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thrombocytopenia congenital heart

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https://www.readbyqxmd.com/read/29756161/risk-factors-for-failure-of-systemic-to-pulmonary-artery-shunts-in-biventricular-circulation
#1
Keti Vitanova, Cornelius Leopold, Jelena Pabst von Ohain, Cordula Wolf, Elisabeth Beran, Rüdiger Lange, Julie Cleuziou
Systemic-to-pulmonary artery shunt placement is an established palliative procedure for congenital heart disease, but it is associated with high morbidity and mortality. Data of all patients with biventricular circulation who underwent systemic-to-pulmonary artery shunt implantation between 2000 and 2016 were reviewed. Endpoints of the study were shunt failure and shunt-related mortality. Shunt failure was defined as any shunt dysfunction requiring intervention or reoperation. Shunt-related mortality was defined as death due to shunt dysfunction...
May 14, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29753820/association-of-pre-operative-cell-counts-with-outcomes-after-surgery-for-congenital-heart-disease
#2
Shannon Jones, Courtney McCracken, Bahaaldin Alsoufi, William T Mahle, Matthew E Oster
BACKGROUND: We examined the association of pre-operative cell count abnormalities, which have been shown to be associated with outcomes in adult cardiac patients, with morbidity and mortality following surgery for congenital heart disease (CHD) in children. METHODS: We performed a retrospective cohort study on 4865 children undergoing cardiac surgery from 2004- 2014. Our exposures of interest were presence of pre-operative lymphopenia (lymphocyte count ≤ 3000 cells/μL), thrombocytopenia (platelet count < 150 x103 / μL), and neutrophilia (neutrophil count ≥ 7000 cells/ μL)...
May 10, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29721915/clinical-biochemical-and-molecular-overview-of-transaldolase-deficiency-and-evaluation-of-the-endocrine-function-update-of-34-patients
#3
M Williams, V Valayannopoulos, R Altassan, W K Chung, A C Heijboer, W T Keng, R Lapatto, P McClean, M F Mulder, A Tylki-Szymańska, M J E Walenkamp, M Alfadhel, H Alakeel, G S Salomons, W Eyaid, M M C Wamelink
BACKGROUND: Transaldolase deficiency (TALDO-D) is a rare autosomal recessive inborn error of the pentose phosphate pathway. Since its first description in 2001, several case reports have been published, but there has been no comprehensive overview of phenotype, genotype, and phenotype-genotype correlation. METHODS: We performed a retrospective questionnaire and literature study of clinical, biochemical, and molecular data of 34 patients from 25 families with proven TALDO-D...
May 2, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29605959/heparin-induced-thrombocytopenia-in-infants-after-heart-surgery
#4
Juma N Abdillah, Qinghua Hu, Xuliang Chen, Xing Chen, Wu Zhou, Wanjun Luo, Lingjin Huang
BACKGROUND:  Heparin-induced thrombocytopenia (HIT) in infants is a rare disorder, and the diagnosis and management of HIT still remains challenging. Argatroban is a synthetic direct thrombin inhibitor (DTI) that is widely used for treating HIT. However, little is known about the efficacy of the activated clotting time (ACT) test in monitoring DTI treatment as an alternative to the routinely used activated partial thromboplastin time (aPTT). METHODS:  Between July 2013 and January 2015, four infants were diagnosed with HIT after surgical correction of congenital anomalies...
April 1, 2018: Thoracic and Cardiovascular Surgeon
https://www.readbyqxmd.com/read/29523490/thrombotic-microangiopathy-in-a-very-young-infant-with-mitral-valvuloplasty
#5
Yuka Matsunaga, Masataka Ishimura, Hazumu Nagata, Kiyoshi Uike, Tadamune Kinjo, Masayuki Ochiai, Kenichiro Yamamura, Hidetoshi Takada, Yoshihisa Tanoue, Masaki Hayakawa, Masanori Matsumoto, Toshiro Hara, Shouichi Ohga
BACKGROUND: Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by systemic or intrarenal platelet aggregation, thrombocytopenia, and red cell fragmentation. Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear. CASE PRESENTATION: A one-month-old infant developed TMA after the initial surgery of double outlet right ventricle...
