PubMed Health⌕ Search

Biomedical subjects

S F Huang

Publications and source records attributed to S F Huang.

At least 55 records · Page 3Linked to original sources
PubMed1996

Segmental defect of the intestinal musculature of a newborn: evidence of acquired pathogenesis.

Two cases of premature newborns with a segmental defect of the intestinal musculature are presented. The clinical diagnoses were intestinal obstruction and perforation, respectively. Emergency laparotomy was performed. In the first case, this showed a dilated loop of ileum and proximally dilated small bowel. The second case had ileal perforation with marked pneumoperitoneum. Pathological examination showed multifocal partial or complete absence of the muscularis propria, with relative preservation of the remaining components of the bowel wall in both cases. In addition, foci of recent muscular necrosis was noted in case 1, and early replacement fibrosis in case 2. These findings suggest that the muscle defect was secondary to muscle injury rather than a primary absence of muscle. The different theories of pathogenesis are discussed.

Anastomosis, Surgical↗
PubMed1996

Left common carotid artery arising from the pulmonary artery in a patient with DiGeorge syndrome.

A female infant, born at 33 weeks' gestation with tetralogy of Fallot, died of severe perinatal asphyxia 6 hours after birth. Necropsy disclosed two associated vascular anomalies: a right aortic arch with a left common carotid artery arising from the pulmonary artery (isolated left common carotid artery) and an aberrant left subclavian artery arising from the descending aorta. Agenesis of the thymus and parathyroid gland was also found, suggesting that the child also had DiGeorge syndrome. Origin of the left common carotid artery from the pulmonary artery is exceedingly rare. When planning surgical treatment it is important to be aware of the possibility of this anomaly occurring in association with congenital heart disease, particularly in the presence of tetralogy of Fallot, right sided aortic arch, or DiGeorge syndrome.

Carotid Artery, Common↗
PubMed1996

Malignant chondroid syringoma.

A case of malignant chondroid syringoma on a foot with multiple bone metastases is presented. An 18-year-old male patient first noticed a protruding mass on the plantar surface of his right foot at 9 years of age. A sweat gland tumor of a benign nature was diagnosed and excised at that time. The tumor recurred three times during the 10 years after surgery and was finally diagnosed as malignant chondroid syringoma. Multiple bone metastases involving the calcaneus, talus and fibula of the lesion side were found after extensive radiologic survey. The patient underwent below-knee amputation with total removal of the fibula. However, pelvic bone metastasis developed 1 year after the amputation. He died of this disease due to brain and diffuse bony metastasis 36 months after the amputation. This is a rare case of malignant chondroid syringoma with a long history but ominous outcome. We recommend that sweat gland tumors be carefully examined and treated more radically when there is a suspicion of malignancy.

Adenoma, Pleomorphic↗
PubMed1996

Fluorescence in situ hybridization evaluation of chromosome deletion patterns in prostate cancer.

Various nonrandom chromosomal aberrations have been identified in prostate carcinoma. These aberrations include deletions of several chromosome regions, particularly the chromosome 8 short arm. Large-scale numerical aberrations, reflected in aberrant DNA ploidy, are also found in a minority of cases. However, it is unclear whether prostate carcinomas contain aberrations of certain chromosome regions that are deleted frequently in other common types of cancer. In this study, we performed dual-color fluorescence in situ hybridization on intact nuclei from touch preparations of 16 prostate cancers. Chromosome copy number was determined using pericentromeric probes, whereas potential chromosome arm deletions were evaluated using yeast artificial chromosome (YAC) and P1 probes. Two YAC probes targeted chromosome 8 short arm regions known to be deleted frequently in prostate cancer. Other YACs and P1s were for chromosome regions, including 1p22, 3p14, 6q21, 9p21, and 22q12, that are deletion targets in a variety of cancers although not extensively studied in prostate cancer. Hybridization efficiencies and signal intensities were excellent for both repeat sequence (alpha-satellite) and single, copy (YAC and P1) fluorescence in situ hybridization probes. Of 16 prostate cancers, 11 had clonal aberrations of 1 or more of the 13 chromosome regions evaluated, and 10 cases (62.5%) had 8p deletions, including 4 cases with 8p deletion in virtually all cells and aneuploidy in only a subset of those deleted cells. Deletions at 3p14, 6q21, and 22q12 were identified in 2, 1, and 1 case, respectively, and each of those cases had a similarly sized cell population with 8p deletion. These studies confirm 8p deletion in the majority of prostate carcinomas. 8p deletions appear to be early events in prostate tumorigenesis, often antedating aneuploidy. Fluorescence in situ hybridization strategies incorporating pericentromeric and single-copy regional chromosome probes offer a powerful and efficient means for determining frequency and progression of oncogenetic events in prostate cancer.

