bluuebunny/crossref_metadata_2025_split huggingface dataset:
Spectrum of renal osteodystrophy in children on continuous ambulatory peritoneal dialysis
Authors: Yalçinkaya Fatoş , Ince Erdal , Tümer Necmiye , Ensari Arzu , Özkaya Nuray 
Published: March 2003

Abstract Background: The prevalence of different types of bone disease in chronic renal failure (CRF) has changed significantly during the last decade. The aim of the present study is to evaluate the spectrum of bone disease in children with CRF undergoing continuous ambulatory peritoneal dialysis (CAPD).
Methods
: Seventeen children with CRF on CAPD aged 7–20 years were evaluated. All patients had received regular vitamin D and calcium carbonate therapy during the 6 months preceding the bone biopsy. Serum calcium, phosphate, alkaline phosphatase and immunoreactive parathyroid hormone (iPTH) levels were measured and hand X‐rays were performed. Transiliac bone biopsies were analyzed for histologic diagnosis.
Results
: High turnover renal osteodystrophy (ROD) was the most common bone disease, present in eight patients (47%). Five patients (29%) had low turnover bone disease, and four (24%) had mixed ROD. The mean age of the high turnover ROD group was higher than that of the low turnover group (14~3 vs. 11~3 years, P<0.05). Seven of the nine patients who had tubulo‐interstitial nephritis were found to have high turnover bone disease. In contrast, none of the patients with glomerulonephritis exhibited high turnover bone lesions. Mean serum calcium levels were found to be significantly higher in the low turnover group compared with the patients with high turnover bone disease (P<0.001). A serum iPTH level >200 pg/mL was 100% sensitive and 66% specific in identifying patients with high turnover ROD.
Conclusion
: The spectrum of bone disease of the children with CRF undergoing CAPD seems to depend on the rate of CRF and primary disease. The risk of developing overt hyperparathyroid bone disease is high in children with slowly progressing forms of renal pathology and especially in those with tubulo‐interstitial disease. In contrast, children with glomerular diseases who had a more rapidly progressive course may have a lesser risk of developing high turnover bone disease. The results of the present study indicate that even routinely prescribed regular vitamin D therapy early in the course of disease may lead to low turnover bone lesion in small children who have CRF due to rapidly progressive forms of renal pathology.
Evaluation by exercise testing of children with mild and moderate valvular aortic stenosis
Authors: Yilmaz Gonca , Özme Şencan , Özer Sema , Tokel Kürşat , Çeliker Alpay 
Published: March 2003

Abstract Background: The aim of the present paper was to determine the factors related to sudden death in aortic stenosis.
Methods
: The factors related to sudden death were investigated in 40 asymptomatic children with mild and moderate aortic stenosis by treadmill testing.
Results
: The QT interval of aortic stenosis cases were significantly longer than those of healthy children with increasing heart rates during exercise.
Conclusions
: A longer QT interval of aortic stenosis cases compared to normal children during exercise is the first sign of myocardial ischemia and leads to fatal ventricular arrhythmias and sudden death. For this reason we recommend that exercise testing should be performed frequently in aortic stenosis patients and that close follow up is necessary for patients with long QT segments that can be a marker for severe arrhythmias.
Echocardiographic evaluation of the ductal morphology in patients with refractoriness to lipo‐prostaglandin E1 therapy
Authors: Takeda, Nobuhiro , Hiraishi, Satoshi , Misawa, Hitoshi , Agata, Yotarou , Horiguchi, Yasunori , Fujino, Nobuyuki , Hirota, Hamao , Kawamura Seiji , Ogawa Natuko 
Published: March 2003

Abstract

Background
: Lipo‐prostaglandin (PG)E1 is effective at lower doses and has fewer side effects than PGE1‐cyclodextrin (CD). Previous studies, however, have suggested that some patients show refractoriness to lipo‐PGE1 in the course of treatment. The present paper examines: (i) whether such cases can be predicted by examining the ductal morphology before and 24 h after the start of lipo‐PGE1 infusion; and (ii) whether PGE1‐CD dilates the ductus arteriosus in patients with refractoriness to lipo‐PGE1.
Methods
: The ductal morphology was evaluated with two echo indices, such as minimal and minimal plus maximal intraluminal diameters of the ductus. Two‐dimensional echocardiography was performed in 24 patients with ductus‐dependent congenital heart disease. The two echo indices were measured before and 24 h after lipo‐PGE1 infusion and also at least twice per week until surgery.
Results
: In 19 of 24 patients, ductal patency was maintained until surgical treatment (group A). The remaining five patients (21%) showed ductal closure during the course of the lipo‐PGE1 therapy (group B). There were no significant differences between the two groups, in either the maximal or minimal diameters, which were examined before and 24 h after treatment. In the five patients of group B, lipo‐PGE1 was replaced with a relatively high dosage of PGE1‐CD (50–100 ng/kg per min), resulting in good ductal patency until surgery.
Conclusions
: Patients with refractoriness to lipo‐PGE1 therapy could not be predicted from initial intraluminal diameters of the ductus using echocardiography. Therefore, serial echocardiographic examinations are important to detect early findings of ductal closure. In addition, PGE1‐CD is still useful as back‐up therapy in such patients.
Pulmonary complications in patients with staphylococcal sepsis
Authors: Çaksen Hüseyİn , Öztürk Mustafa Kürşat , Üzüm Kazim , Yüksel Şaban , Üstünbaş Hasan Basrİ 
Published: March 2003

