Skeletal Muscle Mass Assessment in Pediatric Patients: Development of a Normative Equation and Assessment of Factors Associated With a Low Skeletal Muscle Mass in PICU Patients.

OBJECTIVES: To develop an equation for defining a low skeletal muscle mass (SMM) in children and to investigate risk factors and outcomes associated with low SMM in critically ill pediatric patients. DESIGN: Single-center retrospective pediatric cohorts, 2011-2018. SETTING: Tertiary Emergency and Critical Care Center of Kyushu University Hospital in Japan. PATIENTS: We studied two cohorts of pediatric patients 1-15 years old who underwent abdominal CT at the level of the third lumbar vertebra (L3). First a cohort of trauma patients presented to the emergency department in whom we developed an SMM regression equation. Second, a cohort of patients who had undergone abdominal CT within 3 days of PICU admission. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The equation for estimating normal SMM used sex, age, and weight. Low SMM was defined as less than 80% of normal. In the 112 patients in the PICU cohort, median (range) age was 68 (13-191) months, and 83 (74.1%) had underlying disease. There was low SMM in 54 patients (48.2%). Regarding associations, using odds ratio (OR) and 95% CI, we found that low dietary intake (OR 4.33 [95% CI, 1.37-13.70]; p = 0.013) and the presence of underlying disease (OR 7.44 [95% CI, 2.10-26.30]; p = 0.002) were independently associated with greater odds of low SMM. Low SMM, compared with normal SMM, was also associated with longer hospital stays (42.5 d vs. 20.5 d; p = 0.007; ß, 1.59; 95% CI, 1.09-2.33; p = 0.016). CONCLUSIONS: In this retrospective PICU cohort from a single center in Japan, we found that low SMM at PICU admission was present in almost half the cases. Low SMM, as defined by being less than 80% of the normal, was associated with greater odds of low dietary intake and underlying chronic disease. Furthermore, low SMM was associated with longer hospital stays.

Quantitative indices for an intracranial aneurysm and subarachnoid hemorrhage in early childhood: a case report.

BACKGROUND: Intracranial aneurysms (ICA) rarely occur in children under 3 years of age. Little is known for neuroimaging parameters that predict survival and clinical outcomes of patients with ICA in early childhood. CASE PRESENTATION: A 2-year-old girl showed intracranial hemorrhage due to a rupture of aneurysm at the middle cerebral artery. Quantitative measurements of ischemic damages on the head computed tomography (CT) marked an extremely low score of 2 points with modified Alberta Stroke Program Early CT Score (mASPECTS). She died 15 days after admission. In publications from 2021 to 2022, we found 21 children who were under 3 years of age at onset of ICA. None of them died, but two of three patients who had mASPECTS scores 0-8 showed developmental delay and/or epilepsy as neurological complications. CONCLUSION: Early CT findings are applicable for predicting survival and neurological outcomes of young children with intracranial hemorrhage.

Validation of the PECARN head trauma prediction rules in Japan: A multicenter prospective study.

BACKGROUND: Head trauma in children is one of the most common causes for emergency department visits. Although most trauma cases are minor, identifying those patients who have clinically important traumatic brain injury (ciTBI) is challenging. The Pediatric Emergency Care Applied Research Network (PECARN) head trauma prediction rules identifying children who do not require cranial computed tomography (CT) were validated and are used all over the world. However, these rules have not been validated with large cohort multicenter studies in Asia. OBJECTIVES: To investigate whether the PECARN rules can be safely applied to Japanese children. METHODS: We conducted a multicenter, prospective, observational cohort study. We included children younger than 16 with minor head trauma (Glasgow Coma Scale ≥14) who presented to the six participating centers within 24 h of their injuries between June 2016 and September 2017. The primary analysis was set to calculate the negative predictive value of the patients with very low risk by the PECARN rules, compared with a preset threshold of 99.85%. RESULTS: We included 6585 children of which 463 (7.0%) had head CT scans performed and 23 (0.35%) had ciTBI. There were two patients with ciTBI who were classified as very low risk. The negative predictive value, calculated as 99.96% (95%CI: 99.86-100.00; P = .019), was significantly superior compared with the preset threshold of 99.85%. CONCLUSIONS: The PECARN head trauma prediction rules seemed to be safely applicable to Japanese children. Further studies are needed to determine safety in hospitals where physicians do not have expertise in managing children.

