medical grandrounds eduardo o. yambao jr., md. objectives to discuss a case of hemolytic uremic...
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MEDICAL GRANDROUNDS
Eduardo O. Yambao Jr., MD
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Objectives
• To discuss a case of hemolytic uremic syndrome (HUS) and bilateral renal cortical necrosis (BRCN) resulting from septic abortion.
• To discuss the diagnosis and treatment for HUS and BRCN
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J.J.
• 34 y/o female• Single• CC: Hypogastric Pain
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History of Present Illness
• 2 days PTA • had an induced abortion done
• Few hours after, mild hypogastric pain
• No fever• Took analgesics (aspirin,
naproxen, paracetamol) affording temporary relief
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History of Present Illness
• Few hours PTA • Severe hypogastric pain not relieved by pain meds
• Vaginal bleeding• No fever• No weakness• Consult at OB• Advised immediate
curettage and admitted
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Review of Systems
• General: no weight loss, no pallor, no fever• Chest: no dyspnea, no cough, no colds, no
hemoptysis• Heart: no chest pain, no palpitations• GU: no dysuria, no hematuria, no oliguria• Extremities: no edema
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Past Medical History
• Non hypertensive, non diabetic• History of bronchial asthma – last attack 1
year ago• No known allergies
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Obstetrical History• G2P1 (1-0-1-1)• LMP: October 25, 2008• PMP: September 2008
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Family History
• No hypertension• No diabetes• (+) bronchial asthma – both sides• No cancer
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Personal and Social History
• Non-smoker• Occasional alcoholic beverage drinker• No illicit drug use
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Physical Examination
• Alert, awake, conversant, in pain• BP100/70 HR90 RR19 afebrile• Anicteric sclera, pink palpebral conjunctivae• Thyroid gland not enlarged, no
lymphadenopathy, neck veins not distended• No tonsillopharyngeal congestion, no
lymphadenopathy• Equal chest expansion, no retractions, clear lungs
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Physical Examination• Adynamic precordium, AB 5th LICS MCL, no
murmurs• Abdomen flabby, soft, (+) direct tenderness on
hypogastric area, no guarding, no rebound tenderness, no hepatosplenomegaly
• No CVA tenderness• No edema• No acrocyanosis• Pulses full and equal
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Physical Examination
• Speculum examination : placental tissue plugging the os with minimal bleeding
• Internal examination : dilated cervix 1cm all the way.
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Assessment
• G2P1 (1011), Incomplete Abortion, Induced Abortion, t/c Septic Abortion
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Course in the Ward
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Day of Admission
• Kept on NPO• Underwent stat completion curettage• Cefazolin 1 gram IV single dose given• D5MR 1L x 8 hours with 10 units oxytocin
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Day of Admission
• Post currettage• Cefalexin 500 mg tab, 3x/day• Metronidazole 500 mg tab, 3x/day• Methylergometrine 125 microgram tab,
3x/day for 3 days
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Day of Admission
• BP 80-90 / 60• Hooked to voluven 500 ml, fast drip• Referred to infectious disease service • Impression : septic shock secondary to pelvic
inflammatory disease due to induced abortion• CBC, Blood culture • Discontinue cefalexin
•
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Blood culture:enterococcus faecalis (grp D), sensitive to ampicillin, penicillin
CBC
Hgb 12.9
Hct 37.9
Wbc 28.09
Mye 4
Meta 3
Stab 4
Seg 86
Lym 1
Plt 30k
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Day of Admission
• Start ampiclllin-sulbactam 1.5 gram IV every 12 hours
• Amikacin 750 mg IV every 24 hours • BP 70/50 placed on trendelenburg • Fast drip 200 ml PNSS and regulate to 100
ml/hr
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Course in the Ward• 0610H– Referred to hematology– PT/PTT – Peripheral blood smear – Fibrinogen level– Hematology: facilitate platelet transfusion 6 units
or 1 unit platelet apheresis and 6 units FFP– Impression : Suspect hemolytic crisis: t/c
Hemolytic Uremic Syndrome