Abstract Background: The aim of the present study was to determine the pulmonary findings in patients with sepsis caused by Staphylococcus aureus.
Methods
: The clinical and laboratory findings of 32 cases (82%) of pulmonary involvement (secondary pneumonia) of 39 patients with sepsis caused by S. aureus were studied retrospectively. The criteria for the diagnosis of sepsis were clinical evidence of infection plus hyperthermia/hypothermia, tachycardia, tachypnea and white blood cell abnormalities. Secondary pneumonia was diagnosed in patients who presented with staphylococcal disease at one or more non‐pulmonary sites and who developed radiologic evidence of pulmonary involvement during the course of illness.
Results
: Of the 32 patients, 23 were male and nine were female; the male to female ratio was 2.5/1. The ages of the patients ranged from 2 months to 14 years (7.87~4.71 years). Bronchopneumonic infiltration was bilateral in 18 patients and unilateral in 14 patients (20 patients (62.5%) had lobar consolidation). Pleurisy was noted in 12 (37.5%) patients; it was on the right side in five patients, on the left in five patients and bilateral in two patients. In contrast, pneumatocele and pneumothorax were observed in seven (21.9%) and four (12.5%) patients, respectively. Closed chest tubes were placed through a closed thoracotomy in five children who developed dyspnea, orthopnea with imminent respiratory failure and mediastinal shift. As well as the pulmonary involvement, arthritis was noted in 13 patients, osteomyelitis in 11 patients, rash in six patients, pericarditis in five patients and renal failure in one patient. Staphylococcus aureus was isolated from blood culture in all except for seven cases. While S. aureus was isolated from blood culture in all of the 12 patients with pleurisy, it was isolated from pleural fluid in only two (16.6%) patients. Six of 32 patients died; the mortality rate was 18.75%.
Conclusions
: It was found that the rate of pulmonary involvement was as high as 82% in sepsis caused by S. aureus, and the pulmonary findings, including bronchopneumonic infiltration and lobar consolidation, were frequently seen in S. aureus pneumonia, causing a mortality rate of 18.75%.
Thrombocytopenia: An important indicator for the application of partial exchange transfusion in polycythemic newborn infants?
Authors: Acunas Betül , Çeltik Coskun , Vatansever Ülfet , Karasalihoglu Serap 
Published: March 2003

Abstract Background: The conventional therapeutic approach in polycythemic newborn infants is to apply partial exchange transfusion (PET) when hematocrit value exceeds 70% or when the infant develops symptoms with the exception of plethora.
Methods
: In order to investigate the possibility of using platelet count as a simple criterion implying the PET requirement, we retrospectively reviewed polycythemic newborn infants with respect to the relationship between thrombocytopenia and severity of symptoms, and the association of platelet count and the PET performance. Thrombocytopenia has been defined as a platelet count <150 000/μL.
Results
: We studied 18 polycythemic infants with thrombocytopenia (group 1, 35%) and 34 without it (group 2, 65%). Perinatal asphyxia, gestational toxemia and intrauterine growth retardation, which are the three common causative factors leading to polycythemia, were not significantly different in the two groups. No correlation existed between platelet counts and hematocrit values within each group, but there was a very significant difference between the two groups in terms of severity of clinical findings (P<0001); no difference in terms of moderate findings and moderately significant difference with respect to mild symptoms and asymptomatic situation (P<0.05). Partial exchange transfusion was performed in all patients in group 1, while only 12 infants in group 2 (32%) received transfusion and the difference was statistically significant (P<0.05). A significant rise in platelet counts has been achieved only in group 1, while hematocrit values decreased significantly in both groups following PET.
Conclusions
: This study emphasizes the relationship between thrombocytopenia and the severity of clinical findings and PET performance rate in polycythaemic newborn infants, implying that thrombocytopenia is a possible marker of hyperviscosity, the results of which warrant further investigation.
Six novel mutations of the fibrillin‐1 gene in Korean patients with Marfan syndrome
Authors: Oh Myung Ryurl , Kim Jung Sim , Beck Nam Seon , Yoo Han Wook , Lee Heung Jae , Kohsaka Takao , Jin Dong Kyu 
Published: March 2003

