Guidelines
Revision of diagnostic guidelines for Kawasaki dia(6th revid edition)
Purpo and background of the revision
The diagnostic guidelines for Kawasaki dia(KD)were last revid in20021(5th revision).Major points of revision included:(i)the definition of fever was defined as fever per-sisting5days or more(inclusive of cas in which the fever had subsided before the fifth day in respon to therapy)and(ii)to explicitly state that incomplete KD cas can have coro-nary artery lesions.
After the5th revid edition of the diagnostic guidelines for KD was published,the proportion of patients receiving early treatment incread and the incidence of coronary artery lesions decread nationwide.On the other hand,the number of incomplete KD cas incread yearly from10%to the cur-rent level,which is greater than20%of all KD patients.
In recent years,a standard method for expressing the coro-nary artery internal diameter of Japane children was estab-lished2and allowed us to define coronary artery internal dimensions in terms of standard deviations from the mean,or Z scores).Incorporation of Z scores to facilitate the diagnosis of i
ncomplete KD was a motivating factor for this revision.In the5th edition,the ction titled“Other significant symptoms or findings”was not changed;therefore,the description from the4th edition lasted more than 30 years and was due for an update. In 2017, we consulted with the Japan Kawasaki Dia Society Steering Committee Members regarding the necessity for a revi-sion of the 5th revid edition, and 75% of the committee mem-bers agreed to
revi. The Japan Kawasaki Dia Rearch Center and study group for vasculitis funded by the Ministry of Health, Labor and Welfare also agreed to the revision.
巨蟹座和摩羯座In this revision, the writing group members conducted dis-cussions from 2018 to 2019. The original draft was prented to the 38th Annual Meeting of the Japane Society of Kawa-saki Dia in Wakayama. The draft was revid again, bad on the steering committee members’ suggestions, and the final draft of the 6th revision was completed. In the future it will be interesting to evaluate the impact of the revid guideli-nes on the diagnosis of KD in Japan.
The previous 5th edition1 was published as an article in Japan Today in 2005, and was titled “Diagnostic Guidelines.” The recent format of the “guidelines” has changed and requires full supporting evidence; “diagnostic guidance” or “criteria with clinical findings” may be more appropriat
e as the title for this revision becau there is not enough evidence for the diag-nosis of this dia. However, as the previous title has been familiar with most pediatricians and primary care physicians, we would prefer to u the same title with only the change of the edition number from the fifth to the sixth revid edition. Additionally, becau such clinicians u the guidelines as the diagnostic criteria, it is desirable to be as conci as possi-ble and to be prented as a few brief sheets of 1 or 2 pages.
As we also believe more detailed explanations are neces-sary to describe each item, including many examination findings, an additional “guidebook” will be written by the committee members for publication.
The major changes of the revision are described below.
Principal clinical features
Several changes were made to the six principal clinical fea-tures,which have been well understood and disminated for almost all clinicians in Japan(Tables1–3and Figure1).
1.The requirement for a specific duration of fever was deleted.In Japan,more than90%of KD patients
received high do intravenous immunoglobulin(IVIG)in a single do.Although most pediatricians or primary care physi-cians know that the classic definition of KD required a duration of fever for more than5days,the24th Nation-wide Surveillance reports that approximately9%,25%,and35%of KD patients received the first IVIG treatment on the3rd,4th,and5th days of illness,respectively,and the prevalence of coronary artery lesions(CAL s)has been lower than before. As we expect a continuous decrea in CALs, we modified the fever definition to reflect current practice.
Table1Principal clinical features
1.Fever.
2.Bilateral bulbar conjunctival injection.
3.Changes of lips and oral cavity:reddening of lips,strawberry tongue,
diffu injection of oral and pharyngeal mucosa.
4.Rash(including redness at the site of Bacille Calmette-
Gu e rin(BCG)inoculation).
张国中5.Changes of peripheral extremities:(Initial stage)reddening of palms and
好看的qq头像soles,edema.(Convalescent stage)periungual desquamation.炫酷图片
6.Non-supparative cervical lymphadenopathy.
