Central Auditory Processing Impairment in Renal Failure.

This study is the first to investigate central auditory processing impairment in patients with slight decrease in renal function (PSR), who had no risk factors, evidence of renal injury, and albuminuria. The aim was to determine the scores of dichotic digits test (DDT) and word-in-noise perception (WINP) test in PSR. The survey was cross-sectional-comparative. The case group consisted of 30 PSR, with an estimated glomerular filtration rate of 60-90 mL/min at 1.73 m2. The control group consisted of 60 normal participants. They were matched with the case group based on sex, education level, dominant hand and age. The measures were the 28-item general health questionnaire, mini-mental state examination, Petersburg sleep quality index, acoustic immittance assessment, pure tone audiometry, speech reception threshold evaluation, DDT and WINP test. Both groups had normal stress levels, night sleep, mental states, and hearing thresholds. The mean scores of the WINP test in the right and left ears of the case group were significantly different from the control group (PvRight = 0.026, PvLeft = 0.029 ). The mean difference of DDT scores in the right and left ears of the case group compared to the control group showed significant difference (PvRight = 0.039, PvLeft = 0.048 ). Therefore, slight decrease in renal function can be one of the causes of central auditory processing impairment. Affected patients with normal hearing thresholds may have difficulty in discrimination the pitch of words, and speech perception in competing situations. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-04345-5.

Prevalence of reflux nephropathy in Iranian children with solitary kidney: results of a multi-center study.

BACKGROUND: Given the importance of the function of the remnant kidney in children with unilateral renal agenesis and the significance of timely diagnosis and treatment of reflux nephropathy to prevent further damage to the remaining kidney, we aimed to determine the prevalence of reflux nephropathy in this subgroup of pediatric patients. METHODS: In general, 274 children referred to pediatric nephrologists in different parts of Iran were evaluated, of whom 199 had solitary kidney and were included in this cross-sectional study. The reasons for referral included urinary tract infection (UTI), abnormal renal ultrasonography, being symptomatic, and incidental screening. Demographic characteristics, including age and gender were recorded. History of UTI and presence of vesicoureteral reflux (VUR) were evaluated. RESULTS: Of the 274 children evaluated in this study with the mean age (SD) of 4.71 (4.24) years, 199 (72.6%) had solitary kidney. Among these, 118 (59.3%) were male and 81 (60.7%) were female, 21.1% had a history of UTI, and VUR was present in 23.1%. The most common cause of referral was abnormal renal ultrasonography (40.2%), followed by incidental screening (21.1%), being symptomatic (14.1%), and UTI (5.5%). In 116 children (58.3%), the right kidneys and in 83 (41.7%) the left kidneys were absent. Besides, 14.6% of the participants had consanguineous parents and 3% had a family history of solitary kidney. Upon DMSA scan, the single kidney was scarred in 13.1%, of which only 7.5% were associated with VUR. In addition, proteinuria and hematuria were observed in 6.5% and 1.5% of children, respectively. CONCLUSIONS: The prevalence of reflux nephropathy was 7.5% in children with solitary kidney with a male predominance. Given the relatively high prevalence of reflux nephropathy in these children, screening for VUR in the remnant kidney appears to be essential in this population.

Balanced Reciprocal Translocation t(X;1) in a Girl with Tall Stature and Primary Amenorrhea.

Chromosomal translocations constitute one of the most important, yet uncommon, causes of primary amenorrhea and gonadal dysgenesis. Although X-autosome translocations are frequently associated with streak gonads and clinical features of the Turner syndrome, the majority of X-autosome carriers may present with a variable phenotype, developmental delay, and recognizable X-linked syndrome due to nonrandom X-inactivation. In this article, we describe a healthy 15.5-year-old girl with primary amenorrhea, gonadal dysgenesis, and tall stature without other manifestations of the Turner syndrome. Relevant clinical, biochemical, endocrinological, and cytogenetical evaluations were performed. Initial investigations revealed hypergonadotropic hypogonadism (FSH=134 mIU/mL [normal=10-15 mIU/mL], LH=47.5 [normal=10-15 mIU/mL], and estradiol=24.3 pmol/L). On ultrasound examination of the pelvis, streak ovaries with a hypoplastic uterus were noted. Chromosome study, performed according to routine procedures, revealed an apparently balanced reciprocal translocation involving the short arm of chromosome 1(p2) and the long arm of the X chromosome (q2) in all the cells with the following karyotype: 46,X,t(1;X)(p13;q22). She was placed on hormone replacement therapy. In our patient, X-autosome translocation was associated with gonadal dysgenesis and tall stature. We conclude that t(X;1) may be associated with gonadal dysgenesis without other congenital abnormalities. To our knowledge, normal phenotype with gonadal dysgenesis and tall stature in association with t(X;1) translocation has not been previously reported.

