Cystic Disease Research Articles

Idiopathic Bilateral Wunderlich Syndrome: A Case Report

Wunderlich Syndrome (WS) is a rare condition characterized by spontaneous bleeding in the kidney without any traumatic event. It usually happens unilaterally, but bilateral cases are rare. The most common causes of WS are renal neoplasms, vascular disorders, infections, renal cystic diseases, and anticoagulation states, with idiopathic cases being uncommon. Here, we present a case of bilateral idiopathic Wunderlich Syndrome.

Extrahepatic and extrapulmonary hydatid cysts as primary lesions

Background: Hydatid cyst disease, caused by the larval stage of the parasite Echinococcus granulosus, commonly affects the liver and lungs but can also manifest in various extrahepatic and extrapulmonary locations. Splenic hydatid disease, despite being rare, is the third most common location. This paper presents a case series highlighting the clinical presentation, diagnostic challenges, and management strategies for patients with extrahepatic and extrapulmonary hydatid cysts. Several studies already postulated on sites of hydatid cysts which have statistically significant results. Methods: Patients who were operated on for hydatid disease or cystic lesions, which were later diagnosed as hydatid disease, between September 2022-August 2023 were retrieved retrospectively. Patients with lesions localized outside the liver and the lung as well as in liver and lung were enrolled in the study. Fifty-Two patients with extra-hepatic primary hydatid disease were treated surgically at our clinic. The cysts were located in different part of body. Results has undergone statistical methods like Z-test and Mann-Whitney U test. Any patient of any gender admitted with diagnosis of hydatid cyst in any part of body with age >18 but less than 70 years irrespective of any comorbidities. Results: Surgical techniques like partial or total cystectomy with or without tube drainage are good option for management of extrahepatic and extrapulmonary primary hydatid cysts. There were no complications or mortality in the postoperative period. Hydatid cyst is considered in the differential diagnosis of cystic lesions, especially in endemic areas. Surgical technique planned according to the location of the cyst. Conclusions: Cystectomy is a surgical option in extrahepatic and extrapulmonary hydatid cyst which is evaluate better in this study.

Single-Center Experience of Pediatric Cystic Kidney Disease and Literature Review.

Pediatric cystic kidney disease (CyKD) includes conditions characterized by renal cysts. Despite extensive research in this field, there are no reliable genetics or other biomarkers to estimate the phenotypic consequences. Therefore, CyKD in children heavily relies on clinical and diagnostic testing to predict the long-term outcomes. A retrospective study aimed to provide a concise overview of this condition and analyze real-life data from a single-center pediatric CyKD cohort followed during a 12-year period. Medical records were reviewed for extensive clinical, laboratory, and radiological data, treatment approaches, and long-term outcomes. During the study period, 112 patients received a diagnosis of pediatric CyKD. Male patients were more involved than female (1:0.93). Fifty-six patients had a multicystic dysplastic kidney; twenty-one of them had an autosomal dominant disorder; fifteen had an isolated renal cyst; ten had been diagnosed with autosomal recessive polycystic kidney disease; three had the tuberous sclerosis complex; two patients each had Bardet-Biedl, Joubert syndrome, and nephronophthisis; and one had been diagnosed with the trisomy 13 condition. Genetic testing was performed in 17.9% of the patients, revealing disease-causing mutations in three-quarters (75.0%) of the tested patients. The most commonly presenting symptoms were abdominal distension (21.4%), abdominal pain (15.2%), and oligohydramnios (12.5%). Recurrent urinary tract infections (UTI) were documented in one-quarter of the patients, while 20.5% of them developed hypertension during the long-term follow-up. Antibiotic prophylaxis and antihypertensive treatment were the most employed therapeutic modalities. Seventeen patients progressed to chronic kidney disease (CKD), with thirteen of them eventually reaching end-stage renal disease (ESRD). The time from the initial detection of cysts on an ultrasound (US) to the onset of CKD across the entire cohort was 59.0 (7.0-31124.0) months, whereas the duration from the detection of cysts on an US to the onset of ESRD across the whole cohort was 127.0 (33.0-141.0) months. The median follow-up duration in the cohort was 3.0 (1.0-7.0) years. The patients who progressed to ESRD had clinical symptoms at the time of initial clinical presentation. This study is the first large cohort of patients reported from Croatia. The most common CyKD was the multicystic dysplastic kidney disease. The most common clinical presentation was abdominal distention, abdominal pain, and oliguria. The most common long-term complications were recurrent UTIs, hypertension, CKD, and ESRD.

