DR.ANAND DESHPANDE · Rh incompatibilty. Normoinsulinaemic hypoglycaemia ... Mode of delivery :...
Transcript of DR.ANAND DESHPANDE · Rh incompatibilty. Normoinsulinaemic hypoglycaemia ... Mode of delivery :...
DR.ANAND DESHPANDEMBBS, DCH , DNB [PAEDS]
Consultant Pediatrician ,
•Consultant : Deenanath Mangeshkar Hospital, •Pune
Hon. Asso.Professor Pediatrics, BJGMC PG Teacher MUHS
•Past President IAP Pune
Refractory Hypoglycemia
Dr Anand DeshpandeConsultant Pediatrician
Deenanath Mangeshkar Hospital, Pune
Hon. Associate professor Pediatrics
B J Medical college Pune
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Neonatal Hypoglycaemia
• The most common metabolic disorder of
neonatal period and infancy.
• One of the most preventable cause of
neurological sequelae.
• Requires early diagnosis and prompt intervention
• Exact value at which brain damage occurs ???
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Operational threshold (defined by consensus)
Plasma / whole blood glucose at which clinicians
should consider intervention.
• A blood glucose level (BGL) < 40 mg/dl (plasma glucose level< 45 mg/dl)
• WHO
BGL < 45mg/dl (2.2mmol/L) Hypoglycemia
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Transitional neonatal hypoglycaemia (TNH)
• BGL as low as 30 mg/dl within 1-2 hrs of birth
common in normal
• Occurs in up to 10% of normal newborns
• Represent physiological adaptation to post natal life.
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The levels of insulin falls initially and remains in the basal range for several days without demonstrating the usual brisk response to physiologic stimuli like enteral glucose.
Clinical Manifestations
Features d/t autonomic disturbancesJitteriness
• Tremors
• Pallor and cyanosis
Features d/t cerebral glucopenia• Staring
• Refusal to feed
• Lethargy
• Convulsion
• Apnea8
WHOM TO SCREEN ??
• Birth wt <2kg, GA < 35wks.
• SGA
• LGA
• IDM
• Sick neonate
• Family h/o hypoglycaemia
• Congenital syndromes
• Parenteral nutrition
• Haemolytic disease of newborn 9
Schedule Of Glucose Monitoring
• At Risk Neonates- 2, 6, 12, 24, 48, 72 hrs life.
• Sick Neonates- Every 6-8 hours.
• Neonates On Parenteral Nutrition-
• 1st 72hrs : Every 6-8hrs.
• After 72hrs : Once a day.
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Management Of HypoglycaemiaAsymptomatic
BSL 20-40mg/dl : trial of oral feeds,
Repeat BSL after 1hr
Role of Dextrose Gels
BSL <20 mg/dl: IV Dextrose @6mg/kg/min11
Management Of HypoglycemiaSymptomatic
IV bolus 10% dextrose 2ml/kg
Continuous infusion @ GIR 6-8 mg/kg/min
Increase by 2mg/kg/min,every 30mins
(max 12mg/kg/min)
Continue infusion till 24 hrs after stabilisation,
then taper and stop once GIR 4, BSL >50 consistently12
GIR Calculator
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Recurrent/Resistant Hypoglycemia
• Blood glucose level low despite a GIR 12mg/kg/min
• Stabilisation not achieved despite 7 days of
parenteral glucose therapy.
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Causes Of Recurrent/Resistant Hypoglycemia
Hyperinsulinism(most common cause)
Congenital Acquired
Genetic mutation
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IDM
IUGR
PIH
Birth asphyxia
Polycthemia
Rh incompatibilty
Normoinsulinaemic hypoglycaemia
Counterregulatory hormone deficiency
eg. cortisol def, GH def .
IEM
• Glycogen Storage Disease
• Galactosemia
• Fatty acid oxidation def (MCAD)
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Diagnosis Of Hyperinsulinism
• Based on critical sample i,e when BGL<40 or GIR of 12 is reqd
Critical sample should include
1. Glucose
2. Insulin (>2mIU/ml)
3. Cortisol
4. Beta hydroxy butyrate (<2 mmol/L)
5. Free fatty acid (<1.5mmol/L)
6. Glucagon
(inadequate response to 0.1mg/kg iv glucagon)
*Figures in bracket s/o hyperinsulinism18
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• Besides increasing GIR for resistant hypoglycaemia,certain drugs may be tried.
