8,679 results match your criteria Cyanosis


Pulmonary arteriovenous malformation revealed by a chronic cyanosis.

Acta Cardiol 2019 Jan 16:1-2. Epub 2019 Jan 16.

b Division of Paediatric Radiology , Hôpital Universitaire des Enfants Reine Fabiola (Université Libre de Bruxelles ULB) , Brussels , Belgium.

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http://dx.doi.org/10.1080/00015385.2018.1523974DOI Listing
January 2019

Risk factors for hospitalization of patients with chikungunya virus infection at sentinel hospitals in Puerto Rico.

PLoS Negl Trop Dis 2019 Jan 14;13(1):e0007084. Epub 2019 Jan 14.

Centers for Disease Control and Prevention, Dengue Branch, San Juan, Puerto Rico.

Background: Hospitalization of patients during outbreaks of chikungunya virus has been reported to be uncommon (0.5-8.7%), but more frequent among infants and the elderly. Read More

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http://dx.doi.org/10.1371/journal.pntd.0007084DOI Listing
January 2019

Clinical Cyanosis in a Patient Presenting for Outpatient Colonoscopy: A Case Report of Hemoglobin Kansas.

A A Pract 2019 Jan 10. Epub 2019 Jan 10.

From the Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University, Baltimore, Maryland.

Gene mutations that affect the synthesis or structure of hemoglobin are present in 7% of the world's population. These mutations lead to variant hemoglobinopathies with phenotypes that range from clinically insignificant to fatal. Because monitoring oxygen saturation is standard of care and critical to detecting hypoxemia, it is crucial to recognize factors that might interfere with the measurement of oxygen saturation or decrease oxygen delivery. Read More

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http://dx.doi.org/10.1213/XAA.0000000000000959DOI Listing
January 2019

A neonate with pneumoperitoneum and cyanosis of lower limbs skin during esophagogastroduodenoscopy.

Dig Liver Dis 2019 Jan 3. Epub 2019 Jan 3.

Department of Internal Medicine, Toyama Prefectural Central Hospital, Toyama, Japan.

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http://dx.doi.org/10.1016/j.dld.2018.12.015DOI Listing
January 2019
1 Read

Complex pulmonary arteriovenous fistula in mother and daughter: Case report.

Medicine (Baltimore) 2019 Jan;98(2):e13922

Department of Cardiology, West China Hospital, Sichuan University, Chengdu.

Rationale: Complex pulmonary arteriovenous fistula (PAVF) is unusual, and even rarer in 2 members of a family. PAVF may not appear on chest X-ray or computed tomography imaging, especially in asymptomatic patients, and therapy is limited. Herein, PAVFs occurring in a mother and daughter are described, with the current standard methods of diagnosis and treatment of PAVF. Read More

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http://dx.doi.org/10.1097/MD.0000000000013922DOI Listing
January 2019

Outcome Of Use Of Nasal Continuous Positive Airway Pressure Through Infant Flow Drivers In Neonates With Respiratory Distress In A Tertiary Care Hospital In Pakistan.

J Ayub Med Coll Abbottabad 2018 Oct-Dec;30(4):511-555

Iqbal Memorial Teaching Hospital/Khawaja Muhammad Safdar Medical College Sialkot, Pakistan.

Background: Nasal continuous positive pressure has been used for management of respiratory distress in neonates in various conditions as a primary modality. Objective of the study is to evaluate the frequency of improved outcome and complications of use of nasal CPAP through infant flow drivers in neonates with respiratory distress. The study was conducted from 2nd April 2017 to 2nd October 2017 in neonatal intensive care unit of Allama Iqbal Memorial Teaching Hospital Sialkot. Read More

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January 2019
1 Read

The mysterious "double" arch.

Echocardiography 2019 Jan 9. Epub 2019 Jan 9.

Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.

A child with cyanosis and heart failure was noted to have two different vascular arches of similar caliber on echocardiography. A systematic analysis of the rhythm and flow patterns revealed the mysterious "double" arch. Read More

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http://dx.doi.org/10.1111/echo.14253DOI Listing
January 2019

Blue Is the Warmest Color: The Effect of Cyanosis and Heart Disease on Risk of Adverse Events During Tracheal Intubation.

Pediatr Crit Care Med 2019 Jan;20(1):82-84

Cardiac Intensive Care, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.

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http://dx.doi.org/10.1097/PCC.0000000000001790DOI Listing
January 2019

Recessive congenital methemoglobinemia type II: Hypoplastic basal ganglia in two siblings with a novel mutation of the cytochrome b5 reductase gene.

