Codify By AAPC
ICD 10 Range Metabolic disorders
1
Disorders of aromatic amino-acid metabolism( E70 )
2
Classical phenylketonuria( E70.0 )
3
Other hyperphenylalaninemias( E70.1 )
4
Disorders of tyrosine metabolism( E70.2 )
5
Disorder of tyrosine metabolism, unspecified( E70.20 )
6
Tyrosinemia( E70.21 )
7
Other disorders of tyrosine metabolism( E70.29 )
8
Albinism( E70.3 )
9
Albinism, unspecified( E70.30 )
10
Ocular albinism( E70.31 )
11
X-linked ocular albinism( E70.310 )
12
Autosomal recessive ocular albinism( E70.311 )
13
Other ocular albinism( E70.318 )
14
Ocular albinism, unspecified( E70.319 )
15
Oculocutaneous albinism( E70.32 )
16
Tyrosinase negative oculocutaneous albinism( E70.320 )
17
Tyrosinase positive oculocutaneous albinism( E70.321 )
18
Other oculocutaneous albinism( E70.328 )
19
Oculocutaneous albinism, unspecified( E70.329 )
20
Albinism with hematologic abnormality( E70.33 )
21
Chediak-Higashi syndrome( E70.330 )
22
Hermansky-Pudlak syndrome( E70.331 )
23
Other albinism with hematologic abnormality( E70.338 )
24
Albinism with hematologic abnormality, unspecified( E70.339 )
25
Other specified albinism( E70.39 )
26
Disorders of histidine metabolism( E70.4 )
27
Disorders of histidine metabolism, unspecified( E70.40 )
28
Histidinemia( E70.41 )
29
Other disorders of histidine metabolism( E70.49 )
30
Disorders of tryptophan metabolism( E70.5 )
31
Other disorders of aromatic amino-acid metabolism( E70.8 )
32
Aromatic L-amino acid decarboxylase deficiency( E70.81 )
33
Other disorders of aromatic amino-acid metabolism( E70.89 )
34
Disorder of aromatic amino-acid metabolism, unspecified( E70.9 )
35
Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism( E71 )
36
Maple-syrup-urine disease( E71.0 )
37
Other disorders of branched-chain amino-acid metabolism( E71.1 )
38
Branched-chain organic acidurias( E71.11 )
39
Isovaleric acidemia( E71.110 )
40
3-methylglutaconic aciduria( E71.111 )
41
Other branched-chain organic acidurias( E71.118 )
42
Disorders of propionate metabolism( E71.12 )
43
Methylmalonic acidemia( E71.120 )
44
Propionic acidemia( E71.121 )
45
Other disorders of propionate metabolism( E71.128 )
46
Other disorders of branched-chain amino-acid metabolism( E71.19 )
47
Disorder of branched-chain amino-acid metabolism, unspecified( E71.2 )
48
Disorders of fatty-acid metabolism( E71.3 )
49
Disorder of fatty-acid metabolism, unspecified( E71.30 )
50
Disorders of fatty-acid oxidation( E71.31 )
51
Long chain/very long chain acyl CoA dehydrogenase deficiency( E71.310 )
52
Medium chain acyl CoA dehydrogenase deficiency( E71.311 )
53
Short chain acyl CoA dehydrogenase deficiency( E71.312 )
54
Glutaric aciduria type II( E71.313 )
55
Muscle carnitine palmitoyltransferase deficiency( E71.314 )
56
Other disorders of fatty-acid oxidation( E71.318 )
57
Disorders of ketone metabolism( E71.32 )
58
Other disorders of fatty-acid metabolism( E71.39 )
59
Disorders of carnitine metabolism( E71.4 )
60
Disorder of carnitine metabolism, unspecified( E71.40 )
61
Primary carnitine deficiency( E71.41 )
62
Carnitine deficiency due to inborn errors of metabolism( E71.42 )
63
Iatrogenic carnitine deficiency( E71.43 )
64
Other secondary carnitine deficiency( E71.44 )
