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By: Bruce Alan Perler, M.B.A., M.D.

  • Vice Chair for Clinical Operations and Financial Affairs
  • Professor of Surgery

https://www.hopkinsmedicine.org/profiles/results/directory/profile/0002711/bruce-perler

It is also weighted so that its base is heavy skin care qualifications purchase cheapest benzac, encouraging the individual to acne holes purchase benzac 20 gr line put the cup down between mouthfuls skin care vancouver order benzac on line amex. Other methods of altering the way in which individuals receive their solids or fiuids may have to skin care logos buy 20gr benzac mastercard be individually tailored. I had some success modifying a sauce bottle by attaching a piece of fiexible straw-like tubing to its end so that individuals could use their hand to squeeze the fiuid up the tubing and deliver it to the posterior of the oral cavity. The person for whom this adaptation was made had had a large proportion of the soft palate removed due to cancer. He was finding it difficult to manipulate the fiuid so that it fiowed down his stronger side. The task of drinking was much less taxing for this patient because he was able to deliver the bolus to the posterior of the oral cavity and direct it to his stronger side. Despite our best efforts, it is not always possible for individuals to keep taking food and fiuids orally. Aspiration is one reason why individuals may be fed for the short or longer term nonorally. Individuals who take 10 s or more to prepare every mouthful will be too fatigued to meet all of their nutritional needs orally. A nasogastric tube allows for nutrition to be delivered to the individual without the added pressure of eating while the individual is recovering. Percutaneous endoscopic gastrostomy are generally considered a long-term solution to the issue of non-oral feeding. Here a surgical incision places an entry point permanently into the stomach for direct placement of the nutritional bolus. Percutaneous endoscopic gastrostomies can be removed if patients improve to the point where they no longer need them. Complications with this procedure include, the risk of infection around the stoma site, soreness of the site and leakage out of the stoma. Total parenteral nutrition is reserved for patients who are unable to take nutrition via enteral means, i. It is possible for an individual to receive non-oral feeding and, when safe, commence small amounts of oral intake. This is often done in the middle of the day when the individual is most alert and least likely to be fatigued. Non-oral feeds can be gradually reduced so that the individual experiences the sensation of hunger and is thus more likely to want to eat food orally. In one case, an individual seen clinically who presented with surgical removal of the right portion of the soft palate, a surgical device provided both improved communication and improved swallowing. The speech pathologist accompanied the patient to a prosthedontist who designed and fitted an obturator for the patient. The obturator was a plate-like device that attached to the patient’s existing upper teeth. It had a large bulbous portion at the posterior region that acted to fill the void left by surgical removal of the portion of soft palate tissue. The prosthesis required a tongue impression and it was reshaped so that it was individualized for the patient. Although speech therapy was still required to enhance communication after fitting the obturator, the patient’s communication and swallowing skills were significantly improved when the obturator was used. This device has most commonly been used for individuals who have sections of the oropharynx removed due to cancer, especially after glossectomy. It is also useful for individuals with impaired tongue mobility due to trauma or neurological disorders (Marunick and Tselios, 2004). It is possible, for example, to ‘build up’ the prosthetic palate so that the vault of the hard palate is lowered. Hence the patient with limited tongue movement is more likely to be able to achieve tongue-to-palate contacts, which are required for both speech and swallowing. This type of prosthesis may particularly assist the oral stage of swallowing, assisting with more efficient food preparation and reducing the likelihood of oral residue (Light et al. A palatal lift is another prosthetic device that may be considered when there is minimal or no elevation of the soft palate. In addition to extremely poor soft palate function, the potential candidate should also have a reduced gag refiex. This is to ensure that when the prosthesis is in place the device does not trigger a gag refiex. When the device