bluuebunny/crossref_metadata_2025 huggingface dataset:
Pediatric Spinal Tumors
Authors: Schick Uta , Marquardt Gerhard
Published: April 2003

We present an overview of the clinical outcome and surgical treatment of 34 pediatric spinal tumors, carried out in our center from 1981 to 1999. Follow-ups were available for 25.5 months on average. Clinical history, signs, surgical approach, outcome and radiological reports were obtained by review of the patients’ charts. These pediatric tumors formed a very heterogeneous group, with a predominance of neurinomas and neurofibromas in older children and neuroblastomas or primitive neuroectodermal tumors in younger ones. Ependymomas and astrocytomas were the most frequent intramedullary tumors. Extradural tumors included tumors such as aneurysmal bone cysts, Ewing’s sarcoma, histiocytomas, chordomas and an angiofibroma. Following decompression of the spinal cord, the neurological status improved with good functional recovery in 23 children and had deteriorated at follow-up in 5 children. Relapse occurred in 12 cases, including histiocytomas, chordomas, medulloblastoma and tumors associated with genetic disorders. Eighteen children underwent a combined treatment with chemotherapy and/or radiotherapy. The spectrum of pathology is wide and requires multidisciplinary treatment.
The Impact of Endoscopic Third Ventriculostomy on the Management of Newly Diagnosed Hydrocephalus in Infants
Authors: Javadpour Mohsen , Mallucci Conor , Brodbelt Andrew , Golash Aprajay , May Paul
Published: April 2003

Objectives: To evaluate the role of endoscopic third ventriculostomy (ETV) as a primary treatment for hydrocephalus in children less than 1 year old and to determine its impact as a whole on the reduction of shunts necessary in a new population of hydrocephalic infants. Methods: Data were collected prospectively on 47 infants with newly diagnosed hydrocephalus of all aetiologies who were referred between 1st April 1998 and 30th September 2000. Twenty-one patients (median age 6 weeks, range 34 weeks of gestation to 10 months) underwent ETV, while the remaining 26 patients had insertion of a ventriculoperitoneal shunt. Anatomical criteria and demonstration of third ventricle outflow obstruction on preoperative magnetic resonance imaging were used to select patients for ETV. Results: There was no mortality or major morbidity following ETV. The median follow-up period was 18 (range 8–36) months. During the follow-up period, the ETV remained patent in 7 (33%) of the 21 patients. Of the 14 patients with failed ETV, 11 had insertion of a ventriculoperitoneal shunt, while 3 have undergone successful redo ETV. Therefore, in total 10 patients (48%) of the ETV group remain shunt independent. The best results were obtained in patients with congenital aqueduct stenosis with 71% (5 of 7 patients) success rate, while patients with posthaemorrhagic hydrocephalus did particularly badly with only 1 of 10 patients having a successful ETV. Overall, 10 of 47 (21%) infants with newly diagnosed hydrocephalus have avoided a shunt. Conclusions: Our results suggest that the selective use of ETV as the primary treatment in infants with hydrocephalus is safe and can lead to a reduction in the shunted population of all newly diagnosed hydrocephalic infants by up to 21%. Success of ETV is aetiology, not age dependent.
Preliminary Observations on the Association between Simple Metopic Ridging in Children without Trigonocephaly and the Chiari I Malformation
Authors: Tubbs R. Shane , Elton Scott , Blount Jeffrey P. , Oakes W. Jerry
Published: April 2003

Historically, there have been no accounts of an association between simple metopic ridging and the Chiari I malformation. We prospectively evaluated 50 children with simple metopic ridges and found that 30% of these patients had a Chiari I malformation. During the period in which this screening for simple metopic ridges was performed, only 9% of the total patient population (324) was diagnosed as having a Chiari I malformation. We hypothesize that small decreases in anterior fossa volume in children with a simple metopic ridge and no other clinical findings of trigonocephaly may increase their chance of having a Chiari I malformation that becomes symptomatic. In addition, simple metopic ridging should perhaps be added to the list of bony anomalies associated with the Chiari I malformation, such as atlantal assimilation and retroverted odontoid processes. Future studies aimed at calculating anterior fossa volumes in children with a Chiari I malformation and imaging all children with simple metopic ridges are now necessary to ascertain if there is an increased risk of these patients becoming symptomatic with a hindbrain hernia or having a hindbrain hernia, respectively.
Surgery for Expansion of Spinal Tuberculoma during Antituberculous Chemotherapy: A Case Report
Authors: Nomura Sadahiro , Akimura Tatsuo , Kitahara Tetsuhiro , Nogami Ken-ichiro , Suzuki Michiyasu
Published: April 2003

We report a case of intramedullary spinal tuberculoma in a girl aged 2 years and 6 months. At the age of 18 months, the patient was hospitalized for 2 months with tuberculous meningitis. Tuberculomas then appeared in the thoracic spinal cord, and the patient developed paraparesis despite continuation of chemotherapy. Spinal magnetic resonance imaging demonstrated multiple ring-enhanced lesions in the cord on the T1-weighted image; on the T2-weighted image, the lesions had a bright core surrounded by a hypointense rim. Laminectomy and partial excision were performed, and the paraparesis improved. In addition to antituberculous chemotherapy and dexamethasone, surgical decompression was needed to treat the expansion of the spinal tuberculoma.
Spontaneous Decompression of a Posterior Fossa Arachnoid Cyst: A Case Report
Authors: Pandey P. , Tripathi M. , Chandra P. Sarat , Singh V.P. , Mehta V.S.
Published: April 2003