Table 2Definition of complete or incomplete KD
Number of principal
clinical features
Coronary artery abnormalities (+)Coronary artery abnormalities (À)6
Complete (a)Complete (a)5
Complete (a)Complete (a)4
Complete (b)Incomplete (d)3Incomplete (c)Incomplete (d)
Table 3 Other significant demographic, clinical, echocardio-graphic, and laboratory features
1. Kawasaki dia may be suspected in the prence of fewer than four principal clinical features when the following findings are obrved:
•Elevation of hepatic transaminas early in the cour of the dia.•Incread leukocytes in the urine diment of an infant.•Thrombocytosis in the convalescent pha •Elevation of BNP or NT-pro BNP •Mitral valve regurgitation or pericardial effusion by echocardiography •Enlargement of the gallbladder (hydrops of gallbladder)•Hypoalbuminemia or hyponatremia
2. If a KD patient manifests the following findings, the patient should be considered for admission of a critical care unit.•Hemodynamically significant myocarditis •Hypotention (shock)•Paralytic ileus •Decread level of consciousness
3. Risk scores to predict intravenous immunoglobulin resistance may be applied to guide patient management. The following features are elements of the risk scores for predicting intravenous immunoglobulin resistance.•Leukocytosis with left shift •thrombocytopenia •hypoalbuminemia •hyponatremia •hyperbilirubinemia (jaundice)•elevation of CRP •Age <1year
4. Other non-specific findings which may be obrved in Kawasaki Dia and should not exclude the diagnosis.•Irritability •Cardiovascular: abnormal extra heart sounds, electrocardio-gra
m changes, aneurysm of peripheral arteries other than coronary (axillary etc.),•Gastrointestinal: abdominal pain, vomiting, diarrhea •Hematologic: incread erythrocyte dimentation rate, ane-mia •Dermatologic: micropustular rash, transver grooves across the finger nails.•Respiratory: cough, rhinorrhea, retropharyngeal edema, infiltrate on chest radiograph.•Rheumatologic: pain, swelling.•Neurologic: cerebrospinal fluid pleocytosis, izures, facial nerve palsy, paralysis of the extremities.
BNP, brain natriuretic protein; KD, Kawasaki dia; NT-pro BNP, N terminal pro-brain natriuretic protein.
1.Mortality in the acute pha: <0.1%.
2.Recurrence rate: 3–4%; proportion of siblings’ cas, 1–2%.
3.Nonsuppurative cervical lymphadenopathy (multiple hypoe-choic, enlarged nodes obrved on ultrasound) is less fre-quently encountered (approximately 65%) compared with other principal clinical features during the acute pha. Non-
suppurative cervical lymphadenopathy is obrved in approxi-mately 90% of older children and often can be the first
clinical feature of KD with fever.
To diagno complete or incomplete KD, the exclusion of other febrile illness is esntial.
a: A patient who fulfills the criteria with five or six signs is diagnod as complete KD.
b: A patient who fulfills the criteria with four signs and coro-nary artery abnormality by echocardiography (Figure 1-h) is diag-nod with complete KD.
c: A patient who has three principal clinical features with coro-nary artery abnormality by echocardiography (Figure 1-h) and in whom other febrile illness have been excluded fulfills the crite-ria in “c.”
d: When the patients who fulfill three or four signs in the the principle clinical features without coronary artery dilation but with some features from the list of ‘Other significant clinical fea-tures’ can be diagnod as incomplete KD, if other dias are ruled out.
e: Incomplete KD may also be considered in the prence of only one or two principal clinical features after excluding other diagnos.
2. The clinical feature of “Polymorphous exanthema” was changed to “Rash” and now includes “redness at the site of Bacille Calmette-Gu e rin (BCG) inoculation”. Japane pediatricians have recognized that redness at the site of BCG inoculation is a specific clinical sign that appears at the ont of KD. In the prent revision, we included redness at the site of BCG inoculation as a clinical feature that counts as “rash,” even in the abnce of more diffu dermatologic changes.
