Tamer Çelik

Cerebral Salt Wasting in Status Epilepticus: Two Cases and Review of the Literature

BACKGROUND Cerebral salt wasting is a hypovolemic hyponatremia state, caused by natriuresis and diuresis. The most important element of treatment is to replace the volume and sodium loss and improve the current clinic. PATIENTS We present two children who were treated in the intensive care unit who subsequently developed cerebral salt wasting. Diagnosis was based on hyponatremia associated with high urinary sodium excretion and inappropriately high urine output in the presence of dehydration. As part of the treatment, one patient was given fluid and sodium replacement, measures that were insufficient in the other patient, who also required fludrocortisone treatment. CONCLUSION The status epilepticus may be involved in the etiology of cerebral salt wasting. In both patients, cerebral salt wasting was detected in the posttreatment follow-up evaluations. Cerebral salt wasting is particularly likely to occur in individuals with status epilepticus, and the electrolyte and hydration status of these patients should be monitored closely, even after the convulsions are taken under control.

Metabolic acidosis in a patient with type 1 diabetes mellitus complicated by methanol and amitriptyline intoxication.

Diabetic ketoacidosis (DKA) is a widely known acute metabolic complication of diabetes mellitus (DM), which can be potentially fatal. It is not difficult to diagnose when a patient with DM comes with symptoms such as coma, fruity breath, hyperglycemia, acidosis, and tachypnea. If the patient has not been diagnosed with DM before, then other sicknesses characterized by an increased anion gap should be considered. A 12-year-old boy with type 1 DM and repeated earlier admissions for DKA was admitted to the emergency department in another apparent case of DKA with coma, hyperglycemia, and profound metabolic acidosis. When his condition did not improve with initial treatment, intoxication was suspected as an alternate cause of his condition. Further laboratory tests detected methanol and amitriptyline. The patient underwent hemodialysis and recovered completely. This case illustrates that a seemingly obvious medical condition can mask serious intoxication. This report is the only publication on two different entities characterized by an increased anion gap and at the end the patient has been cured completely without any complications.

Epstein-Barr virus encephalitis with substantia nigra involvement

Infectious mononucleosis due to Epstein–Barr virus (EBV) is a usually benign systemic viral illness common in children. Many studies described nervous system manifestations of infectious mononucleosis with a wide spectrum of neurologic deficits. Neurologic complications of EBV are seen in both acute and reactivate infection. Herein, we describe a patient diagnosed by acute EBV encephalitis with substantia nigra involvement and excellent clinical recovery.

Concurrency of Guillain-Barre syndrome and acute transverse myelitis: a case report and review of literature

Guillain-Barré syndrome and acute transverse myelitis manifest as demyelinating diseases of the peripheral and central nervous system. Concurrency of these two disorders is rarely documented in literature. A 4-year-old girl presenting with cough, fever, and an impaired walking ability was admitted to hospital. She had no previous complaints in her medical history. A physical examination revealed lack of muscle strength of the lower extremities and deep tendon reflexes. MRI could not be carried out due to technical problems; therefore, both Guillain-Barré syndrome and acute transverse myelitis were considered for the diagnosis. Intravenous immunoglobulin treatment was started as first line therapy. Because this treatment did not relieve the patients symptoms, spinal MRI was carried out on the fourth day of admission and demyelinating areas were identified. Based on the new findings, the patient was diagnosed with acute transverse myelitis, and high dose intravenous methylprednisolone therapy was started. Electromyography findings were consistent with acute polyneuropathy affecting both motor and sensory fibers. Therefore, the patient was diagnosed with concurrency of Guillain-Barré syndrome and acute transverse myelitis. Interestingly, while concurrency of these 2 disorders is rare, this association has been demonstrated in various recent publications. Progress in diagnostic tests (magnetic resonance imaging and electrophysiological examination studies) has enabled clinicians to establish the right diagnosis. The possibility of concurrent Guillain-Barré syndrome and acute transverse myelitis should be considered if recovery takes longer than anticipated.

Salbutamol intoxication: is salbutamol a drug-inducing fever? A case report and treatment strategy.

A four-year-old female with salbutamol intoxication was referred to our paediatric emergency medicine unit, due to agitation, tremulousness, sinus tachycardia, mild hypokalaemia and hyperglycaemia. On admission the child was agitated and had a noticeable tremor, an axillary temperature of 38 degrees C and a pulse rate of 185 beats/min. She had no identifiable focus of infection on physical examination to explain her fever. Gastric lavage, activated charcoal, intravenous hydration and electrocardiogram (ECG) monitoring were performed. Her plasma potassium level, blood sugar and QT interval were closely monitored during her hospital stay. Her fever, tachycardia and serum potassium and glucose levels returned to normal and she was discharged in good condition 24 h after admission. The difference of this case from prior cases of salbutamol intoxication was the observation of fever in the absence of evidence of infection. Since the cause of fever was not a reaction to the medication used in the treatment or related to environmental factors, it is assumed that salbutamol is a fever-inducing drug.

Placento-Cranial Adhesions in Amniotic Band Syndrome and the Role of Surgery in Their Management: An Unusual Case Presentation and Systematic Literature Review

Amniotic band syndrome is a group of sporadic congenital anomalies that involve the limbs, craniofacial regions and trunk, ranging from simple digital band constriction to complex craniofacial and central nervous system abnormalities. Placento-cranial adhesions in amniotic band syndrome are extremely rare, and severe conditions are associated with high morbidity and mortality rates. In this study, we pooled placento-cranial adhesion case reports that were published in the medical literature and added an unpublished case from our institution. The purpose of this article was to review and discuss the clinical features and outcomes of placento-cranial adhesions in amniotic band syndrome.

