A Child With a Glasgow Coma Scale Score of 4 After Near-Hanging: A Case Report on Treatment Without Intubation and Targeted Temperature Management.

While prehospital medical interventions are evolving and improving survival rates, the evidence for adequate early prognostic assessment is often insufficient. A 12-year-old Japanese child was found hanging on the roof of his home. After being rescued by his mother, he was transported by an ambulance and a rapid response car (RRC) with doctors, nurses, and paramedics on board, and admitted to our hospital. His initial Glasgow Coma Scale score in the RRC was 4. Although he did not undergo intubation and targeted temperature management (TTM), he had no neurological sequelae upon discharge. To the best of our knowledge, this report is the first to describe the case of a child with a decreased level of consciousness after near-hanging, who was treated without intubation and TTM.

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.

Sneezing-Induced Subclavian Arterial Rupture: A Case of Vascular Ehlers-Danlos Syndrome in a Child.

Vascular Ehlers-Danlos syndrome is a fatal disease caused by a type III collagen mutation that can result in the rupture of blood vessels, the intestinal tract, and/or the uterus. Despite being the most severe form of Ehlers-Danlos syndrome, it is not well known in the pediatric context because it rarely presents serious complications in childhood. In this case, the patient experienced a subclavian artery rupture triggered by sneezing, which was initially managed with an endovascular stent. However, the descending aorta subsequently ruptured, and the patient died. Traditionally, surgical or endovascular treatments have been avoided due to the inherent fragility of blood vessels. Nevertheless, favorable outcomes have been documented with a wait-and-see surgical approach or endovascular treatment, especially when the diagnosis precedes the onset of vascular complications. Notably, celiprolol, a partial ß2-agonist and ß1-blocker, has demonstrated efficacy in preventing vascular complications. Therefore, early diagnosis plays a pivotal role. Raising awareness about this syndrome, along with its management and prophylaxis, holds the potential to enhance the survival rate.

Overall usefulness of newborn screening for congenital hypothyroidism by using free thyroxine measurement.

In Kanagawa Prefecture, Japan, simultaneous measurements of free T4 (FT4) and TSH levels are performed during newborn screening for congenital hypothyroidism (CH). FT4 measurement enables the detection of CH of central origin (CH-C), the incidence of which is estimated to be 1 in 30,833 live births in Kanagawa Prefecture. In this study, we aimed to evaluate the efficacy of FT4 screening when transient CH-C and thyroidal CH (CH-T) with delayed TSH elevation are included as screening targets. Data collected on CH-C patients using a regional survey, as well as data from a database created by a screening organization, were used. Of the 24 CH-C patients who had been born in Kanagawa Prefecture between 1999 and 2008, a positive screening result for FT4 (<0.7 ng/dL) was obtained in 13 newborns; of these, 12 were identified solely through newborn screening. Of the 113 patients for whom positive screening results were obtained during the study period, 5 and 6 were found to have transient CH-C and CH-T with delayed TSH elevation, respectively. Remarkably, 4 out of 5 patients with transient CH-C and all patients with CH-T with delayed TSH elevation were diagnosed through the evaluation of low FT4 at screening. These results indicate that the use of this FT4 screening system facilitates the identification of transient CH-C and CH-T with delayed TSH elevation, thus justifying the inclusion of these entities as screening targets.

Mass screening of newborns for congenital hypothyroidism of central origin by free thyroxine measurement of blood samples on filter paper.

OBJECTIVE: To evaluate the effectiveness of mass screening of newborns for congenital hypothyroidism of central origin (CH-C) by measurement of free thyroxine (FT(4)) and thyroid-stimulating hormone (TSH). DESIGN: Questionnaire-based survey of CH-C patients born between 1999 and 2008 in Kanagawa prefecture, Japan. METHODS: TSH and FT(4) levels in dried blood spots on filter paper were measured using ELISA kits, and CH-C was diagnosed at FT(4) levels below a cutoff of 0.7 ng/dl (9.0  pmol/l). Survey results were collated with the database created by the screening organizer. RESULTS: Twenty-four CH-C patients (18 males) were identified, 14 of whom had multiple pituitary hormone deficiencies (group M), eight had isolated CH-C (group I), and two had undetermined pituitary involvement (group U). In groups M, I, and U, the number of patients with FT(4) levels below the cutoff value at screening was five (36%), seven (88%), and one (50%) respectively; other patients had been diagnosed clinically. Thus, 13 patients were true positives, while nine were false negatives, yielding screening sensitivity of 59.1% and positive predictive value of 11.5%. The calculated sensitivity was 81.8% at a higher cutoff value of 0.9  ng/dl (11.6  pmol/l). The overall incidence of CH-C was estimated at 1 in 30, 833 live births, while that of CH of thyroidal origin (CH-T) is 1 in 3472 live births in Kanagawa prefecture (CH-T/CH-C, 8.9). CONCLUSIONS: Newborn screening with combined FT(4) and TSH measurements can identify a significant number of CH-C patients before manifestation of clinical symptoms, but a more appropriate FT(4) cutoff value should be considered.

Total synthesis of brevenal.

A total synthesis of brevenal is described. The pentacyclic ether core was constructed by the intramolecular allylation of alpha-acetoxy ether and subsequent ring-closing metathesis. Both of the diene side chains were introduced by Wittig olefination and a Horner-Wadsworth-Emmons reaction, respectively, in a highly stereoselective manner.