The%20septic%20appearing%20infant:%20approach%20and%20case%20discussion - PowerPoint PPT Presentation

View by Category
About This Presentation
Title:

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

Description:

'An ill-appearing infant is septic until proven otherwise' but widen your ... The basic Approach to Inborn Errors of Metabolism 'limited repertoire' of symptoms ... – PowerPoint PPT presentation

Number of Views:153
Avg rating:3.0/5.0
Slides: 108
Provided by: jacquelin90
Category:

less

Write a Comment
User Comments (0)
Transcript and Presenter's Notes

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


1
The septic appearing infantapproach 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
  • lt 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
(No Transcript)
25
Urea Cycle Defects
  • Early respiratory alkalosis
  • Marked elevation of ammonia
  • Abnormal plasma amino acids

26
Urea Cycle Defects
  • Ornithine-transcarbamylase (OTC)
  • Carbamyl phosphate synthetase (CPS)

27
(No Transcript)
28
  • Immediate transfer for hemodialysis

29
  • 10 glucose lipids 1 g/kg
  • Minimal proteins
  • Essential amino acids (0.25 g/kg)

30
  • Sodium benzoate 250 mg/kg
  • Hippuric acid
  • Sodium phenylacetate 250 mg/kg
  • Phenylacetylglutamine

31
Organic Acidemia (OAs)
  • Methylmalonic acidemia
  • Propionic acidemia
  • Isovaleric acidemia

32
  • Severe acidosis
  • Ketosis
  • Hyperammonemia
  • Seizures
  • Unusual odor (urine)

33
  • Neutropenia
  • Thrombocytopenia
  • Urine organic acid

34
  • Hydration
  • Glucose infusion
  • Bicarbonate

35
Lactic Acidosis
  • Small for gestational age
  • Dysmorphic features
  • Multiorgan disease
  • Seizures

36
  • Lactate/pyruvate ratio
  • Elevated anion gap
  • Arterial specimen

37
Galactosemia
  • Not manifest until galactose is introduced
  • Most formulas contain lactose
  • No galactose in soy formulas

38
  • Vomiting
  • Lethargy or irritability
  • Feeding difficulties
  • Poor weight gain
  • Convulsion

39
  • Jaundice
  • Hepatomegaly
  • Hypoglycemia
  • Mental Retardation
  • Hepatic Cirrhosis
  • E. coli Sepsis

Reducing substances in urine Must be
done before transfusion
40
Phenylketonuria
  • Phenylalanine hydroxylase
  • Normal at birth
  • Mental retardation
  • Gradual onset
  • Vomiting

41
  • Fair skin
  • Blue eyes
  • Seborrhea or eczema
  • Hypertonia
  • Seizure

42
  • Guthrie test
  • Phenylalanine
  • 48-72 hrs
  • After protein feeding

43
Maple Syrup Disease
  • Decarboxylase
  • Branched chain amino acids
  • Leucine (neurotoxic)
  • Isoleucine
  • Valine

44
  • Precedes screening test results
  • Normal at birth
  • First week
  • May present as early as 24 hours

45
  • Feeding intolerance
  • Lethargy
  • Hypotonia
  • Posturing
  • Seizures

46
  • Typical odor
  • Burnt sugar or caramelized sugar
  • May not be prominent
  • Metabolic acidosis
  • Late finding
  • Hypoglycemia
  • No improvement after correction

47
Newborn Screening
  • Phenylketonuria
  • Maple Syrup Urine Disease
  • Galactosemia
  • Homocystinuria
  • Hypothyroidism
  • Sickle cell disease
  • Biotinidase deficiency
  • HIV

48
Case 2
  • 4-week-old-term infant presented fussy, crying
    irritable
  • Vomited greenish material
  • Tachycardia
  • Slightly distended abdomen

49
Malrotation Volvulus
  • First 2 months
  • Intense constant pain
  • Crying, drawing up their knees
  • Poor feeding
  • Bilious vomiting
  • Abdominal distension
  • No distension in high volvolus

50
Case 3
  • 4-week-old presented fussy with decreased
    appetite
  • Cyanoticdoes not respond to O2
  • Tachycardic
  • Grunting respiration
  • No hepatomegaly
  • Normal Chest X-ray

51
Methemoglobinemia
  • Uncommon cause of cyanosis
  • Can be a cause of death
  • Ferric rather than ferrous
  • Impaired oxygen binding of Hb

52
  • Hemoglobin M
  • Hemoglobin reductase
  • Drugs (benzocaine-Orajel)
  • Idiopathic (70)
  • Symptoms depend on the concentration of
    methemoglobin

53
  • 10-30 Cyanosis
  • 30-50 Tachycardia, fatigue
  • 50-70 Lethargy, stupor
  • gt70 Death

54
  • Cyanosis without cardiac or pulmonary disease
  • Oxygen-unresponsive cyanosis
  • Cyanosis out of proportion to symptoms

55
  • Chocolate brown blood
  • Pulse oximeter read 90s
  • Normal PaO2 despite cyanosis

56
  • lt 30 Not needed
  • 30-70 Methylene blue
  • No response Hyperbaric O2 Exchange
    transfusion

57
  • Methylene blue 1 mg/kg IV
  • 10 ml 1 ampule (10 mg/ml)
  • Reduce methHb to hemoglobin
  • Maximum effect in 30 minutes

58
  • Ineffective in G-6PD deficiency
  • Hemolysis in G-6PD deficiency
  • Alter the pulse oximeter reading

