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emigrants and immigrants, laminopathy, nursing care, palliative care, restrictive dermopathy



  1. Bosque, Elena PhD, NNP


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.