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  1. Ng, B.K., Lim, P.S., Ng, Y.L., Kew, T.Y., Abdul Kadir, A.K., Hatta, M.
    MyJurnal
    Primary malignant melanoma of the vagina is rare but aggressive. Various treatment options include surgery and adjuvant therapy has been advocated but the outcome remained unpredictable. Standard treatment protocol is yet to be established. We report a case of 54-year-old, Para 4+1, with malignant melanoma of the vagina. She underwent wide local excision but the surgical margin was not clear of malignant cells, hence adjuvant radiotherapy was given. Combination chemotherapy was initiated subsequently as her disease disseminated. She succumbed later due to septicaemic shock. The treatment options for vaginal melanoma were reviewed.
    Matched MeSH terms: Glycogen Storage Disease Type VI
  2. Toe BP, Ramli N, Lam SY, Wong KT, Prepageran N
    Ear Nose Throat J, 2015 Feb;94(2):E27-32.
    PMID: 25651356
    Basaloid squamous cell carcinoma (BSCC) is a rare subtype of squamous cell carcinoma. To date, only 95 cases of sinonasal BSCC have been reported in the English-language literature, and they account for 5% of all cases of head and neck BSCC. We describe what we believe is only the second reported case of a sinonasal tract BSCC that metastasized to the liver. The patient was a 36-year-old woman who presented with right-sided nasal obstruction and a foul-smelling discharge. Clinical examination and imaging identified a large, lobulated, enhancing mass in the right nasal cavity. Following excision of the mass, the patient was scheduled for radiotherapy. However, before it could be administered, follow-up imaging detected a metastasis to the liver and lung, and the patient was switched to chemotherapy. Initially, she responded well clinically, but at 5 months postoperatively, a follow-up CT showed an increasing metastatic presence in the liver and bone. The patient died of her disease 1 year after surgery.
    Matched MeSH terms: Glycogen Storage Disease Type VI
  3. Chan KH, Lee CH, Sharif SZ, Hayati F, Sallapan S
    Ann Med Surg (Lond), 2020 Dec;60:438-441.
    PMID: 33251002 DOI: 10.1016/j.amsu.2020.11.035
    Background: Metastatic neuroendocrine tumours (NETs) to the breast are very rare entities.

    Case presentation: A 26-year-old lady presented with anterior neck swelling with symptoms of superior vena cava syndrome for 6 months. Imaging study revealed a mediastinal mass which was preceded with core biopsy which was consistent with high-grade small cell NETs. Despite second-line adjuvant chemotherapy and radiotherapy, her disease became advanced which was confirmed via restaging scan. There were bilateral breast lesions discovered during the scan which was deemed to be metastatic NETs histologically. Despite prompt initiation of treatment, she succumbed 1 year after the radiotherapy due to disease progression.

    Conclusion: High suspicion of an index is needed for diagnosis when patients with known primary NETs present with suspicious breast lesions. Triple assessment is mandatory, however histopathology assessment and immunohistochemistry staining are the mainstay of diagnosis.

    Matched MeSH terms: Glycogen Storage Disease Type VI
  4. Wong L, Kanthasamy SV, Durairaj G, Thangaratnam RR
    Int J Surg Case Rep, 2020;77:48-52.
    PMID: 33137672 DOI: 10.1016/j.ijscr.2020.10.091
    INTRODUCTION: Intussusception usually occurs in the paediatric population. When it occurs in the adult population, it is normally caused by a malignant intraluminal pathology.

    PRESENTATION OF CASE: A 72-year-old female presented to us with right-sided abdominal pain for 3 weeks, associated with vomiting and diarrhoea. She had an appendectomy done 30 years ago and a recent myocardial infarction. Abdominal examination revealed a previous appendectomy scar and tenderness over the right lumbar region. Computed tomography showed ileocaecal intussusception. Right hemicolectomy with a double barrel stoma was performed as she was unstable intraoperatively. Histopathological examination of the tumour showed a well-differentiated neuroendocrine tumour. Subsequent PET scan showed no systemic disease and a reversal of the stoma was done. She remained disease free for a year.

    DISCUSSION: Our patient had undergone a right hemicolectomy despite the high risk of mortality, as there is a high chance of malignancy. Double barrel stoma was done, as she was unstable intraoperatively. Fortunately, she recovered well and had her stoma reversed without any further recurrence of her disease.

    CONCLUSION: Adult patients who present with intussusception should be managed with resection, as there is a high possibility of a malignancy. Early resection should be planned to prevent further spread of the tumour.

    Matched MeSH terms: Glycogen Storage Disease Type VI
  5. Aung YN, Nur AM, Ismail A, Aljunid SM
    Clinicoecon Outcomes Res, 2019;11:505-513.
    PMID: 31447570 DOI: 10.2147/CEOR.S209108
    Purpose: Care at ICUs is expensive and variable depending on the type of care that the patients received. Knowing the characteristics of the patient and his or her disease is always useful for improving health services and cost containment.

    Patients and methods: An observational study was conducted at four different intensive care units of an academic medical institution. Demographic characteristics, disease-management casemix information, cost and outcome of the high costing decile, and the rest of the cases were compared.

    Results: A total of 3,220 discharges were included in the study. The high-cost group contributed 35.4% of the ICU stays and 38.8% of the total ICU expenditure. Diseases of the central nervous system had higher odds to be in the top decile of costly patients whereas the cardiovascular system was more likely to be in the non-high cost category. The high-cost patients were more likely to have death as an outcome (19.2% vs 9.3%; p<0.001). The most common conditions that were in the high-cost groups were craniotomy, other ear, nose, mouth, and throat operations, simple respiratory system operations, complex intestinal operations, and septicemia. These five diagnostic groups made up 43% of the high-cost decile.

    Conclusion: High-cost patients utilized almost 40% of the ICU cost although they were only 10% of the ICU patients. The chances of admission to the ICU increased with older age and severity level of the disease. Central nervous system diseases were the major problem of patients aged 46-69 years old. In addition to cost reduction strategies at the treatment level, detailed analysis of these cases was needed to explore and identify pre-event stage prevention strategies.

    Matched MeSH terms: Glycogen Storage Disease Type VI
  6. Sabariah, M. N., Zainina, S., Faridah, I., Leong, C. F.
    MyJurnal
    Clonal disorders of LGL may either be CD3+ CD56- or CD3- CD56+ phenotype and these have been designated as T-cell leukaemia (T-LGL) or natural killer cell (NK)-LGL leukaemia respectively. Clonality is usually demonstrated by clonal rearrangement of T-cell receptor gene rearrangement or identified by flowcytometry analysis. Most patients with T-LGL will have an indolent course. In this report we described an aggressiveness of disease in a patient with clonal CD3+ LGL leukaemia whose cells also co-expressed CD56 diagnosed by flowcytometry. The patient responded well to interrupt ALL standard risk protocol however succumbed to her disease while waiting for upfront stem cell transplant. This case highlights on both the classical laboratory findings of rare entity of disease as well as a review of the literature pertaining particularly on its management.
    Matched MeSH terms: Glycogen Storage Disease Type VI
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