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Significant Morbidity (significant + morbidity)
Kinds of Significant Morbidity Selected AbstractsUtilization of the Edge-to-Edge Valve Plasty Technique to Correct Severe Tricuspid Regurgitation in Patients with Congenital Heart DiseaseJOURNAL OF CARDIAC SURGERY, Issue 6 2009Yong-chao Cui Significant morbidity and mortality are related to tricuspid valve replacement. Tricuspid valve plasty is still a preferred choice. This report deals with our surgical experience in using the edge-to-edge valve plasty technique to correct severe tricuspid regurgitation in patients with congenital heart disease. Methods: From December 2002 to August 2007, severe tricuspid regurgitation was corrected with a flexible band annuloplasty and edge-to-edge valve plasty technique in nine patients with congenital heart disease. The age ranged from 7 to 62 years (average 24.4 years). Congenital cardiac anomalies included atrioventricular canal in five cases, secundum atrial septal defect in three cases, and cor triatriatum in one case. Results: No hospital death or postoperative morbidity occurred. No or trivial tricuspid regurgitation was present in six cases and mild tricuspid regurgitation in three cases at discharge. The follow-up ranged from 12 months to 70 months (average 39.3 months). No tricuspid stenosis was found. No to mild tricuspid regurgitation was present in eight cases, and moderate tricuspid regurgitation in one case at the latest follow-up. Conclusions: Edge-to-edge valve plasty is an easy, effective, and acceptable additional procedure to correct severe tricuspid regurgitation in patients with congenital heart disease. [source] An alternate-day corticosteroid regimen for pemphigus vulgaris.JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 10 2007A 13-year prospective study Abstract Background, Pemphigus vulgaris (PV) at the early, usually oral and relatively stable stage, represents the majority of PV patients. Treatment modalities usually do not differ compared to those for the fully established disease. Objectives, To prospectively assess a standardized and effective therapeutic approach that aims at less morbidity due to adverse reactions. Methods, The following regimen, also known as Lever's mini treatment (LMT), was used. Forty mg of oral prednisone on alternate days plus 100 mg azathioprine every day were administered until the complete healing of all lesions. A gradual monthly and later bimonthly decrease of prednisone was followed by the tapering of a second immunosuppressive agent, in a one-year period. Results, Seventy-four patients suffering from early-stage-PV, and representing 70% of all PV patients seen through the years 1991,2003, were eligible in the study. Total follow-up period was 76 ± 37 (26,180) months. During the 53 ± 26 months of LMT, 6 (8%) patients dropped out of therapy, 9 (12%) required a change to another treatment, two (3%) died and 57 (77%) achieved a lesion-free condition. Forty-five (61%) patients were in complete remission for 27 ± 29 months. Significant morbidity was estimated 4/74 (5.2%). Disease ,breakthroughs' necessitating treatment adjustments occurred in 30 patients, usually throughout the last phase of therapy and post-treatment follow-up. Conclusion, LMT may be a standardized therapeutic approach for the early and relatively stable stage of PV, resulting in high efficacy, safety and quality of life profile. [source] The Long-Term Evaluation of Tracheostomy in the Management of Severe Obstructive Sleep Apnea,THE LARYNGOSCOPE, Issue 2 2003Gentry W. Thatcher MD Abstract Objective To determine the long-term success and morbidity of tracheostomies for patients with severe obstructive sleep apnea. Study Design A retrospective study of patients who have undergone tracheostomy for documented obstructive sleep apnea at Hennepin County Medical Center since 1979. Methods Retrospective chart review of all tracheostomy patients operated January 1, 1979, to June 30, 1999, were evaluated. Follow-up data from patient records, death certificates, and DMV. Results Seventy-nine patients (70 men and 9 women; age range, 25,70 y; mean age, 47 y) received tracheostomies at a regional sleep disorder center. Respiratory distress index ranged from 45 to 146 (mean value, 81). Tracheostomy eliminated obstructive sleep apnea in all cases. Follow-up ranged from 3 months to 20 years (mean period, 8.3 y). In all, 16 patients had decannulation (range, 2 mo to 13 y): 5 of the patients chose continuous positive airway pressure, 3 grew intolerant of their tracheostomy, 3 had obstructive sleep apnea fully resolve after uvulopalatopharyngoplasty, 3 unknown diagnosis for decannulation, 2 had significant weight loss. Morbidity seen within the first year was primarily limited to granulation tissue, infection, and stoma revision. Fourteen deaths were identified. Average age at time of death was 62 years. Five deaths were cardiopulmonary related, four were from cancer, two were from postoperative complications of unrelated surgery, and one was from aspiration. Tracheostomy-related mortality included one postoperative myocardial infarction and one tracheal-innominate fistula. Conclusion Severe obstructive sleep apnea and its comorbid conditions are effectively treated in the long term with tracheostomy. Initial management of more frequent complication is well tolerated. Significant morbidity and mortality are low. Chances of obstructive sleep apnea resolution allowing decannulation remain poor. [source] Primary rectus abdominis myocutaneous flap for repair of perineal and vaginal defects after extended abdominoperineal resectionBRITISH JOURNAL