1 Departments of Nephrology and 2 Pathology, Rabin Medical Center (Campus Golda), Petach Tikva, Israel, Affiliated with the Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel
Introduction
A 72-year-old woman was admitted to hospital with a 12-h history of fever, pain over the left ear and a non-bloody diarrhoea. Twenty-four hours previously a haemodialysis session had ended uneventfully.
Case
Twelve years earlier the patient had undergone a vagotomy and pyloroplasty. The operation was complicated by splenic bleeding, which necessitated splenectomy. Other relevant past history included end-stage renal failure secondary to bilateral atherosclerotic renal-artery stenosis, ischaemic heart disease highlighted by recurrent episodes of acute pulmonary oedema, hypertension, and cholelithiasis. Chronic haemodialysis had been started 6 months previously. Daily medications included frusemide, nitrates, enalapril, losartan, and low-dose atenolol.
On admission the patient was alert but confused. Vital signs were temperature 38.5°C; blood pressure 90/60 mmHg; and pulse rate 80/min. The PTFE graft in her upper arm did not appear inflamed. Neck stiffness, pulmonary consolidation, or abdominal tenderness were not determined. An ENT consultant diagnosed a left, non-exudative, otitis media. Initial blood examination revealed a leukocytosis (WBC 12 000/mm3). Chest X-ray was normal. Blood cultures were taken and antibiotic therapy with vancomycin, ceftriaxone, and gentamicin, was begun.
Over the next 12 h the patient's condition deteriorated markedly. Both patchy and confluent purpuric rashes developed over wide areas of the patients body. Hypotension proved unresponsive to crystalloid and colloid fluid resuscitation, and high-dose dobutamine and noradrenaline infusions were commenced. Disseminated intravascular coagulation (DIC) became obvious, with thrombocytopenia (platelet count: 32 000/mm3), prolonged PT/PTT times, and the appearance of elevated fibrin split products. Acute respiratory failure necessitated mechanical ventilation.
The patient lapsed into a coma, and died less than a day after admission. Final pre-mortem laboratory results included a serum albumin level of 19 g/l, and a serum LDH level of over 2500 U/l. An autopsy was performed.
What were the two pathophysiological reasons behind this patients death?
Comments
Blood cultures grew Streptococcus pneumoniae, so therefore septic shock, complicated by DIC, was the primary reason for this patients multiple organ failure. However, on post-mortem the second reason for this patient's demise became all too clear. Macroscopic haemorrhage into both adrenal glands was unequivacable. Histopathology revealed haemorrhagic necrosis of both adrenal glands.
The WaterhouseFriderichsen syndromeacute adrenal insufficiency secondary to gross haemorrhage into both adrenal glandsis classically associated with Meningococcal bacteraemia and DIC [1]. But other bacteria are known to cause this devastating medical problem, especially Haemophilus influenzae and Streptococcus pneumoniae [27]. Importantly, a previous splenectomy in any bacteraemic patient is regarded as a possible precipitating factor towards the development of the WaterhouseFriderichsen syndrome [3,711].
Possible clues to the existence of the Waterhouse Friderichsen syndrome include resistant shock in any feverish patient and the dermatological picture known as purpura fulminans. In this setting, high-dose and continuous hydrocortisone therapy may be life-saving.
Although serum cortisol levels and sonographic examination of the adrenal glands may aid in reaching a definite diagnosis of the WaterhouseFriderichsen syndrome, such tests are time-consuming and therefore they should not cause any delay in commencing empiric steroid therapy when the suspicion of the WaterhouseFriderichsen syndrome is high [1,12].
Finally, this patient received a pneumococcal vaccine a short time after her splenectomy, and despite this went on to develop overwhelming Pneumococcal bacteraemia. It must therefore be reiterated that the recent Center for Disease Control (Atlanta, USA) recommendations for polysaccharide Pneumococcal vaccine include (i) a single dose only for any adult over the age of 65 years, and (ii) a single dose for all patients with chronic renal failure (irrespective of age), strengthened by a single revaccination 5 years after the initial vaccination [13].
Teaching point
Notes
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Correspondence and offprint requests to: Professor U. Gafter, Department of Nephrology, Rabin Medical Center (Campus Golda), Petach Tikva, Israel.
References