February 7, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29520672/features-associated-with-pulmonary-arterial-hypertension-in-chinese-hospitalized-systemic-lupus-erythematosus-patients
#6
Shu-Zhen Xu, Yan Liang, Xiang-Pei Li, Xiao-Mei Li, Zong-Wen Shuai, Rui-Xue Leng, Hai-Feng Pan, Dong-Qing Ye
Pulmonary arterial hypertension (PAH) is an increasingly recognized complication of systemic lupus erythematosus (SLE). This study aims to estimate the point prevalence of PAH and identify risk factors for PAH in a large cohort of hospitalized SLE patients. We have collected the medical records of patients hospitalized with SLE at the First Affiliated Hospital of Anhui Medical University and Anhui Provincial Hospital. Resting transthoracic echocardiography (TTE) was used to estimate pulmonary artery pressure (PAP) and PAH was defined as systolic PAP (PASP) > 30 mmHg...
June 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29281614/potential-contribution-of-erythrocyte-microrna-to-secondary-erythrocytosis-and-thrombocytopenia-in-congenital-heart-disease
#7
Nobuhiro Mukai, Yoshinobu Nakayama, Satoshi Murakami, Toshihito Tanahashi, Daniel I Sessler, Sachiyo Ishii, Satoru Ogawa, Natsuko Tokuhira, Toshiki Mizobe, Teiji Sawa, Yasufumi Nakajima
BackgroundChildren with cyanotic heart disease develop secondary erythrocytosis and thrombocytopenia via unknown mechanisms. Mature erythrocyte microRNAs may reflect clinical pathologies and cell differentiation processes pre-enucleation. This study evaluated erythrocyte microRNAs in children with cyanotic heart disease.MethodsErythrocyte microRNAs from children with cyanotic and acyanotic heart disease and without cardiac disease were quantified with Ion PGM System (n=10 per group). Differential expression was confirmed by quantitative PCR (qPCR; n=20 per group)...
January 17, 2018: Pediatric Research
https://www.readbyqxmd.com/read/29250516/use-of-therapeutic-plasma-exchange-during-extracorporeal-life-support-in-critically-ill-cardiac-children-with-thrombocytopenia-associated-multi-organ-failure
#8
Mei Chong, Alejandro J Lopez-Magallon, Lucas Saenz, Mahesh S Sharma, Andrew D Althouse, Victor O Morell, Ricardo Munoz
Background: Thrombocytopenia-associated multi-organ failure (TAMOF) in children is a well-described factor for increased hospital mortality. Low cardiac output syndrome (LCOS) and the effects of cardiopulmonary bypass may manifest with several adverse physiologic and immunologic effects, with varying degrees of thrombocytopenia and multi-organ dysfunction, sometimes very similar to TAMOF. LCOS is a common occurrence in children with critical heart disease, presenting in as much as 23...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29217070/congenital-vascular-tumors
#9
REVIEW
Jeremy A Goss, Arin K Greene
Vascular tumors are benign neoplasms, which result from proliferating endothelial cells. These lesions present during infancy or childhood, may affect any location, and exhibit postnatal growth. Local complications include bleeding, tissue destruction, and pain whereas systemic sequelae include thrombocytopenia, congestive heart failure, and death. Vascular tumors should be differentiated from vascular malformations, which present at birth, have a quiescent endothelium, and grow in proportion to the child. Together, vascular tumors and malformations comprise the field of vascular anomalies...
February 2018: Otolaryngologic Clinics of North America
https://www.readbyqxmd.com/read/29207763/carbamazepine-induced-thrombocytopenia
#10
Ruhi Kumar, Sridhar Chivukula, Goutham Reddy Katukuri, U K Chandrasekhar, K N Shivashankar
Antiepileptic Drugs (AEDs) are commonly associated with haematological disorders, including anaemia, thrombocytopenia, neutropenia and even bone marrow failure. Fatal disorders like aplastic anaemia are uncommon. On exploring through the literature, older AEDs are more associated with haematological alterations than newer AEDs, and careful monitoring is warranted especially with phenytoin, carbamazepine and valproate. The exact cause of these alterations is not established, though immune mechanisms and pharmacology of individual drugs are the proposed mechanisms, a further research along this path is underway...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28880458/determinants-of-platelet-count-in-pediatric-patients-with-congenital-cyanotic-heart-disease-role-of-immature-platelet-fraction
#11
Randa M Matter, Iman A Ragab, Alaa M Roushdy, Ahmed G Ahmed, Hanan H Aly, Eman A Ismail
OBJECTIVES: Congenital heart defects are common noninfectious causes of mortality in children. Bleeding and thrombosis are both limiting factors in the management of such patients. We assessed the frequency of thrombocytopenia in pediatric patients with congenital cyanotic heart disease (CCHD) and evaluated determinants of platelet count including immature platelet fraction (IPF) and their role in the pathogenesis of thrombocytopenia. METHODS: Forty-six children and adolescents with CCHD during pre-catheter visits were studied; median age was 20...
