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Pediatric ascites

Anna K Ermarth, John Pohl, Brittany Esty, Jessica K Sempler, John C Carey, Molly A O'Gorman
We report the first observation of a patient with contgenital chylous ascites (CCA) and Ehlers-Danlos syndrome type VI due to primary lymphatic defect with additional vascular anomaly. CCA is a rare condition, and there is limited understanding of its pathophysiology and treatment options. We also review the patient's treatment course mitigated with octreotide and total parenteral nutritional support, as there are no current established guidelines for CCA. Early recognition of possible association with Ehlers-Danlos syndrome is important for quick intervention and successful management of pediatric patients...
August 2016: ACG Case Reports Journal
Joanna L Gendreau, Christine Knoll, Roberta H Adams, Leon L Su
Sinusoidal obstructive syndrome (SOS) is a potentially fatal form of hepatic injury following hematopoietic stem cell transplant (HSCT). Patients can develop liver dysfunction, portal hypertension, ascites, coagulopathies, and multi-system organ failure. The mortality rate of severe SOS has been reported as high as 98% by Day 100 post-transplant. Defibrotide, which is now approved for the treatment of SOS, has significantly decreased mortality. Defibrotide is a polynucleotide with pro-fibrinolytic, anti-ischemic, and anti-inflammatory activity...
January 20, 2017: Biology of Blood and Marrow Transplantation
Tarek Abdelazeem Sabra, Hideaki Okajima, Atsushi Yoshizawa, Tatsuya Okamoto, Takayuki Anazawa, Shintaro Ygi, Koichiro Hata, Kentaro Yasuchika, Kojiro Taura, Etsuro Hatano, Toshimi Kaido, Shinji Uemoto
PV reconstruction is an important aspect of LDLT, with post-transplant outcomes depending on PV reconstruction methods. However, it is unclear whether the preferential selection of these techniques is dependent on preoperative recipient characteristics. This retrospective study assessed whether preoperative recipient factors differed in pediatric patients who did and did not receive VGs for PV reconstruction. Of 113 pediatric patients who underwent LDLT from January 2010 to July 2015, 31 (27%) underwent PV reconstruction with VGs and the other 82 (73%) without VGs...
January 22, 2017: Pediatric Transplantation
Sriram Pothapregada, Banupriya Kamalakannan, Mahalaskhmy Thulasingham, Srinivasan Sampath
OBJECTIVE: To study the clinical profile and outcome of dengue fever in children at a tertiary care hospital in Puducherry. MATERIALS AND METHODS: All children (0-12 years of age) diagnosed and confirmed as dengue fever from August 2012 to January 2015 were reviewed retrospectively from hospital case records as per the revised World Health Organization guidelines for dengue fever. The diagnosis was confirmed by NS1 antigen-based ELISA test or dengue serology for IgM and IgG antibodies, and the data were analyzed using SPSS 16...
July 2016: Journal of Global Infectious Diseases
Bianca Karber, Lenore Omesi, Sunny Chang, Andrew Handel, Monica Hegedus, Echezona Maduekwe
Congenital ascites is rare, but when it occurs, urinary ascites secondary to posterior urethral valve obstruction is the most common, and tumors are the least. Among the tumors in the pediatric population, the central nervous system tumors are common, but spinal cord tumors are rare. We describe a very rare case of congenital malignant spinal cord glioma presenting as isolated congenital ascites secondary to neurogenic bladder. A female infant was diagnosed sonographically with isolated congenital ascites at 40 weeks' gestational age, with uneventful development prior to 40 weeks' gestational age...
2016: Case Reports in Pediatrics
Parisá Khodayar-Pardo, Andrés Peña Aldea, Ana Ramírez González, Adela Meseguer Carrascosa, Cristina Calabuig Bayo
Extrahepatic portal vein obstruction, although rare in children, is a significant cause of portal hypertension (PHT) leading to life-threatening gastrointestinal bleeding in the pediatric age group. PHT may also lead to other complications such as hyperesplenism, cholangyopathy, ascites, and even hepatopulmonary syndrome and portopulmonary hypertension that may require organ transplantation. Herein we report the case of an asymptomatic 11-month-old infant wherein a hepatomegaly and cavernous transformation of the portal vein was detected by liver ultrasound...
