ICD-10 Diagnosis Code D74.0

Congenital methemoglobinemia

Diagnosis Code D74.0

ICD-10: D74.0
Short Description: Congenital methemoglobinemia
Long Description: Congenital methemoglobinemia
This is the 2017 version of the ICD-10-CM diagnosis code D74.0

Code Classification
  • Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
    • Other disorders of blood and blood-forming organs (D70-D77)
      • Methemoglobinemia (D74)

Information for Medical Professionals

Diagnostic Related Groups
The diagnosis code D74.0 is grouped in the following Diagnostic Related Group(s) (MS-DRG v33.0)


Convert to ICD-9 Additional informationCallout TooltipGeneral Equivalence Map
The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs.

  • Congenital methemoglobinaema with defective methemoglobin-reducing system
  • Congenital methemoglobinemia
  • Congenital methemoglobinemia with abnormal methemoglobins
  • Cytochrome b>3< deficiency
  • Deficiency in enzyme complexes of mitochondrial respiratory chain
  • Familial methemoglobinemia
  • Hereditary methemoglobinemia due to globin chain mutation
  • Hereditary methemoglobinemia, enzymatic type
  • Hereditary nonspherocytic hemolytic anemia due to reduced nicotinamide adenine dinucleotide-methemoglobin reductase deficiency

Index of Diseases and Injuries
References found for the code D74.0 in the Index of Diseases and Injuries:

Information for Patients

Blood Disorders

Also called: Hematologic diseases

Your blood is living tissue made up of liquid and solids. The liquid part, called plasma, is made of water, salts and protein. Over half of your blood is plasma. The solid part of your blood contains red blood cells, white blood cells and platelets.

Blood disorders affect one or more parts of the blood and prevent your blood from doing its job. They can be acute or chronic. Many blood disorders are inherited. Other causes include other diseases, side effects of medicines, and a lack of certain nutrients in your diet.

Types of blood disorders include

  • Platelet disorders, excessive clotting, and bleeding problems, which affect how your blood clots
  • Anemia, which happens when your blood does not carry enough oxygen to the rest of your body
  • Cancers of the blood, such as leukemia and myeloma
  • Eosinophilic disorders, which are problems with one type of white blood cell.

  • Blood differential
  • Blood smear
  • CBC
  • Hematocrit
  • Hemoglobin
  • Low white blood cell count and cancer
  • RBC count
  • RBC indices
  • WBC count

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Methemoglobinemia, beta-globin type Methemoglobinemia, beta-globin type is a condition that affects the function of red blood cells. Specifically, it alters a molecule called hemoglobin within these cells. Hemoglobin within red blood cells attaches (binds) to oxygen molecules in the lungs, which it carries through the bloodstream, then releases in tissues throughout the body. Instead of normal hemoglobin, people with methemoglobinemia, beta-globin type have an abnormal form called methemoglobin, which is unable to efficiently deliver oxygen to the body's tissues. In methemoglobinemia, beta-globin type, the abnormal hemoglobin gives the blood a brown color. It also causes a bluish appearance of the skin, lips, and nails (cyanosis), which usually first appears around the age of 6 months. The signs and symptoms of methemoglobinemia, beta-globin type are generally limited to cyanosis, which does not cause any health problems. However, in rare cases, severe methemoglobinemia, beta-globin type can cause headaches, weakness, and fatigue.
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Autosomal recessive congenital methemoglobinemia Autosomal recessive congenital methemoglobinemia is an inherited condition that mainly affects the function of red blood cells. Specifically, it alters a molecule within these cells called hemoglobin. Hemoglobin carries oxygen to cells and tissues throughout the body. In people with autosomal recessive congenital methemoglobinemia, some of the normal hemoglobin is replaced by an abnormal form called methemoglobin, which is unable to deliver oxygen to the body's tissues. As a result, tissues in the body become oxygen deprived, leading to a bluish appearance of the skin, lips, and nails (cyanosis).There are two forms of autosomal recessive congenital methemoglobinemia: types I and II. People with type I have cyanosis from birth and may experience weakness or shortness of breath related to the shortage of oxygen in their tissues. People with type II have cyanosis as well as severe neurological problems. After a few months of apparently normal development, children with type II develop severe brain dysfunction (encephalopathy), uncontrolled muscle tensing (dystonia), and involuntary limb movements (choreoathetosis); also, the size of their head remains small and does not grow in proportion with their body (microcephaly). People with type II have severe intellectual disability; they can recognize faces and usually babble but speak no words. They can sit unassisted and grip objects but have impaired motor skills that leave them unable to walk. In type II, growth is often slowed. Abnormal facial muscle movements can interfere with swallowing, which can lead to feeding difficulties and further slow growth.People with autosomal recessive congenital methemoglobinemia type I have a normal life expectancy, but people with type II often do not survive past early adulthood.
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