SynthesisPediatric dermatology
Ichthyosis Prematurity Syndrome: A Systematic Review of the Literature.
Synthesis in Pediatric dermatology. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
3 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Ichthyosis Prematurity Syndrome: A Systematic Review of the Literature.Pediatric dermatologyPooled it
- Founder effect and clinical heterogeneity of SLC27A4-related non-syndromic EDD in Réunion.Journal of the European Academy of Dermatology and Venereology : JEADV · 2026Article
- Ichthyosis Prematurity Syndrome Caused by a Novel Homozygous SLC27A4 Mutation in Two Emirati Siblings.Cureus · 2025Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND/
objectivesIchthyosis prematurity syndrome (IPS) is a rare autosomal recessive congenital disorder characterized by premature birth, neonatal respiratory distress, eosinophilia, and a thick, clay-like vernix at birth. This review aims to summarize the available reported cases of IPS, including genetic etiology, clinical features, management, and prognosis.
methodsA search was conducted using MEDLINE, Embase, Scopus, Web of Science, and CINAHL, from inception to September 3, 2024 using the search terms "IPS" or "ichthyosis congenita type IV". This systematic review was registered on the PROSPERO database (CRD42024533988). Any publication reporting a case of IPS was suitable for inclusion. Quality appraisal was performed according to CARE guidelines. Descriptive statistical analysis was performed using R v4.3.1 (R Foundation, Vienna, Austria).
resultsWe identified 26 studies reporting 59 cases of IPS (PROSPERO database: CRD42024533988). While IPS was previously thought to predominantly affect Norwegian and Swedish patients, we identified cases from a broad range of ethnicities. The median duration of the cutaneous scale was 10 weeks, though patients usually have persisting atopy. Long-term prognosis is generally good, although six (10.2%) fulminant cases were identified with an estimated 6-month survival rate of 87.6% (95% confidence interval: 78.8-97.4). Mortality in these cases was attributed to respiratory and/or cardiac sequelae or sepsis. Limitations include heterogeneity in the reporting of cases and low quality of data in several aspects of data collection.
conclusionsThe cutaneous manifestations of IPS resolve over a period of weeks, and patients generally have a favorable long-term prognosis. However, rare cases of death and other complications have been reported.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.