Genotype-phenotype correlations in children with Gitelman syndrome.

BACKGROUND: This study aimed to analyze genotype-phenotype correlations in children with Gitelman syndrome (GS). METHODS: This multicenter retrospective study included 50 Korean children diagnosed with SLC12A3 variants in one or both alleles and the typical laboratory findings of GS. Genetic testing was performed using the Sanger sequencing except for one patient. RESULTS: The median age at the diagnosis was 10.5 years (interquartile range, 6.8;14.1), and 41 patients were followed up for a median duration of 5.4 years (interquartile range, 4.1;9.6). A total of 30 different SLC12A3 variants were identified. Of the patients, 34 (68%) had biallelic variants, and 16 (32%) had monoallelic variants on examination. Among the patients with biallelic variants, those (n = 12) with the truncating variants in one or both alleles had lower serum chloride levels (92.2 ± 3.2 vs. 96.5 ± 3.8 mMol/L, P = 0.002) at onset, as well as lower serum potassium levels (3.0 ± 0.4 vs. 3.4 ± 0.3 mMol/L, P = 0.016), and lower serum chloride levels (96.1 ± 1.9 vs. 98.3 ± 3.0 mMol/L, P = 0.049) during follow-up than those without truncating variants (n = 22). Patients with monoallelic variants on examination showed similar phenotypes and treatment responsiveness to those with biallelic variants. CONCLUSIONS: Patients with GS who had truncating variants in one or both alleles had more severe electrolyte abnormalities than those without truncating variants. Patients with GS who had monoallelic SLC12A3 variants on examination had almost the same phenotypes, response to treatment, and long-term prognosis as those with biallelic variants.

Effects of Apamin on MPP+-Induced Calcium Overload and Neurotoxicity by Targeting CaMKII/ERK/p65/STAT3 Signaling Pathways in Dopaminergic Neuronal Cells.

Parkinson's disease (PD), a neurodegenerative disorder, is characterized by the loss of dopaminergic (DA) neurons. The pathogenesis of PD is associated with several factors including oxidative stress, inflammation, and mitochondrial dysfunction. Ca2+ signaling plays a vital role in neuronal signaling and altered Ca2+ homeostasis has been implicated in many neuronal diseases including PD. Recently, we reported that apamin (APM), a selective antagonist of the small-conductivity Ca2+-activated K+ (SK) channel, suppresses neuroinflammatory response. However, the mechanism(s) underlying the vulnerability of DA neurons were not fully understood. In this study, we investigated whether APM affected 1-methyl-4-phenyl pyridinium (MPP+)-mediated neurotoxicity in SH-SY5Y cells and rat embryo primary mesencephalic neurons. We found that APM decreased Ca2+ overload arising from MPP+-induced neurotoxicity response through downregulating the level of CaMKII, phosphorylation of ERK, and translocation of nuclear factor NFκB/signal transducer and activator of transcription (STAT)3. Furthermore, we showed that the correlation of MPP+-mediated Ca2+ overload and ERK/NFκB/STAT3 in the neurotoxicity responses, and dopaminergic neuronal cells loss, was verified through inhibitors. Our findings showed that APM might prevent loss of DA neurons via inhibition of Ca2+-overload-mediated signaling pathway and provide insights regarding the potential use of APM in treating neurodegenerative diseases.

Erratum: Correction of Text in the Article "Pediatric Emergency Department Utilization and Coronavirus Disease in Daegu, Korea".

This corrects the article on p. e11 in vol. 36, PMID: 33398945.

Pediatric Emergency Department Utilization and Coronavirus Disease in Daegu, Korea.

BACKGROUND: Limited data exist on children's utilization of the emergency department (ED) in the ongoing coronavirus disease 2019 (COVID-19) pandemic. Thus, we aimed to examine ED utilization among pediatric patients and the impact of COVID-19 in one large city affected by the outbreak. METHODS: This retrospective study included data from six EDs in Daegu, Korea. We compared the demographic and clinical data of patients presenting to the ED during the COVID-19 pandemic (February 1st-June 30th 2020) with those of patients who visited the ED in this period during 2018 and 2019. RESULTS: Fewer patients, particularly children visited the EDs during the study period in 2020 than those in the previous (2018/2019) year period: the number of adult patient decreased by 46.4% and children by 76.9%. Although the number of patients increased from the lowest point of the decrease in March 2020, the number of pediatric patients visiting the ED remained less than half (45.2%) in June 2020 compared with that of previous years. The proportion of patients with severe conditions increased in adults, infants, and school-aged children, and consequently resulted in increased ambulance use and higher hospitalization rates. Fewer infants and young children but more school-aged children visited the ED with febrile illnesses in 2020 than in 2018/2019. CONCLUSION: The COVID-19 pandemic has led to a substantial decrease in pediatric ED utilization. These findings can help reallocate human and material resources in the EDs during infectious disease outbreaks.

