Safety of Non-Operating Room Anesthesia With Propofol Sedation in Three Pediatric Patients With Central Sleep Apnea.

Children with central sleep apnea may require sedation for procedures, including brain imaging as part of the evaluation of apnea. However, the safety of deep sedation without a protected airway is not known in this patient population. In this case series, we present 3 children with central sleep apnea who were sedated with propofol for brain imaging in a non-operating room setting. All 3 did well with no complications; those with a home oxygen requirement were on oxygen during the procedure but none experienced apnea, desaturation, or respiratory distress. While obstructive sleep apnea is a known contraindication to deep sedation with propofol, it may be safe in pediatric patients with central sleep apnea. Deep sedation may be a good option for these patients, thereby avoiding the need for general anesthesia and placement of an advanced airway.

A 15-year-old with chest pain: An unexpected etiology.

A 15-year-old female with no significant past medical history presented to the emergency department with 1 day of substernal and pleuritic chest pain, chills, cough, and hematuria. She also had swelling of the face and ankles that resolved by presentation. She was found to have elevated troponin and brain natriuretic peptide during initial workup. Electrocardiogram was normal, but there were significant pleural effusions on chest x-ray. She was strep positive and had blood pressure up to 150/90, prompting admission for cardiac monitoring and cardiology consultation. Blood pressure decreased down to 125/72 without intervention. She was afebrile with unlabored breathing and normal saturations. She was clear to auscultation bilaterally, with no abdominal distension or hepatosplenomegaly, and edema was not evident on exam. There was mild erythema to the bilateral tonsillar pillars. Initial considerations included viral myocarditis, pericarditis, and atypical nephritic syndrome. Workup revealed elevated antistreptolysin antibodies, low C3 complement, negative antineutrophil cytoplasmic antibodies, and negative flu testing. Renal sonography was unremarkable. Cardiology recommended echocardiography, which confirmed pleural effusions but revealed no cardiac abnormalities. Urinalysis revealed hematuria and mild proteinuria. Diagnosis was found to be post-streptococcal glomerulonephritis complicated by fluid overload and left ventricular strain secondary to hypertensive emergency. Post-streptococcal glomerulonephritis is the most common cause of acute glomerulonephritis in children. The mechanism of disease is a proliferation and inflammation of the renal glomeruli secondary to immunologic injury, with deposition of immune complexes, neutrophils, macrophages, and C3 after complement activation. This leads to hematuria, proteinuria, and fluid overload. Edema is present in 65%-90% of patients, progressing to pulmonary involvement in severe cases. Cardiac dysfunction secondary to fluid overload is a potentially fatal outcome in the acute setting. Physicians should consider post-streptococcal glomerulonephritis for patients presenting with hypertension, cardiac/pulmonary pathology, or symptoms of acute heart failure in the context of strep infection.

Characteristics and Outcomes of Patients Receiving Sedation for Voiding Cystourethrography.

Background Voiding cystourethrography (VCUG) is used to diagnose vesicoureteral reflux (VUR); however, it is an invasive procedure and can be psychologically distressing. Procedural sedation is occasionally utilized to alleviate anxiety during VCUG, and some patient populations may get referred more readily for sedation than others. Sedative medications may also impact the results of the test due to their effects on smooth muscle. The goals of this study were to compare patient characteristics between those that were referred for procedural sedation and those that were not and to compare VCUG results between sedated and non-sedated patients. Methodology We performed a retrospective cohort study of patients aged 2-18 years undergoing VCUG during a five-year period. Sedated patients were matched with non-sedated patients controlling for referring provider and procedure year. Exclusion criteria included chronic catheterization, same-day surgery, current intensive care admission, and sedation restrictions. A total of 284 patients were included. Demographic information, medical comorbidities, and VCUG results were analyzed. Results There were no significant differences between sedated and non-sedated patients in any demographic variables. Neurologic, developmental, and gastrointestinal comorbidities were more common in sedated patients. On multivariate analysis, having more than one comorbid condition was the only significant predictor of referral for procedural sedation. There were no significant differences in VCUG results between sedated and non-sedated patients. Conclusions Patients with comorbidities were more likely to receive procedural sedation for VCUG. Procedural sedation did not have a significant impact on test results, suggesting its potential utility in relieving pain and anxiety associated with VCUG.