Abstract Background: Mutations in the FBN1 gene, encoding fibrillin‐1, result in Marfan syndrome (MFS). According to previous reports, the mutations in FBN1 share certain characteristics in each family with variable penetrance and overlapping symptoms, even in the same genotype. In the present study, we report six novel mutations and evaluate the clinical significance of these nucleotide changes.
Methods
: To screen for nucleotide changes in all 65 exons of the FBN1 gene in 38 unrelated Korean patients, we performed polymerase chain reaction, single‐strand conformational polymorphism (SSCP) and sequencing for the shift of the band in SSCP.
Results
: We identified six mutations: a 2253 del 7 b.p., N1043S, C1254S, L1421F, C1895R and S2662P.
Conclusions
: These results suggest that many different mutations are responsible for MFS in the Korean population.
Laparoscopic surgery in neonates and infants weighing less than 5 kg
Authors: Iwanaka Tadashi , Arai Mari , Ito Mitsuhiro , Kawashima Hiroshi , Imaizumi Satohiko 
Published: March 2003

Background
: Laparoscopic surgery in small infants is still an uncommon procedure in Japan. The present study was conducted to evaluate the advantages and disadvantages of laparoscopic surgery in neonates and infants weighing less than 5 kg.
Methods
: Between July 1997 and November 1999, 54 infants underwent laparoscopic surgery. They were evaluated for length of operation, intra‐ and postoperative complications, changes in intra‐operative body temperature, time to postoperative feeding, length of hospital stay and changes in serum levels of C‐reactive protein (CRP), creatinine phosphokinase (CPK) and interleukin (IL)‐6 on days 0, 1 and 4. These parameters in the laparoscopic pyloromyotomy (LP) and laparoscopic fundoplication groups were compared with those in the open pyloromyotomy (OP) and open fundoplication groups, respectively, which were performed during the same period.
Results
: Three laparoscopy cases were converted to open procedures. One case of fundoplication had panperitonitis due to failed gastrostomy and required long‐term parenteral nutrition. Time to postoperative feeding and length of hospital stay in the LP group were significantly shorter than in the OP group. In LP group, intra‐operative body temperature did not markedly decrease during CO2 pneumoperitoneum. Although serum levels of CRP, CPK and IL‐6 were elevated in all groups on postoperative day 1, there were no significant differences between the groups.
Conclusions
: Better quality of life after laparoscopy is a significant advantage over conventional surgical procedures. This advantage not only outweighs the incidence of intra‐ and postoperative complications in small infants, but further emphasizes the need to improve laparoscopic techniques to avoid complications.
Plasma levels of granulocyte elastase–α1‐proteinase inhibitor complex in children with hemolytic uremic syndrome caused by verotoxin‐producing Escherichia coli
Authors: Ishikawa Naoko , Kamitsuji Hidekazu , Murakami Tomohiko , Nakayama Akifumi , Umeki Yayoi 
Published: March 2003

Abstract

Background
: Activated neutrophils play an important role in the pathogenesis of renal injury in humans and in experimental models of hemolytic uremic syndrome (HUS). To evaluate the clinical significance of the circulating granulocyte elastase–α1‐proteinase inhibitor complex (GEPIC), which is a marker of neutrophil activation, we investigated the plasma concentrations of GEPIC in children with hemolytic uremic syndrome (HUS) associated with verotoxin‐producing Escherichia coli (VTEC), VTEC gastroenteritis without HUS and in normal controls.
Methods
: Of 22 children (1–19 years of age; mean age 5.5 years) with VTEC infection, nine were diagnosed with HUS. Plasma GEPIC, soluble thrombomodulin (sTM) and thrombin–antithrombin‐III complex (TAT) levels were measured by ELISA.
Results
: The number of polymorphonuclear leukocytes and the levels of plasma GEPIC in patients with HUS were significantly higher than those in non‐HUS (9850~5091 vs 5278~3327 /μL, P<0.05; 432.1~211.7 vs 188.3~117.0 ng/mL, P<0.01) or control subjects (9850~5091 vs 4728~1977 /μL, P<0.05; 432.1~211.7 vs 105.9~51.1 ng/mL, P<0.001). Furthermore, plasma GEPIC levels showed a positive correlation with sTM (r=0.522; P<0.01), a marker of endothelial cell injury, and TAT (r=0.594; P<0.01), a marker of thrombin activity.
Conclusions
: These results suggest that an increase in circulating GEPIC levels in patients with VTEC‐associated HUS may be related to endothelial injury, which may possibly lead to a more severe episode of this disease.
External beam radiation monotherapy for prostate cancer
Authors: Akakura Koichiro , Furuya Yuzo , Suzuki Hiroyoshi , Komiya Akira , Ichikawa Tomohiko , Igarashi Tatsuo , Tanaka Masashi , Murakami Shino , Ito Haruo 
Published: March 2003

