bluuebunny/crossref_metadata_2025 huggingface dataset:
Payment for Performance: In Sickness and in Health. For Better or for Worse?
Authors: Goldman Richard S.
Published: December 2005

American consumers and payers are increasingly questioning the value of health care as costs increase amid reports of poor quality. Rewarding health care providers who apply evidence-based, clinical practice guidelines (CPGs) to obtain desired patient outcomes seems an intuitive, innovative method to improve health care value. Although there are many unanswered questions about payment for performance (P4P), Congress and the Centers for Medicare and Medicaid Services are developing a rapid timetable to begin P4P. Many private payers have already begun P4P programs. Currently, we lack randomized, controlled studies proving the safety, effectiveness, and patient centeredness of P4P. Many see publicly reporting provider performance as a surrogate for P4P. There is a growing literature documenting the effect of publicly reporting provider performance on the medical community and patient outcomes. It changes provider behavior, but occasionally, in undesirable ways (for example, adverse risk selection, or ‘cherry picking’). Publicly reporting or rewarding provider performance based on applying evidence-based CPGs may also have unforeseen negative patient outcomes. P4P should proceed carefully while considering unintended consequences for patients and providers. P4P should be pilot tested in the proper target patient and provider populations. Thus, we need to study how CPGs interact with one another in specific patient populations. The updating process for CPGs should include evidence-based statements concerning their impact on real patients with multiple chronic illnesses.
What Is Uremia? Retention versus Oxidation
Authors: Vanholder R. , Schepers E. , Meert N. , Lameire N.
Published: December 2005

The uremic syndrome is characterized by the retention of a host of compounds which in healthy subjects are secreted into the urine by the healthy kidneys. These compounds disturb many physiologic functions, resulting in toxicity. However, many of the retained compounds as well as many of the pathophysiologic actions of the known retention solutes remain unknown. In this publication, we review recent information on uremic toxicity. Especially the difficulty to remove compounds, such as protein-bound compounds and larger molecules, seems to play a crucial role. New strategies enhancing their removal might be highly useful. Part of the retained compounds are the result of oxidative processes due to the inflammatory status of uremic patients; however, other compounds are not, and even the concentration of oxidative compounds will be further increased by disturbances of urinary clearance.
Dialysis Patients and Cardiovascular Problems: Can Technology Help Solve the Complex Equation?
Authors: Ronco Claudio , Bowry Sudhi , Tetta Ciro
Published: December 2005

Patients with end-stage kidney disease undergoing chronic hemodialysis (HD) present higher mortality rates compared with the general population. Once patients are on HD, the risk of cardiovascular death is approximately 30 times higher than in the general population and remains 10–20 times higher after stratification for age, gender, and the presence of diabetes. About half the deaths of patients on dialysis are attributed to cardiovascular causes including coronary heart disease, cerebrovascular disease, peripheral vascular disease, and heart failure. The cardiovascular burden of the HD patient arises from three different sources: risks inherent to the patient and the uremic syndrome, traditional risk factors, and risk factors related to the dialysis therapy. Based on these considerations and the fact that several aspects of the dialysis procedure can either add to the cardiovascular burden or modify the existing burden, new technologies should be directed towards the approach of a potential ‘cardioprotective dialysis therapy’; such an approach may be facilitated by the application of new techniques and advanced dialysis machines. Created to make dialysis easy and safe, new machines feature several options that make patient monitoring and online hemodiafiltration therapy routine procedures. These and other features will possibly make dialysis better tolerated and more efficient in protecting patients from undesirable or potentially fatal cardiovascular events.
Association between Inflammation and Malnutrition as Risk Factors of Cardiovascular Disease
Authors: Kaysen George A.
Published: December 2005

Cardiovascular disease is the leading cause of death among dialysis patients. The relative risk of mortality increases as serum albumin concentration and body mass index decline. While these are generally associated with nutritional status, inflammation causes sarcopenia and decreased albumin concentration by reducing synthesis of proteins and increasing their catabolic rate. While inflammation can arise from atherosclerotic blood vessels, systemic inflammation from any source can alter the vascular endothelium and plasma protein composition in ways that promotes vascular injury. High-density lipoprotein synthesis is decreased and the high-density lipoprotein present is less capable of reducing inflammation. Activation of neutrophils favors lipoprotein oxidation. Surprisingly, while obesity is associated with cytokine production in patients without renal failure, as well as among dialysis patients, increased body mass index, whether reflecting muscle mass or adipose tissue, is associated with a decline in mortality rates.
Salt – A Potential ‘Uremic Toxin’?
Authors: Ritz Eberhard , Dikow Ralf , Morath Christian , Schwenger Vedat
Published: December 2005

It has been known for decades that salt (NaCl) determines extracellular volume as well as blood pressure and is one cause of hypertension. The difficulty to control the NaCl balance and thus treat sodium overload and hypertension in patients on dialysis has been recognized by Scribner in the early days of dialysis. In recent years, an impressive body of evidence has accumulated indicating that in essential hypertension, NaCl – blood pressure independently – causes target organ damage such as left ventricular hypertrophy, microalbuminuria, and increased aortic stiffness. It has further been recognized that NaCl increases oxidative stress and, again blood pressure independently, amplifies tissue injury induced by aldosterone. In renal damage models, progression is dramatically accelerated by high NaCl intake. Sodium as a potential culprit in progression to target organ damage in terminal renal failure has not been well investigated so far. However, it is possible, and indeed likely, that sodium plays an adverse role in the genesis of target organ damage in terminal renal failure.
Uric Acid – A Uremic Toxin?
Authors: Nakagawa Takahiko , Mazzali Marilda , Kang Duk-Hee , Sánchez-Lozada L. Gabriela , Herrera-Acosta Jaime , Johnson Richard J.
Published: December 2005

