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

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Presentation on theme: "The septic appearing infant: approach and case discussion Muhammad Waseem, MD Pediatric Emergency Medicine Lincoln Hospital Bronx, NY."— 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 l New diagnoses in ED l Inborn errors of metabolism l Congenital anomalies

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

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

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

7 Organic Aciduria

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

9 l Hypotonia l Hepatomegaly l Hypoglycemia l Breath odor l Sweaty feet or stale urine

10 l Coma l Seizure l Respiratory distress

11 The basic Approach to Inborn Errors of Metabolism

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

13 Clinical presentations l Vomiting l Lethargy l Coma l Seizure

14 l Jaundice l Odor l Body l Urine

15 Inborn error of metabolism l Encephalopathy without acidosis l Encephalopathy with acidosis l Hepatic syndrome

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

17 IEM with acidosis l Organic aciduria l Lactic acidosis

18 Hepatic Syndrome l Galactosemia

19 Acute Evaluation l Glucose l pH & HCO3 l Electrolytes l Ammonia l Lactate l Pyruvate

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

21 l Urine l Organic acids l Amino acids l Ketones l Reducing substances

22 l Hypoglycemia l Acidosis l Hyperammonemia

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

24

25 Urea Cycle Defects l Early respiratory alkalosis l Marked elevation of ammonia l Abnormal plasma amino acids

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

27

28 l Immediate transfer for hemodialysis

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

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

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

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

33 l Neutropenia l Thrombocytopenia l Urine organic acid

34 l Hydration l Glucose infusion l Bicarbonate

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

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

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

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

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

40 Phenylketonuria l Phenylalanine hydroxylase l Normal at birth l Mental retardation l Gradual onset l Vomiting

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

42 l Guthrie test l Phenylalanine l hrs l After protein feeding

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

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

45 l Feeding intolerance l Lethargy l Hypotonia l Posturing l Seizures

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

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

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

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

50 Case # 3 l 4-week-old presented fussy with decreased appetite l Cyanotic;does not respond to O 2 l Tachycardic l Grunting respiration l No hepatomegaly l Normal Chest X-ray

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

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

53 l 10-30%Cyanosis l 30-50%Tachycardia, fatigue l 50-70%Lethargy, stupor l >70%Death

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

55 l Chocolate brown blood l Pulse oximeter read 90s% l Normal PaO 2 despite cyanosis

56 l < 30%Not needed l 30-70%Methylene blue l No responseHyperbaric O 2 Exchange transfusion

57 l Methylene blue1 mg/kg IV l 10 ml 1% ampule (10 mg/ml) l Reduce methHb to hemoglobin l Maximum effect in 30 minutes

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

59 Case #4 l 7-day-old term infant l Poky eater; eats and stops l Crying & irritable after eating < 1 oz l Acts hungry & wants to eat again

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

61 Congenital Heart Defects

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

63 l VSD l AV canal malformation First month

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

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

66 Prostaglandin E 1 z 0.3 X Kg = Number of mg to be added in 50 ml z 0.5 ml/hr will deliver 0.05 microgram/kg/min

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

68 l Apnea l Bradycardia l Hypotension l Seizures l Hyperthermia

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

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

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

72 l Cyanosis l Tachypnea l Murmur may be absent l “Egg on a stick appearance”

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

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

75 l No murmur l Hepatomegaly l Metabolic acidosis

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

77 l Irritable l Crying-not consolable l HR 160, R 50, T 99 0 F l Intermittent grunting l O 2 saturation 97%

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

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

80 l 2 weeks to 6 months l Restlessness, irritability l Incessant crying l Dyspnea l Pallor & sweating (> feeding)

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

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

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

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

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

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

87 l Female l Enlarged clitoris l Labial fusion l Virilization

88 l Electrolytes l Low Na + l High K + l Glucose

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

90 l Fluid & Electrolyte replacement l Urgent l 20 ml/kg Normal Saline l Hydrocortisone l 25 mg IV bolus l 50 mg/m 2 /24 hours

91 l Hyperkalemia l Far better tolerated l Volume restoration l Insulin & glucose contraindicated

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

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

94 l Na 144 l K 6 l Cl110 l Urea 27

95 l pH7.45 l PCO 2 12 l HCO38 l Base deficit12 l Salicylate level98 mg/dl

96 Salicylate poisoning l Tachypnea & respiratory alkalosis l Metabolic acidosis l Fever l Seizure l Coma

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

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

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

100

101 l Target sign l Soft tissue mass with 2 concentric circles of fat density l Absence of cecal gas & stool l Loss of visualization of tip of liver l Paucity of bowel gas (RLQ) l Normal abdominal radiographs do not rule out intussusception

102 l 3 months to 6 years l 80% under 2 years l Male:female 4:1

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

104 l Fluid resuscitation is important l All patients are hypovolemic

105 l Barium enema l Diagnostic & therapeutic l Air enema l Increased success l Lower complications & radiation

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

107 Take home message l The “septic appearing infant” is septic until proven otherwise but think beyond! l Key to diagnosis is high index of suspicion l “Eyes can not see what the mind does not know”


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