bluuebunny/crossref_metadata_2025_split huggingface dataset:
Bexarotene – an alternative therapy for progressive cutaneous T‐cell lymphoma? First experiences
Authors: Bohmeyer J. , Stadler R. , Kremer A. , Nashan D. , Muche M. , Gellrich S. , Luger T. , Sterry W. 
Published: October 2003

Summary Background: A standard therapy for advanced cutaneous T‐cell lymphomas has not yet been defined. Bexarotene is a new retinoid x receptor‐specific retinoid that has been approved for systemic second‐line therapy for cutaneous T‐cell lymphomas in the USA and Europe. In order to evaluate the efficacy of bexarotene in cutaneous T‐cell lymphomas, a pilot trial was initiated. Patients and methods: In a pilot project 10 patients with advanced cutaneous T‐cell lymphomas, who had received a variety of previous treatments, were treated with bexarotene at the departments of dermatology in Münster, Minden and Charité Berlin, Germany. The patients received bexarotene at a dose of 300 mg/m2 body surface daily. According to the percentage of tumour reduction and affected body surface, the response rates were divided in complete and partial remission, stable disease and progressive disease. Laboratory parameters i. e. cholesterol, triglycerides transaminases, T3, T4, and TSH were screened regularly. Results: In 2 patients a short partial remission was achieved; however, after a few weeks progression followed. In 4 patients a lasting stabilisation was obtained. The other 4 patients showed a progressive disease during therapy. 6 patients developed hypertriglyceridemia with levels up to 2000 mg/dl; therapy had to be suspended in 3 patients because of these adverse drug events. Conclusion: Weighing benefits and risks, bexarotene can at present not be recommended as standard therapy in the treatment of patients with progressive cutaneous lymphomas.
Die solare Keratose: Von der Präkanzerose zum präinvasiven Plattenepithelkarzinom – Therapeutischer Ansatz nach dem Bioinduktionsverfahren
Authors: Oster‐Schmidt Claus , Altmeyer Peter , Stücker Markus 
Published: October 2003

Zusammenfassung Hintergrund: Solare Keratosen sind präkanzeröse Veränderungen chronisch UV‐geschädigter Haut, die histologisch dem präinvasiven Stadium eines Plattenepithelkarzinoms entsprechen. Als solche stellen sie eine unbedingt behandlungsbedürftige Hauterkrankung dar, um eine Progression in das invasive Stadium zu verhindern. Therapeutisch kommen sowohl medikamentöse als auch physikalisch‐ablative Methoden in Frage. Hier wird über einen neuartigen Ansatz, die Bioinduktionstherapie mit Imiquimod‐5 %‐Creme, berichtet. Patienten und Methodik: In einer prospektiven Fallserie wurden 7 Patienten mit solaren Keratosen mit Imiquimod‐5 %‐Creme behandelt. Die Creme wurde an 5 Tagen/Woche über zwei Wochen appliziert. Der Therapieerfolg wurde nach Ende der Behandlung in regelmäßigen Abständen, zum Teil auch histologisch, weiter kontrolliert. Ein weiterer unbehandelter Patient wurde als Kontrolle mitgeführt. Ergebnisse: Bei 6 der 7 behandelten Patienten heilten die solaren Keratosen vollständig ab, ein Patient reagierte nicht auf die Therapie. Bei den Patienten traten durchschnittlich 2 Jahre nach Therapieende keine weiteren solaren Keratosen auf. Bei dem Kontrollpatienten heilte die Keratose spontan ab. Als unerwünschte Wirkungen fanden sich unter Therapie Erythem, Ödem und Erosion in unterschiedlicher Ausprägung. Diese bildeten sich komplett zurück. Schlußfolgerungen: Das Konzept der Bioinduktionstherapie mit Imiquimod‐5 %‐Creme stellt einen vielversprechenden Ansatz in der Behandlung von kutanen Präkanzerosen wie der solaren Keratose dar.
Efficacy of zinc supplementation on the severity and duration of diarrhea in malnourished Turkish children
Authors: Polat Tugçin Bora , Uysalol Metin , Çetinkaya Feyzullah 
Published: October 2003

