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Pediatric Clinics of North America

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https://www.readbyqxmd.com/read/29803288/benign-hematology
#1
EDITORIAL
Michael U Callaghan
No abstract text is available yet for this article.
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803287/living-up-to-its-name-advances-in-benign-hematology
#2
EDITORIAL
Bonita F Stanton
No abstract text is available yet for this article.
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803286/management-of-epistaxis-in-children-and-adolescents-avoiding-a-chaotic-approach
#3
REVIEW
Peter Svider, Khashayar Arianpour, Sean Mutchnick
This article provides an organized foundation that facilitates the management of acute epistaxis and an understanding of features that merit further diagnostic workup. Prompt management, including measures such as holding pressure and using nasal packing, takes precedence over comprehensive diagnostic workup. Severe, recurrent, and posteriorly based bleeds should prompt consideration of alternate interventions and expert consultation.
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803285/acquired-aplastic-anemia-what-have-we-learned-and-what-is-in-the-horizon
#4
REVIEW
Süreyya Savaşan
Acquired aplastic anemia (aAA) characterized by peripheral pancytopenia and bone marrow aplasia is a rare and serious disorder. Differential diagnosis includes constitutional bone marrow failure syndromes and myelodysplastic disorders. Autoimmune reaction to altered hematopoietic stem cells highlights the underlying mechanism. Matched related donor allogeneic hematopoietic stem cell transplantation is the ideal pediatric treatment; alternative approaches include immunosuppressive therapy and use of eltrombopag...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803284/red-blood-cell-enzyme-disorders
#5
REVIEW
Rachael F Grace, Bertil Glader
Mature red blood cells are reliant on the glycolytic pathway for energy production and the hexose monophosphate shunt for cell protection from oxidative insults. The most common red blood cell enzyme disorders are characterized by hemolysis but with wide clinical variability. Glucose-6-phosphate dehydrogenase deficiency is the most common red cell enzyme disorder worldwide. Frequent clinical presentations include neonatal jaundice and episodic hemolysis after exposure to oxidative stress. Symptoms of pyruvate kinase deficiency and other glycolytic enzyme disorders include neonatal jaundice, chronic hemolytic anemia, gallstones, and transfusion-related and transfusion-independent iron overload...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803283/congenital-disorders-of-platelet-function-and-number
#6
REVIEW
Ruchika Sharma, Juliana Perez Botero, Shawn M Jobe
Mucocutaneous bleeding symptoms and/or persistent thrombocytopenia occur in individuals with congenital disorders of platelet function and number. Apart from bleeding, these disorders are often associated with additional hematologic and clinical manifestations, including auditory, immunologic, and oncologic disease. Autosomal recessive, dominant, and X-linked inheritance patterns have been demonstrated. Precise delineation of the molecular cause of the platelet disorder can aid the pediatrician in the detection and prevention of specific disorder-associated manifestations and guide appropriate treatment and anticipatory care for the patient and family...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803282/abnormal-uterine-bleeding-in-young-women-with-blood-disorders
#7
REVIEW
Kathryn E Dickerson, Neethu M Menon, Ayesha Zia
Abnormal uterine bleeding is common in adolescents and is thought to affect 9% to 14% of women in their reproductive years. Certain unique aspects of underlying inherited or acquired blood disorders exacerbate the "expected" hormonal imbalance at this age, thereby increasing the morbidity of the underlying problem. A multifactorial etiology demands a collaborative approach between hematologists and gynecologists or adolescent medicine physicians to effectively manage abnormal uterine bleeding in young women with blood disorders...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803281/von-willebrand-disease-diagnostic-strategies-and-treatment-options
#8
REVIEW
Christopher J Ng, Jorge Di Paola
von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. Since its first description in 1926, the diagnosis and management of VWD has significantly improved due to increasing scientific knowledge of the genetics and biology of von Willebrand factor (VWF). This article reviews the molecular structure and function of VWF as well as the clinical symptoms, laboratory-based diagnostic workup, and classification schema for VWD. It highlights current treatment options and state-of-the art research in VWF and VWD...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803280/atypical-hemolytic-uremic-syndrome
#9
REVIEW
Bradley P Dixon, Ralph A Gruppo
Atypical hemolytic uremic syndrome is a rare life-threatening disease of unregulated complement activation. Untreated, the prognosis is generally poor; more than one-half of patients die or develop end-stage renal disease within 1 year. Atypical hemolytic uremic syndrome is characterized by thrombotic microangiopathy with evidence of hemolysis, thrombocytopenia, and renal impairment. This systemic disease affects the kidneys, brain, heart, lungs, gastrointestinal tract, pancreas, and skin. Acquired and genetic abnormalities of complement regulation may be identified in approximately 70% of patients...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803279/pulmonary-embolism-in-children
#10
REVIEW
Sarah Ramiz, Madhvi Rajpurkar
Pulmonary embolism (PE) in children is a rare condition with potential for high mortality. PE incidence is increasing owing to increased survival of children with predisposing conditions, increased use of central venous catheters, and improved awareness and recognition. Although pediatric PE is distinct from adult PE, management guidelines in children are extrapolated from the adult data. Treatment includes thrombolysis or thrombectomy, and pharmacologic anticoagulation. Ongoing clinical trials are evaluating the use of direct oral anticoagulants in children...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803278/the-epidemiology-and-management-of-lung-diseases-in-sickle-cell-disease-lessons-learned-from-acute-and-chronic-lung-disease-in-cystic-fibrosis
