Ten-year epidemiological review of in-hospital patients with Marfan syndrome.

Marfan syndrome is a connective tissue disorder with a worldwide prevalence of 1 in 5,000, without any racial predilection. Major cardiovascular manifestations of Marfan disease often require surgical intervention. The aim of this study was to examine the demographics of patients with Marfan syndrome admitted to Hong Kong hospitals over a 10-year period from 1997 to 2006. We retrospectively reviewed the prospectively collected Hong Kong Health Authority's Clinical Data Analysis and Reporting System and Clinical Medical System. Statistical analysis was performed using SPSS, version 15. A total of 525 patients with Marfan syndrome (310 male, 215 female) were included in this study. For males, mean age at first hospital admission was 19.8 years (range 0-78) and for females, 18.7 years (range 0-60). One hundred and twelve (21.3%) patients (56 male, 56 female) had documented aortoiliac aneurysms and/or dissection, with 74 (66.7%) cases involving the thoracic aorta. Forty-nine (9.3%) patients had major cardiac or aortoiliac operations, with an operative mortality of 5/49 (10.2%). Thirty-seven (7.0%) patients (23 male, 14 female) died during this period, with a mean age at death of 41.0 years (range 0-83) for males and 29.9 years (range 0-59) for females. The majority of these patients died of cardiovascular causes, with four aortic dissections, two ruptured aneurysms, seven with sudden collapse and cardiac arrest, and five with heart failure. In addition, there were other causes of mortality: five perioperative, one congenital, and four pulmonary causes, three with malignancy and one of stroke. The cause of death was unknown in five patients. Patients with aortoiliac diseases have a statistically significant higher mortality rate (p < 0.05). This population-based study shows that significant numbers of patients with Marfan syndrome are admitted to hospital per year, with a significant proportion requiring admissions at a young age. Life span in Marfan patients is markedly shortened, and aortoiliac disease is probably underdiagnosed. A standardized diagnostic and therapeutic follow-up program should be offered to these patients and their families.

Developmental changes of lactose malabsorption in normal Chinese children: a study using breath hydrogen test with a physiological dose of lactose.

The malabsorption of a physiological dose of lactose (0.5 g/kg body weight) was studied in 726 healthy Chinese children, ranging in age from 3 to 18 years, using the breath hydrogen test. The prevalence of lactose malabsorption was found to increase with age; it occurred in less than 15% of preschool-age children and in approximately 45% of younger school-age and 60% of older school-age children. Approximately 70% of adolescents measured showed malabsorption. The critical period of change was from 6 to 7 years of age, with the lactose malabsorption rate rising abruptly from 12 to 43%. The incidence of lactose intolerance in teenagers and adolescents was 27 and 33%, respectively. The great majority of them had only dull abdominal pain. No case of lactose intolerance was seen in children less than 9 years of age. These results indicated that preschool Chinese children can absorb a physiological dose of lactose (equivalent to the average amount of milk consumed daily) without any adverse effects. In contrast, one half of school-age children and two thirds of adolescents were malabsorbers.