is in place the incompetent soft palate is brought level with the hard palate. This device is particularly useful for reducing nasal air emissions by providing a mechanical barrier for air leakage into the nasal cavity. It is also possible to use the palatal lift prosthesis in combination with the palatal augmentation device. Together these devices may be useful for the treatment of speech and swallowing disorders where the deficit lies with the soft palate region (Light et al. This type of surgery is obviously only suitable for individuals who have had portions of the velopharynx surgically removed. This type of surgery would not be suitable for individuals with velopharyngeal insufficiency as a result of neurological deficit. The radial foream free fiaps have the benefit of being able to restore sensation as one of the antebrachial cutaneous nerves is attached to the glossopharyngeal or greater palatine nerve. They work in the same way as the prosthesis, although they have the advantage of being permanent and re-establishing sensation where possible. Selley (1985) has reported on the use of an intra-oral device called a palatal training appliance. The appliance consists of a 1mm stainless steel piece of wire that is bent into a U shape. The device is bent to conform to the shape of the patient’s resting soft palate, with the bottom of the U shape lightly touching the soft palate near the base of the uvula. The aim of the device, which is worn throughout the day, is to stimulate a refiexive swallow. The mere presence of the device in the mouth causes some initial increased salivation. It is possible that the increased sensory stimulus offered by additional saliva in the oral cavity may in fact be more of a catalyst for improving swallowing function, simply by increased practice! In addition these should be evaluated in conjunction with swallowing studies, patient expectations, and patient motivation. These factors in combination will allow the determination of realistic treatment goals (Marunick and Tselios, 2004: 68). An inability to protect the airway during swallowing places the individual at risk of aspiration. The concept behind medialization is that an additional bulk is added to the damaged vocal fold. This is done by either surgically inserting an implant (Type I thyroplasty), or injecting the damaged cord with Tefion or Gelfoam. For Type I thyroplasty the implant is placed between the thyroid cartilage and the vocalis muscle (Woo, 2000). Both the surgical and injection procedures are carried out by a qualified surgeon. These include those with bleeding disorders or compromised immune status, those undergoing chemotherapy and those with poor wound healing. Apart from immobility of the damaged cord, there is also an absence of vocal fold tension and muscle atrophy begins. The medialization thyroplasty and injection procedures aim to reshape the position of the cord and also the stiffness of the damaged cord (Woo, 2000). With the damaged cord bulked up and in a more midline position, the functional cord has a better chance of contacting with it to provide adequate airway closure. Arytenoid adduction may also be performed in conjunction with medialization thyroplasty. Even with successful medialization thyroplasty there may still exist a ‘posterior chink’ or opening. This posterior opening would mean that a portion of the cords was not entirely closed and could allow material to be aspirated through the opening. In order to address this problem, the surgical approach of arytenoid adduction may be used. Again, a qualified surgeon is best placed to recommend the type of surgery most suitable for individual situations.

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Complete blood count and electrolyte panel are frequently obtained during syncope evaluation acne before period order benzac with a visa. The diagnostic yield is low when these are used routinely; however acne in pregnancy buy 20 gr benzac with amex, when these blood tests are conducted in patients with a suspected related diagnosis skin care qualifications benzac 20gr otc. Thus acne description buy 20gr benzac mastercard, specific testing should stem from the assessment by history and physical examination when the nature of the syncope presentation or associated comorbidities suggests a diagnostic or more likely prognostic role for laboratory testing. See Online Data Although data to support biomarker testing are in general relatively weak, there are sufficient data to suggest that Supplements natriuretic peptide is elevated in patients whose subsequent cause for syncope is determined to be cardiac. The ability of troponin and natriuretic peptide 129 