Uric acid might often be regarded as a simple marker of renal disease. Although it is well known that hyperuricemia causes gout which is associated with renal insufficiency and cardiovascular disease, one might think that it could attribute to the intrarenal urate crystal, but not to uric acid per se. In order to clarify the role of uric acid in the kidney, we hypothesized that uric acid causes renal disease. To generate mild hyperuricemia without intrarenal crystal in rats, we used low doses of an uricase inhibitor (2% oxonic acid). Hyperuricemia induced systemic hypertension, glomerular hypertrophy/hypertension, afferent arteriolar sclerosis, and macrophage infiltration in normal rat kidney. In progressive renal disease, such as cyclosporine nephropathy and remnant kidney in rat, uric acid accelerated the progression of renal disease. Thus, we concluded that uric acid is not a simple marker, but a cause of renal disease.
A Critical Assessment of Uremia Research
Authors: Locatelli Francesco , Di Filippo Salvatore , Pozzoni Pietro
Published: December 2005

There are considerably fewer randomized controlled trials investigating hemodialysis (HD) than other fields of internal medicine, and no significant improvements have been observed over time. Only the National Cooperative Dialysis Study and the HEMO trial were based on hard endpoints such as morbidity and mortality, but neither considered on-line hemodiafiltration or super-flux membranes, which are thought to provide a number of advantages in terms of the cardiovascular condition of uremic patients. However, results of well-designed clinical trials showing that increasing convection may improve the clinical outcome of HD patients are still lacking. The need for maximizing removal of uremic toxins calls for more frequent HD sessions, but this may be affected by many organizational problems. Therefore, well-designed, long-term clinical trials are urgently needed to investigate which currently available therapeutic instruments can improve the clinical outcome of uremic patients.
Access Monitoring Is Worthwhile and Valuable
Authors: Besarab Anatole
Published: December 2005

During the past several years, a limited number of small clinical trials have questioned the role of surveillance in the management of vascular accesses, since the prolongation of access longevity until replacement was not altered. Although prolongation of access life span is an important endpoint, it is not the only one. Reduction in thrombotic events reduces the risks to the patient resulting from loss of access patency. The body of evidence suggests that the detection of stenosis and prevention of thrombosis are valuable. When a test indicates the likely presence of a stenosis, venography or fistulography should be used to definitely establish the presence and the degree of the stenosis. In most cases, angioplasty should be performed if the stenosis is greater than 50% by diameter. The value of routine use of any surveillance technique for detecting anatomic stenosis alone without concomitant functional assessment by measurement of access flow, venous pressure, recirculation, or other physiologic parameter has not been established. Stenotic lesions should not be repaired merely because they are present. If such correction is performed, then intra-procedural studies of access flow or intra-access pressure prior to and following percutaneous transluminal angioplasty should be conducted to demonstrate a functional improvement with a ‘successful’ percutaneous transluminal angioplasty.
Strategies to Minimize Tunneled Hemodialysis Catheter Use
Authors: Asif Arif , Merrill Donna , Leon Carlos , Ellis Renee , Pennell Phillip
Published: December 2005

While the use of arteriovenous grafts has recently declined, there has been an astronomical increase in hemodialysis patients dialyzing with tunneled dialysis catheters (TDCs). Recent data have indicated that over 70% of the patients with end-stage renal disease initiate dialysis with a catheter. Additionally, up to 27% of the end-stage renal disease patients in the US are using TDCs as their permanent access, with placement rates having doubled since 1996. Although most modern catheters claim to provide adequate blood flow for dialysis, they are associated with the highest incidence of complications, morbidity and mortality when compared with other types of vascular access. It is for these reasons that the National Kidney Foundation Dialysis Outcomes Quality Initiative guideline 30 as well as the Fistula First Change Concept 7 emphasize limiting the use of catheters and fostering the creation of arteriovenous fistulae. Early referral has clearly been shown to minimize the use of TDCs and maximize fistulae. This report focuses on the role of additional measures that minimize TDC use, such as dialysis modality presentation and peritoneal dialysis, vascular access education, preoperative vascular mapping and salvage of early failure and thrombosed fistulae.
Cause and Consequences of Sympathetic Hyperactivity in Chronic Kidney Disease
Authors: Kotanko Peter
Published: December 2005

