Common metabolic problem Blood glucose in newborns are generally lowerthan older children & adult Fetal glucose level maintained at 2/3 ofmaternal B.glucose by transplacental route Glucose level fall in Ist 1-2 hrs,lowest value atage of 3 hrs, increase and stabilise by 4 hrs. New born – glycogenolysis, gluconeogenesis andexogenous nutrients.
Defined as a blood glucose level of <40mg %regardless of gestational age and whether ornot symptoms are presentWhipple’s triad: low glucose level documented by accurate labmethod Signs and symptoms of hypoglycemia Resolution of signs and symptoms onrestoration of blood glucose levels.
Fetal or Neonatal Hyperinsulinism –↑utilisation of glucose. Decreased production or store Increased utilisation and/or decreasedproduction
Fetal or Neonatal Hyperinsulinism –↑utilisation of glucose. Babies born to Diabetic mothers(15-25 %GDM,25-50% DM) LGA infants-16% Erythroblastosis Islet cell hyperplasia Beckwith-weidemann(macrosomia,microcephaly,omphalocoele,macroglossia,visceromegaly).
Insulin producing tumours(islet celladenoma). Maternal therapy with tocolytics liketerbutaline,ritodrine, OHA and diuretics(chlorothiazide) Glucose infusion through UAC –highglucose into celiac,SMA—stimulate insulinfrom pancreas
Decreased production or store: Prematurity IUGR (15% in SGA) Inadequate calorie intake Delayed onset of feeding
Increased utilisation or decreased production: Perinatal stressSepsis/shock/asphyxia/respiratorydistress/hypothermia/post resuscitation. Exchange transfusionHeparinised blood with low glucose levelCPD blood (relatively hyperglycemic---reactive hypoglcemia Defects in carbohydrate metabolismGlycogen storage diseaseFructose intoleranceGalactosemia
Endocrine deficiencyAdrenal insufficiencyHypothalamic deficiencyHypopituitarism(neonatal emergencies such as apnea, cyanosis, or severehypoglycemia with or without seizures, hyperbilirubinemia, andmicropenis. )Glucagon defEpn deficiency Defects in amino acid metabolismMSUD,propionic acidemia,MMA,tyrosinemia
Polycythemia-higher glucose utilisation by increased mass of RBC Maternal therapy with beta blockers-Prevention of symp stimulation of glycogenolysis&epinephrine induced increase in FFA
SYMPTOMSTremors,jitteriness,irritability,seizures,lethargy,poor feeding,vomiting ,limpness,weak orhigh pitched cry ,cyanosisASYMPTOMATIC. MEASURMENT OF BLOOD GLUCOSEglucometer- 15% lower than plasma levelsLab diagnosis-sample obtained and analyzedpromptly (18mg/dl/hr) CLINICALCONFIRMATION-whipples triad
The major long-term sequelae ofsevere, prolonged hypoglycemia are mentalretardation, recurrent seizure activity, or both. Permanent neurologic sequelae are present in 25–50% ofbabies with severe recurrent symptomatichypoglycemia These sequelae are more likely when alternativefuel sources are limited, as occurs withhyperinsulinemia Anticipation and prevention –key tomanagement of infants with risk factors for HG
Routine screening in babies with riskfacors SGA/Smaller of the discordant twin IDM/LGA Preterm <35 weeks On IVF/TPN Prolonged hypoxia/hypothermia/polycythemia/septicemia/ suspectedIEM
After exchange tranfusion Rh Hemolytic d/s Babies born to mothers on terbutaline/b-blockers/OHA Symptomatic babiesScreening within 1 hr of birth IDM-0,1,3,6 ,12,18.24,48,72 hrs For 72hrs - risk babies ET-2 hrs after infusing CPD blood
Asymptomatic 25-40mg% <25mg% Trial of feeds Parenteral >40 <40 Continue oral feedsand monitor for 48 hrs
Early feeding with glucose water raises BG onlytransiently and asso with rebound hypoglycemia Early introduction of breast feedso maintain stable BG levels without rebound HGo keep ketone levels high---alternate fuel during 1stfew days while baby adapts to DBFo enhances gluconeogenesis
IV therapyIndications – intolerance to oral feeds Symptomatic oral feeds not maintaining glucose levels BG level < 25mg/dl
o IV glucose through a peripheral line or UVCo Urgent treatment- 2 ml/kg(200mg/kg) of 10%dextrose over 2-3 min.o Severe distress – 2-4 ml/kg 25%D(1g/kg glucose)@ 1ml /kg/mtFor eg 2 kg infant-4-8 ml of 25% Dex in 2-4mto In asymptomatic baby with low BG levels initialpush of conc sugar →→hyperinsulinism.Therfore, infusion 5-10 ml of 10% D at 1 ml/mt
Continuing therapy – based on Glucose Infusion RateGIR(mg/kg/min) = % dextrose x ml/kg/day144For eg.86 ml/kg/day of 10% D--GIR 6-8[GIR of 8.33 = 80ml/kg/day of 15%D]
Monitor BG hourly till euglycemic and thereafter 6thhrly If BG > 40mg%,Continue same and monitor When 2 BG values >50 mg%,wean GIR by 2mg/kg/mt6th hrly and start oral feedsStop infusion when baby is stable @4mg/kg/mt for 12hrMonitoring stopped when 2 values on oral feeds>50mg%
If BG < 40 mg%Repeat bolus & increase GIR by 2mg/kg/mtevery 6 hr till euglycemicIf GIR >12 orHG not resolving by day 7steroids/glucagon/diazoxideFurther investigations
Check blood glucose after 30 mts of everychange in infusion rate Monitoring of glucose levels--to ensure adequate correction ofhypoglycemia-To avoid hyperglycemia---diuresis---dehydration
<2kg –parenteral therapy in the 1st hour oflife >2 kg- can be fed hourly, for 3 or 4 feeds,and then 2 hrly As interval increase ,vol ↑ If by 2 hrs ,despite feeding GRBS< 40 mg%--parenteral therapy
Hydrocortisone 10mg/kg/day in 2 div doses MOA-decrease peripheral glucoseutilisation, increase gluconeogenesis,increaseeffects of glucagon Rapidly tapered off in few days Before administration of HC ,obtain bloodsamples for insulin and cortisol levels
Glucagon Mobilising hepatic glycogen stores Infants with good glycogen stores Not in preterms and malnourished 0.025-0.3 mg/kg IM Diazoxide (2-5mg/kg q8h PO) – in persistenthyperinsulinemia Epinephrine Subtotal pancreatectomy