Prenatal Diagnosis and Outcome of Ebstein's Anomaly and Tricuspid Valve Dysplasia in Relation to Lung Hypoplasia

Although not all newborns with Ebstein's anomaly present with severe cardiomegaly in utero, some of them cannot live after birth because of the lung hypoplasia. To clarify the relationship between the intrauterine cardiomegaly and the outcome of the patients with Ebstein's anomaly or tricuspid valve dysplasia, we calculated the ratio of the area of the heart against the thorax (CTAR) in the transverse view of the thorax at the level of the cardiac four-chamber view in the fetal echocardiogram and compared it to the outcome. The study population consisted of four patients with Ebstein's anomaly and one patient with tricuspid valve dysplasia who were diagnosed in utero. The ranges obtained from 53 normal fetal cases were 20%± 8% (mean ± 2 SD) at below 20 week's gestation, 25%± 10% at the gestational age of 21 to 30 weeks, and 29%± 6.4% at the gestational age of 31 to 40 weeks. The CTARs of these five cases measured 81.6%, 51%, 55.2%, 47.5%, and 75.6%, respectively, and were abnormally higher than the normal value. Two fetuses died in utero with severe hydrops fetalis. Two fetuses whose cardiothoracic ratios by chest X ray were 100% died at twelve hours of life. One patient died at 110 days. The cross-sectional area of the thorax was smaller than the normal range in 3 (cases 1, 4, and 5) out of these 5 cases. Thus, we conclude that fetal Ebstein's anomaly and tricuspid valve dysplasia associated with massive tricuspid regurgitation with a large CTAR ratio (higher than 50%) and small thoracic cross-sectional area has a very poor prognosis bothprena-tally and neonatally. (ECHOCARDIOGRAPHY, Volume 11, May 1994)     

Spontaneous rupture of adrenal pheochromocytoma with capsular invasion

A 67-year-old Japanese man developed a sudden onset of severe right-side upper abdominal pain, nausea and vomiting. On hospitalization, physical examination revealed sweating, tachycardia, hypertension and the appearance of peripheral vasoconstriction. An urgent computed tomography scan with contrast demonstrated a large hematoma in the right retroperitoneal space. A phentolamine test and an 131iodine metaiodobenzylguanidine scan suggested pheochromocytoma. An elective right adrenalectomy was successfully performed after pretreatment for sufficient volume replacement with continuous administration of alpha- and beta-adrenergic blocking agents. Pathological diagnosis was an adrenal pheochromocytoma 9.0 x 6.5 cm in diameter with evidence of capsular invasion, which could be associated with a tear in the capsule.     

Color Stability of Compomer after Immersion in Various Media

Purpose: The purpose of this study was to evaluate the effect of various media on the color stability of compomer and to compare these results to those of other materials that could be used in similar clinical circumstances.
Materials and Methods: In this test, six materials (shade A2) were used: four compomers (Dyract, Compoglass F, Xeno, F2000), one composite resin (Clearfil AP-X), and one resin-modified glass ionomer cement (Fuji II LC). There were four test solutions: one alcoholic (whiskey), two low pH soft drinks (Coca Cola, orange juice), and deionized water as a control. A plastic ring mold (9-mm diameter x 1-mm height) was used to prepare 120 disk specimens. For 60 days, the test specimens were immersed in the various media daily for 3 hours then transferred to the deionized water. Color was measured by CIE L* a* b* relative to CIE source against a white background, using a colorimeter. Color change (Δ*) was calculated as Δ*= [(δ*)²+ (δ*)²+ (Δ*)²]^1/2. Color changes (Δ*) were recorded after 1, 7, 30, and 60 days.
Results: The results indicated that compomer and resin-modified glass ionomer were susceptible to discoloration in various solutions over an extended period of time. Composite resin showed minimal perceptible color change. Specimens immersed in whiskey showed a significantly high perceptible color change (p < .0001). Water caused no perceptible color changes.
CLINICAL SIGNIFICANCE
Compomer and resin-modified glass ionomer materials are susceptible to discoloration by various media.     

Interleukin-2 receptor positive T and B cells in children with acute severe asthmatic attack

Subpopulations of interleukin-2 receptor (IL-2R)-positive CD4 and CD8 T cells and IL-2R⁺ CD20 B cells in the peripheral blood lymphocytes as well as serum concentrations of soluble IL-2R (sIL2R) were measured in children aged 1–7 years who suffered acute severe asthmatic attack. Subpopulations of CD4⁺ IL-2R⁺ cells, CD8⁺ IL-2R⁺ cells and CD20⁺ IL-2R⁺ cells in the peripheral blood lymphocytes at acute severe asthmatic attack phase were significantly higher than those at non-asthmatic attack phase (P < 0.02, P < 0.03 and P < 0.02, respectively). Subpopulations of CD20⁺ IL-2R⁺ cells in the peripheral blood lymphocytes significantly decreased 5–10 days after acute severe asthmatic attack (at recovery phase, P < 0.02) and were significantly correlated with clinical severity of asthmatic attack (P < 0.05). These results indicated that activation of both T cells and B cells was important in the pathogenesis of acute asthmatic attack in young children.     

Analysis of the circumstances of death of 56 children suffering from cancer: Proposal for the development of terminal medicine in Japan

In Japan terminal medicine for children dying from cancer has not yet been developed nor has a support system for home terminal care and bereaved families been established. We have analyzed our own experiences in these areas and researched the possibilities of establishing support systems. In the 16 years from 1978 to 1993, 56 children with cancer have been treated and have died at Hamamatsu University Hospital. We analyzed the circumstances of their deaths. We interviewed 25 sets of parents about their acceptance of their child's death. Three children (5%) died unexpectedly during treatment, 27 children (48%) died from the side-effects of intensive treatment, and 26 children (47%) died during terminal care. More children with leukemia and lymphoma died from side-effects than children with solid tumors (P < 0.05). Six out of the 25 families had not yet accepted the loss of their child due to regrets associated with the missed opportunity for terminal care. From our experiences with the five children who received terminal care at home, we recognize the need for a support system run by the hospital and conclude the time is ripe for initiating home-based terminal care in Japan.