Abstract
Background
: Intervention trials have shown that zinc may be efficacious in treating acute diarrhea in children of developing countries. A double‐blind placebo‐controlled study was designed to evaluate the effects of zinc supplementation on the clinical course and duration of diarrhea in malnourished Turkish children.
Methods
: The study group comprised 40 subjects with low zinc levels (Group 1a) and 52 subjects with normal zinc levels (Group 1b). The control group was also comprised of two subgroups: 36 subjects with low zinc levels (Group 2a) and 54 subject with normal zinc levels (Group 2b). Forty‐three percent of children in the study group and 40% of controls had low serum zinc levels (<14 µmol/L), and 43% of subjects in both groups had very low serum zinc concentrations (<10 µmol/L). The study group were given 20 mg zinc per day for 10 days and the control group were given 750 mg glucose per day as a placebo for 10 days.
Results
: The mean duration of diarrhea was shorter and the percentage of children with consistent diarrhea for more than 3−7 days was lower in the study subgroups than in the control subgroups. Prolonged diarrhea was present in 12% of children in the study group, and in 44% and 37% of children in the hypozincemic and normozincemic control subgroups, respectively. The was no significant difference among the four subgroups of children in the number of cases with post‐enrollment diarrhea of a duration of>14 days. Stool frequency over the first 4 days after enrollment was lower in children in the study group.
Conclusion
: It was concluded that zinc supplementation in malnourished children with acute diarrhea may reduce the severity and duration of diarrhea, especially in children with low zinc levels.
Determinants of prognosis of acute transverse myelitis in children
Authors: Miyazawa Reiko , Ikeuchi Yuka , Tomomasa Takeshi , Ushiku Hideo , Ogawa Tetsushi , Morikawa Akihiro 
Published: October 2003

Abstract
Background
: Acute transverse myelitis (ATM) is a severe disorder; recovery requires several months and often leaves neurologic residua. To determine what features of patients with acute transverse myelitis significantly influence prognosis, the authors reviewed reports of ATM in Japanese children published in the last 15 years (from 1987 to 2001).
Methods
: The authors studied reports of 50 Japanese patients (17 boys, 26 girls, 7 children of unspecified sex; mean age ± SD, 8.0 ± 3.8 years). Acute‐phase and demographic features including age, increased deep tendon reflexes, Babinski reflex, sex, preceding infection, decreased deep tendon reflexes, time course of peak neurologic impairment, treatment with prednisolone and/or high‐dose methylprednisolone, and the day of illness when treatment was started were used as independent variables in a regression analysis. The dependent variable was long‐term persistence of neurologic deficits.
Results
: Younger patients and those without increased deep tendon reflexes or a Babinski reflex were more likely to have residual neurologic deficits such as paraplegia or tetraplegia, sensory loss and sphincter disturbance. No relationship was seen between prognosis and sex, preceding infections, decreased deep tendon reflexes, time course of peak neurologic impairment, treatment with prednisolone or high‐dose methylprednisolone, or timing of treatment initiation.
Conclusions
: Age at onset and neurologic features were important for outcome prediction in ATM. Steroid therapy did not associate with better outcome.
Somatostatin infusion withdrawal in the diagnosis of childhood growth hormone deficiency
Authors: Bosnak Mehmet , Dikici Bunyamin , Haspolat Kenan , Dogru Omer , Ozkan Ilker , Ece Aydin 
Published: October 2003

Abstract
Objective
: An evaluation of growth hormone (GH) testing for GH deficiency (GHD) in childhood is confounded by the lack of a world‐wide consensus on the definition of GHD. Although a single GH test remains the most powerful biochemical tool in the evaluation of a child with growth failure, the test remains far from ideal. Withdrawal of somatostatin (SS) infusion is followed by a rebound rise of GH thought to be mediated by endogenous GH‐releasing hormone (GHRH) function. This study was designed to compare the GH response to 90 min SS infusion in children with normal GH secretion versus children with GH deficiency.
Methods
: Ten children with GHD and 10 healthy controls (NC) have been evaluated for GH response to somatostatin infusion withdrawal (SSIW) and compared with response of two provocative tests, glucagon plus propranolol test and L‐Dopa test. All children received constant infusion of somatostatin for 90 min (3 µg/kg per h, Stilamin, Serono, Aubonne, Switzerland). In order to determine GH, blood samples were obtained 90 min before the SS infusion and 0, 15, 30, 45, 60, 75, and 90 min after the cessation of infusion.
Results
: Growth hormone peak levels with SSIW were significantly lower in GH deficient children than in healthy children (2.5 ± 1.2 ng/dL, vs 21.9 ± 5.3 ng/dL, respectively, P < 0.01). No adverse effects were observed during or after somatostatin infusion.
Conclusion
: In the present study, SSIW elicited a significant GH rise in healthy children but not in children with GH deficiency. Although further controlled studies using more data are necessary to expand these findings, the results suggested that children with GH deficiency can be reliably discriminated from healthy children by SSIW.
Serum KL‐6 level in newborns with meconium aspiration syndrome
Authors: Kurotobi Shunji , Maekawa Shu , Hara Tatsuyuki , Hamana Keiko , Inada Nahoko , Kawakami Nobuhiro , Honda Atsuko , Matsuoka Taro , Fujita Hiroshi , Nagai Toshisaburo 
Published: October 2003