Neuroradiol J 2019 Jan 7:1971400918822153. Epub 2019 Jan 7.

Department of Radiology, King Faisal Specialist Hospital and Research Centre, Saudi Arabia.

Recessive congenital methemoglobinemia type II is a very rare autosomal recessive hematologic disorder due to NADH-cytochrome b5 reductase deficiency, usually caused by full-stop mutations or deletions. This disease classically presents with mild neonatal cyanosis, early onset severe progressive developmental delay, movement disorders, and progressive microcephaly. We report two siblings with recessive congenital methemoglobinemia type II whose evaluation revealed a novel p. Read More

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http://journals.sagepub.com/doi/10.1177/1971400918822153
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http://dx.doi.org/10.1177/1971400918822153DOI Listing
January 2019
1 Read

Clofazimine-induced methemoglobinemia: A rare incidence.

J Family Med Prim Care 2018 Nov-Dec;7(6):1573-1575

Department of Medicine, Tata Main Hospital, Jamshedpur, Jharkhand, India.

Clofazimine is commonly used for the treatment of leprosy and chronic use of it can lead to methemoglobinemia, which is a rare but major concern. Iron of hemoglobin remains in the form of ferric (Fe3+) in methemoglobinemia as compared with ferrous form (Fe2+) in normal situation. This transformation prevents oxygen carriage and results in higher level of MetHb in blood which could be dangerous to life. Read More

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http://www.jfmpc.com/text.asp?2018/7/6/1573/246503
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http://dx.doi.org/10.4103/jfmpc.jfmpc_296_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6293906PMC
January 2019
1 Read

[Breve reseña histórica de la primera publicación del síndrome de insuficiencia respiratoria aguda].

Cir Cir 2019 ;87(1):113-122

Instituto Nacional de Ciencias Médicas y Nutrición Dr. Salvador Zubirán, Ciudad de México, México.

Acute respiratory distress syndrome was first described by Ashbaugh, Petty, Levine y Bigellow in 1967 writing in The Lancet. Their study was based on a case series of 12 patients with acute respiratory distress, cyanosis refractory to oxygen therapy, decreased lung compliance and diffuse infiltrates evident on the chest radiograph. Mortality was 58% with greater survival in five patients managed with mechanical ventilation and positive end expiratory pressure. Read More

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http://dx.doi.org/10.24875/CIRU.18000592DOI Listing
January 2019

Terlipressin-Induced Peripheral Cyanosis in a Patient with Liver Cirrhosis and Hepatorenal Syndrome.

Am J Case Rep 2019 Jan 2;20:5-9. Epub 2019 Jan 2.

School of Pharmacy, Master Program in Clinical Pharmacy, College of Pharmacy, Kaohsiung Medical University, Kaohsiung, Taiwan.

BACKGROUND Hepatorenal syndrome (HRS), which is a type of functional renal impairment, is one of the most serious complications in patients with liver cirrhosis. Terlipressin can induce splanchnic vasoconstriction, which increases the renal blood flow and has beneficial effects on HRS. However, terlipressin administration may cause serious ischemic complications such as skin ischemia, peripheral gangrene, and ischemic bowel necrosis. Read More

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http://dx.doi.org/10.12659/AJCR.913150DOI Listing
January 2019
1 Read

Common Arterial Trunk: Physiology, Imaging, and Management.

Semin Cardiothorac Vasc Anesth 2018 Dec 29:1089253218821382. Epub 2018 Dec 29.

3 Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Common arterial trunk (CAT), or truncus arteriosus, is a rare form of cyanotic congenital heart disease and is highly associated with DiGeorge syndrome (microdeletion 22q11.2). Prenatal diagnosis is highly feasible, allowing proper delivery planning and postnatal management. Read More

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http://journals.sagepub.com/doi/10.1177/1089253218821382
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http://dx.doi.org/10.1177/1089253218821382DOI Listing
December 2018
4 Reads

Anomalous Origin of the Right Subclavian Artery in a Patient with D-transposition of the Great Arteries.

Korean J Thorac Cardiovasc Surg 2018 Dec 5;51(6):403-405. Epub 2018 Dec 5.

Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine.

This case report concerns a young patient with an extremely rare combination of d-transposition of the great arteries (d-TGA) and anomalous origin of the right subclavian artery. In our patient, the right subclavian artery originated from the pulmonary artery, which is why he did not show reversed differential cyanosis. We conclude that the presence of an aortic arch anomaly should be considered in patients with d-TGA who do not present with reversed differential cyanosis. Read More

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http://dx.doi.org/10.5090/kjtcs.2018.51.6.403DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6301321PMC
December 2018

Aortopulmonary window in tetralogy of fallot with absent conal septum.

