Lesson 2

Lesson 2

Scarlet fever. Pseudotuberculosis

 

Scarlet nFever.

This disease is the result of infection by streptococci that elaborate none of three pyrogenic (erythrogenic) exotoxins.

Β-hemolytic streptococcus group A

 

The incubation period ranges from 1–7 days, with an average of 3 days. nThe onset is acute and is characterized by fever, vomiting, headache, toxicity, npharyngitis, and chills. Abdominal pain may be present; when this is associated nwith vomiting prior to the appearance of the rash, an abdominal surgical ncondition may be suggested. Within 12–48 hr the typical rash appears.

Generally, temperature increases abruptly and may npeak at 39.6–40º C (103–104º F) on the 2nd day and gradually returns nto normal within 5–7 days in the untreated patient; it is usually normal withi12–24 hr after initiation of penicillin therapy. The tonsils are hyperemic and nedematous and may be covered with a gray-white exudate.

Group A streptococcal pharyngitis with ninflammation of the tonsils and uvula.

 

The pharynx is inflamed and covered by a membrane nin severe cases. The tongue may be edematous and reddened. During the early ndays of illness the dorsum of the tongue has a white coat through which the red nand edematous papillae project (i.e., white strawberry tongue). After several ndays the white coat desquamates; the red tongue studded with prominent papillae npersists (i.e., red strawberry tongue, raspberry tongue). The palate and uvula nmay be edematous, reddened, and covered with petechiae.

This is the ntypical “white strawberry tongue” of scarlet fever,

with a white ncoating with red dots on the surface.

 

Red tongue nwith red dots (red strawberry tongue)

 

Note inflammation of the oropharynx with npetechiae on the soft palate, small red spots caused by group A streptococcal npharyngitis.

 

 

The exanthem is red, is punctate or finely npapular, and blanches on pressure. In some individuals, it may be palpated more nreadily than it is seen, having the texture of gooseflesh or coarse sandpaper. nThe rash appears initially in the axillae, groin, and neck but within 24 hr nbecomes generalized. Punctate lesions generally are not present on the face. nThe forehead and cheeks appear flushed, and the area around the mouth is pale n(i.e., circumoral pallor). The rash is most intense in the axillae and groiand at pressure sites. Petechiae may occur owing to capillary fragility. Areas nof hyperpigmentation that do not blanch with pressure may appear in the deep ncreases, particularly in the antecubital fossae (i.e., pastia lines). In severe ndisease, small vesicular lesions (miliary sudamina) may appear over the nabdomen, hands, and feet.

 

Typical nrashes in scarlet fever

 

Flushed face, ncircumoral pallor (Filatov’s sign)

      

Pastia lines nin skin folds

 

 

Miliary nsudamina and “sand paper” or goose skin (shagreen) sign

 

 

In people nwith very dark skin, the inflammation

and redness nof exanthem is more subtle,

but the rash nis easily seen and felt by the touch.

 

Desquamation begins on the face in fine flakes toward the end of the 1st nwk and proceeds over the trunk and finally to the hands and feet. The duratioand extent of desquamation vary with the intensity of the rash; it may continue nfor as long as 6 wk.

Desquamatioon the skin (fine flakes)

 

 

Desquamatioon the palms and soles (macrolamellar)

 

 

 

This image ndisplays the previously pink, sandpaper-like rash of scarlet fever starting to npeel and improve.

 

Scarlet fever may follow infection of wounds (i.e., surgical scarlet nfever), burns, or streptococcal skin infection. Clinical manifestations are nsimilar to those just described, but the tonsils and pharynx generally are not ninvolved. A similar picture may be observed with certain strains of staphylococci nthat produce an exfoliative toxin.

Scarlet fever must be differentiated from other nexanthematous diseases, including measles (characterized by its prodrome of nconjunctivitis, photophobia, dry cough, and Koplik spots), rubella (disease is nmild, postauricular lymphadenopathy usually is present, and throat culture is nnegative), and other viral exanthems. Patients with infectious mononucleosis, nhave pharyngitis, rash, lymphadenopathy, and splenomegaly as well as atypical nlymphocytes. The exanthems produced by several enteroviruses can be confused nwith scarlet fever, but differentiation can be established by the course of the ndisease, the associated symptoms, and the results of culture. Roseola is ncharacterized by the cessation of fever with the onset of rash and the ntransient nature of the exanthem. Kawasaki disease, drug eruption, and toxic nshock syndrome must also be considered.

 

Septic or severe scarlet fever associated with bacteremia or toxemia may nmanifest high fever and may be complicated by arthritis, jaundice, and hydrops nof the gallbladder. Scarlet fever may be differentiated from Kawasaki disease nby an older age at onset, absence of conjunctival involvement, and recovery of ngroup A streptococci. Streptococcal toxic shock–like syndrome, associated with nthe pyrogenic toxins, produces toxicity, fever, shock, tissue injury n(necrotizing fasciitis, myositis), pneumonia, rash (local or diffuse erythema, nmaculopapular, petechial, desquamation), and multiorgan dysfunction (kidney, nlung, central nervous system). The shock, local tissue injury, older age, and nnonscarlatiniform rash differentiate this syndrome from scarlet fever. nArcanobacterium haemolyticum (formerly Corynebacterium haemolyticum) also produces ntonsillitis, pharyngitis, and a scarlatiniform rash in adolescents and young nadults. Severe sunburn can also be confused with scarlet fever.

DIAGNOSIS. Although 30% nof children with sore throat have a positive throat culture for group A nstreptococci, only 50% of these have a positive antibody response indicative of nactive infection rather than colonization. Streptococcal pharyngitis is nsuggested by age greater than 5 yr, high fever, exudates, tender anterior ncervical lymphadenopathy, scarlatiniform rash, and a history of exposure. nHowever, only 15% of children with pharyngitis and 25% of those with exudates nhave streptococcal infection; 50% of those with streptococcal pharyngitis do nnot have tonsillar exudates. Clinical judgment does not predict which childremay have streptococcal infection, which must be diagnosed by throat culture or nantigen detection.

 

Throat culture is the most useful laboratory aid in reaching a diagnosis nin patients with acute tonsillitis or pharyngitis. Selective media give a higher nyield than sheep blood agar plates. A positive result for a throat culture may nindicate streptococcal pharyngitis, but hemolytic streptococci are commoinhabitants of the nasopharynx in well children. Isolation of a group A nstreptococcus from the pharynx of a child with pharyngeal infection does not nnecessarily indicate that the disease is caused by this organism. Whestreptococci are isolated from children with moderate or severe exudative npharyngitis who have petechiae on the palate and cervical adenitis, the ndiagnosis is more secure. Rapid antigen detection tests are not sufficiently nsensitive to be used without a back-up culture. Treatment is, however, nrecommended for all children with pharyngitis and a positive throat culture or nrapid antigen test for group A streptococci, even though in some cases the nstreptococci represent colonization.