Abstract
Background
: To clarify the implications and limitations of external beam radiation monotherapy for localized prostate cancer, the long‐term outcomes and prognostic factors were investigated.
Methods
: Between 1976 and 1994, 91 patients with untreated prostate cancer were treated with external beam radiation therapy alone. Thirty‐two were classified as T1b, eight were T2a, four were T2b and 47 were T3. Pelvic lymphadenectomy was carried out in 69 cases; 57 were staged as pN0, eight were pN1, four were pN2 and 22 were pNX. Linac X‐rays were used in 55 cases, fast neutron in 15 and a combination of the two in 21. No other therapy was given until relapse and when relapse was evident endocrine therapy was started.
Results
: The observation period ranged from 3 to 206 months with a median of 78 months. Local control rate and disease‐free, cause‐specific and overall survivals at 10 years were 74.0, 49.6, 74.2 and 39.2%, respectively. By univariate analysis, T category, pN category and histologic grade were significant prognostic indicators for disease‐free survival. Multivariate analysis revealed that T category was an independent prognostic factor. In T2b and T3 diseases, pN0/1 patients demonstrated significantly better disease‐free survival than pNX.
Conclusions
: A favorable long‐term outcome was achieved by external beam radiation monotherapy in patients with minimally extended prostate cancer (T1b and T2a). For locally advanced disease (T2b and T3), staging pelvic lymphadenectomy would be useful for the selection of patients.
Endocrine cell distribution and expression of tissue‐associated antigens in human female paraurethral duct: Possible clue to the origin of urethral diverticular cancer
Authors: Ogihara Sigemasa , Kato Haruaki 
Published: March 2003

Abstract
Background
: To investigate (i) what determines the histologic differences seen among female urethral diverticular cancers and (ii) the possible embryologic origin of the female paraurethral duct, we performed a distribution analysis of endocrine cells and a comparative study of tissue‐associated antigens in the female paraurethral duct.
Methods
: Six human female urethras were obtained from surgical and autopsy cases including two cases of urethral diverticular cancer (columnar/mucinous type adenocarcinoma). The urethral and paraurethral epithelia were examined histologically and immunohistochemically.
Results
: Immunoreactive endocrine cells predominated and carcinoembryonic antigen (CEA) was strongly expressed in the larger portion of the paraurethral duct close to the urethral lumen. Conversely, prostate‐specific antigen and prostatic acid phosphatase were positive only in the smaller distal duct. In two cases of adenocarcinoma including endocine cells, cancer cells were strongly positive for CEA.
Conclusions
: This study suggests that the proximal and distal parts of the paraurethral duct have different histologic characteristics and that the pathologic differences seen among female diverticular cancers may result from their cancer‐genesis from different parts of the paraurethral duct.
Preclinical Cushing’s syndrome: Report of seven cases and a review of the literature
Authors: Morioka Masaaki , Fujii Tomohiro , Matsuki Takakazu , Jo Yoshimasa , Kobayashi Tatsuya , Tanaka Hiroyoshi , Ohashi Teruhisa , Kondo Katsuyoshi 
Published: March 2003

