scholarly journals Distal renal tubular acidosis: a systematic approach from diagnosis to treatment

2021 ◽  
Author(s):  
Sabrina Giglio ◽  
Giovanni Montini ◽  
Francesco Trepiccione ◽  
Giovanni Gambaro ◽  
Francesco Emma
Keyword(s):  
Metabolic Acidosis ◽  
Renal Tubular Acidosis ◽  
Molecular Mechanisms ◽  
Clinical Symptoms ◽  
Genetic Disorder ◽  
Treatment Options ◽  
Current Treatment ◽  
Hydrogen Ions ◽  
Renal Tubular

AbstractRenal tubular acidosis (RTA) comprises a group of disorders in which excretion of hydrogen ions or reabsorption of filtered HCO3is impaired, leading to chronic metabolic acidosis with normal anion gap. In the current review, the focus is placed on the most common type of RTA, Type 1 RTA or Distal RTA (dRTA), which is a rare chronic genetic disorder characterized by an inability of the distal nephron to secrete hydrogen ions in the presence of metabolic acidosis. Over the years, knowledge of the molecular mechanisms behind acid secretion has improved, thereby greatly helping the diagnosis of dRTA. The primary or inherited form of dRTA is mostly diagnosed in infancy, childhood, or young adulthood, while the acquired secondary form, as a consequence of other disorders or medications, can happen at any age, although it is more commonly seen in adults. dRTA is not as “benign” as previously assumed, and can have several, highly variable long-term consequences. The present review indeed reports and summarizes both clinical symptoms and diagnosis, long-term outcomes, genetic inheritance, epidemiology and current treatment options, with the aim of shedding more light onto this rare disorder. Being a chronic condition, dRTA also deserves attention in the transition between pediatric and adult nephrology care, and as a rare disease it has a place in the European and Italian rare nephrological diseases network.

scholarly journals A clinical case of renal tubular acidosis type 1 in a 1-month old baby

2017 ◽  
Vol 98 (1) ◽  
pp. 129-131
Author(s):  
E V Voljanjuk ◽  
I J Lutfullin
Keyword(s):  
Metabolic Acidosis ◽  
Renal Tubular Acidosis ◽  
Rare Case ◽  
Metabolic Disorders ◽  
Hydrogen Ions ◽  
Distal Nephron ◽  
First Onset ◽  
Life Threatening ◽  
Renal Tubular

Renal tubular acidosis is a group of tubular diseases of the kidneys, whiсh are characterized by disorders of bicarbonate reabsorption, secretion of hydrogen ions, or a combination of both defects and cause metabolic acidosis with preserved glomerular filtration. Distal renal tubular acidosis is characterized by severe hyperchloraemic metabolic acidosis due to impaired excretion of hydrogen ions in the distal nephron. The prevalence of the primary distal renal acidosis is 1:40 000. Most often the first onset of the disease occurs at the age of 6 months to 2 years. The article presents a rare case of tubular acidosis type 1 in a child at the age of 1 month and 5 days. The presented case demonstrates that renal tubular acidosis can clinically manifest in children during the first months of life leading even at this age to severe metabolic disorders requiring certain raised level of suspicion for this pathology. Rarity of distant tubular acidosis is one of the factors predisposing to difficulty and tardiness of its diagnosis that leads to early disability and high risk of life-threatening conditions.

scholarly journals Encapsulating Peritoneal Sclerosis: Pathophysiology and Current Treatment Options

2019 ◽  
Vol 20 (22) ◽  
pp. 5765 ◽  
Author(s):  
Rajesh M. Jagirdar ◽  
Andreas Bozikas ◽  
Sotirios G. Zarogiannis ◽  
Maria Bartosova ◽  
Claus Peter Schmitt ◽  
...  
Keyword(s):  
Intestinal Obstruction ◽  
Animal Studies ◽  
Clinical Symptoms ◽  
Treatment Options ◽  
Renin Angiotensin System ◽  
Mtor Inhibitors ◽  
Current Treatment ◽  
Therapeutic Interventions ◽  
Ultrafiltration Failure