Congenital cystic diseases of the lung

Congenital cystic diseases of the lung are rare but their incidence is increasing, especially with the widespread use of antenatal ultrasound. Close relationships between airway and respiratory segments and vascular structures during embryonic development can lead to malformations. It should be remembered that congenital cystic lung diseases have the potential to be life-threatening and the possibility of missed diagnosis is high. Surgical resection yields good results in symptomatic lesions, with lobectomy being the most commonly performed surgical approach. Some centers recommend surgical resection even in asymptomatic patients to prevent infection and exclude malignancy.

Unexpected origins: Bronchopulmonary dysplasia and the unusual spectrum of cystic lung disease.

Unexpected origins: Bronchopulmonary dysplasia and the unusual spectrum of cystic lung disease.

Abstract 4358: Genomic profiling of residual kidney structures in dialysis patients

Abstract Background: Chronic kidney disease is frequently associated with persistent inflammation, which results in fibrosis in the stroma, a reduced number of renal tubules, the formation of multiple cystic lesions, ultimately terminating in renal failure and hemodialysis. The high incidence of renal cell carcinoma (RCC) in hemodialysis patients suggests a relationship between tissue remodeling by chronic inflammation and carcinogenesis. However, little is known about the genetic background of cancer development from remaining tubules and cystic lesions in hemodialysis patients. Methods: We enrolled 5 patients under hemodialysis who were accompanied by acquired cystic kidney disease and who underwent total nephrectomy for renal cancer. Surgical specimens were fixed with alcohol-based solution and paraffin-embedded, and after H&E staining, subjected to laser capture microdissection (LCM) to collect remaining tubules and cysts containing approximately 200 cells. DNA was extracted and analyzed for somatic mutations and copy number alteration by whole-exome sequencing. Results: In total, we collected 161 LCM samples, including 118 from proximal tubules, 17 from collecting ducts, and 26 from cysts. The median variant allele frequencies of detected mutations were 0.237 in proximal tubules, 0.133 in collecting duct, and 0.381 in cysts, indicating larger clonal expansion in proximal tubules and cysts than in collecting ducts. Proximal tubules and cysts contained recurrent mutations in FAT1 (11% and 8%, respectively), STAG2 (5% and 8%), and PTEN (3% and 4%), whereas no recurrent driver mutations were identified in collecting ducts. In copy number analysis, 26% of proximal tubules, 35% of collecting ducts, and 80% of cysts had copy number alterations. Of note, samples from cysts had more copy number alterations compared to remaining tubules (on average, 2.3 vs. 1.1). Proximal tubules had recurrent copy number gains of chromosomes 3, 7, 10, 18, and 20, and loss of chromosome 22 and samples from collecting ducts showed gains of chromosome 7 and loss of chromosome 18. In cysts, gains of chromosomes 2, 3, 7, 10, 12, and 16 and loss of chromosomes 15, 16, 21, and 22 were frequently observed. Copy number profile in cysts was similar to that of papillary RCC and acquired cystic disease-associated RCC. Conclusion: In the end-stage cystic kidney, both proximal tubules and cysts exhibited an enrichment of driver mutations commonly found in papillary RCC and RCC with acquired cystic disease, reflecting the fact that the incidence of these two types of RCC increases as the duration of dialysis becomes longer. In addition, clear cell RCC drivers, such as VHL mutation and loss of chromosome 3, were not observed, possibly explaining the rare occurrence of clear cell RCC in hemodialysis patients. Since cysts had more frequent copy number alterations than remaining tubules, cysts in the end-stage kidney might be precursor lesions of RCC. Citation Format: Kosuke Ieiri, Nobuyuki Kakiuchi, Tomonori Hirano, Koichi Watanabe, Hirona Maeda, Hiroko Tanaka, Satoru Miyano, Dai Takamatsu, Takashi Matsumoto, Keisuke Monji, Masaki Shiota, Junichi Inokuchi, Hideki Makishima, Masatoshi Eto, Seishi Ogawa. Genomic profiling of residual kidney structures in dialysis patients [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 1 (Regular Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(6_Suppl):Abstract nr 4358.

