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Liver disease, hemolytic anemia

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https://www.readbyqxmd.com/read/29766398/hepatosplenic-t-cell-lymphoma-a-unifying-entity-in-a-patient-with-hemolytic-anemia-massive-splenomegaly-and-liver-dysfunction
#1
Marianna Mavilia, Agnes McAuliffe, Safina Hafeez, Haleh Vaziri
Hepatosplenic T cell lymphoma (HSTCL) is a rare subtype of peripheral non-Hodgkin lymphoma, of which 20% of cases are associated with chronic immunosuppression. It often occurs in patients with inflammatory bowel disease treated with immunomodulating medications such a thiopurines or TNF-alpha inhibitors. Cytopenias are commonly seen but autoimmune hemolytic anemia (AIHA) is rare. Here we present a young male with longstanding ulcerative colitis on chronic azathioprine, exhibiting several rare features of HSTCL...
May 15, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29668551/fetal-onset-congenital-dyserythropoietic-anemia-type-1-due-to-a-novel-mutation-with-severe-iron-overload-and-severe-cholestatic-liver-disease
#2
Hui-Lin Chin, Le Ye Lee, Pei Lin Koh
We report a rare case of severe congenital dyserythropoietic anemia type 1 with fetal onset. Our patient presented with fetal hydrops from 19 weeks of gestation, requiring multiple intrauterine transfusions. At birth, she had severe hemolytic anemia with severe jaundice, and was subsequently transfusion dependent. She eventually developed severe iron overload and fulminant liver failure before her demise at 5 months of age. Genetic testing revealed a novel mutation in CDAN1.
April 17, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29473172/ceftriaxone-induced-drug-reaction-mimicking-acute-splenic-sequestration-crisis-in-a-child-with-hemoglobin-sc-disease
#3
Nancy L Van Buren, Jed B Gorlin, Robyn C Reed, Jerome L Gottschall, Stephen C Nelson
BACKGROUND: Acute splenic sequestration crisis is a complication of sickle cell disease (SCD) occurring when intrasplenic red blood cell (RBC) sickling prevents blood from leaving the spleen, causing acute splenic enlargement. Although typically seen in young children, it has been reported in older children with hemoglobin (Hb)SC disease, eventually resulting in functional asplenia. Ceftriaxone is a frequently used antibiotic of choice for children with SCD, because of its efficacy against invasive pneumococcal disease...
April 2018: Transfusion
https://www.readbyqxmd.com/read/29395171/de-novo-allergy-and-immune-mediated-disorders-following-solid-organ-transplantation-prevalence-natural-history-and-risk-factors
#4
Nufar Marcus, Achiya Z Amir, Eyal Grunebaum, Anne Dipchand, Diane Hebert, Vicky L Ng, Thomas Walters, Yaron Avitzur
OBJECTIVES: To describe the prevalence, natural course, outcome, and risk factors of post-transplant de novo allergy and autoimmunity. STUDY DESIGN: A cross-sectional, cohort study of all children (<18 years) who underwent a solid-organ transplantation, between 2000 and 2012, in a single transplant center, with a follow-up period of 6 months or more post-transplant and without history of allergy or immune-mediated disorder pretransplant. RESULTS: A total of 626 eligible patients were screened, and 273 patients (160 males; 59%) met the inclusion criteria; this included 111 liver, 103 heart, 52 kidney, and 7 multivisceral recipients...
January 22, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29394216/should-giant-cell-hepatitis-with-autoimmune-hemolytic-anemia-be-considered-a-pediatric-autoimmune-liver-disease
#5
Silvia Nastasio, Marco Sciveres, Giuseppe Maggiore
No abstract text is available yet for this article.
