The Turkish Journal of Pediatrics
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Meningococcal Disease in Children: A Clinical Review
Ahmed Sabra, Jonathan Benger
The Academic Department of Emergency Care, Bristol Royal Infirmary, University Hospitals Bristol NHS Foundation
Trust, Bristol, United Kingdom
|Meningococcal disease is a global burden and remains one of the leading
infectious causes of death in children, with an estimated annual death rate of
170,000 worldwide. Despite these figures, the management of children with
severe meningococcal sepsis and septic shock remains suboptimal. This review
presents an overview of this condition including the epidemiology, pathogenesis,
clinical manifestations, complications, management, and prediction.and prediction.|
meningococcal disease, epidemiology, pathogenesis, prevention, Neisseria
meningitidis, meningococcemia, meningitis, children.
1. Neisseria meningitidis
Meningococcal disease (MCD) is caused by
a bacterial microorganism called Neisseria
meningitidis, a member of the genus Neisseria,
which is an obligate human-specific pathogen
that preferentially colonizes the mucous
membrane of the nasopharynx. Neisseriae are
gram-negative diplococci with two pathogenic
members: N. meningitidis (meningococcus) and
N. gonorrhoeae (gonococcus). These are very
similar in their morphological and cultural
characteristics. In pus from inflammatory
exudates, pathogenic neisseriae are usually
found inside polymorphonuclear pus cells, and
they appear, in gram-stained specimens, as
kidney-shaped pairs with the opposed surfaces
flat or slightly concave,. Other members of
the genus Neisseria are common commensals of
the nasopharynx. These include N. lactamica, N.
polysaccharea, N. subflava and N. sicca, which are
of low pathogenicity,. Colonies of pathogenic
neisseriae (N. meningitidis and N. gonorrhoeae) are
identified by their ability to produce acid from
glucose. On the other hand, they do not ferment
lactose or sucrose, and therefore, they could
be differentiated from low or non-pathogenic
neisseriae4. Despite their similar morphological
and cultural characteristics, meningococcus and
gonococcus are associated with two entirely
different diseases: meningococcal disease and
Neisseria meningitidis has five major serogroups
that are pathogenic for humans: A, B, C, W135,
and Y. Serogroups A, B, and C account for more than 90% of all invasive MCD, while
less than 10% of clinical isolates are from
serogroups W-135 and Y. The classification of
serogroup is determined by the meningococcal
lipopolysaccharide (LPS) capsular antigen.
Serotyping and subtyping are used to further
classify meningococcal strains by the variations
in outer membrane proteins (OMP). These are
classified according to electrophoretic mobility
into five major classes including PorA (class 1
protein) and PorB (class 2 and 3 proteins).
OMP act as cation- or anion-selective porins
controlling the influx of water-soluble molecules
through the outer membrane and are linked
to the severity of the disease.
Meningococcal disease (MCD) is a global
burden, with an estimated annual death rate
of 170,000 worldwide. The disease has an
overall mortality greater than 10% and is even
higher in the developing world, reaching 26%.
Invasive disease is most common among young
children, with a slightly higher incidence in
males (55% of cases) than females,. Up to
10% of the population may be asymptomatic
carriers with nasopharyngeal colonization, but
higher rates among children can be seen in
crowded conditions. Meningococcal carriage
rates are expectedly higher in institutions
such as universities, schools, prisons, and
military institutions. A study by Neal et al.
showed a dramatic increase in carriage rates
among students in their first year at a British university. This was, particularly, in the first
week of the academic year, with meningococcal
carriage rates increasing rapidly from 6.9% (day
1) to 23.1% (day 4).
Meningococcal disease (MCD) occurs
sporadically and in epidemics throughout the
world with seasonal variations. As mentioned
above, there are five major pathogenic
organisms that cause invasive MCD, and their
prevalence varies with time and geographical
location (Fig. 1). Areas such as sub-Saharan
Africa from Ethiopia to Senegal (known as the
meningitis belt), Nepal and India are endemic
for serogroup A, which caused large epidemics
during the nineties, whereas serogroups B
and C tend to be the commonest in Europe
and most of the Americas,,,. In 2009, the
meningitis belt area struggled to cope with
another large epidemic affecting thousands of
people, with a case fatality of up to 11.4%.