65
Ruvalcaba-Myhre-Smith syndrome( E71.440 )
66
Other secondary carnitine deficiency( E71.448 )
67
Peroxisomal disorders( E71.5 )
68
Peroxisomal disorder, unspecified( E71.50 )
69
Disorders of peroxisome biogenesis( E71.51 )
70
Zellweger syndrome( E71.510 )
71
Neonatal adrenoleukodystrophy( E71.511 )
72
Other disorders of peroxisome biogenesis( E71.518 )
73
X-linked adrenoleukodystrophy( E71.52 )
74
Childhood cerebral X-linked adrenoleukodystrophy( E71.520 )
75
Adolescent X-linked adrenoleukodystrophy( E71.521 )
76
Adrenomyeloneuropathy( E71.522 )
77
Other X-linked adrenoleukodystrophy( E71.528 )
78
X-linked adrenoleukodystrophy, unspecified type( E71.529 )
79
Other group 2 peroxisomal disorders( E71.53 )
80
Other peroxisomal disorders( E71.54 )
81
Rhizomelic chondrodysplasia punctata( E71.540 )
82
Zellweger-like syndrome( E71.541 )
83
Other group 3 peroxisomal disorders( E71.542 )
84
Other peroxisomal disorders( E71.548 )
85
Other disorders of amino-acid metabolism( E72 )
86
Disorders of amino-acid transport( E72.0 )
87
Disorders of amino-acid transport, unspecified( E72.00 )
88
Cystinuria( E72.01 )
89
Hartnup's disease( E72.02 )
90
Lowe's syndrome( E72.03 )
91
Cystinosis( E72.04 )
92
Other disorders of amino-acid transport( E72.09 )
93
Disorders of sulfur-bearing amino-acid metabolism( E72.1 )
94
Disorders of sulfur-bearing amino-acid metabolism, unspecified( E72.10 )
95
Homocystinuria( E72.11 )
96
Methylenetetrahydrofolate reductase deficiency( E72.12 )
97
Other disorders of sulfur-bearing amino-acid metabolism( E72.19 )
98
Disorders of urea cycle metabolism( E72.2 )
99
Disorder of urea cycle metabolism, unspecified( E72.20 )
100
Argininemia( E72.21 )
101
Arginosuccinic aciduria( E72.22 )
102
Citrullinemia( E72.23 )
103
Other disorders of urea cycle metabolism( E72.29 )
104
Disorders of lysine and hydroxylysine metabolism( E72.3 )
105
Disorders of ornithine metabolism( E72.4 )
106
Disorders of glycine metabolism( E72.5 )
107
Disorder of glycine metabolism, unspecified( E72.50 )
108
Non-ketotic hyperglycinemia( E72.51 )
109
Trimethylaminuria( E72.52 )
110
Primary hyperoxaluria( E72.53 )
111
Other disorders of glycine metabolism( E72.59 )
112
Other specified disorders of amino-acid metabolism( E72.8 )
113
Disorders of gamma aminobutyric acid metabolism( E72.81 )
114
Other specified disorders of amino-acid metabolism( E72.89 )
115
Disorder of amino-acid metabolism, unspecified( E72.9 )
116
Lactose intolerance( E73 )
117
Congenital lactase deficiency( E73.0 )
118
Secondary lactase deficiency( E73.1 )
119
Other lactose intolerance( E73.8 )
120
Lactose intolerance, unspecified( E73.9 )
121
Other disorders of carbohydrate metabolism( E74 )
122
Glycogen storage disease( E74.0 )
123
Glycogen storage disease, unspecified( E74.00 )
124
von Gierke disease( E74.01 )
125
Pompe disease( E74.02 )
126
Cori disease( E74.03 )
127
McArdle disease( E74.04 )
128
Lysosome-associated membrane protein 2 [LAMP2] deficiency( E74.05 )
129
Other glycogen storage disease( E74.09 )
130
Disorders of fructose metabolism( E74.1 )
131
Disorder of fructose metabolism, unspecified( E74.10 )
132
Essential fructosuria( E74.11 )
133
Hereditary fructose intolerance( E74.12 )
134
