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         Intrauterine Dwarfism:     more detail

81. III. ENDOCRINE, NUTRITIONAL AND METABOLIC DISEASES, AND IMMUNITY
259.3 Ectopic hormone secretion, not elsewhere classified 259.4 dwarfism, NOS constitutional Excludes dwarfism achondroplastic (756.4) intrauterine
http://www.nber.org/mortality/1995/docs/ch03.txt
III. ENDOCRINE, NUTRITIONAL AND METABOLIC DISEASES, AND IMMUNITY DISORDERS Excludes: endocrine and metabolic disturbances specific to the fetus and newborn (775.-) Note: All neoplasms, whether functionally active or not, are classified in Chapter 2. Codes in Chapter 3 (i.e., 242.8, 246.0, 251-253, 255-259) may be used, if desired, to identify such functional activity associated with any neoplasm, or by ectopic endocrine tissue. DISORDERS OF THYROID GLAND (240-246) 240 Simple and unspecified goiter 240.0 Goiter, specified as simple Includes: conditions in 240.9, specified as simple 240.9 Goiter, unspecified Enlargement of thyroid Goiter or struma: NOS diffuse colloid endemic hyperplastic parenchymatous Excludes: congenital (dyshormonogenic) goiter (246.1) 241 Nontoxic nodular goiter Excludes: adenoma of thyroid (226) cystadenoma of thyroid (226) 241.0 Nontoxic uninodular goiter Thyroid nodule Uninodular goiter (nontoxic) 241.1 Nontoxic multinodular goiter Multinodular goiter (nontoxic) 241.9 Unspecified Adenomatous goiter Struma nodosa (simplex) Nodular goiter (nontoxic) NOS 242 Thyrotoxicosis with or without goiter Excludes: neonatal thyrotoxicosis (775.3) ICD-9/Ch03.TXT - pg. 1 242.0 Toxic diffuse goiter Exophthalmic or toxic goiter NOS 242.1 Toxic uninodular goiter Thyroid nodule, toxic or with hyperthyroidism Uninodular goiter, toxic or with hyperthyroidism 242.2 Toxic multinodular goiter 242.3 Toxic nodular goiter, unspecified Any condition in 241.9 specified as toxic or with hyperthyroidism 242.4 Thyrotoxicosis from ectopic thyroid nodule 242.8 Thyrotoxicosis of other specified origin Overproduction of thyroid-stimulating hormone Thyrotoxicosis: factitia from ingestion of excessive thyroid material Use additional E code, if desired, to identify cause, if drug-induced 242.9 Thyrotoxicosis without mention of goiter or other cause Hyperthyroidism NOS 243 Congenital hypothyroidism Congenital thyroid insufficiency Cretinism Use additional code, if desired, to identify associated mental retardation Excludes: congenital (dyshormonogenic) goiter (246.1) 244 Acquired hypothyroidism [Not to be used for underlying cause of death] Includes: hypothyroidism (acquired) myxedema (adult) (juvenile) 244.0 Postsurgical hypothyroidism 244.1 Other postablative hypothyroidism Hypothyroidism following therapy, such as irradiation ICD-9/Ch03.TXT - pg. 2 244.2 Iodine hypothyroidism Hypothyroidism resulting from administration or ingestion of iodide Use additional E code, if desired, to identify drug 244.3 Other iatrogenic hypothyroidism Hypothyroidism resulting from: P-aminosalicylic acid [PAS] Phenylbutazone Resorcinol Use additional E code, if desired, to identify drug 244.8 Other 244.9 Unspecified hypothyroidism Hypothyroidism, primary or NOS Myxedema, primary or NOS 245 Thyroiditis 245.0 Acute thyroiditis Abscess of thyroid Thyroiditis: pyogenic suppurative Use additional code, if desired, to identify organism 245.1 Subacute thyroiditis Thyroiditis: de Quervain's giant-cell granulomatous 245.2 Chronic lymphocytic thyroiditis Autoimmune thyroiditis Lymphocytic thyroiditis (chronic) Hashimoto's disease Struma lymphomatosa 245.3 Chronic fibrous thyroiditis Thyroiditis: ligneous Riedel's ICD-9/Ch03.TXT - pg. 3 245.4 Iatrogenic thyroiditis Use additional code, if desired, to identify cause 245.8 Other and unspecified chronic thyroiditis Chronic thyroiditis NOS 245.9 Unspecified Thyroiditis NOS 246 Other disorders of thyroid 246.0 Disorders of thyrocalcitonin secretion Hypersecretion of calcitonin or thyrocalcitonin 246.1 Dyshormonogenic goiter Congenital goiter Goiter due to enzyme defect in synthesis of thyroid hormone 246.2 Cyst of thyroid Excludes: cystadenoma (226) 246.3 Hemorrhage and infarction of thyroid 246.8 Other Abnormality of thyroid-binding globulin Atrophy of thyroid 246.9 Unspecified DISEASES OF OTHER ENDOCRINE GLANDS (250-259) 250 Diabetes mellitus Excludes: neonatal diabetes mellitus (775.1) nonclinical diabetes (790.2) when complicating pregnancy, childbirth, or the puerperium (648.0) 250.0 Diabetes mellitus without mention