Documentation and Coding: Other Significant Endocrine and Metabolic Disorders

Waterhouse-Friderichsen syndrome 

Meningococcus infection with adrenal gland failure 

Waldenstrom macroglobulinemia 

Cancer with too much protein in the blood 

Defects in the complement system 

Problem with complement cells in immune system 

Cryoglobulinemia 

Abnormal proteins in the blood 

Myxedema coma 

Coma due to underactive thyroid gland; Postsurgical and post irradiation hypothyroidism resulting from the removal or radiation of thyroid tissue is not included in this category. They are located later in this chapter with code E89.0 Postprocedural hypothyroidism. 

Nondiabetic hypoglycemic coma 

Hypoglycemia is an abnormally diminished content of glucose in the blood that may occur in a nondiabetic patient because of excessive insulin produced in the body (hyperinsulinemia), inborn error of metabolism, medications and poisons, alcohol, hormone deficiencies, prolonged starvation, alterations of metabolism associated with infection, or organ failure. Prolonged extreme hypoglycemia may lead to stupor and coma. 

Hypoparathyroidism 

Hypoparathyroidism is a disorder in which there is not enough parathyroid hormone (PTH) secreted from one or more of the parathyroid glands. 

Hyperparathyroidism and other disorders of parathyroid gland 

Hyperparathyroidism is an excessive secretion of the parathyroid hormone (PTH) from one or more of the parathyroid glands. Includes Secondary and Tertiary Hyperparathyroidism 

Hyperfunction of pituitary gland 

Excludes Cushing’s Syndrome, and Nelson’s Syndrome; The most common cause of pituitary hyperfunction is pituitary adenoma. 

Hypofunction and other disorders of the pituitary gland 

Underlying causes of pituitary hypofunction relate to dysfunction of the pituitary itself and/or to the hypothalamus gland. This code includes the listed conditions whether the disorder is in the pituitary or the hypothalamus (Example-Sheehan’s Syndrome, Simmonds’ Disease) 

Pituitary-dependent Cushing's disease 

Too much cortisol due to pituitary gland problem 

Adrenogenital disorders 

Adrenogenital disorders are a group of conditions caused by a family of autosomal recessive disorders of steroid hormone production in the adrenal glands leading to a deficiency of cortisol, the stress fighting hormone. 

Hyperaldosteronism 

Hyperaldosteronism is a disease caused by an excess production of the normal adrenal hormone aldosterone 

Other disorders of adrenal gland 

Other disorders of the adrenal gland may involve the adrenal cortex or the adrenal medulla. (Example-Addison’s Disease) 

Polyglandular dysfunction 

Polyglandular deficiency syndromes (PDS) are characterized by sequential or simultaneous deficiencies in the function of several endocrine glands that have a common cause. (Example-Sipple’s Syndrome, Wermer’s Syndrome) 

Diseases of thymus 

The thymus gland is comprised of lymphatic and epithelial tissue. It processes white blood cells (WBC), which kill foreign cells and stimulates other immune cells to produce antibodies. 

Constitutional tall stature 

Tall stature is height over two standard deviations above the mean for the person's gender and age. There may be a family history of tallness. 

Disorders of Aromatic amino-acid metabolism 

Disorders of aromatic amino-acid metabolism manifest in many ways, causing phenylketonuria, albinism, and other conditions. 

Disorders of Branched-chain amino-acid metabolism and fatty-acid metabolism 

Amino acids are classified based on certain characteristics of their molecular structures with branched chain being one type of molecular structure. Fatty acids are carboxylic acids with long hydrocarbon chains. Conditions classified here involve the inability to break down and use branched chain amino acids and fatty acids. (Example-Maple-Syrup Urine Disease) 

Other disorders of amino-acid metabolism 

Hereditary disorders of amino-acid metabolism can result from defects in breaking down amino acids or getting them into cells. Disorders of amino-acid metabolism in this category include disorders of amino-acid transport, sulfur-bearing amino-acid metabolism, urea cycle metabolism, lysine and hydroxylysine metabolism, ornithine metabolism, and glycine metabolism. 

Other disorders of carbohydrate metabolism 

Carbohydrate metabolism disorders are a rare hereditary group of metabolic disorders caused by deficiency of the enzymes necessary to process certain carbohydrates into sugars such as glucose, galactose, fructose, and glycogen. 

Other sphingolipidosis 

Lipid storage disorders that relate to sphingolipid metabolism. 

Includes: Fabry (-Anderson) disease, Gaucher disease, Krabbe disease, Niemann-Pick disease types A, B, C, and D, which are classified based on the genetic cause and the signs and symptoms; metachromatic leukodystrophy; multiple sulfatase deficiency (MSD); and other and unspecified sphingolipidosis. 

Disorders of Glycosaminoglycan metabolism 

Includes: Sanfilippo Syndrome, Morquio Syndromes, Hurler’s Syndrome, Scheie’s Syndrome 

Disorders of Glycoprotein metabolism 

Includes: Fucosidosis 

Disorders of Purine and pyrimidine metabolism 

Includes: Lesch-Nyhan Syndrome, Hereditary xanthinuria 

Disorders of Porphyrin and bilirubin metabolism 

Includes: Gilbert Syndrome, Crigler-Najjar Syndrome 

Hereditary hemochromatosis 

Too much iron in the body (hemochromatosis); Hemochromatosis that is documented as gestational or neonatal is coded to P78.84 Gestational alloimmune liver disease. 

Amyloidosis 

Amyloidosis is a condition in which abnormal proteins are deposited in various organs and tissues. The deposits damage the tissues and interfere with the function of the involved organ. Amyloidosis occurs in multiple forms: spontaneous, hereditary, secondary to hemodialysis, and resulting from a cancer of the blood cells called myeloma.  

Other and unspecified metabolic disorders 

A variety of metabolic disorders are classified here, including disorders of plasma-protein metabolism, tumor lysis syndrome, mitochondrial metabolism disorders, and metabolic syndrome 

Postprocedural endocrine and metabolic complications and disorders, not elsewhere classified 

Surgery and other procedures such as radiation therapy can impact the ability of the affected endocrine gland to produce hormones in sufficient quantities. Procedures on or near an endocrine gland can compromise function leading to hypofunction or failure of the gland.  

Kearns-Sayre syndrome 

Strabismus disorders relate to the eyes' inability to coordinate focus in the same direction due to conditions affecting the muscles controlling this function. Include laterality and use additional code for other manifestation, such as heart block (I45.9) 

Nephrogenic diabetes insipidus 

Type of diabetes is due to the inability of renal tubules to reabsorb water back into the body. It is not responsive to vasopressin (antidiuretic hormone), and it is characterized by excessive thirst and excessive urine production. It may develop into chronic renal insufficiency. 

Secondary hyperparathyroidism of renal origin  

Parathyroid dysfunction caused by chronic renal failure. Phosphate clearance and vitamin D production are impaired resulting in lowered calcium blood levels and an excessive production of parathyroid hormone.