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Hemolytic disease

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https://www.readbyqxmd.com/read/29226095/atypical-hemolytic-uremic-syndrome-due-to-complement-factor-i-mutation
#1
Abdullah H Almalki, Laila F Sadagah, Mohammed Qureshi, Hatim Maghrabi, Abdulrahman Algain, Ahmed Alsaeed
Atypical hemolytic-uremic syndrome (aHUS) is a rare disease of complement dysregulation leading to thrombotic microangiopathy (TMA). Renal involvement and progression to end-stage renal disease are common in untreated patients. We report a 52-year-old female patient who presented with severe acute kidney injury, microangiopathic hemolytic anemia, and thrombocytopenia. She was managed with steroid, plasma exchange, and dialysis. Kidney biopsy shows TMA and renal cortical necrosis. Genetic analysis reveals heterozygous complement factor I (CFI) mutation...
November 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/29222291/evolving-treatment-paradigms-in-sickle-cell-disease
#2
REVIEW
Ramasamy Jagadeeswaran, Angela Rivers
Sickle cell disease (SCD) is an inheritable hemoglobinopathy characterized by polymerization of hemoglobin S in red blood cells resulting in chronic hemolytic anemia, vaso-occlusive painful crisis, and multiorgan damage. In SCD, an increased reactive oxygen species (ROS) generation occurs both inside the red blood cells and inside the vascular lumen, which augment hemolysis and cellular adhesion. This review discusses the evolving body of literature on the role of ROS in the pathophysiology of SCD as well as some emerging therapeutic approaches to SCD with a focus on the reduction of ROS...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222288/cardiovascular-complications-in-patients-with-sickle-cell-disease
#3
REVIEW
Mark T Gladwin
Sickle cell disease (SCD) is an autosomal recessive disease in which homozygosity for a single point mutation in the gene encoding the β-globin chain produces hemoglobin S molecules that polymerize within the erythrocyte during deoxygenation; the result is sustained hemolytic anemia and vaso-occlusive events. As patients live to adulthood, the chronic impact of sustained hemolytic anemia and episodic vaso-occlusive episodes leads to progressive end-organ complications. This scenario culminates in the development of 1 or more major cardiovascular complications of SCD for which there are no approved or consensus therapies...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222267/new-therapeutic-targets-in-transfusion-dependent-and-independent-thalassemia
#4
REVIEW
M Domenica Cappellini, Irene Motta
β-Thalassemias are characterized by reduced production of β-globin chain, resulting in α/β-chain unbalance and precipitation of α-globin-heme complexes and determining ineffective erythropoiesis. Ineffective erythropoiesis, chronic hemolytic anemia, and compensatory hematopoietic expansion are the disease hallmarks, and they are related to the severity of the chain unbalance. Several clinical forms of β-thalassemia, including the coinheritance of β-thalassemia with hemoglobin E resulting in hemoglobin E/β-thalassemia, have been described...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29219637/first-cases-of-hb-agrinio-described-in-patients-from-the-republic-of-macedonia
#5
Marija Dimishkovska, Maja Kuzmanovska, Svetlana Kocheva, Kata Martinova, Oliver Karanfilski, Zlate Stojanoski, Dijana Plaseska-Karanfilska
Previous molecular analyses of α-thalassemia (α-thal) in the Republic of Macedonia have identified the following genetic defects: -α3.7 (rightward), -(α)20.5 and - -MED I deletions and Hb Icaria [α142, Term→Lys (α2), HBA2: c.427T>A] and polyadenylation signal (polyA) [AATAAA>AATGAA (α2), HBA2: c.*92A>G] point mutations. Here, we report two unrelated patients from the Romani population in the Republic of Macedonia, homozygotes for the α2-globin gene variant Hb Agrinio [α29(B10)Leu→Pro; HBA2: c...
