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Sample records for neurasthenic syndrome treatment

  1. Treatment of West syndrome.

    PubMed

    Sakakihara, Yoichi

    2011-03-01

    West syndrome is one of the most refractory epileptic syndromes in infancy, and many researchers have made great effort to find optimal treatment modalities for this syndrome. In this review, previous literature on optimal treatments of West syndrome and its refractory nature were briefly presented, followed by an introduction of recent publication of expert opinions from the US and Europe. An Asian expert opinion generated by a short questionnaire survey was then presented. It was shown that medically proven optimal treatment of West syndrome is not always the practical treatment of choice in Asian countries. Cost and geographical regions should also be taken into account in making practical choices for treatment of West syndrome. PMID:21196092

  2. Treatment Option Overview (Myelodysplastic Syndromes)

    MedlinePlus

    ... Patient Myelo-proliferative Neoplasms Patient Myelodysplastic Syndromes Treatment Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Health Professional Myelodysplastic ...

  3. Treatment Options for Myelodysplastic Syndromes

    MedlinePlus

    ... Patient Myelo-proliferative Neoplasms Patient Myelodysplastic Syndromes Treatment Myeloproliferative Neoplasms Treatment Myelodysplastic/ Myeloproliferative Neoplasms Treatment Health Professional Myelodysplastic ...

  4. Myofascial pain syndrome treatments.

    PubMed

    Borg-Stein, Joanne; Iaccarino, Mary Alexis

    2014-05-01

    Myofascial pain syndrome (MPS) is a regional pain disorder caused by taut bands of muscle fibers in skeletal muscles called myofascial trigger points. MPS is a common disorder, often diagnosed and treated by physiatrists. Treatment strategies for MPS include exercises, patient education, and trigger point injection. Pharmacologic interventions are also common, and a variety of analgesics, antiinflammatories, antidepressants, and other medications are used in clinical practice. This review explores the various treatment options for MPS, including those therapies that target myofascial trigger points and common secondary symptoms. PMID:24787338

  5. Treatment of Dravet Syndrome.

    PubMed

    Wirrell, Elaine C

    2016-06-01

    Dravet syndrome is among the most challenging electroclinical syndromes. There is a high likelihood of recurrent status epilepticus; seizures are medically refractory; and patients have multiple co-morbidities, including intellectual disability, behaviour and sleep problems, and crouch gait. Additionally, they are at significant risk of sudden unexplained death. This review will focus predominantly on the prophylactic medical management of seizures, addressing both first-line therapies (valproate and clobazam) as well as second-line (stiripentol, topiramate, ketogenic diet) or later options (levetiracetam, bromides, vagus nerve stimulation). Sodium channel agents-including carbamazepine, oxcarbazepine, phenytoin and lamotrigine-should be avoided, as they typically exacerbate seizures. Several agents in development may show promise, specifically fenfluramine and cannabidiol, but they need further evaluation in randomized, controlled trials. In addition to prophylactic treatment, all patients need home-rescue medication and a status epilepticus protocol that can be carried out in their local hospital. Families must be counselled on non-pharmacologic strategies to reduce seizure risk, including avoidance of triggers that commonly induce seizures (including hyperthermia, flashing lights and patterns). In addition to addressing seizures, holistic care for a patient with Dravet syndrome must involve a multidisciplinary team that includes specialists in physical, occupational and speech therapy, neuropsychology, social work and physical medicine. PMID:27264138

  6. What Are the Treatments for Rett Syndrome?

    MedlinePlus

    ... Resources and Publications What are the treatments for Rett syndrome? Skip sharing on social media links Share this: ... 2012, from http://www.rettsyndrome.org/understanding-rett-syndrome/about-rett-syndrome [top] PubMed Health. (2010). Rett syndrome . Retrieved ...

  7. Treatment of Tourette syndrome.

    PubMed

    Kurlan, Roger M

    2014-01-01

    Tourette's syndrome (TS) consists of chronic motor and phonic tics and characteristically begins in childhood. The tics can be disabling and commonly associated behavioral comorbities such as attention deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD), can also cause problems in daily functioning. The underlying etiology and neurobiology of TS remain unknown although genetic factors appear to be important, cortical control of basal ganglia motor function appears to be disturbed and neurochemical abnormalities, particularly involving dopamine neurotransmission, are likely present. The treatment of TS involves appropriate education and support. Tics can be treated with habit reversal cognitive behavioral therapy, medications (most commonly alpha agonists and antipsychotics), local intramuscular injections of botulinum toxin and some severe, refractory cases have responded to deep brain stimulation surgery (DBS). It is important to appropriately diagnose and treat comorbid behavioral disorders that are disrupting function. OCD can be treated with cognitive behavioral therapy, selective serotonin reuptake inhibitors, and atypical antipsychotics. DBS has become a treatment option for patients with disabling OCD despite other therapies. ADHD is treated with appropriate classroom accommodations, behavioral therapy, alpha agonists, atomoxetine or methylphenidate-containing stimulant drugs. PMID:24043501

  8. Tourette Syndrome (TS): Treatments

    MedlinePlus

    ... in treatment and support—which may include teachers, childcare providers, coaches, therapists, and other family members. Taking ... and discipline that is effective with their particular child. Top of Page For More Information Additional information ...

  9. Combination syndrome symptomatology and treatment.

    PubMed

    Tolstunov, Len

    2011-04-01

    Combination syndrome (CS) is one of the most fascinating oral conditions yet is poorly understood and underappreciated in the literature and clinical practice. This article reviews the most important literature on this subject and analyzes the etiology, symptomatology, diagnosis, and current therapeutic modalities for treatment in an attempt to better understand CS. The syndrome represents an example ofa complex pathologic condition of the entire stomatognathic system with a multitude of hard-tissue, soft-tissue, and occlusal changes. These changes, initiated by a certain sequence of events, beginning with a prolonged period of tooth loss, can lead to severe bone atrophy in different regions of the jaws, loss of masticatory function, and the need for complex treatment. Implant rehabilitation of these patients in a preventative approach with cooperation of the entire dental team is emphasized. PMID:21560744

  10. Treatment-refractory Tourette Syndrome.

    PubMed

    Kious, Brent M; Jimenez-Shahed, Joohi; Shprecher, David R

    2016-10-01

    Tourette Syndrome (TS) is a complex neurodevelopmental condition marked by tics and frequently associated with psychiatric comorbidities. While most cases are mild and improve with age, some are treatment-refractory. Here, we review strategies for the management of this population. We begin by examining the diagnosis of TS and routine management strategies. We then consider emerging treatments for refractory cases, including deep brain stimulation (DBS), electroconvulsive therapy (ECT), repetitive transcranial magnetic stimulation (rTMS), and novel pharmacological approaches such as new vesicular monoamine transporter type 2 inhibitors, cannabinoids, and anti-glutamatergic drugs. PMID:26875502

  11. Tourette Syndrome: A Multidimensional Approach to Treatment.

    ERIC Educational Resources Information Center

    Anderson, Donna J.

    1987-01-01

    Describes Tourette syndrome, a chronic, neurological disorder characterized by involuntary muscular movements, uncontrollable sounds, and inappropriate words. Notes that Tourette syndrome is frequently misunderstood and mistreated, presents symptoms, and suggests a multidimensional approach to treatment. (Author/NB)

  12. Greater trochanteric pain syndrome diagnosis and treatment.

    PubMed

    Mallow, Michael; Nazarian, Levon N

    2014-05-01

    Lateral hip pain, or greater trochanteric pain syndrome, is a commonly seen condition; in this article, the relevant anatomy, epidemiology, and evaluation strategies of greater trochanteric pain syndrome are reviewed. Specific attention is focused on imaging of this syndrome and treatment techniques, including ultrasound-guided interventions. PMID:24787333

  13. Treatment of restless legs syndrome.

    PubMed

    Ferini-Strambi, Luigi; Manconi, Mauro

    2009-12-01

    Restless legs syndrome (RLS) is a common condition characterized by an urge to move the legs, accompanied by uncomfortable or unpleasant sensations. Symptoms predominantly occur at rest in the evening or at night, and they are alleviated by moving the affected extremity or by walking. Recent European epidemiological studies reported an overall prevalence of RLS up to 10%, with a female preponderance. The prevalence rates reported in south-eastern Europe are lower, as are those in Asiatic populations. Although the aetiopathogenesis of RLS is still unknown, the rapid and dramatic improvement of RLS with dopaminergic compounds suggests a dopaminergic system dysfunction as the basic mechanism. Extensive data are available for l-dopa and dopamine receptor agonists, especially for pramipexole and ropinirole. Pharmacological treatment should be limited to those patients who suffer from clinically relevant RLS with impaired sleep quality or quality of life. Treatment on demand is a clinical need in RLS cases that present intermittent symptoms. PMID:20123560

  14. Treatments for Shoulder Impingement Syndrome

    PubMed Central

    Dong, Wei; Goost, Hans; Lin, Xiang-Bo; Burger, Christof; Paul, Christian; Wang, Zeng-Li; Zhang, Tian-Yi; Jiang, Zhi-Chao; Welle, Kristian; Kabir, Koroush

    2015-01-01

    Abstract Many treatments for shoulder impingement syndrome (SIS) are available in clinical practice; some of which have already been compared with other treatments by various investigators. However, a comprehensive treatment comparison is lacking. Several widely used electronic databases were searched for eligible studies. The outcome measurements were the pain score and the Constant–Murley score (CMS). Direct comparisons were performed using the conventional pair-wise meta-analysis method, while a network meta-analysis based on the Bayesian model was used to calculate the results of all potentially possible comparisons and rank probabilities. Included in the meta-analysis procedure were 33 randomized controlled trials involving 2300 patients. Good agreement was demonstrated between the results of the pair-wise meta-analyses and the network meta-analyses. Regarding nonoperative treatments, with respect to the pain score, combined treatments composed of exercise and other therapies tended to yield better effects than single-intervention therapies. Localized drug injections that were combined with exercise showed better treatment effects than any other treatments, whereas worse effects were observed when such injections were used alone. Regarding the CMS, most combined treatments based on exercise also demonstrated better effects than exercise alone. Regarding surgical treatments, according to the pain score and the CMS, arthroscopic subacromial decompression (ASD) together with treatments derived from it, such as ASD combined with radiofrequency and arthroscopic bursectomy, showed better effects than open subacromial decompression (OSD) and OSD combined with the injection of platelet-leukocyte gel. Exercise therapy also demonstrated good performance. Results for inconsistency, sensitivity analysis, and meta-regression all supported the robustness and reliability of these network meta-analyses. Exercise and other exercise-based therapies, such as kinesio taping

  15. Newer treatments for fibromyalgia syndrome

    PubMed Central

    Harris, Richard E; Clauw, Daniel J

    2008-01-01

    Fibromyalgia syndrome is a common chronic pain disorder of unknown etiology. The lack of understanding of the pathophysiology of fibromyalgia has made this condition frustrating for patients and clinicians alike. The most common symptoms of this disorder are chronic widespread pain, fatigue, sleep disturbances, difficulty with memory, and morning stiffness. Emerging evidence points towards augmented pain processing within the central nervous system (CNS) as having a primary role in the pathophysiology of this disorder. Currently the two drugs that are approved by the United States Food and Drug Administration (FDA) for the management of fibromyalgia are pregabalin and duloxetine. Newer data suggests that milnacipran, a dual norepinephrine and serotonin reuptake inhibitor, may be promising for the treatment of fibromyalgia. A double-blind, placebo-controlled trial of milnacipran in 125 fibromyalgia patients showed significant improvements relative to placebo. Milnacipran given either once or twice daily at doses up to 200 mg/day was generally well tolerated and yielded significant improvements relative to placebo on measures of pain, patient’s global impression of change in their disease state, physical function, and fatigue. Future studies are needed to validate the efficacy of milnacipran in fibromyalgia. PMID:19337439

  16. [Depressive syndrome in gastroenterology: diagnosis and treatment].

    PubMed

    Tsimmerman, Ia S; Tsimmerman, I Ia

    2007-01-01

    The article presents modern data on depressive syndrome, its prevalence and possible reasons for its growth, the role of psychoemotional stress in the development of anxiety depression (AD), as well as psychosomatic diseases and syndromes and modern views on the mechanisms of their formation. The authors discuss methods of revealing and diagnostic criteria of AD and psychosomatic diseases, including those that develop against the background of masked depression. Associations between depressive syndrome and the development of gastroenterological diseases and psychosomatic syndromes such as peptic ulcer, functional dyspepsia, irritated bowel syndromes, chronic cholecystitis, and chronic duodenal obstruction syndrome are discussed in detail. Special attention is paid to treatment of AD in gastroenterological patients using psycho- and hypnotherapy as well as psychotropic drugs such as antidepressives, anxiolytics, neuroleptics, and nootropic agents. PMID:17665598

  17. Post-Treatment Lyme Disease Syndrome

    MedlinePlus

    ... FAQ Health care providers Educational materials Post-Treatment Lyme Disease Syndrome Recommend on Facebook Tweet Share Compartir It ... ONE 7(1): e29914. HHS Special Webinar on Lyme Disease Persistence frame support disabled and/or not supported ...

  18. Treatment Approaches in Down's Syndrome: A Review.

    ERIC Educational Resources Information Center

    Foreman, Philip J.; Ward, James

    1986-01-01

    The paper reviews research into treatment approaches in Down's Syndrome. Pharmacological treatments reviewed include thyroid therapy, 5-hydroxytryptophan, vitamin therapy, and cell therapy. Other treatments considered are movement patterning, early intervention, and facial surgery. Early educational intervention is seen as the most effective…

  19. Treatment of the genitourinary syndrome of menopause.

    PubMed

    Palacios, S; Mejía, A; Neyro, J L

    2015-10-01

    The vagina, vulva, vestibule, labia majora/minora, and bladder trigone have a high concentration of estrogen receptors; therefore, they are a sensitive biological indicator of serum levels of these hormones in women. The estrogen loss in postmenopausal women produces a dysfunction called genitourinary syndrome of menopause. The principal therapeutic goal in the genitourinary syndrome of menopause is to relieve symptoms. Treatment options, as well as local and systemic hormonal treatment are changes in lifestyle and non-hormonal treatments mainly based on the use of moisturizers and lubricants. New treatments that have recently appeared are ospemifeme, the first selective hormone receptor modulator for dyspareunia and vulvovaginal atrophy treatment, and the use of vaginal laser. This review has been written with the intention of giving recommendations on the prevention and treatment of genitourinary syndrome of menopause. PMID:26366797

  20. [Pharmacological treatment of syndromes of aggressivity].

    PubMed

    Itil, T M

    1978-01-01

    In the treatment of violent-aggressive behavior, four major groups of drugs emerged: 1. Major tranquilizers in the treatment of aggressive-violent behavior associated with psychotic syndromes. 2. Anti-epileptic drugs such as diphenylhydantoin and barbiturates in the treatment of aggressive-violent behavior within the epileptic syndrome. 3. Psychostimulants in the treatment of aggressive behavior of adolescents and children within behavior disturbances. 4. Anti-male hormones such as cyproterone acetate in the treatment of violent-aggressive behavior associated with pathological sexual hyperactivity. Whereas each category of drug is predominantly effective in one type of aggressive syndrome, it may also be effective in other conditions as well. Aggression as a result of a personality disorder is most difficult to treat with drugs. PMID:34189

  1. Examination and Treatment of Cuboid Syndrome

    PubMed Central

    Durall, Chris J.

    2011-01-01

    Context: Cuboid syndrome is thought to be a common source of lateral midfoot pain in athletes. Evidence Acquisition: A Medline search was performed via PubMed (through June 2010) using the search terms cuboid, syndrome, subluxed, locked, fault, dropped, peroneal, lateral, plantar, and neuritis with the Boolean term AND in all possible combinations. Retrieved articles were hand searched for additional relevant references. Results: Cuboid syndrome is thought to arise from subtle disruption of the arthrokinematics or structural congruity of the calcaneocuboid joint, although the precise pathomechanic mechanism has not been elucidated. Fibroadipose synovial folds (or labra) within the calcaneocuboid joint may play a role in the cause of cuboid syndrome, but this is highly speculative. The symptoms of cuboid syndrome resemble those of a ligament sprain. Currently, there are no definitive diagnostic tests for this condition. Case reports suggest that cuboid syndrome often responds favorably to manipulation and/or external support. Conclusions: Evidence-based guidelines regarding cuboid syndrome are lacking. Consequently, the diagnosis of cuboid syndrome is often based on a constellation of signs and symptoms and a high index of suspicion. Unless contraindicated, manipulation of the cuboid should be considered as an initial treatment. PMID:23016051

  2. Dementia syndromes: evaluation and treatment

    PubMed Central

    Scott, Kevin R; Barrett, Anna M

    2008-01-01

    As our population ages, diseases affecting memory and daily functioning will affect an increasing number of individuals, their families and the healthcare system. The social, financial and economic impacts will be profound. This article provides an overview of current dementia syndromes to assist clinicians in evaluating, educating and treating these patients. PMID:17425495

  3. Asperger Syndrome and Medication Treatment

    ERIC Educational Resources Information Center

    Tsai, Luke Y.

    2007-01-01

    Asperger syndrome (AS) is a neurobiological disorder whose core clinical symptoms include impairment in social interaction, impairments in verbal and nonverbal communication, and restricted, repetitive, and stereotyped patterns of behavior, interests, and activities. AS is often accompanied by coexisting neuropsychiatric disorders, including…

  4. [Noonan's syndrome and growth hormone treatment].

    PubMed

    Castinetti, F; Reynaud, R; Brue, T

    2008-09-01

    Noonan's syndrome is a clinical entity associating short stature, facial dysmorphy and congenital cardiomyopathy. In 50 % of cases, PTPN11 mutations are found, transmitted as an autosomal dominant trait. Mutations of other genes (KRAS, SOS1) were also recently reported. Short stature could be due to GH deficiency, abnormal neurosecretory function or GH insensitivity. GH treatment induces height gain, even if only few studies reported data on final height. Response to GH varies, depending on the presence of PTPN11 mutations. No cardiac adverse effects were reported to date with GH treatment in Noonan's syndrome. PMID:18954855

  5. [Laron syndrome: Presentation, treatment and prognosis].

