Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0694563 (
eds
)
1,062
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Sleep-disordered breathing (SDB) is common in patients with
growth hormone
(GH) secreting pituitary adenomas. Since long-term untreated SDB aggravates systemic conditions (hypertension and arrhythmia etc.), the therapeutic outcome of SDB is important in reducing morbidity and mortality rates. But the results of a quantitative analysis of the lowered GH and IGF-1 levels in SDB in a relatively large number of patients are not detailed. Ten consecutive acromegalic patients were studied with a bedside oximeter. Preoperatively they were divided into two groups based on the presence (SDB group = 6 patients) or absence (non-SDB group = 4 patients) of clinical symptoms of SDB such as habitual snoring,
excessive daytime somnolence
and nocturnal apneic episodes. The serum IGF-1 averaged 931.7 ng/ml in SDB group and 898.3 ng/ml in non-SDB group. The oxygen desaturation index (ODI) (the number of oxygen desaturations exceeding 4% from the base line) was 29.1+/-15.4 in the SDB group and 2.5+/-1.8 in the non-SDB group (P=0.01). Other oximeter parameters such as the percent of the time spent at O2 saturation < 90% and the mean and the lowest O2 saturations closely correlated with the degree of the clinical symptoms. A postoperative sleep study was conducted in 5 patients in the preoperative SDB group, 4 months or more after the surgery. The serum GH and IGF-1 levels normalized in 3 patients but remained slightly high in 2. ODI became 9.1+/-5.6, which was significantly lower than the preoperative value (P=0.026). One patient had a complete clinical resolution. The other 4 obtained slight to moderate improvement clinically and oximetrically despite normalized or decreased hormonal levels. This study clarified that the response of SDB to lowering of the GH level varies from one patient to another and persisting SDB despite the normalization of the hormonal levels suggests the involvement of other factors in the production of SDB.
...
PMID:Sleep-disordered breathing in acromegalics--relation of hormonal levels and quantitative sleep study by means of bedside oximeter. 1058 Jul 52
Prader-Willi syndrome (PWS) is an imprinted genetic disorder conferred by loss of paternal gene expression from chromosome 15q11.2-q13. Individuals with PWS have impairments in ventilatory control and are predisposed toward sleep disordered breathing due to a combination of characteristic craniofacial features, obesity, hypotonia, and hypothalamic dysfunction. Children with PWS progress from failure to thrive during infancy to hyperphagia and morbid obesity during later childhood and onward. Similarly, the phenotype of sleep disordered breathing in PWS patients also evolves over time from predominantly central sleep apnea in infants to obstructive sleep apnea (OSA) in older children. Behavioral difficulties are common and may make establishing effective therapy with continuous positive airway pressure (CPAP) more challenging when OSA persists after adenotonsillectomy.
Excessive daytime sleepiness
(
EDS
) is also common in patients with PWS and may continue after OSA is effectively treated. We describe here the characteristic ventilatory control deficits, sleep disordered breathing, and excessive daytime sleepiness seen in individuals with PWS. We review respiratory issues that may contribute to sudden death events in PWS patients during sleep and wakefulness. We also discuss therapeutic options for treating sleep disordered breathing including adenotonsillectomy, weight loss, and CPAP. Lastly, we discuss the benefits and safety considerations related to
growth hormone
therapy.
...
PMID:Disorders of Sleep and Ventilatory Control in Prader-Willi Syndrome. 2893 3