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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

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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

early discharge
Early Discharge
  • New diagnoses in ED
    • Inborn errors of metabolism
    • Congenital anomalies
septic appearing infant
Septic-Appearing infant
  • ABCs
  • Cultures & antibiotics
  • “An ill-appearing infant is septic until proven otherwise” but widen your differential
case 1
Case #1
  • 10-day-old-term infant drinking 3-4 oz at first
  • Decreased appetite & vomiting
  • Sleepy
slide6
“ill appearing”
  • Flat fontanel
  • Dry mucous membrane
  • Enlarged liver
  • Slight hypotonia
  • Glucose 25  40 (after correction)
slide8
Presents in first 2-3 week
  • Septic-appearing
  • Irritability or lethargy
  • Vomiting
slide9
Hypotonia
  • Hepatomegaly
  • Hypoglycemia
  • Breath odor
  • Sweaty feet or stale urine
slide10
Coma
  • Seizure
  • Respiratory distress
slide12
“limited repertoire” of symptoms
  • Non specific
  • Symptoms may overlap
    • E.coli sepsis (galactosemia)
  • Clinically indistinguishable

High index of suspicion

clinical presentations
Clinical presentations
  • Vomiting
  • Lethargy
  • Coma
  • Seizure
slide14
Jaundice
  • Odor
    • Body
    • Urine
inborn error of metabolism
Inborn error of metabolism
  • Encephalopathy without acidosis
  • Encephalopathy with acidosis
  • Hepatic syndrome
iem with no acidosis
IEM with No Acidosis
  • Maple Syrup Urine disease
  • Urea cycle defects
iem with acidosis
IEM with acidosis
  • Organic aciduria
  • Lactic acidosis
hepatic syndrome
Hepatic Syndrome
  • Galactosemia
acute evaluation
Acute Evaluation
  • Glucose
  • pH & HCO3
  • Electrolytes
  • Ammonia
  • Lactate
  • Pyruvate
ammonia level
Ammonia level
  • Susceptible to artifacts
  • Must be placed in ice
  • Immediate processing
  • < 80 mcg/dL
  • Hundreds to thousands
  • Readily traverses BBB
  • Central hyperventilation
slide21
Urine
    • Organic acids
    • Amino acids
    • Ketones
    • Reducing substances
slide22
Hypoglycemia
  • Acidosis
  • Hyperammonemia
hyperammonemia
Hyperammonemia
  • Urea cycle defects
  • Organic acidemia
  • Transient hyperammonemia of the newborn
urea cycle defects
Urea Cycle Defects
  • Early respiratory alkalosis
  • Marked elevation of ammonia
  • Abnormal plasma amino acids
urea cycle defects1
Urea Cycle Defects
  • Ornithine-transcarbamylase (OTC)
  • Carbamyl phosphate synthetase (CPS)
slide29
10% glucose & lipids 1 g/kg
  • Minimal proteins
    • Essential amino acids (0.25 g/kg)
slide30
Sodium benzoate 250 mg/kg
    • Hippuric acid
  • Sodium phenylacetate 250 mg/kg
    • Phenylacetylglutamine
organic acidemia oas
Organic Acidemia (OAs)
  • Methylmalonic acidemia
  • Propionic acidemia
  • Isovaleric acidemia
slide32
Severe acidosis
  • Ketosis
  • Hyperammonemia
  • Seizures
  • Unusual odor (urine)
slide33
Neutropenia
  • Thrombocytopenia
  • Urine organic acid
slide34
Hydration
  • Glucose infusion
  • Bicarbonate
lactic acidosis
Lactic Acidosis
  • Small for gestational age
  • Dysmorphic features
  • Multiorgan disease
  • Seizures
slide36
Lactate/pyruvate ratio
    • Elevated anion gap
    • Arterial specimen
galactosemia
Galactosemia
  • Not manifest until galactose is introduced
  • Most formulas contain lactose
  • No galactose in soy formulas
slide38
Vomiting
  • Lethargy or irritability
  • Feeding difficulties
  • Poor weight gain
  • Convulsion
slide39
Jaundice
  • Hepatomegaly
  • Hypoglycemia
  • Mental Retardation
  • Hepatic Cirrhosis
  • E. coli Sepsis

