Pedia+Notes+EPCapul+5.0

ALLERGY and IMMUNOLOGY IMMUNODEFICIENCY 10 Warning signs of Immunodeficiency

• • • • • • • • • •

8 ear infections infections or more within a year year 2 sinus infections within a year 2 months antibiotics biotics with little little effect 2 pneumonias within 1 year failure gain weight; grow normally Recurrent abscessess Thrush in mouth or skin IV antibiotics antibiotics for for infections infections 2 deep-seated deep-seated infections family history of primary immunodeficiency

General Screening of Immunity

• CBC, differential, platelets • IgG,IgA,IgM,IgE levels • Baseline Antibody Titers Phagocytic Defect

• NBT test • Rebuck skin window •  Chemotaxis • Bacterial assay Complement Defect

•  CH50 • C3 • C4 assay HIV Screening (ELISA)

Positive- Probable AIDS Verify diagnosis by: ELISA HIV HIV test • repeat ELISA • Western blot analysis • CD4 T cell count Negative- Non-AIDS T cell defect test (PPD, Candida antigen, etc.) • CMI skin test • CD4, CD8 assay ratio • Lymphocyte blastogenic assay • T cell enumeration

α-hypotension,peripheral vasodilation, increased vasopermeability, urticaria, angioedema β-positive inotropic & chronotropic effects, bronchodilation, increase cAMP Epinephrine 1:1000 0.01 ml/kg SC/ IM (ped) or 0.3 to 0.5 ml (adult) given q 20 mins prn Px on β blockers may be resistant to epinephrine so higher does may be required or glucagon given insect sting or injected drug: infiltrate 0.1 - 0.2 ml locally to retard absorption of the residual allergen tourniquet applied proximally if injection or sting is on an extremity

Immediate Therapy Rapid ABC’s of resuscitation Epinephrine IV (1:100,000) = 0.01 mg/kg or continuous drip 0.1-0.2 µg/kg/min titrated q 0.1 µg/kg/min to max of 1.5 µg/kg/min Separate IV line no HCO3 infusion Continuous monitoring of CVS status and O2 Rapid HX of triggering event, current medications, HX of asthma, allergies and concomitant medical conditions Subacute H1 blocker – Diphenhydramine 1-2 mg/kg PO,IM,IV Chlorpheniramine 10-20 mg IV,IM Corticosteroids – Hydrocortisone 4-8 mg/kg/dose or methylprednisolone 1-2 mg/kg Iv q 6 h Β2 agonist nebulization q 20 mins of continuous Secondary H2 blocker – Ranitidine IV or PO 2-4 mg/kg/day q 8 h Glucagon 0.1 mg/kg IV if refractory to initial TX Observe at least 4 hours for biphasic anaphylaxis Fluids – Loss of up to 50% intravascular volume may occur resulting in profound hypotension not responsive to epinephrine Antihistamines are not appropriate monotherapy for the Tx of acute anaphylaxis Corticosteroids are used to prevent the biphasic response and to control bronchospasm Bronchodilators are useful adjuncts in TX esp in those with asthma

CARDIOLOGY

ANAPHYLAXIS

Heart Rate Age Awake Mean NB-3mos 85-205 140 3mos-2yrs 100-190 130 2-10yrs 60-140 80 >10yrs 60-100 75 Prob SVT (nQRS) >220 infants, >180children

Criteria for rapid recognition recognition of Anaphylaxis Anaphylaxis

Cardioversion/ Defibrillation: Defibrillation: 0.5-1J/kg (VT); 2-4J/kg (VF/ (VF/ Pulseless VT)

1. Exposure to an allergen within 1 hour & 1 systemic s ystemic sign 2. Urticaria or angioedema & 1 systemic sign Systemic signs: hypotension bronchospasm or dyspnea laryngeal/pharyngeal edema, stridor or dysphonia increased gastrointestinal tract motility

Sleeping 80-160 75-160 60-90 50-90

ECG

Patterns

Acute – explosive onset within seconds to minutes of exposure to triggering event Biphasic – followed by a reaction 3 to 8 hours after initial reaction (5-20% (5 -20% of cases) Protracted – lasts 3 to 21 days from onset of acute reaction Laboratory findings

Elevated plasma histamine Elevated serum tryptase - longer half-life

Treatment

EPINEPHRINE EPINEPHRINE IS THE DRUG OF CHOICE! potent cathecholamine with both α and β adrenergic properties Reverses all pathophysiologic features of anaphylaxis

Pedia Notes

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Normal Axis Newborn 0- (+)180 1- 6 m0 (+)10- (+)125 6mo- 3yr (+)10- (+)110 >3yr 0- (+)90 PR 0.12-0.20sec QRS 0.08-0.12sec ST not >1mm in limb leads; not >2mm in precordial QTc 0.44sec 3-4days; ≤0.45 25 • qR in V1 >3day • upright T in V1 >3day • R in avR≥8mm RVH in Children • RV1 >20, SV6 >7 leads • qR in chest leads • upright T >3yo • RV1≤10mm • T wave inversion in avF • R/S ratio in V1 >1 • RsR’ in V1 • RAD >3mos LVH SV1 >20, RV6 >25 Asymmetric T wave inversion inV5 & V6 SV1 + RV6 >50mm Qwave >30mm in II, III, aVF, V5-6 CVH Direct signs of RVH & LVH LVH + RAD & tall R in V1 RVH + q ≥2 mm in V5 & V6, tall R in V6, & inverted T in V6 Large equiphasic QRS in V2- V4, R + S >60 mm- KatzWachtel phenomenon QTc (corrected QT) - Bazett’s Formula:  ____QTa______ √RR interval where RR interval = # of small squares between R-R x 0.04 sec First Degree AV Block There must be P waves There must be one P wave to each QRS complex com plex P waves have morphology and axis usual for the subject QRS complex must have morphology and axis usual for the subject P-R interval is constant P-R interval is prolonged (i.e. >0.20 sec.)

Pedia Notes

Second degree AV block Mobitz type 1- Wenkebach phenomenon

• • • •

there must be P waves waves there must be QRS complexes P waves must have morphology and axis usual for the subject Progressive prolongation prolongation of P-R interval with each succeeding beat until there is a dropped beat

Second degree AV block Mobitz type 2

• • • •

there must be P waves waves & QRS complexes P waves have have morphology and axis usual usual for the subject subject QRS complex must have morphology axis axis usual for the subject P-R interval of conducted conducted beats may be normal or long but fixed, fixed, then there is a dropped beat

High Grade AV Block

• Some P waves are followed by QRS complexes and some are not • Atrio-ventricular Atrio-ventricular conduction ratio is 3:1 3:1 or higher • P-R interval of beats in which a QRS complex follows follows a P wave may be normal or long but must be constant

Third degree AV block

• Any form of atrial activity may be seen or there may be no atrial activity between atrial and ventricular • no consistent or meaningful relationship between activity. Variable PR and RP intervals. often are • QRS may be normal in shape, duration and axis but more often abnormal and are of constant morphology •  QRS rate is usually constant and lies within the range of 15-70 beats/min.

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• Increased myocardial oxygen requirements and potential to cause ischemia • Decreased splanchnic and hepatic circulation ( ↑AST, ALT) • Anti-Insulin effects: lactic acidosis, hyperglycemia

CHEST XRAY ABNORMAL PATTERN

RAE – AP: >1/3 of the right RVE AP: apex upturned / rounded Lateral: obliteration of the retrosternal space; ½ filled only normally. Displacement of LV posteriorly. Behind shadow of IVC LAE AP: increased distance between the right wall of the left atrium and left main stem bronchus (double density); ≥3.5cm for infants, ≥4.5cm for children; Prominence of left atrial appendage or so called disappearance of cardiac waistline; elevation of left main stem bronchus. Lateral: elevation of left main stem bronchus; discrete bulge in the region of the left atrium which pushes the esophagus posteriorly LVE – AP: prominent left heart border and mid-left heart concavity with apex displaced posteriorly and meets IVC at the diaphragm level MYOCARIDAL INFARCTION IN CHILDREN