OF SURGERY (NOW INCLUDES EUROPEAN JOURNAL OF SURGERY), Issue 4 2005S. W. Bell Background: Significant morbidity can result from perineal wounds, particularly after radiotherapy and extensive resection for cancer. Myocutaneous flaps have been used to improve healing. The purpose of this study was to evaluate the morbidity and results of primary rectus abdominis myocutaneous flap reconstruction of the vagina and perineum after extended abdominoperineal resection. Methods: Thirty-one consecutive patients undergoing one-stage rectus abdominis myocutaneous flap reconstruction of extensive perineal wounds were studied prospectively. Twenty-six patients had surgery for recurrent or persistent epidermoid anal cancer or low rectal cancer, and 21 had high-dose preoperative radiotherapy. Results: Three weeks after the operation, complete healing of the perineal wound was seen in 27 of the 31 patients. There were nine flap-related complications including three patients with partial flap necrosis, two with vaginal stenosis, one with vaginal scarring, one with small flap disunion and two with weakness of the anterior abdominal wall. There were no unhealed wounds at the completion of follow-up (median 9 months). Conclusion: The transpelvic rectus abdominis myocutaneous flap for the reconstruction of large perineal and vaginal wounds achieves wound healing with only moderate morbidity in the majority of patients after extensive abdominoperineal resection with or without radiotherapy. Copyright © 2005 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd. [source] Repeat Trigeminal Nerve Radiosurgery for Refractory Cluster Headache Fails to Provide Long-Term Pain ReliefHEADACHE, Issue 2 2007Shearwood McClelland III MD Objective/Background.,Medically refractory cluster headache (MRCH) is a debilitating condition that has proven resistant to many modalities. Previous reports have indicated that radiosurgery for MRCH provides little long-term pain relief, with moderate/significant morbidity. However, there have been no reports of repeated radiosurgery in this patient population. We present our findings from the first reports of repeat radiosurgery for MRCH. Methods.,Two patients with MRCH underwent repeat gamma knife radiosurgery at our institution. Each fulfilled clinical criteria for treatment, including complete resistance to pharmacotherapy, pain primarily localized to the ophthalmic division of the trigeminal nerve, and psychological stability. Both patients previously received gamma knife radiosurgery (75 Gy) for MRCH with no morbidity, but no long-term improvement of pain relief (Patient 1 = 5 months, Patient 2 = 10 months) after treatment. For repeat radiosurgery, each patient received 75 Gy to the 100% isodose line delivered to the root entry zone of the trigeminal nerve, and was evaluated postretreatment. Pain relief was defined as: excellent (free of MRCH with minimal/no medications), good (50% reduction of MRCH severity/frequency with medications), fair (25% reduction), or poor (less than 25% reduction). Results.,Following repeat radiosurgery, long-term pain relief was poor in both patients. Neither patient sustained any immediate morbidity following radiosurgery. Patient 2 experienced right facial numbness 4 months postretreatment, while Patient 1 experienced no morbidity. Conclusion.,Repeat radiosurgery of the trigeminal nerve fails to provide long-term pain relief for MRCH. Given the reported failures of initial and repeat radiosurgery for MRCH, trigeminal nerve radiosurgery should not be offered for MRCH. [source] Protein-Losing Enteropathy after Fontan OperationCONGENITAL HEART DISEASE, Issue 5 2007Jack Rychik MD ABSTRACT Protein-losing enteropathy (PLE) is a poorly understood and enigmatic disease process affecting patients with single ventricle after Fontan operation. In those afflicted, PLE after Fontan operation results in significant morbidity and mortality. The pathophysiology of the disease is unknown; however, a proposed mechanism incorporates a combination of phenomena including: (1) altered hemodynamics, specifically low cardiac output; (2) increased mesenteric vascular resistance; (3) systemic inflammation; and (4) altered enterocyte basal membrane glycosaminoglycan make-up. A paradigm for the clinical management of PLE after Fontan operation is proposed. [source] Device Closure of Congenital Ventricular Septal DefectsCONGENITAL HEART DISEASE, Issue 2 2007Karim A. Diab MD ABSTRACT Ventricular septal defect is the most common congenital heart malformation. Surgical closure, when indicated, has been practiced for over 50 years with good results; however, surgical closure is still associated with significant morbidity and mortality. Over the past decade, several occluding devices have been developed that made catheter device closure an attractive alternative to surgery with widely satisfactory results. In this article, a comprehensive review of percutaneous and perventricular (hybrid) device closure of each type of ventricular septal defect is presented. [source] Reduction in the Incidence of Squamous Cell Carcinoma in Solid Organ Transplant Recipients Treated with Cyclic Photodynamic TherapyDERMATOLOGIC SURGERY, Issue 5 2010ANDREA WILLEY MD BACKGROUND AND OBJECTIVES Squamous cell carcinomas (SCCs) produce significant morbidity in solid organ transplant recipients (SOTRs), particularly in patients who develop multiple tumors. Topical photodynamic therapy (PDT) has been shown to decrease the number of keratotic lesions in SOTRs, but the duration of the beneficial effect is limited. The aim of this study was to evaluate the potential benefit of cyclic PDT in the prevention of new SCCs in