The Importance of Implementing a Transition Strategy for Patients with Muscular Dystrophy: From Child to Adult-Insights from a Tertiary Centre for Rare Neurological Diseases.

Progress in the field of muscular dystrophy (MD) using a multidisciplinary approach based on international standards of care has led to a significant increase in the life expectancy of patients. The challenge of transitioning from pediatric to adult healthcare has been acknowledged for over a decade, yet it continues to be a last-minute concern. Currently, there is no established consensus on how to evaluate the effectiveness of the transition process. Our study aimed to identify how well patients are prepared for the transition and to determine their needs. We conducted a descriptive, cross-sectional study on 15 patients aged 14 to 21 years. The patients completed a sociodemographic and a Transition Readiness Assessment Questionnaire (TRAQ). We also analyzed the comorbidities of these patients. Our study revealed that only 46.7% of the patients had engaged in a conversation with a medical professional, namely, a child neurologist, about transitioning. A total of 60% of the participants expressed having confidence in their self-care ability. However, the median TRAQ score of 3.6 shows that these patients overestimate themselves. We emphasize the necessity for a slow, personalized transition led by a multidisciplinary team to ensure the continuity of state-of-the-art care from pediatric to adult healthcare services and the achievement of the highest possible quality of life for these patients.

Central pancreatectomy: an oncologically safe option to treat metastases of other neoplasms of the mid-portion of the pancreas?

BACKGROUNDS/AIMS: Standard pancreatic resections are the current approach for patients with resectable, isolated pancreatic metastases of other neoplasms. However, the role of parenchyma-sparing pancreatectomies for such pathology is poorly investigated. The aim of the present study is to assess the oncological safety of central pancreatectomies for pancreatic metastases of other neoplasms. METHODS: A literature search was performed in order to identify patients with central pancreatectomies for pancreatic metastases of other neoplasms. The available data of the patients were extracted and analyzed. RESULTS: A total number of 16 patients were identified. Renal carcinoma was the primary origin for the largest number of these patients (11 patients - 69%). The mean overall survival time was 109 months, with 1-, 5- and 10-year survival rates of 100%, 84%, and 60%, respectively. CONCLUSIONS: Although not often performed, a central pancreatectomy appears to be an oncologically safe surgical procedure in select patients with pancreatic metastases of other neoplasms of the pancreatic body and isthmus. However, no definitive conclusions should be drawn, based on the data provided in the present study, due to the limited number and heterogeneity of the patients.

Clinical value of spleen-preserving distal pancreatectomy: a case-matched analysis with a special emphasis on the postoperative systemic inflammatory response.

BACKGROUND: The impact of splenectomy on outcomes after distal pancreatectomy was assessed in the present study, with a special emphasis on the postoperative systemic inflammatory response. METHODS: Thirty-three patients with spleen-preserving distal pancreatectomy-Kimura technique (SPDP group) were compared with a group of distal pancreatectomies with splenectomy (DPS group). The two groups were 1:1 matched for age, gender, co-morbidities and pathology. RESULTS: No differences between the groups were observed regarding the overall/severe/infectious morbidity, pancreatic fistulae and postoperative diabetes rates (P-values ≥ 0.475). An increased blood loss (P = 0.031) and need for intraoperative transfusions (P = 0.004) was observed in the DPS group. Postoperative platelet count and platelet-to-lymphocyte ratio were significantly higher in the DPS group (P < 0.001). CONCLUSION: Spleen removal during DP is not associated with a higher morbidity but with an increased blood loss and need for intraoperative transfusions. Although the postoperative systemic inflammatory response is higher when the splenectomy is performed, the number of postoperative infectious complications is not influenced. Preservation of the spleen during DP for benign and low-grade malignant tumor of the distal pancreas appears to be worthy and should be the first option whenever is technically feasible and it can be safely achieved.

Central pancreatectomy versus spleen-preserving distal pancreatectomy: a comparative analysis of early and late postoperative outcomes.

BACKGROUND/AIM: The aim of the present study is to compare the postoperative and long-term outcomes of central pancreatectomy (CP) and spleen-preserving distal pancreatectomy (SPDP). METHODS: Clinical, pathological and long-term data were compared between 22 patients who underwent CP and 25 patients who underwent SPDP (2002-2012). RESULTS: The median length of resected pancreas was 8.5 cm in the SPDP group and 5 cm in the CP group (p < 0.001). The median estimated blood loss was significantly lower in the CP group (p = 0.019). Morbidity was 50% for CPs and 40% for SPDPs (p = 0.564). The rate of pancreatic fistulae was 36% for CPs and 40% for SPDPs (p = 0.530). The rate of new-onset diabetes was nil in the successful CP group and 16% in the SPDP group (p = 0.111). CONCLUSION: Morbidity and pancreatic fistula rates are not higher after CP when compared to SPDP. The loss of normal pancreatic tissue is significantly lower for CP, and thus there is potentially better preservation of the pancreatic endocrine functions. CP should be considered only in selected cases when preservation of the pancreas is of utmost importance, especially for lesions situated at the level of the pancreatic neck.

Left-sided duplication of inferior vena cava: clinical implications in a patient with sigmoid adenocarcinoma.

Double inferior vena cava is a rare congenital anomaly, usually detected by computed tomography or magnetic resonance imaging. Although asymptomatic, it may have a clinical significance, as it may mimic a para-aortic lymphadenopaty. A case of left-sided duplication of the inferior vena cava in a patient with sigmoid colon cancer is presented. The diagnostic pitfalls and clinical implications are discussed. Accurate preoperative assessment of such an anatomical variant is of utmost importance, this way potentially life-threatening surgical complications, particularly when a minimally invasive approach is planned, are prevented.