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An unusual ca(u)se of tetany Prof. S.SUNDAR’s unit, Dr. N.ARUN KUMAR, PG
Case presentation ,[object Object],[object Object],[object Object]
h/o presenting illness ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Past history ,[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object]
General examination ,[object Object],[object Object],[object Object]
Systemic examination ,[object Object],[object Object],[object Object],[object Object]
Provisional diagnosis ,[object Object]
Investigations  CBC Values Hb TC DC ESR RBC HCT Platelet  MCV MCH MCHC 11.8 gm% 11000 cells/cumm P51, L38, E6, M4, B1 4/10 4.4 million 34.9% 4.84 lakh 79.3 fl 26.8 pg 33.8 gm/dl
Electrolytes  ELECTROLYTES  VALUES Serum calcium-  Serum magnesium Serum Na  Serum K- Serum chloride Serum HCO3  Serum PTH Serum albumin 5.7 mg%  (N – 8.7-10.2mg%) 1.2 mg%  (N – 1.5-2.3mg%) 134 meq/L 2.92 meq/L  84 meq/L 34 meq/L 9.5 pg/ml  (N – 8-51 pg/ml) 3.8gm/dl
ABG ,[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
DIAGNOSIS ,[object Object]
Gitelman’s syndrome ,[object Object]
Pathogenesis of Gitelman’s syndrome  Inactivating mutations in the gene downregulation Hypocalciuria  Due to associated  Plasma volume contraction
Clinical features Gitelman’s syndrome is mostly a disorder of adults
Laboratory evaluation
Treatment  ,[object Object],[object Object],[object Object]
Causes of hypocalcemia Low PTH levels  High PTH levels Parathyroid agenesis isolated DiGeorge syndrome Vit. D def or impaired 1,25(OH)2 production/action nutritional vit.D def renal insufficiency with impaired  1,25(OH)2 production vit. D resistance Parathyroid destruction surgical radiation infiltration by mets auto immune PTH resistance syndromes PTH receptor mutations pseudo hypoparathyroidism Reduced parathyroid function hypomagnesemia activating CaSR mutations Drugs calcium chelators inhibitors of bone resorption (bisphosphonates, plicamycin) altered vit. D metabolism (phenytoin, ketoconazole)  Miscellaneous causes acute pancreatitis acute rhabdomyolysis hyngry bone syndrome  osteoblastic mets (Ca prostate)
Causes of hypocalcemia Associated with normal / low plasma PO4 Associated with high plasma PO4 Associated with hypoalbuminemia Vit. D def decreased intake decreased absorption  (post-gastrectomy, PBC, intestinal malabsorption) Idiopathic or sporadic hypoparathyroidism Hemodilution Decreased 25(OH) vit. D  generation (liver  disease, anti- convulsants) Post operative hypo-parathyroidism Nephrotic syndrome Decreased calcitriol  renal failure type 1 vit. D dep rickets Acquired hypo- parathyroidism post irradiation amyloidosis Exudative enteropathy Resistance to calcitriol type 2 vit. D dep rickets Pseudo hypo- parathyroidism type 1 type 2 cirrhosis Acute pancreatitis CKD advanced stage Mg deficiency AKI oligoanuric nstage
Clinical features ,[object Object],RAPIDITY SEVERITY
[object Object],[object Object],[object Object],[object Object],[object Object]
Trousseau’s sign: carpal spasm induced by inflation of BP cuff to >20 mmHg of systolic BP for 3 min
Chvostek’s sign: tapping of facial nerve branches    twitching of facial muscles)
Acute hypocalcemia paraesthesias of the lips & extremities muscle cramps tetany laryngeal stridor  convulsions
Chronic hypocalcemia cataracts brittle nails with transverse grooves dry skin decreased or even absent axillary & pubic hair
Diagnostic approach to hypocalcemia ,[object Object],[object Object]
Diagnostic approach to hypocalcemia
 