Abstract 1072: The EsomiR project: A case control study to assess the role of exosomal miRNAs in breast cancer cancerogenesis

Abstract Introduction: One of the unmet needs in Breast Cancer (BC) prevention is how to identify women at higher risk of developing this disease, who might benefit from periodic surveillance and/or from chemoprevention. The goal of this retrospective study is to assess the role miRNAs isolated from extracellular vesicles (EV-miRNAs) as possible early biomarkers for identifying patients with higher risk of developing BC, in order to personalize their screening programme. To this goal, EV-miRNAs from cyst fluid derived from women with Gross Cyst Disease of the Breast (GCDB - a benign disease of the mammary gland associated with a 2-4-fold increase in the risk of developing BC) have been analysed. Methodology: Cyst fluids have been selected among samples from a cohort of 600 patients diagnosed with GCDB between 1985 and 1993, some of whom developed BC during follow-up. EV-miRNAs have been extracted from cyst fluid using the exoRNeasy midi kit (Qiagen), qualitatively evaluated by Bioanalyzer (Agilent) and quantified by Qubit™ using the microRNA Assay Kit (Thermo Fisher Scientific). The MiRNome profiles have been assessed by microarray using an optimised protocol on Agilent platform (labelling and hybridization on SurePrint Human miR Microarrays 8 × 60 K and images acquisition by G2565CA scanner). Results: A total of 117 samples (58 cases and 59 controls, paired on the bases of clinical and pathological variables) have been analysed. Single variable logistic regression analysis on the most expressed EV-miRNAs (logFC >= 6 (n=329)), selected six EV-miRNAs (miR-6076, miR-202-3p, miR-6872-3p, miR-769-3p, miR-5195-3p, miR-4443) and one (miR-4713-3p) negatively and positively associated with BC development, respectively (raw p-value <0.05). A risk score combining the seven EV-miRNAs was derived by multivariable logistic regression modelling and reported an AUC=0.73 (95% [CI] 0.6427-0.8231). The model was refined including clinical variables and applying step-wise logistic regression analysis. The combination of 3 EV-miRNAs (miR-6076, miR-6872-3p, miR-5195-3p) with menopausal status, familiarity and type of cyst yielded an AUC of 0.8 (95% [CI] 0.7015-0.8847). Logistic regression was also performed on Type I cysts (n=78), that are correlated with a higher risk of developing BC, pointing in evidence 3 EV-miRNAs with a negative (miR-6076, miR-6872-3p) or positive (miR-4515) correlation with BC development risk (AUC = 0.74, 95% [CI] 0.6278-0.8475). In the model comprehensive of clinical variables, two of those EV-miRNAs (miR-6872-3p, miR-4515) together with familiarity and menopausal status were selected, giving an AUC of 0.8 (95% [CI] 0.6961-0.8991). Conclusion: This study allowed the identification of a EV-miRNA-based BC risk signature obtained by liquid biopsy on cyst fluid. The final purpose of this project is to transfer this signature in the clinic by testing it prospectively on plasma samples. Citation Format: Barbara Cardinali, Patrizia Piccioli, Francesco Boccardo, Alessandra Rubagotti, Linda Zinoli, Andrea Sciutto, Simona Coco, Giovanna Chiorino, Paola Ostano, Emir Sehovic, Zita Cavalieri, Cristina Bruzzo, Silvia Marconi, Roberta Tasso, Rodolfo Quarto, Davide Ceresa, Paolo Malatesta, Lucia Del Mastro. The EsomiR project: A case control study to assess the role of exosomal miRNAs in breast cancer cancerogenesis [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 1 (Regular Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(6_Suppl):Abstract nr 1072.

