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P74 Paediatrics Toronto Notes 2023

AUTOIMMUNE THROMBOCYTOPENIA

Pathophysiology

• caused by antiplatelet antibodies from maternal ITP or SLE

• passive transfer of anti-platelet antibodies across placenta

Clinical Features

• similar presentation to neonatal alloimmune thrombocytopenia, but thrombocytopenia is usually less

severe and does not tend to cause bleeding

Treatment

• steroids to mother for 10-14 d prior to delivery or I VIg to mother before delivery

• treat neonate with 1Vlg (usually if platelets <50000 or in the unlikely event of bleeding);otherwise

close monitoring for platelet recovery,bleeding

• transfusion of infant with maternal/donor platelets only in severe cases, as antibodies will destroy

transfused platelets

VITAMIN K DEFICIENCY BLEEDING

. see Vitamin K Deficiency, P52

Bronchopulmonary Dysplasia

Definition

. also known as chronic lung disease

• clinically defined as O:requirement for >28 d plus persistent need for oxygen and/or ventilatory

support at 36 wk corrected GA

• damage to developing lungs with prolonged intubation/ventilation, high levels O’

,infections

Investigations

• CXR findings may demonstrate decreased lung volumes, areas of atelectasis,signs of inflammation,

and hyperinflation

Treatment

• no clearly effective treatments

• gradual wean from ventilator, optimize nutrition

• dexamethasone may help decrease inflammation and encourage weaning, but use ofdexamelhasone

is associated with increased risk of adverse neurodevelopmental outcomes

Prognosis

• chronic respiratory failure may lead to pulmonary HTN, poor growth, and right-sided heart failure

• patients with bronchopulmonary dysplasia may continue to have significant impairment and

deterioration in lung function late into adolescence

• some lung abnormalities may persist into adulthood including airway obstruction, airway hyperreactivity, and emphysema

• associated with increased risk of adverse neurodevelopmental outcomes

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P75 Paediatrics Toronto Notes 2023

Cyanosis

Cyanosis

I

r

Peripheral li e - I

'

-

1

1 I I 1

Transient (typical) Sepsis or Deoxygenated

temperature hemoglobin

instability |

Abnormal hemoglobin

I

; T

Cardiovascular

*

(CHD,PPHN)

Respiratory Hematologic Sepsis Methemoglobinemia Carboxyhemoglobinemia

(polycythemia)

Neurologic

1

T I

Lower

(pneumonia,

macroglossia, airway MAS,

hypoplasia,laryngeal pneumothorax,

web/cyst, diaphragmatic hernia,

foreign body) hypoplasia)

Figure 14. Approach to neonatal cyanosis

CNS(asphyxia,

hemorrhage,

seizure,

opioids/sedatives)

Neuromuscular

(myasthenia gravis,

botulism)

Upper

(choanal atresia,

Management

•ABCis

elevated CO2 suggests respiratory cause

hyperoxia test (to distinguish between cardiac and respiratory causes of cyanosis): get baseline

Pa02 in room air, then Pa02 on 100% 02 for 10-15 min

Pa()2 < 150 mmHg: suggests cyanotic CHD or possible PPHN (see Cardiology and Cardiac

Suruerv. 09)

Pa02 >150 mmHg:suggests cyanosislikely due to respiratory or non-cardiac cause

•CXR:look for respiratory abnormalities (pneumothorax, respiratory tract malformations, evidence of

shunting, pulmonary infiltrates) and cardiac abnormalities (cardiomegaly, abnormalities of the great

vessels)

Diaphragmatic Hernia

•see General and Thoracic Surgery. CiS73

Definition

•developmental defect of the diaphragm with herniation of abdominal organs into thorax

•associated with pulmonary hypoplasia and PPHN

Clinical Features

•respiratory distress, cyanosis

•scaphoid abdomen and barrel-shaped chest

•affected side dull to percussion and breath sounds absent, may hear bowel sounds instead

•heart sounds shifted to contralateral side, if left sided diaphragmatic hernia

•asymmetric chest movements, trachea deviated away from affected side

•may present outside of neonatal period

•often associated with other anomalies (cardiovascular, neural tube defects, chromosomal

abnormalities)

•CXR for diagnosis

•CXR: bowel loops in thorax (usually left side), displaced mediastinum

Treatment

•immediate intubation required at birth: DO NOT bag mask ventilate because air will enter stomach

and further compress lungs

•place large bore orogastric tube to decompress bowel

•initial stabilization and management of pulmonary hypoplasia and PPHN if present, hemodynamic

support and surgery when stable j

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P76 Paediatrics Toronto Notes 2023