 

The immunologic response of the host after nexposure to streptococcal antigen can be assessed by measuring antistreptolysiO (ASO) titers. An increase in ASO titer to greater than 166 Todd units occurs nin more than 80% of untreated children with streptococcal pharyngitis withithe first 3–6 wk following infection. This response may be modified or nabolished by early and effective antibiotic therapy. ASO titers may be very nhigh in patients with rheumatic fever; in contrast, they are weakly positive or nnot elevated at all in patients with streptococcal pyoderma; responses ipatients with glomerulonephritis are variable. Group A b-hemolytic streptococci nalso may be recovered from the pharynx of asymptomatic individuals who develop nan antibody response to this organism, indicating that subclinical infectiohas occurred.

 

Individuals with impetigo may react strongly to stimulation by other nstreptococcal extracellular products. Anti-DNase (deoxyribonuclease) B provides nthe best serologic test for streptococcal pyoderma; it begins to rise 6–8 wk nafter infection. Most patients with streptococcal pharyngitis also develop nelevated titers to this enzyme. Patients with pyoderma and pharyngitis also may ndevelop antibody responses to hyaluronidase, but antihyaluronidase (AH) titers nare elevated with less regularity than are ASO titers.

 

A 2-min, inexpensive Streptozyme slide test (Wampole Laboratories, nCranbury, NJ) is designed to detect antibodies against multiple streptococcal nextracellular antigens. This test detects more patients with increased antibody ntiters than any other single test presently available. Nonspecific n(false-positive) reactions have been limited iumber, and the test is capable nof detecting antibody responses within 7–10 days of infection. However, the nstrength of the Streptozyme reagent varies from lot to lot, and it may not be nspecific for antibodies to extracellular products of group A streptococci.

The white blood cell count may or may not be nelevated. Because leukocytosis may occur in many bacterial and viral diseases, nthis finding is nonspecific. Similarly, elevations in the erythrocyte nsedimentation rate and C-reactive protein do not help to establish a specific ndiagnosis.

 

DIFFERENTIAL DIAGNOSIS.

 

Acute pharyngitis that is indistinguishable nclinically from that caused by group A b-hemolytic streptococci may be caused nby many viruses, including Epstein-Barr virus (infectious mononucleosis). A nviral cause may be suggested by failure to isolate streptococci and can be nidentified specifically by viral culture and serologic studies. Infectious nmononucleosis may be suggested by the clinical manifestations, the presence of natypical lymphocytes in the peripheral blood, and a rise in heterophil and nEpstein-Barr viral antibody titers. Acute pharyngitis similar to that caused by nb-hemolytic streptococci may occur in patients with diphtheria, tularemia, ntoxoplasmosis, infection with Mycoplasma or A. haemolyticum, and, rarely, iindividuals with tonsillar tuberculosis, salmonellosis, and brucellosis or ninfections caused by Neisseria gonorrhoeae, Neisseria meningitidis, and nYersinia enterocolitica. These diseases can be differentiated by appropriate ncultures and serologic tests.

 

 Streptococcal pyoderma must be ndifferentiated from staphylococcal skin disease. Often these bacterial species ncoexist. The lesions produced are clinically indistinguishable; distinction is nmade only by culture.

Streptococcal septicemia, meningitis, septic arthritis, and pneumonia npresent signs and symptoms similar to those produced by other bacterial norganisms. The offending pathogen can be established only by culture.

Vinsent’s angina need to be differentiated from streptococcal angina in Scarlet nfever

COMPLICATIONS. nComplications generally reflect extension of streptococcal infection from the nnasopharynx. This may result in sinusitis, otitis media, mastoiditis, cervical nadenitis, retropharyngeal or parapharyngeal abscess, or bronchopneumonia. nHematogenous dissemination of streptococci may cause meningitis, osteomyelitis, nor septic arthritis. Nonsuppurative late complications include rheumatic fever nand glomerulonephritis.

 

PREVENTION. nAdministration of penicillin will prevent most cases of streptococcal disease nif the drug is provided prior to the onset of symptoms. Except for rheumatic nfever (see Chapter 175), indications for prophylaxis are not clear. Oral npenicillin G or V (400,000 U/dose) is provided four times each day for 10 days. nAlternatively, 600,000 U of benzathine penicillin in combination with 600,000 U nof aqueous procaine penicillin may be given as a single intramuscular ninjection. This approach should be used for institutional epidemics. Childreexposed to an individual case at school may be observed carefully.

 

Management of carriers of group A b{beta}-hemolytic streptococci is ncontroversial. It has been suggested that treatment of the carrier precludes the ndevelopment of type-specific immunity, thereby leaving the individual nsusceptible to reinfection later in life. It is probably unnecessary to nre-treat asymptomatic convalescent patients with persistently positive throat ncultures for group A streptococci, since they are generally carriers who do not nhave persistent or recurrent streptococcal infections. Children thought to have nrecurrent streptococcal infections may be carriers who have frequent viral nrespiratory infections masquerading as streptococcal infections. Parental nanxiety may be high after several such episodes. Treatment with a nnon-penicillin antibiotic (e.g., cephalosporin, erythromycin, clindamycin) may nbe useful in eradicating the carrier state but should be reserved for the rare nproblem case.

No streptococcal vaccines nare available for clinical use.

TREATMENT. The goals of ntherapy are to decrease symptoms and prevent septic, suppurative, and nnonsuppurative complications. Penicillin is the drug of choice for the ntreatment of streptococcal infections. All strains of group A b{beta}-hemolytic nstreptococci isolated to date have been sensitive to concentrations of npenicillin achievable in vivo.

Blood and tissue levels of penicillin sufficient nto kill streptococci should be maintained for at least 10 days. Children with nstreptococcal pharyngitis should be treated with penicillin (125–250 mg/dose nthree times a day) for 10 days. Penicillin G or penicillin V may be employed; nthe latter is preferable because satisfactory blood levels are achieved even whethe stomach is not empty. A single intramuscular injection of a long-acting nbenzathine penicillin G (600,000 U for children <60 lb and 1,200,000 U for nchildren >60 lb) nmay be more effective for treatment or prevention of relapse and is indicated nfor all noncompliant patients or those having nausea, vomiting, or diarrhea.

Erythromycin (40 mg/kg/24 hr), clindamycin (30 nmg/kg/24 hr), or cefadroxil monohydrate (15 mg/kg/24 hr) may be used for ntreating streptococcal pharyngitis in patients who are allergic to penicillin. nGenerally, relapse rates are lower with regimens other than penicillin. nTetracyclines and sulfonamides should not be used for treatment, although nsulfonamides may be used for prophylaxis of rheumatic fever.

