Disappearing bone disease is a rare condition and usually affects young adults. Its aetiology is not known. A case of a 12-year-old female child, who had a fall and sustained a fracture mid shaft of femur and supracondylar region, is reported. On follow-up subsequent X-ray showed extensive osteolysis which was progressively affecting the other side of the pelvic girdle and femur. She was put on trial of calcitonin 50 IU by nasal spray for six months but there was no improvement. However, during the past three and half years the disease process had progressively extended to the other side of pelvic girdle and femur with fatal outcome. To the knowledge of the authors such progression in a short time has not been reported in any case so far.
Dengue fever (DF) which is caused by four serotypes of dengue virus may in some cases progress into a life threatening situation of dengue haemorrhage fever (DHF) and dengue shock syndrome (DSS). It has been suggested that sequential infection with different dengue virus serotypes predisposes the patient towards DHF/DSS. We report here a primary dengue infection in a 10-year-old boy progressing from DF to DSS while under clinical observation. The report provides unequivocal evidence for the development of DSS in primary dengue infection caused by virus serotype 4. The close relationship between sequential changes in the levels of tumour necrosis factor (TNF), Interleukin 1 and 6 (IL-1 and IL-6) in the serum, to the clinical progression of the disease from DF to DHF/DSS and then to full recovery implicates a pathogenetic role for the inflammatory cytokines. The child also manifested clinical features consistent with Reye's syndrome and this suggests a common pathogenetic origin for DSS and the Reye-like syndrome induced by dengue virus.
In a study of 1,002 consecutive Malaysian male newborns, 48 (4.8%) were found to have undescended testes (UDT). The rate and laterality of the UDT were associated with lower birth weight (P < 0.001) and prematurity (P < 0.001). Boys with UDT were also more likely to have other congenital abnormalities of the external genitalia, the commonest being hydrocele. No correlation between UDT and maternal age, birth order, social class, or mode of delivery was demonstrated in this study. Although 26/34 (76.5%) of UDT achieved full spontaneous descent by 1 year of age, 1.1% of all infants whose testes remained undescended required regular long-term follow-up with surgical referral and correction at an appropriate time. A premature infant with UDT is more likely to achieve full testicular descent at 1 year of age than a term infant.
Study site: University Hospital, Kuala Lumpur, Malaysia (University Malaya Medical Centre)
Typhoid fever, which is endemic in Malaysia, affects all age groups and it has been stated that classical features described in textbooks were absent in children. The aim of this study was to find out whether this was true in the local setting and hence a retrospective study was undertaken.
Expression of c-erbB3 protein was investigated in 104 primary breast carcinomas comprising nine comedo ductal carcinoma in situ (DCIS), 91 invasive ductal carcinomas and four invasive lobular carcinomas using two monoclonal antibodies, RTJ1 and RTJ2. Of the 91 invasive ductal carcinomas, seven contained the comedo DCIS component adjacent to the invasive component. An immunohistochemical technique was used to evaluate the association between expression of c-erbB3 and clinical parameters and tumour markers such as epidermal growth factor receptor (EGFR), c-erbB2, cathepsin-D and p53 in archival formalin-fixed paraffin-embedded tumour tissues. Our results indicated that RTJ1 and RTJ2 gave identical staining patterns and concordant results. It was found that the overexpression of c-erbB3 protein was observed in 67% (6/9) of comedo DCIS, 52% (44/84) of invasive ductal carcinomas, 71% (5/7) of carcinomas containing both the in situ and invasive lesions and 25% (1/4) of invasive lobular carcinomas. A significant relationship (P < 0.05) was observed between strong immunoreactivity of c-erbB3 protein and histological grade, EGFR and cathepsin-D, but not with expression of c-erbB2, p53, oestrogen receptor status, lymph node metastases or age of patient. However, we noted that a high percentage of oestrogen receptor-negative tumours (59%), lymph node-positive tumours (63%) and c-erbB2 (63%) were strongly positive for c-erbB3 protein. We have also documented that a high percentage of EGFR (67%), c-erbB2 (67%), p53 (75%) and cathepsin-D-positive DCIS (60%) were strongly positive for c-erbB3. These observations suggest that overexpression of c-erbB3 protein could play an important role in tumour progression from non-invasive to invasive and, also, that it may have the potential to be used as a marker for poor prognosis of breast cancer.
