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Related Concept Videos

Diagnosing Acidosis and Alkalosis01:24

Diagnosing Acidosis and Alkalosis

226
Diagnosing acid-base imbalances involves systematically analyzing arterial blood samples, focusing on three key measurements: pH, bicarbonate (HCO3−) concentration, and carbon dioxide partial pressure (PCO2). This analysis follows a four-step process that helps identify the imbalance's underlying cause and nature.
First, the pH level is assessed to determine whether the blood pH is normal (7.35–7.45), low (acidosis), or high (alkalosis).
Next, the PCO2  and...
226
Disorders of Acid-Base Balance01:29

Disorders of Acid-Base Balance

204
The human body maintains a precise pH range of arterial blood between 7.35 and 7.45. Deviations result in either acidosis (pH < 7.35) or alkalosis (pH > 7.45). These conditions are further classified as respiratory or metabolic disorders based on their underlying cause.
Respiratory Acidosis and Alkalosis
Respiratory acidosis occurs due to an increase in the partial pressure of carbon dioxide PCO2 in the blood. It often arises from shallow breathing or impaired gas exchange caused by...
204
Acute Pancreatitis I: Introduction01:27

Acute Pancreatitis I: Introduction

366
Pancreatitis is inflammation of the pancreas, an organ located behind the stomach. It can be either acute or chronic.
Acute pancreatitis is characterized by rapid inflammation of the pancreas, often caused by factors like gallstone blockage or excessive alcohol consumption. Chronic pancreatitis, on the other hand, is a slow, progressive inflammation that may result from long-term alcohol abuse, obstructions in the pancreatic duct, or genetic factors.
The causes of acute pancreatitis include:
366
Renal Regulation of Acid-Base Balance01:29

Renal Regulation of Acid-Base Balance

459
Metabolic reactions in the body produce nonvolatile acids, such as sulfuric acid, which generate an acid load of approximately 1 mEq of H+ per kilogram of body weight daily. Excreting H+ in the urine is essential to balance this acid load.
In the kidneys, cells within the proximal convoluted tubules (PCT) and the collecting ducts secrete hydrogen ions (H+) into the tubular fluid. Specifically, in the PCT, Na+/H+ antiporters secrete H+ while reabsorbing Na+.
However, the intercalated cells in...
459
Gastritis-II: Pathophysiology01:17

Gastritis-II: Pathophysiology

339
Gastritis is marked by disruption of the mucosal barrier that usually protects the stomach tissue from digestive juices and manifests in acute and chronic forms.
In acute gastritis, the gastric mucosa becomes swollen and red and undergoes superficial erosion. Superficial ulceration may lead to bleeding.
In chronic gastritis, persistent or repeated insults lead to chronic inflammatory changes and, eventually, thinning or atrophy of the gastric tissue.
Gastritis can stem from various causes, each...
339
Acid-Base Balance01:25

Acid-Base Balance

405
The human body maintains a narrow pH range regulated through acid-base balance. This balance is crucial as changes in the hydrogen ion concentration can disrupt cell membrane stability, alter protein structures, and change enzyme activities. The normal pH of arterial blood is 7.4, venous blood and interstitial fluid is 7.35, and intracellular fluid averages 7.0.
When the pH of arterial blood rises above 7.45, it results in a condition called alkalosis. Conversely, a drop below 7.35 leads to...
405

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Updated: Jul 7, 2025

Multi-step Preparation Technique to Recover Multiple Metabolite Compound Classes for In-depth and Informative Metabolomic Analysis
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Isovaleric Acidemia: A Case Report.

Elizabeth Zegarra Buitron1, Daniel A Vidal Panduro1, Nancy S Guillén Ramírez2

  • 1Internal Medicine, School of Medicine, Universidad Peruana de Ciencias Aplicadas, Lima, PER.

Cureus
|December 26, 2023
PubMed
Summary
This summary is machine-generated.

Isovaleric acidemia, a leucine metabolism disorder, presents variably. Early diagnosis and management, including diet and supplements, are crucial for preventing crises and ensuring good growth.

Keywords:
chronic intermittent isovaleric acidemiaisovaleric acidemialeucine metabolismmetabolic acidosisorganic acidemia

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Area of Science:

  • Biochemistry
  • Genetics
  • Metabolic Disorders

Background:

  • Isovaleric acidemia is an autosomal recessive metabolic disorder affecting leucine breakdown.
  • It is characterized by variable clinical presentations, including asymptomatic, acute neonatal, and chronic intermittent phenotypes.
  • Infections frequently precipitate catabolic crises in affected individuals.

Observation:

  • A case study of a three-year-old female with recurrent decompensation episodes since age two is presented.
  • The patient experienced encephalopathy during the diagnostic episode but recovered without neurological deficits.
  • The patient has been managed with dietary restrictions and L-carnitine/L-glycine supplementation.

Findings:

  • Diagnosis relies on detecting isovalerylglycine CoA in urine and elevated C5 acylcarnitine in blood.
  • Effective management involves preventing catabolic states through dietary control and supplementation.
  • The presented case demonstrates successful long-term management with positive nutritional and growth outcomes.

Implications:

  • This case highlights the importance of timely diagnosis and consistent management in isovaleric acidemia.
  • Effective treatment strategies can prevent severe complications like neurological sequelae.
  • Optimized dietary management and supplementation support favorable growth and development in affected children.