Waterhouse-Friderichsen syndrome, septic adrenal apoplexy.

Waterhouse-Friderichsen syndrome is a rare but potentially fatal disorder of the adrenal gland characterized by bilateral adrenal hemorrhage. It is classically a result of meningococcal sepsis and presents acutely with features of shock, petechial rashes, abdominal pain, and non-specific symptoms such as headache, fatigue, and vomiting. Treatment consists of fluid resuscitation, corticosteroid replacement, and possibly surgery. The prognosis is poor despite treatment. This chapter will review the etiology, pathogenesis, clinical features, and management of the disease.

Fatal Waterhouse-Friderichsen syndrome in an adult due to serogroup Y Neisseria meningitidis.

Waterhouse-Friderichsen syndrome (WFS), defined as severe adrenal insufficiency due to bilateral adrenal gland haemorrhagic necrosis, occurred in a 59-year-old woman. An underlying serogroup Y Neisseria meningitidis (NM) infection was diagnosed, with a rapid progression to purpura fulminans, disseminated intravascular coagulation and WFS. Intensive treatment including fluid resuscitation, broad-spectrum antibiotic therapy, ventilatory support, platelet and factor replacement were administered. The meningococcaemia in the presence of WFS had a fulminant progression, leading to a fatal outcome within 24 hours of symptom onset. This case details the diagnosis and management challenges of the WFS, a rare complication of NM septicaemia, and describes the identification of a NM serogroup that is rare in Portugal in middle-aged patients.

[Management of severe sepsis using a Cytokin-adsorber]. / Management einer schweren Sepsis unter Einsatz eines Zytokin-Adsorbers.

Based on a representative case, which was treated in our center for severe burn injuries, the severity of the Waterhouse-Friderichsen syndrome and the complexity of the prolonged course of treatment are illustrated. Special attention is focused on the new treatment paradigm using the CytoSorb® adsorber.

[Foudroyant meningococcal sepsis in emergency medicine. Medical treatment and post-exposure prophylaxis]. / Foudroyante Meningokokkensepsis im Notarztdienst. Handlungsempfehlungen und Expositionsprophylaxe.

A paediatric emergency case with a rescue helicopter, which was reported as unexpected cardiac arrest of a 4-year-old child will be described. Based on this case, the symptoms and therapy of meningococcal sepsis and Waterhouse-Friderichsen-Syndrome under emergency conditions will be discussed as well as aspects of post-exposure prophylaxis.

Waterhouse-Friderichsen syndrome and bilateral renal cortical necrosis in meningococcal sepsis.

Waterhouse-Friderichsen syndrome and bilateral renal cortical necrosis (BRCN) are rare complications of meningococcal sepsis associated with high mortality rates. We describe a 20-year-old man who presented with a 1-day history of fever, chills, malaise, and vomiting. He collapsed in the emergency room, requiring mechanical ventilation and intravenous vasopressors for resuscitation. He was noted to be anuric, and computed tomography showed adrenal hemorrhage and BRCN. Blood cultures later confirmed Neisseria meningitidis sepsis, and a biopsy confirmed renal cortical infarction. The patient was treated aggressively with intravenous antibiotics, corticosteroids, and immunoglobulins, in addition to plasmapheresis, dialysis, and supportive measures. He recovered his adrenal function and was discharged from the hospital, but he remains dialysis dependent. To our knowledge, this is the first reported case of concomitant Waterhouse-Friderichsen syndrome and BRCN in a patient with meningococcal sepsis.

Rupert Waterhouse and Carl Friderichsen: adrenal apoplexy.

The Waterhouse-Friderichsen (WFS) syndrome, also known as purpura fulminans, is described as acute hemorrhagic necrosis of the adrenal glands and is most often caused by meningococcal infection. This clinical entity is more frequently seen in the pediatric than the adult population and is associated with a high morbidity and mortality. The initial presenting complaints for patients with the WFS usually include a diversity of nonspecific, vague symptoms such as cough, dizziness, headache, sore throat, chills, rigors, weakness, malaise, restlessness, apprehension, myalgias, arthralgias, and fever. These symptoms are usually abrupt in their onset. Petechiae are present in approximately 50-60% of patients. The clinical diagnosis of WFS may be relatively straightforward or extremely challenging. Patients who appear in the initial and nontoxic-appearing stage without any skin lesions may be difficult to distinguish from a benign viral illness. When a patient presents with fever and petechiae, WFS must be considered, even when the patient has a non-toxic appearance. Due to the rapid progression and often devastating consequences, therapy should be instituted as soon as the diagnosis is suspected.

