approach failure to thrive
DESCRIPTION
Approach Failure to ThriveTRANSCRIPT
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G r a n d R o u n d 1 2 / 2 0 1 0 Page 1
PHYSICAL EXAMINATION
Tuesday, May 11, 2010 At Prasong Tujinda Conference Room
3 4 . 1 3 :
7-8 3 (Infant formula : Similac) 6 10 / , (, , , ) , , , 16-20 onz/day
: 2,700 g, APGAR 9,10 Length 47 cm , , . : 1 3 2 , -,
, : : - 41 160 cm
- 40 163 cm - 5 3 1 13 5 V/S : Temp 37 0C, PR 110/ min, RR 24/min, BP 91/45 mmHg
Length 70 cm (10th P), Wt 8.2 kg (< 3rd P), HC 44.5 cm (25th- 50th P) %BW for Lt = 101.23 %, % Wt for age = 85.4 %, %Ht for age = 90.9 %
Grand Round 12/2010
Case Presentation
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G r a n d R o u n d 1 2 / 2 0 1 0 Page 2
INVESTIGATION
GA : active, not pale, no jaundice, no dyspnea, no cyanosis , no dysmorphic feature HEENT: Ears intact both tympanic membranes, not injected
Nasal turbinate swelling 2+ both Pharynx and tonsils not injected, tonsilar enlarge 2+, no postnasal drip No teeth eruption seen
Eyes- no nystagmus, no xerophthalmia No thyroid gland enlargement CVS : normal S1 S2 , no murmur Lung : clear, equal breath sounds Abdomen : soft. not tender, no hepatospleenomegaly Skin : no rash or dermatitis Nervous system : Alert Motor power grade V/V all extremities No stiff neck
CBC : Hb 12.1 g/dL, Hct 36.4%, WBC 8,190 /mm3, (N 20%, L 65%) Platlet 336,000 /mm3, MCV 75, MCH 25, RDW 13.6 (PBS: normochromic normocytic RBC) BUN 16, Cr 0.3mg/dL Electrolyte : Na 140, K 4.6, Cl 106, HCO3 19 mmol/L, Total Ca 9.5, PO4 5 mg/dL UA : pH 7, sp.gr. 1.010, protein-neg, suger-neg, ketone-neg, blood-neg, WBC 0-1, RBC
0-1, epi.0-1/LPF Thyroid function test : T3 139.8 ng/dl, T4 9.57 ng/dl, TSH 2.03 uU/ml Film bone age : to be presented
Sessions and Speakers
Speaker Est. Time
., . History and Physical examination 10 min .. Approach failure to thrive: Nutritional aspect 10 min
. Approach normal gap metabolic acidosis 15 min
., . Management RTA and Progression 10 min
.. Summary and Discussion 10 min
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G r a n d R o u n d 1 2 / 2 0 1 0 Page 3
Question and answer 5 min
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NEPHROLOGY GRAND ROUND
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Historyy 3 6/12 y 1 3/12 y : y :{ 7-8 6 1 1-2
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Historyy 3 (Infant formula : Similac) 16-20 onz/day 2 2/12 1-2 /y 6
1 6 2 8-9 3 1
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Historyy 3 1/3 /, , (, , , ) , , , , y y
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Historyy 1 3/12 y {Ht 70 cm (10th P), Wt 8.2 kg (< 3rd P), HC 44.5 cm (25th- 50thP ){% Wt for Ht = 101.2 % Normal{% Wt for age = 85.4 % 1st Degree malnutrition{% Ht for age = 90.9 % 1st Degree stuntingy
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Historyy Inadequate calories intake advice impoved nutrition BW 400 g in 2 weeks y {CBC : Hb 12.1%, Hct 36.4 g/dL, MCV 75 MCH 25 RDW 13.6
WBC 8,190 /mm3(N 20%, L 65%) Platelet 336,000 /mm3
{ BUN 16 Cr 0.3 mg/dL
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Investigationsy Electrolyte : Na 140, K 4.6, Cl 106, HCO3 19 mmol/L, Total Ca 9.5, PO4 5
mg/dL, AG 15 (Normal gap metabolic acidosis)y UA : pH 7, sp.gr.1.010, protein-neg, suger-neg, ketone-neg, blood-neg,
WBC 0-1, RBC 0-1, epi.0-1/LPFy Thyroid function test : { T3 139.8 ng/dl (119-218 ng/dl){ T4 9.57 ng/dl (6.8-13.5 ng/dl){ TSH 2.03 uU/ml (0.6-5.5 uU/ml)
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Historyy Genetic FTT Habitual
abortion 3 ) dysmorphic features
41
40
13 1 week 1 week 3 mo. 3
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History
y Plasma organic acid , urine organic acid (28/1/51)- normal y DQ(30/1/51 1 4/12 ) 108 (average)y Electrolyte(27/1/51) {Na 136, K 4.3, Cl 104, HCO3 20 mmol/L, AG 12{ BUN 18 Cr 0.3 mg/dLy Urine electrolyte (29/1/51) urine Cr 71.3 mg/dL, Na 48, K 157, Cl 94 mmol/L,
urine AG 111
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Historyy Nephrology normal gap metabolic acidosis definite diagnosisy Loss F/U nutrition, nephrology y 2 ..
