a case of atypical hemolytic uremic syndrome
TRANSCRIPT
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PROF DR K H NOOR UL AMEEN M5 UNIT
DR.RAKESH PINNINTI
CASE OF JAUNDICE
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Chief complaints
30yr old male Mr.Vishwanathan was admitted withChief complaints of 1) jaundice 2) vomiting 3) fatigue 4) Oliguria Patient had above complaints for 4 days prior to admission
Patient was having altered sensorium since 6hr prior to admission
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Presentation
Patient was apparently doing well 4 days back when he noticed & was also alerted by his family …about yellowish discoloration of his eyes
It was sudden onset, persistent, progressive severity.
He noticed similar discoloration on his tongue, palms & soles the next day.
He felt fatigued, unable to work, rested for the most of the day.
Patient felt nauseated for most part of the day, with reduced appetite, vomiting 3 episodes/day since day of onset of jaundice
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Presentation
No H/O fever, diarrhea, abdominal pain,No H/O chest pain, palpitations, dyspnea, coughNo H/O headache, seizures, LOC, syncope/pre-
syncopeNo H/O recent blood transfusions, contact with
jaundiced person, No H/O malena, hemetemesis, easy bruising/
bleeding from any orifice.No H/O myalgia, arthralgia, skin rashes, mouth
ulcers, weight loss H/O reduced urine output since the day of onset
of jaundice.H/O passing high colored urine.
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History
Past History No H/O similar complaints in the pastNo H/O prior hospitalization, surgeries, drug
intakeNot a K/C/O DMT2, SHT, TB, malignancyPersonal HistorySmoker since 10yr Alcoholic since 8yr H/O alcohol binge in recent
weeks, last consumption 5 days prior to admission.Family HistoryNo similar complaints in the family
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Examination
Patient a adult male aged 30yrs moderately built & nourished.
Conscious, mild disoriented, disinterested in surrounding, looks distressed, afebrile, responding to oral commands & obeys commands, moves all 4 limbs spontaneously.
Vitals Pulse rate 102/min regular. BP 180/110 temp afebrile RR 24/min
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GPE
Pallor ++Icterus ++Puffiness of faceBPPENo clubbing No cyanosisNo lymphadenopathyNo petechiae, ecchymoses, bleeding from
orifices.No elevated JVPNo features of chronic liver disease/ liver failure.
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Systemic examination
Cardiovascular : S1 S2 heard , flow murmurs +
Respiratory : NVBS heard in all lung fields,
inspiratory crepts in B/L lung bases
Per abdomen: soft, no tenderness, liver palpable 2 cm below costal margin, no spleenomegaly, no free fluid, BS normal
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CNS
HMF : Conscious, mild disoriented, disinterested in surrounding, looks distressed, afebrile, responding to oral commands & obeys commands, moves all 4 limbs spontaneously.
CRANIAL NERVES : Normal
SENSORY & MOTOR : Normal
Cerebellar signs : NIL
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List of problems
Alcohol abuse Jaundice, anemia, edemaOliguriaHypertensionInspiratory creptsHepatomegalyAltered sensorium
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Investigations
CBCHb 8.0 gm% TC 8200 mm3DC P65 L32 E3ESR 8/16Plt 95,000 mm3• RFTUrea 10.4 gm%Creat 4.8 gm% URINE ROUTINEALBUMIN +RBC NILDEPOSITS 2-4 EP/mm3
LFT TB 6.5 IB 5.1 SGOT 54.2 SGPT 23.5 ALP 65.0 TOTAL PROTEIN 5.5 ALBUMIN 3.6• RETIC -3.5%• LDH -1736 U/L• DAT -VE
PERIPHERAL SMEAR : MACROCYTIC ANEMIA URINE FOR HB : POSITIVE24HR URINARY PROTIEN : 100 mg%
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Investigations
MP QBC : NEGATIVE MSAT : 1+ PT T 12 sec C 11 sec APTT T 25.2 sec C 27 sec INR 1.1 BT/CT N URIC ACID 9.9 gm% CA2+ 10.5 PHOS 9.6
ANA RF ASO APLA NEGATIVE CRP POSITIVE FDP MILDY ELEVATED (1.4 U) PROTIEN C & S N/A HIV, HBsg, HCV, HAV NEGATIVE STOOL C/S NEGATIVE BLOOD C/S NO GROWTH URINE C/S NO GROWTH
USG ABDOMEN : FATTY LIVERXRAY CHEST : NORMALECG : SINUS TACHY`
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RFT PROGRESSION
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
Hemo-Dialysis started on 16/5/2011
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GH HEMATOLOGY FOR RESCUE
Hb 7.4gm%Tc 8,400DC P62 L22 B4 M6 normoblasts 6ESR 48/100Plt 1.1P.smear : microcytic hypochromic RBC normoblasts macrocytes polychromasia fragmented RBCs ++
IMP : TTP/HUS sug. BMA
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KIDNEY BIOPSY (Apollo)
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ONLINE REFERENCE
Fibrin thrombi & RBC in renal capillaries
Subintimal fibrin, but no inflammation
Healing from prior fibrinoid injury, occluding vessel
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FINAL DIAGNOSIS
ATYPICAL HEMOLYTIC UREAMIC SYNDROME / D-HUS
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TTP vs HUS
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D+ vs. D-
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Oklahoma TTP-HUS Registry
Idiopathic — 37 % Drug-associated — 13 % Autoimmune disease — 13 % Infection — 9 %Pregnancy/postpartum — 7 %Bloody diarrhea prodrome — 6 % Hematopoietic cell transplantation — 4 %
Suspected TTP-HUS
Idiopathic TTP-HUS
Severe ADAMTS13 deficiency
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Disseminated intravascular coagulation is not typically present, but may be seen when there is diffuse tissue ischemia.
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Treatment
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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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