Echocardiography 2018 Dec 25. Epub 2018 Dec 25.

Division of Pediatric Cardiology, Stanford University and University of California San Francisco, San Francisco, California.

Background: Aortopulmonary window is a rare conotruncal defect that is often associated with other congenital heart defects. We present a patient with a previously unreported combination of aortopulmonary window with tetralogy of Fallot with an absent conal septum.

Case Presentation: A term, 2. Read More

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http://dx.doi.org/10.1111/echo.14243DOI Listing
December 2018
1 Read
1.254 Impact Factor

Clinical characteristics and effective treatments of scrub typhus-associated hemophagocytic lymphohistiocytosis in children.

J Adv Res 2019 Jan 18;15:111-116. Epub 2018 Jul 18.

Department of Pediatrics, First Affiliated Hospital of Wenzhou Medical University, Wenzhou 325000, China.

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon and life-threatening disorder that may rarely complicate the clinical course of Orientia tsutsugamushi disease (scrub typhus). Here, we describe the clinical features, laboratory parameters, management, and outcome of 16 children with scrub typhus-associated HLH. All patients satisfied the HLH-2004 diagnostic criteria. Read More

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http://dx.doi.org/10.1016/j.jare.2018.05.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6300568PMC
January 2019
1 Read

Predicted clinical factors associated with the intensive care unit length of stay after total cavopulmonary connection.

J Thorac Cardiovasc Surg 2018 Nov 15. Epub 2018 Nov 15.

Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany; Insure (Institute for Translational Cardiac Surgery), Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany; German Center for Cardiovascular Research, Munich, Germany.

Objectives: A longer length of stay (LOS) in the intensive care unit (ICU) after the total cavopulmonary connection (TCPC) is thought to be a predictive sign of late Fontan failure. This study was performed to determine the clinical risk factors for ICU LOS.

Methods: In total, 483 patients who underwent a TCPC between May 1994 and December 2016 were included the study. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00225223183296
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http://dx.doi.org/10.1016/j.jtcvs.2018.10.144DOI Listing
November 2018
5 Reads

Congenital high airway obstruction with tracheoesophageal fistula: A case report.

Medicine (Baltimore) 2018 Dec;97(51):e13709

Department of Otorhinolaryngology Head and Neck Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, People's Republic of China.

Rationale: Congenital high airway obstruction syndrome (CHAOS) is defined as complete or partial obstruction of the fetal upper airways. Laryngeal atresia is the most frequent cause.

Patient Concern: A male neonate born with poor reactions, weakly spontaneous breathing and cyanosis of the limbs was referred to our hospital. Read More

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http://dx.doi.org/10.1097/MD.0000000000013709DOI Listing
December 2018
1 Read

Methemoglobinemia treated with hyperbaric oxygen therapy: A case report.

Turk J Emerg Med 2018 Dec 13;18(4):176-178. Epub 2018 Apr 13.

Marmara University Pendik Education and Research Hospital, Department of Emergency Medicine, Istanbul, Turkey.

Methemoglobinemia is a rare but clinically important condition that should be considered among differential diagnosis' in cyanosis. The conventional treatments of methemoglobinemia are high flow oxygen and methylene blue therapies. We present a 52-year-old male patient who admitted to emergency room with severe cyanosis and dyspnea after he was exposed to paint thinner and zinc phosphate solution. Read More

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http://dx.doi.org/10.1016/j.tjem.2018.03.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6261104PMC
December 2018
1 Read

Intractable apnoeic seizures in a child with a deletion typically associated with Williams syndrome.

Epileptic Disord 2018 Dec;20(6):530-534

Division of Pediatric Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto.

Williams-Beuren syndrome is rarely associated with epilepsy. One previously reported case showed an association with apnoeic seizures while a few other cases showed an association with infantile epileptic spasms and generalized and focal seizures. We report the case of a 13-month-old boy with a deletion typically associated with Williams-Beuren syndrome, who presented with isolated apnoeic seizures which were refractory to multiple antiepileptic drugs but partially responsive to the ketogenic diet. Read More

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http://dx.doi.org/10.1684/epd.2018.1013DOI Listing
December 2018
1 Read

Extreme exercise dislike of a toddler due to a patent foramen ovale.

Cardiol Young 2018 Dec 11:1-2. Epub 2018 Dec 11.

Fetal and Pediatric Cardiology,University Hospitals Leuven,Herestraat 49, 3000 Leuven,Belgium.