December 8, 2017: Hemoglobin
https://www.readbyqxmd.com/read/29218505/antibacterial-and-antibiofilm-activity-of-lactic-acid-bacteria-isolated-from-traditional-artisanal-milk-cheese-from-northeast-china-against-enteropathogenic-bacteria
#6
Xingyang Cui, Yunjia Shi, Shanshan Gu, Xin Yan, Hongyan Chen, Junwei Ge
The present study aims to investigate the probiotic properties of novel strains of lactic acid bacteria isolated from traditional artisanal milk cheese from Northeast China and to explore their antibacterial activity against enteropathogenic bacteria. Of the 321 isolates, 86 exhibited survival in low pH, resistance to pancreatin, and tolerance to bile salts; of these, 12 inhibited the growth of more than seven enteropathogenic bacteria and exhibited antibiofilm activities against Staphylococcus aureus CMCC26003 and/or Escherichia coli CVCC230...
December 7, 2017: Probiotics and Antimicrobial Proteins
https://www.readbyqxmd.com/read/29218045/factor-h-c-terminal-domains-are-critical-for-regulation-of-platelet-granulocyte-aggregate-formation
#7
Adam Z Blatt, Gurpanna Saggu, Claudio Cortes, Andrew P Herbert, David Kavanagh, Daniel Ricklin, John D Lambris, Viviana P Ferreira
Platelet/granulocyte aggregates (PGAs) increase thromboinflammation in the vasculature, and PGA formation is tightly controlled by the complement alternative pathway (AP) negative regulator, Factor H (FH). Mutations in FH are associated with the prothrombotic disease atypical hemolytic uremic syndrome (aHUS), yet it is unknown whether increased PGA formation contributes to the thrombosis seen in patients with aHUS. Here, flow cytometry assays were used to evaluate the effects of aHUS-related mutations on FH regulation of PGA formation and characterize the mechanism...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29216383/genetic-susceptibility-to-hemolytic-uremic-syndrome-after-shiga-toxin-producing-escherichia-coli-stec-infection-a-centers-for-disease-control-and-prevention-cdc-foodnet-study
#8
Asha R Kallianpur, Yuki Bradford, Rajal K Mody, Katie N Garman, Nicole Comstock, Sarah L Lathrop, Carol Lyons, Amy Saupe, Katie Wymore, Jeffrey A Canter, Lana M Olson, Amanda Palmer, Timothy F Jones
Background: Post-diarrheal hemolytic-uremic syndrome (D+HUS) following Shiga toxin-producing Escherichia coli (STEC) infection is a serious condition lacking specific treatment. Host immune dysregulation and genetic susceptibility to complement hyperactivation are implicated in non-STEC-related HUS. However, genetic susceptibility to D+HUS remains largely uncharacterized. Methods: Patients with culture-confirmed STEC diarrhea, identified through the CDC FoodNet surveillance system (2007-2012), were serotyped and classified by laboratory and/or clinical criteria as suspected, probable, or confirmed D+HUS, or as controls and genotyped at 200 loci linked to non-diarrheal HUS or similar pathologies...
December 6, 2017: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/29215813/kidney-transplantation-in-patients-with-atypical-hemolytic-uremic-syndrome-due-to-complement-factor-h-deficiency-impact-of-liver-transplantation
#9
Sejin Kim, Eujin Park, Sang Il Min, Nam Joon Yi, Jongwon Ha, Il Soo Ha, Hae Il Cheong, Hee Gyung Kang
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare disease that is often associated with genetic defects. Mutations of complement factor H (CFH) are the most common genetic defects that cause aHUS and often result in end-stage renal disease. Since CFH is mainly produced in the liver, liver transplantation (LT) has been performed in patients with defective CFH. METHODS: The clinical courses of four kidney allograft recipients who lost their native kidney functions due to aHUS associated with a CFH mutation were reviewed...