Patients with chronic kidney disease and patients undergoing hemodialysis treatment show a sustained overactivity of the sympathetic nervous system, which originates from signals arising in the failing kidneys and traveling via afferent renal nerves to cardiovascular centers in the brainstem. Additional important factors are increased levels of angiotensin II and asymmetrical dimethylarginine. The sympathetic overactivity contributes to hypertension and cardiovascular morbidity and mortality in that patient population. Sympathetic overactivity can be reduced by adrenergic receptor blockers, centrally acting sympathicolytic drugs such as moxonidine and rilmenidine, angiotensin-converting enzyme inhibition, and angiotensin II type 1 receptor antagonists. Daily short hemodialysis and long nocturnal hemodialysis may reduce the elevated sympathetic activity, possibly because of an increased clearance of asymmetrical dimethylarginine, an endogenous nitric oxide synthase inhibitor. Prospective trials examining the potential impact of both β-blockers and centrally acting sympatholytic drugs on cardiovascular mortality in chronic kid ney disease and hemodialysis patients are very much needed.
Proven Strategies to Reduce Cardiovascular Mortality in Hemodialysis Patients
Authors: Hampl H. , Hennig L. , Rosenberger C. , Gogoll L. , Riedel E. , Scherhag A.
Published: December 2005

Background: In hemodialysis patients, left ventricular hypertrophy (LVH) correlates with mortality. The reason for LVH in uremics is multifactorial. The primary objective of our study was to investigate the effects of a multi-interventional treatment strategy on LVH. Methods: In 230 ambulatory patients, including patients with coronary artery disease, diabetes, diastolic and systolic dysfunction, we continued optimized cardiac therapy (β-blockers, angiotensin-converting enzyme inhibitors and angiotensin receptor blockers) with full anemia correction by intravenous epoetin-β. The dose of epoetin-β for maintaining target hemoglobin (Hb) was 68 ± 23 IU/kg/week. Serial echocardiograms were recorded every 3–6 months. The mean observation period was 4.8 ± 1.2 years. Results: Mean Hb at baseline was 11.2 ± 2.0 versus 14.1 ± 1.4 g/dl (p < 0.001) at study end. There was a significant reduction in left ventricular mass index (LVMI: 159 ± 50.4 vs. 130.2 ± 42.7 g/m2; p < 0.001). In a subgroup of 2/3 of the patients, LVMI returned to normal (169 ± 33 vs. 114 ± 14 g/m2; p < 0.001). Conclusion: Baseline LVMI (p < 0.001), Hb increase (p < 0.03), and triple cardiac therapy (p < 0.03) were significant and independent prognostic factors for a reduction in LVMI. The annual cardiovascular mortality was 5%. Even anemia correction from 12 to 14 g/dl results in further (p < 0.001) regression of LVMI.
Peritoneal Dialysis in Europe: An Analysis of Its Rise and Fall
Authors: Lameire Norbert , Peeters Patrick , Vanholder Raymond , van Biesen Wim
Published: December 2005

There is a wide variability in the utilization of peritoneal dialysis throughout Europe that cannot be explained by differences in medical factors alone. Over the last years, the utilization of peritoneal dialysis has even changed in individual countries. The main factors of this variation are not medical, but rather changes in reimbursement and other financial considerations. The overall structure of the health care in an individual country is to a large extent determining the use of a renal replacement therapy modality.
Chronic Kidney Disease Management – What Can We Learn from South African and Australian Efforts?
Authors: Katz I.J. , Hoy W.E. , Kondalsamy-Chennakesavan S. , Gerntholtz T. , Scheppingen J. , Sharma S. , Butler O. , Shezi E. , Mdleleni G. , Mthombeni D.
Published: December 2005

Background: The prevalence of chronic kidney disease is on the rise. Our objective is to describe two programs to improve the awareness and management of hypertension, renal disease, and diabetes in remote Australian Aboriginal and urban and periurban South African communities. We focus on how the Australian Aboriginal and South African Chronic Disease Outreach Programs have worked together. Methods: The establishment of prevention programs in developing countries is a challenge. The paper evaluates these challenges, including accessing international aid. The programs advocate that regular integrated checks for chronic disease and their risk factors are essential elements of regular adult health care. Programs should be run by primary health workers, following algorithms for testing and treatment, and a backup provided by nurse coordinators. Constant evaluation is essential to develop community health profiles and adapt program structure. Results: Both programs are discussed, including how they are organized to deliver preventative and treatment strategies. The challenges and adaptations required are outlined. Conclusions: It is the aim of the international kidney commu- nity to prevent chronic kidney disease. The South African and Australian groups highlight the need for a systematic and sustained approach to the management of chronic diseases to achieve this goal.
Randomized Trials of Frequent Hemodialysis – Infinite Possibilities
Authors: Suri Rita S. , Garg Amit X.
Published: December 2005

Current understanding of the benefits of daily and nocturnal hemodialysis is limited by the designs used in previous studies which tested these therapies. There is increasing momentum to evaluate these therapies in randomized controlled trials. By establishing the efficacy, safety and delivery of daily and nocturnal hemodialysis, such trials will guide policy and funding decisions regarding the role of these therapies in the treatment of end-stage renal disease. However, in designing trials that fulfill these goals, many questions arise. What study population should be used? How should the intervention be defined? Which outcomes should be evaluated? Several methodological issues particular to trials of frequent dialysis require additional consideration. The ‘ideal’ trial in terms of methods must be balanced against the feasibility of implementation and budgetary constraints. Here, we explore some of these issues.
Debate Forum: Carnitine Supplements Have Not Been Demonstrated as Effective in Patients on Long-Term Dialysis Therapy
Authors: Handelman Garry J.
Published: December 2005