ECG Findings • New onset wide Q waves (>0.035 sec) seen within first few hours and persistent over several years • ST-segment elevation (>2mm) seen within the first few hours •  Diphasic T waves seen within first few days (beginning sharply inverted) then normalizing over time • Prolonged QT interval (>0.44 sec) with accompanying abnormal Q waves • Deep wide Q waves in Leads I, avL, or V6 contrast Q waves in II, III, avF Other criteria •  Elevated creatinine kinase / MB although this is not specific for detection of acute MI in children • Cardiac Troponin I is a more sensitive indicator of early myocardial damage in children – elevated within hours of cardiac injury, persists for 4-7 days, specific for cardiac injury CARDIOVASCULAR MEDICATIONS

• Inotropes: agents that improve myocardial contractility and enhance stroke volume •  Pressors: agents that increase systemic vascular resistance and increase blood pressure • Chronotropic: Increase heart rate • Lusotropic: improve relaxation during diastole and decrease EDP in the ventricles ALPHA-ADRENERGIC MEDICATIONS •  Alpha1-adrenergic effects: Vascular smooth muscle contraction •  Alpha2-adrenergic effects: Vascular smooth muscle relaxation--this is a very mild effect only at low doses of an alpha-adrenergic agent like epinephrine. BETA-ADRENERGIC MEDICATIONS •  Beta1-adrenergic effects:Direct cardiac effects: (a) Inotropy (improved cardiac contractility) (b) Chronotropy (increased heart rate) •  Beta2-adrenergic effects: (a) Vasodilation (b) Bronchodilation CARDIAC MEDS VIA CONTINUOUS INFUSION EPINEPHRINE

• Both an alpha- and beta-adrenergic agent • Indications for its use as a continuous infusion are: low cardiac output state  beta effects will improve cardiac function  alpha effects may increase afterload and decrease cardiac output  septic shock - useful for both inotropy and vasoconstriction • Actions are dose dependent (mcg/kg/min): 0.02-0.08 = mostly beta1 and beta2 stimulation.  increased cardiac output  mild vasodilation 0.1-2.0 = mix of beta1 and alpha1  increase cardiac output  increase SVR = vasoconstriction > 2.0 = mostly alpha1  increase SVR, and may decrease CO by increasing afterload • Side effects include: • Anxiety, tremors,palpitations • Tachycardia and tachyarrhythmias o

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Pedia Notes

NOREPINEPHRINE

• Employed primarily for its alpha agonist effect - increases SVR (and B.P.) without significantly increasing C.O. • Used in cases of low SVR and hypotension such as profound “warm shock” with a normal or high C.O. state • Infusion rates titrated between 0.05 to 1 mcg/kg/min   In general, norepinephrine differs from epinephrine in that at doses used in clinical practice, the vasoconstriction outweighs any increase in cardiac output. i.e. norepinephrine usually increases blood pressure and SVR, often without increasing cardiac output. • Side Effects: • Similar to those of Epinephrine •  Can compromise perfusion in extremities and may need to be combined with a vasodilator e.g. Dobutamine or Nipride •  More profound effect on sphlancnic circulation and myocardial oxygen consumption o

DOPAMINE

•  Intermediate product in the enzymatic pathway leading to the production of norepinephrine; thus, it indirectly acts by releasing norepinephrine. • Directly has alpha, beta and dopaminergic actions (dose-dependent) • Indications are based on the adrenergic actions desired. • Improve renal perfusion 2-5 mcg/kg/min • Improve C.O. in mild to moderate Cardiogenic or Distributive Shock 510mcg/kg/min •  Post-resuscitation stabilization in patients with hypotension (in conjuction with fluid therapy) 10-20mcg/kg/min DOBUTAMINE

•  Synthetic catecholamine with inotropic effect (increases stroke volume) and peripheral vasodilation (decreases afterload) • Positive chronotropic effect (increases HR) • Some lusotropic effect • Overall, improves Cardiac Output by above beta-agonist acitivity Major metabolite is 3-O -methyldobutamine, a potent inhibitor of alphaadrenoceptors.Therefore, vasodilation is possible secondary to this metabolite. •  Usual starting infusion rate is: 5 mcg/kg/min, with the dose being titrated to effect up to 20 mcg/kg/min. • Used in low C.O. states and CHF e.g. myocarditis, cardiomyopathy, myocardial infarction • If BP adequate, can be combined with afterload reducer (Nipride or ACE inhibitor) • In combination with Epi/Norepi in profound shock states to improve Cardiac Output and provide some peripheral vasodilatation o

MILRINONE/AMRINONE

• Belong to new class of agents “Bipyridines” •  Non-receptor mediated activity based on selective inhibition of Phosphodiesterase Type III enzyme resulting in cAMP ac cumulation in myocardium • cAMP increases force of contraction and rate and extent of relaxation of myocardium • Inotropic, vasodilator and lusotropic effect • AMRINONE • First generation agent - limited use now • Long half-life (4.4 hours) with potential for prolonged hypotension after loading dose • Associated with thrombocytopenia • Dosage: Load with 0.75 mg/kg with infusion rate of 5-10 mcg/kg/min • Milrinone is preferred drug from this group • MILRINONE • Increases CO by improving contractility, decreased SVR, PVR (?), lusotropic effect; decreased preload due to vasodilatation • Unique in beneficial effects on RV function • Half-life is 1-2 hours •  Load with 50 mcg/kg over 30 mins followed by 0.3 to 0.75 mcg/kg/min • No increase in myocardial O2 requirement

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VASODILATORS

Classified by site of action • Venodilators: reduce preload - Nitroglycerin • Arteriolar dilators: reduce afterload Minoxidil and Hydralazine • Combined: act on both arterial and venous beds and reduce both pre- and afterload Sodium Nitroprusside (Nipride)

• NITROPRUSSIDE •  Vasodilator that acts directly on arterial and venous vascular smooth muscle. •  Indicated in hypertension and low cardiac output states with increased SVR. • Also used in post-operative cardiac surgery to decrease afterload on an injured heart. • Action is immediate; half-life is short; titratable action. • Toxicity is with cyanide, one of the metabolites of the breakdown of nipride. •  Severe, unexplained metabolic acidosis might suggest cyanide toxicity. • Dose starts at 0.5 mcg/kg/min and titrate to 5 mcg/kg/min to desired effect. May go higher (up to 10 mcg/kg/min) for short periods of time. • NITROGLYCERIN • Direct vasodilator as well, but the major effect is as a venodilator with lesser effect on arterioles. • Not as effective as nitroprusside in lowering blood pressure. • Another potential benefit is relaxation of the coronary arteries, thus improving myocardial regional blood flow and myocardial oxygen demand. • Used to improve myocardial perfusion following cardiac surgery • Dose ranges from 0.5 to 8 mcg/kg/min. Typical dose is 2 mcg/kg/min for 24 to 48 hours post-operatively • Methemoglobinemia is potential side effect • ISOPROTERENOL • Synthetic catecholamine • Non-specific beta agonist with minimal alpha-adrenergic effects. •  Causes inotropy, chronotropy, and systemic and pulmonary vasodilatation. • Indications: bradycardia, decreased cardiac output, bronchospasm (bronchodilator). • No longer available in some markets • Occasionally used to maintain heart rate following heart transplantation. • Dose starts at 0.01 mcg/kg/min and is increased to 1.0 mcg/kg/min for desired effect. • INHALED NITRIC OXIDE • Selective Pulmonary vasodilator •  Dilates only pulmonary capillaries to alveoli participating in gas exchange • Decreases intrapulmonary shunt and improves V/Q matching • Rapidly inactivated by Hgb in pulm. cap. so no systemic side effects (eg hypotension) • Potential for use in ARDS and Pulmonary Hypertension • Currently only approved for use in neonatal Pulmonary Hypertension •  Expensive • Special monitoring equipment required • Dose: Concentration of 0.5-60 ppm in inhaled gas

•  Dosage: initial (low) dose: 0.01 mg/kg = 0.1 cc/kg of 1:10,000 subsequent (high) doses: 0.1 mg/kg = (0.1 cc/kg of 1:1,000) o o o o

ATROPINE

•  Parasympathetic (not an alpha- or beta-adrenergic) agent--acts by blocking cholinergic stimulation of the muscarinic receptors of the heart. • Results in an increase in the sinus rate of the heart. • Little effect on systemic vascular resistance or myocardial contractility. •  Indications:   Bradycardia Second or third degree heart block   Asystole Pulseless electrical activity (electrical mechanical dissociation) Route of Administration: IV, IO, ET, SQ, IM, nebulization •  Dosage: 10 to 20 mcg/kg minimum dose is 0.1 mg--smaller doses may cause reflex bradycardia (central stimulatory effect on the medullary vagal nuclei) maximum (adult) dose is 2 mg o o o o

