Recurrent respiratory papillomatosis (RRP) is a benign disease caused by the human papilloma virus (HPV), characterized by the formation of recurrent, epithelial neoplastic lesions in the airways. While benign, they can cause significant airway obstruction in some cases. Difficulties in treatment arise from the recurrent nature of the lesions despite repeated procedures. Other known procedures that result in deep tissue damage also cause unacceptable collateral damage to the underlying airway mucosa. We describe a case of recurrent papillomatosis that was successfully treated with argon plasma coagulation ( APC) when laser and electrocautery ablation had failed in the past. After the papillomatasis was treated with APC, there is no recurrence on repeat scope at 4 months and 9 months after the initial procedure. The procedure was done as a day case and there is no complication from the procedure. The property of the APC that allows it to cause only superficial thermal damage to the tissue makes it a suitable adjunct therapy to the treatment of papillomas, which are usually superficial lesions.
We report a case of Griscelli Syndrome (GS). Our patient initially presented with a diagnosis of haemophagocytic lymphistiocytosis (HLH). Subsequent microscopic analysis of the patient's hair follicle revealed abnormal distribution of melanosomes in the shaft, which is a hallmark for GS. Analysis of RAB27A gene in this patient revealed a homozygous mutation in exon 6, c.550C>T, p.R184X . This nonsense mutation causes premature truncation of the protein resulting in a dysfunctional RAB27A. Recognition of GS allows appropriate institution of therapy namely chemotherapy for HLH and curative haemotopoeitic stem cell transplantation.
Kimura's disease is rare chronic inflammatory disease with a distinct clinicopathological entity. It has three major components; inflammatory, vascular and fibrosis. It has to be considered as a differential diagnosis in young patient presenting with head and neck swelling. Although of unknown aetiology many hypothesis has been postulated. Inflammation is the most prominent and predominating characteristic in this disease. Although reported to be predominant in Asian literature regarding this disease is scanty. We report a complete clinical-radiological and pathological picture of this disease.
There is a resurgence of tuberculosis globally but lesions affecting the skull are rare. Cases reported are of single, focal lesions as seen on plain x-rays. We report a 34 yearold patient with tuberculosis of the skull where multiple punched out lesions are seen, mimicking that of multiple myeloma.
hemangiopericytoma (hPC) is a rare tumor by definition and intracranial hPC makes up to less than one percent of all the intracranial tumors. It is a dural base tumor and its clinical features and radiological findings are similar to meningiomas. however, cerebellopontine angle hemangipericytoma had only been reported twice and would almost always be misdiagnosed. definite diagnosis is important, as the treatment of hPC is different from meningiomas and acoustic neuromas. we report a case of a young female who presented with atypical symptoms of left cerebellopontine angle mass. A literature review of the nature of the disease, radiological findings, immunohistochemical features and treatment options of the tumor are described.
deep venous thrombosis (dVT) in lower limbs caused by abdominal aortic aneurysm (AAA) is rare. A combination of anatomical obstruction with surrounding inflammatory tissue and reduced blood flow in venous limb were probably the main reasons for the occurrence of the dVT. we report a case of infra-renal AAA presented as lower limb dVT and symptomatic pulmonary embolism.
Prolonged fever in patients can be a diagnostic challenge. Clinicians generally consider infectious diseases, malignant diseases and collagen vascular diseases as possible causes of pyrexia of unknown origin (PUO). Even after extensive evaluation as many as 15 percent of patients with prolonged fever may remain undiagnosed. This case report describes subacute thyroiditis as a cause of prolonged fever and documents how that diagnosis was finally made after 40 days of fever.
The aim of this study was to evaluate the management and clinical outcome of transfusion-dependent thalassaemia children receiving care in the Paediatric Ambulatory Care Centre, Hospital Tuanku Ja'afar Seremban in comparison to The Malaysian Clinical Practice Guidelines. The demography, management and clinical outcome of the patients were documented using a checklist. Information on compliance to chelation agents was obtained through interview. There were twenty-six patients recruited in this study out of thirty seven patients registered in the centre. This study showed that more effort and vigilance should be given to ensure that the management of these patients adheres to the guidelines and clinical outcome of these patients monitored closely.
Study site: Paediatric Ambulatory Care Centre, Hospital Tunku Ja'afar, Seremban.
