Prevalence and risk factors of low vitamin D levels in children and adolescents with familial hypokalemic periodic paralysis.

Patients with familial hypokalemic periodic paralysis (HOKPP) experience episodes of reversible immobility and are at an increased risk of limited sunlight exposure, potentially leading to vitamin D deficiency. However, there is a lack of data on vitamin D levels in this population. We investigated serum vitamin D levels and their associated factors in children with HOKPP. This study included 170 genetically-confirmed children with HOKPP, aged 3-18 years, and 170 age-, sex-, and body mass index (BMI)-matched healthy controls from the Korean Channelopathy Study, a prospective controlled investigation. Anthropometric and clinical characteristics were recorded, and serum levels of calcium, ionized calcium, phosphorus, alkaline phosphatase, 25-hydroxyvitamin D, and intact parathyroid hormone (PTH) were analyzed. Vitamin D deficiency (< 20 ng/mL) was observed in 87.0% of the patients compared to 45.5% of the controls (P < 0.05) during the summer-fall season. During the winter-spring season, 91.7% of the patients and 73.4% of the controls were deficient (P < 0.05). A strong positive correlation was found between onset age of the first paralytic attack and vitamin D levels (r = 0.78, P < 0.01). Conversely, the frequency and duration of paralytic attacks were negatively correlated with vitamin D levels (r = -0.82 and r = -0.65, P < 0.01, respectively). Age, BMI, age at onset, frequency and duration of attacks, and PTH levels were independently associated with vitamin D levels (ß = -0.10, -0.12, 0.19, -0.27, -0.21, and -0.13, P < 0.05, respectively). CONCLUSIONS: Vitamin D deficiency was highly prevalent in children with HOKPP, and vitamin D levels correlated with various disease characteristics. We recommend routine screening for vitamin D levels in these patients to address this prevalent deficiency. Considering the high prevalence of vitamin D deficiency observed, further research on other diseases characterized by reversible immobility is warranted. WHAT IS KNOWN: • A correlation between immobility and low serum vitamin D levels has been established. However, the vitamin D status of patients with familial hypokalemic periodic paralysis (HOKPP) who experience periods of reversible immobility remains unknown. WHAT IS NEW: • Vitamin D deficiency was highly prevalent in children with HOKPP, and vitamin D levels correlated with various disease characteristics.

Sedation methods can determine performance of endoscopic submucosal dissection in patients with gastric neoplasia.

BACKGROUND: Although proper sedation is mandatory for endoscopic procedures such as endoscopic submucosal dissection (ESD), there is no research investigating the effects of sedation on ESD performance and complications. We aimed to evaluate the relationship among sedation methods, clinical outcomes, and complications after ESD for gastric neoplasia. METHODS: We retrospectively reviewed clinical data of 1,367 patients with 1,485 lesions who had undergone ESD for gastric adenoma or early gastric cancer at our tertiary teaching hospital in Seoul, Korea, between January 2008 and May 2011. Of these, 1,035 lesions in 958 patients were included in the intermittent midazolam/propofol injection by endoscopists (IMIE) group, and 450 lesions in 409 patients were included in the continuous propofol infusion with opioid administration by anesthesiologists (CPIA) group. RESULTS: En bloc resection and complete resection rates were higher in the CPIA group than in the IMIE group (CPIA vs. IMIE; en bloc resection, 99.8 and 95.0 %, P < 0.001; complete resection, 94.2 and 88.3 %, P < 0.001). Duration of procedure was shorter in the CPIA group than in the IMIE group (CPIA vs. IMIE; 48.2 ± 32.5 and 57.6 ± 41.3 min, P < 0.001). In multivariate analysis, sedation method was an independent factor associated with en bloc resection and complete resection. Additionally, sedation with CPIA was not a risk factor for bleeding (P = 0.403) or perforation (P = 0.474); however, aspiration pneumonia developed more frequently in patients sedated with CPIA (CPIA vs. IMIE, 4.4 and 1.5 %, P = 0.002). CONCLUSIONS: Sedation with CPIA can improve ESD performance.

[A case of acinar cell carcinoma of pancreas, manifested by subcutaneous nodule as initial clinical symptom].

Pancreas acinar cell carcinoma (ACC) accounts for only 1-2% of pancreatic exocrine malignant tumor. The symptoms of patients with ACC are usually non-specific, for example the anorexia and weight loss. Patients may develop Schmid's triad including subcutaneous fat necrosis, polyarthritis, and eosinophilia. We reported a case of ACC which was manifested by subcutaneous nodule as initial clinical symptom. To our knowledge, this is the first reported case of ACC presenting as subcutaneous fat necrosis in Korea.

[A case of undifferentiated (embryonal) liver sarcoma mimicking klatskin tumor in an adult].

Undifferentiated sarcoma is an uncommon primary malignant tumor of the liver typically occurring in older children. It is also referred to as malignant mesenchymoma, fibromyxosarcoma, or mesenchymal sarcoma. We experienced a case of undifferentiated sarcoma in 72-year-old male. Contrast enhanced liver CT scan revealed a 3.4 cm heterogeneously enhancing, ill-defined, and low attenuated mass in the left liver and subtle intrahepatic duct dilatation. And, in tubogram, there were segmental stenosis and occlusion from the hilum to the proximal common bile duct. We did ultrasonography guided liver biopsy. The pathologic finding revealed infiltrative growth of atypical cells with rhabdoid features. Some atypical cells showed clear cytoplasm, but no organoid pattern was identified. The stroma around atypical cells was filled with eosinophilic hyaline material. These tumor cells were positive for vimentin only, and the tumor was consistent with undifferentiated sarcoma of the liver.