The past century has seen tremendous changes in the scope and practice of pathology laboratories in tandem with the development of the medical services in Malaysia. Major progress was made in the areas of training and specialization of pathologists and laboratory technical staff. Today the pathology laboratory services have entered the International arena, and are propelled along the wave of globalization. Many new challenges have emerged as have new players in the field. Landmark developments over the past decade include the establishment of national quality assurance programmes, the mushrooming of private pathology laboratories, the establishment of a National Accreditation Standard for medical testing laboratories based on ISO 15189, and the passing of the Pathology Laboratory Act in Parliament in mid-2007. The Pathology Laboratory Act 2007 seeks to ensure that the pathology laboratory is accountable to the public, meets required standards of practice, participates in Quality Assurance programmes, is run by qualified staff, complies with safety requirements and is subject to continuous audit. The Act is applicable to all private laboratories (stand alone or hospital) and laboratories in statutory bodies (Universities, foundations). It is not applicable to public laboratories (established and operated by the government) and side-room laboratories established in clinics of registered medical or dental practitioners for their own patients (tests as in the First and Second Schedules respectively). Tests of the Third Schedule (home test blood glucose, urine glucose, urine pregnancy test) are also exempted. The Act has 13 Parts and provides for control of the pathology laboratory through approval (to establish and maintain) and licensing (to operate or provide). The approval or license may only be issued to a sole proprietor, partnership or body corporate, and then only if the entity includes a registered medical practitioner. Details of personnel qualifications and laboratory practices are left to be specified by the Director-General of Health, providing for a formal recognition process and room for revision as pathology practices evolve. Encompassed in the responsibilities of the licensee is the requirement that samples are received and results issued through, and management vested in, a registered medical or dental practitioner. This effectively prohibits "walk-ins" to the laboratory and indiscriminate public screening. The requirement for a person-in-charge in accordance with class and speciality of laboratory ensures that the laboratory is under the charge of the pathology profession. Examined carefully, the requirements of the Act are similar to laboratory accreditation, but are backed by legislation. Many of these details will be spelt out in the Regulations, and these in turn are likely to fall back on National professional guidelines, as accreditation does. Although not at first obvious, enforcement of the Act is based on self-regulation by pathology laboratory professionals. Sincere professional input is thus required to embrace its philosophy, ensure rational and transparent enforcement of legislation, and develop National guidelines for good pathology practices upon which enforcement may be based.
Pathology processes ; Pathology procedure ; Laboratories ; public ; seconds

Western-style medicine was introduced to Malaya by the Portuguese, Dutch and British between the 1500s and 1800s. Although the earliest pathology laboratories were developed within hospitals towards the end of the 19th Century, histopathology emerged much later than the biochemistry and bacteriology services. The University Departments of Pathology were the pioneers of the renal histopathology diagnostic services. The Department of Pathology, University of Malaya (UM) received its first renal biopsy on 19 May 1968. Hospital Universiti Kebangsaan Malaysia (HUKM) and Hospital Universiti Sains Malaysia (HUSM) started their services in 1979 and 1987 respectively. It is notable that the early services in these University centres caterred for both the university hospitals and the Ministry of Health (MOH) until the mid-1990s when MOH began to develop its own services, pivoted on renal pathologists trained through Fellowship programmes. Currently, key centres in the MOH are Kuala Lumpur Hospital, Sultanah Aminah Hospital Johor Bahru and Malacca Hospital. With the inclusion of renal biopsy interpretation in the Master of Pathology programmes, basic renal histopathology services became widely available throughout the country from 2000. This subsequently filtered out to the private sector as more histopathologists embraced private practice. There is now active continuing professional development in renal histopathology through clinicopathological dicussions, seminars and workshops. Renal research on amyloid nephropathy, minimal change disease, IgA nephropathy, fibrillary glomerulonephritis, lupus nephritis and microwave technology have provided an insight into the patterns of renal pathology and changing criteria for biopsy. More recently, there has been increasing involvement of renal teams in clinical trials, particularly for lupus nephritis and renal transplant modulation.
Renal ; Histopathology ; Hospitals ; Malaysia ; Pathology procedure

