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JOURNAL ARTICLE
Prabudh Goel, Veereshwar Bhatnagar, Vikash Srinivasaiahsetty Chennur
BACKGROUND AND OBJECTIVES: More than 20% of patients with extrahepatic portal vein obstruction (EHPVO) may be deemed as nonshuntable due to lack of a suitable vein. The role of "makeshift shunts" or "lesser shunts" assumes importance in such cases. In this report, the authors have shared their experience with the makeshift shunts in the management of portal hypertension in children with emphasis upon anatomic considerations, resolution of symptoms, outcomes after surgery, and shunt patency...
2024: Journal of Indian Association of Pediatric Surgeons
#2
REVIEW
Jordache Ellis, Tassos Grammatikopoulos, James Cook, Akash Deep
Respiratory manifestations of chronic liver disease have a profound impact on patient clinical outcomes. Certain conditions within paediatric liver disease have an associated respiratory pathology. This overlap between liver and respiratory manifestations can result in complex challenges when managing patients and requires clinicians to be able to recognise when referral to specialists is required. While liver transplantation is at the centre of treatment, it opens up further potential for respiratory complications...
March 2024: Breathe
#3
Subhasish Burman, Asish K Das, Aquila A Anwar, Abhijit Maji, Abhishek Khatua
This report describes the understudied co-occurrence of temporomandibular joint ankylosis (TMJA) and extrahepatic portal vein obstruction (EHPVO), exploring a shared pathway involving hypercoagulability. TMJA is an acquired pathology where joint surfaces fuse, causing restricted mouth opening and facial asymmetry. Globally, TMJA is prevalent among 1.5 to 5 patients/million, with a higher incidence in developing countries. While trauma and infections often cause TMJA, the pathogenesis remains unclear in many cases...
February 2024: Curēus
#4
JOURNAL ARTICLE
Qian Zheng, Haiyang Zhang
RATIONALE: Hepatic sinusoidal obstruction syndrome (HSOS), which includes hepatic stasis and portal hypertension, is a rare vascular disorder of the liver. It is often associated with hematopoietic stem cell transplantation. It is also possible to treat this disease using Chinese herbal medicines that contain pyrrolizidine alkaloids (PAs). This disease is extremely rare in children and poses a serious threat to their health. To our knowledge, this is the first case of HSOS in a child with PAs...
March 15, 2024: Medicine (Baltimore)
#5
Qusay Abdoh, Abdalaziz Darwish, Mohammad Alnees, Mahdi Awwad, Duha Najajra, Mai Alsadi, Maysa Alawneh
INTRODUCTION AND SIGNIFICANCE: Portal vein thrombosis (PVT) is not commonly observed in patients, particularly those who have gone through neonatal intensive care unit (NICU) stays and had umbilical catheters. Although PVT can potentially cause hypertension and gastrointestinal bleeding it is highly unusual for this condition to manifest during childhood. CASE PRESENTATION: The authors present a case of a 10-year-old child who developed portal hypertension, esophageal varices, and multiple thrombophilia associated mutations...
March 2024: Annals of Medicine and Surgery
#6
JOURNAL ARTICLE
Srinivas Srinidhi Vadlapudi, Anshu Srivastava, Nidhi Saini, Moinak Sen Sarma, Ujjal Poddar, Surender Kumar Yachha
BACKGROUND: Ascites in children is multifactorial and serum ascites albumin gradient (SAAG) ≥1.1 helps differentiate portal hypertension (PHTN) related from non-PHTN ascites. AIMS: We evaluated the aetiology and diagnostic accuracy of SAAG in children with ascites. METHODS: Children with ascites were retrospectively evaluated. Etiological diagnosis was based on clinical presentation and investigations. All cases with ascitic fluid analysis and a definite diagnosis were included for calculating the utility of SAAG...
February 29, 2024: Digestive and Liver Disease
#7
REVIEW
Chaowapong Jarasvaraparn, Christopher Hartley, Wikrom Karnsakul
Children represent only a small proportion of those infected with the hepatitis C virus (HCV) compared to adults. Nevertheless, a substantial number of children have chronic HCV infection and are at risk of complications including cirrhosis, portal hypertension, hepatic decompensation with hepatic encephalopathy, and hepatocellular carcinoma in adulthood. The overall prevalence of the HCV in children was estimated to be 0.87% worldwide. The HCV spreads through the blood. Children born to women with chronic hepatitis C should be evaluated and tested for HCV due to the known risk of infection...
February 16, 2024: Pathogens
#8
Kentaro Okunushi, Hironobu Kobayashi, Yuri Yoh, Masaya Kunimatsu, Tadashi Shiohama, Tomozumi Takatani, Hiromichi Hamada
We encountered a pediatric case of pulmonary hypertension triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. A 14-year-old girl was brought to the emergency department of our hospital with fever, respiratory distress, and impaired consciousness. She tested positive for SARS-CoV-2 upon a polymerase chain reaction examination and had prolonged hypoxemia without pneumonia. An echocardiography revealed elevated right ventricular pressure. She was diagnosed with pilocytic astrocytoma at the age of 10 years and underwent a resection of a pituitary tumor...
