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Biomedical subjects

Shiu-Feng Huang

Publications and source records attributed to Shiu-Feng Huang.

At least 37 records · Page 2Linked to original sources

Ultrasound biomicroscopy in pigmented conjunctival cystic nevi.

PURPOSE: To report the use of ultrasound biomicroscopy in the clinical diagnosis and management of pigmented conjunctival cystic nevi. METHOD: Two patients, aged 11 and 18 years, with rapidly growing raised conjunctival melanocytic lesions suspected to be inflamed juvenile conjunctival nevus underwent ultrasound biomicroscopic and histopathologic examinations. RESULTS: Ultrasound biomicroscopic examination of the lesions revealed multiple areas of cystic tissue, which is compatible with pathologic finding of compound nevus with epithelial inclusion cysts formation. Furthermore, clear interface was found between the mass and the underlying sclera. CONCLUSION: Pigmented conjunctival nevi may obscure cysts under slit-lamp examination. Ultrasound biomicroscopy is a useful diagnostic adjunct to distinguish cysts in conjunctival lesions. Additionally, this technique may be helpful in delineating the extent of lesions prior to excision biopsy.

Child↗

Adjunctive treatment with interferon alpha-2b may decrease the risk of papilloma-associated conjunctival intraepithelial neoplasm recurrence.

PURPOSE: To report a case of bilateral papilloma virus-positive conjunctival intraepithelial neoplasm and the treatment results of using adjunctive interferon alpha-2b. METHODS: Case report and literature review. RESULTS: A 73-year-old man underwent subtotal excisional biopsy of the 270-degree gelatinous limbal lesion of the right eye to avoid creating a limbal deficiency and cicatricial change. Total excisional biopsy of the temporal elevated leukoplakic limbal lesion was performed on his left eye. Histology examination showed bilateral intraepithelial neoplasia, and human papilloma virus-16 and -18 were detected by polymerase chain reaction in both lesions. Two supplemental perilesional injections of interferon alpha-2b in the right eye were given, and the residual mass decreased in size gradually and completed clinical resolution 7 weeks following initial surgery. Although no recurrence was noticed in the right eye, recurrence of the conjunctival intraepithelial neoplasm lesion was noticed in the left eye. CONCLUSION: Adjunctive therapy might lower CIN recurrence rate, especially in extensive lesions, when surgical excision cannot ensure a tumor-free margin. Our bilateral case provided a good control example for the recurrence with or without adjunctive therapy.

Aged↗

Severe intrahepatic cholestasis in an elderly patient with primary amyloidosis and colon adenocarcinoma.

Hepatic involvement in primary amyloidosis (AL type) is not rare but is often clinically silent. However, presentation with jaundice in AL-type amyloidosis is rare, with an incidence of less than 5% reported in the literature. It is considered to be a preterminal sign. We herein report on a case of primary hepatic amyloidosis presenting with severe intrahepatic cholestasis. Viral, drug, alcohol, and autoimmune etiologies were all excluded. A liver biopsy was performed because of unexplained cholestatic jaundice for 3 months. The pathology showed hepatic amyloidosis with extensive amyloid deposition in the expanded portal tracts and sinusoidal space. The patient received supportive treatment only, because of persistent jaundice, coexistent colon cancer with para-aortic lymph node metastasis, and possibly peritoneal carcinomatosis. Unfortunately, the patient died of sepsis 10 months after the onset of jaundice. We suggest that hepatic amyloidosis must be considered in the differential diagnosis of unexplained cholestatic jaundice.

Adenocarcinoma↗

Pulmonary lymphangioleiomyomatosis: a clinicopathological analysis of ten cases.

BACKGROUND: Lymphangioleiomyomatosis (LAM) of the lung is a very rare disease of unknown etiology which occurs particularly in women of childbearing age. So far, there have been very few reports of LAM in Taiwan. METHODS: Data on all patients with a clinical or pathological diagnosis of LAM in Chang Gung Memorial Hospital from 1990 to 2001 were collected from the pathology files or by consultation with thoracic surgeons, chest physicians, and radiologists of this institution. Cases were confirmed by lung biopsy and high-resolution computed tomographic scanning. Clinical data were obtained from patients' charts. Additional immunostaining for HMB-45 antigen, estrogen receptor, and progesterone receptor was carried out for cases with available paraffin blocks. RESULTS: In total, 10 patients confirmed with a diagnosis of LAM and adequate clinical follow-up information were found from 1991 to 2001. All were females of fertility age. The follow-up periods ranged from 18 to 167 months. Six patients received hormonal therapy. According to the pulmonary function tests and clinical symptoms, 6 patients were in stable condition and 4 showed slow progression of disease by the time of the last follow-up. Two patients died after lung transplantation and abdominal surgery for retroperitoneal LAM, respectively, at another medical center. CONCLUSIONS: The clinicopathological features of our series are similar to those reported from other countries. The clinical course was slowly progressive with no significant response to hormonal therapy. Lung biopsies are important for a diagnosis, but the size and location of the biopsy specimens greatly affect the diagnostic reliability.