SOTRs. METHODS Twelve high-risk SOTRs received cyclic PDT treatments at 4- to 8-week intervals for 2 years. The development of new SCCs (invasive and in situ) performed 12 and 24 months after the start of cyclic PDT were compared with the number of SCCs developed during the year before initiation of cyclic PDT. RESULTS The median reduction in the 12- and 24-month post-treatment counts from the 1-month pretreatment counts was 79.0% (73.3,81.8%) and 95.0% (87.5,100.0%), respectively. Treatments were well tolerated. CONCLUSION Cyclic PDT with 5-aminolevulinic acid may reduce the incidence of SCC in SOTRs. Additional studies with larger numbers of patients and optimized protocols are necessary to further explore the potential benefits of cyclic PDT in the prevention of skin cancer in this high-risk patient population. Dr. Lee is member of the Medical Advisory Board of Dusa Pharmaceuticals, Inc. [source] A Common Tumor, An Uncommon Location: Basal Cell Carcinoma of the Nipple and Areola in a 49-Year-Old WomanDERMATOLOGIC SURGERY, Issue 4 2005Nathan Rosen MD Background. Basal cell carcinoma (BCC) occurring on sun-protected regions is an uncommon phenomenon. BCC of the nipple is an exceedingly rare event. Method. We review the literature on BCC of the female nipple and herein describe the eighth reported case in the English literature. Our patient was treated with Mohs micrographic surgery and sentinel lymph node biopsy. Conclusion. BCC of the nipple are extremely rare tumors with unclear etiology. They can be aggressive and are capable of causing significant morbidity and mortality if they are neglected or improperly treated. With continued reporting of the diagnosis, treatment, and follow-up of these patients, we may gain an understanding of the pathogenesis, as well as the best method of control for these unusual tumors. [source] Use of a Living Dermal Equivalent for a Refractory Abdominal Defect after Pediatric Multivisceral TransplantationDERMATOLOGIC SURGERY, Issue 9 2004Carlos A. Charles MD Background. Primary closure is not always possible after pediatric multivisceral transplantation. Reepithelialization may require extended periods of postoperative time, which can be associated with significant morbidity Objective. The objective was to accelerate secondary wound closure thereby minimizing infection or further complications in a pediatric multivisceral transplant patient. Methods. Five applications of human fibroblast-derived dermis (Dermagraft, Smith and Nephew) were applied to the postsurgical defect of a pediatric multivisceral transplant patient over the course of 8 months. Routine wound care and observation was performed between human fibroblast-derived dermis applications. Results. Human fibroblast-derived dermis stimulated healing and accelerated reepithelialization. Signs of clinical rejection or infection were not observed. Conclusion. Reepithelialization can be aided in the postoperative period in pediatric multivisceral transplant patients with human fibroblast-derived dermis, thereby helping to deter complications associated with secondary wound closure. We have illustrated the successful use of a human fibroblast-derived dermis as an adjunct for wound healing in a complicated surgical defect. [source] Electrosurgery, Pacemakers and ICDs: A Survey of Precautions and Complications Experienced by Cutaneous SurgeonsDERMATOLOGIC SURGERY, Issue 4 2001Hazem M. El-Gamal MD Background. Minimal information is available in the literature regarding the precautions implemented or complications experienced by cutaneous surgeons when electrosurgery is used in patients with pacemakers or implantable cardioverter-defibrillators (ICDs). The literature pertinent to dermatologists is primarily based on experiences of other surgical specialties and a generally recommended thorough perioperative evaluation. Objective. To determine what precautions are currently taken by cutaneous surgeons in patients with pacemakers or ICDs, and what types of complications have occurred due to electrosurgery in a dermatologic setting. Methods. In the winter of 2000, a survey was mailed to 419 U.S.-based members of the American College of Mohs Micrographic Surgery and Cutaneous Oncology (ACMMSCO). Results. A total of 166 (40%) surveys were returned. Routine precautions included utilizing short bursts of less than 5 seconds (71%), use of minimal power (61%), and avoiding use around the pacemaker or ICD (57%). The types of interference reported were skipped beats (eight patients), reprogramming of a pacemaker (six patients), firing of an ICD (four patients), asystole (three patients), bradycardia (two patients), depleted battery life of a pacemaker (one patient), and an unspecified tachyarrhythmia (one patient). Overall there was a low rate of complications (0.8 cases/100 years of surgical practice), with no reported significant morbidity or mortality. Bipolar forceps were utilized by 19% of respondents and were not associated with any incidences of interference. Conclusions. Significant interference to pacemakers or ICDs rarely results from office-based electrosurgery. No clear community practice standards regarding precautions was evident from this survey. The use of bipolar forceps or true electrocautery are the better options when electrosurgey is required. These two modalities may necessitate fewer perioperative precautions than generally recommended, without compromising patient safety. [source] Soft tissue augmentation 2006: filler fantasyDERMATOLOGIC THERAPY, Issue 3 2006Arnold William Klein ABSTRACT:, As an increasing number of patients seek esthetic improvement through minimally invasive procedures, interest