    PubMed

    Latrech, Hanane; Polak, Michel

    2016-01-01

    Laron syndrome is a rare cause of short stature due to an abnormality of growth hormone receptor (GHR). It is characterized by poor phenotype-genotype correlation and geographic predilection essentially in the Mediterranean rim, the Middle East and Indian subcontinent. This syndrome corresponds to an endogenous and exogenous complete insensitivity of GH and manifests by early hypoglycemia, an extremely severe short stature and dysmorphic features contrasting with high levels of circulating GH. To date, treatment with recombinant IGF1 is the only treatment option that has improved the terrible prognosis in these patients but does not actually realize the conditions for genuine replacement therapy. PMID:26564390

  6. Pathophysiology and Treatment of Alien Hand Syndrome

    PubMed Central

    Sarva, Harini; Deik, Andres; Severt, William Lawrence

    2014-01-01

    Background Alien hand syndrome (AHS) is a disorder of involuntary, yet purposeful, hand movements that may be accompanied by agnosia, aphasia, weakness, or sensory loss. We herein review the most reported cases, current understanding of the pathophysiology, and treatments. Methods We performed a PubMed search in July of 2014 using the phrases “alien hand syndrome,” “alien hand syndrome pathophysiology,” “alien hand syndrome treatment,” and “anarchic hand syndrome.” The search yielded 141 papers (reviews, case reports, case series, and clinical studies), of which we reviewed 109. Non-English reports without English abstracts were excluded. Results Accumulating evidence indicates that there are three AHS variants: frontal, callosal, and posterior. Patients may demonstrate symptoms of multiple types; there is a lack of correlation between phenomenology and neuroimaging findings. Most pathologic and functional imaging studies suggest network disruption causing loss of inhibition as the likely cause. Successful interventions include botulinum toxin injections, clonazepam, visuospatial coaching techniques, distracting the affected hand, and cognitive behavioral therapy. Discussion The available literature suggests that overlap between AHS subtypes is common. The evidence for effective treatments remains anecdotal, and, given the rarity of AHS, the possibility of performing randomized, placebo-controlled trials seems unlikely. As with many other interventions for movement disorders, identifying the specific functional impairments caused by AHS may provide the best guidance towards individualized supportive care. PMID:25506043

  7. Neonatal Abstinence Syndrome: Presentation and Treatment Considerations.

    PubMed

    Jones, Hendrée E; Kaltenbach, Karol; Johnson, Elisabeth; Seashore, Carl; Freeman, Emily; Malloy, Erin

    2016-01-01

    This clinical case conference discusses the treatment of a pregnant woman with opioid use disorder in a comprehensive care program that includes buprenorphine pharmacotherapy. The presentation summarizes common experiences that pregnant women who receive buprenorphine pharmacotherapy face, and also what their prenatally opioid-exposed children confront in the immediate postpartum period. It describes the elements of a successful comprehensive care model and corollary neonatal abstinence syndrome treatment regimen. Expert commentary is included on issues that arise in the buprenorphine induction and maintenance throughout the prenatal and postpartum periods and in the treatment of co-occurring mental health problems during both the prenatal and postpartum periods, particularly the treatment of depression. There is also expert commentary on the care of opioid-exposed neonates, with attention to the treatment for neonatal abstinence syndrome. PMID:27244045

  8. Treatment strategy for Boerhaave's syndrome.

    PubMed

    Ochiai, T; Hiranuma, S; Takiguchi, N; Ito, K; Maruyama, M; Nagahama, T; Kawano, T; Nagai, K; Nishikage, T; Noguchi, N; Takamatsu, S; Kawamura, T; Teramoto, K; Iwai, T; Arii, S

    2004-01-01

    Esophageal rupture is a potentially mortal condition. Rapid and correct diagnosis, and urgent surgical treatment with esophagectomy is indicated, but conservative and other surgical treatments have also been reported recently. The treatment strategies for esophageal rupture are discussed here, based on our experiences with four cases during the last 10 years. They were admitted urgently and each was treated by a different method. Three of them underwent emergency operations, one undergoing primary closure of the ruptured esophagus, another received a T-tube insertion from the ruptured site with omental flap, and the third an esophagogastrectomy. The fourth case was treated conservatively. All patients survived and were discharged 36-144 days post treatment. One of them was readmitted for debridement of necrotic rib. In conclusion, the prompt and accurate diagnosis of esophageal rupture is crucial for a subsequent successful treatment. Conservative treatment or operation including esophagectomy will be determined by the severity of the condition. PMID:15209751

  9. Treatment of repetitive use carpal tunnel syndrome

    NASA Astrophysics Data System (ADS)

    Smith, Chadwick F.; Vangsness, C. Thomas; Anderson, Thomas; Good, Wayne

    1995-05-01

    In 1990, a randomized, double-blind study was initiated to evaluate the use of an eight-point conservative treatment program in carpal tunnel syndrome. A total of 160 patients were delineated with symptoms of carpal tunnel syndrome. These patients were then divided into two groups. Both groups were subjected to an ergonomically correct eight-point work modification program. A counterfeit low level laser therapy unit was utilized in Group A, while an actual low level laser therapy unit was utilized in Group B. The difference between Groups A and B was statistically significant in terms of return to work, conduction study improvement, and certain range of motion and strength studies.

  10. [Burnout syndrome: diagnosis, principles of treatment, prophylaxis].

    PubMed

    Ovchinnikov, Yu V; Palchenkova, M V; Kalachev, O V

    2015-07-01

    Burnout syndrome is a socio-psychological phenomenon of emotional, motivational, and physical exhaustion as a result of chronic occupational stress. It is manifested as long-term emotional exhaustion, depersonalization and diminished personal and professional achievements. Burnout syndrome is common among health care workers, 'due to their high dedication, empathy for suffering patients, and decision-making related to life and health of patients. Personal, role and organizational factors influence on development of burnout. The clinical picture of burnout is multifactorial and can be described as a set of psychosomatic and somatic disorders, symptoms of social dysfunction. Diversity and non-specific symptoms of burnout syndrome determine the need for an interdisciplinary approach to its diagnosis. The leading role in solving problems related to stress and emotional burnout plays psychotherapy. The paper presents diagnostic criteria, risk factors, and methods of prevention and treatment of emotional burnout. PMID:26821457

  11. Hepatorenal Syndrome: Aetiology, Diagnosis, and Treatment

    PubMed Central

    Low, G.; Alexander, G. J. M.; Lomas, D. J.

    2015-01-01

    Acute renal impairment is common in patients with chronic liver disease, occurring in approximately 19% of hospitalised patients with cirrhosis. A variety of types of renal impairment are recognised. The most important of these is the hepatorenal syndrome, a functional renal impairment due to circulatory and neurohormonal abnormalities that underpin cirrhosis. It is one of the most severe complications of cirrhosis with survival often measured in weeks to months. A variety of treatment options exist with early diagnosis and appropriate treatment providing the best hope for cure. This paper provides a comprehensive and up-to-date review of hepatorenal syndrome and lays out the topic according to the following sections: pathophysiology, historical developments, diagnostic criteria and limitations, epidemiology, precipitating factors, predictors, clinical and laboratory findings, prognosis, treatment options, prophylaxis, and conclusion. PMID:25649410

  12. Diagnosis and Treatment of Polycystic Ovary Syndrome.

    PubMed

    Williams, Tracy; Mortada, Rami; Porter, Samuel

    2016-07-15

    Polycystic ovary syndrome is the most common endocrinopathy among reproductive-aged women in the United States, affecting approximately 7% of female patients. Although the pathophysiology of the syndrome is complex and there is no single defect from which it is known to result, it is hypothesized that insulin resistance is a key factor. Metabolic syndrome is twice as common in patients with polycystic ovary syndrome compared with the general population, and patients with polycystic ovary syndrome are four times more likely than the general population to develop type 2 diabetes mellitus. Patient presentation is variable, ranging from asymptomatic to having multiple gynecologic, dermatologic, or metabolic manifestations. Guidelines from the Endocrine Society recommend using the Rotterdam criteria for diagnosis, which mandate the presence of two of the following three findings- hyperandrogenism, ovulatory dysfunction, and polycystic ovaries-plus the exclusion of other diagnoses that could result in hyperandrogenism or ovulatory dysfunction. It is reasonable to delay evaluation for polycystic ovary syndrome in adolescent patients until two years after menarche. For this age group, it is also recommended that all three Rotterdam criteria be met before the diagnosis is made. Patients who have marked virilization or rapid onset of symptoms require immediate evaluation for a potential androgen-secreting tumor. Treatment of polycystic ovary syndrome is individualized based on the patient's presentation and desire for pregnancy. For patients who are overweight, weight loss is recommended. Clomiphene and letrozole are first-line medications for infertility. Metformin is the first-line medication for metabolic manifestations, such as hyperglycemia. Hormonal contraceptives are first-line therapy for irregular menses and dermatologic manifestations. PMID:27419327

  13. SURGICAL TREATMENT FOR KYPHOSCOLIOSIS IN COHEN SYNDROME

    PubMed Central

    IMAGAMA, SHIRO; TSUJI, TAICHI; OHARA, TETSUYA; KATAYAMA, YOSHITO; GOTO, MANABU; ISHIGURO, NAOKI; KAWAKAMI, NORIAKI

    2013-01-01

    ABSTRACT Cohen syndrome is a very rare disease. Complication by spinal deformity has been reported, but management and surgery for spinal deformity in Cohen syndrome has not been previously described. The objective of this study was to examine the outcome of surgical treatment for kyphoscoliosis of Cohen syndrome with a literature review. The patient was a 14-year-old male with the characteristics of Cohen syndrome: truncal obesity, mental retardation, arachnodactyly, microcephalia, and a facial malformation. Scoliosis was conservatively treated with a brace at 13 years of age, but the spinal deformity rapidly progressed within a year. Plain radiographs before surgery showed scoliosis of 47° (T5-T11) and 79° (T11-L3), and kyphosis of 86° (T7-L1). One-stage anteroposterior corrective fusion of T4-L3 was scheduled after 2-week Halo traction. Postoperative respiratory management was carefully performed because of Cohen syndrome-associated facial malformation, obesity, and reduced muscle tonus. Respiration was managed with intubation until the following day and no respiratory problems occurred. After surgery, thoracolumbar scoliosis was 28° (correction rate: 65%). Kyphosis was markedly improved from 86° to 20°, achieving a favorable balance of the trunk. The outcome is favorable at 6.5 years after surgery. In conclusion, Cohen syndrome is often complicated by spinal deformity, particularly kyphosis, that is likely to progress even in adulthood. In our patient, spinal deformity progressed within a short period, even with brace treatment. Surgery should be required before progression to the severe spinal deformity with careful attention to general anesthesia. PMID:24640185

  14. Surgical treatment for kyphoscoliosis in Cohen syndrome.

    PubMed

    Imagama, Shiro; Tsuji, Taichi; Ohara, Tetsuya; Katayama, Yoshito; Goto, Manabu; Ishiguro, Naoki; Kawakami, Noriaki

    2013-08-01

    Cohen syndrome is a very rare disease. Complication by spinal deformity has been reported, but management and surgery for spinal deformity in Cohen syndrome has not been previously described. The objective of this study was to examine the outcome of surgical treatment for kyphoscoliosis of Cohen syndrome with a literature review. The patient was a 14-year-old male with the characteristics of Cohen syndrome: truncal obesity, mental retardation, arachnodactyly, microcephalia, and a facial malformation. Scoliosis was conservatively treated with a brace at 13 years of age, but the spinal deformity rapidly progressed within a year. Plain radiographs before surgery showed scoliosis of 47 degrees (T5-T11) and 79 degrees (T11-L3), and kyphosis of 86 degrees (T7-L1). One-stage anteroposterior corrective fusion of T4-L3 was scheduled after 2-week Halo traction. Postoperative respiratory management was carefully performed because of Cohen syndrome-associated facial malformation, obesity, and reduced muscle tonus. Respiration was managed with intubation until the following day and no respiratory problems occurred. After surgery, thoracolumbar scoliosis was 28 degrees (correction rate: 65%). Kyphosis was markedly improved from 86 degrees to 20 degrees, achieving a favorable balance of the trunk. The outcome is favorable at 6.5 years after surgery. In conclusion, Cohen syndrome is often complicated by spinal deformity, particularly kyphosis, that is likely to progress even in adulthood. In our patient, spinal deformity progressed within a short period, even with brace treatment. Surgery should be required before progression to the severe spinal deformity with careful attention to general anesthesia. PMID:24640185

  15. Chronic fatigue syndrome: aetiology, diagnosis and treatment

    PubMed Central

    Avellaneda Fernández, Alfredo; Pérez Martín, Álvaro; Izquierdo Martínez, Maravillas; Arruti Bustillo, Mar; Barbado Hernández, Francisco Javier; de la Cruz Labrado, Javier; Díaz-Delgado Peñas, Rafael; Gutiérrez Rivas, Eduardo; Palacín Delgado, Cecilia; Rivera Redondo, Javier; Ramón Giménez, José Ramón

    2009-01-01

    Chronic fatigue syndrome is characterised by intense fatigue, with duration of over six months and associated to other related symptoms. The latter include asthenia and easily induced tiredness that is not recovered after a night's sleep. The fatigue becomes so severe that it forces a 50% reduction in daily activities. Given its unknown aetiology, different hypotheses have been considered to explain the origin of the condition (from immunological disorders to the presence of post-traumatic oxidative stress), although there are no conclusive diagnostic tests. Diagnosis is established through the exclusion of other diseases causing fatigue. This syndrome is rare in childhood and adolescence, although the fatigue symptom per se is quite common in paediatric patients. Currently, no curative treatment exists for patients with chronic fatigue syndrome. The therapeutic approach to this syndrome requires a combination of different therapeutic modalities. The specific characteristics of the symptomatology of patients with chronic fatigue require a rapid adaptation of the educational, healthcare and social systems to prevent the problems derived from current systems. Such patients require multidisciplinary management due to the multiple and different issues affecting them. This document was realized by one of the Interdisciplinary Work Groups from the Institute for Rare Diseases, and its aim is to point out the main social and care needs for people affected with Chronic Fatigue Syndrome. For this, it includes not only the view of representatives for different scientific societies, but also the patient associations view, because they know the true history of their social and sanitary needs. In an interdisciplinary approach, this work also reviews the principal scientific, medical, socio-sanitary and psychological aspects of Chronic Fatigue Syndrome. PMID:19857242

  16. [Klinefelter's syndrome: diagnosis and treatment. Case report].

    PubMed

    Martini, R

    2001-10-01

    Klinefelter's syndrome affects 1 in 500 men across all ethnic groups but the diagnosis is often delayed because of substantial variations in clinical presentation. A 26 year-old male came to observation for chronic fatigue. His laboratory data and radiological examination were negative. Examination showed eunuchoidal body habitus with sparse facial hair, small and firm testes and no gynecomastia. The patient had heterosexual orientation with regular sexual intercourses but diminished libido. Serum gonadotropin concentrations were raised while serum testosterone concentration was low-normal level. Serum PRL concentration and thyroid function were normal. Seminal analysis revealed azoospermia and peripheral lymphocyte karyotyping showed a 47,XXY karyotype, confirming diagnostic suspicion. Patient was given testosterone enanthate 200 mg intramuscularly every 2 weeks. He noted improvements in fatigue and libido and increase of muscle mass. Since the true prevalence of Klinefelter's syndrome is very high, the diagnosis of this disease should be considered in every men with complaints related to hypogonadism (fatigue, weakness, gynecomastia, infertility, erectile dysfunction, small testis and osteoporosis). Testosterone replacement therapy should be started early to minimize the physical and psychological effects of androgen deficiency. There have been recent advances in the options for the treatment of infertility in patients with Klinefelter's syndrome: however findings that this syndrome may be transmitted by the new assisted reproductive techniques is cause for concern. PMID:11675582

  17. Cushing's Syndrome Masquerading as Treatment Resistant Depression

    PubMed Central

    Anil Kumar, B. N.; Grover, Sandeep

    2016-01-01

    Treatment resistant depression (TRD) is a common clinical occurrence among patients treated for major depressive disorder. A significant proportion of patients remain significantly depressed in spite of aggressive pharmacological and psychotherapeutic approaches. Management of patient with treatment resistant depression requires thorough evaluation for physical causes. We report a case of recurrent depressive disorder, who presented with severe depressive episode without psychotic symptoms, not responding to multiple adequate trials of antidepressants, who on investigation was found to have Cushing's syndrome and responded well to Ketoconazole. PMID:27335521

  18. Cushing's Syndrome Masquerading as Treatment Resistant Depression.

    PubMed

    Anil Kumar, B N; Grover, Sandeep

    2016-01-01

    Treatment resistant depression (TRD) is a common clinical occurrence among patients treated for major depressive disorder. A significant proportion of patients remain significantly depressed in spite of aggressive pharmacological and psychotherapeutic approaches. Management of patient with treatment resistant depression requires thorough evaluation for physical causes. We report a case of recurrent depressive disorder, who presented with severe depressive episode without psychotic symptoms, not responding to multiple adequate trials of antidepressants, who on investigation was found to have Cushing's syndrome and responded well to Ketoconazole. PMID:27335521

  19. What's New in Myelodysplastic Syndrome Research and Treatment?

    MedlinePlus

    ... Next Topic Additional resources for myelodysplastic syndromes What`s new in myelodysplastic syndrome research and treatment? Genetics and ... research unfolds, it may be used to design new drugs or eventually in developing gene therapy. This ...

  20. [Effectiveness of dietetic treatment in nephrotic syndrome].

    PubMed

    Calleja Fernández, A; López Gómez, J J; Vidal Casariego, A; Cano Rodríguez, I; Ballesteros Pomar, M D

    2009-01-01

    We present the case of a male patient suffering from a primary amyloidosis and a nephrotic syndrome who came to dietotherapy clinic. In the first visit we made a nutritional record including an anthropometric evaluation, body composition, biochemistry, and food intake. The patient had an excess of body water, proteinuria, low plasma protein, albumin, prealbumin and HDL cholesterol levels, and high concentrations of total cholesterol, LDL and triglycerides. The consumption of protein and sodium was higher than the recommendation. An individualized diet was made. Six months later, his weight and the excess of body water decreased, but the fat free mass remained unchanged. The levels of albumin and prealbumin increased, the proteinuria decreased. Total cholesterol, LDL and triglycerides decreased until normal levels. Dietetary treatment in nephrotic syndrome is effective to decrease proteinuria, improve cholesterol and triglycerides levels, and to prevent malnutrition. PMID:20049380

  1. Kabuki syndrome: diagnostic and treatment considerations

    PubMed Central

    2012-01-01

    Kabuki syndrome (KS) is a rare genetic disorder first diagnosed in 1981. Unknown by most primary care physicians and clinicians in the mental health fields, children with KS present with unique facial characteristics, mental retardation, health problems and socio-emotional delays that are often mistaken for other diagnostic problems. Literature detailing the psychological and psychosocial features of this disorder is scant, and psychotherapeutic approaches have not been described. In this article, we present a case description and treatment of a child with KS and her family. A brief review of KS is then provided, highlighting its signs and symptoms. Factors related to differential diagnoses are identified to aid primary care and mental health clinicians in better understanding this unique syndrome. Interventions with similar populations are discussed from which a psychological approach to KS is suggested. Finally, implications for primary care physicians are described and suggestions for further research indicated. PMID:23997823

  2. [Neuroleptic malignant syndrome from treatment with antidepressives].

    PubMed

    Heinemann, F; Assion, H J; Laux, G

    1997-05-01

    The neuroleptic malignant syndrome (NMS) is a rare complication in the treatment of neuroleptics. The pathophysiology is not fully known. A dopaminergic transmission block in the basal ganglia and the hypothalamus is thought to be the pathophysiological mechanism of NMS. There are some findings against the single role of dopamine receptor blockade: NMS is rare under neuroleptic treatment, although a strong dopamine receptor blockade is found even with a low dosis of neuroleptics. NMS can develop even after longterm treatment with neuroleptics and is not improved by dopamine agonists within the expected period. NMS may even develop when neuroleptics are reduced. Several cases have been reported of NMS precipitated by medication without a direct effect on dopaminergic system. Only rare case reports describe NMS under antidepressants. We report on all cases of NMS associated with antidepressants and present the different pathophysiological hypotheses on the precipitation of NMS. PMID:9235312

  3. Premenstrual Syndrome: Approaches to Diagnosis and Treatment

    PubMed Central

    Simkin, Ruth J.

