scholarly journals Transient 5-oxoprolinuria and high anion gap metabolic acidosis: clinical and biochemical findings in eleven subjects

1998 ◽  
Vol 44 (7) ◽  
pp. 1497-1503 ◽  
Author(s):  
James J Pitt ◽  
Simon Hauser
Keyword(s):  
Metabolic Acidosis ◽  
Organic Acids ◽  
Organic Acid ◽  
Clinical Features ◽  
Reference Range ◽  
Anion Gap ◽  
Urinary Organic Acid ◽  
Liver Glutathione ◽  
Liver Functions

Abstract We describe biochemical and clinical features of 11 subjects (ages, 1.2–84 years, nine females and two males) with transient 5-oxoprolinuria (0.6–23.6 mol/mol of creatinine, reference range <0.07). A variety of conditions preceded the onset of acidosis, and all had taken acetaminophen (paracetamol), although in therapeutic amounts in most subjects. Metabolic acidosis was documented in nine subjects, and all had an increased anion gap and abnormal liver functions. 5-Oxoproline was the major urinary organic acid in five subjects, whereas the rest had more complex profiles comprising 5-oxoproline and other organic acids, such as lactate, 3-hydroxybutyrate, and 4-hydroxyphenyl lactate. The 5-oxoproline was predominantly of the l-configuration. One subject died during an acidotic episode, and the rest recovered with no apparent long-term ill effects. Urinary 5-oxoproline was within the reference range in six subjects that were re-tested after the anion gap normalized. These findings suggest that acetaminophen, in association with other unidentified factors, is involved in the development of this condition through a mechanism of depletion of liver glutathione stores.

Metabole acidose met verhoogde anion gap ten gevolge van 5-oxoprolinurie bij gelijktijdig gebruik van acetaminofen en flucloxacilline

2021 ◽  
Author(s):  
B. VERDONCK ◽  
Y. BEKHUIS ◽  
S. NEVEN ◽  
H. VERBRUGGE
Keyword(s):  
Metabolic Acidosis ◽  
Sodium Bicarbonate ◽  
Organic Acids ◽  
Metabolic Disorder ◽  
Spontaneous Recovery ◽  
Anion Gap ◽  
Sufficient Evidence ◽  
Recovery Treatment ◽  
Simultaneous Use ◽  
Rare Metabolic Disorder

Metabolic acidosis with increased anion gap due to 5-oxoprolinuria with concomitant use of acetaminophen and flucloxacillin 5-oxoprolinuria is a relatively rare metabolic disorder in which there is accumulation of 5-oxoproline leading to a high anion gap metabolic acidosis. The acquired form is usually caused by simultaneous use of acetaminophen (also known as paracetamol) and flucloxacillin, which is not uncommon. The exact incidence and prevalence of the acquired form are not known. This disorder is most likely under-reported as the test used for diagnosis is not always performed nor easy accessible. Diagnosing 5-oxoprolinuria requires testing urine or serum of a patient for the presence of organic acids, which are highly elevated in 5-oxoprolinuria. Due to the relative rareness of the disorder, cases are easily missed. 5-oxoprolinuria should always be suspected in a patient with unexplained high anion gap metabolic acidosis and/or therapy with flucloxacillin with or without the concomitant use of acetaminophen. Treating 5-oxoprolinuria is straightforward and consists of stopping the offending drug(s) which mostly leads to a spontaneous recovery. Treatment with sodium bicarbonate and/or N-acetylcysteine are also described in the literature, but at present there is not sufficient evidence supporting their use.

scholarly journals Responses of Swamp Cypress (Taxodium distichum) and Chinese Willow (Salix matsudana) Roots to Periodic Submergence in Mega-Reservoir: Changes in Organic Acid Concentration

Forests ◽  
2021 ◽  
Vol 12 (2) ◽  
pp. 203
Author(s):  
Xinrui He ◽  
Ting Wang ◽  
Kejun Wu ◽  
Peng Wang ◽  
Yuancai Qi ◽  
...  
Keyword(s):  
Oxalic Acid ◽  
Organic Acids ◽  
Organic Acid ◽  
Tartaric Acid ◽  
Malic Acid ◽  
Woody Species ◽  
Base Diameter ◽  
Salix Matsudana ◽  
Winter Flooding