in soft tissue augmentation and filling agents is at an all-time high. One reason for this interest is the availability of botulinum toxin type A, which works superbly in the upper face. The rejuvenation of the upper face has created much interest in injectable filling agents and implant techniques that work equally well in the restoration of the lower face. One of the central tenets of soft tissue augmentation is the concept of the three-dimensional face. The youthful face has a soft, full appearance, as opposed to the flat, pulled, two-dimensional look often achieved by more traditional surgical approaches. Injectable filling agents can augment and even at times, replace pulling. Additionally, with the lip as the focal center of the lower face, subtle lip enhancement is here to stay, and is in fact, the number one indication for injectable fillers. Moreover, minimally invasive soft tissue augmentation offers cosmetic enhancement without the cost and recovery time associated with more invasive procedures. As more and more physicians take interest in minimally invasive surgery, courses in cosmetic surgery techniques are becoming increasingly popular at the medical meetings of many specialties. Today, physicians have a much larger armamentarium of techniques and materials with which to improve facial contours, ameliorate wrinkles, and provide esthetic rejuvenation to the face. For a substance or device to be amenable for soft tissue augmentation in the medical community, it must meet certain criteria. It must have both a high "use" potential, producing cosmetically pleasing results with a minimum undesirable reactions, and have a low abuse potential in that widespread or incorrect or indiscriminate use would not result in significant morbidity. It must be nonteratogenic, noncarcinogenic, and nonmigratory. In addition, the agent must provide predictable, persistent correction through reproducible implantation techniques. Finally, the substance, agent or device must be approved by the U.S. Food and Drug Administration, which assures purity, safety, and accessibility, as well as much-needed information regarding use. Having a thorough understanding of the filling agents available, their indications and contraindications, as well as having thorough knowledge of implant technique are vital in providing the patient with an esthetically pleasing result. [source] Use of topical immunomodulators in organ transplant recipientsDERMATOLOGIC THERAPY, Issue 1 2005Bradley T. Kovach ABSTRACT:, Solid organ transplant recipients are a growing population at increased risk for the development of cutaneous premalignant and malignant lesions, resulting in significant morbidity and mortality. Topical immunomodulators, in particular imiquimod, have shown efficacy in the management of multiple malignant, precancerous, and viral conditions. The ability to locally induce an immune response, presumably against tumor and viral antigens, and induce apoptosis makes topical immunomodulators a promising therapeutic option in organ transplant recipients. Although limited, data have begun to accumulate on the use of imiquimod in transplant patients for the management of superficial, nodular, and infiltrative basal cell carcinomas; in situ and invasive squamous cell carcinomas; condyloma acuminata; and common warts. As more experience is gathered, the role of imiquimod and other topical immunomodulators in the care of OTRs will be clarified. The authors reviewed the existing data on the use of topical imiquimod in OTRs with mention of its presumed mechanisms of action and other immunomodulators with potential efficacy against cancerous and precancerous lesions. [source] How hypoglycaemia can affect the life of a person with diabetesDIABETES/METABOLISM: RESEARCH AND REVIEWS, Issue 2 2008Brian M. Frier Abstract Hypoglycaemia is the commonest side-effect of insulin treatment for diabetes, and is the single greatest barrier to achieving and maintaining good glycaemic control. Severe hypoglycaemia (requiring assistance for recovery) is associated with significant morbidity and is feared by most people with type 1 diabetes and their families. It causes stress and anxiety and may influence self-management and glycaemic control. The annual prevalence of severe hypoglycaemia is around 30% in people with type 1 diabetes, and is higher in those with risk factors such as strict glycaemic control, impaired awareness of hypoglycaemia and increasing duration of diabetes. It is also common during sleep (nocturnal hypoglycaemia). Neurological manifestations include coma, convulsions, transient hemiparesis and stroke, while reduced consciousness and cognitive dysfunction may cause accidents and injuries. Cardiac events may be precipitated such as arrhythmias, myocardial ischaemia and cardiac failure. Hypoglycaemia can affect all aspects of life, including employment, driving, recreational activities involving exercise, and travel, and measures should be taken in all of these situations to avoid this potentially dangerous side-effect of insulin therapy. Copyright © 2007 John Wiley & Sons, Ltd. [source] Obesity, bariatric surgery and type 2 diabetes,a systematic reviewDIABETES/METABOLISM: RESEARCH AND REVIEWS, Issue 6 2004Cynthia V. Ferchak Abstract Obesity is endemic in the United States and is closely linked to the development of type 2 diabetes. Both obesity and diabetes are responsible for significant morbidity and mortality. Likewise, both conditions are resistant to treatment. Recent studies have evaluated prevention of type 2 diabetes through intensive lifestyle intervention, while others are examining the impact of bariatric surgery on