2024: Frontiers in Pediatrics
#9
Ying Liu, Ping Zhu, Jiajun Tian
Congenital hepatic fibrosis (CHF) is considered to be a rare autosomal recessive hereditary fibrocystic liver disease, mainly found in children. However, cases of adult CHF with autosomal dominant polycystic kidney disease (ADPKD) caused by PKD1 gene mutation are extremely rare. We report a 31-year-old female patient admitted for esophageal and gastric variceal bleeding. Physical examination revealed significant splenomegaly, biochemical tests showed a slight increase in liver enzymes, and a decrease in platelet count...
2024: Frontiers in Medicine
#10
Kritick Bhandari, Sarmendra Mishra, Kamana Sen, Prasnna Basnet, Pawan Kumar Shah, Manish Yadav
INTRODUCTION: Extrahepatic Portal Vein Obstruction is the most common cause of portal hypertension in children. However, it has a very low prevalence. Esophageal varices due to portal hypertension in children can lead to recurrent episodes of upper gastrointestinal bleeding, which can have a sinister outcome if timely diagnosis and treatment are not initiated. CASE PRESENTATION: A 7-year-old male child presents with recurrent episodes of upper gastrointestinal bleeding for 3 years...
February 8, 2024: International Journal of Surgery Case Reports
#11
JOURNAL ARTICLE
Md Wahiduzzaman Mazumder, Md Benzamin
BACKGROUND: Upper gastrointestinal bleeding (UGIB) is defined as bleeding that occurs proximal to the ligament of Treitz and can sometimes lead to potentially serious and life-threatening clinical situations in children. Globally, the cause of UGIB differs significantly depending on the geographic location, patient population and presence of comorbid conditions. AIM: To observe endoscopic findings of UGIB in children at a tertiary care center of Bangladesh. METHODS: This retrospective study was carried out in the department of Pediatric Gastroenterology and Nutrition of Bangabandhu Shiekh Mujib Medical University, a tertiary care hospital of Bangladesh, between January 2017 and January 2019...
January 16, 2024: World Journal of Gastrointestinal Endoscopy
#12
Ratna Acharya, Kiran Upadhyay
Autosomal recessive polycystic kidney disease (ARPKD) is often associated with hepatobiliary disease in the form of hepatic fibrosis and/or Caroli disease. Combined liver-kidney transplantation (CLKT) is a transplant modality of choice in children with both end-stage renal disease (ESRD) and severe hepatic disease. However, there is no consensus on whether children with ARPKD-associated ESRD without severe hepatic disease can be treated with isolated kidney transplantation (KT) without the need for CLKT. We retrospectively studied the efficacy of isolated KT in children with ARPKD without severe hepatic disease, and followed the course of hepatic disease post KT...
December 21, 2023: Clinics and Practice
#13
JOURNAL ARTICLE
Arghya Samanta, Anshu Srivastava, Rajnikant Yadav, Aditya Kapoor, Anindya Ghosh, Prabhakar Mishra, Moinak Sen Sarma, Ujjal Poddar
OBJECTIVES: Radiological intervention (RI) is the preferred treatment in children with Budd-Chiari syndrome (BCS). We studied the comparative long-term outcome of BCS children, with and without RI and utility of liver and splenic stiffness measurement (LSM, SSM) by 2-dimensional shear wave elastography (2D-SWE) in assessing response. METHODS: Sixty children (40 boys, median age 10.5 [6.5-15.25] years) with BCS (29 newly diagnosed, 31 follow-up) were evaluated. LSM and SSM by 2D-SWE and vascular patency were monitored pre- and postprocedure (≥ 6 months postprocedure) in those undergoing RI...
January 2024: Journal of Pediatric Gastroenterology and Nutrition
#14
JOURNAL ARTICLE
Hamza Hassan Khan, Stuart S Kaufman, Nada A Yazigi, Khalid M Khan
PURPOSE: Limited data exist regarding outcome and morbidity associated with portosystemic shunts in the pediatric transplant population. Our study assesses the outcomes of pediatric patients who underwent a portosystemic shunt procedure, both with and without liver transplantation (LT). METHODS: This study retrospectively reviewed the medical records of pediatric patients aged 0-19 years who underwent shunt placement between 2003 and 2017 at a tertiary care center...
January 2024: Pediatric Gastroenterology, Hepatology & Nutrition
#15
JOURNAL ARTICLE
A Wang, Z J Lu, X F Gu, J P Liu, C L Lu
Objective: To investigate the clinical and pathological features of congenital hepatic fibrosis (CHF). Methods: The clinical and pathological findings of 20 patients diagnosed with CHF from 2017 to 2023 were retrospectively analyzed. Results: Among the 20 patients, 8 were males and 12 were females with a median age of 21.5 years. Mostly patients were admitted to the hospital with cirrhosis, portal hypertension and upper gastrointestinal bleeding. Pathological features were diffuse fibrosis in the portal area, formation of fibrous septa of varying width, segmentation of the liver parenchyma, with hyperplasia of small bile ducts...
