Download - 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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