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Journal of Cystic Fibrosis : Official... Jun 2024To study the prevalence of cystic fibrosis related liver disease (CFLD) as defined by ultrasound (US) and describe difference in clinical and radiological features in...
AIMS
To study the prevalence of cystic fibrosis related liver disease (CFLD) as defined by ultrasound (US) and describe difference in clinical and radiological features in those with CFLD and those without CFLD (nCFLD); with and without portal hypertension (PHT and nPHT).
METHODS
Children with CF (CwCF) from our clinic who had regular screening liver US from 3 years of age were included. Liver parenchyma findings were classified into normal, homogeneous, heterogeneous and nodular. For our study, we defined PHT as US evidence of splenomegaly and/or ascites, abnormal portal flow, varices, ligamentum teres recanalization if present. Demographic, clinical, nutritional and lung function between the two groups-CFLD/nCFLD; and subgroups- PHT and nPHT were compared. Gamma glutamyl transferase (GGT)/ platelet ratio (GPR) as a marker of fibrosis was measured.
RESULTS
From 227 CwCF,40 (17 %) were excluded (below the age of 3 years or alternative cause of liver disease). Of the remaining 187, 107 (57 %) had a normal US, 80 (43 %) had CFLD; 25 (13.4 %) had PHT. There was no significant difference in demographics, BMI-z score, lung function, presence of gastrostomy or pancreatic insufficiency in CFLD vs nCFLD and PHT vs nPHT. CF related diabetes mellitus (CFRD) was significantly associated with CFLD vs nCFLD (P = 0.0086). GGT was higher and platelet count was lower in PHT vs nPHT (P = 0.0256 and P = 0.0001). Nodularity was strongly associated with an elevated GPR (P = 0.016). There was a strong association between nodularity on US and PHT (P = 0.0006).
CONCLUSION
Nodularity is a clear marker for advanced liver disease with higher scores for a non-invasive marker for fibrosis. There was no difference in nutrition and FEV1 between advanced liver disease and absent/ milder liver disease.
PubMed: 38942721
DOI: 10.1016/j.jcf.2024.06.008 -
Cureus May 2024A serous cystic tumor is a rare entity that has a benign course. Its imaging characteristics, such as the presence of multiple cysts with or without nodular enhancement,...
A serous cystic tumor is a rare entity that has a benign course. Its imaging characteristics, such as the presence of multiple cysts with or without nodular enhancement, can simulate other cystic or solid lesions of the pancreas. Identification of the enhancing scar with punctate calcifications on computed tomography (CT) or magnetic resonance imaging (MRI) may be a distinctive finding suggesting this diagnosis. Neuroendocrine tumors of the pancreas are a different and also rare entity. In images, they have early arterial enhancement. In MRI, they are hyperintense on T2 and hypointense on T1, with avid contrast enhancement. A case of a patient with two focal lesions in the pancreas is presented and the importance of integrating clinical findings, semiology in diagnostic images and, if applicable, the histopathological result for the optimal management of pancreatic tumors is illustrated, highlighting the crucial role of a radiologist in this process.
PubMed: 38933621
DOI: 10.7759/cureus.61159 -
Children (Basel, Switzerland) May 2024Pancreatic fluid collections (PFCs) are a well-known complication of pancreatitis. PFCs operative management includes percutaneous, endoscopic or surgical drainage. Even...
Pancreatic fluid collections (PFCs) are a well-known complication of pancreatitis. PFCs operative management includes percutaneous, endoscopic or surgical drainage. Even if in adult patients, endoscopic drainage is a well-established treatment, few data are available in pediatric setting. We report our single-center experience of EUS-guided cystogastrostomy and lumen-apposing metal stent (LAMS) positioning in children with PFCs; this, at the best of our knowledge, has never been reported before. All consecutive children with PFCs between April 2020 and November 2022 were enrolled in this retrospective study. PFCs were preoperatively evaluated with MRI or CT scan. All the procedures were performed under general anesthesia. A LAMS Hot-Axios 10 × 15 mm was placed in all patients. We evaluated technical feasibility and clinical outcomes, including complications and recurrence rates. Follow-up included clinical observation, blood tests and US. EUS-guided cystogastrostomy was performed in 3 children (2 males; median age 13.2 years). Median maximum cyst diameter was 14.7 cm (range 10-22 cm). Technical and clinical success rates were 100%. No intra or post-operative complications occurred. Our experience suggests that this can be considered a safe and feasible treatment of PCFs even in the pediatric population, as long as the procedure is performed by an expert Endoscopist in a pediatric tertiary-level Center.