Hemofiltración continua en pacientes pediátricos en estado crítico / Continuous hemofiltration in pediatric patients in critical state

La hemofiltración es utilizada con cada vez mayor frecuencia en pacientes pediátricos, en un variado número de situaciones clínicas. Se describe retrospectivamente una experiencia de 34 meses con este método aplicado a 17 pacientes, 9 varones edad promedio de 5 años 3 meses. 16 con falla orgánica múltiple, cinco con meningococcemia y riesgo (promedio) de mortalidad de 66 porciento. Se empleó hemofiltración arteriovenosa en 13 y venovenosa en 4 casos,asociada con diafiltración en 12 de los pacientes. Su indicación se debió siempre a edema y oliguria. En todos los casos se empleó heparina sistémica. No se registraron complicaciones de importancia. 9 pacientes (53 porciento) fallecieron debido a la afección de base, incluídos los 5 con meningococcemia y choque. La hemofiltración es un método práctico en pacientes pediátricos en situaciones críticas y puede contribuir a mejorar su manejo

Sonoclot coagulation analysis and plasma exchange in a case of meningococcal septicaemia.

On the basis of a patient with fulminant meningococcaemia and severe disseminated intravascular coagulation (DIC) syndrome, the diagnostic potential of a clot impedance test - Sonoclot coagulation analysis - was used to evaluate plasma exchange. A 17-yr-old girl was treated for a fulminant infection with Neisseria meningitidis in our intensive care unit. She developed severe DIC. Whereas platelet administration caused immediate arterial oxygen desaturation necessitating ventilatory support, plasma exchange improved pulmonary and mental function. Three separate exchanges all improved haemostasis. Sonoclot analysis was used together with routine coagulation analyses to evaluate this DIC treatment. Sonoclot signs, such as lack of the shoulder and peak, prolonged shoulder-peak interval and peak time predicted clinical bleeding manifestations (haematuria, haemoptysis, epistaxis) and were improved by platelet transfusion and plasma exchange. Plasma exchange was successful even at a very low platelet count of < 23 x 10(9).L-1. Sonoclot coagulation analyses were normalised several days before routine coagulation analyses. The Sonoclot gave additional information to routine coagulation studies, correctly indicated insufficient haemostasis and predicted a positive outcome. Also, plasma exchanges and platelet transfusions could be controlled in the management of DIC.

[Recommendations for the diagnosis and therapy of meningitis, Waterhouse-Friderichsen syndrome and encephalitis in childhood]. / Empfehlungen zur Diagnostik und Therapie der Meningitis, des Waterhouse-Friderichsen-Syndroms und der Enzephalitis im Kindesalter.

Representatives of the working group of "Neuropaediatrics" and "Infectology" like to present recommendations on diagnostics and treatment of infectious diseases on the CNS. Individual opinions can not be considered in recommendations always. Therefore we have to understand these recommendations in this way that individual decisions will not be restricted in special situations.

[Intravascular monitoring by axillary artery cannulation in infants]. / Intravasales Monitoring durch Arteria axillaris-Kanülierung im Säuglingsalter.

In patients with respiratory failure and shock serial arterial blood analyses are important to calculate adequate respiratory therapy. During circulatory insufficiency punction of a radial artery can be difficult or impossible, especially in very young patients. The case report of a 9-month old female infant with septic shock and Waterhouse-Friderichsen's syndrome illustrates these problems and is helpful to describe an alternative technique. Percutaneous axillary arterial catheterization with a disposable venous cannula proved to be a simple and quick method to obtain the possibility of arterial monitoring in very young patients with severe hypotension.