The role of anion gap normalization time in the management of pediatric diabetic ketoacidosis.

Introduction: Our aims were to determine whether anion gap normalization time (AGNT) correlates with risk factors related to the severity of diabetic ketoacidosis (DKA) in children, and to characterize AGNT as a criterion for DKA resolution in children admitted with moderate or severe disease. Methods: A ten-year retrospective cohort study of children admitted to the intensive care unit with DKA. We used a survival analysis approach to determine changes in serum glucose, bicarbonate, pH, and anion gap following admission. Using multivariate analysis, we examined associations between patients' demographic and laboratory characteristics with delayed normalization of the anion gap. Results: A total of 95 patients were analyzed. The median AGNT was 8 h. Delayed AGNT (>8 h) correlated with pH < 7.1 and serum glucose >500 mg/dL. In multivariate analysis, glucose >500 mg/dL was associated with an increased risk for delayed AGNT, by 3.41 fold. Each 25 mg/dL elevation in glucose was associated with a 10% increment in risk for delayed AGNT. Median AGNT preceded median PICU discharge by 15 h (8 vs. 23 h). Discussion: AGNT represents a return to normal glucose-based physiology and an improvement in dehydration. The correlation observed between delayed AGNT and markers of DKA severity supports the usefulness of AGNT for assessing DKA recovery.

Demonstration of mucus simulant clearance in a Bench-Model using acoustic Field-Integrated Intrapulmonary Percussive ventilation.

Intrapulmonary Percussive Ventilation (IPV) is a high-frequency airway clearance technique used to help in mucus transport for mechanically ventilated and unventilated patients. Despite the many years of usage, this technique does not provide clear evidence of its intended efficacy. This is mainly attributable to the lack of in vitro observations that show "mucokinesis" towards the direction of the mouth. In the current manuscript, we demonstrate and subsequently propose a mechanism that details the movement of a mucus simulant in the proximal (towards the mouthpiece) direction. Towards this end, a novel method utilizing a high-frequency acoustic field in addition to the conventional air pulsations brought forth by traditional IPV is proposed. Under these conditions, at certain parameter settings, it is shown that the simulant is broken down into much smaller parts and subsequently pushed in the upstream direction gradually over a period of half-hour.

Transcript-Based Diagnosis and Expanded Phenotype of an Intronic Mutation in TPM3 Myopathy.

INTRODUCTION: Congenital myopathies are a broad group of inborn muscle disorders caused by a multitude of genetic factors, often characterized by muscle atrophy and hypotonia. METHODS: Clinical studies, imaging, histology, whole-exome sequencing (WES) and muscle tissue RNA studies. RESULTS: We describe a severe congenital myopathy manifesting at birth with bilateral clubfeet, delayed motor development and hypotonia, becoming evident by 4 months of age. At 3 years of age, the patient had tongue fasciculations, was bedridden, and was chronically ventilated via tracheostomy. Imaging studies demonstrated severe muscle atrophy and, surprisingly, cerebral atrophy; electromyography demonstrated a myasthenic pattern and histological evaluation did not facilitate a definitive diagnosis. Trio WES did not identify a causative variant, except for a non-canonical intronic TPM3 c.118-12G>A variant of uncertain significance. Transcript analysis of muscle tissue from the patient proved the pathogenicity of this homozygous variant, with a 97% reduction in the muscle-specific TPM3.12 transcript. DISCUSSION: This study broadens the phenotypic spectrum of recessive TPM3 disease, highlighting tongue fasciculations and bilateral clubfoot, as well as possibly-related cerebral atrophy. It also shows the importance of a broad approach to genetic analysis and the utility of RNA-based studies, demonstrating efficacy of early genome and transcriptome queries in facilitating rapid and cost-effective diagnosis of congenital myopathies.

Bogota Bag Temporary Abdominal Closure Surgical Technique in Children: A 15-Year Single Center Experience.

BACKGROUND: Temporary abdominal closure (TAC) surgical technique relates to a procedure in which the post-surgical abdominal wall remains open in certain indications. The Bogota bag (BB) technique is a tension-free TAC method that covers the abdominal contents with a sterilized fluid bag. There are very few reports of pediatric patients treated with this technique. OBJECTIVES: To describe our institution's 15 years of experience using the BB technique on pediatric patients. METHODS: A retrospective cohort study describing our experience treating patients with BB was conducted. The medical files of 17 pediatric patients aged 0-18 years were reviewed. RESULTS: Between January 2000 and December 2014, 17 patients were treated with BB at our medical center (6 females, median age 12 years). Indications for BB were a need for a surgical site re-exploration, mechanical inability for primary abdominal closure, and high risk for ACS development. Median BB duration was 5 days and median bag replacement was 2 days. Median ICU length of stay (LOS) was 10 days and hospital LOS was 27 days. The ICU admission and BB procedure was tolerated well by 6 patients who were discharged home without complications. Of the remaining 11 patients, 6 patients died during the admission (35%) and the others presented with major complications not related to the BB but to the patient's primary disease. CONCLUSIONS: This report represents the largest series of children treated with BB. The technique is simple to perform, inexpensive, and has very few complications.

