Hereditary pancreatitis (HP) is a very rare form of early-onset chronic pancreatitis, which usually begins in childhood with a variable spectrum of severity of disease. HP is commonly caused by variants/mutations in the PRSS1 gene as reported in many studies. Therefore, in this study, we aimed to investigate the possible association of PRSS1 gene variants/mutations in a Malaysian Chinese family with HP.
There is very little information in literature describing ethnic variations in etiologic and clinical outcome of acute pancreatitis in the Asian population. This study describes the demographic, etiologic and clinical course of acute pancreatitis among the three main races in Malaysia namely, the Malays, Chinese and Indians. One hundred and thirty-three consecutive patients were admitted for acute pancreatitis for the period January 1994 to July 1999 and they consisted of 77 males and 56 females with a mean age of 43.5 years (SD+/- 14.7). The racial breakdown of acute pancreatitis was: Malays 38 (28.6%), Chinese 19 (14.3%), Indians 75 (56.4%) and 1 (0.8%) patient was an orang asli. The incidence of alcohol association with acute pancreatitis was significantly increased in the males, while gallstone pancreatitis was principally a disease of the female. Alcohol was identified as the predominant factor associated with acute pancreatitis among the Indians (73.3%) and in contrast, gallstone was the commonest associated etiologic factor for the Malays and Chinese. No etiologic factor could be identified in a substantial proportion of the Malay patients (60.5%) when compared to the Chinese (36.8%) and Indians (35%). Severe disease developed in 25% of the cases reviewed but there was no difference in of the rate of severe pancreatitis in terms of ethnic groupings or etiologic factors. The overall mortality rate was 7.5% and the commonest cause of death was multi-organ failure. The study recognises that there are differences in the characteristics of acute pancreatitis among the three major races in the country and this divergence is primarily due to sociocultural habits.
OBJECTIVES: To describe the features and treatment outcomes of IgG4-RD in multi-ethnic patients in Singapore.
METHODS: Retrospective study was performed on IgG4-RD patients identified from patient databases in a tertiary hospital.
RESULTS: Fourty-two patients (76% male) were included; 79% fulfilled the 2011 comprehensive diagnostic criteria for IgG4-RD for definite IgG4-RD. 81% were Chinese and 19% were Malays. Common initial manifestations included jaundice (52%), abdominal pain (36%) and swollen salivary glands (26%). Only 36% had a history of allergy. 83% had ≥ 1 organ involvement. Erythrocyte sedimentation rate, immunoglobulin E, IgG2 and IgG4 levels were elevated in 84%, 100%, 70% and 44% of patients, respectively. The most common histopathological feature was >10 IgG4+ cells per high power field (66%). 94% (34/36) of patients were treated with moderate to high doses of glucocorticoids, including 17 patients with combination immunosuppressants. Of these, all patients responded to therapy by 3 months. With a median (range) follow-up of 4.1 (0.4-13.8) years, 69% (25/36) needed low dose of glucocorticoids to maintain disease remission. Twenty-six per cent had relapse of disease, of which 82% had disease recurrence in the same organs.
CONCLUSIONS: Pancreatitis, lymphoadenopathy and cholangitis were the commonest manifestations in Asians with IgG4-RD. All patients responded to glucocorticoid therapy by 3 months, two-thirds required maintenance therapy with glucocorticoids, and one-quarter developed relapse of disease.