Fontan-associated liver disease (FALD) is one of the main complications after the Fontan procedure, manifesting mostly as liver fibrosis and even cirrhosis, with a high incidence rate and a lack of typical clinical symptoms that seriously affect patient prognosis. The specific cause is unknown, although it is considered to be associated with long-term elevated central venous pressure, impaired hepatic artery blood flow, and other relevant factors. The absence of association between laboratory tests, imaging data, and the severity of liver fibrosis makes clinical diagnosis and monitoring difficult. A liver biopsy is the gold standard for diagnosing and staging liver fibrosis. The most important risk factor for FALD is time following the Fontan procedure; therefore, it is recommended to do a liver biopsy 10 years after the Fontan procedure and to be cautious for the presence of hepatocellular carcinoma. Combined heart-liver transplantation is a recommended choice with favorable outcomes for patients with Fontan circulatory failure and severe hepatic fibrosis.
Humans ; Liver Diseases/pathology* ; Liver Cirrhosis/pathology* ; Liver/pathology* ; Carcinoma, Hepatocellular/pathology* ; Liver Transplantation/adverse effects* ; Fontan Procedure/adverse effects* ; Postoperative Complications/pathology* ; Liver Neoplasms/pathology*

OBJECTIVEThe Pul-stent is a new cobalt alloys stent, specially licensed for pulmonary artery stenosis. The aim of this study was to investigate the value of the stent implantation as the treatment of postoperative pulmonary artery stenosis.

METHODClinical practice was carried out to evaluate effectiveness, safety and maneuverability of Pul-stent in the defined clinical setting. Transthoracic echocardiography, chest roentgenography and electrocardiography were carried out as follow-up studies at 1 and 3 months after procedures.

RESULTTen patients (7 males and 3 females) received Pul-stent implantation in left or right pulmonary arteries (9 stents in left and 1 stent in right). For 9 cases transcatheter stent implantation was performed and in I case hybrid procedure. The median age was 7. 9 years (range 3. 4 - 13. 7 years). The median weight was 22. 3 kg (range 13 - 32 kg). Among them 6 cases were post surgical repair of tetralogy of Fallot, 2 cases were after Fontan procedures, 1 patient was post surgical repair of pulmonary atresia with ventricular septal defect, and 1 patient had stenosis at Glenn pathway. The pressure gradient at the stenosis decreased from (31. 6 ± 10. 2) mmHg(1 mmHg =0. 133 kPa) to (7. 3 ± 10. 3) mmHg, and the diameter of the narrowest segment increased from (4. 0 ± 2. 3) mm to (9. 6 ± 2. 7) mm, the right ventricle to aortic pressure ratio decreased from 0. 54 to 0. 36, all of these improvements were statistically significant (t = 3. 9, -9. 7, 4. 5; P =0. 008, 0. 000, 0. 004). The total procedure time ranged from 55 to 220 min (median 117 min) , and the fluoroscopy time ranged from 9 to 67 min (median 26 min). There were 2 cases of post-stenting pneumorrhagia. No stent fracture, stent malposition and other severe complications were observed. Initial follow-up of 1 and 3-months showed good results with maintenance of improved caliber of the stented vessel, and the gradient across the stenosis area measured by echocardiography was (32. 0 ± 14. 6) mmHg after 3 months. Compared with before stentifng ((40.6 ± 15. 2) mmHg) and 1 month later ((30. 6 ± 13. 6) mmHg), the difference was not statistically significant (t =2. 2,1. 76; P =0. 07, 0. 10).

CONCLUSIONPul-Stent tracking and delivery was excellent, the initial experience has shown that Pul-stent implantation was effective and safe in treating post-operative branch pulmonary artery stenosis. Further follow-up study should be conducted to make sure whether those good results would be kept constant.

Constriction, Pathologic ; Female ; Follow-Up Studies ; Fontan Procedure ; Humans ; Male ; Postoperative Complications ; Pulmonary Artery ; pathology ; Pulmonary Atresia ; Stents

BACKGROUNDThe present study was aimed to compare the effects of bilateral and unilateral bidirectional superior cavopulmonary shunt (b-BDG and u-BDG) on pulmonary artery growth and clinical outcomes.