Abstract
Background
: It has been reported that serum KL‐6 increases in babies with progressing chronic lung disease (CLD). However, there have been few reports assessing KL‐6 in meconium aspiration syndrome (MAS). KL‐6 was measured in neonates with respiratory diseases including MAS.
Methods
: Thirty‐eight neonates with respiratory disease were enrolled in the study. These patients were classified into three groups, 14 patients with respiratory distress syndrome (RDS), 14 with MAS, and 10 with transient tachypnea of the newborn (TTN). The control group consisted of 12 healthy neonates. KL‐6 levels were measured 1 day (median) after the birth. In the RDS group, measurement was repeated just prior to 36 weeks’ postmenstrual age.
Results
: The levels of KL‐6 were 116 ± 40 U/mL in the RDS, 281 ± 138 U/mL in the MAS, and 106 ± 41 U/mL in the TTN groups. KL‐6 levels were significantly higher in the MAS group than in the control group (134 ± 71 U/mL; P < 0.01). In addition, the levels were significantly higher in those with severe MAS than those with mild MAS (P < 0.05). In patients with RDS, KL‐6 increased in patients who developed CLD (P < 0.05), while KL‐6 levels did not change in those who did not develop CLD.
Conclusion
: These data confirm the high level of KL‐6 in CLD, and suggest that KL‐6 is increased in MAS.
Ontogeny of congenital anomalies of the kidney and urinary tract, CAKUT
Authors: Miyazaki Yoichi , Ichikawa Iekuni 
Published: October 2003

AbstractEctopic budding of the initial ureter from the Wolffian duct is the first ontogenic misstep that leads to many congenital anomalies of the kidney and urinary tract (CAKUT). The ectopia results in hypoplastic kidney, ectopia of ureterovesical orifice, urinary outflow obstruction and/or reflux. Recent studies on several mutant mouse models verified that ectopic ureteric budding indeed occurs prior to the formation of CAKUT. Often, the genes involved in navigating the site of ureteric budding also regulate later ontogenic processes of the kidney and other urinary tract systems. These additional functions of the genes underlie the wide spectrum of CAKUT, for example multicystic dysplastic kidney, megaureter and atretic ureter, because the genes regulate the morphogenesis of the many portions of the excretory system through their distinctive cellular functions.
Pathogenesis of human renal dysplasia: An alternative scenario to the major theories
Authors: Shibata Sawako , Nagata Michio 
Published: October 2003

AbstractAmong the various congenital anomalies of the kidney and urinary tract (CAKUT), renal dysplasia may deserve the closest attention due to its frequency and clinical severity. However, the pathogenesis of this disease is still not well known. It is believed that renal dysplasia is caused by a nephron induction deficit due to ampullary inactivity, or an abnormal budding of the ureteric bud from the mesonephric duct. Renal dysplasia is frequently associated with urinary tract obstruction, suggesting an alternative mechanism whereby urinary retention causes abnormal kidney development. Multicystic dysplastic kidney (MCDK) and obstructive renal dysplasia (ORD) are two different phenotypes of dysplasia associated with urinary tract obstruction. From detailed morphological studies using fetal dysplastic kidneys, nephron induction with filtrating function occurs before cysts are formed. Moreover, three‐dimensional reconstruction of dysplastic nephrons showed that cysts developed in the once‐induced nephrons as the result of fluid retention in both MCDK and ORD. In utero urinary tract obstruction may cause urine retention in functioning nephrons and lead to multiple cyst formation in the nephrogenic zone. These findings are common to MCDK and ORD, although there may be a difference in the obstruction site. Expansion of cysts with tubular dilatation (cysts) disturbs the subsequent nephrogenesis by the deregulated expression of growth and transcription factors, and may contribute to the misdevelopment of fetal kidneys.
Genetic disorders of human congenital anomalies of the kidney and urinary tract (CAKUT)
Authors: Nakanishi Koichi , Yoshikawa Norishige 
Published: October 2003

