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 Primary hyperoxaluria, type 1( E72.530 )

• 112 Other specified primary hyperoxaluria( E72.538 )

• 113 Primary hyperoxaluria, unspecified( E72.539 )

• 114 Secondary hyperoxaluria( E72.54 )

• 115 Dietary hyperoxaluria( E72.540 )

• 116 Enteric hyperoxaluria( E72.541 )

• 117 Other secondary hyperoxaluria( E72.548 )

• 118 Secondary hyperoxaluria, unspecified( E72.549 )

• 119 Other disorders of glycine metabolism( E72.59 )

• 120 Other specified disorders of amino-acid metabolism( E72.8 )

• 121 Disorders of gamma aminobutyric acid metabolism( E72.81 )

• 122 Other specified disorders of amino-acid metabolism( E72.89 )

• 123 Disorder of amino-acid metabolism, unspecified( E72.9 )

• 124 Lactose intolerance( E73 )

• 125 Congenital lactase deficiency( E73.0 )

• 126 Secondary lactase deficiency( E73.1 )

• 127 Other lactose intolerance( E73.8 )

• 128 Lactose intolerance, unspecified( E73.9 )

• 129 Other disorders of carbohydrate metabolism( E74 )

• 130 Glycogen storage disease( E74.0 )

• 131 Glycogen storage disease, unspecified( E74.00 )

• 132 von Gierke disease( E74.01 )

• 133 Pompe disease( E74.02 )

• 134 Cori disease( E74.03 )

• 135 McArdle disease( E74.04 )

• 136 Lysosome-associated membrane protein 2 [LAMP2] deficiency( E74.05 )

• 137 Other glycogen storage disease( E74.09 )

• 138 Disorders of fructose metabolism( E74.1 )

• 139 Disorder of fructose metabolism, unspecified( E74.10 )

• 140 Essential fructosuria( E74.11 )

• 141 Hereditary fructose intolerance( E74.12 )

• 142 Other disorders of fructose metabolism( E74.19 )

• 143 Disorders of galactose metabolism( E74.2 )

• 144 Disorders of galactose metabolism, unspecified( E74.20 )

• 145 Galactosemia( E74.21 )

• 146 Other disorders of galactose metabolism( E74.29 )

• 147 Other disorders of intestinal carbohydrate absorption( E74.3 )

• 148 Sucrase-isomaltase deficiency( E74.31 )

• 149 Other disorders of intestinal carbohydrate absorption( E74.39 )

• 150 Disorders of pyruvate metabolism and gluconeogenesis( E74.4 )

• 151 Other specified disorders of carbohydrate metabolism( E74.8 )

• 152 Disorders of glucose transport, not elsewhere classified( E74.81 )

• 153 Glucose transporter protein type 1 deficiency( E74.810 )

• 154 Other disorders of glucose transport( E74.818 )

• 155 Disorders of glucose transport, unspecified( E74.819 )

• 156 Disorders of citrate metabolism( E74.82 )

• 157 SLC13A5 Citrate Transporter Disorder( E74.820 )

• 158 Other disorders of citrate metabolism( E74.829 )

• 159 Other specified disorders of carbohydrate metabolism( E74.89 )

• 160 Disorder of carbohydrate metabolism, unspecified( E74.9 )

• 161 Disorders of sphingolipid metabolism and other lipid storage disorders( E75 )

• 162 GM2 gangliosidosis( E75.0 )

• 163 GM2 gangliosidosis, unspecified( E75.00 )

• 164 Sandhoff disease( E75.01 )

• 165 Tay-Sachs disease( E75.02 )

• 166 Other GM2 gangliosidosis( E75.09 )

• 167 Other and unspecified gangliosidosis( E75.1 )

• 168 Unspecified gangliosidosis( E75.10 )

• 169 Mucolipidosis IV( E75.11 )

• 170 Other gangliosidosis( E75.19 )

• 171 Other sphingolipidosis( E75.2 )

• 172 Fabry (-Anderson) disease( E75.21 )

• 173 Gaucher disease( E75.22 )

• 174 Krabbe disease( E75.23 )

• 175 Niemann-Pick disease( E75.24 )

• 176 Niemann-Pick disease type A( E75.240 )