Other disorders of fructose metabolism( E74.19 )
135
Disorders of galactose metabolism( E74.2 )
136
Disorders of galactose metabolism, unspecified( E74.20 )
137
Galactosemia( E74.21 )
138
Other disorders of galactose metabolism( E74.29 )
139
Other disorders of intestinal carbohydrate absorption( E74.3 )
140
Sucrase-isomaltase deficiency( E74.31 )
141
Other disorders of intestinal carbohydrate absorption( E74.39 )
142
Disorders of pyruvate metabolism and gluconeogenesis( E74.4 )
143
Other specified disorders of carbohydrate metabolism( E74.8 )
144
Disorders of glucose transport, not elsewhere classified( E74.81 )
145
Glucose transporter protein type 1 deficiency( E74.810 )
146
Other disorders of glucose transport( E74.818 )
147
Disorders of glucose transport, unspecified( E74.819 )
148
Other specified disorders of carbohydrate metabolism( E74.89 )
149
Disorder of carbohydrate metabolism, unspecified( E74.9 )
150
Disorders of sphingolipid metabolism and other lipid storage disorders( E75 )
151
GM2 gangliosidosis( E75.0 )
152
GM2 gangliosidosis, unspecified( E75.00 )
153
Sandhoff disease( E75.01 )
154
Tay-Sachs disease( E75.02 )
155
Other GM2 gangliosidosis( E75.09 )
156
Other and unspecified gangliosidosis( E75.1 )
157
Unspecified gangliosidosis( E75.10 )
158
Mucolipidosis IV( E75.11 )
159
Other gangliosidosis( E75.19 )
160
Other sphingolipidosis( E75.2 )
161
Fabry (-Anderson) disease( E75.21 )
162
Gaucher disease( E75.22 )
163
Krabbe disease( E75.23 )
164
Niemann-Pick disease( E75.24 )
165
Niemann-Pick disease type A( E75.240 )
166
Niemann-Pick disease type B( E75.241 )
167
Niemann-Pick disease type C( E75.242 )
168
Niemann-Pick disease type D( E75.243 )
169
Niemann-Pick disease type A/B( E75.244 )
170
Other Niemann-Pick disease( E75.248 )
171
Niemann-Pick disease, unspecified( E75.249 )
172
Metachromatic leukodystrophy( E75.25 )
173
Sulfatase deficiency( E75.26 )
174
Pelizaeus-Merzbacher disease( E75.27 )
175
Canavan disease( E75.28 )
176
Other sphingolipidosis( E75.29 )
177
Sphingolipidosis, unspecified( E75.3 )
178
Neuronal ceroid lipofuscinosis( E75.4 )
179
Other lipid storage disorders( E75.5 )
180
Lipid storage disorder, unspecified( E75.6 )
181
Disorders of glycosaminoglycan metabolism( E76 )
182
Mucopolysaccharidosis, type I( E76.0 )
183
Hurler's syndrome( E76.01 )
184
Hurler-Scheie syndrome( E76.02 )
185
Scheie's syndrome( E76.03 )
186
Mucopolysaccharidosis, type II( E76.1 )
187
Other mucopolysaccharidoses( E76.2 )
188
Morquio mucopolysaccharidoses( E76.21 )
189
Morquio A mucopolysaccharidoses( E76.210 )
190
Morquio B mucopolysaccharidoses( E76.211 )
191
Morquio mucopolysaccharidoses, unspecified( E76.219 )
192
Sanfilippo mucopolysaccharidoses( E76.22 )
193
Other mucopolysaccharidoses( E76.29 )
194
Mucopolysaccharidosis, unspecified( E76.3 )
195
Other disorders of glucosaminoglycan metabolism( E76.8 )
196
Glucosaminoglycan metabolism disorder, unspecified( E76.9 )
197
Disorders of glycoprotein metabolism( E77 )
198
Defects in post-translational modification of lysosomal enzymes( E77.0 )
199
Defects in glycoprotein degradation( E77.1 )
200
Other disorders of glycoprotein metabolism( E77.8 )
201
Disorder of glycoprotein metabolism, unspecified( E77.9 )
