Challenges in managing metabolic crises and arrhythmias in TANGO2 mutation
TANGO2 mutation is a rare genetic mutation characterized by metabolic crises associated with rhabdomyolysis, hypoglycemia, metabolic acidosis, encephalopathy, and life-threatening arrhythmias. There is a paucity of literature regarding the prevention or acute management of these metabolic crises and their sequelae
Journal Club about the Phase 2 study of Selonsertib in Diabetic Kidney Disease to Our Division on 12/9/19.
Also an intro about the Phase 3 study (MOSAIC) we will be launching before the end of the year
ANEMIA IS ASSOCIATED WITH GREATER MORBIDITY AND RESOURCE UTILIZATION IN PEDIA...Texas Children's Hospital
Design: Retrospective cohort study querying the Pediatric Health Information System (PHIS) database, comprised of 50 children’s hospitals over 10 years (01/2008 to 12/2017).
Admissions of patients aged < 21 years (yr) with ICD-9/10 codes for systolic HF were included. Patients with congenital heart disease (CHD) codes were excluded to avoid confounding by polycythemia due to cyanotic CHD.
Demographic and clinical features and procedures during admission reviewed using ICD-9/10 coding.
Outcomes: Primary outcome was composite cardiac death (CCD, defined as ventricular assist device (VAD), heart transplant (HTx), or death during admission), and the secondary outcomes were hospital length of stay (LOS) and billed charges.
Univariate and multivariable analyses performed using generalized estimating equations (GEE) for categorical outcomes and mixed modeling for continuous outcomes, to account for clustering by hospitals - factors with p<0.2 on univariate analysis included in the initial multivariable model, and factors with p<0.05 retained in successive models.
In a hospital admission of a pediatric patient with systolic HF without CHD, anemia is associated with more systemic comorbidities, and greater resource utilization (longer LOS and higher billed charges).
This suggests a need for examining anemia management strategies to optimize pediatric HF outcomes.
[03/2019]
* Taylor, Olson, Marc, Anders
Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy characterized by heart failure secondary to left ventricular systolic dysfunction, typically with an ejection fraction <45%, occurring towards the end of pregnancy or in the months following delivery for which no other cause of heart failure is found.1 Rarely, catastrophic presentations can occur with severe respiratory distress and low cardiac output necessitating mechanical ventilation and circulatory support. Data on the use of extracorporeal membrane oxygenation (ECMO) in PPCM is limited. Little is known about the safety, efficacy, or mortality. The Extracorporeal Life Support Organization (ELSO) maintains an international registry of patients treated with ECMO since 1989 and collects data from over 300 pediatric and adult centers. We sought to examine the ELSO registry for PPCM patients treated with ECMO in order to characterize demographic and clinical features, complications, overall survival to discharge, and variables associated with mortality.
46 patients met inclusion criteria. 2 patients were excluded leaving 44 patients for the analysis. Overall survival to discharge was 56.8% while 75.0% of patients were weaned off ECMO. All patients had one ECMO run except for one patient who had two. All patients were conventionally ventilated.
Cardiovascular (52.3%), renal (36.4%), hemorrhagic (34.1%), mechanical (25.0%), infectious (15.9%), metabolic (15.9%), neurologic (11.4%), and pulmonary (9.1%) complications were reported.
Pre- ECMO variables associated with decreased survival included higher ventilation rate (p=0.03**, OR 0.88 [0.79-0.98]) and support with vasopressor or inotropic agents (p<0.01, OR 0.09 [0.01-0.82]). Decreased survival was also observed in patients with ECMO cannula site bleeding (p=0.02, OR 0.14 [0.02-0.83]).
Our review is the largest to date of PPCM patients supported with ECMO. We identified factors associated with mortality including pre-ECMO ventilation rate, pre-ECMO support with vasopressors, and ECMO cannula site bleeding. We believe our data supports the use of ECMO in PPCM when clinically indicated.
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Journal Club about the Phase 2 study of Selonsertib in Diabetic Kidney Disease to Our Division on 12/9/19.
Also an intro about the Phase 3 study (MOSAIC) we will be launching before the end of the year
ANEMIA IS ASSOCIATED WITH GREATER MORBIDITY AND RESOURCE UTILIZATION IN PEDIA...Texas Children's Hospital
Design: Retrospective cohort study querying the Pediatric Health Information System (PHIS) database, comprised of 50 children’s hospitals over 10 years (01/2008 to 12/2017).
