The MNS glycophorin variant GP.Mur affects differential erythroid expression of Rh/RhAG transcripts.

BACKGROUND: The band 3 macrocomplex (also known as the ankyrin-associated complex) on the red cell membrane comprises two interacting subcomplexes: a band 3/glycophorin A subcomplex, and a Rh/RhAG subcomplex. Glycophorin B (GPB) is a component of the Rh/RhAG subcomplex that is also structurally associated with glycophorin A (GPA). Expression of glycophorin B-A-B hybrid GP.Mur enhances band 3 expression and is associated with lower levels of Rh-associated glycoprotein (RhAG) and Rh polypeptides. The goal of this study was to determine whether GP.Mur influenced erythroid Rh/RhAG expression at the transcript level. MATERIALS AND METHODS: GP.Mur was serologically determined in healthy participants from Taitung County, Taiwan. RNA was extracted from the reticulocyte-enriched fraction of peripheral blood, followed by reverse transcription and quantitative PCR for RhAG, RhD and RhCcEe. RESULTS: Quantification by real-time PCR revealed significantly fewer RhAG and RhCcEe transcripts in the reticulocytes from subjects with homozygous GYP*Mur. Independent from GYP.Mur, both RhAG and RhD transcript levels were threefold or higher than that of RhCcEe. Also, in GYP.Mur and the control samples alike, direct quantitative associations were observed between the transcript levels of RhAG and RhD, but not between that of RhAG and RhCcEe. CONCLUSION: Erythroid RhD and RhCcEe were differentially expressed at the transcript levels, which could be related to their different degrees of interaction or sensitivity to RhAG. Further, the reduction or absence of glycophorin B in GYP.Mur erythroid cells affected transcript expressions of RhAG and RhCcEe. Thus, GPB and GP.Mur differentially influenced Rh/RhAG expressions prior to protein translation.

Prophylactic total thyroidectomy in an 8-year-old girl with multiple endocrine neoplasia type 2A.

We report a genetic carrier of multiple endocrine neoplasia type 2A (MEN 2A) who underwent prophylactic total thyroidectomy. The asymptomatic carrier of MEN 2A, an 8-year-old Taiwanese girl, was admitted for early thyroidectomy. Preoperative basal plasma concentrations of calcitonin and intact parathyroid hormone, and urine vanillylmandelic acid excretion, were normal. Ultrasonography of the thyroid was also normal. Pathology did not reveal any gross lesion but C-cell hyperplasia of the thyroid gland was found microscopically. Prophylactic total thyroidectomy is encouraged and is justified during the first decade of life for MEN 2A carriers.

Determination of beryllium and selenium in human urine and of selenium in human serum by graphite-furnace atomic absorption spectrophotometry.

For human urine beryllium (Be), each sample (500 microl) was diluted (1+1) with Nash reagent (containing 0.2% (v/v) acetylacetone and 2.0 M ammonium acetate buffer at pH 6.0) and then a 20-microl volume of Triton X-100 (0.4%, v/v) aqueous solution was added. An aliquot (10 microl) of the diluted urine mixture was introduced into a graphite cuvette and was atomized according to a temperature program. The method detection limit (MDL, 3sigma) for Be was 0.37 microg/l in the undiluted urine sample and the calibration graph was linear up to 65.0 microg/l. Calibration graphs were prepared by the standard addition method. Accuracies of 98.6-102% were obtained when testing standard reference material (SRM 2670) freeze dried human urine samples. Precision (relative standard deviation, RSD) for urine Be was < or = 2.3% (withinrun, n = 5) and was < or = 3.0% (between-run, n = 3). For human urine and serum selenium (Se), samples (100 microl) were diluted with HNO3 (0.2%, v/v) to make a (1+1) dilution for urine analysis or a (1+4) dilution for serum analysis. An additional aliquot (10 microl) of Triton X-100 (0.1%, v/v) was added to each 200 microl of (1+1) diluted urine (or 20 microl of the Triton X-100 was added to each 500 microl of (1+4) diluted serum) sample. After the diluted sample mixture (10 microl) was introduced into a graphite cuvette, the corresponding chemical modifier (10 microl, containing Ni2+ + Pd + NH4NO3 in HNO3 (0.2%, v/v)) was added to it and the mixture was atomized. The MDL (3sigma) for Se in urine and in serum was 4.4 and 21.4 microg/l in undiluted sample, respectively, and the calibration graphs were linear up to 150 and 400 microg/l. Accuracies of urine Se were 98.9 - 99.4% by testing SRM 2670 (NIST) urine standards with RSD (between-run, n = 3) within 2.9%; and that of serum Se was 97.2% when testing a certified second-generation human serum (No. 29, #664) with RSD (between-run, n = 3) of 1.4%. The proposed method can be applied easily, directly, and accurately to the measurement of Be and Se in real samples (including six urine Se and four serum Se from patients of Blackfoot Disease in Taiwan).

