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MEDICAL GRANDROUNDS . Eduardo O. Yambao Jr., MD . 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. J.J. . 34 y/o female Single
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MEDICAL GRANDROUNDS Eduardo O. Yambao Jr., MD
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
J.J. • 34 y/o female • Single • CC: Hypogastric Pain
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
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
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
Past Medical History • Non hypertensive, non diabetic • History of bronchial asthma – last attack 1 year ago • No known allergies
Obstetrical History • G2P1 (1-0-1-1) • LMP: October 25, 2008 • PMP: September 2008
Family History • No hypertension • No diabetes • (+) bronchial asthma – both sides • No cancer
Personal and Social History • Non-smoker • Occasional alcoholic beverage drinker • No illicit drug use
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
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
Physical Examination • Speculum examination : placental tissue plugging the os with minimal bleeding • Internal examination : dilated cervix 1cm all the way.
Assessment • G2P1 (1011), Incomplete Abortion, Induced Abortion, t/c Septic Abortion
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
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
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
Blood culture:enterococcus faecalis (grp D), sensitive to ampicillin, penicillin
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
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
Peripheral blood smear : predominantlynormocyticnormochromic, spherocytes, no nucleated rbc’s, wbc adequate, thrombocytopenia Fibrinogen : 432.30 mg/dL
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
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
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
Course in the Ward • CXR : pulmonary congestion , no effusion, no infiltrates
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
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
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
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
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
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
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
Course in the Ward • Fifteenth Hosp Day - discharged - will undergo follow-up hemodialysis 3x/wk as an out patient
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
Septic Abortion • Serious complications : 1. Severe hemorrhage 2. Sepsis 3. Acute renal failure
Hemolytic Uremic Syndrome (HUS) • Pentad : 1. hemolytic anemia 2. thrombocytopenia 3. neurological symptoms 4. renal involvement 5. fever
Two Forms of HUS • Diarrhea – associated HUS (D+HUS) • Non Shiga toxin – HUS (D-HUS)
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
Hyaline thrombi in the lumen of glomerular capillary loops (arrows).
Pathogenesis • Typical pathologic lesion 1. platelet aggregation of arterioles and capillaries out of proportion to fibrin deposition 2. endothelial damage 3. lack of inflammatory infiltrate 4. regional differences in microcirculatory involvement
Pathogenesis • Acute cortical or tubular necrosis may occur. • Immunofluorescence studies invariably demonstrate fibrinogen along the glomerular capillary walls and in arterial thrombi.
Pathogenesis • During pregnancy the kidney seems to be particularly susceptible to damage by mechanisms involving intravascular coagulation.
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
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
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 events 2. women of childbearing age