Consulting Child Specialist Neonatal Intensivist

Algorithm: Child with bleeding

PT,APTT N
Evaluate count & function Evaluate BT Consider repeat evaluation for VWD

Hx, PE Platelets AbN N AbN N

Bleeding Time AbN

Platelet aggregation studies (ADP,collagen, ristocetin, epi, arachidonic acid)

Thrombin clotting time (TCT) Mixing studies 1:1 Pt.: pooled plasma

VWF function Antigen cofactor

Contaminn AbN with heparin or throbolytic agent

N (or ristocetin abN)

AbN
Glanzmann thrombo Drug effect (aspirin) AbN Storage pool def. VWD Confirm with: Multimer analysis Family studies

Not corrected Specific factor inhibitor APL syndrome

Corrected

- ve
Fibrinogen

Specific factor assays 8,9,3, vit.k, Isolated factors VWD

Normal

Bernard Soulier Abnormal Uremia Consider Dys/A/Hyp fibrinogren

FDP

DIC

Approach for isolated thrombocytopenia

Hx, PE, CBC Differential Dx, PS, Platelets
Assess platelet morphology Normal Assess for splenomegaly or lymphadenopathy Positive Abnormal Bernard-Soulier Wiscott-Aldrich May-Hegglin anomaly Glanzman thromboa Gray platelet syndrome

Negative

Normal Examination

Congenital Anomalies

Bone Marrow aspirate

ITP Viral induced Drug induced HIV Collagen vascular Infant Hemangioma Aplastic anemia, Fanconi anemia Familial Skeletal Fanconi anemia TAR Cyanotic CHD Eczema Wiscott-Aldrich Hemangiomas Kassabach-Merritt

Malignancy Infection HIV Storage disease Hypersplenism

Diagnostic algorithm of hypoxia & hypercarbia

Hypoxemia, Hypercarbia
Respiratory effort? (respiratory distress)
Yes No Anatomy of respiratory drive or respiratory muscle function:

Mechanical dysfunction of lungs or chest wall

Tachypnes Lung volume Rales & cracles Prolonged insp Or expi Airway noises

induced (Opioids,barbiturates) Brain stem injury Polyneuritis Myopathy

Wheeze Long expi, lung vol Intra-thoracic Obstruction Asthma Bronchiolitis Tracheo-bronchomalacia, FB

Drug

Restrictive ds Croup, FB Epiglottitis

Obstructive ds
Stridor Long inspi Extra-thoracic Obstruction Croup Epiglottitis Foreign Body

Guide to initial Mm of febrile neutropenic patient

Fever (>380C) & neutropenia (<500/mm3)
Evaluate Is vancomycin needed? Indications
Severe

No indication

mucositis Quinolone prophylaxis Colonized with MRSA Pen/Ceph. resistant S. pneumoniae Obvious catheterrelated infection hypotension

Monotherapy

Duotherapy

Ceftazidime or Imipenem

Aminoglycoside plus antipseudomonal -lactam

Reassess after 3 days

Pathway for detection & referral of DDH in newborn

PE in newborn infant
Unstable Uncertain Reassess in 2 weeks Stable No risk factors Risk factors, breech, family Hx

Abnormal exam

Normal exam Periodic follow-up US 3 mo or XR 6 mo

Abnormal exam

Normal exam
Continue periodic follow-up until after ambulation

Refer to Pediatric Orthopedist

Evaluation & Tx of Urinary Incontinence in children

Hx, PE Urinalysis

Nocturnal Enuresis
Maturation delay

Day & Night Incontinence

Continuous Intermittent

Age <5 yrs

Age >5 yrs Enuretic alarm, Biofeedback, Drugs Refractory Child >10 yrs age Ultrasound

Dysfunctional Voider

No Tx

IVP/VCUG

Anticholinergics, Time voiding, Biofeedback

Normal

Ectopic Ureter (female)

Abnormal VCUG Urodynamics Normal

Surgery Neuropathic Bladder

Urodynamics Dysfunctional Voider

Approach to IEM causing chronic encephalopathy

Chronic Encephalopathy
No Mainly gray matter Seizures Blindness Dementia Abnormalities outside the CNS? Muscle Yes RES

Mainly white matter Spasticity Weakness Ataxia CNS only CNS & PNS
Lysosomal storage
(eg, MLD, GLD)

