BACKGROUND AND PURPOSE: This study evaluates the treatment results of patients with dermatofibrosarcoma protuberans. PATIENTS AND METHODS: Between August 1987 and July 1998, 35 consecutive patients with pathologically proved dermatofibrosarcoma protuberans received surgery with or without radiation therapy. Their treatment results were analyzed retrospectively. RESULTS: The patient ages ranged from 5 to 67 years (median 37 years). There were 24 males and 11 females. The anatomic sites of tumor were: trunk in 21, extremity in eight, and head and neck region in six. The maximal dimension of tumor ranged from 1.5 to 25 cm. Surgery techniques included local excision and wide excision with or without graft or flap. Adjuvant radiation therapy was given to 11 patients, with a dose ranging from 46 to 68 Gy (one pre-operative, ten post-operative). At a median follow-up of 50 months (range 11-131 months), there were 11 patients (nine patients without radiation therapy) who developed local failure. Salvage therapy (excision with or without radiation therapy) was given to all of them, and ten achieved disease control. Some patients had treatment-related moderate cosmetic or functional problems. CONCLUSIONS: Dermatofibrosarcoma protuberans is a malignancy of a high cure rate, and adjuvant radiation therapy can reliably decrease the local recurrence rate and prevent mutilation and functional deficit caused by repeated surgery.

Adolescent↗

PubMed2000

The role of radical surgery followed by adjuvant therapy for high-risk early-stage cervical carcinoma patients with pelvic lymph node metastasis.

OBJECTIVES: To identify a subgroup of high-risk node-positive patients in early-stage cervical cancers and to determine the role of radical hysterectomy followed by adjuvant therapy to these patients. STUDY DESIGN: We conducted a retrospective review of 482 surgically-treated patients of clinical stage Ib and IIa cervical carcinoma from July 1986 to December 1994 at Kaohsiung Chang Gung Memorial Hospital. Of these, 96 patients had pelvic lymph node metastases. Clinicopathological variables, including the level of pretreatment squamous cell carcinoma antigen (SCC-Ag), DNA flow cytometry analysis, and the use of different adjuvant therapies were studied. RESULTS: Disease-free survival was significantly worse among patients with S-phase fraction greater than 20% and pretreatment SCC-Ag level above 5 ng/ml. Utilizing these significant variables, we identified two distinct risk groups. Those patients without any of the risk variables were categorized as the low-risk group. Those patients with either one or both risk variables were categorized as the high-risk group. Five-year disease-free survival rates were 74% in the low-risk group and 43% in the high-risk group, (P=0.034). Disease recurred in 30.2% of the low-risk patients and 45. 3% of the high-risk patients. No survival advantages were found by using different adjuvant therapies. CONCLUSIONS: Radical hysterectomy should not be attempted if either the pretreatment SCC-Ag level is above 5 ng/ml or S-phase fraction of the tumor greater than 20% due to its limited value despite applying aggressive postoperative adjuvant therapy.

Adenocarcinoma↗

PubMed2000

Survey of Spirometra erinaceieuropaei in frogs in Taiwan and its experimental infection in cats.

Eighteen of 56 (32.1%) wild Rana limnocharis from central and south Taiwan were found to contain plerocercoids of Spirometra erinaceieuropaei. This is the first report of S. erinaceieuropaei infections in frogs in Taiwan, with the plerocercoids being recovered from the thigh and back muscles or under the skin. Other species of frogs examined, including nine wild R. latouchii, one wild Buergeria robustus and 110 cultured R. rugulosa were free of infection. The plerocercoids were orally inoculated into four cats; three of which were each given a single plerocercoid and one a dose of three plerocercoids. Daily faecal examination showed that two cats started shedding eggs of S. erinaceieuropaei on day 8 postinfection (PI) and the other two on day 10 PI. The highest eggs per gram and eggs per day for a single worm was found to be 428,000 and 14,416,000 respectively. Only the cat inoculated with three plerocercoids shed proglottids in its faeces during the 2 month observation period.

Animals↗

PubMed2000

Temporal reference in Chinese mother-child conversation: morphosyntactic, semantic and discourse-pragmatic resources.

This paper explores two Mandarin-speaking children's (3;2 and 3;3) ability to refer to the past in mother-child conversation. The approach encompasses morphosyntactic, semantic and discourse-pragmatic perspectives. The results show that the children tend to refer to immediate past spontaneously, but rely heavily on elicitation when referring to earlier past. It is suggested that maternal scaffolding functions as a discourse support for children to participate in conversation involving earlier past. When establishing past reference with overt temporal markers, the children resort mainly to aspect markers. In addition, they also rely on semantic and discourse-pragmatic resources for temporal inferencing, such as inherent semantic aspect, shared background knowledge and situational context.

Child, Preschool↗

PubMed2000

Correlation of neurological manifestations and MR images in a patient with Wilson's disease after liver transplantation.