Hypoglycemia

Definition

• glucose <2.6 mmol/L within 72 h of birth OR <3.3 mmol/ L after first 72 h of life

Etiology

• decreased carbohydrate stores: premature, SGA, RDS, maternal HT'

N

• endocrine: hormonal deficiencies (GH, cortisol, epinephrine),insulin excess (infant of diabetic

mother, LGA,Beckwith-YViedemann syndrome/islet cell hyperplasia), hypothalamic-pituitary-adrenal

axis suppression (panhypopituitarism)

• inborn errors of metabolism:fatty acid oxidation defects, galactosemia

• miscellaneous:sepsis, hypothermia, polycythemia, perinatal stress(e.g. asphyxia)

Clinical Findings

• signs often non-specific and subtle:lethargy, poor feeding, irritability, tremors, apnea, cyanosis,

seizures

Management

• identify and monitor infants at risk (pre-feed blood glucose checks) until blood glucose stable and for

at least 12 h (for infant of diabetic mother or LGA) or 36 h (if preterm or SGA)

• in a well at-risk infant, begin oral feeds as soon as possible after birth and ensure regular feeds, check

glucose at 2 h of age

• if significant and/orsymptomatic hypoglycemia, provide glucose IV (D10YV ) and titrate according to

blood sugar levels

• if persistent hypoglycemia (>48 h of life), prolonged glucose IV,severe symptomatic hypoglycemia

(coma, lethargy,seizure), or no predisposing cause,send “critical blood work* during an episode of

hypoglycemia: ABG, ammonia, p-hydroxybutyrate,cortisol, free fatty acids, GH, insulin, lactate,

urine dipstick for ketones

Neonatal Hyperbilirubinemia

Definition

• total serum bilirubin >95th percentile (high-risk zone) on Bhutani nomogram in infants >35 wk GA

Clinical Features

• jaundice typically visible at serum bilirubin levels of 85-120 pmol/L

• visual assessment is misleading, confirm jaundice with blood test

Hyperbilirubinemia

Jaundice is very common - 60N of term

T newborns develop visible jaundice Unconjuyated Conjugated

1

t 1

Pathologic Physiologic Always pathologic

Jaundice in the first 24 h of life and

conjugated hyperbilirubinemia are

always pathological

1 1

Non-Hemolytic

Hematoma

(cephalohematoma)

Polycythemia

Sepsis

Hypothyroidism

Gilbert syndrome

Crigler-Najjar

Hemolytic Hepatic

Infectious

Sepsis

Hep B, TORCH

Metabolic

Galactosemia

Tyrosinemia

a- 1-antitrypsin deliciency

Hypothyroidism

Post-Hepatic

Biliary atresia

Choledochal cyst £

Intrinsic

Membrane

Spherocytosis

Elliptocytosis

Enzyme

G6PD deliciency

PK deficiency

Hemoglobin

Thalassemia

Extrinsic

Immune

ABO incompatability

Rh incompatability

Kell, Dully, etc.

Non-immune

Splenomegaly

Sepsis

AVM

Jaundice must be investigated if:

• It occurs within 24 h of birth

• Conjugated hyperbilirubinemia is

present

• Unconjugated bilirubin rises rapidly

or is excessive for patient's age and

weight

• Persistent jaundice lasts beyond 1-2

wk of life

CF

Drugs

TPN

Idiopathic neonatal hepatitis

Figure 15. Approach to neonatal hyperbilirubinemia

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PHYSIOLOGIC JAUNDICE

Epidemiology

• term infants: onset 3-4 d of life, resolution by 10 d of life

• premature infants: higher peak and longer duration

Pathophysiology

• increased hematocrit and decreased RBC lifespan

• immature glucuronvl transferase enzyme system (slow conjugation of bilirubin)

• increased enterohepatic circulation

Breastfeeding Jaundice

• common: due to a lack of milk production -> dehydration > exaggerated physiologic jaundice

Breast Milk Jaundice

• I in 200 breastfed infants

• glucuronyl transferase inhibitor found in breast milk

• onset 7 d of life, peak at 2-3 wk of life, usually resolved by 6 wk

Table 34. Risk Factors for Jaundice

Maternal Factors Perinatal Factors Neonatal Factors

Ethnic group|e.g. Asian, Indigenous) Birth trauma (cephalohematoma.

ecchymoses)

Complications during pregnancy (infant of Prematurity

diabetic mother. Rh or ABO incompatibility)

Breastfeeding

FMHx/previous child required phototherapy

Difficulty establishing breastfeeding

Infection (sepsis,hepatitis)

