| Dx | CC/MCC | Exclusions | Description |
| D57213 | MCC | 0497:49 codes | Sickle-cell/Hb-C disease with cerebral vascular involvement |
| D57214 | MCC | 0497:49 codes | Sickle-cell/Hb-C disease with dactylitis |
| D57218 | MCC | 0497:49 codes | Sickle-cell/Hb-C disease with crisis with other specified complication |
| D57219 | MCC | 0496:125 codes | Sickle-cell/Hb-C disease with crisis, unspecified |
| D57411 | MCC | 0496:125 codes | Sickle-cell thalassemia, unspecified, with acute chest syndrome |
| D57412 | MCC | 0496:125 codes | Sickle-cell thalassemia, unspecified, with splenic sequestration |
| D57413 | MCC | 0498:47 codes | Sickle-cell thalassemia, unspecified, with cerebral vascular involvement |
| D57414 | MCC | 0498:47 codes | Sickle-cell thalassemia, unspecified, with dactylitis |
| D57418 | MCC | 0498:47 codes | Sickle-cell thalassemia, unspecified, with crisis with other specified complication |
| D57419 | MCC | 0496:125 codes | Sickle-cell thalassemia, unspecified, with crisis |
| D57431 | MCC | 0499:51 codes | Sickle-cell thalassemia beta zero with acute chest syndrome |
| D57432 | MCC | 0499:51 codes | Sickle-cell thalassemia beta zero with splenic sequestration |
| D57433 | MCC | 0499:51 codes | Sickle-cell thalassemia beta zero with cerebral vascular involvement |
| D57434 | MCC | 0499:51 codes | Sickle-cell thalassemia beta zero with dactylitis |
| D57438 | MCC | 0499:51 codes | Sickle-cell thalassemia beta zero with crisis with other specified complication |
| D57439 | MCC | 0499:51 codes | Sickle-cell thalassemia beta zero with crisis, unspecified |
| D57451 | MCC | 0499:51 codes | Sickle-cell thalassemia beta plus with acute chest syndrome |
| D57452 | MCC | 0499:51 codes | Sickle-cell thalassemia beta plus with splenic sequestration |
| D57453 | MCC | 0499:51 codes | Sickle-cell thalassemia beta plus with cerebral vascular involvement |
| D57454 | MCC | 0499:51 codes | Sickle-cell thalassemia beta plus with dactylitis |
| D57458 | MCC | 0499:51 codes | Sickle-cell thalassemia beta plus with crisis with other specified complication |
| D57459 | MCC | 0499:51 codes | Sickle-cell thalassemia beta plus with crisis, unspecified |
| D57811 | MCC | 0496:125 codes | Other sickle-cell disorders with acute chest syndrome |
| D57812 | MCC | 0496:125 codes | Other sickle-cell disorders with splenic sequestration |
| D57813 | MCC | 0500:48 codes | Other sickle-cell disorders with cerebral vascular involvement |
| D57814 | MCC | 0500:48 codes | Other sickle-cell disorders with dactylitis |
| D57818 | MCC | 0500:48 codes | Other sickle-cell disorders with crisis with other specified complication |
| D57819 | MCC | 0496:125 codes | Other sickle-cell disorders with crisis, unspecified |
| D588 | CC | 0501:104 codes | Other specified hereditary hemolytic anemias |
| D589 | CC | 0501:104 codes | Hereditary hemolytic anemia, unspecified |
| D590 | CC | 0502:112 codes | Drug-induced autoimmune hemolytic anemia |
| D5910 | CC | 0503:16 codes | Autoimmune hemolytic anemia, unspecified |
| D5911 | CC | 0503:16 codes | Warm autoimmune hemolytic anemia |
| D5912 | CC | 0503:16 codes | Cold autoimmune hemolytic anemia |
| D5913 | CC | 0503:16 codes | Mixed type autoimmune hemolytic anemia |
| D5919 | CC | 0503:16 codes | Other autoimmune hemolytic anemia |
| D592 | CC | 0502:112 codes | Drug-induced nonautoimmune hemolytic anemia |
| D5930 | MCC | 0504:110 codes | Hemolytic-uremic syndrome, unspecified |
| D5931 | MCC | 0504:110 codes | Infection-associated hemolytic-uremic syndrome |
| D5932 | MCC | 0504:110 codes | Hereditary hemolytic-uremic syndrome |
| D5939 | MCC | 0504:110 codes | Other hemolytic-uremic syndrome |
| D594 | CC | 0504:110 codes | Other nonautoimmune hemolytic anemias |
| D599 | CC | 0504:110 codes | Acquired hemolytic anemia, unspecified |