In particular, this sign is obrved in more than 70% of the patients who ages are from 6 to 20 months old3. When the patient do not show polymorphous exanthema but shows the redness of BCG scar and other 4 principle sign, that patient can be diagnod as not incomplete KD, but (com-plete) KD. The impact of this change to the principal diagnostic clini-cal features will require further study and should be moni-tored.
a b
c d
e f
g h
Fig.1(a)Bulbar conjunctival injection;(b)reddening of lips,strawberry tongue;(c)rash;(d)redness at the site of Bacille Calmette-Gu e rin (BCG)inoculation;(e)reddening of palms with edema;(f)periungual desquamation;(g)non-supparative cervical lymphadenopa-thy;(h)Echocardiographic finding of coronary artery aneurysm.
3.Non-suppurative cervical lymphadenopathy(multiple hypoechoic,enlarged nodes obrved by ultrasound)is less
frequently encountered(approximately65%)than other principal clinical features during the acute pha.
Nonsuppurative cervical lymphadenopathy is obrved in approximately90%of older children and often can be the first clinical feature of KD with fever.This phenomenon is described as“Remark”1at the last part.
4.The preci clinical definitions of complete and incom-plete KD are now clearly delineated as outlined in the appendix
and are bad on the number of principal clini-cal features and the prence of coronary artery abno
rmalities.A patient who fulfills the criteria in“a”or“b”is diagnod as complete KD.A patient who has three prin-cipal clinical features with coronary artery abnormality by echocardiography and in whom other febrile illness have been excluded fulfills the criteria in“c”and is diag-nod as incomplete KD.Patients who fulfill the criteria in“d”are also diagnod as incomplete Kawasaki dia defined as the prence of three or four principal clinical features without coronary artery dilation but with features from the list of“Other significant clinical features”.
Incomplete Kawasaki dia may also be considered in the prence of only one or two principal clinical features after very careful,sufficient obrvation and excluding other diagnos.For the patients,particularly careful consideration of the differential diagnosis is esntial.For reference,in the24th nationwide survey,0.7%and 5.4%of all KD patients were reported as incomplete KD with only one or two clinical features,respectively.
人无完人金无足赤
However,the possibilities of other dia are higher than for incomplete KD,if the coronary artery is not involved and the principal signs are limited to less than two.
Evaluation of the coronary arteries using Z scores
The revid guidelines recommend the u of Z scores for defining coronary artery dilation.When Z-
score of internal coronary artery diameter≥2.5SD units,it is defined as coro-nary artery dilation.However,in ca that the examiner has a difficulty to u Z score, conventional criteria using traditional measurements of inner diameter≥3mm(<5years old)or≥4mm(≥5years old)can be ud for the diagnosis of coronary artery dilation.While Z scores are a more quantitative asss-ment,we realize that they have not been adopted by all centers in Japan.This change of definition may affect the incidence of coronary artery dilation,especially transient dilation and small aneurysm.Asssment of the impact of this change will there-fore be important in future epidemiologic surveys. Other significant demographic,clinical,echocardiographic,and laboratory features
In this ction,we substantially revid the description of specific clinical features that can be associated with KD.
1.Seven clinical features are described that may be helpful in the recognition of incomplete KD cas.We hope that future男烫发发型图片
clinical rearch will provide more accurate diag-nostic tools including the optimum cut-off values for the variables.
Although it is expected that the quantitative cut-off values of all findings in the items will be deter-mined,there have been very few Japane studies of suffi-cient quality.In the future,an algorithm using such cut-off values is expected to be constructed.
2.Clinical findings that warrant referral to a tertiary medical center with experience in treating critically ill pediatric patients怦然心动观后感
are outlined.
3.Risk scores to predict intravenous immunoglobulin resis-tance may be applied to guide patient management.We defined
ven features that are elements of risk scores pre-dicting intravenous immunoglobulin resistance.The fea-tures may be uful in risk stratification of patients.
4.Other non-specific findings that may be obrved in KD and should not exclude the diagnosis have been added.The
eight features may support the diagnosis of KD.空调外机结霜
This summary describes the major changes to the6th revid edition of the KD diagnostic guidelines.We hope that this revision will lead to standardization of the definition of incomplete KD and diagnostic approaches,and will further reduce number of coronary artery lesions suffered by KD patients.