Correlation of cystatin C and creatinine based estimates of renal function in children with hydronephrosis.

INTRODUCTION: The use of a simple and accurate glomerular filtration rate (GFR) estimating method aiming minute assessment of renal function can be of great clinical importance. OBJECTIVES: This study aimed to determine the association of a GFR estimating by equation that includes only cystatin C (Gentian equation) to equation that include only creatinine (Schwartz equation) among children. PATIENTS AND METHODS: A total of 31 children aged from 1 day to 5 years with the final diagnosis of unilateral or bilateral hydronephrosis referred to Besat hospital in Hamadan, between March 2010 and February 2011 were consecutively enrolled. Schwartz and Gentian equations were employed to determine GFR based on plasma creatinine and cystatin C levels, respectively. RESULTS: The proportion of GFR based on Schwartz equation was 70.19± 24.86 ml/min/1.73 m(2), while the level of this parameter based on Gentian method and using cystatin C was 86.97 ± 21.57 ml/min/1.73 m(2). The Pearson correlation coefficient analysis showed a strong direct association between the two levels of GFR measured by Schwartz equation based on serum creatinine level and Gentian method and using cystatin C (r = 0.594, P < 0.001). The linear association between GFR values measured with the two methods included cystatin C based GFR = 50.8+ 0.515 × Schwartz GFR. The correlation between GFR values measured by using serum creatinine and serum cystatin C measurements remained meaningful even after adjustment for patients' gender and age (r = 0.724, P < 0.001). CONCLUSION: The equation developed based on cystatin C level is comparable with another equation, based on serum creatinine (Schwartz formula) to estimate GFR in children.

The main etiologies of acute kidney injury in the newborns hospitalized in the neonatal intensive care unit.

INTRODUCTION: Acute kidney injury (AKI) is one of the most common diseases among the newborns hospitalized in the neonatal intensive care units (NICUs), which is usually resulted from predisposing factors including sepsis, hypovolemia, asphyxia, respiratory distress syndrome (RDS), and heart failure. The goal of this study was to assess main etiologies, relevant risk factors, and early outcome of neonatal AKI. MATERIALS AND METHODS: In a cross- sectional study, 49 consecutive neonates hospitalized in NICU of Besat hospital with diagnosis of AKI from October 2009 to October 2011 were investigated through census sampling method. AKI was diagnosed based on urine output and serum creatinine levels. RESULTS: The prevalence of AKI was 1.54% (49 out of 3166 newborns hospitalized in NICU) with the female: male was 7:1. Thirty-nine patients (79.5%) were full-term neonates. Oliguria was observed in 38 (77.5%) patients. Sepsis was the most common predisposing factor for AKI in 77.5% of patients (n = 38) accompanied with the highest mortality rate among other factors (30.5%). Other leading causes of AKI included hypovolemia secondary to dehydration, followed by hypoxia secondary to RDS, patent ductus arteriosus, posterior urethral valve, asphyxia, and renal venous thrombosis. A positive relationship was observed between neonates' age, sex, urine output, and also between serum creatinine levels with initiation of dialysis. The mortality rate among the newborns hospitalized with AKI was 36.7%. Eighteen (36.7%) newborns were treated with peritoneal dialysis (PD) of whom 10 patients (55.6%) died, 31 patients were managed conservatively of whom five neonate died (25.9%). DISCUSSION: Prognosis of AKI in the oliguric neonates requiring PD is very poor. It is thus recommended to prevent AKI by predicting and rapid diagnosis of AKI in patients with potential risk factors and also by early and effective treatment of such factors in individuals with AKI.

Tuberous sclerosis with hypertension and abdominal pain in a child.

Hypertension in children is not as frequent as adults. In addition, most of the times, we encounter secondary hypertension rather than essential hypertension in children. This demands careful history taking, physical examination, and laboratory and imaging investigations to find the underlying cause. Here, a boy with tuberous sclerosis is reported who presented with hypertension and abdominal pain associated with bilateral renal cystic disease.

Continuous ambulatory peritoneal dialysis for a patient with Bardet-Biedl syndrome.

Bardet-Biedl syndrome (BBS) is a multisystem syndrome with a range of primary and secondary features. Kidney abnormalities are a major cause of morbidity and mortality in BBS and it is a significant genetic cause of chronic kidney disease in children. Conventional approach to end-stage renal disease in these patients is hemodialysis and kidney transplantation afterwards. Continuous ambulatory peritoneal dialysis, however, is not a commonly advocated modality in literature. In this report, we present a boy with BBS who underwent continuous ambulatory peritoneal dialysis, which resulted in control of kidney function impairment and better compliance for his family than hemodialysis before kidney transplantation. Of note, this is a rare case of BBS complicated with end-stage renal disease in the first decade of life.