Extensive Angiokeratomas of the Trunk-An Unsual Presentation of Fabry Disease

2021 ◽  
Vol 7 (04) ◽  
pp. 01-02
Author(s):  
Inusha Panigrahi

A 23 year old man presented with erythematous non-pruritic rash over his trunk since childhood. Family history was non-contributory, and he did not have any major or chronic medical illness in the past. There was no history suggestive of paresthesias, exertional dyspnea or focal neurological deficit.

1995 ◽  
Vol 82 (4) ◽  
pp. 558-566 ◽  
Author(s):  
Francis H. Tomlinson ◽  
Paul J. Kurtin ◽  
Vera J. Suman ◽  
Bernd W. Scheithauer ◽  
Judith R. O'Fallon ◽  
...  

✓ The authors report on a clinicopathological study of 89 surgical patients with histologically proven primary parenchymal brain lymphoma, all diagnosed between January 1975 and December 1990. The cohort included 60 men and 29 women whose median age at diagnosis was 60 years (range 14 to 84 years). The duration of symptoms was less than 8 weeks in 48% of the patients. Symptom groups included focal neurological deficit (73%), neuropsychiatric symptoms (28%), seizures (9%), and increased intracranial pressure (3%). A total of 132 tumors were seen in 89 patients: the most common sites were frontal (32 patients), temporoparietal (31 patients), and basal ganglia (17 patients); multiple lesions were reported in 23 patients. No patient had antecedent of human immunodeficiency virus positivity or acquired immunodeficiency syndrome. A family history of cancer was present in 33% of the patients, three-quarters of whom were first-degree relatives. Histological subtypes (National Cancer Institute Working Formulation) included 64 large cell (72%) and 13 immunoblastic (15%) tumors. Phenotype was determined in 66 patients: 63 were B-cell type and three were T-cell type. Surgical resection was performed in 47% of the cases, with the remainder undergoing biopsy only. All but six patients received radiation therapy. Thirty-one patients received chemotherapy, whereas 46 patients did not; data on the remaining 12 patients were unavailable. The end point of the study was death from any cause. At the time of last contact, 69 of the patients (78%) had died; the median survival time for this study group was 20.9 months. On univariate analysis, prognostic factors significantly associated with survival included age at diagnosis, family history of cancer, and focal neurological deficit. Multivariate analysis revealed four unfavorable prognostic factors: age greater than or equal to 60 years, history of cancer in first-degree relatives, focal deficit, and ependymal contact. After adjustment for these variables, clinical syndrome, size and number of lesions, extent of surgery, histological cell type, radiation dose, and use of chemotherapy were not significantly associated with survival.


2015 ◽  
Vol 2015 ◽  
pp. 1-5
Author(s):  
Ali Zohair Nomani ◽  
Haris Majid Rajput ◽  
Mansoor Iqbal ◽  
Zakir Jan ◽  
Muhammad Irshad ◽  
...  

Subarachnoid hemorrhage (SAH) is a relatively less common but important neurological condition comprising 5% of all the cerebrovascular accidents. In most populations the reported incidence is 6-7 per 100,000 person-years and one-third of survivors become dependent. It is a serious but potentially treatable cause of neurological morbidity. Multiple authors have identified the most unusual novel associations and triggers of subarachnoid bleeds over the past decade. We herein report a rare case of subarachnoid hemorrhage leading to focal neurological deficit in a middle aged man secondary to forceful sneeze.


2006 ◽  
Vol 53 (4) ◽  
pp. 125-131
Author(s):  
I.J. Butkovic

Historically, intra-articular calcaneal fractures have been managed, as a rule, nonsurgically, although surgical treatment has more advocates in the past 20 years. Even today many high specialized orthopedic surgeons have a lot of controversies relating the indications fields of nonsurgical and operative treatment of these fractures. For getting correct decision adequate preoperative evaluation of fracture type, overall patients health and his functional needs is necessary. Generally, elderly patients with sedentary habits, without or with minimal fracture displacement, might be treated successfully with nonsurgical management. Good result after surgical treatment might be predicted in patients younger than 40 years, with a simple fracture pattern and high probability of achieving anatomic reduction. Highly precise and good surgical technique is necessary for getting a good final result with a long learning curve of the surgeon. Smoking, diabetes, chronic medical illness, peripheral vascular disease as well workers compensation claim (!) markedly increase the risk of surgical complications.


Chelovek RU ◽  
2020 ◽  
pp. 18-53
Author(s):  
Sergei Avanesov ◽  

Abstract. The article analyzes the autobiography of the famous Russian philosopher, theologian and scientist Pavel Florensky, as well as those of his texts that retain traces of memories. According to Florensky, the personal biography is based on family history and continues in children. He addresses his own biography to his children. Memories based on diary entries are designed as a memory diary, that is, as material for future memories. The past becomes actual in autobiography, turns into a kind of present. The past, from the point of view of its realization in the present, gains meaning and significance. The au-thor is active in relation to his own past, transforming it from a collection of disparate facts into a se-quence of events. A person can only see the true meaning of such events from a great distance. Therefore, the philosopher remembers not so much the circumstances of his life as the inner impressions of the en-counter with reality. The most powerful personality-forming experiences are associated with childhood. Even the moment of birth can decisively affect the character of a person and the range of his interests. The foundations of a person's worldview are laid precisely in childhood. Florensky not only writes mem-oirs about himself, but also tries to analyze the problems of time and memory. A person is immersed in time, but he is able to move into the past through memory and into the future through faith. An autobi-ography can never be written to the end because its author lives on. However, reaching the depths of life, he is able to build his path in such a way that at the end of this path he will unite with the fullness of time, with eternity.


