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![P A T H O P H Y S I O L O G Y - R H A B D O M Y O L Y S I S
2] ATP depletion
Dysfunction of Na/K ATPase pump
Integrity of myocyte lost
Release of intracellular muscle contents
a) creatine kinase
b) Myoglobin
c) Electrolytes](https://image.slidesharecdn.com/hyper-ck-emia-241115181956-206e0b9e/75/Hyper-CK-emia-case-presentation-medicine-26-2048.jpg)
![PATHOPHYSIOLOGY - RHABDOMYOLYSIS
1] Muscle injury
increase in intracellular free ionised calcium
Activation of proteases
mitochondrial dysfunction
free oxygen radicle
skeletal muscle death](https://image.slidesharecdn.com/hyper-ck-emia-241115181956-206e0b9e/75/Hyper-CK-emia-case-presentation-medicine-27-2048.jpg)
![Rheumatic Fever CASE PRESENTATION [Autosaved].pptx](https://cdn.slidesharecdn.com/ss_thumbnails/casepresentationautosaved-251123182512-9d9b0da4-thumbnail.jpg?width=640&height=640&fit=bounds)
Hyper-CK-emia case presentation medicine
- 1.
- 2. CHIEF COMPLAINTS 30 yearold male No known co morbidities Working in a bakery at Mysore Came to ER with C/o B/L upperlimb and thigh pain for the past 10 days
- 3. HOPI Patient was apparentlynormal before 10 days after which he developed generalized body pain more intensity in B/L upper limb pain followed by B/L thighs 3 days later. Pain was insidious in onset , gradually progressive extending from the shoulder to forearm , not associated with swelling, no aggravating and reliving factors
- 4. HISTORY OF PRESENTINGILLNESS No h/o weakness No h/o trauma No h/o recent strenuous exercise No h/o alcohol / substance abuse No h/o prolonged immobilization No h/o heat exposure No h/o any medications intake No h/o Fever
- 5. No h/o electrocution Noh/o burns injuiry No h/o seizures No h/o difficulty in walking/climbing stairs No h/o fasciculations/ involuntary movements No H/o Abd pain/Nausea/Vomitting/ loose stools No h/o Burning micturition/ decreased micturition/ dark coloured urine No h/o weight loss
- 6. PAST HISTORY N/K/C/O DM/SHTN/BA/PTB/CAD/CKD/CVA/THYROID DISORDER/SEIZUREDISORDER NO History of any drug intake PERSONAL HISTORY Working as a chef at a bakery in Mysore Mixed Diet Not an alcoholic Not a smoker Normal bowel and bladder habits
- 7. GENERAL EXAMINATION O/E patientconscious oriented afebrile NO pallor/icterus/clubbing/cyanosis/lymphadenopathy/pedal edema No gottron papules/helitrope rash/shawl sign/V sign VITALS HR 98/min, regular rhytm, normal volume, no specific character,all peripheral pulses present,no radiofemoral delay BP 130/80 mmHg in Right upper limb, supine posture Spo2 - 98% Temp – 98.5 F CBG – 151 mg/dl
- 8. LOCAL EXAMINATION B/L LOWERLIMB and THIGH – No ulcers No swelling No scars/ sinuses No warmth / tenderness
- 9. SYSTEMIC EXAMINATION CVS –S1S2 +, No murmur RS – BAE + No Added Sounds P/A – soft, No tenderness, BS+ no organomegaly
- 10. CNS- POWER 5/5 all4 limbs Tone – normal No involuntary movements B/L plantar flexor
- 11. DTR: Biceps ++ ++ Triceps++ ++ Supinator ++ ++ Knee ++ ++ Ankle ++ ++
- 12. INITIAL INVESTIGATIONS CBC 7600/16.1 / 2.12 lakh RFT Urea 45/ creat 1.5 Na 134 / k 4.1 / cl 101 TB 0.54/ DB 0.11/ IBD 0.43 SGOT 420/ SGPT 191/ ALP 74 TP 6.0/ALB 3.5/ GLB 2.7
- 13. RBS 168 HBA1C 5.5 Calcium8.9 / phosphorus 4.0/ uric acid 4.5 URINE ROUTINE Color straw yellow Appearance clear Glucose protein – NIL RBC 2-3