of complication Diabetes mellitus without mention of complication or manifestation classifiable to 250.1-250.9 Diabetes (mellitus) NOS ICD-9/Ch03.TXT - pg. 4 250.1 Diabetes with ketoacidosis Diabetic: acidosis without mention of coma ketosis without mention of coma 250.2 Diabetes with coma Diabetic coma (with ketoacidosis) Diabetes with hyperosmolar coma 250.3 Diabetes with renal manifestations Diabetic nephropathy Intercapillary glomerulosclerosis Kimmelstiel-Wilson syndrome 250.4 Diabetes with ophthalmic manifestations Diabetic: cataract retinopathy 250.5 Diabetes with neurological manifestations Diabetic: Diabetic polyneuropathy amyotrophy mononeuropathy 250.6 Diabetes with peripheral circulatory disorders Diabetic: gangrene peripheral angiopathy 250.7 Diabetes with other specified manifestations Excludes: intercurrent infections in diabetic patients 250.9 Diabetes with unspecified complications 251 Other disorders of pancreatic internal secretion 251.0 Hypoglycemic coma Iatrogenic hyperinsulinism Insulin coma Use additional E code, if desired, to identify cause, if drug-induced 251.1 Other hyperinsulinism ICD-9/Ch03.TXT - pg. 5 Hyperinsulinism: Hyperplasia of pancreatic islet beta cells NOS NOS ectopic functional 251.2 Hypoglycemia, unspecified 251.3 Postsurgical hypoinsulinemia [Not to be used for underlying cause of death] Postpancreatectomy hyperglycemia 251.4 Abnormality of secretion of glucagon Hyperplasia of pancreatic islet alpha cells with glucagon excess 251.5 Abnormality of secretion of gastrin Hyperplasia of pancreatic alpha cells with gastrin excess Zollinger-Ellison syndrome 251.8 Other 251.9 Unspecified Islet cell hyperplasia NOS 252 Disorders of parathyroid gland 252.0 Hyperparathyroidism Hyperplasia of parathyroid Osteitis fibrosa cystica generalisata [von Recklinghausen's disease of bone] Excludes: secondary hyperparathyroidism (of renal origin) (588.8) 252.1 Hypoparathyroidism Parathyroiditis (autoimmune) Tetany: parathyroid parathyroprival Excludes: pseudohypoparathyroidism (275.4) pseudopseudohypoparathyroidism (275.4) tetany NOS (781.7) transitory neonatal hypoparathyroidism (775.4) 252.8 Other 252.9 Unspecified ICD-9/Ch03.TXT - pg. 6 253 Disorders of the pituitary gland and its hypothalamic control Includes: the listed conditions whether the disorder is in the pituitary or the hypothalamus Excludes: Cushing's syndrome (255.0) 253.0 Acromegaly and gigantism Arthropathy associated with acromegaly Overproduction of growth hormone 253.1 Other anterior pituitary hyperfunction Excludes: overproduction of: ACTH (255.3) thyroid-stimulating hormone (242.8) 253.2 Panhypopituitarism Cachexia, pituitary Necrosis of pituitary (post-partum) Pituitary insufficiency NOS Sheehan's syndrome Simmonds's disease Excludes: iatrogenic hypopituitarism (253.7) 253.3 Pituitary dwarfism Isolated deficiency of growth hormone Lorain-Levi dwarfism 253.4 Other anterior pituitary disorders Isolated or partial deficiency of an anterior pituitary hormone, other than growth hormone 253.5 Diabetes insipidus Excludes: nephrogenic diabetes insipidus (588.1) 253.6 Other disorders of neurohypophysis Syndrome of inappropriate secretion of antidiuretic hormone 253.7 Iatrogenic pituitary disorders Hypopituitarism: hormone-induced hypophysectomy-induced radiotherapy-induced ICD-9/Ch03.TXT - pg. 7 Use additional E code, if desired, to identify cause 253.8 Other disorders of the pituitary and other syndromes of diencephalohypophyseal origin Abscess of pituitary Cyst of Rathke's pouch Adiposogenital dystrophy Fr”hlich's syndrome 253.9 Unspecified Dyspituitarism 254 Diseases of thymus gland Excludes: aplasia or dysplasia with immunodeficiency (279.2) myasthenia gravis (358.0) 254.0 Persistent hyperplasia of thymus Hypertrophy of thymus 254.1 Abscess of thymus 254.8 Other 254.9 Unspecified 255 Disorders of adrenal glands Includes: the listed conditions whether the basic disorder is in the adrenals or is pituitary induced 255.0 Cushing's syndrome: Cushing's syndrome: NOS iatrogenic idiopathic pituitary-dependent Ectopic ACTH syndrome Overproduction of cortisol Use additional E code, if desired, to identify cause, if drug-induced 255.1 Hyperaldosteronism Conn's syndrome 255.2 Adrenogenital disorders Adrenogenital syndromes, virilizing or feminizing, whether acquired or associated with congenital adrenal hyperplasia consequent on inborn enzyme defects in hormone synthesis ICD-9/Ch03.TXT - pg. 8 Adrenogenital