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Peripheral blood smear : predominantly normocytic normochromic, spherocytes, no nucleated rbc’s, wbc adequate, thrombocytopenia Fibrinogen : 432.30 mg/dL
Feb 18
PTT
Patient 41.9
Control 27.4
PT
Patient 17.1
Act 53.2
Control 11.8
INR 1.46
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Course in the Ward• 1135H– No urine output for 5 hours: – Refer to nephrology : Ischemic Acute
Tubular Necrosis Secondary to Septic Shock
– Stat ultrasound of the whole abdomen : Bilateral renal parenchymal disease,
enlarged uterus with echogenic endometrium, minimal ascites
Na 139
K 4.2
Cl 103
Phos 3.98
Mg -
Calc 5.91
BUN 37
CREA 4.69
Uric A. 7.17
ALT 57
AST 519
CHON 3.7
Alb 1.7
Glob 2
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Course in the Ward
• 1315H– BP70/50 HR110 RR24 JVP12-14– Mottled skin, cold extremities– Post secalon line insertion left femoral– Discontinue voluven, start dopamine 400mg in
250ccD5W x 8ml/hr (10,cg/kg/hr); noradrenaline (levophed) 8mg in 100cc D5W x10 cc/hr
– Transfer to ICU
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Course in the Ward
• First Hosp Day 0820H – CVP 16cmH20 (+) fine rales both lower lung fields
anuric, temp39.1 BP 80/40 o2 sat 83%– Discontinue PNSS, portable CXR stat– Shift to MVM 50%– pulmo referral
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Course in the Ward
• CXR : pulmonary congestion , no effusion, no infiltrates
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Course in the Ward
• First Hosp Day 0915H– O2sat74%, patient is intubated – AC Fio2 100% Vt400 RR20 – Impression of pulmo service : Acute Respiratory
Failure probably secondary to fluid overload versus Acute Respiratory Distress Syndrome (ARDS)
– triple lumen catheter insertion , right– Stat dialysis
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Course in the Ward
• First Hosp Day- Ampicillin-sulbactam discontinued- started with Piperacillin-Tazobactam 2.25 grams IV every 8 hours- after dialysis : given Vancomycin 1 gram IV for 1 dose - CT scan of the abdomen
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Course in the Ward
CT scan of the Whole Abdomen – Prominent uterus, minimal fluid collection in the
cul de sac– bilateral renal cortical necrosis, absence of
contrast excretion may be due to severe hypovolemia or may be a sign of acute renal failure
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Course in the Ward
• Fourth Hosp Day- therapeutic plasma exchange - Piperacillin Tazobactam shifted to Meropenem 500 mg once a day and Levofloxacin 500 mg IV for 1 dose then 200 mg IV every 48 hours
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Course in the Ward
• Seventh Hosp Day- improvement in the platelet count and LDH levels (plt 413,000 and LDH 646 from 2,532)- still anuric - started on Hydrocortisone (Solucortef) 100 mg IV every 8 hours
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Course in the Ward • Eighth Hosp Day
- rpt CXR : clearing of pulmonary congestion, stable vital signs, good oxygen saturation - off inotropes ; extubated- NGT removed, started on soft diet
• Ninth Hosp Day - perm cath was inserted - started on Epoetin 5000 IU 4x/wk
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Course in the Ward
• Eleventh Hosp Day - transferred to a regular room
• Twelfth Hosp Day - IV steroid shifted to Prednisone 5 mg, 1 tablet 2x/day
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Course in the Ward
• Fifteenth Hosp Day - discharged - will undergo follow-up hemodialysis 3x/wk as an out patient
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Final Diagnosis
• Septic Abortion• Septic Shock • Hemolytic Uremic Syndrome (HUS) • Bilateral Renal Cortical Necrosis • s/p completion curettage, s/p perm cath
insertion, right IJ
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Septic Abortion
• Serious complications : 1. Severe hemorrhage2. Sepsis3. Acute renal failure
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Hemolytic Uremic Syndrome (HUS)
• Pentad : 1. hemolytic anemia2. thrombocytopenia3. neurological symptoms4. renal involvement5. fever
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Two Forms of HUS
• Diarrhea – associated HUS (D+HUS)• Non Shiga toxin – HUS (D-HUS)
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Pathogenesis
• Characteristic lesion in HUS is thrombotic microangiopathy
• Hallmark of thrombotic microangiopathies : widespread “hyaline” thrombi in terminal arterioles and capillaries
• Initiating mechanisms : endothelial injury and activation of intravascular thrombosis
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Hyaline thrombi in the lumen of glomerular capillary loops (arrows).