May 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29381966/hemolytic-anemia-in-alcoholic-liver-disease-zieve-syndrome-a-case-report-and-literature-review
#6
Miao-Xia Liu, Xiao-Yu Wen, Ying-Kit Leung, Yi-Jie Zheng, Mei-Shan Jin, Qing-Long Jin, Jun-Qi Niu
RATIONALE: Zieve syndrome, a rarely reported disease resulting from alcohol abuse, consists of a triad of symptoms: hemolytic anemia, cholestatic jaundice, and transient hyperlipidemia. It is largely under-recognized and under-reported, possibly because of unawareness of the condition by physicians. Here, we report a case of Zieve syndrome managed at the Jilin University First Bethune Hospital. PATIENT CONCERNS: A 30-year-old Chinese woman presented with a 4-month history of fatigue, yellowish discoloration of the eyes, and tea-colored urine...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29357939/inherited-thrombotic-thrombocytopenic-purpura-mimicking-immune-thrombocytopenic-purpura-during-pregnancy-a-case-report
#7
Valter Romão de Souza, Ana Beatriz Cavalcante de Oliveira, Ana Maria Vanderlei, Amanda Queiroz da Mota Silveira Aroucha, Bruna Pontes Duarte, Aureli Nunes Machado, Lívia Netto Chaer, Cláudia Wanderley de Barros Correia, Maria da Conceição de Barros Correia, Manuela Freire Hazin Costa
BACKGROUND: Thrombotic thrombocytopenic purpura is a very rare hereditary blood deficiency disorder of ADAMTS13 (von Willebrand factor-cleaving protease) and a life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. The deficiency in ADAMTS13 metalloprotease, which cleaves the von Willebrand factor, may be congenital or acquired. The congenital form is caused by inherited mutations in the ADAMTS13 gene. The diagnosis is challenging due to the nonspecific signs and symptoms, as well as the rarity of the disease...
January 22, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29331477/atypical-presentation-of-pregnancy-related-hemolytic-uremic-syndrome
#8
Salim Baghli, Catherine Abendroth, Umar Farooq, Jennifer A Schaub
The cause of acute kidney injury during pregnancy and in the postpartum period can be particularly challenging to diagnose, especially when it is necessary to differentiate among preeclampsia; eclampsia; hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome; and thrombotic microangiopathies (TMAs). All these disease entities can present with kidney failure, microangiopathic hemolytic anemia, and thrombocytopenia. We present a teaching case of atypical hemolytic uremic syndrome in the postpartum period in a young woman who was found to have mutations of uncertain clinical significance in the complement cascade, including in C3, CFH, and CFI...
January 10, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29325287/-the-diagnostic-value-and-limits-of-diagnostic-parameters-for-wilson-s-disease
#9
X Yang
Wilson disease (WD) is a rare and treatable genetic disorder. This paper describes the new advances and author's long-term experiences in the diagnosis of WD. The characteristics in clinical and routine tests are: the age of presentation can be quite broad, the WD could not be excluded based on age only; the patients usually have mild digestive symptoms but obvious chronic liver disease signs; liver function tests may reveal normal or a mild elevation in bilirubin, ALT and AST, but quite abnormal in serum albumin and prothrombin time in most patients; Coombs-negative hemolytic anemia, normal or markedly subnormal serum alkaline phosphatase (typically < 40 IU/L) are useful for the diagnosis of fulminant WD...
December 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29277896/c5ar-activation-in-the-absence-of-c5a-a-new-disease-mechanism-of-autoimmune-hemolytic-anemia-in-mice
#10
Shahzad N Syed, Eduard Rau, Mareen Ziegelmann, Georgios Sogkas, Bernhard Brüne, Reinhold E Schmidt
IgG Fc receptors (FcγRs) and the C5a anaphylatoxin receptor (C5aR) were identified as key regulators of type II autoimmune injury in mice. However, and with respect to C5aR, the relative importance of C5a for IgG autoantibody-induced cellular destruction remained unclear. Using an experimental model of autoimmune hemolytic anemia (AIHA), we here report marked differences in the development of AIHA between mice lacking C5aR and C5-deficient (Hc0 ) strain, indicating a limited role of C5 in this type of C5aR-regulated disease...