The authors present a case of spontaneous regression of a posterior fossa, supracerebellar arachnoid cyst causing hydrocephalus in a 7-month-old male child. The patient presented with macrocrania, bulging fontanelle and upgaze paresis of the eyes. There was complete improvement of these symptoms on the morning of the day of surgery without any obvious precipitating factors, including trauma. CT scan showed complete resolution of the cyst and hydrocephalus. The child was doing well at 1 year of follow-up. To the best of our knowledge, this is the first case report of spontaneous resolution of a posterior fossa arachnoid cyst.
Outcome and Long-Term Side Effects after Synchronous Radiochemotherapy for Childhood Brain Stem Gliomas
Authors: Benesch Martin , Lackner Herwig , Moser Andrea , Kerbl Reinhold , Schwinger Wolfgang , Oberbauer Rainer , Eder Hans-Georg , Mayer Ramona , Wiegele Karin , Urban Christian
Published: April 2003

Between 1993 and 1999, 11 children with histologically confirmed diffuse and exophytic brain stem glioma (BSG) were treated with intensive induction chemotherapy and simultaneous external beam irradiation. Chemotherapy was performed according to the German/Austrian Pediatric Brain Tumor Study HIT ’91 and included two cycles of ifosfamide (days 1–3), etoposide (days 4–6), methotrexate (days 15 and 22), cisplatin (days 29–31) and cytarabine (days 29–31), separated by a 3-week interval. Maintenance chemotherapy with carmustine, carboplatin and vincristine (8 cycles over a 1-year period) was given in those patients who responded clinically or radiographically to induction chemotherapy. Six of 11 patients showed an objective reduction in tumor size on magnetic resonance imaging and 4 of 11 are alive in good general condition >22, >22, >90 and >92 months, respectively, after diagnosis without radiographic evidence of tumor progression (1 complete remission, 2 partial remissions, 1 stable disease), but suffer from moderate to severe long-term side effects. Three patients died due to disease progression after having achieved a partial remission which lasted 5, 6 and 18 months, respectively, whereas only short-term stabilization was observed in 4 patients who died within 1 year after diagnosis. Acute hematologic toxicity was severe but manageable. This intensive combined modality treatment was toxic but yielded objective responses in more than 50% and long-term survivors in one third of childhood BSG patients.
Symptomatic Spinal Intradural Arachnoid Cysts in the Pediatric Age Group: Description of Three New Cases and Review of the Literature
Authors: Lee H.-J. , Cho D.-Y.
Published: April 2003

Spinal arachnoid cysts are a relatively uncommon lesion that may be either intra- or extradural, and intradural spinal arachnoid cysts are even less common. These cysts are usually asymptomatic but may produce symptoms by compressing the spinal cord or nerve roots suddenly or progressively. We present three cases in the pediatric age group with spinal intradural arachnoid cysts without a preceding history of trauma. Three patients with symptomatic intradural arachnoid cysts were investigated with conventional T1- and T2-weighted magnetic resonance imaging (MRI). The MRI scans demonstrated the intradural arachnoid cysts with slightly lower CSF signal intensity on the gradient echo images and slightly higher signal intensity on T1-weighted images. The first cyst was located at the level T12–L1 and compressed the conus medullaris, with neurogenic bladder and cauda equina syndrome for 2 months. The second was located at the level C5–T1 ventrally, with spastic gait and neurogenic bladder for 4 years. The other was located at T2–3 ventrally, with sudden onset of quadriplegia after jumping rope. The combined treatment of total resection and wide fenestration in our three patients produced an excellent return of neurologic function in each one, except for residual urinary disturbance in case 2. Intradural spinal arachnoid cysts appear to result from an alteration of the arachnoid trabeculae; some such cysts are ascribed anecdotally to previous trauma or arachnoiditis, whereas the majority are idiopathic and congenital. The majority of intradural spinal arachnoid cysts occur in the thoracic region and most are dorsal to the neural elements. Only 10 cases have been reported in which the intradural arachnoid cysts were located anterior to the cervical spinal cord, of which 8 were in the pediatric age group, like our case 2. Myelography, postcontrast CT myelography and MRI have been demonstrated as useful for the diagnosis of intradural arachnoid cysts. MRI is the imaging modality of choice, and the extent, size and nature of the lesion in our cases were well demonstrated by MRI. Surgical treatment is necessary if progressive neurological dysfunction appears in the course of spinal cord compression. Complete surgical excision of the cysts is the best choice of treatment, and wide fenestration and shunting of the cyst to the peritoneum, pleural cavity or right atrium were the modalities of choice. MRI offers a noninvasive and effective means to make the diagnosis of arachnoid cysts easier. Intradural arachnoid cysts may cause progressive myelopathy; however, the postoperative prognosis is good if the operation is performed prior to neurologic deficits.
Monozygotic Twins Discordant for External Hydrocephalus
Authors: Piatt Jr. Joseph H.
Published: April 2003

External hydrocephalus (EH) is a transient, developmental condition in infancy characterized by macrocephaly and prominence of the subarachnoid spaces. The cause is unknown, but many patients have a family history consistent with autosomal dominant inheritance. This report describes a pair of monozygotic twins, only one of whom – the recipient of a twin-twin transfusion – has EH. Whatever the genotype of the twins, their discordant phenotypes suggest that the disproportionate calvarial growth that characterizes EH is set in motion – or not – during a limited, critical temporal window in fetal development.
Pituitary Adenoma Associated with Gigantism and Hyperprolactinemia
Authors: Alikasifoglu A. , Kandemir N. , Akalan N. , Yordam N.
Published: April 2003

