Addisons disease

1. Welcome to clinical meeting
Dr. Eshita Reza Khan
Resident phase A
Pediatric hematology and oncology

2. • Name: Saif
• Age: 8 years,
• Sex: male,
• Address: Pilkhana,
• Date of admission:13. 12. 15.

3. • Chief complaints:
– Progressive darkening of skin – 3 years,
– Recurrent fever – 3 years,
– Generalized weakness and weight loss – 3 years.

4. • H/O Present Illness:
– According to the statement of the informant
mother, the child was reasonably alright 3 years
back. Then he developed progressive darkening
of skin, involving the face, lips, gums and over
bony prominences of the body.

5. • Mother also complains of recurrent fever for the
last 3 years, lasting for several days 1-2 times
every month. For the last 1 month, fever is high
grade and continued in nature, not associated
with chills, rigor or drenching night sweat and
subsided by taking antipyretics.

6. • Mother also told that her child is getting lean and
thin day by day and gives H/O losing 5 kg weight in
the last 6 months.
• The patient also has H/O generalized weakness,
fatigue, less playfulness, poor appetite and cramping
leg pain. He likes salty food and sometimes eats table
salts alone.

7. • But, there is no H/O vomiting, unconsciousness,
convulsion, severe dehydration requiring I/V fluid,
contact with TB patient.
• For above complaints, the child was being treated with
several antibiotics with no response. So he was admitted
in BSMMU for better evaluation and management.

8. • Birth history: uneventful.
• Developmental history: age appropiate,
• Immunization history: as per EPI schedule,
• Past illness:
– Recurrent otitis media, treated by tympanoplasty 1 year
back.
– Fever with convulsion (encephalitis) at 3 years of age.

9. • Family history:
– 2nd issue of consanguineous parents. No F/H of similar
illness.
• Social and personal history:
– Father is a businessman, mother is a housewife, the child
reads in KG.

10. • General examination:
– Appearance:
• Ill looking,
– Pallor: absent,
– Jaundice: absent,
– Cyanosis: absent,

11. – Clubbing: absent,
– Koilonychia: absent,
– Leuconychia: absent.
– Oedema: absent,
– Dehydration: absent.

12. • Skin survey:
– Hyperpigmentation of face,
lips, gingiva, bony prominences.
– BCG mark present.
• Lymph nodes: not palpable,
• Bony tenderness: absent,
• Thyroid: not enlarged.

13. • Vital signs:
– Pulse: 110 beats/minute,
– BP:
• Supine: 70/40 mm of Hg (below the 5th centile for age
and sex)
• Sitting: 50/30 mm of Hg  postural hypotension.
– Temperature: 101˚ F.

14. • Anthropometry:
– Weight: 21 kg, on 5th centile for age,
– Height: 137 cm, on 95th centile for age.
– BMI: 11.2 kg/m², - 4 SD for age and sex.

15. • Locomotor system:
– Look: generalized muscle wasting,
– Feel: generalized muscle tenderness,
– Move: knee extension is restricted due to pain.

16. • Gastro-intestinal system:
– Mouth and oral cavity:
• normal,
– Abdomen:
• Inspection: normal in shape, umbilicus central,
• Palpation:
– Superficial: no tenderness or mass.
– Deep: liver and spleen not palpable, fluid thrill absent,
• Percussion: shifting dullness absent.
• Auscultation: bowel sound present.

17. • Nervous system:
– Higher psychic function: normal,
– Cranial nerves: intact,
– Motor:
• Bulk of the muscles: symmetrically reduced,
• Tone: normal
• Power: normal
• Jerks: normal
• Planter: flexor,
• Co-ordination: present
• Gait: normal
– Sensory: intact.

18. • Respiratory system:
– Inspection: shape of the chest wall normal, no sucostal/ intercostal recession,
– Palpation:
• Trachea central
• Apex in the left 4th ICS medial to midclavicular line,
• Chest expansion normal
• Vocal fremitus normal,
– Percussion: resonant.
– Auscultation:
• Breath vescicular,
• No added sound,
• Vocal resonance normal.

19. • Cardiovascular system:
– Pulses: normal,
– BP: hypotensive with postural hypotension.
– JVP: not raised,
– Precordium:
• Apex beat in the left 4th ICS medial to MCL,
• No thrill, palpable P2 or left parasternal heave,
• S1 and S2 normally audible in all precordial areas with no
murmur.