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SODIUM BICARBONATE

• Use during CPR remains a controversial issue due to lack of evidence showing benefit from receiving bicarbonate. • Elevates blood pH by binding with hydrogen to form water and CO 2 • HCO-3 + H+ => H2CO3 => H2O + CO2 • Must have adequate ventilation to remove CO2 or respiratory acidosis will worsen • Adverse effects of acidosis:   Cardiac  Decrease contractility  Lower threshold for ventricular fibrillation  Decrease responsiveness to catecholamines   Vascular  Decrease systemic vascular resistance  Decrease systemic vascular responsiveness to catecholamines  Increase pulmonary vascular resistance •  Indications:  Pre-existing acidosis Prolonged CPR (after 10 minutes) Pulmonary hypertensive crisis   Hyperkalemia • Route of administration: IV, IO Dosage: 1-2 meq/kg/dose (1 meq/cc or 0.5 meq/cc) o

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CALCIUM

•  Current recommendations for the use of calcium during CPR are restricted to a few specific situations. •  Intracellular calcium plays an important role in the process of cell death, but no studies have shown that transient hypercalcemia worsens outcome after cardiac arrest. • Adverse Effects of Hypocalcemia Decreased myocardial contractility Decreased systemic vascular resistance Decreased catecholamine release Decreased cardiovascular response to catecholamines •  Indications:   Hypocalcemia  Ionized hypocalcemia may result from severe alkalosis or after large transfusions of citrated blood products.   Hyperkalemia   Hypermagnesemia Calcium channel blocker overdose • Route of administration: IV, IO only Calcium chloride--central venous line Calcium gluconate--peripheral venous line •  Dosage: Calcium chloride = 10-20 mg/kg Calcium gluconate = 100-200 mg/kg o o o o

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CARDIAC ARREST MEDICATIONS EPINEPHRINE

• Both an alpha- and beta-adrenergic agent •  During an cardiac arrest, most think it has the greatest benefit by alpha-adrenergic actions, increasing afterload and thus diastolic blood pressure, leading to improved coronary artery perfusion. •  Indications:  Cardiac arrest  Severe bronchospasm Anaphylactic reactions • Route of Administration IV or IO SQ or IM (for bronchospasm) ET (cardiac arrest without IV or IO access) o o o

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Pedia Notes

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LIDOCAINE

RHEUMATIC FEVER

Class 1B antiarrhythmic Decreases automaticity threshold and ventricular fibrillation threshold. Effective in terminating PVCs. Rarely used in pediatric arrests as ventricular tachycardia and ventricular fibrillation are not commonplace. •  Indications: Ventricular Tachycardia  Ventricular Fibrillation  Frequent PVCs • Route of Administration: IV, IO, ET Dosage: 1 mg/kg/dose (may need up to 2.5 mg/kg ET)

Guidelines for the Diagnosis of Initial Attack of Rheumatic Fever (Jones Criteria, Updated 1992) SUPPORTING EVIDENCE OF ANTECEDENT GROUP A MAJOR MINOR STREPTOCOCCAL MANIFESTATIONS MANIFESTATIONS INFECTION Clinical features: Carditis Positive throat culture

ENDOTRACHEAL MEDICATIONS (LEAN)

Polyarthritis

• • • • 

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or rapid streptococcal antigen test

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  Lidocaine   Epinephrine   Atropine  Naloxone (Narcan)

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Arthralgia Fever

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Erythema marginatum Subcutaneous nodules

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CONGENITAL HEART DISEASE

�������� �� ���������� ����� �������� �� �������� �� ������  Aortic Valve Stenosis

Pulmonary valve stenosis  Atrial septal defect Pulmonary ejection murmur (innocent) Pulmonary flow murmur

-Supravalvar AS -Subvalvar AS

-PA stenosis

-Aortic stenosis -PDA, PAPVR, TAPVR 

 Ventricular Septal Defect Endocardial Cushion Defect  Vibratory Innocent Murmur

Mitral Regurgitation  Vibratory innocent murmur MVP syndrome  Aortic stenosis IHSS

-HOCM (IHSS) -Tricuspid regurgitation -TOF

 ACYANOTIC DEFECTS

INC.PBF

NORMAL PBF

L to R Shunts

Obstructive Lesions

LVH or CVH

RVH

- VSD - PDA - ECD

- ASD - PAPVR 

LVH

RVH

- AS or AR  - CoA - MR 

- PS - CoA (infants) - MS

Elevated or increasing streptococcal antibody titer

Laboratory features:

Elevated acute phase reactants: Erythrocyte sedimentation rate C-reactive protein Prolonged PR interval

Chorea • intended only for the diagnosis of the initial attack of acute rheumatic fever and not for recurrences • 5 major and 4 minor criteria and an absolute requirement for evidence (microbiologic or serologic) of recent GAS infection. • Diagnosis of acute rheumatic fever: 2 major criteria or 1 major and 2 minor criteria and meets the absolute requirement. • Chorea may occur as the only manifestation of acute rheumatic fever. • Indolent carditis may be the only manifestation in patients who 1st come to medical attention months after the onset of acute rheumatic fever Criteria for determining activity: • joint symptoms • new significant murmur • increasing heart size • congestive heart failure in the absence of old valvular disease • subcutaneous nodules • rectal temperature >100.4 F for at least 3 consecutive days • sleeping pulse of >100/min • positive C-reactive protein *considered active if any one of the following findings is present RHEUMATIC HEART DISEASE

MR/MS is appreciated on PE LVH/RVH on ECG irregular cardiac borders on CXR *In RF there is also cardiomegaly but with normal ECG findings Rheumatic Carditis Mild carditis – no cardiomegaly & no CHF Moderate carditis – cardiomegaly & CHF Severe carditis – severe CHF & pulmonary edema   Valvulitis Apical systolic murmur (Mitral regurgitation) Apical mid-diastolic murmur (Carey-Coomb’s) Basal diastolic murmur (Aortic regurgitation) Basal systolic murmur (Tricuspid regurgitation) Resting tachycardia Muffled heart sounds Gallop rhythm Pericardial friction rub Congestive heart failure Treatment 1. Mild carditis ASA 80-100mg/kg/day X 2 weeks then 60mg/kg/day X 2 weeks 2. Moderate to severe carditis Prednisone 2mg/kg/day X 2 weeks, Tapering until d iscontinued On the last week of prednisone, start ASA 80-100mg/kg/day X 2 weeks then 60mg/kg/day X 2 weeks • • •

•

• •

CYANOTIC DEFECTS

• •

PBF

•

PBF

• •

LVH or CVH

RVH

- PTA - SV  - TGA-VSD

- TGA-IVS

Pedia Notes

- TAPVR  - HLHS

CVH

LVH

RVH

- TVA - TOF - PVA-IVS - Ebstein’sa. hypopl. PAs - PVOD - SV w/PS - TGA-PS - PTA w/

• •

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INFECTIVE ENDOCARDITIS (Duke criteria) Major criteria

(1) positive blood cultures (two separate cultures for a usual pathogen, two or more for less typical pathogens) and (2) evidence of endocarditis on echocardiography (intracardiac mass on a valve or other site, regurgitant flow near a prosthesis, abscess, partial dehiscence of prosthetic valves, or new valve regurgitant flow)

• Measles and Varicella immunization should be deferred for 11 months after child receives high-dose of IVIG • Even when treated with high-dose of IVIG within the 1st 10 days of illness, 5% of children develop at the least transient coronary artery dilation and 1% develop giant aneurysms. • Careful monitoring is necessary during the administration of gamma globulin because it rarely can cause an allergic-like reaction.

