The short-term effects of intranasal steroids on intraocular pressure in pediatric population.

PURPOSE: To evaluate the effect of intranasal mometasone furoate (INMF) on short-term intraocular pressure (IOP) alterations in children with allergic rhinitis (AR). METHODS: Children diagnosed with AR and to whom INMF nasal spray had been firstly prescribed were enrolled. Cases with any ocular diseases except for refractive errors were excluded. Complete ophthalmologic examinations including IOP measurements using Tonopen XL were performed before the treatment as well as at the first and sixth weeks of follow-up. Demographics and ophthalmologic findings were noted and statistically analyzed. RESULTS: Study population consisted of 62 right eyes of 62 children with a mean age of 8.55 ± 3.14 years. Of them, 29 were female (46.8%) and 33 were male (53.2%). Dilated fundoscopy revealed an enlarged Cup/Disc ratio in 12 eyes (19.4%). Family history of glaucoma was positive in 13 cases (21.0%). Mean best corrected visual acuity was found as 0.05 ± 0.08 logMAR. Initial IOP was 17.1 ± 2.3 mmHg; whereas it was measured as 18.2 ± 2.0 mmHg and 17.3 ± 2.1 mmHg at the first and sixth weeks of follow-up, respectively (p < 0.001). Both at the first and sixth weeks of follow-up, significant IOP rise was present in children with a positive family history of glaucoma (p < 0.001 and p = 0.003, respectively). Besides, increased IOP was found in participants with cupping revealed on fundoscopy at the first week of follow-up (p = 0.044). CONCLUSION: Since children have greater risk for steroid-induced ocular hypertensive response than adults, ophthalmologic evaluation must be recommended in children receiving intranasal steroids.

Accidentally diagnosed ocular abnormalities identified during close screening for retinopathy of prematurity.

PURPOSE: To report the variety of ocular findings which have been identified serendipitously during the screening for retinopathy of prematurity (ROP) in a tertiary referral center during seven-year period. METHODS: The charts of 1568 preterm infants who screened for ROP were reviewed retrospectively. Any ocular lesion except for ROP were noted. All infants had undergone routine ocular examination of the external eye, pupillary light reflex, anterior and posterior segment. Wide-angle digital retinal image acquisition system for any vitreoretinal pathology requiring a close follow-up had been utilized. RESULTS: Abnormal ocular findings other than ROP were diagnosed in 296 infants (19.2%). Tunica vasculosa lentis was the most common finding (25%) followed by vitreous or retinal hemorrhages (17.2%) and retinal white lesions (16.6%). Retina was the most frequently involved anatomic site. Other frequent ocular findings included optic disc cupping, congenital cataract, optic nerve hypoplasia, choroidal nevus, persistent fetal vasculature, lid hemangioma, and tilted disc. However, life-threatening pathologies such as lipemia retinalis and even retinoblastoma were also diagnosed. CONCLUSION: A duly ophthalmologic examination is mandatory in premature infants for ROP screening. During such examinations, ophthalmologists must be aware of coexisting ocular findings; which could be sight-threatening or even life-threatening.

Efficacy of Phenylalanine- and Tyrosine-Restricted Diet in Alkaptonuria Patients on Nitisinone Treatment: Case Series and Review of Literature.

INTRODUCTION: Nitisinone used in alkaptonuria (AKU) can result in keratopathy due to strongly increased tyrosine levels. METHODS: This study aimed to investigate nutritional status and changes in plasma tyrosine and phenylalanine and urinary homogentisic acid (u-HGA) levels in 8 adult AKU patients (mean age, 56.3 ± 4.7 years) who were on tyrosine/phenylalanine-restricted diet together with 2 mg/day nitisinone. RESULTS: The treatment period was 23.4 ± 6.9 months. Daily dietary protein intake was restricted to 0.8-1.0 g/kg/day. Daily tyrosine intake was restricted to 260-450 mg/day for females and 330-550 mg/day for males. Tyrosine/phenylalanine-free amino acid supplements accounted for an average of 56.1% of daily protein intake. The following assessments were performed: anthropometric and plasma tyrosine level measurements every 2 months; ophthalmological examination every 6 months, and nutritional laboratory analyses and measurements of plasma amino acids and u-HGA once in a year. It was targeted to keep the plasma tyrosine level <500 µmol/L. The plasma tyrosine level was <100 µmol/L before the treatment in all patients and around a mean of 582.5 ± 194.8 µmol/L during the treatment. The diet was rearranged if a plasma tyrosine level of >700 µmol/L was detected. The u-HGA level before and after the 1st year of treatment was 1,429.3 ± 1,073.4 mmol/mol creatinine and 33.6 ± 9.5 mmol/mol creatinine, respectively. None of the patients developed keratopathy or experienced weight loss and protein or micronutrient deficiency. CONCLUSION: AKU patients should receive tyrosine/phenylalanine-restricted diet for reducing plasma tyrosine level to the safe range. Tyrosine/phenylalanine-free amino acid supplements can be safely used to enhance dietary compliance. Keratopathy and nutrient deficiency should be frequently monitored.