The database for carnitine supplements in dialysis includes no large-scale randomized trials and no registered trials. Medical practitioners prefer to make treatment decisions based on the outcome of randomized clinical trials, with appropriate controls. Furthermore, registered trials provide a further level of integrity, since trial registration avoids publication bias by ensuring that all outcomes are reported, including trials that are not completed. Positive effects reported from carnitine administration in dialysis patients include decreased erythropoietin dose, increased hematocrit, less intradialytic hypotension, and less fatigue. The evidence for carnitine effectiveness is limited to trials that are mostly open-label and that include no more than a total of 1,000 patients. An analysis of recent carnitine administrations to patients in a large dialysis practice database indicates no overall change in hemoglobin or erythropoietin dose following 6 months of carnitine administration. As outcomes of controlled trials with appropriate power to examine for the benefits of carnitine are not yet available, the dialysis practitioner cannot justify the administration of carnitine.
A Nexus of Progression of Chronic Kidney Disease: Charcoal, Tryptophan and Profibrotic Cytokines
Authors: Schulman Gerald
Published: December 2005

Fibrosis plays a role in the pathogenesis of progressive chronic kidney disease (CKD). The inhibition of the renin-angiotensin system, which promotes fibrosis, has become the standard of care in the treatment of patients with CKD. A novel charcoal compound, AST-120, has been used for over a decade in Japan to prevent progression of CKD. It is thought that the oral administration of AST-120 blocks the intestinal absorption of tryptophan-derived indole. This prevents the hepatic conversion of indole to indoxyl sulfate (IS). IS has been shown to stimulate the production of profibrotic cytokines such as transforming growth factor-beta. AST-120 lowers IS in a dose dependent fashion and does not change the creatinine appearance rate in the urine. Enteric capsules containing Bifidobacterium longum have been shown to prevent progression of CKD in a preliminary study. These findings suggest that prospective clinical trials be undertaken to determine if these other potential methods of inhibiting fibrosis are useful in slowing progressive CKD.
Oxidant and Carbonyl Stress-Related Apoptosis in End-Stage Kidney Disease: Impact of Membrane Flux
Authors: Bordoni Valeria , Piroddi Marta , Galli Francesco , de Cal Massimo , Bonello Monica , Dimitri Petras , Salvatori Gabriella , Ranishta Rattanarat , Levin Nathan , Tetta Ciro , Ronco Claudio
Published: December 2005

Apoptosis is a highly regulated process which mostly affects cell-mediated immunity. In this open-label, randomized, prospective clinical study, we determined the impact in 10 hemodialysis patients treated with high-, medium-, and low-flux membranes on spontaneous or plasma-induced apoptosis, on monocytes, as well as on oxidant and carbonyl stress. High- and medium-flux membranes significantly reduced patients’ plasma-dependent proapoptotic activity on U937 monocytic cell lines. Patients who had the highest levels of plasma-induced proapoptotic activity exhibited the highest plasma levels of advanced oxidation protein products (AOPPs) and carbonyls. Plasma carbonyl residues but not AOPPs were significantly lowered. Finally, a significant correlation could be drawn between the extent of plasma-induced proapoptotic activity and both plasma carbonyl and AOPP levels.
Surgical Treatment Outcomes in Subdural Effusion: A Clinical Study
Authors: Yilmaz N. , Kiymaz N. , Yilmaz C. , Bay A.
Published: December 2005

Objective: In this study, patients who underwent surgery due to subdural effusion were retrospectively analyzed. The location, depth and etiology of the subdural effusion, the surgical approach that was used and the recurrence rates were studied in these patients. Method: A total of 32 patients who were followed up and treated for subdural effusion at the Neurosurgery Clinic of the Yüzüncü Yıl University School of Medicine were included in the study; 18 (56%) of the patients were male and 14 (44%) were female. The surgical techniques applied were surgical burr hole drainage, repeated subdural transaxial puncture and subduroperitoneal shunt approaches. The patients were evaluated by computerized tomography of the brain in week 1 and in the third month after surgery. Recurrences were evaluated based on radiological findings and the clinical condition of the patients. Result: The consciousness level of the patients was proportional to the mass effect of the subdural effusion. Lower recurrence rates were found in patients with a large midline shift resulting from the subdural effusion. In addition, recurrence rates were higher in patients with cerebral atrophy and lower protein content in the subdural effusion fluid. It was observed that these patients responded better to the subduroperitoneal shunt treatment.
Prevalence of Primary Tethered Cord Syndrome Associated with Occult Spinal Dysraphism in Primary School Children in Turkey
Authors: Bademci Gulsah , Saygun Meral , Batay Funda , Cakmak Aytul , Basar Halil , Anbarci Huseyin , Unal Birsen
Published: December 2005

