title: Complex case study: nursing care of an infant with restrictive dermopathy.
abstract: This article is a complex case study of an infant, born at 33 2/7 weeks' gestation, with restrictive dermopathy, who died at 5 weeks of age. The review of perinatal and postnatal courses, as well as the rare disorder of restrictive dermopathy, a lethal genodermatosis laminopathy, is presented. Neonatal problems are identified. These include problems of prematurity, including need for a neutral thermal environment, nutritional support, and sepsis evaluation. Problems related to the diagnosis of restrictive dermopathy include skin abnormalities of mild hydropic appearance and ichthyosis-like exanthem, thrombocytophilia, pain, and respiratory insufficiency that led to death. The specific social issues of a non-English-speaking family from Russia faced with a lethal diagnosis for their infant are discussed. This infant was born in a community hospital with a perinatal center and tertiary-level neonatal intensive care unit and was transferred at 3 weeks of age to a regional pediatric medical center for subspecialist pulmonary, dermatologic, and genetic evaluation, where she died shortly afterward. Finally, recommendations are made for a nursing care plan related to the problems presented in this case.
authors.affiliation: Pediatrix Medical Group, Renton, Washington 98055, USA. ebosque@u.washington.edu
authors.forename: Elena
authors.initials: E
authors.lastname: Bosque
mesh_terms: D000328:Adult; D003144:Communication Barriers; D017809:Fatal Outcome; D005260:Female; D006764:Hospitals, Community; D006801:Humans; D007231:Infant, Newborn; D007235:Infant, Premature, Diseases / Q000175:diagnosis / Q000208:ethnology / Q000235:genetics* / Q000451:nursing*; D015931:Intensive Care, Neonatal / Q000379:methods; D034904:Lamin Type A / Q000235:genetics; D009154:Mutation / Q000235:genetics; D017283:Neonatal Nursing / Q000379:methods*; D010290:Parents / Q000193:education / Q000523:psychology; D012131:Respiratory Insufficiency / Q000235:genetics; D012426:Russia / Q000208:ethnology; D012868:Skin Abnormalities / Q000175:diagnosis / Q000208:ethnology / Q000235:genetics* / Q000451:nursing*; D013577:Syndrome; D019851:Thrombophilia / Q000235:genetics
chemical_list: D034904:Lamin Type A