measurement to infiuence clinical decision making or patient outcome is unknown. There is little biological plausibility linking the remaining elements of broad-panel laboratory testing to the presentation or mechanism of syncope. Cardiovascular Testing: Recommendations the abnormalities found during cardiovascular testing may Cardiovascular causes of syncope are common. As such, cardiovascular testing can be a critical element in the evaluation and management of selected cardiovascular testing. Transthoracic echocardiography can be useful when healthcare providers are concerned about the presence of valvular disease. Although an echocardiogram may not be able to establish the immediate cause of syncope, it provides information for a potential disease substrate related to prognosis. These modalities offer superior spatial resolution in delineating cardiovascular anatomy. They provide information on the structural disease substrate relevant to the overall diagnosis and subsequent evaluation and follow-up in selected patients presenting with syncope. Unexpected findings on echocardiograms to explain syncope are uncommon; a “screening” echocardiogram is of low utility. Subjecting a patient to a treadmill exercise test to reproduce the symptoms or evaluate the hemodynamic response to exertion. However, bradyarrhythmia may ultimately be responsible for exertional syncope as well, and may only be elicited during stress testing. Cardiac Monitoring: Recommendations the choice of monitoring system and duration should be Although cardiac monitoring is often used in the evaluation appropriate to the likelihood that a spontaneous event will of palpitations or intermittent arrhythmias, the following recbe detected and the patient may be incapacitated and unable ommendations and discussion are focused primarily on the to voluntarily trigger the recording system. N/A the technology of cardiac rhythm monitoring is dynamic and advancing at rapid speed. Their selection and usefulness are highly dependent on patient characteristics with regard to the frequency of syncope and the likelihood of an arrhythmic cause of syncope. The effectiveness of any external cardiac Supplements monitoring device for syncope evaluation is related to the duration of monitoring, continuous versus intermittent 11 and 12. The patient activation, before or after an event, allows for symptom rhythm correlation; however, some external loop recorders are of limited use inpatientswhoare temporarilyincapacitatedaroundthetimeof syncope. Theadvantageofanexternal looprecorderoverHoltermonitoringstemsfromalonger 149,153 monitoring period, which confers a higher yield than Holter monitoring and may offer a diagnosis after a negativeHolterevaluation. One prospective, multicenter study of 392 patients (28% with syncope) reported a 4-week diagnostic yield of 24. The advances of new patch-based devices offer another and often less cumbersome means of identifying an arrhythmic cause for syncope. Some practices offer mobile continuous outpatient telemetry devices, which provide real-time arrhythmia monitoring and analysis. Importantly, there was a similar result in the subgroup of patients presenting with syncope or presyncope, with a significantly higher diagnostic yield in the mobile continuous outpatient telemetry group (89% versus 69%; p50. Table 8 Cardiac Rhythm Monitors Types of Monitor Device Description Patient Selection 151–153 Holter monitor A portable, battery-operated device Symptoms frequent enough to be detected Continuous recording for 24–72 h; up to 2 wk with newer within a short period (24–72 h) of monitoring* models Symptom rhythm correlation can be achieved through a patient event diary and patient-activated annotations Patient-activated, A recording device that transmits patient-activated data (live Frequent, spontaneous symptoms likely to recur transtelephonic or stored) via an analog phone line to a central remote within 2–6wk monitor (event monitoring station. However, the diagnostic yield of inpatient telemetry is low in the absence of high suspicion about an arrhythmic cause. In 1 prospective study of 2,240 patients admitted to a telemetry unit, patients admitted for syncope (10%) had low rates of unexpected intensive care transfer, and most were unrelated to arrhythmic conditions. A large, prospective evaluation of 2,106 patients admitted with syncope demonstrated high telemetry use (95%) but a diagnostic yield of only 5%. Electrophysiological Study: Recommendations or with low suspicion of an arrhythmic etiology. A positive response is defined as inducible presyncope or syncope associated with hypotension, with or without bradycardia (less commonly asystole). The