Apamin Suppresses LPS-Induced Neuroinflammatory Responses by Regulating SK Channels and TLR4-Mediated Signaling Pathways.

Neuroinflammation plays a vital role in neurodegenerative conditions. Microglia are a key component of the neuroinflammatory response. There is a growing interest in developing drugs to target microglia and thereby control neuroinflammatory processes. Apamin (APM) is a specifically selective antagonist of small conductance calcium-activated potassium (SK) channels. However, its effect on neuroinflammation is largely unknown. We examine the effects of APM on lipopolysaccharide (LPS)-stimulated BV2 and rat primary microglial cells. Regarding the molecular mechanism by which APM significantly inhibits proinflammatory cytokine production and microglial cell activation, we found that APM does so by reducing the expression of phosphorylated CaMKII and toll-like receptor (TLR4). In particular, APM potently suppressed the translocation of nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB)/signal transducer and activator of transcription (STAT)3 and phosphorylated mitogen-activated protein kinases (MAPK)-extracellular signal-regulated kinase (ERK). In addition, the correlation of NF-κB/STAT3 and MAPK-ERK in the neuroinflammatory response was verified through inhibitors. The literature and our findings suggest that APM is a promising candidate for an anti-neuroinflammatory agent and can potentially be used for the prevention and treatment of various neurological disorders.

Propionic Acidemia with Novel Mutation Presenting as Recurrent Pancreatitis in a Child.

Propionic acidemia (PA) is a rare organic acidemia resulting from a deficiency of the mitochondrial enzyme propionyl-coenzyme A carboxylase. Most cases are diagnosed after the detection of metabolic abnormalities-such as hyperammonemia, metabolic acidosis, and ketosis-associated with complaints of vomiting, feeding difficulties, and hypotonia during the neonatal period. However, in rare late-onset cases, mild or vague symptoms make the diagnosis more challenging. Even though acute pancreatitis is relatively uncommon in children, it can occur in association with PA. We present the case of a 4-year-old child who was admitted owing to the complaint of recurrent pancreatitis and had not previously been diagnosed with having metabolic disease. During inpatient treatment for acute pancreatitis, convulsions occurred with concomitant hyperammonemia, metabolic acidosis, coagulopathy, and shock 1 week after the administration of total parenteral nutrition. He was diagnosed to have PA after a metabolic work-up and confirmed to have novel mutation by molecular genetic analysis. Because children with PA may have acute pancreatitis, although rare, vomiting and abdominal pain should raise a suspicion of acute pancreatitis. On the contrary, even among children who have never been diagnosed with a metabolic disease, if a child has recurrent pancreatitis, metabolic pancreatitis caused by organic acidemia should be considered.

Pomolic Acid Ameliorates Fibroblast Activation and Renal Interstitial Fibrosis through Inhibition of SMAD-STAT Signaling Pathways.

Fibrosis is a common pathological feature in most kinds of chronic kidney disease. Transforming growth factor ß1 (TGF-ß1) signaling is the master pathway regulating kidney fibrosis pathogenesis, in which mothers against decapentaplegic homolog 3 (SMAD3) with signal transducer and activator of transcription 3 (STAT3) act as the integrator of various pro-fibrosis signals. We examine the effects of pomolic acid (PA) on mice with unilateral ureteral obstruction (UUO) and TGF-ß1 stimulated kidney fibroblast cells. UUO mice were observed severe tubular atrophy, and tubulointerstitial fibrosis and extracellular matrix (ECM) deposition at seven days postoperatively. However, PA-treated UUO mice demonstrated only moderate injury, minimal fibrosis, and larger reductions in the expression of ECM protein and epithelial-mesenchymal transition (EMT) progress. PA inhibited the SMAD-STAT phosphorylation in UUO mice. PA effects were also confirmed in TGF-ß1 stimulated kidney fibroblast cells. In this study, we first demonstrated that PA ameliorates fibroblast activation and renal interstitial fibrosis. Our results indicate that PA may be useful as a potential candidate in the prevention of chronic kidney disease.