| D600 | MCC | 0504:110 codes | Chronic acquired pure red cell aplasia |
| D601 | MCC | 0504:110 codes | Transient acquired pure red cell aplasia |
| D608 | MCC | 0504:110 codes | Other acquired pure red cell aplasias |
| D609 | MCC | 0504:110 codes | Acquired pure red cell aplasia, unspecified |
| D6101 | CC | 0505:98 codes | Constitutional (pure) red blood cell aplasia |
| D6102 | CC | 0506:101 codes | Shwachman-Diamond syndrome |
| D6103 | CC | 0507:102 codes | Fanconi anemia |
| D6109 | CC | 0507:102 codes | Other constitutional aplastic anemia |
| D611 | MCC | 0504:110 codes | Drug-induced aplastic anemia |
| D612 | MCC | 0504:110 codes | Aplastic anemia due to other external agents |
| D613 | MCC | 0504:110 codes | Idiopathic aplastic anemia |
| D61810 | MCC | 0508:41 codes | Antineoplastic chemotherapy induced pancytopenia |
| D61811 | MCC | 0508:41 codes | Other drug-induced pancytopenia |
| D61818 | CC | 0508:41 codes | Other pancytopenia |
| D6182 | CC | 0508:41 codes | Myelophthisis |
| D6189 | MCC | 0504:110 codes | Other specified aplastic anemias and other bone marrow failure syndromes |
| D619 | CC | 0504:110 codes | Aplastic anemia, unspecified |
| D62 | CC | 0509:111 codes | Acute posthemorrhagic anemia |
| D65 | MCC | 0510:50 codes | Disseminated intravascular coagulation [defibrination syndrome] |
| D66 | MCC | 0510:50 codes | Hereditary factor VIII deficiency |
| D67 | MCC | 0510:50 codes | Hereditary factor IX deficiency |
| D6800 | CC | 0511:49 codes | Von Willebrand disease, unspecified |
| D6801 | CC | 0511:49 codes | Von Willebrand disease, type 1 |
| D68020 | CC | 0511:49 codes | Von Willebrand disease, type 2A |
| D68021 | CC | 0511:49 codes | Von Willebrand disease, type 2B |
| D68022 | CC | 0511:49 codes | Von Willebrand disease, type 2M |
| D68023 | CC | 0511:49 codes | Von Willebrand disease, type 2N |
| D68029 | CC | 0511:49 codes | Von Willebrand disease, type 2, unspecified |
| D6803 | CC | 0511:49 codes | Von Willebrand disease, type 3 |
| D6804 | CC | 0511:49 codes | Acquired von Willebrand disease |
| D6809 | CC | 0511:49 codes | Other von Willebrand disease |
| D681 | CC | 0510:50 codes | Hereditary factor XI deficiency |
| D682 | CC | 0510:50 codes | Hereditary deficiency of other clotting factors |
| D68311 | CC | 0510:50 codes | Acquired hemophilia |
| D68312 | CC | 0512:52 codes | Antiphospholipid antibody with hemorrhagic disorder |
| D68318 | CC | 0510:50 codes | Other hemorrhagic disorder due to intrinsic circulating anticoagulants, antibodies, or inhibitors |
| D6832 | CC | 0510:50 codes | Hemorrhagic disorder due to extrinsic circulating anticoagulants |
| D684 | CC | 0510:50 codes | Acquired coagulation factor deficiency |
| D6851 | CC | 0513:40 codes | Activated protein C resistance |
| D6852 | CC | 0513:40 codes | Prothrombin gene mutation |
| D6859 | CC | 0514:42 codes | Other primary thrombophilia |
| D6861 | CC | 0513:40 codes | Antiphospholipid syndrome |
| D6862 | CC | 0513:40 codes | Lupus anticoagulant syndrome |
| D6869 | CC | 0514:42 codes | Other thrombophilia |
| D688 | CC | 0510:50 codes | Other specified coagulation defects |
| D689 | CC | 0510:50 codes | Coagulation defect, unspecified |
| D690 | CC | 0510:50 codes | Allergic purpura |
| D693 | CC | 0515:52 codes | Immune thrombocytopenic purpura |
| D6941 | CC | 0515:52 codes | Evans syndrome |
| D6942 | CC | 0515:52 codes | Congenital and hereditary thrombocytopenia purpura |
| D740 | CC | 0516:24 codes | Congenital methemoglobinemia |
| D748 | CC | 0516:24 codes | Other methemoglobinemias |
| D749 | CC | 0516:24 codes | Methemoglobinemia, unspecified |
| D7581 | CC | 0487:133 codes | Myelofibrosis |
| D761 | CC | 0517:39 codes | Hemophagocytic lymphohistiocytosis |
| D762 | CC | 0518:39 codes | Hemophagocytic syndrome, infection-associated |
