Flecainide against fetal supraventricular tachycardia complicated by hydrops fetalis

Cyclic depsipeptide antibiotic aureobasidin A (AbA) and its analogs were tested for the inhibitory activity of P-glycoprotein in multidrug resistant cancer cells as well as for the antifungal activity. Some analogs with lower antifungal activity than AbA showed higher inhibition of P-glycoproteins indicating difference of the structure-activity relationships between the two activities. Among AbA analogs tested, [D-beta-hydroxy-methylvalyl9]-AbA newly prepared by chemical synthesis, which had much lower antifungal activity than AbA, showed 10-fold higher inhibitory activity of P-glycoprotein than AbA.     

Amniotic fluid for screening of lysosomal storage diseases presenting in utero (mainly as non-immune hydrops fetalis)

PURPOSE: We studied the efficacy and safety of a recent technique of keratomileusis for myopia, excimer laser in situ keratomileusis. METHODS: We studied retrospectively 88 eyes of 63 patients who received excimer laser in situ keratomileusis with the Chiron Automated Corneal Shaper and the Summit OmniMed laser under a hinged corneal flap without sutures. RESULTS: Mean follow-up was 5.2 months. Mean spherical equivalent of the manifest refraction before surgery was -8.24 diopters (range, -2.00 to -20.00 diopters). Mean spherical equivalent refraction after surgery was +0.22 +/- 1.42 diopters. Of 40 eyes with a baseline refraction from -2.00 to -6.00 diopters, 25 eyes (63%) had refraction within +/- 0.50 diopter of emmetropia, and 37 eyes (93%) had refraction within +/- 1.00 diopter. In eyes with baseline refraction of -6.12 to -12.00 diopters, postoperative refraction was within +/- 1.00 diopter in 19 (65%) of 29 eyes. In eyes with baseline refraction of -12.10 to -20.00 diopters, postoperative refraction was +/- 1.00 diopter in eight (43%) of 19 eyes. Overall, 64 (72.8%) of 88 eyes had a refraction within +/- 1.00 diopter after surgery. Between three weeks and five months after surgery the change in the mean spherical equivalent refraction was -0.61 diopter in the myopic direction. Uncorrected visual acuity after surgery was 20/20 or better in 31 eyes (36%) and 20/40 or better in 61 eyes (71%). Three eyes (3.6%) lost two lines or more of spectacle-corrected visual acuity, two from progressive myopic maculopathy and one from irregular astigmatism. No eyes had vision-threatening complications. CONCLUSION: Excimer laser in situ keratomileusis under a corneal flap can be an effective method of reducing myopia between -2.00 and -20.00 diopters, with minimal complications. Current surgical algorithms need modification to improve predictability of outcome. Stability of refraction after surgery requires further study.     

A case of spontaneous resolution of nonimmune hydrops fetalis

Indoor average 222Rn concentrations have been measured in more than 400 houses in Istanbul city. The average radon concentrations in the houses were found to be between 10 Bq m-3 and 260 Bq m-3 and the mean value was 50 Bq m-3.     

Sialidosis and galactosialidosis as the cause of non-immunologic hydrops fetalis

Two cases of non-immunological hydrops fetalis (NIHF) presenting with massive ascites are reported; in both patients an oligosaccharid-pattern in the urine typical for sialidosis resp. galactosialidosis was found. The cerebral sonography of both patients showed streaky echo enhancement in the region of the thalamostriatal vessels, which was interpreted as calcification of the vessels. The courses of the patients were characterised by recurrent infections, hepatosplenomegaly and myoclonus. Relevant literature reports on a large variability in the clinical appearance of oligosaccharidoses. The diagnosis of sialidosis is confirmed in cultured fibroblasts by the deficiency of alpha-N-acetylneuraminidase and, in case of galactosialidosis by the additional lack of beta-galactosidase. The precise diagnosis in NIHF is of increasing interest for prenatal diagnostic as well as for neonatological management.     

