To the Parents of the Boy We Lost.

Recently, we took care of a teenage boy in our PICU who had been struggling with a lifelong chronic illness and accompanied him and his parents during the last few days of his life. We had the privilege of getting to know him and his parents quite well during the last few years, as he required hospitalization several times during this time. We saw how extremely dedicated his parents had been to him and his care, how they left no stone unturned and sacrificed everything they could to help him have the best quality of life possible. I happened to not be on service, nor know about the moment when he passed away. I also missed when his funeral took place. I wanted to reach out to his parents, to express how much his life and their love for him had meant to me, too, and to find some closure. So I wrote this short poem for them, and for all the other parents, who have to go through the most difficult time a parent can ever go through-as a pediatric intensivist, a Mom who has never been where they had to go, a fellow human being, and a grieving friend.

Seizure as Presenting Symptom of Multisystem Inflammatory Syndrome in Children.

We describe the case of a 13-year-old girl who presented with a new-onset seizure and fever and subsequently developed severe cardiac dysfunction, coronary artery dilation, and shock due to the surprising diagnosis of multisystem inflammatory syndrome in children (MIS-C). Although the clinical entity we now call MIS-C was first mentioned in the medical literature in April 2020, the full picture of this disease process is still evolving. Neurologic involvement has been described in cases with MIS-C; however, seizures are not a typical presenting symptom. Additionally, because children infected with SARS-CoV-2 are often asymptomatic, a documented preceding COVID-19 infection might not be available to raise suspicion of MIS-C early on. Febrile seizures, meningitis, and encephalitis are childhood illnesses that pediatricians are generally familiar with, but associating these clinical pictures with MIS-C is uncommon. Given the possibility of rapid clinical cardiogenic decline, as seen in our patient, a prompt diagnosis and appropriate monitoring and treatment are of utmost importance. This case report aims to raise awareness that new-onset seizures with fevers can be early or the first presenting symptoms in children with MIS-C, and further workup and close monitoring may be required.

Pediatric ECMO Candidates at Non-ECMO Centers: Transfer, Cannulate, or Treat Locally?

Pediatric inpatient and intensive care specialists working outside of tertiary medical centers confront difficult clinical scenarios related to how best to care for extremely ill children who may or may not benefit from advanced medical technology, and these clinicians are often faced with limited local availability. Extracorporeal membrane oxygenation (ECMO) is a technology that is only available at a subset of tertiary care centers, and the decision to risk the transfer of a child for the potential benefit of ECMO is challenging. This article is aimed at addressing the main factors and ethical principles related to this decision-making: (1) whether ECMO is the standard of care, (2) clinical decision analysis of the risks and benefits, (3) informed consent and education of the parents and/or guardians, and (4) institutional leadership decision-making. A decisional framework is proposed that incorporates a thoughtful shared decision-making algorithm.

Case Report of a Child after Hematopoietic Cell Transplantation with Acute Aspergillus Tracheobronchitis as a Cause for Respiratory Failure.

Rapid respiratory failure due to invasive mycosis of the airways is an uncommon presentation of Aspergillus infection, even in immunocompromised patients, and very few pediatric cases have been reported. Patients with Aspergillus tracheobronchitis present with nonspecific symptoms, and radiologic studies are often noninformative, leading to a delay in diagnosis. Prompt initiation of adequate antifungal therapies is of utmost importance to improve outcome. We report the case of a 9-year-old girl with chronic myelogenous leukemia who developed respiratory distress 41 days after hematopoietic cell transplantation and rapidly deteriorated despite multiple interventions and treatment modalities.

Impact of Microbiota on Resistance to Ocular Pseudomonas aeruginosa-Induced Keratitis.

The existence of the ocular microbiota has been reported but functional analyses to evaluate its significance in regulating ocular immunity are currently lacking. We compared the relative contribution of eye and gut commensals in regulating the ocular susceptibility to Pseudomonas aeruginosa-induced keratitis. We find that in health, the presence of microbiota strengthened the ocular innate immune barrier by significantly increasing the concentrations of immune effectors in the tear film, including secretory IgA and complement proteins. Consistent with this view, Swiss Webster (SW) mice that are typically resistant to P. aeruginosa-induced keratitis become susceptible due to the lack of microbiota. This was exemplified by increased corneal bacterial burden and elevated pathology of the germ free (GF) mice when compared to the conventionally maintained SW mice. The protective immunity was found to be dependent on both eye and gut microbiota with the eye microbiota having a moderate, but significant impact on the resistance to infection. These events were IL-1ß-dependent as corneal IL-1ß levels were decreased in the infected GF and antibiotic-treated mice when compared to the SPF controls, and neutralization of IL-1ß increased the ocular bacterial burden in the SPF mice. Monocolonizing GF mice with Coagulase Negative Staphylococcus sp. isolated from the conjunctival swabs was sufficient to restore resistance to infection. Cumulatively, these data underline a previously unappreciated role for microbiota in regulating susceptibility to ocular keratitis. We predict that these results will have significant implications for contact lens wearers, where alterations in the ocular commensal communities may render the ocular surface vulnerable to infections.

