Some Algorithms in Pediatrics

8/4/2019 Some Algorithms in Pediatrics

1/15

Consulting Child Specialist

Neonatal Intensivist


2/15

Algorithm: Child with bleeding

Hx, PE

PT,APTT Platelets Bleeding Time

Evaluatecount &function

EvaluateBT

Considerrepeatevaluationfor VWD

Platelet aggregationstudies (ADP,collagen,

ristocetin, epi,arachidonic acid)

Thrombin clotting time(TCT)

VWF functionAntigen cofactor

Mixing

studies1:1 Pt.:pooledplasma

Contaminn

withheparin orthrobolytic

agent

N (or

ristocetin abN)

Glanzmann thrombo

Drug effect (aspirin)

Storage pool def.

Specificfactorinhibitor

APLsyndrome

Specificfactor

assays

8,9,3, vit.k,

Isolated

factorsVWD

Fibrinogen

Normal Abnormal

Bernard Soulier

Uremia

VWD

Confirm with:

Multimer analysis

Family studies FDP Consider

Dys/A/Hyp

fibrinogrenDIC

N AbN

N AbN

N AbN N

AbN

AbN

Not corrected Corrected - ve

N AbN


3/15

Approach for isolated thrombocytopenia

Hx, PE, CBC Differential Dx, PS, Platelets

Assess platelet morphology

Assess forsplenomegaly or

lymphadenopathy

Bernard-Soulier

Wiscott-Aldrich

May-Hegglin anomaly

Glanzman thromboaGray platelet syndrome

Bone Marrowaspirate

Normal Examination CongenitalAnomalies

Malignancy

Infection HIV

Storage disease

Hypersplenism

ITP

Viral inducedDrug inducedHIVCollagen vascular

InfantHemangiomaAplastic anemia,Fanconi anemiaFamilial

Skeletal

Fanconi anemia

TAR

Cyanotic CHD

Eczema

Wiscott-Aldrich

Hemangiomas

Kassabach-Merritt

Abnormal

Positive

Negative

Normal


4/15

Diagnostic algorithm of hypoxia & hypercarbia

Hypoxemia, Hypercarbia

Respiratory effort? (respiratory distress)Yes No

Mechanical dysfunction of lungsor chest wall

Anatomy of respiratory drive or

respiratory muscle function:

Drug induced

(Opioids,barbiturates)

Brain stem injuryPolyneuritis

Myopathy

Tachypnes

Lung volumeRales & cracles

Prolonged insp

Or expi

Airway noises

Restrictive ds

Croup, FB

Epiglottitis

Obstructive ds

Stridor

Long inspi

Wheeze

Long expi, lung vol

Extra-thoracicObstruction

Croup

EpiglottitisForeign Body

Intra-thoracic

Obstruction

Asthma

BronchiolitisTracheo-bronchomalacia, FB


5/15

Guide to initial Mm of febrile neutropenic patient

Fever (>380C) & neutropenia (


6/15

Pathway for detection & referral of DDH in newborn

PE in newborn infant

Unstable Uncertain Stable

Reassessin 2 weeks

No riskfactors

Riskfactors,breech,

family Hx

Abnormalexam

Normalexam

Periodicfollow-up

US 3 mo

or

XR 6 mo

Abnormal exam Normal exam

Refer toPediatric

Orthopedist

Continueperiodic

follow-upuntil after

ambulation


7/15

Evaluation & Tx of Urinary Incontinence in children

Hx, PE Urinalysis

Nocturnal Enuresis Day & Night Incontinence

Maturation delay Continuous Intermittent

Age 5 yrs Dysfunctional

VoiderEnuretic alarm,Biofeedback,

DrugsNo Tx IVP/VCUG Anticholinergics,

Time voiding,Biofeedback

Refractory Child

>10 yrs age Ectopic Ureter(female)

Normal

Ultrasound

Abnormal Surgery Urodynamics

VCUGUrod namics

Normal NeuropathicBladder

DysfunctionalVoider


8/15

Approach to IEM causing chronic encephalopathy

Chronic Encephalopathy

Abnormalities outside the CNS?

