The%20septic%20appearing%20infant:%20approach%20and%20case%20discussion

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The septic appearing infantapproach and case
discussion

• Muhammad Waseem, MD
• Pediatric Emergency Medicine
• Lincoln Hospital Bronx, NY

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Another Sepsis Work-up
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Early Discharge

• New diagnoses in ED
• Inborn errors of metabolism
• Congenital anomalies

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Septic-Appearing infant

• ABCs
• Cultures antibiotics
• An ill-appearing infant is septic until proven
  otherwise but widen your differential

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Case 1

• 10-day-old-term infant drinking 3-4 oz at first
• Decreased appetite vomiting
• Sleepy

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• ill appearing
• Flat fontanel
• Dry mucous membrane
• Enlarged liver
• Slight hypotonia

• Glucose 25 ? 40 (after correction)

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Organic Aciduria
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• Presents in first 2-3 week
• Septic-appearing
• Irritability or lethargy
• Vomiting

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

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• Coma
• Seizure
• Respiratory distress
  
  

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The basic Approach to Inborn Errors of Metabolism
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• limited repertoire of symptoms
• Non specific
• Symptoms may overlap
• E.coli sepsis (galactosemia)
• Clinically indistinguishable

High index of suspicion
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Clinical presentations

• Vomiting
• Lethargy
• Coma
• Seizure

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• Jaundice
• Odor
• Body
• Urine

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Inborn error of metabolism

• Encephalopathy without acidosis
• Encephalopathy with acidosis
• Hepatic syndrome

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IEM with No Acidosis

• Maple Syrup Urine disease
• Urea cycle defects

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IEM with acidosis

• Organic aciduria
• Lactic acidosis

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Hepatic Syndrome

• Galactosemia

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Acute Evaluation

• Glucose
• pH HCO3

• Electrolytes
• Ammonia

• Lactate
• Pyruvate

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Ammonia level

• Susceptible to artifacts
• Must be placed in ice
• Immediate processing
• lt 80 mcg/dL
• Hundreds to thousands
• Readily traverses BBB
• Central hyperventilation

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

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• Hypoglycemia
• Acidosis
• Hyperammonemia

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Hyperammonemia

• Urea cycle defects
• Organic acidemia
• Transient hyperammonemia of the newborn

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Urea Cycle Defects

• Early respiratory alkalosis
• Marked elevation of ammonia
• Abnormal plasma amino acids

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Urea Cycle Defects

• Ornithine-transcarbamylase (OTC)
• Carbamyl phosphate synthetase (CPS)

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• Immediate transfer for hemodialysis

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

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

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Organic Acidemia (OAs)

• Methylmalonic acidemia
• Propionic acidemia
• Isovaleric acidemia

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

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• Neutropenia
• Thrombocytopenia
• Urine organic acid

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• Hydration
• Glucose infusion
• Bicarbonate

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Lactic Acidosis

• Small for gestational age
• Dysmorphic features
• Multiorgan disease
• Seizures

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

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Galactosemia

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

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

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• Jaundice
• Hepatomegaly
• Hypoglycemia

• Mental Retardation
• Hepatic Cirrhosis
• E. coli Sepsis

Reducing substances in urine Must be
done before transfusion
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Phenylketonuria

• Phenylalanine hydroxylase
• Normal at birth
• Mental retardation
• Gradual onset
• Vomiting

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

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

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Maple Syrup Disease

• Decarboxylase
• Branched chain amino acids
• Leucine (neurotoxic)
• Isoleucine
• Valine

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

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• Feeding intolerance
• Lethargy
• Hypotonia
• Posturing
• Seizures

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• Typical odor
• Burnt sugar or caramelized sugar
• May not be prominent
• Metabolic acidosis
• Late finding

• Hypoglycemia
• No improvement after correction

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Newborn Screening

• Phenylketonuria
• Maple Syrup Urine Disease
• Galactosemia
• Homocystinuria
• Hypothyroidism
• Sickle cell disease
• Biotinidase deficiency
• HIV

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Case 2

• 4-week-old-term infant presented fussy, crying
  irritable
• Vomited greenish material
• Tachycardia
• Slightly distended abdomen

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Malrotation Volvulus

• First 2 months
• Intense constant pain
• Crying, drawing up their knees
• Poor feeding
• Bilious vomiting

• Abdominal distension
• No distension in high volvolus

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Case 3

• 4-week-old presented fussy with decreased
  appetite
• Cyanoticdoes not respond to O2
• Tachycardic
• Grunting respiration

• No hepatomegaly
• Normal Chest X-ray

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Methemoglobinemia

• Uncommon cause of cyanosis
• Can be a cause of death

• Ferric rather than ferrous
• Impaired oxygen binding of Hb

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• Hemoglobin M
• Hemoglobin reductase
• Drugs (benzocaine-Orajel)
• Idiopathic (70)

• Symptoms depend on the concentration of
  methemoglobin

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• 10-30 Cyanosis
• 30-50 Tachycardia, fatigue
• 50-70 Lethargy, stupor
• gt70 Death

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• Cyanosis without cardiac or pulmonary disease
• Oxygen-unresponsive cyanosis
• Cyanosis out of proportion to symptoms

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• Chocolate brown blood
• Pulse oximeter read 90s
• Normal PaO2 despite cyanosis

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• lt 30 Not needed
• 30-70 Methylene blue
• No response Hyperbaric O2 Exchange
  transfusion

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• Methylene blue 1 mg/kg IV
• 10 ml 1 ampule (10 mg/ml)
• Reduce methHb to hemoglobin
• Maximum effect in 30 minutes

