Effects of potassium citrate/citric acid intake in a mouse model of polycystic kidney disease

The kidney function in a model of autosomal dominant polycystic kidney disease (PKD), the Han:SPRD rat, is dramatically improved by chronic ingestion of a solution of potassium citrate and citric acid (KCitr). This study investigated whether this treatment would also be beneficial in the pcy/pcy mouse, a model of autosomal recessive PKD. Starting at 1 month of age, male CD-1 pcy/pcy and normal CD-1 mice were provided with a solution of 55 mM K(3) citrate/67 mM citric acid or tap water to drink. The pcy/pcy mice on the KCitr solution failed to grow normally and showed elevated plasma urea levels when compared to water-drinking littermates. Growth of normal CD-1 mice was not affected by KCitr intake. The pcy/pcy mice were then provided with a more dilute solution of KCitr to drink: this resulted in greater kidney wet and dry weights and a higher kidney weight/body weight ratio, but no beneficial effects. We conclude that pcy/pcy mice cannot tolerate a high level of KCitr intake and that a lower level is of no benefit. Whether KCitr therapy would be helpful in patients with PKD is still an open question.     

A new surgical technique for ocular fixation in congenital third nerve palsy.

PURPOSE: To present a new technique of ocular fixation to restore and maintain the ocular alignment in primary position for patients with total third nerve paralysis. METHOD: We fixated the globe (medial rectus muscle insertion) to the medial palpebral ligament insertion at the anterior lacrimal crest by using nonabsorbable 5-0 polyester sutures in a prospective study of 5 patients (5 eyes) with congenital total third nerve paralysis. A large recession of the lateral rectus muscle (12 to 16 mm) was also performed in four patients. RESULTS: Four patients achieved satisfactory ocular alignment and one patient had residual exotropia. After an initial exotropic shift, no significant change in ocular alignment was observed during the follow-up period of 6 to 9 months. Mild fullness and congestion over the medial rectus muscle area was observed in the immediate postoperative period in all the patients, which resolved in about two months time. CONCLUSION: This technique of ocular fixation is easy, safe, and effective for the management of exotropia secondary to total third nerve paralysis.     

Comparison of clinical and biometric characteristics between nanophthalmic children and age-matched controls

Purpose:To compare the clinical and biometric characteristics of children presenting with nanophthalmos (NO group) with that of age-matched controls (CO group).Methods: Electronic medical records of 40 children (<18 years of age) with diagnosis of nanophthalmos (NO), presented to a tertiary center in Tamil Nadu between January 2010 and December 2019, were reviewed and compared with 30 age-matched controls (CO) presenting for routine eye examination between October 2019 and December 2019. Clinical parameters compared were best-corrected visual acuity (BCVA), axial length (AxL), keratometry (K), anterior chamber depth (ACD), lens thickness (LT), retinochoroidal scleral thickness (RCS), corneal diameter, central corneal thickness (CCT), intraocular pressure (IOP), lens axial length factor (LAF), and lens thickness/anterior chamber depth ratio (LT/ACD).Results: Mean age of the NO group was 8.95 ± 4.0 years. Mean spherical equivalent (SE) in NO group was 10.87 ± 3.1 D and was inversely correlated to AxL (r = −0.46, P value = 0.003). All biometric parameters (AxL, ACD, LT, RCS, LAF, and LT/ACD), except CCT were significantly different between NO and CO groups. NO group children had 52.5% visual impairment with BCVA ≤ 6/24 and 17.5% had esotropia. Common ocular associations in NO group were amblyopia (64.3%), primary angle-closure glaucoma (PACG) (17.8%), pigmentary retinopathy (14.3%), and retinal detachment (3.6%). Angle-closure disease was seen in 50% of NO group and 30% underwent laser peripheral iridotomy (LPI). There was a significant difference in SE, ACD, and LAF among NO children with AxL <17 mm or >17 mm. Multivariable regression analysis revealed a significant correlation of SE and ACD with AxL.Conclusion: Nanophthalmos in children often present as amblyopia with visual impairment and strabismus. NO group with AxL <17 mm, had angle-closure disease as a common association with significantly lower ACD, higher SE, and LAF. All morphometric characteristics, except CCT, were significantly different between NO and CO groups. Close monitoring with serial biometry in NO group is needed for the timely diagnosis and prompt intervention to avoid visual impairment, due to glaucoma.     

Visual impairment in children with multiple disabilities in schools for children with special needs in South India

Purpose:To understand/assess ocular and functional vision impairment in children with multiple disabilities with a functional vision assessment battery in addition to standard ophthalmic examinations in an outreach setting.Methods:Seven schools for children with special needs, 243 children in total, were screened for ocular disorders and functional vision impairment through school camps.Results:Among them, 37% had refractive errors needing spectacle correction. With standard ocular testing methods, the visual impairment was around 32%, but when functional vision was assessed, the functional vision impairment amounted to 70% in these children. The presence of functional vision impairment was found to be independent of the associated disability. Assessment of visual capacities such as visual closure, saccade pursuits, optic ataxia, and developmental milestones early on can help in suspecting the presence of CVIConclusion:Children with multiple disabilities are more at risk of functional vision impairment, which significantly impairs their ability to function in daily life. A complete functional vision assessment becomes essential to plan early intervention for these children. The significant proportion of vision impairment and functional vision loss in our study indicates the need for coordinated structured programs to address vision-related problems in children with multiple disabilities.