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CPC 17 th April 2008

CPC 17 th April 2008. Female, 48 years old 2 years prior to admission: Proximal muscle weakness left lower extremity 1.5 years prior to admission: Weakness, on wheel chair Muscle atrophy 1 year prior to admission : Upper extremities weakness

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CPC 17 th April 2008

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  1. CPC 17th April 2008

  2. Female, 48 years old 2 years prior to admission: Proximal muscle weakness left lower extremity 1.5 years prior to admission: Weakness, on wheel chair Muscle atrophy 1 year prior to admission : Upper extremities weakness 1 week prior to admission : Quadriparesis

  3. MRI: At 1.5 years prior to admission (lower extremities weakness with muscle atrophy) Cervical spondylosis, cervical canal stenosis C5-T1 Herniated disc C 3-7 Physical therapy/ Orthropaedic surgery: C3-4 with instrument

  4. Asymptomatic Degenerative Disk Disease and Spondylosis of the Cervical Spine: MR Imaging’ Radiology 1987; 164:83-88

  5. Abnormal magnetic-resonance scans of the cervical spine in asymptomatic subjects. A prospective investigation <40 Yrs. Old (N = 167) >40Yrs. Old (N= 97) Major Minor Major Minor Abnormality Abnormality Abnormality Abnormality Herniated disc 5 (3%) 7 (4%) 1 (1%) 4 (4%) Bulging disc 0 8 (5%)1 (1%) 5 (5%) Foraminal stenosis 5 (3%) 7 (4%) 9 (9%) 14 (14%) Disc-space narrowing 3 (2%) 18 (1 1%) 15 (16%) 21 (22%) Degenerated disc 13 (8%) NA 36 (37%) NA Spurs (spondylosis) 5 (3%) 23 (14%) 6 (6%) 33 (34%) Abnormal cords 15 (9%) 15 (9%) 1 (1%) 17 (18%) J Bone Joint Surg Am. 1990;72:1178-1184.

  6. Muscle weakness/motor predominate No cranial nerves involvement No paresthesia No bowel/urinary bladder/autonomic dysfunction Asymmetry to symmetry by clinical course Chronic progressive course Muscle atrophy

  7. Corticospinal tract Anterior horn cell: Amyotrophic lateral sclerosis, Radiculoneuropathy: Demyelination disease Mononeuritis multiplex Neuromuscular junction: Myasthenia gravis Lambert-Eaton myasthenic syndrome Myopathy: Polymyositis, Metabolic disorder

  8. Cervical spondylosis • Compressed fracture C6 • HNP C 3-7, • Kyphoscoliosis • Osteopenia

  9. Metabolic Bone Disease Bone turnover: Primary hyperparathyroidism Secondary hyperparathyroidism Adynamic bone disease Low Bone content: Osteoporosis High bone content: Osteopetrosis Bisphosphonate Abnormal mineralization: Osteomalacia/rickets

  10. Causes of Osteomalacia • Abnormal matrix • Abnormal vitamin D metabolism Gastrointestinal disease Chronic liver disease Chronic kidney disease Hypoparathyroidism • Mineralization inhibitor Aluninum toxicity Fluoride Chronic metabolic acidosis • Hypophosphatasia (AR) • Hypophosphatemia

  11. Labs: • BUN 9 mg %, Cr 0.4 mg %, • Calculated creatinine clearance 151 mL/min • Measured creatinine clearance 68.2 mL/min • Na+ 142 mEq/L, K+ 3.7 mEq/L, Cl- 105 mEq/L, HCO3- 27 mEq/L • Ca2+ 10.4 mg%, PO42- 1.3 mg% • iPTH 72 pg/mL

  12. Daily requirement = 800 mg • Foods • Meat, poultry & fish • Dairy products • Processed foods • Soda

  13. Etiology of Hypophosphatemia • Internal redistribution • Re-feeding • Acute respiratory alkalosis • Hungry bone syndrome • Decreased intestinal absorption • Inadequate intake (< 100 mg/day) • Chronic diarrhea, malabsorption • Vitamin D deficiency or resistance • Aluminum or magnesium ingestion • Increased urinary excretion • Primary hyperparathyroidism • Secondary hyperparathyroidism • Proximal tubule dysfunction • Hereditary hypophosphatemic rickets • Onchogenic osteomalacia

  14. Filtered Phosphate Excreted Phosphate

  15. Renal phosphatewasting • 24 hours urine phosphate = 787.4 mg/day ( < 100 mg/day) • Fractional PO42+ excretion = U PO42+ xPcr P PO42+ xUcr = 26.73% (5 -10 %) • Renal phosphateclearance = U PO42+ x V P PO42+ x 1440 = 27.34 mL/min (5 -15 % mL/min)

  16. Renal threshold phosphate conc (Tm PO42+/GFR) Normal2.0 – 3.5 mg% = 0.9 mg% Lancet1975;309-10

  17. . • Glucosuria • Hypophosphatemia • Hypouricemia • Hypokalemia • Aminoaciduira • Autosomal dominant, recessive, or X-linked • Wilson’s disease, • Galactosemia, tyrosinemia, cystinosis • Multiple myeloma, amyloid, • Heavy metal toxicity, chemotherapeutic drugs (ifosfamide), imatinib mesylate

  18. Hypophosphatemia with renal phosphate wasting • Proximal RTA • Hyperparathyroidism • Mutation of type 2a sodium-phosphate co-transport: Absorptive hypercalciuria type III, nephrolithiasis Hereditary hypophosphatemic rickets with hypercalciuria • Hereditary hypophosphatemic ricket • Tertiary hyperparathyroidism post-kidney transplantation • Onchogenic osteomalacia: High phosphatonin: FGF 23, matrix extracellular phosphaglycoprotein (MEPE), frizzled growth factor 4 (FRP-4)

  19. iPTH stimulate FGF 23

  20. Log FGF-23 Kidney Int 2003, 64 : 2272–2279

  21. CKD, Renal bone disease, Aging

  22. New Engl J Med 2003;348:1656

  23. Mesenchymal tumor cause TIO • Fibrous dysplasia • Hemangiopericytoma • Osteosarcoma • Chrondroblastoma • Chondromyxoid fibroma • Malignant fibrous histiocytoma • Giant cell tumor • hemangioma Mayo clinic 1994

  24. Progressive muscle weakness • Severe metabolic bone disease/Osteomalacia • Hypophosphatemia/Severe renal phosphate wasting • Groin mass • Onchogenic osteomalacia/immobilization osteoporosis • Investigation: • Groin mass excision/immunohistochem staining • Serum FGF-23 concentration • (Octreotide labeled indium-111 scan)

  25. Mesenchymal tumor mass Muscle weakness Acquired high phosphatonin (FGF-23) Severe renal phosphate wasting Severe osteomalacia, immobilization osteopenia

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