Increased incidence of renal and urinary tract anomalies among individuals with fetal alcohol spectrum disorders (FASD).

BACKGROUND: Fetal alcohol spectrum disorders (FASD) in a spectrum of neurodevelopmental conditions resulting from prenatal alcohol exposure (PAE). Animal models have confirmed the toxic effects of PAE on the kidneys and urinary tract, yet the evidence from human studies is contradictory. The purpose of this study was to establish the incidence of renal and urinary tract anomalies (RUTA), impaired kidney function, and hypertension among patients with FASD. METHODS: Children from the FASD Diagnostic Center with FASD diagnosis (FAS, pFAS, or ARND) were offered participation in the study. The control group consisted of patients from the Gastroenterology Department of the same hospital. The patients underwent renal and urinary tract ultrasound examination. The serum creatinine level was also evaluated and the blood pressure was taken twice. Polish OLAF charts were used to determine the percentiles of blood pressure. RESULTS: The incidence of kidney and urinary tract defects in the study group was significantly higher than in the control group (OR: 2.64 [1.60-4.34]). The kidney size among FASD patients was significantly lower (73 mm [60-83] vs. 83 mm [70-96]; p < .001) when compared to the control group. No differences were observed in the estimated glomerular filtration rate. In the study group, significantly lower systolic blood, diastolic blood pressure, percentile of systolic pressure, and diastolic pressure were observed. CONCLUSIONS: RUTA occurred more frequently among patients with FASD compared to the control group, and decreased kidney size was also demonstrated among patients with FASD. However, impaired kidney function and the risk of hypertension were not observed.

Establishing percentile charts for hip joint capsule and synovial cavity thickness in apparently healthy children.

BACKGROUND: The usefulness of musculoskeletal ultrasonography (MSUS) in paediatric population is limited by lack of reference values. One of such parameters is hip joint capsule thickness, postulated as an early measure for synovitis. However, the joint capsule is hardly a distinguished structure from slit synovial cavity in patients with little or no fluid collection. Therefore, in patients without effusion, it is more convenient to measure hip joint capsule thickness together with synovial cavity. The aim of the study was to establish percentile chart for hip joint capsule and synovial cavity thickness (HJC&SCT) in apparently healthy children. MATERIAL AND METHODS: The analysis included 816 US of hip joint in 408 children without musculoskeletal disorders, distributed equally throughout the whole developmental period in 18 one-year subgroups. Hip joints US was performed according to standard protocol including measurement of HJC&SCT in a single rheumatology centre by three investigators. RESULTS: The 3rd, 10th, 25th, 50th, 75th, 90th, and 97th HJC&SCT percentile curves were depicted in the age and height charts for the combined group of girls and boys. The median HJC&SCT values were increasing with age from 3.7 (C10 - C90: 3.3 - 4.2) mm in the first year of life up to 6.7 (5.8 - 7.3) in 16 years old, and above. In a similar way the increase was seen with height from 3.9 (3.5 - 4.7) mm in shorter than 95 cm to 6.9 (6.2 - 7.4) mm in taller than 169 cm subjects. Intra-observer and inter-observer mean precision was less than 1.8 and 12.5%, respectively. CONCLUSION: The developed centile chart for hip joint capsule and synovial cavity thickness in the paediatric population is expected to improve detection of hip joint capsule disorders, including synovitis in juvenile idiopathic arthritis.

Ultrasonographic Features of Hip Joints in Mucopolysaccharidoses Type I and II.

OBJECTIVES: The primary aim of this study was to assess the ultrasonographic features of hip joints in patients with mucopolysaccharidosis (MPS) type I and II in comparison with healthy population. The secondary aims were to correlate these features with clinical measures and to evaluate the utility of ultrasound in the diagnosis of MPS disease. MATERIALS AND METHODS: Sixteen MPS I (n = 3) and II (n = 13) patients were enrolled in the present study and underwent clinical and radiological evaluation, and bilateral high-resolution ultrasonography (US) of hip joints. The distance from the femoral neck to joint capsule (synovial joint space, SJS), joint effusion, synovial hyperthrophy, and local pathological vascularization were evaluated. The results were compared to the healthy population and correlated with clinical and radiological measures. RESULTS: 1. There was a difference in US SJS between children with MPS disease and the normative value for healthy population (7mm). Mean values of SJS were 15.81 ± 4.08 cm (right hip joints) and 15.69 ± 4.19 cm (left joints). 2. No inflammatory joint abnormalities were detected in MPS patients. 3. There was a clear correlation between US SJS and patients' age and height, while no clear correlation was observed between SJS and disease severity. CONCLUSIONS: 1. Patients with MPS I and II present specific features in hip joint ultrasonography. 2. The data suggests that ultrasonography might be effective in the evaluation of hip joint involvement in patients with MPS and might present a valuable tool in facilitating the diagnosis and follow up of the disease.