Genetic factors

Polycythemia

Drugs

TPK

Table 35. Causes of Neonatal Jaundice by Age

<24 h 24-72 h 72-96 h Prolonged (>1wk)

ALWAYSPATHOLOGIC

Hemolytic

Physiologic,polycythemia

Dehydration

(breastfeeding jaundice)

Hemolysis

G6PD deficiency

Pyruvate kinase deficiency

Spherocytosis

Bruising,hemorrhage,

hematoma

Sepsis/congenital infection

Physiologic * breastfeeding

Sepsis

Breast milk jaundice

Prolonged physiologic jaundice

in preterm

Hypothyroidism

Neonatal hepatitis

Conjugation dysfunction

c.g. Gilbert syndrome.

Crigler-Najjar syndrome

Inborn errors of metabolism

e.g. galactosemia

Biliary tract obstruction

e.g. biliary atresia

Rh or ABO incompatibility

Sepsis

Congenital infection (TORCH)

Severe bruising/hemorrhage

PATHOLOGIC JAUNDICE

• all cases of conjugated hyperbilirubinemia;some cases of unconjugated hyperbilirubinemia are

pathologic

Investigations

• unconjugated hyperbilirubinemia

« hemolytic workup:CBC, reticulocyte count, blood group (mother and infant), peripheral blood

smear,Coombs test ( DAT)

• if babv is unwell or has fever:septic workup (CBC and differential, blood and urine cultures ± LP,

CXR)

other:G6PD screen (especially in males), TSH

• conjugated hyperbilirubinemia must be investigated without delay

• consider liver enzymes(AST, ALT), coagulation studies (FT, P I T'

),serum albumin, ammonia,

ISH, TORCH screen,septic workup, galactosemia screen (erythrocyte galactose-1-phosphate

uridyltransferase levels), metabolic screen, abdominal U/S, H1DA scan,sweat chloride

• predicting occurrence ofsevere hyperbilirubinemia

measure either total serum bilirubin (TSB) or transcutaneous bilirubin (TcB) concentration in

all infants between 24 h and 72 h of life and plot on appropriate hyperbilirubinemia treatment

graph. If infant does not require immediate treatment, resultsshould be plotted on predictive

nomogram to determine the risk of progression to severe hyperbilirubinemia and need for repeat

measurement (refer to: http://www.cps.ca/documents/position/hyperbilirubinemia-newborn)

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TREATMENT OF UNCONJUGATED HYPERBILIRUBINEMIA

• to prevent kernicterus

• breastfeeding does not usually need to be discontinued, ensure adequate feeds and hydration

• lactation consultantsupport, mother to pump after feeds

• treat underlying causes (e.g. sepsis)

• phototherapy (blue-green wavelength, not U V light);standard intensive or multiple intensive protocol

depending on severity of hyperbilirubinemia

insoluble unconjugated bilirubin is converted to excretable form via photoisomerization

serum bilirubin should be monitored during and immediately after therapy (risk of rebound

because photoisomerization is reversible when phototherapy is discontinued)

contraindicated in conjugated hyperbilirubinemia; results in “bronzed'

baby

side effects:skin rash, diarrhea, eye damage (eye shield used routinely for prevention),

dehydration

use published guidelines and nomogram (see Figure 16) to determine appropriateness of

phototherapy by stratifying infant risk and assessing if total scrum bilirubin level is above cutoff

for respective gestational age

• exchange transfusion

indications: high bilirubin levels as per published graphs based on age, wk gestation

most commonly performed for hemolytic disease and severe cases of G6PD deficiency

use of I Vlg in case ofsevere hyperbilirubinemia (DAT+) becoming evidence-based practice

“Bronzed” Baby in Infants with

Conjugated Hyperbilirubinemia

Phototherapy results In the production

and accumulation of a lode metabolite

which also imparts a bronze hue on the

baby's skin

/Toni SxumR'

iudt

*

povl >

P

Age

— Intents atlower nsk (>38wk and well

- - Inter.ts at medium risk ( 28wk -t- risk factors or

3M/ V/ wt and well)

^—Intents at higher risk|3»3/VI wk nsk factors)

j

Figure 16. Gold standard in deciding

when to initiate phototherapy for

unconjugated hyperbilirubinemia

Licence number: 4601410094382

KERNICTERUS

Etiology

• unconjugated bilirubin concentrations exceed albumin binding capacity and bilirubin is deposited in

the brain resulting in tissue necrosis and permanent damage (typically basal ganglia or brainstem)

