Waterhouse-Friderichsen Syndrome with Bilateral Adrenal Hemorrhage Associated with Methicillin-Resistant Staphylococcus aureus (MRSA) Bacteremia in an Adult Patient with History of Intravenous Drug Use.

BACKGROUND Waterhouse-Friderichsen syndrome, also known as acute adrenal insufficiency due to adrenal gland hemorrhage, is an uncommon and frequently fatal condition classically presenting with fever, shock, rash, and coagulopathy. Although most often associated with Meningococcemia, many other etiologies have been implicated, including reports of Staphylococcus aureus infection on autopsy examinations. This report details an adult intravenous drug user with adrenal hemorrhage associated with methicillin-resistant Staphylococcus aureus (MRSA) bacteremia. CASE REPORT A 58-year-old man with a history of intravenous drug use presented to the hospital with weakness. Vitals were initially normal and exam findings were notable for decreased right-sided motor strength. Magnetic resonance imaging (MRI) revealed a cervical epidural abscess with spinal cord compression. Despite initiation of broad-spectrum antibiotics and intravenous fluids, the patient progressed to shock, requiring vasopressor administration, and his blood cultures later grew MRSA. Further imaging of the abdomen/pelvis was completed, revealing bilateral adrenal hemorrhage. Random cortisol at that time was 5.6 µg/dL, confirming a diagnosis of critical illness-related corticosteroid insufficiency in addition to likely septic and spinal shock. The patient was initiated on hydrocortisone with improvement in his hypotension. He was transitioned to prednisone and fludrocortisone in addition to 8 weeks of antibiotics after achieving clinical stability. CONCLUSIONS This report brings to attention the risk of adrenal hemorrhage and acute adrenal insufficiency as a sequela of the relatively common illness of Staphylococcus aureus bacteremia. As symptoms of adrenal insufficiency can overlap with septic shock related to the primary condition, this diagnosis requires a high index of suspicion in the critically ill patient.

Waterhouse Friderichsen syndrome complicating fulminant Enterobacter cloacae sepsis in a preterm infant: the unresolved issue of corticosteroids.

Bilateral adrenal hemorrhage can complicate severe sepsis in the neonate and is most commonly attributed to meningococcal disease; however, it can be caused by other etiologic agents as well. We report herein a fatal case of Enterobacter cloacae sepsis in a preterm infant, resulting in massive adrenal hemorrhages. This is the first documented case of adrenal hemorrhage following infection with this pathogen.

[Recombinant human activated protein C in the treatment of children with meningococcal purpura fulminans]. / Proteína C activada humana recombinante en el tratamiento de niños con púrpura fulminante meningocócica.

Meningococcal purpura fulminans (MPF) produces high mortality and morbidity, despite appropriate standard therapy. Administration of recombinant human activated protein C (rhAPC) has been successfully applied in adults with MPF and pediatric studies are under way. We report three pediatric patients with MPF treated with rhAPC as compassionate therapy. In two of these patients, positive clinical and laboratory effects were observed and both children achieved full recovery. The remaining patient died after 36 hours from refractory multiorgan failure. No rhAPC-related adverse effects were detected. The reported cases highlight the usefulness of rhAPC in children with MPF at least as a rescue compassionate treatment. Further clinical trials are needed to better delineate its efficacy and administration schedule in children.

Human recombinant activated protein C in meningococcal sepsis.

A 19-year-old woman presented with purpura fulminans and septic shock; subsequently, progressive coagulopathy, widespread purpura fulminans associated with meningococcemia, severe shock, respiratory, and renal failure developed. This clinical course was associated with depletion of functional protein C levels to < 5%. We describe her clinical course and therapy with human recombinant activated protein C.

Púrpura fulminans asociada a sepsis meningocócica. Presentación de un caso en una paciente adulta / Meningococcal sepsis-associated purpura fulminans. Report of a case in an adult female

Púrpura fulminans es una rara entidad caracterizada por el desarrollo rápido de grandes áreas purpúricas con bordes geográficos, frecuentemente necróticas, asociada con hiperpirexia, hipotensión y coagulopatía de consumo. El examen histopatológico revela la presencia de trombos en los capilares y vénulas cutáneos, junto con hemorragia intersticial y diversos grados de necrosis cutánea.Presentamos un caso de púrpura fulminans asociada a sepsis meningocócica en una paciente adulta previamente sana. (AU)

[Meningococcal epidemic in a boarding school: a rifampicin-resistant secondary case while under chemoprophylaxis]. / Meningokokken-Epidemie in einer Internatsschule: Sekundärerkrankung mit rifampicin-resistentem Erreger unter Chemoprophylaxe.

An epidemic of meningococcal disease after an influenza outbreak in a community of 49 boys (14-18 years) and 8 adults in a boarding-school is reported. The first patient died with all symptoms of the Waterhouse-Friderichsen syndrome. Several hours later, two other boys developed severe septicemia with meningitis and meningitis respectively. N. meningitidis group B susceptible to penicillin and rifampin was isolated. Within the next 8 hours, chemoprophylaxis with rifampin (600 mg twice daily) was started and maintained for 4 days for the whole community. Throat cultures had not been obtained before prophylaxis. Ten other symptomatic boys were admitted to the hospital and treated by penicillin infusion. The results of blood and cerebrospinal fluid cultures were negative, and treatment was therefore discontinued. Five days after the death of the first boy, another boy died with full-blown Waterhouse-Friderichsen syndrome while on chemoprophylaxis. The neisseriae isolated from this patient were rifampin-resistant. Serological investigations in all patients admitted to hospital revealed the existence of concomitant epidemic infection with influenza A and B in this school. We assume that the viral infection made way for the outbreak of the meningococcal disease and for the high rate of secondary meningococcal infection. Chemoprophylaxis with rifampin should not be continued for longer than 2 to 3 days, otherwise the risk of occurrence of rifampin resistant strains of N. meningitidis increases. Hitherto such strains have rarely been isolated in clinically manifest disease.

[Clinical pathology of the Waterhouse-Friderichsen syndrome (author's transl)]. / Zur klinischen Pathologie des Waterhouse-Friderichsen-Syndroms

24 patients (among 35 clinical cases) with meningococcal Waterhouse-Friderichsen syndrome were submitted to pathologic anatomical examinations (including 18 microscopic investigations). Two aspects were found: 12 cases of mild suprarenal hemorrhage (histological evidence only), but associated with massive degeneration of the adrenocortical cells, leptomeningitis, distinct thymic hypertrophy with microthrombi in various organs. The other 12 cases presented massive bilateral suprarenal hemorrhage with medium status thymo-lymphaticus and distinct capillary thromboses in all organs investigated (consumptive coagulopathy).

[Septic shock with disseminated intravascular coagulation and adrenal failure (author's transl)]. / Septischer Schock mit Verbrauchskoagulopathie und Nebennierenausfall

The case is reported of a 15 year-old patient admitted in a state of severe shock with disseminated intravascular coagulation, complicated by unilateral adrenal failure as demonstrated scintigraphically by the absence of I131-labelled cholesterol uptake. Problems arising in the differential diagnosis and therapeutic management are discussed.