The prevalence and associated factors of primary tethered cord syndrome (PTCS) in primary school children were investigated. A cross-sectional study was performed in four demographically different primary schools in Turkey. Demographic, familial and physical data were collected from 5,499 children based on enuresis as a predominant symptom and dermatologic and orthopedic signs as clues of occult spinal dysraphism. Statistical analysis and input of the data were carried out with the SPSS package program 10.00, and logistic regression analysis was used to identify discriminating factors between enuretic children with or without neurologic signs. Of 5,499 analyzed children, 422 (7.7%) had enuresis nocturna, and 19.9% of 422 children had also daytime incontinence. Sixteen of these 422 enuretic children (3.8%) had several dermatologic signs. Five of them had spina bifida on plain radiographies, and 4 of them had cord tethering on lumbar MRI. Fifteen of 422 enuretic children (3.7%) had gait disturbances and orthopedic anomalies without cutaneous manifestations. Six of 15 children had spina bifida on plain graphies and 2 of them had tethered cord syndrome on MRI. The general prevalence of PTCS was found to be 0.1% of 5,499 analyzed children and 1.4% of enuretic children. A good outcome after untethering was found in 83.0% in this series. Practitioners should be aware of these clues of occult spinal dysraphism and resort to further radiologic and neurosurgical assessment. Early surgical intervention may halt the progression of the neurologic deficits and stabilize or reverse symptoms.
The Chiari Pseudotumor Cerebri Syndrome: Symptom Recurrence after Decompressive Surgery for Chiari Malformation Type I
Authors: Fagan Lisa H. , Ferguson Sherise , Yassari Reza , Frim David M.
Published: December 2005

Introduction: The etiology of Chiari malformation type I (CM1) as well as other anomalies associated with CM1 remains poorly defined. We have noted the presence of elevated CSF pressures with small ventricles, consistent with the pseudotumor cerebri (PTC) syndrome in a group of CM1 patients that did not respond over the long term to posterior fossa decompression. In order to better understand this association, we reviewed a series of CM1 patients treated by posterior fossa decompression to define the prevalence and nature of post-Chiari PTC. Methods: We performed a retrospective chart review of 192 patients diagnosed with CM1 and treated by posterior fossa decompression. Patients who failed to respond to surgery were evaluated by CINE MR flow studies to assess presence of CSF flow at the foramen magnum and then by lumbar puncture if flow was present. The diagnosis of Chiari PTC was defined by recurrence of Chiari-like symptoms after decompression, elevated lumbar CSF pressure in the absence of ventriculomegaly, and transient resolution of symptoms with large volume lumbar CSF drainage. Results: Thirty-six of 192 patients did not improve with surgical decompression. Fifteen of 36 operative CM1 patients (41.6%) were found to have Chiari PTC. The most frequent symptoms of CM1/PTC patients were head pain, body aches, and balance difficulties. Three patients also experienced visual complaints. The mean maximum lumbar CSF pressure documented in this cohort was 26 cm of water in adults and 25.3 in children. All patients received treatment for the CM1/PTC that culminated with CSF shunt placement in 14/15. Seven of 9 pediatric patients had significant symptom resolution while 6/6 adult patients remained variably symptomatic. Conclusion: CM1 and PTC co-exist in a surprising percentage of failed operative CM1 patients and present with a syndrome that is difficult to treat. The etiology of this association after Chiari decompression is unclear, though perhaps posterior fossa surgery in the setting of abnormal anatomy and potentially anomalous CSF flow dynamics contributes to CSF malabsorption and resultant or coexistant PTC.
Clinical and Electroencephalographic Features of Infantile Spasms Associated with Malformations of Cortical Development
Authors: Kang Hoon Chul , Hwang Yong Soon , Park Jung Chae , Cho Woo Ho , Kim Soung Hee , Kim Heung Dong , Park Sang Keun
Published: December 2005

The aim of this study was to reveal the clinical and encephalographic (EEG) features of infantile spasms (IS) with malformations of cortical development (MCDs). The clinical features, EEG findings, neuroimaging studies and outcomes of various therapeutic modalities for 27 patients with IS and MCDs were reviewed. Background activities of EEG on the MCDs, i.e. asymmetric hypsarrhythmia, localized persistent polymorphic slowing, asymmetric slowing and diffuse fast activities, were shown in 22, 15, 9 and 2 patients, respectively. Partial epileptiform discharges such as localized paroxysmal fast activities, spindle-shaped fast activities and subclinical seizures were shown in 15, 8 and 10 patients, respectively, and the lateralized prominence of generalized paroxysmal fast activities and generalized sharp and wave discharges in 4 and 5 patients, respectively. MCDs were suspected in 5 patients, as revealed by EEG and/or functional neuroimagings without distinct magnetic resonance imaging lesions, and confirmed by pathologic findings. Of the 11 patients treated with surgical resection, 8 became seizure free. EEG features can be very useful in the identification of underlying cortical dysgenesis in patients with IS. However, in one patient who underwent epilepsy surgery in early infancy, we observed easy bleeding and difficult hemostasis from friable vascular and parenchymal tissues. Various EEG features can be very useful in screening underlying MCDs. In addition, epilepsy surgery can be an effective therapeutic modality in many patients with otherwise medically intractable IS with MCDs. However, surgical intervention in extremely young infants should be performed with caution.
Assessment of Quality of Continuous Intracranial Pressure Recordings in Children
Authors: Eide P.K.
Published: December 2005

