Idiopathic Infantile Arterial Calcification (IIAC) is a rare syndrome of unknown cause characterized by disruption and calcification of the internal elastic laminae of fetal arteries with calcium deposits leading to fibrosis and occlusion of the arteries. The diagnosis is often made at post-mortem examination. However, in a few cases it has been detected prenatally as in this case. Fewer than 10 cases of antenatal detection of this condition have been reported in the literature. In our case, thrombotic microangiopathy was an added feature, which has not been reported thus far in the literature to the best of our knowledge.

Download full-text PDF

Source
http://dx.doi.org/10.3109/15513815.2010.505625DOI Listing

Publication Analysis

Top Keywords

idiopathic infantile
8
infantile arterial
8
arterial calcification
8
reported literature
8
calcification thrombotic
4
thrombotic microangiopathy--a
4
microangiopathy--a unique
4
unique case
4
case idiopathic
4
calcification iiac
4

Similar Publications

Isolated Temporomandibular Arthritis as Presentation of Juvenile Idiopathic Arthritis.

J Craniofac Surg

January 2025

Rheumatology Unit, Department of Childhood and Developmental Medicine, ASST Fatebenefratelli-Sacco, Milano, MI.

Involvement of the temporomandibular joint (TMJ) in patients with juvenile idiopathic arthritis (JIA) has been increasingly reported, affecting up to 87% of cases. This involvement generally occurs after the diagnosis of JIA has been established; however, in the authors' cases, as in a few others documented in the literature, patients presented with isolated TMJ arthritis as the sole joint involvement. The authors performed a narrative literature review on TMJ involvement in JIA and reported 2 cases that presented with isolated TMJ arthritis as the initial manifestation of JIA.

View Article and Find Full Text PDF

This study aimed to discuss the pathogenic hereditary factors of children with steroid-resistant nephrotic syndrome (SRNS) in Guangxi, China. We recruited 89 patients with SRNS or infantile NS from five major pediatric nephrology centers in Guangxi, and conducted a retrospective analysis of clinical data. Whole-exome sequencing analysis was also performed on all patients.

View Article and Find Full Text PDF

A narrative review of monogenic disorders causing nephrolithiasis and chronic kidney disease.

Nephrology (Carlton)

December 2024

Department of Clinical Pharmacy, School of Pharmacy, Tehran University of Medical Sciences, Tehran, Iran.

Article Synopsis
  • - The incidence of genetic diseases linked to chronic kidney disease (CKD) has been on the rise, prompting a review of monogenic causes related to kidney stone disorders.
  • - Identified monogenic conditions include primary hyperoxaluria, cystinuria, and Dent disease, all of which can lead to CKD and possibly progress to end-stage kidney disease (ESKD).
  • - It's important to conduct genetic testing in children with kidney stones, as a significant number of these patients may develop CKD due to underlying genetic disorders.
View Article and Find Full Text PDF
Article Synopsis
  • The study investigates the role of genetic factors in infantile hypertrophic pyloric stenosis and its associated rare diseases.
  • It involves a retrospective analysis of neonates from 2000 to 2022 who underwent surgery for this condition and had additional health complications.
  • The findings indicate that 9.8% of these patients had a rare disease, emphasizing the need for early diagnosis and treatment to improve health outcomes.
View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!