    1985-01-01

    There has been much confusion in the literature over the definition, diagnosis and treatment of premenstrual syndrome (PMS). This article discusses definitions of PMS, incidence, etiology and symptomatology. Diagnosis depends on the timing of symptoms rather than the type. Symptoms commonly occur during the late premenstruum; at ovulation and during the premenstruum; or at ovulation, gradually increasing in severity throughout the luteal phase. To diagnose PMS, three consecutive menstrual cycles must be charted, the symptoms must be limited to the luteal phase, and there must be a complete absence of symptoms for at least one week in the postmenstruum. Rational treatment programs for mild, moderate and severe PMS are proposed. The role of progesterone in treatment is discussed. PMID:21274209

  4. Advances in Tourette syndrome: diagnoses and treatment.

    PubMed

    Serajee, Fatema J; Mahbubul Huq, A H M

    2015-06-01

    Tourette syndrome (TS) is a childhood-onset neurodevelopmental disorder characterized by multiple motor tics and at least one vocal or phonic tic, and often one or more comorbid psychiatric disorders. Premonitory sensory urges before tic execution and desire for "just-right" perception are central features. The pathophysiology involves cortico-striato-thalamo-cortical circuits and possibly dopaminergic system. TS is considered a genetic disorder but the genetics is complex and likely involves rare mutations, common variants, and environmental and epigenetic factors. Treatment is multimodal and includes education and reassurance, behavioral interventions, pharmacologic, and rarely, surgical interventions. PMID:26022170

  5. Treatment strategies for tics in Tourette syndrome

    PubMed Central

    Eddy, Clare M.; Rickards, Hugh E.; Cavanna, Andrea E.

    2011-01-01

    Tourette syndrome (TS) is a chronic neurodevelopmental disorder characterized by tics: repetitive, involuntary movements and vocalizations. These symptoms can have a significant impact on patients’ daily functioning across many domains. Tics tend to be most severe in child and adolescent sufferers, so their presence has the potential to impact a period of life that is both critical for learning and is often associated with the experience of greater social tension and self-consciousness than adulthood. Furthermore, control over tics that lead to physical impairment or self-injurious behaviour is of vital importance in maintaining health and quality of life. There are numerous complicating factors in the prescription of treatment for tics, due to both the side effects associated with alleviating agents and patient characteristics, such as age and comorbid conditions. This review summarizes literature pertaining to the efficacy and safety of both traditionally prescribed and more modern medications. We also discuss the merits of behavioural and surgical techniques and highlight newer emerging treatments. Although treatment response is to some extent variable, there are a number of agents that are clearly useful as first-line treatments for TS. Other interventions may be of most benefit to patients exhibiting refractory tics or more specific symptom profiles. PMID:21339906

  6. The Treatment of Irritable Bowel Syndrome

    PubMed Central

    Weiser, Kirsten; De Lee, Ryan

    2009-01-01

    Irritable bowel syndrome (IBS) is a highly prevalent functional bowel disorder routinely encountered by healthcare providers. Although not life-threatening, this chronic disorder reduces patients’ quality of life and imposes a significant economic burden to the healthcare system. IBS is no longer considered a diagnosis of exclusion that can only be made after performing a battery of expensive diagnostic tests. Rather, IBS should be confidently diagnosed in the clinic at the time of the first visit using the Rome III criteria and a careful history and physical examination. Treatment options for IBS have increased in number in the past decade and clinicians should not be limited to using only fiber supplements and smooth muscle relaxants. Although all patients with IBS have symptoms of abdominal pain and disordered defecation, treatment needs to be individualized and should focus on the predominant symptom. This paper will review therapeutic options for the treatment of IBS using a tailored approach based on the predominant symptom. Abdominal pain, bloating, constipation and diarrhea are the four main symptoms that can be addressed using a combination of dietary interventions and medications. Treatment options include probiotics, antibiotics, tricyclic antidepressants, selective serotonin reuptake inhibitors and agents that modulate chloride channels and serotonin. Each class of agent will be reviewed using the latest data from the literature. PMID:21180545

  7. Treatment options for polycystic ovary syndrome

    PubMed Central

    Badawy, Ahmed; Elnashar, Abubaker

    2011-01-01

    Polycystic ovary syndrome (PCOS) is the most common endocrine disorder in women. The clinical manifestation of PCOS varies from a mild menstrual disorder to severe disturbance of reproductive and metabolic functions. Management of women with PCOS depends on the symptoms. These could be ovulatory dysfunction-related infertility, menstrual disorders, or androgen-related symptoms. Weight loss improves the endocrine profile and increases the likelihood of ovulation and pregnancy. Normalization of menstrual cycles and ovulation could occur with modest weight loss as little as 5% of the initial weight. The treatment of obesity includes modifications in lifestyle (diet and exercise) and medical and surgical treatment. In PCOS, anovulation relates to low follicle-stimulating hormone concentrations and the arrest of antral follicle growth in the final stages of maturation. This can be treated with medications such as clomiphene citrate, tamoxifen, aromatase inhibitors, metformin, glucocorticoids, or gonadotropins or surgically by laparoscopic ovarian drilling. In vitro fertilization will remain the last option to achieve pregnancy when others fail. Chronic anovulation over a long period of time is also associated with an increased risk of endometrial hyperplasia and carcinoma, which should be seriously investigated and treated. There are androgenic symptoms that will vary from patient to patient, such as hirsutism, acne, and/or alopecia. These are troublesome presentations to the patients and require adequate treatment. Alternative medicine has been emerging as one of the commonly practiced medicines for different health problems, including PCOS. This review underlines the contribution to the treatment of different symptoms. PMID:21339935

  8. Irritable bowel syndrome treatment: cognitive behavioral therapy versus medical treatment

    PubMed Central

    Mahvi-Shirazi, Majid; Rasoolzade-Tabatabaei, Sayed-Kazem; Amini, Mohsen

    2012-01-01

    Introduction The study aims to investigate two kinds of treatment in patients suffering from irritable bowel syndrome (IBS) and consequently compares its efficacy on improving the symptoms and mental health of patients; one with just medical treatment and another through a combination of psychotherapy and medical treatment. Material and methods Applying general sampling, 50 IBS patients were selected from among those who used to refer to a Gastroenterology Clinic. After physical and mental evaluations based on ROME-II scale and SCL-90-R questionnaires, the subjects were randomly superseded into: the control group with medical treatment and, the case group with a combination of medical and psychological treatments. The acquired data were then analyzed through t-test and Mann-Whitney U-test. Results The findings show that the mental health of patients receiving cognitive behavioral therapy along with the medical treatment was higher than those of the control group at post-test level. It was observed that the therapy reduces the disability caused by IBS. Comparatively, while the cognitive therapy and medical treatments cured 80% of the patients, those receiving cognitive therapy alone showed an extensive reduction of symptoms. Conclusions Considering the role of cognitive behavioral therapy, it is therefore recommend that such patients be managed by a combined team of gastroenterologists and psychologists. PMID:22457686

  9. Fetal Alcohol Syndrome: Characteristics, Prevention, Treatment and Long Term Outlook.

    ERIC Educational Resources Information Center

    Seward, Cynthia A.; Barber, William H.

    1991-01-01

    This article discusses fetal alcohol syndrome (FAS) including causes, common characteristics, secondary characteristics, prevention, and treatment. Economic implications are noted which suggest that treatment costs are 100 times the cost of prevention programs. (DB)

  10. What Are the Treatments for Cushing's Syndrome?

    MedlinePlus

    ... syndrome: A systematic review and meta-analysis. European Journal of Internal Medicine, 23 (3), 278-282. PMID 22385888 . [top] National Library of Medicine, MedlinePlus. Cushing’s syndrome. Retrieved April ...

  11. Fibromyalgia Syndrome: Etiology, Pathogenesis, Diagnosis, and Treatment

    PubMed Central

    Bellato, Enrico; Marini, Eleonora; Castoldi, Filippo; Barbasetti, Nicola; Mattei, Lorenzo; Bonasia, Davide Edoardo; Blonna, Davide

    2012-01-01

    Fibromyalgia syndrome is mainly characterized by pain, fatigue, and sleep disruption. The etiology of fibromyalgia is still unclear: if central sensitization is considered to be the main mechanism involved, then many other factors, genetic, immunological, and hormonal, may play an important role. The diagnosis is typically clinical (there are no laboratory abnormalities) and the physician must concentrate on pain and on its features. Additional symptoms (e.g., Raynaud's phenomenon, irritable bowel disease, and heat and cold intolerance) can be associated with this condition. A careful differential diagnosis is mandatory: fibromyalgia is not a diagnosis of exclusion. Since 1990, diagnosis has been principally based on the two major diagnostic criteria defined by the ACR. Recently, new criteria have been proposed. The main goals of the treatment are to alleviate pain, increase restorative sleep, and improve physical function. A multidisciplinary approach is optimal. While most nonsteroidal anti-inflammatory drugs and opioids have limited benefit, an important role is played by antidepressants and neuromodulating antiepileptics: currently duloxetine (NNT for a 30% pain reduction 7.2), milnacipran (NNT 19), and pregabalin (NNT 8.6) are the only drugs approved by the US Food and Drug Administration for the treatment of fibromyalgia. In addition, nonpharmacological treatments should be associated with drug therapy. PMID:23213512

  12. Fragile X Syndrome and Targeted Treatment Trials

    PubMed Central

    Lauterborn, Julie; Au, Jacky; Berry-Kravis, Elizabeth

    2014-01-01

    Work in recent years has revealed an abundance of possible new treatment targets for fragile X syndrome (FXS). The use of animal models, including the fragile X knockout mouse which manifests a phenotype very similar to FXS in humans, has resulted in great strides in this direction of research. The lack of Fragile X Mental Retardation Protein (FMRP) in FXS causes dysregulation and usually overexpression of a number of its target genes, which can cause imbalances of neurotransmission and deficits in synaptic plasticity. The use of metabotropic glutamate receptor (mGluR) blockers and gamma amino-butyric acid (GABA) agonists have been shown to be efficacious in reversing cellular and behavioral phenotypes, and restoring proper brain connectivity in the mouse and fly models. Proposed new pharmacological treatments and educational interventions are discussed in this chapter. In combination, these various targeted treatments show promising preliminary results in mitigating or even reversing the neurobiological abnormalities caused by loss of FMRP, with possible translational applications to other neurodevelopmental disorders including autism. PMID:22009360

  13. Current treatment of atypical hemolytic uremic syndrome

    PubMed Central

    Kaplan, Bernard S.; Ruebner, Rebecca L.; Spinale, Joann M.; Copelovitch, Lawrence

    2014-01-01

    Summary Tremendous advances have been made in understanding the pathogenesis of atypical Hemolytic Uremic Syndrome (aHUS), an extremely rare disease. Insights into the molecular biology of aHUS resulted in rapid advances in treatment with eculizumab (Soliris®, Alexion Pharmaceuticals Inc.). Historically, aHUS was associated with very high rates of mortality and morbidity. Prior therapies included plasma therapy and/or liver transplantation. Although often life saving, these were imperfect and had many complications. We review the conditions included under the rubric of aHUS: S. pneumoniae HUS (SpHUS), inborn errors of metabolism, and disorders of complement regulation, emphasizing their differences and similarities. We focus on the clinical features, diagnosis, and pathogenesis, and treatment of aHUS that results from mutations in genes encoding alternative complement regulators, SpHUS and HUS associated with inborn errors of metabolism. Mutations in complement genes, or antibodies to their protein products, result in unregulated activity of the alternate complement pathway, endothelial injury, and thrombotic microangiopathy (TMA). Eculizumab is a humanized monoclonal antibody that inhibits the production of the terminal complement components C5a and the membrane attack complex (C5b-9) by binding to complement protein C5a. This blocks the proinflammatory and cytolytic effects of terminal complement activation. Eculizumab use has been reported in many case reports, and retrospective and prospective clinical trials in aHUS. There have been few serious side effects and no reports of tachphylaxis or drug resistance. The results are very encouraging and eculizumab is now recognized as the treatment of choice for aHUS. PMID:25343125

  14. Overactive bladder syndrome pharmacotherapy: future treatment options

    PubMed Central

    2015-01-01

    Overactive bladder syndrome (OAB) is a lifestyle disease and its incidence increases with age. Although it is not a life-threatening disease, it is known to have a significant impact on the quality of life. The first-choice pharmacological treatment of OAB is antimuscarinics. However, their limited clinical effectiveness and unsatisfactory tolerance profile, combined with the advancement of knowledge on the aetiopathogenesis of the disease, have inspired research on new pharmacotherapy options for OAB. Basic research has provided foundations for the development of new OAB treatments, which seem very promising and can be applied in clinical practice. The mechanisms of the studied compounds are based on their effect on certain receptors and neurotransmitters that contribute to regulating the micturition reflex. These compounds are not only more receptor-specific as compared to currently used drugs, but also some of them are organ-specific. Some of such compounds have already passed the proof-of-concept stage of development and have the therapeutic potential to determine the future of OAB pharmacotherapy. This review focuses on the mechanisms of substances that are now undergoing pre-clinical and clinical tests and their effects on the micturition cycle, while also identifying opportunities for using them with specific groups of patients. Due to the fact that OAB is a disease of symptoms and its aetiopathogenesis is complex, it seems that modern treatment methods should be tailor-made and based on the pathophysiological mechanisms that induce disease symptoms, rather than only treating the symptoms by inhibiting the contractility of the urinary bladder. PMID:26848291

  15. [Irritable Bowel Syndrome treatment: a multidisciplinary approach].

    PubMed

    Shani-Zur, Dana; Wolkomir, Keren

    2015-01-01

    Irritable Bowel Syndrome affects 9-23% of the general population. This diagnosis contributes to more frequent doctor visits and multiple consultations by patients. The current approach to treating IBS is symptomatic and consists of a regimen of first line pharmacological treatment options; the use of anti-depressant drugs is also common. The efficiency of complementary medicine in the treatment of IBS has been studied in the last few years. Qualitative multidisciplinary approach studies, using personalized medicines with complementary therapies are needed. We present the case of a 39-year-old woman with a diagnosis of IBS since 2009, who complained about gastrointestinal symptoms since the age of 13 and severe episodes of spasmodic stomach aches in the last year self-ranked as 10, on a 0-10 scale; 3-4 episodes a month, which last for 5 days, accompanied by severe flatulence and bloating. In addition, she has constipation (one bowel movement every 10 days), alternating with multiple diarrheic bowel movements (6 times a day). Using a multidisciplinary approach, including medicinal care, Chinese medicine, reflexology and naturopathy resulted in significant improvement in symptoms and quality of life, as well as gradual reduction of drugs, approved by her physician. Stomach ache self-ranked now as 1, on a 0-10 scale; and flatulence and bloating self-ranked as mild. Bowel movement frequency increased and is now every other day. She no longer has diarrheic and/or multiple bowel movements. This case report emphasizes the importance of integrative treatment in IBS and its benefit in improving patients' quality of life. PMID:25796677

  16. Gonococcal Conjunctivitis Despite Successful Treatment of Male Urethritis Syndrome.

    PubMed

    Peters, Remco P H; Verweij, Stephan P; McIntyre, James A; Schaftenaar, Erik

    2016-02-01

    We report a case of progressive, cephalosporin-susceptible, Neisseria gonorrhoeae conjunctivitis despite successful treatment of male urethritis syndrome. We hypothesize that conjunctival infection progressed due to insufficient penetration of cefixime and azithromycin and point out that extragenital infection and male urethritis may not be cured simultaneously in settings where the syndromic approach is used. PMID:26760182

  17. [Blue nevus syndrome: endoscopic treatment by sclerosis and banding ligation].

    PubMed

    Sala Felis, T; Urquijo Ponce, J J; López Viedma, B; Pertejo Pastor, V; Berenguer Lapuerta, J

    1999-03-01

    The blue rubber bleb nevus syndrome is a rare entity characterized by the presence of cavernous hemangiomas in the skin and gastrointestinal tract with frequent digestive hemorrhages. Different therapeutic modalities exist: medical treatment, surgical resection; and most recently, endoscopic therapy has been described. We present a patient with blue rubber bleb nevus syndrome treated with combined endoscopic therapy: sclerosis and band ligation. PMID:10228324

  18. Sanfilippo syndrome: causes, consequences, and treatments

    PubMed Central

    Fedele, Anthony O

    2015-01-01

    Sanfilippo syndrome, or mucopolysaccharidosis (MPS) type III, refers to one of five autosomal recessive, neurodegenerative lysosomal storage disorders (MPS IIIA to MPS IIIE) whose symptoms are caused by the deficiency of enzymes involved exclusively in heparan sulfate degradation. The primary characteristic of MPS III is the degeneration of the central nervous system, resulting in mental retardation and hyperactivity, typically commencing during childhood. The significance of the order of events leading from heparan sulfate accumulation through to downstream changes in the levels of biomolecules within the cell and ultimately the (predominantly neuropathological) clinical symptoms is not well understood. The genes whose deficiencies cause the MPS III subtypes have been identified, and their gene products, as well as a selection of disease-causing mutations, have been characterized to varying degrees with respect to both frequency and direct biochemical consequences. A number of genetic and biochemical diagnostic methods have been developed and adopted by diagnostic laboratories. However, there is no effective therapy available for any form of MPS III, with treatment currently limited to clinical management of neurological symptoms. The availability of animal models for all forms of MPS III, whether spontaneous or generated via gene targeting, has contributed to improved understanding of the MPS III subtypes, and has provided and will deliver invaluable tools to appraise emerging therapies. Indeed, clinical trials to evaluate intrathecally-delivered enzyme replacement therapy in MPS IIIA patients, and gene therapy for MPS IIIA and MPS IIIB patients are planned or underway. PMID:26648750

  19. Munchausen Syndrome by Proxy: Evaluation and Treatment.

    ERIC Educational Resources Information Center

    Parnell, Teresa F.; Day, Deborah O.

    Munchausen Syndrome by Proxy (MSBP) is characterized by a significant caretaker, usually a mother, deliberately inducing and/or falsely reporting illness in a child. The potentially fatal outcome of undetected MSBP makes the understanding of this syndrome gravely important. Early detection and effective intervention can be accomplished through the…

  20. What Are the Treatments for Symptoms in Klinefelter Syndrome?

    MedlinePlus

    ... benefits with their health care provider. Treatment for Infertility Between 95% and 99% of XXY men are ... et al. (1999). Klinefelter's syndrome in the male infertility clinic. Human Reproduction , 14 (4), 946-952. [top] ...

  1. [Diabetic foot syndrome: modern approaches to its treatment].

    PubMed

    Danilenko, S Iu; Plekhanov, A N; Markevich, P A

    2012-01-01

    Recent data on the results of treatment of diabetic foot syndrome are reported with special reference to its conservative therapy, surgical and endovascular methods. Modern dressing materials are described. PMID:22690559

  2. Apert Syndrome: Late presentation and treatment challenges

    PubMed Central

    Kheir, Abdelmoneim E M; Hamed, Ahlam A; Maki, Wala M; Hasan, Lina H M

    2014-01-01

    Apert syndrome is a rare autosomal dominant disorder characterized by craniosynostosis, craniofacial anomalies, and severe symmetrical syndactyly of the hands and feet. Anomalies of the viscera, skeleton and cardiovascular system have also been reported… Untreated craniosynostosis leads to inhibition of brain growth and an increase in intracranial and intraorbital pressure. Most cases are sporadic, resulting from new mutations with a paternal age effect. The prognosis of Apert Syndrome depends on the severity of brain malformation and early surgical interventions. We describe a Sudanese infant with Apert syndrome who presented for the first time at the age of three months and had limited options for intervention.

  3. Drug-Hypersensitivity Syndrome: Diagnosis and Treatment

    PubMed Central

    Hamm, Rose L.