Adult↗

Infantile-onset glycogen storage disease type II (Pompe disease): report of a case with genetic diagnosis and pathological findings.

Glycogen storage disease type II (GSD-II), also known as Pompe disease, is a rare autosomial recessive disease due to deficiency of lysosomal acid alpha-glucosidase (GAA). The infantile-onset form is the most severe, and most patients present with hypotonia and cardiomyopathy in early infancy. We report on a typical case of Pompe disease in a patient who died at 8 months of age due to aspiration pneumonia and hypertrophic cardiomyopathy. Genetic studies showed deficient GAA activity and mutation of the GAA gene with Gly615Arg (exon 13, G1845A). On autopsy, glycogen had markedly accumulated in the liver, myocardium and skeletal muscle. The neurons of the anterior horn of the spinal cord and medulla were also involved, but the cortex was spared. These neurological-histologic findings may explain the clinical features of poor motor function, decreased deep tendon reflexes and lack of mental retardation.

Base Sequence↗

Interleukin-6 acts as an antiapoptotic factor in human esophageal carcinoma cells through the activation of both STAT3 and mitogen-activated protein kinase pathways.

The production of interleukin-6 (IL-6) has been discovered in a variety of human tumors. Here we report the expression of IL-6, IL-6 receptor alpha (IL-6Ralpha), and gp130 in human esophageal carcinoma tissues. We further demonstrate that IL-6 protects an esophageal carcinoma cell line CE48T/VGH from apoptosis induced by staurosporine. IL-6 stimulation induced a rapid phosphorylation of gp130 and STAT3, and a dominant-negative STAT3 completely abolished the antiapoptotic effect. IL-6 also activated ERK 1/2 in CE48T/VGH cells. Inhibition of the ERK activation by PD98059 and transfection of a dominant-negative ERK2 completely blocked the protection of IL-6 against apoptosis. Thus, both STAT and MAP kinase pathways are responsible for the IL-6-delivered survival signal in human esophageal carcinoma cells. In contrast, PI3-K inhibitors only partially attenuated the effect of IL-6, suggesting that PI3-K does not play a major role in the antiapoptotic signal of IL-6 in our system. To investigate whether IL-6 could induce the production of antiapoptotic molecules, proteins of the Bcl-2 family were measured. While Bcl-2, Bcl-x(L,), and Bax were not affected, Mcl-1 was induced by IL-6 in human esophageal carcinoma cells. Our results suggest that IL-6 may contribute to the progression of esophageal cancers in an autocrine or paracrine manner.

Apoptosis↗

Novel point mutations in complete androgen insensitivity syndrome with incomplete müllerian regression: two Taiwanese patients.

UNLABELLED: Complete androgen insensitivity syndrome (CAIS) is a relatively rare X-linked disorder caused by androgen receptor gene (AR) mutations that result in complete impairment of genital virilisation. In these individuals, no müllerian derivatives are usually found; however, several sporadic cases of CAIS with müllerian remnants have been reported. In this paper, we report two novel point mutations of the AR gene resulting in two cases of CAIS with incomplete müllerian regression. Molecular studies of cases 1 and 2 showed novel missense mutations of the AR gene, with a methionine to threonine substitution at codon 749 (base 2608 T-->C) in exon 5 and a methionine to lysine substitution at codon 787 (base 2722 T-->A) in exon 6. Both patients received bilateral gonadectomy and inguinal hernia repair. The excised gonads proved to be testes with incomplete regression of the müllerian structures. CONCLUSION: Müllerian structures can be present in androgen insensitivity syndrome and the presence of a uterus therefore does not exclude this disorder. Further study of these patients may promote a better understanding of the pathogenesis.

Androgen-Insensitivity Syndrome↗

Antisense overexpression of BMAL2 enhances cell proliferation.

To identify genes that are frequently downregulated in hepatocellular carcinoma (HCC), a panel of putative underexpressed genes was first established by an in-house cDNA macroarray method. Two different assays, semiquantitative RT-PCR combined with Northern analysis and customized cDNA microarray analysis, were used to screen through these genes and the results were compared. Several genes, some with unknown function, were confirmed to be downregulated by both the methods. The effect of a downregulated gene, BMAL2, on cell proliferation was examined. Overexpression of antisense BMAL2 RNA in 293EBNA cells resulted in reduced cell cycle time, increased plating efficiency in soft agar, diminished TNF-alpha-induced increment of CPP32/caspase-3 activity, and a reduced proportion of cells in the G2 phase with a concomitantly increased proportion of cells in the S phase. In conclusion, by combining three different methods, we have obtained a panel of frequently down regulated genes in HCC, including BMAL2. Antisense overexpression of BMAL2 enhances cell proliferation.