A 5-year-old girl presented with chronic fatigue and extreme exercise intolerance. After countless doctor visits, investigations, and hospital admissions, striking desaturation during exercise test pointed to a cardiovascular problem. Desaturation as a result of right-to-left shunting through a patent foramen ovale during upright exercise was hypothesised. Read More

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http://dx.doi.org/10.1017/S1047951118002044DOI Listing
December 2018
1 Read

Complex Congenital Heart Malformation Including Transposition of the Great Vessels, Presenting as Sudden Unexplained Infant Death.

J Forensic Sci 2018 Nov 28. Epub 2018 Nov 28.

Eastern Virginia Medical School, Physician Assistant Program, 825 Fairfax Avenue, Norfolk, VA, 23507.

Transposition of the great vessels (TGV) is a common congenital heart defect that is difficult to diagnose before birth. Antenatal diagnosis is associated with increased survival. Unusual features such as anomalous pulmonary artery origin may delay cyanosis, decreasing clinical suspicion. Read More

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http://dx.doi.org/10.1111/1556-4029.13967DOI Listing
November 2018
1 Read

Left Atrial Drainage of the Right Superior Vena Cava: A Case Report.

J Tehran Heart Cent 2018 Apr;13(2):84-87

Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran.

An isolated right superior vena cava (RSVC) draining into the left atrium represents a very rare congenital malformation, especially in the absence of a partial anomalous pulmonary venous return. This condition leads to hypoxemia, cyanosis, and clubbing without any other signs of heart defects. We describe an 8-year-old girl, who was referred to our hospital due to unexplained cyanosis. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6246439PMC
April 2018
6 Reads

The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair.

Int J Cardiol 2018 Nov 7. Epub 2018 Nov 7.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK; NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, UK. Electronic address:

Objectives: To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia.

Background: Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging.

Methods: Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Read More

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http://dx.doi.org/10.1016/j.ijcard.2018.11.011DOI Listing
November 2018
2 Reads
4.036 Impact Factor

Acute epiglottitis caused by community-acquired methicillin-resistant in a healthy infant.

Infect Drug Resist 2018 31;11:2063-2067. Epub 2018 Oct 31.

Department of Pediatrics and Child Health, Nihon University School of Medicine, Tokyo, Japan,

was the main causative organism for acute epiglottitis in the pre- type b (Hib) vaccine era. However, with current widespread Hib vaccination, the causative organisms may have changed. Here, we report the case of a healthy infant with acute epiglottitis caused by community-acquired methicillin-resistant (MRSA). Read More

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http://dx.doi.org/10.2147/IDR.S182659DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6219105PMC
October 2018
8 Reads

Anaphylaxis: Ten-year retrospective study from a tertiary-care hospital in Northern Thailand.

Asian Pac J Allergy Immunol 2018 Oct 15. Epub 2018 Oct 15.

Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

Background: The study of anaphylaxis in different geographic areas raises the awareness to improve prevention and medical care.

Objective: To investigate the incidence, causes, characteristics, and management of anaphylaxis in Chiang Mai, Thailand METHODS: We performed a retrospective review, based on ICD-10 electronic medical records of patients who attended the Out-Patient and Emergency Departments at Chiang Mai University Hospital from January 2007 to December 2016.

Results: A total of 441 episodes of anaphylaxis in 433 patients were analyzed. Read More

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http://dx.doi.org/10.12932/AP-210318-0284DOI Listing
October 2018
1 Read

[Anesthetic management for patient with severe cyanosis following bioprosthetic valve stenosis].

Authors:
Jun Zeng Wei Wei

Rev Bras Anestesiol 2018 Nov 13. Epub 2018 Nov 13.

Sichuan University, West China Hospital, Department of Anesthesiology, Chengdu, China. Electronic address:

We presented a 39-year-old female patient with life-threatening hypoxemia after tricuspid valve replacement because of Ebstein's anomaly. And the severe cyanosis is due to bioprosthetic valve stenosis and atrial septal defect. Anesthetic management of a patient with severe obstructive prosthetic valve dysfunction can be challenging. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00347094183003
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http://dx.doi.org/10.1016/j.bjan.2018.06.010DOI Listing
November 2018
8 Reads

A Novel Brain Injury Biomarker Correlates with Cyanosis in Infants with Congenital Heart Disease.

Pediatr Cardiol 2018 Nov 14. Epub 2018 Nov 14.

Division of Pediatric Cardiology, Department of Pediatrics, Cohen Children's Medical Center of New York at Northwell Health, New Hyde Park, NY, 11040, USA.