May 2016: Case Reports in Gastroenterology
Alvaro Lassaletta, Ana Guerreiro Stucklin, Vijay Ramaswamy, Michal Zapotocky, Tara McKeown, Cynthia Hawkins, Eric Bouffet, Uri Tabori
Infants with low-grade glioma (LGG) have a poor survival. BRAFV600E mutation has been identified in pediatric LGG; however, the use of BRAF inhibitors in infants has never been reported. A 2-month-old with V600E mutant hypothalamic/chiasmatic glioma progressed on chemotherapy resulting in profound visual loss, massive ascites, and diencephalic syndrome. Initiation of dabrafenib resulted in rapid and sustained disappearance of clinical symptoms and a profound sustained cytoreduction. BRAF inhibition was safely tolerated with dramatic clinicoradiological response, suggesting early targeted therapy is a viable option in infants with LGG...
November 2016: Pediatric Blood & Cancer
Arin L Madenci, Alyssa Stetson, Christopher B Weldon, Leslie E Lehmann
Hepatic VOD (veno-occlusive disease) is a serious complication of HSCT (hematopoietic stem cell transplantation) and has historically been associated with high mortality. This obstruction to hepatic flow often results in fluid collections in the peritoneal and pleural cavities. Catheter placement to drain ascites or pleural fluid may reduce intra-abdominal hypertension and/or improve respiratory parameters. The safety of these interventions among critically ill, immunocompromised children is unknown. Among 32 HSCT recipients (2000-2012) with severe VOD, we assessed the primary outcome of procedural complication from peritoneal drain placement...
August 2016: Pediatric Transplantation
Y Kawano, K Mizuta, Y Sanada, T Urahashi, Y Ihara, N Okada, N Yamada, H Sasanuma, Y Sakuma, N Taniai, H Yoshida, H Kawarasaki, Y Yasuda, E Uchida
INTRODUCTION: Although hepatic vein stenosis after liver transplantation is a rare complication, the complication rate of 1% to 6% is higher in pediatric living-donor liver transplantation than that in other liver transplantation cases. Diagnosis is very important because this complication can cause hepatic congestion that develops to liver cirrhosis, graft loss, and patient loss. However, this is unlikely in cases where there are no ascites or hypoalbuminemia. OBJECTIVES: Eleven of 167 patients who had undergone pediatric living-donor liver transplantation were identified in the outpatient clinic at Jichi Medical University as having suffered from hepatic vein stenosis, and were enrolled in the study...
May 2016: Transplantation Proceedings
Leyat Tal, Joseph R Angelo, Ayse Akcan-Arikan
Peritoneal dialysis (PD) is generally considered the preferred extracorporeal therapy for neonates with acute kidney injury (AKI). However, there are situations when PD is not suitable, such as in patients with previous abdominal surgery, hyperammonemia and significant ascites or anasarca. Additionally, with a need to start PD soon after catheter placement, there is increased risk of PD catheter leak and infection. Extracorporeal continuous renal replacement therapy (CRRT) is challenging in severely ill neonates as it requires obtaining adequately sized central venous access to accommodate adequate blood flow rates and also adaptation of a CRRT machine meant for older children and adults...
November 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Tarik Alhmoud, Joshua Anspach Hanson, Gulshan Parasher
BACKGROUND: Eosinophilic gastroenteritis (EOGE) is a rare idiopathic disease characterized by eosinophil-predominant inflammation of the stomach and/or intestines. Our aims are to determine the epidemiology, clinical features and outcomes of EOGE cases in a tertiary-care hospital. METHODS: Retrospective cohort study of patients with gastrointestinal eosinophilic infiltration from 2004 through 2014. All relevant specimens were reviewed by an expert pathologist. Significant eosinophilic infiltrate was defined as >25 eosinophils/HPF in the stomach or small intestine and >50 eosinophils/HPF in the colon...
September 2016: Digestive Diseases and Sciences
Fabao Hao, Hongjie Guo, Qianfu Luo, Chunbao Guo
BACKGROUND: Approximately 10% of patients with acute pancreatitis (AP) progress to chronic pancreatitis. Little is known about the factors that affect recurrence of pancreatitis after an initial episode. We retrospectively investigated patients with AP, focusing on their outcomes and the predictors for disease progression. METHODS: Between July 2003 and June 2015, we retrospectively enrolled first-time AP patients with medical records on disease etiology, severity (according to the Atlanta classifications), and recurrence of AP...
May 15, 2016: Journal of Surgical Research
B P Xu, H Wang, Y H Zhao, J Liu, Y Yao, X L Feng, K L Shen
OBJECTIVE: To investigate the clinical manifestations and molecular features of cystic fibrosis in Chinese children. METHOD: A retrospective analysis of two pediatric cystic fibrosis cases diagnosed by gene test in Beijing Children's Hospital, Capital Medical University from 2010 to 2015, and Chinese cystic fibrosis reported patients searched of"cystic fibrosis, Chinese"on Chinese databases (CNKI, Wanfang Data) and PubMed from 1975 to 2015.The clinical manifestations and molecular features were analyzed...
May 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
A Zamprakou, C Berg, B Strizek, A Müller, A Heydweiller, U Gembruch, A Geipel
BACKGROUND: Morgagni hernia presents a rare type of congenital diaphragmatic hernia (CDH, about 2-5 %) (Herman, J Perinatol 21:343-344, 2001), which is characterized by an anterior mainly right-sided defect of the diaphragm. Infrequently, this is combined with a herniation of the liver into the pericardial cavity (Aké, Prenat Diagn 11:719-724, 1991; Stevens, Pediatr Radiol 26:791-793, 1996). This may cause massive pericardial effusion and subsequently lung hypoplasia (Pober et al., Congenital diaphragmatic hernia overview, University of Washington, Seattle, 2015; Ikeda, J Perinat Med 30:336-340, 2002; Hara, J Obstet Gynaecol Res 33:561-565, 2007)...
November 2016: Archives of Gynecology and Obstetrics
Yucel Yankol, Mustafa Ertugrul, Turan Kanmaz, Nesimi Mecit, Ilhan Ocak, Ozlem Durmaz, Koray Acarli, Munci Kalayoglu
OBJECTIVES: Acute liver failure is a rapidly progressive and life-threatening disease in children, whose clinical features differ from those of adults. MATERIALS AND METHODS: This is a review of a single center's experience with pediatric acute liver failure in a region with insufficient deceased donor support. The study is a retrospective review and analysis of 22 pediatric patients with acute liver failure between January 2007 and May 2013. RESULTS: The cause of acute liver failure was indeterminate in 45...
October 2016: Experimental and Clinical Transplantation
Hongjie Guo, Fabao Hao, Chunbao Guo, Yang Yu
Background. Portal cavernoma (PC) is the most critical condition with risk or variceal hemorrhage in pediatric patients. We retrospectively investigated the patients with PC focusing on the predictors for recurrent variceal bleeding. Methods. Between July 2003 and June 2013, we retrospectively enrolled all consecutive patients admitted to our department with a diagnosis of PC without abdominal malignancy or liver cirrhosis. The primary endpoint of this observational study was recurrent variceal bleeding. Independent predictors of recurrent variceal bleeding were identified using the logistic regression model...
2016: Gastroenterology Research and Practice
Barina Aqil, Wei Xie, Reka Szigeti
Distinguishing reactive changes from neoplastic processes during lymphoid tissue evaluation is oftentimes difficult. Ancillary studies, such as flow cytometry, may aid the diagnosis by demonstrating monotypic or polytypic light chain expression on the B cells. The detection of immunoglobulin light chain restricted B cell population is considered a surrogate marker of clonality, which can be confirmed by molecular assays. In general, the presence of a monotypic B cell population in the ascitic fluid is considered lymphomatous involvement rather than a reactive condition...
2016: Annals of Clinical and Laboratory Science
J Panaiotova, D Mitev, V Atanasova, A Nikolov
The detection of isolated ascites is a rare ultrasound finding. A precise examination should be undertaken in all cases fetal ascites. This include following a systematic protocol for diagnosis. The prognosis of the newborn in case of prenatal diagnosed ascites, depends on the etiology of the ascites. This is a case report of 40 years old pregnant women, at 34 weeks of gestation. During routine ultrasound examination at 33 weeks of gestation fetal ascites, has been diagnosed. The aim of our team was to examine the etiology of the ascites...
2015: Akusherstvo i Ginekologii︠a︡
Moinak Sen Sarma, Anshu Srivastava, Surender Kumar Yachha, Ujjal Poddar, Amrita Mathias
OBJECTIVE: To analyze the presentation and predictors of outcome of children with galactosemia. METHODS: Analysis of clinical, laboratory, microbiological profile and outcome of patients fulfilling the diagnostic criteria: i) clinical setting; ii) reduced erythrocyte Gal-1-PUT enzyme activity; and iii) unequivocal response to lactose-free diet. RESULTS: 24 patients; median age of symptom onset and diagnosis: 10 (3-75) d and 55 (15-455) days, respectively...
January 2016: Indian Pediatrics
Marybelle Pe, Eitan Dickman, Mark Tessaro
A 13-year-old adolescent girl with chronic abdominal distention was referred to the pediatric emergency department after the outpatient workup suggested moderate ascites. Point-of-care ultrasonography performed by the emergency physicians ruled out ascites, instead demonstrating a well-circumscribed cystic mass subsequently identified as an ovarian mucinous cystadenoma.
February 2016: Pediatric Emergency Care
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