Ovarian dermoid cyst in a buffalo

AbstractOvarian dermoid cyst is characterised by the presence of hair follicles, sebaceous glands grossly observed as clusters of matted hair in greyish cheesy material in the vicinity of ovaries. Upon examination of 235 buffalo genitalia, five were found to have abnormal ovarian texture and were relatively large in size. Further investigation revealed presence of dermoid cyst in these ovaries. Histopathological analysis showed degenerated primary, secondary and tertiary/Graafian follicles in the ovarian parenchyma, with patches of neuro‐ectodermal rosette‐like structures composed of glial cells. Other extra‐gonadal tissues often less organised were also observed like cartilage, fibrous connective tissue and glandular structures. In two of the specimens, corpus luteum with degenerated luteal cells was also present in the parenchyma of the ovary. These detailed investigations pointed to the infertility problems in buffaloes associated with this condition and often dictated in slaughterhouse‐derived ovaries. It has clinical significance as these structures are often confused with cystic ovarian disease upon rectal palpation, and treatment is unsuccessful in reducing the size of the ovary at the field level.

Genetically encoded red fluorescent pH ratiometric sensor: Application to measuring pH gradient abnormalities in cystic fibrosis cells

Genetically encoded probes to measure in vivo pH are challenging. They must be chloride insensitive and require normalization of the transfection efficiency. Furthermore, probes that emit in the red or far red are advisable to promote in vivo use. The mBeRFP D162T fluorescent protein presents two emission bands with different pH sensitivities. When the probe was cytosolic-expressed in HeLa as control cells and CFBE41o, an epithelial cell line that carries an F508del mutation in the CFTR transporter, the ratiometric measurement between both emission bands allows us to determine the pH, demonstrating that mBeRFP D162T can be used to accurately measure the cytosolic pH differences of these cell lines.Furthermore, we have located the sensor inside or outside the lysosomal membrane to investigate the lysosomal pH gradient. In HeLa cells, our probe detected pH gradient changes under conditions known to alter lysosomal pH. In the CFBE41o cells, which mimic cystic fibrosis disease, we observed a complete loss of lysosomal acidification. Using lumacaftor, a drug that restores functioning CFTR protein, partially brings back the pH gradient. In conclusion, mBeRFP D162T is a valuable tool for measuring in vivo intracellular pH values and lysosomal pH gradient dynamics in physiological or pathological conditions.

Modern paradigm in the diagnosis of cystic diseases of the kidneys

Cystic diseases of the kidneys are a fairly common pathology, which has a negative impact on the course of underlying disease affecting the kidneys, or even being the primary renal pathology. The purpose of this review is to analyze the latest literature data on the etiology, pathogenesis, and diagnosis of cystic kidney diseases. This article highlights some aspects of the pathogenesis, diagnosis, and treatment of cystic kidney diseases in order to deepen knowledge about this pathology. Important nuances of ultrasound diagnosis of cystic kidney diseases are discussed. Modern equipment allows diagnosing almost all variants of cystic kidney disease. Training in ultrasound examination or at least knowledge of sonographic interpretation should be part of training in nephro­logy.

1H, 13C and 15N backbone resonance assignments of hepatocyte nuclear factor-1-beta (HNF1β) POUS and POUHD.

Hepatocyte nuclear factor 1β (HNF1β) is a transcription factor that plays a key role in the development and function of the liver, pancreas, and kidney. HNF1β plays a key role in early vertebrate development and the morphogenesis of these organs. In humans, heterozygous mutations in the HNF1B gene can result in organ dysplasia, making it the most common cause of developmental renal diseases, including renal cysts, renal malformations, and familial hypoplastic glomerular cystic kidney disease. Pathogenic variants in the HNF1B gene are known to cause various diseases, including maturity-onset diabetes of the young and developmental renal diseases. This study presents the backbone resonance assignments of HNF1β POUS and POUHD domains, which are highly conserved domains required for the recognition of double-stranded DNA. Our data will be useful for NMR studies to verify the altered structures and functions of mutant HNF1B proteins that can induce developmental renal diseases, including renal cysts, renal malformations, and familial hypoplastic glomerular cystic kidney disease. This study will provide the structural basis for future studies to elucidate the molecular mechanisms underlying how mutations in HNF1β cause diseases.

