The majority of the Japanese patients with K(ATP) channel hyperinsulinism (84.2%) demonstrated paternally inherited monoallelic mutations that accurately predicted the presence of the focal form.

Long-term, continuous, subcutaneous octreotide infusion is a feasible alternative to surgery especially for patients with monoallelic KATP-channel mutations.

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The optimal scan delays for hepatic arterial and portal venous phases after the bolus-tracking program detected threshold enhancement by 50 H in the lower thoracic aorta for the detection of hypervascular HCCs were 10-15 and 45-55 seconds, respectively.

Our results indicate that the signal intensity and heterogeneity of hepatocellular carcinomas on MR images correlate with the degree of VEGF expression in hepatocellular carcinomas.

To evaluate the role of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) in intravascular large B-cell lymphoma (IVLBCL), we retrospectively analyzed four consecutive IVLBCL patients receiving FDG-PET before treatment between May 2006 and November 2007. Patients were two men and two women (median age 62 years, range 54-76 years). All patients received bone marrow biopsies and random skin biopsies and two of the four patients underwent renal biopsy for diagnosis. Accuracy of FDG-PET for the detection of organ involvements was analyzed by comparing results of pathological findings. Concordant results with respect to bone marrow involvement were accurately obtained for two patients. Skin and renal involvements were undetectable by FDG-PET regardless of positive pathological findings. One patient with a false-negative FDG-PET result showed fewer lymphoma cells in the bone marrow specimen than patients with concordant FDG-PET results. These results suggest false-negative results for some types of organ involvement. Careful interpretation of the results of FDG-PET in IVLBCL is thus required.

Over the past 20 years, there has been remarkable progress in the diagnosis and treatment of congenital hyperinsulinism (CHI). These advances have been supported by the understanding of the molecular mechanism and the development of diagnostic modalities to identify the focal form of ATP-sensitive potassium channel CHI. Many patients with diazoxideunresponsive focal CHI have been cured by partial pancreatectomy without developing postsurgical diabetes mellitus. Important novel findings on the genetic basis of the other forms of CHI have also been obtained, and several novel medical treatments have been explored. However, the management of patients with CHI is still far from ideal. First, state-of-the-art treatment is not widely available worldwide. Second, it appears that the management strategy needs to be adjusted according to the patient's ethnic group. Third, optimal management of patients with the diazoxideunresponsive, diffuse form of CHI is still insufficient and requires further improvement. In this review, we describe the current landscape of this disorder, discuss the racial disparity of CHI using Japanese patients as an example, and briefly note unanswered questions and unmet needs that should be addressed in the near future.Key words 18F-dihydroxyphenylalanine, congenital hyperinsulinism, potassium channel.Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in neonates and infants before weaning.Other terms for CHI, such as "nesidioblastosis" or "persistent hyperinsulinemic hypoglycemia in infancy," are currently less frequently used. "Nesidioblastosis" is a pathological term indicating the continuous proliferation of islet cells from the pancreatic ducts, which is a common finding in healthy newborns and in obese subjects, 1-4 and the term "persistent hyperinsulinemic hypoglycemia in infancy" is no longer favored because symptoms of CHI could develop after infancy or even in adulthood. 5Persistent severe hypoglycemia could lead to permanent brain damage, so appropriate and timely treatment is extremely important for the prognosis of patients with CHI.6-9 Patients resistant to medical treatment previously often underwent near-total pancreatectomy, which involves removal of more than 95% of the total volume of the pancreas. The results, however, were frequently unsatisfactory because many patients remained hypoglycemic after surgery and most patients without hypoglycemia eventually developed postsurgical insulin-dependent diabetes mellitus. 9-11Fortunately, during the past decade, there has been major progress in the understanding and treatment of CHI, and many cases are now curable without postsurgical complications. However, there are still unanswered questions about the cause of CHI and unmet needs of patients that need to be addressed. Importantly, current diagnostic and therapeutic strategies are not uniformly available and applicable throughout the world.We have studied CHI in Japan over the past several years and found that there are regional biological ...

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