An 11-year-old girl presented with excessive growth, headache, left visual loss and seizures. Her growth hormone (GH) and prolactin (PRL) levels were high and magnetic resonance imaging findings showed an invasive macroadenoma. Gross total tumor removal was performed and then radiotherapy and medical therapy were given. During the follow-up, she developed ACTH deficiency, secondary hypothyroidism and hypogonadism requiring replacement therapy. It is still unclear whether the biological characteristics of GH- and PRL-secreting tumors are different in children from those in adults. More data are needed before a definitive conclusion can be established.
Hospital Charges Attributable to Bronchoscopy-Related Complications in Outpatients
Authors: Colt Henri G. , Matsuo Toshihiro
Published: April 2003

Background: Despite literature pertaining to algorithms of care, operating room charges and other financial management issues in medicine, there is a paucity of data pertaining to the fiscal consequences of bronchoscopic practice. Objective: To identify hospital charges directly attributable to bronchoscopy-related complications in outpatients. Methods: A prospective analysis of outpatient bronchoscopy-related complications, clinical outcomes and hospital charges resulting directly from procedure-related adverse events in 660 consecutive outpatients undergoing flexible fiberoptic bronchoscopy (FFB) during a period of 30 consecutive months at the University of California, San Diego Medical Center, was performed. Results: Altogether, 1,009 consecutive outpatient FFBs were performed on 660 patients (mean age 58 years, range16–91 years). Fifty adverse events (5% of all procedures) occurred in 44 patients. These were bronchospasm (31 cases), hemoptysis (5 cases), pneumothorax (3 cases), nausea/vomiting (3 cases), hypoxemia (2 cases), seizure (2 cases), laryngeal spasm (2 cases), chills/fever (1 case) and a vasovagal episode (1 case). Prolonged length of stay in the postprocedure recovery area on 22 occasions (2.2% of all procedures) resulted in USD 6,996 in additional hospital charges. Hospitalization was necessary in only 5 instances (0.5% of all procedures), but resulted in USD 34,500 in additional charges (range for the 5 patients, USD 2,000–11,000) that were directly attributable to a procedure-related complication. Conclusion:  Hospital charges directly attributable to outpatient flexible bronchoscopy-related complications are minimal, but escalate considerably if hospitalization becomes necessary.
Treatment of Recurrent Severe Hemoptysis in Cystic Fibrosis with Tranexamic Acid
Authors: Graff Gavin R.
Published: April 2003

Major hemoptysis is a potentially life-threatening complication of cystic fibrosis (CF) lung disease. Bronchial artery embolization (BAE) along with treatment of a CF pulmonary exacerbation has become the most widely used therapeutic approach for major hemoptysis in CF. However, BAE has been associated with severe complications, especially when bronchial artery to spinal artery anastomoses are present. This case study describes the successful treatment of major hemoptysis in CF with tranexamic acid, in an individual in whom 12 previous BAE procedures had been performed and further procedures were contraindicated secondary to bronchial artery to spinal artery collaterals. Recurrence of the hemoptysis occurred after attempts had been made to withdraw the tranexamic acid. Tranexamic acid was resumed with resolution of the hemoptysis, and the therapy has been used continuously for 13 months without any complications.
A Rare Case of Bronchial Glomus Tumor
Authors: Oizumi Satoshi , Kon Yujiro , Ishida Takashi , Yamazaki Koichi , Itoh Tomoo , Ogura Shigeaki , Dosaka-Akita Hirotoshi , Morikawa Toshiaki , Shimizu Michio , Katoh Hiroyuki , Kawakami Yoshikazu
Published: April 2003

A 48-year-old man was admitted because of bloody sputum in whom a chest computed tomography (CT) scan and fiberoptic bronchoscopy demonstrated a polypoid tumor in the left main bronchus. The tumor was surgically resected, and the pathological and immunohistochemical findings led to diagnosis of the tumor as a bronchial glomus tumor.
Pathogenesis and Pathology of COPD
Authors: Turato Graziella , Zuin Renzo , Saetta Marina
Published: April 2003

Chronic obstructive pulmonary disease (COPD) is characterized by airflow limitation. Since flow is the result of a driving pressure that promotes flow and of an opposing resistance that contradicts it, the reduction in flow observed in COPD has two main components: increased resistance, which is due to airway obstruction, and a loss of the elastic recoil pressure of the lung, which is due to parenchymal destruction. Although it has long been known that the major site of increased resistance in COPD is the peripheral airways, recent studies have shown that central airways are involved in the disease as well. The purpose of this review is to describe the major structural and cellular changes present in peripheral airways, central airways and lung parenchyma of patients with COPD, and to underline the possible mechanisms contributing to airflow limitation in these subjects.
Human Recombinant Interferon-α2a and Interferon-αA/D Have Different Effects on Bleomycin-Induced Lung Injury
Authors: Berkman N. , Kremer S. , Or R. , Lossos I.S. , Christensen T.G. , Goldstein R.H. , Breuer R.
Published: April 2003

