keyword
https://read.qxmd.com/read/38497171/stroke-without-cerebral-arteriopathy-in-sickle-cell-disease-children-causes-and-treatment
#1
JOURNAL ARTICLE
Sarah Liane Linguet, Suzanne Verlhac, Florence Missud, Laurent Holvoet-Vermaut, Valentine Brousse, Ghislaine Ithier, Alexandra Ntorkou, Emmanuelle Lesprit, Malika Benkerrou, Manoëlle Kossorotoff, Berengere Koehl
Cerebral arteriopathy (CA) in children with sickle cell disease (SCD) is classically described as chronic stenosis of arteries in the anterior brain circulation, leading to ischemic stroke. Some studies have however reported strokes in children with SCD but without CA. In order to better understand the etiology and risk factors of these strokes, we retrospectively analyzed ischemic strokes occurring in a large cohort of children over a 13 year-period. Between 2007 and 2020, 25/1500 children with SCD had an ischemic stroke in our center...
March 14, 2024: Haematologica
https://read.qxmd.com/read/37465032/acute-chest-syndrome
#2
JOURNAL ARTICLE
Patrick Meloy, Daniel R Rutz, Amit Bhambri
AUDIENCE: Emergency medicine residents and medical students on emergency medicine rotations. INTRODUCTION: Acute chest syndrome is a life-threatening, potentially catastrophic complication of sickle cell disease.1,2 It occurs in approximately 50% of patients with sickle cell disease, with up to 13% all-cause mortality.1 Most common in children aged 2-4, up to 80% of patients with a prior diagnosis of acute chest syndrome will have recurrence of this syndrome.4 Diagnostic criteria include a new infiltrate on pulmonary imaging combined with any of the following: fever > 38...
January 2023: Journal of education & teaching in emergency medicine
https://read.qxmd.com/read/37215593/clinical-application-of-indocyanine-green-fluorescence-imaging-navigation-for-pediatric-renal-cancer
#3
JOURNAL ARTICLE
Jun Feng, Wei Yang, Hong Qin, Jiatong Xu, Shan Liu, Jianyu Han, Ning Li, Lejian He, Huanmin Wang
BACKGROUND: Indocyanine Green (ICG) fluorescence imaging has been widely used in the surgical treatment of adult renal cancers, but its application in pediatric renal cancers has rarely been reported. This study aims to summarize the experience of ICG fluorescence imaging in pediatric renal cancers and explores its safety and feasibility. METHODS: The clinical features, surgical information, ICG administration regimen, near infrared radiography data in vivo and ex vivo and pathological results of children with renal cancers using ICG navigation were analyzed and summarized...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/36990700/-chinese-experts-consensus-statement-diagnosis-and-treatment-of-cystic-fibrosis-2023
#4
JOURNAL ARTICLE
(no author information available yet)
Cystic fibrosis (CF) is one of the most common autosomal recessive genetic diseases in Caucasians, but CF patients in China are rare, and it was listed as the first batch of rare diseases in China in 2018. In recent years, CF has been gradually recognized in China, and the number of CF patients reported in China in the past 10 years is more than 2.5 times the total number in the previous 30 years, and the total number of CF patients is estimated to be more than 20 000. The research progress of CF gene modification has led to the innovation of CF treatment...
April 12, 2023: Chinese Journal of Tuberculosis and Respiratory Diseases
https://read.qxmd.com/read/36876242/cerebral-fat-embolism-syndrome-in-a-patient-with-homozygous-sickle-cell-disease-in-the-setting-of-multisystem-inflammatory-syndrome-in-children
#5
Kirstin Sepulveda, Tesneem Issa, Gueorgui Dubrocq
Multisystem inflammatory syndrome in children (MIS-C) is a rare progressive inflammatory process temporally associated with exposure to SARS-CoV-2 (COVID-19) in patients 20 years of age and younger. At this time, much of MIS-C is not well understood, including the pathogenesis, long-term implications, and how each variant of the COVID-19 virus affects the progression and severity. We present the unusual case of a 19-year-old man with a history of homozygous sickle cell disease who developed a vaso-occlusive pain crisis and cerebral fat embolism syndrome as a complication of MIS-C secondary to the Omicron variant of COVID-19...
