scholarly journals P1803EFFICACY, SAFETY AND IMPACT ON RENAL FUNCTIONALITY OF ACETAZOLAMIDE TREATMENT IN PATIENTS WITH PMM2-CDG (AZATAX CLINICAL TRIAL)

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Mercedes Serano Guimaré ◽  
Pedro Arango Sancho ◽  
Víctor López Baez ◽  
Elena Codina Sampera ◽  
Yolanda Calzada Baños ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Renal Ultrasound ◽  
Second Phase ◽  
Protein Loss ◽  
Acid Base Balance ◽  
Base Balance ◽  
Renal Prognosis ◽  
Intellectual Deficit ◽  
Glycosylation Defect

Abstract Background and Aims Deficit of phosphomanomutase-(PMM2-CDG) is the most frequent congenital N-glycosylation defect, producing cerebellar syndrome with intellectual deficit and “stroke-like” episodes. It has been associated with renal alterations such as proteinuria, diffuse cortical hyperechogenicity and malformations. We evaluated efficacy, safety and renal repercussion of acetazolamide as a new therapeutic tool. Method First clinical trial in phase II including PMM2-CDG patients (5-21-years). First phase: 6 months treatment group with acetazolamide. Second phase: 5 weeks randomized withdrawal in responders (acetazolamide vs placebo), assessing renal functionality and effects of this medication. Dosing acetazolamide by 8 to 30mg/kg/day in 2-3 doses. Controls were performed at 3, 6, 14, 25 and 30 weeks determining acid-base balance, ionogram, renal function (creatinine) and Pr/Cr, Ca/Cr index and B2-microglobulin in first morning urine. All patients underwent in a bone densitometry study and renal ultrasound. Results 24 patients were included (mean age 12.3 ± 4.5 years). Bicarbonate levels and plasma pH were significantly lower at week 25(p <0.001). 13 patient needs to reduce acetazolamide dose due to excessive metabolic acidosis or asthenia. They showed a decrease in sodium (p=0.06), potassium (p<0.001) and serum calcium(p=0.030), although maintained in low normality limit with a decrease in protein loss(p=0.019) and increase in calcium/creatinine index(p=0.025) without B2-microglobulin alterations. The previously ultrasound renal described findings; the cortical hyperechogenicity was observed in 8.4% without renal dysfunction or associated nephrocalcinosis. One patient presented microlithiasis and another, symptomatic renal lithiasis. Densitometric study 69% of patients presented values in the range of osteopenia at the end of trial (-0.9 to -4.9SD, average of -2.36SD). Conclusion The efficacy of acetazolamide in the neurological symptoms of PMM2-CDG, due to an enzymatic stimulation mechanism mediated by acidosis, generates the possibility of chronic treatment with the drug in this group of patients, with possible renal adverse effects associated with long-term, overshadowing the skeletal and renal prognosis.

The Effect of Long-term Topiramate Treatment on Acid-base Balance in Children with Epilepsy

2016 ◽  
Vol 24 (3) ◽  
pp. 116-121
Author(s):  
김지용 ◽  
남상욱 ◽  
김영미 ◽  
이윤진 ◽  
이훈상 ◽  
...  
Keyword(s):  
Acid Base Balance ◽  
Acid Base ◽  
Base Balance

Long-term regulation of renal Na-dependent cotransporters and ENaC: response to altered acid-base intake

2000 ◽  
Vol 279 (3) ◽  
pp. F459-F467 ◽  
Author(s):  
Gheun-Ho Kim ◽  
Stephen W. Martin ◽  
Patricia Fernández-Llama ◽  
Shyama Masilamani ◽  
Randall K. Packer ◽  
...  
Keyword(s):  
Collecting Duct ◽  
Na Channel ◽  
Thick Ascending Limb ◽  
Acid Base Balance ◽  
Acid Base ◽  
Opposite Pattern ◽  
Α Subunit ◽  
Base Balance ◽  
Acid Loading