Treatment failure, defined as persistence of nstreptococci after a complete course of penicillin, occurs in 5–20% of childreand is more common with oral than with intramuscular therapy. It may be due to npoor compliance, reinfection, the presence of b{beta}-lactamase–producing oral nflora, tolerant streptococci, or presence of a carrier state. Persistent ncarriage of streptococci predisposes a small number of patients to symptomatic nrelapse. Repeating the throat culture after a course of penicillin therapy is nindicated only in high-risk situations, such as in patients with a history of nprevious rheumatic fever. If the throat culture is again positive for group A nstreptococci, some clinicians recommend a second course of treatment. nPersistence after a second course of antibiotics probably indicates a carrier nstate, which has a low risk for the development of rheumatic fever and does not nrequire further therapy.

Patients with severe scarlet fever, streptococcal nbacteremia, pneumonia, meningitis, deep soft tissue infections, erysipelas, nstreptococcal toxic shock syndrome, or complications of streptococcal npharyngitis should be treated parenterally with penicillin, preferably nintravenously. The dose and duration of therapy must be tailored to the nature nof the disease process, with daily doses as high as 400,000 U/kg/24 hr required nin the most severe infections. Severe, necrotizing infections may require the naddition of a second antibiotic (e.g., clindamycin) to ensure complete nbacterial killing.

 

PROGNOSIS. The nprognosis for adequately treated streptococcal infections is excellent; most nsuppurative complications are prevented or readily treated. When therapy is nprovided promptly, nonsuppurative complications are prevented and complete nrecovery is the rule. In rare instances, particularly ieonates or ichildren whose response to infection is compromised, fulminant pneumonia, nsepticemia, and death may occur despite usually adequate therapy.

 

Short nstatement of the material

Scarlet fever nis an acute infectious disease, that is caused by ngroup A b-hemolytic streptococcus, transmitted by an air-droplet way, ncharacterized by intoxication, rashes on a

skin, ntonsillitis, regional lymphadenitis, and strawberry tongue.

Etiology: ngroup A b-hemolytic streptococcus (GABHS).

Epidemiology:

– source nof infection – ill persoot only with scarlet fever, but other forms nof  GABHS infections (sore throat, nerysipelas, streptodermia).

– infectiois transmitted by inhalation of infected airborne droplets, rare with food nand direct contact.

– susceptible norganism – children 2-9 years old.

 

Pathogenesis nAn entrance gate is the mucus membrane of the throat, seldom damaged skin, and nmaternity ways (at delivery).

Pathogenesis has nthree lines:

1.                     nToxic (toxically damage of ncardiovascular, central nervous, endocrine systems).

2.                     nSeptic – primary inflammation in the nplace of infection (tonsillitis, secondary bacterial complication).

3.                     nAllergic – sensibilization by GABHS nproteins (depression of immunity leads to allergic complications – nephritis, narthritis, myocarditis, rheumatism).

Clinical npresentation: Onset is usually acute and is characterized by a nsore throat (often with dysphagia), fever (often above 39 °C), pharyngeal and npurulent tonsilar exudates. Anterior cervical lymph nodes, particularly the njugular-digastric nodes just beneath the angle of the mandible, are tender and nenlarged. Erythema of the soft palate is common, and an enanthema of “doughnut” nlesions on the soft palate. Strawberry tongue. Other features are nausea and nvomiting, headache, abdominal discomfort. One to two days later the rashes like n“sandpaper” appears, first on the neck and then on the trunk and extremities ntill the end of the day.

The eruptiois characterized by dusky red, blanching tiny papules that have a rough ntexture. Papules are usually absent on the face, palms, and soles, but the face ncharacteristically shows flushing with circumoral pallor. On the body, the nrashes are intensified in skin folds and at sites of pressure. In the nantecubital and axillary fosses, linear petechias are seen with accentuation of nthe erythema (Pastia’s lines).

The exanthema nusually lasts 4 to 5 days and then begins to desquamate, first on the face last non the palms and soles. Pharyngitis usually resolves in 5 to 7 days.

Clinical ndiagnostic criteria:

1. Latent nperiod: a few hours – 7 days.

2. Initial or nprodromal period (from the first signs of illness to rashes appearance):

up to 1-2 ndays

• acute nbeginning;

• toxic nsyndrome, hyperthermia;

• in the nthroat: pain, bright hyperemia, pin-point enanthema,

• catarrhal nregional lymphadenitis (photo).

 

3.        Period of exanthema n(rashes):

а) nPhase of height (1 – 2 days)

• maximal nintoxication, fever up to 39-40 °C;

• ntonsillitis: bright hyperemia of the throat marked off a hard palate (photo 31), npinpoint enanthema, hypertrophied tonsils, catarrhal, lacunar (photo 32, 33), nfollicle (photo 34, 35) or necrotizing (photo 36) tonsillitis;

• regional nlymphadenitis;

• pin-point nrashes for a few hours spread all over the body, intensified on the front and nlateral surface of neck, lateral surfaces of trunk (photo 37), abdomen (photo n38, 39), lumbar region (photo 40, 41), iatural skin folds (photo 42), on the nred background of skin, typical intensified in skin folds with hemorrhagic nelements (Pastia’s lines) (photo 43), a skin is rough (“sand paper” sign), pale nperioral triangle (Filatov’s sign) (photo 44, 45);

• white ndermatographia;

• coated ntongue (photo 46) within 2-3 days clears up (photo 47, 48), on 4th – 5th day nbecomes “strawberry” (photo 49, 50);

• sympatic nphase of “scarlet fever” heart (tones are loud, tachycardia, BP is elevated).

 

 

bright nhyperemia of the throat marked off  a nhard palate

 

pin-point nenanthema

Follicular ntonsillitis

 

Lacunar ntonsillitis

Necrotizing ntonsillitis

Typical nrashes localization

 

pin-point nrashes

 

Typical nrashes localization

 

Typical nrashes localization in skin folds

 

Typical nrashes localization in skin folds

 

Pale nperioral triangle

 

Coated ntongue

 

 

Tongue nbegin to clear up

 

 Strawberry tongue

 

b) nFading phase:

• nnormalization of body temperature till 3rd – 4th day of the disease, decrease nof the toxic syndrome;

• rashes and nredness of the skin from 2nd – 3rd up to 6th day turns pale;

• throat: nenanthema disappears from 2nd – 3rd day, hyperemia turns pale till 6-7 day;

• the sizes nof lymphnodes normalized till 4th – 5th day;

• vagus-phase nof scarlet fever heart (bradycardia, dilation of the cardiac dullness borders, nsystolic murmur on the apex, low BP);

• a tongue nturns pale till 10th – 12th day, with enlarged follicles.

