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Hypomelanosis of Ito is a multisystem neurocutaneous syndrome. The pathogenesis hasn′t been clear and typical cutaneous lesions are associated with variable extracutaneous abnormalities involved the central nervous system and musculoskeletal system. Its clinical manifestations include abnormal skin biopsy and chromosomal patterns and brain iconography. The treatments have to be adapted to the individual and prognosis depends on the severity of clinical manifestations and complications.
ABSTRACT
The scholars abroad have reported a group of previously normal children,who develop intractable focal epilepsy or status epilepticus after fever,followed by drug-resistant partial epilepsy and severe cognitive impairment,named febrile infection-related epilepsy syndrome (FIRES).There are unknown etiology,critical condition,poor prognosis and absent of specific changes on cerebrospinal fluid,biopsies and neuroimagings.No therapeutic agent is efficacious and ketogenic diet may be an alternative therapy in some reports.This study describes the clinical characteristics of FIRES.
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Objective To investigate the clinical features of incomplete Kawasaki disease (IKD).Methods The data of 46 Kawasaki disease (KD) patients including 14 IKD patients (IKD group)and 32 classical Kawasaki disease (CKD) patients (CKD group) was analyzed retrospectively,and compared the clinical manifestation,laboratory examination result,the incidence of coronary artery lesion (CAL) and the response to intravenous immunoglobulin (IVIG) of two groups.Results There was no significant difference in gender and age between IKD group and CKD group (P> 0.05 ).In IKD group,the fever time was longer than that in CKD group [( 11.43 ± 9.12) d vs (7.12 ± 2.83 ) d, P < 0.05], but the frequency of conjunctival congestion, lymphadenectasis of neck, indurative edema of palms and soles, changes of lips and oral cavity were less than these in CKD group [57.14%(8/14) vs 93.75%(30/32),28.57%(4/14) vs 59.38%(19/32), 35.71%(5/14) vs 78.12%(25/32),42.86%(6/14) vs 75.00%(24/32)](P< 0.01 or < 0.05).There was no difference in laboratory examination result and incidence of CAL between two groups, but the incidence of IVIG nonresponse in IKD group was higher than that in CKD group [40.0%(4/10) vs 6.45%(2/31 ),P <0.05].Conclusions The frequency of conjunctival congestion, indurative edema of palms and soles, changes of lips and oral cavity are less common in IKD patients comparing with CKD patients.The laboratory examination result and the risk of CAL in IKD patients are similar to CKD ones.Moreover, IKD is not sensitive to IVIG, so the doctors should pay more attention to it.
ABSTRACT
Objective To evaluate the echocardiographic features of coronary artery dilation (CAD) and coronary artery aneurysms (CAA) in Kawasaki disease dynamically.Methods Echocardiography was performed in KD cases for measurement of the diameter of the coronary artery.Results Twelve CAD and four CAA were identified in forty-one patients.In CAD group,left coronary artery (LCA) is more susceptive to dilation than right coronary artery (RCA)(P<0.05).Both LCA and RCA were involved in CAA group,the maximum diameter of coronary artery is 10mm.Six to eighteen days after treatment,LCA were regressed significantly in CAD group(P<0.05),but the diameter of RCA in CAD group and coronary artery in CAA patients have no change(P>0.05).Ten coronary artery lesion patients were followed up,8 CAD cases regressed completely,1 CAA extended,the other CAA regressed completely.Conclusions In CAD group,LCA is more susceptive to dilation than RCA,but it regressed significantly in a short time after treatment.Echocardiography is a non-traumatic method to detect the coronary artery lesions in KD patients dynamically.