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Biomedical subjects

W Y Lam

Publications and source records attributed to W Y Lam.

At least 19 recordsLinked to original sources

Inflammatory myofibroblastic tumor of the nasal cavity.

Inflammatory myofibroblastic tumor (IMT) is a rare tumor with a variable natural history and biologic behavior, ranging from completely benign to malignant with fatal outcome. We report a case of benign IMT in the left nasal cavity with radiologic features mimicking angiofibroma. We also demonstrate the hypervascular nature of this disease on angiography and the contribution of preoperative embolization in assisting surgical excision and minimizing the potential uncontrolled intraoperative bleeding.

Adolescent↗

Pathology of fatal human infection associated with avian influenza A H5N1 virus.

Eighteen cases of human influenza A H5N1 infection were identified in Hong Kong from May to December 1997. Two of the six fatal cases had undergone a full post-mortem which showed reactive hemophagocytic syndrome as the most prominent feature. Other findings included organizing diffuse alveolar damage with interstitial fibrosis, extensive hepatic central lobular necrosis, acute renal tubular necrosis and lymphoid depletion. Elevation of soluble interleukin-2 receptor, interleukin-6 and interferon-gamma was demonstrated in both patients, whereas secondary bacterial pneumonia was not observed. Virus detection using isolation, reverse transcription-polymerase chain reaction and immunostaining were all negative. It is postulated that in fatal human infections with this avian subtype, initial virus replication in the respiratory tract triggers hypercytokinemia complicated by the reactive hemophagocytic syndrome. These findings suggest that the pathogenesis of influenza A H5N1 infection might be different from that of the usual human subtypes H1-H3.

Adolescent↗

Kaposiform hemangioendothelioma: five patients with cutaneous lesion and long follow-up.

Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor of the skin, deep soft tissue, and bone in children, characterized by infiltrating nodules and sheets of spindle cells, and unmistakable resemblance to Kaposi's sarcoma. More than 60 patients with such tumor have been reported so far, and while many have died as a result of extensive disease and severe coagulopathy, the long-term biologic behavior of this tumor remains undetermined. We describe five patients with kaposiform hemangioendothelioma and a mean follow-up of 19 years, ranging from 8 to 35 years. This report emphasizes on the importance of cutaneous lesions being the most commonly affected site, but also for its clinical diversity. Early diagnosis is possible even for a small skin lesion, which may be critical for the treatment of a potentially fatal deep-seated extensive tumor. All five patients are well, and three of them with persistent vascular tumor, which has carried two patients from childhood to adult. Although the behavior of this tumor might have been modified by radiation or interferon in three patients, this series indicates that kaposiform hemangioendothelioma is incapable of metastasis, despite a protracted course of many decades with no tendency for spontaneous regression.

Adult↗

Renal failure and cholestatic jaundice as unusual complications of childhood pustular psoriasis.

Generalized pustular psoriasis (GPP) is uncommon in children, and serious renal and liver complications arising from GPP are rarely reported. We describe a Chinese boy who had suffered from recurrent exacerbations of GPP from the age of 1 year. He developed IgA nephropathy at the age of 9 years. He also had recurrent episodes of oliguric renal failure, hepatomegaly and cholestasis associated with severe exacerbations of GPP. These complications progressed despite early antibiotics and supportive therapy, but responded promptly to intravenous methylprednisolone therapy. Ultimately, acitretin was given and he has successfully been in remission for a year.

Anti-Inflammatory Agents↗

Reduced expression of APC and DCC gene protein in breast cancer.

AIMS: To investigate if the adenomatous polyposis coli (APC) protein and the deleted in colorectal cancer (DCC) protein expression can be demonstrated by an immuno-histochemical method and to study the role of APC and DCC gene inactivation in the development and progression of breast cancer using colorectal cancer as a control model. METHODS AND RESULTS: The reduced or loss of protein expression of the APC and DCC genes was studied in 27 surgical specimens of primary breast cancer using an immunohistochemical method. Reduced or lost expression was identified in 11 out of 27 samples (40.7%) for the APC gene and 15 out of 27 samples (55.6%) for the DCC gene. No statistically significant difference was observed between the reduced or lost protein expression and the histological grading of breast tumour for both the APC and the DCC gene. CONCLUSIONS: Both gene proteins can be demonstrated by the immunohistochemical method. Reduced or loss of APC and DCC gene product were observed in 40.7% and 55.6% cases of primary breast cancer respectively. Further work is required to investigate the significance of the finding.