December 25, 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/29245301/copper-associated-hepatitis-in-a-patient-with-chronic-myeloid-leukemia-following-hematopoietic-stem-cell-transplantation-a-case-report
#11
Ching-Fen Lee, Chi-Hua Chen, Yu-Chuan Wen, Tsung-Yen Chang, Ming-Wei Lai, Tang-Her Jaing
RATIONALE: We report a complicated case of cholestatic hepatitis with suspected autoimmune hemolytic anemia (AIHA) and copper toxicity syndrome after HSCT and donor lymphocyte infusion (DLI). PATIENT CONCERNS: A 19-year-1-month-old girl presented with a history of CML. She underwent matched unrelated donor HSCT and donor lymphocyte infusion subsequently. Three months later, yellowish discoloration of the skin was found, which was accompanied by progressive itchy skin, easy fatigability, insomnia, and dark urine output...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29200157/an-infant-with-unusually-high-unconjugated-hyperbilirubinemia-due-to-coexistence-of-hereditary-spherocytosis-and-gilbert-syndrome
#12
Ivona Butorac Ahel, Kristina Baraba Dekanic, Goran Palcevski, Jelena Roganovic
Hereditary spherocytosis is the most frequent congenital hemolytic anemia and is characterized with variable degree of anemia, jaundice, and splenomegaly. In the case of severe hyperbilirubinemia out of proportion with hemolysis, other causes of hyperbilirubinemia must be considered. Gilbert syndrome (GS) is an autosomal dominant disorder characterized with intermittent hyperbilirubinemia without any other sign and symptom of liver disease as a result of reduced activity of uridine diphosphate-glucuronyl transferase 1A1...
March 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29068997/atypical-hemolytic-uremic-syndrome-induced-by-cblc-subtype-of-methylmalonic-academia-a-case-report-and-literature-review
#13
REVIEW
Minguang Chen, Jieqiu Zhuang, JianHuan Yang, Dexuan Wang, Qing Yang
RATIONALE: Methylmalonic acidemia (MMA) is a common organic acidemia, mainly due to methylmalonyl-CoA mutase (MCM) or its coenzyme cobalamin (VitB12) metabolic disorders. Cobalamin C (CblC) type is the most frequent inborn error of cobalamin metabolism; it can develop symptoms in childhood and often combine multisystem damage, which leads to methylmalonic acid, propionic acid, methyl citrate, and other metabolites abnormal accumulation, causing nerve, liver, kidney, bone marrow, and other organ damage...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28982343/study-of-glucose-6-phosphate-dehydrogenase-deficiency-5-years-retrospective-egyptian-study
#14
Adel A Hagag, Ibrahim M Badraia, Mohamed S Elfarargy, Mohamed M Abd Elmageed, Ehab A Abo-Ali
BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency worldwide that causes a spectrum of diseases including neonatal hyperbilirubinemia, acute and chronic hemolysis after exposure to oxidative stress. AIM OF THE WORK: This five years retrospective study was carried out to study the demographic, clinical and laboratory data of 1000 patients with G6PD deficiency anemia registered in Hematology Unit, Pediatric Department, Tanta University Hospital...
February 13, 2018: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28878866/association-of-autoimmune-hepatitis-type-1-in-a-child-with-evans-syndrome
#15
Chaowapong Jarasvaraparn, Hamayun Imran, Abdul Siddiqui, Felicia Wilson, David A Gremse
Autoimmune hepatitis (AIH) is a progressive liver disease that is often associated with extrahepatic autoimmune disorders. Evans syndrome (ES) is a rare autoimmune disorder, which is characterized by immune thrombocytopenia and autoimmune hemolytic anemia. Association of AIH with ES is rare, especially in children. We report a 3-year-old female with a past medical history of ES who presented with jaundice and significant transaminitis due to AIH type 1. She required multiple treatments with steroids as well as azathioprine, intravenous immunoglobulin and a course of rituximab...