syndrome Female adrenal pseudohermaphroditism Male: macrogenitosomia praecox sexual precocity with adrenal hyperplasia Virilization (female) 255.3 Other corticoadrenal overactivity Overproduction of ACTH 255.4 Corticoadrenal insufficiency Addisonian crisis Addison's disease: NOS tuberculous Adrenal: atrophy (autoimmune) calcification crisis hemorrhage infarction insufficiency NOS 255.5 Other adrenal hypofunction 255.6 Medulloadrenal hyperfunction Catecholamine secretion by pheochromocytoma Note: Not to be used as the primary code for phaeochromocytoma. See note at beginning of Chapter III. 255.8 Other Abnormality of cortisol-binding globulin 255.9 Unspecified 256 Ovarian dysfunction 256.0 Hyperestrogenism 256.1 Other ovarian hyperfunction Hypersecretion of ovarian androgens 256.2 Postablative ovarian failure Ovarian failure: iatrogenic postirradiation postsurgical ICD-9/Ch03.TXT - pg. 9 256.3 Other ovarian failure Premature menopause NOS Primary ovarian failure 256.4 Polycystic ovaries Stein-Leventhal syndrome 256.8 Other 256.9 Unspecified 257 Testicular dysfunction 257.0 Testicular hyperfunction Hypersecretion of testicular hormones 257.1 Postablative testicular hypofunction Testicular hypofunction: iatrogenic postirradiation postsurgical 257.2 Other testicular hypofunction Defective biosynthesis of testicular androgen Testicular hypogonadism Excludes: azoospermia (606.0) 257.8 Other Goldberg-Maxwell syndrome Male pseudohermaphroditism with testicular feminization Testicular feminization 257.9 Unspecified 258 Polyglandular dysfunction and related disorders 258.0 Polyglandular activity in multiple endocrine adenomatosis Wermer's syndrome Note: Not to be used as the primary code for multiple endocrine adenomatosis. See note at beginning of Chapter III. 258.1 Other combinations of endocrine dysfunction Lloyd's syndrome Schmidt's syndrome ICD-9/Ch03.TXT - pg. 10 258.8 Other 258.9 Polyglandular dysfunction, unspecified 259 Other endocrine disorders 259.0 Delay in sexual development and puberty, not elsewhere classified Delayed puberty 259.1 Precocious sexual development and puberty, not elsewhere classified Sexual precocity: NOS constitutional cryptogenic idiopathic 259.2 Carcinoid syndrome Hormone secretion by carcinoid tumors Note: Not to be used as the primary code for carcinoid tumor. See note at beginning of Chapter III. 259.3 Ectopic hormone secretion, not elsewhere classified 259.4 Dwarfism, not elsewhere classified Dwarfism: NOS constitutional Excludes: dwarfism: achondroplastic (756.4) intrauterine (759.7) nutritional (263.2) pituitary (253.3) renal (588.0) progeria (259.8) 259.8 Other Pineal gland dysfunction Werner's syndrome Progeria 259.9 Unspecified Disturbance: endocrine NOS hormone NOS Infantilism NOS ICD-9/Ch03.TXT - pg. 11 NUTRITIONAL DEFICIENCIES (260-269) Excludes: deficiency anemias (280.-, 281.-) 260 Kwashiorkor Nutritional edema with dyspigmentation of skin and hair 261 Nutritional marasmus Nutritional atrophy Severe calorie deficiency Severe malnutrition NOS 262 Other severe protein-calorie malnutrition Malnutrition of third degree according to Gomez classification [weight for age less than 60% of standard] Nutritional edema without mention of dyspigmentation of skin and hair 263 Other and unspecified protein-calorie malnutrition 263.0 Malnutrition of moderate degree Malnutrition of second degree according to Gomez classification [weight for age 60% to less than 75% of standard] 263.1 Malnutrition of mild degree Malnutrition of first degree according to Gomez classification [weight for age 75% to less than 90% of standard] 263.2 Arrested development following protein-calorie malnutrition Nutritional dwarfism Physical retardation due to malnutrition 263.8 Other protein-calorie malnutrition 263.9 Unspecified Dystrophy due to malnutrition Malnutrition (calorie) NOS Excludes: nutritional deficiency NOS (269.9) 264 Vitamin A deficiency 264.0 With conjunctival xerosis 264.1 With conjunctival xerosis and Bitot's spot Bitot's spot in the young child 264.2 With corneal xerosis ICD-9/Ch03.TXT - pg. 12 264.3 With corneal ulceration and xerosis 264.4 With keratomalacia 264.5 With night blindness 264.6 With xerophthalmic scars of cornea 264.7 Other ocular manifestations of vitamin A deficiency Xerophthalmia due to vitamin A deficiency 264.8 Other manifestations of vitamin A deficiency Follicular keratosis due to vitamin A deficiency Xeroderma due to vitamin A deficiency 264.9 