Adenomatous Polyposis Coli Protein↗

Porokeratotic eccrine ostial and dermal duct naevus with dermatomal trunk involvement: literature review and report on the efficacy of laser treatment.

Porokeratotic eccrine ostial and dermal duct naevus (PEODDN) is a rare, benign hamartomatous malformation involving the eccrine sweat duct. The existence of filiform keratinous plugs that represent cornoid lamellae overlying dilated infundibula of eccrine ducts is a distinctive feature and the presence of associated abnormal dermal ducts is frequent. We report a patient with PEODDN who exhibited lesions on the left side of her chest. Cases of PEODDN reported in the literature are reviewed. Our experience in treating this patient with ultrapulsed carbon dioxide laser is also presented.

Eccrine Glands↗

Herpes simplex virus type 2 infection in a 5-year-old boy presenting with recurrent chest wall vesicles and a possible history of herpes encephalitis.

A 5-year-old hyperkinetic but otherwise healthy child presented with recurrent irritable vesicles and erosions of the anterior chest wall; they have been apparent since the age of 15 months. Wound swab cultures yielded herpes simplex virus type-2 (HSV-2) and Western blot serology showed past exposure to both HSV-1 and HSV-2. Skin biopsy results further supported a herpes virus infection. Magnetic resonance imaging of the brain showed right temporal lobe atrophy. An evaluation showed no evidence of sexual abuse in the patient but a Western blot assay of the mother's serum for HSV-2 was positive, while the father's was negative. In view of the diagnosis of HSV-2 infection in such a young patient, the possible routes of transmission and the time of acquisition of infection were explored. We believe the most likely route of infection in this child was postnatal, through intimate contact with the mother.

Child, Preschool↗

Isolation and characterization of a human heart cDNA encoding a new member of the small heat shock protein family--HSPL27.

A novel cDNA clone was isolated from a human adult heart cDNA library. This cDNA clone is similar to the small heat shock protein (smhsp) in both DNA and amino acid sequences, especially in the conserved region. Sequence analysis has shown that the putative novel smhsp, named 27 kDa heat-shock-protein-like protein (HSPL27) is a protein of 241 amino acids with a deduced molecular mass of 26.7 kDa and a deduced pI of 8.0. We have expressed the HSPL27 in E. coli and the expressed protein was found to be present in the soluble fraction of the bacterial cell lysate. Chromosomal mapping data shows that the HSPL27 gene is located at human chromosome 5q11.2.

Adult↗

A novel cDNA encoding a human homologue of ribosomal protein L29.

During the large scale partial sequencing of human heart cDNA clones, a novel clone which is very similar to the rat ribosomal protein L29 in both DNA and amino acid sequences was found. The cDNA encodes a protein with a deduced molecular weight of 17751 (159 aa). It shows 80.4% homology to protein L29 from the large ribosomal subunit of rat and is related to yeast YL43. The putative protein was named human ribosomal protein L29 (hRPL29). hRPL29 has a large excess of basic residues over acidic ones. The large amount of charged residues makes the protein very hydrophilic and the protein has a deduced pI of 12.16. Internal repeats have been characterised in many ribosomal proteins and a tandem repeat of KAKAKAKA was found to be unique to hRPL29. Analysis of gene organisation by Southern blotting shows that of the approximate 10 copies of hrpL29, all but one are pseudogenes. Northern analysis indicated that the mRNA that encodes human L29 is approx. 800 base pairs in length. An intron of hrpL29 has also been cloned and sequenced by polymerase chain reaction using human genomic DNA as the template.

Amino Acid Sequence↗

Late cosmetic results of short fractionation for breast conservation.

