A Case of Atypical Hemolytic Uremic Syndrome

PROF DR K H NOOR UL AMEEN M5 UNIT

DR.RAKESH PINNINTI

CASE OF JAUNDICE

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

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

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.

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

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

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.

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

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

List of problems

Alcohol abuse Jaundice, anemia, edemaOliguriaHypertensionInspiratory creptsHepatomegalyAltered sensorium

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%

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`

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

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

KIDNEY BIOPSY (Apollo)

ONLINE REFERENCE

Fibrin thrombi & RBC in renal capillaries

Subintimal fibrin, but no inflammation

Healing from prior fibrinoid injury, occluding vessel

FINAL DIAGNOSIS

ATYPICAL HEMOLYTIC UREAMIC SYNDROME / D-HUS

TTP vs HUS

D+ vs. D-

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

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 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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