Unspecified Hypovitaminosis A NOS 265 Thiamine and niacin deficiency states 265.0 Beriberi 265.1 Other and unspecified manifestations of thiamine deficiency Other vitamin B1 deficiency states 265.2 Pellagra Deficiency: niacin (-tryptophan) nicotinamide nicotinic acid vitamin PP Pellagra (alcoholic) 266 Deficiency of B-complex components 266.0 Ariboflavinosis Riboflavin deficiency 266.1 Vitamin B6 deficiency Deficiency: pyridoxal pyridoxamine pyridoxine Vitamin B6 deficiency syndrome Excludes: vitamin B6-responsive sideroblastic anemia (285.0) ICD-9/Ch03.TXT - pg. 13 266.2 Other B-complex deficiencies Deficiency: cyanocobalamin folic acid vitamin B12 Excludes: deficiency anemias (281.-) 266.9 Unspecified vitamin B deficiency 267 Ascorbic acid deficiency Deficiency of vitamin C Scurvy Excludes: scorbutic anemia (281.8) 268 Vitamin D deficiency Excludes: vitamin D-resistant rickets and osteomalacia (275.3) 268.0 Rickets, active Excludes: coeliac rickets (579.0) renal rickets (588.0) 268.1 Rickets, late effect Any condition specified as rachitic or due to rickets and present one year or more after onset, or stated to be a late effect or sequela of rickets 268.2 Osteomalacia 268.9 Unspecified Avitaminosis D 269 Other nutritional deficiencies 269.0 Deficiency of vitamin K Excludes: deficiency of coagulation factor due to vitamin K deficiency (286.7) vitamin K deficiency of newborn (776.0) 269.1 Deficiency of other vitamins Deficiency: vitamin E vitamin P 269.2 Unspecified vitamin deficiency ICD-9/Ch03.TXT - pg. 14 269.3 Mineral deficiency, not elsewhere classified Excludes: deficiency: potassium (276.8) sodium (276.1) 269.8 Other nutritional deficiency Excludes: failure to thrive (783.4) feeding problems (783.3) newborn (779.3) 269.9 Unspecified OTHER METABOLIC DISORDERS AND IMMUNITY DISORDERS (270-279) Use additional code, if desired, to identify any associated mental retardation 270 Disorders of amino-acid transport and metabolism Excludes: abnormal findings without manifest disease (791-796) disorders of purine and pyrimidine metabolism (277.2) gout (274.-) 270.0 Disturbances of amino-acid transport Cystinosis Glycinuria (renal) Cystinuria Hartnup disease Fanconi (-de Toni) (-Debr82) syndrome 270.1 Phenylketonuria Hyperphenylalaninemia 270.2 Other disturbances of aromatic amino-acid metabolism Albinism Hypertyrosinemia Alkaptonuria Oasthouse urine disease Disturbances of metabolism of Ochronotic arthritis tyrosine and tryptophan Tyrosinosis Hydroxykynureninuria Tyrosinuria Excludes: vitamin B6-deficiency syndrome (266.1) 270.3 Disturbances of branched-chain amino-acid metabolism Disturbances of metabolism of leucine, isoleucine, and valine Hypervalinemia Leucinosis Maple syrup urine disease ICD-9/Ch03.TXT - pg. 15 270.4 Disturbances of sulphur-bearing amino-acid metabolism Cystathioninuria Homocystinuria Disturbances of metabolism of Hypermethioninemia methionine, homocystine, and cystathionine 270.5 Disturbances of histidine metabolism Histidinemia Hyperhistidinemia Imidazole amino aciduria 270.6 Disorders of urea cycle metabolism Argininosuccinic aciduria Hyperammonemia Citrullinemia Hyperornithinemia Disorders of metabolism of ornithine, citrulline, argininosuccinic acid, arginine, and ammonia 270.7 Other disturbances of straight-chain amino-acid metabolism Hyperglycinemia Other disturbances of metabolism of Hyperlysinemia glycine, threonine, serine, Pipecolic acidemia glutamine, and lysine Saccharopinuria 270.8 Other Ethanolaminuria Iminoacidopathy Hydroxyprolinemia Sarcosinemia Hyperprolinemia 270.9 Unspecified 271 Disorders of carbohydrate transport and metabolism Excludes: abnormality of secretion of glucagon (251.4) diabetes mellitus (250.-) hypoglycemia NOS (251.2) mucopolysaccharidosis (277.5) 271.0 Glycogenosis Glycogen-storage disease 271.1 Galactosemia 271.2 Hereditary fructose intolerance ICD-9/Ch03.TXT - pg. 16 271.3 Intestinal disaccharidase deficiencies and disaccharide malabsorption Intolerance or malabsorption (congenital): glucose-galactose lactose sucrose-isomaltose 271.4 Renal glycosuria Renal diabetes 271.8 Other Hyperoxaluria (primary) Oxalosis 271.9 Unspecified 272 Disorders of lipoid metabolism Excludes: localized cerebral lipidoses (330.1) 272.0 Pure hypercholesterolemia Familial hypercholesterolemia Fredrickson's Type IIa hyperlipoproteinemia Hyper-beta-lipoproteinemia Hyperlipidemia, Group A Low-density-lipoid-type [LDL] hyperlipoproteinemia 272.1 Pure