• 177 Niemann-Pick disease type B( E75.241 )

• 178 Niemann-Pick disease type C( E75.242 )

• 179 Niemann-Pick disease type D( E75.243 )

• 180 Niemann-Pick disease type A/B( E75.244 )

• 181 Other Niemann-Pick disease( E75.248 )

• 182 Niemann-Pick disease, unspecified( E75.249 )

• 183 Metachromatic leukodystrophy( E75.25 )

• 184 Sulfatase deficiency( E75.26 )

• 185 Pelizaeus-Merzbacher disease( E75.27 )

• 186 Canavan disease( E75.28 )

• 187 Other sphingolipidosis( E75.29 )

• 188 Sphingolipidosis, unspecified( E75.3 )

• 189 Neuronal ceroid lipofuscinosis( E75.4 )

• 190 Other lipid storage disorders( E75.5 )

• 191 Lipid storage disorder, unspecified( E75.6 )

• 192 Disorders of glycosaminoglycan metabolism( E76 )

• 193 Mucopolysaccharidosis, type I( E76.0 )

• 194 Hurler's syndrome( E76.01 )

• 195 Hurler-Scheie syndrome( E76.02 )

• 196 Scheie's syndrome( E76.03 )

• 197 Mucopolysaccharidosis, type II( E76.1 )

• 198 Other mucopolysaccharidoses( E76.2 )

• 199 Morquio mucopolysaccharidoses( E76.21 )

• 200 Morquio A mucopolysaccharidoses( E76.210 )

• 201 Morquio B mucopolysaccharidoses( E76.211 )

• 202 Morquio mucopolysaccharidoses, unspecified( E76.219 )

• 203 Sanfilippo mucopolysaccharidoses( E76.22 )

• 204 Other mucopolysaccharidoses( E76.29 )

• 205 Mucopolysaccharidosis, unspecified( E76.3 )

• 206 Other disorders of glucosaminoglycan metabolism( E76.8 )

• 207 Glucosaminoglycan metabolism disorder, unspecified( E76.9 )

• 208 Disorders of glycoprotein metabolism( E77 )

• 209 Defects in post-translational modification of lysosomal enzymes( E77.0 )

• 210 Defects in glycoprotein degradation( E77.1 )

• 211 Other disorders of glycoprotein metabolism( E77.8 )

• 212 Disorder of glycoprotein metabolism, unspecified( E77.9 )

• 213 Disorders of lipoprotein metabolism and other lipidemias( E78 )

• 214 Pure hypercholesterolemia( E78.0 )

• 215 Pure hypercholesterolemia, unspecified( E78.00 )

• 216 Familial hypercholesterolemia( E78.01 )

• 217 Homozygous familial hypercholesterolemia [HoFH]( E78.010 )

• 218 Heterozygous familial hypercholesterolemia [HeFH]( E78.011 )

• 219 Familial hypercholesterolemia, unspecified( E78.019 )

• 220 Pure hyperglyceridemia( E78.1 )

• 221 Mixed hyperlipidemia( E78.2 )

• 222 Hyperchylomicronemia( E78.3 )

• 223 Other hyperlipidemia( E78.4 )

• 224 Elevated Lipoprotein(a)( E78.41 )

• 225 Other hyperlipidemia( E78.49 )

• 226 Hyperlipidemia, unspecified( E78.5 )

• 227 Lipoprotein deficiency( E78.6 )

• 228 Disorders of bile acid and cholesterol metabolism( E78.7 )

• 229 Disorder of bile acid and cholesterol metabolism, unspecified( E78.70 )

• 230 Barth syndrome( E78.71 )

• 231 Smith-Lemli-Opitz syndrome( E78.72 )

• 232 Other disorders of bile acid and cholesterol metabolism( E78.79 )

• 233 Other disorders of lipoprotein metabolism( E78.8 )

• 234 Lipoid dermatoarthritis( E78.81 )

• 235 Other lipoprotein metabolism disorders( E78.89 )

• 236 Disorder of lipoprotein metabolism, unspecified( E78.9 )

• 237 Disorders of purine and pyrimidine metabolism( E79 )

• 238 Hyperuricemia without signs of inflammatory arthritis and tophaceous disease( E79.0 )