 

4. Period of recovery: nfrom 2nd week (for 10-14 days)

• changes othe skin: flakes-like desquamation all over the body except palms and soles n(where it is larger) (photo 51);

• tongue with nenlarged follicles;

• the nvagus-phase of scarlet fever heart continue for 2-4 weeks;

• rise nsensitivity to the streptococcus infection, possibility of complications.

Classification

1. Form:

a) typical;

b) atypical:

• without nrashes;

• effaced;

• extra npharyngeal (burns, wounds, post-natal, after operations, delivery);

• with naggravated symptoms (hypertoxic, hemorrhagic).

2. Severity:

a) mild;

b) moderate;

c) severe: ntoxic, septic, toxic-septic.

3. Course:

a) smooth;

b) uneve(relapses, complications).

 

Complications

By the ncharacter:

• are ninfectious (streptodermia (photo 50, 52), necrotizing tonsillitis (photo 36), nsecondary tonsillitis (photo 54), peritonsilitis (photo 53) peritonsilar nabscess (photo 55), otitis, purulent lymphadenitis, sepsis);

• and nallergic (rheumatism, myocarditis, arthritis, glomerulonephritis).

By the time nof development:

• early n(first week of the disease);

• late (2nd – n3rd week).

By the netiology:

• specific or nprimary (caused by the same streptococcus);

• secondary n(caused by the other bacteria).

 

Streptodermia

 

Peritonsillitis

 

Peritonsilar abscess

 

Laboratory ntests

1.                     nThe diagnose is confirmed by throat nculture with group A b-hemolytic streptococcus.

2.                     nSerology (antistreptolysin O, nantidesoxyribonuclease B) with their grows in 2 weeks may be useful for ndocumenting recent GABHS infection.

3.                     nThe complete blood cell count is nhelpful: usually white blood cell count higher 12500 cells/mm3, nneutrophyllosis, left shift, eosynophylia, elevated ESR ECG, CBC and urinanalysis non the 10th day after the disease began, and on 21th day for possible late ncomplications diagnostic (nephritis, myocarditis).

Diagnosis nexample:

• Scarlet nfever, typical form, exanthema period, severe (toxic) degree, complicated by nthe right side peritonsilar abscess.

• Scarlet nfever, typical form, recovery period, moderate degree, complicated by the nmyocarditis.

 

Differential ndiagnosis: tonsillitis may be seen with diphtheria, nmononucleosis, adenovirus, and micoplasm; rashes may be seen with measles, nrubella, and pseudotuberculosis.

 

Evidences for nobligatory hospitalization of patients with infectious exanthema

1.                     nThe severe form of disease, wheappears need in undertaking of intensive therapy; patients with moderate forms nat age before 3 years.

2.                     nSick children from families with bad nsocial-home conditions, especially in the event of impossibility of their nisolation to prevent infections transmission.

3.                     nAbsence of conditions for examinatioand treatment at home.

4.                     nSick children from closed children institutions.

Advantages of nthe home treatment

1.                     nPossibility of additional infectioby hospital bacteria is completely excluded.

2.                     nRealization of individual care nprinciple for sick child is more full.

3.                     nAvoiding stressful reactions, which ncould appear in case of hospital treatment.

Treatment ihome conditions is possible

1.                     nIn conditions of isolated flat.

2.                     nIn case of satisfactory material nposition of the parents.

3.                     nIn case of parents desire to organize nindividual care and treatment at home.

Treatment

1. Bed nregime during an acute period.

2. Etiological ntreatment for scarlet fever is:

a. In the nmild case penicillin orally (penicillin V) for 10 days 50,000-100,000 EU/kg/day ndivided in 3-4 doses. Erythromycin (or another macrolides) is alternative nantibiotic (30-50 mg/kg/day). The course of treatment is 10 days.

b. In the nmoderate case penicillin intramuscularly (penicillin G), the same dose as ithe mild case. The course of treatment is 10-14 days.

c. In the nsevere case: cefalosporins of the 1st-2nd generation, klindamycin, vancomyciintravenously for 10-14 days.

3. Detoxicatiotherapy:

a. In the nmild case large amount of oral fluids.

b. In the nmoderate and severe cases – Glucose and saline solutions IV.

4. Antihistamines n(in average doses) – pipolphen, suprastin, claritin, cetirizin.

5. Medicine nwhich strengthens vascular wall (vit. C and PP: ascorutin, galascorbin)

6. Control nof fever (when the temperature is more than 38.5-39 °C); in childrebefore 2 mo and in case of perinatal CNS damage, seizures in the history, nsevere heart diseases – when the temperature is up to 38 °C with acetaminophe(paracetamol 10-15 mg/kg not often than every 4 hours (not more than 5 ntimes per day) or ibuprophen 10 mg/kg per dose, not often than every 6 nhours.

7. Local ntreatment with antiseptic fluids (gurgling), UV-insolation. Patient may go home nfrom infection department not earlier the 10th day of the illness, in 10 days nblood analysis, urinalyses, ECG must be done.

 

Prevention: isolatioof the patient on the 10 days, but he mustn’t visit school until 22 day of the ndisease. Contact person (children before 8 years) must be isolated for 7 days n(period of incubation).

YERSINIA PSEUDOTUBERCULOSIS

 

Infection due to Y. pseudotuberculosis is most often seen as a npseudoappendicitis syndrome without diarrhea.

 

ETIOLOGY. Y. npseudotuberculosis is differentiated biochemically from Y. enterocolitica othe basis of ornithine decarboxylase activity, fermentation of sucrose, nsorbitol, cellobiose, and other tests, although some overlap between species nmay be seen. Antisera to somatic O antigens and sensitivity to yersinial phages nmay also be used to differentiate the two species. Subspecies-specific DNA nsequences have been isolated that allow direct probe- and primer-specific ndifferentiation of Y. pestis, Y. pseudotuberculosis, and Y. enterocolitica.

 

EPIDEMIOLOGY. Less is known of the nepidemiology of Y. pseudotuberculosis infections than for Y. enterocolitica or nY. pestis. The seasonal incidence in humans parallels that in wild and domestic nanimals. Transmission from cats and cat-contaminated substances is established. nThere is a low reported incidence in the 5–12-yr age range.

 

PATHOLOGY AND PATHOGENESIS. nThe pathology is similar to that described for Y. enterocolitica, with ileal nand colonic mucosal ulceration and mesenteric adenitis. Necrotizing, nepithelioid granulomas are seen in the mesenteric nodes. The appendix is nfrequently grossly and microscopically normal. Mesenteric nodes are frequently nthe only source of positive cultures. Y. pseudotuberculosis antigens bind ndirectly to HLA class II molecules and function as superantigens, which may npartly explain the clinical syndromes resembling Kawasaki syndrome caused by nthis organism.

