Glossary

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aberrant dentin – abnormal formation of the material that composes the principal mass of a tooth

achondroplasia – an inherited problem with the growth of the cartilage in the long bones and skull, characterized by short stature

Angelman syndrome – a genetic disorder characterized by mental retardation, hyperactivity, and unprovoked laughter

angular cheilitis – inflammation and fissuring from the corners of the mouth; also known as angular stomatitis

anorexia nervosa – a disorder characterized a disturbance in body image, an extreme fear of becoming obese, and an aversion to food, often resulting in life-threatening weight loss

bulimia nervosa – a disorder involving repeated and secretive episodic bouts of binge eating followed by purging, e.g., vomiting, use of laxatives or diuretics, fasting, or vigorous exercise in order to prevent weight gain

bruxism – rubbing, gritting, or grinding together of the teeth, usually during sleep

cachexia – weight loss and wasting during the course of a chronic disease, such as cancer or HIV/AIDS

calculus – calcified deposits formed around teeth; also called tartar

candidiasis – infection with the yeast Candida, especially C. albicans

deLange syndrome – a congenital syndrome characterized by short stature, small heady, delayed development and mental retardation, a number of distinct facial features, and upper limb anomalies; also called Cornelia deLange syndrome and Brachmann-deLange syndrome

dentin – the material that composes the principal mass of a tooth

dentifrice - toothpaste

diabetes – a chronic metabolic disorder in which utilization of carbohydrate is impaired; caused by deficiency of insulin and characterized by hyperglycemia (high blood sugar), glycosuria (glucose in the urine), water and electrolyte loss, ketoacidosis, and coma; long-term complications include neuropathy, retinopathy, nephropathy, generalized degenerative changes in large and small blood vessels, and increased susceptibility to infection

Down syndrome – trisomy 21; a genetic disorder in which an individual has an extra 21st chromosome, typically characterized by low muscle tone, cardiac problems, GI malformations and a distinct facial appearance

early intervention services – established by Part H of P.L. 97-457 of 1986 (now Part C of the IDEA of 1997); community-based therapeutic and educational services for infants and children under 3 years of age with developmental delays

enamel hypoplasia - deficient or defective enamel matrix formation; may be hereditary or acquired

fetal alcohol syndrome - a syndrome resulting from the teratogenic effects of alcohol during fetal development; possible symptoms include developmental delay, short stature, microcephaly and hyperactivity

fragile X syndrome - a syndrome resulting from a fragile or broken site on the X chromosome, often characterized by mental retardation, hypotonia and hyperactivity

galactosemia – an inherited (autosomal recessive) metabolic disorder, marked by the deficiency of the enzyme involved in the metabolism of the carbohydrate galactose; accumulation of galactose 1-phosphate can lead to hepatosplenomegaly with cirrhosis, cataracts, and mental retardation; treatment includes elimination of foods containing galactose (primarily milk and milk products)

gastroesophageal reflux - regurgitation of the contents of the stomach into the esophagus, where they can be aspirated; often results from a failure of the esophageal sphincter to close; commonly leads to feeding problems in infants and children with neuromuscular disorders; also gastroesophageal reflux disease (GERD)

gingiva – also called the gum, the tissue that envelops the alveolar processes of the upper and lower jaws and surrounds the necks of the teeth

gingival hyperplasia – overgrowth of gum tissue

glossitis – inflammation of the tongue

glycogen storage diseases – a group of inherited metabolic disorders (autosomal recessive) characterized by the accumulation of glycogen; there may be enlargement of the liver, heart, or muscle; treatment depends on the enzyme deficiency involved, but often includes avoidance of fasting and supplementation with cornstarch

Head Start– federally-funded preschool program for children ages 3 to 5 years from low-income families; includes children with special needs; educational program for children and parents; includes some health screenings

HIV/AIDS - human immunodeficiency virus (HIV) is the retrovirus that causes acquired immunodeficiency syndrome (AIDS); symptoms of HIV infection can include opportunistic infections, growth problems, diarrhea, developmental regression and immune system dysfunction

hypophosphatemia – abnormally low concentration of phosphates in the blood; the cause can be an inherited (autosomal recessive) disorder of metabolism; premature tooth loss and periodontal disease have been reported with hypophosphatemia

