Euglycemic Ketoacidosis in Spinal Muscular Atrophy.

Euglycemic ketoacidosis is defined by the triad of high anion gap acidosis, increased plasma ketones, and the absence of hyperglycemia. Apart from diabetes mellitus, the disorder may occur in prolonged fasting, excessive alcohol consumption, pregnancy, and inborn errors of metabolism. Here, we highlight the diagnosis of euglycemic ketoacidosis in a pediatric nondiabetic patient with spinal muscular atrophy (SMA) type 1 (Werdnig-Hoffmann disease), who, subsequently to her postoperative admission to the intensive care unit following a spinal surgery, developed high anion gap metabolic acidosis. We discuss the pathophysiology of acid-base disorders in SMA, along with the glucose and fatty acids metabolism, the necessary knowledge for medical practitioners.

Once-weekly dipeptidyl peptidase-4 inhibitors for type 2 diabetes: a systematic review and meta-analysis.

OBJECTIVE: To assess the efficacy and safety of omarigliptin and trelagliptin, novel dipeptidyl peptidase-4 inhibitors administered once-weekly (DPP-4i QW). METHODS: We systematically searched for placebo- and active-controlled randomized trials in adults with type 2 diabetes mellitus. RESULTS: Fifteen primary studies with 5709 participants were included. DPP-4i QW were more effective than placebo in reducing hemoglobin A1c (HbA1c) (Weighted Mean Difference (WMD) -0.63%; 95% CI -0.80, -0.46; I2 = 84%) and had a similar glucose-lowering effect with daily DPP-4i (WMD 0.01%; -0.08, 0.11%; I2 = 34%). Omarigliptin was less effective compared with oral antidiabetic agents, other than daily DPP-4i, (WMD 0.24%; 0.10, 0.38; I2 = 12%). Omarigliptin did not affect body weight (WMD versus placebo 0.60 kg; 0.25, 0.96; I2 = 0%). Risk for any hypoglycemia was similar between DPP-4i QW and placebo (Odds Ratio 1.32; 0.78, 2.22; I2 = 0%). Incidence of other adverse events did not differ between DPP-4i QW and control. CONCLUSIONS: DPP-4i QW were superior to placebo and similar to daily DPP-4i in terms of glycemic control, and were not associated with any specific adverse events. There is limited comparative effectiveness evidence against other agents, while their effect on hard clinical safety outcomes is unknown.

Behçet's Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?

Behçet's disease (BD) represents a multisystemic disorder that combines features of immune-mediated diseases and autoinflammatory disorders. Even though it is recognized that every type or size of vessel can be affected in this disease, there is an inability to describe a coherent model that sufficiently explains the predilection of certain patients with BD for manifesting severe large vessel thrombosis. The inconsistent epidemiologic data and the complex genetic background of BD, along with the controversy of multiple international studies regarding the coexistence of thrombophilia in patients with BD and large vessel thrombosis, make us think that a percentage of these patients may actually suffer from a distinct clinical entity. The stimulus for this concept arose from the clinical observation of three male patients who were admitted to our clinic due to extended vena cava thrombosis. On the occasion of those clinically and laboratory resembling cases, we performed a literature review concerning the epidemiology of BD, associated thrombosis, and coexistent thrombophilic factors, in order to present some evidence, which sustains our hypothesis that certain patients with large vessel thrombosis, who share features of BD and coexistent thrombophilia, should actually be further investigated for the possibility of suffering from a distinct nosological entity.

Transient Monoclonal Gammopathy Induced by Disseminated Staphylococcus aureus Infection.

Monoclonal gammopathy reflects a serological disorder suggesting a plasma cell dyscrasia or a B-cell abnormality. However, it may occasionally be encountered as a transient manifestation in the course of several diseases including infections. This is the first reported case of a transient monoclonal gammopathy IgG lambda light chain associated with a Staphylococcus aureus infection that was complicated with renal abscess and vertebral spondylodiscitis in a previously healthy 68-year-old male. We observed a complete resolution of the gammopathy within three months of medical treatment before the entire restoration of all clinical and laboratory findings. Many invasive and cost-intensive diagnostic procedures had preceded the exclusion of a malignancy. The clinical significance and the exact pathogenesis of transient monoclonality are poorly understood and remain a matter of speculation.