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• Ineffective in G-6PD deficiency
• Hemolysis in G-6PD deficiency
• Alter the pulse oximeter reading

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Case 4

• 7-day-old term infant
• Poky eater eats and stops
• Crying irritable after eating lt 1 oz
• Acts hungry wants to eat again

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• Bounding pulse in upper extremity
• Weak/or no pulses in lower extremities

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• Congenital Heart Defects

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First week

• Hypoplastic left heart syndrome
• TGA
• TAPVR
• Coarctation of aorta

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First month

• Coarctation of aorta
• VSD
• AV canal malformation

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Ductal dependent lesions

• Coarctation of aorta
• Hypoplastic left heart syndrome
• TGA

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• Tricuspid atresia
• Pulmonary atresia
• with intact ventricular septum
• Critical pulmonary stenosis

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Prostaglandin E1

• 0.3 X Kg Number of mg to be added in
  50 ml
• 0.5 ml/hr will deliver 0.05 microgram/kg/min

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• Recognize life-threatening conditions
• Initiate therapy even before precise conditions

• Prostaglandin E1
• 0.05 - 0.1 microgram/kg/min

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• Apnea
• Bradycardia
• Hypotension
• Seizures
• Hyperthermia

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Coarctation of Aorta

• Decreased lower limb pulses
• Acute cardiovascular collapse
• Differential cyanosis

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TGA

• 5 of all CHD
• Aorta from RV
• Pulmonary artery from LV

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• Ductus closure
• minimal mixing of the systemic pulmonary blood
  via foramen ovale
• Hypoxemia

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• Cyanosis
• Tachypnea
• Murmur may be absent
• Egg on a stick appearance

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Hypoplsatic left heart

• Both cyanotic acyanotic
• 25 of all cardiac deaths in 1st wk

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• Pallor
• Tachypnea
• Poor perfusion
• grayish blue color
• Poor to absent peripheral pulses

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• No murmur
• Hepatomegaly
• Metabolic acidosis

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Case 5

• 6-week-old-full-term-infant
• Irritability poor feeding few days
• During feeding
• Pallor breathlessness

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• Irritable
• Crying-not consolable
• HR 160, R 50, T 990 F
• Intermittent grunting
• O2 saturation 97

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• Pale, cool extremities
• Clear lung fields
• Palpable liver 4 cm
• 4 extremities pulse BP equal

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Anomalous Origin of LCA

• Pulmonary Artery
• Low pressure
• Desaturated blood
• Myocardial ischemia

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• 2 weeks to 6 months
• Restlessness, irritability
• Incessant crying
• Dyspnea
• Pallor sweating (gt feeding)

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• Congestive cardiac failure
• Tachypnea
• Tachycardia
• Cardiomegaly
• Hepatomegaly

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• Q-wave in I, aVL left precordium
• Persistent ST-elevation
• T-wave inversion

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Case 6

• 6-day-old girl, lethargic
• Vomiting all night
• Extremely irritable
• Enlarged clitoris with local hairs

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Adrenal Hyperplasia

• Inborn errors of adrenal steroid
• Acute salt-losing crisis
• 2 - 5 weeks
• Ambiguous genitalia

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• 21-hydroxylase deficiency
• 90 of all cases
• 1 in 15,000 live births

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• Male
• Appears normal at birth
• Sexual precocity appears in 6 months
• Large phallus
• Dark skin mucous membrane

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• Female
• Enlarged clitoris
• Labial fusion
• Virilization

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• Electrolytes
• Low Na
• High K
• Glucose

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• Adrenal steroid profile
• 17-hydroxyprogesterone
• Markedly elevated
• Obtain before hydrocortisone administration

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• Fluid Electrolyte replacement
• Urgent
• 20 ml/kg Normal Saline

• Hydrocortisone
• 25 mg IV bolus
• 50 mg/m2/24 hours

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• Hyperkalemia
• Far better tolerated
• Volume restoration
• Insulin glucose contraindicated

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Case 7

• 3-year-old previously healthy girl
• Breathing fast
• 6 vomiting in 2 hours
• Lethargic

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• T 100.30 F, HR 156, R 60
• Clear lung fields
• Glucose 69

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• Na 144
• K 6
• Cl 110
• Urea 27

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• pH 7.45
• PCO2 12
• HCO3 8
• Base deficit 12

• Salicylate level 98 mg/dl

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Salicylate poisoning

• Tachypnea respiratory alkalosis
• Metabolic acidosis
• Fever
• Seizure
• Coma

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Case 8

• 15-month-old girl- fever vomiting
• Sleepy but arousable
• Lethargic
• Intermittent cry followed by vomiting

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• RUQ tenderness
• Scant bowel sounds
• Guaic negative stool

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Intussusception

• Sudden onset
• Triad
• Vomiting
• Colicky abdominal pain
• Heme-positive stool (currant jelly)

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• Target sign
• Soft tissue mass with 2 concentric circles of fat
  density
• Absence of cecal gas stool
• Loss of visualization of tip of liver
• Paucity of bowel gas (RLQ)

• Normal abdominal radiographs do not rule out
  intussusception

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• 3 months to 6 years
• 80 under 2 years
• Malefemale 41

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• Adenoviruses (spring)
• Rotaviruses (summer)
• Rotavirus vaccine

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• Fluid resuscitation is important
• All patients are hypovolemic

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• Barium enema
• Diagnostic therapeutic
• Air enema
• Increased success
• Lower complications radiation

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Child Abuse

• Intracranial hemorrhage
• Inconsistent history
• High index of suspicion

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Take home message

• The septic appearing infant is septic until
  proven otherwise but think beyond!
• Key to diagnosis is high index of suspicion

• Eyes can not see what the mind does not
  know