• 239 Lesch-Nyhan syndrome( E79.1 )

• 240 Myoadenylate deaminase deficiency( E79.2 )

• 241 Other disorders of purine and pyrimidine metabolism( E79.8 )

• 242 Aicardi-Goutieres syndrome( E79.81 )

• 243 Hereditary xanthinuria( E79.82 )

• 244 Other specified disorders of purine and pyrimidine metabolism( E79.89 )

• 245 Disorder of purine and pyrimidine metabolism, unspecified( E79.9 )

• 246 Disorders of porphyrin and bilirubin metabolism( E80 )

• 247 Hereditary erythropoietic porphyria( E80.0 )

• 248 Porphyria cutanea tarda( E80.1 )

• 249 Other and unspecified porphyria( E80.2 )

• 250 Unspecified porphyria( E80.20 )

• 251 Acute intermittent (hepatic) porphyria( E80.21 )

• 252 Other porphyria( E80.29 )

• 253 Defects of catalase and peroxidase( E80.3 )

• 254 Gilbert syndrome( E80.4 )

• 255 Crigler-Najjar syndrome( E80.5 )

• 256 Other disorders of bilirubin metabolism( E80.6 )

• 257 Disorder of bilirubin metabolism, unspecified( E80.7 )

• 258 Disorders of mineral metabolism( E83 )

• 259 Disorders of copper metabolism( E83.0 )

• 260 Disorder of copper metabolism, unspecified( E83.00 )

• 261 Wilson's disease( E83.01 )

• 262 Other disorders of copper metabolism( E83.09 )

• 263 Disorders of iron metabolism( E83.1 )

• 264 Disorder of iron metabolism, unspecified( E83.10 )

• 265 Hemochromatosis( E83.11 )

• 266 Hereditary hemochromatosis( E83.110 )

• 267 Hemochromatosis due to repeated red blood cell transfusions( E83.111 )

• 268 Other hemochromatosis( E83.118 )

• 269 Hemochromatosis, unspecified( E83.119 )

• 270 Other disorders of iron metabolism( E83.19 )

• 271 Disorders of zinc metabolism( E83.2 )

• 272 Disorders of phosphorus metabolism and phosphatases( E83.3 )

• 273 Disorder of phosphorus metabolism, unspecified( E83.30 )

• 274 Familial hypophosphatemia( E83.31 )

• 275 Hereditary vitamin D-dependent rickets (type 1) (type 2)( E83.32 )

• 276 Other disorders of phosphorus metabolism( E83.39 )

• 277 Disorders of magnesium metabolism( E83.4 )

• 278 Disorders of magnesium metabolism, unspecified( E83.40 )

• 279 Hypermagnesemia( E83.41 )

• 280 Hypomagnesemia( E83.42 )

• 281 Other disorders of magnesium metabolism( E83.49 )

• 282 Disorders of calcium metabolism( E83.5 )

• 283 Unspecified disorder of calcium metabolism( E83.50 )

• 284 Hypocalcemia( E83.51 )

• 285 Hypercalcemia( E83.52 )

• 286 Other disorders of calcium metabolism( E83.59 )

• 287 Other disorders of mineral metabolism( E83.8 )

• 288 Hungry bone syndrome( E83.81 )

• 289 Disorders of pyrophosphate metabolism( E83.82 )

• 290 Generalized arterial calcification of infancy with unspecified genetic causality( E83.820 )

• 291 ENPP1 deficiency causing generalized arterial calcification of infancy( E83.821 )

• 292 ENPP1 deficiency causing autosomal recessive hypophosphatemic rickets type 2( E83.822 )

• 293 ABCC6 deficiency causing generalized arterial calcification of infancy( E83.823 )

• 294 ABCC6 deficiency causing pseudoxanthoma elasticum( E83.824 )

• 295 CD73 deficiency causing arterial calcification( E83.825 )

• 296 Other disorders of mineral metabolism( E83.89 )

• 297 Disorder of mineral metabolism, unspecified( E83.9 )

• 298 Cystic fibrosis( E84 )

• 299 Cystic fibrosis with pulmonary manifestations( E84.0 )

• 300 Cystic fibrosis with intestinal manifestations( E84.1 )