INTRODUCTION: Glycohemoglobin (HbA1c) most accurately reflects the previous two to three months of glycaemic control. HbA1c should be measured regularly in all patients with diabetes, and values should be maintained below 7% to prevent the risk of chronic complications. Apart from the genetic variants of haemoglobins many other conditions also known to affect HbA1c measurements. In this study we evaluated the conditions that cause low HbA1c results.
METHODS AND MATERIALS: The data was collected retrospectively HbA1c was measured in our laboratory by Biorad Variant II turbo 2.0. The method is based on chromatographic separation of HbA1c on a cation exchange cartridge. This method has been certified by National Glycohemoglobin Standardization Programme (NGSP). 58437 requests were received in a period of one year (January to December 2011). Medical records were reviewed to identify the conditions that might be associated with these low values.
RESULTS: Among 58437 samples analysed, 53 patients had HbA1c levels < 4.0%. Fourteen patients had haemoglobinopathy. In 34 patients without Hb variants had conditions such as chronic liver disease, chronic kidney disease, haemolytic anaemia, pregnancy, and anaemia of chronic disease. Five non-pregnant individuals who were screened for diabetes mellitus had HbA1c levels < 4%.
CONCLUSION: Our study underscores the importance of that both laboratories and the physicians should be aware of the factors that can influence the HbA1c results. The haematological status should be taken into consideration for proper interpretation of HbA1c results.
INTRODUCTION: Carotid intima media thickness (CIMT) being a cost effective and easily performed technique is useful in the detection of subclinical atherosclerosis and has been shown to be a prognosticator of cardiovascular events. The primary objective of this study was to obtain the distribution of CIMT measurements, highly sensitive C reactive protein (hs-CRP) and assessing health awareness and attitudes of the Malaysian population at cardiovascular disease (CVD) risk and not receiving lipid lowering agents. Secondarily the study sought to assess the significance of the relationship between these measurements against various patient characteristics.
METHODS: Measurements of CIMT are obtained by ultrasonography of 12 sites within the common carotid artery was recorded for 123 subjects from a single centre tertiary hospital of Malaysia who had two or more CVD risk factors but were not receiving lipid lowering therapy. CVD risk factors and lipid and glucose profiles were analyzed with respect to distribution of CIMT and high-sensitivity Creactive protein (hs-CRP) values.
RESULTS: The mean-max CIMT was 0.916±0.129mm (minimum 0.630mm, maximum 1.28mm) and the mean-mean CIMT was 0.743±0.110mm (minimum 0.482mm, maximum 1.050mm) and mean hs-CRP was 0.191mg/dL (minimum 0.030mg/dL, maximum 5.440mg/dL). Multivariate analyses confirmed a significant association between increasing CIMT and increasing age, total and low density lipoprotein cholesterol while log-transformed hs-CRP levels showed significant association with increasing body mass index, waist circumference, high blood glucose and triglyceride levels. Our patients had good health awareness on CVD.
CONCLUSION: Newly defined CIMT measurements and hs-CRP levels may be useful adjunctive tools to screen for atherosclerosis in the Malaysian population. It may help in refining risk stratification on top of traditional clinical assessment.
A Health Technology Assessment (HTA) was conducted in 2011 to evaluate whether transnasal oesophagoscopy (TNE) should be made available at otorhinolaryngology (ORL) clinics in the Ministry of Health facilities. The safety, efficacy or effectiveness and economic implication of using transnasal oesophagoscopy (TNE) were reviewed. This review provides a summary of the HTA and an updated literature review as well as how this technology might potentially affect services in the Ministry of Health.
AIM OF STUDY: This study investigated the consistency in Auditory Brainstem Response (ABR) waveform evaluations between two audiologists (inter-audiologist agreement) and within each of the audiologist (intra-audiologist agreement).
METHODS: Two audiologists from one of the audiology clinics in Kuantan, Pahang, Malaysia were involved in this study. Both audiologists were required to identify and mark the presence of Waves I, III and V in 66 ABR waveforms. Over a one-month interval, each audiologist was required to carry out the same procedure on the same ABR waveforms. This process was continued until we had three separate reviews from each audiologist.