Skin/conn ective tissue

Mitochondrial Amino acid (eg, CRSM syn., ds Leigh ds, Organic MELAS, Alpers acid ds syn) Lysosomal Lysosomal ds storage
(eg, NCL, GM2)

Mitochondrial Amino acid ds (myopathies)

(eg, homocyst)

Lysosomal storage
(eg, MPS, Gaucher, NPD, GM1, Sialosis)

Peroxismal ds
(eg, NALD, IRS)

Lysosomal storage
(eg, Mucolipidoses, MSD)

(eg, GM1 late)

Other
B6 dependency

Peoxismal ds Other
Canavan Alexander

Peroxismal ds
(eg, Zellweger)

Dysmorphic syndrome
(eg, Menkes)

Approach to IEM a/w metabolic acidosis

Metabolic Acidosis What is the anion gap?
Normal Renal HCO3losses Elevated Accumulation of fixed anion Urinary organic acids Carbohydrate ds
(eg, galactosemia)

Plasma ketones

Plasma lactate & pyruvate Oxphos ds / pyruvate oxidation (eg,

PDH def., PC def.)

Lysosomal storage
(eg, cystinosis)

Organic acid ds
(eg, propionic acidemia, methylmalonic acidemia)

Organic acid ds
(eg, 3-ketothilase def., SCOT def, 20 finding in other OA diseases)

Amino acid ds
(eg, hepatorenal tyrosenemia)

Mitochondrial

Organic acid ds (eg, MCD) Carbohydrate ds (20 finding in

disorders of gluconeogenesis)

DDx of congenital hyperammonemia

Hyperammonemia Prematurity/respiratory distress prior to 24 hrs of age Yes
Transient hyperammonemia

No
Inborn error of metabolism Urinary organic acids

Abnormal

Oxphos/pyruvate Organic oxidation ds acidemias

Pyruvate dehydrogenase def Pyruvate carboxylase def Defects of oxidative phosphorylation Propionic acidemia Methylmalonic acidemia Isovaleric acidemia Multiple carboxylase def. Glutaric acidurias HMG CoA lyase def

Disorders of fatty acid oxidation

Acyl-CoA dehydrogenase def Carnitine:acycarnitine transiocase def

Normal

Inborn errors of ureagenesis

DDx of IEM a/w severe hypoglycemia

Hypoglycemia Nonglucose-reducing substance in urine
Positive Carbo. Ds
(eg, galactosemia, HFI)

Negative Ketones Low Plasma FFA High Hepatomegaly Plasma amino acids, NH3, urine organic acids

Amino acid ds
(eg, hepatorenal tyrosenemia)

High FAO ds (eg, MCAD def)

Low Consider: Hyperinsulism

High

Present Plasma lactate

Organic acid Absent ds Plasma hGH, cortisol, T4

Appropriate
(eg, propionic acidemia)

Amino acid ds
(eg, MSUD)

Normal

Abnormal

Carbo. Ds
(eg, GSDI, FDPase def)

Consider:
Ketotic hypoglycemia

Consider:
Ketotic hypoglycemia

Consider:
Endocrinopathy

DDx of inborn errors of ureagenesis

Inborn Errors of Ureagenesis Measure plasma citrulline
Absent/ trace Urinary orotic acid N/ Moderate ASA in plasma & urine Marked

Plasma arginine

Normal

Elevated

Low/N

Elevated

CPS 1 def NAGS def

OTC def

Argininemia

Argininos ucconoc aciduria

Citrulline mia

Urinary lysine, ornithine, arginine

Plasma ornithine, urinary homocitr ulline

HHH

LPI

Approach to child with disproportionate short stature

Infant with disproportionate short stature Examine segments Short limb Rhizomelic Associated findings Skeletal survey Mesomelic Associated findings Skeletal survey Acromelic Associated findings Skeletal survey Define X-ray pattern Spondylo/epiphyseal/diaphyseal Define diagnostic possibilities Make Dx Associated findings Skeletal survey Short trunk

Approach to child with microcephaly

Child with microcephaly Prenatal onset
Measure parents Do ophthalmo Small for gestational age Eye findings

Postnatal onset
Minor anomalies Seizures / neurological signs

Multiple contractures d/t deformities Consider syndromes

Consider appropriate syndromes

Do karyotype/ ophthalmo

Do biochemical studies; CT/MRI/EEG

Signs of IUI
No diagnosis Do confirmatory studies

Do CT/MRI

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 q6h Inj. 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