hyperglyceridemia Endogenous hyperglyceridemia Fredrickson's Type IV hyperlipoproteinemia Hyperlipidemia, Group B Hyper-pre-beta-lipoproteinemia Hypertriglyceridemia, essential Very-low-density-lipoid-type [VLDL] hyperlipoproteinemia 272.2 Mixed hyperlipidemia Broad- or floating-beta-lipoproteinemia Fredrickson's Type IIb or III hyperlipoproteinemia Hypercholesterolemia with endogenous hyperglyceridemia Hyper-beta-lipoproteinemia with pre-beta-lipoproteinemia Hyperlipidaemia, Group C Tubo-eruptive xanthoma Xanthoma tuberosum 272.3 Hyperchylomicronemia Fredrickson's type I or V Hyperlipidemia, Group D hyperlipoproteinemia Mixed hyperglyceridemia ICD-9/Ch03.TXT - pg. 17 272.4 Other and unspecified hyperlipidemia Alpha-lipoproteinemia Hyperlipidemia NOS Combined hyperlipidemia 272.5 Lipoprotein deficiencies A-beta-lipoproteinemia Hypo-beta-lipoproteinemia (familial High-density lipoid deficiency Lecithin cholesterol acyltransferase Hypo-alpha-lipoproteinemia deficiency Tangier disease 272.6 Lipodystrophy Barraquer-Simons disease Progressive lipodystrophy Use additional E code, if desired, to identify cause, if iatrogenic Excludes: intestinal lipodystrophy (040.2) 272.7 Lipidoses Chemically induced lipidosis Disease: Disease: pseudo-Hurler's or mucolipidosis III Anderson's Sandhoff's Fabry's triglyceride-storage, Type I or II Gaucher's Wolman's or triglyceride-storage, I-cell or mucolipidosis I Type III lipoid-storage NOS Mucolipidosis II Niemann-Pick Primary familial xanthomatosis 272.8 Other disorders of lipoid metabolism Hoffa's disease or liposynovitis prepatellaris Launois-Bensaude's lipomatosis 272.9 Unspecified disorders of lipoid metabolism 273 Disorders of plasma protein metabolism Excludes: agammaglobulinemia and hypogammaglobulinemia (279.-) coagulation defects (286.-) hereditary hemolytic anemias (282.-) 273.0 Polyclonal hypergammaglobulinemia Waldenstr”m's hypergammaglobulinemic purpura: 273.1 Monoclonal paraproteinemia Monoclonal gammopathy: NOS associated with lymphoplasmacytic dyscrasias ICD-9/Ch03.TXT - pg. 18 benign Paraproteinemia: benign (familial) secondary to malignant or inflammatory disease 273.2 Other paraproteinemias Cryoglobulinemic: purpura vasculitis Mixed cryoglobulinemia 273.3 Macroglobulinemia Macroglobulinemia (idiopathic) (primary) Waldenstr”m's macroglobulinemia 273.8 Other Abnormality of transport protein Bisalbuminemia 273.9 Unspecified 274 Gout Excludes: lead gout (984.-) 274.0 Gouty arthropathy 274.1 Gouty nephropathy Gouty nephropathy NOS Uric acid nephrolithiasis 274.8 Gout with other manifestations Gouty tophi of: ear heart 274.9 Gout, unspecified 275 Disorders of mineral metabolism Excludes: abnormal findings without clinical significance (790.-) 275.0 Disorders of iron metabolism Hemochromatosis Excludes: anemia: iron deficiency (280.-) sideroblastic (285.0) ICD-9/Ch03.TXT - pg. 19 275.1 Disorders of copper metabolism Hepatolenticular degeneration Wilson's disease 275.2 Disorders of magnesium metabolism Hypermagnesemia Hypomagnesemia 275.3 Disorders of phosphorus metabolism Familial hypophosphatemia Hypophosphatasia Vitamin D-resistant: osteomalacia rickets 275.4 Disorders of calcium metabolism Calcinosis Nephrocalcinosis Hypercalcemia Pseudohypoparathyroidism Hypercalcinuria Pseudopseudohypoparathyroidism Excludes: parathyroid disorders (252.-) vitamin D deficiency (268.-) 275.8 Other 275.9 Unspecified 276 Disorders of fluid, electrolyte, and acid-base balance Excludes: diabetes insipidus (253.5) familial periodic paralysis (359.3) 276.0 Hyperosmolality and/or hypernatremia Na excess Na overload 276.1 Hyposmolality and/or hyponatremia Na deficiency 276.2 Acidosis Acidosis: NOS lactic metabolic respiratory Excludes: diabetic acidosis (250.1) ICD-9/Ch03.TXT - pg. 20 276.3 Alkalosis Alkalosis: NOS metabolic respiratory 276.4 Mixed acid-base balance disorder 276.5 Volume depletion Dehydration Depletion of volume of plasma or extracellular fluid Hypovolemia Excludes: hypovolemic shock: postoperative (998.0) traumatic (958.4) 276.6 Fluid overload Fluid retention 276.7 Hyperpotassemia K overload K excess 276.8 Hypopotassemia K deficiency 276.9 Electrolyte and fluid disorders not elsewhere classified Electrolyte imbalance Hypochloremia Hyperchloremia Excludes: electrolyte imbalance: associated with hyperemesis gravidarum (643.1) following abortion and ectopic or molar pregnancy (634-638 with fourth digit .4, 639.4) 277 Other and unspecified disorders of metabolism 277.0 Cystic fibrosis Fibrocystic disease of the pancreas Mucoviscidosis 277.1 Disorders of porphyrin metabolism Porphyria Porphyrinuria 277.2 Other disorders of purine and pyrimidine metabolism ICD-9/Ch03.TXT - pg. 21 Hypoxanthine-guanine-phosphoribosyltransferase deficiency [HG-PRT deficiency] Lesch-Nyhan syndrome Excludes: gout (274.