2023: Proceedings of the Baylor University Medical Center
https://read.qxmd.com/read/35494947/renin-angiotensin-blockade-reduces-readmission-for-acute-chest-syndrome-in-sickle-cell-disease
#6
JOURNAL ARTICLE
Nneoma Wamkpah, Anuj Shrestha, Gary Salzman, Stephen Simon, Sahil Suman, Alan Poisner, Agostino Molteni
Rationale Acute chest syndrome (ACS) is a life-threatening complication of sickle cell disease (SCD). Current treatment is supportive-supplemental oxygen, transfusions, and antibiotics. Prevention of ACS may reduce morbidity and mortality in patients with SCD. Acute chest syndrome appears similar to pulmonary fat embolism (PFE), a complication of severe skeletal trauma or orthopedic procedures from pulmonary micro-vessel blockage by bone marrow fat. Vascular obstruction and bone marrow necrosis occur in PFE and ACS...
March 2022: Curēus
https://read.qxmd.com/read/33908741/peritoneovenous-shunt-for-intractable-ascites-in-children-a-series-of-4-cases
#7
JOURNAL ARTICLE
Coralie Defert, Jean-Baptiste Marret, Florence Lacaille, Samira Sissaoui, Laurent Dupic, Hubert Lardy, Carmen Capito, Christophe Chardot
Intractable ascites is a rare condition in children mainly caused by cirrhosis or lymphatic disorders. Internal drainage may be considered as rescue therapy. In our department, 4 patients ages from 2 months to 15 years old underwent a peritoneovenous shunt (PVS) placement between 2010 and 2020. The surgically inserted device was a pumping device that enabled to drain ascites from the peritoneum into the venous system via the internal jugular vein (Denver shunt, BD Company, NJ). Immediate efficient drainage was achieved in all cases and lasted up to 9 years...
July 1, 2021: Journal of Pediatric Gastroenterology and Nutrition
https://read.qxmd.com/read/32282316/-maternal-mortality-hospital-profesor-alejandro-posadas-buenos-aires-evolution-2003-2015
#8
JOURNAL ARTICLE
Isabel Miceli, María Elena Borda, Roberto Casale, Alberto Ferreirós
We reviewed the medical records of women with maternal death reported from August 2003 to December 2015 in the Posadas Hospital (Buenos Aires Province, Argentina), and calculated indexes, trends and years of potential life lost. A total of 52 cases fulfilled the criteria of maternal death. The information provided by death certificates was meager. Two deaths were incidental: one occurred post-caesarean section and was caused by fat embolism following liquid silicone breast injection, and the other was the consequence of femicide which involved also the 24-week fetus...
2020: Medicina
https://read.qxmd.com/read/32110249/fat-embolism-fat-embolism-syndrome-and-the-autopsy
#9
REVIEW
Christopher M Milroy, Jacqueline L Parai
Fat embolism is common following trauma and is a common autopsy finding in these cases. It may also be seen in non-traumatic cases and is seen in children as well as adults. In comparison fat embolism syndrome (FES) only occurs in a small number of trauma and non-trauma cases. Clinical diagnosis is based on characteristic clinical and laboratory findings. Fat embolism exerts its effect by mechanical blockage of vessels and/or by biochemical means including breakdown of fat to free fatty acids causing an inflammatory response...
September 2019: Academic Forensic Pathology
https://read.qxmd.com/read/31237663/-respiratory-failure-and-neurological-impairment-in-a-child-with-duchenne-muscular-dystrophy-following-minor-trauma
#10
Helena Wihlborg, Sven Wiklund, Emelie Styring
Fat Embolism Syndrome (FES) is a rare and often lethal condition, associated with trauma or surgery. It is more serious than a typical fat embolism seen after a fracture in a long bone. FES is a triad of symtoms, including respiratory failure, abnormal neurology and petechial bleeding. FES is a diagnosis of exclusion. The incidence of FES is higher among adults compared to children. One possible explanation for this is the greater proportion of fat in the adult bone marrow. Children with Duchennes muscular dystrophy have a significantly increased risk of FES compared to both other children and adults...
