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  1. Looi LM
    Histopathology, 1991 Feb;18(2):133-41.
    PMID: 1901294
    Congo red screening of 27,052 routine biopsy specimens from 22,827 patients over a 5 1/2-year period in the Department of Pathology, University of Malaya detected 186 cases of amyloidosis. The categories of amyloidosis encountered and their prevalences in relation to each other were: systemic AL (5.9%); systemic AA (3.2%); isolated atrial (14%); primary localized cutaneous (7.5%); other primary localized deposits (3.2%); localized intratumour (58%); and dystrophic (8.6%). A third of patients with systemic AL amyloidosis had coexistent immunocyte abnormality. The commonest underlying pathology for systemic AA amyloidosis was leprosy. Notable among the types of localized amyloidosis revealed by this study were isolated atrial amyloidosis, which appeared to complicate chronic rheumatic heart disease, and intratumour amyloidosis complicating nasopharyngeal carcinoma. Other tumours in which amyloid deposits were observed included basal cell carcinoma, islet cell tumour and medullary carcinoma of the thyroid. Dystrophic amyloidosis was observed in fibrotic tissues, such as damaged cardiac valves and osteoarthritic joints. Heredofamilial amyloidosis, senile systemic amyloidosis and degenerative cerebral amyloidosis were notably absent from this study.
    Matched MeSH terms: Amyloidosis/pathology*; Cardiomyopathies/pathology; Neoplasms/pathology; Skin Diseases/pathology
  2. Borucinska JD, Caira JN
    J Fish Dis, 2006 Jul;29(7):395-407.
    PMID: 16866923
    Lesions associated with two species of tapeworms within the digestive tract of wild-caught specimens of the bull shark, Carcharhinus leucas, and the sicklefin weasel shark, Hemigaleus microstoma, from Malaysian Borneo are described. Portions of the glandular stomach and pyloric gut with parasites were removed and fixed in 10% formalin buffered in sea water. Whole mounts, histological sections of tissues with and without worms in situ, and scanning electron microscopy images of detached worms were examined. Both species of cestodes belonged to the trypanorhynch family Tentaculariidae. Heteronybelinia estigmena was found in large numbers parasitizing the pyloric gut of C. leucas; an unidentified tentaculariid was found in relatively small numbers in both the glandular stomach and pyloric gut of H. microstoma. Both species burrowed their scoleces deeply in the mucosa and attached via hooked tentacles and unciniform microtriches of the scolex. The lesions induced by the parasites were marked in both sharks and ranged from acute necrotizing to chronic granulomatous gastroenteritis. Regenerative hyperplasia and intestinal metaplasia of gastric epithelium were also present. The severity and character of pathology was causally linked to the intensity of infection, the attachment mode of the parasites, and to the anatomophysiological relationships within the gut of the host shark.
    Matched MeSH terms: Cestode Infections/pathology; Fish Diseases/pathology; Stomach/pathology; Gastrointestinal Tract/pathology
  3. Low DE, Tang MM, Surana U, Lee JY, Pramano ZAD, Leong KF
    Int J Dermatol, 2019 Oct;58(10):e190-e193.
    PMID: 31192449 DOI: 10.1111/ijd.14518
    Matched MeSH terms: Contracture/pathology; Hearing Loss, Sensorineural/pathology; Skin/pathology; Histiocytosis/pathology
  4. Han HS, Toh PY, Yoong HB, Loh HM, Tan LL, Ng YY
    Vet Dermatol, 2018 Oct;29(5):442-e148.
    PMID: 30066413 DOI: 10.1111/vde.12672
    BACKGROUND: Cutaneous screw-worm myiasis in companion animals either due to the New World screw-worm, Cochliomyia hominivorax or the Old World screw-worm, Chrysomya bezziana, has been reported particularly in tropical and subtropical regions of the world. Although treatment of screw-worm myiasis in dogs has been described, few studies have been conducted regarding its clinical and epidemiological aspects in companion animals.

    OBJECTIVES: To describe clinical and epidemiological aspects of canine and feline screw-worm myiasis.

