Objectives: To assess the prevalence of congenital heart block (CHB) and electrocardiographic (ECG) abnormalities in infants of anti-Ro/SSA-positive women.
Methods: Sixty anti-Ro-positive and 36 anti-Ro-negative patients were prospectively followed before/during pregnancy and underwent weekly fetal echocardiography from 18th to 26th weeks of gestational age. Infants' ECG and/or ECG-Holter were performed at 1, 3, 6 and 12 months. ECG of 200 consecutive neonates were used as a healthy control group.
Results: One of 61 fetuses of anti-Ro-positive mothers developed CHB (20th week); another anti-Ro-positive baby developed second degree atrioventricular (AV) block (30th week). The prevalence of transient first degree AV block detected post-natally was significantly higher in the anti-Ro-positive group, in comparison with healthy controls (P = 0.002). No differences in corrected QT (QTc) interval prolongation prevalence (>/=440 ms) was observed between the anti-Ro-positive and -negative groups, but both were significantly higher than that of the control population (P < 0.001). ECG-Holter showed QTc prolongation in 59% of infants of anti-Ro-positive and in 60% of infants of anti-Ro-negative mothers. Holter QTc was >/=470 ms in four infants of anti-Ro-positive group and two of anti-Ro-negative group. Known acquired causes of QTc prolongation were excluded.
Conclusions: This prospective study confirms the low occurrence of CHB in newborns from anti-Ro-positive mothers. ECG abnormalities (first degree AV block and QTc interval prolongation) are frequent in infants of mothers with autoimmune diseases, independently of maternal disease, autoantibody profile and treatment during pregnancy.
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http://dx.doi.org/10.1093/rheumatology/kem073 | DOI Listing |
BMJ Case Rep
January 2025
Division of Neonatology, Kasturba Medical College Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India.
We report a neonate evaluated for hepatomegaly during hospitalisation and was diagnosed to have hepatoblastoma, an uncommon childhood malignancy. The presence of dysmorphism, macrosomia and congenital heart defect led to the suspicion of congenital overgrowth conditions. The genetic evaluation revealed a pathogenic variant, conclusive of Simpson-Golabi-Behmel syndrome type 1 (SGBS1).
View Article and Find Full Text PDFPediatr Neurol
January 2025
Department of Radiology, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China; Shaanxi Engineering Research Center of Computational Imaging and Medical Intelligence, Xi'an, China. Electronic address:
Background: Preterm infants are at high risk for subsequent neurodevelopmental disability. Early developmental characterization of brain and neurobehavioral function is critical for identifying high-risk infants. This study aimed to elucidate the early evolution of sensorimotor function in preterm neonates by exploring postnatal age-related changes in the brain white matter (WM) and neurobehavioral abilities.
View Article and Find Full Text PDFArq Bras Oftalmol
January 2025
Department of Ophthalmology, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brazil.
Purpose: Congenital epiphora can be related to anomalies of the nasolacrimal duct. This study aimed to assess the distal end of the nasolacrimal duct and the outcomes of endoscopic treatment in children older than 12 months with congenital epiphora.
Methods: This retrospective analysis describes the clinical characteristics, management, and outcomes of symptomatic congenital lacrimal obstruction in 32 lacrimal systems of 23 children.
Riga-Fede disease (RFD) is a rare, benign condition marked by traumatic ulceration on the tongue's ventral side in infants. It arises from friction between the tongue and lower incisors during sucking, potentially worsening into a keratinized lesion if the cause is not addressed. This report details the case of a 1-year-6-month-old male with hydrocephalus, cleft palate, corpus callosum dysgenesis, neuropsychomotor developmental delay, and tracheostomy and gastrostomy needs.
View Article and Find Full Text PDFClin Pediatr (Phila)
February 2025
Department of Pediatric Surgery, St. John's Medical College Hospital, Bangalore, Karnataka, India.
Renal histologic changes in congenital ureteropelvic junction obstruction (UPJO), although well documented, are sparsely studied in children. This study aims to establish a histological grading depending on the glomerular and tubulo-interstitial changes in hydronephrotic kidneys and determine correlation with age at surgery and impact on function post-pyeloplasty. A renal cortical wedge biopsy was obtained after pyeloplasty and histological changes were graded from 1 to 4.
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