Increased systemic acid intake is associated with an increase in apical Na/H exchange in the renal proximal tubule mediated by the type 3 Na/H exchanger (NHE3). Because NHE3 mediates both proton secretion and Na absorption, increased NHE3 activity could inappropriately perturb Na balance unless there are compensatory changes in Na handling. In this study, we use semiquantitative immunoblotting of rat kidneys to investigate whether acid loading is associated with compensatory decreases in the abundance of renal tubule Na transporters other than NHE3. Long-term (i.e., 7-day) acid loading with NH4Cl produced large decreases in the abundances of the thiazide-sensitive Na-Cl cotransporter (TSC/NCC) of the distal convoluted tubule and both the β- and γ-subunits of the amiloride-sensitive epithelial Na channel (ENaC) of the collecting duct. In addition, the renal cortical abundance of the proximal type 2 Na-dependent phosphate transporter (NaPi-2) was markedly decreased. In contrast, abundances of the bumetanide-sensitive Na-K-2Cl cotransporter of the thick ascending limb and the α-subunit of ENaC were unchanged. A similar profile of changes was seen with short-term (16-h) acid loading. Long-term (7-day) base loading with NaHCO3resulted in the opposite pattern of response with marked increases in the abundances of the β- and γ-subunits of ENaC and NaPi-2. These adaptations may play critical roles in the maintenance in Na balance when changes in acid-base balance occur.

scholarly journals Prognostic value of base excess as indicator of acid-base balance in acute heart failure

2020 ◽  
Vol 9 (5) ◽  
pp. 399-405 ◽  
Author(s):  
Hiroki Nakano ◽  
Toshiyuki Nagai ◽  
Yasuyuki Honda ◽  
Satoshi Honda ◽  
Naotsugu Iwakami ◽  
...  
Keyword(s):  
Heart Failure ◽  
Confidence Interval ◽  
Acute Heart Failure ◽  
Base Excess ◽  
Acid Base Balance ◽  
Acid Base ◽  
Heart Failure Patients ◽  
Base Balance ◽  
High Base

Background: Acid-base balance can change as a result of pulmonary oedema and low tissue perfusion in acute heart failure patients. However, its long-term prognostic significance remains to be clarified. Methods: We prospectively examined a cohort of 472 consecutive acute heart failure patients who underwent arterial blood gas analysis on admission between January 2013 and May 2016. Acidaemia, alkalaemia and normal range of base excess were defined as pH <7.38, >7.42 and −2 to 2 mEq/L, respectively. The primary outcome was all-cause death. Results: During a median follow-up period of 714 days, 101 patients died. Although there was no difference in mortality among patients with acidaemia, normal pH and alkalaemia ( p = 0.92), patients with high base excess had the highest mortality compared with others. Multivariable Cox proportional hazard models revealed that high base excess was an independent determinant of mortality (hazard ratio 1.83, 95% confidence interval 1.08–3.13 (high versus normal base excess), hazard ratio 0.81, 95% confidence interval 0.47–1.41 (low versus normal base excess)), even after adjustment for significant prognostic covariates. Furthermore, regarding mortality stratified by base excess and carbon dioxide partial pressure (pCO2), patients with high base excess (>2.1 mEq/L) and high pCO2 (>40 mmHg) had the highest mortality compared with others. Conclusions: High base excess, but not low base excess, on admission was associated with long-term mortality in acute heart failure patients, indicating the importance of evaluating acid-base balance on admission by base excess for stratifying the risk of mortality in patients with acute heart failure.

scholarly journals Molecular Evolution of Podocyte Slit-diaphragm Proteins

2020 ◽  
Author(s):  
NSK Mulukala ◽  
V Kambhampati ◽  
SAH Qadri ◽  
AK Pasupulati
Keyword(s):  
Protein Complexes ◽  
Slit Diaphragm ◽  
Ankyrin Repeats ◽  
Protein Loss ◽  
Acid Base Balance ◽  
Src Homology 3 ◽  
Base Balance ◽  
Src Homology ◽  
Spfh Domain ◽  
First Time