    ANIMALS: Naturally infested dogs and cats, presented to five veterinary clinics in four Malaysian states from September 2017 to February 2018.

    METHODS AND MATERIALS: Cutaneous screw-worm myiasis was diagnosed based on clinical signs and visual examination of burrowing larvae within lesion. Age, breed, gender, anatomical site of infestation and suspected underlying predisposing causes were investigated.

    RESULTS: A total of 55 dogs and 21 cats were included in the study. Intact male mixed breed dogs (mean age 58 months) and intact male domestic short hair cats (mean age 24 months) with suspected fight-related wounds were most commonly presented with exudative and ulcerative lesions associated with screw-worm myiasis. The most common anatomical sites of infestation in the dogs were the external ear canals, followed by the perineum and medial canthus. For the cats, the most commonly affected areas were paws and tail. Five cats with screw-worm myiasis were concurrently infected with sporotrichosis.

    CONCLUSION AND CLINICAL RELEVANCE: Aggression between unneutered animals is a likely underlying cause for cutaneous screw-worm myiasis in both cats and dogs. Sporotrichosis was also a potential predisposing cause for screw-worm myiasis in cats.

    Matched MeSH terms: Cat Diseases/pathology; Dog Diseases/pathology; Screw Worm Infection/pathology; Skin/pathology
  5. Zainol Abidin Z, Mohd Zaki F, Kew TY, Goh BS, Abdullah A
    J Laryngol Otol, 2020 Jul;134(7):603-609.
    PMID: 32713375 DOI: 10.1017/S0022215120001334
    OBJECTIVE: This study aimed to evaluate the association between cochlear nerve canal dimensions and semicircular canal abnormalities and to determine the distribution of bony labyrinth anomalies in patients with cochlear nerve canal stenosis.

    METHOD: This was a retrospective study in which high-resolution computed tomography images of paediatric patients with severe-to-profound sensorineural hearing loss were reviewed. A cochlear nerve canal diameter of 1.5 mm or less in the axial plane was classified as stenotic. Semicircular canals and other bony labyrinth morphology and abnormality were evaluated.

    RESULTS: Cochlear nerve canal stenosis was detected in 65 out of 265 ears (24 per cent). Of the 65 ears, 17 ears had abnormal semicircular canals (26 per cent). Significant correlation was demonstrated between cochlear nerve canal stenosis and semicircular canal abnormalities (p < 0.01). Incomplete partition type II was the most common accompanying abnormality of cochlear nerve canal stenosis (15 out of 65, 23 per cent).

    CONCLUSION: Cochlear nerve canal stenosis is statistically associated with semicircular canal abnormalities. Whenever a cochlear nerve canal stenosis is present in a patient with sensorineural hearing loss, the semicircular canal should be scrutinised for presence of abnormalities.