Minor criteria

(1) predisposing conditions (2) fever (3) embolic-vascular signs (4) immune complex phenomena (glomerulonephritis, arthritis, rheumatoid factor, Osler nodes, Roth spots) (5) a single positive blood culture or serologic evidence of infection (6) echocardiographic signs not meeting the maj or criteria. Definite Endocarditis: Two major criteria, one major and three minor, or five minor criteria KAWASAKI DISEASE Clinical and Laboratory Features

EPIDEMIOLOGIC CASE DEFINITION (CLASSIC CLINICAL CRITERIA) Fever persisting at least 5 days Presence of at least 4 principal features: • Changes in extremities • Acute: Erythema of palms, soles; edema of hands, feet • Subacute: Periungual peeling of fingers, toes in weeks 2 and 3 • Polymorphous exanthema • Bilateral bulbar conjunctival injection without exudates • Changes in lips and oral cavity: Erythema, lips cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosae • Cervical lymphadenopathy (>1.5 cm diameter), usually unilateral Exclusion of other diseases with similar findings 3 Clinical phases:

Acute Febrile Phase ( 1-2 weeks ) fever, conjuctivitis, erythema, rash Subacute Phase ( 2-4 weeks ) begins when the fever stops desquamation, thrombocytosis, coronary aneurysms Convalescent Phase ( 4-6 Weeks ) resolution of all sign & symptoms labs return to normal coronary aneurysms persists Coronary Aneurysms Classification of CAA Small - 8 mm internal diameter Treatment

Acute Stage Intravenous Immunoglobulin: 2g/kg over 10-12 hours With Aspirin (80-100 mkd) q6, until day 14 of illness and afebrile for 48 to 72 hrs This therapy should be instituted within the 1st 10 days of illness and if possible within 7 days of illness. Convalescent Stage Aspirin (3-5 mkd) OD, until the patient shows no evidence of CA changes by 6-8 weeks after the onset of illness. IVIG also should be administered to children presenting after the 10th day of illness Long Term: Coronary Abnormalities Aspirin (3-5 mkd) divided dosed, continued indefinitely High dose intravenous gammaglobulin (IVIG) • effective in preventing the occurrence of coronary artery abnormalities in KD. • Patients treated with IVGG have a significant increase in T suppressor cells, a decrease in circulating activated T helper cells, and a decrease in spontaneous IgG and IgM synthesis. • suggest that IVGG reduces the vasculitis in KD by suppressing the marked immune activation associated with this disease. Pedia Notes

DEVELOPMENT Anterior fontanelles – closed at 7-19 months Posterior fontanelle – closed at 3 months ANTHROPOMETRICS

Length/Height Average Birth Length: 50cm Length: 9-8-5-3cm Height: agex5+80

Head Circumference Average 13-14in 0-4 mos – 2in 5-12mos – 2in 1-2 yrs – 2 in Weight 2-5 yrs – 2 in Average BW: 3000 5-20 yrs – 2 in 1-6mos= age in mos x 600 + BW OR 7-12mos= age in mos x 500 + BW Average: 35cm 1-6yrs=agex2+8 0-3mos 2cm/mo 7-12yrs=agex7-5/2 3-6 1cm/mo 6-9 0.5cm/mo BSA: square root of (wt x ht / 3600) 9-12 0.5cm/mo 1-3yrs 0.25cm/mo 4-6yrs 1cm/yr Height age – age points on the growth curve where the child’s height falls on the 50 th percentile Weight age – age point on the weight curve where the child’s weight falls on the 50 th percentile Midparental height – 7 (for girls) ± 10 Midparental height + 7 (for boys) ± 10 OR For Males: (mother’s height + 13cm + father’s height) ± 5 2 For Females: (Father’s height - 13cm + mother’s height) ± 5 2 Growth Velocity (cm/yr) Ht (cm) measured at Time 2 - Ht (cm) measured at Time 1 X 12 (mos/yr) Number of months between time 2 and time 1 Age Rate (cm/yr) 1-2 month 38 4 months 28 1 year 12 2 years 10 3-4 years 7 5-6 years 6 7-puberty 5 Arm Span Age Boys: 95 th percentile Body proportions Upper segment – sitting height (measure using Harpenden sitting table) Lower segment – measure from upper border of symphysis pubis to floor in standing position US/LS: Birth = 1,7; 10 years 1 Page 6 /epcapul

ENDOCRINOLOGY

Sexual Maturity Rating Girls

Stage 1 2 3 4 5

Breast Preadolescent Breast and papilla elevated as small mound, diameter of areola is increased Breast and areola enlarged, no contour separation Areola and papilla form secondary mound Mature nipple projects, areola part of general breast contour

Pubic Hair Preadolescent Sparse, lightly pigmented, straight, medial border of labia Darker, beginning to curl, increased amount Coarse, curly, abundant but less than in adult Adult feminine triangle, spread to medial surface of thighs

Boys

1 2

Stage

Penis Preadolescent Minimal change  / Enlargement

Testes Preadolescent Enlarged scrotum, pink texture altered Larger

3

Lengthens

4

Larger; Glans and breadth increase in size

Larger, scrotum dark

5

Adult size

Adult size

Pubic Hair None Scanty, long, slightly pigmented Darker, beginning to curl, small amount Resembles adult type, but less quantity; coarse, curly Adult distribution, spread to medial surface of thighs

RED FLAGS Motor Delay

poor head control by 3 months hands still fisted by 4 months unable to hold objects by 7 months does not sit independently by 10 months cannot stand on one leg by 3 years Language Delay

does not turn to sound by 6 months does not babble or use gestures by 12 months no single word utterances by 16 months No 2-word phrases by 2 years No 3-word sentences by 3 years Psychosocial Delay

No social smile by 3 months Not laughing in playful situation by 6 months Hard to console, stiffens when approached by 1 year In constant motion, resists discipline Does not play with other children at 3 years Cognitive delay

- 2 months -6 months - 12 months - 18 months - 2 years - 3 years -4 ½ years - 5 years -5 ½ years

Pedia Notes

Not alert to mother Not searching for dropped objects No object permanence No interest in cause-and-effect games Does not categorize similarities Does not know full name Cannot count sequentially Does not know letters or colors Does not know birthday or address

IDF definition of Metabolic Syndrome in children and adolescents Age 6 to 200 mg/dl (11.1 mmol/l) heavy glycosuria (>55 mmol/l) ketonuria acidosis (pH < 7.3) ( HCO3 < 15 mmol/l) 5% or more dehydrated ± vomiting / drowsy Principle 1:Restoration of vascular volume In shock with poor peripheral perfusion or coma: give 10 cc/kg x 10-30 min Repeat if poor pulses remain Fluid of choice: 0.9 NSS Fluid input > 4li/m2 : incrd risk for cerebral edema IV therapy MODEL 1 Req’ts = Deficit + Maintenance Maintenance: 3 – 9 kg 80 cc/kg/d 10-19 kg 70 cc/kg/d 20-30 kg 60 cc/kg/d 30-50 kg 50 cc/kg/d >50kg 35 cc/kg/d Add deficit to 48 hr MTN; Replace for 48 hrs w/ PNSS Page 7 /epcapul

MODEL 2 Covers maintenance + 10% deficit, give evenly for 48 hrs. 3 – 9 kg 6 cc/kg/hr 10 – 19 kg 5 cc/kg/hr 20 kg 4 cc/kg/hr (max 250 cc/hr) Compute for the fluid requirement of VJ ( BW=35 kg) Deficit: 35 x 60cc= 2100 ml (assume mod dehydration unless shocky) Maintenance: (35 x 50) x 2=35000 Total fluid for 48h: 5600 ml Monitor urine output especially during the first 4-6 hours of therapy Replace urine losses volume per volume Entails frequent changing rate of infusion Potassium supplementation Start as early as the 3rd hour or even earlier as long as the patient is voiding Shift to a glucose containing solution when the blood glucose is down to 200 mgs% Principle 2:Inhibition of lipolysis and correction of hyperglycemia Insulin therapy • Only short-acting insulin is used ( Humulin R, Actrapid ) • Should not be started until shock has been reversed • IV route only • Target fall in blood glucose: 50-100 mg per hour Low dose continuous Insulin Infusion 0.1 u/kg/hr (consider 0.05 u/kg/hr for a young child) ↓ Hourly blood glucose, fluid input and output Neurological status at least hourly Electrolyte 2 hrs after start of IV therapy Monitor ECG for T wave changes How to prepare insulin infusion? Mix 10 ‘U’ SA insulin in 100 cc plain NSS – 0.1 ‘u’/ml Flush tubings with solution For VJ: 35 kg x .1’u’/kg/hr= 3.5 ‘u’ 35 ml/hr So, always prepare a solution as above so that infusion rate is equal to the weight of the patient When do you stop insulin infusion? • acidosis is resolved • patient is awake How to shift to subcutaneous route? • Compute at .15-.25 ‘u’/kg/dose q6h to be given 30 min pre-meals and at MN • D/C infusion 30 min after 1st SQ dose Do not increase insulin levels >100% Dosing Schedule AM 2/3 PM 1/3 Principle 3:Correction of acidosis Sodium Bicarbonate therapy • pH < 7.0 • HCO3 < 5meq/li Half-correction over 30 minutes - 1 hour