Organic acids are critical as secondary metabolites for plant adaption in a stressful situation. Oxalic acid, tartaric acid, and malic acid can improve plant tolerance under waterlogged conditions. Two prominent woody species (Taxodium distichum-Swamp cypress and Salix matsudana-Chinese willow) have been experiencing long-term winter submergence and summer drought in the Three Gorges Reservoir. The objectives of the present study were to explore the responses of the roots of two woody species during flooding as reflected by root tissue concentrations of organic acids. Potted sample plants were randomly divided into three treatment groups: control, moderate submergence, and deep submergence. The concentrations of oxalic acid, tartaric acid, and malic acid in the main root and lateral roots of the two species were determined at four stages. The results showed that T. distichum and S. matsudana adapted well to the water regimes of the reservoir, with a survival rate of 100% during the experiment period. After experiencing a cycle of submergence and emergence, the height and base diameter of the two species showed increasing trends. Changes in base diameter showed insignificant differences between submergence treatments, and only height was significant under deep submergence. The concentrations of three organic acids in the roots of two species were influenced by winter submergence. After emergence in spring, two species could adjust their organic acid metabolisms to the normal level. Among three organic acids, tartaric acid showed the most sensitive response to water submergence, which deserved more studies in the future. The exotic species, T. distichum, had a more stable metabolism of organic acids to winter flooding. However, the native species, S. matsudana, responded more actively to long-term winter flooding. Both species can be considered in vegetation restoration, but it needs more observations for planting around 165 m above sea level, where winter submergence is more than 200 days.

scholarly journals Minding The Gap: Severe Anion Gap Metabolic Acidosis Associated With 5-Oxoproline Secondary To Chronic Acetaminophen Use

2019 ◽  
Vol 14 (4) ◽  
pp. e43-e49
Author(s):  
Claudia Frankfurter ◽  
Kevin Venus ◽  
David Frost
Keyword(s):  
Metabolic Acidosis ◽  
Anion Gap ◽  
Presumptive Diagnosis ◽  
Diffuse Abdominal Pain ◽  
Common Causes ◽  
Alkali Therapy ◽  
History Of ◽  
Douleur Abdominale ◽  
Osmolar Gap

AbstractAn 89-year-old man with multiple comorbidities presented to the emergency department with diffuse abdominal pain and dyspnea. He was found to have a severe anion-gap metabolic acidosis with the normal osmolar gap. An initial panel of investigations for common causes of anion-gap metabolic acidosis was unremarkable. Further history revealed long-term daily acetaminophen use. A presumptive diagnosis of 5-oxoprolinemia secondary to chronic acetaminophen use was made. Despite supportive care, the patient did not survive. There is emerging literature on elevated anion gap metabolic acidosis induced by the accumulation of 5-oxoproline, an intermediate organic acid in the gamma-glutamyl cycle. A quantitative profile of urinary organic acids to measure 5-oxoproline is valuable in confirming the diagnosis. Treatment is largely supportive, consisting of cessation of acetaminophen, alkali therapy, and N-acetylcysteine. Clinicians should consider 5-oxoprolemia in patients who present with an otherwise unexplained anion gap metabolic acidosis and a history of chronic acetaminophen use. RESUMEUn homme de 89 ans souffrant de comorbidités multiples s’est présenté à l’urgence avec douleur abdominale diffuse et dyspnée. On a découvert qu’il souffrait d’une acidose métabolique grave à anions nuls avec un écart osmolaire normal. Un premier groupe d’études sur les causes courantes d’acidose métabolique à intervalle anionique n’a pas été remarquable. D’autres antécédents ont révélé une utilisation quotidienne à long terme de l’acétaminophène. Un diagnostic présumé de 5-oxoprolinémie secondaire à l’utilisation chronique d’acétaminophène a été posé. Malgré des soins de soutien, le patient n’a pas survécu. Il existe une littérature émergente sur l’acidose métabolique à intervalle anionique élevé induite par l’accumulation de 5-oxoproline, un acide organique intermédiaire dans le cycle gamma-glutamyle. Un profil quantitatif d’acides organiques urinaires pour mesurer la 5-oxoproline est utile pour confirmer le diagnostic. Le traitement est largement favorable, consistant en l’arrêt de l’acétaminophène, un traitement alcalin et de la N-acétylcystéine. Les cliniciens devraient envisager l’administration de 5-oxoprolemia chez les patients qui présentent une acidose métabolique par gap anionique autrement inexpliquée et des antécédents d’utilisation chronique d’acétaminophène.

The Routine Investigation of Urinary Organic Acids in Selected Paediatric Patients: Results over a 2 1/2-Year Period

1984 ◽  
Vol 21 (1) ◽  
pp. 45-50 ◽  
Author(s):  
M J Bennett ◽  
Anne Green ◽  
R J Pollitt ◽  
E Worthy
Keyword(s):  
Metabolic Acidosis ◽  
Lactic Acidosis ◽  
Organic Acids ◽  
Organic Acid ◽  
Acid Metabolism ◽  
Diagnostic Yield ◽  
Inborn Errors ◽  
Routine Investigation ◽  
Urinary Organic Acids ◽  
Organic Acid Metabolism

Over a 2 1/2-year period 13 patients with inborn errors of organic acid metabolism, excluding undifferentiated lactic acidosis, have been diagnosed in our laboratories. The diagnostic yield in patients who had not previously been investigated by organic acid chromatography was 1 in 25, the majority of cases having presented with metabolic acidosis. A larger number of non-specific abnormalities were also detected. This type of investigation is beset with pitfalls and is extremely labour intensive.

