METHODS: Delivery records in Hospital Sultanah Nur Zahirah, Terengganu from 1st. January 2016 until 31st. December 2016 were reviewed and analysed.
RESULTS: Out of the 15,837 deliveries, eight cases of MAP were identified. Six out of eight patients had previous caesarean scar with concomitant placenta praevia, the other two patients had previous caesarean scar with history of placenta praevia in previous pregnancies. Seven out of eight cases were suspected to have MAP based on risk factors. Correct diagnosis was made by ultrasound in five patients, all with histologically confirmed moderate/severe degree of abnormal placentation. The other two cases of 'unlikely MAP', demonstrated segmental MAP intra-operatively with histologically confirmed milder degree of abnormal placentation. Total intraoperative blood loss ranged from 0.8 to 20 litres. Prophylactic internal iliac artery balloon occlusion was associated with significantly less blood loss.
CONCLUSION: Antenatal diagnosis is essential in outlining the best management strategy in patients with MAP. Ultrasound may not be accurate in ruling out lower degree of MAP. Apart from having a scarred uterus with concomitant placenta praevia, history of having placenta praevia in previous pregnancy is also a risk factor for MAP. Prophylactic internal iliac artery balloon occlusion is associated with significantly less blood loss and should be considered in cases suspected with MAP.
METHODS: Data from the Malaysia Primary Immunodeficiency Network (MyPIN) with cases of CGD diagnosed from 1991 until 2016 were collated and analysed.
RESULTS: Twenty patients were diagnosed as CGD. Males (N = 13, 65%) outnumber females (N = 7, 35%). CGD is commonest amongst the Malays (65%) followed by the Chinese (15.0%), Indians (10.0%) and natives of Borneo (10.0%), reflecting the ethnic composition of the country. The mean age of diagnosis was 3.7 years. There was a positive family history in 40% of the cases. Abscess was the main presenting feature in 16 patients (80%) with one involving the brain. Pneumonia occurred in 10 (50%) and one with complicated bronchiectasis. Catalase-positive bacteria were the most commonly isolated pathogen with Chromobacterium violaceum predominating (N = 5, 25%) with consequent high mortality (N = 4, 80%). All CGD patients with C. violaceum infection displayed CD4 + (T helper cells) lymphopenia.
CONCLUSION: This study has shown CGD occurs in the major ethnic groups of Malaysia. To the best of our knowledge, this is the first and the largest series of chronic granulomatous disease in South East Asia which may be reflective of similar clinical pattern in the region. C. violaceum infection is associated with a higher mortality in CGD patients in Malaysia. All the CGD patients with C. violaceum infection in this patient series displayed CD4 + (T helper) lymphopenia. We recorded rare clinical manifestation of CGD viz. brain abscess and bronchiectasis.