 

CLINICAL MANIFESTATIONS. nChildren usually present with fever and abdominal pain that is diffuse or nlocalized to the right lower quadrant. Frequently, there is tenderness over the nMcBurney point and strong clinical suspicion of appendicitis. At surgery, the nterminal ileum is thickened and shiny with enlarged mesenteric nodes, which may nappear necrotic. The appendix is normal or only mildy inflammed.

 

 

DIAGNOSIS. Mesenteric adenitis should be nsuspected in children with unexplained fever and abdominal pain. A characteristic npicture of enlarged mesenteric lymph nodes, thickening of the terminal ileum, nand no image of the appendix may appear on ultrasound. Y. pseudotuberculosis is nrarely isolated. It is almost never isolated from stools, and the best source nis an involved mesenteric node. Culture conditions are the same as for Y. nenterocolitica.

 

Serologic tests have been described, but ncommercially available tests or standardized antigens are not available.

 

DIFFERENTIAL DIAGNOSIS. Appendicitis nis the most common diagnosis. Inflammatory bowel disease and nonspecific nintra-abdominal infections are also considered.

 

PREVENTION. Specific preventive measures nother than avoiding exposure to potentially infected animals and careful nfood-handling practices are not apparent. Vaccines for prevention of Y. npseudotuberculosis have not been developed.

 

TREATMENT. nUncomplicated mesenteric adenitis due to Y. pseudotuberculosis is a nself-limited disease, and antimicrobial therapy is not required. Culture-confirmed nbacteremia should be treated with an aminoglycoside in combination with another nagent, as for infections due to Y. enterocolitica.

 

Short statement of the material

Pseudotuberculosis nis an acute infectious disease that is characterized nby the expressed polymorphism of clinical symptoms with predominance of ntoxic-allergic syndrome, rashes like in scarlet fever, the damage of ngastro-intestinal tract, liver; quite often has relapsed motion.

 

Etiology: Yersinia npseudotuberculosis, gram-negative bacillus

 

Epidemiology:

• Source nof infection-wild and home animals (rats, dogs, foxes, cats and other);

• Way of ntransmitting – alimentary;

• Susceptible norganism – children (not infants), adults.

 

Pathogenesis:

1. Entering nthe bacilli to gastrointestinal tract. An entrance gate is a thin bowel n(terminal department and appendix)

2. Enteral nphase: invasion of bacteria in enterocytes, development of local ninflammation, diarrhea, enterotoxin secretion.

3. Regional lymphadenitis n(regional infection).

4. Generalizatio(bacteriemia, toxemia) in severe cases.

5. Parenhymatous nphase: hematogenous distribution of bacteria with forming of the secondary nfocus (lungs, liver, spleen, bones).

6. nImmunological response, recovering from disease.

7. May be secondary nbacteriemia (exacerbations and relapses), because of possible persistency nin lymph nodes.

 

Clinical ncriteria

Incubatioperiod is 3-18 days. Beginning is acute with high temperature, intoxication.

• Polymorphism nof complaints: malaise, fatigue, headache, sleepless, anorexia, narthralgias, muscle pain, sore throat, nausea, abdominal pain, dyspepsia.

• Rashes: nmaculopapulous (like in scarlet fever), may be erythematosus or eveerythema nodosum may developed (photo 56a, 56b);

– The neruption is characterized by dusky red, tiny papules;

– The rashes nare present on face, intensified periorbitally, on the neck (“glasses” symptom, n“hood” symptom (photo 56));

– On the body nthe rashes are intensified in skin folds (photo 57), at the sites of pressure n(red dermographysm), on the hands, feet, (“gloves”,”socks” symptom), round the njoints;

– The nexanthema usually lasts 4 to 5 days and then begins to desquamate, first on the nface, other parts of the body (photo 58) and last on the palms and soles (photo n59, 60).

• Pharyngeal nand tonsilar erythema without the exudates, erythema of the soft palate, nconjunctivitis, corryza demonstrate catarrhal syndrome.

• “Strawberry” ntongue also simulates the scarlet fever (photo 61).

• Abdominal nsyndrome; tenderness during the palpation of abdomen, may be acute nappendicitis.

• Dyspepsia: nnausea, vomiting, liquid feces.

• Hepatomegaly, nrare – splenomegaly, lymphadenopathy.

• Arthritis nof knees (photo 62), elbows, foot and hand small joints or arthralgia.

• Hepatitis nwith or without the jaundice.

• Toxic nmyocarditis.

• Toxic nnephritis, pyelonephritis.

• Bronchitis nor pneumonia may also develop.

 

”hood” symptom

 

Rashes are intensified in skifolds (Pastia lines)

 

“socks” symptom

 

Skin desquamation

 

Desquamation on palms

 

Desquamation on palms

 

Strawberry tongue

 

Arthritis

 

Nodular erythema in case of relapsed course

 

Clinical forms classification

n

Type	Severity	Course
Typical forms Like Scarlet fever Abdominal Arthralgic Icteric (jaundice) Combined Generalized (septic) Atypical  forms Catarrhal Effaced Subclinical	Mild Moderate Severe Indexes of severity: Meningoencephalitic  syndrome Hemorrhagic syndrome Considerable damage of liver Abdominal syndrome Damage of joints Signs of process generalization	Smooth  Uneven with exacerbations and relapses Uneven with complications

Diagnosis nexample:

Pseudotuberculosis, ntypical combined (Scarlet fever like + arthralgic) form, moderate severity, nuneven prolonged duration with exacerbation.

 

Features of npseudotuberculosis at the children of early age

• high and nprolong fever;

• expressed nhepatolienal syndrome;

• systemic nincrease of lymphnodes;

• dyspeptic nsyndrome;

• damage of respiratory ntract, development of pneumonia (very often);

• rarely: nscarlatina rashes, damage of joints;

• prolong, nundulating duration with exacerbation and relapses;

• frequent ncomplications.

Laboratory nfindings

· Complete nblood analyses: leucocytosis, neutrophilia with left shift, eosynophilia, ERS nis enlarged.

· Bacteriological: nYersinia Pseudotuberculosis may be found in feces, urine, blood and mucus.

· Serologically: nincreasing of special antibodies 4 times and more in paired sera (AR, IHAR with ndiagnostic titles 1:200 and more).

· Immune-enzyme nanalysis (ELISA test): Specific antibodies Ig M are positive in an acute phase nof the disease.

 

Differential ndiagnosis should be performed among scarlet fever, measles, nviral hepatitis, typhoid fever, paratyphoid fever, sepsis, enterovirus ninfection, bacterial diarrhea.

 

 

Treatment

Children with nmild form of pseudotuberculosis may be treated at home symptomatically without the netiological medicine.

Hospitalizatiois obvious for:

• Childrewith moderate form of pseudotuberculosis;

• Childrewith severe form of pseudotuberculosis.