Background: Bleomycin (Bleo)-induced lung injury in mice serves as an animal model of pulmonary fibrosis. The pathogenesis of pulmonary fibrosis remains unclear, but it comprises both inflammatory and fibrotic components. The cytokine interferon (IFN)-α is produced by macrophages and may modulate both fibrogenesis and the determination of T lymphocyte phenotype in pulmonary fibrosis. Objective: To investigate the effect of two preparations of recombinant IFN-α (IFN-αA/D and IFN-α2a) on Bleo-induced lung injury in C57BL/6 mice. Methods: Mice were treated by a single intratracheal (IT) instillation of 0.06 mg of Bleo in 0.1 ml of saline or saline alone. One of two different IFN-α preparations, IFN-αA/D or IFN-α2a in saline, or saline alone were administered by daily intraperitoneal injections starting 1 day prior to IT instillation. The treatment groups were as follows: IT Bleo and intraperitoneal saline; IT Bleo and intraperitoneal IFN-α2a; IT Bleo and intraperitoneal IFN-αA/D; IT saline and intraperitoneal IFN-αA/D or IFN-α2a; IT saline and intraperitoneal saline. The animals were sacrificed 14 days after IT instillation. Lung injury was evaluated by total and differential cell count in bronchoalveolar lavage (BAL) fluid, by a semiquantitative morphological index of lung injury and a quantitative image analysis of cellularity and fibrosis fraction and by biochemical analysis of lung hydroxyproline content. Results: In Bleo-treated mice, IFN-α2a treatment caused a significant rise in BAL lymphocytes and in cellularity and fibrosis fractions in lung tissue. In contrast, IFN-αA/D treatment had no effect on Bleo-induced lung injury. Conclusion: IFN-α may enhance Bleo-induced lung injury but this effect varies with different IFN preparations.
Evaluation of a Portable Recording Device (Somnocheck®) for Use in Patients with Suspected Obstructive Sleep Apnoea
Authors: Ficker J.H. , Wiest G.H. , Wilpert J. , Fuchs F.S. , Hahn E.G.
Published: April 2003

Background: Portable recording devices for the diagnosis of obstructive sleep apnoea (OSA) should be carefully evaluated before being used on a routine basis in out-patients. Objective: This study was designed to evaluate the diagnostic accuracy of the new portable recording device, Somnocheck® (SC). Methods: Nocturnal polysomnographies (PSG) and SC recordings were performed simultaneously in the sleep laboratory in 51 patients with suspected OSA, and were analysed manually by a blinded investigator. Results: The apnoea/hypopnoea index (AHI) obtained by manual SC analysis correlated closely with that obtained by PSG (r = 0.98), but the correlation with AHI obtained by automatic SC analysis was markedly lower (r = 0.83). When an AHI ≧10 was defined as indicative of OSA, the sensitivity of manual SC analysis was 0.97 and its specificity 1.00. The sensitivity of automatic analysis was 0.83 and its specificity 0.95. Conclusions: In this experimental setting, the SC showed a very high diagnostic accuracy for the diagnosis of OSA and was able to define its severity precisely. The diagnostic accuracy of manual analysis was found to be superior to that of automatic analysis. Considering the results of this technical evaluation, the SC may also be expected to work reliably when it is used in an ambulatory setting.
Increased Interleukin-5 Levels in Bronchoalveolar Lavage Fluid Is a Major Factor for Eosinophil Accumulation in Acute Eosinophilic Pneumonia
Authors: Nakahara Yoshiaki , Hayashi Shinichiro , Fukuno Yuji , Kawashima Michihiro , Yatsunami Jun
Published: April 2003

Background: Increased interleukin-5 (IL-5) levels have been reported in bronchoalveolar lavage fluid (BALF) from patients with acute eosinophilic pneumonia (AEP); however, it still remains to be determined whether IL-5 is responsible for the eosinophil accumulation in the lung. Objective: We examined the effect of antibodies against cytokines on eosinophil chemotaxis induced by BALF from AEP patients to identify factors responsible for eosinophil accumulation. Methods: We measured a series of specific cytokines, including IL-3, IL-4, IL-5, IL-6, IL-8, GM-CSF, RANTES, MCP-1, MIP-1α and eotaxin, in the BALF from 4 patients with AEP. BALF from 4 patients with chronic eosinophilic pneumonia (CEP) and 13 patients with non-eosinophilic interstitial lung diseases (ILD) were examined as controls. The eosinophil chemotactic activity in the BALF was examined using tissue culture insert furnished with a polycarbonate membrane. Results: The total protein content in BALF from patients with AEP was extremely elevated. Even after standardization with protein concentration, IL-5 levels in AEP patients were significantly higher than those in CEP and ILD. IL-3 and chemokines were rather lower in the AEP group than in the CEP and ILD groups. In AEP BALF, anti-IL-5 neutralizing antibody significantly inhibited eosinophil chemotaxis. Antibodies against IL-3, GM-CSF, and IL-8 did not affect the eosinophil migration. Conclusion: These findings suggest that locally produced IL-5 plays an important role in eosinophil accumulation of AEP.
Bronchiolitis obliterans with Organizing Pneumonia Associated with Acute Coxiella burnetii Infection
Authors: Pérez de Llano L.A. , Veres Racamonde A. , Rivas Bande M.J. , Ortiz Piquer M. , Bal Nieves F. , Rodríguez Feijoo A.
Published: April 2003

We report the case of a 62-year-old man who developed bronchiolitis obliterans with organizing pneumonia (BOOP) associated with acute Coxiella burnetii infection. The diagnosis of BOOP was made by fiber-optic transbronchial biopsy. Treatment with corticosteroids resulted in rapid clinical improvement and complete resolution of airspace opacities. To the best of our knowledge, this is the first well-documented case of BOOP associated with C. burnetii infection. This case suggests that this infection might trigger the development of BOOP.
KL-6, Surfactant Protein A and D in Bronchoalveolar Lavage Fluid from Patients with Pulmonary Sarcoidosis
Authors: Kunitake Ritsuko , Kuwano Kazuyoshi , Yoshida Koichiro , Maeyama Takashige , Kawasaki Masayuki , Hagimoto Naoki , Hara Nobuyuki
Published: April 2003