PubMed: 38929223
DOI: 10.3390/children11060643 -
Pancreatico-renal fistula associated with pancreatic cysts caused by type 1 autoimmune pancreatitis.Clinical Journal of Gastroenterology Jun 2024To our best knowledge, the formation of a pancreatico-renal fistula and the presence of pancreatic fluid collection in the renal subcapsular space have not been reported...
To our best knowledge, the formation of a pancreatico-renal fistula and the presence of pancreatic fluid collection in the renal subcapsular space have not been reported as autoimmune pancreatitis (AIP) complications. We describe a case of a pancreatico-renal fistula associated with type 1 AIP. The patient presented with abdominal and back pain accompanied by pancreatic cystic lesions during an untreated course of AIP. The diagnosis of pancreatico-renal fistula was based on the presence of a left renal subcapsular fluid collection containing pancreatic amylase, disappearance of pancreatic cysts, and a defect in the partial anterior renal fascia observed on imaging studies. Treatment with steroids and percutaneous drainage resulted in improvement. Pancreatic pseudocysts can affect other organs owing to their digestive action. Similar symptoms may occur in patients with AIP.
PubMed: 38922496
DOI: 10.1007/s12328-024-02008-8 -
Surgical Case Reports Jun 2024A chronic expanding hematoma is an uncommon entity described as an organized blood collection that increases in size after the initial hemorrhagic event without...
BACKGROUND
A chronic expanding hematoma is an uncommon entity described as an organized blood collection that increases in size after the initial hemorrhagic event without histological neoplastic features. The standard treatment is complete resection. To our knowledge, this is the first report of a chronic expanding hematoma mimicking a pancreatic cystic tumor that has been successfully resected utilizing a laparoscopic approach.
CASE PRESENTATION
We report the case of a 32-year-old man with a 10-cm chronic expanding hematoma that was preoperatively diagnosed as a cystic pancreatic tumor. Dynamic computed tomography revealed a cyst at the inferior part of the uncinate process of the pancreas without contrast enhancement. His blood biochemical data were within normal limits. The operation initially utilized a laparoscopic approach; however, the procedure was converted to hand-assisted laparoscopic surgery due to capsule adherence to surrounding organs and finally, enucleation of the tumor was performed. Pathological findings revealed a chronic expanding hematoma in the retroperitoneal space.
CONCLUSION
Chronic expanding hematoma in the retroperitoneal space is so rare and sometimes adheres to the surrounding tissue. It is difficult to distinguish hematoma attaching pancreas and pancreatic cyst preoperatively. In rare cases such as this, hand-assisted laparoscopic surgery is a feasible, less invasive procedure for facilitating complete resection and preventing recurrence.
PubMed: 38918294
DOI: 10.1186/s40792-024-01957-z -
JCEM Case Reports Jun 2024Pheochromocytomas (PCCs) and/or paragangliomas (PGLs) are a challenge to diagnose during pregnancy because of elusive signs and testing difficulties. We report a...
Pheochromocytomas (PCCs) and/or paragangliomas (PGLs) are a challenge to diagnose during pregnancy because of elusive signs and testing difficulties. We report a 25-year-old woman with no pertinent medical history who presented to the hospital with hypertension, vision loss, and weakness and was initially diagnosed with preeclampsia. Imaging showed hemangioblastomas in the medulla and thoracic spine, pancreatic cysts, and a renal cyst. The endocrinology service was consulted for possible PCCs associated with von Hippel-Lindau disease (VHL). Serum and urine normetanephrine levels were elevated despite the lack of overt PCCs/PGLs seen on magnetic resonance imaging and magnetic resonance angiography. The patient was medically managed with doxazosin and then labetalol. Despite successful resection of the hemangioblastoma in the medulla, the patient suffered respiratory distress requiring tracheostomy and venous-venous extracorporeal membrane oxygenation (V-V ECMO) and fetal demise. After 3 months, the patient was discharged to rehabilitation. Follow-up genetics were heterozygous for VHL and Lynch syndrome. DOTATATE positron emission tomography/computed tomography scan showed a small hepatic focus of a maximum standard uptake value of 12.1. Altogether, this case illustrates the importance of prompt diagnosis and proper management of PCCs/PGLs during pregnancy and incorporating genetic information during surveillance to lower morbidity and mortality.
PubMed: 38911362
DOI: 10.1210/jcemcr/luae097 -
Revista de Gastroenterologia de Mexico... Jun 2024
PubMed: 38906757
DOI: 10.1016/j.rgmxen.2024.03.002 -
Pancreas Jun 2024Epidermoid cyst in an intra-pancreatic accessory spleen (ECIPAS) is an exceedingly rare pancreatic lesion that is always mistakenly suspected of malignancy...
INTRODUCTION
Epidermoid cyst in an intra-pancreatic accessory spleen (ECIPAS) is an exceedingly rare pancreatic lesion that is always mistakenly suspected of malignancy preoperatively.
