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Background: Hyperinsulinism (HI) due to dysregulation of pancreatic beta-cell insulin secretion is the most common and most severe cause of persistent hypoglycemia in infants and children. In the 65 years since HI in children was first described, there has been a dramatic advancement in the diagnostic tools available, including new genetic techniques and novel radiologic imaging for focal HI; however, there have been almost no new therapeutic modalities since the development of diazoxide. Summary: Recent advances in neonatal research and genetics have improved our understanding of the pathophysiology of both transient and persistent forms of neonatal hyperinsulinism. Rapid turnaround of genetic test results combined with advanced radiologic imaging can permit identification and localization of surgically-curable focal lesions in a large proportion of children with congenital forms of HI, but are only available in certain centers in "developed" countries. Diazoxide, the only drug currently approved for treating HI, was recently designated as an "essential medicine" by the World Health Organization but has been approved in only 16% of Latin American countries and remains unavailable in many under-developed areas of the world. Novel treatments for HI are emerging, but they await completion of safety and efficacy trials before being considered for clinical use. Key Messages: This international consensus statement on diagnosis and management of HI was developed in order to assist specialists, general pediatricians, and neonatologists in early recognition and treatment of HI with the ultimate aim of reducing the prevalence of brain injury caused by hypoglycemia. A previous statement on diagnosis and management of HI in Japan was published in 2017. The current document provides an updated guideline for management of infants and children with HI and includes potential accommodations for less-developed regions of the world where resources may be limited. [ABSTRACT FROM AUTHOR]

Objective: The Royal Australian and New Zealand College of Psychiatrists recommends screening for a range of antibodies in first-episode psychosis, including anti-glutamic acid decarboxylase antibodies. Glutamic acid decarboxylase antibody–associated encephalitis occurs with high antibody titres and may cause cognitive dysfunction, seizures and psychiatric symptoms. However, glutamic acid decarboxylase antibodies are more frequently found in lower titre in association with other autoimmune disorders (such as diabetes mellitus type 1) and in healthy individuals. The utility of testing unselected populations of consumers with psychosis is unclear. The psychiatric manifestations of this disorder are also poorly described. Methods: First, systematic review of cohort and case–control studies that tested for IgG glutamic acid decarboxylase antibodies in psychiatric populations was conducted. Random-effects meta-analysis of odds ratio for antibody positivity in cases with psychosis and controls assessed prevalence. Second, literature review of all published cases and case series of glutamic acid decarboxylase antibody–associated limbic encephalitis was assessed for frequency and description of psychotic symptoms. Results: There were 17 studies, in which 2754 individuals with psychotic disorders were tested for glutamic acid decarboxylase IgG antibodies. Thirty-one consumers with psychosis (0.7%) had positive glutamic acid decarboxylase antibodies compared to 24 controls (1.0%), all at low titre and not fulfilling diagnostic criteria for autoimmune encephalitis. Meta-analysis found no significant difference in rates of glutamic acid decarboxylase antibody positivity (odds ratio = 1.8, 95% confidence interval: [0.90, 3.63]). Literature review found 321 cases of glutamic acid decarboxylase antibody–associated limbic encephalitis, with psychosis in 15 (4.3%) cases. Clinical screening would have identified all cases that presented to psychiatric services. Conclusion: Glutamic acid decarboxylase antibodies were uncommon in consumers with psychosis, with no significant difference in prevalence from controls and no cases of encephalitis identified. In cases with established glutamic acid decarboxylase antibody–associated limbic encephalitis, psychotic symptoms were uncommon and identifiable by clinical assessment. Targeted antibody testing guidelines should be further considered. [ABSTRACT FROM AUTHOR]

The aim of the study was to evaluate the bone-optimal pediatric levels of 25-hydroxy-vitamin D (25OHD) by testing the level at which 25OHD optimally effects calcium, phosphorus, and parathyroid hormone levels in a large population-based dataset. This was an observational retrospective "big-data" study. We analyzed 49 935 25OHD tests from children sampled in Clalit Health Services, Jerusalem district between 2009 and 2019. Associated data were available in the following number of samples: corrected calcium; 18 869, phosphorus: 1241, and PTH: 449. We tested correlations between each parameter and 25OHD, adjusting phosphorus levels by age using a "phosphorus index". Pearson's and Spearman's correlation coefficients were calculated to determine the strength of the correlation between 25OHD and each parameter. There was a significant correlation between 25OHD levels and both PTH and calcium but not for the phosphorus index. The level at which increase in 25OHD continued to cause significant alteration was: for PTH up to 100 nmol/l (40 ng/ml), for corrected calcium it increased beyond 100 nmol/l. Increasing levels of 25OHD levels up to at least 100 nmol/l are associated with improvement in parameters known to be associated with increased bone mineralization. Therefore, one should aim for a 25OHD level of 100 nmo/l. [ABSTRACT FROM AUTHOR]