59
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

60
  • Bounding pulse in upper extremity
  • Weak/or no pulses in lower extremities

61
  • Congenital Heart Defects

62
First week
  • Hypoplastic left heart syndrome
  • TGA
  • TAPVR
  • Coarctation of aorta

63
First month
  • Coarctation of aorta
  • VSD
  • AV canal malformation

64
Ductal dependent lesions
  • Coarctation of aorta
  • Hypoplastic left heart syndrome
  • TGA

65
  • Tricuspid atresia
  • Pulmonary atresia
  • with intact ventricular septum
  • Critical pulmonary stenosis

66
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

67
  • Recognize life-threatening conditions
  • Initiate therapy even before precise conditions
  • Prostaglandin E1
  • 0.05 - 0.1 microgram/kg/min

68
  • Apnea
  • Bradycardia
  • Hypotension
  • Seizures
  • Hyperthermia

69
Coarctation of Aorta
  • Decreased lower limb pulses
  • Acute cardiovascular collapse
  • Differential cyanosis

70
TGA
  • 5 of all CHD
  • Aorta from RV
  • Pulmonary artery from LV

71
  • Ductus closure
  • minimal mixing of the systemic pulmonary blood
    via foramen ovale
  • Hypoxemia

72
  • Cyanosis
  • Tachypnea
  • Murmur may be absent
  • Egg on a stick appearance

73
Hypoplsatic left heart
  • Both cyanotic acyanotic
  • 25 of all cardiac deaths in 1st wk

74
  • Pallor
  • Tachypnea
  • Poor perfusion
  • grayish blue color
  • Poor to absent peripheral pulses

75
  • No murmur
  • Hepatomegaly
  • Metabolic acidosis

76
Case 5
  • 6-week-old-full-term-infant
  • Irritability poor feeding few days
  • During feeding
  • Pallor breathlessness

77
  • Irritable
  • Crying-not consolable
  • HR 160, R 50, T 990 F
  • Intermittent grunting
  • O2 saturation 97

78
  • Pale, cool extremities
  • Clear lung fields
  • Palpable liver 4 cm
  • 4 extremities pulse BP equal

79
Anomalous Origin of LCA
  • Pulmonary Artery
  • Low pressure
  • Desaturated blood
  • Myocardial ischemia

80
  • 2 weeks to 6 months
  • Restlessness, irritability
  • Incessant crying
  • Dyspnea
  • Pallor sweating (gt feeding)

81
  • Congestive cardiac failure
  • Tachypnea
  • Tachycardia
  • Cardiomegaly
  • Hepatomegaly

82
  • Q-wave in I, aVL left precordium
  • Persistent ST-elevation
  • T-wave inversion

83
Case 6
  • 6-day-old girl, lethargic
  • Vomiting all night
  • Extremely irritable
  • Enlarged clitoris with local hairs

84
Adrenal Hyperplasia
  • Inborn errors of adrenal steroid
  • Acute salt-losing crisis
  • 2 - 5 weeks
  • Ambiguous genitalia

85
  • 21-hydroxylase deficiency
  • 90 of all cases
  • 1 in 15,000 live births

86
  • Male
  • Appears normal at birth
  • Sexual precocity appears in 6 months
  • Large phallus
  • Dark skin mucous membrane

87
  • Female
  • Enlarged clitoris
  • Labial fusion
  • Virilization

88
  • Electrolytes
  • Low Na
  • High K
  • Glucose

89
  • Adrenal steroid profile
  • 17-hydroxyprogesterone
  • Markedly elevated
  • Obtain before hydrocortisone administration

90
  • Fluid Electrolyte replacement
  • Urgent
  • 20 ml/kg Normal Saline
  • Hydrocortisone
  • 25 mg IV bolus
  • 50 mg/m2/24 hours

91
  • Hyperkalemia
  • Far better tolerated
  • Volume restoration
  • Insulin glucose contraindicated

92
Case 7
  • 3-year-old previously healthy girl
  • Breathing fast
  • 6 vomiting in 2 hours
  • Lethargic

93
  • T 100.30 F, HR 156, R 60
  • Clear lung fields
  • Glucose 69

94
  • Na 144
  • K 6
  • Cl 110
  • Urea 27

95
  • pH 7.45
  • PCO2 12
  • HCO3 8
  • Base deficit 12
  • Salicylate level 98 mg/dl

96
Salicylate poisoning
  • Tachypnea respiratory alkalosis
  • Metabolic acidosis
  • Fever
  • Seizure
  • Coma

97
Case 8
  • 15-month-old girl- fever vomiting
  • Sleepy but arousable
  • Lethargic
  • Intermittent cry followed by vomiting

98
  • RUQ tenderness
  • Scant bowel sounds
  • Guaic negative stool

99
Intussusception
  • Sudden onset
  • Triad
  • Vomiting
  • Colicky abdominal pain
  • Heme-positive stool (currant jelly)

100
(No Transcript)
101
  • 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

102
  • 3 months to 6 years
  • 80 under 2 years
  • Malefemale 41

103
  • Adenoviruses (spring)
  • Rotaviruses (summer)
  • Rotavirus vaccine

104
  • Fluid resuscitation is important
  • All patients are hypovolemic

105
  • Barium enema
  • Diagnostic therapeutic
  • Air enema
  • Increased success
  • Lower complications radiation

106
Child Abuse
  • Intracranial hemorrhage
  • Inconsistent history
  • High index of suspicion

107
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
About PowerShow.com