Background: KL-6, and surfactant protein A (SP-A) and surfactant protein D (SP-D) derived from alveolar type II cells and/or bronchiolar epithelial cells have been reported to be useful markers for interstitial lung diseases. Objective: The aim of this study was to measure the levels of these molecules in bronchoalveolar lavage fluid (BALF) from patients with pulmonary sarcoidosis to investigate their relationship with other markers of inflammatory activity. Methods: We measured KL-6, SP-A and SP-D levels in BALF from patients with pulmonary sarcoidosis using an ELISA. Results: KL-6 and SP-D, but not SP-A levels were significantly increased in pulmonary sarcoidosis compared with controls. KL-6, SP-A and SP-D levels were significantly correlated with each other. KL-6 and SP-D levels were relatively and significantly correlated with the percentage of lymphocytes in BALF. KL-6, SP-D, but not SP-A levels were significantly correlated with the concentration of albumin in BALF. There was no significant correlation between KL-6, SP-A, or SP-D levels and chest X-ray findings, angiotensin-converting enzyme levels, or CD4/CD8 ratio in BALF. Conclusions: We conclude that KL-6 and SP-D levels in BALF were increased in pulmonary sarcoidosis. Since these markers are specifically derived from epithelial cells, it is considered that KL-6 and SP-D levels are reflecting damage or release of these markers from epithelial cells due to the inflammatory response.
Physiologic Evaluation of 4 Weeks of Nocturnal Nasal Positive Pressure Ventilation in Stable Hypercapnic Patients with Chronic Obstructive Pulmonary Disease
Authors: Nava Stefano , Fanfulla Francesco , Frigerio Pamela , Navalesi Paolo
Published: April 2003

Background: The long-term daily use of noninvasive mechanical ventilation (NIMV) to treat chronic respiratory failure in chronic obstructive pulmonary disease (COPD) patients is not widely recommended, partly because of a lack of clear clinical results and partly because the physiological mechanisms by which the daily application of NIMV would be helpful in these patients have not yet been clarified. Objectives: We designed a physiological study in order to assess the effects of supervised long-term NIMV on gas exchange, respiratory muscle function, pulmonary mechanics and to ascertain the possible effect of the treatment in responders and nonresponders. Methods: Fourteen consecutive inpatients with stable hypercapnic COPD (pH = 7.37 ± 0.01; PaCO2 = 56.73 ± 6.48 mm Hg) underwent 4 weeks of nocturnal NIMV delivered with a bilevel ventilator ‘physiologically’ set to reduce tidal transdiaphragmatic pressure (Pdi) by at least 50% and the amount of dynamic intrinsic positive end-expiratory pressure by 70%. Various measurements were compared with those obtained in a control group of consecutive patients with comparable baseline characteristics who refused NIMV and underwent breathing exercises for the same period of time. Results: By the end of the 4 weeks NIMV had induced a slight but significant (p < 0.01) reduction in resting PaCO2 (53.78 ± 5.64 mm Hg) associated with a decrease in the pressure time product of the diaphragm per minute (from 172 ± 60 to 136 ± 61 cm H2O/l/s; p < 0.05). This latter value was primarily due to a significant shortening of the inspiratory duty cycle, while Pdi and lung mechanics were not modified. Eight of the 13 NIMV-treated patients (1 dropped out for nonmedical reasons) had a clear reduction in PaCO2 (>3 mm Hg or >5% from enrollment) and were classified as responders. The acute reduction in PaCO2 during the first trial with NIMV resulted to be a strong index of the final response. The subgroup of responders had a significantly increased maximal Pdi (from 41 ± 19 to 49 ± 23 cm H2O, p < 0.05) and an enhanced ability of the ventilatory pump to clear CO2 (9.7 ± 3.4 vs. 7.2 ± 2.9 cm H2O × s/min; p < 0.01). No significant changes were observed in the control group. Conclusions: These results suggest that in a remarkable and identifyable proportion of patients with stable hypercapnic COPD, nocturnal NIMV may decrease resting PaCO2, reraising the role of chronically supporting the respiratory pump.
Persistent Spontaneous Pneumothorax in an Adolescent with Marfan’s Syndrome and Pulmonary Bullous Dysplasia
Authors: Rigante Donato , Segni Giuseppe , Bush Andrew
Published: April 2003

A 16-year-old boy with Marfan’s syndrome was admitted with progressive dyspnea due to a large spontaneous pneumothorax. Bullous pulmonary dysplasia was confirmed and pleural tube drainage did not affect the air leak. Complete recovery required surgical resection of the bulla responsible for the ongoing air leak. This case report highlights the issue of management for severe spontaneous pneumothorax in general, showing that the choice of treatment should not depend on the presence of pulmonary bullous dysplasia but on the clinical evaluation of the individual patient.
Concurrent Left-Sided Spontaneous Pneumothorax in Japanese Monogerminal Twins
Authors: Tsukadaira Akihiro , Okubo Yoshio , Ota Masao , Hotta Junichi , Kubo Keishi
Published: April 2003

Monogerminal twin brothers aged 17 were admitted because of concurrent left-sided spontaneous pneumothorax. A familial background of this common disease in association with the human leukocyte antigen (HLA) has been suggested; however, the actual mode of inheritance in association with HLA typing is still uncertain. HLA analysis in this family revealed the HLA-A24, B61 (40), Cw3, DR4, DR53, and DQ3 haplotype in the twins and their father, who also had a medical history of spontaneous pneumothorax. We report these cases as an extremely rare example of familial occurrence of spontaneous pneumothorax.
Monitoring Asthma Severity and Response to Treatment
Authors: Godfrey S.
Published: April 2003