Hypocalcemia and a Positive Metabolic Screen for Severe Combined Immunodeficiency in an 11-Day-Old Male With DiGeorge Syndrome.

22q11 deletion syndrome (22q11DS), also known as DiGeorge syndrome or velocardiofacial syndrome, is the most common human genetic microdeletion. Hypocalcemia secondary to hypoparathyroidism is a common finding in this condition and may present with seizures. We describe a case of an 11-day-old male presenting with hypocalcemic seizures and a positive newborn screen for severe combined immunodeficiency as the primary manifestations of 22q11DS. Given the potential for wide phenotypic variability, clinicians should maintain a high index of suspicion for this syndrome, especially in the neonate presenting with hypocalcemia.

Development of a Pediatric PICC Team Under an Existing Sedation Service: A 5-Year Experience.

OBJECTIVE: To examine our institutional experiences with ultrasound-guided peripherally inserted central catheter (US-PICC) placement by a dedicated US-PICC team under the umbrella of an existing pediatric sedation service. METHODS: Retrospective review of quality data examining 968 US-PICC encounters over a 5-year period from 2012 to 2016. Data for each encounter included line indications, success rate, dwelling time, need for sedation, and incidence of complications including venous thrombosis, infection, and accidental removal. RESULTS: US-PICC lines were successfully placed in 89% of patients with an average age of 5.4 years. Extended antibiotic treatment was the most common indication for US-PICC placement and the mean dwell time was 23 days. Long-term complications were noted in 6.1% of cases, with venous thrombosis and line infection complicating 1.7% and 0.9% of encounters, respectively. CONCLUSION: Results suggest that our endeavor of creating a dedicated US-PICC team under an existing pediatric sedation service is successful with regard to the number of lines placed, success rates, and incidence of complications. This approach may be beneficial to other institutions seeing to maximize resource utilization and streamline patient care.

Contrast-Induced Vomiting in Pediatric Patients Under Propofol Sedation: A Case Series.

Gadolinium-based compounds are frequently used in contrast-enhanced magnetic resonance imaging studies. Rarely, adverse events have been reported with administration of these compounds, of which the most common are nausea and vomiting. Although well established in the adult literature, these adverse effects are less well described in the pediatric population, who often need sedation to complete imaging studies. In this case series, we present 3 children who experienced vomiting shortly after contrast administration while under sedation with propofol, which is known to have antiemetic properties. Although all 3 children recovered without complication, this case series illustrates the serious potential consequences of vomiting while sedated, and providers should be aware of these possible adverse events as pediatric sedation becomes more common outside the operating room.

Acute lymphoblastic leukemia presenting as cholestatic jaundice in a 7-year-old boy.

This is a case of a 7-year-old boy with acute lymphoblastic leukemia presenting with cholestasis and elevated transaminase levels. Acute lymphoblastic leukemia is the most common malignancy in children and can have variable presenting clinical manifestations. However, cholestasis is less commonly encountered in the pediatric population and can be a diagnostic challenge. We present a case of a 7-year-old boy discovered to have elevated transaminase levels while undergoing an evaluation for motor tics, which subsequently progressed to cholestasis and acute liver failure secondary to acute lymphoblastic leukemia. He demonstrated marked improvement after induction therapy and is in clinical remission. Clinicians should be ever mindful of the potentially unique presentations of childhood leukemia.

Bumbling, Stumbling, Fumbling: Weakness, Steppage Gait, and Facial Droop in a 3-Year-Old Male.

A previously healthy, unimmunized, 3-year-old Caucasian boy presented to the emergency department with right-sided facial droop, clumsiness, and intermittent bilateral hip pain. Two weeks ago, he had 24 hours of self-resolving rhinorrhea and fever. Examination was significant for right facial nerve palsy, lower extremity pain, areflexia of his right lower extremity, and diminished reflexes of his left lower extremity. He was admitted for urgent magnetic resonance imaging of the brain. Cerebrospinal fluid (CSF) protein was 85 mg/dL with elevated albumin and immunoglobulin, and CSF white blood cell was 3 cells/mm3. Serum Mycoplasma immunoglobulin (Ig) M and IgG were elevated. There was concern for Guillain-Barré syndrome (GBS). He was started on intravenous IG (IVIG) and was treated for presumed Mycoplasma infection. Weakness and gait disturbances in a child can present the clinician with a diagnostic challenge. Gait disturbance may indicate a neurological lesion anywhere from the central nervous system to the peripheral nerves, neuromuscular junction, or muscle. In the present case, the combination of peripheral facial palsy, presumed neuropathic pain, gait difficulties, and areflexia in the setting of an antecedent respiratory illness were suggestive of GBS. The cornerstone treatments involve hospitalization to facilitate continuous monitoring for serious sequelae, such as acute respiratory failure and cardiac dysrhythmia, followed by immunotherapy with IVIG or plasma exchange. Gait disturbance and weakness in a child is a diagnostic challenge. GBS is the most common cause of acute paralysis in the Western world and should remain high on the clinician's differential diagnosis. However, patients with GBS may also present nonclassically with extremity pain and cranial nerve palsies.