A rare progressive neurometabolic disease (L-2-hydroxyglutaric aciduria) diagnosed by urinary organic acid analysis

2006 ◽  
Vol 37 (03) ◽  
Author(s):  
S Vlaho ◽  
S Posselt ◽  
V Boda ◽  
M Baz Bartels ◽  
S Parbel ◽  
...  
Keyword(s):  
Organic Acid ◽  
Urinary Organic Acid ◽  
Organic Acid Analysis ◽  
Urinary Organic Acid Analysis

An Unusual Case of Severe Anion Gap Metabolic Acidosis in a 3-year-old Girl

2021 ◽  
Vol 42 (Supplement 1) ◽  
pp. S46-S51
Author(s):  
Ryan M. Fredericks ◽  
George Sam Wang ◽  
Christine U. Vohwinkel ◽  
Jessica Kraynik Graham
Keyword(s):  
Metabolic Acidosis ◽  
Unusual Case ◽  
Anion Gap

Clinical features and long-term outcome of ipsilateral delayed endolymphatic hydrops

2019 ◽  
Vol 69 (3) ◽  
Author(s):  
Andrea Albera ◽  
Claudia Cassandro ◽  
Carmine F. Gervasio ◽  
Sergio Lucisano ◽  
Marco Boldreghini ◽  
...  
Keyword(s):  
Clinical Features ◽  
Endolymphatic Hydrops ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Delayed Endolymphatic Hydrops

Possibilities and prospects in early orthopaedic rehabilitation of children with ectodermal dysplasia and congenital edentulism. clinical features and practical experience

2020 ◽  
pp. 40-50
Author(s):  
V.G. Galonsky ◽  
N.V. Tarasova ◽  
E.S. Surdo ◽  
A.V. Gradoboev
Keyword(s):  
Preschool Children ◽  
Clinical Features ◽  
Ectodermal Dysplasia ◽  
Dental Treatment ◽  
Practical Experience ◽  
Dental Clinic ◽  
Pedagogical Tools ◽  
Psychological Types ◽  
Clinical Cases

The article is devoted to the issue of early orthopaedic rehabilitation of youngest preschool children with ectodermal dysplasia and congenital edentulism. The essence and details of children’s psychophysiological development at this age are revealed, as well as main pedagogical tools making it possible to adapt the children to the specific environment of the dental clinic and motivate them for undergoing long-term multistage orthopaedic dental treatment. Clinical approaches and peculiarities in the dentist’s work with children of different psychological types as well as particular aspects in their adaptation to removable dentures are described. Two clinical cases of effective orthopaedic rehabilitation of 3-years-old children with ectodermal dysplasia and congenital edentulism using full removable lamellar dentures.

Ketamine Treatment in Depression: A Systematic Review of Clinical Characteristics Predicting Symptom Improvement

2020 ◽  
Vol 20 (15) ◽  
pp. 1398-1414 ◽  
Author(s):  
Darby J.E. Lowe ◽  
Daniel J. Müller ◽  
Tony P. George
Keyword(s):  
Clinical Features ◽  
Clinical Characteristics ◽  
Bipolar Depression ◽  
Antidepressant Effect ◽  
Future Research ◽  
Symptom Improvement ◽  
Randomized Controlled ◽  
Treatment Of Depression ◽  
Future Direction

Ketamine has been shown to be efficacious for the treatment of depression, specifically among individuals who do not respond to first-line treatments. There is still, however, a lack of clarity surrounding the clinical features and response periods across samples that respond to ketamine. This paper systematically reviews published randomized controlled trials that investigate ketamine as an antidepressant intervention in both unipolar and bipolar depression to determine the specific clinical features of the samples across different efficacy periods. Moreover, similarities and differences in clinical characteristics associated with acute versus longer-term drug response are discussed. Similarities across all samples suggest that the population that responds to ketamine’s antidepressant effect has experienced chronic, long-term depression, approaching ketamine treatment as a “last resort”. Moreover, differences between these groups suggest future research to investigate the potential of stronger efficacy towards depression in the context of bipolar disorder compared to major depression, and in participants who undergo antidepressant washout before ketamine administration. From these findings, suggestions for the future direction of ketamine research for depression are formed.

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