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https://www.readbyqxmd.com/read/29340523/a-case-of-severe-glutathione-synthetase-deficiency-with-novel-gss-mutations
#1
H Xia, J Ye, L Wang, J Zhu, Z He
Glutathione synthetase deficiency (GSSD) is a rare inborn error of glutathione metabolism with autosomal recessive inheritance. The severe form of the disease is characterized by acute metabolic acidosis, usually present in the neonatal period with hemolytic anemia and progressive encephalopathy. A case of a male newborn infant who had severe metabolic acidosis with high anion gap, hemolytic anemia, and hyperbilirubinemia is reported. A high level of 5-oxoproline was detected in his urine and a diagnosis of generalized GSSD was made...
January 11, 2018: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
https://www.readbyqxmd.com/read/29314195/methylene-blue-induced-heinz-body-hemolytic-anemia-in-a-premature-neonate
#2
Lewis Vanhinsbergh, Sabita Uthaya, Barbara J Bain
No abstract text is available yet for this article.
January 5, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29313481/case-report-severe-plasmodium-vivax-malaria-mimicking-sepsis-in-a-neonate
#3
Suryadi N N Tatura, Elizabeth Clarissa Wowor, Jose M Mandei, Rocky Wilar, Sarah M Warouw, Johnny Rompis, Priscilla Kalensang, Joseph Tuda
Severe congenital malaria associated with Plasmodium vivax is uncommon. In Indonesia, most congenital malaria cases are due to Plasmodium falciparum infections. Most cases of congenital or neonatal malaria in endemic areas are diagnosed from peripheral smear as part of routine sepsis workup. Differentiating congenital and acquired neonatal malaria is very difficult. The case presented in this study describes severe P. vivax malaria with cholestatic jaundice and sepsis-like signs and symptoms in neonates. The mother was asymptomatic and the neonate was successfully treated with intravenous artesunate...
January 8, 2018: American Journal of Tropical Medicine and Hygiene
https://www.readbyqxmd.com/read/29284213/pathologic-and-molecular-characterization-of-streptococcus-dysgalactiae-subspecies-equisimilis-infection-in-neonatal-piglets
#4
Sang-Ik Oh, Jong Wan Kim, Ji-Youl Jung, Myeongju Chae, Yu-Ran Lee, Jong Ho Kim, ByungJae So, Ha-Young Kim
Streptococcus dysgalactiae subspecies equisimilis (SDSE) is an emerging pathogen in animals and humans. Here, we describe two clinical swine cases of SDSE infection presenting with lameness, neurological signs, or sudden death. Pathological examination indicated suppurative arthritis, encephalitis, and multifocal abscesses in the kidney and heart. β-hemolytic colonies from the joint samples of each case were identified as SDSE. The isolates showed low minimum inhibitory concentration values for β-lactams and presented the same virulence gene profile (slo-/sagA+/pSTKP8+)...
December 28, 2017: Journal of Veterinary Science
https://www.readbyqxmd.com/read/29259893/neonatal-pyknocytosis-in-a-preterm-dizygotic-twin
#5
Alberto Berardi, Eleonora Balestri, Goretta Bonacorsi, Claudio Chiossi, Giovanni Palazzi, Eugenio Spaggiari, Fabrizio Ferrari
Infantile pyknocytosis (IP) is a rare, self-limited neonatal haemolytic anaemia that may require multiple blood transfusions. Only a little more than 50 cases have been reported in the medical literature, and the great majority of them concerns term infants. The etiology of IP is not well understood; most likely it results from a transient extra-corpuscular factor, whose nature is unknown, transmitted from mother to child or, alternatively, from a deficiency of an anti-oxidative agent. We report the case of two preterm twins, one of which suffered from IP and developed severe anaemia at age 2 wk, while the other was unaffected...
November 8, 2017: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/29247156/melatonin-promotes-brain-derived-neurotrophic-factor-bdnf-expression-and-anti-apoptotic-effects-in-neonatal-hemolytic-hyperbilirubinemia-via-a-phospholipase-plc-mediated-mechanism
#6
Yong Luo, Mei Peng, Hong Wei
BACKGROUND Melatonin therapy shows positive effects on neuroprotective factor brain-derived neurotrophic factor (BDNF) expression and neuronal apoptosis in neonatal hemolytic hyperbilirubinemia. We hypothesized that melatonin promotes BDNF expression and anti-apoptotic effects in neonatal hemolytic hyperbilirubinemia through a phospholipase (PLC)-mediated mechanism. MATERIAL AND METHODS A phenylhydrazine hydrochloride (PHZ)-induced neonatal hemolytic hyperbilirubinemia model was constructed in neonatal rats...
