Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0728731 (prematurity)
 7,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The article describes pregnancies and labors of five women with myotonic muscular dystrophy and their four severely involved infants, and reviews the pertinent literature. Three of the four neonatal cases died by 3 weeks of age of respiratory failure or aspiration; the fourth infant is now 4 months old and has respiratory and swallowing difficulties. The symptoms of myotonic dystrophy worsen during pregnancy. A high rate of fetal loss occurs due to spontaneous abortion, prematurity, and neonatal involvement with the disease. Prolonged labor has been described as a consistent complication, but the evidence does not justify this conclusion. Although many neonates with myotonic dystrophy are asymptomatic, severely affected newborns have a recognizable disorder unrelated to the severity of the maternal disease. The most common clinical manifestations in the neonate are arthrogryposis involving predominantly the lower extremilies, generalized hypotonia and weakness, and pharyngeal weakness. Less constant features include polyhydramnios, facial diplegia, diaphragmatic paralysis, respiratory failure, decreased motility of the gastrointestinal tract, congenital cataracts, and electrocardiographic abnormalities.
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PMID:Clinical effects of myotonic dystrophy on pregnancy and the neonate. 77 28

Neuromuscular disorder can cause neonatal respiratory insufficiency. One male term newborn with Werdnig-Hoffmann disease and two preterm infants with congenital myotonic dystrophy Curshmann-Steinert-Batten are reported. Hydramnion, few spontaneous movements, prematurity, increasing respiratory insufficiency, hypotonia and myopathic facies of the mother are typical for congenital myotonic dystrophy.
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PMID:[Neuromuscular diseases as a cause of neonatal respiratory insufficiency]. 196 Dec 9

We report the clinical experience of 18 patients with the congenital form of myotonic dystrophy, the majority of whom were diagnosed during the neonatal period and monitored from 5 to 14 years. Prematurity associated with congenital myotonic dystrophy gives rise to the severest clinical manifestations. Among them, respiratory involvement is common and is the leading cause of death in the neonatal period. Weakness and foot deformities secondary to muscle involvement are the predominant clinical features of this group of patients from birth to age 3 or 4 years. Once muscle strength improves, learning disabilities and behavioral disturbances become the main clinical problems. All our patients, when tested after 5 years of age, had intelligence quotients under 65, clearly below the average intelligence quotient of their mothers (IQ = 80). There is no relationship between the degree of mothers' and patients' disease. No patient has presented problems with routine immunizations, and no complications were observed in the 7 patients who underwent surgery under general anesthesia. Among the surviving patients, no correlation can be established between severity of disease in the neonatal period and the magnitude of sequelae as teenagers. Mental and behavioral disturbances are the factors which mainly influence the long-term management and prognosis of this cohort of individuals.
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PMID:Presentation, clinical course, and outcome of the congenital form of myotonic dystrophy. 757 59