Prophylactic use of levosimendan in preoperative setting for surgical repair of congenital heart disease in children.

Introduction: Low cardiac output syndrome (LCOS) is a significant cause of morbidity and the leading cause of mortality after pediatric cardiac surgery. Levosimendan has been shown safe and effective in pediatrics to treat LCOS. We aimed to review our local strategy with preoperative prophylactic Levosimendan infusion to minimize LCOS after heart surgery in identified high-risk patients. Methods: Retrospective monocentric study. As there is no reliable cardiac output measurement in children, we recorded hemodynamic parameters as surrogates of cardiac output after extracorporeal circulation through an electronic patient survey system at different time points. Results: Seventy-two children received Levosimendan before surgery between 2010 and 2019. As expected, most patients were newborns and infants with prolonged open-heart surgeries. Median cardiopulmonary bypass time was 182 [137-234] min, and aortic clamping time was 95 [64-126] min. The postoperative hemodynamic parameters, vasoactive-inotropic score, and urine output remained stable throughout the first 48 h. Only a tiny portion of the patients had combined surrogate markers of LCOS with a maximal median arterial lactate of 2.6 [1.9-3.5] mmol/L during the first six postoperative hours, which then progressively normalized. The median arterio-venous difference in oxygen saturation was 31 [23-38] % between 12 and 18 h post-surgery and gradually decreased. The median venous-to-arterial CO2 difference was the highest at 10 [7-12] mmHg between 12 and 18 h post-surgery. Nine patients (13%) required extracorporeal membrane oxygenation. No patient required dialysis or hemofiltration. Mortality was 0%. Conclusion: Before congenital heart surgery, preoperative prophylactic administration of Levosimendan seems effective and safe for decreasing occurrence and duration of LCOS in high-risk children.

Acute life-threatening presentation of vitamin D deficiency rickets.

CONTEXT: Clinical manifestations of vitamin D deficiency rickets are widely described; however cardiorespiratory arrest is an extremely rare presentation. OBJECTIVE: The aim of this paper is to present the symptoms of severe vitamin D deficiency rickets and to highlight the importance of vitamin D prophylaxis in infants. RESULTS: We report a case of a 16-month-old infant who presented to emergency room with a stridor that evolved into a full cardiorespiratory arrest secondary to hypocalcemia. Medical history revealed that the infant was exclusively breastfed without vitamin D supplementation until the age of 10 months. Due to cultural habits, his diet was also grossly deficient in dairy products. Physical exam revealed clinical signs of rickets. Laboratory test showed severe hypocalcemia, elevated alkaline phosphatase, normal serum phosphorous, decreased 25(OH) cholecalciferol, increased intact parathyroid hormone level, and normal urine calcium excretion. The radiography of the wrist showed evidence of cupping, fraying, metaphyseal widening, and demineralization of the distal radial and ulnar metaphyses. The bone mineral density of the lumbar spine measured by dual x-ray absorptiometry showed a Z-score below -2 SD. His cardiorespiratory arrest secondary to hypocalcemia was therefore attributed to severe nutritional rickets. CONCLUSION: Vitamin D deficiency rickets can be life threatening. Vitamin D supplementation is therefore crucial, especially in breastfed infants and some ethnic minorities (dark-skinned people, poor sun exposure), more at risk for developing severe rickets if not supplemented.

Sperm of Doradidae (Teleostei: Siluriformes).