    2012-01-01

    Drug-induced hypersensitivity syndrome is a systemic autoimmune disorder that results in mucocutaneous symptoms ranging in severity from mild pruritus to life-threatening skin and mucosal loss, with different nomenclature depending on the severity of the symptoms. The purpose of this article is to review the recent advances in understanding the pathology of drug-induced hypersensitivity syndrome, as well as current recommendations for both medical and wound management. PMID:24527369

  4. Acupuncture for treatment of irritable bowel syndrome

    PubMed Central

    Manheimer, Eric; Cheng, Ke; Wieland, L. Susan; Min, Li Shih; Shen, Xueyong; Berman, Brian M; Lao, Lixing

    2013-01-01

    Background Irritable bowel syndrome (IBS) is a common, costly, and difficult to treat disorder that impairs health-related quality of life and work productivity. Evidence-based treatment guidelines have been unable to provide guidance on the effects of acupuncture for IBS because the only previous systematic review included only small, heterogeneous and methodologically unsound trials. Objectives The primary objectives were to assess the efficacy and safety of acupuncture for treating IBS. Search methods MEDLINE, the Cochrane Central Register of Controlled Trials, EMBASE, the Cumulative Index to Nursing and Allied Health, and the Chinese databases Sino-Med, CNKI, and VIP were searched through November 2011. Selection criteria Randomized controlled trials (RCTs) that compared acupuncture with sham acupuncture, other active treatments, or no (specific) treatment, and RCTs that evaluated acupuncture as an adjuvant to another treatment, in adults with IBS were included. Data collection and analysis Two authors independently assessed the risk of bias and extracted data. We extracted data for the outcomes overall IBS symptom severity and health-related quality of life. For dichotomous data (e.g. the IBS Adequate Relief Question), we calculated a pooled relative risk (RR) and 95% confidence interval (CI) for substantial improvement in symptom severity after treatment. For continuous data (e.g. the IBS Severity Scoring System), we calculated the standardized mean difference (SMD) and 95% CI in post-treatment scores between groups. Main results Seventeen RCTs (1806 participants) were included. Five RCTs compared acupuncture versus sham acupuncture. The risk of bias in these studies was low.We found no evidence of an improvement with acupuncture relative to sham (placebo) acupuncture for symptom severity (SMD-0.11, 95%CI −0.35 to 0.13; 4 RCTs; 281 patients) or quality of life (SMD = −0.03, 95%CI −0.27 to 0.22; 3 RCTs; 253 patients). Sensitivity analyses based on study

  5. Treatment of complex regional pain syndrome

    PubMed Central

    Resmini, Giuseppina; Ratti, Chiara; Canton, Gianluca; Murena, Luigi; Moretti, Antimo; Iolascon, Giovanni

    2015-01-01

    Summary Complex Regional Pain Syndrome (CRPS) is a multifactorial and disabling disorder with complex etiology and pathogenesis. Goals of therapy in CRPS should be pain relief, functional restoration, and psychological stabilization, but early interventions are needed in order to achieve these objectives. Several drugs have been used to reduce pain and to improve functional status in CRPS, despite the lack of scientific evidence supporting their use in this scenario. They include anti-inflammatory drugs, analgesics, anesthetics, anticonvulsants, antidepressants, oral muscle relaxants, corticosteroids, calcitonin, bisphosphonates, calcium channel blockers and topical agents. NSAIDs showed no value in treating CRPS. Glucocorticoids are the only anti-inflammatory drugs for which there is direct clinical trial evidence in early stage of CRPS. Opioids are a reasonable second or third-line treatment option, but tolerance and long term toxicity are unresolved issues. The use of anticonvulsants and tricyclic antidepressants has not been well investigated for pain management in CRPS. During the last years, bisphosphonates have been the mostly studied pharmacologic agents in CRPS treatment and there are good evidence to support their use in this condition. Recently, the efficacy of intravenous (IV) administration of neridronate has been reported in a randomized controlled trial. Significant improvements in VAS score and other indices of pain and quality of life in patients who received four 100 mg IV doses of neridronate versus placebo were reported. These findings were confirmed in the open-extension phase of the study, when patients formerly enrolled in the placebo group received neridronate at the same dosage, and these results were maintained at 1 year follow-up. The current literature concerning sympathetic blocks and sympathectomy techniques lacks evidence of efficacy. Low evidence was recorded for a free radical scavenger, dimethylsulphoxide (DMSO) cream (50%). The

  6. Treatment of complex regional pain syndrome.

    PubMed

    Resmini, Giuseppina; Ratti, Chiara; Canton, Gianluca; Murena, Luigi; Moretti, Antimo; Iolascon, Giovanni

    2015-01-01

    Complex Regional Pain Syndrome (CRPS) is a multifactorial and disabling disorder with complex etiology and pathogenesis. Goals of therapy in CRPS should be pain relief, functional restoration, and psychological stabilization, but early interventions are needed in order to achieve these objectives. Several drugs have been used to reduce pain and to improve functional status in CRPS, despite the lack of scientific evidence supporting their use in this scenario. They include anti-inflammatory drugs, analgesics, anesthetics, anticonvulsants, antidepressants, oral muscle relaxants, corticosteroids, calcitonin, bisphosphonates, calcium channel blockers and topical agents. NSAIDs showed no value in treating CRPS. Glucocorticoids are the only anti-inflammatory drugs for which there is direct clinical trial evidence in early stage of CRPS. Opioids are a reasonable second or third-line treatment option, but tolerance and long term toxicity are unresolved issues. The use of anticonvulsants and tricyclic antidepressants has not been well investigated for pain management in CRPS. During the last years, bisphosphonates have been the mostly studied pharmacologic agents in CRPS treatment and there are good evidence to support their use in this condition. Recently, the efficacy of intravenous (IV) administration of neridronate has been reported in a randomized controlled trial. Significant improvements in VAS score and other indices of pain and quality of life in patients who received four 100 mg IV doses of neridronate versus placebo were reported. These findings were confirmed in the open-extension phase of the study, when patients formerly enrolled in the placebo group received neridronate at the same dosage, and these results were maintained at 1 year follow-up. The current literature concerning sympathetic blocks and sympathectomy techniques lacks evidence of efficacy. Low evidence was recorded for a free radical scavenger, dimethylsulphoxide (DMSO) cream (50%). The same level

  7. Congenital myasthenic syndromes: pathogenesis, diagnosis, and treatment

    PubMed Central

    Engel, Andrew G.; Shen, Xin-Ming; Selcen, Duygu; Sine, Steven M.

    2015-01-01

    The congenital myasthenic syndromes are diverse disorders linked by abnormal signal transmission at the motor endplate that stem from defects in single or multiple proteins. Multiple endplate proteins are affected by mutations of single enzymes required for protein glycosylation, and deletion of PREPL exerts its effect by activating adaptor protein 1. Finally, neuromuscular transmission is also impaired in some congenital myopathies. The specific diagnosis of some syndromes is facilitated by clinical clues pointing to a disease gene. In absence of such clues, exome sequencing is a useful tool for finding the disease gene. Deeper understanding of disease mechanisms come from structural and in vitro electrophysiologic studies of the patient endplate, and from engineering the mutant and wild-type gene into a suitable expression system that can be interrogated by appropriate electrophysiologic and biochemical studies. Most CMS are treatable. Importantly, however, some medication beneficial in one syndrome can be detrimental in another. PMID:25792100

  8. Alien limb syndrome secondary to multimodal treatment of recurrent oligodendroglioma.

    PubMed

    Gallant, Rachel E; Bonney, Phillip A; Sughrue, Michael E; Bharucha, Kersi J; Battiste, James D

    2015-10-01

    We present a 41-year-old man who experienced alien limb syndrome as a complication of treatment for recurrent Grade III oligodendroglioma of the right parietal lobe. Alien limb syndrome is a rare phenomenon in which a limb performs involuntary actions and the affected individual feels a sense of estrangement towards the limb. It occurs most commonly as a result of corticobasal syndrome, though a variety of other etiologies have been reported. It is rarely associated with focal lesions, such as stroke or tumors. PMID:26094559

  9. Surgical treatment of posterior nutcracker syndrome presented with hyperaldosteronism.

    PubMed

    Deser, Serkan Burc; Onem, Kadir; Demirag, Mustafa Kemal; Buyukalpelli, Recep

    2016-05-01

    Posterior nutcracker syndrome is caused by the compression of left renal vein between the abdominal aorta and the vertebral body. Most seen symptoms are haematuria, left flank pain, abdominal pain and varicocele. The nutcracker syndrome may lead to left renal vein thrombosis due to blood congestion within compression of the vessel. Both endovascular and open surgical interventions can relieve symptoms; however, traditional surgical repair is still considered as the gold standard. Here, we present the surgical treatment of a 36-year old female with complaints of hypertension, hyperaldosteronism and diagnosed with posterior nutcracker syndrome. PMID:26892192

  10. PFAPA syndrome: a review on treatment and outcome.

    PubMed

    Vanoni, Federica; Theodoropoulou, Katerina; Hofer, Michaël

    2016-01-01

    The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) is the most common cause of periodic fever in childhood. The current pharmacological treatment includes corticosteroids, which usually are efficacious in the management of fever episodes, colchicine, for the prophylaxis of febrile episodes, and other medication for which efficacy has not been proven so far. Tonsillectomy is an option for selected patients. Usually PFAPA syndrome resolves during adolescence, but there is increasing evidence that this condition may persist into adulthood. PMID:27349388

  11. Immune reconstitution inflammatory syndrome during treatment of Whipple's disease.

    PubMed

    Vayssade, Marielle; Tournadre, Anne; D'Incan, Michel; Soubrier, Martin; Dubost, Jean-Jacques

    2015-03-01

    Immune reconstitution inflammatory syndrome is a rare complication of the treatment of Whipple's disease. Here, we report the case of a 65-year-old man treated for Whipple's disease affecting the joints, with positive Tropheryma whipplei PCR in CSF, who developed fever and nodular eruption on the trunk, arms and face in association with biological inflammatory syndrome 10 days after initiation of antimicrobial treatment. Skin manifestations and the patient's general condition improved on corticosteroids (0.5mg/kg prednisone), but as steroids were gradually tapered, new nodules appeared below a prednisone dose of 10-15mg. One year after starting treatment, lumbar puncture showed asymptomatic meningitis with negative T. whipplei PCR results which had regressed spontaneously. Two years after the diagnosis, on prednisone 5mg daily and antimicrobial treatment, the patient had only transient, episodic nodular rash without fever or inflammatory syndrome. PMID:25553832

  12. Carotid Stump Syndrome: Pathophysiology and Endovascular Treatment Options

    SciTech Connect

    Lakshminarayan, Raghuram; Scott, Paul M.; Robinson, Graham J.; Ettles, Duncan F.

    2011-02-15

    Carotid stump syndrome is one of the recognised causes of recurrent ipsilateral cerebrovascular events after occlusion of the internal carotid artery. It is believed that microemboli arising from the stump of the occluded internal carotid artery or the ipsilateral external carotid artery can pass into the middle cerebral artery circulation as a result of patent external carotid-internal carotid anastomotic channels. Different pathophysiologic causes of this syndrome and endovascular options for treatment are discussed.

  13. Diagnosis and treatment of restless legs syndrome.

    PubMed

    Sales, Samantha; Sanghera, Manjit K; Klocko, David J; Stewart, R Malcolm

    2016-07-01

    Restless legs syndrome (RLS) is a disorder characterized by an irresistible urge to move the legs during rest, usually accompanied by uncomfortable sensations in the affected extremity or extremities. RLS can manifest at any age but prevalence increases with advancing age. This article describes the symptoms of RLS, associated comorbidities, and how to diagnose and manage RLS. PMID:27306327

  14. Atomoxetine Treatment of ADHD in Children with Comorbid Tourette Syndrome

    ERIC Educational Resources Information Center

    Spencer, Thomas J.; Sallee, F. Randy; Gilbert, Donald L.; Dunn, David W.; McCracken, James T.; Coffey, Barbara J.; Budman, Cathy L.; Ricardi, Randall K.; Leonard, Henrietta L.; Allen, Albert J.; Milton, Denai R.; Feldman, Peter D.; Kelsey, Douglas K.; Geller, Daniel A.; Linder, Steven L.; Lewis, Donald W.; Winner, Paul K.; Kurlan, Roger M.; Mintz, Mark

    2008-01-01

    Objective: This study examines changes in severity of tics and ADHD during atomoxetine treatment in ADHD patients with Tourette syndrome (TS). Method: Subjects (7-17 years old) with ADHD ("Diagnostic and Statistical Manual of Mental Disorders, DSM-IV") and TS were randomly assigned to double-blind treatment with placebo (n = 56) or atomoxetine…

  15. Treatment of eosinophilic cellulitis (Wells syndrome) - a systematic review.

    PubMed

    Räßler, F; Lukács, J; Elsner, P

    2016-09-01

    Eosinophilic cellulitis (Wells syndrome) is a rare inflammatory skin disease defined by erythematous, tender, sometimes urticarial plaques, possibly with vesicles and bullae, and granulomatous eosinophilic infiltrates in the dermis. Usually the disease has a benign course with spontaneous remission within a few weeks. Nevertheless, recurrences are quite frequent and may occur for several years. The objective of this study was to review the so far reported treatment options for Wells syndrome in a systematic manner. This systematic review is based on a search on Medline, Embase and Cochrane Central Register for English and German articles from 1970 to 2015. Advices on the treatment of Wells syndrome are limited predominately to case reports or to small case series. There are no randomized controlled trials, and control groups are missing. A variety of treatment options for Wells syndrome were reported including topical and systemic corticosteroids, antihistamines, cyclosporine, dapsone, azathioprine, griseofulvin, doxycycline, minocycline, antimalarial medications, oral tacrolimus/topical tacrolimus, sulfasalazine, interferon alpha and gamma, TNF alpha inhibitors, colchicine and PUVA therapy. As well-designed, randomized controlled trials are missing, no guidelines for the treatment of this disease can be given. Due to the small number of patients and the frequent misdiagnosis of this clinical entity, the aim of this systematic overview is to call attention to this rare condition and to help clinicians to diagnose and treat Wells syndrome effectively. Due to the good prognosis and tendency to resolve, systemic treatment should be limited to cases resistant to local therapy or with widespread lesions. PMID:27357601

  16. [Successful sunitinib treatment of a patient with Stauffer's syndrome].

    PubMed

    Küronya, Zsófia; Kovács, Eszter; Lahm, Erika; Géczi, Lajos

    2014-09-01

    Several potential biomarkers of response to targeted therapies are being evaluated in metastatic renal cell carcinoma (RCC) to improve drug development and to determine which patient may benefit the most from the different treatment options. Stauffer's syndrome is a paraneoplastic syndrome that presents with the elevation of hepatic enzymes without hepatic metastases in patients with renal cell carcinoma. Hereby we report the case of our patient who suffered from multiple peritoneal metastases of renal cell cancer accompanied by Stauffer's syndrome. During his course of disease, the change in the serum gamma-glutamyl transferase (GGT) and alkaline phosphatase (ALP) levels correlated well with the extent of metastatic spread. Hypertension, grade 2 hand-foot syndrome and hypothyreosis also developed in relation to the successful sunitinib treatment. These side effects are predictive biomarkers in patients responding well to sunitinib. As other potential causes of increased GGT and ALP were excluded, the elevation of these enzymes were attributed to Stauffer's syndrome. During treatment, magnetic resonance imaging (MRI) follow-up showed complete regression, while the serum GGT and ALP levels halved. In this case, besides the known biomarkers, changes in serum GGT and ALP levels correlated well with the regression of metastatic renal cell carcinoma. To our knowledge, this is the first case published in the medical literature to show GGT and ALP levels in Stauffer's syndrome as potential biomarkers. PMID:25260079

  17. Mucoadhesive polymers in the treatment of dry X syndrome.

    PubMed

    Partenhauser, Alexandra; Bernkop-Schnürch, Andreas

    2016-07-01

    Mucoadhesive polymers are an essential tool in the treatment of diseases where dry mucosal surfaces are involved. In this review, we focus on the application of mucoadhesive polymers in the context of dry eye, dry mouth, and dry vagina syndrome, collectively named 'dry X syndrome'. With a prolonged residence time on mucosal membranes, mucoadhesive materials are as targeted treatment option, with the mucosa as an intended site of action. Thus, mucoadhesive polymers are able to ease local irritation or itching, alleviate chewing difficulties, improve tear-film break-up time, and help to restore physiological conditions. Here, we discuss the different classes of mucoadhesive material and their performance in the treatment of dry X syndrome. PMID:26944445

  18. Targeting SREBPs for treatment of the metabolic syndrome.

    PubMed

    Soyal, Selma M; Nofziger, Charity; Dossena, Silvia; Paulmichl, Markus; Patsch, Wolfgang

    2015-06-01

    Over the past few decades, mortality resulting from cardiovascular disease (CVD) steadily decreased in western countries; however, in recent years, the decline has become offset by the increase in obesity. Obesity is strongly associated with the metabolic syndrome and its atherogenic dyslipidemia resulting from insulin resistance. While lifestyle treatment would be effective, drugs targeting individual risk factors are often required. Such treatment may result in polypharmacy. Novel approaches are directed towards the treatment of several risk factors with one drug. Studies in animal models and humans suggest a central role for sterol regulatory-element binding proteins (SREBPs) in the pathophysiology of the metabolic syndrome. Four recent studies targeting the maturation or transcriptional activities of SREBPs provide proof of concept for the efficacy of SREBP inhibition in this syndrome. PMID:26005080

  19. Cost of the treatment of myelodisplastic syndrome in Brazil

    PubMed Central

    Clark, Otávio; Faleiros, Enéas José de Matos

    2011-01-01

    Introduction Myelodysplastic syndrome is an incurable and rare hematological disease that affects the production of blood cells. One aim of treatment is to maintain the blood-cell count to near-normal levels. This is mainly achieved with hematopoietic- growth factors and transfusions. Our objective was to determine the cost of supportive treatment/care for patients with low and intermediate I risk myelodysplastic syndrome in respect to private healthcare plans in Brazil. Method We adapted the National Comprehensive Cancer Network treatment guidelines for intermediate risk myelodysplastic syndrome patients to the Brazilian reality, adopting a decision tree to explore treatment combinations. Then, we calculated the costs for each branch of the tree, according to national prices. We also estimated total costs for a cohort of 100 patients, distributed across treatment combinations according to the expected epidemiology. We assumed a horizon of one year of treatment. Results The mean cost of treatment for low and intermediate I risk myelodysplastic syndrome is US$ 42,758/patient/year. This cost can vary from US$ 24,282 to US$ 121,952, according to patient characteristics and the treatment used. Overall, patients that require immunotherapy with antithymocyte globulins are associated with the highest cost. Those that achieve disease stability solely with the use of erythropoietin were associated with the lowest cost. Conclusion In Brazil, treatment of low and intermediate I risk myelodysplastic syndrome is associated with a mean cost of the order of US$ 42,700/patient/year. New types of therapy have the potential to change this scenario if they can diminish the requirements for supportive care. PMID:23284240

  20. Boerhaave's syndrome: The importance of early diagnosis and treatment.

    PubMed Central

    Curci, J J; Horman, M J

    1976-01-01

    Boerhaave's syndrome, spontaneous esophageal rupture, is associated with a 70% survival with surgical intervention. Mortality and morbidity are increased in direct proportion to the time between diagnosis and appropriate surgical intervention. Sepsis, hypovolemia and shock are the predominant causes of morbidity and mortality in Boerhaave's syndrome. Two cases of Boerhaave's syndrome are presented which were diagnosed rapidly, and were managed surgically, resulting in survival of the patients. A review of the literature is also presented with emphasis on the clinical and roentgenologic methods of diagnosis of spontaneous esophageal rupture. Particular attention is given to the fact that early diagnosis and treatment will unquestionably reduce the morbidity of this syndrome. Images Fig. 1. Fig. 2. Fig. 3. PMID:1267496

  1. Fragile X syndrome. Molecular and clinical insights and treatment issues.

    PubMed Central

    Hagerman, R J

    1997-01-01

    The fragile X syndrome is the most common inherited cause of mental retardation that is known. The prevalence of mental retardation from this syndrome ranges from 1 in 1,250 to 1 in 4,000 in the general population, although the prevalence of female carriers has been reported to be as high as 1 in 259. The discovery of the FMR1 gene mutation in 1991 has simplified diagnosis, enhanced our understanding of the spectrum of involvement in the fragile X syndrome, and stimulated research regarding the normal function of the FMR1 protein in brain development. Advances have also occurred in the treatment of the fragile X syndrome, and psychopharmacologic and educational interventions are reviewed here. Images Figure 1. PMID:9109330

