A study of the cost and effect of newborn screening for Congenital Adrenal Hyperplasia in Texas

Congenital Adrenal Hyperplasia (CAH), due to 21-Hydroxylase deficiency, has an estimated incidence of 1:15,000 births and can result in death, salt-wasting crisis or impaired growth. It has been proposed that early diagnosis and treatment of infants detected from newborn screening for CAH will decrease the incidence of mortality and morbidity in the affected population. The Texas Department of Health (TDH) began mandatory screening for CAH in June, 1989 and Texas is one of fourteen states to provide neonatal screening for the disorder.^ The purpose of this study was to describe the cost and effect of screening for CAH in Texas during 1994 and to compare cases first detected by screen and first detected clinically between January 1, 1990 and December 31, 1994. This study used a longitudinal descriptive research design. The data was secondary and previously collected by the Texas Department of Health. Along with the descriptive study, an economic analysis was done. The cost of the program was defined, measured and valued for four phases of screening: specimen collection, specimen testing, follow-up and diagnostic evaluation.^ There were 103 infants with Classical CAH diagnosed during the study and 71 of the cases had the more serious Salt-Wasting form of the disease. Of the infants diagnosed with Classical CAH, 60% of the cases were first detected by screen and 40% were first detected because of clinical findings before the screening results were returned. The base case cost of adding newborn screening to an existing program (excluding the cost of specimen collection) was $357,989 for 100,000 infants. The cost per case of Classical CAH diagnosed, based on the number of infants first detected by screen in 1994, was \$126,892. There were 42 infants diagnosed with the more benign Nonclassical form of the disease. When these cases were included in the total, the cost per infant to diagnose Congenital Adrenal/Hyperplasia was $87,848. ^

THE ROLE OF ADRENAL AND THYROID HORMONES IN GASTRIC DEVELOPMENT (THYROXINE, PARIETAL CELL, CORTICOSTERONE)

The role of adrenal and thyroid hormones on the development of chief and parietal cells was studied in the rat. Administration of corticosterone or thyroxine in the first and second postnatal weeks resulted in the precocious appearance of pepsinogen in the oxyntic gland mucosa and an increase in basal acid output. When pups were adrenalectomized or made hypothyroid, both pepsinogen and basal acid secretion were lowed. Corticosterone injection increased pepsinogen content and acid secretion to levels higher than those of control in hypothyroid and adrenalectomized rats while thyroxine had no such effect in adrenalectomized rats. Morphologically, chief cells responded to corticosterone or thyroxine with increases in both zymogen granules and RER. Chief cells, however, contained less zymogen granules and RER in adrenalectomized and hypothyroid rats. Corticosterone was effective in restoring the normal morphological appearance of chief cells in the hypothyroid rats while thyroxine had no effect in the adrenalectomized rats. In response to corticosterone or thyroxine, parietal cells in normal animals appeared to contain more mitochondria, tubulovesicles and intracellular canaliculi than those of control. Unlike chief cells, parietal cells retained normal ultrastructure in the absence of adrenal and thyroid hormones. These data indicate that (1) corticosterone is necessary for the functional and morphological development of chief cells; (2) the morphological development of parietal cells does not appear to depend upon corticosterone, (3) the effect of thyroxine on the development of chief and parietal cells is due to corticosterone. ^