1 / 107

The septic appearing infant: approach and case discussion

The septic appearing infant: approach and case discussion. Muhammad Waseem, MD Pediatric Emergency Medicine Lincoln Hospital Bronx, NY. Another Sepsis Work-up. Early Discharge. New diagnoses in ED Inborn errors of metabolism Congenital anomalies. Septic-Appearing infant. ABCs

sandra_john
Download Presentation

The septic appearing infant: approach and case discussion

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. The septic appearing infant:approach and case discussion Muhammad Waseem, MD Pediatric Emergency Medicine Lincoln Hospital Bronx, NY

  2. Another Sepsis Work-up

  3. Early Discharge • New diagnoses in ED • Inborn errors of metabolism • Congenital anomalies

  4. Septic-Appearing infant • ABCs • Cultures & antibiotics • “An ill-appearing infant is septic until proven otherwise” but widen your differential

  5. Case #1 • 10-day-old-term infant drinking 3-4 oz at first • Decreased appetite & vomiting • Sleepy

  6. “ill appearing” • Flat fontanel • Dry mucous membrane • Enlarged liver • Slight hypotonia • Glucose 25  40 (after correction)

  7. Organic Aciduria

  8. Presents in first 2-3 week • Septic-appearing • Irritability or lethargy • Vomiting

  9. Hypotonia • Hepatomegaly • Hypoglycemia • Breath odor • Sweaty feet or stale urine

  10. Coma • Seizure • Respiratory distress

  11. The basic Approach to Inborn Errors of Metabolism

  12. “limited repertoire” of symptoms • Non specific • Symptoms may overlap • E.coli sepsis (galactosemia) • Clinically indistinguishable High index of suspicion

  13. Clinical presentations • Vomiting • Lethargy • Coma • Seizure

  14. Jaundice • Odor • Body • Urine

  15. Inborn error of metabolism • Encephalopathy without acidosis • Encephalopathy with acidosis • Hepatic syndrome

  16. IEM with No Acidosis • Maple Syrup Urine disease • Urea cycle defects

  17. IEM with acidosis • Organic aciduria • Lactic acidosis

  18. Hepatic Syndrome • Galactosemia

  19. Acute Evaluation • Glucose • pH & HCO3 • Electrolytes • Ammonia • Lactate • Pyruvate

  20. Ammonia level • Susceptible to artifacts • Must be placed in ice • Immediate processing • < 80 mcg/dL • Hundreds to thousands • Readily traverses BBB • Central hyperventilation

  21. Urine • Organic acids • Amino acids • Ketones • Reducing substances

  22. Hypoglycemia • Acidosis • Hyperammonemia

  23. Hyperammonemia • Urea cycle defects • Organic acidemia • Transient hyperammonemia of the newborn

  24. Urea Cycle Defects • Early respiratory alkalosis • Marked elevation of ammonia • Abnormal plasma amino acids

  25. Urea Cycle Defects • Ornithine-transcarbamylase (OTC) • Carbamyl phosphate synthetase (CPS)

  26. Immediate transfer for hemodialysis

  27. 10% glucose & lipids 1 g/kg • Minimal proteins • Essential amino acids (0.25 g/kg)

  28. Sodium benzoate 250 mg/kg • Hippuric acid • Sodium phenylacetate 250 mg/kg • Phenylacetylglutamine

  29. Organic Acidemia (OAs) • Methylmalonic acidemia • Propionic acidemia • Isovaleric acidemia

  30. Severe acidosis • Ketosis • Hyperammonemia • Seizures • Unusual odor (urine)

  31. Neutropenia • Thrombocytopenia • Urine organic acid

  32. Hydration • Glucose infusion • Bicarbonate

  33. Lactic Acidosis • Small for gestational age • Dysmorphic features • Multiorgan disease • Seizures

  34. Lactate/pyruvate ratio • Elevated anion gap • Arterial specimen

  35. Galactosemia • Not manifest until galactose is introduced • Most formulas contain lactose • No galactose in soy formulas

  36. Vomiting • Lethargy or irritability • Feeding difficulties • Poor weight gain • Convulsion

  37. Jaundice • Hepatomegaly • Hypoglycemia • Mental Retardation • Hepatic Cirrhosis • E. coli Sepsis * Reducing substances in urine * Must be done before transfusion

  38. Phenylketonuria • Phenylalanine hydroxylase • Normal at birth • Mental retardation • Gradual onset • Vomiting

  39. Fair skin • Blue eyes • Seborrhea or eczema • Hypertonia • Seizure

  40. Guthrie test • Phenylalanine • 48-72 hrs • After protein feeding

  41. Maple Syrup Disease • Decarboxylase • Branched chain amino acids • Leucine (neurotoxic) • Isoleucine • Valine

  42. Precedes screening test results • Normal at birth • First week • May present as early as 24 hours

  43. Feeding intolerance • Lethargy • Hypotonia • Posturing • Seizures

  44. Typical odor • Burnt sugar or caramelized sugar • May not be prominent • Metabolic acidosis • Late finding • Hypoglycemia • No improvement after correction

  45. Newborn Screening • Phenylketonuria • Maple Syrup Urine Disease • Galactosemia • Homocystinuria • Hypothyroidism • Sickle cell disease • Biotinidase deficiency • HIV

  46. Case #2 • 4-week-old-term infant presented fussy, crying & irritable • Vomited greenish material • Tachycardia • Slightly distended abdomen

  47. Malrotation &Volvulus • First 2 months • Intense & constant pain • Crying, drawing up their knees • Poor feeding • Bilious vomiting • Abdominal distension • No distension in high volvolus

  48. Case # 3 • 4-week-old presented fussy with decreased appetite • Cyanotic;does not respond to O2 • Tachycardic • Grunting respiration • No hepatomegaly • Normal Chest X-ray

More Related