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Historyy : 2,700 g, APGAR 9,10
Length 47 cm , , .y : 2
7-8 1 1 -
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Historyy : y :{ 41 160 cm { 40 163 cm { 5 3 1 13
, 5
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Physical examinationy V/S : Temp 37 0C, PR 110/ min, RR 24/min, BP 91/45 mmHg y GA : active, not pale, no jaundice, no dysmorphic featurey Ht 88.5 cm (3rd P), Wt 10.7 kg (3rd P), HC 46 cm (< 3rd P )y U:L segment 1.2: 1{% Wt for Ht = 85.6 % 1st Degree westing{% Wt for age = 76.4 % 1st Degree malnutrition{% Ht for age = 92.2 % 1st Degree stunting
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160 cm 100 kg 163 cm 64.7 kg
MPH 148.5-161.5 cm
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Physical examinationy GA : active, not pale, no jaundice, no dysmorphic featurey HEENT: {Ears intact tympanic membranes both, not injected{Nasal turbinate swelling 2+ both {Pharynx and tonsils not injected, tonsilar enlargement 2+, no
postnasal drip{No teeth eruption seen{Eyes- no nystagmus, no xerophthalmia{No thyroid gland enlargement
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Physical examinationy CVS : normal S1 S2 , no murmur y Lung : clear, equal breath soundsy Abdomen : soft. not tender, no hepatospleenomegaly, no mass y Skin : no rash or dermatitisy Nervous system : Alert{Cranial nerve intact{Motor power grade V/V all extremities{No stiff neck
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Problem listyMalnutrition yHistory of low calorie intakeyNormal gap metabolic acidosisyFamily history of habitual abortion
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Failure to thriveDefinition{Weight below the 3th percentile for age and sex{Weight for age curve falls across two major percentile lines{Weight gain is less than expected
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HISTORY AND PHYSICALHISTORY AND PHYSICALEXAMINATIONEXAMINATION
Organic CauseOrganic Cause
Cause Not Cause Not ObviousObvious
Feeding Feeding DisorderDisorder
or or
BehavioralBehavioral
oror
PsychosocialPsychosocial
EtiologyEtiology
InvestigationInvestigation
and and
ManagementManagement
as Indicatedas Indicated
Laboratory Screening Laboratory Screening TestsTests
PositivePositive NegativeNegative
Treatment MalnutritionTreatment Malnutrition
andand
MultidisciplinaryMultidisciplinary
ServicesServices
INFANT WHO HAS FTTINFANT WHO HAS FTT
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Differential diagnosisInappropriate intake Increase needs Increase losses
Discomfort or pain associated with eating
Increase metabolic rate(underlying medical conditions)Heart diseasesChronic lung diseasesHyperthyroidismOSADiencephalic syndrome
Increase losses from stool or urineCarbohydrate intoleranceFood protein allergyBulimia nervosaCeliac diseaseShort bowel syndromeIBDPancreatitisCystic fibrosisChronic liver or renal diseasesDMInborn error metabolismRenal tubular acidosis
Oromotor dysfunctionBehavioral issuesDecreased appetiteNon nutrient liquidChronic diseasesMedicationPsychosocial problems Inflammation /infection
Dietary restriction : eating disorders, poverty
Increase activity
Child abuse
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. .
