Exstrophy-epispadias complex: are the kidneys and kidney function spared?

BACKGROUND: Exstrophy-epispadias complex (EEC) is a complex malformation of the lower abdominal wall, bladder, and pelvic floor, which necessitates multiple successive reconstruction procedures. Surgical and infectious complications are frequent. Our aim was to evaluate kidney function in these patients. METHODS: This cross-sectional study included patients with EEC, followed since birth in a pediatric urology clinic, who underwent nephrological evaluation (blood pressure (BP) measurement and blood and urine chemistries) and imaging studies (urinary tract ultrasound and DMSA kidney scan) during 2017-2020. RESULTS: Forty-three patients (29 males), median age 9 years (interquartile range 6-19), were included. Eleven (26%) used clean intermittent catheterization (CIC) for bladder drainage. At least one sign of kidney injury was identified in 32 (74%) patients; elevated BP, decreased kidney function (estimated glomerular filtration rate (eGFR) < 90 ml/min/1.73 m2), and proteinuria/albuminuria were detected in 29%, 12%, and 36% of patients, respectively. Urinary tract dilatation (UTD) was found in 13 (37%) ultrasound examinations. Parenchymal kidney defects were suspected in 46% and 61% of ultrasound and DMSA scintigraphy, respectively. UTD was significantly associated with DMSA-proven kidney defects (p = 0.043) and with elevated BP, 39% vs. 20% in those without UTD. Decreased eGFR and elevated BP were less frequent among patients on CIC than among patients who voided spontaneously: 10% vs. 14% and 18% vs. 36%, respectively. Recurrent UTIs/bacteriuria and nephro/cystolithiasis were reported by 44% and 29% patients, respectively. CONCLUSION: The high rate of signs of kidney injury in pediatric patients with EEC dictates early-onset long-term kidney function monitoring by joint pediatric urological and nephrological teams. A higher resolution version of the Graphical abstract is available as Supplementary information.

Increased rate of major birth malformations in infants with neonatal "asymmetric crying face": a hospital-based cohort study.

Asymmetric crying face (ACF) is a minor anomaly found in 3-8 per 1,000 births, which may be associated with other anomalies. Previous studies on this topic included small groups of selected subjects, resulting in large variations in findings. The aim of this study was to examine the characteristics and associated anomalies of newborn infants with ACF compared with the general population of newborn infants. The study included newborn infants delivered between 1993 and 2003 at the Department of Neonatology of Rabin Medical Center, Israel. Charts of all newborns diagnosed with ACF were reviewed for obstetric and neonatal details, then compared with non-ACF newborns. ACF was diagnosed in 258 of 67,289 newborns (0.38%), with left-side predominance (77%). Major malformations were found in 7% of ACF infants, 3.5-fold higher than in the total Israeli population. Mild anomalies were present in 15% of the ACF group, and deformations in 4.6%. There was a higher rate of forceps deliveries in the ACF group (RR = 2.73, 95% CI = 1.37-5.42). ACF was more prevalent among females, and the male:female ratio was lower in the ACF group (0.86 vs. 1.06, P = 0.05). The rate of low-birth-weight infants was 3.9% among ACF infants and 9.6% in the control group (RR = 0.41, 95% CI = 0.23-0.76). No significant between-group difference was found for rates of primiparity, macrosomia, prematurity, postmaturity, or size-for-gestational-age. Thus, ACF is associated with a high rate of major malformations. This should prompt clinicians to seek for additional birth defects in ACF infants.

Possible association of Guillain-Barré syndrome and hepatitis A vaccination.

We report a case of Guillain-Barré syndrome in a previously healthy child who received a hepatitis A vaccination (HAVRIX) 5 days before onset of symptoms. No other precipitating factor could be identified. The relevant literature is reviewed and discussed.