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Blackwater fever

Blackwater fever. Dr Josh Davis Staff Specialist Immunology & Infectious Diseases John Hunter Hospital. Background. 54 yo married school teacher Felty’s Syndrome Diagnosed 1994. Splenomegaly. RF positive RA currently quiescent Chronic neutropaenia 2ry to Felty’s No G-CSF or antibiotics

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Blackwater fever

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  1. Blackwater fever Dr Josh Davis Staff Specialist Immunology & Infectious Diseases John Hunter Hospital

  2. Background • 54 yo married school teacher • Felty’s Syndrome • Diagnosed 1994. Splenomegaly. RF positive • RA currently quiescent • Chronic neutropaenia 2ry to Felty’s • No G-CSF or antibiotics • Nphils 0.3-0.5 since at least 1998 • GIH in 8/04. Settled spontaneously. Awaiting upper and lower endoscopies • No Regular Medications

  3. Presenting Illness • Well until 4 hours prior to presentation • At 18:00, crampy abdominal pain, loose stools (no blood), chills and rigors. • Went to MMH ED • O/E. T=40.1 degrees. Frank haematuria. Hypotensive 80 systolic. Nil else localising

  4. Initial Management • CXR clear, FBC 103/2.9/244, Bilirubin 153, other LFTs normal • ? Pyelonephritis in setting of febrile neutropaenia • Fluid resuscitation, Cefepime, Gentamicin

  5. Next 8 Hours • Haemoglobin plummeted • Developed respiratory failure with bibasal crepitations and bilateral new pulmonary infiltrates • ? Autoimmune haemolytic anaemia • ?Infection

  6. Progress • Transferred to JHH ICU • Rapidly developed ARDS, septic shock, DIC • Intubated, required noradrenaline infusion • Transfused for massive haemolysis • Blood cultures flagged positive

  7. Day 1 • Presumptive diagnosis: Clostridial bacteraemia with consequent massive haemolysis (Grew C.perfringens) • CT Abdomen revealed small liver collection • CT-guided pigtail drain placed in collection • Nearly exsanguinated into abdomen from liver puncture. Impossible to ventilate

  8. Day 1 • Transferred to OT for laparotomy “moribund” not expected to survive • On Nad 0.40mcg/kg/min, Vasopressin, Pressure Control Ventilation, 80% oxygen. BP 60-70 sys • APTT>100, PT 46, Fibrinogen 0.2 • 5 litres blood drained from abdomen. Liver packed

  9. Blood products first 4 days • Packed Cells • 46 units • FFP • 34 units • Cryoprecipitate • 52 units • Pooled platelets • 15 bags

  10. Progress • Survived against all odds • Developed ARF requiring CVVH then haemodialysis for 1 month • Discharged from ICU day 18, from hospital 46 • Received 6 weeks IV antibiotics (penicillin changed to metronidazole because of cholestasis) for liver abscess • Still awaiting scopes. Has dysphagia . . . . .

  11. Clostridial Bacteraemia 31/3/05 Josh Davis

  12. Clostridia - Microbiology • Anaerobic, spore-forming, gram positive rods. • 90 species exist – the most important in humans are: • C tetani – Tetanus • C botulinum – Gas gangrene • C difficile – Pseudomembranous colitis • C perfringens – see below • C septicum – bactraemia with colonic Ca.

  13. Clostridium perfringens - Microbiology • Ubiquitous in soil and faeces • Found in every soil sample ever tested except for the sands of the sahara • Found in the faeces of every vertebrate species tested • Originally called C.welchii • Secretes >12 exotoxins, most of which are lethal (to mice)

  14. Clostridium perfringens - Microbiology • Alpha - Toxin • AKA Lecithinase or Phospholipase C • Directly destroys RBC membranes, causing haemolysis • Enterotoxin • Food poisoning • Others • Haemolysins, DNAses, collagenase, protease, hyaluronidase • Spread through tissue and cause necrosis (gas gangrene)

  15. C.perfringens – Clinical Syndromes • Soft tissue infections • Simple wound infection (polymicrobial) • Crepitant cellulitis (does not invade healthy muscle) • Clostridial myonecrosis (gas gangrene) • Intraabdominal infections (see irrelevant aside) • Emphysematous cholecystitis • Enteritis necroticans • Typhlitis • Pelvic infections post-TOP • Primary bacteraemia • Food poisoning • Mild, no treatment required

  16. C. Perfringens bacteraemiaCase series – Rechner et al.1 • Rural US hospital all positive blood cultures 1990-1997 • Clostridia were 74 of 63,000 (0.12%) • C.perfringens most common, followed by C.septicum • 48% had underlying malignancy • Mortality was 58% • 52% were found to have a GI source • Only one patient had massive intravascular haemolysis 1- Rechner et al. CID 2001; 33: 349-53

  17. Massive haemolysis associated with C.perfringens bacteraemia • Literature review 1999, Alvarez et al.1 • 19 cases in entire world literature • 11/13 rapidly fatal (85% mortality) • Mean time from diagnosis to death=8 hours 1 –Alvarez et al. Massive hemolysis in Clostridium perfringens infectionHaematologica. 1999 Jun;84(6):571-3.

  18. C.perfringens bacteremia - summary • 1) Rare – (approx 0.1% of bacteraemias) • 2) Usually associated with underlying malignancy or debility • 3) Usual primary source is colonic lesion • 4) High mortality 40-60% • 5) Treatment=Penicillin, debridement • 6) Massive haemolysis very rare and almost always fatal within hours.

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