CASE SUMMARY
A 25-year-old male incidentally found a giant mass in the left upper abdomen neighboring the hilum of the spleen. The patient denied any obvious discomfort. Except for a slightly elevated carbohydrate antigen 19-9 (CA19-9, 43.5 U/ml), no abnormal laboratory test results were found. Contrast-enhanced computed tomography (CT), conventional ultrasound (US), and magnetic resonance imaging (MRI) were performed. The patient received a laparoscopic distal pancreatectomy. The final pathology showed ECIPAS. The postoperative course was uneventful and no signs of recurrence during 2 years of follow-up.
DISCUSSION
For an incidental pancreatic cystic lesion (PCL), ECIPAS should be considered in the differential diagnosis. ECIPAS may mimic pancreatic cystadenoma. Imaging follow-up or surgical removal may be useful for the exclusion of malignant risks in ECIPAS.
PubMed: 38904669
DOI: 10.1097/MPA.0000000000002383 -
Cureus May 2024Hydatid disease is caused by the tapeworm and is a zoonosis that endemically affects certain geographic areas with a high prevalence in animal husbandry. Due to... (Review)
Review
Hydatid disease is caused by the tapeworm and is a zoonosis that endemically affects certain geographic areas with a high prevalence in animal husbandry. Due to globalization, the pathology can also be encountered beyond these preferred geographic areas. It predominantly affects the liver and lungs, with pancreatic localizations of hydatid cysts being rare and posing a challenge for differential diagnosis and surgical tactics. The present study aimed to provide a recent scoping of the literature on this type of localization, analyzing demographic data, therapeutic management, and postoperative outcomes. It was observed that females are more frequently affected in pancreatic hydatid localizations (p < 0.001), with the most common symptomatology represented by abdominal pain. The preferred localization was at the level of the pancreatic tail (32.5%), followed by cephalic localizations (25%). The preferred surgical approach was open surgery, with an observed preference for open surgery in specific localizations, such as the head, isthmus, and body of the pancreas (p < 0.001). Radical procedures are more commonly used than conservative ones (52.5% vs. 47.5%), and paradoxically, although less invasive, procedures such as inactivation and drainage are associated with more frequent complications (p = 0.03). This type of localization, due to the elements of local anatomical topography, requires adequate preparation in biliopancreatic surgery, considering that sometimes preoperative diagnosis is not oriented, and intraoperative records may require extensive interventions. Our research encompassed a thorough review of literature spanning the last decade using PubMed and Google Scholar databases, focusing specifically on cases involving primary hydatid cysts found within the pancreas. Thirty-three relevant articles were published between 2014 and 2024. In addition, we presented a unique case study that illustrates this uncommon occurrence.
PubMed: 38903307
DOI: 10.7759/cureus.60797 -
Clinical Proteomics Jun 2024Biomarkers play a crucial role in advancing precision medicine by enabling more targeted and individualized approaches to diagnosis and treatment. Various biofluids,...
Biomarkers play a crucial role in advancing precision medicine by enabling more targeted and individualized approaches to diagnosis and treatment. Various biofluids, including serum, plasma, cerebrospinal fluid (CSF), saliva, tears, pancreatic cyst fluids, and urine, have been identified as rich sources of potential for the early detection of disease biomarkers in conditions such as cancer, cardiovascular diseases, and neurodegenerative disorders. The analysis of plasma and serum in proteomics research encounters challenges due to their high complexity and the wide dynamic range of protein abundance. These factors impede the sensitivity, coverage, and precision of protein detection when employing mass spectrometry, a widely utilized technology in discovery proteomics. Conventional approaches such as Neat Plasma workflow are inefficient in accurately quantifying low-abundant proteins, including those associated with tissue leakage, immune response molecules, interleukins, cytokines, and interferons. Moreover, the manual nature of the workflow poses a significant hurdle in conducting large cohort studies. In this study, our focus is on comparing workflows for plasma proteomic profiling to establish a methodology that is not only sensitive and reproducible but also applicable for large cohort studies in biomarker discovery. Our investigation revealed that the Proteograph XT workflow outperforms other workflows in terms of plasma proteome depth, quantitative accuracy, and reproducibility while offering complete automation of sample preparation. Notably, Proteograph XT demonstrates versatility by applying it to various types of biofluids. Additionally, the proteins quantified widely cover secretory proteins in peripheral blood, and the pathway analysis enriched with relevant components such as interleukins, tissue necrosis factors, chemokines, and B and T cell receptors provides valuable insights. These proteins, often challenging to quantify in complex biological samples, hold potential as early detection markers for various diseases, thereby contributing to the improvement of patient care quality.
PubMed: 38902643
DOI: 10.1186/s12014-024-09497-2