Alterations in the fecal microbiota in patients with advanced cystic fibrosis liver disease after 6 months of elexacaftor/tezacaftor/ivacaftor

BackgroundCystic fibrosis associated liver disease (CFLD) carries a significant disease burden with no effective preventive therapies. According to the gut-liver axis hypothesis for CFLD pathogenesis, dysbiosis and increased intestinal inflammation and permeability permit pathogenic bacterial translocation into the portal circulation, leading to hepatic inflammation and fibrosis. Evaluating the effect of CFTR (cystic fibrosis transmembrane conductance regulator) modulation with elexacaftor/tezacaftor/ivacaftor (ETI) may help determine the role of CFTR in CFLD and increase understanding of CFLD pathogenesis, which is critical for developing therapies. We aimed to characterize the fecal microbiota in participants with CF with and without advanced CFLD (aCFLD) before and after ETI. MethodsThis is an ancillary analysis of stool samples from participants ages ≥12 y/o enrolled in PROMISE (NCT04038047). Included participants had aCFLD (cirrhosis with or without portal hypertension, or non-cirrhotic portal hypertension) or CF without liver disease (CFnoLD). Fecal microbiota were defined by shotgun metagenomic sequencing at baseline and 1 and 6 months post-ETI. ResultsWe analyzed 93 samples from 34 participants (11 aCFLD and 23 CFnoLD). Compared to CFnoLD, aCFLD had significantly higher baseline relative abundances of potential pathogens Streptococcus salivarius and Veillonella parvula. Four of 11 aCFLD participants had an initially abnormal fecal calprotectin that normalized 6 months post-ETI, correlating with a significant decrease in S. salivarius and a trend towards decreasing V. parvula. ConclusionsThese results support an association between dysbiosis and intestinal inflammation in CFLD with improvements in both post-ETI, lending further support to the gut-liver axis in aCFLD.

Surgical excision of the right ventricular hydatid cyst: a case report

Hydatid cyst is described as an endemic situation predominantly observed in different undeveloped regions of the world which is caused by Echinococcus granulosus tapeworm. This disease usually affects the lungs or liver. Cardiac location is reported in less than 2 % to 0.5% of patients in all cases and as in our case the intraventricular location is seen seldomly. A 38-year-old female patient with diagnosed systemic hydatid cyst disease was consulted with chest pain, palpitation, and shortness of breath. Transthoracic echocardiography and cardiac computerized tomography imaging showed the location of the cardiac hydatid cyst was the right ventricle. She underwent elective surgery swiftly and was discharged uneventfully.

Diagnosis, Management, and Prognosis of Cystic Fibrosis-Related Liver Disease in Children.

Cystic fibrosis (CF) is a multifaceted disorder predominantly investigated for its pulmonary manifestations, yet patients with CF also exhibit a spectrum of extrapulmonary manifestations, notably those involving the hepatobiliary system. The latter constitutes the third leading cause of morbidity and mortality in individuals with CF. Cystic fibrosis-related liver disease (CFLD), with an escalating prevalence, manifests diverse clinical presentations ranging from hepatomegaly to cirrhosis and hepatopulmonary syndrome. Consequently, early detection and appropriate management are imperative for sustaining the health and influencing the quality of life of CF patients afflicted with CFLD. This review aims to consolidate existing knowledge by providing a comprehensive overview of hepatobiliary manifestations associated with CF. It delineates the clinical hepatobiliary manifestations, diagnostic methodologies, incorporating minimally invasive markers, and therapeutic approaches, encompassing the impact of novel CFTR modulators on CFLD. Given the exigency of early diagnosis and the intricate management of CFLD, a multidisciplinary team approach is essential to optimize care and enhance the quality of life for this subset of patients. In conclusion, recognizing CF as more than solely a pulmonary ailment, the authors underscore the imperative for further clinical investigations to establish a more robust evidence base for CFLD management within the continuum of this chronic disease.