Most of these cases were reported from one
epidemic focus including Northern Nigeria
and Niger, which is again characterized by the
predominance of serogroup A. In contrast
to serogroup A, MCD due to serogroup W-
135 occurs in smaller numbers and has been
associated mainly with outbreaks during the
Hajj (pilgrimage to Mecca) season.
In the United Kingdom (UK), about twothirds
of cases were due to group B, onethird
to group C and less than 5% to other
groups. However, the epidemiology in the
UK has changed in recent years. This is
explained by the dramatic decline in the number
of cases caused by serogroup C following
the implementation of a new meningococcal
serogroup C conjugate (MCC) vaccination
program, which has successfully reduced the
morbidity and mortality rate from serogroup
C disease. As in other parts in the world, in Turkey, the epidemiology of N. meningitidis
is also changing. Previously, carriage rate
due to serogroups A and C accounted for
most isolates,. Nonetheless, recent studies
show that W-135 and B are currently the
commonest serogroups causing meningococcal
meningitis,. This current trend may be
attributed to pilgrims travelling to Saudi Arabia
for the Hajj.
| ||Fig. 1. Distribution of predominant N. meningitidis
serogroups before meningococcal serogroup C conjugate
Nasopharyngeal carriage is the reservoir of
pathogenic meningococci in 5-20% of the
general population, and man is the only known
reservoir. Nasopharyngeal colonization usually
remains asymptomatic and does not progress
further. However, following colonization of
the nasopharyngeal area of the upper airway
tract by meningococci, bloodstream spread may
ensue. This type of invasion is typically seen
following upper respiratory tract infections.
The development of invasive MCD is dependent
upon a wide variety of bacterial, host and
3.1. Bacterial Factors
Some meningococcal strains (virulent strains)
are more likely to cause invasive MCD.
Virulence of the meningococci is determined
by the ability to release endotoxins and adhere
to and invade nasopharyngeal epithelium. This
is achieved through the presence of surfaceexpressed
proteins, such as pili and OMP,
polysaccharide capsule and lipooligosaccharide
(LOS),. Type IV pili expressed by N. meningitidis
are essential for selective adherence to host
non-ciliated epithelia, and this gives the
meningococci their ability for colonization and
transmission. Meningococcal LOS (endotoxin)
is another factor implicated in meningococcal
interaction with host epithelial cells and
constitutes up to 50% of the outer membrane
of pathogenic neisseriae. LOS is biochemically
similar to LPS of gram-negative bacteria, in that
it contains a lipid A subcomponent. In addition
to interaction with host epithelial cells, it is
also a major factor contributing to the human
proinflammatory response to meningococci.
LOS may alter the permeability of the bloodbrain
barrier, which is important for the invasion
of the central nervous system. Furthermore,
it causes the activation of macrophages and release of tumor necrosis factor, a primary
mediator of meningococcal septic shock. This
outlines the importance of LOS as a virulence
component involved in multiple steps in the
pathogenesis of both meningococcal meningitis
and meningococcemia. OMP that act as cationor
anion-selective porins (PorA and PorB),
controlling the influx of water-soluble molecules
through the outer membrane, are linked to
the severity of the disease. The surfaceexposed
loops of PorA are greatly involved
in activating the human immune system by
inducing the production of bactericidal and
opsonophagocytic antibodies. Once through
the epithelium, N. meningitidis enters into the
bloodstream, where the capsular polysaccharide
enhances the survival of meningococcus by
resisting phagocytic killing.
3.2. Host Factors
Age is an important factor, which is evident by
the variation in incidence of MCD in different
age groups. The peak incidence of the disease
is in the first year of life, which could be
explained by the loss of maternal antibody.
