A young boy of 18 years was admitted at department of Neurology, Dhaka Medical College Hospital with the complaints of progressive generalized hyper-pigmentation, gradual loss of vision, hearing impairment, abnormal behaviors and one episode of seizure. Examination finding revealed, abnormal behaviors, generalized hyper pigmentation of skin, oral mucosa, gum, tongue and palmer creases. He has diffuse hair loss, bilateral primary optic atrophy, bilateral sensoryneural deafness. All routine investigations revealed normal findings except, CSF protein were elevated, biochemical features (very high ACTH, low basal cortisol) of primary adrenal failure, Magnetic resonance imaging (MRI) of the head showed bilateral symmetrical white matter abnormalities in parieto-occipital regions. The diagnosis of Adreno-leukodystrophy (ALD) was strongly suggested from the medical history, biochemical and radiological (MRI) findings of brain. The purpose of our report is to highlight this very rare nontreatable disease to all. A patient of neuropsychiatric symptoms with Addison's disease we must think about ALD, because it's progression can be delayed with early diagnosis and supportive treatments, it's incidence can be reduced by genetic counseling.Key words: Adrenoleukodystrophy (ALD); Addison's disease; Very long chain fatty acid (VLCFA). DOI: 10.3329/jbcps.v28i3.6514J Bangladesh Coll Phys Surg 2010; 28: 189-192
doi:10.3329/jafmc.v4i2.1843 JAFMC Bangladesh Vol.4(2) 2008 33-37
Background/Objective: Stroke is the fifth leading cause of death in the US and the leading cause of long-term disability for adults. Research has shown that some ethnic groups have worse health outcomes after suffering from an acute stroke. It has been shown that certain ethnic groups used EMS services less and took longer to arrive at the hospital for acute stroke care. Since time is of the essence for stroke treatment, our study may provide insight on factors that may lead to the delay in the acute stroke management. Project Methods: In this retrospective chart review, we will look at patients who suffered from an ischemic or hemorrhagic stroke. From each patient’s electronic medical record, demographic information, time from acute stroke onset to hospital stroke activation, stroke severity, usage of EMS services, duration of hospital stay, co-morbid conditions, and stroke outcome will be recorded. Two-way ANOVA is used for statistical analysis. Results: Data was not able to be collected at this point. We hypothesize that patients of some ethnic groups have longer delay of ED care initiation from the stroke onset, lesser usage of EMS services, and higher rates of co-morbid conditions. We also hypothesize that these factors are correlated with stroke outcome at the time of hospital discharge. In contrast, the distance between home and nearest hospital with stroke care expertise is not different for different ethnic groups. Conclusion and Potential Impact: The data collected in this study can further explain why some ethnic groups have worse stroke outcomes than others. If the underlying factors responsible for these differences include factors such as failure to use EMS services, steps can then be taken to provide remedy and to promote better acute stroke outcomes for people of all ethnic groups within our community.
BackgroundThe incidence of neoplasms of the appendix is low and accounts for 1% of all cancers. It is often an incidental finding, detected in 0.2 -0.3% of all appendicectomy specimens. PurposeWe studied the incidence of primary lesions of the appendix in a cohort of patients who underwent appendicectomy as part of cytoreductive surgery for gynaecological malignancies. Methods A total of 182 cases were identified from the database of the Department of Histopathology over the course of 6 years (2015-2020). The histopathology reports for these cases were reviewed, and data including patient age, primary gynaecological cancer type, macroscopic abnormality in appendix, presence of a primary lesion of the appendix and its type was collated. ResultsThe median age of the entire cohort was 54 years (range 17-83 years). An appendiceal lesion was identified in 12/182 cases (0.5%), whose median age was 61years (range 48-77 years). The appendiceal pathology included low grade appendiceal neoplasm in 4/12 (33%), well differentiated (grade 1) neuroendocrine tumour in 2/12 (17%), sessile serrated lesion with no dysplasia in 4/12 (33%) and hyperplastic polyp in 2/12 (17%) cases. There was no association with any particular gynaecological cancer type with the presence of appendiceal lesions. A macroscopic abnormality in the form of dilatation of appendix was noted in one of the 12 cases (8%). A tumour nodule was identified grossly in another case whilst metastatic tumour deposits were confirmed microscopically in 4/12 cases (33%). ConclusionAppendicectomy in the course of cytoreductive surgery provides an opportunity to identify occult primary appendiceal lesions, particularly in patients over the age of 45. Appendiceal lesions are most often an incidental finding and given the poor correlation between gross appearance of the appendix and microscopic findings in the majority of these lesions, the entire appendix should be submitted for histological assessment.
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