Regimen

• half-bed nregimen in mild cases;

• bed regimein moderate cases;

• straight bed nregimen in severe cases.

Diet:

• Icteric n(jaundice) form – N 5;

• Abdominal n(intestinal) form – N 4;

• Other forms n– N 15.

 

Etiological:

• Chloramphenicol n(orally) 10-15 mg/kg 3 or 4 times per day during all period of pyrexia

and plus 3 ndays (average duration is 14 days).

• Alternative nantibiotics (Reserve): cefalosporins of the 3rd or 4th generation (IM, nIV),

• In severe ncases combination together with aminoglycosides of the 3rd generatio(IM, IV).

Pathogenetical:

• Disintoxication: noral with large amount of alkaline fluids (in mild cases), or parenteral with nglucose-saline solutions (in moderate, severe cases);

• nGlucocorticoids 1-3 mg/kg (in equivalent to prednisolone) as a short course 3-5 ndays (in severe cases), for 2-4 wks in case of myocarditis;

• Control nof fever and myalgia (when the temperature is more than 38.5-39 °C); in childrebefore 2 mo and in case of perinatal CNS damage, seizures in the history, nsevere heart diseases – when the temperature is up to 38 °C with acetaminophe(paracetamol 10-15 mg/kg not often than every 4 hours (not more than 5 ntimes per day) or ibuprophen 10 mg/kg per dose, not often than every 6 nhours;

• Antihistamines n(in average doses) – pipolphen, suprastin, claritin, cetirizin;

• NSAIDs icase of arthritis, carditis, nodular erythema (ibuprophen, aspirin, voltaren,

indomethaciin average doses).

 

Prophylaxis:

1.                     nDeratization, disinfection.

2.                     nRight keeping of products.

3.                     nLooking after people from the nepidemic focus for 18 days with bacteriological investigation.

 

Kawasaki disease

Kawasaki disease (KD), also known as Kawasaki nsyndrome, lymph node syndrome and mucocutaneous lymph node nsyndrome, is an autoimmune ndisease in which the medium-sized blood vessels throughout the body become ninflamed. It is largely seen in children under five years of age. It affects nmany organ systems, mainly those including the blood vessels, skin, mucous membranes and lymph nodes; however, its nrare but most serious effect is on the heart where it can cause nfatal coronary nartery aneurysms in untreated children. Without treatment, mortality nmay approach 1%, usually within six weeks of onset. With treatment, the nmortality rate is less than 0.01% in the U.S. There is often a pre-existing nviral infection that may play a role in its pathogenesis. The conjunctivae and oral mucosa, along with nthe epidermis (skin), become erythematous (red and inflamed). Edema is often seen in the nhands and feet and one or both of the cervical lymph nodes are noften enlarged. Also, a remittent fever, often 40°C (104°F) or higher, is ncharacteristic of the acute phase of the disease. In untreated children, the nfebrile period lasts on average approximately 10 days, but may range from five nto 25 days. The disorder was first described in 1967 by Dr. Tomisaku Kawasaki nin Japan

Epidemiology

Kawasaki disease affects boys more than girls with people nof Asian ethnicity, particularly Japanese and Korean people are most nsusceptible as well as people of Afro-Caribbean ethnicity. The disease was rare nin Caucasians until the last few decades and incidence rate fluctuates from ncountry to country.

Currently, Kawasaki disease is the most commonly ndiagnosed pediatric vasculitis in the world. By far the highest incidence of nKawasaki disease occurs in Japan, nwith the most recent study placing the attack rate at 218.6 per 100,000 nchildren <5 years of age (~1 in 450 children). At this present attack rate, nmore than 1 in 150 children in Japan will develop Kawasaki disease during their nlifetime.

However, its incidence in the United States is nincreasing. Kawasaki disease is predominantly a disease of young children, with n80% of patients younger than five years of age. Approximately 2,000-4,000 cases nare identified in the United States each year.

In the United Kingdom, estimates nof incidence rate vary because of the rarity of Kawasaki disease. However, nKawasaki disease is believed to affect fewer than 1 in every 25,000 people.^[54] nIncidence of the disease doubled from 1991 to 2000 however, with 4 cases in per n100,000 children in 1991 compared with a rise of 8 cases per 100,000 in 2000.

History

The disease was first reported by Dr. Tomisaku Kawasaki nin a four-year-old child with a rash and fever at the Red Cross Hospital in Tokyo, Japan in January of 1961, and later published na report on 50 similar cases. Later Yamamoto and colleagues were persuade that nthere is definite cardiac involvement when they studied and reported 23 cases, nof which 11(48%) patients had abnormalities detected by an electrocardiogram. It was nnot until 1974 that the first description of this disorder was published in the nEnglish language literature. in the year 1976 Melish et al., described the same nillness in 16 children in Hawaii. Melish and Kawasaki had independently ndeveloped the same diagnostic criteria for the disorder, which are still used ntoday to make the diagnosis of classic KS.

A question was raised whether the disease only started during the period nbetween 1960 and 1970, but later a preserved heart of a 7 year old boy died in 1870 was examined and nshowed three aneurysms of the coronary arteries with clots, as well as npathologic changes consistent with KS. KS is now recognized worldwide. In the nUnited States and other developed nations, it appears to have replaced acute nrheumatic fever as the most common cause of acquired heart disease in children.

Classification

Systemic vasculitis is ainflammatory condition affecting both veins and arteries throughout the nbody, and is usually caused by a proliferation of cells associated with an immune response to a pathogen, or autoimmunity. nSystemic vasculitides may be classified according to the type of cells involved nin the proliferation, as well as the specific type of tissue damage occurring nwithin the vein or arterial walls. Under this classification scheme for nsystemic vasculitis, Kawasaki disease is considered to be a necrotizing vasculitis n(also called necrotizing angeititis), which may be identified histologically by the noccurrence of necrosis n(tissue death), fibrosis, and nproliferation of cells associated with inflammation in the inner layer of the nvascular wall. Other diseases featuring necrotizing vasculitis include Polyarteritis nodosa, Wegener’s granulomatosis, Henoch-Schönlein purpura nand Churg-Strauss nsyndrome. Kawasaki disease may be further classified as a nmedium-sized-vessel vasculitis, affecting medium and small sized blood vessels, nsuch as the smaller cutaneous vasculature (veins and arteries in the skin) that nrange from 50 to 100µm nin diameter. KD is also considered to be a primary childhood vasculitis, a ndisorder associated with vasculitis that mainly affects children under the age nof 18. A recent, consensus-based evaluation of vasculitides occurring primarily nin children resulted in a classification scheme for these disorders, to both ndistinguish them and suggest a more concrete set of diagnostic criteria for neach. Within this classification of childhood vasculitides, Kawasaki disease nis, again, a predominantly medium-sized vessel vasculitis.