November 20, 2023: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
#16
JOURNAL ARTICLE
Chaowapong Jarasvaraparn, Jessica Thoe, Andrew Rodenbarger, Howard Masuoka, R Mark Payne, Larry Wayne Markham, Jean P Molleston
BACKGROUND: Fontan-associated liver disease (FALD) refers to structural and functional changes of the liver caused by the physiology of the Fontan palliation. Currently, liver biopsy is the gold standard to assess liver fibrosis of FALD. AIM: Investigate biomarkers correlating with severity of liver biopsy fibrosis in FALD. METHODS: A retrospective study of post-Fontan patients ≥ 10 years of age who underwent liver biopsy was conducted...
January 13, 2024: Digestive and Liver Disease
#17
REVIEW
F Y Tian, X Dong, X H Hou, R Y Yuan, Y W Pan, D Zhang
Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023...
January 2, 2024: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
#18
JOURNAL ARTICLE
Bettina E Hansen, Shannon M Vandriel, Pamela Vig, Will Garner, Douglas B Mogul, Kathleen M Loomes, David A Piccoli, Elizabeth B Rand, Irena Jankowska, Piotr Czubkowski, Dorota Gliwicz-Miedzińska, Emmanuel M Gonzales, Emmanuel Jacquemin, Jérôme Bouligand, Lorenzo D'Antiga, Emanuele Nicastro, Henrik Arnell, Björn Fischler, Étienne Sokal, Tanguy Demaret, Susan Siew, Michael Stormon, Saul J Karpen, Rene Romero, Noelle H Ebel, Jeffrey A Feinstein, Amin J Roberts, Helen M Evans, Shikha S Sundaram, Alexander Chaidez, Winita Hardikar, Sahana Shankar, Ryan T Fischer, Florence Lacaille, Dominique Debray, Henry C Lin, M Kyle Jensen, Catalina Jaramillo, Palaniswamy Karthikeyan, Giuseppe Indolfi, Henkjan J Verkade, Catherine Larson-Nath, Ruben E Quiros-Tejeira, Pamela L Valentino, Maria Rogalidou, Antal Dezsőfi, James E Squires, Kathleen Schwarz, Pier Luigi Calvo, Jesus Quintero Bernabeu, Andréanne N Zizzo, Gabriella Nebbia, Pinar Bulut, Ermelinda Santos-Silva, Rima Fawaz, Silvia Nastasio, Wikrom Karnsakul, María Legarda Tamara, Cristina Molera Busoms, Deirdre Kelly, Thomas Damgaard Sandahl, Carolina Jimenez-Rivera, Jesus M Banales, Quais Mujawar, Li-Ting Li, Huiyu She, Jian-She Wang, Kyung Mo Kim, Seak Hee Oh, Maria Camila Sanchez, Maria Lorena Cavalieri, Way Seah Lee, Christina Hajinicolaou, Chatmanee Lertudomphonwanit, Orith Waisbourd-Zinman, Cigdem Arikan, Seema Alam, Elisa Carvalho, Melina Melere, John Eshun, Zerrin Önal, Dev M Desai, Sabina Wiecek, Raquel Borges Pinto, Victorien M Wolters, Jennifer Garcia, Marisa Beretta, Nanda Kerkar, Jernej Brecelj, Nathalie Rock, Eberhard Lurz, Niviann Blondet, Uzma Shah, Richard J Thompson, Binita M Kamath
BACKGROUND AND AIMS: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is the first-approved pharmacologic therapy for cholestatic pruritus in ALGS. Since long-term placebo-controlled studies are not feasible or ethical in children with rare diseases, a novel approach was taken comparing 6-year outcomes from maralixibat trials with an aligned and harmonized natural history cohort from the Global ALagille Alliance (GALA) study...
December 25, 2023: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
#19
JOURNAL ARTICLE
Sittisak Honsawek, Nichaphat Bovornsethanant, Thamonwan Woraruthai, Paisarn Vejchapipat, Wanvisa Udomsinprasert, Yong Poovorawan
BACKGROUND: Biliary atresia (BA) is a severe congenital disorder with progressive obstructive cholangiopathy in young children. The inflammatory process has been recognized as one of the pathological mechanisms driving bile duct injury. Since interleukin-34 (IL-34) has been reportedly linked to several pathological liver disorders, including inflammation, the current study aimed to analyze circulating IL-34 and the association of circulating IL-34 with hepatic deterioration and clinical outcomes in post-Kasai BA children...
December 15, 2023: International Immunopharmacology
#20
JOURNAL ARTICLE
S R Margaryan, A Yu Razumovsky, Z B Mitupov, A I Gurevich, E A Titova
To date, side-to-side splenorenal shunt (SRS) and its analogues (splenosuprarenal shunts (SSRS)) are mainly used for portal hypertension. These are total portosystemic shunts characterized by total blood shunt from portal vein into inferior vena cava. The latter is fraught with a significant risk of complications such as pulmonary hypertension, decreased portal liver perfusion, liver failure and hepatic encephalopathy. Prevention of these complications is still an urgent problem in modern surgery. However, we proposed a new method of treatment, i...
2023: Khirurgiia
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