Moreover, it seems that asymptomatic carriage
contributes to increasing the immunity against
the disease in older populations. Host factors
are still not fully understood. Older patients
with hereditary complement deficiencies are
more likely to acquire MCD and have more
severe infection than others. For example,
patients who have deficiencies in the terminal
component C5 to C9 of the complement
cascade will usually suffer from recurrent
infection with gram-negative bacteria, caused
almost solely by meningococci,,, whereas
absent or malfunctioning properdin results
in an increase in both risk and severity
of MCD. Also, there is an increased risk
among persons with acquired complement
deficiencies, such as nephrotic syndrome,
systemic lupus erythematosus and hepatic
failure. This highlights the importance of the
complement system in defense against MCD.
Anatomical or functional asplenia are other
recognized factors predisposing to MCD. Other
host factors that may affect the outcome and
severity of MCD include protein C or protein S
deficiency and decreased endothelial expression
of thrombomodulin and protein C receptors,
which are linked to the severity of the disease
and development of purpura fulminans.
3.3. Environmental Factors
The transmission and development of
invasive MCD has been associated with
many environmental factors. Crowded living
conditions, low socioeconomic status and
antecedent viral infections, particularly
influenza, are recognized factors with increased
risk of MCD. Other factors include both active
and passive smoking. Maternal smoking has
been demonstrated to be a significant risk
factor for the development of invasive MCD in
infants,. The effect of smoking is presumed to
be due to disrupting the respiratory epithelium
with a decrease in mucociliary function and
hence reduced bacterial clearance. During
outbreaks, other factors that are associated with
higher risk of acquiring invasive MCD include
intimate kissing with multiple partners, being
a university student and preterm at birth,
binge drinking, marijuana-related activities,
bar patronage, and attendance at a party of
adolescents or young adults. The increased
risk of invasive MCD associated with these
activities may be explained by a combination
of factors that could facilitate transmission,
including overcrowding, intimate contact with
carriers, poor ventilation, sharing of drinking
glasses and cigarettes, active and passive
smoking, and smoking-associated coughing.
Natural immunity against N. meningitidis
frequently develops after repeated colonization
with different serogroups or serotypes.
Additionally, enteric bacteria that express crossreactive
antigen and non-pathogenic neisseriae
contribute to the development of natural
immunity against meningococcal infection.
Immunity can also be induced artificially
by vaccination. Prevention includes primary
prevention by vaccination and secondary
prevention with chemoprophylaxis for close
contacts of patients with MCD.
4.1. Primary Prevention
Vaccines against groups A, C, W135, and
Y meningococci have been licensed in the
United States of America (USA), the UK and
other countries,,. Polysaccharide vaccines
including the tetravalent vaccine (Menomune®)
against serogroups A, C, W-135, and Y were first developed 30 years ago, and research
studies showed that in adults, the vaccine
induced the production of suitable levels of
bactericidal antibodies, which were maintained
for up to one year following immunization.
The limitation of these polysaccharide vaccines
is that they are ineffective in young children and
induce only short-term protection. However, a
new approach to improve the immunogenicity
of these polysaccharide vaccines was achieved by
chemical conjugation to a carrier protein, which
transformed the vaccine to a T-cell-dependent
antigen inducing long-term immunity,. A
promising phase 2 study provides evidence for
the efficacy of a novel tetravalent meningococcal
(MEN-ACWY) vaccine in infants by using a
nontoxic mutant of diphtheria toxin as the
carrier protein and aluminium phosphate as
an adjuvant. An earlier successful attempt
led to the development of the MCC vaccine,
which changed the healthcare practice in
the UK with the implementation of a new
vaccination program in November 1999.