Carcinoma↗
PubMed1995

Abnormal splenic and thymic IL-4 and TNF-alpha expression in MRL-lpr/lpr mice.

The MRL-lpr/lpr and MRL-(++) mice were studied for the expression of cytokines in the spleen, lymph node, thymus, kidney and brain through the reverse transcription-polymerase chain reaction (RT-PCR). The frequencies of IL-4 and TNF-alpha expression in the thymus and spleen were significantly higher in MRL-lpr/lpr mice than in MRL-(++) mice from the age of 17 to 32 weeks. More importantly, IL-4 transcript was demonstrated in the early rather than in the terminal stage of the lupus disease. At the 20th week, MRL-lpr/lpr mice with active disease exhibited higher concentrations of IL-1 alpha, IL-6 and TNF-alpha in serum than MRL-(++) mice. Interestingly, in MRL-lpr/lpr but not MRL-(++) mice, the IL-6 concentration in cultured supernatants of the thymic cells was significantly higher than that of the splenic or lymph node cells. On the other hand, IL-6 and IL-1 beta were expressed in the brain and kidney of MRL-lpr/lpr mice but not of MRL-(++) mice. Cultured MRL-lpr/lpr mesangial cells could also express IL-6 but to a lesser extent. These results suggest that the abnormal splenic and thymic IL-4 and TNF-alpha expression may predispose the development of autoimmune reactions. The expression of IL-1 beta and IL-6 in the brain and kidney may be implicated in the damage of these two organs in MRL-lpr/lpr mice.

Animals↗
PubMed1995

Ring chromosomes in dermatofibrosarcoma protuberans are composed of interspersed sequences from chromosomes 17 and 22.

Ring chromosomes are found in most dermatofibrosarcoma protuberans (DFSPs), and recent reports demonstrate that portions of the DFSP ring chromosomes derive from chromosome 17. In this study we characterized ring chromosomes in three DFSPs using a combined approach of karyotyping, chromosome painting, and comparative genomic hybridization. Chromosome painting demonstrated that the ring chromosomes in each DFSP were composed of discontinuous, interwoven sequences from chromosomes 17 and 22. Amplification of chromosomes 17 and 22 sequences was confirmed in each of these cases by comparative genomic hybridization, and over-representation of chromosomes 17 and 22 sequences was also demonstrated by comparative genomic hybridization in 1 of 2 cytogenetically unremarkable DFSPs. We conclude that amplification of chromosomes 17 and 22 sequences, in ring form, is a characteristic aberration in DFSP.

Adult↗
PubMed1995

[Current clinical approaches and gene mutation study of beta-thalassemia major].

Forty-one cases of beta-thalassemia major were assessed for their clinical manifestations and gene mutation. The age distribution was from 1 to 18 years old. Patient's initial clinical symptoms began mostly before 2 years of age (90.2%). Patient's initial hematological data included mean hemoglobin value, 5.8 +/- 1.2 gm/dl, hemoglobin F value, 85.0 +/- 12.1%, hemoglobin A2 value 2.3 +/- 1.8%, reticulocyte count 9.2 +/- 9.0%. Eight different point mutations were characterized. Of these mutations, C to T substitution at nucleotide (nt) 654 of intervening sequence (IVS) 2, accounting for 46.3% of mutant beta-globin genes, is the most common mutation in our series, followed by frameshift codons 41/42 with a four nucleotides (TCTT) deletion for 31.7%; A to G substitution at position -28 of the promotor area for 8.5%; A to T substitution at codon 17 for 6.1%; frameshift codons 27/28 (insertion of C) for 2.4%; G to T substitution at nucleotide 1 of IVS-1 for 2.4%; frameshift codons 71/72 (insertion of A) and IVS-1 3' end TAG-->GAG for 1.2%. The first four mutations account for 92.6% of all beta-globin gene mutations in our series. As to mutations in each individual, the incidence of compound heterozygotes of two different mutations is much higher than homozygotes of a single mutation, 78.0% vs. 22.0%. Compound heterozygotes of C to T substitution at nt 654 of IVS-2 and frameshift codons 41/42 with a four nucleotides deletion is the most common pattern of beta-thalassemia mutation in our patients (41.5%). Patients with beta(0)/-28 beta(+) compound heterozygote mutation had much delayed initial symptoms than beta (0)/beta(0) homozygote mutation, but clinical manifestation may be aggravated when the mutation combined with glucose-6-phosphate dehydrogenase deficiency. Severity of iron overload was significantly correlated with total transfusion amount and patient's age in simple regression analysis (p < 0.001). Splenectomy may effectively prolong transfusion interval, maintain higher hemoglobin level before each transfusion and palliate clinical symptoms (p < 0.01). Iron-chelating agent therapy can effectively lower the total amount of serum ferritin. Higher severity of iron overload correlates with higher incidence of EKG and cardiac abnormalities in patients with beta-thalassemia major.