HR 1/3 HN 2/3 HR 1/3 HN 2/3

THYROID STORM

Precipitating factors for thyroid storm Infection Surgery (thyroidal and nonthyroidal) Therapy with radioactive iodine Administration of iodinated contrast dyes or ingestion of large, stable iodine loads Withdrawal of antithyroid medication Amiodarone therapy Ingestion of excessive amounts of exogenous thyroid hormone Diabetic ketoacidosis Congestive cardiac failure Hypoglycemia Toxemia of pregnancy Parturition and the immediate postpartum state Severe emotional stress Acute manic crisis Pulmonary embolism Cerebral vascular accident Bowel infarction Acute trauma Tooth extraction Vigorous palpation of thyroid gland The predictive clinical scale for thyroid storm (Burch and Wartofsky)

Scoring Parameter taken into consideration points Thermoregulatory dysfunction, Temperature (oral) 99-99.9°F 37.2-37.7°C 5 100-100.9°F 37.8-38.2°C 10 101-101.9 °F 38.3-38.8 °C 15 102-102.9°F 38.9â39.3°C 20 103-103.9°F 39.4-39.9°C 25 >104 °F >40 °C 30 CNS effects Absent 0 Mild (agitation) 10 Moderate (delirium, psychosis, extreme 20 lethargy) Severe (seizures, coma) 30 GI-hepatic dysfunction Absent 0 Moderate (diarrhea, nausea/vomiting, 10 abdominal pain) Severe (unexplained jaundice) 20 Tachycardia (beats/min) 99-109 5 110-119 10 120-129 15 130-139 20 >40 25 Congestive cardiac failure Absent 0 Mild (pedal edema) 5 Moderate (bibasal rales) 10 Severe (pulmonary edema) 15 Atrial fibrillation Absent 0 Present 10 Precipitating event Absent 0 Present 10 A cumulative score of >45 is highly suggestive of thyroid storm, 25-44 is suggestive of impeding storm, and 37.5ºC sustained hyperventilation or excessive 25-50% muscular activity hypermetabolic states 25-75% for burns: 2% increase per 1% BSA with burns diarrhea and vomiting volume per volume sweating 10-25% room temperature > 31ºC 30% per ºC rise > 31ºC newborn under radiant warmer or 25% phototherapy Less Fluids needed hypothermia very high humidity humidified inspired air oliguria or anuria sedated or paralyzed Electrolyte Na+ K+

Daily Requirement (meq/kg/day) 2.5-3.0 2.0-2.5

ELECTROLYTES

Na 135-145; K 3.5-5; Ca 2.1-2.6; HCO3 22-26 IVF

pLR

FLUIDS & ELECTROLYTES

ml/100 cal expended 50-55 0-5 30 15

Holliday-Segar Method Weight Daily Requirements 3-10 kg 100 ml/kg 11-20 kg 1000 ml + 50 ml/kg for each kg > 10 kg > 20 kg 1500 ml + 20 ml/kg for each kg > 20 kg Pedia Notes

12% per ºC fall below 37.5ºC 30% 25% Individualized 40%

Determine the fluid deficit Severity of Dehydration Infant Child (>10 kg) mild 50 cc/kg 30 cc/kg moderate 100 cc/kg 60 cc/kg severe 150 cc/kg 90 cc/kg determine the maintenance fluid requirement give the ½ of the fluid deficit over the 1st 8 hours then ½ over the next 16 hours re-assess hydration status periodically for moderate to severe dehydration, check serum electrolytes

IVF

Daily Water Loss Area obligatory urine volume stool water Skin Lungs

Requirements 1500 ml/m2/day 30-50 meq/m2/day 20-40 meq/m2/day

Water Na+ K+

Na+ K+ ClHCO3(meq/L) (meq/L) (meq/L) (meq/L) 130 4 109 28 (lactate) 154 154 51 51 -

pNSS D5 0.3NaCl D5IMB 25 D5NR 140

20 5

22 98

D5NM

13

40

40

27 (acetate) 16 (acetate)

Mg++ Ca++ (mg/dL) (mg/dL) 3 -

-

3 -

-

3

-

HYPONATREMIA

Fast correction: -4mL/kg/dose of 3% NaCl -3% NaCl= 1mL (2meqs/mL NaCl + 4mL sterile water) -Total Na required= (M+D) –bolus M= 3meqs/kg/day D= (desired Na – actual Na) x o.6 x wt Page 9 /epcapul

HYPERNATREMIA

Total water required for 2 days = (M for 2 days +D) – bolus Ideal TBW (in liters)= wtx 0.6; ideal serum Na 140 Water deficit= ideal TBW – actual TBW Actual TBW= ideal TBW x ideal serum Na/actual serum Na

sodium bicarbonate- 2meq/kg IV over 30minutes (except for ERD patients) Polystyrene sulphonate resins-0.5-1gm/kg PO or PR Q4-6hours Sodium Bicardonate Base deficit Wt (kg)x distribution of NaHCO3(0.3) HYPOCALCEMIA

CORRECTED SODIUM

Glucose in mg/dL Na+ + Glucose -100 x 1.6 100 Glucose in mmol/L Na+ + Glucose -5.6 x 1.6 5.6 HYPOKALEMIA

Fast correction 0.5meqs/kg/dose in PNSS diluent x 1hour x 3-5 doses (max 40meqs/L) Example: Wt 20kg: (0.5meg/kg/dose K? x 20kg =10meq/hr Compute how much diluent is required. Central line (200meq/L concentration) 200meq = 10meq 1000mL x X=50mL Order: Give 10meq K in 50mL NSS x 1hr Peripheral line (60meq/L concebtration) 60meq = 10meq 1000mL x X=170mL Order: Give 10meq K in 170mL NSS x 1hr Bedside Pediatric Nephrology PO correction is potassium chloride of 4-6meg/kg/day given in divided doses. Parenteral correction Intermittent Dosing: (for symptomatic hypokalemia) 0.5 to 1.0meq/kg/hr (maximum 30meq/hr) with maximum infusion rate of 0.5meg/kg/hr and given Q2-4hours until symptoms resolve. Continuous Dosing: (for non-symptomatic hypokalemia) 0.2-0.3meg/kg/hr for 24hours *always consider the possibility of Magnesium deficiency especially among patients with refractory hypokalemia. Magnesium is a important co-factor for the activity of the Na-K-ATPase pump which is necessary for potassium homeostasis. Hariett Lane: Oral: Child: 1-4meg/kg/24hr ÷BID-QID Adult: 40-100meq/24hr ÷BID-QID IV: Child: 0.5-1meq/kg/dose given as an infusion of 0.5meq/kg/hr x 1-2hr Max: 1meq/kg/hr. This may be used in critical situations(i.e. hypokalemia with arrhythmia) Adult: Serum K >2.5meq/L: Replete at rates up to 10meq/hr. Total dosage not to exceed 200meq/24hr Serum K 20 kg – 20 mg OD 10-20kg – 10 mg OD - NSAID induced gastric and duodenal ulcer : 20 mg OD x 4-8 wks - GERD 10-20 mg OD x 2-4 wks - symptomatic GERD w/ esophageal lesions 20 mg OD x 4 wks -maintenance of healing of erosive esophagitis – 20 mg OD up to 12 mos Eradication of H. pylori BID x 1 wk: Omeprazole 20 mg + Amox 1000 mg + clarithromycin 500 mg BID x 1 wk: Omeprazole 20 mg + Metro 500 mg + clarithromycin 250 mg Pedia Notes

