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Central nervous system complications and management in sickle cell disease.
DeBaun MR, Kirkham FJ
(2016) Blood 127: 829-38
MeSH Terms: Acute Disease, Adolescent, Adult, Age Factors, Anemia, Sickle Cell, Brain Infarction, Cerebral Hemorrhage, Chronic Disease, Headache Disorders, Humans, Male
Show Abstract · Added July 20, 2016
With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ∼11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA (3% and 10%, respectively). In high-income countries, RCTs (Stroke Prevention in Sickle Cell Anemia [STOP], STOP II) have demonstrated that regular blood transfusion therapy (typically monthly) achieves primary stroke prevention in children with SCA and high transcranial Doppler (TCD) velocities; after at least a year, hydroxycarbamide may be substituted (TCD With Transfusions Changing to Hydroxyurea [TWiTCH]). Also in high-income countries, RCTs have demonstrated that regular blood transfusion is the optimal current therapy for secondary prevention of infarcts for children with SCA and strokes (Stroke With Transfusions Changing to Hydroxyurea [SWiTCH]) or silent cerebral infarcts (Silent Infarct Transfusion [SIT] Trial). For adults with SCD, CNS complications continue to be a major cause of morbidity and mortality, with no evidence-based strategy for prevention.
© 2016 by The American Society of Hematology.
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11 MeSH Terms
Risk-benefit analysis of lumbar puncture to evaluate for nontraumatic subarachnoid hemorrhage in adult ED patients.
Migdal VL, Wu WK, Long D, McNaughton CD, Ward MJ, Self WH
(2015) Am J Emerg Med 33: 1597-601
MeSH Terms: Adolescent, Adult, Aged, Aged, 80 and over, Emergency Service, Hospital, Female, Headache, Hospitalization, Humans, Male, Middle Aged, Retrospective Studies, Risk Assessment, Spinal Puncture, Subarachnoid Hemorrhage, Tomography, X-Ray Computed, Young Adult
Show Abstract · Added April 6, 2017
OBJECTIVE - The objective of the study is to compare the risks and benefits of lumbar puncture (LP) to evaluate for subarachnoid hemorrhage (SAH) after a normal head computed tomographic (CT) scan.
METHODS - This was an observational study of adult emergency department patients at a single hospital who presented with headache and underwent LP after a normal head CT to evaluate for SAH. Lumbar puncture results classified as indicating a SAH included xanthochromia in cerebrospinal fluid (CSF) or red blood cells in the final tube of CSF with an aneurysm or arteriovenous malformation on cerebral angiography. An LP-related complication was defined as hospitalization or a return visit due to symptoms attributed to the LP. Proportions of the study patients who had SAH diagnosed by LP and who experienced an LP-related complication were compared.
RESULTS - The study included 302 patients, including 2 (0.66%) who were diagnosed with SAH based on LP (number needed to diagnose, 151); both of these patients had a known intracranial aneurysm. Eighteen (5.96%) patients experienced an LP-related complication (P < .01 compared with number with SAH diagnosed; number needed to harm, 17). Complications included 12 patients with low-pressure headaches, 4 with pain at the LP site, and 2 with contaminated CSF cultures.
CONCLUSION - The yield of LP for diagnosing SAH in adults with nontraumatic headache after a normal head CT was very low. The severity of LP-related complications was low, but complications were more common than SAH diagnoses. Lumbar puncture may not be advisable after a normal head CT to evaluate for SAH, particularly in patients with low-risk clinical features for SAH.
Copyright © 2015 Elsevier Inc. All rights reserved.
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17 MeSH Terms
Reply: To PMID 24529619.
DeBaun MR, Dowling MM
(2014) J Pediatr 165: 646
MeSH Terms: Anemia, Sickle Cell, Cerebral Infarction, Female, Headache, Hemoglobins, Humans, Male, Migraine Disorders
Added October 7, 2014
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8 MeSH Terms
Annals of Emergency Medicine journal club. Do more rules make us safer? Clinical decision rules, patient safety, and the role of emergency physicians in health care: answers to the January 2014 journal club questions.