Gastric Drainage in the Treatment of Near-Fatal Food-Induced Anaphylaxis.

Anaphylaxis should be treated with early administration of intramuscular Adrenalin; however, fatalities may still occur even with this therapy. We report a patient with near-fatal anaphylaxis due to milk whose symptoms persisted and were resistant to any therapeutic attempt; however, the patient had a prompt resolution of the anaphylaxis shortly after a nasogastric tube placement with gastric drainage, suggesting that this procedure ended the ongoing absorption of additional allergen from the gastrointestinal tract. We suggest that nasogastric drainage of gastric contents should be considered as part of the therapy in severe food-induced anaphylaxis.

Treatment Outcomes of Stenotrophomonas maltophilia Bacteremia in Critically Ill Children: A Multicenter Experience.

OBJECTIVES: Stenotrophomonas maltophilia is a gram-negative opportunistic bacterium that may cause a myriad of clinical diseases in immunocompromised individuals. We aimed to describe the clinical characteristics, risk factors, mortality, and treatment of S. maltophilia bacteremia in critically ill children, a topic on which data are sparse. DESIGN: A multicenter observational retrospective study in which medical charts of critically ill children with S. maltophilia bacteremia were reviewed between 2012 and 2017. SETTING: Data were collected from each of the four largest PICUs nationwide, allocated in tertiary medical centers to which children with complex conditions are referred regularly. PATIENTS: A total of 68 suitable cases of S. maltophilia bacteremia were retrieved and reviewed. MEASUREMENTS AND MAIN RESULTS: The total occurrence rate of S. maltophilia isolation had increased significantly during the study period (r = 0.65; p = 0.02). The crude mortality was 42%, and the attributed mortality was 18%. Significant risk factors for mortality were a longer length of hospital stay prior to infection (33 d in nonsurvivors vs 28 in survivors; p = 0.03), a nosocomial source of infection (p = 0.02), presentation with septic shock (p < 0.001), and treatment with chemotherapy (p = 0.007) or carbapenem antibiotics (p = 0.05) prior to culture retrieval. On multivariate analysis, septic shock (odds ratio, 14.6; 95% CI, 1.45-147.05; p = 0.023) and being treated with chemotherapy prior to infection (odds ratio, 5.2; 95% CI, 1.59-17.19; p = 0.006)] were associated with mortality. The combination of ciprofloxacin, trimethoprim-sulfamethoxazole, and minocycline resulted in the longest survival time (p < 0.01). CONCLUSIONS: The significant attributed mortality associated with S. maltophilia bacteremia in critically ill children calls for an aggressive therapeutic approach. The findings of this investigation favor a combination of trimethoprim-sulfamethoxazole, ciprofloxacin, and minocycline.

Purpuric rash and fever among hospitalized children aged 0-18 years: Comparison between clinical, laboratory, therapeutic and outcome features of patients with bacterial versus viral etiology.

BACKGROUND: The evaluation of children with purpuric rash and fever (PRF) is controversial. Although many of them have viral infections, on occasion such patients may be infected with Neisseria meningitidis. We described all children aged 0-18 years with PRF in southern Israel during the period 2005 ̶ 2016 and compared their microbiologic, laboratory, clinical and outcome characteristics in relation to various etiologies of this syndrome. METHODS: Data were summarized from electronic patient and microbiology files. Viral diagnoses were made by serology and/or PCR. RESULTS: Sixty-nine children with PRF were admitted; 30 (43.48%), 9 (13.04%) and 30 (43.48%) had a syndrome of bacterial, viral or non-established etiology, respectively. N. meningitidis infection was diagnosed in 16/69 (23.19%) patients and in 16/30 (53.33%) patients with bacterial etiology; 14/30 (46.67%) patients suffered from a non-invasive bacterial disease (9 with Rickettsial disease). Adenovirus and Influenza B (3 and 2 cases, respectively) represented the most frequent etiologic agents among patients with viral etiology. More patients with PRF of bacterial etiology were older, of Bedouin ethnicity, looked ill on admission, had higher rates of meningitis and were treated more frequently with antibiotics compared with patients with non-bacterial PRF. Fatality rates among patients with bacterial, viral and non-established etiology were 5/30 (16.7%), 0% and 2/39 (5.1%). CONCLUSIONS: Although PFR was uncommon, high rates of meningococcal infections were recorded in children with PRF, which was associated with high fatality rates. Rickettsial infections were frequent, emphasizing the need for a high index of suspicion for this disease in endemic geographic areas.