Background: Bad quality of continuous intracranial pressure (ICP) recordings may potentially give wrong diagnostic information. Method: This study examined the ICP recordings of 33 children undergoing ICP monitoring during a 12-month period. Their ICP recordings were stored as raw data files and analyzed retrospectively. An automatic algorithm applied to every subsequent 6-second time window of each recording assessed the quality of ICP recordings. Time windows containing 4–18 cardiac beat-induced single ICP waves were accepted; time windows containing artifact-induced pressure waves due to noise in the signal were rejected. For every ICP recording, the number of accepted and rejected time windows were determined. In addition, the raw data ICP signal was assessed by visual inspection to verify the automatic algorithm. Two methods of computing mean pressure of a time window are compared; mean ICP was either computed according to current practice independent of the presence or absence of single ICP waves or computed only for single ICP waves within the time window (meanSW ICP). Results:In 4 of the 33 patients (12%), more than 70% of the time windows of their ICP recordings were rejected; in 3 patients 95–100% of time windows were rejected. Visual inspection of the raw ICP signals confirmed the absence of single ICP waves. In these cases, computing meanSW ICP, not by mean ICP, revealed the bad signal quality. Conclusions: This retrospective study showed that in 3 of 33 patients (9%) the quality of the ICP recordings was so bad that they gave wrong diagnostic information. One way of obtaining quality control is to compute meanSW ICP, determined only for cardiac beat-induced single ICP waves within a time window.
Spinal Intradural-Intramedullary Cavernous Malformation
Authors: Bakır Abdurrahman , Savas Ali , Yilmaz Erdal , Savaş Berna , Erden Esra , Cağlar Şükrü , Sener Özden
Published: December 2005

Cavernous angiomas or cavernomas are uncommon vascular malformations of the central nervous system and spinal involvement is much rarer especially in pediatric patients. We report a case of spinal intradural-intramedullary cavernous angioma in a 14-year-old male child. The cavernoma was located at the level of C6–C7 at the dorsal part of the spinal cord. The diagnosis was made with MRI and the patient underwent surgical treatment. The cavernoma was totally removed with laminotomy and microsurgical techniques. Somatosensory evoked potential monitoring was also used peroperatively. The clinical, radiological and surgical features of this rare case were presented and discussed with reference to the literature.
Dual Occurrence of Pineal Germinoma and Testicular Seminoma
Authors: Ho Chi Long , Deruytter Marc J
Published: December 2005

A 16-year-old male presented with obstructive hydrocephalus secondary to pineal germinoma. There have been many reported cases of abdominal metastasis of pineal germinoma after ventriculoperitoneal shunting. Endoscopic ventriculostomy was preferred in our case, thus avoiding iatrogenic peritoneal seeding, but spinal metastasis was unavoidable. Metastatic infiltration of the ventricular system and spinal meninges is the commonest mode of spread. Later, the patient underwent orchidectomy for an asymptomatic left testicular seminoma. He responded to chemotherapy, and had a complete recurrence-free remission for more than 10 years. As far as we know there are only a handful of reported cases of dual occurrence of two primary germ cell tumors (GCT), i.e. gonadal seminoma and pineal germinoma with spinal seeding. We also addressed the controversial subject of radiation versus chemotherapy in the management of patients with pineal germinomas. A review of the relevant literature and recommendations for future treatment of similar cases are discussed.
Fatal Haemorrhage in Medulloblastoma following Ventricular Drainage
Authors: Elgamal Essam A. , Richards Peter G. , Patel U. Jash
Published: December 2005

Haemorrhage in medulloblastoma is reported to be very rare. The authors report a case of a 13-year-old boy who presented with headache, unsteadiness, diplopia and papilloedema due to posterior fossa medulloblastoma causing obstructive hydrocephalus. Six hours following placement of an external ventricular drain, he suddenly became comatose with respiratory arrest. The cause was marked upward herniation of the anterior vermis and downward herniation of the cerebellar tonsils due to massive spontaneous intratumoural haemorrhage extended into the ventricular system. This atypical clinical course of fatal haemorrhage in medulloblastoma after insertion of external ventricular drainage is reported and the literature discussed.
Giant Terminal Lipomyelocystocele
Authors: Gupta Deepak Kumar , Ramdurg Shashank , Mahapatra A.K.
Published: December 2005

Terminal myelocystocele is a rare form of occult spinal dysraphism in which the hydromyelic caudal spinal cord and the subarachnoid space are herniated through a posterior spina bifida. A 1-year-old female child presented with a large lumbosacral mass (30 × 20 × 10 cm), flaccid paraplegia and urinary incontinence since birth. Magnetic resonance imaging revealed a low-lying conus (with associated conus lipoma) and a dilated central canal surrounded by a meningocele suggestive of terminal lipomyelocystocele and was operated on successfully. In our experience, this was a giant terminal lipomyelocystocele and such a large lesion has not been reported in the literature before. Terminal myelocystocele should be included in the differential diagnosis of congenital lesions presenting as a lumbosacral mass and operated early.
Neurenteric Cyst of the Upper Cervical Spine: Excision via Posterior Approach
Authors: Tuzun Yusuf , Izci Yusuf , Sengul Goksin , Erdogan Fazli , Suma Selami
Published: December 2005

Neurenteric cyst of the cervical spine is an uncommon congenital lesion and usually associated with many craniocervical anomalies. Solitary neurenteric cyst of the upper cervical spine is very rare. Surgery is the main treatment method. An 11-year-old female child presented with the symptoms of neck pain that radiated to both shoulders and arms. Magnetic resonance imaging of the patient showed a cystic mass lesion ventral to the spinal cord and lying at the level from C1 to C3. She underwent surgical resection via a posterior approach and complete cyst resection was performed. Although removal of a neurenteric cyst through a posterior approach may result in spinal cord injury, this method is easier and safer than the anterior approach, especially in children.
Alagille Syndrome and Aneurysmal Subarachnoid Hemorrhage
Authors: Tumialán Luis M. , Dhall Sanjay S. , Tomak Patrick R. , Barrow Daniel L.
Published: December 2005