AbstractVertebrates kidneys contribute to the homeostasis by regulating electrolyte, acid-base balance, and prevent protein loss into the urine. Glomerular podocytes constitute blood-urine barrier and podocyte slit-diaphragm, a modified tight junction contributes to the glomerular permselectivity. Nephrin, podocin, CD2AP, and TRPC6 are considered to be crucial members, which largely interact with each other and contribute to the structural and functional integrity of the slit-diaphragm. In this study, we analyzed the distribution of these four-key slit-diaphragm proteins across the organisms for which the genome sequence is available. We found that nephrin has a diverse distribution ranging from nematodes to higher vertebrates whereas podocin, CD2AP, and TRPC6 are predominantly restricted to the vertebrates. In the invertebrates nephrin and its orthologs consist of more immunoglobulin-3 and immunoglobulin-5 domains when compared to the vertebrates wherein, CD80-like C2-set Ig2 domains were predominant. Src Homology-3 (SH3) domain of CD2AP and SPFH domain of podocin are highly conserved among vertebrates. Although the majority of the TRPC6 and its orthologs had conserved ankyrin repeats, TRP, and ion transport domains, the orthologs of TRPC6 present in Rhincodon typus and Acanthaster planci do not possess the ankyrin repeats. Intrinsically unstructured regions (IURs), which are considered to contribute to the interactions among these proteins are largely conserved among orthologs of these proteins, suggesting the importance of IURs in the protein complexes that constitute slit-diaphragm. This study for the first time reports the evolutionary insights of vertebrate slit-diaphragm proteins and its invertebrate orthologs.

scholarly journals Tissue accumulation and the effects of long-term dietary copper contamination on osmoregulation in the mudflat fiddler crab Minuca rapax (Crustacea, Ocypodidae)

2020 ◽  
Author(s):  
MV Capparelli ◽  
JC McNamara ◽  
MG Grosell
Keyword(s):  
Carbonic Anhydrase ◽  
Atpase Activity ◽  
Fiddler Crab ◽  
Carbonic Anhydrase Activity ◽  
Copper Accumulation ◽  
Acid Base Balance ◽  
Dietary Copper ◽  
Base Balance ◽  
Copper Delivery

AbstractWe examined copper accumulation in the hemolymph, gills and hepatopancreas, and hemolymph osmolality, Na+ and Cl- concentrations, together with gill Na+/K+-ATPase and carbonic anhydrase activities, after dietary copper delivery (0, 100 or 500 µg Cu/g) for 12 days in a fiddler crab, Minuca rapax. In contaminated crabs, copper concentration decreased in the hemolymph and hepatopancreas, but increased in the gills. Hemolymph osmolality and gill Na+/K+-ATPase activity increased while hemolymph [Na+] and [Cl-] and gill carbonic anhydrase activity decreased. Excretion likely accounts for the decreased hemolymph and hepatopancreas copper titers. Dietary copper clearly affects osmoregulatory ability and hemolymph Na+ and Cl- regulation in M. rapax. Gill copper accumulation decreased carbonic anhydrase activity, suggesting that dietary copper affects acid-base balance. Elevated gill Na+/K+-ATPase activity appears to compensate for the ion-regulatory disturbance. These effects of dietary copper illustrate likely impacts on semi-terrestrial species that feed on metal contaminated sediments.

scholarly journals Modern anesthesia in obstetrics as a component of the concept of safe anesthesia

2020 ◽  
pp. 280-282
Author(s):  
R.O. Tkachenko
Keyword(s):  
Regional Anesthesia ◽  
Epidural Anesthesia ◽  
Local Anesthetic ◽  
Acid Base Balance ◽  
Combined Spinal Epidural ◽  
Rapid Effect ◽  
Base Balance ◽  
Combined Spinal Epidural Anesthesia ◽  
Spinal Epidural