| D763 | CC | 0519:39 codes | Other histiocytosis syndromes |
| D7801 | CC | 0520:78 codes | Intraoperative hemorrhage and hematoma of the spleen complicating a procedure on the spleen |
| D7802 | CC | 0520:78 codes | Intraoperative hemorrhage and hematoma of the spleen complicating other procedure |
| D7811 | CC | 0521:76 codes | Accidental puncture and laceration of the spleen during a procedure on the spleen |
| D7812 | CC | 0521:76 codes | Accidental puncture and laceration of the spleen during other procedure |
| D7821 | CC | 0520:78 codes | Postprocedural hemorrhage of the spleen following a procedure on the spleen |
| D7822 | CC | 0520:78 codes | Postprocedural hemorrhage of the spleen following other procedure |
| D7831 | CC | 0520:78 codes | Postprocedural hematoma of the spleen following a procedure on the spleen |
| D7832 | CC | 0520:78 codes | Postprocedural hematoma of the spleen following other procedure |
| D7833 | CC | 0520:78 codes | Postprocedural seroma of the spleen following a procedure on the spleen |
| D7834 | CC | 0520:78 codes | Postprocedural seroma of the spleen following other procedure |
| D7881 | CC | 0522:18 codes | Other intraoperative complications of the spleen |
| D7889 | CC | 0522:18 codes | Other postprocedural complications of the spleen |
| D800 | CC | 0523:44 codes | Hereditary hypogammaglobulinemia |
| D801 | CC | 0524:46 codes | Nonfamilial hypogammaglobulinemia |
| D802 | CC | 0524:46 codes | Selective deficiency of immunoglobulin A [IgA] |
| D803 | CC | 0523:44 codes | Selective deficiency of immunoglobulin G [IgG] subclasses |
| D804 | CC | 0523:44 codes | Selective deficiency of immunoglobulin M [IgM] |
| D805 | CC | 0523:44 codes | Immunodeficiency with increased immunoglobulin M [IgM] |
| D806 | CC | 0523:44 codes | Antibody deficiency with near-normal immunoglobulins or with hyperimmunoglobulinemia |
| D807 | CC | 0523:44 codes | Transient hypogammaglobulinemia of infancy |
| D808 | CC | 0523:44 codes | Other immunodeficiencies with predominantly antibody defects |
| D809 | CC | 0523:44 codes | Immunodeficiency with predominantly antibody defects, unspecified |
| D810 | CC | 0523:44 codes | Severe combined immunodeficiency [SCID] with reticular dysgenesis |
| D811 | CC | 0523:44 codes | Severe combined immunodeficiency [SCID] with low T- and B-cell numbers |
| D812 | CC | 0523:44 codes | Severe combined immunodeficiency [SCID] with low or normal B-cell numbers |
| D8130 | CC | 0525:35 codes | Adenosine deaminase deficiency, unspecified |
| D8131 | CC | 0525:35 codes | Severe combined immunodeficiency due to adenosine deaminase deficiency |
| D8132 | CC | 0525:35 codes | Adenosine deaminase 2 deficiency |
| D8139 | CC | 0525:35 codes | Other adenosine deaminase deficiency |
| D814 | CC | 0523:44 codes | Nezelof's syndrome |
| D815 | CC | 0525:35 codes | Purine nucleoside phosphorylase [PNP] deficiency |
| D816 | CC | 0523:44 codes | Major histocompatibility complex class I deficiency |
| D817 | CC | 0523:44 codes | Major histocompatibility complex class II deficiency |
| D8182 | CC | 0523:44 codes | Activated Phosphoinositide 3-kinase Delta Syndrome [APDS] |
| D8189 | CC | 0523:44 codes | Other combined immunodeficiencies |
| D819 | CC | 0523:44 codes | Combined immunodeficiency, unspecified |
| D820 | CC | 0523:44 codes | Wiskott-Aldrich syndrome |
| D821 | CC | 0523:44 codes | Di George's syndrome |
| D830 | CC | 0523:44 codes | Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function |
| D831 | CC | 0523:44 codes | Common variable immunodeficiency with predominant immunoregulatory T-cell disorders |
| D832 | CC | 0523:44 codes | Common variable immunodeficiency with autoantibodies to B- or T-cells |
| D838 | CC | 0523:44 codes | Other common variable immunodeficiencies |