 
Treatment of hypocalcemia ,[object Object],RAPIDITY SEVERITY ACCOMPANYING COMLICATIONS
Treatment of  acute symptomatic hypocalcemia ,[object Object],[object Object],[object Object]
Treatment of  acute symptomatic hypocalcemia (contd…) ,[object Object],[object Object],[object Object]
Treatment of  chronic hypocalcemia: Treatment in PTH deficiency ,[object Object],[object Object],[object Object],[object Object]
Treatment of  chronic hypocalcemia (contd…) ,[object Object],[object Object],[object Object]
Goal of treatment To bring serum calcium to low normal range To avoid hypercalciuria To avoid nephrolithiasis
 

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An Unusual case of Tetany

  • 1. An unusual ca(u)se of tetany Prof. S.SUNDAR’s unit, Dr. N.ARUN KUMAR, PG
  • 2.
  • 3.
  • 4.
  • 5.
  • 6.
  • 7.
  • 8.
  • 9.
  • 10. Investigations CBC Values Hb TC DC ESR RBC HCT Platelet MCV MCH MCHC 11.8 gm% 11000 cells/cumm P51, L38, E6, M4, B1 4/10 4.4 million 34.9% 4.84 lakh 79.3 fl 26.8 pg 33.8 gm/dl
  • 11. Electrolytes ELECTROLYTES VALUES Serum calcium- Serum magnesium Serum Na Serum K- Serum chloride Serum HCO3 Serum PTH Serum albumin 5.7 mg% (N – 8.7-10.2mg%) 1.2 mg% (N – 1.5-2.3mg%) 134 meq/L 2.92 meq/L 84 meq/L 34 meq/L 9.5 pg/ml (N – 8-51 pg/ml) 3.8gm/dl
  • 12.
  • 13.
  • 14.
  • 15.
  • 16. Pathogenesis of Gitelman’s syndrome Inactivating mutations in the gene downregulation Hypocalciuria Due to associated Plasma volume contraction
  • 17. Clinical features Gitelman’s syndrome is mostly a disorder of adults
  • 19.
  • 20. Causes of hypocalcemia Low PTH levels High PTH levels Parathyroid agenesis isolated DiGeorge syndrome Vit. D def or impaired 1,25(OH)2 production/action nutritional vit.D def renal insufficiency with impaired 1,25(OH)2 production vit. D resistance Parathyroid destruction surgical radiation infiltration by mets auto immune PTH resistance syndromes PTH receptor mutations pseudo hypoparathyroidism Reduced parathyroid function hypomagnesemia activating CaSR mutations Drugs calcium chelators inhibitors of bone resorption (bisphosphonates, plicamycin) altered vit. D metabolism (phenytoin, ketoconazole) Miscellaneous causes acute pancreatitis acute rhabdomyolysis hyngry bone syndrome osteoblastic mets (Ca prostate)
  • 21. Causes of hypocalcemia Associated with normal / low plasma PO4 Associated with high plasma PO4 Associated with hypoalbuminemia Vit. D def decreased intake decreased absorption (post-gastrectomy, PBC, intestinal malabsorption) Idiopathic or sporadic hypoparathyroidism Hemodilution Decreased 25(OH) vit. D generation (liver disease, anti- convulsants) Post operative hypo-parathyroidism Nephrotic syndrome Decreased calcitriol renal failure type 1 vit. D dep rickets Acquired hypo- parathyroidism post irradiation amyloidosis Exudative enteropathy Resistance to calcitriol type 2 vit. D dep rickets Pseudo hypo- parathyroidism type 1 type 2 cirrhosis Acute pancreatitis CKD advanced stage Mg deficiency AKI oligoanuric nstage
  • 22.
  • 23.
  • 24. Trousseau’s sign: carpal spasm induced by inflation of BP cuff to >20 mmHg of systolic BP for 3 min
  • 25. Chvostek’s sign: tapping of facial nerve branches  twitching of facial muscles)
  • 26. Acute hypocalcemia paraesthesias of the lips & extremities muscle cramps tetany laryngeal stridor convulsions
  • 27. Chronic hypocalcemia cataracts brittle nails with transverse grooves dry skin decreased or even absent axillary & pubic hair
  • 28.
  • 29. Diagnostic approach to hypocalcemia
  • 30.  
  • 31.  
  • 32.
  • 33.
  • 34.
  • 35.
  • 36.
  • 37. Goal of treatment To bring serum calcium to low normal range To avoid hypercalciuria To avoid nephrolithiasis
  • 38.