  2. Plant-derived therapeutics for the treatment of metabolic syndrome

    PubMed Central

    Graf, Brittany L; Raskin, Ilya; Cefalu, William T; Ribnicky, David M

    2011-01-01

    Metabolic syndrome is defined as a set of coexisting metabolic disorders that increase an individual’s likelihood of developing type 2 diabetes, cardiovascular disease and stroke. Medicinal plants, some of which have been used for thousands of years, serve as an excellent source of bioactive compounds for the treatment of metabolic syndrome because they contain a wide range of phytochemicals with diverse metabolic effects. In order for botanicals to be effectively used against metabolic syndrome, however, botanical preparations must be characterized and standardized through the identification of their active compounds and respective modes of action, followed by validation in controlled clinical trials with clearly defined endpoints. This review assesses examples of commonly known and partially characterized botanicals to describe specific considerations for the phytochemical, preclinical and clinical characterization of botanicals associated with metabolic syndrome. PMID:20872313

  3. Diagnosis and treatment of acute extremity compartment syndrome.

    PubMed

    von Keudell, Arvind G; Weaver, Michael J; Appleton, Paul T; Appelton, Paul T; Bae, Donald S; Dyer, George S M; Heng, Marilyn; Jupiter, Jesse B; Vrahas, Mark S

    2015-09-26

    Acute compartment syndrome of the extremities is well known, but diagnosis can be challenging. Ineffective treatment can have devastating consequences, such as permanent dysaesthesia, ischaemic contractures, muscle dysfunction, loss of limb, and even loss of life. Despite many studies, there is no consensus about the way in which acute extremity compartment syndromes should be diagnosed. Many surgeons suggest continuous monitoring of intracompartmental pressure for all patients who have high-risk extremity injuries, whereas others suggest aggressive surgical intervention if acute compartment syndrome is even suspected. Although surgical fasciotomy might reduce intracompartmental pressure, this procedure also carries the risk of long-term complications. In this paper in The Lancet Series about emergency surgery we summarise the available data on acute extremity compartment syndrome of the upper and lower extremities in adults and children, discuss the underlying pathophysiology, and propose a clinical guideline based on the available data. PMID:26460664

  4. Advances in the Treatment of Fragile X Syndrome

    PubMed Central

    Hagerman, Randi J.; Berry-Kravis, Elizabeth; Kaufmann, Walter E.; Ono, Michele Y.; Tartaglia, Nicole; Lachiewicz, Ave; Kronk, Rebecca; Delahunty, Carol; Hessl, David; Visootsak, Jeannie; Picker, Jonathan; Gane, Louise; Tranfaglia, Michael

    2010-01-01

    The FMR1 mutations can cause a variety of disabilities, including cognitive deficits, attention-deficit/hyperactivity disorder, autism, and other socioemotional problems, in individuals with the full mutation form (fragile X syndrome) and distinct difficulties, including primary ovarian insufficiency, neuropathy and the fragile X-associated tremor/ataxia syndrome, in some older premutation carriers. Therefore, multigenerational family involvement is commonly encountered when a proband is identified with a FMR1 mutation. Studies of metabotropic glutamate receptor 5 pathway antagonists in animal models of fragile X syndrome have demonstrated benefits in reducing seizures, improving behavior, and enhancing cognition. Trials of metabotropic glutamate receptor 5 antagonists are beginning with individuals with fragile X syndrome. Targeted treatments, medical and behavioral interventions, genetic counseling, and family supports are reviewed here. PMID:19117905

  5. Hepatorenal syndrome: Update on diagnosis and treatment.

    PubMed

    Baraldi, Olga; Valentini, Chiara; Donati, Gabriele; Comai, Giorgia; Cuna, Vania; Capelli, Irene; Angelini, Maria Laura; Moretti, Maria Ilaria; Angeletti, Andrea; Piscaglia, Fabio; La Manna, Gaetano

    2015-11-01

    Acute kidney injury (AKI) is a common complication in patients with end-stage liver disease and advanced cirrhosis regardless of the underlying cause. Hepatorenal syndrome (HRS), a functional form of kidney failure, is one of the many possible causes of AKI. HRS is potentially reversible but involves highly complex pathogenetic mechanisms and equally complex clinical and therapeutic management. Once HRS has developed, it has a very poor prognosis. This review focuses on the diagnostic approach to HRS and discusses the therapeutic protocols currently adopted in clinical practice. PMID:26558188

  6. Hepatorenal syndrome: Update on diagnosis and treatment

    PubMed Central

    Baraldi, Olga; Valentini, Chiara; Donati, Gabriele; Comai, Giorgia; Cuna, Vania; Capelli, Irene; Angelini, Maria Laura; Moretti, Maria Ilaria; Angeletti, Andrea; Piscaglia, Fabio; La Manna, Gaetano

    2015-01-01

    Acute kidney injury (AKI) is a common complication in patients with end-stage liver disease and advanced cirrhosis regardless of the underlying cause. Hepatorenal syndrome (HRS), a functional form of kidney failure, is one of the many possible causes of AKI. HRS is potentially reversible but involves highly complex pathogenetic mechanisms and equally complex clinical and therapeutic management. Once HRS has developed, it has a very poor prognosis. This review focuses on the diagnostic approach to HRS and discusses the therapeutic protocols currently adopted in clinical practice. PMID:26558188

  7. [Factors influencing psychotherapeutic treatment outcome of various syndromes].

    PubMed

    Hartmann, Sebastian; Zepf, Siegfried

    2004-12-01

    The authors investigated specific and unspecific factors influencing the psychotherapeutic treatment of various syndromes using a questionnaire which systematically replicated the Consumer Reports Study performed in the USA in 1994. The authors were particularly concerned with the degree to which certain psychotherapeutic methods - psychoanalysis, depth psychology-based psychotherapy and behavioral therapy - produced differing results following treatment of syndromes. Using cluster-analysis, two groups of syndromes could be distinguished: Patients with depressive symptoms, stress-related disorders and/or relationship problems (depression-group) and patients with anxiety disorders and/or eating-related disorders (anxiety-group). With the help of cart-analysis (Classification and Regression Trees) it was possible to identify factors influencing the improvement of symptoms. The method of treatment had not a specific effect on the improvement of symptoms. In both groups the most important predictor was the length of treatment. Furthermore in the depression group the sex of the patients and a possible restriction of the treatment by the health insurance companies influenced the treatment results and in the anxiety group the frequency of treatment and the age of the patients. PMID:15551189

  8. Successful Surgical Treatment for Thoracoabdominal Aortic Aneurysm with Leriche Syndrome

    PubMed Central

    Chong, Byung Kwon; Kim, Joon Bum

    2015-01-01

    Thoracoabdominal aortic aneurysm accompanied by Leriche syndrome is an extremely rare combination of aortic diseases, the surgical management of which has not been described to date. We report the successful treatment of one such case through open surgical repair of the thoracoabdominal aorta. PMID:25883898

  9. Treatment of the acquired von Willebrand syndrome.

    PubMed

    Budde, Ulrich; Scheppenheim, Sonja; Dittmer, Rita

    2015-12-01

    Acquired von Willebrand syndrome (aVWS) accounts for 22% of patients with abnormal von Willebrand factor. Most patients with known pathophysiological mechanisms suffer from cardiovascular, myeloproliferative and lymphoproliferative disorders. Less frequent associations are of autoimmune origin, due to hyperfibrinolysis, adsorption to tumor cells, reduced synthesis and prolonged circulation. The mechanisms leading to aVWS is hitherto not known in patients with liver and kidney diseases, drug use, glycogen storage disease, virus infections and at least 18 other disease entities. Diagnosis is complicated by the battery of tests needed, and their inherent rather low sensitivity and specificity for aVWS. Thus, even in acute bleeding situations it may take days until a firm diagnosis is settled and specific therapies can be initiated. The main aim is to shed more light onto this, compared with inherited von Willebrand disease, rare disease which affects at least 2-3% of the older population. PMID:26577336

  10. [Current approaches to the diagnosis and treatment of irritable bowel syndrome].

    PubMed

    Kharchenko, N V; Chernenko, V V

    2001-01-01

    Modern definition, classifications, diagnosis and treatment of irritable bowel syndrome (IBS) are presented together with results of treatment of 30 patients with irritable bowel syndrome with the new selective calcium-channel blocker dicetel. PMID:11881362

  11. Post-treatment lyme syndrome and central sensitization.

    PubMed

    Batheja, Shweta; Nields, Jenifer A; Landa, Alla; Fallon, Brian A

    2013-01-01

    Central sensitization is a process that links a variety of chronic pain disorders that are characterized by hypersensitivity to noxious stimuli and pain in response to non-noxious stimuli. Among these disorders, treatments that act centrally may have greater efficacy than treatments acting peripherally. Because many individuals with post-treatment Lyme syndrome (PTLS) have a similar symptom cluster, central sensitization may be a process mediating or exacerbating their sensory processing. This article reviews central sensitization, reports new data on sensory hyperarousal in PTLS, explores the potential role of central sensitization in symptom chronicity, and suggests new directions for neurophysiologic and treatment research. PMID:24026711

  12. Irritable bowel syndrome: a concise review of current treatment concepts.

    PubMed

    Wall, Geoffrey C; Bryant, Ginelle A; Bottenberg, Michelle M; Maki, Erik D; Miesner, Andrew R

    2014-07-21

    Irritable bowel syndrome (IBS) is one of the most common gastrointestinal disorders causing patients to seek medical treatment. It is relatively resource intensive and the source of significant morbidity. Recent insights into the pathophysiology and treatment of IBS has given clinicians more options than ever to contend with this disorder. The purpose of our paper is to review older, "classic" treatments for IBS as well as newer agents and "alternative" therapies. We discuss the evidence base of these drugs and provide context to help develop appropriate treatment plans for IBS patients. PMID:25083054

  13. Irritable bowel syndrome: A concise review of current treatment concepts

    PubMed Central

    Wall, Geoffrey C; Bryant, Ginelle A; Bottenberg, Michelle M; Maki, Erik D; Miesner, Andrew R

    2014-01-01

    Irritable bowel syndrome (IBS) is one of the most common gastrointestinal disorders causing patients to seek medical treatment. It is relatively resource intensive and the source of significant morbidity. Recent insights into the pathophysiology and treatment of IBS has given clinicians more options than ever to contend with this disorder. The purpose of our paper is to review older, “classic” treatments for IBS as well as newer agents and “alternative” therapies. We discuss the evidence base of these drugs and provide context to help develop appropriate treatment plans for IBS patients. PMID:25083054

  14. Treatment timing and multidisciplinary approach in Apert syndrome

    PubMed Central

    Fadda, Maria Teresa; Ierardo, Gaetano; Ladniak, Barbara; Di Giorgio, Gianni; Caporlingua, Alessandro; Raponi, Ingrid; Silvestri, Alessandro

    2015-01-01

    Summary Apert syndrome is a rare congenital disorder characterized by craniosynostosis, midface hypoplasia and symmetric syndactyly of hands and feet. Abnormalities associated with Apert syndrome include premature fusion of coronal sutures system (coronal sutures and less frequently lambdoid suture) resulting in brachiturricephalic dismorphism and impaired skull base growth. After this brief explanation it is clear that these anatomical abnormalities may have a negative impact on the ability to perform essential functions. Due to the complexity of the syndrome a multidisciplinary (respiratory, cerebral, maxillo-mandibular, dental, ophthalmic and orthopaedic) approach is necessary in treating the psychological, aesthetic and functional issues. The aim of this paper is to analyse the different functional issues and surgical methods trying to enhance results through a treatment plan which includes different specialities involved in Apert syndrome treatment. Reduced intellectual capacity is associated to the high number of general anaesthesia the small patients are subject to. Therefore the diagnostic and therapeutic treatment plan in these patients has established integrated and tailored surgical procedures based on the patients’ age in order to reduce the number of general anaesthesia, thus simplifying therapy for both Apert patients and their family members. PMID:26330906

  15. [Hurler syndrome: early diagnosis and treatment].

    PubMed

    Leroux, S; Muller, J-B; Boutaric, E; Busnel, A; Lemouel, F; Andro-Garçon, M; Neven, B; Valayannopoulos, V; Vinceslas, C

    2014-05-01

    Hurler syndrome, the most severe form of mucopolysaccharidosis type I (MPS I), is a rare lysosomal storage disease. The overall incidence of MPS I is 0.99-1.99/100,000 live births. Accumulation of glycosaminoglycans causes the progressive dysfunction of multiple organs. We report the case of a 3-week-old newborn who was hospitalized in the Neonatal Intensive Care Unit for feeding problems. Coarse facial features and gingival hypertrophy, associated with axial hypotonia, upper airway obstruction, and moderate hepatomegaly, led to the early diagnosis of MPS I at 3 weeks of age and was confirmed by an abnormally elevated amount of dermatan and heparan sulphate in the urine and complete deficiency of alpha-L-iduronidase lysosomal enzyme activity. The child was homozygous for the p.W402X mutation, located on chromosome 4p16.3 of the alpha-L-iduronidase (IDUA) gene. The clinical condition gradually deteriorated until the age of 4 months, with thoracic and lumbar dysostoses, glaucoma, cerebral ventricular dilatation and cervical spinal stenosis, dilated cardiomyopathy, and umbilical hernia. Early diagnosis allowed enzyme replacement therapy (iaronidase, Aldurazyme(®), Genzyme) started at the age of 5 months, which provided stabilization of the heart disease, significant regression of rhinologic symptoms, and regression of hepatomegaly. Cord blood hematopoietic stem cell transplantation was performed at 11 months of age, allowing optimal preservation of cognitive development. PMID:24698225

  16. Cardio-renal syndrome type 5: epidemiology, pathophysiology, and treatment.

    PubMed

    Soni, Sachin S; Ronco, Claudio; Pophale, Rupesh; Bhansali, Ashish S; Nagarik, Amit P; Barnela, Shriganesh R; Saboo, Sonali S; Raman, Anuradha

    2012-01-01

    The cardio-renal syndromes (CRS) recently were defined systematically as disorders of the heart or kidney whereby dysfunction of one organ leads to dysfunction of another. Five types of CRS are defined. The first four types describe acute or chronic cardio-renal or renocardiac syndromes. Type 5 CRS refers to secondary cardio-renal syndrome or cardio-renal involvement in systemic conditions. It is a clinical and pathophysiological entity to describe the concomitant presence of renal and cardiovascular dysfunction. Type 5 CRS can be acute or chronic and it does not strictly satisfy the definition of CRS. However, it encompasses many conditions in which combined heart and kidney dysfunction is observed. Because this entity has been described only recently there is limited information about the epidemiology, clinical course, and treatment of this condition. PMID:22365162

  17. [Postural orthostatic tachycardia syndrome (POTS)--pathophysiology, diagnostics, and treatment].

    PubMed

    Rek, Marta; Kaczmarek, Krzysztof; Cygankiewicz, Iwona; Wranicz, Jerzy K; Ptaszyński, Paweł

    2014-01-01

    Postural orthostatic tachycardia syndrome (POTS) is one of the most common presentation of orthostatic intolerance. The syndrome is described as a multifactorial affliction. Main symptoms consist of persistent orthostatic tachycardia (heart rate increase at least 30 beats/min, lasting at least 10 min after assumic vertical position) with high noradrenalin serum concentration (measured in stand-up position). Additionally patients with POTS tend to have lover total blood volume. POTS is generally classified into dysatonomia disorders Symptoms in patients affected with POTS are chronic. The syndrome occurs predominantly in young women (approximately 80%). Due to complexity and variable intensity of symptoms POTS can severely impair daily activity and quality of life in otherwise healthy people. The correct diagnosis and identification of potential pathophysiological mechanisms of POTS is necessary before treatment administration. Adequate therapy can significantly reduce symptoms giving the patients a chance for a normal life. PMID:25546918

  18. [Treatment for dementia in parkinsonian syndromes. Efficacy of cholinesterase inhibitors].

    PubMed

    Liepelt, I; Maetzler, W; Blaicher, H-P; Gasser, T; Berg, D

    2008-01-01

    In parkinsonian syndromes dementia frequently occurs in the disease progress. The cholinergic system has been proposed as playing a key role in cognitive disturbances. Therefore the application of cholinesterase inhibitors (ChEI) is also hotly argued for dementia associated with parkinsonian syndromes. This review focuses on the specific symptoms of dementia in Parkinson's disease (PDD), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). The effect of cholinergic treatment on cognition and behaviour is reported and critically discussed. There is evidence that medication with some ChEIs reduces cognitive disturbances and to a lesser extent improves activities of daily living in PDD. Behavioural symptoms also seem to be positively influenced by treatment with ChEIs in both PDD and DLB. The effect of treatment with cholinesterase inhibitors in PSP and CBD warrants more carefully designed studies including sufficient numbers of patients. PMID:17687535

  19. [PECULIARITIES OF TREATMENT VERTEBROGENIC PAIN SYNDROMES].

    PubMed

    Klymenko, O; Belska, I

    2015-01-01

    The results of studying the pathogenetic features of back pain at vertebrogenic of Spine, and disorders of life of patients in relation to it. Application of therapy with the standard treatment and the complex medicine of the vitamins B. PMID:27089720

  20. [TREATMENT DILEMMAS IN BEHÇET'S SYNDROME].