Adolescent↗
PubMed1995

Density of muscarinic receptors in rat myocardium during early sepsis.

The muscarinic receptor changes in two subcellular fractions of rat myocardium during sepsis, the sarcolemma (SL) and light vesicles (LV), were studied. [3H]-quinuclidinyl benzilate ([3H]-QNB) was used as a radioligand. Sepsis was induced by cecal ligation and puncture (CLP). The septic rats had higher pulse rates and slightly higher blood glucose levels than control rats. The marker enzyme assays revealed that the SL fraction was enriched with 5'-nucleotidase and the Na(+)-K(+)-ATPase activity increased over 20-fold, while the LV fraction showed very little enrichment when compared with the homogenate. [3H]-QNB binding studies showed that Bmax increased by 58.8% in SL with no changes in LV during early sepsis (9 h post-CLP), but there was no significant change in the Kd value. These data indicate that muscarinic cholinergic receptors in rat heart SL increase during early sepsis. Since the muscarinic cholinergic receptors mediate parasympathetic modulation of myocardial contractility, changes in the number of muscarinic receptors in the cardiac SL may have a pathophysiologic significance in the development of hemodynamic changes during sepsis.

Animals↗
PubMed1994

Prenatal diagnosis of X-linked hydrocephalus in a Chinese family with four successive affected pregnancies.

We report on a woman with four successive pregnancies affected with X-linked hydrocephalus (XLH). The first child had prenatal craniocentesis and died in utero. The second child had a postnatal shunting operation, but suffers from severe growth and mental retardation at 5 years of age. In the third pregnancy, prenatal ultrasound detected hydrocephalus at the 16th and 20th weeks of gestation and the pregnancy was terminated. In the fourth pregnancy, ultrasound scanning at the 17th and 20th weeks of gestation revealed no remarkable findings, but hydrocephalus was detected at the 24th week. Autopsy confirmed the prenatal diagnosis. DNA polymorphism analysis of the Bc1I site of exons 17-18 of factor VIII gene of the woman and her last two fetuses seemed to be compatible with a linkage between the XLH locus and factor VIII gene. Although XLH has a variable presentation of ventriculomegaly, ultrasound scanning is still a useful tool for prenatal diagnosis at present. Earlier and more accurate prenatal diagnosis will be feasible with molecular analysis of the XLH locus or its flanking regions.

Adult↗
PubMed1994

Infantile endodermal sinus tumor presenting with vaginal bleeding: report of a case.

Endodermal sinus tumor (EST) of the vagina is extremely rare and primarily affects infants. We report on a six-month-old female infant with EST of the vagina who presented with vaginal spotting of one month's duration. Pelvic ultrasound and computerized tomography showed a 3.8 x 3.5 cm heterogeneous mass between the bladder and the rectum. The serum alpha-fetoprotein (AFP) level was high (1270 ng/mL) and the beta-human chorionic gonadotropin was undetectable. She received surgical intervention followed by chemotherapy. The patient was disease-free and serum AFP remained undetectable during the eight-month follow-up period.

Endodermal Sinus Tumor↗
PubMed1994

Persistent hyperinsulinemic hypoglycemia due to nesidiodysplasia in a neonate.

A large, one-hour-old female baby was noted to have hypoglycemia immediately after birth. Although early feeding and then intravenous glucose infusion with a dose gradually increased to 30 mg/kg/min were given, the baby still had recurrent hypoglycemia 14 days after birth. High serum insulin/glucose ratios (> 2.5) were noted twice during hypoglycemic episodes. At the same time, the baby's serum growth hormone, thyroid hormone and cortisol levels were within normal ranges. Hyperinsulinism due to nesidiodysplasia was diagnosed and a near-total pancreatectomy was performed at the age of 14 days. After the operation, euglycemia was achieved and there was no episode of hypoglycemia even after fasting for eight hours. Our experience confirms that surgery is the treatment of choice for patients, including neonates, with hyperinsulinemic hypoglycemia.

Female↗
PubMed1994

Hypomelanosis of Ito associated with West syndrome: report of a case.