| D839 | CC | 0523:44 codes | Common variable immunodeficiency, unspecified |
| D8481 | CC | 0523:44 codes | Immunodeficiency due to conditions classified elsewhere |
| D84821 | CC | 0523:44 codes | Immunodeficiency due to drugs |
| D84822 | CC | 0523:44 codes | Immunodeficiency due to external causes |
| D8489 | CC | 0523:44 codes | Other immunodeficiencies |
| D849 | CC | 0523:44 codes | Immunodeficiency, unspecified |
| D89810 | CC | 0526:45 codes | Acute graft-versus-host disease |
| D89811 | CC | 0526:45 codes | Chronic graft-versus-host disease |
| D89812 | CC | 0526:45 codes | Acute on chronic graft-versus-host disease |
| D89813 | CC | 0526:45 codes | Graft-versus-host disease, unspecified |
| D89833 | CC | No Excl | Cytokine release syndrome, grade 3 |
| D89834 | CC | No Excl | Cytokine release syndrome, grade 4 |
| D89835 | CC | No Excl | Cytokine release syndrome, grade 5 |
| E035 | MCC | 0527:718 codes | Myxedema coma |
| E0501 | MCC | 0528:59 codes | Thyrotoxicosis with diffuse goiter with thyrotoxic crisis or storm |
| E0511 | MCC | 0528:59 codes | Thyrotoxicosis with toxic single thyroid nodule with thyrotoxic crisis or storm |
| E0521 | MCC | 0528:59 codes | Thyrotoxicosis with toxic multinodular goiter with thyrotoxic crisis or storm |
| E0531 | MCC | 0528:59 codes | Thyrotoxicosis from ectopic thyroid tissue with thyrotoxic crisis or storm |
| E0541 | MCC | 0528:59 codes | Thyrotoxicosis factitia with thyrotoxic crisis or storm |
| E0581 | MCC | 0528:59 codes | Other thyrotoxicosis with thyrotoxic crisis or storm |
| E0591 | MCC | 0528:59 codes | Thyrotoxicosis, unspecified with thyrotoxic crisis or storm |
| E060 | CC | 0529:7 codes | Acute thyroiditis |
| E0800 | MCC | 0530:352 codes | Diabetes mellitus due to underlying condition with hyperosmolarity without nonketotic hyperglycemic-hyperosmolar coma (NKHHC) |
| E0801 | MCC | 0530:352 codes | Diabetes mellitus due to underlying condition with hyperosmolarity with coma |
| E0810 | MCC | 0530:352 codes | Diabetes mellitus due to underlying condition with ketoacidosis without coma |
| E0811 | MCC | 0530:352 codes | Diabetes mellitus due to underlying condition with ketoacidosis with coma |
| E0852 | CC | 0531:45 codes | Diabetes mellitus due to underlying condition with diabetic peripheral angiopathy with gangrene |
| E08641 | MCC | 0530:352 codes | Diabetes mellitus due to underlying condition with hypoglycemia with coma |
| E0900 | MCC | 0530:352 codes | Drug or chemical induced diabetes mellitus with hyperosmolarity without nonketotic hyperglycemic-hyperosmolar coma (NKHHC) |
| E0901 | MCC | 0530:352 codes | Drug or chemical induced diabetes mellitus with hyperosmolarity with coma |
| E0910 | MCC | 0530:352 codes | Drug or chemical induced diabetes mellitus with ketoacidosis without coma |
| E0911 | MCC | 0530:352 codes | Drug or chemical induced diabetes mellitus with ketoacidosis with coma |
| E0952 | CC | 0532:46 codes | Drug or chemical induced diabetes mellitus with diabetic peripheral angiopathy with gangrene |
| E09641 | MCC | 0530:352 codes | Drug or chemical induced diabetes mellitus with hypoglycemia with coma |
| E1010 | MCC | 0533:355 codes | Type 1 diabetes mellitus with ketoacidosis without coma |
| E1011 | MCC | 0533:355 codes | Type 1 diabetes mellitus with ketoacidosis with coma |
| E1052 | CC | 0534:46 codes | Type 1 diabetes mellitus with diabetic peripheral angiopathy with gangrene |
| E10641 | MCC | 0533:355 codes | Type 1 diabetes mellitus with hypoglycemia with coma |
| E1100 | MCC | 0530:352 codes | Type 2 diabetes mellitus with hyperosmolarity without nonketotic hyperglycemic-hyperosmolar coma (NKHHC) |
| E1101 | MCC | 0530:352 codes | Type 2 diabetes mellitus with hyperosmolarity with coma |