Approach to failure to thrive : Nutritional aspect
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Investigationsy CBC : Hb 10.8%, Hct 32.8 g/dL, MCV 72.7 MCH 23.9 RDW 14.4
WBC 9,500 /mm3(N 35%, L 56%) Platelet 349,000 /mm3 (PBS: normochromic normocytic RBC)
y BUN 18 Cr 0.3 mg/dLy Electrolyte : Na 139, K 3.9, Cl 109, HCO3 19 mmol/L,
AG 11 (normal gap) Total Ca 8.7, PO4 4.6, Mg 2.0 mg/dL y UA : pH 7, sp.gr.1.010, protein-neg, suger-neg, ketone-neg, blood-
neg, WBC 0-1, RBC 0-1, epi.0-1/LPF
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Film bone age : 3 yr (CA 3 4/12yr)
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Metabolic Acidosis
Figure obtained from MKSAP Edition 14
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Management y Nutrition : Poor intake Food record * 3 daysy Psychiatry : no psychiatric problems, y Endocrine : dysmorphic feature of growth hormone
deficiency, growth velocity (6.4 cm/yr) ,Film BA=CA, growth hormone deficiency
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Management y Nephrology Normal gap metabolic acidosis R/O
Renal tubular acidosis y Urine pH 6.5-7y Urine electrolytey urine Cr 81.7 mg/dL, Na 28, K 22.5, Cl 31mmol/Ly urine AG 19.5 abnormal H+ secretion
y Imp: Distal renal tubular acidosis (RTA type 1)
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HCO3 loading test
Urine pH 8.2 (on shohl solution 10 ml po q8 hr )Urine electrolyte Na 196, K 55.2, Cl 99 mmol/L, urine Cr 56.7 mg/dl
Electrolyte Na 139, K 4.3, Cl 99, HCO3 23 mmol/L, AG 10 BUN 15.9 Cr 0.4 mg/dl
Urine gas pH 7.94, CO2 37.9, O2 165, HCO3 83.1
VBG pH 7.42, CO2 42.1, O2 36, HCO3 27
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Interpretationy FeHCO3= 2.5 % ( < 5){ Interpretation: normal HCO3 reabsorptiony Urine CO2- blood CO2 = 0.8 ( > 20){ Interpretation: abnormal distal acidification
Imp: Distal RTA (type 1)
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Approach to renal tubular acidosis
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Treatment of RTA
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Proximal RTAy A mixture of Na+ and K+ salts, preferably citrate, is
preferable.y 10 to 15 meq of alkali/kg may be required per day to stay
ahead of urinary losses.y Thiazide diuretic may be beneficial if large doses of
alkali are ineffective or not well tolerated.
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Distal RTAy Bicarbonate wasting is negligible in adults who can generally be
treated with 1 to 2 meq/kg of sodium citrate or bicarbonate.y Potassium citrate, alone or with sodium citrate, is indicated for
persistent hypokalemia or for calcium stone disease.y For patients with hyperkalemic distal RTA, high-sodium, low-
potassium diet plus a thiazide or loop diuretic if necessary.
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Hyperkalemic RTAy Treatment and prognosis depends on the underlying cause.y Potassium-retaining drugs should always be withdrawn.y Fludrocortisone therapy may also be useful in
hyporeninemic hypoaldosteronism, preferably in combination with a loop diuretic such as furosemide to reduce the risk of extracellular fluid volume expansion.
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Progression
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After Rx 2 weeks 4 weeks 6 weeks
BW 10.9 kg 10.9 kg 11.2 kgInvestigation Na140 K 4.2
Cl 107 HCO3 22 mmol/LAG 11BUN 14.7 Cr0.4 mg/dL
UA : pH 5, sp.gr. 1.020, prot-neg, sugar-neg, acetone-neg ,WBC 0-1,RBC 0-1, epi 0-1Urine Ca/Cr 0.16
Treatment M. pot cit (1 mEq/kg/day)
M. pot cit(1 mEq/kg/day)
M. pot cit (1.2 mEq/kg/day)
U/S KUB (2/3/53): RK 5.9*3.4*3.3 cm , LK 6.1*3.1*2.4 cm No evidence of nephrocalcinosis, chronic parenchymatous disease of kidneys, no evidence of stone, mass or hydronephrosis
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Take home massage1. Evaluation of Failure to Thrive involves careful History&PE,
observation of feeding session, and should not include routine lab or other diagnostic testing
2. Nutritional deprivation in the infant and toddler age group can have permanent effects on growth and brain development
3. Review causes of Normal-anion gap Metabolic Acidosis{ Renal vs. GI losses
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Take home massage4. Distinguish RTA Types 1, 2 and 4{ Type 1: renal stones, hypercalciuria, high urine pH despite metabolic
acidosis{ Type 2: acetazolamide and bicarbonate wasting; Fanconi syndrome{ Type 4: aldosterone deficiency and hyperkalemia