Fetal supraventricular tachycardia complicated by hydrops fetalis: a role for direct fetal intramuscular therapy

Maternally administered digoxin for the treatment of fetal supraventricular tachycardia (SVT) complicated by hydrops fetalis may be ineffective secondary to poor transplacental drug transfer. We present our experience with eight pregnancies treated with transplacental therapy or combined maternal and direct fetal intramuscular therapy. Response to treatment following maternal intravenous administration (MIV) of digoxin or a combination of fetal intramuscular (FIM) digoxin and MIV is described for eight hydropic fetuses during nine successful pharmacologic conversions. The MIV digoxin was administered using standard loading and maintenance protocols. FIM was administered at a dose of 88 micrograms/kg q 12-24 hours, to a maximum of three injections in the fetal buttock. Time to onset of the first two hours of sinus rhythm (TO2 degrees), time to onset > 90% sinus rhythm (TO > 90%), and time to resolution of hydrops fetalis (HF) were noted. The mean heart rate was 257 +/- 36 beats/minute and the mean gestational age was 29 +/- 4.8 weeks. Fetal SVT was due to a reentrant mechanism in all cases. For the three fetuses that underwent successful cardioversion following MIV digoxin (all required additional maternal antiarrhythmic drugs), TO2 degrees was 145 +/- 114 hours, TO > 90% was 176 +/- 55 hours, and HF resolved in 41 +/- 37 days. Initial combined FIM and MIV therapy in four fetuses resulted in a TO2 degrees of 5.5 +/- 4 hours, TO > 90% of 22 +/- 14 hours, and resolution of HF in 25 +/- 21 days. For the two failed cardioversions with transplacental treatment alone (one fetus had recurrent SVT with hydrops after initial successful cardioversion with MIV), TO2 degrees was 203 +/- 180 hours and TO > 90% was 313 +/- 270 hours. Once FIM was begun in these fetuses, TO2 degrees was 17 +/- 7 hours and TO > 90% was 60 +/- 13 hours; HF resolved in 45 days in one fetus, whereas the other fetus never had resolution of hydrops despite 100 days of antiarrhythmic therapy. Direct fetal intramuscular injection of digoxin combined with transplacental therapy appears to shorten the time to initial conversion of SVT and to sustain sinus rhythm in the fetus with SVT complicated by hydrops fetalis.     

Immunohistochemical distribution of surfactant apoproteins in hypoplastic lungs of nonimmunologic hydrops fetalis

Forty-three patients with nonimmunologic hydrops fetalis (NIHF), including 32 patients (74%) with hypoplastic lung, were immunohistochemically examined for the expression of surfactant apolipoproteins (SPs), using anti-gamma G immunoglobulins against human SP-A with a molecular weight (MW) of 35 K and SP-B with a MW of 5 K compared with that in 59 patients in a control group and 45 patients with hypoplastic lung induced by causes other than NIHF. In the control group, SP-A was expressed in the lungs from 23 gestational weeks and became more numerous and intense in alveolar type II cells after 31 gestational weeks, whereas SP-B began to be expressed from 20 gestational weeks, and almost all patients showed a diffuse positivity after 26 gestational weeks. In the NIHF group, SP-A expression was generally weak, even after 31 gestation weeks. Moreover, most of the patients showing a weak expression of SP-A were also associated with hypoplastic lung and had a clinical history of persistent intrauterine pleural effusion of more than 2 weeks. Conversely, the immunoreactivity of SP-B was well preserved in NIHF cases either with or without hypoplastic lung. These results suggest that in the NIHF lung, there is a possible delay in the functional maturation or development of SP-A synthesis by alveolar type II cells, and this retardation of the functional maturation in type II cells also participates in the postnatal respiratory insufficiency in NIHF.     

Prune-belly syndrome in a female, complicated by intestinal malrotation after successful antenatal treatment of hydrops fetalis

The authors report a female case of prune-belly syndrome having an antenatal sonographic diagnosis of hydrops fetalis and treated prenatally with transplacental digitalis administration. The findings of this case suggest that the clinical entity includes various phenotypes or aberrants having different origins.     