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Pathogenesis
• Typical pathologic lesion 1. platelet aggregation of arterioles and
capillaries out of proportion to fibrin deposition
2. endothelial damage3. lack of inflammatory infiltrate4. regional differences in microcirculatory
involvement
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Pathogenesis
• Acute cortical or tubular necrosis may occur. • Immunofluorescence studies invariably
demonstrate fibrinogen along the glomerular capillary walls and in arterial thrombi.
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Pathogenesis
• During pregnancy the kidney seems to be particularly susceptible to damage by mechanisms involving intravascular coagulation.
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Pathogenesis
• Evidence of renal involvement is present in the majority of patients with HUS
• Microscopic hematuria (78%) are the most consistent findings
• More than 90% of patients with HUS have significant renal failure, one third of whom are anuric
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Acute Renal Failure in Pregnancy
• Acute renal failure (ARF) in pregnancy bears a high risk of bilateral renal cortical necrosis (BRCN) and consequently of chronic renal failure
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Renal Cortical Necrosis (RCN)
• Rare cause of acute renal failure in developed countries
• Still occurs in developing countries due to poor health facilities
• Occurs in 2 peaks : 1. early infancy – severe perinatal events2. women of childbearing age
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Pathogenesis
• Causes of RCN can be divided into two groups: Obstetric and non-obstetric
• Obstetric complications : 1. abruptio placentae 2. septic abortion 3. eclamptic toxaemia 4. post-partum haemorrhage 5. intrauterine fetal demise
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Renal Cortical Necrosis (RCN)
• Due to poor health facilities, RCN is still a cause of morbidity and mortality in developing countries. The damage is permanent and functional loss is irreversible.
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Renal Cortical Necrosis (RCN)
• Septic abortion continued to be an important cause of RCN and endotoxin-mediated endothelial damage leads to vascular thrombosis and subsequent renal ischemia in patients with septic abortion
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Clinical Manifestations • most common :
1. CNS changes2. purpura or bleeding from other sites3. malaise4. abdominal pain5. fever
• CNS symptoms : mild confusion and headache to frank paresis, aphasia, paresthesias, visual problems, seizures, and coma
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Clinical Manifestations
• Additional clinical findings: 1. petechiae 2. icterus secondary to intravascular hemolysis 3. indirect hyperbilirubinemia4. signs of involvement of other organs.
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Diagnostic Tests
• The microangiopathic hemolytic anemia of HUS is distinguished from that of DIC by the absence of gross deviations from normal in the prothrombin and partial thromboplastin times.
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Diagnostics Tests
• Definite diagnosis for BRCN : renal biopsy • CT scan : representative and specific imaging
procedure of kidneys for BRCN • A very unstable hemodynamic status with
coagulopathy in the early period of hospital was not suitable for the procedure of renal biopsy.
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Treatment
• Plasmapheresis may remove the recently identified inhibitory antibodies against vWF protease from the circulation and supply larger amounts of the protease enzyme
• Plasma exchange should be performed daily until remission is achieved, remission being normalization of platelet count, or resolution of neurologic symptoms, or both.
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Treatment
• Supportive measures : 1. dialysis2. antihypertensive medications3. blood transfusions4. management of neurologic complications
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Treatment
• plasma exchange (plasma pheresis combined with fresh frozen plasma replacement) is currently the treatment of choice and is superior to plasma infusion alone
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Treatment
• Plasma exchange (PE) has been shown in several case series to produce response rates of approximately 80% and survival rates greater than 90%.
• The most important determinant of long term survival is the presence or absence of a serious underlying medical condition.
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Treatment
• Severe renal insuffiency resulting from HUS often requires dialysis.
• Renal transplantation has also been performed
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