Successfully reported this slideshow.
We use your LinkedIn profile and activity data to personalize ads and to show you more relevant ads. You can change your ad preferences anytime.

Pediatric Hypoglycemia

case presentation of hypoglycemia, Approach to hypoglycemia, pathophysiology, differential diagnosis, treatment and management, comparison and case presentation

  • Login to see the comments

Pediatric Hypoglycemia

  1. 1. PEDIATRIC HYPOGLYCEMIA Dr. Fahad Fayyaz Butt Pediatric Resident 2
  2. 2. CASE: 2y, boy Complaints: Vomiting  2days Loose stools-2days lethargic 1 day
  3. 3. INITIAL IMPRESSION: •Lethargic and sick lookingConsciousness •deep rapid breathingBreathing •PaleCirculation
  4. 4. PRIMARY ASSESSMENT •MaintainableAirway •Deep rapid breathing 50/min, Spo2 99%Breathing •Delayed capillary refill >3secs, HR: 150/m, BP 85/50Circulation •Lethargic arousable to voice, Glucose: 40mg/dlDisability •No rash, Temp:37CExposure
  5. 5. SYSTEMIC EXAM: CNS: lethargic, no neck stiffness , negative kernig and brudzinski , closed fontanelle, no focal deficit CVS: S1+S2+No murmur, no gallop or muffled heart sounds + weak peripheral pulses GIT: Soft, No hepatosplenomegaly, no mass, non-tender Chest: Bilateral equal air entry, no crackles ENT: Normal throat and ears
  6. 6. CBG : Hypocount: 40 mg/dl pH: 7.03 PCO2: 6.5 PaO2: 80 HCO3: 1.6 Base Excess: -28 Lactate: 13.9 Electrolytes: normal
  7. 7. SEVERITY:  Respiratory Distress Vs. Respiratory failure Hypovolemic shock; Compensated Shock DIFFERENTIAL DIAGNOSIS: Acute Gastroenteritis with severe dehydration Sepsis Causes of Anion gap Metabolic Acidosis Inborn error of metabolism
  8. 8. TREATMENT GIVEN IN EMERGENCY : IV 0.9% NaCl Boluses IV D10% Bolus Working Diagnosis: •Gastroenteritis and Severe Dehydration
  9. 9. Past medical history: No prior hospitalization or a severe presentation in ER Patient sometime looks sweaty at night and wakes up hungry. Birth History: Term , NVD, Admitted in NICU after birth due to hypoglycemia for few hours , Birth Weight: 3.5kg (mother has no reports) Vaccination: Current Nutritional: On regular family diet, high amount of milk intake. Development: Age -appropriate Family history:  one of two siblings.  Consanguineous marriage , both parents are well  No history of miscarriage, still born or developmental delay, significant Metabolic disorder in family
  10. 10. SUMMARY: 2 year old boy Vomiting/loose stool and lethargy for 2 days Severe dehydration with hypoglycemia CBG with Metabolic acidosis No significant past medical/family history. Patient sometime looks sweaty at night and wakes up hungry.
  11. 11. INVESTIGATIONS: Labs: Patient value CBC WBC 33.1 x 10^3 ABN:22.9 HGB 9.3 g/dl PLT 587 x 10^3 CRP <5 mg/L LFTs Albumin 4.9 g/dL ALP 283U/L ALAT 181 U/L T. Bil 0.1 mg/dL Total Protein 8.3 g/dL PT 12.5 secs APTT 32.4 secs INR 1.39
  12. 12. Labs: Patient value Reference Value Urea and electrolytes Sodium 135 135-145 Potassium 4.1 3.5-5.5 Chloride 106 ? HCO3 5 18-25 Urea 31 ? creatinine 0.3 ? CPK 123 0-228
  13. 13. Labs: Patient value Blood Culture No growth Urine Routine WBC: 0-3 RBC: Nil Culture No growth Stool Routine No Pus Cells No RBCs No Ova And Parasites Culture No Growth Rota & Adenovirus Negative Ammonia 43 umol/L , ref: 17-68 INVESTIGATIONS:
  14. 14. COURSE IN THE HOSPITAL: Day 1: •Admitted to ICU •IV NaHCO3 •IV D10% +0.45%NaCl •IV Ceftriaxone •Clinical Status: Markedly Improved • After 24hours in ICU: • CBG: pH: 7.4, pCO2: 32, HCO3: 20.1 • Lac:2.7 Glu: 85mg/dl
  15. 15. Day 2 & 3 : Shifted to ward Full Oral ,Vitally stable, had no episode of fever Discharged with instructions
  16. 16. Review of Differential Diagnosis: Acute Gastroenteritis with severe dehydration Sepsis (unlikely) Causes of Anion gap Metabolic Acidosis (tissue hypoxia) Inborn error of metabolism
  17. 17. Since then Child was admitted in Hospital for a total of five times in one year with Identical picture: Chief complaints Blood gas picture : Low blood glucose and metabolic acidosis Course in hospital PROGRESS
