OBJECTIVES:Clinical-laboratory and evolutionary analysis of twenty-eight patients with Wilson's disease.METHODS:Twenty-eight children (twelve females and sixteen males) with Wilson's disease were evaluated retrospectively between 1987 and 2009, with a follow-up of 72 months (1 – 240 months). The clinical, laboratory, and histologic features at diagnosis were recorded at the end of the study.RESULTS:The median age at diagnosis was 11 years (2 – 18 years). Twelve patients were asymptomatic, seven had hepatitis symptoms, five had raised aminotransferase levels, three had hepatomegaly associated with neurological disorders, one had fulminant hepatitis with hemolytic anemia, and six patients presented with a Kayser-Fleischer ring. A histological analysis revealed that six children had chronic hepatitis, seven had cirrhosis, two had steatosis, one had portal fibrosis, and one had massive necrosis. The treatment consisted of D-penicillamine associated with pyridoxine for 26 patients. Adverse effects were observed in the other two patients: one presented with uncontrollable vomiting and the other demonstrated elastosis perforans serpiginosa. At the end of the study, all 26 treated patients were asymptomatic. Twenty-four of the patients were treated with D-penicillamine and pyridoxine, and two were treated with trientine and zinc sulfate. A liver transplant was performed in one patient with fulminant hepatitis, but the final patient died 48 hours after admission to the intensive care unit.CONCLUSIONS:Family screenings associated with early treatment are important in preventing Wilson's disease symptoms and potentially fatal disease progression. The study suggests that Wilson's disease must be ruled out in children older than two years presenting with abnormal levels of hepatic enzymes because of the heterogeneity of symptoms and the encouraging treatment results obtained so far.

Amnioreduction performed to relieve maternal symptoms did not statistically increase the risk of pregnancy complications with severe polyhydramnios in single pregnancies.

Few reports have described the clinical and prognostic characteristics of endometrial cancer, which is increasing worldwide, in large patient series in Brazil. Our objective was to analyze the clinicopathological characteristics, prognostic factors, and outcomes of patients with endometrial cancer treated and followed at a tertiary Brazilian institution over a 10-year period.This retrospective study included 703 patients diagnosed with endometrial cancer who were treated at a public academic tertiary hospital between 2008 and 2018. The following parameters were analyzed: age at diagnosis, race, body mass index, serum CA125 level before treatment; histological type and grade, and surgical stage. Outcomes were reported relative to histological type, surgical staging, serum CA125, lymph-vascular space involvement (LVSI), and lymph-node metastasis. The median patient age at diagnosis was 63 (range, 27-93) years (6.4% were <50 years). Minimally invasive surgeries were performed in 523 patients (74.4%). Regarding histological grade, 468 patients (66.5%) had low-grade endometrioid histology and 449 patients (63.9%) had stage I tumors. Tumors exceeded 2.0 cm in 601 patients (85.5%). Lymphadenectomy was performed in 551 cases (78.4%). LVSI was present in 208 of the patients' tumors (29.5%). Ninety-three patients (13.2%) had recurrent tumors and 97 (13.7%) died from their malignant disease. The robust prognostic value of FIGO stage and

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The complications of thrombocytopenic patients with autoimmune hepatitis are elevated; nevertheless, with appropriate attention and care, they can be resolved. The association between two severe pathologies appears to increase the risk of prematurity and fetal growth restriction, demanding specialized prenatal care, as well as surveillance of newborn well-being.

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Objectives The 2018 FIGO (International Federation of Gynecology and Obstetrics) cervical cancer staging system changed from a clinical system to a clinical/pathologic/radiologic system with stages IIIC1 and IIIC2 indicating positive pelvic and para-aortic lymph nodes, respectively. We evaluated a nationwide hospital database for the impact on survival of lymph node involvement. Methods The National Cancer Database from 2004-2015 was queried for patients with cervical cancer, yielding 115,819 patients. Patients with metastatic disease (22,569), non-adeno/ squamous histologies (5,909), unknown nodal status (60,695), or unknown survival time (9,473) were excluded. Survival was compared using Cox proportional hazard model based on nodal status (node-negative [N0], positive pelvic nodes [IIIC1], or positive para-aortic nodes [IIIC2]). Univariate (UVA) and multivariate analyses (MVA) were done for the overall cohort, followed by UVA by T stage. Results 17,173 patients were eligible. Lymph node involvement negatively affected survival in the overall cohort (UVA IIIC1 Hazard Ratio [HR] 2.0, p<0.001, IIIC2 HR 3.85, p<0.001, MVA IIIC1 HR 1.36, p<0.001, IIIC2 HR 2.14, p<0.001) and in FIGO stages IB-III individually. In FIGO IB, the effect of IIIC2 was most pronounced (HR=5.38, p<0.001 versus HR 1.5 p=0.001 for IIIC1 disease) compared to FIGO III (HR 1.698, p<0.001 for IIIC2 versus HR 1.19 p=0.02 for IIIC1). Within FIGO IB, there was no difference in survival for IIIC1 compared to N0 for FIGO IB1 and IB2. Conclusions In this study, lymph node involvement negatively affects prognosis in cervical cancer. The impact on survival varies by T stage with the greatest effect seen in stage IB.