RESULTS: There was a high inter-audiologist ABR waveform identification agreement (over the range 81.71-89.77%), but a lower intra-audiologist ABR waveform identification agreement (over the range 50%-78%) for both audiologists. Our results also showed a high intra-audiologist ABR latency agreement within 0.2 ms (>90%), but a slightly lower inter-audiologist latency agreement (75-84%) within 0.2 ms.
CONCLUSION: Our results support the need for the clinic to implement further strategies for improving the respective lower agreements and consistencies. These include conducting a continuous education program and using an objective algorithm to support their interpretations.
Study site:; International Islamic University, Malaysia (IIUM) Hearing
and Speech Clinic
Spontaneous thyroid haemorrhages are rare. There are reported cases occurring in thyroid nodules and cysts but none in thyroid malignancies. We describe a 48 year old who presented to the on-call ENT team with a rapidly progressing neck swelling that was interfering with his airway. After resuscitation, the patient underwent a right lobectomy to stop the bleeding. Histology showed a thyroid follicular carcinoma. As per the regional multidisciplinary team discussion, he underwent a completion thyroidectomy followed by radioactive iodine treatment. We conclude that spontaneous haemorrhages of the thyroid gland can occur in malignancies and stress the importance of early histological diagnosis.
Tuberculosis, an ancient disease continues to be a health care burden in Malaysia in the 21st century. Extrapulmonary tuberculosis is a less common presentation of tuberculosis and in particular peripancreatic tuberculous lymphadenitis is rarely seen. We report a case of a young immunocompetent boy presenting with a two month history of non productive chronic cough associated with loss of appetite and loss of weight. Investigations including CT Scan and Endoscopic ultrasound revealed multiple mediastinal lymphadenopathy and peripancreatic lymphadenopathy with central caseation necrosis. Histology of cervical lymph node was suggestive of tuberculosis and mycobacterium PCR was positive. The patient was subsequently treated with antituberculous therapy and had marked clinical improvement of his symptoms. This case outlines a rare presentation of a common disease and the application of newer investigative tools in making the diagnosis.
Hemobilia is a rare but potentially lethal condition. The commonest cause of hemobilia is trauma, accounting up to 85% of all cases. Hemobilia caused by gallstones is very rare. Most of the cases of hemobilia are either managed conservatively or treated by embolization. Surgery is indicated only when there is an associated surgical condition or when embolization fails. We report a case of a 72-year-old patient with massive hemobilia caused by gallstone erosion to the adjacent artery, diagnosed intraoperatively. The complication was successfully managed by cholecystectomy and repair of the bleeding vessel. This case highlights the importance that hemobilia should be suspected in patients presenting with upper gastrointestinal bleeding. Although rare, massive hemobilia can be life threatening, leading to significant morbidity and mortality. Therefore, a high index of suspicion and timely intervention are important.
Tracheal agenesis is a rare congenital airway anomaly which presents as an airway emergency at birth. We report a case of late premature Chinese infant with tracheal agenesis type II (by Floyd's classification) who presented with severe respiratory distress at birth. He had multiple failed attempts at intubations with accidental oesophageal intubation and ventilation. Tracheal agenesis with tracheo-oesophageal fistula was suspected from an emergency optical laryngoesophagoscopy done. The infant was subsequently stabilized on oesophageal ventilation. The diagnosis was confirmed on CT scan and parents were counseled regarding the poor outcome and decided for withdrawal at day 7 of life.
AIM: This was a randomized single blinded study to determine optimal size for Ambu®LMA (ALMA) among Malaysian adult population.
METHODS: One hundred and twenty six non-paralyzed anaesthetized adult patients were block randomized into size 3, 4 and 5 Ambu®LMA. Optimal size is defined primarily by oropharyngeal pressure (OLP). Pharyngeal injury and ease of insertion are also taken into consideration.
RESULTS: Mean OLP was significantly higher for Size 4 and 5 compared to size 3 (p<0.001) but similar between size 4 and 5. Number of insertion attempts and insertion time were similar between sizes. Size 5 required more manipulations during insertion (p<0.005) and had higher pharyngeal injury (p=0.001) compared to size 3 and 4.
DISCUSSION: We recommend size 4 ALMA as the optimal size for Malaysian adults in view of the higher OLP compared to size 3, yet less pharyngeal injury than size 5 in spontaneously breathing patients.