Disease: Ichthyosis prematurity syndrome
- A case of a Japanese neonate with congenital ichthyosiform erythroderma diagnosed as Netherton syndrome
- A case of severe trichothiodystrophy 3 in a neonate due to mutation in the GTF2H5 gene: Clinical report
- A common deletion mutation in European patients with Sjögren-Larsson syndrome
- A compound heterozygous mutation of the SPINK5 gene in a Taiwanese boy with Netherton syndrome
- A founder mutation for ichthyosis prematurity syndrome restricted to 76 kb by haplotype association
- A Japanese infant with localized ichthyosis linearis circumflexa on the palms and soles harbouring a compound heterozygous mutation in the SPINK5 gene
- A new SLC27A4 mutation associated with ichthyosis prematurity syndrome and compartment syndrome
- A Nonsense Variant in the <em>ST14</em> Gene in Akhal-Teke Horses with Naked Foal Syndrome
- A novel mutation in the connexin 26 gene (GJB2) in a child with clinical and histological features of keratitis-ichthyosis-deafness (KID) syndrome
- A spontaneous Fatp4/Scl27a4 splice site mutation in a new murine model for congenital ichthyosis
- Altered lamellar body secretion and stratum corneum membrane structure in Netherton syndrome: differentiation from other infantile erythrodermas and pathogenic implications
- An infant with Netherton syndrome and persistent pulmonary hypertension requiring extracorporeal membrane oxygenation
- Ankyloblepharon-ectodermal dysplasia-clefting syndrome misdiagnosed as epidermolysis bullosa and congenital ichthyosiform erythroderma: Case report and review of published work
- Antenatal Findings of Keratitis-Ichthyosis-Deafness Syndrome
- Assignment of the locus for ichthyosis prematurity syndrome to chromosome 9q33.3-34.13
- Autosomal recessive congenital ichthyosis caused by a pathogenic missense variant in CLDN1
- Bi-allelic TARS Mutations Are Associated with Brittle Hair Phenotype
- Brain and cerebellar hemidysplasia in a case with ipsilateral body dysplasia and suspicion of CHILD syndrome
- Ciliopathy: Sjögren-Larsson Syndrome
- Claudin-1 gene mutations in neonatal sclerosing cholangitis associated with ichthyosis: a tight junction disease
- Clinical and genetic characterization of Chanarin-Dorfman syndrome patients: first report of large deletions in the ABHD5 gene
- Clinical and molecular characteristics of autosomal recessive congenital ichthyosis in Thailand
- Clinical and molecular implications of structural changes to desmosomes and corneodesmosomes
- Clinical expression and new SPINK5 splicing defects in Netherton syndrome: unmasking a frequent founder synonymous mutation and unconventional intronic mutations
- Collodion baby and respiratory distress syndrome
- Comparative functional characterization of novel non-syndromic <em>GJB2</em> gene variant p.Gly45Arg and lethal syndromic variant p.Gly45Glu
- Complex case study: nursing care of an infant with restrictive dermopathy
- Congenital diaphragmatic hernia and early lethality in PIGL-related disorder
- Congenital erythroderma
- Corneodesmosomal cadherins are preferential targets of stratum corneum trypsin- and chymotrypsin-like hyperactivity in Netherton syndrome
- Disruption of AP1S1, causing a novel neurocutaneous syndrome, perturbs development of the skin and spinal cord
- EGFR mutations cause a lethal syndrome of epithelial dysfunction with progeroid features
- Epidermal barrier abnormalities in exfoliative ichthyosis with a novel homozygous loss-of-function mutation in CSTA
- Epidermal hyperproliferation in mice lacking fatty acid transport protein 4 (FATP4) involves ectopic EGF receptor and STAT3 signaling
- Experience of low-dose dexamethasone use in the respiratory management of ichthyosis prematurity syndrome
- Fatal neonatal nephrocutaneous syndrome in 18 Roma children with EGFR deficiency
- FATP4 missense and nonsense mutations cause similar features in Ichthyosis Prematurity Syndrome
- Fatty acid transport protein 1 can compensate for fatty acid transport protein 4 in the developing mouse epidermis
- Fatty acid transport protein 4 is required for incorporation of saturated ultralong-chain fatty acids into epidermal ceramides and monoacylglycerols
- Fatty acid transporters in skin development, function and disease
- Genetic activation of Nrf2 reduces cutaneous symptoms in a murine model of Netherton syndrome
- Haim-Munk syndrome and Papillon-Lefèvre syndrome are allelic mutations in cathepsin C
- Ichthyosis prematurity syndrome caused by a novel fatty acid transport protein 4 gene mutation in a German infant
- Ichthyosis prematurity syndrome caused by a novel missense mutation in FATP4 gene-a case report from India
- Ichthyosis Prematurity Syndrome due to a Novel SLC27A4 Homozygous Mutation in an Italian Patient
- Ichthyosis prematurity syndrome in two Omani siblings, caused by homozygous c.1A > G mutation in the FATP4 gene
- Ichthyosis prematurity syndrome in two Omani siblings, caused by homozygous c.1A > G mutation in the FATP4 gene
- Ichthyosis prematurity syndrome mimics keratitis-ichthyosis-deafness syndrome at birth: Use of electron microscopy and genetic testing
- Ichthyosis prematurity syndrome with separation of fetal membranes and neonatal asphyxia
- Ichthyosis prematurity syndrome: a case report and review of known mutations