Cyanotic heart lesions are a complex subset of congenital heart disease (CHD) in which patients are desaturated until surgical repair or palliation. We hypothesized that a direct relationship would exist between degree of desaturation and presence of systemic inflammation and brain injury in unrepaired patients less than 1 year of age. The pre-operative desaturation with augmented systemic inflammation would predict a more complex post-operative course. Read More

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http://link.springer.com/10.1007/s00246-018-2023-4
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http://dx.doi.org/10.1007/s00246-018-2023-4DOI Listing
November 2018
5 Reads

Breath-Holding Spells in Pediatrics: A Narrative Review of the Current Evidence.

Curr Pediatr Rev 2018 Nov 12. Epub 2018 Nov 12.

Department of Paediatrics, The Chinese University of Hong Kong, Shatin. Hong Kong.

Background: Breath-holding spells are common, frightening, but fortunately benign events. Familiarity with this condition is important so that an accurate diagnosis can be made.

Objective: To familiarize physicians with the clinical manifestations, diagnosis, evaluation, and management of children with breath-holding spells. Read More

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http://dx.doi.org/10.2174/1573396314666181113094047DOI Listing
November 2018
1 Read

Cor triatriatum dexter with right ventricular hypoplasia: Role of multimodality imaging in decision making.

Echocardiography 2018 12 9;35(12):2113-2116. Epub 2018 Nov 9.

Cleveland Clinic Children's Hospital, Cleveland, Ohio.

Cor Triatriatum Dexter (CTD) is a rare congenital anomaly involving the systemic venous valves. Failure of regression of the right-sided sinus venosus valve leads to abnormal septation of the right atrium and a variety of right atrial and tricuspid valve obstructive lesions. The presentation can be varied ranging from asymptomatic to persistent neonatal cyanosis. Read More

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http://doi.wiley.com/10.1111/echo.14175
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http://dx.doi.org/10.1111/echo.14175DOI Listing
December 2018
10 Reads

Phlegmasia Cerulea Dolens with Compartment Syndrome.

Ann Vasc Dis 2018 Sep;11(3):355-357

Trauma Unit, Department of Surgery, Faculty of Medicine, Prince of Songkla University, Songkla, Thailand.

Venous thromboembolism (VTE) is a major healthcare problem that results in significant mortality, morbidity, and expenditure of resources. It compounds with pulmonary embolism (PE) and deep vein thrombosis (DVT). Phlegmasia cerulea dolens (PCD) is an uncommon but potentially life-threatening complication of acute DVT characterized by marked swelling of the extremities with pain and cyanosis, which in turn may lead to arterial ischemia and ultimately gangrene with high amputation and mortality rates. Read More

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https://www.jstage.jst.go.jp/article/avd/11/3/11_cr.18-00030
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http://dx.doi.org/10.3400/avd.cr.18-00030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6200621PMC
September 2018
6 Reads

[Clinical characteristics and genetic features of benign infantile epilepsy with PRRT2 mutation].

Zhonghua Er Ke Za Zhi 2018 Nov;56(11):818-823

Department of Neurology, National Center for Children's Health (Beijing) , Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.

To summarize the detailed clinical characteristics and genetic features of benign infantile epilepsy with PRRT2 mutation, in order to improve the understanding of the disease. The clinical data and genetic results of 40 benign infantile epilepsy patients with PRRT2 mutation who were diagnosed and treated in the neurology department of National Center for Children's Health (Beijing) , Beijing Children's Hospital affiliated to Capital Medical University from January 2002 to October 2017 and their affected family members were analyzed. Forty benign infantile epilepsy patients were recruited for this study, with 18 males and 22 females. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2018.11.005DOI Listing
November 2018
5 Reads

Asxl1 exerts an antiproliferative effect on mouse lung maturation via epigenetic repression of the E2f1-Nmyc axis.

Cell Death Dis 2018 Nov 2;9(11):1118. Epub 2018 Nov 2.

Department of Integrative Bioscience and Biotechnology, Sejong University, Seoul, 05006, Korea.

Although additional sex combs-like 1 (ASXL1) has been extensively described in hematologic malignancies, little is known about the molecular role of ASXL1 in organ development. Here, we show that Asxl1 ablation in mice results in postnatal lethality due to cyanosis, a respiratory failure. This lung defect is likely caused by higher proliferative potential and reduced expression of surfactant proteins, leading to reduced air space and defective lung maturation. Read More

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http://www.nature.com/articles/s41419-018-1171-z
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http://dx.doi.org/10.1038/s41419-018-1171-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6215009PMC
November 2018
4 Reads

Myh10 deficiency leads to defective extracellular matrix remodeling and pulmonary disease.

