Non-umbilical cutaneous metastases from pancreatic adenocarcinomas are extremely rare. Only a few cases have been reported in the literature. An 83-year-old Korean woman, with no previous medical history, presented with a painful nodule on her scalp. Histologic examination of the nodule revealed a metastatic adenocarcinoma, and immunohistochemical staining was positive for cytokeratin (CK) 7 and CK 19. These findings were consistent with a metastatic carcinoma of pancreatic origin. An abdominal computed tomography scan identified a mass on the pancreatic head and multiple enlarged lymph nodes. Pathological examination of an endoscopic ultrasound-guided fine needle biopsy of the pancreatic mass determined that it was a poorly differentiated carcinoma. The patient refused any treatment owing to her old age and short life expectancy. Four months later, the disease progressed rapidly, and the patient died.
Intradialytic blood pressure abnormalities are associated with adverse outcomes in patients with end-stage renal disease on dialysis. Vascular calcification is a common complicating feature, but whether this complication results in intradialytic blood pressure abnormalities remains uncertain. Therefore, this study investigated the relationship between coronary artery calcium score and intradialytic blood pressure abnormalities in patients with end-stage renal disease on maintenance hemodialysis. Thirty-six patients who received nongated chest computed tomography scans were included. Intradialytic hypotension was defined as a minimum intradialytic systolic blood pressure of <100 mmHg or a pre-dialysis blood pressureminimum intradialytic systolic blood pressure >30 mmHg. Intradialytic hypertension was defined as >10 mmHg increase in systolic blood pressure (pre-to post-dialysis). Patients were classified as 22 (61.1%) with coronary artery calcium score <400 and 14 (38.9%) with coronary artery calcium score ≥400. Median systolic and diastolic blood pressures were equivalent, but median pulse pressure was higher in patients with coronary artery calcium score ≥400 than in those with scores <400. Coronary artery calcium score was comparable according to both intradialytic hypotension and hypertension, and had no correlation with systolic blood pressure fall and nadir systolic blood pressure. Coronary artery calcium score predicted the occurrence of cardiovascular events and all-cause mortality (hazard ratio 1.001 and 1.001; p=0.058 and 0.010). Coronary vascular calcification could be irrelevant to intradialytic blood pressure abnormalities in patients with end-stage renal disease on dialysis.
Rational:Kallmann syndrome (KS) is a genetic gonadotropin-releasing hormone deficiency associated with hyposmia or anosmia and characterized by various modes of inheritance.Patient concerns:A 16-year-old male did not reach puberty and was associated with hypogonadotropic hypogonadism and anosmia. His magnetic resonance imaging of brain revealed the absence of the olfactory bulb.Diagnosis:His karyotype was 46 XY. Sanger sequencing of the KAL1 gene revealed no mutations. Diagnostic exome sequencing identified a prokineticin-receptor 2 (PROKR2) gene variant, c.337T > C (p.Tyr113His), previously reported to be a pathogenic mutation; we confirmed the presence of the mutation via Sanger sequencing of the coding exons of PROKR2. His apparently unaffected mother and sister, but not his father, were heterozygous for the PROKR2 Tyr113His mutation.Lessons:This work advances our understanding of the role played by PROKR signaling and the mode of inheritance of the gene in patients with KS.
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