Background. Anesthesia should be selected individually for each labor. Systemic analgesia of labor includes suggestive analgesia, narcotic analgesics, local infiltration and regional blockade, inhalation analgesia. It should be noted that there is no analgesic, sedative or local anesthetic that does not penetrate the placenta, affecting the fetus in any way. Objective. To describe modern anesthesia in obstetrics. Materials and methods. Analysis of literature sources on this issue. Results and discussion. Three groups of antispasmodics are used for analgesia: neurotropic (atropine, scopolamine), myotropic (papaverine, drotaverine) and neuromyotropic (baralgin). The main non-steroidal anti-inflammatory drugs used for this purpose include metamizole sodium, ketorolac tromethamine, diclofenac sodium. Inhalation autoanalgesia with nitrous oxide (N2O) is effective only in 30-50 % of women. When the concentration of N2O exceeds 50 %, the sedative effect increases and oxygenation decreases, which leads to the loss of consciousness and protective laryngeal reflexes. Such analgesia is indicated for low-risk patients who have refused from regional anesthesia. Epidural anesthesia (EDA) is the gold standard of labor anesthesia. The advantages of EDA include the option to change the degree of analgesia, the ability to continue pain relief until the end of labor and the minimal impact on the condition of both child and mother. Before manipulation, be sure to determine the platelet count and heart rate of the fetus. It is recommended to start EDA in the latent stage of labor. In patients with uterine scarring, early EDA is a mandatory component of medical care. The woman’s wish is the main indication for EDA. Indications for early catheterization of the epidural space include the presence of twins, preeclampsia, obesity, respiratory tract with special features. Headache is the most common complication of EDA. The use of pencil-point spinal needles minimizes the frequency of this complication. Adequate analgesia for uncomplicated labor should be performed with minimal concentrations of anesthetics with the least possible motor block. Local anesthetics (lidocaine, bupivacaine (Longocaine, “Yuria-Pharm”), ropivacaine) are used for EDA). Combined spinal-epidural anesthesia provides a rapid effect and long-term analgesia. For this purpose, 0.25 % Longocaine heavy (“Yuria-Pharm”) 2 mg and fentanyl 20 μg are administered intrathecally, followed by 0.225 % Longocaine 10 mg and fentanyl 20 μg epidurally. The technique of epidural dural puncture is a modification of combined spinal-epidural anesthesia. This technique improves the caudal spread of analgesia compared to the epidural technique without the side effects seen with spinal-epidural anesthesia. The ideal local anesthetic should be safe for both mother and fetus, provide sufficient analgesia with minimal motor block, and not affect labor process. A single spinal injection of opioids may be effective, but it should be limited in time. The use of systemic opioids during labor increases the need for resuscitation of newborns and worsens the condition of their acid-base balance compared to basic regional anesthesia. Catheter techniques can be used in case of the increased labor duration. Nalbuphine (“Yuria-Pharm”), which eliminates the side effects of regional anesthesia, can also be successfully used. Analgesic effect of paracetamol (Infulgan, “Yuria-Pharm”) in case of intravenous administration exceeds the analgetic effect of tramadol, and the effect on the newborn condition according to the Apgar scale does not differ (Meenakshi et al., 2015). Paracetamol (Infulgan) is moderately effective for perineal pain on the first day after delivery. The possibility of use during lactation is an another advantage of paracetamol. Conclusions. 1. Pain during labor is an extremely stressful factor, so women should have access to quality analgesia and anesthesia. 2. There is no analgesic, sedative or local anesthetic that does not penetrate the placenta, affecting the fetus. 3. EDA is the gold standard of labor anesthesia. 4. Combined spinal-epidural anesthesia provides rapid effect and long-term analgesia. 5. The use of systemic opioids during labor increases the need for resuscitation of newborns and worsens the condition of their acid-base balance. 6. Nalbuphine and Infulgan have been used successfully for labor pain relief.

scholarly journals THE INDICATORS OF BASAL METABOLISM IN INFLAMMATORY SYNDROME BACTERIAL ORIGIN: PREDICTORS OF PROGNOSIS OR THE NEED FOR TIMELY CORRECTION?

2019 ◽  
Vol 64 (2) ◽  
pp. 122-127
Author(s):  
Yu. L. Ketsko ◽  
A. V. Lunina ◽  
O. A. Gusyakova ◽  
E. V. Petrovskaya ◽  
A. V. Lyamin ◽  
...  
Keyword(s):  
Antibacterial Therapy ◽  
Nutritional Therapy ◽  
Basal Metabolism ◽  
Protein Loss ◽  
Acid Base Balance ◽  
Logistic Analysis ◽  
Therapy Strategy ◽  
Base Balance ◽  
Inflammatory Syndrome

