Pattern of Vascular Involvement in Egyptian Patients with Budd-Chiari Syndrome: Relation to Etiology and Impact on Clinical Presentation.

INTRODUCTION AND AIM: Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction. This work aims to analyze the pattern of vascular involvement in Egyptian patients with BCS, demonstrates its relation to etiology and shows its impact on clinical presentation. MATERIAL AND METHODS: The current retrospective study was conducted at The Tropical Medicine Department, Ain Shams University on one hundred Egyptian patients with confirmed diagnosis of primary BCS who were presented to the Budd-Chiari Study Group (BCSG) from April 2014 to May 2016 by collecting clinical, laboratory and radiological data from their medical records. RESULTS: Isolated hepatic vein occlusion (HVO) was the most common pattern of vascular involvement (43%), followed by combined HVO and inferior vena cava (IVC) compression by enlarged caudate lobe (32%), then combined HVO and IVC stenosis/webs (21%), and lastly isolated IVC occlusion (4%). Ascites was more significantly encountered in BCS patients with HVO than in those with isolated inferior vena cava (IVC) occlusion and patent HVs (P = 0.005). Abdominal pain was significantly encountered in patients with occluded three major HVs (P = 0.044). Behcet's disease was significantly detected in isolated IVC occlusion. Protein C deficiency was significantly detected in patients with combined HVO and IVC compression. CONCLUSION: 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.

Therapeutic superiority of combined propranolol with short steroids course over propranolol monotherapy in infantile hemangioma.

UNLABELLED: Infantile hemangiomas are the most common benign childhood tumor that may have functional and/or cosmetic complications. We aimed to compare the clinical efficacy of propranolol alone and propranolol primed with systemic corticosteroids on the outcome of infantile hemangioma. A prospective randomized study included 40 infants aged less than 9 months with cutaneous hemangiomas. Patients were randomly assigned into two groups: group A were given oral prednisolone for the initial 2 weeks combined with oral propranolol, while group B were given oral propranolol alone for 6 months. The median age of the studied patients was 4.5 months (ranged 4 weeks-8 months). Sequential determination of the dimensions of the hemangiomas based on direct measurement and photographic analysis were performed. A significant reduction in the size of the lesions was found in group A in the 2-, 4-, and 8-week evaluation compared to group B (p < 0.001) with no statistical difference in the ultimate 6 month response (p = 0.134). Multiple logistic regression showed that early treatment before 6 months of age (OR 9.82, p = 0.007) and combined treatment with propranolol and prednisolone (OR 10.71, p = 0.006) were the predictors of best response. CONCLUSION: Combining propranolol with corticosteroids gives a faster response and should be considered in treating life- or function-threatening hemangiomas.

Uterine development in patients with Turner syndrome: relation to hormone replacement therapy and karyotype.

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-five 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 configuration, even at a late start of hormone replacement therapy and regardless of karyotype.