• incidence increases as serum bilirubin levels increase above 340 pmol/ L

• can occur at lower levels in presence ofsepsis, meningitis, hemolysis, hypoxia, acidosis, hypothermia,

hypoglycemia, and prematurity

Clinical Features

• up to 15% of infants have no obvious neurologic symptoms

• early stage:lethargy, hypotonia, poor feeding, emesis (acute bilirubin encephalopathy)

• mid stage: hypertonia, high pitched cry,opisthotonic posturing (back arching), bulging fontanelle,

seizures, pulmonary hemorrhage

• late stage (during first year and beyond): hypotonia,delayed motor skills, extrapyramidal

abnormalities (choreoathetoid CP), gaze palsy, mitral regurgitation,sensorineural hearing loss

Prevention

• exchange transfusion,1Vlg if indicated

BILIARY ATRESIA

Definition

• atresia of the extrahepatic bile ducts which leads to cholestasis and increased conjugated bilirubin

after the first wk of life

• progressive obliterative cholangiopathy

Epidemiology

. incidence: I in 10000-15000 live births

• associated anomalies in 10-35% of cases:situs inversus, congenital heart defects, polysplenia

Clinical Features

• dark urine, pale stool, jaundice (persisting for >2 wk), abdominal distension,hepatomegaly

Diagnosis

• conjugated hyperbilirubinemia, abdominal U/S, operative cholangiogram

• HIDA scan (may be bypassed in favour of biopsy if timing of diagnosis is critical)

• liver biopsy

Treatment

• surgical drainage procedure

• hepatoportoenterostomy ( Kasai procedure; most successful if age <8 wk)

• two-thirds will eventually require liver transplantation

• vitamins A,D, E, and K; dietshould be enriched with medium-chain triglycerides to ensure adequate

fat ingestion

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Necrotizing Enterocolitis

Influence ol EnteralMutrition on Occurrences

of Recrotizing Enterocolitisin Very-tow BirtliIfcight Infants

J Pedatr Gastroenterol Nutr 2015:61(4):445 450

Study:Case-controlstudy of wry-low-birth-weiglit

(VlBI)nlantsand occurrences of HECwrtlr in 30

dotMi.

Population:1028 VLBW infantsin neonatal intensive

care unit Jan 2003-Hay 2008.

Primary Outcome: NEC defined using stage >2 of

modified Bell criteria.

Resmlts:55 infantsdeveloped MEC with in 30 d of life

(5 4%) lltose mtii NEC had higher odds ol having

been fed breast milk <7 d (OS:4.02|, not harmg

achieved full enteralfeeding during the first oin (OR:

3.S0).and having had parenteral feeding (Of: 2.10).

Conclusions Occurience ol NEC can be reduced

with breast nilk feeding beyond 7d and early full

enteralfeeding.

Definition

• intestinal inflammation associated with focal or diffuse ulceration and necrosis

• primarily affecting terminal ileum and colon

Epidemiology

• affects 1 -5% of preterm newborns admitted to NICU

Pathophysiology

• postulated mechanism of bowel ischemia: mucosal damage and enteral feeding > bacterial growth >

bowel necrosis/gangrene/perforation

Risk Factors

• prematurity (immature defenses)

• asphyxia,shock (poor bowel perfusion)

• hyperosmolar feeds

• enteral feeding with formula (breast milk can be protective)

• sepsis

Clinical Features

• usually presents at age 2-3 wk

• distended abdomen, diminished bowel sounds, feeding intolerance

• increased amount of gastric aspirate/vomitus with bile staining

• frank or occult blood in stool

• signs of bowel perforation (sepsis,shock, peritonitis, DIC)

Investigations

• AXR: pneumatosis intestinalis (intramural air is a hallmark of NEC),free air, fixed loops, ileus,

thickened bowel wall, portal venous gas

CBC, ABG,lactate, blood culture,electrolytes

• high or low WBC, low platelets, hyponatremia, acidosis, hypoxia, hypercapnia

Treatment

• NPO (7-10 d), vigorous IV fluid resuscitation, decompression with NG tube,supportive therapy

. TPN

• antibiotics(usually ampicillin, gentamicin ± metronidazole if risk of perforation x 7-10 d)

• serial AXRs detect early perforation (40% mortality in perforated NEC)

• peritoneal drain/surgery if perforation

• surgical resection of necrotic bowel and surgery for complications(e.g. perforation,strictures)

Persistent Pulmonary Hypertension of the Newborn

Definition

• persistence of fetal circulation as a result of persistent elevation of pulmonary vascular resistance