Klinefelter syndrome – a chromosomal anomaly with chromosome count 47, XXY sex chromosome constitution; also called XXY syndrome

Lowe syndrome – an inherited syndrome (X-linked recessive) with hydrophthalmia, cataracts, mental retardation, aminoaciduria, reduced ammonia production by the kidney, and vitamin D-resistant rickets; also called oculocerebrorenal syndrome, Lowe-Terrey-MacLachlan syndrome

malocclusion – misaligned bite, can be the result of missing teeth (e.g., because of caries or congenital defect); children with hyper- or hypotonia are also at increased risk for malocclusion

mandible – the bone that forms the lower jaw

Marfan syndrome – an inherited (autosomal dominant) connective tissue multisystemic disorder characterized by skeletal changes (long limbs, joint laxity, pectus), cardiovascular defects (aortic aneurysm, mitral valve prolapse), and ectopia lentis

maxilla – the upper jaw bone; supporting the superior teeth and taking part in the formation of the orbit, hard palate, and nasal cavity and containing the maxillary sinus

morphogenesis – differentiation of cells and tissues in the early embryo that establishes the form and structure of the various organs and parts of the body

muscular dystrophy – a general term for a number of hereditary, progressive degenerative disorders affecting skeletal muscles, and often other organ systems; types of muscular dystrophy include Duchenne and Becker muscular dystrophy, spinal muscular atrophy and myotonic dystrophy

neutropenia – abnormally small numbers of neutrophils (white blood cells) in the blood; types of neutropenia include cyclic neutropenia (an autosomal recessive disorder) and agranulocytosis; neutropenia has been associated with periodontal disease

organic acid disorders – a group of inherited disorders characterized by the excretion of non-amino organic acids, usually because of deficient enzyme activity of a specific step in amino acid catabolism; disorders include maple syrup urine disease, propionic acidemia, methylmalonic acidemia ,and isovaleric academia; the usual clinical presentation includes encephalopathy, vomiting, poor feeding, neurologic symptoms, and lethargy progressing to coma; also called organic acidemias or organic acidurias

Papillon-Lefevre syndrome – a rare inherited (autosomal recessive) condition characterized by hyperkeratosis (thickening of the epidermis) of the palms and soles, with periodontal disease and premature loss of primary and permanent teeth

periodontal ligament – an “elastic sling” that surrounds the root of the tooth in the bone, can be destroyed by gingivitis

phenylketonuria – an inherited (autosomal recessive) metabolic disorder, marked by the deficiency of the enzyme that converts phenylalanine (an amino acid) to tyrosine; accumulation of phenylalanine in the blood can lead to mental retardation and other neurologic problems; treatment includes a low-phenylalanine diet and a phenylalanine-free medical food

Rett syndrome - an X-linked disorder marked by progressive neurological deterioration, seizures and cognitive impairment

Smith-Lemli-Opitz syndrome - an autosomal recessive condition characterized by dysmorphic features, short stature, abnormal facies, psychomotor retardation, and genital anomalies in males

stomatitis – inflammation of the mucous membrane of the mouth.

trisomy 18 - a genetic disorder in which an individual has an extra 18th chromosome, characterized by mental retardation, abnormal skull shape, malformed ears, and cardiac defects; the condition is usually fatal within several weeks or months of birth

urea cycle disorders – a group of inherited disorders of nitrogen metabolism through the urea cycle (also called the Krebs cycle), resulting in the accumulation of ammonia; the usual clinical presentation in early infancy is cerebral edema and lethargy, anorexia, hyper- or hypoventilation, seizures, and coma; treatment involves medications to allow alternative pathway excretion of excess nitrogen and reducing the amount of nitrogen in the diet (low protein)

WIC - USDA Supplemental Nutrition Program for Women, Infants, and Children; a federally-funded community program that provides foods, infant formula, and nutrition education to pregnant women, infants, and children under 5 years of age, and breastfeeding mothers

Williams syndrome - a genetic disorder of chromosome 7 characterized by distinctive facial features, growth and developmental delays, varying degrees of learning disabilities, cardiac defects and sometimes hypercalcemia in infancy

xerostomia – dryness of the mouth