Protein: gm/k/d x wt x 4; requirement 0.5-3gm/k/d Lipid: gm/k/d x wt x 9; requirement 0.5-4gm/k/d CHO: gm/100cc x vol x 4 (eg. D5=5gm/100cc) CALORIC DISTRIBUTION OF CHO, COOH, CHON OF TOTAL CALORIES GIVEN CHO 60-70% CHON 10-15% Fats 20-30% Breastmilk: 20cal/oz; VCO: 7.7cal/cc; Cereal 12.4cal/scoop 1gm Nitrogen = 6.25gm protein Supplements for Severe Malnutrition: 50% MgSO4 2ml (2mmol/mL) IM x 1 dose; Zn 1mkd until diarrhea stops; Cu 0.1mkd; Folic acid 5mcg/k/d; Fe 3mkd; Vit A (if not given w/in 6mos) 1yr 200,000iu Age REE Multiplication factor 0-1 55 Maintenance 0.2 1-3 57 Acitivity 0.1-0.25 4-6 48 Fever 0.13/deg >38C 7-10 40 Simple Trauma 0.2 11-14 32M/ 28F Multiple Injuries 0.4 15-18 27M/ 25F Burns/ GI surgery 0.5-1 Total Daily Energy Req: Sepsis 0.4 REE + REE x Total factors Growth 0.5 FORMULA FOR CALORIC REQUIREMENT FOR CATCH-UP GROWTH Get the height, weight get ideal weight for actual height kcal/kg=RDA for age (kcal/kg) x ideal wt/ht actual wt CHON=CHON recommended for age x ideal wt/ht Actual wt * start by 50-70% of caloric requirement * increase calories by 20 kcal/kg/k every 2 days until caloric requirement is reached * increase protein by 0.5 g/kg every 2 days until catch up is reached RDA for CHON (g/kg/day) 0-6 mos 2.2 7-12 mos 1.5 1-2 yrs 1.1 3-8 yrs 0.95 9-13 yrs 0.95 14-18 yrs 0.85 RECOMMENDED ENERGY INTAKE (kcal/kg) per kg per day Infants 0-6mos 108 650 6-12 mo 98 852 Children 1-3 yr 102 1,300 4-6 yr 90 1,800 7-10 yr 70 2,000 Males 11-14 yr 55 2,500 15-18 45 3,000 19-24 40 2,900 25-50 30 2,900 > 50 30 2,300 Females 11-14 yr 47 2,200 15-18 40 2,200 19-24 38 2,200 25-50 36 2,200 > 50 30 1,900 Pregnant +300 Lactating +500 CALORIC REQUIREMENT FOR PARENTERAL NUTRITION Neonate 90-120 cal/kg 20 kg 1500 + 20 cal/kg in excess of 20 kg Page 11 /epcapul

FAT requirement in parenteral nutrition 0-12 mos 2 g/kg/day 1-8 yr 4 g/kg/day > 8 yr 2.5 g/kg/day CARBOHYDRATE reqt VLBW ( 8 yr 1-1.5 NORMAL ELECTROLYTE REQT Na 2-4 meq/kg/day K 2-3 Cl 2-3 Mg 0.25-0.5 Ca Infants 300-400 mg/kg/day Children 100-200 Adolescent 50-100 Phosphorous Infants 1-1.5 mmol/kg/day Children 1 Adolescent 0.5-1 F75 DIET: 75 cal/100 cc Skimmed milk powder 25g Veg. oil 20g Sugar 60g Rice (cereal) powder 60g Water to make 1,000 ml *give 100-130 cc/kg/day F100 DIET: 100 cal/100 cc Skimmed milk powder 80g Veg. oil 60g Sugar 50g Water to make 1,000 ml * minimum daily intake of 120-200 ml/kg RESOMAL (ORS for malnourished patients) Dilute 1 L of ORS with 1 L water Add 45 ml of 10% KCl Add 50 g sucrose Composition: Na 45 mmol/L K 40 mmol/L Sugar 25 g/L

(or whole liquid milk 85-295) Rice 15g Vegetable oil 3-5g Cane sugar 3g Water to make 200 ml * 130 ml/kg provides 110 cal/kg 2nd Diet: Lactose free w/ reduced starch 75cal/100g Whole egg 64g Rice 3g Vegetable oil 4g Glucose 3g Water to make 200 ml * if finely ground cooked chicken meat is used instead of egg. Provides 70 cal/100 * 145 ml/kg provides 110 cal/kg

FEEDING REGIMEN (ENTERAL NUTRITION) 1. Intermittent/bolus – more physiologic - should only be used for gastric feed - start at 1-5 ml/kg/bolus - every 3-6 hrs - deliver over 30-120 min (2 hrs) 2. Continuous – better tolerated in px w/ feeding intolerance & significant GER - for critically ill px - start 1-2 ml/kg/hr in child/adol - can be increased by 1-2 ml/hr - concentration shld be inc before volume

NUTREN JUNIOR (same prep as peptamen) 1 cal/ml 12% CHON 35% COOH 53% CHO MCT 25% Lactose free/ gluten free Per 100 ml CHON 3g CHO 13.3g COOH 3.9 g

NUTRITIONAL GUIDELINE Energy – caloric goal = 125% RDA based on wt/ht at 50 th percentile * glucose polymer to in to 24-27 cal/mg formula * MCT infant formula * MCT oil supplement 1-2 ml/k/d 2-4 doses * supplemental nighttime NGT feeding Essential Fatty acids – corn oil Protein intake (infants) 2-3 g/k/d (child) 0.5-1 g/k/d Children Hospital Formulary - started at 10-20 cc/kg/d as bolus or cont. - advance by 12 mos 200,000 IU Zinc 1mg/kg/d Copper (infants) 0.2-0.6 mg/day (child/adol) 1-2 mg/day MgSO4 50% - 2ml IM/SQ Folic acid 5 ucg/kg/d Iron – to start only in the 2 nd  week of illness when infection is better controlled at a dose of 3mg/kg/d Page 12 /epcapul

MICRONUTRIENTS FOR UPBUILDING Vitamin A Folic Acid 800 ucg/prep (5 ucg/kg/d) D1-LD 5 mg or 5 tabs D2 – 1 mg or 1 tab Zinc – 1-2 mg/kg/d Copper 0.2-0.6 mg/d (infant) 1-2 mg/d (children) FOR ACUTE DIARRHEA Zinc 6mo : 20 mg/d for 10-14 days Test dose for Intralipid < 5kg : 0.1 g/kg x 1 hr > 5kg 0.01 g/min x 10-15 min TOTAL PARENTERAL NUTRITION (TPN)

amino acids make fluid D7.5/D10 NaCl (2.5 meq/ml) – 3 meq/kg KCl (2 meq/ml) – 2 meq/kg Ca gluconate 10% - wt x 3, or wt x 300/100 MgSO4 (25% 1meq/ml, 50% 2meq/ml) -0.2 meq/kg NEONATAL CHOLESTASIS

CHOLERETIC DRUGS UDCA 250mg/tab, 15-45 mkd Rifampicin 5mkd Cholestyramine 4-16 g/d Phenobarital 3-10 mkd Vitamin A – 2,500-25,000 IU/day • Clusivol drops /0.6ml = 4,000 IU • Clusivol syrup /5ml = 2,500 IU • Nutrilin drops /ml = 5,000 IU • Nutrilin syrup /5ml = 1,500 IU • Enervon C drops /ml = 3,500 IU • Enervon C syrup /5ml = 100 IU Vitamin D 400-1,200 IU/day as D3 • Clusivol drops /0.6ml = 400 IU • Clusivol syrup /5ml = 500 IU • Nutrilin drops /ml = 333.33 IU • Nutrilin syrup /5ml = 100 IU • Enervon C drops /ml = 200 IU • Enervon C syrup /5ml = 200 IU • Rocaltrol (Calcitriol) 0.25ucg/cap = 0.05-0.2 ucg/kg/d Vitamin E 15mg/d -200 mg/kg/d or alpha tocopherol acetate (squibb) [100 or 200 or 400 IU/cap] 25-200 IU/kg/d, 1 cap at least q5 days in infants 100 IU = 65 mg Vitamin K 1-5 mg/d Ca (elemental) • 50-200 mg/kg/d • 25-100 mg/kg/d • Up to 800-200 mg/d • Ca Sandoz /5ml =110mg elemental • Ca Sandoz /tab =500mg elemental • *Corrected Ca = (40-actual)x.02 + actual Phosphorous (elemental) • 25-50 mg/kg/d up to 500 mg/d Mg – Mg oxide 1-2 meq/kg/d PO • deficiency: serum Mg 2sec is pathologic - >3 sec indicate risk for bleeding - evaluates extrinsic pathway - prolonged when facter 1,2,5,7,10 deficient - if prolonged in chronic liver dse – suggest poor prognosis NORMAL PT/PTT IN HEALTHY PRETERM PT PTT Day1 13(10.6-16.2) 53(27.5-79.4) 5 12.5(10-15.3) 50.5(26.9-74) 30 11.8(10-13.6) 44.7(26.9-62.5) 90 12.3(10-14.6) 37.5(28.3-50.7) 180 12.5(10-15) 37.5(21.7-53.3) Adult 12.4(10.8-13.9) 33.5(26.6-40.3) Factor VIII- non-hepatic - only factor not made in liver - can be used to differentiate liver dse fr DIC (may be N or inc in liver dse) Vit K deficiencies Give Vit K 1mg/kg IM/IV, min: 1mg in FT Measure PT 4-6 hrs after ROLE OF LIVER IN COAGULATION produce coag factors except von willebrand produce & brkdown factors integral to fibrinolysis eg plasminogen & plasminogen activator clears activated clotting factors fr circ Albumin – principal serum protein - synthesized only in rough endoplasmic reticulum of hepatocytes at 150 mg/k/d - half life: 20 d - maintains colloid osmotic pressure - bind/carrier of bilirubin, Ca, other dr ugs - in pts w/ ascites: may be dec due to inc in the distribution vol rather than dec synthesis - often sign of chronic rather than acute liver dse (since long half life) Other nonhepatic causes of low albumin poor nutrition, nephrotic (urine loss), protein losing enteropathies (fr gut), inc degradation rate (poorly understood) Page 13 /epcapul