The authors report the case of a 21-year-old female with a known history of Alagille syndrome (AGS) who was found to have a basilar terminus aneurysm without evidence of rupture. Prior to intervention, the patient’s hospital course became complicated by multiple medical problems associated with AGS. Subsequently, the patient had an acute neurological decline. An unenhanced CT of the head demonstrated diffuse subarachnoid hemorrhage, intraparenchymal hematoma and intraventricular hemorrhage. AGS is an autosomal dominant arteriodysplastic syndrome with multiple organ system involvement caused by a mutation in the Jagged1 gene. Intracranial hemorrhage is one of the many complications observed in this patient population. While there are multiple case reports in the literature reviewing the spectrum of cerebrovascular events and abnormalities, intracranial aneurysmal rupture has only recently been described. To our knowledge, this is the third reported case of documented aneurysmal subarachnoid hemorrhage in a patient with AGS. The authors present a brief review of the vascular abnormalities both intracranial and systemic seen in AGS. The genomic abnormalities of this syndrome are also reviewed with particular attention to the Jagged1 gene and the Notch receptor signaling pathway which may reveal elements of the pathophysiology involved in aneurysm formation and rupture in AGS patients. In light of the increased incidence of intracranial hemorrhage in AGS and the possible link to aneurysmal subarachnoid hemorrhage, establishing the incidence of intracranial aneurysms in AGS and the role of screening these patients is indicated.
Mycotic Aneurysms of Aortic Root and Aorta-to-Left Atrial Fistula Complicating Bicuspid Aortic Valve Endocarditis
Authors: Özerkan Filiz , Duygu Hamza , Nalbantgil Sanem , Çınar Cahide Soydaş
Published: December 2005

Unlike root abscess, fistula formation is quite uncommon in aortic valve endocarditis. In this report, we describe a patientwith subacute bicuspid aortic valve endocarditis complicated by aortic insufficiency, mycotic aneurysms of the aortic root and fistulous communication between the aorta and the left atrium and his recovery upon surgical treatment.
Changes in Localization of Amino Acids in the Detached Cat Retina
Authors: Sasoh Mikio , Ma Ning , Ito Yoshikazu , Esaki Koji , Uji Yukitaka
Published: December 2005

Purpose: To investigate the distribution of amino acids (glutamate, aspartate, glutamine, GABA, glycine) in detached retinas with minimum postmortem artifact and to clarify the relation between amino acid distribution and histopathological change in the outer portion of detached retinas. Methods: Unilateral retinal detachment was produced in cats by injecting 0.25% sodium hyaluronate into the subretinal space using a glass micropipet. The eyes were fixed by perfusion for 10 min, 1, 3, 6 and 24 h, 2, 3 and 7 days after detachment and then examined under conventional light- and electron-microscopic immunocytochemistry. Results: For glutamate, aspartate and glutamine, the inner segments and perikarya of the photoreceptor cells, which were not immunopositive in the normal retinas, showed various degrees of immunoreactivity immediately after retinal detachment. Photoreceptor cells with the strong immunoreactivity developed necrosis. The staining pattern of GABA and glycine scarcely changed during the course of retinal detachment. Conclusions: Excess intracellular glutamate, aspartate and glutamine in photoreceptor cells may cause a part of neuronal death after retinal detachment.
49,XXXXY Syndrome with Diabetes Mellitus
Authors: Kim Hee Jin , Kim Dongmin , Shin Jae Min , Chung Hyun-Kyung , Lee Gilho
Published: December 2005

49,XXXXY syndrome is a rare sex chromosome aneuploidy and characterized by mental retardation, skeletal defects, craniofacial anomalies and hypogonadism. The increased frequency of diabetes mellitus in patients with Klinefelter syndrome and other types of X-chromosome polysomy has been reported, but no cases of diabetes mellitus in adult with 49,XXXXY syndrome have been reported so far. We report an 18-year-old patient with 49,XXXXY syndrome accompanying diabetes mellitus.
Application of Newly Developed Amniotic Membrane Ointment for Photorefractive Keratectomy in Rabbits
Authors: Kim Tae Hyun , Lee Dong Yeoul , Rho Jee Hyun , Rho Sae Heun , Yoo Kyung Won , Ahn Hee Bae , Yoo Young Hyun , Park Woo Chan
Published: December 2005

We developed amniotic membrane ointment (AMO), and the effect of instilling the AMO after photorefractive keratectomy (PRK) was investigated with respect to inflammatory cell infiltration into the corneal stroma, apoptosis of keratocytes, and suppression of lipid peroxidation of cellular walls. The PRK procedure was performed on both eyes of 10 white rabbits. One eye of each rabbit (the experimental eye) was instilled with the AMO and the other eye of the rabbit (the control eye) with a base ointment 0, 8 and 16 h after the PRK procedure. Corneal specimens were collected 24 h after the PRK procedure. Hematoxylin-eosin stain and TUNEL assay were conducted to demonstrate polymorphonuclear and apoptotic cells, respectively. To assess lipid peroxidation, immunohistochemical staining with an antibody to malondialdehyde was undertaken. Compared to the control, the cornea instilled with the AMO had significantly less polymorphonuclear cells infiltrating into the corneal stroma as well as keratocytes subjected to apoptosis. These corneas also showed a significantly less extent of lipid peroxidation than the control. These data support that instillation of the AMO effectively reduced the recruitment of polymorphonuclear cells, the induction of apoptosis in keratocytes, and lipid peroxidation induced by PRK. Thus, this study could provide basic data on the clinical application of the AMO in the days ahead.
Frequency of Chromosome 17 Aneuploidy in Primary and Recurrent Pterygium by Interphase-Fluorescence in situ Hybridization
Authors: Kamis Umit , Kerimoglu Hurkan , Ozkagnici Ahmet , Acar Hasan
Published: December 2005

