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PROF DR K H NOOR UL AMEEN  M5 UNIT DR.RAKESH PINNINTI  CASE OF JAUNDICE
Chief complaints  <ul><li>30yr old male Mr.Vishwanathan was admitted with </li></ul><ul><li>Chief complaints of </li></ul>...
Presentation  <ul><li>Patient was apparently doing well 4 days back when he noticed & was also alerted by his family …abou...
Presentation  <ul><li>No H/O fever, diarrhea, abdominal pain, </li></ul><ul><li>No H/O chest pain, palpitations, dyspnea, ...
History <ul><li>Past History  </li></ul><ul><li>No H/O similar complaints in the past </li></ul><ul><li>No H/O prior hospi...
Examination <ul><li>Patient a adult male aged 30yrs moderately built & nourished. </li></ul><ul><li>Conscious, mild disori...
GPE <ul><li>Pallor ++ </li></ul><ul><li>Icterus ++ </li></ul><ul><li>Puffiness of face </li></ul><ul><li>BPPE </li></ul><u...
Systemic examination <ul><li>Cardiovascular  :  S1 S2 heard , flow murmurs + </li></ul><ul><li>Respiratory  :  NVBS heard ...
CNS <ul><li>HMF :  Conscious, mild disoriented, disinterested in surrounding, looks distressed, afebrile, responding to or...
List of problems <ul><li>Alcohol abuse  </li></ul><ul><li>Jaundice, anemia, edema </li></ul><ul><li>Oliguria </li></ul><ul...
Investigations  <ul><li>CBC </li></ul><ul><li>Hb  8.0 gm%  </li></ul><ul><li>TC  8200 mm3 </li></ul><ul><li>DC  P65 L32 E3...
Investigations  <ul><li>MP QBC : NEGATIVE </li></ul><ul><li>MSAT :  1+ </li></ul><ul><li>PT  T 12 sec  C  11 sec </li></ul...
RFT PROGRESSION Hemo-Dialysis started on 16/5/2011 14/5 15/5 17/5 20/5  21/5 22/5 24/5 26/5 Date 104 96 148 180 99 89 179 ...
GH HEMATOLOGY FOR RESCUE <ul><li>Hb 7.4gm% </li></ul><ul><li>Tc  8,400 </li></ul><ul><li>DC P62 L22 B4 M6 normoblasts 6 </...
KIDNEY BIOPSY (Apollo)
ONLINE REFERENCE Fibrin thrombi & RBC  in renal capillaries Subintimal fibrin, but no inflammation Healing from prior fibr...
 
FINAL DIAGNOSIS ATYPICAL HEMOLYTIC UREAMIC SYNDROME  / D-HUS
TTP vs HUS
D+ vs. D-
Oklahoma TTP-HUS Registry <ul><li>Idiopathic — 37 %  </li></ul><ul><li>Drug-associated — 13 %  </li></ul><ul><li>Autoimmun...
 
Disseminated intravascular coagulation is not typically present, but may be seen when there is diffuse tissue ischemia.
Treatment
Prognosis  Index contains three adverse prognostic factors (age >40, hemoglobin <9 g/dL, temperature >38.5ºC).  Subjects w...