• Samples need to be taken to investigate the cause before introduction of drugs
Investigations To Be Done In Resistant Hypoglycaemia
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BLOOD URINE
Serum insulin levels, c peptide
thyroid hormones
Urine ketones
Serum cortisone levels Urine reducing substances
GH levels Urine GCMS
Serum ammonia
CASE 1
Baby details-
Full term
Male Child
Date of birth : 12/11/19
Birth weight : 3.6 kg
Mode of delivery : LSCS i/v/o previous previous LSCS
Mother’s Details-
G6A4
Gestation Diabetes Mellitus on Insulin 21
• Clinical Course –
Baby admitted in NICU with asymptomatic hypoglycaemia
(BGL 18) started with full oral feeds & IV fluid D10% with
GIR of 6 mg/kg/min.
Repeat Blood glucose level (BGL) still lowShifted to IV
fluid D12.5 % with GIR of 8 mg/kg/min.
After 24 hours of IV glucose therapy, 2 consecutive BGLs >
50 mg/dl infusion tapered off at the rate of 2 mg/kg/min
every 6 hourly and subsequently stopped. 22
Non persistent/ transient hyperinsulinaemic hypoglycaemia.
• Responded well to parenteral glucose therapy.
• Didnt require critical sample and investigation.
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CASE 2 Baby Details –
Fullterm (39 + 2 weeks)
Date of birth : 03/04/14
Birth weight : 4.22 kg
Mode of delivery : LSCS i/v/o Meconium Stained Liquor
Mother’s Details –
Gravida 3
Bad obstetric history – 2 neonatal deaths, cause unknown
2nd degree Consanguinity
Pregnancy uneventful24
• Clinical Course –
Baby NCIAB, bag and mask ventilation given for 10 sec, admitted in NICU.
On admission – baby pink, euthermic, hairy pinna +,
lethargic, vitals normal, mild respiratory distress +.
Blood glucose level (BGL) with glucose strips –
was not recordable.
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Baby had one seizure episodeIV fluids D10% started with GIR of 6 mg/kg/min along with trophic oral feeds
GIR was increased by 2mg/kg/min upto 16 by DOL 4 despite which baby failed to maintain BGL
Critical sample was sent –Insulin 69.4 mIU/L, Glucose 45 mg/dl Cortisol 110.
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• Baby was started on oral diazoxide @ 10 mg/kg/day later shifted to inj octreotide@10mcg/kg/day after which BGL maintained,
IV fluids were weaned and stopped.
Child still receives injection octreotide monthly.
PET Scan done
Diffuse uptake of dye in pancreas
s/o islet cell hyperplasia
most likely Nesidioblastosis / PHHI
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F-DOPA PET Scan was done - Diffuse uptake of dye in
pancreas s/o islet cell hyperplasia – most likely
Nesidioblastosis/PHHI
F Dopa PET Scan: 100 % specificity and 75 % sensitivity
in detecting focal disease
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Pancreatic beta cell and insulin secretion
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AGENT DOSES ADMINISTRA
TIONSIDE
EFFECTS
Calcium channel blocker
0.25 -0.8 mg/kg/day
Orally, q 8hrly Hypotension,long term safety not known
Diazoxide 10-15mg/kg/day(max 30mg/kg/day)
Oral,q 8hrly Hirsutism,fluid retention,cardiac failure,GI disturbances
Octreotide 7-12mcg/kg/day(max 40mcg/kg/day)
Subcutaneous,q 4-6hrly
Cholelithiasis
Glucagon Bolus 0.5-1mgInfusion 1-20mcg/kg/hr
Intermittent bolusContinuous infusion
HyponatraemiaThrombocytopenia
DexamethasoneHydrocortisone
0.25mg/kg/dose1-2.5mg/kg/dose
Intravenous q12hrlyIntravenous q 6hrly
Growth suppressionHypertension
CASE 3
Baby Details –Fullterm
Male child
Date of birth : 27/10/10
Birth weight : 2.7 kg
Mode of delivery : LSCS
i/v/o fetal distress
Mother’s Details –
Primigravida
No significant history32
•Clinical Course
Baby had seizures on day 1 of life, BSL done – was low.