type 2 diabetes. This article presents an overview of the impact of bariatric surgical and lifestyle interventions on the prevention and treatment of type 2 diabetes. Although studies using a variety of bariatric surgical techniques are included, the focus is on two interventions in particular: the Roux-en-Y gastric bypass and the laparoscopic silicone gastric banding procedure. Outcomes of these procedures are further contrasted with recent lifestyle intervention studies, in particular, the Diabetes Prevention Program study. Gastric bypass studies have been associated with a 99 to 100% prevention of diabetes in patients with IGT and an 80 to 90% clinical resolution of diagnosed early type 2 diabetes. Gastric banding procedures are associated with a lower median (50,60%) clinical remission of type 2 diabetes. Lifestyle intervention studies of obese and glucose-intolerant patients have achieved a 50% reduction in the progression of IGT to diabetes over the short term, with no reported resolution of the disease. Weight loss by any means in the obese patient appears to prevent progression to type 2 diabetes, at least in the short term. Furthermore, sustained weight loss through bariatric surgical intervention is associated both with prevention of progression of IGT and with clinical remission of early type 2 diabetes. Copyright © 2004 John Wiley & Sons, Ltd. [source] The epidemiology of diabetic limb sepsis: an African perspectiveDIABETIC MEDICINE, Issue 11 2002Z. G. Abbas Abstract We review the epidemiology of foot and hand sepsis in adult diabetes patients in Africa. Limb sepsis in these patients is associated with significant morbidity and mortality. The pathogenesis of diabetic foot infections in these patient populations appears to be similar to that for patients in industrialized countries ,ulcers and underlying peripheral neuropathy being the most important risk factors. Prevention of peripheral neuropathy through aggressive glycaemic control may be the most important primary control measure for foot infections. The tropical diabetic hand syndrome (TDHS) is being increasingly seen in diabetes patients in certain parts of Africa. The syndrome is acute, usually follows minor trauma to the hand, and is associated with a progressive synergistic form of gangrene. The major risk factors for TDHS are unknown but recent data suggest poor glycaemic control is associated with poor outcome. Treatment of TDHS requires aggressive surgery. Hence, preventive efforts for both foot and hand sepsis include aggressive glucose control, and education on hand and foot care and the importance of seeking medical attention promptly at the earliest onset of symptoms. Diabet. Med. 19, 895,899 (2002) [source] Angiolipoma of the esophagus: a rare clinical dilemmaDISEASES OF THE ESOPHAGUS, Issue 3 2006E. H. Jensen SUMMARY., Benign tumors of the esophagus are a rare but diverse group of lesions. Although non-malignant in biology, their presence can cause significant morbidity, including dysphagia, bleeding, gastrointestinal obstruction, and even asphyxiation. Diagnosis is frequently made using radiographic and endoscopic means, even in the absence of definitive biopsy. If discovered early, endoscopic or minimally invasive techniques may be used to excise these lesions, with essentially 100% cure rates. However, if discovered late, open excision or even esophagectomy may be required. Angiolipoma represents perhaps one of the rarest of the benign entities to affect the esophagus, with only a few cases reported in the current literature. We present the case of an 85-year-old man who developed complete esophageal obstruction due to a large, pedunculated angiolipoma, requiring open surgical excision. [source] White-eyed blowout fracture: Another lookEMERGENCY MEDICINE AUSTRALASIA, Issue 3 2009Patrick Mehanna Abstract Orbital floor fractures have the potential to cause significant morbidity both in the short and long terms and commonly present to the ED for initial assessment. Although treatment of the majority of these injuries involves clinic review and possible later surgery, there is a specific subset that present to emergency clinically suggestive of a head injury. This subset, ,white-eyed blowout', usually occurring under 18 years of age, with a history of trauma and little sign of soft tissue injury, describes a trap door orbital floor fracture with herniation and acute entrapment of orbital muscle and is regarded as a maxillofacial emergency. The injury presents with marked nausea, vomiting, headache and irritability suggestive of a head injury that commonly distracts from the true aetiology. It requires prompt diagnosis and treatment to avoid permanent morbidity. We present three cases and discuss their management. [source] Areca nut-abuse liability, dependence and public healthADDICTION BIOLOGY, Issue 1 2002A. Winstock It may, however, stigmatize substance use, especially when western diagnostic criteria are applied to essentially culture-bound substances. However, when the pattern of use of a substance results in significant personal harm, then whether dependence exists or not is less important than developing an appropriate response, if its use is associated with significant morbidity and mortality. Such is the case with areca nut, the fourth most commonly used drug in the world after tobacco, alcohol and caffeine. In this paper I will explore its use and properties with respect to its abuse and dependence liability and consider the associated implications for public health. [source] Ethanol lock therapy for the treatment of catheter-related infections in haemophilia patientsHAEMOPHILIA, Issue 6 2009M. RAJPURKAR