We present a case of dermatomyositis together with polycythemia as initial manifestations of a particularly rare type of prostate cancer. A 69-year-old man was hospitalized for facial erythema and symptoms of fatigue. Physical evaluation, serum creatinine phosphokinase and electromyography were consistent with dermatomyositis. In parallel, the hemoglobin level was 18.5 g/dL, serum erythropoietin levels were low normal and no JAK2 mutation was found. Given a strong suspicion of a paraneoplastic syndrome the patient underwent abdominal computed tomography revealing a prostate mass, enlarged iliac lymph nodes and a fracture of L1 due to metastasis. The unusual paraneoplastic manifestations prompted a more thorough immunohistologic examination of the needle biopsy specimen taken from the prostate, which led to the diagnosis of large cell neuroendocrine prostate carcinoma. It is a most rare type of prostate cancer, carrying a poor prognosis. To our knowledge, this is the first case in the literature associating a neuroendocrine cancer of the prostate with dermatomyositis.
Isovaleric acidemia is a rare branched-chain organic acidemia. The authors describe a 3.5-year-old girl with isovaleric acidemia and acute abdominal pain associated with bilious emesis. Elevated serum amylase and abdominal ultrasonography demonstrating an enlarged and edematous pancreas, along with the presence of peripancreatic exudates, confirmed the presence of acute pancreatitis. The patient recovered quickly with intravenous hydration, pancreatic rest, and administration of intravenous L-carnitine. Pancreatitis should be ruled out in the context of vomiting in any patient with isovaleric acidemia. Conversely, branched-chain organic acidemias should be included in the differential diagnosis of any child with pancreatitis of unknown origin.
Objective: This study compares the preoperative ultrasound findings of all children with a clinical picture of acute appendicitis on the basis of intraoperative and histopathological findings to assess the feasibility of this approach in preoperatively distinguishing between uncomplicated and complicated cases. Methods: This retrospective study includes 224 pediatric patients who underwent ultrasound prior to appendectomy at our institution between January 2016 and February 2022. Logistic regression analysis was used to investigate the association between sonographic and intraoperative histopathological findings. Results: Of the 224 participants, 61.1% were intraoperatively diagnosed with uncomplicated appendicitis (59.8% male). Multivariate logistic regression analysis revealed that patients with a higher appendiceal diameter, presence of appendicolith, and peritonitis were more likely to suffer from complicated appendicitis. Finally, the common anatomical position of the appendix and an appendiceal diameter greater than 6 mm had the highest sensitivity (94.6% and 94.5%, respectively) for predicting complicated appendicitis, with the most specific (99.3%) sonographic finding being the existence of an abscess. Conclusions: Preoperative abdominal ultrasound in children with a clinical diagnosis of acute appendicitis can distinguish between uncomplicated and complicated appendicitis in most cases of pediatric appendicitis. A higher appendiceal diameter, the presence of appendicolith, and peritonitis are parameters noted by ultrasound that strongly predict complicated appendicitis.
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