METHODSThe 51 subjects enrolled in this study were divided into two groups: those receiving b-BDG (n = 21) and those receiving u-BDG (n = 30). Clinical records were reviewed retrospectively at a mean of 43.3 months after BDG procedures. Chi square and t-tests were performed to analyze the data.

RESULTSLeft and right pulmonary artery diameters increased 27% - 37% in both groups. The pulmonary artery index increased 37.2% after b-BDG and 27.0% after u-BDG. b-BDG patients experienced a significant decrease in mean hemoglobin concentration and hematocrit value, and a correlated change in postoperative diameter of left pulmonary artery (LPA) and pulmonary artery index (y = 0.2719, x = -1.8278; R = 0.564, P = 0.008). The change ratio of hemoglobin and postoperative LPA were also correlated in b-BDG patients (y = -0.0522x + 0.3539; R = -0.479, P = 0.028). Only one b-BDG patient versus twelve u-BDG patients needed total cavopulmonary connections 31.8 months after BDG surgery (P = 0.0074). Moreover, only one (4.8%) b-BDG patient but eight u-BDG patients (26.7%) developed pulmonary arteriovenous malformations.

CONCLUSIONSb-BDG increases bilateral pulmonary blood flow and promotes growth of bilateral pulmonary arteries, with preferable physiological outcomes to u-BDG. Results may imply that subsequent Fontan repair may not always be needed.

Cardiopulmonary Bypass ; methods ; Child ; Child, Preschool ; Female ; Fontan Procedure ; methods ; Heart Defects, Congenital ; physiopathology ; surgery ; Humans ; Infant ; Lung ; pathology ; surgery ; Male ; Pulmonary Circulation ; Retrospective Studies ; Treatment Outcome

The management of pulmonary atresia with intact ventricular septum (PA/IVS) remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular (Fontan or one and a half ventricle repair) strategies. Although outcomes have been improved, these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials (RCTs) comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival, and secondary criteria included exercise function, arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless, several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review, we will discuss the currently available data.
Arrhythmias, Cardiac ; physiopathology ; prevention & control ; Cardiac Valve Annuloplasty ; methods ; mortality ; Fontan Procedure ; methods ; mortality ; Heart Defects, Congenital ; mortality ; pathology ; surgery ; Heart Ventricles ; abnormalities ; pathology ; surgery ; Humans ; Pulmonary Atresia ; mortality ; pathology ; surgery ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

Fontan surgery is a widely used palliative procedure that significantly improves the survival period of patients with complex congenital heart disease (CHD). However, it does not decrease postoperative complication rate. Previous studies suggested that elevated mean pulmonary artery pressure (mPAP) and vascular resistance lead to decreased exercise tolerance and myocardial dysfunction. Therapy with endothelial receptor antagonists (Bosentan) has been demonstrated to improve the patients' prognosis. A double-blind, randomized controlled trial was performed to explore the efficacy of Bosentan in treating patients who underwent the Fontan procedure. Eligible participants were randomly divided into Bosentan group and control group. Liver function was tested at a local hospital and the results were reported to the phone inspector every month. If the results suggested abnormal liver function, treatment would be adjusted or terminated. All the participants finished the follow-up study, with no patients lost to follow-up. Unblinding after 2-year follow-up, no mortality was observed in either group. However, secondary end-points were found to be significantly different in the comparable groups. The cardiac function and 6-min walking distance in the Bosentan group were significantly superior to those in the control group (P=0.018 and P=0.027). Bosentan could improve New York Heart Association (NYHA) functional status and improve the results of the 6-min walking test (6MWT) in Fontan patients post-surgery, and no other benefits were observed. Furthermore, a primary meta-analysis study systematically reviewed all the similar clinical trails worldwide and concluded an overall NYHA class improvement in Fontan patients who received Bosentan treatments.
Adolescent ; Child ; Double-Blind Method ; Female ; Follow-Up Studies ; Fontan Procedure ; Heart Defects, Congenital ; drug therapy ; pathology ; surgery ; Humans ; Liver ; drug effects ; pathology ; Male ; Palliative Care ; Prognosis ; Sulfonamides ; administration & dosage ; Treatment Outcome