hemodynamic response to the tilt maneuver determines 214 whether there is a cardioinhibitory, vasodepressor, or mixed response. There is general consensus that a tilt-table angle of 70 degrees for 30 to 40 minutes would provide optimal yield. This observation during tilt-table testing cannot necessarily define a causal etiology or be entirely conclusive of a refiex mechanism for syncope in the clinical setting. Correlation of tilt-table–induced findings to patients’ clinical presentation is critically important to prevent consequences of false-positive results from tilt-table testing. Prolonged convulsions and marked postictal confusion are uncommon in patients with syncope associated with convulsive movements,226 and fatigue is frequent after refiex syncope 226 and may be confused with a postictal state. Tilt-table testing has been shown to be of value in this clinical setting when a detailed history cannot clearly determine whether the convulsive movements were secondary to syncope, given the need for objective evidence to help distinguish this entity from true epileptic seizures. In a prospective study of 15 patients with recurrent unexplained seizure-like episodes who were unresponsive to antiepileptic therapy,223 67% had convulsive movements associated with hypotension and bradycardia during tilt-table testing. In another study of 74 patients with a questionable diagnosis of epilepsy (because of drug-refractory seizures or clinically suspected not to be true epilepsy), a cardiac diagnosis was established in 42% of patients, with. Neurological Testing: Recommendations persistent and often progressive generalized weakness, fatigue, visual blurring, cognitive slowing, leg buckling, and 3. These symptoms central or peripheral autonomic nervous system damage or may be provoked or exacerbated by exertion, prolonged dysfunction. Its causes should be sought so as to provide effistanding, meals, or increased ambient temperature. Such care may be provided by a neurologist, cardiologist, internist, or other physician who has sufficient training to treat these complicated patients. Central autonomic degenerative disorders include multiple system atrophy,241 Parkinson’s disease,242 and Lewy Body dementia. The evidence suggests that routine neurological Recommendations testing is of very limited value in the context of syncope evalMany patients undergo extensive neurological investigation uation and management; the diagnostic yield is low, with 36,77,78,251–260 after an uncomplicated syncope event, despite the absence very high cost per diagnosis. Neurological imaging may be indicated if significant head injury as a result of syncope is suspected. Management of Cardiovascular Conditions vant to and within the context of the specific stated cardiac the writing committee reviewed the evidence to support reccondition. Arrhythmic Conditions: Recommendations isting guideline recommendations in the present guideline, Cardiac arrhythmia is a common cause of syncope, and the except for the specific cardiac conditions in Sections 4. Management of paroxysmal and occult on initial evaluation—poses addipatients with syncope and heart disease would include treattional challenges and may warrant a more extensive evaluaing the immediate cause of syncope and further assessing tion (Section 3. The evidencecontinuestosupport,withoutchangefromthepreviousrecommendation,thenotionthatpermanentpacemakerimplantation is reasonable for syncope in patients with chronic bifascicular block when other causes have been excluded. The use of adenosine triphosphate in the evaluation of syncope in older patients continues to evolve. The writing committee has reached a consensus not to make a new recommendation on its use for syncope evaluation because of the limited data at this time. Comprehensive guidelines exist turepublished since publicationofthesedisease-specificguidefor diagnosis and management of many of these diseases, lines was performed to ensure that prior recommendations including sections on syncope. If new published data were of syncope is discussed in patients with underlying structural available, they were incorporated into the present document. Treatment of syncope is based on the specific cause of syncope, whereas treatment for the underlying cardiomyopathy impacts the long-term prognosis. A review of evidence supports previously published recommendations for patients with syncope in the presence of underlying cardiomyopathy. The mechanism is often hemodynamic, as opposed to arrhythmic, because of inability to augment and sustain cardiac output. In patients with valvular heart disease causing syncope, treatment is recommended by the latest guidelines.

