We aimed to discuss the prenatal diagnosis and pathological features of sirenomelia, and to review current embryogenic theories. We observed two sirenomelic fetuses that were at the 19th and 16th gestational week respectively. In the former, transvaginal ultrasound revealed severe oligohydramnios and internal abortion, whereas bilateral renal agenesis, absence of a normally tapered lumbosacral spine, and a single, dysmorphic lower limb were detected in the latter. In both cases, X-rays and autoptic examination allowed categorization on the basis of the skeletal deformity. Subtotal sacrococcygeal agenesis was present in both cases. Agenesis of the urinary apparatus and external genitalia and anorectal atresia were also found. Classification of sirenomelia separately from caudal regression syndrome is still debated. Recent advances in the understanding of axial mesoderm patterning during early embryonic development suggest that sirenomelia represents the most severe end of the caudal regression spectrum. Third-trimester ultrasonographic diagnosis is usually impaired by severe oligohydramnios related to bilateral renal agenesis, whereas during the early second trimester the amount of amniotic fluid may be sufficient to allow diagnosis. Early antenatal sonographic diagnosis is important in view of the dismal prognosis, and allows for earlier, less traumatic termination of pregnancy.
Objective. To determine the efficacy of dynamic gadolinium-enhanced magnetic resonance imaging (MRI) of the wrist in the evaluation of disease activity in patients with rheumatoid arthritis (RA).Methods. Thirty-six patients with RA (with different degrees of disease activity) and 5 healthy controls were studied. MRI was performed with a low-field (0.2T), extremity-dedicated machine. After an intravenous bolus injection of gadolinium-diethylenetriamine pentaacetic acid, 20 consecutive fast spin-echo images of 3 slices of the wrist were obtained every 18 seconds.Results. The curves of synovial membrane enhancement identified the following 2 groups: controls and RA patients in remission, and RA patients with active or intermediately active disease. Both the rate of early enhancement (REE) and relative enhancement (RE) were significantly higher in patients with active RA than in those with inactive RA and controls. The REE and RE were significantly correlated with the number of swollen joints (P < 0.00001 and P ؍ 0.003, respectively), the number of tender joints (P < 0.00001 and P ؍ 0.004, respectively), the Ritchie index (P ؍ 0.0002 for both REE and RE), the Disease Activity Score (P ؍ 0.0004 and P ؍ 0.0008, respectively), the Health Assessment Questionnaire (HAQ) (P ؍ 0.0002 and P ؍ 0.0007, respectively), early morning stiffness (P ؍ 0.001 and P ؍ 0.009, respectively), the C-reactive protein level (P ؍ 0.015 and P ؍ 0.03, respectively), the erythrocyte sedimentation rate (P ؍ 0.03, RE only), and ␣2 globulins (P ؍ 0.036 and P ؍ 0.028, respectively).Conclusion. Our data support use of dynamic MRI for discriminating active from inactive RA. Enhancement curves are associated not only with laboratory and clinical indicators of inflammation, but also with the HAQ, a relevant predictor of RA functional outcome. This technique can be repeated frequently and is an excellent candidate for the ideal method for the followup of patients with RA.
The congenital anomalies and anatomical variants of the bile and pancreatic ducts present a complex spectrum of frequent alterations, which are worthy of attention in both the clinical and surgical settings and are readily identified by MRCP.
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