- Ichthyosis Prematurity Syndrome: A Rare Form but Easily Recognizable Ichthyosis
- Ichthyosis prematurity syndrome: a well-defined congenital ichthyosis subtype
- Ichthyosis prematurity syndrome: clinical evaluation of 17 families with a rare disorder of lipid metabolism
- Ichthyosis Prematurity Syndrome: From Fetus to Adulthood
- Ichthyosis prematurity syndrome: Two new cases
- Identification of a premature stop codon mutation in the PHGDH gene in severe Neu-Laxova syndrome-evidence for phenotypic variability
- Identification of novel FATP4 mutations in a Japanese patient with ichthyosis prematurity syndrome
- Impacts of deletion and ichthyosis prematurity syndrome-associated mutations in fatty acid transport protein 4 on the function of RPE65
- Inherited ichthyoses: molecular causes of the disease in Czech patients
- Interactions between FATP4 and ichthyin in epidermal lipid processing may provide clues to the pathogenesis of autosomal recessive congenital ichthyosis
- Intronic mutations affecting splicing of MBTPS2 cause ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome
- Keratitis-ichthyosis-deafness syndrome with lethal p.Ala88Val variant and severe hypercalcemia
- LEKTI demonstrable by immunohistochemistry of the skin: a potential diagnostic skin test for Netherton syndrome
- LEKTI is localized in lamellar granules, separated from KLK5 and KLK7, and is secreted in the extracellular spaces of the superficial stratum granulosum
- Lethal, neonatal ichthyosis with increased proteolytic processing of filaggrin in a mouse model of Netherton syndrome
- Management of congenital ichthyoses: European guidelines of care, part two
- Matriptase initiates activation of epidermal pro-kallikrein and disease onset in a mouse model of Netherton syndrome
- Mixed sclerosing dysplasia of the bone associated with ovarian and skin problems
- Molecular analysis of a series of Israeli families with Comèl-Netherton syndrome
- Mutations in the fatty acid transport protein 4 gene cause the ichthyosis prematurity syndrome
- Myeloid-specific fatty acid transport protein 4 deficiency induces a sex-dimorphic susceptibility for nonalcoholic steatohepatitis in mice fed a high-fat, high-cholesterol diet
- Neonatal skin barrier: structure, function, and disorders
- Netherton syndrome with extensive skin peeling and failure to thrive due to a homozygous frameshift mutation in SPINK5
- Netherton syndrome: disease expression and spectrum of SPINK5 mutations in 21 families
- Netherton syndrome: report of two Taiwanese siblings with staphylococcal scalded skin syndrome and mutation of SPINK5
- Novel compound heterozygous FATP4 mutations caused ichthyosis prematurity syndrome in Spanish sisters
- Novel duplication mutation in the patatin domain of adipose triglyceride lipase (PNPLA2) in neutral lipid storage disease with severe myopathy
- Novel mutations in FATP4 gene in two families with ichthyosis prematurity syndrome
- Ocular manifestations in the inherited DNA repair disorders
- Permanent neonatal diabetes with arthrogryposis multiplex congenita and neurogenic bladder - a new syndrome?
- Pleomorphic ichthyosis: proposed name for a heterogeneous group of congenital ichthyoses with phenotypic shifting and mild residual scaling
- Prenatal sonographic assessment and perinatal course of ichthyosis prematurity syndrome
- Preterm birth in Sjögren-Larsson syndrome
- Requirement of fatty acid transport protein 4 for development, maturation, and function of sebaceous glands in a mouse model of ichthyosis prematurity syndrome
- Role of fatty acid transport protein 4 in metabolic tissues: insights into obesity and fatty liver disease
- Role of fatty acid transporters in epidermis: Implications for health and disease
- Severe hypernatremic dehydration in an infant with Netherton syndrome
- Sjögren-Larsson syndrome in clinical practice
- Skin permeability barrier formation by the ichthyosis-causative gene <em>FATP4</em> through formation of the barrier lipid ω-<em>O</em>-acylceramide
- SPINK5 and Netherton syndrome: novel mutations, demonstration of missing LEKTI, and differential expression of transglutaminases
- The Causative Gene in Chanarian Dorfman Syndrome Regulates Lipid Droplet Homeostasis in C. elegans
- The prevalence, risk of premature births, mortality and causes of death of cleft lip with or without palate in South Korea: a nationwide population-based cohort study
- The spectrum of pathogenic mutations in SPINK5 in 19 families with Netherton syndrome: implications for mutation detection and first case of prenatal diagnosis
- Trichothiodystrophy: from basic mechanisms to clinical implications
- Trichothiodystrophy: PIBIDS syndrome
- Truncating mutation in intracellular phospholipase A₁ gene (DDHD2) in hereditary spastic paraplegia with intellectual disability (SPG54)
- Understanding fetal factors that contribute to preterm birth: Sjögren-Larsson syndrome as a model
- Upregulation of interleukin-33 in the epidermis of two Japanese patients with Netherton syndrome
- Vitamin D deficiency rickets complicating Dorfman-Chanarin syndrome
- Vulnerable child syndrome in the neonatal intensive care unit: A review and a new preventative intervention with feasibility and parental satisfaction data