20. • Salient feature:
– Saif, 8 years old boy, 2nd issue of consanguineous parents
from Pilkhana, Dhaka, was admitted with complaints of
progressive darkening of skin for the last 3 years, More
affected are the face, lips, gums and the skin over bony
prominences. He also has H/O recurrent fever for last 3
years, which is high grade and continued in nature for the
last 1 month.

21. • The child also has H/O cramping muscle pain, craving for salt
and salty food, generalized weakness, fatigue and significant
weight loss.
• But there is no H/O vomiting, convulsion, unconsciousness
or contact with TB patient.

22. • On examination, the child was ill looking, emaciated,
hyperpigmented skin, more in the face, lips and gingiva and
bony prominences. Patient was febrile (101˚F), hypotensive
for age and sex with postural drop, BMI was 11.2 kg/m²(-4
SD), all other systemic examination revealed no
abnormalities.

23. Provisional diagnosis
?

24. Provisional diagnosis
Addison’s disease.
Differential diagnosis
Adrenoleucodystrophy.

25. Addisons disease
Points in favor Points against
Hyperpigmentation No H/O vomiting, convulsion,
unconsciousness, repeated IV fluids
Weight loss, weakness, fatigue,
muscle cramps
Salt craving
Hypotension, postural drop

26. Adrenoleukodystrophy
Points in favor Points against
Signs of adrenal failure Hyperpigmentation No H/O of
developmental delay
Weight loss, weakness,
fatigue, muscle cramps
No abnormalities in
muscle tone, power
and reflexes.
Salt craving
Hypotension, postural
drop
Signs of neurolgic
involvement
Reads in KG at 8 years.

27. Investigations:

28. • Complete blood count:
– Hemoglobin: 10.2 g/dl,
– WBC: 12600/cumm,
– N: 50%, L: 36%
– Platelates: 5.3 lac/cumm,
– ESR: 78 mm in 1st hour.

29. • S. Cortisol:
– Basal at 8 am: 48.70 nmol/l; normal: 101-690 nmol/l
– At 5 pm: 29.5 nmol/l; normal: half of basal value.
• S. ACTH: 450 pg/ml normal: 8.3-57 pg/ml

30. • S. electrolytes:
– Na: 136 mmol/l,
– K: 4.9 mmol/l,
– Cl: 100 mmol/l,
– TCO2: 25 mmol/l
• Blood glucose: 4.4 mmol/l.

31. • CRP: 48 mg/l
• Mantoux test:
– Diameter of skin induration:
18 mm at 72 hours.

32. • Chest X ray:
– suggestive of
microcardia.

33. • Ultra-sonogram of abdomen:
– Size of the adrenals
are normal,
mild splenomegaly.
• X ray skull:
– Normal.

34. • S. ALT: 11 u/l,
• S. Creatinine: 0.39 mg/dl,
• ANA: negative ( 1.7 IU/ml),
• Urine R/M/E: normal.

35. Final diagnosis:
Addison’s disease due to Tuberculosis

36. Management

37. • Counseling,
• Supportive:
– Diet: normal,
– 5% dextrose with ½ normal saline: 1500 ml daily.
– Inj. Ceftriaxon: 1 gm 12 hourly,
– Tab. Paracetamol: ½ tab 8 hourly, if temp > 100˚F.

38. R: 12.6 mg/kg,
I: 11mg/kg,
E: 22 mg/kg
P: 33 mg/kg
• Specific management:
– Tab. Rimstar 4 FDC
• 1 ¾ tab daily before breakfast,
– Tab. Isoniazide 100 mg
• 1 tab daily before breakfast,
– Tab. Pyridoxine 25 mg
• 1 tab daily.

39. • Future Plan:
– Investigation:
• Gastric aspirate for AFB,
• S. Calcium, S. TSH.
– Management:
• To add: Tab. Hydrocortisone 10 mg/m²/day after fever
subsides.

40. • Follow up plan:
– Clinical:
• General well being,
• Temperature,
• Blood pressure.
• Body weight,
• Pigmentation.
– Lab:
• CRP, RBS, S. Electrolyte
• CBC, S. ALT.