Systolic hypertension in critical aortic stenosis and the effect of valve replacement

Isradipine (lomir) is a new highly selective arteriolar vasodilator of the dihydropyridine group, characterized by a slight cardiodepressive effect. The drug effect on the central hemodynamics and oxygen transport was studied after cardiovascular surgery. Arterial pressure and total peripheral vascular resistance were reliably reduced at the peak of the drug action, as was the left-ventricular stroke work and oxygen consumption. A tendency to increase of cardiac output and cardiac index was observed. Pulmonary vascular resistance and pressure in the pulmonary artery did not reliably change. The drug increased the intrapulmonary shunt.     

Surgical commissurotomy of the aortic valve: outcome of open valvotomy in neonates with critical aortic stenosis

The prevalence of the soil-transmitted helminthiases is extremely high in some rural areas in China. The endemic foci of these human parasites constitute one of the most neglected public health problems. A large scale survey on human parasites (1988-1992) was conducted in China. The prevalences of the soil-transmitted helminthiases in the 30 provinces, autonomous regions and municipalities were reported and correlated with ecological factors (Yu et al, 1994; Xu et al, 1995). In this paper, we re-examined and analyzed the reported results with a spatial pattern test statistic, the D statistic. The values of the D statistic indicated that the spatial correlation of the prevalence of the soil-transmitted helminthiases in China was significant. We also found that the spatial correlation of the prevalence could largely be explained by the paddy field area per capita. The correlation of the prevalence of the soil-transmitted helminthiases and the paddy field area per capita was higher than the correlation of the prevalence with the factors found previously by Xu et al (1995).     

The safety of dipyridamole-thallium imaging in patients with critical aortic valve stenosis and angina

BACKGROUND: Screening pretransplantation recipient sera for percent panel reactive antibodies (%PRA) by an anti-human globulin (AHG) assay may identify recipients who are at risk for graft rejection or development of posttransplantation coronary artery disease. However, the pretransplantation AHG-%PRA does not always correlate with the occurrence of graft rejection or coronary artery disease. METHODS: We compared the predictive capacity of the AHG-%PRA with that of an enzyme-linked immunoassay (EIA)-based PRA assay that identifies immunoglobulin G bound to soluble human leukocyte antigen (sHLA) class I molecules from pooled platelets of 240 random donors (sHLA-EIA), and that of an EIA-based assay that detects immunoglobulin G anti-HLA class I antibodies bound to sHLA derived from individual HLA-typed cell cultures (PRA-STAT). The pretransplantation sera from 130 cardiac allograft recipients were comparatively tested and results evaluated. RESULTS: Although AHG-%PRA- and sHLA-EIA-determined PRA results were comparable, neither assay discriminated potential recipients at risk for rejection or coronary artery disease. However, cardiac allograft recipients with pretransplantation PRA-STAT sera > 10% were at risk for (1) graft rejection (77% vs 56%, p < .05); (2) more rejections/recipient (1.9 vs 1.0, p < .02); (3) graft rejection within 30 days (92% vs 38%, p < .001); or (4) development of coronary artery disease (48% vs 23%, p < .05) than recipients with pretransplantation PRA-STAT sera < 10%. CONCLUSIONS: PRA-STAT analysis of pretransplantation sera from potential cardiac allograft recipients may be more clinically informative about HLA alloimmunity and a better predictor of adverse clinical events than either AHG-%PRA- or sHLA-EIA-determined PRA.     

Subaortic stenosis caused by two discrete membranes

A case of subaortic stenosis caused by two discrete membranes is presented. Following demonstration of the two distinct sites of obstruction by intraventricular pressure gradients and angiocardiogram, the two membranes were excised through a transaortic approach. The importance of being aware of the occurrence of this rare anomaly is stressed, and its surgical implications are discussed.     