Pulmonary Embolism Mimicking Infectious Pleuritis.

The diagnosis of pulmonary thromboembolism (PE) is often delayed because it is usually misdiagnosed as pneumonia or deep vein thrombosis. We report an unusual case of PE misdiagnosed as viral pleuritis on the first arrival at the emergency department (ED) in our hospital. A 14-year-old girl with no previous significant medical history was referred to the ED with pleuritic and chest pain with low-grade fever 4 days before admission. Echography showed a small amount of left pleural effusion. A 12-lead electrocardiogram was normal. She received a diagnosis of viral pleuritis. Two days before admission, she revisited ED with dyspnea and exacerbated pain. Echography showed slight increase in left pleural effusion. She had the same diagnosis. The chest pain remained at the same level. On the day of admission, she presented to ED with vomiting, watery diarrhea, abdominal pain, chest pain, and respiratory distress. Laboratory findings showed hypoalbuminemia and proteinuria. Echography showed a moderate amount of pleural effusion on both sides and no dilatation of the right cardiac ventricle. Contrast-enhanced chest computed tomography was performed to search the cause of the respiratory distress, which showed filling defects with contrast material in pulmonary arteries. A 12-lead electrocardiogram showed an S1Q3T3 pattern. She received a diagnosis of PE caused by nephrotic syndrome. Pulmonary thromboembolism can mimic infectious pleuritis and lead to misdiagnosis and/or delayed diagnosis. Thus, risk factors of PE should be considered in pediatric patients presenting with symptoms suggesting infectious pleuritis.

Severe acidemia on arrival not predictive of neurologic outcomes in post-cardiac arrest patients.

PURPOSE: This study aimed to determine whether severe acidemia (pH <7.2) on arrival at the emergency department (ED) is a predictive factor for neurologic outcomes of post-cardiac arrest patients treated with targeted temperature management (TTM). MATERIALS AND METHODS: Data in the National Disaster Medical Center, a tertiary care hospital, were used to perform a case-control study on post-cardiac arrest patients treated with TTM from January 2013 to April 2015. The case group comprised patients with good neurologic outcomes (cerebral performance categories 1 and 2), whereas the control group comprised patients with poor neurologic outcomes (cerebral performance categories 3-5). Exposure was defined as arterial pH less than 7.2 on arrival at the ED. RESULTS: We identified 32 patients matching our criteria, of which 13 had good outcomes and 19 poor outcomes. Arterial pH on arrival was not significantly associated with neurologic outcomes (P = .47; odds ratio, 0.5; 95% confidence interval, 0.09-2.61). In 24 patients with cardiogenic causes of cardiac arrest, pH on arrival was not significantly associated with neurologic outcomes (P = .68; odds ratio, 0.5; 95% confidence interval, 0.09-2.73) after matched-pair analysis by age, sex, and presence of light reflex. CONCLUSION: Severe acidemia on arrival at the ED is not a significant predictive factor for neurologic outcomes in post-cardiac arrest patients treated with TTM, particularly in patients with cardiogenic causes of cardiac arrest. These results suggest that treatment should not be withheld in post-cardiac arrest patients with severe acidemia.

Delayed diagnosis in a child with strangulated mesenteric hernia.

Background: Strangulated intestinal obstruction is a life-threatening condition that should be considered as a differential diagnosis in children with shock. However, it has pitfalls in diagnosis and can lead to diagnostic errors. Case Presentation: A 3-month-old male patient presented with a pale complexion lasting 2 h and abnormal crying. He was in shock with lactic acidosis, altered mental status, and slight abdominal distension. He required volume resuscitation, vasoactive agents, and transfusion. On Day 2, he had marked abdominal distension and acute kidney injury, which required continuous kidney replacement therapy. Contrast-enhanced computed tomography revealed extensive intestinal ischemia. It took 33.5 h from his arrival to the computed tomography, leading to operative management. The small intestine had entered a mesenteric hiatus, leading to ischemia. He was diagnosed with strangulated mesenteric hernia. Conclusion: In this case, four pitfalls led to delayed diagnosis. Factors for diagnostic errors specific to strangulated intestinal obstruction and intensive care should be noted.