Abstract
Background
: Adrenal adenomas showing autonomous cortisol secretion without specific endocrine symptoms are sometimes discovered in patients with adrenal incidentalomas. This entity has been described as subclinical or preclinical Cushing’s syndrome (PCS), but the endocrine data of reported cases have varied and the diagnostic criteria of PCS have been uncertain.
Methods
: We report seven Japanese cases of PCS due to a unilateral, solitary adrenal adenoma with examination of the endocrine data of these patients. The diagnostic parameters of subtle hypercortisolism and the risk of postoperative adrenal insufficiency and surgical indications are discussed and reviewed.
Results
: In the present cases, the most frequently found biochemical parameters of autonomous cortisol secretion were a low adrenocorticotropic hormone (ACTH) level (100%) and insufficient suppression of cortisol by low‐dose dexamethasone (85.7%). Unilateral accumulation of radiopharmaceuticals in tumors was also frequently observed (100%). A postoperative hydrocortisone supplement was given to six of the seven patients for 5–122 days. It was not given to case 4, because a moderate response of 11‐deoxycortisol to metyrapone was identified. Plasma ACTH levels and the diurnal rhythm of plasma cortisol rapidly recovered within 3 weeks postoperatively in six of the seven cases.
Conclusion
: This entity is heterogeneous and various degrees of cortisol excess have been observed. It should be diagnosed in the wide spectrum and the risk of adrenal insufficiency after surgery should be evaluated by dynamic tests such as the corticotropin‐releasing hormone (CRH) test. Based on the results of the present study and a review of the literature, PCS patients may not require hydrocortisone supplement therapy for a long period.
Prognostic value of tumor‐associated macrophage count in human bladder cancer
Authors: Hanada Toshikatsu , Nakagawa Masayuki , Emoto Akio , Nomura Takeo , Nasu Nobuyoshi , Nomura Yoshio 
Published: March 2003

Abstract
Background
: We determined the tumor‐associated macrophage (TAM) count to investigate its importance in predicting clinical outcome or prognosis in patients with bladder cancer.
Methods
: The TAM count and microvessel count (MVC) were determined immunohistochemically in 63 patients with bladder cancer, including 40 superficial bladder cancers and 23 invasive bladder cancers. To examine the relationship between TAM count and clinical outcome or prognosis in bladder cancer, cystectomy rates, distant metastasis rates, vascular invasion rates and 5 year survival rates were compared between patients with low (< 67) and high (≥ 67) TAM counts.
Results
: The TAM count in invasive bladder cancers (154.22 ± 11.98) was significantly higher than in superficial bladder cancers (49.05 ± 7.76; P < 0.0001). The MVC in invasive bladder cancers (71.55 ± 10.44) was also significantly higher than in superficial bladder cancers (47.02 ± 5.57; P < 0.05). There was a positive correlation between TAM count and MVC (r = 0.30; P = 0.02). Immunohistochemical staining using CD68/horseradish peroxidase monoclonal antibody showed more infiltrating cells in invasive than superficial bladder cancers. Patients with a high TAM count (≥ 67) showed significantly higher rates of cystectomy, distant metastasis and vascular invasion than those with a lower TAM count (< 67). The 5 year survival rate estimated using the Kaplan– Meier method was significantly lower in patients with a high TAM count than in those with a low TAM count (P < 0.0001).
Conclusions
: Our results suggest that determination of TAM count in bladder cancer tissues is of value to predict the clinical outcome or prognosis and to select appropriate treatment strategies in patients with bladder cancer.
Cowper's syringocele causing voiding disturbance in an adult
Authors: Awakura Yasuo , Nonomura Mitsuo , Fukuyama Takuo 
Published: March 2003

Abstract
Background
: A 21‐year‐old man presented with a sudden onset of miction pain and post‐void dribbling. The diagnosis was Cowper's syringocele due to the findings of retrograde urethrography and urethroscopy.
Methods/Results
: The patient underwent transurethral unroofing of the syringocele. The present case is of interest because Cowper's syringocele is usually diagnosed in male infants and children and is rarely found in adults.
Conclusion
: This lesion should be included in the differential diagnosis of voiding disturbances in adults.
Characterization of a newly established cell line derived from human adrenocortical carcinoma
Authors: Ueno Munehisa , Nakashima Jun , Akita Masumi , Ban Shin‐Ichi , Nakanoma Takashi , Iida Masahiro , Deguchi Nobuhiro 
Published: March 2003

Abstract Background: ACT‐1, a new cell line of human adrenocortical carcinoma, has been established and successfully maintained in culture. This study examined the biological characteristics of the cells.Methods: The tumor cells were isolated from a surgical specimen of the tumor thrombus and cultured in monolayer.Results: Histologically, the primary tumor was composed of a solid proliferation of large polygonal cells. A part of the atrophic adrenal cortex remained at the periphery of the tumor. The cultured ACT‐1 cells were spindle‐shaped in morphology and grew exponentially with an approximate population doubling time of 24 h. A chromosomal analysis revealed a modal number of 61 with consistent structural abnormalities of add(3)(q11), add(9)(p11), and add(16)(q11). The expression of 3β‐hydroxysteroid dehydrogenase was observed in the ACT‐1 cells as well as in normal human adrenal glands.Conclusions: The ACT‐1 cell line provides a reproducible model system which gives good insight into the oncogenesis of adrenocortical carcinoma.