June 18, 2019: Läkartidningen
https://read.qxmd.com/read/29279787/acute-chest-syndrome-in-children-with-sickle-cell-disease
#11
REVIEW
Shilpa Jain, Nitya Bakshi, Lakshmanan Krishnamurti
Acute chest syndrome (ACS) is a frequent cause of acute lung disease in children with sickle cell disease (SCD). Patients may present with ACS or may develop this complication during the course of a hospitalization for acute vaso-occlusive crises (VOC). ACS is associated with prolonged hospitalization, increased risk of respiratory failure, and the potential for developing chronic lung disease. ACS in SCD is defined as the presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray...
December 1, 2017: Pediatric Allergy, Immunology, and Pulmonology
https://read.qxmd.com/read/29169714/-idiopathic-nephrotic-syndrome
#12
JOURNAL ARTICLE
O Boyer, V Baudouin, E Bérard, C Dossier, V Audard, V Guigonis, I Vrillon
Nephrotic syndrome (NS) is defined by massive proteinuria and hypoalbuminemia, with resulting hyperlipidemia and edema. The most common cause of NS in children is idiopathic nephrotic syndrome (INS), also called nephrosis. Its annual incidence has been estimated to 1-4 per 100,000 children and varies with age, race, and geography. Many agents or conditions have been reported to be associated with INS such as infectious diseases, drugs, allergy, vaccinations, and malignancies. The disease may occur during the 1st year of life, but it usually starts between the ages of 2 and 7 years...
December 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://read.qxmd.com/read/27524390/why-are-some-patients-with-duchenne-muscular-dystrophy-dying-young-an-analysis-of-causes-of-death-in-north-east-england
#13
JOURNAL ARTICLE
H J A Van Ruiten, C Marini Bettolo, T Cheetham, M Eagle, H Lochmuller, V Straub, K Bushby, M Guglieri
INTRODUCTION: Duchenne muscular dystrophy (DMD) is the most common inherited muscle disease in children. Recent years have seen an increase in age of survival into adulthood following the introduction of proactive standards of care. We reviewed mortality in DMD in our population in order to identify potential underlying risk factors for premature death and improve clinical care. METHOD: A retrospective case note review of all deaths in the DMD population over the last 10 years in North East England...
November 2016: European Journal of Paediatric Neurology: EJPN
https://read.qxmd.com/read/26622143/spontaneous-ophthalmic-artery-occlusion-in-children-due-to-hyperhomocysteinemia
#14
JOURNAL ARTICLE
Virender Sachdeva, Ravi Garg, Avinash Pathengay, Ramesh Kekunnaya
Ophthalmic artery occlusion usually presents as a sudden onset profound decrease in vision in the middle-aged and elderly patients following periocular procedures (retrobulbar injection/glabellar fat injection), embolism from the heart or after prolonged systemic surgery. In this report, we describe three children with spontaneous ophthalmic artery occlusion who presented with unilateral loss of vision and diagnosed elsewhere as optic atrophy whose detailed history and examination were suggestive of ophthalmic artery occlusion...
May 2015: Oman Journal of Ophthalmology
https://read.qxmd.com/read/25459015/chylothorax-and-chylous-ascites-management-and-pitfalls
#15
REVIEW
Juan C Lopez-Gutierrez, Juan A Tovar
Leakage of lymph from the lymphatic ducts causes chylothorax (CT) or chylous ascitis (CA). This may happen for unknown reasons during fetal life or after birth and may also be caused by trauma after thoracic surgery or by other conditions. Fetal CT and CA may be lethal particularly in cases with fetal hydrops that sometimes benefit of intra-uterine instrumentation. After birth, symptoms are related to the amount of accumulated fluid. Sometimes, severe cardio-respiratory compromise prompts active therapy. Most patients with CT or CA benefit from observation, rest, and supportive measures alone...