The UK was, in fact, the first country to start
mass vaccination with the MCC vaccine, which
was integrated into the routine immunization
schedule for infants, administered as one dose
for those under 5 years of age, and as a catchup
school-based immunization campaign for
adolescents. The benefits of the new MCC
vaccine have been demonstrated in many
research studies-. These included high levels
of herd immunity and reduced morbidity and
mortality from laboratory-confirmed serogroup
C disease in England and Wales,. MCD
due to serogroup C in different age groups is
almost nonexistent after five years of the MCC
vaccination program. An effective vaccine
against serogroup B is not yet available for
routine use in young children,,. The major
challenge in developing a vaccine targeting the
serogroup B capsular polysaccharide is its poor
immunogenicity in humans. This could be
explained by the cross-reactivity with human
neural antigens that express structurally similar
antigens,. Several attempts have been made
to develop a reliable vaccine against serogroup
B. For example, a nine-valent meningococcal B
PorA vaccine (NonaMen®) has been developed
with promising results inducing suitable anti-
4.2. Secondary Prevention
Meningococcal meningitis and septicemia are
both notifiable diseases. Protection of close
contacts is possible via administration of
effective chemoprophylaxis, which includes
rifampicin, ceftriaxone or ciprofloxacin,,.
The rationale of antibiotic prophylaxis is to
eliminate nasopharyngeal carriage in close
contacts and thus prevent the development and
transmission of pathogenic strains. Although
evidence suggests that other antibiotic regimens
such as azithromycin or a combination
of rifampicin and erythromycin may be
effective in eradicating nasopharyngeal
carriage, only rifampicin, ciprofloxacin and
ceftriaxone are currently recommended for the
chemoprophylaxis of MCD in the UK national
guidelines, and the USA.
4.3. Other Measures
Other preventive measures include public
education, reducing overcrowding in living
quarters and workplaces, and isolation of
patients for 24 hours after start of antibiotics
with concurrent disinfection of discharges.
Good communication and involvement of
parents, school, nursery, and college are other
measures that may reduce any unnecessary
concerns and contain the disease at the time
of epidemics. Additionally, all risk factors such
as smoking, binge drinking, and attending
of overcrowded places should be addressed,
especially during outbreaks of the disease.
5. Clinical Manifestations
Following colonization of the nasopharyngeal
area by meningococci and then bloodstream
spread, invasive MCD may manifest in various
infectious syndromes. The spectrum of MCD
ranges from occult bacteremia, which is selflimited,
to severe sepsis resulting in death
within a few hours. Invasive MCD tends to
manifest mainly in two major forms, meningitis
and septicemia, with the predominant features
of cardiovascular collapse and cutaneous
manifestations of clotting disorder. In Europe,
the commonest presentation is actually a mixed
picture of both meningitis and septicemia
(60-66%), followed by septicemia alone (22-
25%) and lastly meningitis alone,. The
septicemia only presentation tends to have
a greater mortality rate than the meningitis
only presentation. A large retrospective study
between 1977 and 1993 reported a 19%
mortality rate for children with meningococcal
septicemia, 11% for those with mixed picture of
sepsis and meningitis and 1.2% for meningitis
only. About half of the patients who die of
MCD do so within 24 hours of admission.
Data from the developing world showed a
higher proportion of fatalities, with more than
70% dying within 24 hours of admission,.