SERUM ALBUMIN LEVELS g/dL 1-3mos 3.4 4-6mos 3.46 7-12 mo 3.62 13-24 mo 3.63 25-36mo 4.11 3-8yr 4 9-16 yr 4.25

12-18 M F 18-49 M F

+1 SD 0.72 0.36 0.6 0.8 0.78 0.65 0.7

14.5 14

13 12

15.5 14

13.5 12

MCV

serum albumin and PT are most impt parameters need liver transplant HEPATOPULMONARY SYNDROME

1. Hypoxemia 2. Intrapulmonic right to left shunting of blood 3. Liver disease Patient with chronic liver disease with history of shortness of breath or exercise inteolerance and clinical examination findings of cyanosis (particularly of the lips & fingers), digital clubbing, and O2 sats ferric absorbed - Increases absorption: Gastric acid, some sugars, aa, Bile - Decreased absorption: Oxalate, phosphates - Stimulate inc absorption: 1. iron def, 2. hypoxia, 3. erythropoiesis HEMORRHOIDS

Daflon – micronized purified flavonoid fraction chronic conditions & venous insufficiency: 2 tabs/day acute hemorrhoidal attacks: 3tabs BID x 4 d, 2 tabs BID x 3 days Antibiotics in Gut Obstruction (rationale) • Blood flow to the obstructed bowl decreases as the bowel dilates •  Blood flow is shifted away from the mucosa with loss of mucosal integrity • Bacteria proliferates in the stagnant bowel with a predominance of coliforms and anaerobes • Rapid proliferation of bacteria coupled with loss of mucosal integrity allows bacterial translocation across the bowel wall potentially resulting in endotoxinemia, bacteremia and sepsis Bowel gas •  Air is usually demonstrable radiographically in the stomach of a normal infant immediately after birth • Within 1 hour, air may reach the proximal portion of the small intestine and segments of the colon • Air may become visible in the distal parts of the colon as early as the 3rd hour or as late as 18 hours

measures the average volume of a red blood cell categorizes red blood cells by size. Formula (2-10 yrs old) Lower limit: 70 fL + age in years Upper limit: 84 fL + ( age in yrs x 0.6 ), until upper limit of 96 is r eached What’s the MCV range? Give LL and UL of a 7 years old. Answer: LL: 77 fL; UL: 88.2 fL RETICULOCYTE COUNT

Measures erythrocyte production Expressed as % of circulating rbc’s Take up reticulin stain (supravital): bec of inc RNA N = 0.5 % to 1.5 % or = .005 to .015 Reticulocyte index

Anemic patient --> increased retic so have to correct: retic observed x px Hct / 0.45 Example: Hb 50 Hct 0.15 Retic count=.045= 4.5 % Corrected retic = 4.5% x .15/.45 = 1.5 % ( N = 0.5-1.5%) Absolute Retic Count

More accurate Compute as ff: RBC (in n x 1012 ) x # retic/1000 rbc x 1000 Normal = 40,000 – 100,000/uL Example: Compute for absolute retic count : Hb 90 RBC 3 x 1012 /L Retic .015 Answer: 45,000 retics / uL IRON DEFICIENCY ANEMIA

- microcytic, hypochromic, increased RDW Therapy: daily total dose of 4-6mg/kg of elemental iron in 3 divided doses Response to therapy Time after Iron administration Response 12-24hr Replacement of intracellular iron enzyme; subjective improvement, decreased irritability, increased appetite 24-48 hrs Initial bone marrow response; erythroid heperplasia 48-72 hrs Reticulocytosis, peaking at 5-7 days 4-30 days Increase in hemoglobin levels 1-3 months Repletion of stores

HEMATOLOGY and ONCOLOGY APLASTIC ANEMIA

ANEMIA

Measured Hgb > 2 SD below the mean for age Age Mean 1 mo 14 2 mo 11.5 3-6 mo 11.5 .5-2 y 12 2-6 y 12.5 6-12 y 13.5 Pedia Notes

-2SD 10 9 9.5 10.5 11.5 11.5

Severe ANC 500-1000 Very Severe ANC 200-500 BLEEDING

Get Urinalysis with RBC, if RBC8 weeks Clinical Features high fever, chills, severe prostration, and irritability extensive necrotic and ulcerative lesions: oropharyngeal and nasal tissues , skin, gastrointestinal tract , vagina and uterus Gram-negative septicemia ANC Hyperuricemia >Hyperkalemia >Hyperphosphatemia >Hypocalcemia >Hypercalcemia >Renal failure Hydration

-Should be given at the rate of 3000mL/m2/day to maintain urine output of >100mL/m2/hr or >5mL/kg/hr Alkalinization of urine

-Increase solubility of urates -maintain urine pH 6.5 to 7.5 -maintain urine specific gravity 38.5C or 2 SD above normal for age in the absence of external stimulus, long-term drug or painful stimulus, or otherwise unexplained persistent elevations over 0.5-4 hours period OR for children 6 hours ≤ 6 hours Configuration Stellate, avulsion Linear Depth >1cm ≤ 1 cm Mechanism of injury Missile, crush, burn, Sharp surface (glass, frostbite knife) Dentalized Present Absent conataminants (dirt) Present Absent Immunization Schedule History of Non-tetanus prone wound Tetanus prone wound (all tetanus (clean minor wound) other wounds) immunization Td1  TIG Td TIG Unknown or Yes No Yes Yes < 3 doses 3 or more No2 No No3 No doses Td Tetanus and diphtheria toxoid absorbed (adult) TIG Tetanus immune globulin 1  Yes if wound >24 hours old For children 7 years Td preferred to tetanus toxoid alone 2  Yes if >10 years since last booster 3  Yes if > 5 years since last booster Neonatal tetanus – suggested system of scoring to assess prognosis at time of admission and subsequently The severity of the disease is inversely proportionate to the score: 0 recovery improbable; 15 recovery Reassessment of score should be done 24 hourly An unchanged or lower score at subsequent assessment signified ineffective management or complications and calls for modification of treatment Page 15 /epcapul

Score Age of onset of sx in days (incubation period) Interval between first symptom and fisrt spasm in hours (onset interval) Spasms: duration in minutes Temperature C variation from normal Pneumonia and/or atelectasis

0

1

2

3

1-4

5-8

9-12

>12

48

No spontaneous spasms

Persistent prolonged

>2

3

>2-1-1week in FT, >2 weeks in PT DB >2 mg/dl or >20% of TSB To establish etiology of hyperbilirubinemia

Baseline TB, DB, IB CBC with PC PBS, Coomb’s test,Reticulocyte count Mother’s and baby’s blood type Skin color is not reliable

Policy on Improvised Bilirubin Lights

10 fluorescent bulbs at 20 watts each Distance of 20 inches or 50cm from the patient Duration of use should not be more than 2000 hours Stop photo when: 13 ±0.7 (FT); 10.7 ±1.2 (PT) Prophylactic phototherapy

Extensive bruisingin VLBW Diagnosis of hemolytic disease Reminders:

Determine bilirubin levels every 8-12 hours Follow fluid balance carefully. Increase TFI if on phototherapy. Avoid if with liver disease or obstructive jaundice (DB >2mg/dl) because of risk of bronze baby syndrome Anticipate revound of 25% after phototherapy is discontinued Cover eyes & genitals with black cloth to protect from radiation Discontinue if patient becomes hyperthermic Potential Complications

Impaired maternal-fetal bonding Retinal damage Diarrhea / ileus Dehydration Hyperthermia Skin rashes Bronze baby syndrome EXCHANGE TRANSFUSION Indications

Correction of anemia Removal of sensitized RBCs Reduction of TSB Immune thrombocytopenia Equivocal efficacy: Treatment of sepsis, RDS, DIC Consider for the following conditions:

Rh incompatibility ABO incompatibility with eigher bilirubin >20 mg/dl or lesser if clinical condition warrants or evidence of kernicterus at any level Hyperbilirubinemia due to other causes: VLBW infants, BW in kg X 10  exchange necessary Metabolic-toxic conditions: hyperammonemia in UCDs and drug overdose Techniques for exchange transfusion:

a. b. c. d. e. f. g. h. i.