December 16, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/29244079/a-non-hemolytic-group-b-streptococcus-strain-exhibits-hypervirulence
#7
Claire Gendrin, Jay Vornhagen, Blair Armistead, Pallavi Singh, Christopher Whidbey, Sean Merillat, David Knupp, Robert Parker, Lisa M Rogers, Phoenicia Quach, Lakshminarayan M Iyer, L Aravind, Shannon D Manning, David M Aronoff, Lakshmi Rajagopal
Group B Streptococci (GBS) are gram-positive bacteria that are a leading cause of neonatal infections. Most invasive isolates are -hemolytic and hemolytic activity is critical for GBS virulence. While non-hemolytic GBS strains are occasionally isolated, they are often thought to be virulence attenuated. Here, we show that a non-hemolytic GBS strain (GB37) isolated from a septic neonate exhibits hypervirulence. Substitution of tryptophan to leucine (W297L) in the sensor histidine kinase CovS results in constitutive kinase signaling, leading to decreased hemolysis and increased activity of the GBS hyaluronidase, HylB...
December 13, 2017: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/29209311/codevelopment-of-microbiota-and-innate-immunity-and-the-risk-for-group-b-streptococcal-disease
#8
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/29177106/anti-m-alloimmunization-management-and-outcome-at-a-single-institution
#9
Bethany Stetson, Scott Scrape, Kara Beth Markham
Objective  The objective of this study was to review the management strategies and outcomes in gravidas with anti-M alloimmunization over 15 years. Study Design  Data collected from 195 pregnant patients with anti-M antibodies from July 2000 through June 2016 were reviewed retrospectively. We analyzed indirect antiglobulin test titer results, paternal or fetal/neonatal M antigen status, antepartum course, and perinatal outcomes. Results  Anti-M antibodies were found in 146 women and 195pregnancies. Among those with positive indirect antiglobulin tests, 193 pregnancies had titers at or below 1:4...
October 2017: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/29070139/-analysis-of-correlation-between-igg-titer-of-pregnant-women-and-neonatal-hemolytic-complications-of-different-blood-groups
#10
Hai-Hui Ye, Hong-Hai Huang, Xiao-Lin Wang, You-Jun Pi
OBJECTIVE: To study the relationship between IgG titer of pregnant women and hemolytic disease of newborn(HDN) with different blood groups. METHODS: Four hundred pregnant women, including pregnant women with type O blood, were selected from May 2014 to January 2015 in our hospital for inspection and a couple of different blood groups, the IgG titer of pregnant women were detected in the inspection process. According to neonatal HDN, newborns were divided into 2 groups: HDN group(85 cases) and non-HDN group(315 cases)...
October 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29043829/postpartum-acute-hemolytic-transfusion-reactions-associated-with-anti-lea-in-two-pregnancies-complicated-by-preeclampsia
#11
Marcia Marchese
Lewis blood group antibodies, which are mostly naturally occurring and considered clinically insignificant, have rarely been documented as a cause of acute hemolytic transfusion reactions (AHTRs). This report presents two cases of AHTRs caused by anti-Lea occurring in postpartum black females (one group B, one group AB) whose pregnancies were complicated by preeclampsia. Neither anti-Lea was detected by automated solid-phase red cell adherence technology in pre-transfusion testing. Therefore, red blood cell units, compatible by electronic crossmatch, were issued and transfused...
September 2017: Immunohematology
https://www.readbyqxmd.com/read/28991323/-neonatal-infection-and-universal-screening-for-streptococcus-agalactiae-group-b-%C3%AE-hemolytic-streptococcus-in-the-pregnant-woman-technical-recommendation
#12
(no author information available yet)
No abstract text is available yet for this article.
June 2017: Revista Chilena de Infectología: órgano Oficial de la Sociedad Chilena de Infectología
https://www.readbyqxmd.com/read/28988250/prevalence-of-glucose-6-phosphate-dehydrogenase-deficiency-in-neonates-in-egypt
#13
Soheir Abo Elella, Mahaa Tawfik, Naglaa Barseem, Wafaa Moustafa
BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked disorder which causes neonatal jaundice in most cases, and under certain conditions, can cause a spectrum of hemolytic manifestations. OBJECTIVE: To determine the local prevalence of G6PD deficiency in newborns. DESIGN: Cross-sectional. SETTING: University hospital. METHODS: Infants born during 2015 were prospectively screened for G6PD deficiency...
September 2017: Annals of Saudi Medicine
https://www.readbyqxmd.com/read/28982343/study-of-glucose-6-phosphate-dehydrogenase-deficiency-5-years-retrospective-egyptian-study
#14
Adel Abd Elhaleim 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 an 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...