This high mortality rate may be explained by
the poorly developed healthcare system, limited
resources, late presentation, and difficulties
accessing immediate healthcare, in addition to
differences in socioeconomic and environmental
5.1. Meningococcal Septicemia
This syndrome results from the systemic release
of various mediators in response to bacteria
endotoxins leading to generalized increase
in capillary permeability. Meningococcemia
is characterized by shock and disseminated
intravascular coagulation (DIC). Diagnosis
is not always straightforward because classic
clinical features may be absent or non-specific
at initial presentation. Initially, there may
be a prodrome of an upper respiratory tract
infection followed by high fever, poor feeding,
lethargy, malaise, headache, and nausea. Then,
within a few hours, the toxic picture of septic
shock and DIC becomes apparent. Cold hands
and feet, leg pains and abnormal skin color
(skin mottling or pallor) were reported to be
early signs of MCD, which precede the typical
symptoms by several hours. These findings
attracted public attention and were included
in the recently published guidelines by the
Scottish Intercollegiate Guidelines Network
(SIGN). However, no data are available
about the predictive values of these nonspecific
symptoms. Should these be low, this
would unnecessarily increase the workload
of emergency and primary care physicians
and the burden on the healthcare system by
unnecessary admissions. Hence, more research
in this area is clearly justified to give a precise
answer. Skin rash is another characteristic
feature of this syndrome, which may begin as
a non-blanching rash (erythematous macules)
or petechiae progressing to purpuric lesions
and large hemorrhagic areas. However, the interpretation of non-blanching rash should be
done in the context of the overall picture. Only a
small percentage of children with non-blanching
rash will have MCD, whereas the rest are likely
to have viral illnesses. An important feature
to differentiate meningococcal rash is that it
is unlikely to be confined to the distribution
of the superior vena cava. Thompson et
al. reported petechial rash as being the
first and most common (42–70% of cases)
classic symptom to emerge and that parents
are usually alerted to act by this symptom.
This is expected following the intense public
education campaigns about MCD quoting nonblanching
rash as an important warning sign.
Cases of fulminant meningococcemia can also
be complicated by massive adrenal hemorrhage
(Waterhouse-Friderichsen syndrome), which is
characterized by a rapidly progressive course
of irreversible shock and DIC with massive
mucosal and skin hemorrhages.
Diagnosis of meningococcemia is confirmed
by cultures from blood, cerebrospinal fluid
(CSF) or skin lesion aspirate. Detection
of meningococcal DNA by polymerase chain
reaction (PCR) is another useful test to confirm
the diagnosis, particularly for patients who
received prior antibiotics. A quick detection
of meningococci is possible with Gram stain
of buffy coat preparations of blood, CSF or
skin lesion biopsy/aspirate.
5.2. Meningococcal Meningitis
The invasion of the meninges and crossing
of the blood-brain barrier with the sequential
liberation of endotoxins and activation of
pro- and anti-inflammatory cytokines are the
underlying pathophysiological processes of the
clinical picture from meningococcal meningitis.
This may also result in brain edema and high
intracranial pressure. Patients presenting with
meningitis share similar symptoms and signs of
other types of meningitis. These include fever,
headache, neck stiffness, nausea, vomiting,
impaired consciousness, photophobia, and
seizures. The classic meningeal signs such as
Kernig sign, Brudzinski sign and fever may be
absent in neonates and small infants,, and
therefore the threshold to admit to hospital
and treat should be lower. In contrast to
meningococcemia, meningococcal meningitis is
usually straightforward to diagnose; however,
atypical presentation with focal neurology without the characteristic rash may make the
diagnosis of MCD more difficult. Children
with meningitis are generally better than
those with meningococcemia alone, and
as mentioned earlier, have a relatively good
prognosis,. When meningitis is associated
with septicemia, it may present with sudden
onset and rapidly progressive manifestations
of shock, purpura and reduced level of
consciousness. The prodrome of meningitis
resembles that of meningococcemia including
symptoms of upper respiratory tract infection,
but the course is more insidious.
The diagnosis of meningococcal meningitis is
confirmed by examining the CSF including
culture. Biomedical analysis shows a low
CSF: blood glucose ratio and high protein,
whereas microscopy shows high neutrophils
and gram-negative intracellular diplococci.
Other possible tests include those mentioned
earlier under meningococcemia such as PCR
5.3. Rare Presentations
Meningococcal disease (MCD) may rarely
present in other forms such as upper respiratory
tract infection, tonsillitis, pneumonia,
septic arthritis, pericarditis, peritonitis,
osteomyelitis, conjunctivitis, endophthalmitis,
or chronic meningococcemia.
Chronic meningococcemia is a rare clinical
manifestation of MCD presenting as recurrent
attacks of fever, arthralgia and maculopapular
rash with normal periods in the interim when
symptoms may disappear completely,. The
nature of this condition makes it more difficult
to diagnose, and it is commonly misdiagnosed
as collagen or autoimmune disease such as
Henoch-Schönlein purpura. The diagnosis of
chronic meningococcemia is usually confirmed
by blood culture taken during febrile episodes,
but several blood cultures may need to be
performed, as false-negative results are high,.
