Pediatric

1. Adrenal gland
Dr. Noura Noraddin
Faculty of medicine

2. Adrenal gland:
It is located above the kidney
It secretes different types of hormones
It has 2 parts:
 Cortex
 Medulla
 Cortex:
 Zona glomeruloza
 Zona fasiculata
 Zona reticularis

3. Adrenal gland cortex hormones:
3 zones:
 Zona glomerulosa
 Zona fasciculate
 Zona reticularis
It is responsible for:
3 S hormones
Salt hormones
Sugar hormones
Sex hormones
3/S

4. Adrenal gland cortex hormones:
ZG:
Salt hormone:
Mineralocorticoids:
Aldosterone& Corticosterone
ZF: Sugar hormone:
Glucocorticoids:
Cortisol& Cortisone
ZR: Sex hormone:
Androgens:
Estrogen& Testosterone
3/S

5. Adrenal gland medulla hormones:
Catecholamines:
 Adrenaline
 Noradrenaline
Hormone of fight or flight

6. Adrenal gland medulla hormones:
Catecholamines:
 Adrenaline
 Noradrenaline
Acute adrenal insufficiency:
Low aldosterone& corticosterone:
Abdominal pain, vomiting, diarrhea& dehydration
Delirium or confusion, hypoglycemia& general weakness
in addition to hypotension, hyperkalemia, hyponatremia& shock

7. Chronic adrenal insufficiency:
Addison disease:
It grows very slowly over months
Suppression of gland by prolonged steroid use
When the patient developed any stress as illness it becomes clear
Weakness, skin pigmentation& striae, hypotension, hypoglycemia,
Anorexia, salt craving, abdominal pain, muscle pain, depression, hair loss,
sexual dysfunction.

9. Causes of Addison disease:
ADDISON
 Autoimmune
 Drugs: ketoconazole, DIC
 Degenerative: amyloidosis
 Infectious agents: TB& HIV& iatrogenic
 Secondary causes: hypopituitarism
 Other causes: adrenal hemorrhage
 Neoplasia& Nelson syndrome

10. Clinical picture of Addison disease:
FATIGUED
 Fatigue
 Antibodies anti-adrenal- antithyroid, antiparietal cells
 Triad of: hyponatremia. Hypokalemia& azotemia
 Increased pigmentation of skin& tongue
 Gastrintestinal S: anorexia& weight loss
 Nausea& vomiting
 Electrolytes disturbance
 Dehydration

11. Diagnosis of Addison disease:
 ACTH stimulation test
 With tetracosactisone

13. Cushing syndrome causes:
Exogenous:
Iatrogenic   CRH& ACTH
Endogenous:
Pituitary adenoma:  ACTH
Para-neoplastic tumor
Adrenal carcinoma or nodular adrenal hyperplassia

14. Cushing syndrome investigation:
Dexamethasone suppression test
If ACTH pituitary tumor:
 Cortisol after low dose
 Cortisol after high dose
If ectopic ACTH:
 Cortisol after low& high dose

15. Cortisone excess= Cushing syndrome
Clinical picture:
 Generalized weakness
 Bone osteoporosis
 Hyperglycemia
 Mood disorder
 Moon face
 Glaucoma
 Cataract
 Obesity
 Striae
 HTN
 CMP
Lemon on sticks

16. Cortisone excess= Cushing syndrome
Clinical picture:
MOON FACE
 Menstrual changes
 Osteoporosis
 Obesity
 Neurosis
 Facial plethora
 Altered muscle
 Calor (hot skin)
 Elevated blood pressure

20. In labor room receiving newborn:
Write birth certificate:
Age& Sex

22. Disorders of the sex:
Congenital& acquired
Congenital adrenal hyperplasia:
Gives the clinical picture of ambiguous genitalia ‫المبهمة‬ ‫التناسلية‬ ‫األعضاء‬
Common form is virilized female due to OCP during pregnancy

23. Phenotype=genotype
True hermaphroditism:
Both male& female genitalia are present
Pseudohermaphroditism:
Only one sex genitalia present but it is abnormal;
Large clitosis& labia majora in females
Small penis& undescended testis in males

33. Blood test for:
ACTH
Cortisol
Electrolytes
Progesterone
17- ketosteroids
21-hydroxylase
Karyotyping:
Chromosomal study

34. Abdominal U/S for:
Internal organs as uterus& vagina for females
Or undescended testis in males
Ovotestis
Biopsy& histopathology:
Buccal smear for bar bodies
Determine the real sex
Ovo-testis of abdominal gonad in rare cases (true hermaphrodite)

35. Treatment:
 Identify sex early
 Give the name clearly
 Dress& behavior must go with sex
 Give hormonal replacement therapy
 Surgical reconstruction of genital organs
 Hydrocortisone
 Flodrocortisone
 Estrogen or testosterone

39. Sex hormones:
Delayed puberty& precocious puberty
Precocious puberty:
Development of secondary sex characters before the expected age
Usually before 10 years in boys& before 8 years in girls.
Secondary sexual characters:
 Breast
 Acne vulgaris
 Change in voice
 Dark coarse pubic hair
Secondary sexual characters:
 Thelarche
 Pubarche
 Menarche

41. True precocious puberty:
Occurrence of spermatogenesis or ovulation due to activation of:
Hypothalmic-pituitary axis LH&FSH
Causes:
 Infection
 Trauma
 Tumor
Pseudoprecocious puberty:
Appearance of secondary sexual characters only due to:
Increase androgen-estrogen pathway

42. Investigations:
Radiology:
Bone age
Brain tumor
Endocrinology:
17-ketosteroid, pregnandiol, FSH&LH
N.B.
Dangerous in boys!
It is mostly malignant tumor

43. Management:
Search for the cause of abnormal hormone secretion
Give counter hormonal therapy
Surgical treatment

44. Causes of gynecomastia: MAKE BREAST
 Marjuana
 Alcohol
 Klinefelter syndrome
 Estrogen excess
 Baby ( maternal estrogen)
 Receptor blocker (kketoconaole, calcium blocker, H2 blocker)
 Elderly
 Antineoplastic agents
 Spironolactone
 Tumors ( adrenal or testicular)

45. Medulla hormones:
Adrenaline, noradrenaline& dopamine
They are fight or flight hormones
They help the body to respond to stress by Adrenaline rush
Pheochromocytoma: is a tumor of adrenal medulla
Neuroblastoma is a tumor of nerve ganglia or medulla

46. Pheochromocytoma:
10% familial
10% malignant
10% extra-adrenal

47. Symptoms of pheochromocytoma:
Abdominal pain
Constipation
Weight loss
Headache
Anxiety
Signs of pheochromocytoma:
Hypertension
Hyperglycemia
Tachycardia
Numbness
Sweating
Tremor
Pallor

48. Symptoms of pheochromocytoma:
6 Ps
Pallor
Palpitation
Perspiration
Panic attacks
Pain of the head
Postural dizziness
Paradoxical hypertension

49. Clinical picture:
Abdominal bloating
Watery diarrhea
Hypertension
Anemia
Fever

50. Investigations:
Urine VMA
Blood …. Tests…
Treatment:
Supportive& specific
Control hypertension by labetalol
Surgery & radiation of the tumor

51. The role of androgens:

52. Thank you