Encapsulating peritoneal sclerosis (EPS) is a life-threatening complication of long-term peritoneal dialysis (PD), which may even occur after patients have switched to hemodialysis (HD) or undergone kidney transplantation. The incidence of EPS varies across the globe and increases with PD vintage. Causative factors are the chronic exposure to bioincompatible PD solutions, which cause long-term modifications of the peritoneum, a high peritoneal transporter status involving high glucose concentrations, peritonitis episodes, and smoldering peritoneal inflammation. Additional potential causes are predisposing genetic factors and some medications. Clinical symptoms comprise signs of intestinal obstruction and a high peritoneal transporter status with incipient ultrafiltration failure. In radiological, macro-, and microscopic studies, a massively fibrotic and calcified peritoneum enclosed the intestine and parietal wall in such cases. Empirical treatments commonly used are corticosteroids and tamoxifen, which has fibrinolytic properties. Immunosuppressants like azathioprine, mycophenolate mofetil, or mTOR inhibitors may also help with reducing inflammation, fibrin deposition, and collagen synthesis and maturation. In animal studies, N-acetylcysteine, colchicine, rosiglitazone, thalidomide, and renin-angiotensin system (RAS) inhibitors yielded promising results. Surgical treatment has mainly been performed in severe cases of intestinal obstruction, with varying results. Mortality rates are still 25–55% in adults and about 14% in children. To reduce the incidence of EPS and improve the outcome of this devastating complication of chronic PD, vigorous consideration of the risk factors, early diagnosis, and timely discontinuation of PD and therapeutic interventions are mandatory, even though these are merely based on empirical evidence.

scholarly journals Comprehensive Review of Uterine Fibroids: Developmental Origin, Pathogenesis, and Treatment

2021 ◽  
Author(s):  
Qiwei Yang ◽  
Michal Ciebiera ◽  
Maria Victoria Bariani ◽  
Mohamed Ali ◽  
Hoda Elkafas ◽  
...  
Keyword(s):  
Molecular Mechanisms ◽  
Treatment Options ◽  
Uterine Fibroids ◽  
Current Treatment ◽  
Future Perspectives ◽  
Invasive Treatment ◽  
Treatment Interventions ◽  
Comprehensive Review ◽  
Non Invasive

Abstract Uterine fibroids are benign monoclonal neoplasms of the myometrium, representing the most common tumors in women worldwide. To date, no long-term or non-invasive treatment option exists for hormone-dependent uterine fibroids due to the limited knowledge about the molecular mechanisms underlying the initiation and development of uterine fibroids. This paper comprehensively summarizes the recent research advances on uterine fibroids, focusing on their risk factors, development origin, pathogenetic mechanisms, and treatment options. Additionally, we describe the current treatment interventions for uterine fibroids. Finally, future perspectives on uterine fibroids studies are summarized. Deeper mechanistic insights into tumor etiology and uterine fibroids’ complexity can contribute to the newer targeted therapies.

Acid-Base Disorders

2018 ◽  
Author(s):  
Aaron Skolnik ◽  
Jessica Monas
Keyword(s):  
Metabolic Acidosis ◽  
Renal Tubular Acidosis ◽  
Metabolic Alkalosis ◽  
Respiratory Acidosis ◽  
The Body ◽  
Anion Gap ◽  
Hydrogen Ions ◽  
Acid Base Balance ◽  
Acid Base ◽  
Renal Tubular

Under physiologic conditions, the acid-base balance of the body is maintained via changes in ventilation that eliminate carbon dioxide, buffering of acid loads, and renal excretion of hydrogen ions. Failure to maintain the pH of the blood between 7.35 and 7.45 can result in life-threatening conditions. This review details the pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes of acid-base disorders. Figures show the relationship between hydrogen ions and blood pH, proximal tubular bicarbonate reabsorption, the secretion of hydrogen ions, renal ammonia production, ammonium diffusion, metabolic alkalosis, electrocardiographic changes in hypokalemia and hyperkalemia, pseudoinfarction caused by hyperkalemia, and an algorithmic approach to suspected acid-base disorders. Tables list causes of high–anion gap metabolic acidosis, metabolic acidosis with a normal anion gap, type 1 renal tubular acidosis, type 4 renal tubular acidosis and aldosterone resistance, metabolic alkalosis, respiratory acidosis, and respiratory alkalosis; treatment of hyperkalemia; and a stepwise approach for the evaluation of suspected acid-base disorders. This review contains 9 highly rendered figures, 9 tables, 64 references, and a list of pertinent Web sites.

