Replacement Therapy for Gaucher Disease during Pregnancy: A Case Report

none4noGaucher disease is a lysosomal storage disorder due to deficiency of glucocerebrosidase enzyme. In this study, a case of enzyme-treated woman during her pregnancy was reported.Giannubilo, Stefano Raffaele; Pasculli, Angela; Tidu, Elisa; Ciavattini, AndreaGiannubilo, Stefano Raffaele; Pasculli, Angela; Tidu, Elisa; Ciavattini, Andre

The Multifaced Actions of Curcumin in Pregnancy Outcome

Curcumin, also known as diferuloylmethane, is the main polyphenolic substance present in the rhizomes of Curcuma longa L. This plant showed many beneficial effects and has been used since ancient times for both food and pharmaceutical purposes. Due to its pleiotropic functions, curcumin consumption in the human diet has become very common thanks also to the fact that this natural compound is considered quite safe as it does not have serious side effects. Its functions as an anti-inflammatory, anti-oxidant, neuroprotective, immunomodulatory, anti-toxicant, anti-apoptotic, and anti-diabetic compound are already known and widely demonstrated. There are numerous studies concerning its effects on various human pathologies including cancer, diabetes and arthritis while the studies on curcumin during pregnancy have been performed only in animal models. Data concerning the role of curcumin as anti-inflammatory compound suggest a possible use of curcumin in managing pregnancy complications such as Preeclampsia (PE), Gestational Diabetes Mellitus (GDM), Fetal Growth Restriction (FGR), PreTerm Birth (PTB), and exposure to toxic agents and pathogens. The aim of this review is to present data to support the possible use of curcumin in clinical trials on human gestation complications

HELLP Syndrome and Differential Diagnosis with Other Thrombotic Microangiopathies in Pregnancy

Thrombotic microangiopathies (TMAs) comprise a distinct group of diseases with different manifestations that can occur in both pediatric and adult patients. They can be hereditary or acquired, with subtle onset or a rapidly progressive course, and they are particularly known for their morbidity and mortality. Pregnancy is a high-risk time for the development of several types of thrombotic microangiopathies. The three major syndromes are hemolysis, elevated liver function tests, and low platelets (HELLP); hemolytic uremic syndrome (HUS); and thrombotic thrombocytopenic purpura (TTP). Because of their rarity, clinical information and therapeutic results related to these conditions are often obtained from case reports, small series, registries, and reviews. The collection of individual observations, the evolution of diagnostic laboratories that have identified autoimmune and/or genetic abnormalities using von Willebrand factor post-secretion processing or genetic–functional alterations in the regulation of alternative complement pathways in some of these TMAs, and, most importantly, the introduction of advanced treatments, have enabled the preservation of affected organs and improved survival rates. Although TMAs may show different etiopathogenesis routes, they all show the presence of pathological lesions, which are characterized by endothelial damage and the formation of thrombi rich in platelets at the microvascular level, as a common denominator, and thrombotic damage to microcirculation pathways induces “mechanical” (microangiopathic) hemolytic anemia, the consumption of platelets, and ischemic organ damage. In this review, we highlight the current knowledge about the diagnosis and management of these complications during pregnancy

Analysis of deceleration area of fetal heart rate during the second stage of labor and its relation to acidaemia at delivery.

Objective To determine the value of deceleration area of fetal heart rate as indicator of low arterial pH and a significative fetal distress during the second stage of labor. Study design A retrospective analysis of deceleration area of fetal heart rate recordings in 171 women with a physiologic pregnancy, was performed for the last hour preceding delivery. All the pregnancies progressed to term and did not require induction of labor. The deceleration area was calculated, after digital analysis, with Autocad System 2004 with FHR from 140 to 120 bpm. The accordance analysis and the quantification of threshold value of the deceleration area, indicative of fetal acidaemia, were calculated by unvaried (linear regression) analysis with statistical significance set at p < 0.05. Results Stepwise and multiple regression analysis showed a statistical significant association between umbilical artery pH, Hct, PO2, PCO2 and SO2 at birth used as the dependent variable and the deceleration area, calculated at 140 bpm, at 130 bpm and 120 bpm, in the last 60 and 30 minutes of labor, used as the indipendent variable. Stepwise regression showed that the number of deceleration had an indipendent statistically significant association with umbilical artery pH and Hct. The quantification study of threshold value of deceleration area, indicative of fetal acidaemia, evidenced that during an antenatal fetal heart rate, recorded for 60 and 30 minutes before delivery, total deceleration area was ”d84.41 cm2 and ”d60.12 cm2 for FHRb at 140 bpm, ”d73.93 cm2 and ”d44.36 cm2 for FHRb at 130 bpm and ”d35.50 cm2 and ”d32.22 cm2 for FHRb at 120 bpm (p < 0.001). Conclusion We propose a new and very simple methodological approach to support classical cardiotocography which will be able not only to calculate the cut-off of fetal wellbeing at each FHRb, but also let us know about the fetus capacity to compensate any hypoxical insult