Intracortical osteosarcoma: report of a case.

Intracortical osteosarcoma is the rarest anatomic variation of osteosarcoma. There have been only 12 cases reported in the English-language literature. We present a case of osteosarcoma in an 18-year-old Taiwanese man that originated within the cortex of the tibial diaphysis. The initial radiograph revealed a lytic mass confined to the cortex, mimicking a benign bone lesion. Histopathologic examination of the biopsy specimen showed an osteoblastic osteosarcoma mingled with some fibroblastic foci. He underwent en bloc resection, and a metallic prosthetic intercalary stem was used to replace the larger bone defect. Adjuvant chemotherapy was administered before and after the operation. He was free of disease during 40 months of follow-up. A review of all reported cases of intracortical osteosarcoma revealed that the initial method of treatment plays an important role in local recurrence and distant metastasis. Local excision and curettage leads to the worst results. The outcomes of more recently reported cases have improved because of early awareness of the possibility of malignancy and advances in chemotherapy. However, whether patients with intracortical osteosarcoma have a different prognosis from those with conventional osteosarcoma cannot be determined, because of the small number of intracortical osteosarcoma cases available for analysis.

Nonpeptide oxytocin antagonists: potent, orally bioavailable analogs of L-371,257 containing a 1-R-(pyridyl)ethyl ether terminus.

Structure-activity studies on the oxytocin antagonist 1 (L-371,257) have identified a new series of high affinity, receptor-selective OT antagonists in which the N-acetyl-4-piperidinyl ether terminus in 1 has been replaced with a 1-(aryl)ethoxy group.

Ultrasonographic findings of papillary thyroid carcinoma and their relation to pathologic changes.

To elucidate the ultrasonographic findings in papillary thyroid carcinoma and their relation to pathologic changes, 47 patients on whom thyroid ultrasonography had been performed and whose thyroid tissues were pathologically proven to be papillary thyroid carcinoma were studied. The ultrasonographic findings were reviewed and correlated with the pathologic changes. Fifty-three out of 94 thyroid lobes had papillary carcinoma. The ultrasonographic features of the affected thyroid lobes were hypoechoic in 46, isoechoic in 6 and hyperechoic in 1. The ultrasonographic texture of all 53 lesions was heterogeneous. The margin was clear in 11 lesions and unclear in 42. Cystic degeneration was found in 15 lesions. Discrete particles representative of microcalcifications were found in 25 lesions. Halo signs were found in 7 lesions. Lymph node enlargement was detected in 4 cases. The sensitivity and specificity of each ultrasonic finding in predicting the respective pathologic feature were: unclear margin for tumor invasion, 84% and 31%; cystic degeneration for cysts, 42% and 79%; discrete particles for microcalcification, 50% and 52%; halo sign for total encapsulation, 42% and 88%; and lymph node enlargement for lymph node metastasis, 18% and 100%, respectively. These data suggest that ultrasonographic findings in papillary carcinoma were usually hypoechoic and heterogeneous. An unclear margin in sonography is fairly sensitive for the prediction of tumor invasion.

Pleural non-Hodgkin's lymphoma developing from long-standing pyothorax: report of a case.

A 47-year-old Taiwanese man with a long-standing history of left chronic tuberculous empyema complained of having a painful swelling over the left lower chest wall for a four-month period. Suspecting acute exaggeration of infection, open drainage was performed. Multiple yellow-grayish, fragile substances protruding from the calcified empyema wall were found. Pathologic studies revealed a diffuse large B-cell non-Hodgkin's lymphoma and removal of the tumors en bloc, with the empyema peel and part of the chest wall, was performed two weeks later. The postoperative course was smooth, but the patient refused further chemotherapy. He was well, without local recurrence or metastasis after nine months of follow-up. Malignant lymphoma developing from long-standing pyothorax is very rare, and has been reported only in Japanese series. The relationship between chronic pyothorax and the subsequent development of malignant lymphoma is discussed and the literature is reviewed.

Structure elucidation of ficellomycin.

The structure of ficellomycin, an antibiotic previously discovered by Argoudelis et al., is elucidated as valyl-2-[4-guanidyl-1-azabicyclo[3.1.0]hexan-2-yl]glycine (1) by NMR, MS, and derivatization studies. The 1-azabicyclo[3.1.0]hexane moiety in 1 represents an unusual ring system making ficellomycin a unique natural product compound.