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  • ISSN: 2576-0092
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    Volume 2, Issue 1
    Original Research Article
    James E Squires*, Daniel Wallihan, Kelly Bradley, Suraj Serai, Mantosh Rattan, Alexander Miethke
    Objective: The liver is a complex, multifunctional organ involved in a variety of critical processes. Accurate determination of liver function in children is difficult and current biomarkers often fail to truly assess functional capacity. Advances in magnetic resonance imaging (MRI) with hepatocyte-specific, gadolinium-based agents have enabled improved liver imaging. Since a functional hepatocyte is essential for timely elimination of these agents, hepatic retention may correlate with impaired function. We aimed to determine the utility of quantitative measures of liver enhancement with contrast-enhanced MRI as a biomarker to serve as a global indicator of whole organ function in children and young adult patients with primarily pediatric pathology.
    Methods: We performed a single-center, retrospective review of consecutive MRI examinations using gadoxetate disodium between 9/1/2010 and 9/1/2014. After exclusion criteria, 64 patient scans were analyzed and grouped according to presence (n=45) or absence (n=19) of liver disease. Quantitative enhancement measurements were performed comparing the signal intensity of the liver on the precontrast images relative to the 20 minute delayed hepatocyte phase. Specific measurements included relative liver enhancement (RLE) as well as ratios of liver enhancement compared to spleen (LSR), paraspinal muscle (LMR), and aorta (LAR).
    Results: Mean hepatic enhancement ratios significantly differed between patients with chronic liver disease and controls without parenchymal disease (LSR p<0.0001, LMR p<0.0001, RLE p<0.01). LSR demonstrated the greatest diagnostic performance at a cutoff value of 1.65 (AUC 0.9, sensitivity 89%, specificity 84%). Furthermore, LSR ratios differentiated liver disease subpopulations from individuals without liver disease. Finally, enhancement ratios differentiated patients with normal and abnormal MELD/PELD scores.
    Conclusion: Liver enhancement measurements on MR examinations using gadoxetate disodium in the hepatocyte phase can be used as a biomarker for identifying children with liver disease and quantifying the degree of dysfunction.
    Case Report
    Sravanthi Nandavaram*, Bisma Alam, Ioana Amzuta
    Pulmonary vascular malformation is an unusual cause of hemoptysis. These abnormal vascular communications between pulmonary circulations or between pulmonary and systemic circulations can result in significant dyspnea, hemoptysis and hypoxemia. Pulmonary vascular malformations can take various forms. Here we present a case of an abnormal collection of thrombosed arteries and veins, without the classic features of pulmonary arterio-venous malformation and presented as a focal opacity on imaging, that can be easily confused as air space process or lung consolidation. Identification and treatment of these abnormal vascular communications is vital as they might result in life threatening hemoptysis.
    Case Report
    Mohsen El Kossi*
    Caroli’s disease is a hereditary disorder invariably associated with biochemical changes characteristic of cholestasis and/or chronic kidney disease (CKD). Two cases of Caroli’s disease with CKD presented with hypomagnesemia. Urinary fractional excretion of magnesium was inappropriately high. Magnesium mal-absorption and inappropriate urinary wasting are potential explanations of hypomagnesemia.
    These case reports raise physician awareness about hypomagnesemia as one of the potential biochemical abnormalities that complicates the rare disorder of Caroli’s disease and the possible appropriate course of treatment. They also attract investigators’ interest for more research to explore the exact cause of this abnormality in this condition.
    Case Report
    Francesca Dini*, Cristina Tuoni, Ilaria Vannozzi, Benedetta Toschi, Elisabetta Alberti, Margherita Nardi, Veronica Bertini, Angelo Valetto, Matteo Giampietri, Marco Vuerich, Massimiliano Ciantelli, Antonio Boldrini, Paolo Ghirri
    Congenital leukemia is a rare disease with particular biological and clinical characteristics, which differs from those of older children. Its prognosis is generally poor. Its clinical manifestation may vary (hyperleukocytosis, thrombocytopenia, organomegaly) and some patients can develop cutaneous infiltration by leukemic cells (leukemia cutis). We describe a dysmorphic patient with thrombocytopenia hiding a congenital leukemia with fatal outcome. At birth he presented cutis laxa, multiple dysmorphic, thrombocytopenia and hepatosplenomegaly, initially orienting towards the diagnosis of a syndrome. Afterwards, pancytopenia and coagulopathy led to the diagnosis of congenital leukemia. His clinical features didn’t fit with any of the syndromes described in literature as associated with an increased risk of leukemia (i.e. Down syndrome, Fanconi’s anemia). This suggests a possible new association between a severe neonatal leukemia cutis and a dysmorphic syndrome characterized by cutis laxa (i.e. TALDO deficiency?).

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