ARNTL Transcription Factors↗

Expression of deleted in malignant brain tumor-1 (DMBT1) molecule in biliary epithelium is augmented in hepatolithiasis: possible participation in lithogenesis.

Deleted in malignant brain tumor-1 (DMBT1) is a mucin-like molecule participating in mucosal immune defense. Given that bovine gallbladder mucin, which accelerates cholesterol crystallization, is a DMBT1 homolog, DMBT1 expression was examined immunohistochemically in biliary epithelial cells in livers with hepatolithiasis (N = 25), primary sclerosing cholangitis (N = 7), large bile duct obstruction (N = 12), and control normal livers (N = 10). DMBT1 protein was determined in the hepatic bile samples of hepatolithiasis (N = 12) and other hepatobiliary diseases (N = 8) by immunoblot. While DMBT1 was faintly expressed in normal livers (20%), it was significantly augmented in hepatolithiasis (76%) (P < 0.05). DMBT1 was mildly expressed in primary sclerosing cholangitis and large bile duct obstruction. DMBT1 protein was detected frequently in hepatic bile samples of hepatolithiasis (50%) (P < 0.05), but in the other bile samples. The percentage of cholesterol in intrahepatic calculi was significantly higher in the patients with DMBT1-positive bile. Augmented expression and secretion of DMBT1 in intrahepatic large bile ducts in hepatolithiasis suggests its role in lithogenesis.

Agglutinins↗

Idiopathic pulmonary fibrosis in a child.

Idiopathic pulmonary fibrosis (IPF) is a rare disease of unknown etiology and is usually associated with a poor prognosis. Up to the present, less than 50 cases of IPF in children have been reported in the English literature, and no case has ever been reported from Taiwan. Herein we report on a 2-year-old boy with IPF presenting with a rapid onset of dyspnea followed by respiratory failure. The diagnosis of IPF was verified with an open lung biopsy. Despite intravenous methylprednisolone pulse therapy and empiric nitric oxide treatment, he expired on the 35th day after admission due to profound hypoxemia. A diagnosis of IPF should be included in the differential diagnosis for patients presenting with unexplained shortness of breath and pulmonary interstitial infiltrations.

Adrenal Cortex Hormones↗

Endoscopic mucosal resection using a pure cut and hemoclip method for colonic nonpolypoid neoplasms.

BACKGROUND: Colonic mucosal neoplastic lesions can be classified morphologically into polypoid and nonpolypoid types. The nonpolypoid type has a greater malignancy potential than does the polypoid type. Removing these lesions and obtaining an integral specimen for histopathologic assessment during colonoscopy are very important. This study evaluates the safety and integrity of specimens obtained by endoscopic mucosal resection (EMR) using the pure cut current and hemoclip method. METHODS: Fourteen nonpolypoid colonic neoplasms, which were removed by EMR using the pure cut and hemoclip method between April 2001 and April 2002, were studied. There were 9 male and 4 female patients and the mean age was 57.8 +/- 15.5 (range, 32 - 80) years. EMR was conducted in cases where the lesions were diagnosed as neoplastic tumors by magnification colonoscopy and the indigo carmine dye spray method. RESULTS: The study revealed 11 flat type neoplasms and 3 laterally spreading tumors. The mean size of the lesions was 10.7 +/- 5.6 (range, 6 - 25) mm. All lesions were completely removed. Histopathologically, there were 1 adenocarcinoma and 13 adenomas (3 with mild dysplasia, 7 with moderate dysplasia, and 3 with severe dysplasia). The mean number of hemoclips used was 2.14 +/- 0.66 (range, 1 - 3) pieces. No bleeding or perforation was noted following EMR. CONCLUSION: EMR using the pure cut and hemoclip method is a useful means of obtaining an integral specimen for accurate pathologic assessment. This method provides a safe and minimally invasive technique for managing colonic non-polypoid lesions.

Adult↗

Co-existence of a huge pseudocyst and mucinous cystadenoma: report of a case and the value of magnetic resonance imaging for differential diagnosis.