Orthotopic liver transplantation (OLT) has been applied to patients with Wilson's disease (WD) for correction of irreversible liver cirrhosis. However, the neurological outcome and the correlation between clinical manifestations and neuroimage findings after OLT remain uncertain. We present a WD patient who showed an improvement in both liver functions and neurological manifestations after OLT. Serum levels of ceruloplasmin and copper returned to normal rapidly after the operation. His ataxic gait was improved 5 months later and dysmetria and tremor disappeared 11 months later. The high signal intensities on T2-weighted brain magnetic resonance images regressed at bilateral thalami 5 months later and disappeared in bilateral thalami and red nuclei 16 months after OLT. We conclude that the neurological improvement could be expected in WD patients after OLT. The improvement was correlated with the MRI changes in red nuclei and bilateral thalami.

Adult↗

PubMed2000

The role of pretreatment squamous cell carcinoma antigen in predicting nodal metastasis in early stage cervical cancer.

PURPOSE: To evaluate whether the presence of pelvic lymph node metastasis can be predicted by pretreatment squamous cell carcinoma antigen (SCC-Ag) levels in early stage squamous cervical carcinoma. MATERIALS AND METHODS: Between 1994 and 1998, 284 patients with stage Ib and IIa cervical squamous cell carcinoma undergoing radical hysterectomy had preoperative SCC-Ag determination. The correlation between clinicopathological findings on SCC-Ag levels were examined. The Mann-Whitney U test was used to statistically analyze differences between node positive and negative patients. Multiple regression analysis and a multiple logistic model were employed to examine the effect of clinicopathological findings on SCC-Ag levels. RESULTS: Of the 284 patients, 56 patients were found to have nodal metastasis. Median serum levels and 90% ranges of SCC-Ag were 0.74 microg/l (0.5-7.8) in the 228 nodal negative patients and 4.33 microg/l (0.5-48.5) in the 56 nodal positive patients (p<0.001). Lymph node metastasis and tumor size were found to have a significant impact on SCC-Ag levels. Around 86% of the patients with SCC-Ag levels below 8 microg/l showed no nodal metastasis, while about 65% of the patients with serum levels above 8 microg/l exhibited nodal metastasis. Multivariate analyses confirmed that only lymph node metastasis had a significant impact on the SCC-Ag levels exceeding 8 microg/l. CONCLUSION: For predicting nodal metastasis preoperatively, SCC-Ag levels greater than 8 microg/ l can be considered a high-risk zone for nodal metastasis.

Antigens, Neoplasm↗

PubMed2000

Characterization and mapping of resistance to Oidium lycopersicum in two Lycopersicon hirsutum accessions: evidence for close linkage of two Ol-genes on chromosome 6 of tomato.

The cultivated tomato is susceptible to powdery mildew (Oidium lycopersicum). Several accessions of wild species are resistant. In this study we describe (i) the genetics and mapping of resistance to O. lycopersicum in G1.1290, one of the resistant accessions in Lycopersicon hirsutum, (ii) fine mapping of Ol-1 originated from L. hirsutum G1.1560, another resistant accession of L. hirsutum, and (iii) tests of allelism for resistance in G1.1290 and G1.1560. Initially, it is demonstrated that the resistance in G1.1290 to O. lycopersicum is controlled by an incompletely dominant gene, designated Ol-3. By using an advanced breeding line (ABL) containing introgression fragment(s) from G1.1290, Ol-3 was found to be associated with several RFLP and SCAR markers on chromosome 6. By using these markers, Ol-3 was mapped between markers TG25/SCAF10 and H9A11 on chromosome 6. Secondly, after testing some F3 lines and their progenies from the cross between L. esculentum cv Moneymaker and L. hirsutum G1.1560, we provided more evidence for the map position of Ol-1 to lie between SCAF10 and H9A11, indicating that Ol-1 and Ol-3 are in the same chromosome region. Thirdly, although allelism tests could not discriminate between Ol-1 and Ol-3, (indirect) evidence suggested that these two genes are not identical. They might instead represent functional genes of a cluster of Ol-homologues.

Alleles↗

PubMed2000

Hypokalemic muscular paralysis causing acute respiratory failure due to rhabdomyolysis with renal tubular acidosis in a chronic glue sniffer.

CASE REPORT: A 34-year-old male was admitted to the emergency department with the development of quadriparesis and respiratory failure due to hypokalemia after prolonged glue sniffing. The patient was subsequently given mechanical ventilatory support for respiratory failure. He was weaned from the ventilator 4 days later after potassium replacement. Toluene is an aromatic hydrocarbon found in glues, cements, and solvents. It is known to be toxic to the nervous system, hematopoietic system, and causes acid-base and electrolyte disorders. Acute respiratory failure with hypokalemia and rhabdomyolysis with acute renal failure should be considered as potential events in a protracted glue sniffing.