Septal perforation from intranasal cocaine abuse is well recognised. We present a case of progressive septal as well as palatal perforation. Progression from septal perforation to palatal perforation occurred after cessation of intranasal cocaine abuse. This patient had a weakly positive cytoplasmic antineutrophilic cytoplasmic antibody (C-ANCA) but no histologic evidence of Wegener's Granulomatosis. The differential diagnosis for septal and palatal perforation is reviewed. This case represents the fifth reported case of palatal perforation secondary to cocaine abuse in the literature, and the second associated with positive C-ANCA.
A retrospective analysis of 182 HIV positive Malaysians was done in two centers, the University Hospital Kuala Lumpur (UHKL) and the General Hospital Kuala Lumpur (GHKL) from March 1997 to February 1998. Demographic and clinical data were analyzed. The analysis showed that 130 out of 182 patients had mucocutaneous disorders (71.4%). In the study there were 125 males (96.2%) and 5 females (3.8%). The majority of the patients were in the age group from 20 to 50 years. The patients who presented with mucocutaneous disease also had low CD4+ T lymphocyte counts, and most of them had AIDS defining illnesses. The number of cases with generalized hyperpigmentation was very high (35.7%), followed by papular eruptions (29.1%) and xerosis (27.5%). Seborrheic dermatitis was seen in 19.2% of the cases and psoriasis in 7.7%. The most common infections were oral candida 35.7%, tinea corporis and onychomycosis 9.9%, and herpes infection 4.3%. However, mucocutaneous manifestations of Kaposi's sarcoma were rare. The results suggested that mucocutaneous findings are useful clinical predictors of HIV infection or a sign of the presence of advanced HIV infection.
From April through June 1997, 29 previously healthy children aged <6 years (median, 1.5 years) in Sarawak, Malaysia, died of rapidly progressive cardiorespiratory failure during an outbreak of hand, foot, and mouth disease caused primarily by enterovirus 71 (EV71). The case children were hospitalized after a short illness (median duration, 2 days) that usually included fever (in 100% of case children), oral ulcers (66%), and extremity rashes (62%). The illness rapidly progressed to include seizures (28%), flaccid limb weakness (17%), or cardiopulmonary symptoms (of 24 children, 17 had chest radiographs showing pulmonary edema, and 24 had echocardiograms showing left ventricular dysfunction), resulting in cardiopulmonary arrest soon after hospitalization (median time, 9 h). Cardiac tissue from 10 patients showed normal myocardium, but central nervous system tissue from 5 patients showed inflammatory changes. Brain-stem specimens from 2 patients were available, and both specimens showed extensive neuronal degeneration, inflammation, and necrosis, suggesting that a central nervous system infection was responsible for the disease, with the cardiopulmonary dysfunction being neurogenic in origin. EV71 and possibly an adenovirus, other enteroviruses, or unknown cofactors are likely responsible for this rapidly fatal disease.
'Amyotrophic dermatomyositis' (ADM) is used to describe a small subgroup of patients with the typical skin rash associated with dermatomyositis but without muscle involvement. Lung involvement in ADM is rare. We report on the management of a patient with ADM associated with pulmonary fibrosis at presentation, and her response to corticosteroid treatment.
Age has been suggested to modify systemic lupus erythematosus expression. In this study we have attempted to study 13 patients with late onset (40 years and above) and 90 with early onset disease (below 40 years) to determine whether age-related differences in disease expression exist and whether the genetic make-up influences the age of disease onset. We found that patients with late onset disease initially presented with pericarditis (31% vs 3%, P<0.005) and a lower incidence of malar rash (31% vs 57%, p<0.05). During the disease course, there was a lower incidence of mucocutaneous symptoms especially malar rash (p<0.005) and psychosis (p<0.05) in the late onset group. Serological parameters were similar in both groups. There was a prevalence of HLA-DQA1*0103 in Chinese patients with late onset disease (pcorr=0.004). These findings suggest that a subgroup of late onset patients may experience milder disease and that the risk conferred by the HLA-DQA1*0103 may be significant among these patients.
Acute epiglotiitis is a disease of rapid onset and progression of symptoms and has been well described in children. The importance of being aware of this diagnosis is to prevent an acute upper airway obstruction that can be potentially fatal. Here we describe two cases of adult acute epiglottitis where the patients had severe symptoms of sorethroat but a normal looking oropharynx.