Appraisal of the ″pediatric ARDS: consensus recommendations from the pediatric acute lung injury consensus conference″ with the AGREE II instrument

Background/aim: The ″Pediatric Acute Lung Injury Consensus Conference″ (PALICC) was convened in order to develop a taxonomy to define pediatric acute respiratory distress syndrome (ARDS). The Appraisal of Guidelines for Research and Evaluation (AGREE) assesses the quality of guidelines. The aim of this study is to evaluate the new pediatric ARDS guideline using the AGREE II instrument. To the best of our knowledge, this is the first assessment of the new pediatric ARDS clinical practice guideline in the English literature. Materials and methods: Four appraisers assessed the new pediatric ARDS guideline with the AGREE II instrument. At the end of the evaluation each appraiser rated the overall quality of the guidelines. Results: Results of the assessment were editorial independence 100%, clarity of presentation 94%, scope and purpose 89%, stakeholder involvement 78%, rigor of development 78%, and applicability 78%. Conclusion: The new pediatric ARDS guideline received good scores especially with respect to editorial independence and clarity of presentation. Our overall AGREE II review of the PALICC guideline indicates that it has been created using high quality methodology and should be recommended for use and implementation as currently published.

Retrospective analysis of children with myelin oligodendrocyte glycoprotein antibody-related disorders

BACKGROUND Knowledge has been expanding on myelin oligodendrocyte glycoprotein (MOG) antibody-associated central nervous system disorders. We delineate the clinical and paraclinical findings and outcome of our pediatric patients with MOG antibody seropositive disease. METHODS We retrospectively analyzed the clinical presentation, cerebrospinal fluid findings, magnetic resonance imaging (MRI) studies, course and outcome of children seropositive for anti-MOG IgG. RESULTS Total 20 children with neurological symptoms and serum anti-MOG IgG were identified from six centers in Turkey. Median age at onset was 9 years (mean 8.8 ± 5.0 years, range: 1.5-16.5 years). Final diagnoses were acute disseminated encephalomyelitis (ADEM) (n = 5), ADEM + optic neuritis (n = 4), neuromyelitis optica spectrum disorder (NMOSD) (n = 3), myelitis (n = 2), relapsing optic neuritis (n = 2), multiphasic DEM (n = 3), and unclassified relapsing demyelinating disease (n = 1). Seven/20 (35%) children experienced a single episode while 13/20 (65%) had a least one relapse during follow-up. On MRI, subcortical white matter, brainstem, and corpus callosum were preferentially involved regions. Full recovery was observed in 15/20 (75%) children. CONCLUSION MOG autoimmunity in children has a wide clinical spectrum, tendency to relapse, and a favourable outcome compared with other relapsing demyelinating diseases.

An Adolescent Case of Osteopetrosis with Portal Hypertension as well as Mandibula Osteomyelitis.

Osteopetrosis is a clinical syndrome characterized by the failure of osteoclasts to resorb bone. Excessive bone density can interfere with vital tissues and structures, causing serious problems of the body. Hematopoietic insufficiency, disturbed tooth eruption, nerve entrapment syndromes, and growth impairment may develop in a patient with osteopetrosis. Herein, we present an adolescent girl diagnosed with non-infantile type of osteopetrosis with rare complications of the disease like mandibular osteomyelitis and portal hypertension (PHT) without liver cirrhosis. To our knowledge, this is the first pediatric case with osteopetrosis related PHT.

PP07.6 – 2810: A rare cause of status epilepticus in infant; Alpha lipoic acid intoxication

Objective Alpha lipoic acid (ALA), also known as thiotic acid, is a cofactor found in a number of multienzyme complexes. Lipoic acid is a powerful antioxidant and is widely used as supplementary treatment of diabetic neuropathy. There is no reported dose of safety in children. Methods A 14-month-old previously healthy girl was referred to our hospital with the diagnosis of drug intoxication. She was admitted to the emergency department with lethargy and involuntary movements for several hours after ingesting unknown number of pills of 600 mg ALA belonging to a diabetic parent at home. On admission she was lethargic, bilateral extensor toe sign, and having myoclonic seizures involving all extremities. She had no fever and laboratory examinations were normal except mild methabolic ascidosis (pH: 7.24). The seizures were unresponsive to bolus midazolam injection, fenitoin infusion at 20 mg/kg dose and levetiracetam infusion at 20 mg/kg dose. She was taken to the pediatric intensive care unit with the diagnosis of status epilepticus. Results After failure of the treatment with midazolam infusion (up to 1 mg/kg/hour) she was intubated and thiopental sodium infusion was started. Her myoclonic seizures was controlled with the thiopental sodium infusion (3 mg/kg/hour). After 48 hour intubation and mechanical ventilation the thiopental sodium infusion was gradually reduced and stopped. After withdrawing the thiopental sodium infusion she was seizure free, levetiracetam continued as anticonvulsant drug and she was discharged at the 8. day. Conclusion Alpha lipoic acid and derivatives cause side effects in children like refractory convulsions. As diabetes and the frequency of diabetic neuropathy increase, possibility of ALA usage and intoxication in children living in the same house also rises. When faced with refractory convulsions in children who have had no disease before, intoxication by medicaments with ALA should be taken into consideration.