It is also an autoimmune form of vasculitis, and is not nassociated with ANCA antibodies, unlike other vasculitic disorders associated nwith them, such as wegener’s ngranulomatosis, microscopic polyangiitis, nand Churg-Strauss nsyndrome. This categorization is considered essential for nappropriate treatment.

Signs nand symptoms

 

(A) Bilateral, non-exudative conjunctivitis with nperilimbal sparing – “conjunctival injection”.

(B) Strawberry ntongue and bright red, swollen lips with vertical cracking and nbleeding.

(C) Erythematous nrash involving perineum.

(D) Erythema of the palms, which is often accompanied by painful, brawny nedema of the dorsa of the hands.

 (E) Erythema of the soles, and nswelling dorsa of the feet. (F) Desquamation of the fingers.

(G) Erythema and induration at the site of a previous vaccination with Bacillus Calmette-Guérin n(BCG).

(H) Perianal erythematous desquamation.

A child nshowing characteristic “strawberry tongue” seen in Kawasaki disease

 

 

Kawasaki disease often begins with a high and persistent fever that is not very nresponsive to normal treatment with paracetamol n(acetaminophen) or ibuprofen. nit is the most prominent symptom in Kawasaki disease, which is a characteristic nsign of the acute phase of the disease, is normally high (above 39-40º C), nremittent and followed by nextreme irritability  recent reports says it is even present ipatients with atypical or incomplete KD, nevertheless recent reports says it is nnot present on 100% of cases. The first day of fever is considered the first nday of illness, and the duration of fever is on average one to two weeks; ithe absence of treatment, it may extend for three to four weeks, Prolonged nfever is associated with higher incidence of cardiac involvement. It responds npartially to antipyretic drugs and does not cease with the introduction of nantibiotics. However, when appropriate therapy is started – intravenous immunoglobulin n(IVIG) and aspirin – the fever is ngone after two days.

Bilateral conjunctival injection was nreported by many publications to be the most common symptom after fever,  it typically involves the bulbar conjunctivae, nis not accompanied by suppuration, it is not painful. It usually begins shortly nafter the onset of fever during the acute stage of the disease.  Anterior uveitis may be present on slit-lamp nexamination. iritis ncan occur too.

 

Inflammation of the mucous nmembranes in the mouth, along with erythema (redness), edema (swelling) with nfissures (cracks in the lip surface), desquamation (peeling) and nexsudation of the lips nare also evident.

The oropharynx nmucosa has enanthema and the tongue maintains an unusual red appearance termed n”strawberry ntongue” (marked erythema with prominent gustative papillae).

Keratic precipitates (detectable by a slit lamp but usually too nsmall to be seen by the unaided eye), and swollen lymph nodes may also be npresent and can be the first manifestation of the disease.

Rashes noccur early in the disease, and the cutaneous rash observed in patients with KD nis non-specific, polymorphic, non-itchy and normally nobserved up to the fifth day of fever. Cutaneous exanthema may comprise macular-papular nerythematous and fissure lesions, the most common type, in addition to urticariform type rash, npurpuric, multiform-like nerythema. and peeling of the skin in the genital area, hands, and nfeet (especially around the nails and on the palms and soles) may occur ilater phases.

 

 

Some of these symptoms may come and go during the course of the illness. It nis a syndrome affecting multiple organ systems, and in the acute stage of KD, nsystemic inflammatory changes are evident in many organs.

 Myocarditis, pericarditis, valvulitis, aseptic meningitis, pneumonitis, nlymphadenitis, and hepatitis nmay be present and are manifested by the presence of inflammatory cells in the naffected tissues.  If left untreated, nsome symptoms will eventually relent, but coronary artery aneurysms will not nimprove, resulting in a significant risk of death or disability due to myocardial infarction n(heart attack).  If treated in a timely nfashion, this risk can be mostly avoided and the course of illness cut short.

 

 

Less commomanifestations

·  nHigh-grade fever (greater tha39 °C or 102 °F; often as high as 40 °C or 104 °F), The nduration of fever is on average one to two weeks; in the absence of treatment, nit may extend for three to four weeks. However, when appropriate therapy is nstarted the fever is gone after two days.

·  nRed eyes (conjunctivitis) bilateral nwithout pus or drainage, also known as “conjunctival injection”.

·  nAnterior uveitis.

·  nBright red, chapped, or cracked lips.

·  nRed mucous membranes in the nmouth.

·  nStrawberry tongue, white ncoating on the tongue or prominent red bumps (papillae) non the back of the tongue.

·  nRed palms of the hands and the soles of nthe feet.

·  nPeeling (desquamation) palms and soles n(later in the illness); peeling may begin around the nails.

·  nRash which may take many forms, nnon-specific, polymorphic, non-itchy, but not vesicle-bullous lesions, and appears non the trunk.

·  nSwollen lymph nodes (frequently nonly one lymph node is swollen, and is usually on one side), particularly ithe neck area.

·  nJoint pain (arthralgia) and swelling, nfrequently symmetrical, Also arthritis ncan occur.

·  nIrritability.

·  nTachycardia (rapid heart nbeat).

·  nBeau’s lines (transverse ngrooves oails).

·  nMay find breathing difficult.

Complications

X-ray showing aneurysmal enlargement of nthe coronary arteries, which is a complication in a Kawasaki syndrome

 

The cardiac complications are the most important aspect nof the disease. Kawasaki disease can cause vasculitic changes (inflammation of nblood vessels) in the coronary arteries and subsequent coronary artery aneurysms. nThese aneurysms can lead to myocardial ninfarction (heart attack) even in young children. Overall, about n10–18% of children with Kawasaki disease develop coronary artery aneurysms with nmuch higher prevalence among patients who are not treated early in the course nof illness. Kawasaki disease and rheumatic fever are the nmost common causes of acquired heart disease among children in the United nStates.

Causes

Like all autoimmune diseases, the cause of Kawasaki ndisease is presumably the interaction of genetic and environmental factors, npossibly including an infection. The specific cause is unknown, but current theories center primarily non immunological causes for the ndisease. Evidence increasingly points to an infectious etiology, but debate ncontinues on whether the cause is a conventional antigenic substance or a superantigen. Children’s Hospital Boston nreported that “some studies have found associations between the occurrence nof Kawasaki disease and recent exposure to carpet cleaning or residence near a nbody of stagnant water; however, cause and effect have not beeestablished.” Other data suggests possible correlation of KD with tropospheric nwind patterns

An association has been identified with a SNP in the ITPKC gene, which codes aenzyme that negatively nregulates T-cell activation. An additional factor that suggests genetic susceptibility nis the fact that regardless of where they are living, Japanese children are nmore likely than other children to contract the disease. The HLA-B51 serotype has beefound to be associated with endemic instances of the ndisease

Diagnosis

Kawasaki disease can only be diagnosed clinically (i.e. nby medical signs nand symptoms). nThere exists no specific laboratory test for this condition. It is difficult to nestablish the diagnosis, especially early in the course of the illness, and nfrequently children are not diagnosed until they have seen several health care nproviders. Many other serious illnesses can cause similar symptoms, and must be nconsidered in the differential ndiagnosis, including scarlet fever, toxic shock nsyndrome, juvenile idiopathic narthritis, and childhood mercury poisoning (infantile acrodynia).

