Introduction We aimed to examine the impact of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic on diabetic ketoacidosis (DKA) rates in children with type 1 diabetes (T1D). Methods A retrospective cross-sectional study of 11 Israeli pediatric emergency departments (ED) was conducted. Children with T1D who attended the ED between March 1, 2020 and May 31, 2020 were compared with those who attended the ED between March 1, 2019 and May 31, 2019. Results Overall, 150 and 154 children with T1D attended the EDs during the 3-month study periods in 2020 and 2019, respectively. Among patients with established T1D, DKA rates significantly increased in 2020 compared to 2019 [38/64 (59.3%) vs 31/74 (41.9%); p < 0.043]. There was a non-statistically significant trend toward a higher rate of DKA in patients with newly diagnosed T1D [46/86 (53.4%) vs 31/80 (38.7%); p = 0.063]. No differences were observed in the rates of severe DKA in 2020 compared to 2019 among patients with established T1D [10/64 (15.6%) vs 6/74 (8.1%); p = 0.184], and newly diagnosed T1D [16/86 (18.6%) vs 14/80 (17.5%); p = 0.858]. No differences were observed in the rates of intensive care unit admissions in 2020 compared to 2019 among patients with established T1D [14/64 (21.8%) vs 14/74 (18.9%); p = 0.672], and newly diagnosed T1D [26/86 (30.2%) vs 21/80 (26.2%); p = 0.977]. Conclusions Increased rates of DKA in children with established T1D were observed during the first 3 months of the outbreak in Israel. The findings suggest that the severity of DKA at ED presentation in children with T1D was not influenced by the pandemic.

SummaryIt is commonly accepted that the presence of high amounts of maternal T cells excludes Omenn syndrome (OS) in severe combined immunodeficiency (SCID). We report a SCID patient with a novel mutation in the recombination activating gene (

Primary adrenal insufficiency, or Addison's disease, is very rare in the pediatric population. The diagnosis of Addison's disease is usually suspected in the presence of hyponatremia and hyperkalemia, or when adrenal crisis develops. Pediatricians often are unaware of other presenting symptoms of the disease. As a consequence, diagnosis is often delayed by months and even years. One of the presenting signs of adrenal insufficiency is hyperpigmentation. We present the diagnosis of Addison's disease in an 11-year-old boy complaining of skin color changes that were misinterpreted as "progressive cyanosis". When skin color changes occur in a child, pediatricians should think of hyperpigmentation as a presenting sign of adrenal insufficiency.

A 14-month-old female infant presented with recurrent episodes of acute gastroenteritis accompanied by severe metabolic acidosis and hypoglycemia. Physical examination showed hepatomegaly. Laboratory evaluation revealed elevated hepatic enzymes, prolonged prothrombin time, hyperuricemia, and extremely elevated lactate and alanine levels. Glucagon injection during hypoglycemia resulted in a further decrease of blood glucose. She was treated with glucose-containing intravenous fluids, with rapid improvement and normalization of her blood pH and glucose levels. Hormonal assessment during two episodes of hypoglycemia indicated growth hormone (GH) deficiency. However, as isolated GH deficiency could not explain all other concomitant features, such as severe lactic acidosis, hepatomegaly, impaired liver function, and hyperuricemia, the possibility of a combined defect was suggested. Further lymphocytic enzymatic investigation revealed fructose-1,6-diphosphatase deficiency and molecular genetic analysis demonstrated frame shift mutation in the FBP1 gene. This enzyme deficiency causes a rare metabolic disorder not previously described in combination with GH deficiency.