5. Mainstay of treatment of RTABicarbonate therapy
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Renal Tubular Acidosis
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1. Normal anion gap metabolic acidosis2. Urine pH > 5.5
Renal Tubular Acidosis
Investigations
Diagnosis renal tubular acidosis
Pathophysiology
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ProximalRTA
(type 2 )
Distal RTA
( type 1 )
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Na+
K+
Na-KATPase
3 Na+
2K+
ATPase
ATPase
H+
H+
K+
H2O
OH- HCO3-
Cl-
luminal basolateral
RTA Type 4
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ProximalRTA
DistalRTA
Type 4RTA
HCO3-( mmol/L )
Usually12 20
variable Greater than 17
Urine pH Variable,> 5.5 if above threshold
> 5.5 Usually< 5.5
Serum K reduced Usually reduced
increased
Dose of HCO3
10 15 mmol/kg/D
4 14mmol/kg/D
1 3mmol/kg/D
Characteristic of RTA
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Na+
K+
Na-KATPase
3 Na+
2K+
ATPase
ATPase
H+
H+
K+
H2O
OH- HCO3-
Cl-
luminal basolateral
Cl-
_
_
_
_
K+
K+
K+
-
Na+
H+
Na+
glucose
phosphate
amino acid
3Na+
2K+
HCO3-
Na+
OH- +CO2
H2O
Na K
ATPase
Na+
Na+
Na+
HCO3-
HCO3-
HCO3-
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Na+
Na+
Na+
Na+
Na+
Na+
-
Na+
K+
Na-KATPase
3 Na+
2K+
ATPase
ATPase
H+
H+
K+
H2O
OH- HCO3-
Cl-
luminal basolateral
Cl-
Na+
Na+
Na+
Na+
Na+
Na+
_ _
_ _
_ _
_ _
K+
K+
K+
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ProximalRTA
DistalRTA
Type 4RTA
HCO3-( mmol/L )
Usually12 20
variable Greater than 17
Urine pH Variable,> 5.5 if above threshold
> 5.5 Usually< 5.5
Serum K reduced Usually reduced
increased
Dose of HCO3
10 15 mmol/kg/D
4 14mmol/kg/D
1 3mmol/kg/D
Characteristic of RTAno nephrocalcinosis nephrocalcinosis
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1. Normal anion gap metabolic acidosis2. Urine pH > 5.5
Renal Tubular Acidosis
Investigations
Diagnosis renal tubular acidosis
Pathophysiology
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ProximalRTA
DistalRTA
Urine anion gap
UsuallynegativeIf severepositive
Positive
Urine osmolar gap
< 100 > 100
HCO3 loading test
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cation = anion
measured cation + = measured anion +unmeasured cation unmeasured anion
( Na+ + K+ ) + = Cl- +unmeasured cation unmeasured anion
( Na+ + K+ ) Cl- = unmeasured anion -unmeasured cation
Urine anion gap
NH4+
-
Na+
K+
Na-KATPase
3 Na+
2K+
ATPase
ATPase
H+
H+
K+
H2O
OH- HCO3-
Cl-
luminal basolateral
Cl-NH3
NH4+
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cation = anion
measured cation + = measured anion +unmeasured cation unmeasured anion
( Na+ + K+ ) + = Cl-- +unmeasured cation unmeasured anion
( Na+ + K+ ) Cl- = unmeasured anion -unmeasured cation
Urine anion gap
NH4+
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ProximalRTA
DistalRTA
Urine anion gap
UsuallynegativeIf severepositive
Positive
Urine osmolar gap
> 100 < 100
HCO3 loading test
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urine osmole calculated urine osmole
Urine osmolar gap
2 (Na+K)+ urea + glucose2.8 18
NH4+ no NH4+
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ProximalRTA
DistalRTA
Urine anion gap
UsuallynegativeIf severepositive
Positive
Urine osmolar gap
> 100 < 100
HCO3- loading test
FE HCO3-
Urine CO2 -Bl CO2
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> 20< 20> 20Urine CO2-Blood CO2
5 15< 5> 15FEHCO3-
RTA type 4
DistalRTA
ProximalRTA
HCO3-loading test
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Na+
K+
Na-KATPase
3 Na+
2K+
ATPase
ATPase
H+
H+
K+
H2O
OH- HCO3-
Cl-
luminal basolateral
Cl-HCO3-
H2CO3
H2O +CO2
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> 20< 20> 20Urine CO2-Blood CO2
5 15< 5> 15FEHCO3-
RTA type 4
DistalRTA
ProximalRTA
HCO3-loading test
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1. Normal anion gap metabolic acidosis2. Urine pH > 5.5
Renal Tubular Acidosis
Investigations
Diagnosis renal tubular acidosis
4. HCO3-loading test
- FE HCO3- = 27 x 0.4 x 100 = 2.5%23 56.7
- Urine CO2-Blood CO2 = 37.9 42.1= - 4.2
DistalRTA
4. U/S KUB : no nephrocalcinosis
3. Urine anion gap = positive
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Etiology of distal RTA
Primary
1.Familial- AD : AE1 gene- AR : with deafness : rd RTA1
or ATP6B1genewithout deafness :
rd RTA2 orr ATP6N1B
2. Sporadic
Secondary
1.Autoimmune2.Drug-toxic3.Tubulointersitial
disease
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1-Grand rou..2-NEPHROLOGY_update[1]3-renal tubular acidosis