Liddle's syndrome (LS) is a rare heritable form of hypertension that often affects young patients. It is caused by gain-of-function mutations of the kidney epithelial sodium channel (ENaC) and it is classically associated with hypokalemia and suppression of renin and aldosterone. LS is characterized by responsiveness to ENaC inhibitors but not to mineralocorticoid receptor inhibitors. Consequently the most effective treatment is amiloride. This drug is not used in pregnancy, as it has not been sufficiently studied during gestation. However for pregnant LS patient amiloride is the most effective drug in decreasing blood pressure. Herein we report the case of a LS patient, who has been followed up by a multidisciplinary teamwork during her first pregnancy. Hypertension worsened after the 25th week of gestation and amiloride was safely administered, firstly in combination with hydrochlorothiazide (the only formulation commercially available in Italy) and, thereafter, as a single drug. Genetic testing was performed in the patient's family in order to support diagnosis and clinical management.
There exists a huge gap between protocols issued by scientific bodies and evidence derived by system biology studies on the multifactorial origin of threatened preterm delivery and their different associations with neonatal outcome. The objective of this prospective study was the analysis obstetrical and neonatal outcome in a cohort of pregnant patients treated for the risk of preterm delivery according to maternal and fetal assessment determined by amniotic fluid samples. Methods. Threatened preterm delivery and premature rupture of membranes between 24 + 1 and 32 + 6 weeks of gestation were treated by prolonged tocolytic regimens and if necessary by antibiotics for maternal infections when intra-amniotic inflammation (IAI) was excluded on the basis of negative white blood cell count in the amniotic fluid, or opposite, by delivery after a course of betamethasone and 48 hours maintenance tocolysis. Twenty-three cases were compared with 22 historical controls treated by the same teams according to the 48 hours treat and wait criteria. In addition to this, cases with normal and abnormal amniotic fluid white blood cell were compared. Results. Maternal and fetal conditions at admission were not significantly different between the study and control cohort for all maternal and fetal variables. Clinical indices were significantly improved as regard to latency from admission to delivery, number of newborns admitted to neonatal intensive care unit and length of stay in neonatal intensive care unit. Not any perinatal death or sepsis occurred in the study cohort. Overall, improved neonatal outcomes were observed in the study cohort. Composite major neonatal eventful outcomes occurred in 26% of cases vs. 50% in controls. The limited number of cases was not powered enough to reach a statistical significance for these variables. Continued tocolysis on demand and full regimen of mono or combined antibiotic regimen for maternal infection achieved significantly longer delay between admission to delivery with improved in neonatal outcome in cases negative for IAI: only 2 of 14 newborns suffered of major neonatal complications vs. 4 of 9 newborns delivered for IAI. Conclusions. Fetuses without IAI can be treated conservatively and their stay in utero prolonged without harm. However, we confirmed that when IAI is already active in utero a worse neonatal outcome is already partly predetermined. These positive findings must be interpreted with cautions given the limited number of cases considered by this study.
SUMMARY Length of biliary (CBD) and/or pancreatic (PD) sphincter of Oddi (SO) was measured during perendoscopic or intraoperative manometry in 21 control subjects and in 46 patients with biliary disease. When the high resting pressure (HPZ) and the phasic wave zone (PAZ) were considered, SO length was, in the control group, 9 5±0.5 mm (M±SE) and 9.4±0*6 mm at the level of the CBD-SO, and 7.7±O06 mm and 6-9±0*5 mm at the level of the PD-SO. The physiological sphincter length appeared substantially shorter than the anatomical one, as reported in the literature. No significant differences were found between controls and patients with CBD stones. Endoscopic sphincterotomy without manometry reduced mean sphincter length by 80-6±5-5% (mean±SE) and this was increased to 95-2±3.5% when the length of the sphincterotomy was tailored to the manometric findings. Surgical partial sphincterotomy reduced sphincter length by only 46-7±10-3%.The physiological length of the sphincter of Oddi (SO) may be measured manometrically. Manometric estimates vary from 3-14 mm,"W but this has not been systematically studied in man. In particular, information concerning the pancreatic sphincter is scanty.Autoptic (cast, histology, radiology) and x-ray in vivo measurements have reported that the biliary sphincter ranges from 8 to 20 or even 27 mm in length,9 the pancreatic sphincter from 3-5 to 18 mm57`0 and the ampullary sphincter from 0 to 12 mm.5 [7][8][9][10] Knowledge of SO length may be of interest in understanding pancreaticobiliary diseases and may be useful during sphincterotomy. The aim of the present work was therefore to measure SO length in both the biliary and the pancreatic tract. Control subjects and patients with biliary disease were examined by perendoscopic or intraoperative manometry, in some instances before and after papillotomy.
In this study the effects of glucagon 1–29 peptide and the metabolically inactive portion glucagon 1–21 have been evaluated on sphincter of Oddi (SO) motor activity. A triple lumen catheter perfused by a minimally compliant infusion system was used to record intraoddian pressures at endoscopy. A strictly blind evaluation of tracings was performed. Neither peptide administration appeared to induce any relevant variation of SO resting pressure nor frequency, amplitude and duration of phasic contractions. Data of the present study would not support the use of glucagon whenever a spasmolytic effect on the SO is looked for.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.