Sézary syndrome is a rare form of primary cutaneous T cell lymphoma. It is a distinct systemic variant of mycosis fungoides, marked by erythroderma, lymphadenopathy and circulating cerebriform lymphocytes in the peripheral blood. We report a case of Sézary syndrome in a 61-year-old Malay man with a five-year history of indurated plaques, ulcers and tumours on the head and trunk, with characteristic findings on physical examination, skin biopsy, electron microscopy, immunophenotyping and peripheral blood film. A literature review on Sézary syndrome is presented.
A 38-year-old woman presented with right-sided parotid swelling, dry mouth and dry eyes of one year duration. Her Schirmer's test and sialometry were positive and histopathology showed lymphoplasmacytic infiltration. She also had concomitant normochromic , normocytic anaemia and mild haematuria. Her anti-nuclear antibody titre was also positive, 1:640, with a speckled pattern on immunofluorescence. We discuss the atypical presentation of systemic lupus erythematosus, particularly parotitis and secondary Sjogren's syndrome.
A 53-year-old acromegalic woman had cerebrospinal fluid rhinorrhoea following transphenoidal surgery for a pituitary microadenoma. A continuous lumbar spinal fluid drainage catheter was inserted and on the sixth postoperative day, she developed hyponatremia with features of syndrome of inappropriate antidiuretic hormone (SIADH) requiring hypertonic saline administration. Over-drainage is potentially hazardous and close biochemical monitoring is required. To our knowledge, this is the first reported case of SIADH caused by continuous lumbar drainage in an adult.
The incidence of synchronous bilateral infiltrating breast cancer has been reported to be 2%. However, synchronous unilateral infiltrating ductal carcinoma and infiltrating lobular carcinoma (ILC) are very rarely reported. We present a woman with palpable ILC who was later found to have synchronous well-circumscribed ductal carcinoma on further imaging. We also discuss the use of diagnostic approaches such as ultrasonography, mammography and histopathology. This case highlights the importance of careful assessment of concurrent lesions in the breast in the presence of an existing carcinoma.
A 40-year-old Malay woman presented with increasing lethargy, palpitation and shortness of breath, 17 years after a mitral and aortic valve replacement. A Starr-Edwards prosthetic valve replaced the mitral valve, and a Bjork-Shiley prosthetic valve replaced the aortic valve. Biochemical parameters demonstrated intravascular haemolysis, as evidenced by haemoglobin 7.8 g/dL, reticulocyte count 8.4%, lactate dehydrogenase 2,057 IU/L and low haptoglobulin levels (less than 6 mg/dL). Transoesophageal echocardiography revealed a paravalvular leakage over the mitral valve. The haemoglobin levels remained persistently low despite frequent blood transfusions. She successfully underwent a second mitral valve replacement. Her anaemia resolved subsequently.
A 45-year-old Malay lady developed brisk vesicular, plaque-like reaction to a Mantoux test concomitant with a diagnosis of acute myeloid leukaemia (AML). The lesion resolved one month after chemotherapy. Similar lesions developed later after she was bitten by mosquitoes on the forearms. She also had the lesions over her cheek. A skin biopsy showed infiltration of the dermis with neutrophils and some monocytoid cells. The lesion resolved one week after prednisolone therapy.
We report a case of cardiac arrest due to hyperkalaemia following administration of suxamethonium during a procedure to facilitate a change of endotracheal tube in a septic patient. The cause of this rare but fatal complication is briefly described and discussed. In view of this, suxamethonium should be used with great caution in patients with burns and other forms of physical injury, in a number of nervous system disorders, and in critically ill patients requiring prolonged ITU care.
A prospective comparative study was carried out on thirty-seven consecutive patients presenting with bleeding oesophageal varices at University Hospital, Kuala Lumpur. All patients received injection sclerotherapy if active bleeding was seen at the time of initial endoscopy, followed by repetitive courses of sclerotherapy to obliterate the varices. Predominant aetiological factors were hepatitis-B cirrhosis (43%) and alcoholic cirrhosis (30%). Chinese ethnic group accounted for 62.5% of hepatitis-B cirrhotics and Indian 73% of alcoholic cirrhotics. After excluding patients lost to follow-up, analysis of the remaining thirty-four patients showed reduced long-term survival in patients with Child's C disease. Log-rank analysis of survival curves between hepatitis-B cirrhosis and alcoholic cirrhosis in patients with Child's C liver disease showed no significant difference in long-term survival (p = 0.07). However, six deaths were seen in hepatitis-B cirrhosis compared to one death in alcoholic cirrhosis in the first eight months of follow-up. Most patients died from progressive liver failure. Median survival for Child's C hepatitis-B cirrhosis was 7.5 months whereas this had not been reached for Child's C alcoholic cirrhosis (median follow-up 11.6 months). We conclude that variceal haemorrhage in Child's C hepatitis-B cirrhosis is a bad prognostic sign and is associated with reduced survival with a median survival of 7.5 months despite control of the variceal bleed.
