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Non-cirrhotic portal hypertension - diagnosis and management.
Khanna R, Sarin SK. Khanna R, et al. J Hepatol. 2014 Feb;60(2):421-41. doi: 10.1016/j.jhep.2013.08.013. Epub 2013 Aug 23. J Hepatol. 2014. PMID: 23978714 Free article. Review.
NCPF/IPH is a disorder of young adults or middle aged women, whereas EHPVO is a disorder of childhood. Early age acute or recurrent infections in an individual with thrombotic predisposition constitute the likely pathogenesis. ...Persistent growth failure, symptomat …
NCPF/IPH is a disorder of young adults or middle aged women, whereas EHPVO is a disorder of childhood. Early age acute or recurren
Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome.
Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S, Belman AL. Pohl D, et al. Neurology. 2016 Aug 30;87(9 Suppl 2):S38-45. doi: 10.1212/WNL.0000000000002825. Neurology. 2016. PMID: 27572859 Review.
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating CNS disorder with predilection to early childhood. ADEM is generally considered a monophasic disease. However, recurrent ADEM has been described and defined as multiphasic disseminated e …
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating CNS disorder with predilection to early childhood. AD …
Recurrent childhood myoglobinuria.
Tein I, DiMauro S, DeVivo DC. Tein I, et al. Adv Pediatr. 1990;37:77-117. Adv Pediatr. 1990. PMID: 2264536 Review.
Recurrent heritable childhood myoglobinuria is a potentially fatal entity (mortality up to 35%) in which prompt diagnosis and treatment are critical. ...We have found important differences between the childhood and adult-onset cases. Of 77 cases of adult-onse
Recurrent heritable childhood myoglobinuria is a potentially fatal entity (mortality up to 35%) in which prompt diagnosis and
The epileptic encephalopathies of infancy and childhood.
Wirrell E, Farrell K, Whiting S. Wirrell E, et al. Can J Neurol Sci. 2005 Nov;32(4):409-18. doi: 10.1017/s0317167100004388. Can J Neurol Sci. 2005. PMID: 16408569 Review.
The epileptic encephalopathies comprise a group of devastating seizure syndromes which begin in infancy and early childhood and usually result in intractable epilepsy. While some syndromes are relatively easily diagnosed early in their course, others take time to evolve, h …
The epileptic encephalopathies comprise a group of devastating seizure syndromes which begin in infancy and early childhood and usual …
Recurrent status epilepticus in posterior reversible encephalopathy syndrome as initial feature of pediatric lupus: A newly diagnosed case and literature review.
Nasri A, Bedoui I, Mrissa R, Riahi A, Derbali H, Zaouali J, Messelmani M, Mansour M. Nasri A, et al. Brain Dev. 2016 Oct;38(9):835-41. doi: 10.1016/j.braindev.2016.03.011. Epub 2016 Apr 8. Brain Dev. 2016. PMID: 27068876 Review.
INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a recently described clinico-neuroradiological syndrome with several predisposing conditions. ...RESULTS: We describe the case of a 12-year old girl who presented with recurrent status epilepticus …
INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a recently described clinico-neuroradiological syndrome with sev …
Convulsive status epilepticus in children.
Gross-Tsur V, Shinnar S. Gross-Tsur V, et al. Epilepsia. 1993;34 Suppl 1:S12-20. doi: 10.1111/j.1528-1157.1993.tb05901.x. Epilepsia. 1993. PMID: 8462488 Review.
Status epilepticus (SE) occurs most commonly in infancy and childhood. Children with prior neurological abnormalities are most susceptible. More than 90% of cases are convulsive and the majority are generalized. ...The etiology can be classified as idiopathic, remote sympt …
Status epilepticus (SE) occurs most commonly in infancy and childhood. Children with prior neurological abnormalities are most suscep …
The expanding spectrum of herpesvirus infections of the nervous system.
Kleinschmidt-DeMasters BK, Gilden DH. Kleinschmidt-DeMasters BK, et al. Brain Pathol. 2001 Oct;11(4):440-51. doi: 10.1111/j.1750-3639.2001.tb00413.x. Brain Pathol. 2001. PMID: 11556690 Free PMC article. Review.
Acute meningitis is seen with VZV and HSV-2, and benign recurrent meningitis with HSV-2. Combinations of meningitis/ encephalitis and myelitis/radiculitis are associated with Epstein Barr Virus (EBV); myelitis with VZV, CMV, EBV, and HSV-2; and ventriculitis/encephalitis w …
Acute meningitis is seen with VZV and HSV-2, and benign recurrent meningitis with HSV-2. Combinations of meningitis/ encephalitis and …
Efficacy of adjunctive vagus nerve stimulation in patients with Dravet syndrome: A meta-analysis of 68 patients.
Dibué-Adjei M, Fischer I, Steiger HJ, Kamp MA. Dibué-Adjei M, et al. Seizure. 2017 Aug;50:147-152. doi: 10.1016/j.seizure.2017.06.007. Epub 2017 Jun 17. Seizure. 2017. PMID: 28666193 Free article. Review.
RATIONALE: Dravet Syndrome (DS) is a severe epileptic encephalopathy of childhood involving intractable seizures, recurrent status epilepticus and cognitive decline. ...
RATIONALE: Dravet Syndrome (DS) is a severe epileptic encephalopathy of childhood involving intractable seizures, recurrent
Childhood Sjogren syndrome presenting as acute brainstem encephalitis.
Matsui Y, Takenouchi T, Narabayashi A, Ohara K, Nakahara T, Takahashi T. Matsui Y, et al. Brain Dev. 2016 Jan;38(1):158-62. doi: 10.1016/j.braindev.2015.05.005. Epub 2015 May 23. Brain Dev. 2016. PMID: 26006751 Review.
In retrospect, she had exhibited a preceding history of recurrent conjunctivitis and strong halitosis that could be considered as sicca symptoms. ...In children presenting with unknown acute febrile encephalopathy, Sjogren syndrome should be included in the differen …
In retrospect, she had exhibited a preceding history of recurrent conjunctivitis and strong halitosis that could be considered as sic …
Variants of ATP1A3 in residue 756 cause a separate phenotype of relapsing encephalopathy with cerebellar ataxia (RECA)-Report of two cases and literature review.
Biela M, Rydzanicz M, Szymanska K, Pieniawska-Smiech K, Lewandowicz-Uszynska A, Chruszcz J, Benben L, Kuzior-Plawiak M, Szyld P, Jakubiak A, Szenborn L, Ploski R, Smigiel R. Biela M, et al. Mol Genet Genomic Med. 2021 Sep;9(9):e1772. doi: 10.1002/mgg3.1772. Epub 2021 Aug 2. Mol Genet Genomic Med. 2021. PMID: 34342181 Free PMC article. Review.
BACKGROUND: Variants in ATP1A3 cause well-known phenotypes-alternating hemiplegia of childhood (AHC), rapid-onset dystonia-parkinsonism (RDP), cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss (CAPOS), and severe early infantile epileptic …
BACKGROUND: Variants in ATP1A3 cause well-known phenotypes-alternating hemiplegia of childhood (AHC), rapid-onset dystonia-parkinsoni …
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