BACKGROUND AND PURPOSE: The number of fractions of radiation therapy (RT) used after breast conserving surgery varies widely and accounts for a significant proportion of the workload in a modern radiotherapy department. Internationally, 'standard' therapy ranges from 3 to 7 weeks of daily treatment with or without a boost. Short RT schedules have the attraction of reducing workload but raise concern about an increased risk of late effects and poorer cosmetic outcome. MATERIALS AND METHODS: In a randomized trial, 186 women with T1 or T2, pathologically node-negative breast cancer had cosmetic and various normal tissue effects data collected prospectively. The breast RT prescription was 44 Gy in 16 daily fractions to a tangent pair. RESULTS: Median follow-up is 6.7 years. Actuarial 5-year breast recurrence was 6%. Overall cosmetic results at 5 years were good or excellent in 89% and 96% as reported by physicians and patients, respectively, and were stable between 2 and 5 years. Breast discomfort, erythema, edema and induration were related to both surgery and RT. At 5 years, 20% had breast discomfort, 18% had induration, 6% had erythema and 3% had some degree of breast edema. Fewer patients had these effects at 5 years than immediately after primary surgery. The presence of induration prior to starting RT was associated with a greater likelihood of breast induration 3 or more years following RT (P = 0.02). Thirteen percent of patients, generally those with large breasts, developed mild inframammary telangiectasia by 5 years. CONCLUSIONS: Results are comparable to those reported from centers employing more conventional fractionation. Short fractionation produces acceptable cosmetic results for the majority of women if there are no contraindications to RT and in the absence of significant post-operative breast induration.

Aspirin↗

Effect of naturally occurring visual acuity differences between two eyes in stereoacuity.

Stereoacuity was measured in 30 subjects with a naturally occurring visual acuity (VA) difference between the eyes. The stereoacuity was measured by a modified Howard's apparatus using the staircase method and VA was measured with log MAR charts. Stereoacuity was worse in subjects with a large VA difference between the two eyes; the correlation between stereoacuity and VA difference was significant (r = 0.76, P < 0.001). Neither the VA of the worse eye nor of the better eye contributed to the reduction in stereoacuity. The deterioration was more obvious if VA difference between the two eyes was one line or more (correlation coefficient, r= 0.88, P < 0.001). This study also reinforces the use of a > or = 70% stereothreshold when attempt stereoacuity results to compare with other studies.

Adolescent↗

Analysis of expressed sequence tags from a fetal human heart cDNA library.

Single-pass sequencing of randomly selected cDNA clones to generate expressed sequence tags (ESTs) has been widely used to identify novel genes and to study gene expression in a variety of tissues. We have generated 2244 ESTs from a human fetal heart library (GenBank Accession Nos. R30692-30774 and R56965-58824), which we present in this report. Of these, 51.7% showed no homology to known genes or were similar only to other ESTs, while 48.4% demonstrated homology to known transcripts. A total of 764 ESTs corresponding to known genes were used to study gene expression patterns in the fetal heart and to analyze differences in these patterns from those observed in the adult heart. These analyses demonstrate the utility of ESTs and sequence-tagged clones in comparative studies of gene expression in the cardiovascular system, and they reveal that differential gene expression underlies the structural and functional characteristics of the developing heart.

Adult↗

Cutaneous protothecosis: report of a case in Hong Kong.

We report a patient who presented with recalcitrant ulcerated papules and plaques on both legs. Histopathology revealed protothecosis, and subsequent culture of the lesions grew Prototheca wickerhamii. Gradual resolution of the ulcers occurred on treatment with itraconazole.

Aged↗

Tufted angioma with complete regression.

We describe 2 patients with tufted angioma (angioblastoma of Nakagawa) which regressed spontaneously. Both patients had a solitary but large lesion on the trunk. The 1st patient, a 3-month-old girl, presented with a 6-cm plaque which began to regress 2 years later. It regressed completely over 1 year and had not recurred after a further 6-year follow-up. The other patient, a 3-month-old boy, presented with a 12-cm mass of coalescent angiomatous papules which slowly regressed over 3-years, while small new lesions appeared in the adjacent contiguous skin. Eventually, gradual and near complete regression was observed after 8 years. The histology in both cases was typical, but bore some resemblance to the recently described kaposiform hemangioendothelioma, lobular capillary hemangioma and spindle cell hemangioendothelioma.

Female↗

Cutaneous Rosai-Dorfman disease presenting as a suspicious breast mass.

Cutaneous Rosai-Dorfman disease in a male patient presented as a clinically malignant left breast mass. The intra-operative frozen section indicated a benign inflammatory lesion and a simple excision was performed. The disease was apparently limited to the skin and subcutis of the left breast with no lymph nodes or other organs involvement. While the lesion demonstrated the characteristic morphology of Rosai-Dorfman disease, the diagnosis of granulomatous mastitis, epithelioid sarcoma and other cutaneous xanthohistiocytic proliferations were considered. In the absence of lymphadenopathy, a high index of diagnostic awareness is required to recognize the uncommon, solitary cutaneous Rosai-Dorfman disease.

Adult↗