Acid-Base Disorders

2015 ◽  
Author(s):  
Aaron Skolnik ◽  
Jessica Monas
Keyword(s):  
Metabolic Acidosis ◽  
Renal Tubular Acidosis ◽  
Metabolic Alkalosis ◽  
Respiratory Acidosis ◽  
The Body ◽  
Anion Gap ◽  
Hydrogen Ions ◽  
Acid Base Balance ◽  
Acid Base ◽  
Renal Tubular

Under physiologic conditions, the acid-base balance of the body is maintained via changes in ventilation that eliminate carbon dioxide, buffering of acid loads, and renal excretion of hydrogen ions. Failure to maintain the pH of the blood between 7.35 and 7.45 can result in life-threatening conditions. This review details the pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes of acid-base disorders. Figures show the relationship between hydrogen ions and blood pH, proximal tubular bicarbonate reabsorption, the secretion of hydrogen ions, renal ammonia production, ammonium diffusion, metabolic alkalosis, electrocardiographic changes in hypokalemia and hyperkalemia, pseudoinfarction caused by hyperkalemia, and an algorithmic approach to suspected acid-base disorders. Tables list causes of high–anion gap metabolic acidosis, metabolic acidosis with a normal anion gap, type 1 renal tubular acidosis, type 4 renal tubular acidosis and aldosterone resistance, metabolic alkalosis, respiratory acidosis, and respiratory alkalosis; treatment of hyperkalemia; and a stepwise approach for the evaluation of suspected acid-base disorders. This review contains 9 highly rendered figures, 9 tables, 64 references, and a list of pertinent Web sites.

scholarly journals Biopsy-Proven Type 1 Renal Tubular Acidosis in a Patient with Metabolic Acidosis

2012 ◽  
Vol 27 (1) ◽  
pp. 119 ◽  
Author(s):  
Seok Hui Kang ◽  
Jin Kim ◽  
Jong Won Park
Keyword(s):  
Metabolic Acidosis ◽  
Renal Tubular Acidosis ◽  
Renal Tubular

scholarly journals Vulpinic Acid as a Natural Compound Inhibits the Proliferation of Metastatic Prostate Cancer Cell by Inducing Apoptosis

2021 ◽  
Author(s):  
Demet Cansaran Duman ◽  
Gamze Guney Eskiler ◽  
Betül Çolak ◽  
Elif Sozen Kucukkara
Keyword(s):  
Prostate Cancer ◽  
Metastatic Prostate Cancer ◽  
Molecular Mechanisms ◽  
Treatment Options ◽  
Annexin V ◽  
Current Treatment ◽  
Pcr Analysis ◽  
G1 Arrest ◽  
Advanced Analysis ◽  
Induced Apoptosis

Abstract Lichen secondary metabolites have drawn considerable attention in recent years due to limitations of current treatment options. Vulpinic acid (VA) obtained from Letharia vulpina lichen species exerts a remarkable cytotoxic effect on different cancer types. However, the therapeutic efficacy of VA in metastatic prostate cancer (mPC) cells has not been investigated. In the present study, we aimed to identify VA-mediated cytotoxicity in PC-3 mPC cells compared with control cells. After identification of the cytotoxic concentrations of VA, VA induced apoptosis was analyzed by Annexin V, cell cycle, acridine orange and propidium iodide staining and RT-PCR analysis. Our findings showed that VA significantly decreased the viability of PC-3 cells (p < 0.01) and caused a considerable early apoptotic effect through G0/G1 arrest, nuclear bleebing and the activation of particularly initiator caspases. Therefore, VA may be a potential treatment option for mPC patients. However, the underlying molecular mechanisms of VA-induced apoptosis with advanced analysis should be further performed.

