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We used noninvasive positive pressure ventilation (NPPV) with a helmet-type mask in two young children with acute severe bronchial asthma. The intervention was well-tolerated, because the helmet-type mask caused no pain or discomfort, as compared to the face mask. Tracheal intubation can be avoided by the start of the NPPV. Thus, the respiratory symptoms improved by the use of NPPV in children with acute severe asthma with respiratory muscle fatigue and failure of medical treatment.

We report a 2-year-old girl with tumor necrosis factor receptor–associated periodic syndrome (TRAPS) who is the youngest proband diagnosed in Japan. Recurrent fever had started at her 6 months of age, and she had the familial history of recurrent fever, suggesting underlying genetic disorder, in her father and grandfather. Careful clinical observation of characteristics of fever with disease course and the familial history of recurrent fever may lead to diagnosis of TRAPS in early infancy.

電顕的に診断された菲薄性基底膜症候群について，アンケート調査による臨床的検討を行った。(1)全国42施設にアンケート調査表を送付し，34施設 (81.0%) から回答を得た。(2)家系に腎疾患歴を有するI群73家系87例と，孤発例と考えられるII群26家系26例について検討した。(3)I群の腎疾患家族歴のうち，腎不全歴は5家系5例に，腎死は1例に認められた。(4)性別，発症年齢，最終年齢，平均観察期間，発見動機，初診時ならびに最終観察時の検尿・BUN・Cr，初診時と最終観察時の諸検査の推移については，両群間で差は認めず，腎機能低下例はなかった。(5)感染罹患に伴った尿所見悪化例は，I群29.9%，II群30.8%であった。(6)尿所見改善例はI群で9例認め，うち女性が8例であった。(7)投薬例 は，I群17.2%，II群23.1%で，いずれも積極的な治療は施行されていなかった。以上より，本疾患の中・長期的予後は，良好であることが示唆された。

The presence of a cytokine storm is recognized as a condition possibly involved in the mechanism for the onset, aggravation and progression of acute encephalitis/encephalopathy. Plasma exchange and slow hemodiafiltration have been used as the means of blood purification to deal with acute encephalitis/encephalopathy. It has been suggested that blood purification can lead to diminution of the cytokine storm, possibly enabling correction of multiple organ failure and disseminated intravascular coagulopathy (DIC). However, additional studies are needed as to the possibility of achieving the diminution of the intracerebral cytokine storm with this approach.

So as to determine the value of brain natriuretic peptide in the plasma as a biochemical marker for myocarditis of Kawasaki disease, we studied 69 patients. The blood samples, electrocardiograms and cross-sectional echocardiograms were obtained before the commencement of treatment and in the convalescent phase. Results: The mean concentration of brain natriuretic peptide in the plasma was 73.2 ± 107.7 (mean ± SD) pg/ml in the acute phase, and 7.9 ± 7.5 pg/ml in the convalescent phase. We checked the electrocardiograms to find abnormal Q waves, elevation or depression of the ST segments, change in the pattern of the QRS complexes, and flattening or inversion of the T wave, all believed to be markers of myocarditis in Kawasaki disease. Those in whom the concentrations were greater than 50 pg/ml in the acute phase showed abnormal electrocardiograms more frequently than did those in whom the values were less than 50 pg/ml (21/29 vs 3/40, p < 0.0001 odds ratio 32.4). Amplitudes of the T wave in standard limb leads were measured both in the acute and convalescent phases, and the differences calculated. We regarded the sum total of these differences as representing “flattening T wave”, and we named this variable as the total suppressed T wave voltage. We examined the correlation between the variable and the levels of brain natriuretic peptide in the plasma during the acute phase, demonstrating a significant correlation (r = 0.500, p < 0.0001). We conclude, therefore, that the concentration of brain natriuretic peptide measured in the plasma can be a useful biochemical marker for the myocarditis of Kawasaki disease. When the titer is over 50 pg/ml, the patient probably has an abnormal electrocardiogram and is most likely to have myocarditis.

BACKGROUND Brain natriuretic peptide (BNP) is a cardiac hormone and plasma levels of it increase in patients with congestive heart failure and in those with acute myocardial infarction. Kawasaki disease (KD) is a well-known generalized vasculitis and the most prominent features of this disease are the cardiovascular manifestations, which involve the pericardium, myocardium, endocardium and coronary arteries. It was hypothesized that the plasma concentrations of BNP in patients with KD might be increased and that plasma BNP might be a useful biological marker of cardiovascular manifestations in patients with KD. METHODS Blood was obtained to measure and compare plasma BNP concentrations in the acute (n = 32) and convalescent (n = 35) phases of KD and in the acute phase of the patients with viral infection (n = 26), which included adenovirus, influenza, measles and herpes group virus infection. In patients with KD, two-dimensional echocardiography was performed to check for pericardial effusion and coronary arterial lesions and to measure the dimensions of the left ventricle at diastole and the shortening fraction of the left ventricle (LVSF). RESULTS The mean plasma BNP concentration in patients with KD in the acute phase was 55.0 +/- 39.5 pg/mL, but was 6.8 +/- 7.3 pg/mL in patients with viral infection. The plasma BNP concentration in patients with KD in the acute phase was significantly higher than in patients with viral infection (P < 0.0001). In 31 cases of KD, the plasma BNP concentrations were measured both in the acute and convalescent phases. The mean plasma BNP concentration in the acute phase of KD was 55.3 +/- 40.1 pg/mL and in the convalescent phase was 5.9 +/- 5.7 pg/mL. The level of plasma BNP decreased significantly in the convalescent phase (P < 0.0001). The mean BNP level in patients with KD with pericardial effusion (n = 8) in the acute phase was 80.3 +/- 43.4 pg/mL and that in patients without pericardial effusion (n = 24) was 46.5 +/- 35.1 pg/mL. The BNP level in patients with pericardial effusion was significantly higher than that of patients without pericardial effusion (P < 0.05). There was no significant correlation between the plasma concentrations of BNP in the acute phase of KD and LVSF (r = -0.161, P = 0.39, n = 31). CONCLUSION It was shown that the plasma BNP concentration increased in the acute phase of KD and decreased to within normal range in the convalescent phase. Further examinations are needed to clarify the mechanism by which the elevated levels of plasma BNP occur in the acute phase of KD. However, plasma BNP might be a useful biological marker of the cardiovascular manifestations in patients with KD.

In a family with Alport syndrome, molecular analysis of the COL4A5 gene, which encodes the alpha 5(IV) chain of glomerular basement membrane collagen, revealed a GGA-->AGA change in exon 31, resulting in substitution of an arginine for a glycine in position 852 of the polypeptide chain, between interruptions 16 and 17 of the triple-helical collagenous domain. The mutation causes the MaeI restriction sites, and could be easily diagnosed in the family members through restriction analysis. This one point mutation can be expected to interrupt type IV collagen molecules.