    Matched MeSH terms: Vestibulocochlear Nerve Diseases/pathology; Cochlear Nerve/pathology; Hearing Loss, Sensorineural/pathology; Semicircular Canals/pathology
  6. Ngoh CLY, Goh GHS, Wong WK
    Med J Malaysia, 2019 02;74(1):97-98.
    PMID: 30846674
    Thymoma is a rare mediastinal tumour that can be accompanied by different paraneoplastic syndromes. Here we report a case of Type A thymoma associated with relapsing minimal change disease (MCD). This case highlights: (1) The need to balance rapid prednisolone weaning against risk for relapse in an elderly patient at risk for steroid-induced complications. (2) The addition of calcineurin inhibitor in relapsed thymoma-related MCD, to achieve steroid sparing effects. Resection of the offending tumour and prompt immunosuppressive therapy are critical in getting best renal and overall outcomes in this rare entity.
    Matched MeSH terms: Nephrosis, Lipoid/pathology; Paraneoplastic Syndromes/pathology; Thymoma/pathology; Thymus Neoplasms/pathology
  7. Iyngkaran N, Yadav M, Boey CG
    Arch Dis Child, 1989 Sep;64(9):1256-60.
    PMID: 2817945
    Eleven infants who were suspected clinically of having cows' milk protein sensitive enteropathy were fed with a protein hydrolysate formula for six to eight weeks, after which they had jejunal and rectal biopsies taken before and 24 hours after challenge with cows' milk protein. When challenged six infants (group 1) developed clinical symptoms and five did not (group 2). In group 1 the lesions developed in both the jejunal mucosa (four infants at 24 hours and one at three days), and the rectal mucosa, and the injury was associated with depletion of alkaline phosphatase activity. Infants in group 2 were normal. It seems that rectal injury that develops as a direct consequence of oral challenge with the protein in reactive infants may be used as one of the measurements to confirm the diagnosis of cows' milk protein sensitive enteropathy. Moreover, ingestion of such food proteins may injure the distal colonic mucosa without affecting the proximal small gut in some infants.
    Matched MeSH terms: Food Hypersensitivity/pathology*; Intestinal Mucosa/pathology*; Jejunum/pathology; Rectum/pathology*
  8. Leong CF, Raudhawati O, Cheong SK, Sivagengei K, Noor Hamidah H
    Pathology, 2003 Oct;35(5):422-7.
    PMID: 14555387
    AIMS: Epithelial membrane antigen (EMA) or MUC1 belongs to a heterogeneous group of heavily glycosylated proteins and is expressed in most normal and epithelial neoplastic cells. EMA is also expressed in plasma cells, anaplastic large cell lymphoma (Ki-1 antigen), malignant histiocytosis and erythroleukaemia. In 1996, Cheong et al. (Hematology 1996; 1: 223) demonstrated the positive expression of EMA in monoblasts. Since there were very few useful markers for differentiating subtypes of acute myeloid leukaemia with a monocytic component from the those without, a study was conducted to evaluate the prevalence of EMA expression and its relationship with known markers for monocytic-macrophage lineage (CD11c, CD14 and intracellular CD68) in monocytes and monoblasts.

    METHODS: EMA detection was performed by flow cytometry in monocytes and monoblasts. EMA expression was compared with other known markers of monocytic-macrophage lineage (CD11c, CD14 and intracellular CD68). Samples of purified monocytes were obtained from 20 healthy volunteers. Twenty-two cases of monocytic AML (M4 and M5) were studied and controls were selected from 20 cases of acute lymphoblastic leukaemia (ALL) and 18 cases of non-monocytic AML (M0, M1, M2, M3, and M7).

    RESULTS: EMA was shown to be expressed strongly on the surface of all purified monocytes. EMA expression was observed on blast cells in 18/22 (81.8%) cases of AML M4 and M5, but not in that of non-monocytic AML or ALL. In this study EMA monoclonal antibody has demonstrated a strong association (P<0.001) with all the other known markers of monocytic-macrophage lineage in acute leukaemia subtypes. EMA had also shown 100% specificity and 81.8% sensitivity in the diagnosis of AML M4 and M5.

    CONCLUSIONS: The monoclonal antibody EMA (clone E29) is a useful marker in the classification of acute myeloid leukaemia and can be used as a supplementary analysis for the diagnosis of acute leukemia with monocytic involvement.