Nat Commun 2018 11 2;9(1):4600. Epub 2018 Nov 2.

Department of Developmental Genetics, Max Planck Institute for Heart and Lung Research, Bad Nauheim, 61231, Germany.

Impaired alveolar formation and maintenance are features of many pulmonary diseases that are associated with significant morbidity and mortality. In a forward genetic screen for modulators of mouse lung development, we identified the non-muscle myosin II heavy chain gene, Myh10. Myh10 mutant pups exhibit cyanosis and respiratory distress, and die shortly after birth from differentiation defects in alveolar epithelium and mesenchyme. Read More

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http://www.nature.com/articles/s41467-018-06833-7
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http://dx.doi.org/10.1038/s41467-018-06833-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6214918PMC
November 2018
13 Reads
10.742 Impact Factor

Case 4: Atypical Cause of Cyanosis While Bathing in an 18-month-old Boy.

Pediatr Rev 2018 Nov;39(11):568-569

Department of Pediatrics at McGovern Medical School, Memorial Hermann Children's Hospital, Houston TX.

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http://dx.doi.org/10.1542/pir.2017-0159DOI Listing
November 2018
1 Read

LEIGH SYNDROME: A CASE REPORT WITH A MITOCHONDRIAL DNA MUTATION.

Rev Paul Pediatr 2018 Oct-Dec;36(4):519-523. Epub 2018 Oct 29.

Centro Hospitalar do Médio Ave, Nova de Famalicão, Portugal.

Objective: Leigh syndrome is a neurodegenerative disorder with an incidence of 1:40,000 live births. It presents wide clinical, biochemical, and genetic heterogeneity, but with homogenous neuropatoradiological alterations. There is no specific treatment, and the prognosis is reserved. Read More

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http://dx.doi.org/10.1590/1984-0462/;2018;36;4;00003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322804PMC
October 2018
15 Reads

Outcomes and swallowing evaluations after injection laryngoplasty for type I laryngeal cleft: Does age matter?

Int J Pediatr Otorhinolaryngol 2018 Dec 13;115:10-18. Epub 2018 Sep 13.

Paul C. Gaffney Division of Pediatric Hospital Medicine, UPMC Children's Hospital of Pittsburgh, USA; University of Pittsburgh School of Medicine, USA. Electronic address:

Objectives: To improve the recognition of differences in presentation amongst patients with type 1 laryngeal clefts of various ages and better understand the age dependent outcomes of injection laryngoplasty. A second aim was to analyze the discrepancies between swallow assessment modalities in various age groups with type I laryngeal clefts undergoing injection laryngoplasty.

Methods: A retrospective review of electronic medical records of patients who underwent injection laryngoplasty from 2009 through 2015 at a tertiary care children's hospital. Read More

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http://dx.doi.org/10.1016/j.ijporl.2018.09.006DOI Listing
December 2018
1 Read

Clinical and molecular characterization of Korean children with infantile and late-onset Pompe disease: 10 years of experience with enzyme replacement therapy at a single center.

Korean J Pediatr 2018 Oct 23. Epub 2018 Oct 23.

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.

Purpose: Pompe disease (PD) is an autosomal recessive disorder caused by a deficiency of acid alpha-glucosidase resulting from pathogenic GAA variants. The present study described clinical features, genotypes, changes before and after enzyme replacement therapy (ERT), and long-term outcomes in patients with infantile-onset PD (IOPD) and late-onset PD (LOPD) in one tertiary medical center.

Methods: Five Korean patients (two males and three females) diagnosed with PD between 2002 and 2013 at Samsung Medical Center in Seoul, Republic of Korea. Read More

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http://kjp.or.kr/journal/view.php?doi=10.3345/kjp.2018.06968
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http://dx.doi.org/10.3345/kjp.2018.06968DOI Listing
October 2018
10 Reads

Changes in corneal thickness in patients with high-altitude pulmonary edema after systemic oxygen therapy.

Indian J Ophthalmol 2018 Nov;66(11):1554-1557

Department of Community Medicine, Army College of Medical Sciences, New Delhi, India.

Purpose: High-altitude pulmonary edema (HAPO) is an acute medical emergency occurring typically in lowlanders, who ascend rapidly to heights of 3000 m or more. It presents with marked dyspnea on exertion, fatigue with minimal-to-moderate effort, prolonged recovery time, and dry cough with manifestations of cyanosis, tachycardia, tachypnea, and temperature which generally does not increase beyond 38.5°C. Read More

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http://www.ijo.in/text.asp?2018/66/11/1554/244073
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http://dx.doi.org/10.4103/ijo.IJO_642_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6213672PMC
November 2018
4 Reads

Anomalous Connection of the Right Superior Vena Cava to the Left Atrium in a Child with Bilateral Superior Vena Cavae: An Unusual Cause of Cyanosis.

