Development of a disease severity score for newborns with collodion membrane - 14/02/14
Abstract |
Background |
Collodion membrane in the neonate may be the initial presentation of a number of different conditions. There is a lack of data correlating the extent of clinical involvement to the underlying disease and prognosis.
Objective |
We sought to identify features predictive of the final outcome and complications in a cohort of patients with collodion membrane, using a disease severity score.
Methods |
This was a retrospective cohort study of newborns with collodion membrane at a tertiary care institution over a period of 31 years. We designed and applied a 0- to 15-point severity score and correlated the results with the final diagnoses and complications. Data on demographics, membrane shedding, and treatment were collected.
Results |
We identified 29 cases. Congenital ichthyosiform erythroderma and lamellar ichthyosis were the most common final diagnoses with 7 of 29 cases (24%) each; 3 patients were given the diagnosis of a syndromic ichthyosis. The classic nonsyndromic ichthyoses had higher average score results (7.33) than the syndromic ichthyoses (2.0) and other presentations (4.0), (P = .0097). Patients with major complications had higher, but nonsignificant, average severity scores (P = .64).
Limitations |
The retrospective design and small number of patients with a syndromic ichthyosis are limitations.
Conclusions |
Prospective studies are required to validate the proposed disease severity score.
Le texte complet de cet article est disponible en PDF.Key words : collodion baby, collodion membrane, congenital ichthyosis, disorders of cornification, neonatal diseases, nonsyndromic ichthyosis, syndromic ichthyosis
Abbreviations used : ARCI, CIE, HI, LI, RXLI, UCI
Plan
Funding sources: None. |
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Disclosure: Dr Pope declares that she has been an investigator for and has received grants from Amgen and Pierre Fabre; these relationships are not relevant to this manuscript and do not create conflicts of interest. Drs Rubio-Gomez and Weinstein have no conflicts of interest to declare. |
Vol 70 - N° 3
P. 506-511 - mars 2014 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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