Hypomelanosis of Ito (HI) is a rare neurocutaneous disorder characterized by hypopigmented macules along the Blaschko line and multiple abnormalities, including seizures, developmental retardation, ophthalmologic defects, and chromosome mosaicism. We report a four-month-old female baby with typical skin lesions on the right upper limb, chest, and scalp, noted at the age of one week. She also had a seizure disorder which manifested as neck flexion and hand gripping with series formation from the age of three months. Electroencephalogram (EEG) demonstrated modified hypsarrhythmia. The characteristic clinical and electroencephalographic features favored the diagnosis of West syndrome. Magnetic resonance imaging revealed an absence of abnormal neuronal migration. The auditory brainstem evoked response, nerve conduction velocity, and ophthalmologic examinations were all within normal limits. The seizure responded to adrenocorticotropic hormone therapy on the 12th day of initial therapy, and the EEG became normal. However, another different pattern of seizures characterized by staring and lateral gaze in a right upward direction occurred two weeks later. Carbamazepine and phenobarbital were added to the therapeutic regimen. She was regularly followed up at the outpatient department every two weeks after one-and-a-half months of hospitalization. Infantile spasms with evidence of hypsarrhythmias in the EEG recurred at seven months of age. Oral steroid therapy reduced seizure frequency by 80%. As far as we know, this is the first case report of HI combined with infantile spasms in Taiwan.

Female↗
PubMed1994

Changes of alpha 1-adrenergic receptors in human liver during intraabdominal sepsis.

We studied changes in alpha 1-adrenergic receptors in human liver plasma membranes during intraabdominal sepsis using [3H]prazosin as a radioligand. Human liver tissues were obtained from nonseptic patients undergoing elective abdominal surgery (control group) and from patients with sepsis requiring laparotomy as a therapeutic measure (septic group). Septic patients were further divided into three subgroups on the basis of septic severity scores: mild sepsis (< 21), moderate sepsis (22 to 32) and severe sepsis (> 33). Plasma membranes were prepared by means of sucrose gradient centrifugation and were purified fivefold on the basis of the enrichment of the activity of the marker enzyme, 5'-nucleotidase. [3H]prazosin-binding studies show that the maximal binding capacity was increased by 49.6% (p < 0.01) in mild sepsis, relatively unchanged in moderate sepsis and decreased by 33.4% (p < 0.05) in severe sepsis (in femtomoles per milligram: 193.7 +/- 5.7 for control [n = 6], 289.8 +/- 23.4 for mild sepsis [n = 4], 192.3 +/- 16.3 for moderate sepsis (n = 4), 129.1 +/- 18.3 for severe sepsis [n = 5]). We found a significant inverse correlation (r = 0.85, p < 0.01) between changes in the densities of alpha 1-adrenergic receptors and septic severity scores. These data indicate that alpha 1-adrenergic receptors in human liver plasma membranes undergo dynamic changes during the development of sepsis--that is, the receptor number increased in mild sepsis, returned to a normal level in moderate sepsis and finally decreased in severe sepsis.(ABSTRACT TRUNCATED AT 250 WORDS)

5'-Nucleotidase↗
PubMed1993

Dermatophyte pseudomycetoma: a case report.

A 25-year-old man presented with several prominent subcutaneous masses in the occipital region of the scalp. He had a long history of tinea capitis and tinea corporis infection. Histopathology of the occipital lesions showed mycelial aggregates in the deep dermis and subcutis. Cultures of the excised material and superficial scales grew a fungus identified as Microsporum ferrugineum. We propose the term 'dermatophyte pseudomycetoma' to describe this distinctive mycosis.

Adult↗
PubMed1993

Intrauterine death of co-twin in the third trimester: a case report of twin-to-twin transfusion syndrome and cord accident.

Intrauterine death of one twin in a monochorionic twin pregnancy is associated with increased mortality and morbidity of the survivor. The exact cause is usually difficult to determine even after delivery. The choice of management for the mother and the viable co-twin is difficult. We report on a case of intrauterine death of one twin with monochorionic placentation. Twin-to-twin transfusion syndrome was suggested by the sonographic findings and evidence of a vascular communication. The co-twin survived for another 39 days without complications after the diagnosis of fetal death. Postmortem examination showed severe torsion and constriction of the umbilical cord at the fetal end with focal deficiency of Wharton's jelly. It is likely that torsion and constriction of the cord was responsible for the one intrauterine fetal death; this so-called "twist amputation" of the cord, on the other hand, contributed to the favorable outcome in the surviving co-twin.

Adult↗