202
Disorders of lipoprotein metabolism and other lipidemias( E78 )
203
Pure hypercholesterolemia( E78.0 )
204
Pure hypercholesterolemia, unspecified( E78.00 )
205
Familial hypercholesterolemia( E78.01 )
206
Pure hyperglyceridemia( E78.1 )
207
Mixed hyperlipidemia( E78.2 )
208
Hyperchylomicronemia( E78.3 )
209
Other hyperlipidemia( E78.4 )
210
Elevated Lipoprotein(a)( E78.41 )
211
Other hyperlipidemia( E78.49 )
212
Hyperlipidemia, unspecified( E78.5 )
213
Lipoprotein deficiency( E78.6 )
214
Disorders of bile acid and cholesterol metabolism( E78.7 )
215
Disorder of bile acid and cholesterol metabolism, unspecified( E78.70 )
216
Barth syndrome( E78.71 )
217
Smith-Lemli-Opitz syndrome( E78.72 )
218
Other disorders of bile acid and cholesterol metabolism( E78.79 )
219
Other disorders of lipoprotein metabolism( E78.8 )
220
Lipoid dermatoarthritis( E78.81 )
221
Other lipoprotein metabolism disorders( E78.89 )
222
Disorder of lipoprotein metabolism, unspecified( E78.9 )
223
Disorders of purine and pyrimidine metabolism( E79 )
224
Hyperuricemia without signs of inflammatory arthritis and tophaceous disease( E79.0 )
225
Lesch-Nyhan syndrome( E79.1 )
226
Myoadenylate deaminase deficiency( E79.2 )
227
Other disorders of purine and pyrimidine metabolism( E79.8 )
228
Aicardi-Goutieres syndrome( E79.81 )
229
Hereditary xanthinuria( E79.82 )
230
Other specified disorders of purine and pyrimidine metabolism( E79.89 )
231
Disorder of purine and pyrimidine metabolism, unspecified( E79.9 )
232
Disorders of porphyrin and bilirubin metabolism( E80 )
233
Hereditary erythropoietic porphyria( E80.0 )
234
Porphyria cutanea tarda( E80.1 )
235
Other and unspecified porphyria( E80.2 )
236
Unspecified porphyria( E80.20 )
237
Acute intermittent (hepatic) porphyria( E80.21 )
238
Other porphyria( E80.29 )
239
Defects of catalase and peroxidase( E80.3 )
240
Gilbert syndrome( E80.4 )
241
Crigler-Najjar syndrome( E80.5 )
242
Other disorders of bilirubin metabolism( E80.6 )
243
Disorder of bilirubin metabolism, unspecified( E80.7 )
244
Disorders of mineral metabolism( E83 )
245
Disorders of copper metabolism( E83.0 )
246
Disorder of copper metabolism, unspecified( E83.00 )
247
Wilson's disease( E83.01 )
248
Other disorders of copper metabolism( E83.09 )
249
Disorders of iron metabolism( E83.1 )
250
Disorder of iron metabolism, unspecified( E83.10 )
251
Hemochromatosis( E83.11 )
252
Hereditary hemochromatosis( E83.110 )
253
Hemochromatosis due to repeated red blood cell transfusions( E83.111 )
254
Other hemochromatosis( E83.118 )
255
Hemochromatosis, unspecified( E83.119 )
256
Other disorders of iron metabolism( E83.19 )
257
Disorders of zinc metabolism( E83.2 )
258
Disorders of phosphorus metabolism and phosphatases( E83.3 )
259
Disorder of phosphorus metabolism, unspecified( E83.30 )
260
Familial hypophosphatemia( E83.31 )
261
Hereditary vitamin D-dependent rickets (type 1) (type 2)( E83.32 )
262
Other disorders of phosphorus metabolism( E83.39 )
263
Disorders of magnesium metabolism( E83.4 )
264
Disorders of magnesium metabolism, unspecified( E83.40 )
265
Hypermagnesemia( E83.41 )
266
Hypomagnesemia( E83.42 )
267
Other disorders of magnesium metabolism( E83.49 )
268