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Multinucleated giant cells ies the vaccine has demonstrated signifcant reduction D skin care vitamins and minerals purchase benzac 20gr without prescription. A 60-year-old woman with history of diabetes mellitus are older or equal to skin care wiki cheap 20 gr benzac otc 50 years old; therefore tretinoin 025 acne order benzac australia, the vaccine and hypertension presents with a painful erythematous would be indicated for use in this patient acne 9 year old daughter discount benzac line. The patient was most likely started on zidovudine, a has 9/10 pain and complains of trouble sleeping. Chronic suppressive therapy for herpes simplex infecpain medication tion can be achieved with any of the 3 agents. Acyclovir dosing for suppression is 400 mg 2 times daily or 200 mg tid-qid, while famcicloAnswers vir dosing is 250 mg 2 times daily. The patient is employed at a health care facility, infection (“blueberry mufn baby”) including purpuric and therefore required to have either serologic evidence macules, thrombocytopenia, hepatomegaly, and microof immunity to or a 2-dose vaccination series with varicephaly. Nonvaccination is not an option in this case given infected cells is “owl’s eye” basophilic intranuclear incluthe profession of the patient and the potential risk to her sions. Given recent studmumps or Candida skin test antigens: a novel immunotherapy ies demonstrating a signifcant decrease in incidence of for warts. Safety and immunogenicity sure to vaccinia virus: case definition and guidelines of data colof glycoprotein-D adjuvant genital herpes vaccine. Impact of suppressive antiviral applied immune response modifier for the treatment of external therapy on the health-related quality of life of patients with recurgenital warts. Polymorphisms in the genes for genital warts with imiquimod 5% cream followed by surgiherpesvirus entry. Association of p53 polymortions: epidemiology, pathogenesis, symptomatology, diagnosis, phism with skin cancer. Verrucous carcinoma of the foot assological and virological findings in patients with focal epithelial ciated with human papillomavirus type 16. Once, twice, or three times notherapy of warts with mumps, Candida, and Trichophyton daily famciclovir compared with acyclovir for the oral treatskin test antigens: a single-blinded, randomized, and controlled ment of herpes zoster in immunocompetent adults: a rantrial. J Cutan Med Surg controlled, dose-ranging trial of peroral valaciclovir for epi2003;7:449–454. New York: Oxford; podofilox gel in the treatment of external genital and/or perianal 2001. New York: prednisone for the treatment of herpes zoster: a randomized, Marcel Dekker; 2002. The pustules may rupture leaving with scarring contagious honey-colored crusts • Treatment: topical antibiotics, systemic antibiotics may be • Treatment: topical mupirocin indicated • Bullous impetigo is a toxin-mediated erythroderma Furuncles/Carbuncles (Fig. Periorificial and flexural accentuation may be • Clinical observed • Erythematous and irregular appearing linear streaks • Nikolsky sign present (extension of a blister resulting in the skin, extending from the primary infection site from lateral pressure to the border of an intact blister) toward regional lymph nodes. Streaks may tender and • Diagnosis: frozen section tissue analysis to exclude toxic warm. Due to inhalation of anthrax spores > nonspecific • Aerobes: (usually gram-negative organisms), ampicilsymptoms: low-grade fever and a nonproductive cough. Usually fatal • Intravenous immunoglobulin • Chest x-ray: widened mediastinum with hemorrhagic pleural effusions Actinomycosis • Gastrointestinal anthrax: due to ingestion of infected meat • Caused by Actinomyces israelii, a filamentous, anaerobic, products. Mainly affects the cecum gram-positive bacteria • Cutaneous anthrax: occurs 1 to 7 days after skin exposure > • Cutaneous disease includes cervicofacial disease (lumpy “Malignant pustule”: central area of coagulation necrosis jaw) or cutaneous mycetoma (Maduromycosis) (ulcer with eventual eschar), edema, and vesicles filled • Clinical: with bloody or clear fluid (actually not pustular) > • Cervicofacial—abscess with draining sinus, usually at ruptures to leave a black eschar and scar. Perivascular inflammation • Vector: human body louse (Pediculus humanus corporis) with thrombi and extravasation of red blood cells • Humans are the natural reservoir • Giemsa stain of tissue: small coccobacillary intracel• Incubation period of 7 to 14 days lular bacteria • Clinical • Treatment: (self-limited