* Reducing substances in urine

* Must be done before transfusion

phenylketonuria
Phenylketonuria
  • Phenylalanine hydroxylase
  • Normal at birth
  • Mental retardation
    • Gradual onset
  • Vomiting
slide41
Fair skin
  • Blue eyes
  • Seborrhea or eczema
  • Hypertonia
  • Seizure
slide42
Guthrie test
    • Phenylalanine
    • 48-72 hrs
    • After protein feeding
maple syrup disease
Maple Syrup Disease
  • Decarboxylase
  • Branched chain amino acids
    • Leucine (neurotoxic)
    • Isoleucine
    • Valine
slide44
Precedes screening test results
  • Normal at birth
  • First week
  • May present as early as 24 hours
slide45
Feeding intolerance
  • Lethargy
  • Hypotonia
  • Posturing
  • Seizures
slide46
Typical odor
    • Burnt sugar or caramelized sugar
    • May not be prominent
  • Metabolic acidosis
    • Late finding
  • Hypoglycemia
    • No improvement after correction
newborn screening
Newborn Screening
  • Phenylketonuria
  • Maple Syrup Urine Disease
  • Galactosemia
  • Homocystinuria
  • Hypothyroidism
  • Sickle cell disease
  • Biotinidase deficiency
  • HIV
case 2
Case #2
  • 4-week-old-term infant presented fussy, crying & irritable
  • Vomited greenish material
  • Tachycardia
  • Slightly distended abdomen
malrotation volvulus
Malrotation &Volvulus
  • First 2 months
  • Intense & constant pain
    • Crying, drawing up their knees
    • Poor feeding
  • Bilious vomiting
  • Abdominal distension
    • No distension in high volvolus
case 3
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
methemoglobinemia
Methemoglobinemia
  • Uncommon cause of cyanosis
  • Can be a cause of death
  • Ferric rather than ferrous
  • Impaired oxygen binding of Hb
slide52
Hemoglobin M
  • Hemoglobin reductase
  • Drugs (benzocaine-Orajel)
  • Idiopathic (70%)
  • Symptoms depend on the concentration of methemoglobin
slide53
10-30% Cyanosis
  • 30-50% Tachycardia, fatigue
  • 50-70% Lethargy, stupor
  • >70% Death
slide54
Cyanosis without cardiac or pulmonary disease
  • Oxygen-unresponsive cyanosis
  • Cyanosis out of proportion to symptoms
slide55
Chocolate brown blood
  • Pulse oximeter read 90s%
  • Normal PaO2 despite cyanosis
slide56
< 30% Not needed
  • 30-70% Methylene blue
  • No response Hyperbaric O2 Exchange transfusion
slide57
Methylene blue 1 mg/kg IV
  • 10 ml 1% ampule (10 mg/ml)
  • Reduce methHb to hemoglobin
  • Maximum effect in 30 minutes
slide58
Ineffective in G-6PD deficiency
  • Hemolysis in G-6PD deficiency
  • Alter the pulse oximeter reading
case 4
Case #4
  • 7-day-old term infant
  • Poky eater; eats and stops
  • Crying & irritable after eating < 1 oz
  • Acts hungry & wants to eat again
slide60
Bounding pulse in upper extremity
  • Weak/or no pulses in lower extremities
first week
First week
  • Hypoplastic left heart syndrome
  • TGA
  • TAPVR
  • Coarctation of aorta
first month
First month
  • Coarctation of aorta
  • VSD
  • AV canal malformation
ductal dependent lesions
Ductal dependent lesions
  • Coarctation of aorta
  • Hypoplastic left heart syndrome
  • TGA
slide65
Tricuspid atresia
  • Pulmonary atresia
    • with intact ventricular septum
  • Critical pulmonary stenosis
prostaglandin e 1
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
slide67
Recognize life-threatening conditions
  • Initiate therapy even before precise conditions
  • Prostaglandin E1
  • 0.05 - 0.1 microgram/kg/min
slide68
Apnea
  • Bradycardia
  • Hypotension
  • Seizures
  • Hyperthermia
coarctation of aorta
Coarctation of Aorta
  • Decreased lower limb pulses
  • Acute cardiovascular collapse
  • Differential cyanosis
slide70
TGA
  • 5% of all CHD
  • Aorta from RV
  • Pulmonary artery from LV
slide71
Ductus closure
