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Addisons disease

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Addisons disease

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Addisons disease

  1. 1. Welcome to clinical meeting Dr. Eshita Reza Khan Resident phase A Pediatric hematology and oncology
  2. 2. • Name: Saif • Age: 8 years, • Sex: male, • Address: Pilkhana, • Date of admission:13. 12. 15.
  3. 3. • Chief complaints: – Progressive darkening of skin – 3 years, – Recurrent fever – 3 years, – Generalized weakness and weight loss – 3 years.
  4. 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. 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. 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. 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. 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. 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. 10. • General examination: – Appearance: • Ill looking, – Pallor: absent, – Jaundice: absent, – Cyanosis: absent,
  11. 11. – Clubbing: absent, – Koilonychia: absent, – Leuconychia: absent. – Oedema: absent, – Dehydration: absent.
  12. 12. • Skin survey: – Hyperpigmentation of face, lips, gingiva, bony prominences. – BCG mark present. • Lymph nodes: not palpable, • Bony tenderness: absent, • Thyroid: not enlarged.
  13. 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. 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. 15. • Locomotor system: – Look: generalized muscle wasting, – Feel: generalized muscle tenderness, – Move: knee extension is restricted due to pain.
  16. 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. 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. 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. 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. 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. 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. 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. 23. Provisional diagnosis ?
  24. 24. Provisional diagnosis Addison’s disease. Differential diagnosis Adrenoleucodystrophy.
  25. 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. 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. 27. Investigations:
  28. 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. 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. 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. 31. • CRP: 48 mg/l • Mantoux test: – Diameter of skin induration: 18 mm at 72 hours.
  32. 32. • Chest X ray: – suggestive of microcardia.
  33. 33. • Ultra-sonogram of abdomen: – Size of the adrenals are normal, mild splenomegaly. • X ray skull: – Normal.
  34. 34. • S. ALT: 11 u/l, • S. Creatinine: 0.39 mg/dl, • ANA: negative ( 1.7 IU/ml), • Urine R/M/E: normal.
  35. 35. Final diagnosis: Addison’s disease due to Tuberculosis
  36. 36. Management
  37. 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. 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. 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. 40. • Follow up plan: – Clinical: • General well being, • Temperature, • Blood pressure. • Body weight, • Pigmentation. – Lab: • CRP, RBS, S. Electrolyte • CBC, S. ALT.

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