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Related Concept Videos

Hypoxia01:23

Hypoxia

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Hypoxia is a medical condition characterized by an inadequate oxygen supply to body tissues. It typically manifests as a bluish discoloration of the skin and mucosae, especially in fair-skinned individuals, when hemoglobin (Hb) saturation drops below 75%.
Types of Hypoxia
There are four primary types of hypoxia, each resulting from a different cause:
1. Anemic hypoxia: This type occurs due to insufficient oxygen delivery caused by a lack of red blood cells (RBCs) or RBCs with abnormal or...
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Phase II Reactions: Methylation Reactions01:17

Phase II Reactions: Methylation Reactions

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Methylation is a phase II biotransformation process involving the attachment of a methyl group to a substrate. Enzymes known as methyltransferases orchestrate this reaction.
The mechanism of methylation unfolds in two stages. The first stage sees a methyltransferase enzyme facilitating the transfer of a methyl group from S-adenosylmethionine (SAM) to the substrate, forming S-adenosylhomocysteine (SAH). The second stage involves further metabolism of SAH into homocysteine, which can be recycled...
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Hemoglobin01:24

Hemoglobin

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Hemoglobin is a globular protein made up of four subunits. Two of these subunits are alpha chains, and the other two are beta chains. Each subunit contains a molecule of heme, which has an iron atom and can bind to oxygen. When an oxygen molecule binds to one heme group, it changes the shape of hemoglobin, making it easier for the other heme groups to bind oxygen as well.
When all four heme groups are bound to oxygen, the resulting molecule is called oxyhemoglobin. As a result, arterial blood...
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Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

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Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
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Hypersensitivity Reactions: Cytolytic Reactions01:01

Hypersensitivity Reactions: Cytolytic Reactions

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Type II hypersensitivity involves IgG and IgM antibodies targeting cell surface antigens, leading to cell destruction. This can occur through complement activation, antibody-dependent cell-mediated cytotoxicity (ADCC), or acting as opsonins for phagocytosis. When excessive, these reactions cause significant tissue damage.Drug-induced hemolytic anemia is a common example, where drugs like penicillin or cephalosporins bind to red blood cells, forming drug-protein complexes. These complexes...
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Electron Transport Chain: Complex I and II01:46

Electron Transport Chain: Complex I and II

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The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
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Biochemical Measurement of Neonatal Hypoxia
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Methemoglobinemia: Living with Dormant Devil.

Dhiraj J Trivedi1, Bandi Joshiraj2, Vijay Bidkar3

  • 1Department of Biochemistry, SDM College of Medical Sciences and Hospital, Dharwad, Karnataka India.

Indian Journal of Clinical Biochemistry : IJCB
|April 22, 2017
PubMed
Summary

Congenital methemoglobinemia, a condition of high methemoglobin levels, can remain dormant. This case highlights accidental discovery during pre-operative assessment, showing a patient lived without complications despite 27.7% methemoglobin.

Keywords:
CyanosisHemoglobin derivativeMethemoglobinNADH methemoglobin reductase

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Area of Science:

  • Biochemistry
  • Hematology
  • Genetics

Background:

  • Methemoglobin is an oxidized form of hemoglobin.
  • Methemoglobinemia results from NADH methemoglobin reductase deficiency or inactivity, leading to excessive methemoglobin accumulation.
  • This condition can be congenital or acquired.

Observation:

  • A case of dormant congenital methemoglobinemia was discovered during a routine preoperative assessment.
  • The patient presented with low oxygen saturation even at 100% fraction of inspired oxygen (FIO2) and central cyanosis.
  • The patient had 27.7% methemoglobin but had been asymptomatic throughout life.

Findings:

  • Congenital methemoglobinemia can be asymptomatic and undetected until specific medical evaluations.
  • Significant methemoglobin levels (27.7%) do not always correlate with clinical complications in all individuals.
  • Successful surgical intervention (tympanoplasty and mastoidectomy) was achieved under local anesthesia with appropriate precautions.

Implications:

  • Highlights the importance of thorough preoperative assessments, especially for unexplained cyanosis or hypoxia.
  • Suggests that congenital methemoglobinemia may have a broader spectrum of clinical presentation than previously recognized.
  • Emphasizes the need for individualized patient management, considering both methemoglobin levels and overall clinical status.