    Matched MeSH terms: Bone Marrow Cells/pathology; Leukemia, Monocytic, Acute/pathology; Monocytes/pathology; Stem Cells/pathology
  9. Iyngkaran N, Yadav M, Boey CG, Lam KL
    J Pediatr Gastroenterol Nutr, 1988 Sep-Oct;7(5):667-74.
    PMID: 3183870
    A series of 31 infants, 28 with cow's milk protein sensitive enteropathy (CMPSE) and 3 controls, was studied for severity and extent of mucosal damage of the upper small bowel in relation to the development of clinical symptoms. Following challenge with the offending cow's milk, 18 infants (Group 1) developed severe mucosal changes at both the proximal and distal small bowel mucosa and all of these infants presented with clinical symptoms. The other 10 infants (Group 2) who did not develop clinical symptoms following the challenge had less severe damage to the distal small bowel mucosa as compared to the proximal region. The histological score of both the proximal and distal postchallenge biopsies were significantly lower in Group 2 as compared to Group 1 infants. The mucosal disaccharidase and alkaline phosphatase levels were depleted in both the proximal and distal biopsies following challenge but the depletion was greater in the proximal than the distal biopsies. It is suggested that the extent and severity of mucosal damage to the proximal duodenum and jejunum have a critical bearing on the development of clinical symptoms.
    Matched MeSH terms: Diarrhea, Infantile/pathology; Food Hypersensitivity/pathology*; Intestinal Mucosa/pathology*; Jejunum/pathology
  10. Ng CY, Hayati F, Nadarajan C
    BMJ Case Rep, 2020 Sep 09;13(9).
    PMID: 32912885 DOI: 10.1136/bcr-2020-235174
    Malignant melanoma is cancer of the skin which commonly metastasises to the stomach. There have been no reported cases of emphysematous gastritis secondary to metastasis of malignant melanomas, to date. However, a 61-year-old woman with metastatic malignant melanoma of the left great toe presented to us with symptoms of severe left hypochondrium pain associated with high-grade fever, gross abdominal distension and recurrent vomiting. Two months earlier, metastasis was observed to have spread to the stomach and inguinal lymph nodes. At this stage, the patient opted for traditional medication instead of definitive surgery and chemotherapy. Radiological imaging revealed an emphysematous change to the stomach which was radiologically consistent with gastric malignant melanoma. Unfortunately, the patient succumbed to this rare condition.
    Matched MeSH terms: Gastritis/physiopathology; Lymphatic Metastasis/pathology; Melanoma/pathology*; Skin Neoplasms/pathology*; Stomach/pathology*
  11. Hilmi I, Tan YM, Goh KL
    World J Gastroenterol, 2006 Mar 07;12(9):1435-8.
    PMID: 16552816
    AIM: To determine the demography and clinical presentation of CD and secondly to determine any differences in the prevalence between the different ethnic groups in a multiracial Asian population.

    METHODS: Patients with CD who were seen in 2001-2003 in the University of Malaya Medical Centre (UMMC) were enrolled in this study. Prevalence of disease was calculated for the group as a whole and by race with hospital admissions per ethnic group as the denominator.

    RESULTS: Thirty-four patients were diagnosed to have CD. Basic demographic data of patients; male:female 17:17; mean age 29.1 years (+/-13.5 years); ethnic group: Malays 5 (14.7%), Chinese 12 (35.3%) and Indians 17 (50%).Twenty-six (76.5%) were diagnosed under the age of 40 and 8 (23.5%) were diagnosed over the age of 40. Location of the disease was as follows:ileocolonic 13 (38.2%), terminal ileum only 9 (26.5%), colon only 8 (23.5%), and upper gastrointestinal 4 (11.8%). Sixteen (47.1%) had penetrating disease, 9 (26.5%) had stricturing disease and 9 (26.5%) had non-penetrating and non-stricturing disease. The hospital admission prevalence of CD was 26.0 overall, Indians 52.6, Chinese 6.9, and Malays 9.3 per 10(5) admissions per ethnic group. The difference between Indians and Malays: [OR 5.67 (1.97, 17.53) P<0.001] was statistically significant but not between the Indians and the Chinese [OR 1.95 (0.89, 4.35) P=0.700]. The difference between the Chinese and the Malays was also not statistically significant. [OR 2.90 (0.95, 9.42) P=0.063].

    CONCLUSION: The clinical presentation of CD is similar to the Western experience. Although the overall prevalence is low,there appears to be a clear racial predominance among the Indians.

