A case of fetal intestinal volvulus without malrotation causing severe anemia.

Fetal intestinal volvulus without malrotation is a rare, life-threatening disease. Left untreated, hemorrhage from necrotic bowel tissue will lead to severe fetal anemia and even intrauterine death. We encountered a case of fetal intestinal volvulus causing severe anemia, which was diagnosed postnatally and successfully treated with surgical intervention.

Role of levothyroxine supplementation in extremely low birth weight infants who have transient hypothyroidism without thyroid-stimulating hormone elevation.

BACKGROUND: Preterm infants show transient hypothyroxinemia without thyroid-stimulating hormone (TSH) elevation. In addition, the degree of neurodevelopmental delay in preterm infants become severe according to the decreasing gestational age (GA). Because of the crucial role of thyroid hormones in brain development, hypothyroxinemia has the potential to cause developmental delay; however, the effectiveness of thyroxine (T4) supplementation on developmental outcomes remains controversial. To resolve these issues, we evaluated the clinical course of transient hypothyroxinemia and the effects of levothyroxine (LT4) supplementation in extremely low birth weight (ELBW) infants. METHODS: Serum levels of free T4 (FT4) and TSH were examined in 36 ELBW infants from 7 days after birth. LT4 (5-10 microg/kg/day) was orally administered to 18 of 36 infants with a low serum FT4 level (< 0.4 ng/dL) or normal serum FT4 levels and a clinical manifestation of hypothyroidism, whereas remaining 18 patients without a low serum FT4 level or clinical hypothyroidism were not given LT4 supplementation as control subjects. Infants were followed-up at a corrected age of 12 months, and clinical outcome was compared between infants that received LT4 and those that did not. RESULTS: ELBW infants showed low serum FT4 levels without TSH elevation. During hospitalization and at follow-up, LT4-administered infants with low serum FT4 levels showed a shorter GA compared with the control group. There were no other statistically significant differences in clinical outcomes at 12 months of corrected age between LT4-administered and control groups. CONCLUSIONS: Our results show that shorter GA is associated with lower serum FT4 levels. Shorter GA is known to cause developmental delay, however, LT4 supplementation prevents the developmental delay of ELBW infants with transient hypothyroxinemia.

PET imaging analysis with 64Cu in disulfiram treatment for aberrant copper biodistribution in Menkes disease mouse model.

UNLABELLED: Menkes disease (MD), an X-linked recessive disorder of copper metabolism caused by mutations in the copper-transporting ATP7A gene, results in growth failure and severe neurodegeneration in early childhood. Subcutaneous copper-histidine injection is the standard treatment for MD, but it has limited clinical efficacy. Furthermore, long-term copper injection causes excess copper accumulation in the kidneys, resulting in renal dysfunction. To attempt to resolve this issue, we used PET imaging with (64)Cu to investigate the effects of disulfiram on copper biodistribution in living mice serving as an animal model for MD (MD model mice). METHODS: Macular mice were used as MD model mice, and C3H/He mice were used as wild-type mice. Mice were pretreated with 2 types of chelators (disulfiram, a lipophilic chelator, and d-penicillamine, a hydrophilic chelator) 30 min before (64)CuCl2 injection. After (64)CuCl2 injection, emission scans covering the whole body were performed for 4 h. After the PET scans, the brain and kidneys were analyzed for radioactivity with γ counting and autoradiography. RESULTS: After copper injection alone, marked accumulation of radioactivity ((64)Cu) in the liver was demonstrated in wild-type mice, whereas in MD model mice, copper was preferentially accumulated in the kidneys (25.56 ± 3.01 percentage injected dose per gram [%ID/g]) and was detected to a lesser extent in the liver (13.83 ± 0.26 %ID/g) and brain (0.96 ± 0.08 %ID/g). Copper injection with disulfiram reduced excess copper accumulation in the kidneys (14.54 ± 2.68 %ID/g) and increased copper transport into the liver (29.42 ± 0.98 %ID/g) and brain (5.12 ± 0.95 %ID/g) of MD model mice. Copper injection with d-penicillamine enhanced urinary copper excretion and reduced copper accumulation in most organs in both mouse groups. Autoradiography demonstrated that disulfiram pretreatment induced copper transport into the brain parenchyma and reduced copper accumulation in the renal medulla. CONCLUSION: PET studies with (64)Cu revealed that disulfiram had significant effects on the copper biodistribution of MD. Disulfiram increased copper transport into the brain and reduced copper uptake in the kidneys of MD model mice. The application of (64)Cu PET for the treatment of MD and other copper-related disorders may be useful in clinical settings.

Oxidative 3,3,3-trifluoropropylation of arylaldehydes.

A reaction between (E)-trimethyl(3,3,3-trifluoroprop-1-en-1-yl)silane (1) and arylaldehydes 2 was triggered by fluoride anions to afford aryl 3,3,3-trifluoropropyl ketones 3 in moderate to good yield. A mechanistic study of this reaction indicated that it occurred via an allyl alkoxide (4). A subsequent 1,3-proton shift of the benzylic proton of 4 forms 3. This reaction involves oxidative 3,3,3-trifluoropropylation of an arylaldehyde to afford 4,4,4-trifluoro-1-arylbutan-1-one.

Simple synthesis of ß-trifluoromethylstyrenes using (E)-trimethyl-(3,3,3-trifluoroprop-1-enyl)silane.

(E)-trimethyl-(3,3,3-trifluoroprop-1-enyl)silane (1) was synthesized as a reagent for use in Hiyama cross-coupling reactions for the production of ß-trifluoromethylstyrene derivatives. Cross-coupling of 1 with electronically diverse aryl iodides was achieved by treatment with CsF in the presence of catalytic amounts of palladium to afford the desired products in moderate to good yields.