Transient Regional Wall Motion Abnormality and Increased Wall Thickness of the Left Ventricle in Acute Myopericarditis Occurring in the Puerperium

An unusual sequence of echocardiographic abnormalities of a 25-year-old female with acute myopericarditis was described. She presented with shortness of breath and a high body temperature after the birth of her first child. Regional asynergy and increased thickness of the left ventricle were transiently observed by echocardiography. It is considered that these abnormalities resulted from inflammatory changes in heart muscle such as edema, which was ascribable to acute myopericarditis in the puerperium

非侵襲的新指標である E' max/V 100 を用いた高血圧性肥大心の左室機能評価 : 非肥心， ST-T 変化を伴わない肥大心および ST-T 変化を伴う肥大心における比較検討

In forty-one essential hypertensive (EHT) patients with and without left ventricular hypertrophy (LVH), the left ventricular (LV) contractile performance was determined noninvasively using echocardiography. Classification was made with respect to the LVH, as measured by the sum of end-diatolic posterior wall thickness and interventricular septal thickness, and the presence of ST-T changes on electrocardiogram. Patients who had neither LVH nor ST-T changes formed Hl-subgroup (H1; n=22), those who had LVH without ST-T changes served as HZ-subgroup (H2; n =8), and those with LVH accompanied by ST-T changes constituted HS-subgroup (H3; n = 11), Sixteen normal volunteers served as normal control (N). LV systolic phase indices such as ejection fraction (EF), mean velocity of circumferential fiber shortening (mVcf) and end-systolic wall stress (ESWS), and diastolic indices such as isovolumic relaxation time (IVRT) and PR-AC interval were compared among each subgroup and normal subjects. All systolic and diastolic indices showed a depressed LV function in H3. Of these variables, the only IVRT could separate H2 from H1, suggesting deteriorated diastolic function at an early stage of hypertrophy. By altering LV systolic loading, peak systolic pressure-end-systolic volume relation, E’ max, and E' max-volume intercept at 100 mmHg peak systolic pressure ratio, E' max/V 100, were designated and these indices were used for the expression of the myocardial contractile state. E' max and E’ max/V 100 were significantly lower in H2 and H3 than in the control group, indicating depressed myocardial contractility. The value of these variables in Hl did not differ from N, indicating a normal level of inotropic state. E’ max/V 100 in H3, 0.13±0.04 mmHg/ml2, was significantly less than in H2, 0.23±0.05 (p<0.01), and the value in H2 was significantly lower than that in H1, 0.36 ±0.07 (p< 0.01), indicating a validity of E' max/V 100 to differentiate each EHT subgroup. It is concluded that in patients with LVH induced by pressure overload the LV function is declined, furthermore, LV contractile performance is more impaired when LVH is accompanied by ST-T changes. E’ max/V 100 is highly sensitive in identifying the presence of LV contractile impairment and may be a useful approach to the quantitation of LV. performance

Waardenburg Syndrome with Isolated Deficiency of Myenteric Ganglion Cells at the Sigmoid Colon and Rectum

Waardenburg syndrome (WS) has the characteristic clinical features caused by the embryologic abnormality of neural crest cells. WS patients sometimes suffer from functional intestinal obstruction. When it is Hirschsprung disease (HD), the WS is diagnosed as type 4 WS. We report a case of WS which did not have myenteric ganglion cells in the sigmoid colon and rectum. Whether to diagnosis this case as type 1 or 4 WS is controversial. Moreover, this is the third report which has peristalsis failure caused by abnormal myenteric plexus. In all three cases, the eosinophils had aggregated in the myenteric layer of the transition zone. During embryonic life, enteric ganglion cells migrate to the myenteric layer from the proximal to the distal side sequentially and, subsequently, to the submucosal layer through the circular muscle. Therefore, we hypothesize that myenteric ganglion cells that had already migrated were eliminated by an eosinophil-mediated mechanism in these three cases. We believe this report may be helpful to elucidate the pathogenesis of some types of HD

Fluctuations in C-Reactive Protein in a Hepatoblastoma Patient with Thrombocytosis

We observed the changes in serum levels of interleukin 6 (IL-6) and C-reactive protein (CRP) in a patient with hepatoblastoma exhibiting thrombocytosis. The concomitant changes of IL-6 and CRP concentrations after the initiation of chemotherapy, in the absence of infection, suggested that the IL-6, which is synthesized in hepatoblastoma cells and induces thrombocytosis, also stimulated CRP production in the present case. IL-6 is thought to play an important role in thrombocytosis in hepatoblastoma