Medicine
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Primary biliary non-Hodgkin's lymphoma (PBNHL) is a rare disease with only 41 cases reported since 1982. The incidence of PBNHL in patients with malignant cholangiocarcinoma was 0.6%, and PBNHL accounted for 0.4% of extranodal non-Hodgkin's lymphoma, and only 0.016% of all non-Hodgkin's lymphoma cases. ⋯ This case reminds us that although PBNHL is rare, making accurate diagnosis difficult preoperatively, PBNHL should be considered when encountering a case with Epstein-Barr virus infection and those typical imaging findings.
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Observational Study
Differences in the clinicopathological features of pancreatic head carcinoma in dorsal and ventral pancreas: A single institution retrospective review.
The embryonic development of the pancreas originates from dorsal and ventral anlagen, and the pancreatic cancer arising from dorsal or ventral pancreas may have different clinical pathology features. This study aims to explore whether there are differences in clinicopathological features and prognosis of pancreatic head carcinoma arising from dorsal or ventral pancreas. Between January 2014 and February 2018, 101 patients with resectable pancreatic head cancer who underwent pancreaticoduodenectomy in our institution were retrospectively reviewed. ⋯ The D group was more likely to invade the common bile duct (78.6% vs 59.3%, P = .042) and duodenum (71.4% vs 44.1%, P = .006) than the V group. In addition, the survival outcome of V group was better than D group (median overall survival [OS], 15.37 months vs 10.53 months, P = .048, median DFS 9.73 months vs 5.93 months, P = .046). The clinicopathological features of pancreatic head carcinoma arising from dorsal or ventral pancreas are different, and the pancreatic head carcinoma arising from ventral pancreas has a better survival outcome.
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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a chronic inflammatory disorder of the central nervous system. It is characterized by the appearance on magnetic resonance imaging of punctate and curvilinear gadolinium enhancement in the pons and cerebellum, and is exquisitely responsive to steroid treatment. The etiology of CLIPPERS remains unclear, although its pathogenesis reflects immune-mediated processes. The accurate diagnosis of this disease is very important for both its management and prognosis. ⋯ A gene variation library of CLIPPERS syndrome was established, which lays the foundation for the further accumulation of data, and will allow the etiology and pathogenesis of the disease to be explored.
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Case Reports
Acute pancreatitis associated with duodenal obstruction induced by groove pancreatitis: A case report.
Groove pancreatitis (GP) is a rare form of chronic pancreatitis. Since GP presents with nonspecific symptoms, it can be challenging to diagnose. Duodenal obstruction is often caused by malignant diseases; however, when associated with acute pancreatitis, it is rarely induced by groove pancreatitis. ⋯ Groove pancreatitis should be considered in the differential diagnosis of patients presenting with acute pancreatitis and duodenal obstruction. These data can help to make a precise diagnosis and develop an appropriate treatment plan.
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Case Reports
Admixed phenotype of NEDD4L associated periventricular nodular heterotopia: A case report.
Periventricular nodular heterotopia-7 (PVNH7) is a neurodevelopmental disorder associated with improper neuronal migration during neurogenesis in cortex development caused by pathogenic variants in the NEDD4L gene. ⋯ We successfully identified the causative mutation NM_001144967.2(NEDD4L):c.2677G>A:p.Glu893Lys explaining symptoms overlapping those reported for PVNH7. Symptoms shared with the brother were not explained by this variant, since he was not a carrier of the pathogenic NEDD4L variant. These are most likely not extended phenotypes of PVNH7, rather an independent clinical entity caused by a yet unidentified genetic factor in the family, highlighting thus the importance of thorough evaluation of symptomatology and genomic findings in affected and unaffected family members, when such data are available.