    Matched MeSH terms: Colon/pathology; Crohn Disease/pathology*; Ileum/pathology; Gastrointestinal Tract/pathology
  12. Hishamnuri WNAD, Nakagun S, Maezawa M, Sakaguchi K, Akiyama N, Watanabe KI, et al.
    J Vet Diagn Invest, 2019 Nov;31(6):852-855.
    PMID: 31551023 DOI: 10.1177/1040638719875501
    A 19-mo-old Holstein heifer was inactive and dyspneic. Physical examination revealed wheezing, exophthalmos, a cervical mass, and lymphadenopathy. Cytology of the cervical mass and lymph nodes showed predominantly large atypical lymphocytes. Lactate dehydrogenase and thymidine kinase activities were elevated. Although nested PCR for bovine leukemia virus (BLV) using blood was positive, quantitative PCR showed a low number of provirus copies. Autopsy revealed enlargement of most lymph nodes examined, as well as white masses of various sizes in muscles of the left hindlimb and thoracic and abdominal organs. Histopathology revealed severe infiltration with neoplastic lymphocytes in these organs. The cervical mass was immune-positive for B-cell markers. The final diagnosis was thymic B-cell lymphoma with BLV infection.
    Matched MeSH terms: Cattle Diseases/pathology; Thymus Neoplasms/pathology; Lymphoma, B-Cell/pathology; Enzootic Bovine Leukosis/pathology
  13. Wong KT, Dick D, Anderson JR
    Neuromuscul Disord, 1996 May;6(3):163-6.
    PMID: 8784803
    This report describes a 56-yr-old man with a dominantly inherited disorder affecting four generations and characterized by bilateral ptosis and dysphagia. Muscle biopsy showed only minor light microscopic abnormalities but electron microscopy revealed fibres containing paracrystalline mitochondrial inclusions. Southern analysis of mitochondrial DNA obtained from muscle did not reveal mitochondrial gene deletions. An extensive search eventually identified the characteristic intranuclear filaments of oculopharyngeal muscular dystrophy (OPMD). Abnormal mitochondria are non-specific epiphenomena in OPMD but a potential source of confusion with a late-onset mitochondrial cytopathy. This case further emphasizes the necessity for a diligent search for the diagnostic intranuclear filaments when oculopharyngeal muscular dystrophy is suspected clinically.
    Matched MeSH terms: Mitochondria, Muscle/pathology*; Muscular Dystrophies/pathology*; Muscular Dystrophies/physiopathology*; Muscle, Skeletal/pathology*; Muscle Fibers, Skeletal/pathology
  14. Zeehaida M, Zueter A, Zairi NZ, Zunulhisham S
    Trop Biomed, 2015 Sep;32(3):545-50.
    PMID: 26695216 MyJurnal
    Trichuris Dysentery Syndrome (TDS) is a severe persistent trichuriasis associated with heavy worm build-up in the colon that continues to be neglected and underestimated in endemic countries. Trichuriasis is most prevalent in children in tropical countries, and that increases the risk of TDS. We reported a series of four preschool children of both genders chronically having TDS over a period ranging from several months to years presenting with anaemia. The hemoglobin levels ranged from 4.6 to 9.1 g/dl on first admissions. Despite treatment, the cases were reported to have failure to thrive with persistent anaemia. It was concluded that TDS should be considered in endemic areas among children presenting with chronic bloody diarrhea and anaemia.
    Matched MeSH terms: Anemia/pathology; Colon/pathology*; Dysentery/pathology*; Trichuriasis/pathology*
  15. Tan YF, Teng CL, Chua KB, Voon K
    J Infect Dev Ctries, 2017 Mar 31;11(3):215-219.
    PMID: 28368854 DOI: 10.3855/jidc.9112
    INTRODUCTION: Pteropine orthoreovirus (PRV) is an emerging zoonotic respiratory virus that has spilled over from bats to humans. Though initially found only in bats, further case studies have found viable virus in ill patients.

    METHODOLOGY: PubMed was queried with the keywords of Nelson Bay orthoreovirus OR Pteropine orthoreovirus OR Melaka orthoreovirus OR Kampar orthoreovirus, and returned 17 hits.

    RESULTS: Based on prevalence studies, the presence of PRV has been reported in Malaysia and Vietnam, both developing countries. Other case reports also provide further evidence of the presence of PRV in the Southeast Asian region. Despite the absence of PRV in their home countries, travellers from Hong Kong and Japan to Indonesia have returned to their countries ill with this virus, indicating that local communities in Indonesia might be affected by this virus.