Tumefactive Gallbladder Sludge at US: Prevalence and Clinical Importance.

Purpose To evaluate the prevalence of tumefactive sludge of the gallbladder detected at ultrasonography (US) and to assess whether any clinical and imaging differences exist between benign and malignant tumefactive sludge. Materials and Methods The institutional review board approved this retrospective study. The requirement for informed consent was waived. The study included a cohort (n = 6898) of patients with gallbladder sludge drawn from all adults (n = 115 178) who underwent abdominal US between March 2001 and March 2015. Tumefactive sludge was identified according to the following US findings: (a) nonmovable mass-like lesion and (b) absence of posterior acoustic shadowing at B-mode US and vascularity at color Doppler US. Follow-up examinations were arranged to ascertain whether the results showed true sludge or gallbladder cancer. Risk factors for malignant tumefactive sludge based on clinical and US characteristics were identified with multivariate logistic regression analysis. Results The prevalence of gallbladder and tumefactive sludge at abdominal US during the observation period was 6.0% (6898 of 115 178) and 0.1% (135 of 115 178), respectively. Twenty-eight (20.7%) patients were lost to follow-up. Of the 107 with tumefactive sludge, 15 (14%) were confirmed to have malignant tumefactive sludge. The risk factors for malignant tumefactive sludge were old age (odds ratio [OR], 1.06; P = .035), female sex (OR, 5.48; P = .014), and absence of hyperechoic spots within the sludge (OR, 6.78; P = .008). Conclusion Although the prevalence of tumefactive sludge at US was rare, a considerable proportion of patients had a malignancy. Careful follow-up is essential, especially for older patients, women, and those with an absence of hyperechoic spots at US. © RSNA, 2016 Online supplemental material is available for this article.

Liver Imaging Reporting and Data System on CT and gadoxetic acid-enhanced MRI with diffusion-weighted imaging.

OBJECTIVES: To assess major imaging features of Liver Imaging Reporting and Data System (LI-RADS) on contrast-enhanced CT and gadoxetic acid-enhanced MRI and to estimate whether the combination of signal intensity favouring HCC on hepatobiliary phase (HBP) and diffusion-weighted images (DWI) can act as a major feature in LI-RADS. METHODS: Four hundred twenty one patients with 445 observations were included. Major features of LI-RADS on CT and MRI as well as HBP and DWI features were assessed. Diagnostic performances of LR-5 according to LI-RADS v2014 and modified LI-RADS which incorporate combination of HBP and DWI were assessed. Pairwise comparisons of the receiver operating characteristic (ROC) curves were performed. RESULTS: For HCCs, capsule appearance had the highest rate of discordance between CT and MRI (32.7%), followed by washout appearance (22.2%). Specificity (75%) of LR-5 of LI-RADS v2014 was lower than that (77.1-79.2%) of modified LI-RADS. Area under the ROC curve of modified LI-RADS (0.755-0.775) was not significantly different from that of LI-RADS v 2014 (0.709) (p > 0.05). CONCLUSIONS: There were substantial discordances between CT and MRI for capsule and washout appearances in hepatic observations, and combination of gadoxetic acid-enhanced MRI and DWI might be able to be incorporated as a major feature of LI-RADS. KEY POINTS: • Major imaging features of LI-RADS showed substantial discordances on CT and MRI. • An observation may be categorized differently depending on used imaging exam. • CT and MRI should both be performed for LR-3 and LR-4 observations. • Combination of gadoxetic acid-enhanced MRI and DWI may be a major feature.

Differentiation of mass-forming focal pancreatitis from pancreatic ductal adenocarcinoma: value of characterizing dynamic enhancement patterns on contrast-enhanced MR images by adding signal intensity color mapping.