Authors:
Saad Khoshhal

Pediatr Cardiol 2018 Oct 23. Epub 2018 Oct 23.

Department of Pediatric Cardiology, College of Medicine, Taibah University, Madinah, Kingdom of Saudi Arabia.

Anomalous drainage of the right superior vena cava into the left atrium (LA) has been previously reported in the medical literature. We present a unique case of a child who had two superior vena cavae, a left superior vena cava that drained normally through the coronary sinus to the right atrium, and a right superior vena cava that drained anomalously to the LA. Read More

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http://dx.doi.org/10.1007/s00246-018-2013-6DOI Listing
October 2018
1 Read

High incidence of ductal closure or narrowing at birth in patients with right ventricular outflow tract obstruction with normal orientation of the ductus arteriosus.

Cardiol Young 2019 Jan 24;29(1):54-58. Epub 2018 Oct 24.

1Department of Pediatrics,Graduate School of Medical Sciences,Kyushu University,Fukuoka,Japan.

Background: Ductal patency is mandatory to manage patients with ductal-dependent pulmonary circulation. The aim of this study is to elucidate the morphological and haemodynamic features of ductus arteriosus with right ventricular outflow tract obstruction, and investigate the appropriate perinatal management.Patients and methodsPatients with prenatal diagnosis of right ventricular outflow tract obstruction at our institution between 2010 and 2015 were included in the study. Read More

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http://dx.doi.org/10.1017/S1047951118001798DOI Listing
January 2019
1 Read

Iron deficiency and cyanotic breath-holding spells: The effectiveness of iron therapy.

Pediatr Hematol Oncol 2018 Oct 23:1-10. Epub 2018 Oct 23.

c Department of Clinical Pathology , Assiut University Hospital , Assiut , Egypt.

Aim: Frequent cyanotic breath holding spells cause fear and severe anxiety to parents. This study aimed to evaluate clinical, laboratory and treatment characteristics of children with cyanotic breath holding spells.

Methods: Included were 180 children (mean age: 1. Read More

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http://dx.doi.org/10.1080/08880018.2018.1491659DOI Listing
October 2018
12 Reads

Superior vena cava syndrome in a patient with locally advanced lung cancer with good response to definitive chemoradiation: a case report.

J Med Case Rep 2018 Oct 20;12(1):301. Epub 2018 Oct 20.

Department of Radiation Oncology, Indiana University School of Medicine, 535 Barnhill Drive, Cancer Care Pavilion 041, Indianapolis, IN, 46202-5289, USA.

Background: The incidence of superior vena cava syndrome within the United States is roughly 15,000 cases per year. Superior vena cava syndrome is a potentially life-threatening medical condition; however, superior vena cava syndrome is not fatal in the majority of cases. Superior vena cava syndrome encompasses a collection of signs and symptoms resulting from obstruction of the superior vena cava, including swelling of the upper body of the head, neck, arms, and/or breast. Read More

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https://jmedicalcasereports.biomedcentral.com/articles/10.11
Publisher Site
http://dx.doi.org/10.1186/s13256-018-1843-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6195746PMC
October 2018
7 Reads

Congenital bilateral dacryocystocele.

Eur Ann Otorhinolaryngol Head Neck Dis 2018 Oct 15. Epub 2018 Oct 15.

Hospital Pedro Hispano, Unidade Local de Saúde de Matosinhos Otorhinolaryngology, Travessa do Padre Amaro, number 169/229, Matosinhos 4455-033, Portugal.

Introduction: Newborns are obligatory nasal breathers. Therefore, nasal obstruction can lead to cyanosis and desaturation. In spite of being very rare, congenital bilateral dacryocystocele is a possible etiology for neonatal respiratory distress. Read More

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http://dx.doi.org/10.1016/j.anorl.2017.10.006DOI Listing
October 2018
1 Read

Perioperative management of critical right ventricular inflow obstruction from right atrial rhabdomyoma.

Ann Card Anaesth 2018 Oct-Dec;21(4):430-432

Manchester Academic Health Science Centre, Cardiothoracic Anaesthesia and Intensive Care, Manchester University, Manchester University NHS Foundation Trust, Manchester, UK; Department of Anaesthesiology and Intensive Care, The Prince Charles Hospital, The University of Queensland, Critical Care Research Group, Brisbane, Australia; Department of Anaesthesiology and Intensive Care, Philipps University, Marburg, Germany.