- 14. PROCAL 0.79 BLOOD C/S– No growth Urine C/S – No Growth Viral Markers - Negative Scrub – Negative Dengue – Negative Lepto - Negative
- 15. TSH/FT3/FT4 NORMAL serum cortisol– 426 nmol/L CK total 17190 Urine myoglobin 1.8 mg/L
- 16. PERIPHERAL SMEAR RBC –NCNC RBC WBC – TC/DC Normal PLATELET- Adequate No hemoparasites / NO atypical/ immature cells ANA 1: 100 WEAK POSITIVE / REFLEX NEGATIVE
- 17. USG ABDOMEN Fatty liver– 14 cm NO IHBRD NO splenomegaly NO Ascites Kidney normal size/ CMD maintained 2D ECHO Normal LV size NO RWMA IVC collapsed EF 62%
- 18. FINAL DIAGNOSIS RHABDOMYOLYSIS -? Cause Probably secondary to viral myositis
- 19. NEPHROLOGY opinion wasobtained in view of Rhabdomyolysis Patient was started on forced alkaline diuresis Patient was treated with maintainance NS and ½ NS with bicarbonate alternatively Electrolytes and RFT monitored daily NEUROLOGY opinion was obtained to do Electromyography and Muscle biopsy if warrented EMG was deferred from Neuro side, Patient was treated as Viral myositis with INJ METHYLPED 1 gram was given for 3 days, followed up with oral steroids RHEUMATOLOGY opinion was obtained advised to continue oral steroids and hydration RFT CK total was monitored gradual decrease in CK total -2098 at discharge after 10 days of admission
- 20.
- 21. NON NEUROMUSCULAR CAUSES– ELEVATED CK Endocrine disorders Hyperthyroidism (rare) Hypothyroidism Hyperparathyroidism Acromegaly Cushing syndrome Metabolic disturbances Hyponatremia Hypokalemia Hypophosphatemia Muscle trauma
- 22. Medications Statins Fibrates Antiretrovirals Beta-blockers Clozapine Angiotensin II receptorblockers Hydroxychloroquine Isotretinoin Colchicine
- 23. Others Celiac disease Malignancy Surgery Pregnancy Cardiac disease Acutekidney disease Viral illness Predisposition to malignant hyperthermia
- 24. NEUROMUSCULAR CAUSES Muscle dystrophies Duchenneand Becker muscular dystrophies Dystrophin mutations in female carriers Limb girdle Myotonic dystrophy Metabolic and mitochondrial disorders of muscle Carnitine palmitoyltransferase II deficiency McArdle disease Myoadenylate deaminase deficiency Mitochondrial myopathies Pompe disease (acid maltase deficiency)
- 25. Inflammatory myopathies Dermatomyositis Inclusion bodymyositis Polymyositis Others Familial elevated creatine kinase Sarcoid myopathy Charcot-Marie-Tooth disease Other congenital diseases
- 26. P A TH O P H Y S I O L O G Y - R H A B D O M Y O L Y S I S 2] ATP depletion Dysfunction of Na/K ATPase pump Integrity of myocyte lost Release of intracellular muscle contents a) creatine kinase b) Myoglobin c) Electrolytes
- 27. PATHOPHYSIOLOGY - RHABDOMYOLYSIS 1]Muscle injury increase in intracellular free ionised calcium Activation of proteases mitochondrial dysfunction free oxygen radicle skeletal muscle death
- 28. RISK FACTORS 1) Male 2)Age less than 10/ more than 60 years 3) BMI> 40 4) Dehydration 5) Chronic lipid lowering drugs
- 29. NON TRAUMATIC EXERTIONALCAUSES A) prolonged strenuous unaccustomed activity B) Exertional heat stroke C) Hypokalemia – postassium loss in sweat leads to decreased vascular perfusion of muscle D) Sickle cell trait(Excessive exercise)
- 30. E) Hyperkinetic states: a) Seizures b) Delirium tremens c) psychotic agitation d) amphetamine overdose F) Inherited disorders: a) Glycogen storage disorder- McArdles syndrome b) Lipid – fatty acid oxidation disorders c) mitochondrial disorders d) Muscular dystrophy.