INTRODUCTION: Breast cancer is the commonest female cancer in Singapore. It is steadily rising with an incidence of 53.1 cases per 100,000 persons per year among women. Screening for detection of early lesions which are highly curable helps to reduce mortality.
METHODS: Over three afternoon sessions in December 2003, 224 female patients aged 40-65 years, participated in interviews conducted by the National Healthcare Group Polyclinics, Singapore. The survey sought information on mammographic screening history, the time interval since the previous mammographic screening, and the reasons for not going for the screening.
RESULTS: The survey found that only 26.4 percent (28 out of 106) among those aged 40 to 49 years had mammographic screening done within the past one year, and 43.2 percent (51 out of 118) among those aged 50 to 65 years had screening done within the last two years. Chinese women were twice more likely than Malay women to have a mammogram done. The commonest reasons for not wanting to have mammographic screening among women who did not have a mammogram done or had mammogram done more than two years ago, were lack of time (42.5 percent), fear of pain during the procedure (26.9 percent), and the belief that cancer would not happen to them (24.6 percent).
CONCLUSION: Despite publicity on breast cancer being the commonest cancer among women in Singapore and cure being possible if the malignancy was detected early, close to half of the women aged 40-65 years old who attended the National Healthcare Group Polyclinics did not have mammographic screening done. One-quarter of the women who did not have mammogram screening did not do so as they did not think cancer would happen to them.
Study site: NHG Polyclinics, Singapore
One hundred and eighty-three patients undergoing surgery were interviewed twenty-four hours following surgery to assess the quality of pain relief they received in the immediate postoperative period. Interviews were conducted using a standard questionnaire for all patients. They were asked to (1) rate the quality of pain relief they obtained on a Visual Pain Analogue Scale (VPAS-0 being no pain and 10 being the worst imaginable pain); (2) state whether they were happy and satisfied with the pain relief they received; (3) if dissatisfied, they were asked to give their reasons. 37.7% (69 patients) had moderate to severe pain--pain score greater than 6 on the VPAS. Most of these patients had undergone abdominal or major orthopaedic surgery. 32.7% (60 patients) were unhappy with their postoperative pain control. The main reasons for complaint from the patients were that analgesic injections were either not given promptly or were not given at all. The survey also highlighted the inadequate under-administration of narcotic injections in the postoperative period despite orders being written up. It showed there is an urgent need for setting up an Acute Pain Service for better postoperative pain control. An anaesthesiology based Acute Pain Service was started in October 1992.
Acute respiratory distress syndrome (ARDS) associated with severe respiratory syncytial virus infection is rare. We report a 5-month-old Indian girl who was admitted to our intensive care ward with severe respiratory failure who fulfilled the criteria for ARDS using both Murray's Lung Injury Score of > 2.5 and the American-European Consensus Conference definition for ARDS. She developed diffuse bilateral alveolar infiltrates, severe hypoxaemia (PaO2/FiO2 < 100) and required high PEEP (> 15 cm H2O) 24 hours after admission. RSV was isolated from her nasopharyngeal secretion. She also had clinical features suggestive of a primary immunodeficiency and had laboratory evidence of combined T and B cell defect. There was unsustained clinical improvement with a dose of surfactant administered at 36 hours of PICU stay, and she continued to deteriorate and succumbed after 19 days in the PICU.
Supernumerary derivative (22) syndrome is one of the rare genomic syndromes. It is characterised by severe mental retardation, microcephaly, failure to thrive, ear anomalies, preauricular tags or sinus, cleft palate or high arch palate, microganathia, renal anomalies, congenital cardiac defects and genital abnormalities in males. In 99 percent of the cases, one of the parents is a balanced carrier of a translocation between chromosome 11 and chromosome 22. We report the first known case, a female neonate, of supernumerary derivative (22) syndrome from Malaysia.
Candida species is now being increasingly recognised as an important cause of endocarditis especially in immunocompromised patients. A case of Candida albicans endocarditis in a child with acute lymphoblastic leukemia (ALL) is reported. The child did not have a central venous catheter at any time. Treatment consisted of intravenous amphotericin B and fluconazole for 3 weeks followed by oral fluconazole for 2 weeks. No surgical resection was necessary. We highlight here the importance of echocardiography in the management of prolonged febrile neutropenia and discuss the dilemma of continuing chemotherapy in such patients.