January 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/28422132/haploinsufficiency-for-ankrd11-flanking-genes-makes-the-difference-between-kbg-and-16q24-3-microdeletion-syndromes-12-new-cases
#12
Francesca Novara, Berardo Rinaldi, Sanjay M Sisodiya, Antonietta Coppola, Sabrina Giglio, Franco Stanzial, Francesco Benedicenti, Alan Donaldson, Joris Andrieux, Rachel Stapleton, Astrid Weber, Paolo Reho, Conny van Ravenswaaij-Arts, Wilhelmina S Kerstjens-Frederikse, Joris Robert Vermeesch, Koenraad Devriendt, Carlos A Bacino, Andrée Delahaye, S M Maas, Achille Iolascon, Orsetta Zuffardi
16q24 deletion involving the ANKRD11 gene, ranging from 137 kb to 2 Mb, have been associated with a microdeletion syndrome characterized by variable cognitive impairment, autism spectrum disorder, facial dysmorphisms with dental anomalies, brain abnormalities essentially affecting the corpus callosum and short stature. On the other hand, patients carrying either deletions encompassing solely ANKRD11 or its loss-of-function variants were reported in association with the KBG syndrome, characterized by a very similar phenotype, including mild-to-moderate intellectual disability, short stature and macrodontia of upper incisors, with inter and intrafamilial variability...
June 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28393783/coagulopathies-in-cyanotic-cardiac-patients-an-analysis-with-three-point-of-care-testing-devices-thromboelastography-rotational-thromboelastometry-and-sonoclot-analyzer
#13
Vandana Bhardwaj, Poonam Malhotra, Suruchi Hasija, Ujjwal Kumar Chowdury, Neha Pangasa
INTRODUCTION: In the last few years, viscoelastic point-of-care (POC) coagulation devices such as thromboelastography (TEG), rotational thromboelastometry (ROTEM), and Sonoclot (SON) analyzer have been increasingly used in major surgeries for timely assessment and management of coagulopathies. The aim of the present study was to evaluate coagulation profile of cyanotic cardiac patients with TEG, ROTEM, and SON analyzer. In addition, we assessed the correlation of standard laboratory coagulation tests and postoperative chest drain output (CDO) with the parameters of POC testing devices...
April 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28382967/thrombotic-thrombocytopenic-purpura
#14
REVIEW
Johanna A Kremer Hovinga, Paul Coppo, Bernhard Lämmle, Joel L Moake, Toshiyuki Miyata, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic haemolytic anaemia and a variable degree of ischaemic organ damage, particularly affecting the brain, heart and kidneys. Acute TTP was almost universally fatal until the introduction of plasma therapy, which improved survival from <10% to 80-90%. However, patients who survive an acute episode are at high risk of relapse and of long-term morbidity...
April 6, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28212920/neonatal-lupus-follow-up-in-infants-with-anti-ssa-ro-antibodies-and-review-of-the-literature
#15
REVIEW
Antonio Alberto Zuppa, Riccardo Riccardi, Simonetta Frezza, Francesca Gallini, Rita Maria Paola Luciano, Giovanni Alighieri, Costantino Romagnoli, Sara De Carolis
Neonatal Lupus Syndrome (NLS) is a distinct clinical entity caused by transplacental passage of maternal anti-SSA/Ro antibodies (Ab). Mothers may have systemic lupus erythematosus, Sjögren syndrome, or other connective tissue disease, or may be completely healthy at the time of giving birth. NLS includes several clinical manifestations: complete congenital heart block (CCHB) and cutaneous lupus are the most common, while hepatobiliary disease, hematological manifestations and central nervous system involvement may occur...
