Category: E88 - Other and unspecified metabolic disorders

 Chapter Notes

 Section Notes

 E88

Other and unspecified metabolic disorders

Use additional codes for associated conditions

histiocytosis X (chronic) (C96.6)

 E88.0

Disorders of plasma-protein metabolism, not elsewhere classified

monoclonal gammopathy (of undetermined significance) (D47.2)

polyclonal hypergammaglobulinemia (D89.0)

Waldenström macroglobulinemia (C88.00)

disorder of lipoprotein metabolism (E78.-)

 E88.01

Alpha-1-antitrypsin deficiency

AAT deficiency

 E88.02

Plasminogen deficiency

Dysplasminogenemia

Hypoplasminogenemia

Type 1 plasminogen deficiency

Type 2 plasminogen deficiency

Code also, if applicable, ligneous conjunctivitis (H10.51-)

Use additional code for associated findings, such as:

hydrocephalus (G91.4)

otitis media (H67.-)

respiratory disorder related to plasminogen deficiency (J99)

 E88.09

Other disorders of plasma-protein metabolism, not elsewhere classified

Bisalbuminemia

 E88.1

Lipodystrophy, not elsewhere classified

Whipple's disease (K90.81)

 E88.10

Lipodystrophy, unspecified

Lipodystrophy NOS

 E88.11

Partial lipodystrophy

Acquired partial lipodystrophy (APL)

Barraquer-Simons lipodystrophy

Familial partial lipodystrophy (FPLD)

 E88.12

Generalized lipodystrophy

Acquired generalized lipodystrophy (AGL)

Berardinelli-Siep syndrome

Congenital generalized lipodystrophy (CGL)

Lawrence syndrome

 E88.13

Localized lipodystrophy

Injection lipodystrophy

Insulin lipodystrophy

 E88.14

HIV-associated lipodystrophy

Code first any human immunodeficiency virus [HIV] disease (B20)

Use additional code for adverse effect, if applicable, to identify drug (T37.5X5-)

 E88.19

Other lipodystrophy, not elsewhere classified

 E88.2

Lipomatosis, not elsewhere classified

Lipomatosis NOS

Lipomatosis (Check) dolorosa [Dercum]

 E88.3

Tumor lysis syndrome

Tumor lysis syndrome (spontaneous)

Tumor lysis syndrome following antineoplastic drug chemotherapy

Use additional code for adverse effect, if applicable, to identify drug (T45.1X5)

 E88.4

Mitochondrial metabolism disorders

disorders of pyruvate metabolism (E74.4)

Kearns-Sayre syndrome (H49.81)

Leber's disease (H47.22)

Leigh's encephalopathy (G31.82)

Mitochondrial myopathy, NEC (G71.3)

Reye's syndrome (G93.7)

 E88.40

Mitochondrial metabolism disorder, unspecified

 E88.41

MELAS syndrome

Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes

 E88.42

MERRF syndrome

Myoclonic epilepsy associated with ragged-red fibers

Code also progressive myoclonic epilepsy (G40.3-)

 E88.43

Disorders of mitochondrial tRNA synthetases

ARS2-related mitochondrial disorders

LBSL

Leukoencephalopathy with brainstem - spinal cord involvement - lactate elevation

Leukoencephalopathy with thalamus - brainstem involvement - high lactate

LTBL

Mitochondrial aminoacyl-tRNA synthetase disorders

Code also, if applicable, associated condition such as:

leukoencephalopathy (G93.49)

 E88.49

Other mitochondrial metabolism disorders

 E88.8

Other specified metabolic disorders

 E88.81

Metabolic syndrome and other insulin resistance

Use additional codes for associated manifestations, such as:

obesity (E66.-)

 E88.810

Metabolic syndrome

Dysmetabolic syndrome

 E88.811

Insulin resistance syndrome, Type A

 E88.818

Other insulin resistance

Insulin resistance syndrome, Type B

 E88.819

Insulin resistance, unspecified

 E88.82

Obesity due to disruption of MC4R pathway

Use additional code, if applicable, to identify associated manifestations, such as polyphagia (R63.2)

Use additional code to identify body mass index (BMI), if known (Z68.-)

 E88.89

Other specified metabolic disorders

Launois-Bensaude adenolipomatosis

adult pulmonary Langerhans cell histiocytosis (J84.82)

 E88.9

Metabolic disorder, unspecified

 E88.A

Wasting disease (syndrome) due to underlying condition

Cachexia due to underlying condition

Code first underlying condition

cachexia NOS (R64)

nutritional marasmus (E41)

failure to thrive (R62.51, R62.7)