Clinical picture of endemic cretinism in central Apennines (Montefeltro).

Endemic cretinism is still present in an endemic goiter area of the central Apennines (Montefeltro) (goiter prevalence 55%; mean urinary iodine level 39 micrograms/g creatinine). Clinical and biochemical features of patients with myxedematous, neurologic, and mixed cretinism were studied. Also, in this area, as in most other, neurologic cretinism is more prevalent than myxedematous and mixed forms. The hormonal profiles of the three types of cretinism were clearly different. Nevertheless, all myxedematous cretins had some neurologic disorders (hyperreflexia, increased muscle tone, disorder of gait, Babinski sign, hypoacusia) that were similar to those present in neurologic cretins. These findings suggest that neurologic damage is very similar in all forms of endemic cretinism, reflecting a diffuse insult to the developing fetal nervous system. Furthermore, these data support the hypothesis that the primary pathophysiologic event in the different types of endemic cretinism is represented by maternal and fetal hypothyroidism, while differences may be explained by the extent and duration of postnatal hypothyroidism. All the cretins were over 35 of age, suggesting a severe iodine deficiency in the past decades, and a progressive improvement of nutritional status resulted in "silent iodine prophylaxis." However, recent studies have revealed the persistence of a moderate iodine deficiency, a high prevalence of neurologic hypoacusia, and reduction of mental performance in normal schoolchildren of this area. These findings constitute strong evidence in favor of adequate iodine prophylaxis.

Effects of ursodeoxycholic acid on serum liver enzymes in patients with hepatitis C virus-related chronic liver disease.

OBJECTIVE: To study the effect of ursodeoxycholic acid (UDCA) on serum liver enzyme levels [alanine aminotransferase (ALT) and gamma-glutamyl transferase (GGT)] in 101 patients with hepatitis C virus-related chronic liver disease. METHODS: Forty-nine patients were assigned to receive UDCA (450 mg/day) over a period of 6 months and 52 to receive no treatment. RESULTS: In the UDCA group, serum ALT and GGT levels significantly improved. ALT values decreased from pre-treatment levels of 157.0 +/- 62.6 IU/l to 82.5 +/- 46.4 IU/l (P < 0.05), and GGT fell from 141.3 +/- 86.2 IU/l to 66.0 +/- 49.5 IU/l (P < 0.001). No significant change occurred in the mean ALT and GGT levels in the control group. CONCLUSION: Although our encouraging preliminary results must be validated by double-blind histological trials, UDCA may be an alternative treatment for patients who fail to respond to interferon therapy.