[SEVERE HYPERNATREMIA IN A TEENAGE GIRL SUFFERING FROM ANOREXIA NERVOSA].

INTRODUCTION: Hypernatremic dehydration is an uncommon condition, whose main treatment is fluid administration. In cases of extreme hypernatremia, that treatment paradoxically poses a real danger to the patient's morbidity and mortality. In very rare cases, restrictive type anorexia nervosa causes extreme dehydration that can result in elevated sodium levels. In this case study we present a teenage girl who suffers from restrictive anorexia nervosa and was admitted to the pediatric intensive care unit (PICU) with an acute confusional state, with extremely severe hypernatremia up to 203 mEq/L, combined with hyperglycemia up to 700 mg/dL. Under hypotonic electrolyte solution treatment, a gradual sodium level decrease of 8 mEq/L per day was achieved with complete normalization of sodium without any neurological damage. Moreover, glucose levels were normalized rapidly and spontaneously without additional treatment with insulin.

[HAIR STRAIGHTENING - NOT STRAIGHTFORWARD].

INTRODUCTION: The rising popularity of hair straightening in younger ages has become a medical issue, since glycolic acid and formaldehyde may be present, even in "formaldehyde-free" labeled products. Formaldehyde - a colorless material, evaporates during the hair straightening procedure, inhaled and absorbed into the blood stream causing oxidative stress and cytotoxic damage to the proximal tubule cells leading to acute kidney injury (AKI). Glycolic acid is processed to glyoxylate and eventually to oxalate, whose deposition may also cause AKI. We present three cases of female teenagers with AKI, recently after a hair straightening procedures. All patients had features of tubular damage and kidney biopsies in the first 2 cases showed acute tubular necrosis (ATN), one with oxalate deposition and the other with unidentified depositions. Two cases required acute dialysis, and shortly after commencing it, kidney function rapidly improved. We believe that in the presented cases, the prompt dialysis cleared a nephrotoxic ingredient, allowing improvement of renal function, therefore preventing long-standing and maybe, even permanent damage. This case presentation highlights the danger of hair straightening products in pediatric populations aiming to increase its index of suspicion among adult and pediatric nephrologists.

HLH Syndrome in a Community Hospital: The Challenge of an Early Diagnosis.

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory cytokine storm. It can be secondary to infections, malignancies, autoimmune diseases, or the manifestation of genetic disorders, including primary immune deficiency. HLH requires a high index of suspicion and is challenging for community hospitals. Methods: Medical records of children with HLH admitted to the Meir Medical Center in Israel between 2014 and 2017 were reviewed. Results: Nine children met ≥5/8 HLH-2004 criteria. The median age was 1.1 year, and 78% of the patients were aged <2 years. All patients had prolonged fever, cytopenia, and elevated soluble interleukin-2 receptor, and 89% had elevated ferritin levels. Of three children who underwent gene panel evaluation, one had heterozygote genetic variants of UNC13D and STXBP2 of unclear significance, whereas the other two had no variants. Infection was identified in 8 of 9 patients: adenovirus, HHV6, EBV, and Streptococcus Group A. Only 2 patients received HLH-2004 therapy (dexamethasone, etoposide, cyclosporin-A) and the others received dexamethasone and/or intravenous gamma globulins (IVIG), with rapid resolution of fever (median 2 days). One patient (11%) died of Pseudomonas septicemia and multiorgan failure. At a median follow-up of 7 years (range 2.6-8.1 years), all others (8/9) are long-term survivors with no recurrent HLH, but 2 patients developed adenovirus-related bronchiolitis obliterans. Conclusion: Children presenting with prolonged fever and abnormal blood counts should be evaluated with ferritin, triglycerides, and fibrinogen levels which indicate possible HLH. Early intervention with corticosteroids and/or IVIG may prevent deterioration, spare them from chemotherapy and provide time for more elaborate testing to identify true HLH. Unfortunately, mortality remains a significant risk for these children.

In the emergency department, children with common infections may have a severe complication called Hemophagocytic Lymphohistiocytosis or HLH. HLH can be life threatening if not rapidly recognized. HLH is rare and challenging for doctors in community hospitals. We describe nine patients who presented to a community hospital who were later diagnosed with HLH, posing a dilemma for physicians. Most (78%) were less than 2 years, all had prolonged fever, abnormal blood counts, elevated marker of HLH called soluble interleukin­2 receptor and 8 of 9 had elevated ferritin, which can be a marker of HLH. HLH could be genetic therefore three children had genetic studies, with one having minor abnormalities, but the contribution to HLH is unclear. Infection as cause for HLH was identified in 8 of 9 patients. Chemotherapy that is used for severe HLH was required for 2 patients and the others received steroids and/or intravenous gamma globulin with rapid improvement. One patient who received chemotherapy and had suppressed immunity died of a severe bacterial infection. Others (8 of 9) are long-term survivors with no evidence of recurrent HLH. Two patients developed a pulmonary complication from adenovirus known as bronchiolitis obliterans. We conclude that children presenting with prolonged fever and abnormal blood counts should be evaluated with ferritin and other markers of possible HLH. Early intervention may prevent deterioration, may spare them from chemotherapy, and allow further assessment of true HLH. However, the death of one (11%), demonstrates the significant risks to these children.