Gupta M, Barrett TW, Schriger DL
(2014) Ann Emerg Med 63: 774-81
MeSH Terms: Decision Support Techniques, Female, Headache Disorders, Primary, Humans, Male, Radiography, Subarachnoid Hemorrhage
Added January 20, 2015
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7 MeSH Terms
Acute myopia and angle closure glaucoma from topiramate in a seven-year-old: a case report and review of the literature.
Rapoport Y, Benegas N, Kuchtey RW, Joos KM
(2014) BMC Pediatr 14: 96
MeSH Terms: Anticonvulsants, Child, Diagnosis, Differential, Female, Fructose, Glaucoma, Angle-Closure, Headache, Humans, Myopia, Seizures, Topiramate
Show Abstract · Added March 19, 2018
BACKGROUND - A case is reported of acute bilateral myopia and angle closure glaucoma in a 7-year-old patient from topiramate toxicity. This is the second known reported case of topiramate induced acute angle closure glaucoma and third known reported case of topiramate induced acute myopia in a pediatric patient.
CASE PRESENTATION - This case presents a 7-year-old who had recently begun topiramate therapy for seizures and headache. She developed painless blurred vision and acute bilateral myopia, which progressed to acute bilateral angle closure glaucoma. After a routine eye exam where myopia was diagnosed, the patient presented to the emergency room with symptoms of acute onset blurry vision, tearing, red eyes, swollen eyelids, and photophobia. The symptoms, myopia, and angle closure resolved with topical and oral intraocular pressure lowering medications, topical cyclopentolate, and discontinuation of topiramate.
CONCLUSION - Acute angle closure glaucoma is a well-known side effect of topiramate, but is rarely seen in children. It cautions providers to the potential ophthalmic side effects of commonly used medications in the pediatric population. It highlights the need to keep a broad differential in mind when encountering sudden onset blurry vision in the primary care clinic, the need for careful consideration of side effects when starting topiramate therapy in a child, and the need for parental counseling of side effects.
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11 MeSH Terms
Annals of Emergency Medicine Journal Club. Measuring and explaining computed tomography use in the United States and Canada: a consideration of health economics and use versus appropriateness, and interpreting potential conflict of interest: answers to the November 2013 Journal Club questions.
Schriger DL, Callaham ML, Barrett TW
(2014) Ann Emerg Med 63: 479-89
MeSH Terms: Abdominal Pain, Dyspnea, Emergency Service, Hospital, Female, Headache, Humans, Male, Tomography, X-Ray Computed
Added January 20, 2015
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8 MeSH Terms
Headache and migraine in children with sickle cell disease are associated with lower hemoglobin and higher pain event rates but not silent cerebral infarction.
Dowling MM, Noetzel MJ, Rodeghier MJ, Quinn CT, Hirtz DG, Ichord RN, Kwiatkowski JL, Roach ES, Kirkham FJ, Casella JF, DeBaun MR
(2014) J Pediatr 164: 1175-1180.e1
MeSH Terms: Adolescent, Anemia, Sickle Cell, Biomarkers, Blood Transfusion, Cerebral Infarction, Child, Child, Preschool, Cross-Sectional Studies, Female, Headache, Hemoglobins, Humans, Logistic Models, Magnetic Resonance Imaging, Male, Migraine Disorders, Multivariate Analysis, Pain, Recurrence, Risk Factors, Severity of Illness Index
Show Abstract · Added February 19, 2014
OBJECTIVE - To identify risk factors for headache and migraine in children with sickle cell disease and test the hypothesis that either or both are independently associated with silent cerebral infarcts.
STUDY DESIGN - In this cross-sectional study, we evaluated the health history, laboratory values, and brain magnetic resonance imaging findings of participants with sickle cell disease (hemoglobinSS or hemoglobinSβ°-thalassemia) with no history of overt stroke or seizures. Participants characterized headache severity and quality. Migraine was defined by International Headache Society criteria modified for increased sensitivity in children. Neuroradiology and neurology committees adjudicated the presence of silent cerebral infarction by review of magnetic resonance imaging and standardized examination by pediatric neurologists.
RESULTS - The cohort included 872 children (51.1% males), ranging in age from 5 to 15 years (mean age, 9.1 years). Of these children, 317 (36.4%) reported recurrent headaches, and 132 (15.1%) reported migraines. In multivariable logistic regression analyses, both were associated with lower steady-state hemoglobin (P = .01 for headaches; P < .01 for migraines) and higher pain rate (P < .01 for headaches; P < .01 for migraines), defined as the number of admissions requiring opioids in the previous 3 years. The presence of silent cerebral infarction was not associated with recurrent headaches or migraines. Only 1.9% (6 of 317) of children with recurrent headaches received medication for headache prophylaxis.