| E1110 | MCC | 0530:352 codes | Type 2 diabetes mellitus with ketoacidosis without coma |
| E1111 | MCC | 0530:352 codes | Type 2 diabetes mellitus with ketoacidosis with coma |
| E1152 | CC | 0535:46 codes | Type 2 diabetes mellitus with diabetic peripheral angiopathy with gangrene |
| E11641 | MCC | 0530:352 codes | Type 2 diabetes mellitus with hypoglycemia with coma |
| E1300 | MCC | 0530:352 codes | Other specified diabetes mellitus with hyperosmolarity without nonketotic hyperglycemic-hyperosmolar coma (NKHHC) |
| E1301 | MCC | 0530:352 codes | Other specified diabetes mellitus with hyperosmolarity with coma |
| E1310 | MCC | 0530:352 codes | Other specified diabetes mellitus with ketoacidosis without coma |
| E1311 | MCC | 0530:352 codes | Other specified diabetes mellitus with ketoacidosis with coma |
| E1352 | CC | 0536:46 codes | Other specified diabetes mellitus with diabetic peripheral angiopathy with gangrene |
| E13641 | MCC | 0530:352 codes | Other specified diabetes mellitus with hypoglycemia with coma |
| E15 | CC | 0533:355 codes | Nondiabetic hypoglycemic coma |
| E221 | CC | 0537:4 codes | Hyperprolactinemia |
| E222 | CC | 0538:18 codes | Syndrome of inappropriate secretion of antidiuretic hormone |
| E228 | CC | 0537:4 codes | Other hyperfunction of pituitary gland |
| E229 | CC | 0537:4 codes | Hyperfunction of pituitary gland, unspecified |
| E230 | CC | 0539:21 codes | Hypopituitarism |
| E232 | CC | 0540:19 codes | Diabetes insipidus |
| E240 | CC | 0541:23 codes | Pituitary-dependent Cushing's disease |
| E242 | CC | 0541:23 codes | Drug-induced Cushing's syndrome |
| E243 | CC | 0541:23 codes | Ectopic ACTH syndrome |
| E244 | CC | 0541:23 codes | Alcohol-induced pseudo-Cushing's syndrome |
| E248 | CC | 0541:23 codes | Other Cushing's syndrome |
| E249 | CC | 0541:23 codes | Cushing's syndrome, unspecified |
| E270 | CC | 0542:22 codes | Other adrenocortical overactivity |
| E271 | CC | 0542:22 codes | Primary adrenocortical insufficiency |
| E272 | CC | 0542:22 codes | Addisonian crisis |
| E273 | CC | 0542:22 codes | Drug-induced adrenocortical insufficiency |
| E2740 | CC | 0542:22 codes | Unspecified adrenocortical insufficiency |
| E2749 | CC | 0542:22 codes | Other adrenocortical insufficiency |
| E275 | CC | 0542:22 codes | Adrenomedullary hyperfunction |
| E321 | CC | 0543:11 codes | Abscess of thymus |
| E3400 | CC | 0544:11 codes | Carcinoid syndrome, unspecified |
| E3401 | CC | 0544:11 codes | Carcinoid heart syndrome |
| E3409 | CC | 0544:11 codes | Other carcinoid syndrome |
| E3601 | CC | 0545:78 codes | Intraoperative hemorrhage and hematoma of an endocrine system organ or structure complicating an endocrine system procedure |
| E3602 | CC | 0545:78 codes | Intraoperative hemorrhage and hematoma of an endocrine system organ or structure complicating other procedure |
| E3611 | CC | 0521:76 codes | Accidental puncture and laceration of an endocrine system organ or structure during an endocrine system procedure |
| E3612 | CC | 0521:76 codes | Accidental puncture and laceration of an endocrine system organ or structure during other procedure |
| E40 | MCC | 0546:9 codes | Kwashiorkor |
| E41 | MCC | 0546:9 codes | Nutritional marasmus |
| E42 | MCC | 0546:9 codes | Marasmic kwashiorkor |
| E43 | MCC | 0546:9 codes | Unspecified severe protein-calorie malnutrition |
| E440 | CC | 0546:9 codes | Moderate protein-calorie malnutrition |
| E441 | CC | 0546:9 codes | Mild protein-calorie malnutrition |
| E45 | CC | 0546:9 codes | Retarded development following protein-calorie malnutrition |
| E46 | CC | 0546:9 codes | Unspecified protein-calorie malnutrition |
| E5111 | CC | 0547:34 codes | Dry beriberi |
| E5112 | CC | 0547:34 codes | Wet beriberi |
| E512 | CC | 0547:34 codes | Wernicke's encephalopathy |
