Outcome of West Syndrome: A Critical Review

2020 ◽  
Vol 09 (01) ◽  
pp. 001-006
Author(s):  
Sudhir Adhikari ◽  
Deepa Gautam
Keyword(s):  
Age At Onset ◽  
Neurodevelopmental Outcome ◽  
Hormonal Treatment ◽  
Severe Disabilities ◽  
Clinical Syndrome ◽  
West Syndrome ◽  
Oral Steroid ◽  
Unknown Etiology ◽  
Sustained Remission

AbstractWest syndrome is a genetically heterogeneous electro-clinical syndrome starting in early infancy. Short-term goal of therapy is spasm control and with standard hormonal or vigabatrin treatment, spasms can be controlled in 60 to 80% of patients in 2 weeks to 3 months period. Hormonal treatment with oral steroid is an alternative therapy to injectable adrenocorticotropin hormone, especially in low resource areas. Vigabatrin is preferred in tuberous sclerosis patients. Long-term aim of treatment is sustained remission of seizures and better neurodevelopmental outcome. About 50 to 70% of children are spasm free for prolonged duration, but epilepsy with multiple seizure types including Lennox–Gastaut syndrome is evident in 20 to 40% of children in long-term follow-up. Though hypsarrhythmia is helpful for the diagnosis, prognostic role of the resolution of electroencephalographic abnormalities is still uncertain. Seizures can be controlled in 40 to 60% of the patients, but only 12 to 40% children have normal neurodevelopmental outcome and a third of children are left with severe disabilities. Children with unknown etiology and normal development at spasm onset have better clinical outcome. Young age at onset, nonstandard therapy have less favorable outcome. Surgery is helpful for spasm control in patients with structural lesions and refractory spasms even in the absence of structural lesions.

scholarly journals NEONATAL SEIZURES;

2018 ◽  
Vol 21 (05) ◽  
pp. 1048-1053
Author(s):  
Arshad Mahmood ◽  
Syed Qamar Zaman ◽  
Shahid Mahmud
Keyword(s):  
Cerebral Palsy ◽  
Neurodevelopmental Outcome ◽  
Birth Asphyxia ◽  
Unknown Etiology ◽  
Neonatal Seizures ◽  
Inborn Errors ◽  
Cross Sectional ◽  
Normal Delivery ◽  
Female Ratio

Objective: To study the types, etiology and long term neurodevelopmentaloutcome in neonates with seizures. Study Design: A descriptive cross-sectional study. Placeand Duration of Study: PNS Shifa Naval hospital Karachi from Jan 2011 to Feb 2014. StudyPopulation: Ninety six neonates of either gender presented with seizures at NICU PNS ShifaNaval hospital Karachi were studied. Method: All neonates with seizures were evaluated.The seizures were classified according to the simiology. They were investigated according toNICU protocol to confirm the underlying diagnosis and timely management. The patients afterdischarge were regularly followed up for one year to assess the long term neurodevelopmentaloutcome. Results: A total of 96 neonates with seizures were studied and it was observedthat 60 (62.5%) were male babies and 56 (58.33%) were term with a male to female ratio of1.6:1. Majority of the neonatal seizures were seen in 1stweek of life (85%). The most commontype of seizures was clonic 40 (41.67%) followed by subtle 20 (20.84%), mixed 16 (16.67%),tonic 10 (10.41%), myoclonic 5 (5.20%) and unclassified 5 (5.20%). Antiepileptics were usedin 82 (85.41%) patients. Phenobarbitone 49 (59.76%) was most commonly prescribed drug.The most common cause of seizures was birth asphyxia 48 (50%) followed by metabolic 16(16.68%), sepsis 10 (10.41%), intracranial hemorrhage 6 (6.25%), bilirubin encephalopathy 4(4.16%), inborn errors of metabolism 2 (2.08%), birth trauma 2 (2.08%) and unknown etiology 5(5.20%). 25 (26.04%) patients develop adverse neurodevelopmental outcome i.e. cerebral palsywith epilepsy 10 (40%) and cerebral palsy without epilepsy 05 (20%), developmental delay 10(40%). Mortality in the study was 12 (12.5%). Conclusions: Clonic seizures are commonestin neonates apart from infants and children who have GTCS. The most common etiology ofseizures in neonates is birth asphyxia. Phenobarbitone is still the most commonly prescribedantiepileptic. Quick assessment, timely diagnosis and aggressive management according tothe etiology are necessary to prevent the morbidity and mortality associated with neonatalseizures. Long term neurodevelopmental outcome is worse in patients with birth asphyxiaespecially with low Apgar score at 5 minutes. Normal delivery and birth asphyxia were the majorrisk factors for cerebral palsy

