Our study aimed to assess uterine development in Turner syndrome patients and its relation to dose and type of estrogen therapy; and karyotype. Pelvic ultrasound was used to assess uterine size and shape, and ovarian volume in 40 Turner syndrome patients. Information on hormone replacement therapy was collected from patients ' notes. Among the 40 patients studied, 57.5 % started estrogen therapy and 30 % were taking progestins. Sixty-fi ve per cent had immature uterus, 17.5 % had fully mature uterus and 17.5 % had transitional uterus. Uterine volume was associated with age (p < 0.001), height (p = 0.002), weight (p = 0.001), years of estrogen use (p < 0.001), estrogen dose (p = 0.016), current estrogen use (p = 0.001) and Tanner breast stage (p < 0.001). Uterine volume was not affected by the type of estrogen used (p = 0.40) and karyotype (p = 0.40). Patients with Turner syndrome treated with estrogen (of adequate dose and duration) may attain a normal, mature uterine size and confi guration, even at a late start of hormone replacement therapy and regardless of karyotype.
Isolated HVs occlusion was the most common pattern of vascular involvement in Egyptian patients with primary BCS. Vascular pattern of involvement affected the clinical presentation and was related to the underlying thrombophilia in those patients.
Aim of the work
To test the reliability of fibroscan in detection of fibrosis in patients with Budd Chiari syndrome before and after endovascular intervention (after elimination of hepatic congestion).
Background
transient elastography (TE) is a noninvasive methodology that has been used to monitor liver stiffness in patients with chronic viral hepatitis. One of the limitations for accurate assessment of liver fibrosis by TE is the liver congestion. Liver congestion can result from Budd Chiari syndrome (BCS).The treatment of BCS is through restoring the flow of the blood between the portal vein to the inferior vena cava, which will lead to decongestion of the liver.TE, will be tested after liver decongestion for proper detection of liver fibrosis.
Patients and methods
This was a prospective cohort study conducted on 25 Egyptian patients with confirmed diagnosis of primary Budd-Chiari Syndrome (BCS) in the period from June 2017 to September 2019. TE was performed three days before endovascular intervention and three months after it. Liver biopsy was taken during the intervention for assessment of METAVIR score. Comparison was done between TE assessments before and after intervention in detection of the degree of liver fibrosis in comparison to METAVIR score measured in liver biopsy.
Results
FVLM was the most common hypercoagulable cause in the involved patients.
There was significant drop in Liver Stiffness Measurements (LSM) measured three months post-intervention indicating improvement of liver fibrosis after relieving liver congestion but still not correlated to the METAVIR scores measured in the liver biopsy.
Conclusion
Liver congestion has high impact on Liver stiffness measurement giving overestimation which improves significantly after decongestion of the liver by the endovascular intervention.
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