| E518 | CC | 0547:34 codes | Other manifestations of thiamine deficiency |
| E519 | CC | 0547:34 codes | Thiamine deficiency, unspecified |
| E530 | CC | 0547:34 codes | Riboflavin deficiency |
| E550 | CC | 0548:34 codes | Rickets, active |
| E640 | CC | 0546:9 codes | Sequelae of protein-calorie malnutrition |
| E662 | CC | 0549:13 codes | Morbid (severe) obesity with alveolar hypoventilation |
| E700 | CC | 0550:140 codes | Classical phenylketonuria |
| E701 | CC | 0550:140 codes | Other hyperphenylalaninemias |
| E7020 | CC | 0550:140 codes | Disorder of tyrosine metabolism, unspecified |
| E7021 | CC | 0550:140 codes | Tyrosinemia |
| E7029 | CC | 0550:140 codes | Other disorders of tyrosine metabolism |
| E7030 | CC | 0550:140 codes | Albinism, unspecified |
| E70310 | CC | 0550:140 codes | X-linked ocular albinism |
| E70311 | CC | 0550:140 codes | Autosomal recessive ocular albinism |
| E70318 | CC | 0550:140 codes | Other ocular albinism |
| E70319 | CC | 0550:140 codes | Ocular albinism, unspecified |
| E70320 | CC | 0550:140 codes | Tyrosinase negative oculocutaneous albinism |
| E70321 | CC | 0550:140 codes | Tyrosinase positive oculocutaneous albinism |
| E70328 | CC | 0550:140 codes | Other oculocutaneous albinism |
| E70329 | CC | 0550:140 codes | Oculocutaneous albinism, unspecified |
| E70330 | CC | 0550:140 codes | Chediak-Higashi syndrome |
| E70331 | CC | 0550:140 codes | Hermansky-Pudlak syndrome |
| E70338 | CC | 0550:140 codes | Other albinism with hematologic abnormality |
| E70339 | CC | 0550:140 codes | Albinism with hematologic abnormality, unspecified |
| E7039 | CC | 0550:140 codes | Other specified albinism |
| E7040 | CC | 0550:140 codes | Disorders of histidine metabolism, unspecified |
| E7041 | CC | 0550:140 codes | Histidinemia |
| E7049 | CC | 0550:140 codes | Other disorders of histidine metabolism |
| E705 | CC | 0550:140 codes | Disorders of tryptophan metabolism |
| E7081 | CC | 0551:8 codes | Aromatic L-amino acid decarboxylase deficiency |
| E7089 | CC | 0551:8 codes | Other disorders of aromatic amino-acid metabolism |
| E709 | CC | 0550:140 codes | Disorder of aromatic amino-acid metabolism, unspecified |
| E710 | CC | 0550:140 codes | Maple-syrup-urine disease |
| E71110 | CC | 0550:140 codes | Isovaleric acidemia |
| E71111 | CC | 0550:140 codes | 3-methylglutaconic aciduria |
| E71118 | CC | 0550:140 codes | Other branched-chain organic acidurias |
| E71120 | CC | 0550:140 codes | Methylmalonic acidemia |
| E71121 | CC | 0550:140 codes | Propionic acidemia |
| E71128 | CC | 0550:140 codes | Other disorders of propionate metabolism |
| E7119 | CC | 0550:140 codes | Other disorders of branched-chain amino-acid metabolism |
| E712 | CC | 0550:140 codes | Disorder of branched-chain amino-acid metabolism, unspecified |
| E71310 | CC | 0552:39 codes | Long chain/very long chain acyl CoA dehydrogenase deficiency |
| E71311 | CC | 0553:39 codes | Medium chain acyl CoA dehydrogenase deficiency |
| E71312 | CC | 0554:39 codes | Short chain acyl CoA dehydrogenase deficiency |
| E71313 | CC | 0555:39 codes | Glutaric aciduria type II |
| E71314 | CC | 0556:39 codes | Muscle carnitine palmitoyltransferase deficiency |
| E71318 | CC | 0557:39 codes | Other disorders of fatty-acid oxidation |
| E7132 | CC | 0558:39 codes | Disorders of ketone metabolism |
| E7139 | CC | 0559:41 codes | Other disorders of fatty-acid metabolism |
| E7150 | CC | 0560:36 codes | Peroxisomal disorder, unspecified |
| E71510 | CC | 0561:36 codes | Zellweger syndrome |
| E71511 | CC | 0562:36 codes | Neonatal adrenoleukodystrophy |
| E71518 | CC | 0563:36 codes | Other disorders of peroxisome biogenesis |
| E71520 | CC | 0564:36 codes | Childhood cerebral X-linked adrenoleukodystrophy |
| E71521 | CC | 0565:36 codes | Adolescent X-linked adrenoleukodystrophy |