The aim of the study was to determine the comparative significance of basal metabolism indicators and inflammatory process markers for the closest prognosis of patients in ICUs with systemic inflammation syndrome caused with infectious genesis. The paper presents the results of a retrospective analysis of the plan of treatment of 198 patients with confirmed clinical and laboratory signs of bacterial infection. The number of leukocytes, platelets, the percentage of lymphocytes, the concentration of procalcitonin, creatinine, bilirubin, C-reactive protein, acid-base balance values were determined in patients. Patients were assessed on a SOFA scale. The type of biomaterial for microbiological research was determined depending on the intended source of infectious inflammation. Identification of the isolated microorganisms was carried out using the MALDI ToF mass spectrometry method, followed by determination of antibiotic resistance. Empirical antibiotic therapy was prescribed upon admission of the patient to the ICU. Patients were switched to etiotropic antibacterial therapy after 48 hours, taking into account the results of a microbiological study. Determination of basal metabolic rate was carried out using the method of indirect calorimetry (CCM Express, Medical Graphics) with the calculation of basal metabolism, respiratory coefficient, absolute and relative amount of macronutrients. The calculation of the protein was made on the basis of the nitrogen level of the daily urine urea. Logistic analysis (ROC analysis) revealed that the antibacterial therapy strategy used, age, gender of patients, tinctorial properties of microorganisms isolated from patients, do not affect the nearest prognosis of the disease. On the contrary, SOFA score, the concentration of procalcitonin in the blood, and such parameters of the metabolic status as the need for kilocalories per kilogram of actual body weight and the percentage of protein calories significantly affect the nearest prognosis of the disease. The percentage of calories derived from protein metabolism, in the main exchange has a high reliable predictive value. The results of the study confirm the importance of adequate nutritional therapy in the treatment of patients with infectious inflammatory syndrome, including the calculation and correction of protein loss and total daily calorie.

scholarly journals Diseño e implementación de una aplicación móvil de realidad aumentada, para la enseñanza de química general en ingeniería

2021 ◽  
Vol 2 (4) ◽  
pp. 6246-6258
Author(s):  
Andrea de las Mercedes Arias Padilla ◽  
Tania Gloria Tapia Opazo ◽  
Natacha Alejandra Pino Acuña ◽  
Juan José Gutiérrez Álvarez
Keyword(s):  
Augmented Reality ◽  
Learning Process ◽  
New Technologies ◽  
Second Phase ◽  
Acid Base Balance ◽  
Chemistry Teaching ◽  
Nuevas Tecnologías ◽  
Base Balance ◽  
The Subject ◽  
Teaching Learning

La química es una ciencia básica que incluye conceptos abstractos, fenómenos y objetos que no pueden verse a simple vista, lo que dificulta un aprendizaje significativo y homogéneo en los estudiantes, además de obstaculizar la motivación en el proceso de aprendizaje de esta ciencia. Por lo anterior, este trabajo propone como objetivo general el diseño e implementación de una aplicación móvil con tecnología de Realidad Aumentada (RA) para utilizar como apoyo en el proceso de enseñanza-aprendizaje de la Química General en carreras de ingeniería de la Universidad de La Frontera, y que nos permita determinar el grado de motivación que genera en los estudiantes. La propuesta, tiene como primera etapa el diseño de la aplicación móvil con uso de RA en la temática de Equilibrio ácido-base, utilizando el motor de videojuegos multiplataforma Unity y el SDK de Vuforia, y una segunda etapa de prueba de la implementación a estudiantes de primer año de Ingeniería, donde además se aplicó una encuesta de percepción. En esta última, los estudiantes manifestaron una gran aceptación de la aplicación, además de señalar que la incorporación de nuevas tecnologías en la enseñanza de la química permite una mayor motivación e interés en la clase.   Chemistry is a basic science that includes abstract concepts, phenomena and objects that can not be seen with the naked eye. That  makes more difficult for the students to obtain a meaningful and homogeneous learning, and makes more difficult to get motivation in the Chemistry learning process. That is why the general objective of this work is the design and implementation of an application using augmented reality  in order to use the application as a complementary material in the Chemistry teaching-learning  process, in the subject General Chemistry for engineering careers in Universidad de la Frontera, and to determinate how motivated the students can be after using this application. The first phase in this process is the designing of the application using augmented reality with the subject acid-base balance, using the videogames multiplatform motor Unity and the Vuforia´s SDK. The second phase was  testing the application on first year engineer students in Universidad de la Frontera, it was also added a poll to have an opinion of them. They were satisfied with the application and declared that the use of new technologies increases the motivation for the subject.  

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