-) oroticaciduric anemia (281.4) 277.3 Amyloidosis Amyloidosis: NOS inherited systemic nephropathic neuropathic (Portuguese) (Swiss) secondary Familial Mediterranean fever Hereditary cardiac amyloid 277.4 Disorders of bilirubin excretion Syndrome: Crigler-Najjar Dubin-Johnson Gilbert's Rotor's Excludes: hyperbilirubinemias specific to the perinatal period (774.-) 277.5 Mucopolysaccharidosis Gargoylism Hurler's syndrome Hunter's syndrome 277.6 Other deficiencies of circulating enzymes Alpha 1-antitrypsin deficiency Hereditary angioedema 277.8 Other 277.9 Unspecified 278 Obesity and other hyperalimentation Excludes: hyperalimentation NOS (783.6) poisoning by vitamin preparations (963.5) 278.0 Obesity Excludes: adiposogenital dystrophy (253.8) obesity of endocrine origin NOS (259.9) 278.1 Localized adiposity Fat pad ICD-9/Ch03.TXT - pg. 22 278.2 Hypervitaminosis A 278.3 Hypercarotinemia 278.4 Hypervitaminosis D 278.8 Other 279 Disorders involving the immune mechanism 279.0 Deficiency of humoral immunity Agammaglobulinaemia: Hypogammaglobulinaemia NOS brulous 279.1 Deficiency of cell-mediated immunity Di George syndrome Wiskott-Aldrich syndrome Nezelof syndrome Excludes: ataxia-telangiectasia (334.8) 279.2 Combined immunity deficiency Agammaglobulinemia, Swiss type Combined immunity deficiency syndrome Reticular dysgenesis Thymic: alymphoplasia aplasia or dysplasia with immunodeficiency 279.3 Unspecified immunity deficiency 279.4 Autoimmune disease, not elsewhere classified Autoimmune disease NOS 279.8 Other 279.9 Unspecified ICD-9/Ch03.TXT - pg. 23

82. References
De Prins FA Van Assche FA (1982) intrauterine growth retardation and Laron Z et al (1972) The syndrome of familial dwarfism and high plasma
http://www.unu.edu/unupress/food2/UID03E/uid03e0b.htm
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References
Adamson ED (1993): Activities of growth factors in pre-implantation embryos. J. Cell Biochem Acta Endocrinol (Copenh) Baker H et al (1975): Vitamin profile of 174 mothers and new born at parturition. Am. J. Clin. Nutr Basset NS et al (1990): The effect of maternal starvation on plasma insulin-like growth factor I concentration in the late gestation ovine fetus. Pediatr. Res An introduction to Fetal Physiology . Orlando Academic Press. Am. J. Clin. Nutr Am. J. Obstet Gynecol Lancet J. Obstet. Gynaecol Br. Commonw Borle AB (1981): Control, modulation and regulation of cell calcium. Rev. Physiol. Biochem. Pharmacol J. Am. Diet. Assoc Bulmer M (1970): The Biology of Twinning in Man . Oxford University Press: Oxford. Butherworth RF et al (1991): Thiamine deficiency and Wernicke's Encephalopaty in AIDS. Metab. Brain Dis. Perinatal Mortality . Livingstone: Edinburg. Am. J. Clin. Nutr Malnutrition and the infant brain . Wiley-Liss Inc: New York, pp 207-224. . 1st ed. Stockton Press: New York. J. Nutr. Biochem

83. Information Center For Rare Diseases And Orphan Drugs - Sections
mental retardationunusual facies-intrauterine growth retard. meretoja syndrome microcephalic osteodysplastic primordial dwarfism taybi linder type
http://www.raredis.org/modules/sections/index.php?op=viewarticle&artid=27

84. Mesothelioma - RARE DISEASES
Mesomelic dwarfism cleft palate camptodactyly; Mesomelic dwarfism Langer type Microcephaly, holoprosencephaly, and intrauterine growth retardation
http://mesothelioma.me.uk/rare diseases.html

85. NEJM -- Laron Dwarfism And Mutations Of The Growth Hormone-receptor Gene
Laron dwarfism is associated with resistance to growth hormone (GH). intrauterine Growth Retardation and Postnatal Growth Failure Associated with
http://content.nejm.org/cgi/content/abstract/321/15/989
HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Please sign in for full text and personal services Volume 321:989-995 October 12, 1989 Number 15 Next Laron dwarfism and mutations of the growth hormone-receptor gene
S Amselem, P Duquesnoy, O Attree, G Novelli, S Bousnina, MC Postel-Vinay, and M Goossens
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Source Information Service de Biochimie and INSERM, C.H.U. Henri Mondor, Creteil, France.