    PubMed

    Zeller, Lior; Ling, Edoard; Abu-Shakra, Mahmoud

    2016-02-01

    Behçet's disease is an inflammatory systemic disorder, characterized by a relapsing and remitting course, it manifests with oral and genital ulcerations, skin lesions, uveitis, vasculitis, central nervous system and gastrointestinal involvement. The main histopathological finding is widespread vasculitis of the arteries and veins. Therapy is variable and depends largely on the severity of the disease and organ involvement. There is common practice to treat with anticoagulation in patients suffering from vessel thrombosis, but there are no control trials to support this tendency. Anticoagulation treatment can cause major bleeding events in patients suffering from aneurysms. In this case report we describe a treatment dilemma in a patient suffering from deep vein thrombosis and pulmonary aneurysms. PMID:27215119

  1. Ibrutinib and Azacitidine for Treatment of Higher Risk Myelodysplastic Syndrome

    ClinicalTrials.gov

    2016-04-26

    Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndrome; Previously Treated Myelodysplastic Syndrome; Refractory Anemia With Excess Blasts in Transformation; Secondary Myelodysplastic Syndrome

  2. Coagulation syndrome: Delayed perforation after colorectal endoscopic treatments

    PubMed Central

    Hirasawa, Kingo; Sato, Chiko; Makazu, Makomo; Kaneko, Hiroaki; Kobayashi, Ryosuke; Kokawa, Atsushi; Maeda, Shin

    2015-01-01

    Various procedure-related adverse events related to colonoscopic treatment have been reported. Previous studies on the complications of colonoscopic treatment have focused primarily on perforation or bleeding. Coagulation syndrome (CS), which is synonymous with transmural burn syndrome following endoscopic treatment, is another typical adverse event. CS is the result of electrocoagulation injury to the bowel wall that induces a transmural burn and localized peritonitis resulting in serosal inflammation. CS occurs after polypectomy, endoscopic mucosal resection (EMR), and even endoscopic submucosal dissection (ESD). The occurrence of CS after polypectomy or EMR varies according previous reports; most report an occurrence rate around 1%. However, artificial ulcers after ESD are largely theoretical, and CS following ESD was reported in about 9% of cases, which is higher than that for CS after polypectomy or EMR. Most cases of post-polypectomy syndrome (PPS) have an excellent prognosis, and they are managed conservatively with medical therapy. PPS rarely develops into delayed perforation. Delayed perforation is a severe adverse event that often requires emergency surgery. Since few studies have reported on CS and delayed perforation associated with CS, we focused on CS after colonoscopic treatments in this review. Clinicians should consider delayed perforation in CS patients. PMID:26380051

  3. Treatment of Cushing's Syndrome: An Endocrine Society Clinical Practice Guideline

    PubMed Central

    Nieman, Lynnette K.; Biller, Beverly M. K.; Findling, James W.; Murad, M. Hassan; Newell-Price, John; Savage, Martin O.; Tabarin, Antoine

    2015-01-01

    Objective: The objective is to formulate clinical practice guidelines for treating Cushing's syndrome. Participants: Participants include an Endocrine Society-appointed Task Force of experts, a methodologist, and a medical writer. The European Society for Endocrinology co-sponsored the guideline. Evidence: The Task Force used the Grading of Recommendations, Assessment, Development, and Evaluation system to describe the strength of recommendations and the quality of evidence. The Task Force commissioned three systematic reviews and used the best available evidence from other published systematic reviews and individual studies. Consensus Process: The Task Force achieved consensus through one group meeting, several conference calls, and numerous e-mail communications. Committees and members of The Endocrine Society and the European Society of Endocrinology reviewed and commented on preliminary drafts of these guidelines. Conclusions: Treatment of Cushing's syndrome is essential to reduce mortality and associated comorbidities. Effective treatment includes the normalization of cortisol levels or action. It also includes the normalization of comorbidities via directly treating the cause of Cushing's syndrome and by adjunctive treatments (eg, antihypertensives). Surgical resection of the causal lesion(s) is generally the first-line approach. The choice of second-line treatments, including medication, bilateral adrenalectomy, and radiation therapy (for corticotrope tumors), must be individualized to each patient. PMID:26222757

  4. History of Nephrotic Syndrome and Evolution of its Treatment

    PubMed Central

    Pal, Abhijeet; Kaskel, Frederick

    2016-01-01

    The recognition, evaluation, and early treatment of nephrotic syndrome in infants and children originate from physicians dating back to Hippocrates. It took nearly another 1000 years before the condition was described for its massive edema requiring treatment with herbs and other remedies. A rich history of observations and interpretations followed over the course of centuries until the recognition of the combination of clinical findings of foamy urine and swelling of the body, and measurements of urinary protein and blood analyses showed the phenotypic characteristics of the syndrome that were eventually linked to the early anatomic descriptions from first kidney autopsies and then renal biopsy analyses. Coincident with these findings were a series of treatment modalities involving the use of natural compounds to a host of immunosuppressive agents that are applied today. With the advent of molecular and precision medicine, the field is poised to make major advances in our understanding and effective treatment of nephrotic syndrome and prevent its long-term sequelae. PMID:27303658

  5. Coagulation syndrome: Delayed perforation after colorectal endoscopic treatments.

    PubMed

    Hirasawa, Kingo; Sato, Chiko; Makazu, Makomo; Kaneko, Hiroaki; Kobayashi, Ryosuke; Kokawa, Atsushi; Maeda, Shin

    2015-09-10

    Various procedure-related adverse events related to colonoscopic treatment have been reported. Previous studies on the complications of colonoscopic treatment have focused primarily on perforation or bleeding. Coagulation syndrome (CS), which is synonymous with transmural burn syndrome following endoscopic treatment, is another typical adverse event. CS is the result of electrocoagulation injury to the bowel wall that induces a transmural burn and localized peritonitis resulting in serosal inflammation. CS occurs after polypectomy, endoscopic mucosal resection (EMR), and even endoscopic submucosal dissection (ESD). The occurrence of CS after polypectomy or EMR varies according previous reports; most report an occurrence rate around 1%. However, artificial ulcers after ESD are largely theoretical, and CS following ESD was reported in about 9% of cases, which is higher than that for CS after polypectomy or EMR. Most cases of post-polypectomy syndrome (PPS) have an excellent prognosis, and they are managed conservatively with medical therapy. PPS rarely develops into delayed perforation. Delayed perforation is a severe adverse event that often requires emergency surgery. Since few studies have reported on CS and delayed perforation associated with CS, we focused on CS after colonoscopic treatments in this review. Clinicians should consider delayed perforation in CS patients. PMID:26380051

  6. Treatment of the irritable bowel syndrome.

    PubMed

    Friedman, G

    1991-06-01

    Individualization of treatment for patients with IBS is predicated on a thorough analysis of the patient's symptoms, consideration of the reasons for seeking health care, evaluation of symptom-precipitating factors, elimination of confounding features, and the absolute knowledge of the absence of organic illness. Collecting and codifying appropriate historical data allow the physician to educate the patient with respect to the origin of his symptoms, and to enlist the patient as a partner in his future health care. There is no single, universally accepted therapeutic agent available for the treatment of the IBS patient. As a result, treatment is directed at reducing the frequency and intensity of triggering factors as well as ameliorating the symptoms when they arise. Symptoms evoked by psychologic factors may be effectively reduced by psychotherapy or hypnotherapy. Situational anxiety may be treated for brief periods by using antianxiety agents such as diazepam, chlordiazepoxide, buspirone, or similar agents. Depressive reactions may be reduced with suitable doses of antidepressant agents such as amitriptyline. Smooth muscle hyperreactivity may be dulled with small amounts of selected anticholinergics, which are usually most effective in reducing meal-induced discomfort. Peppermint oil may be of additional benefit. Gas-related symptoms require elimination of contributory dietary factors, such as lactose-containing foods, sorbitol, or fructose, as well as certain oligosaccharides. Simethecone, charcoal, or beanase may be helpful. Functional constipation is best treated with graded doses of insoluble or soluble fiber. Diarrheal episodes may be reduced with either loperamide or diphenoxylate. Careful, continued follow-up assessment of therapeutic endeavors, a sincere interest in the patient's concerns, and surveillance for intercurrent organic illness are the cornerstones of complete ongoing care. PMID:2066156

  7. Polycystic ovary syndrome: update on diagnosis and treatment.

    PubMed

    Setji, Tracy L; Brown, Ann J

    2014-10-01

    Polycystic ovary syndrome is now a well-recognized condition affecting 6%-25% of reproductive-aged women, depending on the definition. Over the past 3 decades, research has launched it from relative medical obscurity to a condition increasingly recognized as common in internal medicine practices. It affects multiple systems, and requires a comprehensive perspective on health care for effective treatment. Metabolic derangements and associated complications include insulin resistance and diabetes, hyperlipidemia, hypertension, fatty liver, metabolic syndrome, and sleep apnea. Reproductive complications include oligo-/amenorrhea, sub-fertility, endometrial hyperplasia, and cancer. Associated psychosocial concerns include depression and disordered eating. Additionally, cosmetic issues include hirsutism, androgenic alopecia, and acne. This review organizes this multi-system approach around the mnemonic "MY PCOS" and discusses evaluation and treatment options for the reproductive, cosmetic, and metabolic complications of this condition. PMID:24859638

  8. The Dynamic Treatment of the Low Back Strain Syndrome

    PubMed Central

    Woodhead, B. H.; Fowler, J. R.

    1964-01-01

    The modalities at present in use for the treatment of soft-tissue injuries responsible for the low back strain syndrome are predominantly passive, yet similar soft-tissue injuries elsewhere in the body receive and respond to an early program of active movement. To assess the feasibility of such an approach to the low back strain syndrome, a program of therapeutic exercises with and without the addition of heat and massage was employed for the treatment of this condition. Exercises alone were found to be as effective as a program of “heat, massage and exercises” in resolving the presenting complaints. At the same time this dynamic approach served to reduce the undesirable effects, both local and general, of the enforced inactivity of the patient. PMID:14145468

  9. Burning mouth syndrome: a review on diagnosis and treatment.

    PubMed

    Coculescu, E C; Radu, A; Coculescu, B I

    2014-01-01

    Burning mouth syndrome (BMS) is defined as a chronic pain condition characterized by a burning sensation in the clinically healthy oral mucosa. It is difficult to diagnose BMS because there is a discrepancy between the severity, extensive objective pain felt by the patient and the absence of any clinical changes of the oral mucosa. This review presents some aspects of BMS, including its clinical diagnosis, classification, differential diagnosis, general treatment, evolution and prognosis. PMID:25713611

  10. Burning mouth syndrome: a review on diagnosis and treatment

    PubMed Central

    Coculescu, EC; Radu, A; Coculescu, BI

    2014-01-01

    Burning mouth syndrome (BMS) is defined as a chronic pain condition characterized by a burning sensation in the clinically healthy oral mucosa. It is difficult to diagnose BMS because there is a discrepancy between the severity, extensive objective pain felt by the patient and the absence of any clinical changes of the oral mucosa. This review presents some aspects of BMS, including its clinical diagnosis, classification, differential diagnosis, general treatment, evolution and prognosis. PMID:25713611

  11. Treatment with cimetidine of atypical fasciitis panniculitis syndrome.

    PubMed Central

    Naschitz, J E; Yeshurun, D; Rosner, I; Abrahamson, J E; Misselevitch, I; Boss, J H

    1990-01-01

    Three patients presented with septal fasciitis and panniculitis, associated with clinical and laboratory features which precluded straight-forward classification into eosinophilic fasciitis, localised scleroderma, or lupus erythematosus profundus. Treatment with cimetidine caused the remission of cutaneous manifestations and the extracutaneous abnormalities, such as nailfold capillary disturbances and the presence of antithyroid antibodies, improved. It is concluded that features of eosinophilic fasciitis or localised scleroderma and certain additional atypical elements should be categorised as atypical fasciitis-panniculitis syndrome. Images PMID:2241270

  12. [Pharmaceutical treatment of irritable bowel syndrome].

    PubMed

    Inoue, M

    1992-11-01

    In the treatment of IBS best results could be obtained by implementing a comprehensive program for the patients. This might include a through examination, an explanation of the condition to the patients, psychologic managements, and correction of any bad habits, as well as drug therapy. The aim of drug therapy of IBS is the relief of the symptoms: such as abdominal pain, disturbed bowel function, anxiety or depression. As there is no drug which is effective in relieving the entire range of symptoms, drug should be chosen according to specific symptoms. Tranquilizers and antispasmodics may be the most commonly used drugs, however their efficacy is limited. To postprandial pain antispasmodics or trimebutine are most effective when prescribed before meal. Antidepressant are beneficial for the depressive state. Bulking agents are preferable mainly in relieving constipation, and loperamide is effective in treating diarrhea. PMID:1363124

  13. [Diagnosis and treatment of 'body packer' syndrome].

    PubMed

    Henebiens, M; van Geloven, A A W; Gouma, D J

    2007-08-25

    A body packer is someone who carries drugs such as heroine or cocaine, packed in rubber or plastic, in his/her body in order to smuggle them. These people can present with symptoms that vary from mild abdominal complaints to respiratory insufficiency and even death. Physical examination and additional radiology tests are helpful for the diagnosis. Any packages can usually be seen on a plain abdominal X-ray. Detailed information on the number of drug packages, their exact location in the gastrointestinal tract and complications, such as small intestine obstruction or perforation, can be derived from a CT scan. In most patients conservative treatment suffices. Surgery for body packing, i.e. the removal of the packages by gastrotomy or enterotomy, is often followed by serious complications related to contamination of the peritoneum that frequently occurs. PMID:17902559

  14. Intraoperative Floppy Iris Syndrome: Pathophysiology, Prevention, and Treatment

    PubMed Central

    Flach, Allan J.

    2009-01-01

    Purpose: To extend upon previous reports, observations, and discussions of intraoperative floppy iris syndrome (IFIS) with the goal of providing new insight into the syndrome’s pathophysiology, prevention, and treatment. Methods: Following a review of IFIS and its relationship to autonomic pharmacology, evidence for anatomic changes following exposure of humans and other animals to autonomic drugs is described. The clinical implications for these findings are discussed as they relate to the treatment and prevention of this syndrome. Results: IFIS has been associated with the use of adrenergic antagonists even after they have been discontinued years prior to surgery. Some investigators believe that this persistence of IFIS reflects anatomic structural change. Evidence from laboratory experiments and human clinical studies using topically applied and systemic autonomic drugs supports the possibility of anatomic changes coexisting with IFIS observed during cataract surgery. Conclusions: IFIS is a relatively rare syndrome, often associated with the use of systemic α-blockers and conditions that influence dilator muscle tone. Laboratory and clinical evidence supports the possibility of anatomic changes following the use of autonomic drugs. The persistence of IFIS years after cessation of treatment with α-blockers suggests that the potential risks of discontinuing these drugs prior to cataract surgery outweigh potential benefits. PMID:20126500

  15. Treatment of carcinoid syndrome with recombinant interferon alpha-2a.

    PubMed

    Di Bartolomeo, M; Bajetta, E; Zilembo, N; de Braud, F; Di Leo, A; Verusio, C; D'Aprile, M; Scanni, A; Barduagni, M; Barduagni A [corrected to Barduagni, M

    1993-01-01

    The prognosis and the quality of life of patients with carcinoid tumors is related either to symptoms from the substances secreted or to progressive tumor growth. Medical treatment with cytotoxic agents is of marginal value for increasing life expectancy and reducing clinical symptoms. Recent studies with interferon have shown interesting results. In the present investigation, 22 patients with carcinoid tumors and syndrome were treated with recombinant interferon alpha-2a (r-IFN alpha-2a) at the dose of 6 x 10(6) IU intramuscularly daily for 8 weeks and three times weekly thereafter. The primary tumor was localized in the foregut (n = 11), midgut (n = 7), hindgut (n = 1), and unknown site (n = 3). Most cases had liver metastasis. Seventeen patients had elevated 5-hydroxyindoloacetic acid (5-HIAA) excretion and 5 had flushing and/or diarrhea as the only clinical manifestation. Six cases presented a complete syndrome (flushing, diarrhea and 5-HIAA excretion). Control of symptoms was obtained in 80% and a 5-HIAA level reduction in 58% of the patients. The interferon treatment was more effective for control of the carcinoid syndrome than for control of tumor growth. The treatment was well tolerated and fever, myalgia, anorexia and fatigue were the most frequent side-effects. PMID:7686766

  16. Polycystic ovary syndrome: a review for dermatologists: Part II. Treatment.

    PubMed

    Buzney, Elizabeth; Sheu, Johanna; Buzney, Catherine; Reynolds, Rachel V

    2014-11-01

    Dermatologists are in a key position to treat the manifestations of polycystic ovary syndrome (PCOS). The management of PCOS should be tailored to each woman's specific goals, reproductive interests, and particular constellation of symptoms. Therefore, a multidisciplinary approach is recommended. In part II of this continuing medical education article, we present the available safety and efficacy data regarding treatments for women with acne, hirsutism, and androgenetic alopecia. Therapies discussed include lifestyle modification, topical therapies, combined oral contraceptives, antiandrogen agents, and insulin-sensitizing drugs. Treatment recommendations are made based on the current available evidence. PMID:25437978

  17. Filgrastim for the treatment of hematopoietic acute radiation syndrome.

    PubMed

    Farese, A M; MacVittie, T J

    2015-09-01

    The U.S. Food and Drug Administration (FDA) recently approved Neupogen(®) (filgrastim) for the treatment of patients with radiation-induced myelosuppression following a radiological/nuclear incident. It is the first medical countermeasure currently approved by the FDA for this indication under the criteria of the FDA "animal rule". This article summarizes the consequences of high-dose radiation exposure, a description of the hematopoietic acute radiation syndrome (H-ARS), the use of hematopoietic growth factors in radiation accident victims and current available treatments for H-ARS with an emphasis on the use of Neupogen in this scenario. PMID:26488033

  18. Serotonin dysregulation in Fragile X Syndrome: implications for treatment

    PubMed Central

    Hanson, Alicia C; Hagerman, Randi J

    2014-01-01

    Summary Fragile X Syndrome (FXS) is a trinucleotide repeat disorder that results in the silencing of the Fragile X Mental Retardation 1 gene (FMR1), leading to a lack of the FMR1 protein (FMRP). FMRP is an mRNA-binding protein that regulates the translation of hundreds of mRNAs important for synaptic plasticity. Several of these pathways have been identified and have guided the development of targeted treatments for FXS. Here we present evidence that serotonin is dysregulated in FXS and treatment with the selective serotonin reuptake inhibitor (SSRI) sertraline may be beneficial for individuals with FXS, particularly in early childhood. PMID:25606361

  19. [Treatment of irritable bowel syndrome with dicetelium and spasmomen].

    PubMed

    Nedogoda, S V; Parshev, V V

    2000-01-01

    The aim of the study was to compare clinical efficacy of new spasmolytics--pinaverium bromide (dicetel, Solvay Pharma) and otilonium bromide (spasmomen, Menarini-Berlin-Chemie)--in the treatment of irritable bowel syndrome. Effects appearing during the treatment with anxiolytic drug tofisopam (grandaxin, Egis) after using dicetel and spasmomen were also studied. The results indicate marked clinical efficiency of dicetel and spasmomen which improve vegetative functions and psychoemotional status. Grandaxin provides further improvement of clinical symptoms and additionally corrects psychoemotional status. PMID:11220900

  20. [Pyridostigmine in the treatment of postural orthostatic tachycardia syndrome].

    PubMed

    Can, Ilknur; Tholakanahalli, Venkatakrishna

    2014-09-01

    A 34-year-old female patient was admitted with the complaints of inability to stand upright, palpitations, dizziness, and fatigue in the upright posture for the last one year. She was found to stand upright for less than one minute without symptoms. Tilt table testing showed that, compared to baseline her heart rate increased 55 beats/min in the fifth minute of the test with the symptoms of palpitations, fatigue and sweating without any significant change in her blood pressure. Postural orthostatic tachycardia syndrome was diagnosed, and pyridostigmine treatment was started. Four months after treatment her symptoms were relieved so that she was able to function as a nurse. PMID:25362946

  1. Current options for treatment of hypothenar hammer syndrome.

    PubMed

    Hui-Chou, Helen G; McClinton, Michael A

    2015-02-01

    Hypothenar hammer syndrome is a rare vascular condition resulting from injury to the ulnar artery at the level of Guyon canal. The ulnar artery at the wrist is the most common site of arterial aneurysms of the upper extremity and is particularly prone to injury. Signs and symptoms include a palpable mass, distal digital embolization to long, ring, or small fingers, pain, cyanosis, pallor, coolness, and recurrent episodes of vasospasm. Modalities for diagnosis, evaluation, and surgical planning include duplex study, contrast arteriography, and computerized tomographic angiography (CTA). Management includes medical, nonoperative, or operative treatments. Appropriate treatment options are reviewed. PMID:25455356

  2. [Cervical myofascial pain syndrome. Narrative review of physiotherapeutic treatment].