CONCLUSION - Recurrent headaches and migraines are common and undertreated in children with sickle cell disease. Low hemoglobin levels and high pain rates are associated with recurrent headaches and migraines; whereas, silent cerebral infarction is not.
Copyright © 2014 Elsevier Inc. All rights reserved.
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21 MeSH Terms
Do more rules make us safer? Clinical decision rules, patient safety, and the role of emergency physicians in health care: January 2014 Annals of Emergency Medicine Journal Club.
Gupta M, Barrett TW, Schriger DL
(2014) Ann Emerg Med 63: 84-5
MeSH Terms: Decision Support Techniques, Female, Headache Disorders, Primary, Humans, Male, Radiography, Subarachnoid Hemorrhage
Added January 20, 2015
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7 MeSH Terms
The index case for the fungal meningitis outbreak in the United States.
Pettit AC, Kropski JA, Castilho JL, Schmitz JE, Rauch CA, Mobley BC, Wang XJ, Spires SS, Pugh ME
(2012) N Engl J Med 367: 2119-25
MeSH Terms: Aspergillosis, Aspergillus fumigatus, Brain, Cerebellum, Cerebral Infarction, Cerebrospinal Fluid, Diagnosis, Differential, Disease Outbreaks, Drug Contamination, Fatal Outcome, Glucocorticoids, Headache, Humans, Injections, Epidural, Intracranial Hemorrhages, Low Back Pain, Male, Meningitis, Fungal, Middle Aged, Tomography, X-Ray Computed, United States
Show Abstract · Added August 14, 2014
Persistent neutrophilic meningitis presents a diagnostic challenge, because the differential diagnosis is broad and includes atypical infectious causes. We describe a case of persistent neutrophilic meningitis due to Aspergillus fumigatus in an immunocompetent man who had no evidence of sinopulmonary or cutaneous disease. An epidural glucocorticoid injection was identified as a potential route of entry for this organism into the central nervous system, and the case was reported to the state health department.
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21 MeSH Terms
Functional abdominal pain in childhood and adolescence increases risk for chronic pain in adulthood.
Walker LS, Dengler-Crish CM, Rippel S, Bruehl S
(2010) Pain 150: 568-72
MeSH Terms: Abdominal Pain, Adolescent, Adult, Chronic Disease, Female, Headache, Humans, Interviews as Topic, Longitudinal Studies, Male, Pain, Pain Measurement, Prevalence, Risk Factors, Young Adult
Show Abstract · Added March 5, 2014
A few studies of long-term outcomes for pediatric functional abdominal pain (FAP) have assessed acute non-abdominal pain at follow-up, but none has assessed chronic pain. We followed a cohort of pediatric patients with FAP (n=155) and a well control group (n=45) prospectively for up to 15 years. Participants ranged in age from 18 to 32 years at a follow-up telephone interview. FAP patients were classified as Resolved (n=101) versus Unresolved (n=54) at follow-up, based on whether they reported symptoms consistent with the adult Rome III criteria for a functional gastrointestinal disorder. Headache symptoms and reports of chronic non-abdominal pain also were assessed at follow-up. In the Unresolved group, 48.1% reported one or more sites of chronic non-abdominal pain at follow-up, compared to 24.7% in the Resolved group and 13.3% in the control group, p<0.01. More than half (57.4%) of the Unresolved group endorsed symptoms consistent with International Headache Society criteria for headache, compared to 44.6% of the Resolved group and 31% of controls, p<0.05. One-third of the Unresolved group reported both headache and one or more sites of chronic non-abdominal pain at follow-up, compared to 17.8% of the Resolved group and 4.4% of controls. Youth with FAP that persists into adulthood may be at increased risk for chronic pain and headache. Examination of central mechanisms that are common across chronic pain disorders may enhance understanding of this subgroup of FAP.
Copyright (c) 2010 International Association for the Study of Pain. Published by Elsevier B.V. All rights reserved.
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15 MeSH Terms