  18. 18. 2ND EPISODE: Complaints: runny nose and cough ,fever and vomiting. hypocount: 35mg/dl in ER CBG showed severe Metabolic Acidosis Critical Sample was taken
  19. 19. Indications for critical Sample : 1. Recurrent hypoglycemia 2. Not explained by history and physical examination
  20. 20. HOW TO COLLECT THE SAMPLE: 1. Gray-top tube :Glucose, lactate, 2. Red -top tube : Insulin, GH, cortisol, 3. Filter Paper: Acylcarnitine profile 4. Dark green-top tube : FFA, 5. Lavender-top tube : C-peptide from 2 mL in a lavender-top tube (0.5 mL minimum). 6. Send next voided urine for quantitative determination of organic acids, reducing substances and ketones
  21. 21. CRITICAL SAMPLE IN LH Blood FFA Insulin C-peptide Growth Hormone Cortisol Lactate Acylcarnitine on filter paper Ammonia Urine Urinary Ketones Urinary Organic acids Urinary Reducing Substances
  22. 22. HYPOGLYCEMIA • Definition • Clinical Features • Pathophysiology • Differential diagnosis • History and PE • LH approach • Management
  23. 23. DEFINITION OF HYPOGLYCEMIA : Laboratory Serum: less than 50mg/dl Whipple’s triad
  24. 24. Hypoglycemia Insulin Hepatic Glucose output Glucose Glucagon Epinephrine Lipolysis FFA and glycerol Ketones Growth Hormone Insulin Sensitivity Glucose utilization Cortisol Protein Breakdown gluconeog enesis
  25. 25. Excessive Utilization of Glucose: Hyperinsulinism Defect in Ketogenesis Defect in Ketolysis Fatty acid Oxidation Defects Under Production of Glucose: Glycogen storage disease Gluconeogenesis defect Hormonal Defects: Cortisol or growth hormone deficiency
  26. 26. HYPERINSULINISM Insulin Glucagon Gluconeo GENESIS Glyco GENESIS Lipo GENESIS Hypoglycemia Pathogenesis FFA Low Insulin High C-peptide High Growth Hormone High Cortisol High Lactate Normal AcylCarnitine Low Urinary Ketones Low Urinary Organic Acids Absent
  27. 27. CORTISOL DEFICIENCY Cortisol Reduced protein Breakdown Gluconeo GENESIS Hypoglycemia Pathogenesis FFA Raised Insulin Low C-peptide low Growth Hormone High Cortisol Low Lactate Normal Acylcarnitine Normal Urinary Ketones Raised Urinary Organic Acids Absent
  28. 28. GROWTH HORMONE DEFICIENCY Growth Hormone Insulin sensitivity Peripheral glucose Uptake Hypoglycemia Pathogenesis FFA Raised Insulin Low C-peptide low Growth Hormone Low Cortisol High Lactate Normal Acylcarnitine Normal Urinary Ketones Raised Urinary Organic Acids Absent
  29. 29. GLUCONEOGENESIS DEFECTS 1. Glu. 6 Phosphatase 2. Fru. 1.6 Diphoshaphatase 3. PEP Cabroxykinase Gluconeo GENESIS Hypoglycemia Pathogenesis FFA Raised Insulin Low C-peptide Low Growth Hormone High Cortisol High Lactate High Acylcarnitine Normal Urinary Ketones High Urinary Organic Acids Absent Lactate Alanine Glycerol 3 P
  30. 30. GLYCOGENOLYSIS DEFECT 0, Gly. Synthase 3, Debranching Enzy. 6, Liver Phophorylase Enzy. 9, Phosphorylase kinase Glucose Release Hypoglycemia Pathogenesis FFA Raised Insulin Low C-peptide low Growth Hormone High Cortisol High Lactate Normal to High Acylcarnitine Normal Urinary Ketones high Urinary Organic Acids Absent Ketone production
  31. 31. FATTY ACID OXIDATION DEFECTS Acyl CoA Dehydrogenase Beta oxidation of FA Hypoglycemia Pathogenesis FFA High Insulin Low C-peptide low Growth Hormone High Cortisol High Lactate Normal to High Total carnitines Total Acylcarnitines Acylcarnitine Urinary Ketones Low Urinary Organic Acids Dicarboxylic acids Ketone production
  32. 32. KETOGENESIS DEFECTS HMG CoA Lyase HMG CoA synthase Ketone production Hypoglycemia Pathogenesis Urinary Ketones Low Urinary Organic Acids Dicarboxylic acids FFA High Insulin Low C-peptide low Growth Hormone High Cortisol High Lactate Normal to High Total carnitines Total Acylcarnitines Acylcarnitine
  33. 33. KETOLYSIS DEFECTS SCOT def. Beta Ketothiolase deficiency Ketone utilization Hypoglycemia Pathogenesis FFA High Insulin Low C-peptide low Growth Hormone High Cortisol High Lactate Normal acylcarnitines - Urinary Ketones High Urinary Organic Acids -