#11
REVIEW
Shaina M Willen, Michael R DeBaun
Although sickle cell disease and cystic fibrosis are two of the most common monogenic diseases presenting in childhood worldwide, cystic fibrosis and sickle cell disease enjoy vastly different funding and collaborative research efforts. Pulmonary complications in cystic fibrosis have well established guidelines and multidisciplinary involvement focusing on comorbidities, routine monitoring, infectious complications, nutrition, and treatment recommendations. These guidelines can provide a framework on which to build knowledge of lung disease in sickle cell disease...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803277/genetic-therapies-for-sickle-cell-disease
#12
REVIEW
Rajeswari Jayavaradhan, Punam Malik
Sickle cell disease is the most prevalent monogenic disorder worldwide and curative therapies are limited to hematopoietic stem cell transplant to the few with matched donors. Gene therapy has curative potential, whereby autologous hematopoietic stem cells are genetically modified and transplanted, which would not be limited by matched donors, resulting in 1-time, life-long correction devoid of immune side effects. Significant progress has been made to clinically translate gene therapy for sickle cell disease using lentivirus vectors carrying antisickling genes...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803276/a-scientific-renaissance-novel-drugs-in-sickle-cell-disease
#13
REVIEW
Ahmar U Zaidi, Matthew M Heeney
We have entered an era of exploding interest in therapeutics for sickle cell disease. The expansion in our understanding of sickle cell disease pathophysiology has enhanced the range of potential therapeutic targets. From induction of fetal hemoglobin to antiadhesion molecules, we are potentially on the cusp of making life-altering modifications for individuals with sickle cell disease. This disease population cannot afford to let the current momentum wane. Studies exploring combinations of therapies affecting multiple steps in the pathophysiology and exploring novel and clinically relevant outcomes are incumbent...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803275/treatment-options-for-sickle-cell-disease
#14
REVIEW
Emily Riehm Meier
Sickle cell disease (SCD) complications begin with the polymerization of sickle hemoglobin (HbS). Thus, SCD therapies are focused on preventing HbS production or reducing the circulating amount of HbS. Hydroxyurea treatment has become more widespread, whereas the number of evidence-based indications for erythrocyte transfusion is small. Hematopoietic stem cell transplant is a curative option for SCD but less than 25% of patients have a suitable donor. This article focuses on supportive and preventive care improvements and the benefits of hydroxyurea...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29803274/evolving-complexity-in-hemophilia-management
#15
REVIEW
Stacy E Croteau
Rapid expansion of therapeutic options have increased the complexity of hemophilia care. Previously, on-demand therapy aimed to reduce morbidity and early mortality; however, now aggressive prophylaxis, particularly in children, encourages an active lifestyle. Accurate diagnosis, recognition of early threats to musculoskeletal health, and optimization of therapy are critical for both males and females affected by hemophilia. The diversity of emerging hemophilia therapies, from modified factor protein concentrates, to gene therapy, to nonfactor hemostatic strategies, provide an exciting opportunity to target unmet needs in the bleeding disorder community...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29502923/new-understanding-of-mechanisms-and-new-hope-for-treatments
#16
EDITORIAL
Bonita F Stanton
No abstract text is available yet for this article.
April 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29502922/erratum
#17
(no author information available yet)
No abstract text is available yet for this article.
April 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29502921/approach-to-inborn-errors-of-metabolism-in-pediatrics
#18
EDITORIAL
Ayman W El-Hattab, V Reid Sutton
No abstract text is available yet for this article.
April 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29502920/newborn-screening-history-current-status-and-future-directions
#19
REVIEW
Ayman W El-Hattab, Mohammed Almannai, V Reid Sutton
Newborn screening programs aim to achieve presymptomatic diagnosis of treatable disorders allowing for early initiation of medical care to prevent or reduce significant morbidity and mortality. Many of the conditions included in the newborn screening panels are inborn errors of metabolism; however, screening for endocrine, hematologic, immunologic, and cardiovascular diseases, and hearing loss is also included in many panels. Newborn screening tests are not diagnostic and therefore diagnostic testing is needed to confirm or exclude the suspected diagnosis...
April 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29502919/complex-phenotypes-in-inborn-errors-of-metabolism-overlapping-presentations-in-congenital-disorders-of-glycosylation-and-mitochondrial-disorders
#20
REVIEW
Thatjana Gardeitchik, Jeroen Wyckmans, Eva Morava
Congenital disorders of glycosylation (CDG) and mitochondrial disorders have overlapping clinical features, including central nervous system, cardiac, gastrointestinal, hepatic, muscular, endocrine, and psychiatric disease. Specific abnormalities orienting the clinician toward the right diagnostic approach include abnormal fat distribution, coagulation abnormalities, together with anticoagulation abnormalities, hyperinsulinism, and congenital malformations in CDG. Diabetes, sensorineural deafness, and depression are very rare in CDG but common in mitochondrial disease...
April 2018: Pediatric Clinics of North America
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