PEDIATRIC ENDOCRINOLOGY😊

PEDIATRIC ENDOCRINOLOGY

Q. A 5-year-old baby presents with recurrent hypoglycemia, short stature, and micropenis. His bone age is found to be 2.5 years. What is the diagnosis?
Ans.
Congenital hypopituitarism

Isolated GH Deficiency

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  • GH does not play a role in the growth of the fetus.

Characteristic of GH deficiency

  • Short stature
    • Proportionate short stature:
      • US: LS ratio normal
  • Obesity
  • Birth weight & length are normal at birth

Delayed

  • Dentition
  • Puberty
  • Milestones
  • Bone age < chronological age
  • High pitched voice
  • Can be genetic

Diagnosis

  • 1. GH stimulation test
    • Dynamic testing
      • Done using arginine/clonidine/insulin glucagon, levodopa
      • Insulin like growth factor with OGTT stimulation
    • Peak GH level <10 ng/ml
      • GH deficiency
  • 2. Bone age <Chronological age

Treatment

  • Recombinant GH injections
    • subcutaneously for 5 days /week
      • with a gap of 2 days
        • (before the fusion of the epiphysis)
    • For at least 1-2 years
      • for the results to be apparent

Adverse Effects of GH Therapy

  • Pseudotumor cerebri
  • Gynecomastia
  • Impaired glucose tolerance

Adrenal Disorders

The adrenal cortex of the fetus releases

  • In early pregnancy:
    • Cortisol
  • In 2nd trimester:
    • DHEA

The fetal adrenal gland primarily produces 

  • Dehydroepiandrosterone sulfate (DHEA-S)
  • crucial for growth and development.

Fetal growth

  • D/t Fetal Insulin / IGF
  • Not d/t Maternal Insulin / GH
    • Insulin do not cross Placenta
  • Normal Newborn
    • Fetal Hyperglycemia
      • Stimulates Insulin → Fetal growth
  • In Newborn of Diabetic mother
    • Maternal hyperglycemia → ↑↑↑ Fetal insulin → Macrosomia
    • Fetal Hypoglycemia

Cushing Syndrome

https://www.notion.so/Endocrinology-2350a7eb0c778013b881eae70ee85bf0?source=copy_link#2360a7eb0c7780cca2dcf2bd90362bea

21 Hydroxylase Deficiency

https://www.notion.so/Disorders-of-Sexual-Development-DSD-in-46-XX-2200a7eb0c7780eeab70eaf702e48085?source=copy_link#2200a7eb0c778011a086dcd87f2958bd

Congenital Hypothyroidism

https://www.notion.so/Thyroiditis-and-Hypothyroidism-20b0a7eb0c77808e96a1d4702a40717e?source=copy_link#2450a7eb0c77801b806ed3ff66b20ba8

Diabetic Ketoacidosis in Children

Q. A 10-year-old child presented with tachypnea and severe dehydration. His random blood glucose was 524mg/dl, ABG showed a pH of 7.0, HCO3- of 5mmol/L, and urine showed moderate ketones. How do you manage the case?
  • Ans. Severe Diabetic ketoacidosis

Diagnostic Criteria

  • Blood glucose >200 mg/dl along with metabolic acidosis (pH <7.3) or
  • HCO3 <18 mEq/L along with ketonemia or moderate/large ketonuria.

Management

  • In the 1st hour:
    • Normal saline
      • Mild - 10 ml/kg
      • Moderate to severe- 20 ml/kg
      • The amount of fluid is decreased from the total fluid correction in dehydration correction
      • Shock- rapid bolus of normal saline
  • After 1st hour
    • Start regular insulin infusion @ 0.05-0.1 U/kg/hr continued till acidosis has improved
    • Dehydration correction over 48 hrs
    • Slow transition of subcutaneous insulin and diabetic diet
  • Other measures
    • Treat infections
    • Fever