AbstractCongenital abnormalities of the kidney and urinary tract, CAKUT are common in humans, occurring at a frequency of approximately 1 in 500 fetal ultrasound examinations. CAKUT are major causes of chronic renal failure in infants and young children, but little is known about the molecular pathogenesis of these disorders. To date, several gene mutations have been identified as a cause of human CAKUT: these include PAX2, KAL, EYA1, AGTR2 and HNF‐1β. At present, there is only limited information regarding how mutations alter gene expression during development to cause some CAKUT. The most convincing information comes from the multiorgan malformation syndromes with specific gene mutations. However, these syndromes are relatively rare, and most CAKUT appear to occur in isolation. The goal of this review is to provide an overview of these genetic disorders for CAKUT. An understanding of the genetic aspects of human CAKUT will help to unravel the pathogenesis of these disorders and may facilitate the design of genetic screening tests for early diagnosis and appropriate genetic counseling. Moreover, a deeper insight into the relationship between abnormal genes and the pathogenesis of abnormalities of CAKUT will provide an etiological classification of CAKUT. In addition, the importance of developing a registry of patients with various forms of CAKUT is discussed. This information will allow us to combine molecular biology and classical epidemiologic methods, and to continue expanding our knowledge regarding CAKUT.
Ultrasound screening for renal and urinary tract anomalies in healthy infants
Authors: Tsuchiya Masami , Hayashida Mari , Yanagihara Takeshi , Yoshida Junko , Takeda Sachiyo , Tatsuma Noriko , Tsugu Hitoshi , Hino Yoshiaki , Munakata Emiko , Murakami Mutsumi 
Published: October 2003

Abstract
Background
: Nearly 30% of childhood cases of chronic renal failure in Japan are attributed to congenital anomalies of the kidney and urinary tract (CAKUT), and the number is increasing. Urine screening at school facilitates early diagnosis and treatment of glomerulonephritis, but early screening for anomalies is currently not in practice. The authors evaluated the value of early abdominal ultrasonography screening in 1‐month‐old infants.
Methods
: The following characteristics of kidneys were assessed: presence versus absence, size, symmetry of size, position, separation of the central echo complex (CEC), abnormal echogenicity, and other abnormal findings. The bladder and ureter were checked for abnormalities in bladder shape and wall, as well as retrovesical ureteral dilation. Criteria for abnormalities included kidney length of ≤35 mm, or ≥60 mm; a difference in length of left and right kidneys of 10 mm or more; and CEC separation of Society for Fetal Urology (SFU) grade 2 or higher.
Results
: Beginning in April 1994 and continuing until September 2001, screening of 5700 1‐month‐old infants yielded 198 positive cases (3.5%) of CAKUT. Most frequent was abnormal CEC separation (approximately 60% of all abnormalities), followed by abnormal renal size or size asymmetry (30%). Further investigation yielded a specific diagnosis in 32 cases (0.6%) of all subjects. Most prevalent was obstructive uropathy (15 cases); 8 children underwent surgery. Small kidneys and vesicoureteral reflux were next in frequency.
Conclusions
: Ultrasonograpy was effective for early detection of renal and urinary tract anomalies. Ultrasound screening in early infancy may permit early treatment, that can prevent renal dysfunction.
Changing concepts in urological management of the congenital anomalies of kidney and urinary tract, CAKUT
Authors: Nakai Hideo , Asanuma Hiroshi , Shishido Seiichiro , Kitahara Satoshi , Yasuda Kosaku 
Published: October 2003

