Early predictors of high-flow nasal cannula oxygen treatment failure in patients with respiratory distress admitted to the pediatric emergency department.

BACKGROUND: High-flow nasal cannula (HFNC) therapy is a relatively new method used in patients with respiratory distress. The aim of the study was to evaluate the outcomes and to determine the baseline predictors of HFNC treatment failure in children with acute respiratory distress/failure in the pediatric emergency department. METHODS: Children with respiratory distress/failure aged 1 month to 18 years who underwent HFNC therapy with the pre-established protocol were retrospectively analyzed. HFNC therapy was used in respiratory and non-respiratory pathologies. HFNC failure was defined as the need for escalation to non-invasive ventilation or invasive mechanical ventilation. HFNC responders and non-responders were compared based on baseline clinical data. RESULTS: Of the 524 cases (median age:13 months; 292 males / 232 females), 484 (92.4%) had respiratory tract and 40 (7.6%) had non-respiratory tract pathologies. HFNC therapy was unsuccessful in 62 (11.8%) patients. The success rates were 81% and 55% in respiratory and non-respiratory diseases, respectively. In children with respiratory system pathologies, the pre-treatment venous pCO2 level (p: 0.045; OR: 0.958; 95%CI: 0.821-0.990) and the clinically important radiological finding on chest X-ray (lobar infiltration, atelectasis, pleural effusion) (p: 0.045; OR: 3.262; 95%CI: 1.178-9.034) were the most significant parameters in predicting HFNC failure. In children with non-respiratory pathologies, the pre-treatment venous lactate level (p: 0.008; OR: 1.558; 95%CI: 1.125-2.158) was a significant predictor of HFNC failure. There were no cases of pneumothorax or any other reported adverse effects related to HFNC therapy. CONCLUSIONS: HFNC treatment is a safe oxygen therapy in children with respiratory distress/failure due to various etiologies in the emergency department. The lower venous pCO2 level increases and the clinically important radiological finding on chest radiograph decreases the success of HFNC treatment in respiratory pathologies. The higher venous lactate level is a predictor of HFNC treatment failure in non-respiratory pathologies.

Variant ataxia-telangiectasia in a child presenting with laryngeal dystonia.

BACKGROUND: Dystonia is a common hyperkinetic movement disorder in children; however, making an early and definitive diagnosis of dystonia can sometimes be challenging for clinicians. CASE: Herein, we report a case of a 16 years-old girl presenting with laryngeal dystonia due to compound heterozygosity of a known pathogenic and a novel variant in the ATM gene. Serum alpha-fetoprotein level was elevated. Serum IgG, IgA, IgM and IgE levels were within normal range. Treatment with L-DOPA had no benefit. Her symptoms were dramatically improved by localized botulinum toxin injections. CONCLUSION: Mutations in the ATM gene show a wide phenotypic spectrum from severe classical early-onset ataxia-telangiectasia (A-T) to late-onset milder variant A-T. Our findings highlight the importance of recognizing laryngeal dystonia as one of the clinical signs of A-T.

Pediatric case of persistent hiccups associated with hypertrophic olivary degeneration.

BACKGROUND: Hypertrophic olivary degeneration (HOD) is a rare degenerative disorder that is thought to occur subsequent to a disruption of the dentate-rubro-olivary pathway. CASE: We report a pediatric case of unilateral HOD presented with persistent hiccups and palatal tremor. Radiological examination of diaphragm was normal considering ultrasound and chest x-ray. On T2WI (weighted images) and Fluid Attenuated Inversion Recovery (FLAIR) images, hyperintense enlargement of the right inferior olivary nucleus was seen. No abnormal enhancement was detected on post-contrast scans and no evidence of restricted diffusion was seen. Susceptibility weighted imaging (SWI) sequences revealed a chronic hemorrhage involving the medulla oblongata and cerebellum. Cranial magnetic resonance imaging (MRI) findings were consistent with unilateral HOD. Palatal tremor and dentate-rubral tremor are frequent presentation of HOD, however to our knowledge persistent hiccups had not yet been reported in children with HOD. CONCLUSION: We highlight a pediatric case of unilateral HOD, which presented with persistent hiccups. Awareness of clinical and radiological findings of HOD is important to avoid misinterpretation as a mass lesion, an ischemic event, or a demyelinating disease and provide adequate management.