Transient Cardiac Injury in Adolescents Receiving the BNT162b2 mRNA COVID-19 Vaccine.

BACKGROUND: Vaccines are paramount in the effort to end the coronavirus disease 2019 global epidemic. BNT162b2 is approved for the vaccination of adolescents over 16 years of age. Systemic adverse events were scarce though the pretested cohort of this age group was relatively small. The aim of the current study is to raise awareness for potential adverse reactions. METHODS: This is a case series of patients diagnosed with perimyocarditis following vaccination. Patients were compiled from 3 pediatric medical centers in Israel through a network of pediatricians and data regarding those cases was collected. In addition, incidence of perimyocarditis during the vaccination period was compared with previous years. RESULTS: All patients were males 16-18 years old, of Jewish descent, who presented with chest pain that began 1-3 days following vaccination (mean, 2.1 days). In 6 of the 7 patients, symptoms began following the 2nd dose and in 1 patient following the 1st dose. All cases were mild and none required cardiovascular or respiratory support. The incidence of perimyocarditis during the vaccination period was elevated in comparison to previous years. CONCLUSIONS: This case series describes a time association between coronavirus disease 2019 vaccine and perimyocarditis in adolescents. All cases were mild, although only long-term follow-up can reveal the true impact of this cardiac injury. While it seems that the incidence of perimyocarditis during the vaccination campaign period is increased, a more comprehensive data collection on a wider scale should be done. We hope this report will serve as a reminder to report events and allow for analysis of potential adverse reactions.

Variability of Care of Infants With Severe Respiratory Syncytial Virus Bronchiolitis: A Multicenter Study.

BACKGROUND: Viral bronchiolitis caused by respiratory syncytial virus (RSV) is a common childhood disease accounting for many hospitalizations worldwide. Some infants may clinically deteriorate, requiring admission to an intensive care unit. We aimed to describe diagnostic and therapeutic measures of bronchiolitis in Israeli pediatric intensive care units (PICUs) and evaluate intercenter variability of care. METHODS: Medical records of all RSV-infected infants admitted to 5 Israeli PICUs over 4 RSV seasons were retrospectively reviewed. RESULTS: Data on 276 infants with RSV-positive bronchiolitis, admitted to the participating PICUs were analyzed. Most of the infants were males with a mean admission age of 4.7 months. Approximately half of the infants had pre-existing conditions such as prematurity, cardiac disease or chronic lung disease. Respiratory distress was the most common symptom at presentation followed by hypoxemia and fever. There was significant variation in the methods used for RSV diagnosis, medical management and respiratory support of the infants. Furthermore, utilization of inhalational therapy and transfusion of blood products differed significantly between the centers. Although a bacterial pathogen was isolated in only 13.4% of the infants, 82.6% of the cohort was treated with antibiotics. CONCLUSIONS: Significant variation was found between the different PICUs regarding RSV bronchiolitis diagnosis, medical management and respiratory support, which may not be accounted for by the differences in baseline and clinical characteristics of the infants. Some of these differences may be explained by uneven resource allocations. This diversity and the documented routine use of medications with weak evidence of efficacy calls for national guidelines for bronchiolitis management.

Combined immunodeficiency in a patient with mosaic monosomy 21.

Monosomy 21 is an extremely rare genetic disorder presenting with a wide array of symptoms. Recurrent infections, some life threatening, have been reported in several monosomy 21 patients and attributed to an, as of yet, undefined immunodeficiency. Here we report on a 3-year-old boy with mosaic monosomy 21 who presented with clinical and laboratory evidence of immunodeficiency. Despite suffering from infections highly suggestive of a cell-mediated immune defect, the patient's T cells displayed normal counts, subsets and proliferation capability. T cell receptor repertoire was diverse, and de novo T cell production was intact. Consistent with earlier case reports, our patient displayed mildly low B cell counts with hypogammaglobulinemia. B cell subsets demonstrated mainly naïve and marginal zone B cells that have not undergone class switch. Subsequently, IgG, IgA and IgE levels were near absent, whereas IgM level was normal. De novo B cell production and B cell receptor diversity were normal. Together, these results are indicative of a defect in immunoglobulin class switching as the principal cause of immunodeficiency in monosomy 21. A better understanding of the immunodeficiency in this syndrome will enable targeted treatment and prevention of infections in order to prevent morbidity and mortality in these patients.