Acidosis, Renal Tubular↗

PubMed2000

Unusual presentation of mesangial proliferative glomerulonephritis in HELLP syndrome associated with acute renal failure.

Acute renal failure in pregnancy is not common in industrialized countries. HELLP syndrome (hemolysis, elevated liver enzyme, and low platelets) was one of the causes of acute renal failure in pregnancy, but renal pathological findings in case of acute renal failure had rarely been reported. We reported an unusual case of HELLP syndrome with acute renal failure requiring renal replacement therapy and which histopathologic findings of kidney biopsy showed mesangial proliferative glomerulonephritis and her renal function completely recovered after immediate artificial abortion, supportive management, transfusion of blood products, and hemodialysis.

Abortion, Therapeutic↗

PubMed2000

Continuous arteriovenous hemodiafiltration in the acute treatment of hyperammonaemia due to ornithine transcarbamylase deficiency.

BACKGROUND: Acute hyperammonemia caused by urea cycle disorder is a medical emergency for which immediate managements should be taken to minimize permanent brain damage. Among different enzyme defects, ornithine transcarbamylase deficiency (OTC) is one of the most common enzyme defect in urea cycle disorders. We utilized continuous renal replacement therapy techniques in the acute treatment of hyperammonemia due to ornithine transcarbamylase deficiency. PATIENTS AND METHODS: Three male neonates with elevated serum ammonia levels were shown, based on urine organic acid analysis and serum amino acid studies, to have OTC deficiency. Administration of sodium benzoate and sodium phenylacetate for activating alternative nitrogen waste pathway were used associated with protein restriction. Other modalities, including blood exchange transfusion, peritoneal dialysis, continuous renal replacement therapy were utilized in an attempt to lower serum ammonia concentration. RESULTS: We report the successful use of continuous arteriovenous hemofiltration (CAVH), continuous arteriovenous hemodialysis (CAVHD), continuous arteriovenous hemodiafiltration (CAVHDF) in the acute management of hyperammonemia due to OTC deficiency. We also compared the ammonia clearance between peritoneal dialysis, exchange transfusion, CAVH, CAVHD and CAVHDF. It demonstrated the evidence that CAVHDF provides the best ammonia clearance. CONCLUSION: Continuous renal replacement therapy including CAVH, CAVHD, and CAVHDF may be the alternative techniques for acute management of hyperammonemia in inborn error of metabolism when dialysis machine is not available. Our data suggests CAVHDF provides the best ammonia clearance.

Hemodiafiltration↗

PubMed2000

Severe hypercalcaemia with normal serum calcitriol in a diabetic patient with chronic renal failure, autoimmune hepatitis and disseminated tuberculosis.

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Arthritis, Gouty↗

PubMed2000

Spontaneous variability of cardiac output in ventilated critically ill patients.

OBJECTIVE: To define the magnitude of spontaneous cardiac output variability over time in sedated medical intensive care unit patients attached to a continuous cardiac output monitor, and to determine whether high level positive end-expiratory pressure or inverse inspiratory-to-expiratory (I:E) ratio ventilation resulted in greater variability over time than low positive end-expiratory pressure with conventional I:E ratio ventilation. DESIGN: Prospective study. SETTING: Medical intensive care unit in a tertiary medical center. PATIENTS: A total of 22 hemodynamically stable acute respiratory failure patients with a pulmonary artery catheter inserted for hemodynamic monitoring INTERVENTIONS: After being sedated, patients were randomized ultimately to receive pressure control ventilation first at setting A (high positive end-expiratory pressure [15 cm H2O] with conventional I:E ratio [1:2]) and then at setting B (low positive end-expiratory pressure [5 cm H2O] with inverse I:E ratio [2:1]), or vice versa, and then at setting C (low positive end-expiratory pressure [5 cm H2O] with conventional I:E ratio [1:2]). Each ventilation setting period lasted 1 hr. MEASUREMENTS AND MAIN RESULTS: Cardiac output (CO) was measured continuously. The continuous CO value displayed was updated every 30-60 secs. The updated value reflected an average of the previous 3-6 mins. The coefficient of variation (CV) of CO for each setting in each patient was calculated to represent the spontaneous variability. The mean CO+/-SD and CV of each setting was 5.7+/-1.8 L/min and 4.4% for setting A, 5.6+/-1.5 L/min and 4.6% for setting B, and 5.9+/-1.7 L/min and 4.8% for setting C. Analysis of variance revealed no significant differences between the CVs of the three settings. The 95% confidence interval for the COs for each setting was approximately the mean CO+/-0.1 x mean CO measured. CONCLUSIONS: In critically ill sedated medical intensive care unit patients with stable hemodynamics, the spontaneous variability of cardiac output over time was not significant. High positive end-expiratory pressure (15 cm H2O) and inverse ratio ventilation (2:1) did not contribute to increased spontaneous variability of cardiac output.

APACHE↗