Melatonin excretion in normal children and in tuberous sclerosis complex with sleep disorder responsive to melatonin
Circadian Rhythm; Male; Case-Control Studies; Child; Humans; Adolescent; Female; Child Preschool; Administration Oral; Reference Values; Tuberous Sclerosis; Antioxidants; Melatonin; Sleep Wake Disorders; Antioxidants/pk [Pharmacokinetics]; Circadian Rhythm; Melatonin/aa [Analogs & Derivatives]; Melatonin/pk [Pharmacokinetics]; Sleep Wake Disorders/co [Complications]; Tuberous Sclerosis/co [Complications]; 0 (Antioxidants); 2208-40-4 (6-sulfatoxymelatonin); Antioxidants/ad [Administration & Dosage]; JL5DK93RCL (Melatonin); Melatonin/ad [Administration & Dosage]; Melatonin/ur [Urine]; Sleep Wake Disorders/pp [Physiopathology]; Tuberous Sclerosis/pp [Physiopathology]; sleep disturbance/disorders; trajectory; characteristics; melatonin
To determine normal melatonin excretion patterns in healthy children without sleep disorder and to compare these with those of patients with tuberous sclerosis complex and sleep disorder responsive to exogenous melatonin, we measured 6-sulfatoxymelatonin excretion in 21 healthy children and in 7 patients with tuberous sclerosis complex and sleep disorder responsive to melatonin (a 5 mg oral dose increasing total sleep time). Total excretion, cosinor percentage, and acrophase time of 6-sulfatoxymelatonin excretion were estimated. In normal children, total 6-sulfatoxymelatonin excretion was range 11.1 to 40.2 microg (mean 19.0 microg, SD 7.4 microg); cosinor percentage rhythm range was 52.9% to 100% (mean 87%, median 94%); and acrophase time range was 23 hours, 54 minutes to 10 hours, 42 minutes (mean 5 hours, 54 minutes; median 4 hours, 12 minutes). Fifth and 95th percentiles were 11.1 to 29.0 microg, 57.8% to 99.9%, and 2 hours, 1 minute to 10 hours, 4 minutes. In tuberous sclerosis, normal patterns of melatonin excretion were seen in responders. Circadian patterns of melatonin excretion were similar in children and adults. We propose that exogenous melatonin can act by a simple sedative action.
Hancock E; O'Callaghan F; English J; Osborne J P
Journal of Child Neurology
2005
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1177/08830738050200010301" target="_blank" rel="noreferrer noopener">10.1177/08830738050200010301</a>
Neurogenetic disorders and treatment of associated seizures.
Humans; Treatment Outcome; Epilepsies; Angelman Syndrome/dt [Drug Therapy]; Anticonvulsants/tu [Therapeutic Use]; Epilepsies; Rett Syndrome/dt [Drug Therapy]; Seizures/dt [Drug Therapy]; Tuberous Sclerosis/dt [Drug Therapy]; Angelman Syndrome/co [Complications]; Anticonvulsants/ad [Administration & Dosage]; Anticonvulsants/ae [Adverse Effects]; KT Synthesis; Myoclonic/co [Complications]; Myoclonic/dt [Drug Therapy]; Rett Syndrome/co [Complications]; Seizures/co [Complications]; Tuberous Sclerosis/co [Complications]
Seizures are a frequent complication associated with several neurogenetic disorders. Antiepileptic medications remain the mainstay of treatment in these patients. We summarized the available data associated with various antiepileptic therapies used to treat patients with neurogenetic disorders who experienced recurrent seizures. A MEDLINE search was conducted to identify articles and abstracts describing the use of antiepileptic therapy for the treatment of various neurogenetic syndromes. Of all the neurogenetic syndromes, only autism spectrum disorders, Angelman syndrome, Rett syndrome, Dravet syndrome, and tuberous sclerosis complex were identified as having sufficient published information to evaluate therapy. Some efficacy trends were identified, including frequent successes with valproic acid with clonazepam for epilepsy with Angelman syndrome; valproic acid, stiripentol, and clobazam (triple combination therapy) for epilepsy with Dravet syndrome; and vigabatrin for infantile spasms associated with tuberous sclerosis complex. Due to a paucity of information regarding the mechanisms by which seizures are generated in the various disorders, approach to seizure control is primarily based on clinical experience and a limited amount of study data exploring patient outcomes. Although exposure of the developing brain to antiepileptic medications is of some concern, the control of epileptic activity is an important undertaking in these individuals, as the severity of eventual developmental delay often appears to correlate with the severity of seizures. As such, early aggressive therapy is warranted. 2013 Pharmacotherapy Publications, Inc.
2013
Faulkner MA; Singh Sanjay P
Pharmacotherapy
2013
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Journal Article
<a href="http://doi.org/10.1002/phar.1201" target="_blank" rel="noreferrer">10.1002/phar.1201</a>