    PubMed

    Capó-Juan, M A

    2015-01-01

    Pain is a complex and multifactorial phenomenon that depends on the interaction of biopsychosocial factors. Between 15-25% of adults suffer from chronic pain at some point in their lives. Cervical chronic pain is considered a public health problem affecting 9.6% men and 21.9% women, according to the latest National Health Survey 2011-12. A high percentage of medical consultations due to muscle pain turn out to be myofascial pain syndrome (MPS). Its existence implies the presence of myofascial trigger points which can be latent or active throughout the whole population. The aim of this review is to update knowledge in the various therapies applied by the physiotherapist in the treatment of this syndrome at cervical level. From the review it appears that some of the most used techniques that may be useful in the short or medium term are: ischemic compression and/or trigger point pressure release and dry needling. Furthermore, various combinations of treatment modalities are used to treat this syndrome, taking other aspects into account, such as education. PMID:25963463

  3. Successful treatment of Netherton's syndrome with topical calcipotriol.

    PubMed

    Godic, Aleksandar; Dragos, Vlasta

    2004-01-01

    We present a case of a 9-year-old boy with Netherton syndrome (NS) and skin manifestation of ichthyosis linearis circumflexa (ILC) who was successfully treated with topical 0.05% calcipotriol ointment bid. It was applied every fourth day on the same body area, which measured from 18% to 27% of the total body surface. Significant improvement of erythema and scaling was noted two weeks after the beginning of the treatment, with nearly total remission one week later, when the treatment was suspended. Remission lasted three to four weeks, when a few lesions of ILC appeared on his trunk and limbs and the treatment began again. The patient responded well each time he was treated. No adverse effects, suggestive of hypercalcemia or nephrocalcinosis, were noted during the treatment period which lasted for nine months. To evaluate calcipotriol's long-term efficacy and safety it should be tested on a larger group of patients with NS. PMID:15197002

  4. Mind/Body Psychological Treatments for Irritable Bowel Syndrome

    PubMed Central

    Fresé, Michael P.; Rapgay, Lobsang

    2008-01-01

    Currently, the goal of treatment for those with irritable bowel syndrome (IBS) is to improve the quality of life through a reduction in symptoms. While the majority of treatment approaches involve the use of traditional medicine, more and more patients seek out a non-drug approach to managing their symptoms. Current forms of non-drug psychologic or mind/body treatment for IBS include hypnotherapy, cognitive behavioral therapy and brief psychodynamic psychotherapy, all of which have been proven efficacious in clinical trials. We propose that incorporating the constructs of mindfulness and acceptance into a mind/body psychologic treatment of IBS may be of added benefit due to the focus on changing awareness and acceptance of one's own state which is a strong component of traditional and Eastern healing philosophies. PMID:18317547

  5. Irritable bowel syndrome: a mild disorder; purely symptomatic treatment.

    PubMed

    2009-04-01

    (1) Patients frequently complain of occasional bowel movement disorders, associated with abdominal pain or discomfort, but they are rarely due to an underlying organ involvement. Even when patients have recurrent symptoms, serious disorders are no more frequent in these patients than in the general population, unless other manifestations, anaemia, or an inflammatory syndrome is also present; (2) There is currently no way of radically modifying the natural course of recurrent irritable bowel syndrome; (3) The effects of antispasmodics on abdominal pain have been tested in about 20 randomised controlled trials. Pinaverium and peppermint essential oil have the best-documented efficacy and only moderate adverse effects. Antispasmodics with marked atropinic effects do not have a favourable risk-benefit balance; (4) Tricylic antidepressants seem to have only modest analgesic effects in this setting. In contrast, their adverse effects are frequent and they have somewhat negative risk-benefit balances. Nor has the efficacy of selective serotonin reuptake inhibitor antidepressants (SSRIs) been demonstrated; (5) Alosetron and tegaserod carry a risk of potentially life-threatening adverse effects and therefore have negative risk-benefit balances; (6) Seeds of plants such as psyllium and ispaghul, as well as raw apples and pears, have a limited impact on constipation and pain. Osmotic laxatives are effective on constipation. Symptomatic treatments for constipation can sometimes aggravate abdominal discomfort; (7) Loperamide has been poorly assessed in patients with recurrent irritable bowel syndrome with diarrhoea. It modestly slows bowel movement but does not relieve pain or abdominal discomfort; (8) Dietary measures have not been tested in comparative trials. Some patients are convinced that certain foods provoke a recurrence of irritable bowel syndrome, but restrictive diets carry a risk of nutritional deficiencies; (9) Various techniques intended to control emotional and

  6. Childhood opsoclonus-myoclonus syndrome: diagnosis and treatment.

    PubMed

    Blaes, Franz; Dharmalingam, Backialakshmi

    2016-06-01

    Opsoclonus-myoclonus syndrome (OMS) is a rare and primarily immune-mediated disease in children and adults. The main symptoms include opsoclonus, myoclonus and ataxia. In children, the symptoms also include irritability, and, over a long-term course, learning and behavioural disturbances. OMS can be idiopathic, parainfectious or occur as a paraneoplastic (tumour-associated) syndrome. Paraneoplastic OMS in children is almost exclusively associated with neuroblastoma, whereas in adults, small cell lung cancer and breast cancer are the main underlying tumours. An autoimmune pathophysiology is suspected because childhood OMS patients have functionally active autoantibodies, proinflammatory changes in the cytokine network and immunotherapy responses. Children appear to respond regularly to immunosuppressive treatment. However, although the neurological symptoms show a good response, most children continue to show neuropsychological disturbances. PMID:27095464

  7. Cushing’s syndrome in childhood: update on genetics, treatment, and outcomes

    PubMed Central

    Lodish, Maya

    2015-01-01

    Purpose of review To provide an update on the genes associated with Cushing’s syndrome in children, as well as to familiarize the clinician with recent treatment guidelines and outcome data for children with Cushing’s syndrome. Recent findings The list of genes associated with Cushing’s syndrome continues to grow. In addition, treatment for childhood Cushing’s syndrome is evolving. As long-term follow-up data on children becomes available, clinicians need to be aware of the issues that require attention. Summary Knowledge of the specific genetic causes of Cushing’s syndrome has potential implications for treatment, surveillance, and counseling. Advances in surgical technique, radiation modalities, and medical therapies offer the potential for additional treatment options in Cushing’s syndrome. Early identification and management of post-treatment morbidities in children treated for Cushing’s syndrome is crucial in order to optimize care. PMID:25517021

  8. Treatment of Lennox-Gastaut syndrome: overview and recent findings

    PubMed Central

    van Rijckevorsel, Kenou

    2008-01-01

    Lennox-Gastaut syndrome (LGS) is a rare, age-related syndrome, characterized by multiple seizure types, a specific electro-encephalographic pattern, and mental regression. However, published data on the etiology, evolution, and therapeutic approach of LGS are contradictory, partly because the precise definition of LGS used in the literature varies. In the most recent classification, LGS belongs to the epileptic encephalopathies and is highly refractory to all antiepileptic drugs. Numerous treatments, medical and non-medical, have been proposed and results mostly from open studies or case series have been published. Sometimes, patients with LGS are included in a more global group of patients with refractory epilepsy. Only 6 randomized double-blind controlled trials of medical treatments, which included patients with LGS, have been published. Overall, treatment is rarely effective and the final prognosis remains poor in spite of new therapeutic strategies. Co-morbidities need specific treatment. This paper summarizes the definition, diagnosis and therapeutic approach to LGS, including not only recognized antiepileptic drugs, but also “off label” medications, immune therapy, diet, surgery and some perspectives for the future. PMID:19337447

  9. Deep brain stimulation for the treatment of uncommon tremor syndromes

    PubMed Central

    Ramirez-Zamora, Adolfo; Okun, Michael S.

    2016-01-01

    ABSTRACT Introduction: Deep brain stimulation (DBS) has become a standard therapy for the treatment of select cases of medication refractory essential tremor and Parkinson’s disease however the effectiveness and long-term outcomes of DBS in other uncommon and complex tremor syndromes has not been well established. Traditionally, the ventralis intermedius nucleus (VIM) of the thalamus has been considered the main target for medically intractable tremors; however alternative brain regions and improvements in stereotactic techniques and hardware may soon change the horizon for treatment of complex tremors. Areas covered: In this article, we conducted a PubMed search using different combinations between the terms ‘Uncommon tremors’, ‘Dystonic tremor’, ‘Holmes tremor’ ‘Midbrain tremor’, ‘Rubral tremor’, ‘Cerebellar tremor’, ‘outflow tremor’, ‘Multiple Sclerosis tremor’, ‘Post-traumatic tremor’, ‘Neuropathic tremor’, and ‘Deep Brain Stimulation/DBS’. Additionally, we examined and summarized the current state of evolving interventions for treatment of complex tremor syndromes. Expert c ommentary: Recently reported interventions for rare tremors include stimulation of the posterior subthalamic area, globus pallidus internus, ventralis oralis anterior/posterior thalamic subnuclei, and the use of dual lead stimulation in one or more of these targets. Treatment should be individualized and dictated by tremor phenomenology and associated clinical features. PMID:27228280

  10. Electrocortical changes associated with minocycline treatment in fragile X syndrome

    PubMed Central

    Andrea, Schneider; Jacena, Leigh Mary; Patrick, Adams; Rawi, Nanakul; Tasleem, Chechi; John, Olichney; Randi, Hagerman; David, Hessl

    2016-01-01

    Minocycline normalizes synaptic connections and behavior in the knockout mouse model of fragile X syndrome (FXS). Human-targeted treatment trials with minocycline have shown benefits in behavioral measures and parent reports. Event-related potentials (ERPs) may provide a sensitive method of monitoring treatment response and changes in coordinated brain activity. Measurement of electrocortical changes due to minocycline was done in a double-blind, placebo-controlled crossover treatment trial in children with FXS. Children with FXS (Meanage 10.5 years) were randomized to minocycline or placebo treatment for 3 months then changed to the other treatment for 3 months. The minocycline dosage ranged from 25–100 mg daily, based on weight. Twelve individuals with FXS (eight male, four female) completed ERP studies using a passive auditory oddball paradigm. Current source density (CSD) and ERP analysis at baseline showed high-amplitude, long-latency components over temporal regions. After 3 months of treatment with minocycline, the temporal N1 and P2 amplitudes were significantly reduced compared with placebo. There was a significant amplitude increase of the central P2 component on minocycline. Electrocortical habituation to auditory stimuli improved with minocycline treatment. Our study demonstrated improvements of the ERP in children with FXS treated with minocycline, and the potential feasibility and sensitivity of ERPs as a cognitive biomarker in FXS treatment trials. PMID:23981511

  11. Magnesium for Treatment of Reversible Cerebral Vasoconstriction Syndrome

    PubMed Central

    Mijalski, Christina; Dakay, Katarina; Miller-Patterson, Cameron; Saad, Ali; Silver, Brian

    2015-01-01

    We describe 2 cases of reversible cerebral vasoconstriction syndrome (RCVS) with refractory headache aborted by intravenous magnesium. Case 1 is a 53-year-old woman with subarachnoid hemorrhage due to RCVS presented with refractory headache and persistent vasospasm, despite aggressive treatment with calcium channel blockers (CCBs) and systemic corticosteroids. Subsequently, she experienced dramatic relief of symptoms with intravenous magnesium therapy. She continued oral maintenance therapy and remained symptom free. Case 2 is a 71-year-old female with bilateral temporo-occipital infarcts due to RCVS, presented with refractory headache and persistent vasospasm on transcranial Doppler (TCD), despite aggressive treatment with CCBs. She experienced dramatic relief of symptoms with intravenous magnesium and resolution of vasospasm on TCD. Magnesium may be beneficial for the treatment of refractory headaches in patients with RCVS. Future studies are needed to determine whether it should be considered as a first-line agent. PMID:27366294

  12. Treatment of infertility in women with polycystic ovary syndrome.

    PubMed

    Bouchard, P

    2010-05-01

    The treatment of infertility in polycystic ovary syndrome (PCOS) associates lifestyle measures, and the use of drugs to induce ovulation. In this endeavour, clomifene citrate (CC) should be used as the first line of treatment, followed eventually by low dose gonadotrophin stimulation, as a second line. In rare cases, in case of failure of the CC treatment, ovarian drilling i.e. laparoscopic ovarian surgery (LOS), and finally assisted reproduction techniques can be used, if needed. Overall, ovulation induction (representing the CC-gonadotrophin paradigm) is highly effective with a cumulative singleton live birth rate of 72%. The use of insulin sensitizers i.e. metformin in PCOS should be restricted to women with glucose intolerance and/or insulin resistance. Based on recent data available, the routine use of this drug, alone, in ovulation induction is not recommended. PMID:20394912

  13. [SYNDROME OF DIABETIC FOOT: MODERN APPROACHES OF COMPLEX TREATMENT].

    PubMed

    Galimov, O V; Khanov, V O; Saifullin, R R; Valieva, G R; Okroyan, V P

    2015-01-01

    The article presents the results of treatment of 201 patients with neuroischemic form of diabetic foot syndrome. The research included 158 women and 43 men of the middle age of 62.5 ± 11.2 years. The complex approach was applied in treatment including medicamentous treatment, revasculization of extremity, an application of modern combined collagenous coating and foot relieving using silicone insoles and orthopedic footwear. The endovascular and open reconstructive operations were performed in order to obtain the revasculization of extremity. Given complex approach allowed reducing the terms of hospital stay, the rate of ulcerous defects recurrences and relapses of ischemia of lower extremities during one year after endovascular interventions. PMID:26983262

  14. [Infantile Munchausen syndrome. Etiology, diagnostic criteria, and treatment].

    PubMed

    Morales-Franco, B; de la Morena-Fernández, M L

    1995-01-01

    In this paper, we study a special kind of child abuse, the Münchhausen Syndrome by proxy, which consists of a group of diseases that some parents invent in their own children. These parents describe false symptoms of disease in their children, or manipulate the bodies of their children in order to cause alterations. Their purpose is that the doctor will carry out diagnostic tests and medical treatments with the children who really aren't ill. Our objective is to understand this syndrome, so we can report the factors that influence its appearance, study the diagnostic guidelines and the importance of doing a correct differential diagnostic with other real pathologies, and we analyze the preventive and therapeutic measures that health professionals must carry out in children and in their parents. When these professionals understand this syndrome they will be able to detect it before children are subjected to more unnecessary suffering. And, they will be able to prevent that these aggressions on children continue. PMID:8582571

  15. SAPHO Syndrome: Current Developments and Approaches to Clinical Treatment.

    PubMed

    Firinu, Davide; Garcia-Larsen, Vanessa; Manconi, Paolo Emilio; Del Giacco, Stefano R

    2016-06-01

    SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare autoimmune disease which, due to its clinical presentation and symptoms, is often misdiagnosed and unrecognized. Its main features are prominent inflammatory cutaneous and articular manifestations. Treatments with immunosuppressive drugs have been used for the management of SAPHO with variable results. To date, the use of anti-TNF-α agents has proved to be an effective alternative to conventional treatment for unresponsive or refractory SAPHO cases. TNF-α is a pro-inflammatory cytokine and pivotal regulator of other cytokines, including IL-1 β, IL-6, and IL-8, involved in inflammation, acute-phase response induction, and chemotaxis. IL-1 inhibition strategies with anakinra have shown efficacy as first and second lines of treatment. In this review, we will describe the main characteristics of biological drugs currently used for SAPHO syndrome. We also describe some of the promising therapeutic effects of ustekinumab, an antibody against the p40 subunit of IL-12 and IL-23, after failure of multiple drugs including anti-TNF-α and anakinra. We discuss the use and impact of the new anti-IL-1 antagonists involved in the IL-17 blockade, in particular for the most difficult-to-treat SAPHO cases. PMID:27108452

  16. Minimally invasive surgery for the treatment of ureteral stump syndrome

    PubMed Central

    Alenezi, Husain; Eltiraifi, Abdelmoniem E.; Alomar, Mohammad

    2015-01-01

    Objective: The aim was to highlight the advantages and the feasibility of treating ureteral stump syndrome (USS) by different minimally invasive procedures. Materials and Methods: Four patients with USS who were treated by different minimally invasive surgery approaches depending on their presentation and findings on radiologic investigations. Results: Three patients had complete resolution of their symptoms, whereas the fourth patient had persistence of urinary tract infection. Conclusion: Minimally invasive surgery is a valid treatment option for patients with USS with possible less morbidity than conventional open surgical excision. PMID:26692664

  17. Treatment strategies for Middle East respiratory syndrome coronavirus

    PubMed Central

    Modjarrad, Kayvon

    2016-01-01

    Middle East respiratory syndrome coronavirus (MERS-CoV), an emerging infectious disease of growing global importance, has caused severe acute respiratory disease in more than 1600 people, resulting in almost 600 deaths. The high case fatality rate, growing geographic distribution and vaguely defined epidemiology of this novel pathogen have created an urgent need for effective public health countermeasures, including safe and effective treatment strategies. Despite the relatively few numbers of cases to date, research and development of MERS-CoV therapeutic candidates is advancing quickly. This review surveys the landscape of these efforts and assesses their potential for use in affected populations. PMID:26866060

  18. Treatment of primary Sjögren syndrome.

    PubMed

    Saraux, Alain; Pers, Jacques-Olivier; Devauchelle-Pensec, Valérie

    2016-08-01

    Primary Sjögren syndrome (pSS) is a progressive autoimmune disease characterized by sicca and systemic manifestations. In this Review, we summarize the available data on topical and systemic medications, according to clinical signs and disease activity, and we describe the ongoing studies using biologic drugs in the treatment of pSS. Expanding knowledge about the epidemiology, classification criteria, systemic activity scoring (ESSDAI) and patient-reported outcomes (ESSPRI) is driving active research. Treatment decisions are based on the evaluation of symptoms and extraglandular manifestations. Symptomatic treatment is usually appropriate, whereas systemic treatment is reserved for systemic manifestations. Sicca is managed by education, environment modification, elimination of contingent offending drugs, artificial tears, secretagogues and treatments for complications. Mild systemic signs such as fatigue are treated by exercise. Pain can require short-term moderate-dose glucocorticoid therapy and, in some cases, disease-modifying drugs. Severe and acute systemic manifestations indicate treatment with glucocorticoids and/or immunosuppressant drugs. The role for biologic agents is promising, but no double-blind randomized controlled trials (RCTs) proving the efficacy of these drugs are available. Targets for new treatments directed against the immunopathological mechanisms of pSS include epithelial cells, T cells, B-cell overactivity, the interferon signature, proinflammatory cytokines, ectopic germinal centre formation, chemokines involved in lymphoid cell homing, and epigenetic modifications. PMID:27411907

  19. Evaluation and treatment of critical asthma syndrome in children.

    PubMed

    Wade, Alexander; Chang, Christopher

    2015-02-01

    The heterogeneity of asthma is illustrated by the significantly different features of pediatric asthma compared to adult asthma. One phenotype of severe asthma in pediatrics includes atopy, lack of reduction in lung function, and absence of gender bias as the main characteristics. Included in the NIH NAEPP EPR-3 are recommendations for the treatment and management of severe pediatric asthma and critical asthma syndrome, such as continuous nebulization treatments, intubation and mechanical ventilation, heliox, and magnesium sulfate. In addition, epinephrine, intravenous immunoglobulin, intravenous montelukast, extracorporeal membrane oxygenation, and many biological modulators currently under investigation are additional current and/or future treatment modalities for the severe pediatric asthmatic. But, perhaps the most important strategy for managing the severe asthmatic is preventative treatment, which can significantly decrease impairment and risk, particularly for severe acute exacerbations requiring emergency care and/or hospitalization. In order for preventative therapy to be successful, several challenges must be met, including selecting the correct therapy for each patient and then ensuring compliance or adherence to a treatment plan. The heterogeneity of asthma renders the former difficult in that not all patients will respond equally to the same treatment; the latter is only helpful if the correct treatment is employed. Strategies to ensure compliance include education of caregivers and patients and their families. As newer medications are introduced, options for individualized or customized medicine increase, and this may pave the way for significant decreases in morbidity and mortality in severe pediatric asthma. PMID:24488329

  20. [Botulinum toxin for the treatment of pain syndromes].