True and pseudoaneurysms of the visceral arteries are uncommon. They represent 0.1-0.2 percent of all vascular aneurysms. Visceral artery aneurysms (VAAs) should be treated due to their propensity to rupture and associated high mortality. We describe a 58-year-old man with pseudoaneurysm of the inferior pancreaticoduodenal artery and who presented with post-motor vehicle accident abdominal pain and a pulsatile epigastic mass. Computed tomography (CT) showed a pseudoaneurysm of the visceral artery, and selective mesenteric angiography showed the aneurysm to be arising from the inferior pancreaticoduodenal artery. The aneurysm was successfully treated with endovascular coil embolisation. CT angiogram at one month post-procedure revealed persistent occlusion of the aneurysm. To the best of our knowledge, this is the first reported pseudoaneurysm of inferior pancreaticoduodenal artery secondary to blunt abdominal trauma from a motor vehicle accident and also the first reported VAA from Malaysia.
Subdural collections caused by Salmonella infection are rarely encountered in children. We present two cases caused by non-typhi Salmonella, one a four-and-a-half-month-old boy presenting with subdural effusion, and the other, a 16-month-old boy with empyema. The diagnosis was confirmed on blood and subdural pus cultures. One patient had status epilepticus following focal fit, and the other had prolonged fever without any localising signs of infection on admission. They responded well to prompt surgical drainage and prolonged systemic antibiotic therapy. Contrary to previous reports, both patients showed favourable outcome in terms of neurological sequelae.
Over the last 8 years, 22 consecutive cases of subarachnoid haemorrhage were found to have no obvious cause on angiography. The age, neurological status and CT-scan appearance in 16 cases were studied together with the completeness and quality of the angiogram. Limited angiography was done in cases with advanced age, poor neurology or severe hypertension. There is a need for more repeat angiographic studies in the presence of vasospasm whether focal or generalised, as indicated by the fact that only 5 out of the 17 cases with vasospasm had repeated satisfactory studies.
In a 30-month prospective study, between January 1987 and June 1989, 101 of 64,424 Malaysian neonates (1.6 per 1000 livebirths) born in the Maternity Hospital, Kuala Lumpur were found to have subaponeurotic haemorrhage shortly after delivery. The incidence was highest in neonates weighting 4000 gm or more. There was no significant difference in incidence of this condition in neonates of different ethnic origins. Hypoprothrombinemia was present in only 5/101 (5.0%) of the affected neonates. Sixty seven (66.3%) of the neonates with subaponeurotic haemorrhage had history of trial of vacuum extraction. The incidence of subaponeurotic haemorrhage was significantly higher in neonates delivered by vacuum extraction than by other modes of delivery in this hospital (41.4 per 1000 livebirths in neonates delivered by vacuum extraction versus 1.0 per 1000 livebirths in neonates delivered by other modes). Those neonates who developed subaponeurotic haemorrhage without trial of vacuum extraction had a history of either prolonged labour or difficult delivery. Thirty-three (32.7%) of the neonates with subaponeurotic haemorrhage developed anaemia which required blood transfusion and 3/33 (9.1%) were in shock. Fifty seven (56.4%) of the neonates with subaponeurotic haemorrhage developed hyperbilirubinemia due to the haemorrhage. Four (7.0%) of them had severe unconjugated hyperbilirubinemia which required exchange transfusion. The results of this study suggest that subaponeurotic haemorrhage in Malaysian neonates was commonly associated with vacuum extraction and was not a benign condition.
A study to determine the sensitivity and specificity of the Directogen Group A Streptococcal Test was carried out in a primary care clinic in University Hospital, Kuala Lumpur. The study also looked at the prevalence of streptococcal pharyngitis in the clinic. We found that the rapid test has a sensitivity of 90.9% and a specificity of 90.7%. Viral pharyngitis is the commonest form (63%) of pharyngitis followed by Group A Beta haemolytic streptococcal pharyngitis (14.2%). The presence of exudates, tonsillar enlargement and the presence of tender cervical lymph nodes have a significantly higher association with streptococcal pharyngitis.
Study site: Primary care clinic, University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia
Twenty-nine patients (16 males, 13 females) with spontaneous pneumothorax admitted into Hospital Universiti Sains Malaysia, Kubang Kerian, from September 1984 to September 1990 were reviewed. Their ages ranged from newborn to 75 years. The commonest chief presenting symptom was dyspnoea (69%), followed by chest pain (35%). Four patients had chronic obstructive airway disease, 7 had pneumonia, 2 had pulmonary tuberculosis, one patient had emphysema while 4 patients had multiple underlying lung disorders. The left and right lungs were involved with equal frequency. Bilateral pneumothorax occurred in one patient. Most patients had a single episode of pneumothorax but recurrent pneumothoraces occurred in 3 patients (10%). Six patients were observed conservatively, 20 patients required chest tube insertion alone and 3 patients also required pleurodesis. Death occurred in 8 patients (28%) mainly due to coexisting infection and respiratory failure.
A 47-year old man had hypersplenism from massive splenomegaly, the cause of which was undetermined for 2 years. He was initially asymptomatic though there was mild pancytopenia. However, 18 months after presentation he manifested both clinical and haematological deterioration, almost succumbing to sepsis. Splenectomy finally provided a definite diagnosis of follicular lymphoma and also restored his blood counts to within normal range.