Hereditary spherocytosis (HS) is a condition characterized by jaundice, hepato-splenomegaly tumors, and the presence of spherocytes. It accounts for about 70% of the reported cases of congenital hemolytic anemia in Japan. Many researchers state that this pathologic condition primarily arises from an abnormality of the red blood cell membrane. We have encountered a familiy, all the members (23-year-old daughter, 20-year-old son, 53-year-old-father, and 46-year-old-mother) of which had their cases diagnosed as HS by peripheral blood examination and electron microscopy. Excluding the farher, the peripheral blood examination also revealed anemia in the other family members. Moreover, the common HLA was found in all the members of these two families including the one on the mother's side, but human parvovirus B19 was not detected at all. We are providing the socalledprophylactic visiting care for the two families, while giving them information about HS which is arelatively easily-misunderstood hereditary disease. We did not find any report about married coupleswith hereditary spherocythosis. In this paper, we report our findings so far obtained through visiting care with some bibliographic discussion on HS.

Objective: The Sequential Organ Failure Assessment (SOFA) score is a useful tool in defining the clinical conditions and describing the acute morbidity of patient populations with critical illness. This study was performed to assess the usefulness of the SOFA score in predicting the prognosis among cardiac or thoracic aortic postoperative patients. Methods: In total, 123 patients who entered the intensive care unit after a cardiac or thoracic aortic operation from August 2019 to December 2020 were retrospectively investigated. The SOFA score cut-off value from the admission day to postoperative day 3 calculated in the first 60 patients (derivation group) was validated in the latter 63 patients (validation group). Additionally, the Japan SCORE cut-off value calculated in the derivation group was validated in the validation group. Results: The perioperative mortality rate, in-hospital mortality rate and hospital transfer rate were 4.9, 7.3, and 13.2%, respectively. A SOFA score cut-off value of ≥7 for prediction of in-hospital mortality resulted in a sensitivity of 100% and specificity of 81% on postoperative day 2, followed by high sensitivity of 100% and specificity of 95% on postoperative day 3. A SOFA score cut-off value of ≥6 for prediction of hospital transfer resulted in a sensitivity of 57% and a specificity of 67% on postoperative day 2. Conclusion: The SOFA score on postoperative day 2 provides good discriminatory power for in-hospital mortality among cardiac or thoracic aortic postoperative patients. The SOFA scoring system could be useful for predicting short-term prognosis of patients who undergo cardiac or thoracic aortic surgery.

OBJECTIVE: We conducted a retrospective, multi-institutional, collaborative study to accumulate cases of neuroendocrine carcinoma of the endometrium, to clarify its clinicopathologic features, treatment, prognosis and prognostic factors to collate findings to establish future individualized treatment regimens. To our knowledge, this is the largest case study and the first study to statistically analyze the prognosis of this disease. METHODS: At medical institutions participating in the Kansai Clinical Oncology Group/Intergroup, cases diagnosed at a central pathologic review as neuroendocrine carcinoma of the endometrium between 1995 and 2014 were enrolled. We retrospectively analyzed the clinicopathologic features, treatment, prognosis and prognostic factors of this disease. RESULTS: A total of 65 cases were registered from 18 medical institutions in Japan. Of these, 42 (64.6%) cases were diagnosed as neuroendocrine carcinoma of the endometrium based on the central pathological review and thus included in the study. Advanced International Federation of Gynecology and Obstetrics stages (stage III and IV) and pure type small cell neuroendocrine carcinoma cases had a significantly worse prognosis. Upon multivariate analysis, only histologic subtypes and surgery were significant prognostic factors. Pure type cases had a significantly worse prognosis compared to mixed type cases and complete surgery cases had a significantly better prognosis compared to cases with no or incomplete surgery. CONCLUSION: Our findings suggest that complete surgery improves the prognosis of neuroendocrine carcinoma of the endometrium. Even among cases with advanced disease stages, if complete surgery is expected to be achieved, clinicians should consider curative surgery to improve the prognosis of neuroendocrine carcinoma of the endometrium.
Carcinoma, Large Cell ; Carcinoma, Neuroendocrine ; Carcinoma, Small Cell ; Endometrial Neoplasms ; Endometrium ; Female ; Gynecology ; Japan ; Medical Oncology ; Multivariate Analysis ; Obstetrics ; Prognosis ; Retrospective Studies

We report a case of debranching thoracic endovascular aortic repair for Kommerell's diverticulum with right-sided aortic arch in 78-year-old women. The computed tomography (CT) demonstrated Kommerell's diverticulum with a right-sided aortic arch and the trachea and esophagus were compressed by the diverticulum. The diverticulum had a maximum diameter of 32 mm, and surgical intervention was chosen because of the aneurysmal change and the possibility of rupture. We performed endovascular aortic repair for Kommerell's diverticulum with a right-sided aortic arch because of low lung function and low frailty. The patient was discharged on the 21st postoperative day. There was no evidence of aortic event during 2 years follow up.