Survival trends of extracorporeal membrane oxygenation support for pediatric emergency patients in regional and metropolitan areas in Japan.

BACKGROUND: To assess the performance of pediatric extracorporeal membrane oxygenation (ECMO) centers, outcomes were compared between metropolitan and other areas. METHODS: A retrospective cohort study was conducted at three regional centers on Kyushu Island and the largest center in the Tokyo metropolitan area of Japan. The clinical outcomes of patients of ≤15 years of age who received ECMO during 2010-2019 were investigated, targeting the survival and performance at discharge from intensive care units (ICUs), using medical charts. RESULTS: One hundred and fifty-five patients were analyzed (regional, n = 70; metropolitan, n = 85). Survival rates at ICU discharge were similar between the two areas (64%). In regional centers, deterioration of Pediatric Cerebral Performance Category (PCPC) scores were more frequent (65.7% vs. 49.4%; p = 0.042), but survival rates and ΔPCPC scores (PCPC at ICU discharge-PCPC before admission) improved in the second half of the study period (p = 0.005 and p = 0.046, respectively). Veno-arterial ECMO (odds ratio [OR], 3.00; p < 0.03), extracorporeal cardiopulmonary resuscitation (OR, 8.98; p < 0.01), and absence of myocarditis (OR, 5.47; p < 0.01) were independent risk factors for deterioration of the PCPC score. A sub-analysis of patients with acute myocarditis (n = 51), the main indicator for ECMO, revealed a significantly higher proportion of cases with deteriorated PCPC scores in regional centers (51.9% vs. 25.0%; p = 0.049). CONCLUSIONS: The survival rates of pediatric patients supported by ECMO in regional centers were similar to those in a metropolitan center. However, neurological outcomes must be improved, particularly in patients with acute myocarditis.

Rapid and long-lasting efficacy of high-dose ambroxol therapy for neuronopathic Gaucher disease: A case report and literature review.

Gaucher disease (GD) is a lysosomal storage disorder caused by a deficiency in the GBA1-encoded enzyme, ß-glucocerebrosidase. Enzyme replacement therapy is ineffective for neuronopathic Gaucher disease (nGD). High-dose ambroxol has been administered as an alternative treatment for a group of patients with nGD. However, little is known about the clinical indication and the long-term outcome of patients after ambroxol therapy. We herein report a case of a female patient who presented with a progressive disease of GD type 2 from 11 months of age and had the pathogenic variants of p.L483P (formerly defined as p.L444P) and p.R502H (p.R463H) in GBA1. A combined treatment of imiglucerase with ambroxol started improving the patient's motor activity in 1 week, while it kept the long-lasting effect of preventing the deteriorating phenotype for 30 months. A literature review identified 40 patients with nGD, who had received high-dose ambroxol therapy. More than 65% of these patients favorably responded to the molecular chaperone therapy, irrespective of p.L483P homozygous, heterozygous or the other genotypes. These results highlight the long-lasting effect of ambroxol-based chaperone therapy for patients with an expanding spectrum of mutations in GBA1.

Clinical parameter-guided initial resuscitation in adult patients with septic shock: A systematic review and network meta-analysis.

Aim: To identify the most useful tissue perfusion parameter for initial resuscitation in sepsis/septic shock adults using a network meta-analysis. Methods: We searched major databases until December 2022 for randomized trials comparing four tissue perfusion parameters or against usual care. The primary outcome was short-term mortality up to 90 days. The Confidence in Network Meta-Analysis web application was used to assess the quality of evidence. Results: Seventeen trials were identified. Lactate-guided therapy (risk ratios, 0.59; 95% confidence intervals [0.45-0.76]; high certainty) and capillary refill time-guided therapy (risk ratios, 0.53; 95% confidence intervals [0.33-0.86]; high certainty) were significantly associated with lower short-term mortality compared with usual care, whereas central venous oxygen saturation-guided therapy (risk ratio, 1.50; 95% confidence intervals [1.16-1.94]; moderate certainty) increased the risk of short-term mortality compared with lactate-guided therapy. Conclusions: Lactate or capillary refill time-guided initial resuscitation for sepsis/septic shock patients may decrease short-term mortality. More research is essential to personalize and optimize treatment strategies for septic shock resuscitation.