    • minimal mixing of the systemic & pulmonary blood via foramen ovale
    • Hypoxemia
slide72
Cyanosis
  • Tachypnea
  • Murmur may be absent
  • “Egg on a stick appearance”
hypoplsatic left heart
Hypoplsatic left heart
  • Both cyanotic & acyanotic
  • 25% of all cardiac deaths in 1st wk
slide74
Pallor
  • Tachypnea
  • Poor perfusion
    • grayish blue color
  • Poor to absent peripheral pulses
slide75
No murmur
  • Hepatomegaly
  • Metabolic acidosis
case 5
Case # 5
  • 6-week-old-full-term-infant
  • Irritability & poor feeding few days
    • During feeding
  • Pallor & breathlessness
slide77
Irritable
  • Crying-not consolable
  • HR 160, R 50, T 990 F
  • Intermittent grunting
  • O2 saturation 97%
slide78
Pale, cool extremities
  • Clear lung fields
  • Palpable liver 4 cm
  • 4 extremities pulse & BP equal
anomalous origin of lca
Anomalous Origin of LCA
  • Pulmonary Artery
    • Low pressure
    • Desaturated blood
  • Myocardial ischemia
slide80
2 weeks to 6 months
  • Restlessness, irritability
  • Incessant crying
  • Dyspnea
  • Pallor & sweating (> feeding)
slide81
Congestive cardiac failure
    • Tachypnea
    • Tachycardia
    • Cardiomegaly
    • Hepatomegaly
slide82
Q-wave in I, aVL & left precordium
  • Persistent ST-elevation
  • T-wave inversion
case 6
Case # 6
  • 6-day-old girl, lethargic
  • Vomiting all night
  • Extremely irritable
  • Enlarged clitoris with local hairs
adrenal hyperplasia
Adrenal Hyperplasia
  • Inborn errors of adrenal steroid
  • Acute salt-losing crisis
    • 2 - 5 weeks
  • Ambiguous genitalia
slide85
21-hydroxylase deficiency
    • 90% of all cases
    • 1 in 15,000 live births
slide86
Male
    • Appears normal at birth
    • Sexual precocity appears in 6 months
    • Large phallus
    • Dark skin & mucous membrane
slide87
Female
    • Enlarged clitoris
    • Labial fusion
    • Virilization
slide88
Electrolytes
    • Low Na+
    • High K+
  • Glucose
slide89
Adrenal steroid profile
    • 17-hydroxyprogesterone
    • Markedly elevated
    • Obtain before hydrocortisone administration
slide90
Fluid & Electrolyte replacement
    • Urgent
    • 20 ml/kg Normal Saline
  • Hydrocortisone
    • 25 mg IV bolus
    • 50 mg/m2/24 hours
slide91
Hyperkalemia
    • Far better tolerated
    • Volume restoration
    • Insulin & glucose contraindicated
case 7
Case # 7
  • 3-year-old previously healthy girl
  • Breathing fast
  • 6 vomiting in 2 hours
  • Lethargic
slide93
T 100.30 F, HR 156, R 60
  • Clear lung fields
  • Glucose 69
slide94
Na 144
  • K 6
  • Cl 110
  • Urea 27
slide95
pH 7.45
  • PCO2 12
  • HCO3 8
  • Base deficit 12
  • Salicylate level 98 mg/dl
salicylate poisoning
Salicylate poisoning
  • Tachypnea & respiratory alkalosis
  • Metabolic acidosis
  • Fever
  • Seizure
  • Coma
case 8
Case # 8
  • 15-month-old girl- fever & vomiting
  • Sleepy but arousable
  • Lethargic
  • Intermittent cry followed by vomiting
slide98
RUQ tenderness
  • Scant bowel sounds
  • Guaic negative stool
intussusception
Intussusception
  • Sudden onset
  • Triad
    • Vomiting
    • Colicky abdominal pain
    • Heme-positive stool (“currant jelly”)
slide101
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 donot rule out intussusception
slide102
3 months to 6 years
  • 80% under 2 years
  • Male:female 4:1
slide103
Adenoviruses (spring)
  • Rotaviruses (summer)
  • Rotavirus vaccine
slide104
Fluid resuscitation is important
  • All patients are hypovolemic
slide105
Barium enema
    • Diagnostic & therapeutic
  • Air enema
    • Increased success
    • Lower complications & radiation
child abuse
Child Abuse
  • Intracranial hemorrhage
  • Inconsistent history
  • High index of suspicion
take home message
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”