This article has been cited by other articles:
  • Stoffel, W., Jenke, B., Block, B., Zumbansen, M., Koebke, J. (2005). Neutral sphingomyelinase 2 (smpd3) in the control of postnatal growth and development. Proc. Natl. Acad. Sci. U. S. A. [Abstract] [Full Text]
  • Laron, Z. (2004). Laron Syndrome (Primary Growth Hormone Resistance or Insensitivity): The Personal Experience 1958-2003. J Clin Endocrinol Metab [Abstract] [Full Text]
  • Besson, A., Salemi, S., Gallati, S., Jenal, A., Horn, R., Mullis, P. S., Mullis, P. E. (2003). Reduced Longevity in Untreated Patients with Isolated Growth Hormone Deficiency. J Clin Endocrinol Metab [Abstract] [Full Text]
  • Pantel, J., Grulich-Henn, J., Bettendorf, M., Strasburger, C. J., Heinrich, U., Amselem, S. (2003). Heterozygous Nonsense Mutation in Exon 3 of the Growth Hormone Receptor (GHR) in Severe GH Insensitivity (Laron Syndrome) and the Issue of the Origin and Function of the GHRd3 Isoform.

86. Login
Seckel syndrome (MIM 210600) comprises intrauterine growth retardation, severeproportionately short The osteodysplastic type II of primordial dwarfism.
http://dx.doi.org/10.1086/303023
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87. Dwarfism And Impaired Gut Development In Insulin-Like Growth Factor II MRNA-Bind
an inability to make the transition from intrauterine to extrauterine life . The molecular pathways leading to dwarfism and intestinal hypoplasia are
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=400488

88. Dorlands Medical Dictionary
Robinow s syndrome, dwarfism associated with increased interorbital characterized by intrauterine growth retardation and postnatal dwarfism with a small
http://www.mercksource.com/pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszS

89. Clinical Case : Hydrops And Congenital Anomalies
Ultrasound showed light hydramnios and intrauterine growth retardation (IUGR) IUGR, ie microcephalic dwarfism, dysmorphic facies and hypoplasia of the
http://www.hydropsfetalis.org/Clinical_case.html
Home About hydrops fetalis About us Other links
CLINICAL CASE
FRANÇAIS
Miscarriage (9 weeks) Clinical synopsis
  • Thick placenta with many blood clots
  • Umbilical cord too long by 7 cm (no placental pathology was investigated)
  • Microcephaly or rather the weight of brain and the cranium were normal for 14-15 weeks. Extensive autolysis of the brain should be noted.
  • Triangular long face : bird-headed appearance
  • Receding forehead
  • Hypoplastic eyes with hypertelorism and microphthalmia (according to the pathologist)
  • Narrow-shaped and downslanting or antimongoloid slant of palpebral fissures (as shown on pictures)
  • Microtia with malformed and low-set ears (not rimmed), hypoplasia of lobes
  • Protrusion of the nose (very prominent and pointed)
  • Important micrognathia and retrognathia
  • Short neck with oedema
  • Tapering fingers and toes with hypoplastic nails (according to the pathologist)
  • Abnormal space between the two big toes and the second toes (as shown on pictures)
  • Absence of epiphyseal ossification at the phalanges : clinodactyly, camptodactyly ?
  • Legs bone asymmetry : absence or hypoplasia of fibula on the right-hand side
  • Micropenis (hypoplastic testes in pelvis were not examined under a microscope) but the "micropenis" may be normal for a fetus whose weight was 67 grammes...

90. Growth Assessment And Growth Failure: Etiologies Of Abnormal Growth
implicated in the short stature of LeriWeill dyschondrosteosis dwarfism. Reproduced with permission from Usher R, McLean F. intrauterine growth of
http://www.meadjohnson.com/professional/newsletters/ppv4n2a.html
Growth Assessment and Growth Failure:
Etiologies of Abnormal Growth Daniel B. Kessler, M.D., F.A.A.P.
Director, Arizona Child Study Center
Clinical Associate Professor of Pediatrics
Susan S. Baker, M.D., Ph.D.
Division of Pediatric Gastroenterology
Children's Hospital of Buffalo
Lawrence A. Silverman, M.D.
Division of Pediatric Endocrinology
Goryeb Children's Hospital
Atlantic Health System Published 2004 Preface Introduction top Normal growth begins in utero . Fetal growth is affected by a variety of issues, including intrinsic fetal factors, maternal factors, and uteroplacental pathology. Chromosomal Derangements top Common among the factors that affect fetal growth are chromosomal abnormalities. Of these, the most common is Down syndrome, affecting 1 in 600 live born infants. Babies with Down syndrome, on average, weigh 500 gm less than unaffected babies and are 2-3 cm shorter. The average height of an adult with Down syndrome is significantly less than population average. Specific growth curves exist to track affected children. By employing disease-specific growth curves, changes in height would be more obvious than on the population curve. For instance, Hashimoto thyroiditis with subsequent hypothyroidism may be an endocrine cause of growth failure in children with Down syndrome. The concomitant decline in growth rate would be easily noted on a disease-specific curve but may be overlooked in a child who is always well below the curve. While growth hormone may increase final height in children with Down syndrome, this is an off-label use, and there is no widespread consensus on this matter.