Aim:To investigate chromosome 17 numerical aberrations by using fluorescence in situ hybridization (FISH) in pterygia and to find out whether there is any association between chromosome 17 aneuploidy and recurrent pterygia. Methods: Pterygium tissue samples were taken from 21 patients by surgical excision. Eighteen of them had primaryand 3 had recurrent pterygium. Peripheral whole blood interphase cells obtained from 11 healthy subjects were assigned as control group. The cells from pterygium tissue and peripheral blood were incubated with a hypotonic solution and fixed in order to obtain interphase nuclei. FISH analysis with chromosome-17-specific α-satellite DNA probe was performed on both the interphase nuclei of pterygium tissue (of patients) and peripheral whole blood cells of controls. Results: The mean percentage of chromosome 17 aneuploidy was 4.71% for the pterygia group and 4.41% for the controls. No significant difference of chromosome 17 aneuploidy was observed between the patients and the controls. When the group of patients with recurrences was compared with the group without recurrences, there was a significant difference in the frequency of chromosome 17 aneuploidy (U = 17, p = 0.029). Conclusions: Chromosome 17 aneuploidy is probably not an important factor in the formation of pterygium, but it may be related to recurrence.
Transient Left Ventricular Apical Ballooning
Authors: Rosenmann David , Balkin Jonathan , Butnaru Adi , Wanderman Kenneth , Klutstein Marc , Tzivoni Dan
Published: December 2005

Background: Transient left ventricular (LV) apical ballooning is characterized by acute onset of chest pain with reversible balloon-like LV motion abnormality, hypercontractile basal segments, ST segment elevation or T-wave inversion in anterior chest leads and mild cardiac enzyme rise in the absence of significant coronary disease. Methods: We describe 5 patients (4 females) with anteroapical ballooning who were hospitalized with acute myocardial infarction and showed ST segment elevation in anterior chest leads. Results: Echocardiogram demonstrated apical ballooning with normal or hypercontractile contraction of the basal segments. Four patients had severe mitral incompetence and one had mild incompetence. All patients had also systolic anterior motion and 4 had a significant LV outflow (LVOT) gradient. All patients underwent cardiac catheterization soon after admission showing non-significant narrowing of the coronary arteries. At discharge 4 patients had normal LV function and 1 was mildly impaired. Conclusions: LV apical ballooning is relatively rare. It should be suspected in older patients, mainly women, with severe mitral incompetence and LVOT gradient.
Anti-Irritants: Myth or Reality? An Overview
Authors: Ford Christina , Maibach Howard I.
Published: December 2005

Anti-irritants, whether naturally occurring or man-made, are substances that provide a soothing effect to irritated skin and reduce damage by a variety of mechanisms, including reduced absorption and/or biochemical manipulation of noxious chemicals. These products show promise in reducing irritation caused by acute and chronic exposure to known irritant chemicals; however, the data on anti-irritants are incomplete. Controlled trials of the efficacy of proposed anti-irritants substances in reducing irritant contact dermatitis in human and animal models are reviewed. Based on our literature and hand search, anti-irritants seem promising in treating and preventing a variety of irritant contact dermatitis conditions, but their true effects remain sub judice. Many studies do not allow deduction of clinical effects. Further experimentation must be performed to assess sensitivity and specificity of each anti-irritant to their specific irritant-inducing substance.
Factors Determining Patient Choice of Device for GH Therapy
Authors: Wickramasuriya B.P.N. , Casey A. , Akhtar S. , Zia R. , Ehtisham S. , Barrett T.G. , Shaw N.J. , Kirk J.M.W.
Published: December 2005

Aim: To assess the factors determining patient choice of GH device, and whether offering free patient choice improves compliance with GH therapy. Methods: A prospective cross-sectional study performed on patients offered free choice of GH device in a regional growth clinic. In a subgroup having home delivery, GH compliance was assessed using ampoule counts. Results: 125 patients (74 (59%) male), median (range) 9.30 (1.0–18.3) years were commenced on GH from January 2001 to March 2004, and offered free choice of device. 68 (54%) chose a needled device, and 57 (46%) needle-free. There was no statistical difference in age, sex or diagnostic category between the two groups. Light blue devices were more likely to be chosen by males (p = 0.056). Questionnaires giving reasons for choosing a device were available in 40, and a further 50 gave reasons for both choosing a specific device and not choosing others. Other than choice of needled/needle-free device, the factor most likely to determine choice was ‘ease of use’. Only 6 (4.8%) subsequently changed device, and compliance remained high but unchanged at ∼90%. Conclusions: There are no specific features which determine what GH device a patient will choose. For those units offering free patient choice, a wide range of different devices should be made available.