August 18, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28721327/two-catastrophes-in-one-patient-drug-reaction-with-eosinophilia-and-systemic-symptoms-and-toxic-shock-syndrome
#16
Moayed Ibrahim, Diana L Nunley
A 70-year-old, immunocompromised patient presented to the emergency room (ER) five weeks after she was started on clopidogrel. She complained of skin eruption, mouth ulcers, fatigue, and myalgia over the past two weeks. Labs showed severe hyponatremia, acute kidney injury, rhabdomyolysis, hyperkalemia, and elevated liver enzymes. She was treated with steroids and discharged after her condition improved. However, a month later, she returned to the ER, complaining of nausea, vomiting, diarrhea, dizziness, chills, and shortness of breath over the past two days...
June 15, 2017: Curēus
https://www.readbyqxmd.com/read/28700492/an-unusual-association-between-hemophagocytic-lymphohistiocytosis-mixed-connective-tissue-disease-and-autoimmune-hemolytic-anemia-a-case-report
#17
Amar H Kelkar, Anushi A Shah, Sherri L Yong, Zohair Ahmed
RATIONALE: In the adult patient, hemophagocytic lymphohistiocytosis (HLH) is uncommon and frequently difficult to diagnose due to its nonspecific presentation and numerous complications. PATIENT CONCERNS: Herein, we present the case of a 25-year-old female who initially presented for evaluation of persistent fevers and fatigue. She was found to have splenomegaly, generalized lymphadenopathy, pancytopenia, and acute hepatic failure. DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Her course was further complicated by the development of nephrotic syndrome and autoimmune hemolytic anemia (AIHA)...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28588179/childhood-wilson-disease-bangladesh-perspective
#18
M Rukunuzzaman, A B Karim, M Nurullah, F Sultana, M W Mazumder, M A Rahman, S B Billah, F Begum, M Oliullah
Wilson's disease (WD) is an autosomal recessive disorder affecting copper metabolism causing copper induced damage to various organs. In children liver is commonly involved. Central nervous system, eyes, RBC, kidneys, brain and bones may also be affected. Aim of the study is to evaluate clinical & laboratory profile of Wilson's disease in children. This cross sectional descriptive study was conducted at the department of Paediatric Gastroenterology and Nutrition, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, from January 2011 to December, 2013...
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28324773/haptoglobin-is-frequently-low-in-patients-with-myelofibrosis-clinical-relevance
#19
Paolo Strati, Lucia Masarova, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Srdan Verstovsek
A recent study, showing the absence of paroxysmal nocturnal hemoglobinuria clones in myelofibrosis, has reopened the debate around the role of decreased haptoglobin in this disease. We present here a large prospective analysis of the clinical significance of low haptoglobin in 152 patients with myelofibrosis. Low haptoglobin (<32mg/dL) was observed in 50 patients (33%). Decreased haptoglobin did not associate with low hemoglobin levels, positive Coombs test or abnormal liver function tests, suggesting it is not result of autoimmune hemolytic anemia or liver cirrhosis...
June 2017: Leukemia Research
https://www.readbyqxmd.com/read/28225953/drug-reaction-with-eosinophilia-and-systemic-symptoms-dress-and-its-relation-with-autoimmunity-in-a-reference-center-in-mexico
#20
Juan Manuel Ruiz Matta, Silvia Méndez Flores, Judith Domínguez Cherit
BACKGROUND: Drug reaction with eosinophilia and systemic symptoms is a severe adverse drug reaction, with a reported mortality of 10%. Long-term outcomes involve organic failure and autoimmune diseases in some populations. OBJECTIVE: To evaluate the clinical prognosis of patients with drug reaction with eosinophilia and systemic symptoms. METHODS: We conducted a retrospective review at a referral hospital in Mexico City in a period of 22 years (1992-2013), looking up for records with diagnosis of DRESS according to RegiSCAR criteria...
January 2017: Anais Brasileiros de Dermatologia
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