Classically, five days of fever plus four of five diagnostic criteria must nbe met in order to establish the diagnosis.

The criteria are: (1) erythema of the lips or noral cavity or cracking of the lips; (2) rash on the trunk; (3) swelling or nerythema of the hands or feet; (4) red eyes (conjunctival injection) (5) nswollen lymph node in the neck of at least 15 millimeters.

n

 

 

n

 

Many children, especially infants, eventually diagnosed nwith Kawasaki disease do not exhibit all of the above criteria. In fact, many nexperts now recommend treating for Kawasaki disease even if only three days of nfever have passed and at least three diagnostic criteria are present, nespecially if other tests reveal abnormalities consistent with Kawasaki ndisease. In addition, the diagnosis can be made purely by the detection of ncoronary artery aneurysms in the proper clinical setting.

Investigations

A physical examination will demonstrate many of the nfeatures listed above.

Blood tests

·  nComplete blood count (CBC) nmay reveal normocytic anemia nand eventually thrombocytosis

·  nErythrocyte sedimentation rate n(ESR) will be elevated

·  nC-reactive protein (CRP) nwill be elevated

·  nLiver function tests may nshow evidence of hepatic inflammation and low serum albumin

Other optional tests

·  nElectrocardiogram may show nevidence of ventricular ndysfunction or, occasionally, arrhythmia due to myocarditis

·  nEchocardiogram may show subtle coronary nartery changes or, later, true aneurysms.

·  nUltrasound or computerized ntomography may show hydrops (enlargement) of the gallbladder

·  nUrinalysis may show white nblood cells and protein in the urine (pyuria and proteinuria) without nevidence of bacterial growth

·  nLumbar puncture may show nevidence of aseptic nmeningitis

·  nAngiography was nhistorically used to detect coronary artery aneurysms and remains the gold nstandard for their detection, but is rarely used today unless coronary artery naneurysms have already been detected by echocardiography.

·  nTemporal artery biopsy

Treatment

Children with Kawasaki disease should be hospitalized and ncared for by a physician who has experience with this disease. When in aacademic medical center, care is often shared between pediatric cardiology and pediatric infectious disease nspecialists (although no specific infectious agent has been identified as yet).^[38] nIt is imperative that treatment be started as soon as the diagnosis is made to nprevent damage to the coronary arteries.

Intravenous nimmunoglobulin (IVIG) is the standard treatment for Kawasaki disease nand is administered in high doses with marked improvement usually noted withi24 hours. If the fever does not respond, an additional dose may have to be nconsidered. In rare cases, a third dose may be given to the child. IVIG by nitself is most useful within the first seven days of onset of fever, in terms nof preventing coronary artery aneurysm.

Salicylate therapy, particularly aspirin, remains aimportant part of the treatment (though questioned by some) but salicylates nalone are not as effective as IVIG. Aspirin therapy is started at high doses nuntil the fever subsides, and then is continued at a low dose when the patient nreturns home, usually for two months to prevent blood clots from forming. nExcept for Kawasaki disease and a few other indications, aspirin is otherwise nnormally not recommended for children due to its association with Reye’s syndrome. Because nchildren with Kawasaki disease will be taking aspirin for up to several months, nvaccination against varicella and influenza is required, as nthese infections are most likely to cause Reye’s syndrome.

Corticosteroids have also been used, especially wheother treatments fail or symptoms recur, but in a randomized controlled trial, nthe addition of corticosteroid to immune globulin and aspirin did not improve noutcome. Additionally, corticosteroid use in the setting of Kawasaki disease is nassociated with increased risk of coronary artery aneurysm, and so its use is ngenerally contraindicated in this setting. In cases of kawasaki disease nrefractory to IVIG, cyclophosphamide and plasma exchange have been investigated nas possible treatments, with variable outcomes.

There are also treatments for iritis and other eye nsymptoms. Another treatment may include the use of Infliximab (Remicade). nInfliximab works by binding tumour necrosis factor alpha.^[53]

Prognosis

With early treatment, rapid recovery from the acute nsymptoms can be expected and the risk of coronary artery aneurysms greatly nreduced. Untreated, the acute symptoms of Kawasaki disease are self-limited (i.e. nthe patient will recover eventually), but the risk of coronary artery ninvolvement is much greater. Overall, about 2% of patients die from ncomplications of coronary vasculitis. Patients who have had Kawasaki disease nshould have an echocardiogram initially every few weeks, and then every one or ntwo years to screen for progression of cardiac involvement.

It is also not uncommon that a relapse of symptoms may noccur soon after initial treatment with IVIG. This usually requires nre-hospitalization and re-treatment. Treatment with IVIG can cause allergic and nnon-allergic acute reactions, aseptic meningitis, fluid overload and, rarely, nother serious reactions. Overall, life-threatening complications resulting from ntherapy for Kawasaki disease are exceedingly rare, especially compared with the nrisk of non-treatment. There is also evidence that Kawasaki disease produces naltered lipid nmetabolism that persists beyond clinical resolution of the disease.

 

References:

1.                     nManual of children’s infectious ndiseases / O. Ye. Fedortsiv, I. L. Horishna, I. M. Horishniy. – TERNOPІL : UKRMEDKNYHA, n2010. – 382 p. – ISBN 978-966-673-145-9 n

2.                     nManual of Childhood Infections: The nBlue Book (Oxford Specialist Handbooks in Paediatrics) by Mike Sharland, Andrew nCant and al. Published by  Oxford nUniversity Press Inc., New York, 2011 , p. 881  nISBN: 978-019-957-358-5.

3.                     nIllustrated Textbook nof Paediatrics, 4th Edition.  nPublished by  Lissauer & nClayden, 2012, p. 552 ISBN: 978-072-343-566-2.

4.                     n Nelson Textbook of Pediatrics, 19th nEdition Kliegman, Behrman. Published by Jenson & Stanton, 2011, 2608.  ISBN: 978-080-892-420-3.

5.                     nOxford Textbook of Medicine: Infection by David Warrell, Timothy M. Cox, John Firth nand Mili Estee Torok , Published by Wiley-Blackwell, 2012

6.                     nhttp://www.merckmanuals.com/professional/index.html