OBJECTIVES: To evaluate the value of dynamic enhancement patterns on contrast-enhanced MR images by adding signal intensity colour mapping (SICM) to differentiate mass-forming focal pancreatitis (MFFP) from pancreatic ductal adenocarcinoma (PDAC). METHODS: Forty-one clinicopathologically proven MFFPs and 144 surgically confirmed PDACs were enrolled. Laboratory and MR imaging parameters were used to differentiate MFFP from PDAC. In particular, enhancement patterns on MR images adding SICM were evaluated. By using classification tree analysis (CTA), we determined the predictors for the differentiation of MFFP from PDAC. RESULTS: In the CTA, with all parameters except enhancement pattern on SICM images, ductal obstruction grade and T1 hypointensity grade of the pancreatic lesion were the first and second splitting predictor for differentiation of MFFP from PDAC, in order. By adding an enhancement pattern on the SICM images to CTA, the enhancement pattern was the only splitting predictor to differentiate MFFP from PDAC. The CTA model including enhancement pattern on SICM images has sensitivity of 78.0 %, specificity of 99.3 %, and accuracy of 94.6 % for differentiating MFFP from PDAC. CONCLUSION: The characterization of enhancement pattern for pancreatic lesions on contrast-enhanced MR images adding SICM would be helpful to differentiate MFFP from PDAC. KEY POINTS: • SICM was useful to characterize enhancement pattern. • Enhancement pattern on SICM was the only splitting predictor on CTA. • This model may be useful for differentiating MFFP from PDAC.

Identification of Imaging Predictors Discriminating Different Primary Liver Tumours in Patients with Chronic Liver Disease on Gadoxetic Acid-enhanced MRI: a Classification Tree Analysis.

OBJECTIVES: To identify predictors for the discrimination of intrahepatic cholangiocarcinoma (IMCC) and combined hepatocellular-cholangiocarcinoma (CHC) from hepatocellular carcinoma (HCC) for primary liver cancers on gadoxetic acid-enhanced MRI among high-risk chronic liver disease (CLD) patients using classification tree analysis (CTA). METHODS: A total of 152 patients with histopathologically proven IMCC (n = 40), CHC (n = 24) and HCC (n = 91) were enrolled. Tumour marker and MRI variables including morphologic features, signal intensity, and enhancement pattern were used to identify tumours suspicious for IMCC and CHC using CTA. RESULTS: On CTA, arterial rim enhancement (ARE) was the initial splitting predictor for assessing the probability of tumours being IMCC or CHC. Of 43 tumours that were classified in a subgroup on CTA based on the presence of ARE, non-intralesional fat, and non-globular shape, 41 (95.3 %) were IMCCs (n = 29) or CHCs (n = 12). All 24 tumours showing fat on MRI were HCCs. The CTA model demonstrated sensitivity of 84.4 %, specificity of 97.8 %, and accuracy of 92.3 % for discriminating IMCCs and CHCs from HCCs. CONCLUSIONS: We established a simple CTA model for classifying a high-risk group of CLD patients with IMCC and CHC. This model may be useful for guiding diagnosis for primary liver cancers in patients with CLD. KEY POINTS: • Arterial rim enhancement was the initial splitting predictor on CTA. • CTA model achieved high sensitivity, specificity, and accuracy for discrimination of tumours. • This model may be useful for guiding diagnosis of primary liver cancers.

Magnetic resonance imaging with gadoxetic acid for local tumour progression after radiofrequency ablation in patients with hepatocellular carcinoma.

OBJECTIVES: To develop and validate a prediction model using magnetic resonance imaging (MRI) for local tumour progression (LTP) after radiofrequency ablation (RFA) in hepatocellular carcinoma (HCC) patients. METHODS: Two hundred and eleven patients who had received RFA as first-line treatment for HCC were retrospectively analyzed. They had undergone gadoxetic acid-enhanced MRI before treatment, and parameters including tumour size; margins; signal intensities on T1-, T2-, and diffusion-weighted images, and hepatobiliary phase images (HBPI); intratumoral fat or tumoral capsules; and peritumoural hypointensity in the HBPI were used to develop a prediction model for LTP after treatment. This model to discriminate low-risk from high-risk LTP groups was constructed based on Cox regression analysis. RESULTS: Our analyses produced the following model: 'risk score = 0.617 × tumour size + 0.965 × tumour margin + 0.867 × peritumoural hypointensity on HBPI'. This was able to predict which patients were at high risk for LTP after RFA (p < 0.001). Patients in the low-risk group had a significantly better 5-year LTP-free survival rate compared to the high-risk group (89.6 % vs. 65.1 %; hazard ratio, 3.60; p < 0.001). CONCLUSION: A predictive model based on MRI before RFA could robustly identify HCC patients at high risk for LTP after treatment. KEY POINTS: • Tumour size, margin, and peritumoural hypointensity on HBPI were risk factors for LTP. • The risk score model can predict which patients are at high risk for LTP. • This prediction model could be helpful for risk stratification of HCC patients.