• 301 Meconium ileus in cystic fibrosis( E84.11 )

• 302 Cystic fibrosis with other intestinal manifestations( E84.19 )

• 303 Cystic fibrosis with other manifestations( E84.8 )

• 304 Cystic fibrosis, unspecified( E84.9 )

• 305 Amyloidosis( E85 )

• 306 Non-neuropathic heredofamilial amyloidosis( E85.0 )

• 307 Neuropathic heredofamilial amyloidosis( E85.1 )

• 308 Heredofamilial amyloidosis, unspecified( E85.2 )

• 309 Secondary systemic amyloidosis( E85.3 )

• 310 Organ-limited amyloidosis( E85.4 )

• 311 Other amyloidosis( E85.8 )

• 312 Light chain (AL) amyloidosis( E85.81 )

• 313 Wild-type transthyretin-related (ATTR) amyloidosis( E85.82 )

• 314 Other amyloidosis( E85.89 )

• 315 Amyloidosis, unspecified( E85.9 )

• 316 Volume depletion( E86 )

• 317 Dehydration( E86.0 )

• 318 Hypovolemia( E86.1 )

• 319 Volume depletion, unspecified( E86.9 )

• 320 Other disorders of fluid, electrolyte and acid-base balance( E87 )

• 321 Hyperosmolality and hypernatremia( E87.0 )

• 322 Hypo-osmolality and hyponatremia( E87.1 )

• 323 Acidosis( E87.2 )

• 324 Acidosis, unspecified( E87.20 )

• 325 Acute metabolic acidosis( E87.21 )

• 326 Chronic metabolic acidosis( E87.22 )

• 327 Other acidosis( E87.29 )

• 328 Alkalosis( E87.3 )

• 329 Mixed disorder of acid-base balance( E87.4 )

• 330 Hyperkalemia( E87.5 )

• 331 Hypokalemia( E87.6 )

• 332 Fluid overload( E87.7 )

• 333 Fluid overload, unspecified( E87.70 )

• 334 Transfusion associated circulatory overload( E87.71 )

• 335 Other fluid overload( E87.79 )

• 336 Other disorders of electrolyte and fluid balance, not elsewhere classified( E87.8 )

• 337 Other and unspecified metabolic disorders( E88 )

• 338 Disorders of plasma-protein metabolism, not elsewhere classified( E88.0 )

• 339 Alpha-1-antitrypsin deficiency( E88.01 )

• 340 Plasminogen deficiency( E88.02 )

• 341 Other disorders of plasma-protein metabolism, not elsewhere classified( E88.09 )

• 342 Lipodystrophy, not elsewhere classified( E88.1 )

• 343 Lipodystrophy, unspecified( E88.10 )

• 344 Partial lipodystrophy( E88.11 )

• 345 Generalized lipodystrophy( E88.12 )

• 346 Localized lipodystrophy( E88.13 )

• 347 HIV-associated lipodystrophy( E88.14 )

• 348 Other lipodystrophy, not elsewhere classified( E88.19 )

• 349 Lipomatosis, not elsewhere classified( E88.2 )

• 350 Tumor lysis syndrome( E88.3 )

• 351 Mitochondrial metabolism disorders( E88.4 )

• 352 Mitochondrial metabolism disorder, unspecified( E88.40 )

• 353 MELAS syndrome( E88.41 )

• 354 MERRF syndrome( E88.42 )

• 355 Disorders of mitochondrial tRNA synthetases( E88.43 )

• 356 Other mitochondrial metabolism disorders( E88.49 )

• 357 Other specified metabolic disorders( E88.8 )

• 358 Metabolic syndrome and other insulin resistance( E88.81 )

• 359 Metabolic syndrome( E88.810 )

• 360 Insulin resistance syndrome, Type A( E88.811 )

• 361 Other insulin resistance( E88.818 )

• 362 Insulin resistance, unspecified( E88.819 )

• 363 Obesity due to disruption of MC4R pathway( E88.82 )

• 364 Other specified metabolic disorders( E88.89 )

• 365 Metabolic disorder, unspecified( E88.9 )

• 366 Wasting disease (syndrome) due to underlying condition( E88.A )