Rhabdomyoma is the most common cardiac tumor in infancy and commonly located in the ventricles causing outflow obstruction or arrhythmias. We report a rare pediatric (7 month old) case of a right atrial rhabdomyoma presenting with severe cyanosis and low cardiac output from significant tricuspid inflow obstruction with right to left shunt across a stretched patent foramen ovale. We present an emergency cardiac surgery for right atrial tumor resection, and the management of separating the patient with failing right ventricle from cardiopulmonary bypass using a Glenn shunt, since extracorporeal membrane oxygenation (ECMO) or nitric oxide was not available. Read More

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http://www.annals.in/text.asp?2018/21/4/430/243539
Publisher Site
http://dx.doi.org/10.4103/aca.ACA_233_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6206783PMC
October 2018
17 Reads

[Postoperative abnormal liver function in children with heart surgery].

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2018 Sep;43(9):1007-1013

Department of Cardiovascular Surgery, Xiangya Hospital, Central South University, Changsha 410008, China.

Objective: To investigate the incidence of postoperative abnormal liver function test (aLFT) for the children with heart surgery, and to analyze the clinical characteristics and risk factors.
 Methods: A total of 143 children younger than 18 years old who underwent heart surgery in 2017 were enrolled in this study. The liver function were examined one day preoperation and consecutive 5 days after operation. Read More

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http://dx.doi.org/10.11817/j.issn.1672-7347.2018.09.012DOI Listing
September 2018
6 Reads

[Venous Thromboembolism in Pediatric Age: A 15 Year Retrospective Review].

Acta Med Port 2018 Sep 28;31(9):489-495. Epub 2018 Sep 28.

Serviço de Pediatria. Centro Hospitalar Tondela-Viseu. Viseu, Portugal.

Introduction: Pulmonary thromboembolism and deep venous thrombosis occur in pediatric age, with unknown incidence, morbidity and mortality. Our aim is to review the epidemiology, clinical presentation, complementary diagnostic tests and prognosis of patients with pulmonary thromboembolism and deep venous thrombosis.

Material And Methods: Retrospective, descriptive and analytical study of pediatric patients admitted to a Level II hospital for pulmonary thromboembolism and deep venous thrombosis, between 2000 and 2014. Read More

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http://dx.doi.org/10.20344/amp.9639DOI Listing
September 2018
1 Read

Non-traumatic acute ischemia of the lower limbs: Report of 112 cases.

Tunis Med 2018 Mar;96(3):178-181

Introduction: Acute limb ischemia is a common pathology. Etiological diagnosis is not always easy to make, and the prognosis depends mainly on the underlying cause and time management.

Aim: To report the experience of our service, and analyze the different aspects of ischemia and their support in the light of a review of the literature. Read More

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March 2018
10 Reads

Investigations for infants admitted with apparent life-threatening event (ALTE): Necessity or liability? Assessment at a tertiary care hospital.

J Pak Med Assoc 2018 Oct;68(10):1513-1516

Department of Pediatrics, King Abdullah Specialist Children Hospital, Riyadh, Saudi Arabia, and King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.

The retrospective study was planned to assess the outcome of extensive investigations done for infants admitted with apparent life-threatening event (ALTE), and comprised chart review of 275 infants admitted to King Abdul-Aziz Medical City (KAMC), Riyadh, Saudi Arabia, between 2012 and 2015. The outcome of each investigation was compared in terms of whether the test was performed or not performed, and, if performed, whether the test report was normal or abnormal. Also, sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) was assessed for each test. Read More

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October 2018
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Single-centre experience in stenting arterial shunts for adult CHD patients with single-ventricle physiology and pulmonary blood flow dependent on arterial shunts.

Cardiol Young 2018 Dec 12;28(12):1431-1435. Epub 2018 Oct 12.

Department of Congenital Cardiology,Leeds General Infirmary,Leeds,UK.

Objectives: The objectives of this study were to describe the approach to stenting arterial shunts in adult congenital patients with single-ventricle physiology and to assess the medium-term clinical and haemodynamic outcomes following stent insertion.

Background: Adult patients with single-ventricle physiology and pulmonary blood flow dependent on a surgically placed arterial shunt who did not progress to venous palliation are extremely challenging to manage. Progressive cyanosis secondary to narrowing of the shunt has a marked impact on exercise tolerance and results in intolerable well-being for these patients. Read More

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https://www.cambridge.org/core/product/identifier/S104795111
Publisher Site
http://dx.doi.org/10.1017/S1047951118001464DOI Listing
December 2018
2 Reads