The critical care unit. In search of management

Administration of inducing doses of dexamethasone (10 microg/100 g) to adrenalectomized rats results, within 2-5 min, in import of the glucocorticoid receptor from liver cytoplasm into mitochondria, as demonstrated by Western blotting and by electron microscopy. Furthermore, glucocorticoid receptor (GR) synthesized in an in vitro reticulocyte system programmed with the respective mRNA, enters within minutes to added rat liver mitochondria in the form of intact GR, as demonstrated by Western blotting using either monoclonal or polyclonal antibodies against different domains of GR. In vitro studies show that the import is dependent on temperature and/or activation of the hormone-GR complex. These results, in connection with the presence in the human and rodent mitochondrial genome of sequences showing partial homology to the nuclear glucocorticoid response elements, support the hypothesis that the well documented effects of glucocorticoids on mitochondrial functions result from a direct interaction of the GR complex with the mitochondrial genome.     

Survival of a preterm neonate with late onset hydrops fetalis due to parvovirus B19 infection

This paper presents a case of non-immune hydrops (NIH) which presented late at 28 weeks' gestation during an otherwise asymptomatic pregnancy. A severely hydropic female infant was born with significant cardiorespiratory compromise; however, aggressive resuscitation and postnatal treatment resulted in a favourable outcome. Parvovirus B19 DNA was subsequently detected in postnatal blood samples by nested polymerase chain reaction. This case emphasizes that survival is achievable even in those cases where intrauterine transfusion is not performed. It highlights the importance of prompt delivery and intensive neonatal support in such situations.     

In utero decompression of umbilical cord angiomyxoma followed by vaginal delivery

An enlarging cystic-solid cord abnormality was found at prenatal identification to be an angiomyxoma. Partial in utero decompression of the 16 x 12 x 5 cm cystic component allowed uncomplicated spontaneous vaginal delivery at 36 weeks' gestation. Doppler studies had shown variable systolic/diastolic ratios in the solid component of the angiomyxoma.     

Echocardiographic hemodynamic and morphometric predictors of survival after two-ventricle repair in infants with critical aortic stenosis

OBJECTIVES: The purpose of this study was to identify echocardiographic hemodynamic and morphometric factors that would predict which infants with critical aortic stenosis could undergo relief of left ventricular outflow obstruction as opposed to the Norwood procedure. BACKGROUND: Echocardiographic predictors of survival in infants with critical aortic stenosis after two-ventricle repair have been mainly limited to morphometric factors, which have limitations. Echocardiographic hemodynamic predictors of survival in these patients have not previously been studied. METHODS: Doppler color flow mapping and pulsed Doppler techniques were used to obtain hemodynamic measurements of flow in the ascending, transverse and descending aorta, the ductus arteriosus, and across the aortic and mitral valves in infants with critical aortic stenosis. Morphometric measurements of the left heart structures were obtained, and comparisons were made between survivors and nonsurvivors for the hemodynamic and morphometric factors. RESULTS: Twenty-eight infants (mean age 1 +/- .6 days, mean weight 3.6 +/- .6 kg) with critical aortic stenosis were evaluated. Nineteen had a two-ventricle repair initially attempted, and nine had a Norwood operation. Among the patients with a two-ventricle repair, the hemodynamic factors associated with survival after two-ventricle repair included predominant or total antegrade flow in the ascending (p < 0.01) and transverse aorta (p < 0.05). Aortic valve gradient, mitral valve inflow and direction of flow in the ductus arteriosus and descending aorta were unrelated to outcome. The morphometric factors associated with survival after two-ventricle repair included the indexed aortic annulus (p < 0.0002), aortic root (p < 0.003), ascending aorta (p < 0.008) and left ventricular long-axis length (p < 0.01). Left ventricular volume, mass, ejection fraction and mitral valve area were not related to outcome after two-ventricle repair. CONCLUSIONS: In infants with critical aortic stenosis, predominant or total antegrade flow in the ascending and transverse aorta was associated with survival after two-ventricle repair. Determination of a one- versus two-ventricle repair remains a complex issue in infants with critical aortic stenosis. In addition to established morphometric predictors, hemodynamic information on the direction of flow in the aorta may help to define candidates for the Norwood operation.