AbstractRecent advancement in ultrasonographic evaluation has prompted early detection and diagnosis of congenital anomalies in the kidney and urinary tract (CAKUT) in the asymptomatic phase. Consequently, early surgical intervention has become possible in the asymptomatic phase for the purpose of controlling manifestations early, thereby avoiding renal functional deterioration. However, some lesions detected by ultrasonography have been shown to often resolve or disappear without intervention. Thus, it has become more important to identify and understand the natural history of CAKUT.For the precise evaluation of the results of surgical intervention, one must understand the maturational process of renal function during infancy. Without considering this process, we cannot differentiate the renal significance of the surgical management from the natural course of CAKUT. Recent advancement in the field of radioisotopic studies has also made a major contribution to the more precise assessment of renal function.Recent progress in the understanding of the pathophysiology and the natural history of CAKUT has helped rationalize its treatment and management. Improvement in the surgical techniques and tools, together with improvements in pediatric anesthesiology, have made an appreciably positive impact on the outcome.Herein, we present the emerging concepts in the urological management of CAKUT, specifically, multicystic dysplastic kidney, vesicoureteral reflux, congenital hydronephrosis, ectopic ureters and ureteroceles.
Early head cooling in newborn piglets is neuroprotective even in the absence of profound systemic hypothermia
Authors: Iwata Osuke , Iwata Sachiko , Tamura Masanori , Nakamura Tomohiko , Sugiura Masatoshi , Ogiso Yoshifumi , Takashima Sachio 
Published: October 2003

Abstract
Background
: Selective head cooling in the newborn infant has been proposed as a neuroprotective treatment with a lower level of systemic adverse effect than that of systemic hypothermia. However, the efficacy is not confirmed as well as that of systemic hypothermia. In order to analyze the safety and efficacy of selective head cooling, 25 newborn piglets were randomly selected for either normothermic or hypothermic treatment.
Methods
: Global hypoxic insult was induced by lowering the oxygen concentration to the maximal level to maintain the background electroencephalogram (EEG) voltage under 7 µV for 45 min. The core temperature of normothermic piglets was maintained between 38.5°C and 39°C, while prophylactic cooling was applied to the hypothermic piglets at the same time of the insult. Very mild systemic hypothermia by 1°C was induced in addition to selective head cooling with 10°C coolant temperature. Animals were killed for histopathological examination seven hours after the end of the insult.
Results
: Two normothermic piglets died while all hypothermic piglets survived. Neuropathological findings were significantly severer in the normothermic group than in the hypothermic group. Intracranial pressure was significantly lower, and EEG recovery was significantly better in the hypothermic piglets. There was no significant difference in the lowest oxygen concentration, degrees of acidosis, blood lactate, and blood pressure between the groups, although heart rate was significantly lower in the hypothermic group.
Conclusions
: We have demonstrated that early head cooling was effective in preventing some of the earliest brain damage due to hypoxic insult even in the absence of profound systemic hypothermia.
Flexible endoscopy of aerodigestive tract in small infants
Authors: Lee Yu‐Sheng , Soong Wen‐Jue , Jeng Mei‐Jy , Cheng Chin‐Yuan , Shen Chung‐Min , Sun John , Chen Chiung‐Fang , Hwang Betau 
Published: October 2003

Abstract
Background
: Flexible endoscopy (FE) is a useful method for diagnosing airway problems. Congenital or acquired airway lesions in infants may lead to respiratory distress that requires comprehensive investigation and management. This study was designed to evaluate the use of FE in small infants.
Methods
: Infants who had symptoms of respiratory distress and received FE when they were less than 1‐year‐old were studied and their medical history, diagnoses, interventions, and complications from FE were investigated.
Results
: The study population consisted of 568 small infants (334 boys and 234 girls) who weighed 5.1 ± 2.4 kg, and received FE when they were 4.5 ± 3.6 months of age. Most patients (91.2%) received diagnostic FE and the remainder (8.8%) received therapeutic procedures. Stridor (38.0%) was the most common indication for FE and laryngomalacia (33.3%) was the most frequent finding. Synchronous FE diagnosis was found in 351 (61.8%) cases. No major complications associated with FE were found.
Conclusion
: Flexible endoscopy allows direct visualization of dynamic motion of the small aerodigestive tract. Laryngomalacia was the most common FE finding of respiratory distress in small infants. Synchronous FE lesions were frequently found in this young age group and it necessitated a thorough investigation of the entire aerodigestive tract.