Prepare fresh whole blood (mother’s blood type if ABO incompatibility): should be cross-mathced with maternal blood if ABO/Rh incompatibility Place a UVC after aspirating gastric contents A two-volume exchange (DVET): 80-85% turnover. A onevolume exchange only 60%. Allow 1-2 minute per cycle; ½ hour per volume, so 1 hour for DVET Pre exchange studies: CBC with PC< bilirubin Post exchange studies: CBC with PC, bilirubin, RBS, K, Ca – taken 6-12 hours post exchange, blood CS is controversial A CVP of 5-8 cm H2O be maintained at all times Keep thermoregulated during procedure Resume feeds 4 hours after exchange

Calculations

Total blood volume (TBV) Term or >1kg: 80cc/kg Preterm or 1015mL is considered extensive. NECROTIZING ENTEROCOLITIS

Risk Factors 5 Is Ischemia Immaturity Immunologic Infection Intake Stage Systemic Stage I NEC Nonspecific: suspect apnea, decreased HR, lethargy, temperature instability Stage IIA Mild Same NEC

Stage IIB Moderate NEC

Mild acidosis, ↓APC

Stage IIIA Advanced NEC

Respiratory / Metabolic acidosis, assis vent for apnea, decreased BP, decreased UP, neutropenia, DIC Deteriorating VS and laboratory indices

Stage IIIB

Intestinal Gastric residuals; guiac + stools

Radiographic Nonspecific

Prominent abdominal distention ± tenderness, (-) bowel sounds, gross blood in stools Abdominal wall edema, tenderness ± palpable mass Spreading edema, erythema, abdominal induration

Ileus, dilated bowel loops, focal areas of pneumatosis intestinalis Extensive pneumatosis intestinalis Prominent ascites, persistent sentinel loops with no perforation

Page 17 /epcapul

OXYGENATION INDEX (OI)

METABOLIC ACIDOSIS   COMPENSATION Respiratory – changes in paCO2 If actual paCO2 = expected paCO 2 COMPENSATED METAB ACIDOSIS If actual paCO2 < expected paCO2 METAB ACIDOSIS WITH RESP ALKALOSIS If actual paCO2 > expected paCO2 METAB ACIDOSIS WITH RESP ACIDOSIS

–

OI = (MAP X FiO2 X 100) / Postductal PaO2 MAP – mean airway pressure OI > 15 signifies severe respiratory compromise 30-35 failure to respond to the existing mode of ventilator support >40 80% risk of death, ECMO

•

– – •

– •

Screening ROP – 4-6 weeks chronologic age, 31-33 wks PCA

– •

≤ 1500, AOG ≤ 28 weeks, unstable course Hearing – wt>1.5, off vent/meds 0.008 X � pCO2  - Overlapping Metab acidosis or alkalosis? So, Compute for expected HCO 3 If actual HCO3 < expected HCO 3 RESP ACIDOSIS WITH METABOLIC ACIDOSIS If actual HCO3 > expected HCO 3 RESP ACIDOSIS WITH METABOLIC ALKALOSIS • • •

Cardiothymic shadow Clearly defined Still discernible Hazy, barely discernible Up to lung periphery

Air Bronchogram Perihilar within CT shadow Just past CT borders Past 2/3 lung

•

•

•

• •

•

• • •

Cardiac borders no longer visible

–

•

–

NEPHROLOGY

•

–

OSMOLALITY

Osmolality = 2(Na) + BUN/18 + Glucose/2.8 nv: 220-320 nCVP: 5-10cm ARTERIAL BLOOD GAS

Compute for the ∆pH Compute for the expected bicarbonate when it is abnormal Primary Expected Change Disorder HCO3 pCO2 SBE Metabolic 26 (0.7xHCO3) + ≥5 alkalosis (21±2) Acute Respi [(pCO2-40) ÷ >45 or ∆pH = =0 Acidosis 10] + 24 0.008 x (pCO240) Chronic Respi [(pCO2-40) ÷ 3] >45 or ∆pH = 0.4 x (pCO2Acid +24 0.003 x (pCO2- 40) 40) Acute Respi [(40-pCO2) ÷5] expected HCO 3 RESP ACIDOSIS WITH METABOLIC ALKALOSIS –

 

pH < 7.35

pH 7.35 – 7.4 pH 7.4 –7.45 pH > 7.45

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part comp

pCO2 m et abol ic < 35 acidosis

pCO2 metabolic 35-45 acidosis

comp m et abol ic

comp re sp ir at or y r esp ir at or y

acidosis

alkalosis

alkalosis

normal

normal

metabolic alkalosis

comp

pCO2 respiratory respiratory > 45 acidosis acidosis

comp

part comp

metabolic

metabolic

a lka lo si s

a lk alo si s

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Respiratory Alkalosis • • •

pH >7.46, pCO2 0.017 X � pCO2, then compute for expected HCO3 –concomittant metab acidosis or metab a lkalosis – – –

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Oxygenation Status

At room air, sea level: PaO2 80-100 normal or acceptable PaO2 < 80 mild hypoxemia PaO2 < 60 moderate hypoxemia PaO2 < 40 severe hypoxemia On oxygen support: PaO2 80-100 corrected hypoxemia PaO2 > N overcorrected hypoxemia PaO2 < N uncorrected hypoxemia

Estimated GFR = Ht (cm)x 0.5(children/adol girls) or 0.7 (Adol boys) Serum creatinine mg/dL Estimated GFR (mL/min/1.73m2)=kL/Pcr L (length/height, cm) Pcr- plasma creatinine k- constant k LBW during first year of life 0.33 Term AGA during first year of life 0.45 Children and Adolescent girls 0.55 Adolescent boys 0.70 Creatinine Clearance (mL/min/1.73m2) =Urine cr x Urine vol x 1.73 Plasma cr 1440 BSA Normal Values of GFR Age GFR (mean) mL/min/1.73m2 Neonates 34wk gestational age 2-8 days 39 4-28 days 47 30-90 days 58 1-6 mo 7 6-12 mo 103 12-19 mo 127 2 yr-adult 127

Range mL/min/1.73m2 11-15 15-28 40-65 17-60 26-68 30-86 39-114 49-157 62-191 89-165

Computing for pHR

Normal pCO2 = 40 and normal pH = 7.4 If actual pCO 2 > 40: pHR = (40 – Actual pCO 2) x 0.05 + 7.4 10 If actual pCO 2 < 40: pHR = (40 – Actual pCO 2) x 0.1 + 7.4 10 If pHR compared to actual pH is: < 0.03 → purely respiratory > 0.03 →  compensated •

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Laboratory Indices for Prerenal vs Intrinsic Acute Renal Failure Index Prerenal Intrinsic Renal Specific gravity >1.020 500 20 3-plus (+ + +) if quantitation not performed OR Cellular casts: may be red blood cell, hemoglobin, granular, tubular, or mixed Neurologic Seizures:in the absence of offending drugs or known disorder metabolic derangements (e.g., uremia, ketoacidosis, or electrolyte imbalance) OR Psychosis:in the absence of offending drugs or known metabolic derangements (e.g., uremia, ketoacidosis, or electrolyte imbalance) Hematologic Hemolytic anemia, with reticulocytosis disorder OR Leukopenia: