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Liver disease in children

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https://www.readbyqxmd.com/read/29159839/increased-renal-echogenicity-in-children-with-appendicitis
#1
Hila Tal, Daniel Ben-Mordechay, Aviva Ben-Shlush, Lisa Raviv-Zilka, Michalle Soudack
OBJECTIVES: Ultrasound (US) is an important modality for the detection of acute appendicitis in children but has limited sensitivity and specificity. Therefore, additional US findings may contribute to the diagnosis. In our experience, children with acute appendicitis often have increased renal cortical echogenicity on US imaging. The purpose of this study was to examine the association of increased renal cortical echogenicity with appendicitis. METHODS: This study included 240 consecutive pediatric patients with no renal or liver disease who underwent US examinations for suspected appendicitis between February 2014 and January 2016...
November 21, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/29152259/prevalence-of-malnutrition-and-risk-of-undernutrition-in-hospitalised-children-with-liver-disease
#2
Ronghua Yu, Yizhong Wang, Yongmei Xiao, Lili Mo, Aishu Liu, Dan Li, Ting Ge, Guangjun Yu, Ting Zhang
Nutritional status of 380 hospitalised children aged from 1 month to 5 years with liver disease was evaluated in a single paediatric centre. The total prevalence of stunting (height-for-age Z (HAZ) < -2), underweight (weight-for-age Z (WAZ) < -2) and wasting (weight-for-height Z < -2) was 9·8, 9·0 and 7·9 %, respectively. The overall nutritional risk (-2 ≤ Z < -1) of stunting, underweight and wasting was 11·8, 12·9 and 12·6 %. The prevalence of undernutrition was significantly higher in children with cholestasis than children without cholestasis (stunting, 17·5 %/4·4 %, P < 0·001, and underweight, 14·9 %/4·9 %, P < 0·001)...
2017: Journal of Nutritional Science
https://www.readbyqxmd.com/read/29150327/diagnostic-accuracy-of-point-shear-wave-elastography-in-the-detection-of-portal-hypertension-in-pediatric-patients
#3
M Burak Özkan, M C Bilgici, E Eren, G Caltepe
PURPOSE: The purpose of this study was to determine the usefulness of point shear wave elastography (p-SWE) of the liver and spleen for the detection of portal hypertension in pediatric patients. MATERIALS AND METHODS: The study consisted of 38 healthy children and 56 pediatric patients with biopsy-proven liver disease who underwent splenic and liver p-SWE. The diagnostic performance of p-SWE in detecting clinically significant portal hypertension was assessed using receiver operating characteristic (ROC) curves...
November 14, 2017: Diagnostic and Interventional Imaging
https://www.readbyqxmd.com/read/29149474/interventions-for-old-world-cutaneous-leishmaniasis
#4
REVIEW
Julio Heras-Mosteiro, Begoña Monge-Maillo, Mariona Pinart, Patricia Lopez Pereira, Emely Garcia-Carrasco, Pedro Campuzano Cuadrado, Ana Royuela, Irene Mendez Roman, Rogelio López-Vélez
BACKGROUND: Cutaneous leishmaniasis, caused by a parasitic infection, is considered one of the most serious skin diseases in many low- and middle-income countries. Old World cutaneous leishmaniasis (OWCL) is caused by species found in Africa, Asia, the Middle East, the Mediterranean, and India. The most commonly prescribed treatments are antimonials, but other drugs have been used with varying success. As OWCL tends to heal spontaneously, it is necessary to justify the use of systemic and topical treatments...
November 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29147139/insights-into-the-management-of-wilson-s-disease
#5
REVIEW
Mohmadshakil Kathawala, Gideon M Hirschfield
Wilson's disease is a rare, inherited autosomal recessive disease of copper metabolism, in which the causative gene, ATP7B, results in absent or reduced function of the ATP7B transporter important for biliary excretion of copper and incorporation of copper into caeruloplasmin. Affected patients accumulate excessive copper within the liver, brain and other tissues. A disease mainly of children, adolescents and young adults; clinical features vary from the asymptomatic state to chronic liver disease, acute liver failure, and neuropsychiatric manifestations...
November 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/29146214/-treatments-of-steroid-dependent-nephrotic-syndrome-in-children
#6
A Couderc, E Bérard, V Guigonis, I Vrillon, J Hogan, V Audard, V Baudouin, C Dossier, O Boyer
Primary nephrotic syndrome (NS) is the most common glomerular disease in children. It is characterized by massive proteinuria and hypoalbuminemia. It typically has a sudden onset and more than 70% of patients will experience at least one relapse. An immunological origin has long been postulated, although the precise molecular mechanisms underlying the disease remain debated. Steroids are the first-line therapy with cumulative dose and duration of initial treatment varying among countries. Steroid-sparing agents may be indicated in case of steroid-dependency or frequent relapses...
November 13, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29144045/the-triad-of-pruritus-xanthomas-and-cholestasis-two-cases-and-a-brief-review-of-the-literature
#7
Hadley J Pearson, Joy L Mosser, Stephanie K Jacks
When encountered in children, xanthomas are most commonly associated with a group of disorders known as familial hyperlipidemias. Aside from cosmetic concerns, these xanthomas are typically asymptomatic, but when generalized pruritus is a prominent associated symptom, clinicians should consider a different set of diagnoses that includes cholestasis of the liver. In this article we present two illustrative cases of children with cholestatic disease, pruritus, and xanthomas and discuss other disorders that may include this triad...
November 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/29141316/-a-clinical-analysis-of-micafungin-treatment-of-pulmonary-invasive-fungal-infection-in-pediatric-patients-with-acute-leukemia-or-post-hematopoietic-stem-cells-transplantation
#8
K Huang, K Y Qiu, L L Deng, J P Fang, Y Li, H X Guo, D H Zhou
Objective: To investigate the efficacy and safety of micafungin (MCF) for pulmonary invasive fungal disease (PIFD) in pediatric patients with acute leukemia or post hematopoietic stem cells transplantation. Method: Twenty-five neutropenic PIFD children with acute leukemia or post hematopoietic stem cells transplantation in Sun Yat-sen Memorial Hospital of Sun Yat-sen University were selected from January 2012 to June 2015, including 12 males and 13 females, age range 2-15 (average 6.2±2.0) years. There were 12 cases of acute leukemia (AL) after chemotherapy, 4 cases of acute leukemia (AL) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and 9 cases of β-thalassemia major after allo-HSCT...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29134448/hyperammonemia-associated-with-distal-renal-tubular-acidosis-or-urinary-tract-infection-a-systematic-review
#9
Caterina M Clericetti, Gregorio P Milani, Sebastiano A G Lava, Mario G Bianchetti, Giacomo D Simonetti, Olivier Giannini
BACKGROUND: Hyperammonemia usually results from an inborn error of metabolism or from an advanced liver disease. Individual case reports suggest that both distal renal tubular acidosis and urinary tract infection may also result in hyperammonemia. METHODS: A systematic review of the literature on hyperammonemia secondary to distal renal tubular acidosis and urinary tract infection was conducted. RESULTS: We identified 39 reports on distal renal tubular acidosis or urinary tract infections in association with hyperammonemia published between 1980 and 2017...
November 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29132978/intensive-care-in-severe-malaria-report-from-the-task-force-on-tropical-diseases-by-the-world-federation-of-societies-of-intensive-and-critical-care-medicine
#10
REVIEW
Dilip R Karnad, Mohd Basri Mat Nor, Guy A Richards, Tim Baker, Pravin Amin
Severe malaria is common in tropical countries in Africa, Asia, Oceania and South and Central America. It may also occur in travelers returning from endemic areas. Plasmodium falciparum accounts for most cases, although P vivax is increasingly found to cause severe malaria in Asia. Cerebral malaria is common in children in Africa, manifests as coma and seizures, and has a high morbidity and mortality. In other regions, adults may also develop cerebral malaria but neurological sequelae in survivors are rare...
November 8, 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/29131462/outcomes-of-liver-transplantation-in-pediatric-recipients-with-cardiovascular-disease
#11
Márcio Miranda Brito, Joao Seda Neto, Eduardo A Fonseca, Renata Pugliese, Vera B Danesi, Helry L Candido, Adriana Porta, Cristian V B Borges, Gilda Porta, Paulo Chapchap, Irene Kazue Miura
LT exerts considerable stress on the heart perioperatively. Limited data exist on impact of cardiovascular diseases on LT children. This study evaluated the outcomes of children with CVD who underwent LT and compared with pretransplant findings. From 518 LT recipients, 82 (15.8%) had CVD. Sixty patients were classified as low-risk adjustment for congenital heart surgery 1 (RACHS 1 and 2). Five patients were classified as RACHS ≥3. The most common echocardiographic finding in the CVD patients (25/82) was ASD...
November 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29130075/association-between-cytokines-and-liver-histology-in-children-with-nonalcoholic-fatty-liver-disease
#12
Emily R Perito, Veeral Ajmera, Nathan M Bass, Philip Rosenthal, Joel E Lavine, Jeffrey B Schwimmer, Katherine P Yates, Anna Mae Diehl, Jean P Molleston, Karen F Murray, Ann Scheimann, Ryan Gill, David Glidden, Bradley Aouizerat
Background: Reliable non-invasive markers to characterize inflammation, hepatocellular ballooning, and fibrosis in nonalcoholic fatty liver disease (NAFLD) are lacking. We investigated the relationship between plasma cytokine levels and features of NAFLD histology to gain insight into cellular pathways driving NASH and to identify potential non-invasive discriminators of NAFLD severity and pattern. Methods: Cytokines were measured from plasma obtained at enrollment in pediatric participants in NASH Clinical Research Network studies with liver biopsy-proven NAFLD...
September 2017: Hepatol Commun
https://www.readbyqxmd.com/read/29128761/inborn-errors-of-immunity-underlying-fungal-diseases-in-otherwise-healthy-individuals
#13
REVIEW
Juan Li, Donald C Vinh, Jean-Laurent Casanova, Anne Puel
It has been estimated that there are at least 1.5 million fungal species, mostly present in the environment, but only a few of these fungi cause human disease. Most fungal diseases are self-healing and benign, but some are chronic or life-threatening. Acquired and inherited defects of immunity, including breaches of mucocutaneous barriers and circulating leukocyte deficiencies, account for most severe modern-day mycoses. Other types of infection typically accompany these fungal infections. More rarely, severe fungal diseases can strike otherwise healthy individuals...
November 9, 2017: Current Opinion in Microbiology
https://www.readbyqxmd.com/read/29128061/the-genetics-of-pediatric-nonalcoholic-fatty-liver-disease
#14
REVIEW
Nidhi P Goyal, Jeffrey B Schwimmer
Nonalcoholic fatty liver disease (NAFLD) is the leading cause of chronic liver disease in children. Severe fibrosis and cirrhosis are potential consequences of pediatric NAFLD and can occur within a few years of diagnosis. Observations suggest that genetics may be a strong modifying factor in the presentation, severity, and natural history of the disease. There is increasing interest in determining at-risk populations based on genetics in the hope of finding genotypes that correlate to NAFLD phenotype. Ultimately, the hope is to be able to tailor therapeutics to genetic predispositions and decrease disease morbidity in children with NAFLD...
February 2018: Clinics in Liver Disease
https://www.readbyqxmd.com/read/29126168/endocrine-disruptors-and-developmental-origins-of-nonalcoholic-fatty-liver-disease
#15
Lindsey S Treviño, Tiffany A Katz
Nonalcoholic fatty liver disease (NAFLD) is a growing epidemic worldwide, particularly in countries that consume a Western diet, and can lead to life-threatening conditions such as cirrhosis and hepatocellular carcinoma (HCC). With increasing prevalence of NAFLD in both children and adults, an understanding of the factors that promote NAFLD development and progression is crucial. Environmental agents, including endocrine-disrupting chemicals (EDCs), that have been linked to other diseases may play a role in NAFLD development...
November 6, 2017: Endocrinology
https://www.readbyqxmd.com/read/29122851/guidelines-on-the-management-of-abnormal-liver-blood-tests
#16
Philip N Newsome, Rob Cramb, Suzanne M Davison, John F Dillon, Mark Foulerton, Edmund M Godfrey, Richard Hall, Ulrike Harrower, Mark Hudson, Andrew Langford, Anne Mackie, Robert Mitchell-Thain, Karen Sennett, Nicholas C Sheron, Julia Verne, Martine Walmsley, Andrew Yeoman
These updated guidelines on the management of abnormal liver blood tests have been commissioned by the Clinical Services and Standards Committee (CSSC) of the British Society of Gastroenterology (BSG) under the auspices of the liver section of the BSG. The original guidelines, which this document supersedes, were written in 2000 and have undergone extensive revision by members of the Guidelines Development Group (GDG). The GDG comprises representatives from patient/carer groups (British Liver Trust, Liver4life, PBC Foundation and PSC Support), elected members of the BSG liver section (including representatives from Scotland and Wales), British Association for the Study of the Liver (BASL), Specialist Advisory Committee in Clinical Biochemistry/Royal College of Pathology and Association for Clinical Biochemistry, British Society of Paediatric Gastroenterology, Hepatology and Nutrition (BSPGHAN), Public Health England (implementation and screening), Royal College of General Practice, British Society of Gastrointestinal and Abdominal Radiologists (BSGAR) and Society of Acute Medicine...
November 9, 2017: Gut
https://www.readbyqxmd.com/read/29122831/fifteen-minute-consultation-liver-disease-in-children
#17
Jake P Mann, Kathy Gallagher, Emer Fitzpatrick, Anil Dhawan
Liver disease in children can present in many ways from the frequently encountered prolonged neonatal jaundice to the comparatively rare acute liver failure. In this article, we will discuss 'red flags' of liver disease, the initial investigations required and when to refer to a specialist liver centre. Across all presentations, the degree of elevation of alanine aminotransferase or aspartate aminotransferase provides only little diagnostic information. Measurement of clotting is vital, and coagulopathy should be followed by a trial of intravenous vitamin K before being repeated...
November 9, 2017: Archives of Disease in Childhood. Education and Practice Edition
https://www.readbyqxmd.com/read/29122442/serum-fetuin-a-levels-in-obese-children-with-biopsy-proven-nonalcoholic-fatty-liver-disease
#18
V Pampanini, E Inzaghi, D Germani, A Alterio, A Puglianiello, A Alisi, V Nobili, S Cianfarani
BACKGROUND AND AIMS: Fetuin-A has been proposed as a marker of liver damage in adults with obesity-related NAFLD. The aim of this study was to test serum fetuin-A concentrations in obese children with NAFLD diagnosed either by ultrasonography or by liver biopsy and to determine its applicability as predictive tool in pediatric NAFLD. METHODS AND RESULTS: Metabolic parameters and fetuin-A levels were investigated in 81 obese children with NAFLD diagnosed by biopsy, 79 obese children with NAFLD defined by liver ultrasonography and 23 lean subjects...
October 3, 2017: Nutrition, Metabolism, and Cardiovascular Diseases: NMCD
https://www.readbyqxmd.com/read/29118811/obese-children-with-metabolic-syndrome-have-3-times-higher-risk-to-have-nonalcoholic-fatty-liver-disease-compared-with-those-without-metabolic-syndrome
#19
Dimitrios Papandreou, Mirey Karavetian, Zacharoula Karabouta, Eleni Andreou
Background: The aim of this study was to investigate the relationship between metabolic syndrome (MS) and nonalcoholic fatty liver disease (NAFLD) in obese children. One hundred and twenty-five subjects aged 11-12 years old participated in the study. Methods: Anthropometric and biochemical indices were measured, including lipid and liver profile, blood glucose, serum insulin, and liver ultrasound. Results: Forty-four children (58.6%) were found to have MS...
2017: International Journal of Endocrinology
https://www.readbyqxmd.com/read/29114140/analysis-of-hospital-records-of-children-with-hydatid-cyst-in-south-of-iran
#20
Anahita Sanaei Dashti, Mohammad Rahim Kadivar, Abdolvahab Alborzi, Esmaeel Sadeghi, Gholam Reza Pouladfar, Neda Bagherian, Naser Honar, Masoomeh Khalifeh
The clinical manifestations of hydatidosis are various and related to anatomic location. Defining frequent symptoms and signs of the disease is imperative for early management of it. The aim of this report was to analyse the clinical features of infected children with hydatid cysts located in different organs. In this study, medical charts of 57 children between 3 and 16 years of age with hydatid cyst admitted to Pediatric Wards of Nemazee Hospital were evaluated over a 12 year period (from 2003 to 2014, prospectively)...
December 2017: Journal of Parasitic Diseases: Official Organ of the Indian Society for Parasitology
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