2020 ◽  
Vol 15 (4) ◽  
pp. 420-422
Author(s):  
Dhruvkumar M. Patel ◽  
Mukundkumar V. Patel ◽  
Jayanti K. Gurumukhani ◽  
Maitri M. Patel ◽  
Himal J. Mahadevia ◽  
...  

Background: Hypoglycemia may rarely present as hemiparesis and sometimes it is difficult to differentiate from ischemic stroke. When random blood sugar (RBS) value is between 50 and 80 mg % in patients presenting with focal neurological deficit, no guideline exists to consider the possibility of hypoglycemia before initiating thrombolytic therapy. Clinical Case: A 58-year-old male, who was a known case of diabetes and hypertension, was brought to the emergency room with acute onset of right hemiparesis and dysarthria of 90 minutes duration. His NIHSS Score was 9, blood pressure was 150/90 mm of Hg and RBS was 79 mg% on admission. His CT scan brain was normal and was considered for thrombolysis. Resident doctor not aware of previous sugar repeated RBS before thrombolysis which was surprisingly 60 mg% 60 minutes after the first RBS. Even though he was a candidate for thrombolysis, intravenous 25 % dextrose was administered considering the possibility of hypoglycemia. He made a complete recovery within 20 minutes and thrombolytic therapy was withheld. : In Diabetic patients with focal neurological deficit and RBS less than 80 mg% on admission, RBS should be rechecked and in appropriate cases should be challenged with IV dextrose considering the possibility of hypoglycemia before commencing thrombolytic therapy.


2021 ◽  
Vol 9 ◽  
pp. 205031212110343
Author(s):  
Bedru Jemal ◽  
Zemedu Aweke ◽  
Simeneh Mola ◽  
Sileshi Hailu ◽  
Sileshi Abiy ◽  
...  

Background: An emerging respiratory disease abbreviated as coronavirus disease 2019 was first reported in December 2019 in Wuhan city of China. The virus is zoonotic and tends to be transmitted between animals to humans and humans to humans. The major route of transmission of coronavirus disease 2019 is droplet and close contact. The Ethiopian Ministry of Health has initiated training for health care workers at a different level. Thus, the main objective of this study is to assess the knowledge, attitudes, and practices of health workers in Ethiopia toward coronavirus disease 2019 and its prevention techniques. Method: An institution-based multicenter cross-sectional study was conducted in each of eight teaching and referral hospitals. A total of 422 Ethiopian healthcare workers were selected for the assessment of knowledge, attitude, and practice toward coronavirus disease 2019. Data were collected using a structured questionnaire. A logistic regression model was used to identify factors associated with the attitude and knowledge of healthcare workers toward coronavirus disease 2019 at a significance level of p < 0.05. Result: Three hundred ninety-seven healthcare workers participated in the study, with a response rate of 94%. Among these, 88.2% and 94.7% of respondents had good knowledge and positive attitudes, respectively. A respondent with a history of chronic medical illness (odds ratio: 0.193, 95% confidence interval: 0.063–0.593), social media, telecommunication, and television/radio as a source of information were significantly associated with knowledge (odds ratio: 3.4, 95% confidence interval: 1.5–7.4, OR: 4.3, 95% confidence interval: 1.3–14.3 and odds ratio: 3.2, 95% confidence interval: 1.4–7.2). In addition, respondents with a history of chronic medical illness were significantly associated with a negative attitude toward coronavirus disease 2019. Conclusion: The knowledge and attitude were good while; the practice was relatively low. Sources of information such as social media, telecommunication, and television/radio were positively associated with healthcare workers' knowledge about coronavirus disease 2019.


2010 ◽  
Vol 30 (6) ◽  
pp. 1055-1072 ◽  
Author(s):  
SHANNON MCDERMOTT

ABSTRACTOver the past 50 years, self-neglect among older people has been conceptualised in both social policy and the academy as a social problem which is defined in relation to medical illness and requires professional intervention. Few authors, however, have analysed the concept of self-neglect in relation to critical sociological theory. This is problematic because professional judgements, which provide the impetus for intervention, are inherently influenced by the social and cultural context. The purpose of this article is to use critical theory as a framework for interpreting the findings from a qualitative study which explored judgements in relation to older people in situations of self-neglect made by professionals. Two types of data were collected. There were 125 hours of observations at meetings and home assessments conducted by professionals associated with the Community Options Programme in Sydney, Australia, and 18 professionals who worked with self-neglecting older people in the community gave in-depth qualitative interviews. The findings show that professional judgements of self-neglect focus on risk and capacity, and that these perceptions influence when and how interventions occur. The assumptions upon which professional judgements are based are then further analysed in relation to critical theory.


Medical Care ◽  
2004 ◽  
Vol 42 (6) ◽  
pp. 512-521 ◽  
Author(s):  
Seth Himelhoch ◽  
Wendy E. Weller ◽  
Albert W. Wu ◽  
Gerard F. Anderson ◽  
Lisa A. Cooper

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