Genotypic and phenotypic features in Turkish patients with classic nonketotic hyperglycinemia

Metabolic Brain Disease - Nonketotic hyperglycinemia is an autosomal recessive inborn error of glycine metabolism, characterized by deficient activity of the glycine cleavage enzyme system. Classic...     

Pediatric red cell and platelet transfusions

The aim of pediatric transfusions should be based on the concept of avoiding unnecessary transfusions without jeopardizing the patient safety and providing correct blood components when there are well founded indications to transfuse. Despite considerable efforts from transfusion services to increase transfusion safety, transfusions are still associated with preventable and unpreventable adverse effects that may, in the worst case, have severe and fatal consequences. Transfusions to pediatric patients constitute a small proportion of all transfusions but have higher incidence of adverse events compared to adults. Pediatric transfusions consist of intrauterine transfusions, top-up transfusions to neonates and young children, exchange transfusions in the management of hemolytic disease of newborn (HDN), in addition to sickle cell crisis, chronic transfusion therapy in thalassemia patients, massive transfusion in trauma, HLA- and HPA-compatible platelets in immunized patients and neonates with fetal neonatal alloimmune thrombocytopenia (FNAIT). Packed red cells (PRCs) and platelet (PLT) concentrates are the most utilized blood components and will be reviewed here.     

Aspects of urinary tract infections and antimicrobial resistance in hospitalized urology patients in Asia: 10-Year results of the Global Prevalence Study of Infections in Urology (GPIU)

Objectives

To assess Asian data from Global Prevalence Study on Infections in Urology (GPIU study) which has been performed more than 10 years.

Relationships between autonomic nerve function and gastric emptying in patients with autoimmune gastritis

Purpose

Autonomic nervous system dysfunction exists in autoimmune diseases. Symptoms of autoimmune gastritis are not specific, and some patients may present symptoms suggestive of delayed gastric emptying. This study aims to investigate whether any autonomic dysfunction exists in autoimmune gastritis patients, and if so, to clarify the relationship between the autonomic nervous dysfunction, delayed gastric emptying, and gastrointestinal symptoms.

Methods

75 patients (50 women, mean age 56.73 ± 11.77) diagnosed with autoimmune gastritis were investigated by means of autonomic nervous system and gastric emptying tests. All patients underwent a standardized scintigraphic gastric emptying study and five tests evaluating autonomic nervous system. Patients with autonomic nervous system dysfunction were then analyzed and compared by means of existence of delayed gastric emptying and gastrointestinal symptoms.

Results

62 patients had autonomic nervous system dysfunction (14 mild, 40 moderate, and 8 severe autonomic dysfunction). The mean total score of autonomic tests was 3.85 ± 2.35. Total autonomic score of patients (n = 60) with delayed gastric emptying was significantly higher than patients (n = 15) with normal gastric emptying (4.68 ± 1.7 vs. 1.53 ± 0.58, p < 0.001). Mean gastroparesis cardinal symptom index was significantly higher in patients (n = 60) with delayed gastric emptying half-time compared to patients (n = 15) with normal gastric emptying half-time (1.89 ± 1.16 vs 0.4 ± 0.3, p < 0.001).

Conclusions

Most of patients with autoimmune gastritis also have autonomic nerve dysfunction. There is a close relationship between autonomic nervous system dysfunction and delayed gastric emptying. Gastroparesis cardinal symptom index has a high sensitivity and specificity in predicting both autonomic nerve function and delay in gastric emptying.