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A Case of Atypical Hemolytic Uremic Syndrome

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A Case of Atypical Hemolytic Uremic Syndrome

  1. 1. PROF DR K H NOOR UL AMEEN M5 UNIT DR.RAKESH PINNINTI CASE OF JAUNDICE
  2. 2. Chief complaints <ul><li>30yr old male Mr.Vishwanathan was admitted with </li></ul><ul><li>Chief complaints of </li></ul><ul><li>1) jaundice </li></ul><ul><li>2) vomiting </li></ul><ul><li>3) fatigue </li></ul><ul><li>4) Oliguria </li></ul><ul><li>Patient had above complaints for 4 days prior to admission </li></ul><ul><li>Patient was having altered sensorium since 6hr prior to admission </li></ul>
  3. 3. Presentation <ul><li>Patient was apparently doing well 4 days back when he noticed & was also alerted by his family …about yellowish discoloration of his eyes </li></ul><ul><li>It was sudden onset, persistent, progressive severity. </li></ul><ul><li>He noticed similar discoloration on his tongue, palms & soles the next day. </li></ul><ul><li>He felt fatigued, unable to work, rested for the most of the day. </li></ul><ul><li>Patient felt nauseated for most part of the day, with reduced appetite, vomiting 3 episodes/day since day of onset of jaundice </li></ul>
  4. 4. Presentation <ul><li>No H/O fever, diarrhea, abdominal pain, </li></ul><ul><li>No H/O chest pain, palpitations, dyspnea, cough </li></ul><ul><li>No H/O headache, seizures, LOC, syncope/pre-syncope </li></ul><ul><li>No H/O recent blood transfusions, contact with jaundiced person, </li></ul><ul><li>No H/O malena, hemetemesis, easy bruising/ bleeding from any orifice. </li></ul><ul><li>No H/O myalgia, arthralgia, skin rashes, mouth ulcers, weight loss </li></ul><ul><li>H/O reduced urine output since the day of onset of jaundice. </li></ul><ul><li>H/O passing high colored urine. </li></ul>
  5. 5. History <ul><li>Past History </li></ul><ul><li>No H/O similar complaints in the past </li></ul><ul><li>No H/O prior hospitalization, surgeries, drug intake </li></ul><ul><li>Not a K/C/O DMT2, SHT, TB, malignancy </li></ul><ul><li>Personal History </li></ul><ul><li>Smoker since 10yr </li></ul><ul><li>Alcoholic since 8yr H/O alcohol binge in recent weeks, last consumption 5 days prior to admission. </li></ul><ul><li>Family History </li></ul><ul><li>No similar complaints in the family </li></ul>
  6. 6. Examination <ul><li>Patient a adult male aged 30yrs moderately built & nourished. </li></ul><ul><li>Conscious, mild disoriented, disinterested in surrounding, looks distressed, afebrile, responding to oral commands & obeys commands, moves all 4 limbs spontaneously. </li></ul><ul><li>Vitals Pulse rate 102/min regular. </li></ul><ul><li>BP 180/110 </li></ul><ul><li>temp afebrile </li></ul><ul><li>RR 24/min </li></ul>
  7. 7. GPE <ul><li>Pallor ++ </li></ul><ul><li>Icterus ++ </li></ul><ul><li>Puffiness of face </li></ul><ul><li>BPPE </li></ul><ul><li>No clubbing </li></ul><ul><li>No cyanosis </li></ul><ul><li>No lymphadenopathy </li></ul><ul><li>No petechiae, ecchymoses, bleeding from orifices. </li></ul><ul><li>No elevated JVP </li></ul><ul><li>No features of chronic liver disease/ liver failure. </li></ul>
  8. 8. Systemic examination <ul><li>Cardiovascular : S1 S2 heard , flow murmurs + </li></ul><ul><li>Respiratory : NVBS heard in all lung fields, </li></ul><ul><li>inspiratory crepts in B/L lung bases </li></ul><ul><li>Per abdomen: soft, no tenderness, liver palpable 2 cm below costal margin, no spleenomegaly, no free fluid, BS normal </li></ul>
  9. 9. CNS <ul><li>HMF : Conscious, mild disoriented, disinterested in surrounding, looks distressed, afebrile, responding to oral commands & obeys commands, moves all 4 limbs spontaneously. </li></ul><ul><li>CRANIAL NERVES : Normal </li></ul><ul><li>SENSORY & MOTOR : Normal </li></ul><ul><li>Cerebellar signs : NIL </li></ul>