Imaging features of subcentimeter hypointense nodules on gadoxetic acid-enhanced hepatobiliary phase MR imaging that progress to hypervascular hepatocellular carcinoma in patients with chronic liver disease.

BACKGROUND: With the advent of 3-T magnetic resonance imaging (MRI) and diffusion-weighted imaging (DWI), many subcentimeter hepatic nodules have been frequently detected in patients with chronic liver disease, and the prediction of subcentimeter nodules that progress to hypervascular hepatocellular carcinoma (HCC) is important. PURPOSE: To reveal the imaging features of subcentimeter hypointense nodules on gadoxetic acid-enhanced hepatobiliary phase MR images in patients with chronic liver disease that may be related with progression to hypervascular HCC. MATERIAL AND METHODS: This study included 109 patients with 135 subcentimeter hypointense nodules (103 hepatocellular carcinomas [HCCs] and 32 benign nodules) on gadoxetic acid-enhanced hepatobiliary phase. For each subcentimeter nodule, the following imaging features were analyzed: (i) hyperintensity on T2-weighted (T2W) image or DWI; (ii) arterial hypervascularization; and (iii) hypointensity on a portal or late phase. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of those imaging features for predicting subcentimeter nodules that progressed to hypervascular HCC were evaluated. Volume doubling time (VDT) of subcentimeter nodules that progressed to hypervascular HCC was compared to that of growing benign nodules. RESULTS: Arterial hypervascularization with washout yielded the highest specificity (90.6%) and PPV (95.4%) for prediction of subcentimeter nodules that progressed to hypervascular HCC. Combining hyperintensity on T2W image or DWI and arterial hypervascularization with washout did not raise the specificity (90.6%) for prediction of subcentimeter nodules that progressed to hypervascular HCC. Mean VDT (112.2 ± 106.3 days) of subcentimeter nodules that progressed to hypervascular HCC during follow-up was significantly shorter than those of benign subcentimeter nodules (1258 ± 766.5 days, P < 0.001). CONCLUSION: Arterial hypervascularization with washout in subcentimeter hypointense nodules on gadoxetic acid-enhanced hepatobiliary phase in patients with chronic liver disease is strongly related with progression to hypervascular HCC.

The role of diffusion-weighted MR imaging for differentiating benign from malignant bile duct strictures.

OBJECTIVES: To assess the added value of diffusion-weighted imaging (DWI) to conventional magnetic resonance imaging (MRI) for differentiating benign from malignant bile duct strictures. METHODS: Twenty-seven patients with a benign stricture and 42 patients with a malignant stricture who had undergone gadoxetic acid-enhanced MRI with DWI were enrolled. Qualitative (signal intensity, dynamic enhancement pattern) and quantitative (wall thickness and length) analyses were performed. Two observers independently reviewed a set of conventional MRI and a combined set of conventional MRI and DWI, and receiver operating characteristic (ROC) curve analysis was assessed. RESULTS: Benign strictures showed isointensity (18.5-70.4 %) and a similar enhancement pattern (22.2 %) to that of normal bile duct more frequently than malignant strictures (0-40.5 % and 0 %) on conventional MRI (P < 0.05). Malignant strictures (90.5-92.9 %) showed hypervascularity on arterial and portal venous phase images more frequently than benign strictures (37.0-70.4 %) (P < 0.01) On DWI, all malignant strictures showed hyperintensity compared with benign cases (70.4 %) (P < 0.001). Malignant strictures were significantly thicker and longer than benign strictures (P < 0.001). The diagnostic performance of both observers improved significantly after additional review of DWI. CONCLUSIONS: Adding DWI to conventional MRI is more helpful for differentiating benign from malignant bile duct strictures than conventional MRI alone. KEY POINTS: • Accurate diagnosis and exclusion of benign strictures of bile duct are important. • Diffusion-weighted MRI helps to distinguish benign from malignant bile duct strictures. • DWI plus conventional MRI provides superior diagnostic accuracy to conventional MRI alone.

Added value of diffusion-weighted MRI for evaluating viable tumor of hepatocellular carcinomas treated with radiotherapy in patients with chronic liver disease.