October 3, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28950852/a-chronological-study-of-the-bacterial-pathogen-changes-in-acute-neonatal-bacterial-conjunctivitis-in-southern-china
#15
Song Tang, Ming Li, Hongbo Chen, Guo Ping, Chun Zhang, Shusheng Wang
BACKGROUND: The aim of the project is to retrospectively study the changes in bacterial pathogens in acute neonatal bacterial conjunctivitis from 2002 to 2016 in Southern China. The results may provide the guidance for drug choice for acute neonatal bacterial conjunctivitis. METHODS: Secretion specimens for bacterial culture were taken from 485 cases with clinically diagnosed acute bacterial neonatal conjunctivitis. Bacterial pathogens were detected by Gram staining and subsequent bacterial culture...
September 26, 2017: BMC Ophthalmology
https://www.readbyqxmd.com/read/28926834/heme-oxygenase-activity-and-heme-binding-in-a-neonatal-mouse-model
#16
Stephanie Kourula, Joyce Ang, Hui Zhao, Flora Kalish, Peter Vandenabeele, Karl G Sylvester, Ronald J Wong, David K Stevenson
BACKGROUND: Severe hemolytic disease of the newborn leads to the release of pro-oxidative free heme (FH). Heme oxygenase (HO) is primarily responsible for detoxifying FH. OBJECTIVE: To investigate the protective effects of HO in a model of heme overload. METHODS: For in vitro studies, NIH3T3 HO-1-luc cells were incubated with 10, 30, or 60 µM FH or methemalbumin (MHA). HO-1 promoter activity was assessed 3, 6, and 24 h after treatment. Cell survival was indexed by viability assays...
September 20, 2017: Neonatology
https://www.readbyqxmd.com/read/28912922/obstetric-outcomes-of-twin-pregnancies-in-japanese-women-aged-40-and-older
#17
Shunji Suzuki
BACKGROUND: The aim of this study was to compare the obstetric outcomes of twin pregnancies between Japanese women aged ≥ 40 years and their younger counterparts aged 25 - 29. METHODS: This was a retrospective study of twin pregnancies managed at Japanese Red Cross Katsushika Maternity Hospital between 2002 and 2016. Women aged 40 and older at delivery (n = 117) were compared with women aged 25 - 29 at delivery (n = 536). RESULTS: Although the women ≥ 40 years old were more likely to have increased risks of HELLP (hemolytic, elevated liver enzymes and low platelet) syndrome and very low birth weight neonates, there were no measurable differences in obstetric outcomes such as hypertensive disorders, premature delivery and neonatal asphyxia between the two groups...
October 2017: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/28905285/effect-of-bacteriocin-and-exopolysaccharides-isolated-from-probiotic-on-p-aeruginosa-pao1-biofilm
#18
Vivek Sharma, Kusum Harjai, Geeta Shukla
Microorganisms develop biofilms on indwelling medical devices and are associated with biofilm-related infections, resulting in substantial morbidity and mortality. Therefore, to prevent and control biofilm-associated infections, the present study was designed to assess the anti-biofilm potential of postbiotics derived from probiotic organisms against most prevalent biofilm-forming Pseudomonas aeruginosa PAO1. Eighty lactic acid bacteria isolated from eight neonatal fecal samples possessed antibacterial activity against P...
September 14, 2017: Folia Microbiologica
https://www.readbyqxmd.com/read/28899405/neonatal-hemolytic-anemia-does-not-always-indicate-thalassemia-a-case-report
#19
Arwa A Al-Harazi, Bilguis M Al-Eryani, Butheinah A Al-Sharafi
BACKGROUND: Congenital erythropoietic porphyria is a rare autosomal recessive disorder that affects heme-porphyrin synthesis. This disorder is due to the genetic defect of uroporphyrinogen III cosynthase. This defect results in the accumulation of high amounts of uroporphyrin I in all tissues, leading to clinical manifestations ranging from mild to severe chronic damage of the skin, cartilage and bone. Hypertrichosis, erythrodontia and reddish-colored urine are often present, as well as hemolytic anemia accompanied by hepatosplenomegaly...
September 12, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28883277/siblings-with-congenital-thrombotic-thrombocytopenic-purpura
#20
Yasutomo Funakoshi, Masahiko Okada, Masanori Matsumoto, Koichi Kokame, Hiroyuki Moriuchi
Congenital thrombotic thrombocytopenic purpura (TTP) is a rare hereditary deficiency of ADAMTS13 (von Willebrand factor-cleaving protease) characterized by thrombocytopenia and microangiopathic hemolytic anemia. The spectrum of the clinical phenotype is wide, ranging from asymptomatic episodes of thrombocytopenia to life-threatening multiorgan failure. Reportedly, some patients develop isolated thrombocytopenia during childhood. We herein report sibling cases of congenital TTP. An 11-year-old boy with thrombocytopenia accompanied by influenza virus infection was referred to our hospital...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
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