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Ethylmalonic encephalopathy
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
Manifestations of vascular abnormality (Figures 102.2, 102.3, 102.5–102.9, 102.11, 102.12) are typical in this disease [5–8], and these features are quite unique among metabolic disorders. Acrocyanosis (Figures 102.5) may be the mildest manifestation and it may be associated with edema of the extremities. Patients also have episodic showers of petechiae, often associated with infection. One of our patients (Figure 102.2) was originally investigated for meningococcemia before referral to us. There may also be ecchymoses (Figures 102.7 and 102.9) or hemorrhagic streaks (Figures 102.8). Ethylmalonic encephalopathy may masquerade as a hematologic disorder and our index patient (Figure 102.9) suffered from progressive pancytopenia (Figure 102.10), in addition to progressive psychomotor retardation. She presented at birth with severe thrombocytopenia unresponsive to cortisone or immunoglobulins [5]. From the second year of life, the patient needed an increasing number of transfusions of platelets and red blood cells. She gradually developed hypersensitivity against HLA identical thrombocytes and finally died in a cardiovascular arrest secondary to severe anemia. In another patient, leukocytosis and thrombocytosis were prominent [10].
Diagnostic Approach to Rash and Fever in the Critical Care Unit
Published in Cheston B. Cunha, Burke A. Cunha, Infectious Diseases and Antimicrobial Stewardship in Critical Care Medicine, 2020
Lee S. Engel, Charles V. Sanders, Fred A. Lopez
Meningococcemia can occur sporadically or in epidemics and is more commonly diagnosed during the winter months. Neisseria meningitidis is primarily spread by respiratory droplets that require close, prolonged contact for transmission [15]. The risk of infection is highest in infants, asplenic patients, alcoholics, patients with complement deficiency, and persons who live in dormitories (coeds, military personnel, or prisoners). Initial symptoms include cough, headache, sore throat, nausea, and vomiting. Acute meningococcemia progresses rapidly, and patients typically appear ill, with high spiking fevers, tachypnea, tachycardia, mild hypotension, and a characteristic petechial rash [16,17]. Signs and symptoms of meningeal irritation such as headache, vomiting, and change in consciousness occur in up to 88% of patients with meningococcemia [16,18].
Unexplained Fever In Infectious Diseases: Section 2: Commonly Encountered Aerobic, Facultative Anaerobic, And Strict Anaerobic Bacteria, Spirochetes, And Parasites
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
Meningococcal disease can induce problems of unexplained fever in the following circumstances: chronic meningococcemia (without meningitis). This clinical form is manifested initially by a prodromal episode of upper respiratory illness, or a viral exanthem. A few days or weeks after so-called recovery, the patient develops fever, weakness, headache, petechial rash, splenomegaly, arthralgia or arthritis and, in severe cases, hypotension and prostration. The pattern of fever is intermittent and the infection may last for weeks or months, with afebrile periods lasting up to 10 days. Without adequate diagnosis and treatment, severe complications may appear, e.g., carditis, nephritis and, especially, meningitis. This clinical picture must be distinguished from that of recurrent meningococcal meningitis.40
Predictors of meningococcal vaccine uptake in university and college students: a systematic review and meta-analysis
Published in Journal of American College Health, 2022
Joanna Whisnant, Jacqueline Martin-Kerry, Lydia Flett, Peter Knapp
Diagnosing IMD quickly and accurately is important as its onset can be insidious, but it is often mistaken for more common, less serious illnesses.28 Its most common clinical presentations include bacteremia (30% of cases), bacteremic pneumonia (15% of cases), and meningitis (50% of cases).28,29 A subset of bacteremia cases present as meningococcemia, the most dangerous manifestation as patients often fail to respond to treatment, and can die within hours of disease onset.28,30 While IMD has a case-fatality rate of 8–15%, meningococcemia itself has a case-fatality rate of up to 40%, with up to 20% of meningococcemia survivors experiencing sequelae such as amputation or neurological and hearing impairment.18 A recent systematic review by Olbrich et al.31 found that IMD survivors without physical sequelae faced both short- and long-term negative impacts on self-esteem, physical, and mental health including anxiety, learning difficulties, and emotional and behavioral difficulties. These impacts were worse in survivors with physical sequelae.
The potential utility of liposomes for Neisseria vaccines
Published in Expert Review of Vaccines, 2021
Myron Christodoulides, Maria Victoria Humbert, John E Heckels
N. meningitidis lives as a commensal of the human nasopharyngeal microbiota, but more rarely can behave as an opportunistic pathogen to cause Systemic (Invasive) Meningococcal Disease (SMD). Meningococcal infection has caused historically significant mortality and morbidity worldwide [7] and SMD can be classified into four clinically distinctive disorders: i) shock without meningitis (fulminant septicemia), ii) shock and meningitis, iii) meningitis without shock and iv) meningococcemia without shock or meningitis (mild SMD, where patients usually present with fever and may also have a petechial rash) [8]. Meningitis is the most common presentation of SMD and shock without meningitis has the highest mortality rate. SMD is a consequence of unrestrained compartmentalized intravascular and intracranial meningococcal growth and host inflammation. Like gonococci, meningococci can also cause atypical infections of other anatomical sites, including the genitourinary tract and eye [5].
Endogenous Endophthalmitis
Published in Ocular Immunology and Inflammation, 2018
Emmett T. Cunningham, Harry W. Flynn, Nidhi Relhan, Manfred Zierhut
Shah et al17 described two non-immunocompromised adults from London, UK, with unilateral endogenous Meningococcus endophthalmitis in the setting of arthropathy. Neither had evidence of sepsis, meningismus, or fever often seen in patients with meningococcemia. Both presented with light perception vision and panuveitis associated with hypopyon formation, and one had acute ocular hypertension. In each case, erythrocyte sedimentation rates and C-reactive protein levels were markedly elevated, yet blood cultures were negative, and the diagnosis resulted from PCR-based identification of Neisseria meningitides ctrA gene DNA. Despite aggressive treatment with combined intravitreal and systemic antibiotics, vision either failed to improve or progressed to no light perception. The authors cited three additional reports of endogenous Meningococcus endophthalmitis with joint involvement and noted the generally poor outcome in these patients. They also noted the presence of fever, meningitis, a petechial or purpuric skin rash, and other signs of sepsis in a sizable proportion of patients with meningococcemia, but stressed the importance of considering the diagnosis of endogenous Meningococcus endophthalmitis in patients without these findings, but with a recent history of acute arthritis with or without fever – particularly the very young, the elderly, and those with non-age-related causes for immunosuppression. They also commented on the utility of commonly available PCR-based assays applied to both vitreous and joint fluid for diagnosis.