| E71522 | CC | 0566:36 codes | Adrenomyeloneuropathy |
| E71528 | CC | 0567:36 codes | Other X-linked adrenoleukodystrophy |
| E71529 | CC | 0568:36 codes | X-linked adrenoleukodystrophy, unspecified type |
| E7153 | CC | 0569:36 codes | Other group 2 peroxisomal disorders |
| E71540 | CC | 0570:36 codes | Rhizomelic chondrodysplasia punctata |
| E71541 | CC | 0571:36 codes | Zellweger-like syndrome |
| E71542 | CC | 0572:36 codes | Other group 3 peroxisomal disorders |
| E71548 | CC | 0573:36 codes | Other peroxisomal disorders |
| E7200 | CC | 0550:140 codes | Disorders of amino-acid transport, unspecified |
| E7201 | CC | 0550:140 codes | Cystinuria |
| E7202 | CC | 0550:140 codes | Hartnup's disease |
| E7203 | CC | 0574:1 code | Lowe's syndrome |
| E7204 | CC | 0550:140 codes | Cystinosis |
| E7209 | CC | 0550:140 codes | Other disorders of amino-acid transport |
| E7210 | CC | 0550:140 codes | Disorders of sulfur-bearing amino-acid metabolism, unspecified |
| E7211 | CC | 0550:140 codes | Homocystinuria |
| E7212 | CC | 0550:140 codes | Methylenetetrahydrofolate reductase deficiency |
| E7219 | CC | 0550:140 codes | Other disorders of sulfur-bearing amino-acid metabolism |
| E7220 | CC | 0550:140 codes | Disorder of urea cycle metabolism, unspecified |
| E7221 | CC | 0550:140 codes | Argininemia |
| E7222 | CC | 0550:140 codes | Arginosuccinic aciduria |
| E7223 | CC | 0550:140 codes | Citrullinemia |
| E7229 | CC | 0550:140 codes | Other disorders of urea cycle metabolism |
| E723 | CC | 0550:140 codes | Disorders of lysine and hydroxylysine metabolism |
| E724 | CC | 0550:140 codes | Disorders of ornithine metabolism |
| E7250 | CC | 0550:140 codes | Disorder of glycine metabolism, unspecified |
| E7251 | CC | 0550:140 codes | Non-ketotic hyperglycinemia |
| E7252 | CC | 0550:140 codes | Trimethylaminuria |
| E72530 | CC | 0575:144 codes | Primary hyperoxaluria, type 1 |
| E72538 | CC | 0575:144 codes | Other specified primary hyperoxaluria |
| E72539 | CC | 0575:144 codes | Primary hyperoxaluria, unspecified |
| E7259 | CC | 0550:140 codes | Other disorders of glycine metabolism |
| E7281 | CC | 0550:140 codes | Disorders of gamma aminobutyric acid metabolism |
| E7289 | CC | 0550:140 codes | Other specified disorders of amino-acid metabolism |
| E729 | CC | 0550:140 codes | Disorder of amino-acid metabolism, unspecified |
| E7400 | CC | 0550:140 codes | Glycogen storage disease, unspecified |
| E7401 | CC | 0550:140 codes | von Gierke disease |
| E7402 | CC | 0550:140 codes | Pompe disease |
| E7403 | CC | 0550:140 codes | Cori disease |
| E7404 | CC | 0550:140 codes | McArdle disease |
| E7405 | CC | 0576:137 codes | Lysosome-associated membrane protein 2 [LAMP2] deficiency |
| E7409 | CC | 0550:140 codes | Other glycogen storage disease |
| E7420 | CC | 0550:140 codes | Disorders of galactose metabolism, unspecified |
| E7421 | CC | 0550:140 codes | Galactosemia |
| E7429 | CC | 0550:140 codes | Other disorders of galactose metabolism |
| E744 | CC | 0550:140 codes | Disorders of pyruvate metabolism and gluconeogenesis |
| E74810 | CC | 0577:55 codes | Glucose transporter protein type 1 deficiency |
| E74818 | CC | 0577:55 codes | Other disorders of glucose transport |
| E74819 | CC | 0577:55 codes | Disorders of glucose transport, unspecified |
| E74820 | CC | 0577:55 codes | SLC13A5 Citrate Transporter Disorder |
| E74829 | CC | 0577:55 codes | Other disorders of citrate metabolism |
| E7489 | CC | 0577:55 codes | Other specified disorders of carbohydrate metabolism |
| E7500 | CC | 0578:26 codes | GM2 gangliosidosis, unspecified |
| E7501 | CC | 0578:26 codes | Sandhoff disease |
| E7502 | CC | 0578:26 codes | Tay-Sachs disease |
| E7509 | CC | 0578:26 codes | Other GM2 gangliosidosis |
| E7510 | CC | 0578:26 codes | Unspecified gangliosidosis |