Intestinal Microbiota of Mice Influences Resistance to Staphylococcus aureus Pneumonia.

Th17 immunity in the gastrointestinal tract is regulated by the intestinal microbiota composition, particularly the presence of segmented filamentous bacteria (sfb), but the role of the intestinal microbiota in pulmonary host defense is not well explored. We tested whether altering the gut microbiota by acquiring sfb influences the susceptibility to staphylococcal pneumonia via induction of type 17 immunity. Groups of C57BL/6 mice which differed in their intestinal colonization with sfb were challenged with methicillin-resistant Staphylococcus aureus in an acute lung infection model. Bacterial burdens, bronchoalveolar lavage fluid (BALF) cell counts, cell types, and cytokine levels were compared between mice from different vendors, mice from both vendors after cohousing, mice given sfb orally prior to infection, and mice with and without exogenous interleukin-22 (IL-22) or anti-IL-22 antibodies. Mice lacking sfb developed more severe S. aureus pneumonia than mice colonized with sfb, as indicated by higher bacterial burdens in the lungs, lung inflammation, and mortality. This difference was reduced when sfb-negative mice acquired sfb in their gut microbiota through cohousing with sfb-positive mice or when given sfb orally. Levels of type 17 immune effectors in the lung were higher after infection in sfb-positive mice and increased in sfb-negative mice after acquisition of sfb, as demonstrated by higher levels of IL-22 and larger numbers of IL-22(+) TCRß(+) cells and neutrophils in BALF. Exogenous IL-22 protected mice from S. aureus pneumonia. The murine gut microbiota, particularly the presence of sfb, promotes pulmonary type 17 immunity and resistance to S. aureus pneumonia, and IL-22 protects against severe pulmonary staphylococcal infection.

Group A streptococcal bacteremia without a source is associated with less severe disease in children.

We analyzed characteristics of 86 Group A streptococcal bacteremia cases at Boston Children's Hospital from 1992 to 2012. Twenty-three percent of children had severe disease, using intensive care unit admission (18), disability (7) or death (2) as indicators. Children with bacteremia without a source (30% of cases) were less likely to have severe disease than children with focal infections in adjusted models.

Postoperative adrenal crisis in an adolescent with Loeys-Dietz syndrome and undiagnosed adrenoleukodystrophy.

PURPOSE: We present an unusual case involving an adolescent who experienced cardiovascular collapse postoperatively. He had more than one rare life-threatening genetic disorder, and herein we review the anesthetic management of a patient with Loeys-Dietz syndrome (LDS) and adrenoleukodystrophy. CLINICAL FEATURES: A 12-yr-old male (41.5 kg, American Society of Anesthesiologists' physical status III) with LDS, a connective tissue disorder, underwent posterior spinal fusion for spondylolisthesis. This patient demonstrated many signs of LDS: bifid uvula, retrognathia, dilated aortic root, aortic arch aneurysm, inguinal hernias and vertebral subluxations, and multiple areas of tortuous intracranial vessels. A fibreoptic intubation was performed and a balanced anesthetic was administered, consisting of opioids and a low-dose volatile agent. The patient was stable throughout surgery, and at the end of the procedure, his trachea was extubated and he was admitted to the intensive care unit. During the postoperative period, the patient became acutely hemodynamically unstable. Initially, the intensive care team considered the differential diagnoses of postoperative hemorrhage, postoperative sepsis, and cardiac failure secondary to aortic dissection. Supportive care was instituted, and these diagnoses were systematically ruled out. The differential diagnosis was expanded, and the patient was treated with corticosteroids after baseline cortisol levels were drawn. Later he was found to have primary adrenal failure, and it was determined by biochemical and genetic blood analysis that he also had adrenoleukodystrophy. CONCLUSIONS: Although Occam's razor states that physicians should exercise diagnostic parsimony when treating patients, it is possible for a patient to have two or more life-threatening unrelated genetic disorders. Consequently, diagnosticians must always develop and test new hypotheses when treating patients.