The course of this condition is variable, ranging
from spontaneous recovery to progression to
systemic complications. Generally, it has an
excellent prognosis for patients treated with
appropriate antibiotic therapy, with a cure rate
The rates of complications and sequelae were
linked to the severity of MCD associated with
a specific strain of N. meningitidis: serogroup
C serotype 2a. These complications include
skin infarction, adrenal hemorrhage, reactive
arthritis, endocarditis, myocarditis, renal
infarction, lung abscess, subdural effusion
or empyema, and brain abscess. Another
fatal complication is basilar artery occlusion
secondary to intracerebral purpuric lesions,
which manifests with collapse and respiratory
arrest in an apparently improving patient. Most
patients who survive the disease fully recover;
however, a significant number of patients
will suffer permanent neurological sequelae
such as intellectual impairment and cranial
nerve deficits including deafness,, and
peripheral amputations,. Other recognized
but rare complications are avascular necrosis
with growth disturbances and late skeletal
deformities, seizures, blindness, hemiparesis or
quadriparesis, and obstructive hydrocephalus.
Cataract and uveitis are reported as well. All
these complications are assumed to be related
to vasculitis, DIC and hypotension of severe
MCD. As these pathophysiological processes
are mostly associated with septicemia, this
may explain the higher morbidity and mortality
rates from meningococcemia rather than from
This review utilizes the published definitions
of systemic inflammatory response syndrome
(SIRS), severe sepsis and septic shock defined by
Goldstein and the Members of the International
Consensus Conference on Paediatric Sepsis.
Table I presents the categorization of these
syndromes in line with the published consensus
and Figure 2 illustrates these various syndromes
and their overlap.
8. Clinical Management: Therapeutic Goals
Meningococcal disease (MCD) is a medical
emergency. Early recognition of invasive MCD
is crucial to successful disease management.
Management requires immediate treatment
of the underlying infection and its systemic
manifestations. If there is any suspicion of
meningococcal infection, antibiotic therapy
should be initiated immediately. A benefit of general practitioners (GPs) immediately
administering antibiotic (e.g. parenteral
benzylpenicillin) to patients with suspected
meningococcal septicemia was demonstrated,
showing that these patients are 2.5 times less
likely to die than those not given penicillin.
Many other studies support the early use of
antibiotics, showing a higher mortality rate
from delays in administrating antibiotics-
aff3#aff3 In contrast, however, a controversial
study suggested that antibiotic therapy in
the community increased mortality. In that
study, the average Glasgow Meningococcal
Septicaemia Prognostic (GMSP) score for
patients who received penicillin was noticeably
higher than for those who did not. Since the
severity of the disease correlates well with
poor outcome, it is important to consider
the confounding potential of severity on the
findings of that study. Also, most GPs justified
their decision when penicillin was not given
as being due to uncertainty in the diagnosis,
and hence this is another potential source of
bias. The authors recommended conducting
a randomized controlled trial to provide a
definitive answer. However, this would be
unethical unless stronger evidence is available
from large prospective studies that control
for any possible biases. Hence, GPs should
continue giving antibiotics in line with the
recommendation from national health agencies such as SIGN and the Health Protection
Agency. Penicillin, chloramphenicol and thirdgeneration
cephalosporins are all antibiotics
recognized in the treatment of MCD. Resistance
to both penicillin and chloramphenicol has
been reported,, and hence, third-generation
cephalosporins (cefotaxime and ceftriaxone)
are currently the mainstream antibiotics used,
with proven good CSF penetration.
| ||Table I. Definitions of SIRS, Sepsis, Severe Sepsis and Septic Shock*|
Following admission to the hospital, the
main target of therapy is to maintain
adequate microcirculation. Therefore, volume
resuscitation to restore the intravascular
compartment and inotropes to support the
myocardium are the main approaches to
the management of meningococcal septic
shock. Failure to administer adequate fluids or
inotropes was associated with an increased risk
of death in MCD. An audit recently conducted
in the UK showed failure in more than 60%
of cases to follow a consensus guideline on
emergency management of children with severe
sepsis and septic shock, with most children receiving inadequate fluid resuscitation and
inotropic support during the golden hours
following presentation. This failure mainly
affected children presenting with shock, and
may have resulted in a higher mortality rate.
