Transcript Type 1 DM

Pediatric ED Case Conference
Date: 2006/04/25
Presented by R1 劉政忠
Instructor: MA 張玉吉吉 醫師
MA 吳孟書 醫師
General data
 Name: 張X浩
 Chart number: 20947975
 Age: 3 week 2 day-old
 Gender: Male
 Body weight : 3kg
Vital sign
 2006-3-29 12:43 (0hr0min)
 T:36 , P:165 , R: 65
 E4V5M6, SpO2:93%
 檢傷分類: 3級
 病患主訴因食慾差
 Chief complaints: SOB for 2 days
Present illness
 Vomiting for 2 days
 No cough
 Poor appetite(+)
 看起來很乾?
Past history
 Nil
PAT
 Appearance: ?
 Breathing: tachypnea
 Circulation: ?
Physical examination
 General appearance: poor activity
 HEENT: lip cyanosis, dry lip
 Chest: bil coarse BS, wheezing(+), rales(+),
retraction(+)
 Heart: RHB
 Abdomen:?
 Extremity:?
 Skin:?
ED initial impression
 r/o GER
 r/o aspiration pneumonia
Further history taking
 Dyspnea? Cough? Foreign body? Cyanosis?
 Vomiting? Content? Projectile? Feeding? Diarrhea?
Urine amount? Body weight change?
 Fever?
 Activity? Appetite?
 Drug hx?
 Birth hx: BBW? IUGR? Preterm baby?CHD?
 Maternal hx: GDM? PROM?
Further PE
 Appearance: TICLS
 HEENT: frontanelle, tears, dry mouth or mucosa,
throat, nasal flaring, accessory muscle use
 Chest: retraction, breathing sound
 Heart: murmur
 Abdomen: soft or distension, tenderness, bowel
sound, umbilicus, mass
 Skin: rash, turgor, pallor, cyanosis, CRT
Detail Hx from admission
note(1)
 Birth hx: G1P1 GA:39+weeks, NSD, APGAR
score? Born in LMD, BBW: 2850g, BH: 50cm, NB
screen:?
 Drug hx: 八寶粉topical use?
 Vaccine: HBV on 95-3-10
 Maternal hx: 20y/o,G1P1, No maternal fever , no
PROM , no chorioamnionitis, no maternal GDM ,
no PIH , no eclampsia , and no use of drug during
pregnancy
Detail Hx from admission
note(2)
 3 days ago….
 Vomiting
 decreased appetite (80~100cc to 20~30 per
meal)
 progressive tachypnea, cyanosis when
feeding
 BW: 2850 to 2880g (<3rd percentile)
Detail PE from admission
note(1)
 General appearance : ill looking, poor activity and
irritable
 HEENT : grossly normal , no facial dysmorphism ,
no anterior or posterior frontanel bulging , no icteric
sclera , no neck mass , no periorbital edema
 Heart : regular heart beat , no murmur
 Chest : coarse breath sound , no wheeze , no rales ,
tachypnea and obvious subcostal or supraclavicular
retraction , mild nasal flaring
Detail PE from admission
note(2)
 Abdomen : soft , flat , no distension , normoactive
bowel sound , no abnormal palpable mass , no pus
discharge from umbilical area, no
hepatosplenomegaly, no perianal redness
 Genital organ : grossly normal
 Extremity :no edema, distal four limb : warm and
pink , no limb cyanosis , peripheral pulse palpable ,
capillary refill time < 2 sec
 Skin: no rash
 Neurologic examination : muscle power : +5/+5 ,
no hypotonia
How about this baby?
 Respiratory distress + dehydration + poor
activity => ?
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Infection: pneumonia, UTI, viral infection…
Endocrine and metabolism: electrolyte
imbalance, Inborn errors of metabolism,
Hypoglycemia…
GI: pyloric stenosis, AGE, T-E fistula,
obstruction…
Initial order 3/29 13:18
(0hr35min)
 CXR
 CBC/DC, CRP, AST, ALT, BUN, Cr, Na, K,
Cl, Ca, sugar, CRP, B/C*1
 IVF: D5S1/4 run 15cc/hr
 O2 hood
 A+B inhalation st
 NPO
Lab—hemogram
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WBC
Hemoglobin
Hematocrit
Platelets
Segment
Band
Lymphocyte
Monocyte
Eosinophil
21400
15.5
43.9
60000
48.0 %
1.0 %
41.0 %
5.0 %
5.0 %
Lab--biochemistry
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Sugar
BUN (B)
Creatinine(B)
AST (GOT)
ALT/GPT
Calcium
Na
K
Cl
CRP
615 !!