Guidelines for the management of asthma rely on various methods of assessing severity and how it changes in order to select the appropriate treatment. These methods have been developed from the basic clinical skills of history taking and physical examination to which attempts at improving objectivity have been added, such as the use of asthma diaries, the home recording of peak expiratory flow (PEF) or measurements of bronchial reactivity. The present review was an attempt to determine to what extent monitoring asthma by various techniques is justified. Unfortunately, diaries and home recording of PEF are easily faked and generally unreliable while objective tests, such as the measurement of bronchial reactivity or studies of induced sputum cannot be undertaken often enough for monitoring, and there is certainly the need for prospective longitudinal controlled studies.
Multiple Access to Sterile Syringes for Injection Drug Users: Vending Machines, Needle Exchange Programs and Legal Pharmacy Sales in Marseille, France
Authors: Moatti J.P. , Vlahov D. , Feroni I. , Perrin V. , Obadia Y.
Published: April 2003

Objective: In Marseille, southeastern France, HIV prevention programs for injection drug users (IDUs) simultaneously include access to sterile syringes through needle exchange programs (NEPs), legal pharmacy sales and, since 1996, vending machines that mechanically exchange new syringes for used ones. The purpose of this study was to compare the characteristics of IDUs according to the site where they last obtained new syringes. Methods: During 3 days in September 1997, all IDUs who obtained syringes from 32 pharmacies, four NEPs and three vending machines were offered the opportunity to complete a self-administered questionnaire on demographics, drug use characteristics and program utilization. Results: Of 485 individuals approached, the number who completed the questionnaire was 141 in pharmacies, 114 in NEPs and 88 at vending machines (response rate = 70.7%). Compared to NEP users, vending machine users were younger and less likely to be enrolled in a methadone program or to report being HIV infected, but more likely to misuse buprenorphine. They also had lower financial resources and were less likely to be heroin injectors than both pharmacy and NEP users. Conclusions: Our results suggest that vending machines attract a very different group of IDUs than NEPs, and that both programs are useful adjuncts to legal pharmacy sales for covering the needs of IDUs for sterile syringes in a single city. Assessment of the effectiveness and cost-effectiveness of combining such programs for the prevention of HIV and other infectious diseases among IDUs requires further comparative research.
Prognostic Value of Soluble Tumor Necrosis Factor Receptors 1 and 2 in Multiple Sclerosis Patients Treated with Interferon Beta-1b
Authors: Laske C. , Oschmann P. , Tofighi J. , Kühne B.S. , Diehl H. , Bregenzer T. , Kraus J. , Chatzimanolis N. , Bauer R. , Traupe H. , Kaps M.
Published: April 2003

The objective of this study was to investigate the effect of interferon (IFN) beta-1b on the serum levels of soluble tumor necrosis factor receptor 1 (sTNF-R1) and sTNF-R2 in patients with multiple sclerosis (MS) in correlation with clinical and magnetic resonance image (MRI) activity. Serum samples were obtained every 3 months from 24 patients treated with 8 × 106 U of IFN beta-1b every other day (treatment group) and from 21 patients without any immunomodulatory therapy (control group) over a 15-month observation period. The cytokine receptor levels were assessed by ELISA. Cranial MRI was performed every 6 months to determine the burden of disease. In the treatment group, the MRI responders had significantly larger mean values for the area under the concentration-time curve of sTNF-R1 (p = 0.04) and sTNF-R2 (p = 0.01) when compared to the MRI nonresponders during the 15-month observation period. With regard to an increase in sTNF-R1 and -2 of more than 20% during the first 3 months of treatment, we observed a sensitivity of 33 and 58%, respectively, a specificity of 90 and 60%, respectively, and a positive predictive value of 80 and 64%, respectively, for MRI response during the 15-month observation period. A decrease in sTNF-R1 and -2 of more than 20% during the first 3 months of treatment had a sensitivity of 40 and 20%, respectively, a specificity of 100 and 100%, respectively, and a positive predictive value of 100 and 100%, respectively, for further MRI nonresponse (during the 15-month observation period). The present data suggest that assessment of sTNF-Rs may contribute to the identification of subgroups of patients who are likely to respond better than others to treatment with IFN beta-1b. This could help to establish a cost-effective prescription pattern for this expensive treatment, which is of importance for the future management of patients with MS.
Repair of Inguinal Hernias Using the Mesh Technique during Extraperitoneal Pelvic Lymph Node Dissection
Authors: Stolzenburg Jens-Uwe , Pfeiffer Heidemarie , Neuhaus Jochen , Sommerfeld Manfred , Dorschner Wolfgang
Published: April 2003

Purpose: This article describes our experience of using a totally extraperitoneal approach for endoscopic pelvic lymphadenectomy and inguinal hernia repair with the mesh technique in one procedure. Materials and Methods: A total of 52 patients underwent modified pelvic lymph node dissection for the staging of prostate cancer. Eight of them had hernia defects; 1 was recurrent. Five patients with direct and 3 patients with indirect inguinal hernias were treated by totally extraperitoneal hernia repair with the placement of a mesh measuring at least 10 × 15 cm (prolene mesh with incision and flap). Results: The mean duration of the lymphadenectomy itself was decreased from 150 min (first 20 patients) to 70 min (n = 21–52). The mean additional procedure time for hernioplasty was 15 min. The overall lymph node-positive rate was 9.6%. The complication rate was 7.7%. Four patients developed symptomatic lymphoceles, 1 of whom developed deep venous thrombosis. No complications occurred which were attributed to hernia repair. Morbidity did not rise, and hospitalization time did not increase for the patients who underwent hernioplasty. There were no recurrences or neuralgias on follow-up up to 2 years. Conclusions: By avoiding entry into the peritoneal cavity, the extraperitoneal approach obviates intra-abdominal complications (ileus, bowel injury, peritonitis) in both techniques. The extraperitoneal approach for pelvic lymph node dissection allows concomitant inguinal hernia to be repaired with low morbidity and within an acceptable operating time.
Collecting Duct Carcinoma
Authors: Tulunay Özden , Küpeli Saadettin , Okçu Aylin Heper , Baltacı Sümer , Bircan Sema , Orhan Diclehan
Published: April 2003