On examination hyperpigmentation, normal genitalia
Investigations done –
Serum Cortisol – 3.98 (54-287), S 17 – OH Progesterone – raised, S ACTH – 1250 (10-70pg/ml), S Testosterone – 0.47 (3-10ng/ml)
CT Abdomen – Adrenal Gland not visualised
MRI Brain – Normal pituitary gland
Serum Electrolytes – Within normal range
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Treatment –
Started with inj hydrocortisone @ 5mg/kg/day and IV D 10 %
Shifted to oral tablet Hysone
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Congenital Adrenal Hypoplasia
• Frequent cause of adrenal failure in boys.
• Failure of development of adrenal cortex.
• Onset in infancy or 1st year of life.
• Mutation of NROB 1 gene / DAX 1 protein.
• X linked recessive inheritance.
• Hypogonadotropic hypogonadism
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CASE 4• Baby Details –
Fullterm
Birth weight : 2.8 kg
Mode of delivery : LSCS i/v/o previous LSCS
Mother’s Details –
3rd degree consanguineous marriage
G4L1A1D1
D1 – Male child died due to liver disease – jaundice on day of life 15 with abdominal distension, poor feeding & lethargy. USG – Gall bladder not seen, but HIDA scan excretory ? Biliary atresia. Died at 2 months of age
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• Clinical Course –
BCIAB, shifted with mother, started on BF + topfeed, developed icterus DOL 2,had hyperbilirubinemia phototherapy and discharged thereafter.
Baby readmitted and referred to NICU DMH on DOL 15with icterus, lethargy and poor feeding along with hypoglycemia.
On examination – baby was icteric, mild hepatomegaly, other systemic examination were normal.
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• Investigations Direct hyperbilirubinemia (total 13.8, direct 5.16), Deranged coagulation profile (PT – 55/INR 4.47, aPTT 90), SGOT/PT – 79/77, ALP - 1720, GGT – 61, Protein - 7.2 (albumin 3.5), Serum electrolytes 137/6.2, Ammonia 159, Lactate – 7.2, Serum ketones – absent, Urine acetone – negative, urine NGRS+ve.
USG Abdomen done – gall bladder not visualised.
Ophthalmic examination revealed –
b/l eyes nuclear sclerosis with normal fundus.38
Baby was started on ISOMIL (non lactose containing milk substitute), injection meropenem & Vit. A, D, E, K
Blood culture revealed candida positivity – started on injection fluconazole, also urine culture was positive for klebsiella pneumoniae sensitive to colistin and was started with same.
Galactose Epimerase enzyme assay was normal
GALT (Galactose – 1 – phosphouridyl transferase) enzyme assay was low.
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GALACTOSEMIA
• 2 forms( classic/complete transferase deficiency and partial transferase deficiency)
• Starts from 2nd half of 1st wk of life
• Accumulation of galactose 1P (injury to kidney,liver,brain)
• Newborns present with heptaomegaly,cholestasis,seizures,gram -ve sepsis,cataract
• Injury may begin prenatally d/t transplacental glucose transfer
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GALACTOSEMIA
• 2 forms( classic/complete transferase deficiency &partial transferase deficiency).
• Starts from 2nd half of 1st wk of life.
• Accumulation of galactose 1P (injury to kidney,liver,brain)
• Newborns present with hypoglycaemia, heptaomegaly,
• cholestasis,seizures,gram -ve sepsis,cataract.
• Injury may begin prenatally d/t transplacental galactose
transfer.
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CONCLUSION
• Hypoglycaemia is a biochemical sign of a variety of diorders of varying degrees of significance.
• It presents either with non specific symptoms or asymptomatically.
• It can have profound long term neurological consequences (20% of PHHI cause significant brain damage)
• The critical sample being drawn when the patient is hypoglycaemic is crucial to make the diagnosis.
• Treatment depends on the underlying condition.
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