Summary., Central venous access devices (CVAD) are increasingly being used for optimal delivery of clotting factor concentrates in patients with haemophilia with poor peripheral venous access. The utility of CVAD is particularly well recognized in young patients starting factor prophylaxis and in patients with inhibitors undergoing immune tolerance induction (ITI). A catheter-related infection (CRI) remains the most common complication of CVAD in haemophilia patients and is the most frequent indication for its removal. Additionally, in some patients the infection results in significant morbidity and mortality and also contributes to failure of the ITI regimen. Ethanol-lock therapy (ELT) is a treatment modality that has been used to treat CRI in patients with indwelling catheters for home parenteral nutrition and chemotherapy. The aim of this study was to report the success in treating CRI in haemophilia patients using ELT. Three severe haemophilia A patients undergoing ITI regimen who developed CVAD infections resistant to conventional management with antibiotics were treated by ELT according to the institutional technique. All three patients responded well to ELT with clearance of the CVAD infection. There were no adverse side effects. To our knowledge, this is the first report of ELT in patients with haemophilia. The role of ELT needs to be investigated in larger studies for treatment of CRI in patients with bleeding disorders. [source] The Colorado Haemophilia Paediatric Joint Physical Examination Scale: normal values and interrater reliabilityHAEMOPHILIA, Issue 1 2007M. R. HACKER Summary., ,Persons with haemophilia often experience their first joint haemorrhage in early childhood. Recurrent bleeding into a joint may lead to significant morbidity, specifically haemophilic arthropathy. Early identification of the onset and progression of joint damage is critical to preserving joint structure and function. Physical examination is the most feasible approach to monitor joint health. Our group developed the Colorado Haemophilia Paediatric Joint Physical Examination Scale to identify earlier signs of joint degeneration and incorporate developmentally appropriate tasks for assessing joint function in young children. This study's objectives were to establish normal ranges for this scale and assess interrater reliability. The ankles, knees and elbows of 72 healthy boys aged 1 through 7 years were evaluated by a physical therapist to establish normal ranges. Exactly 10 boys in each age category from 2 to 7 years were evaluated by a second physical therapist to determine interrater reliability. The original scale was modified to account for the finding that mild angulation in the weight-bearing joints is developmentally normal. The interrater reliability of the scale ranged from fair to good, underscoring the need for physical therapists to have specific training in the orthopaedic assessment of very young children and the measurement error inherent in the goniometer. Modifications to axial alignment scoring will allow the scale to distinguish healthy joints from those suffering frequent haemarthroses. [source] Microlaryngoscopic repair of iatrogenic pharyngeal pouch perforations: Treatment of choice?HEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 2 2007FRCS (ORL-HNS), Vinidh Paleri MS Background. Endoscopic stapled diverticulostomy (ESD) has become the preferred technique for managing pharyngeal pouches. Iatrogenic perforation, created during stapling, is a rare but serious complication with significant morbidity and mortality. The conventional management in these instances is to convert it to an external procedure and excise the pouch. Methods. Iatrogenic perforations were noticed after stapling in 3 cases in our series of 73 patients who underwent ESD. They were repaired using microlaryngoscopic techniques. Results. All patients had an unremarkable postoperative course. Conclusions. Selected cases with iatrogenic perforations can be repaired primarily and observed with excellent outcome, obviating the need for an external pouch excision. © 2006 Wiley Periodicals, Inc. Head Neck, 2007 [source] Angiofibroma of the larynx: Report of a case with clinical and pathologic literature review,HEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 8 2002Matthew H. Steele MD Abstract Background Angiofibromas are uncommon vascular tumors with a strong predilection for the nasopharynx of adolescent males. Although they are slow growing and histologically benign, they have the potential to cause significant morbidity with laryngeal involvement. Methods We describe the clinical characteristics, histopathologic findings, differential diagnosis, preoperative evaluation, and management of a case of laryngeal angiofibroma. Results The patient was initially seen with a 2½-year history of progressive dyspnea and dysphagia. Preoperative evaluation suggested a vascular mass involving the left supraglottic larynx. A partial laryngopharyngectomy was performed without complication. The patient is alive and disease free 3 years postoperatively. Final histopathologic diagnosis is consistent with angiofibroma. Conclusions Laryngeal angiofibroma is an extremely rare entity. Adequate preoperative imaging is necessary to confirm the vascularity of this lesion, because ill-planned biopsy may lead to significant blood loss. The role of preoperative embolization of other laryngeal vascular lesions has been well documented and may be useful in the management of laryngeal angiofibroma. © 2002 Wiley Periodicals, Inc. Head Neck 24: 805,809, 2002 [source] Lipoprotein (a) in Chronic Renal Failure: Effect of Maintenance