Remission Rates of Children and Adolescents with Thyrotoxicosis Treated with Antithyroid Drugs

PEDIATRICS ◽  
1980 ◽  
Vol 65 (3) ◽  
pp. 550-556
Author(s):  
Roberta J. Collen ◽  
Elliot M. Landaw ◽  
Solomon A. Kaplan ◽  
Barbara M. Lippe
Keyword(s):  
Medical Therapy ◽  
Remission Rate ◽  
Age At Onset ◽  
Laboratory Data ◽  
Antithyroid Drugs ◽  
Sustained Remission ◽  
Table Analysis ◽  
One Year

The clinical course of 65 children with hyperthyrodism in whom antithyroid medication was initiated has been reviewed retrospectively. Fifty-five patients received only this projected medical therapy with propylthiouracil or methimazole. Sustained remission in excess of one year following cessation of drugs occurred in 31 patients (56%). All but two achieved that remission within 4.5 years. Of the 31 patients, 24 have been followed-up for more than two years off medication with only one relapse. Twenty-four patients are still under medical therapy, 14 for longer than 4.5 years. By methods of life-table analysis, the data on all 65 patients gave an estimate of 25% remitting within the first two years and a median remission time of 4 to 4.5 years. These results suggest a continuing remission rate of 25% every two years for up to six years of follow-up. Analysis of presenting variables (assessment of age at onset of disease, sex, family history, initial laboratory data, gland size, and degree of exophthalmos) that might have distinguished which patients were likely to undergo remission was attempted. There were no clearly significant differences with any of these variables although several suggestive differences emerged. These included a tendency toward earlier remission in patients older than 13 years, in boys of any age, and in patients of any age or sex in whom initial triiodothyronine (T3 RIA) < was 645 ng/100 ml. An approach to long-term medical management is proposed.

Corticosteroids in Inflammatory Bowel Disease patients: a practical guide for physicians.

2020 ◽  
Vol 15 ◽  
Author(s):  
Maria Carla Di Paolo ◽  
Cristiano Pagnini ◽  
Maria Giovanna Graziani
Keyword(s):  
Severe Disease ◽  
Unknown Etiology ◽  
History Of Disease ◽  
Bowel Diseases ◽  
Literature Evidence ◽  
History Of ◽  
Inflammatory Bowel ◽  
Favorable Safety ◽  
Pass Metabolism

: Inflammatory bowel diseases (IBDs) are chronic conditions characterized by unknown etiology and pathogenesis with deregulation of mucosal immunity. Among possible treatments, corticosteroids, already available from the 50’, are still the mainstay of treatment for moderate-severe disease. Nonetheless, the use of steroids is still largely empirical and solid evidence about therapeutic schemes are lacking. Moreover, due to the important side-effects and for the unsatisfactory impact on long-term natural history of disease, the steroid sparing has become an important therapeutic goal in IBD management. Besides conventional steroids, the so called “low bioavailability” steroids, which are steroids with high affinity for peripheral receptors and elevated hepatic first-pass metabolism, have demonstrated efficacy and more favorable safety profile. In the present review of the literature evidence of efficacy and safety of conventional and low bioavailability steroids in IBD patients are evaluated, and practical suggestions for a correct use in clinical practice are presented according to the current clinical guidelines.

