This study was carried out to ascertain the aetiology of exudative pleural effusions when other diagnostic investigations such as pleural fluid and sputum examination for cytology and acid fast bacilli fail to yield a definitive diagnosis and to differentiate between tuberculosis and malignancy in cases suspicious of malignancy.
Infection associated hemophagocytic syndrome is increasingly recognized as a potentially fatal complication of dengue fever. It should be suspected with prolonged fever beyond seven days associated with hepatosplenomegaly, hyperferritinemia, worsening cytopenias and development of multiorgan dysfunction. Surge of similar pro-inflammatory cytokines observed in dengue associated hemophagocytic syndrome and multiorgan dysfunction may indicate they are part of related inflammatory spectrum. A proportion of patients recovered with supportive therapy, however most required interventions with corticosteroids, intravenous immunoglobulin or chemotherapy. We report three cases of dengue associated IAHS with good outcome following early recognition and treatment with dexamethasone and intravenous immunoglobulin.
Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome) is caused by deficient activity of the enzyme, N-acetylgalactosamine-4-sulfatase, resulting in impaired degradation of the glycosaminoglycan dermatan sulfate. Patients experience a range of manifestations including joint contractures, short stature, dysostosis multiplex, coarse facial features, decreased pulmonary function, cardiac abnormalities, corneal clouding and shortened life span. Recently, clinicians from institutions in the Asia-Pacific region met to discuss the occurrence and implications of delayed diagnosis and misdiagnosis of MPS VI in the patients they have managed. Eighteen patients (44% female) were diagnosed. The most common sign presented by the patients was bone deformities in 11 patients (65%). Delays to diagnosis occurred due to the lack of or distance to diagnostic facilities for four patients (31%), alternative diagnoses for two patients (15%), and misleading symptoms experienced by two patients (15%). Several patients experienced manifestations that were subtler than would be expected and were subsequently overlooked. Several cases highlighted the unique challenges associated with diagnosing MPS VI from the perspective of different specialties and provide insights into how these patients initially present, which may help to elucidate strategies to improve the diagnosis of MPS VI.
Three children aged 3-11 years had ultrasonography of the urinary tract for the investigation of dysuria and haematuria. A bladder mass was seen in these 3 children. One child had computed tomography scan, cystoscopy and bladder biopsy because rhabdomyosarcoma was considered. The biopsy revealed an inflammatory process. The urine culture of the other 2 children revealed E. coli. On ultrasonography, the inflammatory mass may appear homogeneously hypoechoic or may contain moderate level echoes. The mucosal surface of the mass may be smooth or lobulated. It is important to consider an infective cause for a bladder mass in children because computed tomography, cystoscopy and biopsy may be avoided.
Kimura's disease is a chronic inflammatory disorder of unknown etiology commonly seen among orientals and characterized histologically by lymphatic follicles, vascular proliferation, and marked eosinophilic infiltration. It has a predilection for the head and neck region. The lesion is benign but can be mistaken to be a malignant lesion.
There is presently no line of distinction between oral lichen planus and other oral lichenoid lesions. The aim of this study is to determine using histomorphometry, the differences between these lesions. Paraffin sections from 7 normal buccal epithelium, 19 oral lichen planus (LP), 14 oral lichenoid lesions (LL) and 7 discoid lupus erythematosus-like lesions (DLE-ll) were selected. The nuclear volume (V(N)) and cellular-volume (V(CELL)) of the epithelium were assessed using an image analyser. The V(N) and V(CELL), derived for both basal and spinal strata in LP and DLE-ll were 2.3 times more than that of normal tissues. There was a significant difference between LP and LL (P < 0.005) and between LL and DLE-ll (P < 0.001), but not between LP and DLE-ll. In conclusion, there appears to be a difference between LP, LL and DLE-ll and V(N) and V(CELL) may serve as potential discriminators between these groups of lesions.
We report a case of a lady presenting with a lesion in the distal radius with classical radiological features of a giant-cell tumour. These tumours are often resected without preliminary histological confirmation. A biopsy done in this patient showed it to be tuberculosis.