Disorders of calcium metabolism( E83.5 )
269
Unspecified disorder of calcium metabolism( E83.50 )
270
Hypocalcemia( E83.51 )
271
Hypercalcemia( E83.52 )
272
Other disorders of calcium metabolism( E83.59 )
273
Other disorders of mineral metabolism( E83.8 )
274
Hungry bone syndrome( E83.81 )
275
Other disorders of mineral metabolism( E83.89 )
276
Disorder of mineral metabolism, unspecified( E83.9 )
277
Cystic fibrosis( E84 )
278
Cystic fibrosis with pulmonary manifestations( E84.0 )
279
Cystic fibrosis with intestinal manifestations( E84.1 )
280
Meconium ileus in cystic fibrosis( E84.11 )
281
Cystic fibrosis with other intestinal manifestations( E84.19 )
282
Cystic fibrosis with other manifestations( E84.8 )
283
Cystic fibrosis, unspecified( E84.9 )
284
Amyloidosis( E85 )
285
Non-neuropathic heredofamilial amyloidosis( E85.0 )
286
Neuropathic heredofamilial amyloidosis( E85.1 )
287
Heredofamilial amyloidosis, unspecified( E85.2 )
288
Secondary systemic amyloidosis( E85.3 )
289
Organ-limited amyloidosis( E85.4 )
290
Other amyloidosis( E85.8 )
291
Light chain (AL) amyloidosis( E85.81 )
292
Wild-type transthyretin-related (ATTR) amyloidosis( E85.82 )
293
Other amyloidosis( E85.89 )
294
Amyloidosis, unspecified( E85.9 )
295
Volume depletion( E86 )
296
Dehydration( E86.0 )
297
Hypovolemia( E86.1 )
298
Volume depletion, unspecified( E86.9 )
299
Other disorders of fluid, electrolyte and acid-base balance( E87 )
300
Hyperosmolality and hypernatremia( E87.0 )
301
Hypo-osmolality and hyponatremia( E87.1 )
302
Acidosis( E87.2 )
303
Acidosis, unspecified( E87.20 )
304
Acute metabolic acidosis( E87.21 )
305
Chronic metabolic acidosis( E87.22 )
306
Other acidosis( E87.29 )
307
Alkalosis( E87.3 )
308
Mixed disorder of acid-base balance( E87.4 )
309
Hyperkalemia( E87.5 )
310
Hypokalemia( E87.6 )
311
Fluid overload( E87.7 )
312
Fluid overload, unspecified( E87.70 )
313
Transfusion associated circulatory overload( E87.71 )
314
Other fluid overload( E87.79 )
315
Other disorders of electrolyte and fluid balance, not elsewhere classified( E87.8 )
316
Other and unspecified metabolic disorders( E88 )
317
Disorders of plasma-protein metabolism, not elsewhere classified( E88.0 )
318
Alpha-1-antitrypsin deficiency( E88.01 )
319
Plasminogen deficiency( E88.02 )
320
Other disorders of plasma-protein metabolism, not elsewhere classified( E88.09 )
321
Lipodystrophy, not elsewhere classified( E88.1 )
322
Lipomatosis, not elsewhere classified( E88.2 )
323
Tumor lysis syndrome( E88.3 )
324
Mitochondrial metabolism disorders( E88.4 )
325
Mitochondrial metabolism disorder, unspecified( E88.40 )
326
MELAS syndrome( E88.41 )
327
MERRF syndrome( E88.42 )
328
Disorders of mitochondrial tRNA synthetases( E88.43 )
329
Other mitochondrial metabolism disorders( E88.49 )
330
Other specified metabolic disorders( E88.8 )
331
Metabolic syndrome and other insulin resistance( E88.81 )
332
Metabolic syndrome( E88.810 )
333
Insulin resistance syndrome, Type A( E88.811 )
334
Other insulin resistance( E88.818 )
335
Insulin resistance, unspecified( E88.819 )
336
Other specified metabolic disorders( E88.89 )
337
Metabolic disorder, unspecified( E88.9 )
338
Wasting disease (syndrome) due to underlying condition( E88.A )