Identification of Novel Allosteric Modulators of Metabotropic Glutamate Receptor Subtype 5 Acting at Site Distinct from 2-Methyl-6-(phenylethynyl)-pyridine Binding.

As part of the G-protein coupled receptor (GPCR) family, metabotropic glutamate (mGlu) receptors play an important role as drug targets of cognitive diseases. Selective allosteric modulators of mGlu subtype 5 (mGlu5) have the potential to alleviate symptoms of numerous central nervous system disorders such as schizophrenia in a more targeted fashion. Multiple mGlu5 positive allosteric modulators (PAMs), such as 1-(3-fluorophenyl)-N-((3-fluorophenyl)-methylideneamino)-methanimine (DFB), 3-cyano-N-(1,3-diphenyl-1H-pyrazol-5-yl)-benzamide (CDPPB), and 4-nitro-N-(1,3-diphenyl-1H-pyrazol-5-yl)-benzamide (VU-29), exert their actions by binding to a defined allosteric site on mGlu5 located in the seven-transmembrane domain (7TM) and shared by mGlu5 negative allosteric modulator (NAM) 2-methyl-6-(phenylethynyl)-pyridine (MPEP). Actions of the PAM N-{4-chloro-2-[(1,3-dioxo-1,3-dihydro-2H-isoindol-2-yl)methyl]phenyl}-2-hydroxybenzamide (CPPHA) are mediated by a distinct allosteric site in the 7TM domain different from the MPEP binding site. Experimental evidence confirms these findings through mutagenesis experiments involving residues F585 (TM1) and A809 (TM7). In an effort to investigate mGlu5 PAM selectivity for this alternative allosteric site distinct from MPEP binding, we employed in silico quantitative structure-activity relationship (QSAR) modeling. Subsequent ligand-based virtual screening prioritized a set of 63 candidate compounds predicted from a library of over 4 million commercially available compounds to bind exclusively to this novel site. Experimental validation verified the biological activity for seven of 63 selected candidates. Further, medicinal chemistry optimizations based on these molecules revealed compound VU6003586 with an experimentally validated potency of 174 nM. Radioligand binding experiments showed only partial inhibition at very high concentrations, most likely indicative of binding at a non-MPEP site. Selective positive allosteric modulators for mGlu5 have the potential for tremendous impact concerning devastating neurological disorders such as schizophrenia and Huntington's disease. These identified and validated novel selective compounds can serve as starting points for more specifically tailored lead and probe molecules and thus help the development of potential therapeutic agents with reduced adverse effects.

Progressive Feeding Intolerance Secondary to a Congenital Spinal Teratoma in a Four-week-old Female.

A poorly feeding neonate presents the clinician with a diagnostic challenge. Feeding difficulties and irritability may be due to sepsis, congenital heart disease, inborn errors of metabolism, non-accidental head trauma, as well as a vast variety of other pathologies. Teratomas are rare pediatric tumors that can occasionally present in the immediate neonatal period and can manifest in the infant's central nervous system (CNS) with non-specific symptoms of poor feeding, lethargy, and somnolence. Operative resection remains the cornerstone of treatment; however, there is no well-defined role for adjuvant chemotherapy or radiation in these treatments. We report a case of a four-week-old female presenting with progressive feeding intolerance secondary to a near holocord thoracic spinal teratoma. Her tumor was surgically resected and she was treated with adjuvant chemotherapy and radiation for 13 months and is now in clinical remission. While rare, intramedullary spinal cord lesions should be considered in the differential diagnosis of infants presenting with poor feeding and hypotonia.