- 31. SIGNS AND SYMPTOMS Myalgias muscleweakness myoglobinuria. full triad is observed in only 1 to 10 percent of cases.
- 32. ●Pain –Muscle pain,- proximal muscle groups, such as the thighs and shoulders, and in the lower back and calves stiffness and cramping. ●Weakness –Weakness usually occurs in the same muscle groups affected by pain or swelling proximal legs most frequently involved.
- 33. Swelling –When itoccurs, detectable swelling in the extremities generally develops with fluid repletion. •Myoedema, which is nonpitting and is apparent at presentation or develops after rehydration •Peripheral edema, which is pitting and occurs with rehydration (particularly in patients with AKI) Urine changes — Dark coloured 10% of cases. Myoglobin appears in urine – plasma concentration > 1.5mg/dl Visible changes – 100-300mg/dl
- 34. Fluid and electrolyteabnormalities — ●Hypovolemia results from "third-spacing" due to the influx of extracellular fluid into injured muscles and increases the risk of AKI . ●Hyperkalemia and hyperphosphatemia Hyperkalemia is more common in patients with oliguric AKI . ●Severe hyperuricemia . ●Metabolic acidosis is common, and an increased anion gap may be present. Initial hyocalcemia followed by hypercalcemia
- 35. Acute kidney injury—15 – 50% The risk of AKI is lower in patients with CK levels at admission less than 15 to 20,000 units/L HEME pigment – tubular obstruction Direct Proximal tubular epithelial injury vasoconstriction
- 36. Compartment syndrome after fluidresuscitation, with worsening edema of the limb and muscle may occur after traumatic bone fracture or prolonged limb compression. Disseminated intravascular coagulation — due to the release of thromboplastin and other prothrombotic substances from the damaged muscle.
- 37. Other organ involvement ●Liverinjury –25% elevated transaminases ●Neurologic – Altered mental status (eg, confusion, agitation) may result from the underlying etiology (eg, toxins, drugs, trauma, or electrolyte abnormalities) of rhabdomyolysis . ●Pulmonary – Respiratory failure or acute respiratory distress syndrome may accompany rhabdomyolysis due to the underlying etiology (infection, illicit drugs, metabolic myopathy)
- 38. LABS •CBC – infection •RFT– AKI •ELECTROLYTES – hyperkalemia/hypocalcemia/hyperphosphatemia •LFT – Transaminitis •Coagulation profile/ D dimer/ Fibrinogen – DIC •ABG – Metabolic acidosis •ECG – cardiac arrhythmia •C/S •Toxicology screening
- 39. CK – > 5000units / L – non exertional > 10000 units/L – exertional Rise within 2-12 hours of muscle injury- maximum within 24 -72 hrs Half life 1.5 days URINE MYOGLOBIN Not sensitive/ not detected in >65% Half life 2-3 hrs Appears before CK elevation
- 40. First rule outnon neuromuscular causes NO available treatment Weigh the extensive/ expensive/ invasive evaluation against the limited treatment options available RECOMMENDATIONS FOR MUSCLE BIOPSY IN ASYMPTOMATIC ELEVATED CK - abnormal findings on EMG - CK > 3times the upper limit of normal -AGE <25 - Exercise intolerance
- 41. Combined testing –possibility of diagnosis 28% Findings non specific in 30-45% Findings normal 30-40% - Idiopathic elevated CK