A previous study showed similar results
with hospital treatment being suboptimal
in 71% of patients, with higher fatalities in
patients with longer times from illness onset
| ||Fig. 2. The overlap of various syndromes.|
As with any other patient with severe sepsis,
the management of these patients may also
include, subject to severity, ventilatory support,
hemofiltration, steroid therapy, administration
of activated protein C, and administration of
blood and blood products. The instigation of
these therapies should be considered without
delay within the framework of goal-directed
therapy. This implies stepwise management
with certain therapeutic endpoints being
achieved within a specific time interval. The
guidelines published by the American College
of Critical Care Medicine recommended the
therapeutic endpoints presented in Table II.
Despite increasing awareness of the concept of
goal-direct therapy since the mid-eighties, it
was not linked to outcome and reported until
the late 1990s. Subsequently, a substantive
change in the management of septic patients
was applied with studies reporting a decrease
in mortality rate both in children and adults
following this management approach. The
Surviving Sepsis Campaign is a global
initiative aiming to improve the management
of sepsis based on the concept of early goaldirected
therapy or evidence-based goals.
This provides guidelines (adult and pediatric)
and sepsis management bundles, which are
accessible via the internet from anywhere in
9. Outcome and Prediction
Several investigators have identified unfavorable
prognostic features in patients with MCD
using clinical and laboratory parameters. Many
studies have been undertaken to generate
prognostic scores using these parameters as
predictors of a patient’s outcome and risk of
mortality-. All available scoring systems
were primarily developed to predict death,
either specifically in MCD,-, or generically
in a critically-ill pediatric population,,.
More than 25 specific scoring systems have
been developed for prediction in MCD,,-
,-. However, not all of these scores are
widely used. The GMSP score is the most
well-known (Table III). Children presenting
with a GMSP score of ≥8 are at an increased
risk of death,. There are two other generic
scores widely used that have been validated for
use in MCD. These are the Pediatric Index of
Mortality (PIM) and Pediatric Risk of Mortality
(PRISM). Some of these scores were developed
on the basis of an extended period of time
(e.g. values over 24 hours) rather than at a
single point on first medical contact. Others
were calculated from the information collected
at the time of the first face-to-face contact,
or one hour after, between the patient and a
doctor from the pediatric intensive care unit
Despite the extensive research done about
prediction in MCD, there is a scarcity of work
about predicting the level of supportive therapy
(fluid and inotrope therapy) required. Hence,
there is a need for more research to identify
the important predictors of management
requirements. This would improve the
management of MCD, with the ultimate goal
of increasing the survival rate and decreasing
| ||Table II. The Therapeutic Goals In and Beyond the First Hour of Presentation|
| ||Table III. Glasgow Meningoccoccal Septicaemia Prognostic (GMSP) score90|
Meningococcal disease continues to be one
of the main infectious causes of childhood
mortality. In spite of modern therapies,
mortality is 5-10% in developed countries
and much higher in the developing world,,.
Early recognition, aggressive resuscitation and
normalization of all physiological parameters,
with prompt referral to a specialist PICU for
severe cases, may lead to a significant reduction
in the case fatality rate. This overview of
MCD has identified a number of key issues
relating to improving the management of this
condition. However, many studies identified a
failure of recognition and management of severe
sepsis and septic shock despite the availability
of evidence-based published guidelines. There
is insufficient published work addressing this
issue to date. There is therefore a need for more
research to explore these shortcomings.
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