HemodialysisHEMODIALYSIS INTERNATIONAL, Issue 4 2003Om Prakash Kalra Background:,Coronary artery disease accounts for significant morbidity and mortality in patients with chronic kidney disease (CKD). Besides the higher prevalence of traditional risk factors, several uremia-related factors may play a role in accelerated atherosclerosis, such as elevated levels of lipoprotein (a) (Lp(a)). The effect of maintenance hemodialysis (MHD) on Lp(a) levels is not well understood. The present work was carried out to study the Lp(a) levels in Stage 4 and Stage 5 CKD patients as well as the effect of MHD on Lp(a) levels in patients with Stage 5 CKD. Methods:,The study subjects included 15 patients with Stage 4 CKD, 15 patients with Stage 5 CKD, and 15 age- and sex-matched healthy controls. Plasma Lp(a) was measured by ELISA in all the subjects at the time of entry into the study and after 4 weeks of MHD in patients with Stage 5 CKD. Patients on MHD were dialyzed two to three times weekly for 4 hr during each session. Results:,Mean Lp(a) levels were significantly higher in patients with CKD than in control patients. In patients with Stage 4 CKD, the Lp(a) level was 34.0 ± 19.5 mg/dL, whereas in Stage 5 CKD the level was 49.0 ± 30.9 and in healthy controls it was 22.2 ± 16.4. In patients with Stage 5 CKD, 4 weeks of MHD led to a significant fall in Lp(a) levels by 23.6% (P < 0.001). Conclusions:,The results of this study show that increases in Lp(a) levels start early during the course of CKD and become more pronounced with increased severity of disease. Initiation of MHD lowers Lp(a) levels and may have a long-term beneficial effect on cardiovascular morbidity and mortality. [source] Outcomes of a patient-to-patient outbreak of genotype 3a hepatitis C,HEPATOLOGY, Issue 2 2009Mark E. Mailliard Between March 2000 and July 2001, at least 99 persons acquired a hepatitis C virus genotype 3a (HCV-3a) infection in an oncology clinic. This nosocomial HCV outbreak provided an opportunity to examine the subsequent clinical course in a well-defined cohort. This was a retrospective/prospective observational study of the short-term significant health outcomes of a large, single-source, patient-to-patient HCV-3a outbreak. Outbreak patients or their legal representatives consenting to study were enrolled between September 2002 and December 2007. We measured history and physical examinations, medical records, HCV serology, HCV RNA and genotype, liver enzymes, histology, response to antiviral therapy, and liver-related morbidity and mortality. Sixty-four of the 99 known HCV-3a outbreak patients participated. During a 6-year period, six patients developed life-threatening complications from liver disease, three died, one received a liver transplant, and two were stable after esophageal variceal banding or diuretic therapy of ascites. Thirty-three patients underwent antiviral therapy, with 28 achieving a sustained viral remission. One patient acquired HCV-3a infection sexually from an outbreak patient and was successfully treated. Eleven study patients died of malignancy, including two that had achieved a sustained viral remission after antiviral therapy. Conclusion: Our patient cohort had a nosocomial source and an oncologic or hematologic comorbidity. Compared with previous HCV outcome studies, a patient-to-patient HCV outbreak in an oncology clinic exhibited significant morbidity and mortality. Attention is needed to the public health risk of nosocomial HCV transmission, emphasizing infection control, early diagnosis, and therapy. (HEPATOLOGY 2009.) [source] Endogenous ursodeoxycholic acid and cholic acid in liver disease due to cystic fibrosisHEPATOLOGY, Issue 6 2004Jeffery L. Smith Focal biliary cirrhosis causes significant morbidity and mortality in cystic fibrosis (CF). Although the mechanisms of pathogenesis remain unclear, bile acids have been proposed as potential mediators of liver injury. This study examined bile acid composition in CF and assessed altered bile acid profiles to determine if they are associated with incidence and progression of liver injury in CF-associated liver disease (CFLD). Bile acid composition was determined by gas,liquid chromatography/mass spectrometry in bile, urine, and serum samples from 30 children with CFLD, 15 children with CF but without liver disease (CFnoLD), and 43 controls. Liver biopsies from 29 CFLD subjects were assessed histologically by grading for fibrosis stage, inflammation, and disruption of the limiting plate. A significantly greater proportion of endogenous biliary ursodeoxycholic acid (UDCA) was demonstrated in CFnoLD subjects vs. both CFLD subjects and controls (2.4- and 2.2-fold, respectively; ANOVA, P = .04), and a 3-4 fold elevation in endogenous serum UDCA concentration was observed in both CFLD subjects and CFnoLD subjects vs. controls (ANOVA, P < .05). In CFLD, there were significant correlations between serum cholic acid and hepatic fibrosis, inflammation, and limiting plate disruption as well as the ratio of serum cholic acid/chenodeoxycholic acid to hepatic fibrosis, inflammation, and limiting plate disruption. In conclusion, elevated endogenous UDCA in CFnoLD suggests a possible protective role against liver injury in these patients. The correlation between both cholic acid and cholic acid/chenodeoxycholic acid levels with histological liver injury and fibrosis progression suggests a potential monitoring role for these bile acids in CFLD. (HEPATOLOGY 2004;39:1673,1682.) [source] New insights into the pathobiology of portal hypertensionHEPATOLOGY RESEARCH, Issue 10 2009Praveen Guturu Portal Hypertension is a frequent complication of cirrhosis and causes