January 1, 2018: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29215086/genetic-predisposition-to-infection-in-a-case-of-atypical-hemolytic-uremic-syndrome
#10
Lambertus van den Heuvel, Kristian Riesbeck, Omaima El Tahir, Valentina Gracchi, Mariann Kremlitzka, Servaas A Morré, A Marceline van Furth, Birendra Singh, Marcin Okrój, Nicole van de Kar, Anna M Blom, Elena Volokhina
Most cases of hemolytic uremic syndrome (HUS) are caused by infection with enterohemorrhagic Escherichia coli (EHEC). Genetic defects causing uncontrolled complement activation are associated with the more severe atypical HUS (aHUS). Non-EHEC infections can trigger the disease, however, complement defects predisposing to such infections have not yet been studied. We describe a 2-month-old patient infected with different Gram-negative bacterial species resulting in aHUS. Serum analysis revealed slow complement activation kinetics...
November 13, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/29214674/neutrophil-extracellular-traps-in-plasma-from-dogs-with-immune-mediated-hemolytic-anemia
#11
C Lawson, S A Smith, M O'Brien, M McMichael
BACKGROUND: Neutrophil extracellular traps (NETs) are part of the innate immune response and are essential in local pathogen control, but are associated with pathological inflammation, organ damage, autoimmunity, and thrombosis. Immune-mediated hemolytic anemia (IMHA) is a pro-inflammatory, prothrombotic disease associated with high mortality. HYPOTHESIS/OBJECTIVES: Neutrophil extracellular traps (NETs) are a feature of the inflammatory process in dogs with IMHA...
December 7, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/29214442/shiga-toxin-triggers-endothelial-and-podocyte-injury-the-role-of-complement-activation
#12
REVIEW
Carlamaria Zoja, Simona Buelli, Marina Morigi
Shiga toxin (Stx)-producing Escherichia coli (STEC) is the offending agent in post-diarrhea-associated hemolytic uremic syndrome (HUS), a disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney failure, with thrombi occluding the renal microvasculature. Endothelial dysfunction has been recognized as the trigger event in the development of microangiopathic processes. Glomerular endothelial cells are susceptible to the toxic effects of Stxs that, via nuclear factor kappa B (NF-κB) activation, induce the expression of genes encoding for adhesion molecules and chemokines, culminating in leukocyte adhesion and platelet thrombus formation on the activated endothelium...
December 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29214337/sleep-disordered-breathing-in-patients-with-sickle-cell-disease
#13
REVIEW
Vikram M Raghunathan, Peter L Whitesell, Seah H Lim
Sickle cell disease is one of the most common hereditary hemoglobinopathies worldwide, and its vaso-occlusive and hemolytic crises cause considerable patient morbidity. A growing body of evidence has shown that sleep-disordered breathing, and in particular, obstructive sleep apnea, occurs at high frequency in the sickle cell population, and that there is significant overlap in the underlying pathophysiology of these two conditions. Through a variety of mechanisms including nocturnal hypoxemia and increased oxidative stress, production of pro-inflammatory cytokines, and endothelial dysfunction, sickle cell anemia and sleep-disordered breathing potentiate each other's clinical effects and end-organ complications...
December 6, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/29209311/codevelopment-of-microbiota-and-innate-immunity-and-the-risk-for-group-b-streptococcal-disease
#14
REVIEW
Julia Kolter, Philipp Henneke
The pathogenesis of neonatal late-onset sepsis (LOD), which manifests between the third day and the third month of life, remains poorly understood. Group B Streptococcus (GBS) is the most important cause of LOD in infants without underlying diseases or prematurity and the third most frequent cause of meningitis in the Western world. On the other hand, GBS is a common intestinal colonizer in infants. Accordingly, despite its adaption to the human lower gastrointestinal tract, GBS has retained its potential virulence and its transition from a commensal to a dangerous pathogen is unpredictable in the individual...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29208248/kidney-diseases-associated-with-alternative-complement-pathway-dysregulation-and-potential-treatment-options
#15
REVIEW
Prateek Sanghera, Mythili Ghanta, Fatih Ozay, Venkatesh K Ariyamuthu, Bekir Tanriover
Atypical hemolytic uremic syndrome and C3 glomerulopathy (dense deposit disease and C3 glomerulonephritis) are characterized as inappropriate activation of the alternative complement pathway. Genetic mutations affecting the alternative complement pathway regulating proteins (complement factor H, I, membrane cofactor protein and complement factor H-related proteins) and triggers (such as infection, surgery, pregnancy and autoimmune disease flares) result in the clinical manifestation of these diseases. A decade ago, prognosis of these disease states was quite poor, with most patients developing end-stage renal disease...