    CONCLUSIONS: This work aims to bring to light this emerging zoonotic respiratory virus circulating among developing countries in Southeast Asia. To improve the understanding of PRV of the medical and scientific community in the Southeast Asian region, this work introduces the general features of PRV, reports of imported PRV, prevalence, and clinical features of PRV. Gaps in knowledge about PRV have also been identified in this work, and we hope that future studies can be undertaken to improve our understanding of this virus.

    Matched MeSH terms: Reoviridae Infections/pathology; Respiratory Tract Infections/pathology; Zoonoses/pathology; Communicable Diseases, Emerging/pathology
  16. Vannas A, Hogan MJ, Wood I
    Am J Ophthalmol, 1975 Feb;79(2):211-9.
    PMID: 46719
    Eleven corneal specimens from nine patients with Salzmann's nodular degeneration of the cornea, together with all available clinical information, were collected for this study. The specimens were examined by light and electron microscopy. An antecedent keratitis was diagnosed by history and microscopic findings in every case. The corneal epithelium showed degenerative changes, its thickness varied, and in nodular areas it often consisted of only a single layer of flattened epithelial cells by light microscopy. Bowman's membrane was missing over the nodules, and in this zone there was excessive secretion of a basement membrane-like material. Hyaline degeneration of collagen, cellular debris, and electron-dense hyaline deposits were seen in the collagen of the nodules. The number of fibrocytes in the nodules varied from many that were active to a few that were degenerating. External irritation because of poor epithelial protection was interpreted as a causative factor, although other tissue repair mechanisms may also have played a role.
    Matched MeSH terms: Cicatrix/pathology; Cornea/pathology*; Eye Diseases/pathology; Keratitis/pathology
  17. Parra-Medina R, Lopez-Correa P, Gutierrez V, Polo F
    Malays J Pathol, 2018 Aug;40(2):199-202.
    PMID: 30173239
    A 43-year-old man presented with two-month history of fatigue, weakness, paleness, rectal bleeding, sweating, and weight loss of 10 kg in the past one month. A complete blood count revealed anaemia. The patient underwent a right hemicolectomy. The microscopic examination revealed an adenosquamous carcinoma associated with a mucinous adenocarcinoma in a patient with microsatellite instability due to loss of MLH1 and PMS2 expression and retention of MSH2 and MSH6 expression in both the squamous and glandular components. We also observed an atypical immunohistochemical phenotype in the adenocarcinoma component showing CK7 expression and reduced CK20 and CDX2 expression.
    Matched MeSH terms: Adenocarcinoma, Mucinous/pathology*; Colonic Neoplasms/pathology*; Neoplasms, Multiple Primary/pathology*; Carcinoma, Adenosquamous/pathology*
  18. Eachempati P, Aggarwal H, Shenoy VK, Baliga M
    J Coll Physicians Surg Pak, 2018 Sep;28(9):S187-S189.
    PMID: 30173693 DOI: 10.29271/jcpsp.2018.09.S187
    Oral mucosal melanoma is rare and more aggressive than cutaneous melanoma. Hard palate and maxillary alveolar crest are most commonly involved. Multidisciplinary team approach is necessary for successful management of this tumor. The main treatment modality is surgical resection, which usually results in impaired mastication, deglutition, speech, oral competence and significant cosmetic deformity. Here, a rare case of oral mucosal melanoma of mandibular gingiva in a 44-year man is reported, who was treated by en-block mandibular resection followed by adjuvant therapy with high dose interferons (IFN) - 2b. Following two weeks of healing period, prosthetic rehabilitation of the patient was done with an interim removable denture prosthesis, which effectively limited the unfavourable effects of surgery and helped him in resocialisation.
    Matched MeSH terms: Gingival Neoplasms/pathology*; Mandible/pathology*; Melanoma/pathology*; Mouth Mucosa/pathology*
  19. Phang ZH, Saw XY, Nor NFBM, Ahmad ZB, Ibrahim SB
    BMC Cancer, 2018 Nov 14;18(1):1112.
    PMID: 30428857 DOI: 10.1186/s12885-018-5012-3
    BACKGROUND: Sacral chordoma is a locally aggressive malignant tumour originating from ectopic notochordal cells. The natural history of sacral chordoma is a slow growing tumour arising at the midline of the lower sacrum that can invade the sacrum and progressively increase in size expanding cranially and anteriorly. Metastasis is very rare even when the tumour is large. Sacral chordoma affects males more than females and is more commonly found in middle age and elderly patients.