OBJECTIVE: The purpose of this article is to evaluate the added value of diffusion-weighted imaging (DWI) to the diagnostic performance of conventional MRI in diagnosing viable hepatocellular carcinoma (HCC) tumors treated with radiotherapy in patients with chronic liver disease. MATERIALS AND METHODS: Twenty-nine patients with viable tumor and 35 patients without viable tumor were enrolled. We assessed the signal intensity of viable tumor compared with irradiated liver on MRI and DWI. Signal intensity ratios and apparent diffusion coefficient (ADC) ratios of viable tumor to nonirradiated liver were also assessed on DWI with ADC maps. Two observers reviewed conventional MRI and combined MRI and DWI and rated them using a 5-point scale. Diagnostic performance was evaluated using a receiver operating characteristic (ROC) curve. RESULTS: Viable tumors showed hyperintensity on T2-weighted and arterial phase images (16/29 [55.2%]) and hypointensity on portal (22/29 [75.9%]), 3-minute late (19/29 [65.5%]), and hepatobiliary phase (23/29 [79.3%]) images. Twenty-seven (93.1%) viable tumors showed hyperintensity on DWI and hypointensity on ADC maps. Mean signal intensity ratios and ADC ratios of viable tumor on DWI with ADC maps were significantly higher and lower than those of irradiated liver. Diagnostic performance (area under the ROC curve) improved significantly after adding DWI, and interobserver agreement was moderate for conventional MRI (κ = 0.450) and good after adding DWI (κ = 0.748). CONCLUSION: Adding DWI to conventional MRI can improve the detection of viable HCC tumors treated with radiotherapy compared to conventional MRI alone.

Differentiation of malignant from benign focal splenic lesions: added value of diffusion-weighted MRI.

OBJECTIVE: The objective of our study was to evaluate the added value of diffusion-weighted imaging (DWI) for distinguishing between malignant and benign focal splenic lesions. MATERIALS AND METHODS: This study included 53 patients with 11 malignant and 42 benign splenic lesions who underwent gadoxetic acid-enhanced MRI and DWI. Qualitative and quantitative analyses were conducted for splenic lesions. Two blinded observers evaluated the two image sets--that is, the conventional MR images alone versus the combined conventional MR and DW images--and scored their confidence for malignancy of splenic lesions. The Fisher exact test and Mann-Whitney U test were performed, and diagnostic performance values (ROC curve analysis) were evaluated. RESULTS: All malignant lesions showed a progressive hypovascular enhancement pattern, whereas the hypervascular enhancement patterns were shown in only benign lesions (n = 20, 47.6%) (p < 0.05). The mean apparent diffusion coefficient (ADC) of the malignant lesions (0.73 × 10(-3) mm(2)/s) was significantly lower than that of the benign lesions (1.21 × 10(-3) mm(2)/s) (p < 0.001). The addition of DW images to conventional MR images showed a significant improvement for predicting malignant splenic lesions (area under ROC curve [Az] without DW images vs with DW images: 0.774 vs 0.983 for observer 1 and 0.742 vs 0.986 for observer 2) (p < 0.001). In addition, the diagnostic accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of combined conventional MR and DW images were higher than those of conventional MR images alone. CONCLUSION: The addition of DWI to conventional MRI improves differentiation of malignant from benign splenic lesions.

Value of diffusion-weighted MRI for differentiating malignant from benign intraductal papillary mucinous neoplasms of the pancreas.

OBJECTIVE: The purpose of this study was to evaluate whether the use of diffusion-weighted MRI (DWI) increases diagnostic accuracy in the differentiation of malignant from benign intraductal papillary mucinous neoplasms (IPMNs) of the pancreas over the accuracy of contrast-enhanced MRI with MRCP. MATERIALS AND METHODS: A total of 61 patients with surgically resected IPMNs (19 malignant, 42 benign) who underwent gadoxetic acid-enhanced MRI, DWI, and MRCP were included. Two blinded observers evaluated two image sets, that is, conventional MRI with MRCP images versus combined conventional MRI with MRCP and DW images, and scored their confidence for malignancy of IPMNs. Qualitative analyses of the IPMNs were also conducted. Diagnostic performance (ROC curve analysis), accuracy, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were evaluated. The Fisher exact test was used to compare groups. RESULTS: The diagnostic performance (area under the ROC curve [Az]) with respect to predicting malignancy of IPMNs improved significantly for both observers after additional review of DW images (p < 0.05). The diagnostic accuracy, sensitivity, specificity, PPV, and NPV of combined conventional and DW images were higher than those of conventional MR images alone. Diffusion restriction was more often present in malignant IPMNs (78.9%) than in benign IPMNs (16.7%) (p < 0.001) with excellent interobserver agreement (ĸ = 0.965). CONCLUSION: Compared with conventional MRI alone, adding DWI to conventional MRI improves diagnostic accuracy with increased specificity for differentiating malignant from benign IPMNs of the pancreas.