December 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29207719/approach-to-a-pregnant-woman-with-anti-d-anti-c-reactivity-pattern-a-diagnostic-conundrum
#16
Preeti Rai, Geetika Sharma, Deeksha Singh, Jyoti Garg
The Rhesus G antigen is present on all RBCs that are C+ and also on most D+ RBCs. Due to this co-distribution of G with either C or D antigen, it mimics a reactivity pattern of anti C + anti D on Indirect Antiglobulin Test (IAT), though the role of Anti G in causing Hemolytic Disease of Newborn (HDN) is controversial. The differentiation of anti D, anti C, and anti G is essential particularly in pregnant females. We hereby report a rare case of anti G with anti D and anti C in a pregnant woman with emphasis on approach to identify anti D+C+G and its implications...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/29202700/characterization-of-lpah2-gene-corresponding-to-lipopeptide-synthesis-in-bacillus-amyloliquefaciens-hab-2
#17
Pengfei Jin, Haonan Wang, Wenbo Liu, Weiguo Miao
BACKGROUND: Bacillus spp. have prominent ability to suppress plant pathogens and corresponding diseases. Previous analyses of Bacillus spp. revealed numerous gene clusters involved in nonribosomal synthesis of cyclic lipopeptides with distinct antimicrobial action. The 4'-phosphopantetheinyl transferase (PPTase) encoded by sfp gene is a key factor in lipopeptide synthesis in Bacillus spp. In previous study, B. amyloliquefaciens strain HAB-2 was found to inhibit a broad range of plant pathogens, which was attributed to its secondary metabolite lipopeptide...
December 4, 2017: BMC Microbiology
https://www.readbyqxmd.com/read/29200157/an-infant-with-unusually-high-unconjugated-hyperbilirubinemia-due-to-coexistence-of-hereditary-spherocytosis-and-gilbert-syndrome
#18
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...
December 1, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29194292/fetal-splenomegaly-a-review
#19
Marta Moreira, Rafael Brás, Daniela Gonçalves, Inês Alencoão, Gonçalo Inocêncio, Maria Rodrigues, Jorge Braga
Enlargement of the fetal spleen is usually found secondary to systemic diseases and is frequently associated with hepatomegaly. By far, the most common causes of fetal splenomegaly are infectious. Other etiologies responsible for this sign are hemolytic anemia, congestive cardiac failure, metabolic disorders, and rarely, leukemia, lymphoma, and histiocytosis.We report a case of prenatal splenomegaly diagnosed at 35 weeks, confirmed in the postnatal period. The postnatal workup showed the newborn had a familial type 3 form of hemophagocytic lymphohistiocytosis (HLH)...
November 29, 2017: Ultrasound Quarterly
https://www.readbyqxmd.com/read/29193117/the-development-of-d-antibodies-after-d-mismatched-kidney-transplantation-in-a-setting-of-reduced-immunosuppression
#20
Thomas H P M Habets, Joris Vanderlocht, Ron J M H E Straat, Tim C van Smaalen, Gerard M J Bos, Erik A Beckers, Maarten H L Christiaans, Yvonne M C Henskens
BACKGROUND: D antigens are not taken into account in the allocation of solid organs. Female transplant recipients with D antibodies as a consequence of D-mismatched kidney transplantation may develop hemolytic disease of the fetus and newborn in future pregnancies. We examined D antibody development in transplant recipients who received D-mismatched kidney transplantation in absence of D prophylaxis and in a setting of reduced immunosuppression. STUDY DESIGN AND METHODS: From 1993 until 2015, a total of 1355 kidney patients received transplantations in our center of whom 156 received a D-mismatched graft...
November 29, 2017: Transfusion
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