• classified as primary (absence of risk factors) orsecondary

Epidemiology

• incidence 1.9 in 1000 live births

Clinical Features

• usually presents within 12 h of birth with severe hypoxemia/cyanosis;some may have only mild

respiratory distress

Pathophysiology

• elevated pulmonary pressures cause R -> L shunt through PDA,foramen ovale -> decreased pulmonary

blood flow and hypoxemia -> further pulmonary vasoconstriction

Risk Factors

• secondary PPHN: asphyxia, meconium aspiration syndrome, RDS,sepsis, pneumonia,structural

abnormalities(e.g.diaphragmatic hernia, pulmonary hypoplasia)

• more common in term or post-term infants

Investigations

• measure pre- and post-ductal oxygen levels

• hyperoxia test to exclude CHD

. EGG (RV strain)

• echo reveals increased pulmonary arterial pressure and a R -> L shunt across PDA and patent foramen

ovale; also used to rule out other cardiac defects

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Treatment

• maintain good oxygenation (SaO * >95%) inat-risk infants

• 02 given early and tapered slowly,minimize stress and metabolic demands,maintain normalblood

gases, circulatory support

• mechanical ventilation,high frequency oscillation in a sedated muscle-relaxed infant

• nitric oxide, surfactant

• extracorporeal membrane oxygenation used in some centres when other therapy fails

Respiratory Distress in the Newborn

Clinical Features

• tachypnea:RR >60/min;tachycardia:HR >160/min

• grunting, subcostal/intercostal indrawing, nasal flaring

• duskiness,central cyanosis

• decreased air entry, crackles on auscultation

Differential Diagnosis of Respiratory Distress

• see Table 33 under S'eonatal Resuscitation, P72

Investigations

• labs: CBC,blood gas,glucose,blood culture

• imaging:CXR

• ifindicated: ECG, echo, LP (CSF cell count, culture,and chemistry)

Table 36. Distinguishing Features of RDS, TTN, MAS

RDS TTN MAS

Etiology Surfactant deficiency *

pool lung

compliance due to high alveolar surface fluid •»accumulation offluid in

tension -» atelectasis -»

*

surface area for gas exchange

*

hypoxia acidosis *

respiratory

distress

“Hyaline membrane disease*

Preterm

Maternal DM

Preterm delivery

Male sex

Delayed resorption of fetal lung Meconium is sterile but causes airway

obstruction,chemical inflammation,and

peribronchial lymphatiesand surfactant inactivation leading lochemical

vascular spaces <

*

tachypnea pneumonitis

"Wet lung syndrome"

Gestational Age

Risk Factors

Term and post-term

Meconium-stained amniotic fluid

Post- term delivery

Usually term and late preterm

Maternal DM

Maternal asthma

Male sex

Macrosomia (> 4500 g)

Elective Cesarean section or

short labour

late preterm delivery

Clinical Features Respiratory distress within first few Tachypnea within the first few

hours ol life,worsens over next 24-72 It hours of tile tretractions.

Hypoxia

Cyanosis

CXR Findings Homogenous infiltrates

Airbronchograms

Decreased lung volumes

May resemble pneumonia|GBS)

If severe,"white-out" withno

differentiation of cardiac border

Prenatal corticosteroids (e.g.

Celcstone 12 mg q24 h x 2 doses)il

risk of preterm delivery

*

34 wk

Monitor lecilhin:spliingomyehn IT'S)

ratio with amniocentesis,L/S >2:1

indicates lung maturity

Resuscitation

Oxygen

Ventilation

Surfactant (decreases alveolar surface IV fluids and HG lube feeds if too

tension,improves lung compliance,and tachypneic to feed orally

maintains functional residual capacity)

IBW

Acidosis,sepsis

Hypothermia

Second born twin

Respiratory distress within houis ol birth

Small airway obstruction, chemical pneumonitis

tachypnea,barrel chest with audible crackles

Hypoxia

Hyperinflation

Patchy atelectasis

Patchy and coarse Infillralcs

10- 20% have pneumothorax

grunting,nasal flaring

Often HO hypoxia or cyanosis

Perihilar infiltrates

"Wet silhouette;" fluid in

fissures

Prevention Where possible,avoidance of

elective Cesarean delivery,

particularly before 38 wkGA

If infant isdepressed at birth,intubate and

suction below vocal cords

Avoidance of factors associated within utero

passage of meconium (e.g.post-term delivery)

Supportive

Oxygen if hypoxic

Ventilator support|e.g. CPAP)

