Cardiac pathophysiology in twin-twin transfusion syndrome: new insights into its evolution.

OBJECTIVES: In twin-twin transfusion syndrome (TTTS), unbalanced transfer of vasoactive mediators and fluid from the donor to the recipient cotwin alters their cardiovascular function. The aims of this study were to describe the impact of TTTS on fetal cardiac function in a large cohort of monochorionic-diamniotic (MCDA) pregnancies, and determine the early hemodynamic response to selective fetoscopic laser photocoagulation (SFLP). METHODS: Echocardiography was performed in 145 MCDA pregnancies, including 26 uncomplicated MCDA, 61 TTTS Stages I+II and 58 TTTS Stages III+IV pregnancies, prior to SFLP for TTTS. Echocardiographic data after SFLP were available in a subset of 41/119 (34%) TTTS cases at a mean of 1.7 ± 1.9 days. Mitral (MAPSE) and tricuspid (TAPSE) annular systolic excursion, myocardial performance index (MPI), tissue Doppler velocities (E', A', S') and filling pressures (E/E') were measured and transformed into Z-scores. Ventricular pressure was estimated from peak atrioventricular regurgitation velocity. RESULTS: Left ventricular hemodynamics of the recipient twin were affected in early TTTS. In all stages of TTTS, left MPI and E/E' of the recipient twin were elevated in comparison to those of the donor (all P < 0.05), with reduced recipient left S' in TTTS Stages III+IV (P < 0.001). Ventricular pressure was elevated for gestational age (median, 42 mmHg (range, 20-65 mmHg)) in 11 of 14 recipients in which this parameter was measured. Between-group difference in right ventricular E/E' was significant only in TTTS Stages III+IV recipients compared with TTTS Stages I+II (P = 0.007) and uncomplicated MCDA (P = 0.041). Recipient left and right MPI decreased while S', MAPSE and TAPSE increased after SFLP; in donors, left E/E' and cardiac output increased. CONCLUSIONS: Cardiac function of the recipient twin in TTTS is abnormal, even in the early stages of the disease. Left ventricular filling pressures are elevated and systolic function is decreased before abnormalities in the right heart become apparent. SFLP produces rapid hemodynamic improvement. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Sporulation in Bacillus subtilis is independent of membrane fatty acid composition.

Growth and sporulation of a Bacillus subtilis mutant deficient in branched fatty acid synthesis (gene symbol bfmB) were examined. The mutant, which produces an acyl-coenzyme A:acyl carrier protein transacylase with reduced affinity for branched fatty acid primers, could grow in media containing any one of a wide range of low-molecular-weight fatty acids having branched, cyclic, saturated, or unsaturated carbon chains. The fatty acid composition of cellular lipids depended on the compound used to support growth. Cultures of the bfmB mutant grown in the presence of 3-methylcrotonate contained an unusually high fraction (73%) of straight-chain fatty acids in the cellular lipids. The mutant sporulated with any one of the precursors of branched fatty acids in the medium; isolated spores contained mainly this branched fatty acid and only 10% or less straight-chain fatty acids regardless of the straight-chain fatty acid content of vegetative cells. Exceptional were spores grown in the presence of cyclobutane-carboxylic acid, which contained 28% straight-chain fatty acids. The branched fatty acid composition of spores could be modified greatly by changing the supply of precursors in the medium.

Biochemical and genetic characterization of an auxotroph of Bacillus subtilis altered in the Acyl-CoA:acyl-carrier-protein transacylase.

We have analyzed a mutation of Bacillus subtilis (bfmB) that results in an acyl-CoA:acyl-carrier-protein transacylase with low affinity for branched acyl-CoA substrates; it maps in the acf-hisH region of the chromosome. The aceA mutation, present in the parent of the bfmB mutant, causes a deficiency in pyruvate dehydrogenase and maps in the pycA-pyrA region. Strains carrying the bfmB mutation synthesize branched-chain fatty acids at a rate sufficient for normal growth only if branched acyl-CoA precursors are present in the medium. They grow well if the medium is supplemented with 0.1 mM 2-methylbutyrate, isobutyrate or isovalerate, or with 1.0 mM isoleucine or valine; leucine does not support growth. Growth supported by valine and isoleucine is inhibited by butyrate and other straight short-chain fatty acids at concentrations (0.1 mM) which do not inhibit growth of the standard strain; the inhibition is prevented by short branched fatty acids which are converted to long-chain fatty acids appearing as activity of B. subtilis is controlled by separate enzymatic sites for the acyl-CoA precursors of branched and straight-chain fatty acids. Whether these sites are contained in one or two enzymes is not known.

Continuous Cultivation for Apparent Optimization of Defined Media for Cellulomonas sp. and Bacillus cereus.

Steady-state continuous culture was used to optimize lean chemically defined media for a Cellulomonas sp. and Bacillus cereus strain T. Both organisms were extremely sensitive to variations in trace-metal concentrations. However, medium optimization by this technique proved rapid, and multifactor screening was easily conducted by using a minimum of instrumentation. The optimized media supported critical dilution rates of 0.571 and 0.467 h for Cellulomonas and Bacillus, respectively. These values approximated maximum growth rate values observed in batch culture.

Giant adrenal myelolipoma and testicular interstitial cell tumor in a man with congenital 21-hydroxylase deficiency.

The occurrence of a giant myelolipoma of the adrenal gland reported in a patient with congenital adrenal hyperplasia (21-hydroxylase deficiency). Associated significant findings include a massive proliferation of adrenocortical cells as an integral part of the myelolipoma and coincidental tumor of the interstitial cells of the testis. The clinical, radiologic, endocrinologic, and pathologic features of this case are correlated with a review of the literature. The additional myelolipomas are also reported here for the first time. Similar lesions have been induced experimentally in rats and provide further evidence suggesting a hyperplastic rather than a neoplastic nature for this complex lesion, at least in its earlier stages.

Clear cell sarcoma with melanogenesis.

An example of clear cell sarcoma, from the great toe of a young woman having an otherwise classic history and appearance is described, and a malignant giant cell component is reported for the first time. Histochemical and electron microscopic studies clearly demonstrate melanogenesis. The controversy over tissue of origin of this uncommon neoplasm is discussed and evidence in support of neurectodermal derivation is presented.