scholarly journals Mycotoxins in Children’s Food: Problem and Halal Management

2019 ◽  
Vol 1 (1) ◽  
pp. 16-38
Author(s):  
Mosaad A. Abdel-Wahhab ◽  
Aziza A. El-Nekeety ◽  
Soher E. Aly
Keyword(s):  
Indoor Air ◽  
Clinical Symptoms ◽  
Bone Marrow Failure ◽  
Treatment Options ◽  
Pulmonary Hemorrhage ◽  
Childhood Exposure ◽  
Toxicological Effects ◽  
Food Knowledge ◽  
Contaminated Food

Mycotoxins are ubiquitous compounds found in the natural life cycle of food- producing plants. They have a range of diverse chemical and physical properties and toxicological effects on man and animal. Mycotoxins are considered the most important contaminants of the food chain due to their chronic adverse effects on health and the economy. Mycotoxins are known as the 21th century “Great Masquerader” due to its complex natural history involving different tissues and resembling different diseases at each stage in its evolution. Mycotoxins can induce a variety of clinical symptoms including epistaxis, conjunctivitis, coughing, apnea, wheezing, vomiting and nausea. Some mycotoxins induce acute pulmonary hemorrhage, bone marrow failure and pneumonia. Knowledge about these symptoms enables the clinician to ask questions for possible exposure to the main classes of mycotoxins to protect children from sources of such exposure. These sources may include food, clothes, furniture and indoor air at home. Early childhood exposure to mycotoxins may be critical determinants of later health effects. Exposure in utero and through early infancy may additionally be important. Several well-known diseases such as neural tube defects, liver and esophageal cancers are associated with the consumption of mycotoxin-contaminated food. Knowledge of previous short or long term exposure to mycotoxins may help paediatricians to more accurately diagnose and provide treatment options to children and their families. The current review discusses the problems associated with the occurrence of different common mycotoxins in children’s food and the possible halal strategies to counteract these problems.  

MICROSURGICAL TREATMENT OF SYMPTOMATIC SACRAL PERINEURIAL CYSTS

Neurosurgery ◽  
2007 ◽  
Vol 60 (6) ◽  
pp. 1059-1066 ◽  
Author(s):  
Dongsheng Guo ◽  
Kai Shu ◽  
Rudong Chen ◽  
Changshu Ke ◽  
Yanchang Zhu ◽  
...  
Keyword(s):  
Patient Outcomes ◽  
Treatment Options ◽  
Current Treatment ◽  
Cerebrospinal Fluid Leakage ◽  
Local Defect ◽  
Microsurgical Treatment ◽  
The Past ◽  
Return Visits

Abstract OBJECTIVE The aim of this study was to investigate the microsurgical results of symptomatic sacral perineurial cysts of 11 patients and to discuss the treatment options of the past 10 years. METHODS We retrospectively reviewed the records of 11 patients with symptomatic sacral perineurial cysts who underwent microsurgical treatment at Tongji Hospital, Huazhong University of Science and Technology from 1993 through 2006. The philosophy was to perform total or partial cyst wall removal, to imbricate the remaining nerve sheath if possible, and to repair local defect with muscle, Gelfoam (Pharmacia & Upjohn, Kalamazoo, MI), and fibrin glue. Patient outcomes were assessed by comparing the preoperative and postoperative examination results. The average follow-up time obtained from return visits to the neurosurgery clinic or by telephone questionnaires ranged from 2 months to 13 years. A literature search and analysis of current treatment options were performed. RESULTS Nine of the 11 patients (82%) experienced complete or substantial relief of their preoperative symptoms. One patient (Patient 4) experienced worsening of bladder dysfunction after surgery and recovered slowly to subnormal function during the subsequent 2 months. The symptoms of Patient 9 did not resolve, and magnetic resonance imaging showed that the cyst had reoccurred. The patient underwent reoperation 3 months later without any improvement. One patient (Patient 11) experience a cerebrospinal fluid leakage complication. Neither new postoperative neurological defects nor infection were observed in our series. In the literature, there are six different treatment options under debate and controversially discussed. CONCLUSION Microsurgical treatment yielded the best long-term resolution of patient symptoms to date and should be recommended to appropriately selected patients.

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