A Pitfall of Wheezing - A Large Mediastinal Mass Presenting as Persistent Wheezing: A Case Report.

Anchoring bias is the tendency to pursue only the most salient feature, which can lead to closed-minded thinking in the early stage of the diagnostic process. Wheezes are one of the most frequent chief complaints and highly likely to become an anchoring bias. We described a patient initially receiving a diagnosis of asthma after presenting with persistent wheezes; however, there was no improvement upon treatment for asthma and eventually, an anterior mediastinal mass was found. The patient's respiratory condition suddenly deteriorated when he was placed in a prone position and eventually extracorporeal membrane oxygenation (ECMO) was introduced. We must recognize the danger of anchoring bias with any symptoms. A wheezing patient with an atypical clinical course should undergo further investigations, given the possibility of other etiologies such as a mediastinal tumor. In addition, we have to pay close attention to the patient's position when a mediastinal tumor is suspected.

Flavin Adenine Dinucleotide-Related Adverse Event Necessitating Cardiopulmonary Resuscitation in an Infant.

Mitochondrial rescue drugs, such as vitamin B2, are a treatment modality which can be considered in unexplained cases of cardiac arrest or impaired consciousness in which mitochondrial diseases are considered in the differential. Although case reports exist of children developing a drop in their blood pressure following administration of intravenous flavin adenine dinucleotide (FAD) sodium as vitamin B2, we present the first reported case of a child requiring cardiopulmonary resuscitation (CPR) following FAD sodium infusion for severe bradycardia and hypotension. Intravenous infusion of FAD sodium should be administered as slowly as possible, with careful monitoring of blood pressure and heart rate.

Severe encephalopathy associated with SARS-CoV-2 Omicron BA.1 variant infection in a neonate.

INTRODUCTION: The coronavirus disease 2019 (COVID-19), including the Omicron variant, is less severe in children than in adults. To date, there has been no detailed description of COVID-19-associated severe encephalopathy due to the Omicron variant during the neonatal and early infantile periods. CASE PRESENTATION: During the outbreak of the Omicron variant, a 29-day-old male presented with a pale and ill appearance. The patient was intubated for mechanical ventilation owing to recurrent apnea, which subsequently turned out to be a breath-holding that may have been caused by seizure. In addition, nonconvulsive status epilepticus was observed. Total duration of repetitive seizure activities was approximately 30 min per hour when seizures were most severe. Brain magnetic resonance imaging (MRI) on day 14 revealed extensive hyperintensity in the T2 sequence, hypointensity in the fluid-attenuated inversion recovery (FLAIR) sequence in the deep and subcortical white matter, and diffusion restriction in the corpus callosum. The Omicron BA.1 variant of the severe acute respiratory syndrome coronavirus 2 was detected in his respiratory sample. Follow-up MRI on day 45 revealed multiple cystic cavitations. CONCLUSION: Although COVID-19 is not severe in most children, life-threatening conditions such as COVID-19-associated severe encephalopathy can occur during the neonatal and early infantile periods.

Predictive values of early head computed tomography for survival outcome after cardiac arrest in childhood: a pilot study.

Predicting outcomes of children after cardiac arrest (CA) remains challenging. To identify useful prognostic markers for pediatric CA, we retrospectively analyzed the early findings of head computed tomography (CT) of patients. Subjects were non-traumatic, out-of-hospital CA patients < 16 years of age who underwent the first head CT within 24 h in our institute from 2006 to 2018 (n = 70, median age: 4 months, range 0-163). Of the 24 patients with return of spontaneous circulation, 14 survived up to 30 days after CA. The degree of brain damage was quantitatively measured with modified methods of the Alberta Stroke Program Early CT Score (mASPECTS) and simplified gray-matter-attenuation-to-white-matter-attenuation ratio (sGWR). The 14 survivors showed higher mASPECTS values than the 56 non-survivors (p = 0.035). All 3 patients with mASPECTS scores ≥ 20 survived, while an sGWR ≥ 1.14 indicated a higher chance of survival than an sGWR < 1.14 (54.5% vs. 13.6%). Follow-up magnetic resonance imaging for survivors validated the correlation of the mASPECTS < 15 with severe brain damage. Thus, low mASPECTS scores were associated with unfavorable neurological outcomes on the Pediatric Cerebral Performance Category scale. A quantitative analysis of early head CT findings might provide clues for predicting survival of pediatric CA.