17
0.5
23
22
10.4
125
5.7
96
0.83
3/29 14:45(2hr02min)
 Admission to NICU
3/29 15:10(2hr27min)
 IVF 改 half saline run 15cc/hr
 補ketone body(B) => 3+
Admission impression
 Vomiting and severe dehydration
 Severe metabolic acidosis
 Respiratory distress and cyanosis episode, r/o
sepsis
 Leukocytosis and thrombocytopenia
Admission order 3/29 16:06(day1)
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IVF: D2.5S1/2 IV+ PO 140cc/q8h (150cc/kg/day)
NPO
Check F/S stat and q8h
On OG tube
U/C, U/A, urine GBS Ag
Sugar, NA, K, Ca, Cl, ABG
Ampicillin 70mg q6h iv + GM 7mg q8h ivf
U/A
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Color
Turbidity
SP.Gravity
pH
Leukocyte
Nitrite
Protein
Glucose
Yellow
Clear
1.025
5.0
Negative
Negative
Negative
1.000
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Ketone
2+
Urobilinogen 0.1
Bilirubin
Negative
Blood
2+
RBC
6
WBC
0
Epith-Cell
0
ABG and biochemistry
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TEMP
PH
PCO2
PO2
HCO3
SBE
SAT
37°C
7.148
7.0
130.5
2.4
-26.5
97.9%
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Sugar
715
Calcium
10.3
Na
128
K
4.8
Cl
96
AG: 29.6
Osmo(C): 301.7
Hospital course 3/29 (day 1)
 16:20 on nasal CPAP
 16:45 NS challenge 40cc, jusomine 8mL ivf
 21:00 F/S:524
N/S challenge 30cc
IVF 改 HS run 40cc/hr
RI 10U in N/S 500 rum 3mL/hr
 21:25 on endo
Hospital course 3/30 (day 2)
 01:00 F/S:266, RI line 改run 2mL/hr
 07:00 jusomin 8mL ivf
IVF改D0.225 + 5mL KCl run 140/q8h
consult endocrine
check Osmo(B): 301
=> OG: normal
 23:00 F/S:216 RI line改run 0.5mL/hr
Hospital course
 3/31(day 3): extubation
DC RI line 改 NPH 0.5U SC q8h
 4/1(day 4): try feeding
 4/4(day 7): DC anti
 4/6(day 9): NPH 1U SC q8h
 4/12(day 15): 轉5L
 4/17(day 20): 轉7L, NPH1.25U SC q8h
 4/18(day 21): MBD and OPD F/U
Other study during hospitalization
 Abdominal echo=> negative findings
 Serum amylase and lipase=> normal
 C peptite=> 0.34(low 0.47~3.15)
 Insulin antibody=> P11.0(>10)
 HbA1C=> 9.6.%
 Urine GBS Ag, U/C and B/C=> negative
Final diagnosis
1. Neonatal DM (type 1 DM) under NPH
control, complicated with DKA and severe
dehydration
2. Thrombocytopenia post blood transfusion
3. Respiratroy distress due to severe metabolic
acidosis with respiratory compensation
Discussion
DDx of hyperglycemia in infant
DKA in childhood
Management of DKA
Neonatal DM
The ill-appearing infant:DDx(1)
 Infectious: Bacterial sepsis, Meningitis, Urinary
tract infection, Viral infections…
 Cardiac: CHD, SVT, MI, Pericarditis, Myocarditis,
Kawasaki disease
 Endocrine: CAH
 Metabolic: Hyponatremia, hypernatremia, Cystic
fibrosis, Inborn errors of metabolism,
hypoglycemia
Textbook of Pediatric Emergency Medicine, 5th edition
The ill-appearing infant:DDx(2)
 Drugs/toxins—aspirin, carbon monoxide
 Hematologic: Severe anemia, Methemoglobinemia
 Gastrointestinal: Gastroenteritis with dehydration,
Pyloric stenosis, Intussusception, Necrotizing
enterocolitis, Appendicitis, Volvulus
 Neurologic: Infant botulism, Child abuse=> ICH
Textbook of Pediatric Emergency Medicine, 5th edition
Hyperglycemia in infant: DDx
More often in premature infant
 Excess glucose administration
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IVF, feeding
 Inability to metabolize glucose
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Premature, sepsis, stress
 Neonatal DM
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Rare, 2 days ~ 6 weeks, C-peptide may be normal or transiently low
 Medications
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Caffine, theophylline, steroid, phenytoin
 Type 1 DM
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Vary rare, low to absent C-peptide level
LANGE clinical manual, Neonatology: management, principle, on-call
problems, disease and drugs. 5th edition(2004)
DKA in childhood
 Type 1 diabetes: the most common pediatric
endocrine disorder
 Prevalence: 1/400
 Islet-cell autoantibody, insulin antibody, glutamic
acid decarboxylase antibody
 27 ~ 40% of new-onset diabetics present in DKA
 The leading cause of mortality in diabetics < 24 y/o
 cerebral edema: the leading cause of mortality
Definition
 metabolic acidosis (pH <7.25 to 7.30 or
serum bicarbonate <15 mEq/L)
 hyperglycemia (serum glucose >300 mg/dL)
 ketonemia
Clinical feature
 Polyuria, polydipsia, dehydration, ketotic
breath, hyperpnea, nausea, vomiting,
abdominal pain, general malaise, coma
 The most common cause: poor compliance
 Viral illness and focal infections such as
urinary tract infection or gastroenteritis
Life-threatening complications
 Cerebral edema
 Cardiovascular collapse
 Profound metabolic acidosis
 Hyperkalemia
 Hypokalemia
 Hypophosphatemia
Risk Factors for Cerebral Edema
in DKA
 Elevated BUN
 Low PCO2
 Treatment with bicarbonate
 Failure of measured serum [Na] to rise
steadily with correction of hyperglycemia
 Age <3 y
 New-onset diabetes
Initial lab evaluation
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CBC
Glucose
Electrolyte (Na, K, Ca, P, Cl)
blood gas
Serum osmolality
ketones
Urinary ketones
Lactate
Management(1)
 10–20 mL/kg NS bolus until hemodynamically
stable
 Then begin 0.45% NS(or 0.9%NS) at 1.5 times
maintainance
 K (if patient is urinating)
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<5.5 mEq/L=> add 30 mEq/L.