Mainly graymatter

Seizures

Blindness

Dementia

Mainly white matter

Spasticity

Weakness

Ataxia

Muscle Skin/connectivetissue

RES

CNS only CNS & PNS

Mitochondrial(eg, CRSM syn.,Leigh ds,MELAS, Alperssyn)

Lysosomalstorage(eg, NCL, GM2)

OtherB6 dependency

Amino acidds

Organicacid ds

Lysosomalds(eg, GM1 late)

Peoxismalds

OtherCanavan

Alexander

Lysosomalstorage(eg, MLD, GLD)

Peroxismal

ds(eg, NALD, IRS)

Mitochondrial(myopathies)

Amino acidds(eg, homocyst)

Lysosomal

storage(eg,Mucolipidoses,MSD)

Dysmorphicsyndrome(eg, Menkes)

Lysosomalstorage(eg, MPS,Gaucher,NPD, GM1,

Sialosis)Peroxismalds(eg,Zellweger)

YesNo


9/15

Approach to IEM a/w metabolic acidosis

Metabolic Acidosis

What is the anion gap?

Normal Elevated

Renal HCO3-losses

Accumulation of fixed anion

Carbohydrate ds(eg, galactosemia)

Lysosomal storage(eg, cystinosis)

Amino acid ds(eg, hepatorenal tyrosenemia)

Mitochondrial

Urinaryorganic acids

Plasmaketones

Plasmalactate &pyruvate

Organic acid

ds(eg, propionicacidemia,methylmalonicacidemia)

Organic acid

ds(eg, 3-ketothilasedef., SCOT def, 20finding in other OAdiseases)

Oxphos ds /pyruvateoxidation (eg,PDH def., PC def.)

Organic acidds (eg, MCD)

Carbohydrateds (20 finding indisorders ofgluconeogenesis)


10/15


11/15

DDx of IEM a/w severe hypoglycemia

Hypoglycemia

Nonglucose-reducing substance in urine

Positive Negative

Carbo. Ds(eg, galactosemia,HFI)

Amino acid ds(eg, hepatorenaltyrosenemia)

Ketones Plasma aminoacids, NH3,

urine organicacids

Organic acidds(eg, propionicacidemia)

Amino acid ds(eg, MSUD)

Low High

Plasma FFA Hepatomegaly

High Low Present Absent

FAO ds

(eg, MCADdef)

Consider:

Hyperinsulism

Plasma lactate Plasma hGH,

cortisol, T4

High Normal Appropriate Abnormal

Carbo. Ds

(eg, GSDI,

FDPase def)

Consider:

Ketotic

hypoglycemia

Consider:

Ketotic

hypoglycemia

Consider:

Endocrinopathy


12/15

DDx of inborn errors of ureagenesis

Inborn Errors of Ureagenesis

Measure plasma citrulline

Absent/

trace

N/ Moderate

Marked

Urinary orotic acid Plasma arginine ASA in

plasma &urine

Normal Elevated Low/N Elevated

CPS 1 def

NAGS def

OTC def Argininemia Argininosucconoc

aciduria

Citrullinemia

Urinarylysine,ornithine,arginine

Plasmaornithine,urinaryhomocitr

ulline

LPI HHH


13/15

Approach to child with disproportionate short stature

Infant with disproportionate short stature

Examine segments

Short limb Short trunk

Rhizomelic Mesomelic Acromelic

Associated

findings

Associated

findings

Associated

findings

Associated

findingsSkeletalsurvey

Skeletalsurvey

Skeletalsurvey

Skeletalsurvey

Define X-raypattern

Spondylo/epiphyseal/diaphyseal

Define diagnostic possibilities

Make Dx


14/15

Approach to child with microcephaly

Child with microcephaly

Prenatal onset Postnatal onset

Measureparents

Do

ophthalmo

Small forgestational

age

Eye findings Minoranomalies

Seizures /neurological

signs

Multiplecontractures

d/tdeformities

Considerappropriatesyndromes

Do karyotype/ophthalmo

Dobiochemical

studies;CT/MRI/EEG

Considersyndromes

Signs of IUI

Doconfirmatorystudies

No diagnosis

Do CT/MRI


15/15

Treatment of Acute Severe Asthma

Airway, Oxygen, IV Fluids+ for Dehydration, Cardioscope

Inj. Hydrocortisone 10 mg/kg stat then 2.5-5 mg/kg/dose q6hInj. Methylpred 1-2 mg/kg q6h, IV/IM Dex 1-2 mg/kg q6h

Salbuta/Levo nebulization q20 min 3 or

Ipratropium neb. Q20 min 3, then q6h for 24-48 hrs

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Some Algorithms in Pediatrics