Video-EEG telemetry is often used to support the diagnosis of non-epileptic seizures (NES). Although rare, some patients may have both epileptic seizures (ES) and NES. It is crucially important to identify such patients to avoid the hazards of inappropriate anticonvulsant withdrawal. To delineate the electroclinical characteristics and diagnostic problems in this group of patients, we studied the clinical, EEG and MRI features of 14 consecutive patients in whom separate attacks, considered to be both NES and ES were recorded using video-EEG telemetry. Only two patients were drug-reduced during the telemetry. Most patients had their first seizure (ES or NES) in childhood (median age 7 years; range: 6 months-24 years); 8/14 patients were female. Brain MRI was abnormal in 10/14 patients. Interictal EEG abnormalities were present in all patients; 13/14 had epileptiform and 1/14 only background abnormalities. Over 70 seizures were recorded in these 14 patients: in 12/14 patients, the first recorded seizure was a NES (p < 0.001), and 7 of these patients had at least one more NES before an ES was recorded. Only 3/14 patients had more than 5 NES before an ES was recorded. Recording a small number of apparently NES in an individual by no means precludes the possibility of additional epilepsy. Particular care should be taken, and multiple (> 5) seizure recording may be advisable, in patients with a young age of seizure onset, interictal EEG abnormalities, or a clear, potential aetiology for epilepsy.
An acute abdomen in pregnancy can be caused by pregnancy itself, be predisposed to by pregnancy or be the result of a purely incidental cause. These various conditions are discussed. The obstetrician often has a difficult task in diagnosing and managing the acute abdomen in pregnancy. The clinical evaluation is generally confounded by the various anatomical and physiological changes occurring in pregnancy itself. Clinical examination is further hampered by the gravid uterus. The general reluctance to use conventional X-rays because of the pregnancy should be set aside when faced with the seriously ill mother. A reluctance to operate during pregnancy adds unnecessary delay, which increases morbidity for both mother and fetus. Such mistakes should be avoided as prompt diagnosis and appropriate therapy are crucial. A general approach to acute abdominal conditions in pregnancy is to manage these problems regardless of the pregnancy.
Rectal duplications are rare anomalies. Recently, we observed four cases of rectal duplication, each presenting with different clinical features including chronic constipation, a prolapsing rectal "polyp, " a "growth" from the vulva, and acute retention of urine. The variety of clinical presentations may lead to delay in diagnosis and multiple operations.
Fine needle aspiration (FNA) cytology is now an integral part of the pre-operative investigation of breast lesions and the therapeutic protocol is today often planned on the basis of cytodiagnosis. However, from time to time the cytological picture may be equivocal or inconclusive. In recent years, nucleolar organizer region (NOR) scores have been explored for potential value in the diagnosis of malignancy as the scores in malignant nuclei are seen to be higher than in benign or reactive nuclei. With a view to applying NOR scoring in the evaluation of cytologically equivocal cases, we adopted the argyrophil technique for staining NOR s (AgNOR) in FNA cytological smears of 56 breast lesions, comprising 31 benign and 25 malignant lesions. Histological correlation was possible in 26 of these cases (17 malignant and 9 benign) and AgNOR scoring was done on paraffin sections of these as well. There was a significant difference between mean AgNOR scores in benign and malignant lesions in the cytological smears (P < 0.001). The AgNOR scores ranged from 2.5 to 5.0 per cell in benign lesions and 5.8 to 17.2 per cell in malignant lesions. None of the cases fell into the gray zone of overlap. One malignant lesion that was cytologically equivocal showed a mean AgNOR score of 6.08. The AgNOR scores on histological sections also showed a statistically significant difference (P < 0.001) between benign and malignant lesions with mean scores ranging from 1.34 to 2.58 dots per cell in benign lesions and scores of 2.42 to 5.28 dots per cell in malignant lesions. However, the scores overlapped in four cases and therefore it was considered unsuitable for routine diagnostic work. From this preliminary study, we conclude that an FNA AgNOR score of 5.0 and less strongly favours a benign lesion whereas a score above 5.0 would be in favour of a malignant lesion. A larger study would be needed to verify our impression that AgNOR scoring can be useful in cytologically equivocal cases.
We reviewed biopsy records for 37 cases of oral histoplasmosis for patient characteristics, clinical features, and histopathologic findings. These represented cases diagnosed in the Division of Stomatology, Institute for Medical Research, Kuala Lumpur between July 1967 and October 1994. All were male patients who ranged in age from 11 to 79 years (mean age, 56.7 years). There were 40.6% Malays, 37.8% Chinese, 18.9% Indians, and 2.7% other races. Five patients with mouth lesions as the initial presenting lesions were proven to be cases of disseminated histoplasmosis. In the remaining cases apart from the biopsy-proven oral histoplasmosis lesions, the extent of the disease elsewhere was unknown. The majority of these lesions involved the gingiva, tongue, and palate in decreasing order of frequency. The most frequent presenting symptom was oral mucosal ulceration. Squamous cell carcinoma and tuberculosis were the two most common clinical differential diagnoses. Our present findings compare favorably with published reports from other regions.