2.5–3.5 mEq/L=> add 40 mEq/L
<2.5 mEq/L=> consider adding more
Management(2)
 RI at 0.1 units/kg/hr after intravenous fluid
bolus. Adjust dose to maintain glucose
decline at 50–100 mg/dL/hr.
 Add dextrose to intravenous fluids when BS
is <200–250 mg/dL.
Management(3)
 Avoid bicarbonate therapy in DKA. Only
consider
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arterial pH<6.9
impaired cardiac contractility and vascular tone
life-threatening hyperkalemia.
 Follow serum electrolytes q2h.
 Follow serum glucose every hour
Neonatal DM, NDM(1)
 Hyperglycemia that occurs during the first
one month of life
 Require insulin tx and last more than 2 weeks
 A rare dx with incidence:1/500000 neonates
 IUGR, SGA, hyperglycemia, dehydration,
glucosuria, acidosis with or without ketouria
Permanent neonatal diabetes mellitus manifesting as diabetic ketoacidosis.
Journal of the Formosan Medical Association. 102(12):883-6, 2003 Dec.
NDM(2)
 Transient NDM
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Insulin can be discontinued after several weeks or months
Maturational delay of the adenyl cyclase-cAMP system of islet cells
 Permanent NDM
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pancreatic agenesis or hypoplasia or absent beta cells
Gene mutations: ATP-sensitive potassium channel subunit Kir6.2
 Type 1 DM
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Autoimmune dx
Manifests later in life
Permanent neonatal diabetes mellitus manifesting as diabetic ketoacidosis.
Journal of the Formosan Medical Association. 102(12):883-6, 2003 Dec.
NDM(3)
 In a series of 57 infants with NDM
 18=> Transient
 13=> Transient with recurrence between
7~20 y/o
 26=> Permanent
Long-term course of neonatal diabetes. N Engl J Med 1995; 333:704.
Case report of TNDM
Age of onset BBW(gw)
Case 1
33 days
3200
Duration of
Insulin tx
5 months
Case 2
49 days
3400
4.9 months
Case 3
49 days
2400
10 months
Case 4
60 days
1300
2 weeks
Different faces of non-autoimmune diabetes of infancy. Acta Paediatrica. 87(1):95-7, 1998 Jan
Case report of PNDM
 A case of PNDM in a 3-day-old female infant.
 This full-term neonate was born small for gestational age.
 Respiratory distress, poor activity, hypothermia, poor
feeding, dehydration, and ketoacidosis.
 After insulin therapy and fluid replacement, her condition
became stable.
 Serum C-peptide level to be low for her age.
 During the first year of life she had catch-up growth, but
insulin therapy was still required.
Permanent neonatal diabetes mellitus manifesting as diabetic ketoacidosis.
Journal of the Formosan Medical Association. 102(12):883-6, 2003 Dec.
PNDM in Oman(1)
 All children(5) diagnosed as having PNDM
between 1991 and 1995 in Oman were included in
the study
 The mean incidence: 2.2 per 100 000 live
births/year
 IUGR was noted in all (mean BBW 1.86 kg)
 DKA in 80%
 Circulating islet cell antibody: (-)
Permanent neonatal diabetes mellitus: clinical presentation and epidemiology in Oman Archives of Disease
in Childhood Fetal & Neonatal Volume 80(3), May 1999, pp 209F-212F
PNDM in Oman(2)
Thanks for your attention!!