The Link between Autosomal Dominant Polycystic Kidney Disease and Chromosomal Instability: Exploring the Relationship.

In autosomal dominant polycystic kidney disease (ADPKD) with germline mutations in a PKD1 or PKD2 gene, innumerable cysts develop from tubules, and renal function deteriorates. Second-hit somatic mutations and renal tubular epithelial (RTE) cell death are crucial features of cyst initiation and disease progression. Here, we use established RTE lines and primary ADPKD cells with disease-associated PKD1 mutations to investigate genomic instability and DNA damage responses. We found that ADPKD cells suffer severe chromosome breakage, aneuploidy, heightened susceptibility to DNA damage, and delayed checkpoint activation. Immunohistochemical analyses of human kidneys corroborated observations in cultured cells. DNA damage sensors (ATM/ATR) were activated but did not localize at nuclear sites of damaged DNA and did not properly activate downstream transducers (CHK1/CHK2). ADPKD cells also had the ability to transform, as they achieved high saturation density and formed colonies in soft agar. Our studies indicate that defective DNA damage repair pathways and the somatic mutagenesis they cause contribute fundamentally to the pathogenesis of ADPKD. Acquired mutations may alternatively confer proliferative advantages to the clonally expanded cell populations or lead to apoptosis. Further understanding of the molecular details of aberrant DNA damage responses in ADPKD is ongoing and holds promise for targeted therapies.

Prominent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice

Cystic fibrosis-related liver disease (CFLD) is a chronic cholangiopathy that increases morbidity and mortality in patients with CF. Current treatments are unsatisfactory, and incomplete understanding of CFLD pathogenesis hampers therapeutic development. We have previously shown that mouse CF cholangiocytes respond to lipopolysaccharide with excessive inflammation. Thus, we investigated the role of the gut-liver axis in the pathogenesis of CFLD. Wild-type (WT), whole-body Cftr knockout (CFTR-KO) and gut-corrected (CFTR-KO-GC) mice were studied. Liver changes were assessed by immunohistochemistry and single-cell transcriptomics (single-cell RNA sequencing), inflammatory mediators were analysed by proteome array, faecal microbiota by 16S ribosomal RNA sequencing and gut permeability by FITC-dextran assay. The livers of CFTR-KO mice showed ductular proliferation and periportal inflammation, whereas livers of CFTR-KO-GC mice had no evident pathology. Single-cell RNA sequencing analysis of periportal cells showed increased presence of neutrophils, macrophages and T cells, and activation of pro-inflammatory and pathogen-mediated immune pathways in CFTR-KO livers, consistent with a response to gut-derived stimuli. CFTR-KO mice exhibited gut dysbiosis with enrichment of Enterobacteriaceae and Enterococcus spp., which was associated with increased intestinal permeability and mucosal inflammation, whereas gut dysbiosis and inflammation were absent in CFTR-KO-GC mice. Treatment with nonabsorbable antibiotics ameliorated intestinal permeability and liver inflammation in CFTR-KO mice. Faecal microbiota transfer from CFTR-KO to germ-free WT mice did not result in dysbiosis nor liver pathology, indicating that defective intestinal CFTR is required to maintain dysbiosis. Defective CFTR in the gut sustains a pathogenic microbiota, creates an inflammatory milieu, and alters intestinal permeability. These changes are necessary for the development of cholangiopathy. Restoring CFTR in the intestine or modulating the microbiota could be a promising strategy to prevent or attenuate liver disease. Severe cystic fibrosis-related liver disease (CFLD) affects 10% of patients with cystic fibrosis (CF) and contributes to increased morbidity and mortality. Treatment options remain limited due to a lack of understanding of disease pathophysiology. The cystic fibrosis transmembrane conductance regulator (CFTR) mediates Cl- and HCO3- secretion in the biliary epithelium and its defective function is thought to cause cholestasis and excessive inflammatory responses in CF. However, our study in Cftr-knockout mice demonstrates that microbial dysbiosis, combined with increased intestinal permeability caused by defective CFTR in the intestinal mucosa, acts as a necessary co-factor for the development of CFLD-like liver pathology in mice. These findings uncover a major role for the gut microbiota in CFLD pathogenesis and call for further investigation and clinical validation to develop targeted therapeutic strategies acting on the gut-liver axis in CF.