  10. 10. List of problems <ul><li>Alcohol abuse </li></ul><ul><li>Jaundice, anemia, edema </li></ul><ul><li>Oliguria </li></ul><ul><li>Hypertension </li></ul><ul><li>Inspiratory crepts </li></ul><ul><li>Hepatomegaly </li></ul><ul><li>Altered sensorium </li></ul>
  11. 11. Investigations <ul><li>CBC </li></ul><ul><li>Hb 8.0 gm% </li></ul><ul><li>TC 8200 mm3 </li></ul><ul><li>DC P65 L32 E3 </li></ul><ul><li>ESR 8/16 </li></ul><ul><li>Plt 95,000 mm3 </li></ul><ul><li>RFT </li></ul><ul><li>Urea 10.4 gm% </li></ul><ul><li>Creat 4.8 gm% </li></ul><ul><li>URINE ROUTINE </li></ul><ul><li>ALBUMIN + </li></ul><ul><li>RBC NIL </li></ul><ul><li>DEPOSITS 2-4 EP/mm3 </li></ul><ul><li>LFT </li></ul><ul><li>TB 6.5 </li></ul><ul><li>IB 5.1 </li></ul><ul><li>SGOT 54.2 </li></ul><ul><li>SGPT 23.5 </li></ul><ul><li>ALP 65.0 </li></ul><ul><li>TOTAL PROTEIN 5.5 </li></ul><ul><li>ALBUMIN 3.6 </li></ul><ul><li>RETIC -3.5% </li></ul><ul><li>LDH -1736 U/L </li></ul><ul><li>DAT -VE </li></ul>PERIPHERAL SMEAR : MACROCYTIC ANEMIA URINE FOR HB : POSITIVE 24HR URINARY PROTIEN : 100 mg%
  12. 12. Investigations <ul><li>MP QBC : NEGATIVE </li></ul><ul><li>MSAT : 1+ </li></ul><ul><li>PT T 12 sec C 11 sec </li></ul><ul><li>APTT T 25.2 sec C 27 sec </li></ul><ul><li>INR 1.1 </li></ul><ul><li>BT/CT N </li></ul><ul><li>URIC ACID 9.9 gm% </li></ul><ul><li>CA2+ 10.5 </li></ul><ul><li>PHOS 9.6 </li></ul><ul><li>ANA RF ASO APLA NEGATIVE </li></ul><ul><li>CRP POSITIVE </li></ul><ul><li>FDP MILDY ELEVATED (1.4 U) </li></ul><ul><li>PROTIEN C & S N/A </li></ul><ul><li>HIV, HBsg, HCV, HAV NEGATIVE </li></ul><ul><li>STOOL C/S NEGATIVE </li></ul><ul><li>BLOOD C/S NO GROWTH </li></ul><ul><li>URINE C/S NO GROWTH </li></ul>USG ABDOMEN : FATTY LIVER XRAY CHEST : NORMAL ECG : SINUS TACHY`
  13. 13. RFT PROGRESSION Hemo-Dialysis started on 16/5/2011 14/5 15/5 17/5 20/5 21/5 22/5 24/5 26/5 Date 104 96 148 180 99 89 179 71 Urea 4.8 4.9 5.2 13.0 8.8 6.7 8.2 7.2 Creat
  14. 14. GH HEMATOLOGY FOR RESCUE <ul><li>Hb 7.4gm% </li></ul><ul><li>Tc 8,400 </li></ul><ul><li>DC P62 L22 B4 M6 normoblasts 6 </li></ul><ul><li>ESR 48/100 </li></ul><ul><li>Plt 1.1 </li></ul><ul><li>P.smear : microcytic hypochromic RBC </li></ul><ul><li>normoblasts </li></ul><ul><li>macrocytes </li></ul><ul><li>polychromasia </li></ul><ul><li>fragmented RBCs ++ </li></ul><ul><li>IMP : TTP/HUS sug. BMA </li></ul>
  15. 15. KIDNEY BIOPSY (Apollo)
  16. 16. ONLINE REFERENCE Fibrin thrombi & RBC in renal capillaries Subintimal fibrin, but no inflammation Healing from prior fibrinoid injury, occluding vessel
  17. 18. FINAL DIAGNOSIS ATYPICAL HEMOLYTIC UREAMIC SYNDROME / D-HUS
  18. 19. TTP vs HUS
  19. 20. D+ vs. D-
  20. 21. Oklahoma TTP-HUS Registry <ul><li>Idiopathic — 37 % </li></ul><ul><li>Drug-associated — 13 % </li></ul><ul><li>Autoimmune disease — 13 % </li></ul><ul><li>Infection — 9 % </li></ul><ul><li>Pregnancy/postpartum — 7 % </li></ul><ul><li>Bloody diarrhea prodrome — 6 % </li></ul><ul><li>Hematopoietic cell transplantation — 4 % </li></ul><ul><li>Suspected TTP-HUS </li></ul><ul><li>Idiopathic TTP-HUS </li></ul><ul><li>Severe ADAMTS13 deficiency </li></ul>
  21. 23. Disseminated intravascular coagulation is not typically present, but may be seen when there is diffuse tissue ischemia.
  22. 24. Treatment
  23. 25. Prognosis Index contains three adverse prognostic factors (age >40, hemoglobin <9 g/dL, temperature >38.5ºC). Subjects with zero, one, two, or all three of these adverse factors had 6-month mortality rates of 12, 14, 31, and 62 percent, respectively. Atypical hemolytic–uremic syndrome has a poor prognosis, with death rates as high as 25% and progression to end-stage renal disease in half the patients
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