disease); doxycycline or chloram• Fever, headache phenicol, quinolones • Maculopapular rash occurs on days 4 to 7 • Begins on the axilla and trunk and spreads peripherally BoutonneuSe Fever (meDiterranean Fever) • Can become hemorrhagic with necrosis • Causative agent is R. Fitzpatrick’s proctocolitis, results in scarring/chronic lymphatic Dermatology in General Medicine, 8th Ed. You note 15 mm induration at the test (application of heat) site, to which the patient eagerly informs you that he • M. Initiate 4 drug therapy for 6 months – Skin disease rare: plaques, nodules, ulcers B. A Hansen patient has many patches and plaques, no leprosy) anesthesia, and normal facies. Six months after starting – No satisfactory antimicrobial treatment, often treatment with clofazamine, rifampin, and dapsone, she utilize surgery and grafting in treatment reports increased erythema of her existing lesions and – Strict growth limited to fatty tissue beneath the pain in her extremities and visual disturbances. Intracytoplasmic inclusions within endothelial cells may Part A—Match the following diseases with their correspondbe seen in: ing virulence factors: A. Trimethoprim-sulfamethoxizole Part B—Match the following diseases with the corresponding B. None of the above be preferable to use it as a screening test in this population. This patient has borderline leprosy and is experiencWood’s lamp shows coral red fluorescence. Pseudomonas aeruginosa ity due to syphilitic bone involvement and is a feature of early (< 2 years old) congenital syphilis. A gravely ill patient originally complaining of severe saddle-nose deformity, Higomenaki sign, and 8th cranial headache develops an eruption with metallic-grey irregnerve deafness are classic for late congenital syphilis. Doxycycline and can show bacteria within vacuoles of histiocytes 354 chapter 18 Bacterial DiSeaSeS (Mikulicz cells). Fitzpatrick’s Color Atlas & Synopsis of Clinical • Scaly, erythematous, annular plaques, with active borDermatology, 5th Ed. Fitzpatrick’s Color Atlas & Synopsis of Clinical gen earlier and at a lower concentration than other tests Dermatology, 5th Ed. New York: McGraw-Hill; 2005, • Histology: infections may be gelatinous with numerous p. Which fungus produces characteristic barrel-shaped • Caused by Prototheca wickerhamii, an achloric algae presarthroconidia that are easily aerosolized, and of particuent in stagnant water lar exposure-danger to laboratory workersfi Coccidiomycosis immitis contaminated soil); importantly, person-to-person transC. Paracoccidioides brasiliensis • the organism is of low virulence; most persons infected E. Sporothrix schenckii are immunosuppressed; use of glucocorticoids (in any form) is a risk factor for protothecosis 3. What dermatophyte is a common cause of tinea cruris, • Clinical: but does not cause tinea capitisfi Trichophyton tonsurans confined to the site of inoculation; disseminated disease and even fatal disease can occur in the 4. Which organism may cause extracellular asteroid bodies, immunocompromised with yeast surrounded by brightly eosinophilic spiculesfi Blastomyces dermatitidis • Tenosynovitis was reported from a contaminated scleB. Paracoccidioides brasiliensis • Histology: the diagnostic finding is a classic “morula” E. Sporothrix schenckii that consists of a specialized sporangia with a central endospore surrounded by a corona of molded endo5. A biopsy demonstrates interconnected, lemon-shaped spores forming a soccer ballor berry-like shape yeast, of about 9 fim in diameter, which are enmeshed • Culture: the species grows readily upon Sabouraud within thickened collagen bundles. Piedraia hortae • Surgical intervention is utilized, often in combination with pharmacologic therapy 6. A healthy patient presents with a macular pigmented • Successful treatment of localized disease has been lesion upon the sole. Which of the following is not a dimorphic fungal ents with skin lesions, a persistent fever, and a positive organismfi Which of the following drugs is most often employed to the Lower Sonoran Desert life zone of the southwestern treat fusarium infectionsfi Voriconazole mon cause of tinea capitis in the United States is Trichophyton tonsurans. Sporothrix schenckii is a dimorphic organism, which cells or Medlar bodies in tissuefi Lacazia loboi is the causative species of lobomycosis, a rare fungal infection common to the Amazon 10. Trichophyton concentricum tion ultimately yields a cytokine milieu that creates slowC. Trichophyton tonsurans Phaeoannellomyces werneckii) is a dematiaceous (melanin-producing) yeast with remarkable halotol11.