Resuscitation

Oxygen

Ventilatory support

Surfactant

Inhaled nitric oxide

Extracorporeal membrane oxygenation for

PPHN

Hypoxemia

Hypercapnia

Acidosis

PPHN

Pneumothorax

Pneumomediastinum

Chemical pneumonitis

Secondary surfactant inhibition

Respiratory failure

Dependent on severity,mortality up to 20%

Treatment

Complications In severe prematurity and/or Hypoxemia

prolonged ventilation, increased risk of Hypercapnia

bronchopulmonary dysplasia Acidosis

PPHN r1

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Prognosis Dependent on GA at birth and severity Recovery usually expected in

of underlying lung disease:long-term 24-72h

risks of chronic lung disease

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PNEUMONIA

• see Respirolog)'

, P93

• consider in infants with prolonged (>18 h) or premature rupture of membranes, maternal fever or

other signs and symptoms of chorioamnionitis, or if mother is GBS positive

• suspect if infant exhibits respiratory distress, temperature instability,or WBC islow (<5

elevated (>30 x 109/L),or left-shifted

• symptoms may be non-specific (e.g. lethargy, apnea, tachycardia, poor perfusion, poor feeding)

• investigations: CXR (hazy lung and/or distinct infiltrates, may be difficult to differentiate from KDS),

blood and CSF cultures

• neonates with pneumonia should be admitted to the NICU and given empiric antibioticsfor

management

x 109/L),

Retinopathy of Prematurity

• see Ophthalmology.OP40

Sepsis in the Neonate

Table 37. Sepsis Considerations in the Neonate (Sfy

Chronic Perinatal Infections Early Onset (<72 h) Late Onset (72 h • 28 d)

Acquired after birth CHEAP TORCHES

Risk factors:preterm infants in NICU

Pathogens:coagulase-negaliveStaphylococcus most common,

GBS.anaerobes,E.coli,Klebsiella

Vertical transmission,95%present within 24 halter birth

Risk factors:

Maternal infection:UTI,GBS positive,previous child with GBS,

sepsis,or meningitis

Maternal fever/leukocytosis/chorioamnionitis

Prolonged rupture of membranes|»18h)

Preterm labour

Pathogens:GBS,E.coli,Listeriaare most common

Pneumonia morecommon withearly-onset sepsis

Chicken pox/shingles

Hepatitis B/C/D/E

Enteroviruses

AIDS (HIV)

Parvovirus B19 (erythema infectiosum)

Toxoplasmosis

Other

Rubella virus

Cytomegalovirus/Coxsackievirus

Hb .

Clinical Features

• no reliable absolute indicator of occult bacteremia in infants <3 mo, most specific result has been

WBC<5

*

10 "

/ 1.

• temperature instability (hypo/hyperthermia)

• respiratory distress, cyanosis, apnea

• tachycardia/bradycardia

• lethargy,irritability

• poorfeeding, vomiting,abdominal distension, diarrhea

• hypotonia,seizures,lethargy

• jaundice, hepatomegaly, petechiae, purpura

Investigations

• suspicion of neonatal sepsis requires "full septic workup"

CBC, blood and urine cultures, LP (CSI:

analysis: cell count, glucose, protein, culture, and PCR for

viruses) ± CXR

LP must be conducted if blood culture is positive due to increased risk of meningitis

Management

• supportive care

• IV antibiotics:typically ampicillin + cefotaxime or ampicillin + gentamicin chosen asfirst-line

empiric therapy

• choice of antibiotic and duration of treatment dependent on symptoms, culture results, maternal GBS

status, and local resistance patterns

• if meningitissuspected, consider IV ampicillin + cefotaxime ± vancomycin at meningitic doses

• addition of IV acyclovir if HSV infection suspected

Eveiy STI

Syphilis

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Skin Conditions of the Neonate

Table 38. Common Neonatal Skin Conditions

Neonatal Skin Condition Description

Vasomotor Response Iransienlmottling when exposed tocold:usually normal, particulaity il premature

(Cutis Marmorata, Acrocyanosis)

VernixCaseosa

Congenital DermalMelanocytosis

Soft,creamy, white layer covering baby at birth

Sidle gicy patches over lower back, bullocks,and lower limbs (may look like bruises):prevalence

varies with ethnicity (Asian * Black *

Hispanic

’

While): typically lades within first 2 yr of life

Capillary Hemangioma Raised red lesion,which increases in sire after birth and involutes:SONresolved by S yr.90% by

9 yr

Erythema loxicum Neonatorum Yellow-while papules/pustules surrounded by erythema,eosinophils within the lesions:common

rash,resolves in 5-7 d

12 mm lirm while pearly papules on nasal bridge,cheeks,and palate: sell-resolves within llrsl few

weeks of life

Hyperpigmented macular base withpustules,seen more commonly inBlack infants:maybe present

at birth:no treatment needed

Transient macular vascular malformation olthe eyelids and/or neck (“Angel Kiss" or "Stork Bile"):

most lesions disappear by1yr

Inflammatory papules and pustules mainly on face:sell-resolving usually within 4 mo

Milia

Transient Pustular Melanosis

Nevus Simplex (SalmonPatch)

Neonatal Acne

Nephrology

Common Paediatric Renal Diseases

Table 39. Common Manifestations of Renal Disease

Age Symptoms Common Causes

flank Mass Hydronephrosis,polycystic disease lautosomal dominant or recessive subtypes),tumour

Renal vein thrombosis,asphyxia,mallormallon, trauma

Neonate

Hematuria

Anuria/Oliguria Bilateral renal agenesis,obstruction,asphyxia

Child and

Adolescent

Cola/Red Coloured Urine Acute GN (e.g. post streptococcal.HSP.IgA nephropathy, etc.),hemoglobinuria (hemolysis),

myoglobinuria (rhabdornyolysls)

Gross Hematuria Utologic disease (e.g. nephrolithiasis,trauma, etc.),till,acute GN

Edema Nephrotic syndrome,nephritis,acute/chronic renal failure,consider cardiac or liver disease

Hypertension GN. renal failure,dysplasia (consider coarctation,drugs,endocrine causes)

Polyuria DM, central and nephrogenic Dl,renal fanconi's syndrome (genetic/metabolic/acguired

causes),hypercalcemia,polyuric renal failure (renal dysplasia)

Proteinuria Orthostatic,nephrotic syndrome (MCD.etc.),GN

Oliguria Dehydration. AMI.interstitial nephritis,acute or chronic kidney disease (i.e.renal failure)

Urgency UII,vulvovaginitis

Hemolytic Uremic Syndrome

Definition

• simultaneous occurrence of the triad of:

1. non -immune microangiopathic hemolytic anemia

2. thrombocytopenia

3. acute renal injury

L J

Epidemiology

• annual incidence of 1 -2 in IOOOOO in Canada

• most common cause of acute renal failure in children +

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P83Paediatrics Toronto Notes 2023

Etiology

• STEC-HUS:90% of paediatric HUS; caused by Shiga toxin-producing E.coli (usually 0157:H7)

• atypical HUS: 10% of paediatric HUS;caused by hereditary mutations in complement pathway, nonShiga toxin infections (e.g.S. pneumoniae, HIV),and drugs (cisplatin, oral contraceptives, cyclosporin

tacroliumus, and others)

Pathophysiology

• toxin binds, invades, and destroys colonic epithelial cells, causing bloody diarrhea

• toxin enters the systemic circulation, attaches to,and injures endotheliai cells (especially in the

kidney), causing a release of endothelial products(e.g. von Willebrand factor, platelet aggregating

factor)

• platelet/fibrin thrombi form in multiple organ systems (e.g. kidney, pancreas, brain) resulting in

thrombocytopenia

• RBCs are forced through occluded vessels, resulting in fragmented RBCs (schistocytes) that are

removed by the reticuloendothelial system (hemolytic anemia)

Clinical Features

• initial presentation of abdominal pain and diarrhea, followed by bloody diarrhea; within 5-7 d begins

to show signs of anemia,thrombocytopenia, and renal insufficiency

• pallor, jaundice (hemolysis), edema, petechiae. HTN,decreased urine output

Investigations

• CBC (anemia, thrombocytopenia), blood smear (schistocytes), electrolytes (due to fluid loss), renal

function, urinalysis(microscopic hematuria),stool cultures,and verotoxin/shigella toxin assay

Management

• mainly supportive: nutrition, hydration, ventilation (if necessary), blood products

• dialysis ifsymptomatic uremia, refractory electrolyte abnormality (e.g.hyperkalemia), or severe fluid

overload

• STEC-HUS: avoid antibiotics, NSAlDs, and antidianheal agents; no treatments associated with

improved outcomes

• atypical HUS:plasma exchange or eculizumab

Prognosis

• STEC-HUS: <5% mortality rate, 30% develop long-term renal damage (e.g. HTN,proteinuria,

decreased GER)

• atypical HUS: worse prognosis compared to STEC-HUS, 50% of cases result in death or dialysisdependent renal disease

Nephritic Syndrome

(§s Definition

• acute or chronic syndrome affecting the kidney, characterized by glomerular injury'and inflammation

• defined by hematuria (>5 RBCs per high-powered microscope field),presence of dysmorphic RBCs,

and RBC casts on urinalysis

• often accompanied by at least one of: proteinuria (<50 mg/kg/d), edema, HTN, azotemia, and oliguria