scholarly journals Oxidative stress in common variable immunodeficiency

2021 ◽  
Vol 19 ◽  
pp. 205873922110024
Author(s):  
Sevgen Tanir Basaranoglu ◽  
Sukru Cekic ◽  
Emine Kirhan ◽  
Melahat Dirican ◽  
Sara S. Kilic
Keyword(s):  
Oxidative Stress ◽  
Common Variable Immunodeficiency ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Infectious Complications ◽  
Clinical Syndrome ◽  
University Hospital ◽  
Unknown Etiology ◽  
Healthy Controls ◽  
Oxidative Stress Biomarkers ◽  
Common Variable

Common variable immunodeficiency (CVID) is a heterogenous group of immunologic disorders of unknown etiology. Alterations of the normal cellular balance due to an increase in reactive oxygen species and/or decrease in antioxidant defense may lead to increased oxidative stress. We aimed to evaluate the levels of oxidative stress biomarkers in patients with CVID who had different presentations. We investigated the serum catalase (CAT), erythrocyte superoxide dismutase (SOD), erythrocyte reduced glutathione as antioxidants and serum malondialdehyde levels as lipid peroxidation marker in patients with CVID in Uludag University Hospital Department of Pediatric Allergy and Immunology’s outpatient clinics. In the analysis, there were 21 patients and 27 matched healthy controls. The median levels of CAT in patients with CVID was significantly lower than in healthy controls ( p = 0.04). Among the patients with CVID, 19% had autoimmune disease, one had Sjögren’s syndrome, one had autoimmune alopecia, one had juvenile rheumatoid arthritis, and one had chronic inflammatory demyelinating polyneuropathy. Patients with autoimmune complications had significantly lower CAT levels compared to the ones without autoimmune diseases ( p = 0.03). The patients without non-infectious complications (NICs) had lower SOD levels than the patients with NICs ( p = 0.05). The analysis of oxidative stress markers in the patients with CVID suggested a series of abnormalities in the anti-oxidant system. The clinical syndrome associations may be a useful tool for future studies to set prediction markers for the prognosis of patients with CVID.

scholarly journals Oral melatonin as a new tool for neuroprotection in preterm newborns: study protocol for a randomized controlled trial

Trials ◽  
2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Francesca Garofoli ◽  
Stefania Longo ◽  
Camilla Pisoni ◽  
Patrizia Accorsi ◽  
Micol Angelini ◽  
...  
Keyword(s):  
Preterm Birth ◽  
Preterm Infants ◽  
Neurodevelopmental Outcome ◽  
Lipid Peroxidation Product ◽  
Cerebral Magnetic Resonance Imaging ◽  
Neurodevelopmental Impairment ◽  
Anti Oxidant ◽  
Preterm Newborns