Intravenous fuids should not be given unless the • High temperature child is lethargic or unconscious and shocked acne on neck buy cheap benzac 20gr online. A suggested • Severe anaemia regimen for fuid resuscitation for a child with malnutrition and acute dehydration is shown in box 8 acne yahoo answers buy cheap benzac 20 gr. Final In severe cases of cerebral malaria they may also present with: Report skin care addiction discount benzac on line, February 1998 acne pads buy benzac us. Performance of Health Workers in the Management • Treat hypoglycaemia of Seriously Sick Children at a Kenyan Tertiary Hospital: Before and after a Training Intervention. Implementing • Treat using local anti-malarial guidelines ensuring accurate locally appropriate guidelines and training to improve care of dosing serious illness in Kenyan hospitals: a story of scaling-up (and down and left and right). Normal ranges of heart rate and respiratory rate in children from birth to 18 years of age: a systematic review of • Give cautious fuids if there is impaired perfusion or shock, observational studies. The global burden of disease: comprehensive will cause harm: response to ‘mortality after fuid bolus in assessment of mortality and disability from diseases, injuries African children with severe infection’. Predictors of correct treatment of children with fever seen at Oxford: Oxford University Press, 2003. Guidelines for care at the of health worker treatment practices for uncomplicated frst-referral level in developing countries. Use of antimalarial drugs for children’s fevers in immunization/topics/malaria/en/ (accessed 3rd October district medical units, drug shops and homes in eastern 2014). Lancet; 2004; b364: in patients with severe febrile illness in Tanzania: a prospective 1896-98. The doctor makes a presumptive diagnosis of meningococcal disease and gives her intramuscular penicillin and refers her to hospital by ambulance. She receives appropriate resuscitation and Summary emergency treatment in the emergency department and is transferred to the Intensive Care Unit. She develops multiple Both meningitis and organ failure and requires inotropes and ventilation. Three fngers on her left hand become necrotic meningococcal septicaemia and require amputation. Prompt diagnosis A ffteen-year-old boy presents to hospital with fever, vomiting and lethargy. Over the next few hours Mortality of those reaching he becomes irritable and drowsy. After a blood culture is taken, he is started on ceftriaxone and hospital remains 5-10% with intravenous fuids. The frst case (the “meningitis belt”) epidemics occur every 5-10 is an example of meningococcal septicaemia whilst years with rates of 500 cases/100 000 population/ case 2 is an example of meningococcal meningitis. It is vital that serogroups A, B and C, are responsible for the majority all doctors that may treat sick children have a good of cases. Serogroup W-135 has been particularly understanding of how to diagnose and treat this associated with pilgrims attending the Haj religious condition, as it occurs worldwide and is currently festival in Saudia Arabia. The disease is characterised the leading infective cause of death in children in the by local clusters or outbreaks and there is a winter developed world. The factors Neisseria meningitidis (meningococcus) is a capsulated associated with pathogenicity are not well understood gram-negative diplococcus. Risk factors include: serogroups based on the polysaccharide that makes up • Age (<1 year of age) their capsule. They can be further serotyped and • Overcrowding subtyped based on proteins in the outer membrane of the bacterium. Purifed polysaccharide vaccines have been developed interstitial space and hypovolaemia) and pathological vasospasm against serogroups A, C, Y and W-135, but they are poorly and vasodilatation. Tese vaccines may be useful for controlling outbreaks and epidemics, but are not suitable for use as part of a primary vaccination • Generalised endothelial injury activates procoagulant pathways. A conjugated group C vaccine has been developed where • Anticoagulant pathways (protein-C and fbrinolytic) are downthe polysaccharide antigen is conjugated to a carrier protein. It clinical FeatUreS as already led to a decrease in the number of confrmed cases in these Patients who present early may have very non-specifc symptoms countries. The disease may progress very rapidly, so a high index of suspicion needs to be maintained if the diagnosis is to be made pathophySioloGy early enough for treatment to be efective. The classical feature of the Development of the disease involves: disease is a petechial or purpuric rash (purple rash, which does not • Colonisation of the nasopharynx fade on pressure), but up to 20% of cases may have no