Collecting duct carcinoma of the kidney is a rare variant of renal cell carcinoma. Hematuria is the most common presenting symptom. This tubulopapillary tumor arises from or differentiates forward to medullary collecting tubules. It is typically centered on the medulla of the kidney and extends into the cortex with a desmoplastic reaction. There are few reports documenting the clinical presentation and long-term prognosis. We describe a case of this uncommon tumor with clinical, histological and immunohistochemical features and review the literature.
Natural History of Refluxing Distal Ureteral Stumps following Upper Tract Surgery in Children with Ectopic Ureter or Ureterocele
Authors: Kim Kun Suk , Kim Yongjae , Oh Seung-June
Published: April 2003

Background: It is a difficult problem to manage refluxing distal ureteral stumps after an upper tract approach in the ectopic ureter and ureterocele. We evaluated the natural history of the remaining refluxing distal ureteral stumps through a close follow-up. Patients and Methods: From 1991 to 1998, nine patients with the ectopic ureter or ureterocele, whose age ranged from one to 20 (median 5.0) months, had undergone an upper tract surgery for ectopic ureter (2) or ectopic ureterocele (7). The upper tract surgery for ureterocele or ectopic ureter included a total nephrectomy (2), a partial nephrectomy (6) or a proximal ureteroureterostomy (1), leaving distal ureteral stump. We evaluated the clinical results of the remaining ureteral stumps every 6 or 12 months. The postoperative follow-up period was 51 (median; range 21–96) months. Results: Among the 9 patients, 5 showed disappearance of the reflux and a markedly reduced stump size in the follow-up study. Improvement was noted in 4 patients within 6 months and in 1 by 20 months postoperatively. Two patients subsequently underwent a bladder level surgery due to problems not associated with the ureteral stump itself. Two patients have been followed up for 30 and 39 months, but without any improvement. An intermittent asymptomatic bacteriuria was noted in all patients during the follow-up, but no patient suffered from symptomatic urinary tract infection requiring an additional surgery. Conclusions: Since refluxing distal ureteral stumps were spontaneously resolved in more than half of the cases and did not cause significant problems during the follow-up, it is not necessary to remove the refluxing ureter simultaneously at the time of the upper tract surgery.
Percutaneous Resection of Renal Transitional Carcinoma: Venous Injury and Its Conservative Management
Authors: Goel M.C. , Roberts J.G.
Published: April 2003

An interesting observation encountered at percutaneous resection of a renal transitional cell carcinoma and its conservative management is described. During resection of the tumour sudden heavy haematuria was encountered. Nephroscopic inspection revealed the blood to be coming from behind a loop-generated flap, the raising of which allowed entry of the endoscope into a sizeable vein and thence upwards into the renal vein and inferior vena cava (IVC). On table nephrostogram confirmed contrast outlining the renal vein and IVC. Placement and clamping of a nephrostomy tube was followed by stabilization of the patient and the bleeding stopped. The urine became clear within 24 h. Later on repeat nephrostogram showed an intact pelvicalyceal system with an acceptable tumour clearance. This case highlights the importance of a trial of conservative management in the treatment of bleeding complications during nephroscopy.
Rapidly Relapsing Squamous Cell Carcinoma of the Renal Pelvis Associated with Paraneoplastic Syndromes of Leukocytosis, Thrombocytosis and Hypercalcemia
Authors: Er Özlem , Coşkun Hasan Şenol , Altinbaş Mustafa , Akgün Hülya , Çetın Mustafa , Eser Bülent , Ünal Ali
Published: April 2003

A case history is reported here in which leukocytosis, thrombocytosis and hypercalcemia associated with rapidly relapsing squamous cell carcinoma (SCC) of the renal pelvis were observed. In a 58-year-old man, SCC of the renal pelvis was documented during nephrolithotomy, and right nephrectomy was performed. Local relapse of the tumor occurred rapidly in 2 months’ time and hypercalcemia, leukocytosis and thrombocytosis worsened in accordance with tumor volume. Cranial computerized tomography (CT), thorax CT and bone scintigraphy were negative for metastasis. The serum parathyroid hormone level was 28 pg/ml (normal 9– 55 pg/ml). To disclose leukocytosis and thrombocytosis, peripheral smear and bone marrow aspiration were performed and no pathologic finding regarding any hematologic disorder was found; the samples were also BCR-ABL negative and Philadelphia chromosome negative. Production of several factors by tumor cells may be responsible for this paraneoplastic syndrome. The association of SCC of the renal pelvis with this triple paraneoplastic syndrome is an extremely rare occurrence.
Anisodamine Counteracts Lipopolysaccharide-Induced Tissue Factor and Plasminogen Activator Inhibitor-1 Expression in Human Endothelial Cells: Contribution of the NF-κB Pathway
Authors: Ruan Qiu-Rong , Zhan g Wei-Jian , Hufnagl Peter , Kaun Christoph , Binder Bernd R. , Wojta Johann
Published: April 2003

