[Show abstract][Hide abstract] ABSTRACT: To evaluate infliximab (IFX) in patients with Kawasaki disease (KD) that was unresponsive to additional intravenous immunoglobulin (IVIG) therapy and subsequent rescue with supplementary plasma exchange (PE) in patients unresponsive to treatment.
We studied 76 patients with KD who received IVIG therapy twice and were unresponsive to additional IVIG.
Seventy were treated with IFX alone (92.1%). Six patients who were unresponsive IFX (7.9%) were further treated by PE. This resulted in disappearance of fever and other clinical symptoms, and improvement of laboratory data. There was no severe life-threatening adverse events.Twelve of the 76 cases had developed coronary artery dilatation, and 3 had coronary artery aneurysm within 1 month of disease onset. At the end of follow-up, in all cases, coronary artery lesions were suppressed or reversed.
Treatment of intractable KD with sequential IVIG, IFX, and PE treatments in a step-wise protocol was effective.
The Journal of pediatrics 02/2014; 164(5). DOI:10.1016/j.jpeds.2014.01.020 · 3.79 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Beriberi-heart disease is rare in advanced countries, so accurate diagnosis may be elusive. We present the case of a 2 year-old girl with mild mental developmental disability who developed beriberi-heart disease, presenting with dyspnea, right-sided heart failure, and pulmonary hypertension after she caught a cold. This patient was abnormally fastidious about her food and fluid intake： she would not eat almost exclusively. When admitted to our hospital, her serum thiamine level was remarkably depressed. We were certain she was free from other diseases that can cause pulmonary hypertension. After receiving a thiamine supplement, cardiac function was improved and pulmonary pressure became normal. Neurological symptoms (e.g. hypotonia, lack of deep tendon reflex) also improved. This patient appeared to have secondary pulmonary hypertension induced by thiamine deficiency, based on a complete cure after nutritional status improved.
[Show abstract][Hide abstract] ABSTRACT: The treatment of Kawasaki disease patients who fail to respond to initial i.v. immunoglobulin (IVIG) therapy is controversial. The aim of the present study was to investigate the long-term efficacy of plasma exchange (PE) treatment for refractory Kawasaki disease.
A total of 125 Kawasaki disease patients refractory to IVIG were treated with PE. Coronary artery lesions (CAL) before PE, in the acute period, and during the late period were examined retrospectively.
Residual sequelae requiring medical treatment occurred in six cases in the late period. The outcomes of treatment tended to be better when PE was begun in the early stage. Sequelae remained in 2.8% of patients in whom PE was initiated prior to day 9 after onset, and were present in 15% of patients in whom PE was started on or after day 10. The 105 patients whose coronary arteries were normal before PE had no sequelae (residual sequelae: 0%). Dilatation was present before PE in 14 patients, but remained in only two patients in the late period (residual sequelae, 14.3%). In four of the six patients in whom aneurysms had already formed before PE, the lesions had advanced into giant aneurysms, but in the other two patients they returned to the normal range (residual sequelae, 66.6%).
The outcomes of PE for Kawasaki disease refractory to IVIG are favorable, and the effectiveness of this treatment is excellent, particularly if it is initiated before CAL arise.
Pediatrics International 02/2012; 54(1):99-103. DOI:10.1111/j.1442-200X.2011.03487.x · 0.73 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We had the unique opportunity of following the electrocardiographic (ECG) course of a 13-year-old male with sinus dysfunction and atrial flutter who subsequently developed a Brugada-type ECG pattern associated with sick sinus syndrome at 25 years old. Family history showed that the patient's mother and maternal grandfather suddenly died while sleeping at night. When the patient was 13 years old, he lost consciousness after running a marathon. The patient was diagnosed with sinus dysfunction and atrial flutter, and he underwent pacemaker implantation at 15 years old. ECG examinations performed between 13 and 20 years old showed incomplete right bundle branch block and ST elevation with early depolarization. On ECG examinations performed when the patient was 21 years old and thereafter, the V(2) lead always showed a saddleback-type ST elevation. At 25 years old, the late potential was positive and the electrophysiological study induced ventricular fibrillation. A challenge test with pilsicainide showed remarkable ST elevation by the V(2) lead. The 24-h Holter ECG monitoring showed remarkable ST elevation after eating a snack and during night time when the patient was asleep. The patient was diagnosed with Brugada syndrome and an implantable cardioverter-defibrillator was implanted. Genetic analysis did not reveal mutation of the SCN5A gene.
[Show abstract][Hide abstract] ABSTRACT: We report three cases of hemophagocytic lymphohistiocytosis (HLH) in infants within the first 6 weeks of life. Diagnosis of HLH was made early after symptoms started. All three cases were successfully treated with dexamethasone and none relapsed, indicating that not all cases of HLH in very young infants are familial.