    CASE PRESENTATION: A 25 years old female had neglected an extremely large midline sacral mass for 2 years. On presentation to hospital, she had been bed bound for the past 2 years. The sacral mass was so large that it prevented her from lying down supine and sitting on the wheelchair comfortably. Clinical examination showed a 40 cm × 30 cm × 20 cm hard mass over the sacrum that involved both buttocks and the gluteal fold. Neurological exam of bilateral lower limb was normal. Computed Tomography Scan of the Pelvis showed a large destructive sacrococcygeal mass measuring 43 cm × 38 cm × 27 cm with extension into the presacral space resulting in anterior displacement of the rectum, urinary bladder and uterus; and posterior extension into the dorsal soft tissue with involvement of the gluteus, piriformis, and left erector spinae muscles. Biopsy taken confirmed Chordoma. This patient was managed by a multidisciplinary team in an Oncology referral centre. The patient had undergone Wide En Bloc Resection and Sacrectomy, a complex surgery that was associated with complications namely bleeding, surgical site infection and neurogenic bowel and bladder. Six months post operatively the patient was able to lie supine and sit on wheelchair comfortably. She required extensive rehabilitation to help her ambulate in future.

    CONCLUSION: This is a rare case of neglected sacral chordoma in a young female treated with Wide En Bloc Resection and Sacrectomy associated with complications of this complex surgery. Nevertheless, surgery is still worthwhile to improve the quality of life and to prevent complications secondary to prolonged immobilization. A multidisciplinary approach is ideal and team members need to be prepared to address the complications once they arise.

    Matched MeSH terms: Chordoma/pathology; Sacrum/pathology; Spinal Neoplasms/pathology; Neglected Diseases/pathology
  20. Alwi M
    Catheter Cardiovasc Interv, 2006 May;67(5):679-86.
    PMID: 16572430 DOI: 10.1002/ccd.20672
    Pulmonary atresia with intact ventricular septum (PAIVS) is a disease with remarkable morphologic variability, affecting not only the pulmonary valve but also the tricuspid valve, the RV cavity and coronary arteries. With advances in interventional techniques and congenital heart surgery, the management of PAIVS continues to evolve. This review is an attempt at providing a practical approach to the management of this disease. The basis of our approach is morphologic classification as derived from echocardiography and angiography. Group A, patients with good sized RV and membranous atresia, the primary procedure at presentation is radiofrequency (RF) valvotomy. Often it is the only procedure required in this group with the most favourable outcome. Patients with severely hypoplastic RV (Group C) are managed along the lines of hearts with single ventricle physiology. The treatment at presentation is patent ductus arteriosus (PDA) stenting with balloon atrial septostomy or conventional modified Blalock Taussig (BT) shunt. Bidirectional Glenn shunt may be done 6-12 months later followed by Fontan completion after a suitable interval. Patients in Group B, the intermediate group, are those with borderline RV size, usually with attenuated trabecular component but well developed infundibulum. The treatment at presentation is RF valvotomy and PDA stenting +/- balloon atrial septostomy. Surgical re-interventions are not uncommonly required viz. bidirectional Glenn shunt when the RV fails to grow adequately (11/2 - ventricle repair) and right ventricular outflow tract (RVOT) reconstruction for subvalvar obstruction or small pulmonary annulus. Catheter based interventions viz. repeat balloon dilatation or device closure of patent foramen ovale (PFO) may also be required in some patients.
    Matched MeSH terms: Heart Defects, Congenital/pathology; Heart Septal Defects/pathology; Heart Ventricles/pathology*; Pulmonary Atresia/pathology
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