Group A streptococcal brain abscess in children: two case reports and a review of the literature.

Brain abscesses caused by group A Streptococcus (GAS) are infrequently encountered in children. We present two cases of brain abscess (one cerebellar and one located in the temporal lobe) due to GAS infection occurring in close temporal proximity in previously healthy young children living in different geographic areas of southern Israel. The relevant literature since 2000, in the context of recent epidemiological data reporting an increase in the incidence of invasive GAS infections, is reviewed.

Healthcare workers mobile phone usage: A potential risk for viral contamination. Surveillance pilot study.

BACKGROUND: Mobile phones are commonly used by healthcare workers (HCW) in the working environment, as they allow instant communication and endless resource utilisation. Studies suggest that mobile phones have been implicated as reservoirs of bacterial pathogens, with the potential to cause nosocomial infection. This study aimed to investigate the presence of Respiratory Syncytial Virus, Adenovirus and Influenza Virus on HCWs mobile phones and to identify risk factors implied by HCWs practice of mobile phones in a clinical paediatric environment. METHODS: Fifty HCWs' mobile phones were swabbed over both sides of the mobile phone, for testing of viral contamination during 8 days in January 2015. During the same period, a questionnaire investigating usage of mobile phones was given to 101 HCWs. RESULTS: Ten per cent of sampled phones were contaminated with viral pathogens tested for. A total of 91% of sampled individuals by questionnaire used their mobile phone within the workplace, where 37% used their phone at least every hour. Eighty-nine (88%) responders were aware that mobile phones could be a source of contamination, yet only 13 (13%) disinfect their cell phone regularly. CONCLUSION: Mobile phones in clinical practice may be contaminated with viral pathogenic viruses. HCWs use their mobile phone regularly while working and, although the majority are aware of contamination, they do not disinfect their phones.

Intravenous maintenance fluids revisited.

Intravenous maintenance fluid therapy aims to replace daily urinary and insensible losses for ill children in whom adequate enteric administration of fluids is contraindicated or infeasible. The traditional determination of fluid volumes and composition dates back to Holliday and Segar's seminal article from 1957, which describes the relationship between weight, energy expenditure, and physiologic losses in healthy children. Combined with estimates of daily electrolyte requirements, this information supports the use of the hypotonic maintenance fluids that were widely used in pediatric medicine. However, using hypotonic intravenous fluids in a contemporary hospitalized patient who may have complex physiologic derangements, less caloric expenditure, decreased urinary output, and elevated antidiuretic hormone levels is often not optimal; evidence over the last 2 decades shows that it may lead to an increased incidence of hyponatremia. In this review, we present the evidence for using isotonic rather than hypotonic fluids as intravenous maintenance fluid.

[Severe and uncommon complications of anticholinesterase intoxication in children].

BACKGROUND: The Northern region of the Negev desert is an endemic area of organophosphate and carbamate intoxications in Bedouin children. Most victims are intoxicated by drinking the poisonous material kept by the parents in soft drink bottles. Signs and symptoms of intoxication are commonly known and generally include various effects on the central nervous system, usually a decreased level of consciousness in children, cholinergic muscarinic (sweating, rhinorrhea, miosis, vomiting) and nicotinic (weakness) effects. Specific therapy includes Atropine Sulphate and Oximes. PURPOSE AND RESULTS: To describe the course of disease of four (out of 47) children admitted to the Division of Pediatrics with organophosphate or carbamate poisoning during a two-year period. The four children 3-17 years of age ingested the poisonous material: organophosphate chlorpyrifos (2 children); carbamate methomyl (one child) and an unidentified compound in another child. Three of the four patients ingested the poison in a suicide attempt. All 4 children suffered from severe and uncommon complications, including severe respiratory failure from different etiologies. In addition, two of the four suffered from a neurological deficit causing prolonged disability. Three had renal failure necessitating hemofiltration in one case. One child had severe hemodynamic failure and arrhythmias necessitating, among other therapy, the insertion of a temporary pace maker. Two children had (laboratory) pancreatitis. One of the children with severe respiratory failure died after 38 days of extracorporeal membrane oxygenation. CONCLUSIONS: Intoxications by anticholinesterase compounds are not uncommon among Bedouin children in the Negev. This public health threat should be prevented and completely eradicated by the health authorities. Severe intoxication, especially in cases arising after suicide attempts, wherein the amount of the poisonous material is large, may be complicated by life threatening, multi-organ failure during and after the initial phase of poisoning and may progress into prolonged disability and death.

Organophosphate poisoning-induced acute renal failure.