[A case of primary small intestinal malignant lymphoma found based on multiple pulmonary nodules with cavitation].

A 72-year-old man complained of dyspnea and epigastric pain. He was admitted to our hospital with progressive dyspnea and abnormal chest radiograph findings. Chest CT scan on admission showed multiple nodular shadows with and without air-bronchograms, vessels or cavitation. Transbronchial and percutaneous lung biopsy specimens demonstrated poorly differentiated carcinoma. Pulmonary metastases were suspected, but their primary origin was unknown. Chest and abdominal CT scans on the 18th hospital day showed a giant tumor of the small intestine and rapid progression of the pulmonary tumor, forming cavitation. The patient's condition worsened, and he died on the 51st hospital day. At autopsy, a final diagnosis of T-cell lymphoma of the small intestine and pulmonary metastases was obtained. This is a rare case which was found primarily based on the characteristic radiologic features of pulmonary metastases.

Parvovirus B19-Infected Tubulointerstitial Nephritis in Hereditary Spherocytosis.

BACKGROUND: Human parvovirus B19 (B19V) causes glomerulopathy or microangiopathy, but not tubulopathy. We experienced an 11-year-old girl with spherocytosis who developed acute kidney injury on a primary infection of B19V. She presented with anuria, encephalopathy, thrombocytopenia, and coagulopathy, along with no apparent aplastic crisis. METHODS: Continuous hemodiafiltration, immunoglobulin, and intensive therapies led to a cure. RESULTS: A kidney biopsy resulted in a histopathological diagnosis of tubulointerstitial nephritis without immune deposits. The virus capsid protein was limitedly expressed in the tubular epithelial cells with infiltrating CD8-positive cells. CONCLUSIONS: Viral and histopathological analyses first demonstrated B19-infected tubulointerstitial nephritis due to the aberrant viremia with hereditary spherocytosis.

Paediatric abdominal compartment syndrome and shock associated with gastric volvulus improved rapidly by gastric suction in the emergency department.

Abdominal compartment syndrome (ACS) is not rare and has a high mortality in the paediatric intensive care unit. However, there are few reports about this in the emergency department (ED). We report an 8 month-old male patient with ACS in shock associated with gastric volvulus who improved rapidly by a simple procedure in the ED. He had congenital comorbidities, including multiple cranial anomalies and was transferred because of decreased mental status. He had compensated shock with cold and mottled skin of the lower extremities, paediatric Glasgow Coma Scale of E3V5M6 and prominence of the left upper abdomen. The abdominal X-ray showed a considerably distended stomach. Soon after aspiration of gastric contents, shock and mental status improved. Physicians should consider ACS in the differential diagnosis of shock with abdominal distention. ACS may be ameliorated by gastric suction. Gastric volvulus can induce shock and decreased mental status, particularly in patients with comorbidities.

Hidden diagnosis behind viral infection: the danger of anchoring bias.

Anchoring bias is one of the most common diagnostic biases that may lead to closed-minded thinking and could result in unnecessary tests, inappropriate patient management and even misdiagnosis. A 4-year-old boy was brought to the emergency department because of shaking chills. On the basis of bilateral swollen preauricular areas, high level of serum amylase and the prevalence of mumps, he initially received a diagnosis of mumps in spite of the shaking chills. However, blood culture turned out to be positive for two different kinds of bacteria. The patient finally received a diagnosis of polymicrobial bacteraemia resulting from suppurative appendicitis. We must consider and rule out bacteraemia in the differential diagnosis for patients who present with shaking chills, even in the presence of symptoms or information consistent with a more common viral infection such as mumps. In addition, intra-abdominal infection should be ruled out in the presence of polymicrobial enterobacteriaceae bacteraemia.