In this study we aimed to investigate whether the therapeutic efficacy of anisodamine in the treatment of bacteraemic shock could – at least in part – be brought about by its direct interference with the lipopolysaccharide (LPS)-induced activation of endothelial cells. Thus, we investigated the effect of anisodamine on LPS-induced expression of plasminogen activator inhibitor-1 (PAI-1) and tissue factor (TF), two major markers of endothelial activation. PAI-1 was measured in the conditioned media of human umbilical vein endothelial cells (HUVEC) by a specific enzyme-linked immunosorbent assay (ELISA) whereas TF activity was measured in the lysates of these cells by using a single step clotting assay. Results obtained in these assays were confirmed on the level of specific mRNA expression by Northern blotting using specific probes for human PAI-1 or TF. In order to evaluate a possible contribution of the NF-ĸB pathway on the effects observed, electrophoretic mobility shift assays (EMSA) were performed using nuclear extracts from HUVEC and NF-ĸB-binding oligonucleotides. When HUVEC were treated with 1 µg/ml LPS a significant increase in PAI-1 and TF activity was observed compared with cells incubated without LPS. Anisodamine dose-dependently inhibited this LPS-induced upregulation of PAI-1 and TF. Anisodamine alone had no effect on the constitutive expression of PAI-1 and TF in these cells. These effects were also confirmed on the level of specific PAI-1 and TF mRNA expression by Northern blotting. Furthermore, we could show by EMSA that anisodamine completely abolished LPS-induced NF-ĸB DNA binding activity in nuclear extracts from HUVEC treated with LPS together with anisodamine. Thus, we provide evidence that anisodamine counteracts endothelial cell activation by inhibiting LPS-induced PAI-1 and TF expression in these cells. Its interference with the NF-ĸB pathway might – at least in part – contribute to this effect. The ability of anisodamine to counteract LPS effects on endothelial cells might be one underlying mechanism explaining its efficacy in the treatment of bacteraemic shock.
Awareness of the Benefit of Periconceptional Folic Acid Supplementation in South America
Authors: Castilla Eduardo E. , da Graça Dutra Maria , Lopez-Camelo Jorge S. , Rittler Mónica , Orioli Iêda M.
Published: April 2003

Awareness of the benefit of periconceptional supplementation with folic acid was surveyed in 2,810 postpartum women by the Latin American Collaborative Study of Congenital Malformations (ECLAMC) during 1996. While 48.8% of the women claimed to know that some vitamins were good for the baby in gestation, only 0.5% mentioned folic acid. Although 13.9% of the women declared to know about the beneficial role of folic acid, only 2.2% were able to mention a correct list of foods rich in folic acid. Women delivering in private (n = 1,065) hospitals had taken vitamins, including folic acid, and had heard about its beneficial role more frequently than those within the public system (n = 1,745). Nevertheless, the public series had a larger proportion of vitamin users who started taking vitamins during the first month of gestation. Women aged 20–34 years rated better than both age extremes in stating they had taken vitamins, in actually having taken vitamins, and in having taken vitamins including folic acid. There was no significant difference between age groups, as to the time of pregnancy when vitamins were started, nor to the women`s knowledge about beneficial vitamins. Adolescent women had the lowest awareness rate concerning folic acid (11%), when compared with the two older groups (15%). Based on these results, staple food fortification seems to be the recommended strategy for the prevention of birth defects in South America.
From Family to Pharmacogenetics
Authors: Hamet Pavel , Gaudet Daniel , Bouchard Gérard , Pausova Zdenka , Tremblay Johanne , Jomphe Michèle , Larochelle Pierre , Tremblay Gérald , Kotchen Theodore A. , Cowley Allen W. , Gossard Francis
Published: April 2003

Hypertension is a complex disease in which environment and heredity interact. It is a polygenic trait that can be studied by investigating its intermediate phenotypes. We have selected the population from the Saguenay-Lac St. Jean region because it is a relative genetic isolate. We have found that the prevalence of hypertension at every age level was greater than in a control Canadian population. A sib pair analysis was used to measure familial correlation and heritability of more than 200 phenotypes among which were renal functions, anthropometric measures and cardiac morphology and reactivity. Very strong familial correlations were estimated for several cardiovascular phenotypes. During a pharmacological infusion of norepinephrine, we observed that systolic blood pressure was increased as expected but that the heritable component of this phenotype increased from null at baseline to close to 70%. Preliminary total genome scans completed on the DNA of the participants have selected several regions linked to these phenotypes. This type of study opens the door to pharmacogenetic studies in which sib pairs are analyzed for their concordant or discordant response to a medication.
Role of Genetic Diagnosis Registers in Ongoing Consultation with the Community
Authors: Modell Bernadette , Darlison Matthew , Khan Maren , Harris Rodney
Published: April 2003

The objective of genetic screening is informed choice. The UK Confidential Enquiry into Genetic Counselling by Non-Geneticists (CEGEN) aims to assess the attainment of this objective at the community level. Antenatal screening for thalassaemias and sickle cell disorders has long been standard practice in the UK. National registers of patients and of prenatal diagnoses identify all known conceptions with a fetus with thalassaemia major, and their outcomes. CEGEN viewed the obstetric records of women with an affected pregnancy in 1990–1994: risk had been detected in only half the cases. Hence nationally only half the couples at risk for thalassaemia have access to an informed choice. Most uninformed couples were British Pakistanis: the records revealed that screening had a low priority because of an assumption that they would decline genetic abortion for religious reasons. CEGEN showed that in fact over 70% of British Pakistanis request prenatal diagnosis, providing it is offered in the first trimester. This work demonstrates the power of a confidential enquiry based on genetic diagnosis registers for both assessing the delivery of screening, and eliciting the preferences of the population. The results offer an appropriate guide to acceptable service development.