Imaging of Perirenal and Intrarenal Lymphatic Vessels: Anatomy-based Approach.

The lymphatic system (or lymphatics) consists of lymphoid organs and lymphatic vessels. Despite the numerous previously published studies describing conditions related to perirenal and intrarenal lymphoid organs in the radiology literature, the radiologic findings of conditions related to intrarenal and perirenal lymphatic vessels have been scarcely reported. In the renal cortex, interlobular lymphatic capillaries do not have valves; therefore, lymph can travel along the primary route toward the hilum, as well as toward the capsular lymphatic plexus. These two lymphatic pathways can be opacified by contrast medium via pyelolymphatic backflow at CT urography, which reflects urinary contrast agent leakage into perirenal lymphatic vessels via forniceal rupture. Pyelolymphatic backflow toward the renal hilum should be distinguished from urinary leakage due to urinary injury. Delayed subcapsular contrast material retention via pyelolymphatic backflow, appearing as hyperattenuating subcapsular foci on CT images, mimics other subcapsular cystic diseases. In contrast to renal parapelvic cysts originating from the renal parenchyma, renal peripelvic cysts are known to be of lymphatic origin. Congenital renal lymphangiectasia is mainly seen in children and assessed and followed up at imaging. Several lymphatic conditions, including lymphatic leakage as an early complication and acquired renal lymphangiectasia as a late complication, are sometimes identified at imaging follow-up of kidney transplant. Lymphangiographic contrast material accumulation in the renal hilar lymphatic vessels is characteristic of chylo-urinary fistula. Chyluria appears as a fat-layering fluid-fluid level in the urinary bladder or upper urinary tract. Recognition of the anatomic pathway of tumor spread via lymphatic vessels at imaging is of clinical importance for accurate management at oncologic imaging. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

A cystic and bullous lung disease associated with a PIK3CA-related overgrowth syndrome.

A bullous and cystic lung disease diagnosed in the context of a clinically and genetically diagnosed PROS https://bit.ly/48GnoJy.

Ayurvedic Concept in Understanding and Management of PCOD

PCOD stands for poly cystic ovarian disease is the most challenging edocrinological disease affecting 27% of women in today’s era. Also known as infertility queen, is the first and foremost cause of infertility. There becomes hormonal imbalance in the body targetting different organs and manifesting different symptoms. To balance the disturbed hormones, targeting organs and cure the symptoms manifested becomes a challenging task. Ayurveda describes concept of PCOD under yoni vyapad, artava vyapad and jataharinis. The treatment protocol includes diet, activities, medicine, panchakarma and yogasanas. This holistic approach as mentioned in ayurveda can reverse PCOD to normal stage .

Analytical Validation of Loss of Heterozygosity and Mutation Detection in Pancreatic Fine-Needle Aspirates by Capillary Electrophoresis and Sanger Sequencing.

Pancreatic cystic disease, including duct dilation, represents precursor states towards the development of pancreatic cancer, a form of malignancy with relatively low incidence but high mortality. While most of these cysts (>85%) are benign, the remainder can progress over time, leading to malignant transformation, invasion, and metastasis. Cytologic diagnosis is challenging, limited by the paucity or complete absence of cells representative of cystic lesions and fibrosis. Molecular analysis of fluids collected from endoscopic-guided fine-needle aspiration of pancreatic cysts and dilated duct lesions can be used to evaluate the risk of progression to malignancy. The basis for the enhanced diagnostic utility of molecular approaches is the ability to interrogate cell-free nucleic acid of the cyst/duct and/or extracellular fluid. The allelic imbalances at tumor suppressor loci and the selective oncogenic drivers are used clinically to help differentiate benign stable pancreatic cysts from those progressing toward high-grade dysplasia. Methods are discussed and used to determine the efficacy for diagnostic implementation. Here, we report the analytical validation of methods to detect causally associated molecular changes integral to the pathogenesis of pancreatic cancer from pancreatic cyst fluids.