significant morbidity and mortality. Increased intrahepatic resistance is the primary factor but portal hypertension is also associated with changes in systemic and porto-sytemic collateral circulation. Cirrhosis is a state of vasoregulatory imbalance with excess vasoconstrictors and less vasodilators in hepatic circulation and the reverse is true for systemic circulation. Multiple pathophysiologic mechanisms including endothelial dysfunction, sinusoidal remodeling and angiogenesis are involved in increasing resistance in hepatic vascular bed. Current evidence suggests that these changes in vasoreactivity contribute to a significant proportion of intrahepatic vascular resistance and that they are reversible, providing an attractive target for therapeutic intervention. [source] Un-promoted issues in inflammatory bowel disease: opportunities to optimize careINTERNAL MEDICINE JOURNAL, Issue 3 2010J. M. Andrews Abstract Inflammatory bowel diseases (IBD), comprising Crohn's disease (CD) and ulcerative colitis (UC), are chronic inflammatory disorders of the gut, which lead to significant morbidity and impaired quality of life (QoL) in sufferers, without generally affecting mortality. Despite CD and UC being chronic, life-long illnesses, most medical management is directed at acute flares of disease. Moreover, with more intensive medical therapy and the development of biological therapy, there is a risk that management will become even more narrowly focused on acute care, and be directed only at those with more severe disease, rather than encompassing all sufferers and addressing important non-acute issues. This imbalance of concentration of medical attention on ,high-end' care is in part driven by the need to perform and publish randomized clinical trials of newer therapies to obtain registration and licensing for these agents, which thus occupy a large proportion of the recent IBD treatment literature. This leads to less attention on relatively ,low-technology' issues including: (i) the psychosocial burden of chronic disease, QoL and specific psychological comorbidities; (ii) comorbidity with functional gastrointestinal disorders (FGIDs); (iii) maintenance therapy, monitoring and compliance; (iv) smoking (with regard to CD); (v) sexuality, fertility, family planning and pregnancy; and (vi) iron deficiency and anaemia. We propose these to be the ,Un-promoted Issues' in IBD and review the importance and treatment of each of these in the current management of IBD. [source] Severe cutaneous reactions caused by barbiturates in seven Iranian childrenINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 11 2009Setareh Mamishi MD Background, The severe adverse cutaneous reactions of erythema multiforme (EM), Stevens,Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare mucocutaneous diseases associated with significant morbidity and mortality. The most common cause is antiepileptic drugs, particularly carbamazepine and lamotrigine, as well as the barbiturates group (phenobarbital and phenytoin). In this article, we present seven children with severe adverse cutaneous reactions caused by barbiturates. Case Reports, The age of the affected children was between 2 and 11 years and they all had a history of taking barbiturates. Their symptoms started 1,3 weeks after the initiation of barbiturates, including a prodrome characterized by 2,3 days of malaise, fever, cough and anorexia, after which the skin and mucosal lesions appeared and worsened. The skin lesions varied from rash to large bullae, plus different forms of mucous membrane involvement. The offending drugs (barbiturates) were stopped immediately and care was largely supportive. Conclusion, As a result of the morbidity and/or mortality associated with EM, SJS and TEN, physicians should keep in mind their differential diagnosis when cutaneous reactions are observed in patients undergoing barbiturate therapy. Furthermore, although TEN and SJS are life-threatening diseases, early detection and appropriate care can lead to a decrease in the incidence of death. The strategies described here seem to be successful and safe because, despite the serious conditions, our patients responded well. All survived. [source] Cutaneous manifestations of chikungunya fever: observations made during a recent outbreak in south IndiaINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 2 2008Arun C. Inamadar MD Background, Chikungunya fever is an Aedes mosquito-borne Arbo viral illness with significant morbidity. Methods, In a recent outbreak of the disease in south India, the dermatologic manifestations of 145 patients attending a tertiary care hospital were recorded. Results, All age groups were affected, including newborns. Some of the cutaneous features were observed during the acute stage of the illness, and others during convalescence or thereafter. Pigmentary changes were found to be the most common cutaneous finding (42%), followed by maculopapular eruption (33%) and intertriginous aphthous-like ulcers (21.37%). Lesions with significant morbidity were generalized vesiculobullous eruptions (2.75%), found only in infants, lymphedema, and intertriginous aphthous-like ulcers. Exacerbation of existing dermatoses, such as psoriasis, and unmasking of undiagnosed Hansen's disease were observed. A perivascular lymphocytic infiltrate was a consistent histopathologic finding in all types of skin lesions. All patients responded well to symptomatic, conservative treatment. Conclusions, The cutaneous findings hitherto not reported may be the result of the African genotype of the virus detected during this outbreak in India. [source] | |||||||||||||||||||||||||||||||||||||||||||