BACKGROUND: Acute kidney injury as a direct complication of organophosphate poisoning has rarely been described and its etiology is unclear. CASE: A 17-year-old adolescent girl was admitted to the pediatric intensive care unit after a suicidal attempt with chlorpyrifos, an organophosphate insecticide compound. The patient developed acute kidney injury followed by renal failure, necessitating renal replacement therapy. She was treated with continuous venovenous hemofiltration with full resolution of her renal function. CONCLUSIONS: Organophosphate poisoning can lead to renal failure, which, with proper treatment, may be reversible but, if left unattended, might aggravate the clinical course of the disease. Physicians should be aware of this rare complication.

Lethal brain edema, shock, and coagulopathy after scorpion envenomation.

We report the case of a 2-year-old Bedouin boy in whom developed severe and unusual complications after being stung, most probably, by the yellow scorpion Leiurus quinquestriatus hebraeus. Five hours after arrival to the emergency department, the boy had multisystem organ failure involving the central nervous system (seizure activity followed by coma with dilated, nonreactive pupils, and severe brain edema), shock (noncardiogenic), disseminated intravascular coagulation, renal failure, hepatic failure, and watery diarrhea, causing his death. In view of the relevant literature, we discuss the pathophysiologic events ultimately leading to his death.

Non invasive positive pressure ventilation in infants with respiratory failure.

OBJECTIVE: To determine whether non-invasive positive pressure ventilation (NIPPV) delivered via nasal prongs can alleviate the need for tracheal intubation and invasive ventilation in infants admitted to the pediatric intensive care unit (PICU) with impending respiratory failure, and to find predictive factors for success or failure with this mode. DESIGN: A single center retrospective cohort study. SETTING: PICU in a university affiliated hospital. PATIENTS: During the 14 months of the study period we recovered 22 NIPPV episodes in 19 infants (median age 65 days) with impending respiratory failure. The patient's respiratory failure etiologies were bronchiolitis (n = 13), pertussis (n = 3), and other respiratory conditions (n = 6). MEASUREMENTS AND RESULTS: In 64% of the cases, intubation was prevented and the patients were weaned off to spontaneous breathing (Responders group). 36% failed NIPPV and had to be intubated and invasively ventilated (Non-responders group). Apneic episodes were the indication for ventilation in 11 patients (50%) with a 73% success rate in preventing invasive ventilation. Hypoxemic respiratory failure was present in nine patients (41%) and the rate of success was 44%. Two patients with post extubation respiratory distress, improved with NIPPV. Responders and non-responders did not differ with regard to demographics or disease severity prior to initiation of NIPPV. After initiating NIPPV respiratory rate and the need for sedation were lower in the NIPPV responders. CONCLUSIONS: In a set group of patient population such as infants with apnea secondary to bronchiolitis NIPPV may be successful to reduce the need for invasive ventilation. Our study failed to detect any physiological or clinical markers which could distinguish between so called "responders" and "non-responders" before initiating NIPPV.

Dose-related effects of hyperoxia on the lung inflammatory response in septic rats.

We evaluated the effects of hyperoxia on pulmonary inflammatory changes in sepsis induced by cecal ligation and puncture (CLP) in rats. Seven groups were studied: sham-operated rats breathing air for 20 or 48 h; CLP breathing air for 20 or 48 h; and CLP + 100% oxygen for 20 h, or 70% oxygen for 48 h, or 100% oxygen intermittently (6 h/d) for 48 h. Video microscopy was used to monitor lung macromolecular leak, microvascular flow velocity, and shear rates, and lung morphometry was used for leukocyte infiltration and solid tissue area. Cell counts, tumor necrosis factor α, and nitrites were determined in peripheral blood and lung lavage fluid. Expression of adhesion molecules in blood leukocytes was evaluated by flow cytometry. Cecal ligation and puncture induced inflammation manifested in leukopenia, left shift, thrombocytopenia, increased expression of L selectin and CD11, increased serum and lavage fluid tumor necrosis factor α and leukocytes, and increased lung tissue area, macromolecular leak, and sequestration of leukocytes. Inhalation of 100% oxygen for 20 h increased nitrites (P < 0.01) and decreased leukocyte count in lavage fluid (P < 0.05) and attenuated lung macromolecular leak and changes in solid tissue area (P < 0.01). Inhalation of 70% oxygen (48 h) attenuated expression of adhesion molecules (P < 0.001) but failed to attenuate markers of lung inflammation. In contrast, intermittent 100% oxygen exerted favorable effects on markers of inflammation, attenuated leukocyte expression of L selectin and CD11 (P < 0.01), decreased pulmonary sequestration of leukocytes (P < 0.001), and ameliorated changes in macromolecular leak (P < 0.01) and lung solid tissue area (P < 0.05). Our data support the beneficial effects of safe subtoxic regimens of normobaric hyperoxia on the systemic and pulmonary inflammatory response following CLP.