Spermatic characteristics were studied in 10 species representing several distinct groups within the catfish family Doradidae. Interestingly, different types of spermatogenesis, spermiogenesis and spermatozoa are correlated with intrafamilial groups previously proposed for Doradidae. Semi-cystic spermatogenesis, modified Type III spermiogenesis, and biflagellate sperm appear to be unique within Doradidae to the subfamily Astrodoradinae. Other doradid species have sperm with a single flagellum, cystic spermatogenesis, and spermiogenesis of Type I (Pterodoras granulosus, Rhinodoras dorbignyi), Type I modified (Oxydoras kneri), or Type III (Trachydoras paraguayensis). Doradids have an external mode of fertilization, and share a few spermatic characteristics, such as cystic spermatogenesis, Type I spermiogenesis and uniflagellate sperm, with its sister group Auchenipteridae, a family exhibiting sperm modifications associated with insemination and internal fertilization. Semi-cystic spermatogenesis and biflagellate spermatozoa are also found in Aspredinidae, and corroborate recent proposals that Aspredinidae and Doradoidea (Doradidae+Auchenipteridae) are sister groups and that Astrodoradinae occupies a basal position within Doradidae. The co-occurrence in various catfish families of semi-cystic spermatogenesis and either biflagellate spermatozoa (Aspredinidae, Cetopsidae, Doradidae, Malapturidae, Nematogenyidae) or uniflagellate sperm with two axonemes (Ariidae) reinforces the suggestion that such characteristics are correlated. Semi-cystic spermatogenesis and biflagellate sperm may represent ancestral conditions for Loricarioidei and Siluroidei of Siluriformes as they occur in putatively basal members of each suborder, Nematogenyidae and Cetopsidae, respectively. However, if semi-cystic spermatogenesis and biflagellate sperm are ancestral for Siluriformes, cystic spermatogenesis and uniflagellate sperm have arisen independently in multiple lineages including Diplomystidae, sister group to Siluroidei.

Differences in prey capture in grass shrimp, Palaemonetes pugio, collected along an environmental impact gradient.

The waterways and associated salt marshes along the western border of Staten Island, New York (Arthur Kill) have long been under environmental duress. Environmental threats include industrial and municipal discharges, oil spills, and possible leachate from landfills. These impacts are compounded due to the low flushing of this body of water. Grass shrimp, Palaemonetes pugio, inhabiting the Arthur Kill are, therefore, potentially at risk of exposure to metal and organic pollutants. Successful prey capture (of live brine shrimp, Artemia franciscana) was used to compare the relative health of shrimp collected from three sites along an environmental impact gradient. Study sites included a relatively unimpacted harbor (Great Kills Harbor, GK) and two creeks adjoining the Arthur Kill (Nassau Creek, NC, and Richmond Creek, RC). Shrimp originating from GK exhibited a rate of prey capture (6.3 prey h(-1)) that was about two times greater (p < 0.05) than that of shrimp originating from a creek behind a series of landfills (RC, 3.2 prey h(-1)). The rate of prey capture for shrimp collected from a creek impacted by historic smelting activities (NC) was intermediate (5.4 prey h(-1)). Laboratory studies with shrimp from a pristine site (Tuckerton, NJ) exposed to RC conditions (i.e., sediment and water) for eight weeks indicate that reduced prey capture can be induced in healthy shrimp. Finally, video analysis suggests that reduced prey capture in RC shrimp may not be the result of less effort, but rather the combination of (1) 80% fewer (p < 0.05) prey being captured with a lunge type of attack and (2) a greater reliance (p < 0.05) on a less efficient grab type of foraging behavior (64% success rate for RC versus 87% success rate for GK; p = 0.058). These results indicate that sublethal toxicity in environmentally impacted populations can occur and that prey capture may be used to assay the relative health of field specimens. Additionally, impaired prey capture may have important implications for the energy flow within impacted environments.

Transcriptional regulation of the TFIIH transcription repair components XPB and XPD by the hepatitis B virus x protein in liver cells and transgenic liver tissue.

Human hepatitis B virus is a risk factor for the development of hepatocellular carcinoma. The hepatitis B virus x protein (HBx) has been shown to inactivate the p53 tumor suppressor protein and impair DNA repair, cell cycle, and apoptosis mechanisms. Herein we report that HBx represses two components of the transcription-repair factor TFIIH, XPB (p89), and XPD (p80), both in p53-proficient and p53-deficient liver cells. This inhibition is observed while HBx maintains its transactivation function. Expression of HBx in liver cells results in down-regulation of endogenous XPB and XPD mRNAs and proteins; this inhibition is not observed with other TFIIH subunits, XPA or PCNA. In liver tissue from HBx transgenics, XPB and XPD proteins are down-regulated in comparison to matched normal liver tissue. HBx has been shown to interact with Sp1 transcription factor and affects its DNA binding activity. Sp1 is essential for the basal promoter activity of XPB in liver cells and Drosophila SL2 cells. In the Sp1-deficient SL2 cells, HBx-induced XPB and XPD inhibition is Sp1-dependent. In summary, our results provide evidence that HBx represses the expression of key TFIIH proteins at least in part through Sp1 elements; this repression may impair TFIIH function in DNA repair mechanisms.