Pseudohypoaldosteronism type I in patient admitted with neonatal abstinence syndrome: A case report and review of literature

Pseudohypoaldosteronism (PHA) type 1 is a rare genetic disorder affecting one in 47,000-80,000 newborns. An autosomal dominant type which is restricted to the kidney (also known as rPHA1) and an autosomal recessive type which is more severe and presents with systemic symptoms were reported. A 3-h-old male infant was admitted to the neonatology ward due to high pitch cries, jitteriness, mild tremors, and excessive sleep with a positive history of maternal addiction. For the 1st time, after 9 days, the infant developed hyperkalemia (7 mEq/L) with mixed respiratory alkalosis, and metabolic acidosis (PH: 7.42, Pco2: 22 mm-Hg, Hco3: 12 mmol/L) and blood pressures of 84/44 mm-Hg were recorded. Even after initial treatment, once again the patient developed hyperkalemia resistant to therapy. Hyponatremia was also noted. Hormonal assays were collected, and congenital adrenal hyperplasia (a common differential diagnosis in neonatal hyperkalemia) was ruled out. Elevated renin and aldosterone levels were reported. On the 20th day, hyperkalemia-induced cardiac arrhythmia was demonstrated in the 2nd lead (K+ - 7.5 mEq/L). We report that this case as PHA type 1 is the rare but life-threatening cause of neonatal hyperkalemia. Initial therapy can prevent the possible occurrence of sudden death

Predictors of Thyroid Gland Invasion in Laryngeal Squamous Cell Carcinoma

Introduction: Laryngeal squamous cell carcinoma (SCC) can invade the thyroid gland leading to unnecessary thyroidectomies with subsequent hypothyroidism and hyperparathyroidism. Thus, clinicopathological variables should be defined in order to predict thyroid gland invasion preoperatively.   Materials and Methods: We performed a retrospective analysis of 1,465 patients with laryngeal SCC referred to our center between March 2009 and January 2016. Among these patients, 60 individuals underwent total laryngectomy and either thyroid lobectomy and isthmectomy or total thyroidectomy.   Results: Thyroid gland invasion was observed in 20% of samples. The following variables were associated with thyroid gland invasion: transglottic spread of the tumor (odds ratio [OR]: 2.04, 95% confidence interval [CI]: 1.15–5.81, P=0.004), thyroid cartilage involvement (OR: 1.53, 95% CI: 0.94–2.50, P=0.02), and anterior commissure involvement (OR: 5.75, 95% CI: 0.86–38.42, P=0.01). In addition, the largest dimension of the tumor was significantly associated with thyroid gland involvement (r=0.36, 95% CI 0.05–0.67, P=0.004). Multivariate linear regression analysis confirmed these findings.   Conclusion: The rate of thyroidectomies performed in cases of laryngeal SCC is much higher than the actual rate of thyroid gland invasion. Thus, preoperative evaluation to find transglottic spread of the tumor, thyroid cartilage, and anterior commissure involvement should be considered

Sirtuin3 ensures the metabolic plasticity of neurotransmission during glucose deprivation

Neurotransmission is an energetically expensive process that underlies cognition. During intense electrical activity or dietary restrictions, the glucose level in the brain plummets, forcing neurons to utilize alternative fuels. However, the molecular mechanisms of neuronal metabolic plasticity remain poorly understood. Here, we demonstrate that glucose-deprived neurons activate the CREB and PGC1α transcriptional program, which induces expression of the mitochondrial deacetylase Sirtuin 3 (Sirt3) both in vitro and in vivo. We show that Sirt3 localizes to axonal mitochondria and stimulates mitochondrial oxidative capacity in hippocampal nerve terminals. Sirt3 plays an essential role in sustaining synaptic transmission in the absence of glucose by providing metabolic support for the retrieval of synaptic vesicles after release. These results demonstrate that the transcriptional induction of Sirt3 facilitates the metabolic plasticity of synaptic transmission

Seizure-Control Effect of Levatiracetam on Juvenile Myoclonic Epilepsy and Other Epileptic Syndromes: Literature Review of Recent Studies

How to Cite This Article: Hashemiaghdam A, Sharifi A, Miri M, Tafakhori A. Seizure-Control Effect of Levatiracetam on Juvenile Myoclonic Epilepsy and Other Epileptic Syndromes: Literature Review of Recent Studies. Iran J Child Neurol. Spring 2015;9(2):1-8.AbstractObjectiveVarious epileptic syndromes may present in adolescence and Juvenile Myoclonic Epilepsy (JME) is known to be the most common idiopathic generalized epileptic syndrome presenting itself with different types of seizure activity. The exact etiology of JME is still unknown, but hypoxia, storage disease, toxic-metabolic disorders, drug reactions, and neurodegenerative disorders have been revealed to cause disease manifestation. Previous research shows that JME includes 5–10% of all cases diagnosed with epilepsy. It is estimated to include 18% of idiopathic generalized epilepsies. Females are at higher risk of developing this condition. Levatiracetam (LEV) is an anti-epileptic drug that has become one of the most used drugs for the management of epileptic syndromes. It has less drug interactions, milder side effects, and broad-spectrum efficacy to make it an ideal drug to control seizures. Different mechanisms of actions have made LEV a novel anti-epileptic drug. This new medication can be used as a mono- or add-on therapy to previous anti-epileptic drugs. One of the clinically valuable pharmacological aspects of LEV is that it can be started at a high therapeutic dosage and is well tolerated. The median starting dosage varied according to patients underlying disease, age, and disease severity. We have also discussed the effect of LEV on other epileptic syndromes, which showed promising results in both adults and children. In childhood epilepsy, there is evidence proving that a higher rate of behavioral disturbances with neurological disorders can be improved by LEV therapy. Finally, our review showed the beneficial effects of LEV on seizure-control in different epileptic syndromes especially as a monotherapy

Involvement of NO/NMDA-R pathway in the behavioral despair induced by amphetamine withdrawal

Abrupt discontinuation of chronic amphetamine consumption leads to withdrawal symptoms including depression, anhedonia, dysphoria, fatigue, and anxiety. These irritating symptoms may result in continuing to take the drug or can lead to suicidal behavior. Past studies have shown the involvement of various biologic systems in depression induced following amphetamine withdrawal (AW). However, there is no evidence about the relation between nitric oxide (NO) with NMDA receptors on depression following AW. In this study, we examined the involvement of the NO/NMDA pathways on depressive-like behaviors after 24 h withdrawal following 5 continuous days of amphetamine administration in male NMRI mice. Behavioral tasks used for depression assessment included the forced swimming test (FST), the Splash test and the open field test (OFT). In order to evaluate the role of NO/NMDA pathways animals treated with MK-801 (NMDA-R antagonist), Aminoguanidine (AG), a selective iNOS inhibitor, Nω-Nitro-L-arginine (L-NNA), a non-selective NOS inhibitor and 7-Nitro indazole (7-NI), a selective nNOS inhibitor. We also measured the level of nitrite in the hippocampus. Our data showed that AW induced the depressive-like effect in the FST and the Splash test. We showed that administration of AG, L-NNA, and MK-801 mitigated AW induced depression, however, 7-NI was failed to decrease depressive-like behaviors. Also, the antidepressant-like effect of co-injection of sub-effective doses of MK-801 with AG suggested that inducible nitric oxide synthase (iNOS) is associated with NMDA-R in AW induced depression. In conclusion, both NO and NMDA-R pathways are involved and related to each other in depression induced following AW. © 201