91. Estudio Colaborativo Latino Americano De Malformaciones Congénitas
For families with children who have Russell Silver intrauterine growth retardation . This is a medical center that specializes in dwarfism.
http://eclamc.ioc.fiocruz.br/links_es_02_usa.htm
USA D : Sindrome de Down): Search by specific disease (e.g. D : Down syndrome)

92. Birth Disorder Information Directory - P
Prader (Labhart) Willi (Fancone) Syndrome (Cryptorchidism with dwarfism and Subnormal Pseudotorch Syndrome (intrauterine InfectionLike Syndrome with
http://www.bdid.com/defectp.htm

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P-4 Deletion Syndrome p47-PHOX, Deficiency of p67-PHOX, Deficiency of Pachydermopreiostosis (Hypertrophic Osteoarthropathy, Primary or Idiopathic) Pachyonychia Congenita Pagod Syndrome Pagon Bird Detter Syndrome (Anemia Sideroblastic Spinocerebellar Ataxia) Pagon Syndrome Pallister Syndrome Pallister Hall Syndrome (Hypothalamic Hamartoblastoma, Hypopituitarism, Imperperforate Anus, and Postaxial Polydactyly) Pallister Killian Syndrome

93. Prominent Congenital Defects In Nebraska Beef Cattle, G85-759-A
dwarfism. dwarfism occurs in all breeds of cattle. and intrauterine fetalinfections with BVD and bluetongue viruses have also been incriminated as
http://ianrpubs.unl.edu/beef/g759.htm
G85-759-A
Prominent Congenital Defects in Nebraska Beef Cattle
This NebGuide describes congenital defects that affect the economic value of beef cattle, and provides recommendations on how to minimize them in breeding programs. J. L. Johnson, DVM, Veterinary Pathologist (University of Nebraska)
H. W. Leipold, DVM, Veterinary Pathologist (Kansas State University)
Donald B. Hudson, DVM, Extension Veterinarian
Previous Category Catalog Order Info
Congenital defects are abnormalities of structure or function present at birth. They may be caused by genetic or environmental factors, or a combination of both. The causes of many defects remain unknown. Developmental defects may be lethal, semi-lethal, or compatible with life, causing very little effect or only aesthetic effect. With the increasing use of artificial insemination (AI) in cattle, defects are no longer rare occurrences and are important collectively to the cattle industry. Congenital defects cause economic losses by increasing perinatal calf mortality, decreasing maternal productivity, and decreasing the value of defective calves and their relatives. Susceptibility to injurious agents varies with fetal development and decreases with age. Before Day 14 of gestation in cattle (period of pre-attachment), the zygote or embryo is resistant to agents that produce the incidence of congenital malformations (teratogens), but is susceptible to genetic mutations and abnormality of chromosome numbers or structures (chromosomal aberrations). During the embryonic period (Days 14-42), the embryo is highly susceptible to teratogens, but this decreases with embryonic age as critical periods for the various organs are passed. The fetus (Day 42+) becomes increasingly resistant to teratogenic agents with age, except for the late differentiating structures, such as cerebellum, palate, and urogenital system.

94. Ultrasonic Diagnosis Of Thanatophoric Dwarfism In Utero -- Cremin And Shaff 124
There are several types of shortlimbed neonatal dwarfism. Intrauterineradiological diagnosis is possible and the clinician may be alerted to the
http://radiology.rsnajnls.org/cgi/content/abstract/124/2/479
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Ultrasonic diagnosis of thanatophoric dwarfism in utero
BJ Cremin and MI Shaff
There are several types of short-limbed neonatal dwarfism. Before the delineation of thanatophoric dwarfism many dwarfs who died early in infancy were labeled "achondroplastic." Intrauterine radiological diagnosis is possible and the clinician may be alerted to the possibility of the condition by noting short squat limbs with a relatively large head on ultrasound examination, especially in cases of hydramnios.
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95. Principles Of Pediatric Dermatology - Chapter 32 : GENODERMATOSES
The syndrome must be differentiated from others in which there is intrauterinedwarfism. CARTILAGEHAIR HYPOPLASIA SYNDROME
http://www.drmhijazy.com/english/chapters/chapter32_2.htm
CHAPTER 32 GENODERMATOSES Contents Search
Recessive epidermolysis bullosa
The recessive dystrophic epidermolysis bullosa has the same cutaneous features of the dominant type. Skin manifestations Large, flaccid often-hemorrhagic bullae appear after birth or early in infancy. Mucous membrane manifestations Severe scarring of the mucous membranes of the mouth, pharynx, esophagus and the condition may be fatal. Nail dystrophy is a common complication of the recessive type of epidermolysis bullosa. Secondary bacterial infection is common and causes more complications.
Fig. 256. Epidermolysis, Recessive
Dystrophic Type
Fig. 257. Epidermolysis Bullosa
Differential Diagnosis
  • Bullous diseases
    In infancy, differentiation of epidermolysis bullosa from other bullous eruptions is rarely a problem. Benign chronic bullous disease of childhood may mimic inverse types of EB since the blistering occurs around the genitalia, perineum and buttocks. However, histology of these conditions is diagnostic and the presence of linear IgA on direct immunofluorescence may help to confirm the diagnosis. Dominant bullous ichthyosiform erythroderma.
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