An unusual neurologic presentation of pediatric neuroinvasive West Nile virus infection: ophthalmoplegia.

BACKGROUND: West Nile virus (WNV) is an uncommon arbovirus infection and is usually asymptomatic in pediatric patients and due to its rarity is not very well known by clinicians. CASE: We present a 5-year-old girl admitted to the Pediatric Emergency Service with fever, vomiting, neck stiffness, walking difficulty and sudden deviation of eyes who was diagnosed with a neuroinvasive WNV infection. CONCLUSIONS: Ophthalmoplegia is an unusual presentation of neuroinvasive WNV and there are no published pediatric cases with ophthalmoplegia in the literature.

The association between vitamin D levels and the clinical severity and inflammation markers in pediatric COVID-19 patients: single-center experience from a pandemic hospital.

Vitamin D has an immunomodulating property that regulates the inflammatory response. In this study, the aim was to evaluate the relationship between vitamin D levels and clinical severity and inflammation markers in children and adolescents with COVID-19. The clinical and laboratory records of 103 pediatric cases with COVID-19, whose vitamin D levels had been measured, were retrospectively reviewed. The cases were divided into groups according to their clinical severity (asymptomatic, mild, and moderate-to-severe) and vitamin D levels. The moderate-to-severe clinical group had significantly higher inflammation markers (CRP, procalcitonin, fibrinogen, D-dimer) and a lower lymphocyte count compared to both the mild and asymptomatic groups. The 25 OH vitamin D levels were also significantly lower (p < 0.001), and the ratio of vitamin D deficiency was 70.6% in the moderate-to-severe group. The vitamin D-deficient group had a significantly higher age and fibrinogen levels while also having a lower lymphocyte count compared to the insufficient and normal groups. The 25 OH vitamin D level was correlated positively with the lymphocyte count (r = 0.375, p = <0.001), and negatively with age (r = -0.496, p = <0.001), CRP (r = -0.309, p = 0.002) and fibrinogen levels (r = -0.381, p = <0.001). In a logistic regression analysis, vitamin D deficiency, D-dimer, and fibrinogen levels on admission were independent predictors of severe clinical course.Conclusion: This study revealed an association between vitamin D deficiency and clinical severity, in addition to inflammation markers in pediatric COVID-19 cases. Prophylactic vitamin D supplementation may be considered, especially in the adolescent age group. What is Known: • • The pathology of COVID-19 involves a complex interaction between the SARS-CoV-2 and the immune system. Hyperinflammation/cytokine storm is held responsible for the severity of the disease. • Vitamin D has multiple roles in the immune system that can modulate the body reaction to an infection. What is New: • • Clinically more severe group had significantly lower vit D levels and significantly higher inflammation markers. • Lower 25 OH vit D levels were associated with higher inflammation markers, suggesting an important role of vitamin D in the clinical course of COVID-19 in children and adolescents probably by regulating the systemic inflammatory response.

A novel mutation in SLC2A1 gene causing GLUT-1 deficiency syndrome in a young adult patient.

Üstyol A, Takahashi S, Hatipoglu HU, Duman MA, Elevli M, Selçuk Duru HN. A novel mutation in SLC2A1 gene causing GLUT-1 deficiency syndrome in a young adult patient. Turk J Pediatr 2019; 61: 946-948. GLUT-1 deficiency syndrome is a rare, frequently unrecognized metabolic encephalopathy that is probably underdiagnosed. Although developmental delay, acquired microcephaly, spasticity, and impaired coordination were initially described as the classic findings, mild cases with no pronounced neuromotor compromise have since been included in the broad clinical spectrum with new mutations being identified more recently. We report a case of myoclonic seizures not responding to anti-epileptics since the age of one year in a 17-year-old patient with a normal phenotype and neuromotor development. Previously unreported p.Phe389Leu mutation was determined in the SLC2A1 gene in our patient. This case will be useful in clarifying the phenotype of GLUT-1 deficiency and reveals a new pathogenic mutation.

Aortic Intima-Media Thickness and Mean Platelet Volume in Children With Type 1 Diabetes Mellitus.

BACKGROUND: Diabetes mellitus type 1 is the most common endocrine metabolic disorder occurring in childhood and adolescence due to the autoimmune destruction of pancreatic beta cells as a result of various environmental factors interacting with an underlying genetic predisposition. Diabetes is a risk factor for early onset atherosclerosis, and the high mortality rate seen in these patients is partially related to cardiovascular diseases. OBJECTIVES: This study was conducted to compare mean platelet volume as a marker of early atherosclerosis with aortic intima-media thickness in children with type 1 diabetes and to identify its correlation with known cardiovascular risk factors. PATIENTS AND METHODS: The study included 27 patients between age range of 6 and 17 years that were diagnosed with type 1 diabetes and 30 healthy children of the same age range who did not have any chronic disease. In both groups, we used the color Doppler ultrasound to measure children's aortic intima-media thickness and identify their mean platelet volumes. RESULTS: There was no significant difference between the groups regarding gender distribution, age, High-Density Lipoprotein (HDL) and Low-Density Lipoprotein (LDL) cholesterol levels (P > 0.05). Also no significant difference could be documented between the patient and control groups regarding the aortic intima-media thickness and mean platelet volume (P > 0.05). However, there was a significant correlation between aortic intima-media thickness and mean platelet volume (r = 0.351; P < 0.05). CONCLUSIONS: In the present study, there was no evidence of early atherosclerosis in children with type 1 diabetes. However, mean platelet volume having a significant correlation with aortic intima-media thickness may be useful as an early marker of atherosclerosis.

Hypo- and hypervolemic edema in children with steroid sensitive nephrotic syndrome.

BACKGROUND/AIM: The mechanism of edema formation in nephrotic syndrome is still poorly understood. We aimed to evaluate the volume status in children with steroid-sensitive nephrotic syndrome (SSNS) and to emphasize the importance of echocardiography in demonstrating of volume changes. MATERIALS AND METHODS: Thirty-two SSNS patients and 30 healthy children were enrolled in this study. The volume statuses of patients were evaluated by clinical and laboratory features, including fractional sodium excretion (FENa) and distal sodium/potassium exchange (UK/UNa+K ratio). Inferior vena cava collapsibility index (IVCCI), left atrial diameter (LAD), aortic diameter (AD), and left ventricular mass index (LVMI) were measured using conventional echocardiographic methods. RESULTS: FENa was lower in children with NS; however, the distal K/Na ratio of the patient and control groups did not differ. In addition, IVCCI, LAD, AD, and LVMI were not different among groups. When evaluating the volume status of patients, 8 patients (25%) were hypovolemic while 24 patients (75%) were nonhypovolemic (normovolemic or hypervolemic). LAD was significantly lower in hypovolemic patients. CONCLUSION: The majority of children with SSNS are normovolemic or hypervolemic and echocardiography is an easy and valuable method for the evaluation of volume status in these patients.