Serum copeptin as a biomarker of hypertension in children with obesity.

BACKGROUND: Hypertension (HT) in obesity has been reported frequently in children in recent years. The role of copeptin and uric acid here are not well known. We aimed to investigate the relationship between HT and serum copeptin and uric acid levels in children with obesity. METHODS: We included 80 children with obesity who were admitted to our hospital between April 2018 and September 2018. The patients were separated into two groups: hypertensive and non-hypertensive. Serum copeptin levels were measured using the enzyme-linked immunosorbent assay. RESULTS: Copeptin levels were significantly higher in patients with HT than in those without (p = 0.0001). In addition, serum uric acid levels in patients with HT were significantly higher, while the serum potassium levels were significantly lower (p = 0.01) than in those without HT (p = 0.001). In correlation analyses, a positive correlation was detected between blood sodium and copeptin levels (p = 0.037). CONCLUSIONS: Hypertensive children with obesity had higher serum copeptin and uric acid and lower blood potassium levels. Moreover, copeptin levels were positively correlated with blood sodium levels. Thus, in addition to copeptin, serum uric acid, potassium, and sodium levels may be important in the diagnosis and follow-up of children with HT.

Cause of recurrent rhabdomyolysis, carnitine palmitoyltransferase II deficiency and novel pathogenic mutation.

Carnitine palmitoyltransferase II (CPT II) deficiency is an autosomal inherited metabolic disorder in which the ß-oxidation of the long chain fatty acids is defective. The clinical presentation may be in various forms; it presents itself in the severe form during neonatal and infantile periods and as the less severe myopathic form in the school age and adolescence. While the severity of the rhabdomyolysis attacks varies, occasionally the clinical course may be complicated with acute renal failure. Acylcarnitine analysis may help in the diagnosis of CPT II, but its normality does not indicate the absence of the disease. If there is strong suspicion, genetic analysis should be performed on the cases. In this article, we present a 15-year-old male patient who had two rhabdomyolysis attacks triggered by infection and starvation. Acylcarnitine analysis of the case was normal, CPT II deficiency was considered when the history was evaluated, and CPT II gene c.137A>G (p.Gln46Arg) homozygous novel pathogenic mutation was detected. CPT II deficiency is one of the most common causes of metabolic rhabdomyolysis in patients with recurrent episodes of rhabdomyolysis.

Cytotoxic lesions of the corpus callosum in children: Etiology, clinical and radiological features, and prognosis.

OBJECTIVES: Cytotoxic lesions of the corpus callosum (CLOCCs) are secondary lesions associated with entities like infection manifested by restricted diffusion on diffusion-weighted cranial magnetic resonance imaging. Our objectives are to evaluate the clinic-radiological spectrum of pediatric patients with cytotoxic lesions of the corpus callosum (CC). METHODS: Children (0-18 years) admitted between February 2017 and May 2020 with splenial lesions showing diffusion restriction on MRI, either isolated or within involvement of other parts of the brain, were included retrospectively. The primary lesions of the CC (e.g. acute disseminated encephalomyelitis, acute ischemic infarction, and glioblastoma multiforme) were excluded. CLOCCs were divided into infection-associated, metabolic disorder-associated, and trauma-associated lesions, as well as CLOCCs involving other entities. Data were collected from the medical databases. RESULTS: Forty-one patients were determined to have CLOCCs. Twenty-five (61%) were infection-associated, nine (22%) were trauma-associated, and three (7%) were metabolic disorder-associated cases, including 2 inherited disorders of metabolism. There were four (10%) patients with other entities, three with epilepsy, and one had an apparent life-threatening event. Six patients had a known etiology among the infection-associated group; one had multisystem inflammatory syndrome caused by COVID-19 and one had been infected by COVID-19 without any complications. All the infection-associated patients with isolated splenial lesions recovered totally, although six patients required intensive care hospitalization. Four trauma-associated patients had sequela lesions. CONCLUSIONS: CLOCCs are associated with a spectrum of diseases, including the new coronavirus, COVID-19 infection. Infection-associated CLOCCs has the best prognosis, although severe cases may occur. Sequelae are possible based on the etiology.

Efficacy and Side Effect Profile of Clobazam in Children with Different Etiologies of Epilepsy from a Single Center.

OBJECTIVES: Clobazam is a long-acting antiepileptic drug that belongs to benzodiazepines used in the polytherapy of childhood epilepsy. In this study, our aim is to retrospectively evaluate the effectiveness and side effect profile of clobazam in children with different etiologies of epilepsy, mostly drug resistant. METHODS: Forty patients aged 0-18 years that were admitted to Okmeydani Training and Research Hospital pediatric neurology outpatient clinic between January 2017-January 2019 and prescribed clobazam were included in this study. The data of the patients who gave informed consent were extracted retrospectively from the outpatient clinic files. The patients with no seizures over 50% reduction in seizures were classified as clobazam-responsive, whereas the patients with less than 50% reductions in seizures, patients who had no response, and who manifested side effects and stopped using the drug were classified as clobazam-unresponsive. RESULTS: Twenty-three of the patients (57.5%) were male, 17 were (42.5%) were female. The average onset age of epilepsy was 31.8±37.2 months, while the average age for the prescription of clobazam was 70.6±48.9 months. The types of seizures were focal in 23 patients (57.5%) and generalized in 17 (42.5%) patients. Thirty-three (82.5%) patients had been using double or triple combinations of eight different antiepileptic drugs when clobazam was added to their treatment and accepted as drug-resistant epilepsy. The etiology of twenty one patients (52.5%) was unknown. In the remaining 19 patients (47.5%), the most common cause was structural and others were genetic, infectious and metabolic. Thirty one of the patients (77.5%) were responsive to clobazam. Of them, fifteen (37.5%) had no seizures, and 16 had a reduction in seizures (>50%). Nine (22.5%) patients were accepted as unresponsive to clobazam. The mean dose per kg was 0.7±0.3 mg/kg/day with a median of 0.63 mg/kg/day. Side effects of clobazam were encountered in 18 patients (45%); these resulted in the cessation of administration in only six (15%) patients. The side effects that cause the cessation of clobazam were sedation, refusal to take the drug due to the taste, irritability, hypersalivation, and malaise. Four patients (10%) had their doses reduced, seven patients (17.5%) responsive to clobazam although with side effects continued taking the drug as prescribed. The most common side effects of all were hyperactivity and sedation consecutively. CONCLUSION: Clobazam is an effective treatment for ensuring seizure freedom in pediatric epilepsy, mostly drug-resistant. The side effects are at tolerable levels in patients who are responsive to the drug.