The value of gadoxetic acid-enhanced and diffusion-weighted MRI for prediction of grading of pancreatic neuroendocrine tumors.

BACKGROUND: Parenchyma-preserving resection for the treatment of benign pancreatic neuroendocrine tumors (NETs) has been tried, and preoperative prediction of benign pancreatic NET is important. Recently, diffusion-weighted imaging (DWI) of abdomen magnetic resonance imaging (MRI) has been used to characterize benign and malignant tumors and DWI might be helpful in prediction of benign pancreatic NETs. PURPOSE: To evaluate the value of gadoxetic acid-enhanced MRI and DWI in predicting benign pancreatic NETs for determination of parenchyma-preserving resection. MATERIAL AND METHODS: Our ethics committee approved this study with a waiver of informed consent given its retrospective design. We searched radiology and pathology databases from November 2010 to July 2012 to identify patients who underwent surgery for pancreatic NETs (<4 cm). Twenty patients in the benign group and 14 patients in the non-benign group were included in this study. Two radiologists analyzed the morphologic features, signal intensity on MR images including DWI (b = 800), and dynamic enhancement pattern of the tumors with consensus. The tumor-to-parenchyma ratio and tumor apparent diffusion coefficients (ADCs) were quantitatively assessed. RESULTS: The benign pancreatic NETs were more often round (7/20, 35%) or ovoid (13/20, 65%) in shape and less hypovascular on the arterial phase (3/20, 15%) than were the non-benign pancreatic NETs (1/14, 7.1% and 5/14, 35.8%; 7/14, 50% respectively; P < 0.05). Main pancreatic duct dilatation by tumors was demonstrated only in non-benign pancreatic NETs (4/14, 28.4%; P = 0.021). ADC values and ratios were significantly different between benign pancreatic NETs (mean, 1.48 × 10(-3 )mm(2)/sec, 1.11 ± 0.25, each) and non-benign pancreatic NETs (mean, 1.04 × 10(-3 )mm(2)/sec, 0.74 ± 0.13, each) (P < 0.01). Other qualitative and quantitative analyses between benign and non-benign pancreatic NETs were not significantly different (P > 0.05). CONCLUSION: Abdominal MRI with DWI may be useful for differentiating benign pancreatic NETs from non-benign pancreatic NETs, which might be helpful for determination of parenchyma-preserving resection.

Clinical characteristics and outcomes of COVID-19 in children and adolescents with diabetes in Daegu, South Korea.

PURPOSE: Children with comorbidities have a higher risk of severe, coronavirus disease 2019 (COVID-19). This study investigated the clinical features and outcomes of COVID-19 in children and adolescents with diabetes between January and March 2022. METHODS: We retrospectively reviewed the medical records of 123 children and adolescents (73 with type 1 diabetes and 50 with type 2 diabetes, 59 males and 64 females) aged <18 years who had been diagnosed with diabetes. Data were collected from 7 academic medical centers in Daegu, South Korea. RESULTS: Thirty-five children with diabetes were diagnosed with COVID-19 (18 with type 1 and 17 with type 2 diabetes). Eighteen of the 35 children with diabetes and COVID-19 and 50 of the 88 children with diabetes alone received a COVID-19 vaccination. No significant differences were observed between patients with diabetes and COVID-19 and patients with diabetes alone in the type of diabetes diagnosed, sex, age, body mass index, hemoglobin A1c, or vaccination status. All children with diabetes and COVID-19 had mild clinical features and were safely managed in their homes. Fourteen children had a fever of 38℃ or higher that lasted for more than 2 days, 11 of whom were not vaccinated (p=0.004). None experienced post-COVID-19 conditions. CONCLUSION: All children and adolescents with pre-existing diabetes had mild symptoms of COVID-19 due to low disease severity, high vaccination rates, uninterrupted access to medical care, and continuous glucose monitoring. Unvaccinated children with diabetes who experienced COVID-19 presented with higher and more frequent fevers compared to vaccinated children.