Abstract Background Prevention of neurodevelopmental impairment due to preterm birth is a major health challenge. Despite advanced obstetric and neonatal care, to date there are few neuroprotective molecules available. Melatonin has been shown to have anti-oxidant/anti-inflammatory effects and to reduce brain damage, mainly after hypoxic ischemic encephalopathy. The planned study will be the first aiming to evaluate the capacity of melatonin to mitigate brain impairment due to premature birth. Method In our planned prospective, multicenter, double-blind, randomized vs placebo study, we will recruit, within 96 h of birth, 60 preterm newborns with a gestational age ≤ 29 weeks + 6 days; these infants will be randomly allocated to oral melatonin, 3 mg/kg/day, or placebo for 15 days. After the administration period, we will measure plasma levels of malondialdehyde, a lipid peroxidation product considered an early biological marker of melatonin treatment efficacy (primary outcome). At term-equivalent age, we will evaluate neurological status (through cerebral ultrasound, cerebral magnetic resonance imaging, vision and hearing evaluations, clinical neurological assessment, and screening for retinopathy of prematurity) as well as the incidence of bronchodysplasia and sepsis. We will also monitor neurodevelopmental outcome during the first 24 months of corrected age (using the modified Fagan Test of Infant Intelligence at 4–6 months and standardized neurological and developmental assessments at 24 months). Discussion Preterm birth survivors often present long-term neurodevelopmental sequelae, such as motor, learning, social-behavioral, and communication problems. We aim to assess the role of melatonin as a neuroprotectant during the first weeks of extrauterine life, when preterm infants are unable to produce it spontaneously. This approach is based on the supposition that its anti-oxidant mechanism could be useful in preventing neurodevelopmental impairment. Considering the short- and long-term morbidities related to preterm birth, and the financial and social costs of the care of preterm infants, both at birth and over time, we suggest that melatonin administration could lead to considerable saving of resources. This would be the first study addressing the role of melatonin in very low birth weight preterm newborns, and it could provide a basis for further studies on melatonin as a neuroprotection strategy in this vulnerable population. Trial registration ClinicalTrials.gov NCT04235673. Prospectively registered on 22 January 2020.

scholarly journals Determinants of Outcomes of Acute Kidney Injury: Clinical Predictors and Beyond

2021 ◽  
Vol 10 (6) ◽  
pp. 1175
Author(s):  
Emaad M. Abdel-Rahman ◽  
Faruk Turgut ◽  
Jitendra K. Gautam ◽  
Samir C. Gautam
Keyword(s):  
Acute Kidney Injury ◽  
Kidney Disease ◽  
Kidney Injury ◽  
Complete Recovery ◽  
Severe Form ◽  
Clinical Syndrome ◽  
Current Evidence ◽  
Clinical Predictors ◽  
End Stage

Acute kidney injury (AKI) is a common clinical syndrome characterized by rapid impairment of kidney function. The incidence of AKI and its severe form AKI requiring dialysis (AKI-D) has been increasing over the years. AKI etiology may be multifactorial and is substantially associated with increased morbidity and mortality. The outcome of AKI-D can vary from partial or complete recovery to transitioning to chronic kidney disease, end stage kidney disease, or even death. Predicting outcomes of patients with AKI is crucial as it may allow clinicians to guide policy regarding adequate management of this problem and offer the best long-term options to their patients in advance. In this manuscript, we will review the current evidence regarding the determinants of AKI outcomes, focusing on AKI-D.

scholarly journals Efficacy of long‐term adrenocorticotropic hormone therapy for West syndrome: A retrospective multicenter case series

2021 ◽  
Author(s):  
Shimpei Baba ◽  
Tohru Okanishi ◽  
Yoichiro Homma ◽  
Takeshi Yoshida ◽  
Tomohide Goto ◽  
...  
Keyword(s):  
Hormone Therapy ◽  
Adrenocorticotropic Hormone ◽  
Case Series ◽  
West Syndrome

scholarly journals Long-Term Neurodevelopmental Outcome of Monochorionic and Matched Dichorionic Twins

PLoS ONE ◽  
2009 ◽  
Vol 4 (8) ◽  
pp. e6815 ◽  
Author(s):  
Karien E. A. Hack ◽  
Corine Koopman-Esseboom ◽  
Jan B. Derks ◽  
Sjoerd G. Elias ◽  
Martin J. K. de Kleine ◽  
...  
Keyword(s):  
Neurodevelopmental Outcome

390: Long-term neurodevelopmental outcome in twin-to-twin transfusion syndrome (TTTS) in the eurofoetus trial

2008 ◽  
Vol 199 (6) ◽  
pp. S118 ◽  
Author(s):  
Lisa Örtqvist ◽  
Bussieres Laurence ◽  
Staraci Stephanie ◽  
Fermanian Christophe ◽  
Ville Yves
Keyword(s):  
Neurodevelopmental Outcome
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