Posterior reversible encephalopathy syndrome associated with focal segmental glomerulosclerosis in a child.

Posterior reversible encephalopathy syndrome (PRES) is a rare neurological entity, typically manifested by reversible oedema in the parieto-occipital lobes. It is usually associated with primary hypertension, autoimmune diseases and immunosuppressants. Renal disease is an uncommon cause of PRES. We report a case of an 11-year-old boy with STimulator of INterferon Genes-associated vasculopathy with onset in infancy complicated by focal segmental glomerulosclerosis leading to hypertension and PRES. The patient presented with headache, acute bilateral visual loss and hypertension. Brain MRI showed atypical features revealed by parieto-occipital haemorrhage. The child improved few days after antihypertensive therapy. Follow-up MRI showed complete resolution of haemorrhage. It is important to keep high index of suspicion for the uncommon association of PRES with underlying kidney disease with or without immunosuppressive agents. This combination is the first to our knowledge to be described in paediatric population. Atypical MRI features such as haemorrhage should be kept in mind. Symptoms are reversible within days to weeks with early diagnosis and treatment.

Pancreatic mesothelial cyst.

A 60-year-old woman was investigated for abdominal pain and increasing asthenia. Abdominal CT revealed a 25 mm hypodense cystic lesion in the tail of the pancreas. MRI showed a multiloculated cystic lesion, T1-hypointense and T2-hyperintense lesion, without wall enhancement. Endoscopic ultrasound detected a 25 mm multi-loculated cystic lesion, with regular margin and without pancreatic duct communication. Diagnosis of pancreatic mucinous cystadenoma was discussed and the patient was referred to surgery. She underwent distal pancreatectomy with spleen preservation. Pathological examination revealed the diagnosis of pancreatic mesothelial cyst. Histologically, the cyst was multiloculated, lined by cuboidal epithelium, ovoid nuclei and amphophilic cytoplasm, without mucin deposition or cytological atypia. Immunohistochemistry examination revealed positive staining for cytokeratin 5/6, vimentin and calretinin. At 1-year follow-up, she is in her usual health, without any symptoms.

Intraplatelet Vascular Endothelial Growth Factor and Platelet-Derived Growth Factor: New Biomarkers in Carcinoembryonic Antigen-Negative Colorectal Cancer?

BACKGROUND/AIM: Colorectal cancer (CRC) is associated with high incidence and mortality rates. Carcinoembryonic antigen (CEA), a prognostic biomarker for recurrent CRC following curative resection, suffers from low sensitivity, especially in early-stage screening. Intraplatelet angiogenesis regulators (IPAR), such as vascular endothelial growth factor (VEGF) and platelet-derived growth factor (PDGF), have been identified as important regulators of tumor growth in CRC. The aim of this study was to confirm the higher preoperative level of IPAR (VEGF and PDGF) in CRC patients compared to controls and to measure IPAR in CEA-negative CRC patients. METHODS: The data and blood of 30 CRC patients and 30 presumably healthy controls were prospectively analyzed and compared. RESULTS: We confirmed elevated preoperative intraplatelet VEGF and PDGF levels in CRC patients compared to controls. Importantly, IPAR were significantly elevated even in CEA-negative CRC patients. CONCLUSION: Elevated preoperative intraplatelet VEGF and PDGF levels in CRC patients suggest new possibilities for postoperative monitoring in CRC patients, especially when CEA is negative.

Post-operative recurrence of Crohn's disease after definitive stoma: an underestimated risk.

INTRODUCTION: Crohn's disease (CD) is a progressive inflammatory disease affecting the entire gastrointestinal tract. The need for a definitive stoma (DS) is considered as the ultimate phase of damage. It is often believed that the risk of further disease progression is small when a DS has been performed. AIMS: The goals of the study were to establish the rate of CD recurrence above the DS and to identify predictive factors of CD recurrence at the time of DS. METHODS: We retrospectively reviewed all medical records of consecutive CD patients having undergone DS between 1973 and 2010. We collected clinical data at diagnosis, CD phenotype, treatment, and surgery after DS and mortality. Stoma was considered as definitive when restoration of continuity was not possible due to proctectomy, rectitis, anoperineal lesions (APL), or fecal incontinence. Clinical recurrence (CR) was defined as the need for re-introduction or intensification of medical therapy, and surgical recurrence (SR) was defined as a need for a new intestinal resection. RESULTS: Eighty-three patients (20 males, 63 females) with a median age of 34 years at CD diagnosis were included. The median time between diagnosis and DS was 9 years. The median follow-up after DS was 10 years. Thirty-five patients (42%) presented a CR after a median time of 28 months (2-211) and 32 patients (38%) presented a SR after a median time of 29 months (4-212). In a multivariate analysis, APL (HR = 5.1 (1.2-21.1), p = 0.03) and colostomy at time of DS (HR = 3.8 (1.9-7.3), p = 0.0001) were associated factors with the CR. CONCLUSION: After DS for CD, the risk of clinical recurrence was high and synonymous with surgical recurrence, especially for patients with APL and colostomy.

Reemergence of Splenectomy for ITP Second-line Treatment?

INTRODUCTION: Corticosteroids are still the standard first-line treatment for immune thrombocytopenic purpura (ITP). As second-line therapy, splenectomy and Rituximab are both recommended. The aim of our study was to compare the efficacy of Rituximab to splenectomy in persistent or chronic ITP patients. METHODS: Between January 1999 and March 2015, we retrospectively selected all consecutive patients who underwent an ITP second-line treatment: Rituximab or splenectomy. The distinction between open (OS) and laparoscopic splenectomy (LS) was analyzed. Primary outcome was composite: hospitalization for bleeding and/or thrombocytopenia and death from hemorrhage or infection. Secondary outcomes were based on response (R) and complete response (CR) rates as defined by the American Society of Hematology. RESULTS: Ninety-six patients were included: 30 patients received Rituximab, 37 underwent OS, and 29 underwent LS. The follow-up was 30, 60, and 120 months in Rituximab, LS, and OS groups, respectively. At 30 month, the primary outcome-free survival rate was higher in splenectomy groups (84% for OS, 86% for LS) than Rituximab group (47%) (P = 0.0002). Similarly, at 30 month, R and CR rates were higher for OS (86.5% and 75.7%, respectively) and LS groups (93.1% and 89.7%) than Rituximab (46.7% and 30%) (P = 0.0001). Moreover, R rates remained elevated at 60 month for OS and LS groups (83.7% and 89.6% respectively) and 78.4% at 120 month for OS group. CONCLUSION: We observed that splenectomy for ITP second-line treatment was more effective than Rituximab regarding maintenance of R, CR, and overall response rates. OS and LS had similar efficacy.