Epidemiology

• highest incidence in children ages 5- 15 vr

Etiology

• humoral immune response to a variety of etiologic agents -

> immunoglobulin deposition >

complement activation, leukocyte recruitment, release of growth factors/cytokines -> glomerular

inflammation and injury -> porous podocytes -> hematuria + RBC casts ± proteinuria

• HTN secondary to fluid retention and increased renin secretion by ischemic kidneys

Nephritic Syndrome

PHAROH

Proteinuria (<50 mg/kg/d)

Hematuria

Azotemia

RBC casts

Oliguria

HTN

Table 40,Major Causes of Nephritic Syndrome

Decreased C3 Normal C3

Primary

(idiopathic)

Post-infectious 611(streptococcal infectionis IgA nephropathy

the most common)

Membranoproliferathre

TypeI(SO-80%)

Type II(>80%)

Idiopathic rapidly progressive GN

Anti-EBM disease

SLE HSP(very common)

Polyarteritis nodosa

Granu!omalos:s with polyangiitis

Goodpasture'

s syndrome

Secondary

(systemic disease) Bacterial endocarditis

Abscess or shuntnephritis

Cryoglobulinemia

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P81Paediatrics Toronto Notes 2023

Clinical Features

• often asymptomatic;some overlap in clinical findings for nephritic and nephrotic syndrome

• gross hematuria, mild-moderate edema, oliguria, HTN

• signs and symptomssuggestive of underlying systemic causes (e.g.fever, arthralgias,rash,dyspnea,

pulmonary hemorrhage)

Investigations

• urine

dipstick (hematuria,0 to 2+ proteinuria) and microscopy (>5 RBCs per high-powered microscope

field,acanthocytes,RBC casts)

first morning urine protein/creatinine ratio (<200 mg/mmol)

• blood work

CBC, electrolytes,Cr,BUN, albumin

impaired renalfunction ( Cr and BUN) resulting in t pH and electrolyte abnormalities

(hyperkalemia, hyperphosphatemia, hypocalcemia)

mild anemia on CBC (secondary to hematuria)

hypoalbuminemia (secondary to proteinuria)

appropriate investigations to determine etiology:C3/C4 levels,serologic testing for recent

streptococcal infection (ASOT, anti-hyaluronidase, anti-streptokinase,anti-NAD,anti-DNAse B),

ANA,anti-DNA antibodies, ANCA,serum IgA levels, anti-GB.Vl antibodies

• renal biopsy should be considered only in the presence of acute renal failure, no evidence of

streptococcal infection, normal C3/C4

Management

• treat underlying cause

• symptomatic

renal insufficiency:supportive (dialysisif necessary), proper hydration

HTN:salt and fluid restriction (but not at expense of renal function),ACEI or ARBs for chronic

persistent HTN (not acute cases because ACEI or ARBs may decrease GFR further)

edema:salt and fluid restriction, possibly diuretics (avoid ifsignificant intravascular depletion)

• corticosteroidsifindicated:IgA nephropathy, lupus nephritis, etc.

• post-streptococcal GN should be monitored for complications long term (annual BP,urinalysis)

Prognosis

• dependent on underlying etiology

• complications include HTN, heart failure, pulmonary edema,chronic kidney injury (requiring renal

transplant)

Nephrotic Syndrome

Definition

• clinical syndrome affecting the kidney, characterized by significant proteinuria, peripheral edema,

hypoalbuminemia, and hyperlipidemia

Epidemiology

• highest incidence in children 2-6 yr, M>1

;

Etiology

• primary (idiopathic):nephrotic syndrome in the absence ofsystemic disease (most common cause in

paediatrics)

glomerular inflammation ABSENT on renal biopsy:MCD (85%),focal segmental

glomerulosclerosis

glomerular inflammation PRESENT on renal biopsy: membranoproliferative GN,IgA

nephropathy

• secondary':nephrotic syndrome associated with systemic disease or due to another process causing

glomerular injury (<10% in paediatrics)

autoimmune:SLE,DM, juvenile idiopathic arthritis

• genetic sickle cell disease,Alportsyndrome

infections:hepatitis B/C,post-streptococcal, infective endocarditis, HUS,HIV

malignancies:leukemia,lymphoma

medications:captopril, penicillamine, NSAIDs,antiepileptics

vasculitides:HSP,granulomatosis with polyangiitis

• congenital:congenital nephropathy of the Finnish type, Denys-Drash syndrome,etc.

<§)

Nephrotic Syndrome - HELP

Hy poalbuminemia (<20 g/L)

Edema

Lipids elevated

Proteinuria (>50mg/kg/d)

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