acute cholangitis but pancreatitis occurs rarely. Recurrent bouts of infection lead to portal hypertension, fibrosis of the liver and ultimately end up with an orthotopic liver transplant (OLT). Regular follow-ups are important for disease surveillance and monitoring.

While typical ultrasound patterns of ciliopathy-related cystic kidney diseases have been described in children, ultrasound findings can overlap between different diseases and atypical patterns exist. In this study, we assessed the presence of the \"salt and pepper\" pattern in different renal ciliopathies and looked for additional ultrasound features.
This single-center, retrospective study included all patients with a molecular-proven diagnosis of renal ciliopathy, referred to our center between 2007 and 2017. Images from the first and follow-up ultrasound exams were reviewed. Basic ultrasound features were grouped into patterns and compared to genetic diagnoses. The \"salt and pepper\" aspect was described as enlarged kidneys with heterogeneous, increased parenchymal echogenicity.
A total of 41 children with 5 different renal ciliopathies were included (61% male; median age, 6 years [range, 3 days to 17 years]). The \"salt and pepper\" pattern was present in 14/15 patients with an autosomal recessive polycystic kidney disease (ARPKD). A similar pattern was found in 1/4 patients with an autosomal dominant polycystic kidney disease and in 1/11 patients with HNF1B mutation. Additional signs found were areas of cortical sparing, comet-tail artifacts, and color comet-tail artifacts.
Although the \"salt and pepper\" ultrasound pattern is predominantly found in ARPKD, it may be detected in other ciliopathies. The color comet-tail artifact is an interesting sign when suspecting a renal ciliopathy in case of enlarged hyperechoic kidneys with no detectable microcysts on B-mode grayscale ultrasound.

BACKGROUND: Adventitial cystic disease is relatively rare vascular disease, frequently occurred in the popliteal artery. No definitive treatment has been established yet.
UNASSIGNED: A 53-year-old woman presenting intermittent claudication of the right leg was diagnosed as adventitial cystic disease of popliteal artery. Percutaneous balloon dilation yielded an immediate recurrence. The disease was successfully treated by bypass grafting utilizing the short saphenous vein to replace the part of the popliteal artery containing the adventitial cyst. No postoperative complication was found six months after surgery.
CONCLUSIONS: Comparing to a great saphenous vein, a short saphenous vein as a material of bypass graft has a significant advantage, as only a single surgical field is necessary.
CONCLUSIONS: We propose that bypass graft surgery employing a short saphenous vein is worth considering as a treatment of adventitial cystic disease at the popliteal artery.

BACKGROUND: Biliary diseases during pregnancy are not uncommon and are frequently due to cholelithiasis. Choledochal cyst during pregnancy is rare. The management of biliary pathologies during pregnancy poses a challenge as the window of opportunity to carry out any interventions with minimal risk is small.
METHODS: We report the case of a lady who was diagnosed with a large type I choledochal cyst during the second trimester of pregnancy. Due to increasing symptoms and recurrent cholangitis, she was managed with antibiotics and surgical resection. She remained well and delivered a healthy baby boy.
CONCLUSIONS: Clinicians need to consider choledochal cyst as a differential in pregnant patients presenting with upper abdominal fullness.

In this study, we describe our diagnosis and treatment of two patients who presented with femoral vessel compression caused by a cystic lesion in the groin. One case was diagnosed as adventitial cystic disease (ACD) of the common femoral artery resulting in leg claudication and the other was diagnosed as a ganglion cyst (GC) causing femoral vein compression and unilateral leg swelling. The operative findings differed between these two cases with respect to the dissection of the cyst and femoral vessel, but the postoperative histological examination results were similar. The pathogenesis of ACD and GC is not fully understood, and further investigation is needed to delineate the exact pathology of these uncommon conditions.

Arginine vasopressin (AVP) has a key role in osmoregulation by facilitating water transport in the collecting duct. Recent evidence suggests that AVP may have additional effects on renal function and favor cyst growth in polycystic kidney disease. Whether AVP also affects kidney structure in the general population is unknown. We analyzed the association of copeptin, an established surrogate for AVP, with parameters of renal function and morphology in a multicentric population-based cohort. Participants from families of European ancestry were randomly selected in three Swiss cities. We used linear multilevel regression analysis to explore the association of copeptin with renal function parameters as well as kidney length and the presence of simple renal cysts assessed by ultrasound examination. Copeptin levels were log-transformed. The 529 women and 481 men had median copeptin levels of 3.0 and 5.2 pmol/L, respectively (P<0.001). In multivariable analyses, the copeptin level was associated inversely with eGFR (β=-2.1; 95% confidence interval [95% CI], -3.3 to -0.8; P=0.002) and kidney length (β=-1.2; 95% CI, -1.9 to -0.4; P=0.003) but positively with 24-hour urinary albumin excretion (β=0.11; 95% CI, 0.01 to 0.20; P=0.03) and urine osmolality (β=0.08; 95% CI, 0.05 to 0.10; P<0.001). A positive association was found between the copeptin level and the presence of renal cysts (odds ratio, 1.6; 95% CI, 1.1 to 2.4; P=0.02). These results suggest that AVP has a pleiotropic role in renal function and may favor the development of simple renal cysts.

Lymphangiomyomatosis (LAM) is a rare cystic interstitial lung disease that exclusively affects women of child bearing age and is associated with vascular proliferation of smooth muscle cells in the lung. We report a case of young female with pulmonary LAM presenting with recurrent pneumothorax.