| E7511 | CC | 0578:26 codes | Mucolipidosis IV |
| E7519 | CC | 0578:26 codes | Other gangliosidosis |
| E7523 | CC | 0579:24 codes | Krabbe disease |
| E7525 | CC | 0579:24 codes | Metachromatic leukodystrophy |
| E7526 | CC | 0579:24 codes | Sulfatase deficiency |
| E7527 | CC | 0579:24 codes | Pelizaeus-Merzbacher disease |
| E7528 | CC | 0579:24 codes | Canavan disease |
| E7529 | CC | 0579:24 codes | Other sphingolipidosis |
| E754 | CC | 0578:26 codes | Neuronal ceroid lipofuscinosis |
| E7601 | CC | 0580:37 codes | Hurler's syndrome |
| E7602 | CC | 0580:37 codes | Hurler-Scheie syndrome |
| E7603 | CC | 0580:37 codes | Scheie's syndrome |
| E761 | CC | 0580:37 codes | Mucopolysaccharidosis, type II |
| E76210 | CC | 0580:37 codes | Morquio A mucopolysaccharidoses |
| E76211 | CC | 0580:37 codes | Morquio B mucopolysaccharidoses |
| E76219 | CC | 0580:37 codes | Morquio mucopolysaccharidoses, unspecified |
| E7622 | CC | 0580:37 codes | Sanfilippo mucopolysaccharidoses |
| E7629 | CC | 0580:37 codes | Other mucopolysaccharidoses |
| E763 | CC | 0580:37 codes | Mucopolysaccharidosis, unspecified |
| E768 | CC | 0580:37 codes | Other disorders of glucosaminoglycan metabolism |
| E769 | CC | 0580:37 codes | Glucosaminoglycan metabolism disorder, unspecified |
| E7871 | CC | 0581:41 codes | Barth syndrome |
| E7872 | CC | 0581:41 codes | Smith-Lemli-Opitz syndrome |
| E791 | CC | 0525:35 codes | Lesch-Nyhan syndrome |
| E792 | CC | 0525:35 codes | Myoadenylate deaminase deficiency |
| E7981 | CC | 0525:35 codes | Aicardi-Goutieres syndrome |
| E7982 | CC | 0525:35 codes | Hereditary xanthinuria |
| E7989 | CC | 0525:35 codes | Other specified disorders of purine and pyrimidine metabolism |
| E799 | CC | 0525:35 codes | Disorder of purine and pyrimidine metabolism, unspecified |
| E800 | CC | 0525:35 codes | Hereditary erythropoietic porphyria |
| E801 | CC | 0525:35 codes | Porphyria cutanea tarda |
| E8020 | CC | 0525:35 codes | Unspecified porphyria |
| E8021 | CC | 0525:35 codes | Acute intermittent (hepatic) porphyria |
| E8029 | CC | 0525:35 codes | Other porphyria |
| E803 | CC | 0582:41 codes | Defects of catalase and peroxidase |
| E83820 | CC | 0583:4 codes | Generalized arterial calcification of infancy with unspecified genetic causality |
| E83821 | CC | 0583:4 codes | ENPP1 deficiency causing generalized arterial calcification of infancy |
| E83823 | CC | 0583:4 codes | ABCC6 deficiency causing generalized arterial calcification of infancy |
| E83825 | CC | 0583:4 codes | CD73 deficiency causing arterial calcification |
| E840 | MCC | 0584:5 codes | Cystic fibrosis with pulmonary manifestations |
| E8411 | MCC | 0584:5 codes | Meconium ileus in cystic fibrosis |
| E8419 | CC | 0584:5 codes | Cystic fibrosis with other intestinal manifestations |
| E848 | CC | 0584:5 codes | Cystic fibrosis with other manifestations |
| E849 | CC | 0584:5 codes | Cystic fibrosis, unspecified |
| E850 | CC | 0585:10 codes | Non-neuropathic heredofamilial amyloidosis |
| E851 | CC | 0585:10 codes | Neuropathic heredofamilial amyloidosis |
| E852 | CC | 0585:10 codes | Heredofamilial amyloidosis, unspecified |
| E853 | CC | 0585:10 codes | Secondary systemic amyloidosis |
| E854 | CC | 0585:10 codes | Organ-limited amyloidosis |
| E8581 | CC | 0585:10 codes | Light chain (AL) amyloidosis |
| E8582 | CC | 0585:10 codes | Wild-type transthyretin-related (ATTR) amyloidosis |
| E8589 | CC | 0585:10 codes | Other amyloidosis |
| E859 | CC | 0585:10 codes | Amyloidosis, unspecified |
| E870 | CC | 0586:17 codes | Hyperosmolality and hypernatremia |
| E871 | CC | 0586:17 codes | Hypo-osmolality and hyponatremia |
| E8720 | CC | 0586:17 codes | Acidosis, unspecified |
| E8721 | CC | 0586:17 codes | Acute metabolic acidosis |