Admissions of patients aged < 21 years (yr) with ICD-9/10 codes for systolic HF were included. Patients with congenital heart disease (CHD) codes were excluded to avoid confounding by polycythemia due to cyanotic CHD.
Demographic and clinical features and procedures during admission reviewed using ICD-9/10 coding.
Outcomes: Primary outcome was composite cardiac death (CCD, defined as ventricular assist device (VAD), heart transplant (HTx), or death during admission), and the secondary outcomes were hospital length of stay (LOS) and billed charges.
Univariate and multivariable analyses performed using generalized estimating equations (GEE) for categorical outcomes and mixed modeling for continuous outcomes, to account for clustering by hospitals - factors with p<0.2 on univariate analysis included in the initial multivariable model, and factors with p<0.05 retained in successive models.
In a hospital admission of a pediatric patient with systolic HF without CHD, anemia is associated with more systemic comorbidities, and greater resource utilization (longer LOS and higher billed charges).
This suggests a need for examining anemia management strategies to optimize pediatric HF outcomes.
[03/2019]
* Taylor, Olson, Marc, Anders
Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy characterized by heart failure secondary to left ventricular systolic dysfunction, typically with an ejection fraction <45%, occurring towards the end of pregnancy or in the months following delivery for which no other cause of heart failure is found.1 Rarely, catastrophic presentations can occur with severe respiratory distress and low cardiac output necessitating mechanical ventilation and circulatory support. Data on the use of extracorporeal membrane oxygenation (ECMO) in PPCM is limited. Little is known about the safety, efficacy, or mortality. The Extracorporeal Life Support Organization (ELSO) maintains an international registry of patients treated with ECMO since 1989 and collects data from over 300 pediatric and adult centers. We sought to examine the ELSO registry for PPCM patients treated with ECMO in order to characterize demographic and clinical features, complications, overall survival to discharge, and variables associated with mortality.
46 patients met inclusion criteria. 2 patients were excluded leaving 44 patients for the analysis. Overall survival to discharge was 56.8% while 75.0% of patients were weaned off ECMO. All patients had one ECMO run except for one patient who had two. All patients were conventionally ventilated.
Cardiovascular (52.3%), renal (36.4%), hemorrhagic (34.1%), mechanical (25.0%), infectious (15.9%), metabolic (15.9%), neurologic (11.4%), and pulmonary (9.1%) complications were reported.
Pre- ECMO variables associated with decreased survival included higher ventilation rate (p=0.03**, OR 0.88 [0.79-0.98]) and support with vasopressor or inotropic agents (p<0.01, OR 0.09 [0.01-0.82]). Decreased survival was also observed in patients with ECMO cannula site bleeding (p=0.02, OR 0.14 [0.02-0.83]).
Our review is the largest to date of PPCM patients supported with ECMO. We identified factors associated with mortality including pre-ECMO ventilation rate, pre-ECMO support with vasopressors, and ECMO cannula site bleeding. We believe our data supports the use of ECMO in PPCM when clinically indicated.
Use of inotropic and vasoconstrictor medications in the pediatric heart failu...Texas Children's Hospital
* Raysa Morales-Demori, MD
Inotropes and vasoconstrictor medications are frequently used in the pediatric heart failure population for the acute and chronic management in this condition; however certain agents, such as Dobutamine, have been associated with increased inpatient mortality
Type of study: Retrospective cohort study
Database: Pediatric Health Information System
Study period: 01/2004 – 12/2017
Inclusion criteria:
All patients ≤ 21 year
Heart failure diagnoses ICD-9 (428.xx) or ICD-10 (i50.xx)
Dobutamine and Dopamine use has fallen out of favor in the pediatric HF population
Dobutamine and Norepinephrine have the highest odds ratio of inpatient mortality
* Raysa Morales-Demori, MD
Type of study: Retrospective cohort study
Database: Pediatric Health Information System
Study period: 01/2004 – 12/2017
Inclusion criteria:
All patients ≤ 21 year
Heart failure diagnoses ICD-9 (428.xx) or ICD-10 (i50.xx)
Digoxin use was associated with decreased inpatient mortality
In patients with CHD (24% vs 76%, p<0.001)
In Extreme Risk of Mortality (17% vs 83%, p<0.001)
Relative Hyperoxia in cyanotic congenital heart disease on veno-arterial ECMO...Texas Children's Hospital
Extracorporeal membrane oxygenation (ECMO) is an
established intervention for respiratory or cardiorespiratory
support in children with congenital heart disease (CHD)
when all other interventions have failed. Hyperoxia
following successful resuscitation has been associated with
increased mortality in pediatric and adult studies,
including, specifically, hyperoxia during ECMO
management. We hypothesized that this effect may be
pronounced in patients with lower arterial oxygen
saturation at baseline, such as those with cyanotic CHD. We
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ECMO is a risk factor for mortality in a large multicenter
registry analysis.