Two cases of either peripheral odontogenic fibroma (POF) (WHO type) or peripheral ameloblastoma are reported. Their immunohistochemical characteristics were investigated in an attempt to clarify their histogenesis. The results showed that the epithelial component of this neoplasm tended to retain its distinct odontogenic character and expressed a keratin profile different from that of the overlying oral epithelium from which both cases most probably originated. The connective tissue element of these tumors was vimentin-positive and S-100 protein negative, confirming their mesodermal nature but precluding the possibility of ectomesenchymal derivation. No reactivity for desmin was noted.
X-ray microradiography of surgically excised breast specimens offers the possibility of morphological characterization of calcifications. When combined with digital imaging techniques there exists added potential for obtaining valuable basic quantitative morphometric information regarding differences between microcalcifications in tissues exhibiting evidence of fibrocystic change, benign and malignant tumours. A total of 157 excised breast specimens from 84 patients were microradiographed using a Softex Super Soft X-ray unit and Kodak AA high resolution industrial film. A Quantimet 570C image analysis system was used to digitize and analyse the microradiographs. Of the 157 microradiographs, 51 (from 30 patients) revealed microcalcification clusters. The existence of significant differences between the three identified categories of tissue were indicated by clustering parameters. These included the number of particles per cluster, area of clusters, maximum distance to nearest neighbour, and geometric mean distance to nearest neighbour. The distribution pattern index (DPI), another of the clustering parameters used in this study, has been observed to be a particularly powerful discriminator. The value for fibrocystic change was found to be significantly smaller (0.514) than that for benign tumour (0.796) whilst that for benign tumour was observed to be significantly larger than that for malignant tumour (0.604) at a p-value of less than 0.05 (Kruskal-Wallis one-way analysis of variance).
Primary hyperparathyroidism (PHPT) is an intriguing condition. Routine automated biochemical screening has made the diagnosis commonplace in developed countries and the disease is diagnosed early in its course when it is often asymptomatic. In developing countries or in recent immigrants from these countries, PHPT is often seen in an advanced stage with bone involvement. Associated dietary deficiencies may alter the biochemical profile and cause a diagnostic dilemma. It is important to include it in the differential diagnosis of pathological fractures. We report three cases of PHPT presenting with pathological fractures and discuss their diagnosis and management.
Fifty-four per cent of 52 patients presenting to the University of Malaya Medical Centre with a myelopathy for which appropriate investigations uncovered no definite etiology, subsequently developed clinically definite or probable multiple sclerosis. In the subgroup of patients with a presentation indicative of acute/subacute transverse myelopathy, 14 or 52% also went on to develop clinically definite or probable multiple sclerosis, a far higher proportion than previously recorded in the literature. This finding is probably a further manifestation of racial difference in the behaviour of multiple sclerosis. For the group as a whole, the only factor which appeared to be associated with an increased risk of developing multiple sclerosis was female sex; 67% of 33 female patients went on to develop multiple sclerosis after a mean follow-up period of 5.5 years. Other factors such as age of onset, racial composition, level of spinal cord involvement, presence of fever and CSF finding were found not to be important.
An outbreak of food poisoning resulting in 13 deaths in children occurred in Malaysia during the Chinese Festival of the Nine-Emperor Gods in 1988. The offending food was a Chinese noodle called 'Loh See Fun' (LSF). The source was traced to a factory where a banned food preservative was added to make the LSF. The food poisoning was attributable to aflatoxins and boric acid. The clinical features included vomiting, pyrexia, diarrhoea, abdominal pain, anorexia, giddiness, seizures, and eventual coma. Initially, many presented with a Reye-like syndrome. Eleven post-mortem examinations were performed. The pathological findings included extensive coagulative necrosis of the liver with proliferative 'ductal/ductular metaplasia of the hepatocytes'. Giant cell formation, central vein sclerosis, bile stasis, and steatosis were also noted. There was presence of acute tubular necrosis, superficial upper gastrointestinal erosions, and ensuing encephalopathy. The eventual cause of death is acute hepatic and renal failure.
Aerodigestive amyloidosis is a rare disorder characterized by fibrillar protein deposition in the aerodigestive tree. We present a case of a 19-year-old Chinese gentleman whose diagnosis was initially missed as he presented with features suggestive of severe bronchial asthma and was intubated and ventilated. He subsequently presented 2 years later with severe stridor and required emergency tracheostomy. Current literature is reviewed for the histopathology, common clinical features, radiological findings and treatment options for aerodigestive amyloidosis.