Pulse oral corticosteroids in pediatric chronic inflammatory demyelinating polyneuropathy. 2020

Amanda B Rogers, and Craig M Zaidman, and Anne M Connolly
Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.

Childhood onset chronic inflammatory demyelinating polyneuropathy (CIDP) often requires long-term immunomodulatory therapy. We report a comprehensive review of our treatment of pediatric CIDP with a focus on high-dose weekly corticosteroids ("pulse oral corticosteroids"), a treatment method that is not commonly reported. We retrospectively reviewed medical records of pediatric patients with CIDP treated at our center between 2000 and 2018 for whom we had at least 12 mo follow-up. Here, we describe the demographics, disease course, treatment regimens, and long-term outcomes of these patients. Twenty-five patients were identified for analysis. Pulse oral corticosteroid monotherapy was the predominant maintenance treatment in 56% of patients. Patients were followed for a median of 4 y. Side effects were seen in a minority of patients. The probability of a normal exam or being off treatment at last follow-up was similar regardless of predominant maintenance therapy. Pulse oral corticosteroid therapy is a safe and effective long-term treatment option in children with CIDP.

UI MeSH Term Description Entries
D007155 Immunologic Factors Biologically active substances whose activities affect or play a role in the functioning of the immune system. Biological Response Modifier,Biomodulator,Immune Factor,Immunological Factor,Immunomodulator,Immunomodulators,Biological Response Modifiers,Biomodulators,Factors, Immunologic,Immune Factors,Immunological Factors,Modifiers, Biological Response,Response Modifiers, Biological,Factor, Immune,Factor, Immunological,Factors, Immune,Factors, Immunological,Modifier, Biological Response,Response Modifier, Biological
D007223 Infant A child between 1 and 23 months of age. Infants
D007319 Sleep Initiation and Maintenance Disorders Disorders characterized by impairment of the ability to initiate or maintain sleep. This may occur as a primary disorder or in association with another medical or psychiatric condition. Disorders of Initiating and Maintaining Sleep,Insomnia,Sleeplessness,Chronic Insomnia,DIMS (Disorders of Initiating and Maintaining Sleep),Early Awakening,Insomnia Disorder,Nonorganic Insomnia,Primary Insomnia,Psychophysiological Insomnia,Rebound Insomnia,Secondary Insomnia,Sleep Initiation Dysfunction,Transient Insomnia,Awakening, Early,Dysfunction, Sleep Initiation,Dysfunctions, Sleep Initiation,Insomnia Disorders,Insomnia, Chronic,Insomnia, Nonorganic,Insomnia, Primary,Insomnia, Psychophysiological,Insomnia, Rebound,Insomnia, Secondary,Insomnia, Transient,Insomnias,Sleep Initiation Dysfunctions
D007508 Irritable Mood Abnormal or excessive excitability with easily triggered anger, annoyance, or impatience. Mood, Irritable,Irritable Moods,Moods, Irritable
D008297 Male Males
D011241 Prednisone A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver. Dehydrocortisone,delta-Cortisone,Apo-Prednisone,Cortan,Cortancyl,Cutason,Dacortin,Decortin,Decortisyl,Deltasone,Encorton,Encortone,Enkortolon,Kortancyl,Liquid Pred,Meticorten,Orasone,Panafcort,Panasol,Predni Tablinen,Prednidib,Predniment,Prednison Acsis,Prednison Galen,Prednison Hexal,Pronisone,Rectodelt,Sone,Sterapred,Ultracorten,Winpred,Acsis, Prednison
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D005938 Glucocorticoids A group of CORTICOSTEROIDS that affect carbohydrate metabolism (GLUCONEOGENESIS, liver glycogen deposition, elevation of BLOOD SUGAR), inhibit ADRENOCORTICOTROPIC HORMONE secretion, and possess pronounced anti-inflammatory activity. They also play a role in fat and protein metabolism, maintenance of arterial blood pressure, alteration of the connective tissue response to injury, reduction in the number of circulating lymphocytes, and functioning of the central nervous system. Glucocorticoid,Glucocorticoid Effect,Glucorticoid Effects,Effect, Glucocorticoid,Effects, Glucorticoid

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