Magnetic resonance imaging based kidney volume assessment for risk stratification in pediatric autosomal dominant polycystic kidney disease

IntroductionIn the pediatric context, most children with autosomal dominant polycystic kidney disease (ADPKD) maintain a normal glomerular filtration rate (GFR) despite underlying structural kidney damage, highlighting the critical need for early intervention and predictive markers. Due to the inverse relationship between kidney volume and kidney function, risk assessments have been presented on the basis of kidney volume. The aim of this study was to use magnetic resonance imaging (MRI)-based kidney volume assessment for risk stratification in pediatric ADPKD and to investigate clinical and genetic differences among risk groups.MethodsThis multicenter, cross-sectional, and case-control study included 75 genetically confirmed pediatric ADPKD patients (5–18 years) and 27 controls. Kidney function was assessed by eGFR calculated from serum creatinine and cystatin C using the CKiD-U25 equation. Blood pressure was assessed by both office and 24-hour ambulatory measurements. Kidney volume was calculated from MRI using the stereological method. Total kidney volume was adjusted for the height (htTKV). Patients were stratified from A to E classes according to the Leuven Imaging Classification (LIC) using MRI-derived htTKV.ResultsMedian (Q1-Q3) age of the patients was 6.0 (2.0–10.0) years, 56% were male. There were no differences in sex, age, height-SDS, or GFR between the patient and control groups. Of the patients, 89% had PKD1 and 11% had PKD2 mutations. Non-missense mutations were 73% in PKD1 and 75% in PKD2. Twenty patients (27%) had hypertension based on ABPM. Median htTKV of the patients was significantly higher than controls (141 vs. 117 ml/m, p = 0.0003). LIC stratification revealed Classes A (38.7%), B (28%), C (24%), and D + E (9.3%). All children in class D + E and 94% in class C had PKD1 variants. Class D + E patients had significantly higher blood pressure values and hypertension compared to other classes (p > 0.05 for all).DiscussionThis study distinguishes itself by using MRI-based measurements of kidney volume to stratify pediatric ADPKD patients into specific risk groups. It is important to note that PKD1 mutation and elevated blood pressure were higher in the high-risk groups stratified by age and kidney volume. Our results need to be confirmed in further studies

Brain MRI findings in infants with primary congenital glaucoma

<b>Background: </b> Congenital glaucoma appears in the first months of life, eventually at birth. Isolated congenital glaucoma is characterized by minor malformations of the irido-corneal angle of the anterior chamber of the eye. Clinical manifestations include tearing, photophobia and enlargement of the globe appearing in the first months of life. Imaging technology such as optical coherence tomography and measurement of central corneal thickness may play an important role in the assessment of children with suspected or known glaucoma. However, no MRI findings of the CNS in patients with primary congenital glaucoma (PCG) were reported in the literature. The purpose of this study was to investigate MRI findings of the brain in infants with PCG. <b> Methods: </b> We reviewed the radiological, histopathological and clinical characteristics of infants with primary congenital glaucoma. The records of 17 patients with PCG were reviewed and the MRIs of the brain and associated manifestations were analyzed. <b> Results: </b> Three patients with PCG had abnormal MRI findings suggesting agenesis of the corpus callosum. Two infants had delayed myelinization of the brain. <b> Discussion: </b> Significant abnormal optic nerve excavation and increased corneal diameters in 2 patients with delayed myelinization may suggest that intraocular pressure can be more striking and more severe, revealing a close relationship with PCG and abnormal myelinization in the white matter. Studies with more patients are needed to confirm these results

THE MANAGEMENT OF TUBO-OVARIAN ABSCESS

Objective: To determine the management of tubo-ovarian abscess (TOA) and put out the complications in patients operated on

Histopathological effects of maternal hair dye use on the cornea

The aim of this study is to investigate and compare the histopathological effects of hair dye additives, 2-amino-5-nitrophenol (2A5NP) and 2-nitro-p-phenylendiamin (2NPPD) on cornea of neonates from pregnant rats that have been administered these additives subcutaneously. The study included 90 neonates of 26 nulligravida wistar-albino rats among which ten were given 100 mg/kg/day 2A5NP (Group I), ten rats received 150 mg/kg/day 2NPPD (Group II) and control rats received saline (Group III) injections subcutaneously between 7th and 15th gestational days. No sign of toxicity was observed during the treatment and there was no gross abnormality in both the study and control groups. Histopathological changes of cornea were seen in 22 of 30 newborn rats in Group I (73.4%), in 23 of 30 rats in Group II (76.7%) and only 5 of 30 rats in the control saline injected Group III (16.7%). Histopathological effect of the two additives were statistically significant when compared to the control group (Chi-square: 27.63, p = 0.0001), but there was no difference between the effects of 2A5NP and 2NPPD additives on cornea (Chisquare:0.089, p = 0.766). The present experimental study on rats confirmed the histopathological effect of 2A5NP and 2NPPD on cornea beyond doubt. In the light of which, we can speculate that maternal exposure of hair dyes during pregnancy has some teratogenic effects on newborn rat cornea

RHO Gene Polymorphisms in Patients with Pterygium

Pterygium is one of the most common ocular surface diseases, and characterized by inflammatory infiltrates, proliferation, fibrosis, angiogenesis, and extracellular matrix breakdown. We investigated the association of polymorphisms in the RHO genes RHOA, RHOB, RHOC, RHOD, and RND3 (RHOE). The results of this study demonstrate for the first time the association of RHO genes with the pterygium. We displayed that the RHO gene polymorphisms were significantly associated with pterygium in the Turkish population

Protein Expressions of the Small GTPase Rho Proteins in Pterygial Tissue and Leukocytes of Patients with Pterygium

Pterygium is a benign fibrovascular proliferation that develops from the conjunctiva and invades the cornea. The etiology of this disorder remains unclear. Current treatment of pterygium is surgical. The postoperative recurrence rate of pterygium is reported be high. To the best of our knowledge, these results are the first to demonstrate the contribution of proteins expressions of the small GTPase Rho proteins in patients with pterygium. Our data showed that leukocyte RhoA, RhoB, RhoD, and RhoE protein expressions were markedly elevated in primary pterygium. However, no significant modifications were noted in pterygial tissues

The effects of Nigella sativa oil, thymoquinone, propolis, and caffeic acid phenethyl ester on radiation-induced cataract

The findings obtained in the study might suggest that propolis, CAPE, NSO, and TQ could prevent cataractogenesis in ionizing radiation-induced cataracts in the lenses of rats, wherein propolis and NSO were found to be more potent