Co-existence of a pancreatic pseudocyst and a neoplastic cyst is rare and their differential diagnosis is difficult if the patient has an atypical history as well as subclinical symptoms. The formation of a pseudocyst under such circumstances is usually the result of downstream ductal obstruction by the neoplasm. Two large cysts were found in a 43-year-old woman who had symptoms of gastric outlet obstruction that were the result of external compression by one of the cysts. Magnetic resonance imaging was superior to computed tomography, discriminating between the internal contents and surrounding tissue of the two cysts, enabling the correct preoperative diagnosis of a pseudocyst co-existing with a mucinous cystadenoma to be made. It was most unusual for the pseudocyst to be located downstream of the mucinous tumour, ruling out ductal obstruction by the tumour in its pathogenesis. A possible explanation for the pseudocyst formation in this case was pancreatic juice accumulation in the space of the lesser sac after pancreatic parenchymal destruction by the mucinous tumour.

Adult↗

Hepatic hydatid cysts with superinfection in a non-endemic area in Taiwan.

Hepatic hydatid cysts are extremely rare in Taiwan. We report a case of complicated, multivesicular, hydatid cysts in the liver and a secondary infection with Klebsiella pneumoniae. The cysts were detected in an early stage by ultrasonography and computed tomography and treated successfully with radical resection.

Aged↗

Liver cell adenoma with concomitant hepatocellular carcinoma: report of two cases.

Malignant transformation of liver cell adenoma and unequivocal hepatocellular carcinoma in resected liver cell adenomas are rare. We report two cases of liver cell adenoma with concomitant hepatocellular carcinoma. The first occurred in a 19-year-old male with an asymptomatic liver mass and was discovered incidentally. He had a history of nephrotic syndrome treated with oral prednisolone for 15 years. The second occurred in a 46-year-old female who was admitted due to right tubo-ovarian abscess. Computerized tomography scanning incidentally found a 10-cm liver mass in the right lobe. There was no history of oral contraceptive use. Both patients were negative for serum hepatitis B surface antigen and anti-hepatitis C virus antibody. Neither had cirrhosis. These two cases imply that malignant transformation of liver cell adenoma does occur, and that liver cell adenomas should be excised when malignant transformation is suspected.

Adenoma, Liver Cell↗

Carcinoid tumor of the middle ear: a case report.

Middle ear (ME) carcinoid tumor is exceedingly rare. We present a case of ME carcinoid tumor in a 29-year-old woman whose chief complaints were right otalgia, aural fullness, hearing loss, and facial palsy of 5 days duration. A reddish bulging mass behind the right eardrum and right facial palsy staged as grade IV on the House-Brackmann scale were noted. The high-resolution computed tomography of the temporal bone showed a soft-tissue density mass in the right middle ear and antrum without bony invasion. Although the initial diagnosis was ME adenoma made by the intraoperative frozen section report, the tumor was resected completely by a modified conservative ossicles-preservation method. The patient's initial symptoms of otalgia, aural fullness, and hearing loss immediately improved after surgery, and facial palsy diminished in the following months. No recurrence was noted after 12 months of follow-up.

Adult↗

Amniotic membrane transplantation for corneal perforation related to vitamin A deficiency.

Corneal perforation is one of the most vision-threatening consequences of vitamin A deficiency. Amniotic membrane transplantation was performed in a 36-year-old man who presented with bilateral corneal ulcers. There was a small, sharply demarcated superficial ulcer with crevices of surrounding keratinized epithelium converging on the ulcer and minimal infiltration at the inferior periphery of the right cornea. A 3-mm perforation on a 9 x 7-mm area of stromal melting was noticed in the left cornea. The serum vitamin A level was 14.0 microg/dL (normal, 24.57 to 105.71 microg/dL) at presentation. A biopsy of the right corneal epithelium was performed to confirm the diagnosis. Multilayered amniotic membrane transplantation with debridement of the necrotic corneal stroma was performed on the left cornea. In addition to systemic vitamin A replacement, topical antibiotics and lubricants were also administered. The cornea ulcers healed in 10 days along with the restoration of the serum vitamin A level. The visual acuity improved from 20/250 to 20/20 in the right eye and from light perception to hand movement in the left eye at 3 months. Amniotic membrane transplantation could save eyes with corneal perforation related to vitamin A deficiency before reconstructive surgeries, such as penetrating keratoplasty, are necessary.

Adult↗

Congenital esophageal duplication cyst: report of two cases.

Esophageal duplication cysts account for a small percentage of benign esophageal tumors and are frequently asymptomatic. Diagnosis is difficult, and patients often seek treatment when complications such as respiratory distress, bleeding, or infection develop. Surgical excision is recommended at the time of cyst discovery whether or not symptoms are present. We report 2 newborn cases with esophageal duplication cysts. Symptoms included respiratory distress and palpable neck mass. Computed tomography demonstrated cystic masses in the retropharyngeal space. Both patients underwent surgical resection of the cyst and the symptoms were relieved. Histopathologic examination revealed esophageal duplication cysts.

Esophageal Cyst↗