<b><i>Introduction:</i></b> Distal renal tubular acidosis (dRTA) is a disease that may develop either primarily or secondarily, resulting from urinary acidification defects in distal tubules. Hearing loss may accompany primary forms of dRTA. This study aims to determine the characteristics of hearing loss due to different gene mutations in patients with dRTA. <b><i>Methods:</i></b> Behavioral and electrophysiological audiological evaluations were performed after otolaryngology examination in 21 patients with clinically diagnosed dRTA. Radiological imaging of the inner ear (<i>n</i> = 9) was conducted and results of genetic analyses using next-generation sequencing method (<i>n</i> = 16) were included. <b><i>Results:</i></b> Twenty-one patients with dRTA from 20 unrelated families, aged between 8 months and 33 years (median = 12, interquartile range = 20), participated. All patients with <i>ATP6V1B1</i> mutations (<i>n</i> = 9) had different degrees of hearing loss. There was one patient with hearing loss in patients with <i>ATP6V0A4</i> mutations (<i>n</i> = 6). One patient with the <i>WDR72</i> mutation had normal hearing. Large vestibular aqueduct syndrome (LVAS) was detected in 6 (67%) of 9 patients whose radiological evaluation results were available. <b><i>Conclusions:</i></b> LVAS is common in patients with dRTA and may influence the type and severity of hearing loss in these patients. The possibility of both congenital and late-onset and progressive hearing loss should be considered in dRTA patients. A regular audiological follow-up is essential for the early detection of a possible late-onset or progressive hearing loss in these patients.

Breast milk is the first source of omega-3 fatty acids (FA) for infants. We hypothesized that maternal omega-3 FAs supplementation affects the FA composition of breast milk. Thirtysix women received 950 mg omega-3 polyunsaturated (PUFA) supplementation per day for 9 months from 22-24 weeks of pregnancy until the 6 months of lactation and 26 women were enrolled as controls. Demographic data and nutritional status were taken by a questionnaire. Breast milk samples were collected at just after birth and 6 months of lactation. Fatty acids were analyzed by gas chromatography mass spectrometry (GC-MS). Data was assessed using SPSS 22.0 software. The supplementation increased the level of docosahexaenoic acid (DHA) and eicosapentaenoic acid (EPA) while decreased the level of saturated fatty acids (SFA) in breast milk. The ratio of omega-6/omega-3 FAs was also decreased in the supplemented mothers' milk. In conclusion the maternal supplementation of omega-3 FAs improved the DHA and EPA levels.

In the title NiII complex, [Ni(C10H11O2)2(C6H6N2O)2(H2O)2]·2H2O, the divalent Ni ion occupies a crystallographically imposed centre of symmetry and is coordinated by two O atoms from the carboxylate groups of two 2,4,6-trimethylbenzoate (TMB) ligands [Ni—O = 2.0438 (12) Å], two N atoms from the pyridyl groups of two isonicotinamide (INA) ligands [Ni—N = 2.1506 (15) Å] and two water molecules [Ni—O = 2.0438 (12) Å] in a slightly distorted octahedral geometry. The coordinating water molecules are hydrogen bonded to the non-coordinating carboxylate O atom of the TMB ligand [O...O = 2.593 (3) Å], enclosing an S(6) hydrogen-bonding motif. Two solvent water molecules are also present in the formula unit. In the crystal, a network of intermolecular N—H...O and O—H...O hydrogen bonds link the complexes into a three-dimensional array. Hirshfeld surface analysis indicates that the most important contributions for the crystal packing are from H...H (59.8%), O...H/H...O (20.2%) and C...H/H...C (13.7%) interactions.