April 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28149700/retrospective-study-of-prognostic-factors-in-pediatric-invasive-pneumococcal-disease
#16
Nan-Chang Chiu, Hsin Chi, Chun-Chih Peng, Hung-Yang Chang, Daniel Tsung-Ning Huang, Lung Chang, Wei-Te Lei, Chien-Yu Lin
Streptococcus pneumoniae remains the leading causative pathogen in pediatric pneumonia and bacteremia throughout the world. The invasive pneumococcal disease (IPD) is known as isolation of S. pneumoniae from a normally sterile site (e.g., blood, cerebrospinal fluid, synovial fluid, pericardial fluid, pleural fluid, or peritoneal fluid). The aim of this study is to survey the clinical manifestations and laboratory results of IPD and identify the prognostic factors of mortality. From January 2001 to December 2006, a retrospective review of chart was performed in a teaching hospital in Taipei...
2017: PeerJ
https://www.readbyqxmd.com/read/28051234/neonatal-lupus-erythematosus-with-congenital-heart-block-in-twins
#17
Lamia Gargouri, Faiza Safi, Bayen Maalej, Souad Mallek, Fatma Turki, Imen Majdoub, Malek Akrout, Dorra Abid, Samir Kamoun, Abdelmajid Mahfoudh
Background - Neonatal lupus erythematosus is an uncommon acquired autoimmune disease caused by transplacental passage of maternal antibodies SSA/Ro, SSB/La or U1 ribonucleoproteins. The most common clinical manifestations are skin rash, cardiac lesions, thrombocytopenia, anemia and hepatosplenomegaly. Complete congenital heart block is usually irreversible needing a pacemaker implantation in two-thirds of cases. Cases report - We report neonatal lupus erythematosus with complete congenital heart block in twins...
July 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/27987519/-autoimmune-disorder-secondary-to-digeorge-syndrome-a-long-term-follow-up-case-report-and-literature-review
#18
Y Xie, J Q Guo, Y Hua, W H Zhao, Q Sun, X T Lu
DiGeorge syndrome is the most common chromosome microdeletion disease. The classical complications include congenital heart disease, hypothyroidism, immunodeficiency, facial abnormalities, and hypocalcemia. According to whether there is an absence or hypoplasia of the thymus, DiGeorge syndrome can be divided into two types, complete DiGeorge syndrome and partial DiGeorge syndrome. The patient was a female born with congenital heart disease, facial abnormalities and cleft palate. When the patient went to school, she had learning difficulty and had problems in communication and personal social behavior...
December 18, 2016: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/27753765/risk-adjustment-for-congenital-heart-surgery-score-as-a-risk-factor-for-candidemia-in-children-undergoing-congenital-heart-defect-surgery
#19
Fabio de Araujo Motta, Libera Maria Dalla-Costa, Marisol Dominguez Muro, Andrea Lenzi, Gledson Luiz Picharski, Marion Burger
BACKGROUND: Candida species are the primary cause of invasive fungal infection in hospitalized children. There are few data on risk factors for postoperative candidemia in pediatric patients with congenital heart defects. This study aimed to identify risk factors for candidemia in patients with congenital heart defects who underwent cardiac surgery. METHODS: This was a case-control study conducted in patients admitted to a pediatric cardiology intensive care unit from January 2006 to December 2013...
November 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/27681010/preterm-thrombocytopenia-and-delay-of-ductus-arteriosus-closure
#20
Vinay Vamadev Kulkarni, Sourabh Dutta, Venkataseshan Sundaram, Shiv Sajan Saini
OBJECTIVES: To evaluate whether preterm thrombocytopenia within 24 hours of birth is associated with delayed closure of patent ductus arteriosus (PDA) and higher proportion of hemodynamically significant PDA (Hs-PDA). METHODS: Neonates (gestation 260/7-336/7 weeks, age <24 hours) with known platelet count and PDA on echocardiogram were prospectively enrolled. Asphyxia, congenital infections, structural heart disease, major malformations and clinical sepsis were exclusions...
October 2016: Pediatrics
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