  34. 34. Excessive Utilization of Glucose: Hyperinsulinism Defect in Ketogenesis Defect in Ketolysis Fatty acid Oxidation Defects Under Production of Glucose: Glycogen storage disease Gluconeogenesis defect Hormonal Defects: Cortisol or growth hormone deficiency
  35. 35. HISTORY: History Of presenting Illness Age: • Neonate to 2 years of life: MC age of Presentation • Toddlers or older children: Toxin intake Trigger: • Period Fasting Specific Foods • Milk products • fruit juices Past Medical History • Prior ER visit or Hospitalizations: labelled as seizure disorders or other disorders. Birth History • Antenatal Preeclampsia , GDM • Natal LGA • Postnatal Hypoxic injury at birth Family history • Unexplained deaths in family • Affected members in family Developmental Milestones • Appropriate for age
  36. 36. PHYSICAL EXAM: General Examination: Dysmorphic features: Mid facial defects Growth Charts : Failure to thrive Short stature Neonate: Macrosomia Vital Signs RR: Deep rapid breathing Temperature: Hypothermia or fever Systemic Examination: GIT: Hepatomegaly Umbilical hernia or omhpalocele Skin : Hyperpigmentation Eye: Cataract Genitalia Ambiguous genitalia
  37. 37. APPROACH
  38. 38. Hypoglycemia Urine Ketones Non-glucose Reducing substances Galactosemia H. Fructose Intolerance Approach Continued…
  39. 39. Ketones Positive Negative Hyperinsulinemia + Low FFA + High Insulin And C-peptide FA Oxidation Defects +High FFA +Undetectable Insulin & C- Peptide Approach Continued…
  40. 40. Ketones Positive Serum Lactate High Normal 1. Growth Hormone 2. Adrenal Insufficiency 3. Idiopathic Ketotic Hypoglycemia Approach Continued…
  41. 41. High Serum Lactate Hepatomegaly Glycogen storage disease GSD1 Gluconeogenesis defects No Hepatomegaly Organic Acidopathy Ketolysis Defect
  42. 42. TREATMENT
  43. 43. Glucose <50mg/dl Conscious Oral trial Recheck Drowsy IV access IV D10% Commence IV infusion <50mg/dl: adjust infusion >50mg/dl: Introduce oral feeds No IV access IM Glucagon Target: 60- 140mg/dl
  44. 44. HYPOGLYCEMIA • Definition • Clinical Features • Pathophysiology • Differential diagnosis • History and PE • Critical sample • LH approach • Management
  45. 45. CRITICAL SAMPLE OF THE PATIENT Blood Patient values Normal range FFA Not done? - Insulin 3.2 4.0-16 C-peptide 1.8 1.8-4.7 Growth Hormone 5.2 0-3 Cortisol 1681 69-328 Lactate 11.4 0.5-2.2 Acylcarnitine on filter paper Increased Level of all Acylcarnitine Total and Free Carnitines 70.4 39.4 35.0-84.0 24.0-63.0
  46. 46. CRITICAL SAMPLE IN LH Urine Patient Values Urinary Ketones 3+ Urinary Organic acids Lactic Acids Urinary Reducing Substances +Glucose (taken after glucose infusion) Urinary Amino Acids All in normal range except borderline increased levels of Alanine, Valine , Phenylalanine and Leucine
  47. 47. OTHER TESTS Tests Values Normal Range TSH 1.37 0.80-6.26 T3 4.7 3.96-8.14 T4 13.8 10.45-22.35
  48. 48. 2 year old boy Vomiting/loose stool and lethargy for 2 days Admitted 5 times with Severe dehydration & hypoglycemia No significant past medical/family history. Patient sometime looks sweaty at night and wakes up hungry. Labs: 1. CBG: Metabolic acidosis with hypoglycemia 2. Critical Sample: Lactic acidosis , rest of profile normal 3. Urinary tests done: Ketone 3+ 4. Other tests: CPK: Normal , TFTs: Normal Case : Ketotic Hypoglycemia for evaluation
  49. 49. Glucose Acidosis Lactic acid Ketones Hepatomegaly Others tests Patient Low Present Present Present Absent Normal, mild deranged LFTs Hyperinsulinism Low Absent Absent Absent Absent Insulin and C- peptide FA Oxidation defects Low Absent Absent Absent Present Acylcarnitine profile Elevated dicarboxylic acids Hormone deficiency Low Present Absent Present Absent Hormone levels P.E. Gluconeogenesis Low Present Present Present Present Hyperuricemia Hyperlipidemia Glycogen storage Disease Low Present +/- Present Present Present Deranged LFTs Ketogenesis defects Low Present Absent Absent Present Elevated acetoacetate levels Ketolysis Defects Low Present +/- Present Present Absent Elevated dicarboxylic acids
  50. 50. Questions and suggestions

×