The effect of myopic defocus on myopia progression was assessed in a two-year prospective study on 94 myopes aged 9-14 years, randomly allocated to an undercorrected group or a fully corrected control group. The 47 experimental subjects were blurred by approximately +0.75 D (blurring VA to 6/12), while the controls were fully corrected. Undercorrection produced more rapid myopia progression and axial elongation (ANOVA, F(1,374)=14.32, p<0.01). Contrary to animal studies, myopic defocus speeds up myopia development in already myopic humans. Myopia could be caused by a failure to detect the direction of defocus rather than by a mechanism exhibiting a zero-point error.
The Nipah virus is a newly identified paramyxovirus responsible for an outbreak of fatal encephalitis in Malaysia and Singapore. This paper reports the follow up clinical and magnetic resonance imaging findings in 22 affected subjects. Of 13 patients with encephalitis, one died, one was lost to follow up, and seven recovered. Among the four remaining patients, one had residual sixth nerve palsy, another suffered from severe clinical depression, and a third patient had evidence of retinal artery occlusion. One patient with delayed onset Horner syndrome had a single lesion in the cervical spinal cord. The brain magnetic resonance findings were stable or improved in nine patients over 18 months of follow up. Among a second group of nine asymptomatic seropositive abattoir workers, magnetic resonance examination in seven subjects revealed discrete small lesions in the brain; similar to those detected in encephalitis patients. These findings suggest that in addition to encephalitis, the newly discovered Nipah virus affects the spinal cord and the retina. Late clinical and radiological findings can occur in Nipah virus infections as with other paramyxoviruses.
Paediatric minor head injuries (MHI) are just as common in both bigger and smaller towns in Malaysia. Urban-based MHI are due more to motor vehicular injuries compared to rural-based MHI which are mainly due to non-motor vehicular injuries. The main objectives of this study were to compare incidence of admitted patients to accident and emergency departments of hospitals in two different settings in Malaysia, namely: Ipoh (urban-based) and Kota Bharu (rural-based); and to correlate to demographical characteristics, types of accident, clinical signs and symptoms, radiological and computed tomography (CT) findings, management; and finally, to determine clinical predictors of intracranial injury in MHI.
Diabetic nephropathy is now the number one cause of end stage renal failure in Malaysia. This places a huge burden on patients and the health care system especially in developing countries with limited health care resources, such as in Sarawak in East Malaysia. This study describes the prevalence of proteinuria/microalbuminuria in diabetic patients treated in Klinik Kesihatan Tanah Puteh. Early detection of proteinuria/microalbuminuria allows remedial measures to be taken to retard the progression of nephropathy. Forty-eight percent of the cases had proteinuria and microalbuminuria was found in 16%. Seventy-eight percent of cases with proteinuria were on treatment with angiotensin-converting-enzyme inhibitors or angiotensin-receptor blockers. Seventy-five percent of patients had hypertension but only 6% achieved the targeted BP of < 130/80 mmHg.
Study site: Diabetic clinic of Klinik Kesihatan Tanah Puteh, Kuching, Sarawak, Malaysia
In the Asia Pacific region Human Immunodeficiency virus (HIV) is often acquired in individuals already infected with hepatitis B virus (HBV). The immune suppression caused by HIV infection reduces cellular immune response against HBV and liver inflammation may improve, but the risk of developing cirrhosis is not. HBV infection does not affect the progression of HIV disease. Anti-retroviral agents may be directly hepatotoxic and cause ALT elevations in patients with chronic hepatitis. Highly active anti-retroviral therapy (HAART) improves immunity and as cytotoxic lymphocyte responses improve, hepatitis flares can occur, usually r within 3 months of initiation of HAART. These hepatitis flares may be followed by normalization of ALT and clearance of HBVDNA. If lamivudine is included in the HAART regime, hepatitis flares may not occur till late and these late flares signal the development of lamivudine resistant HBV strains (90% of HBV/HIV co-infection). Treatment options for chronic HBV infection include interferon (IFN), and nucleoside analogues. Lamivudine, adefovir dipivoxil, tenofovir disoproxil fumarate (DF) are nucleoside analogues with activity against both HBVDNA polymerase and HIV reverse transcriptase. The latter two compounds have added activity against lamivudine resistant HBVDNA. Lamivudine should be avoided in the initial treatment of both hepatitis B as well as HIV because of the high incidence of resistance. Interferon should be considered first for treatment of HBV in HIV co-infected individuals and is usually unsuccessful in the later stages of HIV infection when immune suppression is extreme. As new and improved agents in HAART continue to prolong survival, the use of liver transplantation for cirrhotic patients co-infected with HIV and HBV may increase.