The Japanese Pediatric Continuous Renal Replacement Therapy (jpCRRT) Registry: Study Protocol.

BACKGROUND: The use of continuous renal replacement therapy (CRRT) in critically ill children is rapidly increasing, but the standard of care has not yet been established and prognosis remains poor. To develop optimal CRRT strategies, we launched a research project generating the Japanese Pediatric CRRT registry, a multicenter registry of CRRT in Japanese pediatric intensive care units (PICUs), to investigate the actual status of CRRT in recent years in PICUs, where data are lacking. METHODS: This manuscript presents a protocol for planning a multicenter prospective registry. As of April 2023, 15 Japanese PICUs are voluntarily participating. Patients enrolled are those <16 years of age who enter the PICUs of the collaborating institutions, require CRRT, and have the guardians' consent. CRRT is defined as anticipated to be required for >24 hours, and CRRT connected to extracorporeal membrane oxygenation is also included. The registry is an online registry system managed by the University Hospital Medical Information Network. The primary outcomes are Pediatric Cerebral Performance Category Scale at PICU discharge and 6 months post-discharge (deaths included), persistent need for dialysis, and PICU readmission within 6 months. The secondary outcomes are adverse events during and immediately after CRRT initiation, and initial circuit life span. CONCLUSIONS: This project will examine the differences in outcomes of CRRT in PICUs in specific patient and treatment groups and will be used to design future interventional studies. We will also aim to establish a platform for a multicenter registry study in Japanese PICUs, considering the current lack of such a platform.

Possible Role of High-Dose Barbiturates and Early Administration of Parenteral Ketogenic Diet for Reducing Development of Chronic Epilepsy in Febrile Infection-Related Epilepsy Syndrome: A Case Report.

We describe the efficacy of high-dose barbiturates and early administration of a parenteral ketogenic diet (KD) as initial treatments for acute status epilepticus (SE) in an 8-year-old girl with febrile infection-related epilepsy syndrome (FIRES). The patient was admitted to our hospital with refractory focal SE. Abundant epileptic discharges over the left frontal region were observed on electroencephalogram (EEG). Treatment with continuous infusion of thiamylal for 4 hours, increased incrementally to 40 mg/kg/h, successfully ended the clinical SE, and induced a burst-suppression coma. The infusion rate was then gradually decreased to 4 mg/kg/h over the next 12 hours. Parenteral KD was administered from days 6 to 21 of illness. Continuous infusion of thiamylal was switched to midazolam on day 10 without causing seizures or EEG exacerbations. The patient has remained seizure free in the 15 months since hospital discharge. The effectiveness of KD for the treatment of FIRES has attracted attention amongst clinicians, but KD treatment may need to last for 2 to 4 days before it can stop SE, a time period that could cause irreversible brain damage. Considering the severity of SE in our patient and the dose of barbiturates needed to treat it, we consider this case to have had a good clinical outcome. The results suggest that rapid termination of seizure using high-dose barbiturates in conjunction with early administration of parenteral KD could reduce the development of chronic epilepsy in patients with FIRES.

Isolated mediastinal lymphadenitis caused by Mycobacterium malmoense in an immunocompromised child.

Mycobacterium malmoense is a nontuberculous mycobacteria (NTM), that is uncommon in areas other than Northern Europe. We describe the case of mediastinal lymphadenitis caused by M. malmoense in a 4-year-old boy who has a past medical history of disseminated Bacille de Calmette et Guérin (BCG) infection. He presented with persistent high fever and computed tomography revealed mediastinal lymphadenopathy. We identified M. malmoense by hsp65 gene analysis from a lymph node biopsy sample. We treated him with rifampicin, ethambutol and clarithromycin with reference to the guidelines of the British Thoracic Society. M. malmoense can cause severe infections including mediastinal lymphadenitis in children with susceptibility to acid-fast bacteria (AFB).