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Ectopic atrial tachycardia (EAT) is one of the most common forms of persistent supraventricular tachycardia in children.
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As surgical and medical outcomes of children with congenital heart disease improve, it is expected that the pediatric population with heart failure (HF) will increase.
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Qualifications:
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FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
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Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
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Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
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Adaptation Towards the Sense of Smell:
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Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
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Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
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Characteristics of Smell:
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Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
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Primitive, less old, and new olfactory systems with different path
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2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
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Challenges in managing metabolic crises and arrhythmias in TANGO2 mutation - PCICS 2018
1. Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine, Texas Children’s Hospital
Challenges in managing metabolic crises and arrhythmias in TANGO2 mutation
INTRODUCTION CASE DESCRIPTION DISCUSSION AND IMPLICATIONS
TANGO2 mutation is a rare genetic mutation
characterized by metabolic crises associated
with rhabdomyolysis, hypoglycemia,
metabolic acidosis, encephalopathy, and life-
threatening arrhythmias. There is a paucity of
literature regarding the prevention or acute
management of these metabolic crises and
their sequelae
Fig 1: EKG on day of admission to CICU showing normal sinus
rhythm. Type 1 Brugada. ST depression in inferolateral leads.
Prolonged QTc (518msec), Nonspecific ST and T wave abnormality
•Challenge to medical team to manage his
arrhythmias with isoproterenol in the setting of
Brugada pattern on EKG
•Evidence of Brugada syndrome only presents
during metabolic crises, which also provokes
arrhythmias
•No clinical correlates associated with his
worsening metabolic crises to facilitate
projection of his trajectory of illness, which made
it difficult to manage him outpatient and at
outside facilities
•Due to presence of Brugada syndrome, lack of
treatment options if isoproterenol and
magnesium were inadequate
•Underlying condition made him ineligible for
ECMO and heart transplant
•Despite many metabolic crises, all of which were
associated with rhabdomyolysis, kidney function
has always remained normal
•Moral and ethical challenges due to
degenerative nature of disease and difficulty in
determining when care should be limited to
preserve quality of life
REFERENCES
1. Lalani, Seema R., et al. “Recurrent Muscle Weakness with Rhabdomyolysis, Metabolic
Crises, and Cardiac Arrhythmia Due to Bi-Allelic TANGO2 Mutations.” The American
Journal of Human Genetics, vol. 98, no. 2, 2016, pp. 347–357.
Jill Zender, RN, CPNP-AC, Raysa Morales-Demori, MD, Marc Anders, MD
• Presented in 2014, at 2 years of age, with difficulty walking, progressive
neurologic decline, hypoglycemia, and an episode of unresponsiveness
• Whole Exome Sequencing obtained that demonstrated a homozygous
pathogenic variant of p.Gly154Arg in the TANGO2 gene in March 2016
• Presented in March 2018 with fatigue, hematuria, right lower back and
bilateral leg pain, hypoglycemia and Creatinine Kinase (CK) >64,000 u/L
without evidence of Acute Kidney Injury (AKI)
• On EKG had findings consistent with Brugada Syndrome, which was not
present on previous EKGs, and QTc >500 ms
• Throughout CICU admission, never developed AKI despite CK >64,000 u/L
for over a week
• Began to have multiple episodes of ventricular bigeminy and Torsades de
pointes (TdP), which were initially treated with Magnesium sulfate
(maintaining magnesium level >2.4 mg/dL) and esmolol infusion
• Transitioned to isoproterenol because ventricular bigeminy and TdP more
prevalent with heart rate <120 bpm
• Since initial CICU admission, has required readmission multiple times for
management of metabolic crises and arrhythmias
• Each presentation has been characterized by significantly elevated CK levels
without AKI, frequent arrhythmias and marked neurologic decline with
difficulty speaking and swallowing
• When metabolic crises resolve and CK levels normalize, his arrhythmia
burden ceases and he regains motor function
• It remains unclear what leads to the occurrence and resolution of each crises