rash or an atypical maculopapular rash. Symptoms of meningitis include: Both innate and acquired immune mechanisms are responsible for • Headache host protection. The resultant disease process may be focal infection (normally meningitis), septicaemia or both. About 60% of cases in • Fever Europe have evidence of meningitis and septicaemia, while about • Vomiting 20% have meningitis only and 20% septicaemia only. Endotoxin and other bacterial factors cause a host response that results in • Photophobia much of the damage. In infants, particularly, • Raised intracranial pressure the features can be very non-specifc; they frequently present with only: • Recent or prolonged seizures • Irritability • Cardiorespiratory compromise • Refusal to eat • Coagulopathy • Drowsiness • Infection at the site. If a positive microbiological diagnosis can be made from a skin Death is usually caused by refractory raised intracranial pressure. Unless contra-indication exists, patients with suspected meningitis Typically the rash spreads rapidly and can lead to widespread necrosis should have a lumbar puncture, but it should be done promptly and and gangrene of skin and underlying tissues. The rash is a visible should not delay giving the antibiotics by more than thirty minutes. It should only be used to exclude other diaGnoSiS causes for focal neurological signs or to investigate complications of Because of the need for immediate treatment once the disease meningitis. They may also ofer false reassurance since in fulminant treatment infections the white cell count, C-reactive protein and lumbar puncture may all be normal early in the disease. The initial initial assessment and resuscitation diagnosis is based on clinical history and examination. In hospital, assessment and cultures are more likely to be positive if taken before antibiotics are resuscitation of vital functions should occur together, with given. Priorities are: Tere have been a number of reports suggesting that major morbidity 1. Some experts believe that too few lumbar punctures • All patients should receive a high concentration of inspired are done and this remains a controversial area. Treat • Shock is recognised by the presence of an increased heart rate and shock aggressively if present. The patient should be immediately) and a decreased level of consciousness examined for the typical rash but this may not always be present. As soon as intravenous access is obtained, take blood for culture, biochemical (including • Activity against meningococci that are less sensitive to penicillin glucose) and haematological tests, and give antibiotics (see (due to a diferent penicillin binding protein) or resistant to later). Determine whether major neurological compromise exists: even if the cause for them is not immediately obvious. Other complications that may need treatment include: Patients with meningitis rather than septicaemia may develop raised intracranial pressure. This is particularly common, causes major morbidity if unrecognised, and is easy to treat. Determine the • Fluctuating or decreasing level of consciousness blood glucose when intravenous access is frst obtained • Unequal, dilated or poorly reacting pupils • Hypokalaemia • Focal neurological signs • Hypomagnesaemia • Abnormal posturing • Seizures • Hypocalcaema • Hypertension accompanied by tachycardia or bradycardia • Anaemia • Papilloedema is sometimes seen. As a result, many paediatric intensivists give a femoral line may be inserted as it is associated with less morbidity hydrocortisone in a replacement dose (1mg. The use of inotropes/vasopressors should be guided by clinical coagulopathy assessment and markers of ‘global metabolic status’: Deranged clotting is commonly seen as part of the septic process and blood products are often required to correct this. Long-term problems related to renal or myocardial function are less Choice of vasoactive drug should be guided by the clinical picture common. The haemodynamic picture Patients remain infectious for 24 hours after receiving a cephalosporin can change frequently during the frst 48 hours and high doses and should be isolated during this period. Widespread thrombosis and haemorrhagic necrosis of the skin and underlying tissues is called • If infection is due to serogroup A, W-135 or Y, contacts should “purpura fulminans”. When the thrombosis involves large vessels, also receive the quadrivalent conjugate vaccine. The combination of Further information on meningitis and meningococcal disease is ischaemia, necrosis and oedema can cause compartment syndrome. It has been suggested that fasciotomies are only indicated in the frst 24 hours after reFerenceS onset of purpura fulminans and only for compartment syndrome Law R.

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