October 2014: Seminars in Pediatric Surgery
https://read.qxmd.com/read/25102805/a-case-of-tuberculosis-cutis-colliquativa-treated-with-rifampicin-and-isoniazid
#16
LETTER
Ada Lo Schiavo, Roberto Cozzi, Rossella Alfano, Francesca Romano, Maddalena La Montagna, Tobia Caccavale, Raffaella Capasso, Eugenia Elisabetta Mea, Stefano Caccavale
No abstract text is available yet for this article.
2014: Acta Dermatovenerologica Croatica: ADC
https://read.qxmd.com/read/23431527/fat-embolism-syndrome-in-an-adolescent-before-surgical-treatment-of-an-isolated-closed-tibial-shaft-fracture
#17
JOURNAL ARTICLE
Cayce B Nawaf, Derek M Kelly, William C Warner, James H Beaty, Leslie Rhodes, Jeffrey R Sawyer
Fat embolism syndrome (FES) occurs most commonly in adults with high-energy trauma, especially fractures of the long-bones and pelvis. Because of unique age-related physiologic differences in the immature skeleton, as well as differences in fracture management in pediatric patients, FES is rare in children. To our knowledge, this is the first case report of FES occurring before surgical fixation of a closed tibial shaft fracture in an adolescent. A 16-year-old, 109 kg, Caucasian adolescent boy developed FES after closed diaphyseal fractures of the distal tibia and fibula, showing signs of respiratory distress and mental status changes...
December 2012: American Journal of Orthopedics
https://read.qxmd.com/read/23217369/fatal-embolic-events-in-childhood
#18
REVIEW
Roger W Byard
Lethal embolic events in children are an uncommon occurrence that may be first detected at autopsy. Emboli consist of thrombi, tumours, infective organisms, fat, air and foreign body material. The most significant circulations that may be obstructed are the pulmonary, coronary and cerebral. Rare conditions such as arteriovenous malformations may act as sources for emboli in children, and the brain is at particular risk from paradoxical embolism. The latter is facilitated by right to left communications in the heart such as atrial and ventricular septal defects and also from pulmonary vascular abnormalities such as hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) and arteriovenous fistulas...
January 2013: Journal of Forensic and Legal Medicine
https://read.qxmd.com/read/21406723/how-i-treat-acute-chest-syndrome-in-children-with-sickle-cell-disease
#19
REVIEW
Scott T Miller
Acute chest syndrome describes new respiratory symptoms and findings, often severe and progressive, in a child with sickle cell disease and a new pulmonary infiltrate. It may be community-acquired or arise in children hospitalized for pain or other complications. Recognized etiologies include infection, most commonly with atypical bacteria, and pulmonary fat embolism (PFE); the cause is often obscure and may be multifactorial. Initiation of therapy should be based on clinical findings. Management includes macrolide antibiotics, supplemental oxygen, modest hydration and often simple transfusion...
May 19, 2011: Blood
https://read.qxmd.com/read/19727925/transcatheter-closure-of-a-left-circumflex-coronary-artery-fistula-in-two-children-using-the-amplatzer-vascular-plug
#20
JOURNAL ARTICLE
Chi-Di Liang, Sheung-Fat Ko, Ying-Jui Lin, Chih Yuan Fang
A left circumflex coronary artery fistula (CAF) is a rare anomaly. This report describes two young children with progressive left coronary artery dilation due to left CAF demonstrated by serial echocardiography. Cardiac catheterization performed for both children confirmed the presence of a markedly tortuous and dilated left circumflex artery, with the CAF draining directly into the right ventricle. Transcatheter closure of the CAF using the Amplatzer vascular plug was successfully accomplished without any complications...
November 2009: Pediatric Cardiology
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