    PubMed

    Ferreira, Joaquim J; Couto, Marina; Costa, João; Coelho, Miguel; Rosa, Mário M; Sampaio, Cristina

    2006-01-01

    Although botulinum toxin (BoNT) is being used for therapeutic purposes for more than 20 years, the list of potential new indications continues to increase and includes various pain syndromes. The pain relief experienced by patients with dystonia and spasticity from intramuscular BoNT injections suggested that other chronic skeletal-muscles pain conditions may also benefit. BoNT inhibits the release of acetylcholine at the neuromuscular junction thereby reducing striatal muscle contractions and the proposed analgesic property was initially attributed to muscular relaxation. A specific analgesic BoNT effect is difficult to conclude from studies where pain is conditioned by other associated symptoms like dystonia, muscle contraction or spasticity. One alternative is to critically appraise clinical trials where BoNT was studied as the active intervention and pain evaluated as an outcome. From this analysis there is convincing evidence for the effectiveness of BoNT in the treatment of pain associated with cervical dystonia. For all other pain syndromes there have been relatively few, small sized, placebo-controlled studies (myofascial pain syndrome, chronic neck and low back pain, piriformis syndrome and fibromyalgia) and the results of these studies have been contradictory or non conclusive. To establish the analgesic properties of BoNT there is a need for appropriately designed, exploratory randomized controlled studies in well accepted human models of nociceptive or neuropathic pain. This does not exclude the subsequent need to conduct pragmatic trials to evaluate the effectiveness of BoNT in conditions where the improvement of pain or any associated clinical sign or symptom may be of clinical relevance. PMID:17058384

  1. Meditation over medication for irritable bowel syndrome? On exercise and alternative treatments for irritable bowel syndrome.

    PubMed

    Asare, Fredrick; Störsrud, Stine; Simrén, Magnus

    2012-08-01

    Complimentary alternative treatment regimens are widely used in irritable bowel syndrome (IBS), but the evidence supporting their use varies. For psychological treatment options, such as cognitive behavioral therapy, mindfulness, gut-directed hypnotherapy, and psychodynamic therapy, the evidence supporting their use in IBS patients is strong, but the availability limits their use in clinical practice. Dietary interventions are commonly included in the management of IBS patients, but these are primarily based on studies assessing physiological function in relation to dietary components, and to a lesser degree upon research examining the role of dietary components in the therapeutic management of IBS. Several probiotic products improve a range of symptoms in IBS patients. Physical activity is of benefit for health in general and recent data implicates its usefulness also for IBS patients. Acupuncture does not seem to have an effect beyond placebo in IBS. A beneficial effect of some herbal treatments has been reported. PMID:22661301

  2. Syndrome Co-Occurrence and Treatment Outcomes in Youth Mental Health Clinics

    ERIC Educational Resources Information Center

    Doss, Amanda Jensen; Weisz, John R.

    2006-01-01

    Despite widespread speculation that syndrome co-occurrence undermines treatment outcomes, this hypothesis has not been fully examined within clinical care settings. To address this gap, the authors investigated the relation between syndrome co-occurrence and outcome among 325 clinically referred youths. For every syndrome, higher initial severity…

  3. Treatment of Hyperthyroidism in Down Syndrome: Case Report and Review of Literature.

    ERIC Educational Resources Information Center

    Ali, Fawzi E.; Al-Busairi, Waleed A.; Al-Mulla, Fatema A.

    1999-01-01

    A case of an institutionalized adult male with Down syndrome and hyperthyroidism is reported. After treatment with radioactive iodine, he was found to be markedly hypothyroid when he was reviewed 11 weeks later. Three treatment options for hyperthyroidism in Down syndrome are reviewed: surgery, medical treatments, and radiotherapy. (Author/CR)

  4. [TOS (thoracic outlet syndrome)--a challenge to conservative treatment].

    PubMed

    Lindgren, K A

    1997-10-01

    Functional impairment and pain in the upper extremities may indicate a functional deficit in the thoracic outlet. Static work posture, trauma and whiplash injury may be predisposing factors. The younger generation who often spend long hours in front of a computer are in danger of becoming a future risk group. The primary care physician should be familiar with the syndrome which can be identified by careful clinical examination. Timely intervention can prevent much of the disabling symptomatology. Treatment is primarily conservative and should be aimed at the restoration of functional capacity. As in other disorders, the individual constellation of symptoms is dependent on circumstantial factors, an aspect meriting particular attention in treatment and follow-up. Optimisation of ergonomic conditions is important feature of treatment, and long-term follow-up is necessary. Transient exacerbation is not an indication for surgical treatment. If cervical and thoracic outlet function has normalised but the patient still has symptoms, then the differential diagnosis should be reconsidered. Examination and treatment of patients with pain in the upper extremities requires the collaboration of the physician and physical and occupational therapists. Treatment can be delivered in the primary care setting. PMID:9411397

  5. [Amifostine used in the treatment of patients with myelodysplastic syndrome].

    PubMed

    Li, Shu-Xia; Zhu, Hong-Li; Lu, Xue-Chun; Fan, Hui; Yao, Shan-Qian; Ma, Jian; Yang, Qing-Ming; Cai, Li-Li; Zhuang, Xiao-Meng; Yang, Yang

    2007-02-01

    The study was aimed to investigate the curative effects and adverse effects of amifostine in the treatment of patients with myelodysplastic syndrome (MDS). Amifostine (AMF) was used alone (4/12) or combined with recombinant human erythropoietin (rh-EPO) (8/12) in 12 MDS patients. The therapeutic regimen was adopted with AMF 0.4 g/day for 5 days, then took a break of 2 days and then went on for 3 weeks consecutively, that was reputed as one treatment cycle. rh-EPO 6 000 U was used for 3 days per week. The results showed that 12 patients all attained hematological improvement in peripheral blood. 11 cases showed major effective response rate (91.7%), while 1 case showed minor response rate (8.3%). The effective response rate of hemoglobin, leukocytes and platelets was 100%, 75% and 58.3% respectively. The intervals of red cell transfusions (RCT) in 2 cases living on red cell transfusion before AMF treatment were prolonged after AMF treatments, and the amount of each RCT was decreased obviously. The side effect was usually discomfort of digestive system, but all patients can endure. In conclusion, Amifostine is a potential drug in the treatment of MDS patients with safety especially to those elder patients who often suffered from other multiple organ disfunctions, and the curative effect will be improved by more treatment cycles. PMID:17490528

  6. [Bran in the treatment of irritable bowel syndrome].

    PubMed

    Dubinin, A V; Kabanov, A V; Kirkin, B V; Kolkunova, G K; Igorianova, N A

    1987-01-01

    The paper deals with the problem of using wheat bran in the treatment of patients with irritable colon syndrome expressed as spastic constipation. The effectiveness of the treatment with the wheat bran only (in a dose of 30-35 g/day), and in combination with drugs was comparatively studied in 105 patients. The bran fractions differing in the particle size, in the content of cellulose, starch and vitamins were used in the treatment. The combined therapy proved to be advantageous only in the rate of the clinical effect, while the acceleration of the movement along the large intestine did not depend on the treatment type. A long-term (during one year) follow-up of the patients showed that the bran intake led to the cessation of the disease relapse; when the bran was abolished the symptoms of the disease appeared in 11 out of 12 cases. The highest effect was recorded with the bran fraction containing 55.3% cellulose, 18.3% lignin, 157 micrograms tocopherol and the lowest amount of starch--18.0%. A conclusion has been made that the wheat bran are effective in the treatment and prevention of intestinal diseases, the effectiveness of the treatment depends on the summary content of food fibers in the nutrition. PMID:3031879

  7. Treatment of irritable bowel syndrome in China: A review

    PubMed Central

    Li, Chun-Yan; Li, Shu-Chuen

    2015-01-01

    Irritable bowel syndrome (IBS) is a common, chronic, functional gastrointestinal disorder with a high incidence rate in the general population, and it is common among the Chinese population. The pathophysiology, etiology and pathogenesis of IBS are poorly understood, with no evidence of inflammatory, anatomic, metabolic, or neoplastic factors to explain the symptoms. Treatment approaches are mainly focused on symptom management to maintain everyday functioning and to improve quality of life for patients with IBS. However, prescribed medications often result in significant side effects, and many IBS sufferers (particularly Chinese) do not improve. Instead of taking a variety of conventional medications, many have turned to taking traditional Chinese medicine or integrated Chinese and Western medicine for remedy. A number of clinical trials have shown that Chinese herbal, acupuncture or integrative therapies presented improved treatment outcomes and reduced side effects in IBS patients. The purpose of this review article is to examine the treatment approaches of IBS that have been published in recent years, especially in China, to assess the possible differences in treating IBS between China and other countries. This would provide some useful information of unique treatment approach in clinical practice for physicians in the management of IBS in China, thus offering more treatment options for IBS patients with potentially better treatment outcomes while reducing the medical cost burden. PMID:25741137

  8. Treatment of scorpion envenoming syndrome -- need for scientific magnanimity.

    PubMed

    Murthy, K Radha Krishna

    2013-04-01

    Scorpion envenoming syndrome results in a severe autonomic storm with a massive release of catecholamines, increased levels of angiotensin II, an increase in glucagon, cortisol, thyroid hormones; either suppressed insulin levels or hyperinsulinaemia (insulin resistance), hyperglycaemia; increased circulating free fatty acid levels. These hormonal alterations could be responsible for the pathogenesis of a variety of clinical manifestations. Under these conditions, scorpion envenoming syndrome with myocardial damage, cardiovascular disturbances, peripheral circulatory failure, respiratory and cardiac pulmonary oedema, and many other clinical manifestations resulting in a syndrome of fuel-energy deficits and an inability to use the existing metabolic substrates by vital organs causing multisystem organ failure and death. Based on animal experiments in which insulin administration reversed the metabolic and ECG changes induced by scorpion envenoming and treating the poisonous scorpion sting victims with insulin, we consider that insulin has a primary metabolic role in preventing and reversing the cardiovascular, haemodynamic, and neurological manifestations and pulmonary oedema induced by scorpion envenoming. Administration of insulin-glucose infusion to scorpion sting victims appears to be the physiological basis for the control of the metabolic response when that has become a determinant to survival. The mordality of treatment is continuous infusion of regular crystalline insulin at the rate of 0.3 U/g glucose and glucose at the rate of 0.1g/kg body weight/hour, with supplementation of potassium as needed and maintenance of fluid, electrolytes and acid-base balance is required. This treatment should be given at the earliest on admission and continued for the next 48-72 hours. Antiscorpion serum could also be given independently or along with insulin-glucose infusion. PMID:24475558

  9. Treatment benefits on metabolic syndrome with diet and physical activity.

    PubMed

    Dragusha, Gani; Elezi, Abdulla; Dragusha, Shpend; Gorani, Daut; Begolli, Luljeta

    2010-05-01

    The research has included 422 patients aged between 25 to 60, of whom 341 were men and 81 women. The purpose of research was to determine impact of diet and physical activity in the treatment of metabolic syndrome during the six month period. Processing of results through descriptive and discriminative analysis have indicated that 6 month treatment with diet and physical activity have had an impact in the: waistline decrease by 6.05 cm or 5.50% among males, and 4.92 cm or 5.10% among females; body mass index (BMI) decrease by 1.78 or 6.20% among males, and 2.3 or 8.16% among females; decrease of blood triglycerides levels by 0.35 mmol/L or 16.28% among males, and 0.27 mmol/L or 13.30% among females; increase of blood cholesterol HDL-C by 0.48 mmol/L or 34.78% among males, and 0.06 mmol/L or 4.28% among females; systolic arterial pressure decreased by 15 mmHg or 10.18%, and diastolic blood pressure by 8.74 mmHg or 9.47% among males, and systolic arterial pressure decreased by 7.39 mmHg or 5.17%, and diastolic blood pressure decreased by 5.18 mmHg or 5.75% among females; the level of blood glucose decreased by 0.45 mmol/L or 7.04% among males, and by 0.64 mmol/L or 9.92% decreased among females. The results show that physical exercise and diet are important factors in reducing the values symptoms of metabolic syndrome. In order to improve symptoms of metabolic syndrome, it is necessary to keep on with healthy diet and physical exercise that means the change of lifestyle. PMID:20507300

  10. Complementary and alternative medicine for treatment of irritable bowel syndrome

    PubMed Central

    Shen, Yi-Hao A.; Nahas, Richard

    2009-01-01

    Abstract OBJECTIVE To review the evidence supporting selected complementary and alternative medicine approaches used in the treatment of irritable bowel syndrome (IBS). QUALITY OF EVIDENCE MEDLINE (from January 1966), EMBASE (from January 1980), and the Cochrane Database of Systematic Reviews were searched until March 2008, combining the terms irritable bowel syndrome or irritable colon with complementary therapies, alternative medicine, acupuncture, fiber, peppermint oil, herbal, traditional, yoga, massage, meditation, mind, relaxation, probiotic, hypnotherapy, psychotherapy, cognitive therapy, or behavior therapy. Results were screened to include only clinical trials, systematic reviews, and meta-analyses. Level I evidence was available for most interventions. MAIN MESSAGE Soluble fibre improves constipation and global IBS symptoms. Peppermint oil alleviates IBS symptoms, including abdominal pain. Probiotic trials show overall benefit for IBS but there is little evidence supporting the use of any specific strain. Hypnotherapy and cognitive-behavioural therapy are also effective therapeutic options for appropriate patients. Certain herbal formulas are supported by limited evidence, but safety is a potential concern. All interventions are supported by systematic reviews or meta-analyses. CONCLUSION Several complementary and alternative therapies can be recommended as part of an evidence-based approach to the treatment of IBS; these might provide patients with satisfactory relief and improve the therapeutic alliance. PMID:19221071

  11. Therapeutic Strategies for the Treatment of Severe Cushing's Syndrome.

    PubMed

    Alexandraki, Krystallenia I; Grossman, Ashley B

    2016-03-01

    Severe Cushing's syndrome presents an acute emergency and is defined by massively elevated random serum cortisol [more than 36 μg/dL (1000 nmol/L)] at any time or a 24-h urinary free cortisol more than fourfold the upper limit of normal and/or severe hypokalaemia (<3.0 mmol/L), along with the recent onset of one or more of the following: sepsis, opportunistic infection, intractable hypokalaemia, uncontrolled hypertension, heart failure, gastrointestinal haemorrhage, glucocorticoid-induced acute psychosis, progressive debilitating myopathy, thromboembolism or uncontrolled hyperglycaemia and ketocacidosis. Treatment focuses on the management of the severe metabolic disturbances followed by rapid resolution of the hypercortisolaemia, and subsequent confirmation of the cause. Emergency lowering of the elevated serum cortisol is most rapidly achieved with oral metyrapone and/or ketoconazole; if parenteral therapy is required then intravenous etomidate is rapidly effective in almost all cases, but all measures require careful supervision. The optimal order and combination of drugs to treat severe hypercortisolaemia-mostly in the context of ectopic ACTH-secreting syndrome, adrenocortical carcinoma or an ACTH-secreting pituitary adenoma (mainly macroadenomas)-is not yet established. Combination therapy may be useful not only to rapidly control cortisol excess but also to lower individual drug dosages and consequently the possibility of adverse effects. If medical treatments fail, bilateral adrenalectomy should be performed in the shortest possible time span to prevent the debilitating complications of uncontrolled hypercortisolaemia. PMID:26833215

  12. Antiviral Treatment Guidelines for Middle East Respiratory Syndrome

    PubMed Central

    Chong, Yong Pil; Song, Joon Young; Seo, Yu Bin; Choi, Jae-Phil

    2015-01-01

    Middle East respiratory syndrome (MERS) is an acute infectious disease of the respiratory system caused by the new betacoronavirus (MERS coronavirus, MERS-CoV), which shows high mortality rates. The typical symptoms of MERS are fever, cough, and shortness of breath, and it is often accompanied by pneumonia. The MERS-CoV was introduced to Republic of Korea in May 2015 by a patient returning from Saudi Arabia. The disease spread mostly through hospital infections, and by the time the epidemic ended in August, the total number of confirmed diagnoses was 186, among which 36 patients died. Reflecting the latest evidence for antiviral drugs in the treatment of MERS-CoV infection and the experiences of treating MERS patients in Republic of Korea, these guidelines focus on antiviral drugs to achieve effective treatment of MERS-CoV infections. PMID:26483999

  13. Testosterone Supplementation Therapy in the Treatment of Metabolic Syndrome

    PubMed Central

    Kovac, Jason R.; Pastuszak, Alexander W.; Lamb, Dolores J.; Lipshultz, Larry I.

    2016-01-01

    Metabolic syndrome (MetS) is a clinical complex of risk factors including increased waist circumference, high triglycerides, low HDL cholesterol, high blood pressure and insulin resistance whose presence increases the likelihood of developing diabetes and cardiovascular disease. With a quarter of the American adult population affected, MetS has been referred to as the most significant public health threat of the 21st century. While lifestyle modification and weight loss are recommended, no specific pharmacological treatment is known. Given that low levels of testosterone have been implicated in the pathogenesis of MetS and an inverse relationship exists between circulating testosterone and the development of MetS, it is tempting to speculate that men with MetS may benefit from testosterone supplementation therapy (TST). As such, this review seeks to examine the role of testosterone and the use of TST as a treatment modality in men with MetS. PMID:25387223

  14. Restless legs syndrome: clinical presentation diagnosis and treatment.

    PubMed

    Wijemanne, Subhashie; Jankovic, Joseph

    2015-06-01

    Restless legs syndrome (RLS) is a circadian disorder of sensory-motor integration that may be related to genetically determined dysregulation of iron transport across the blood-brain barrier. Dopamine agonists (DAs) have been considered the first-line therapy, but with the growing appreciation of problems associated with long-term treatment, particularly augmentation and impulse control disorder, alpha-2-delta drugs, such as gabapentin, are now considered the first line of treatment in patients with troublesome RLS. Opioids can be considered as an alternative therapy, particularly in patients with DA-related augmentation. In more severe cases, a combination therapy may be required. Intravenous iron therapy may be considered on those patients with refractory RLS. PMID:25979181

  15. The role of renal autotransplantation in treatment of nutcracker syndrome.

    PubMed

    Salehipour, Mehdi; Rasekhi, Alireza; Shirazi, Mehdi; Haghpanah, Abdolreza; Jahanbini, Shahrokh; Eslahi, Seyed Ali

    2010-03-01

    To report our experience with renal autotransplantation in treatment of gross hematuria caused by nutcracker Syndrome (NCS). Between September 2005 and January 2008, four patients of mean age 25.5 years (range: 23-28) with gross hematuria were diagnosed to have NCS. Investigations revealed isolated hematuria on urinalysis, a bloody efflux from left ureteral orifice by urethrocystoscopy, dilatation of left renal vein (LRV) with significant difference in peak systolic velocity in colour doppler ultrasonography (CDUS) and dilatation and compression of LRV between aorta and superior mesenteric artery in MRA. After operation, hematuria dis-appeared in all patients. No vascular or urological complication was seen. Follow up ranged from 4 to 24 months. In conclusion, autotransplatation of left kidney is very effective for the treatment of symptomatic NCS. PMID:20228506

  16. Behavioural treatments for Tourette syndrome: an evidence-based review.

    PubMed

    Frank, Madeleine; Cavanna, Andrea Eugenio

    2013-01-01

    Tourette syndrome (TS) is a disorder characterised by multiple motor and vocal tics and is frequently associated with behavioural problems. Tics are known to be affected by internal factors such as inner tension and external factors such as the surrounding environment. A number of behavioural treatments have been suggested to treat the symptoms of TS, in addition to pharmacotherapy and surgery for the most severe cases. This review compiled all the studies investigating behavioural therapies for TS, briefly describing each technique and assessing the evidence in order to determine which of these appear to be effective. Different behavioural therapies that were used included habit reversal training (HRT), massed negative practice, supportive psychotherapy, exposure with response prevention, self-monitoring, cognitive-behavioural therapy, relaxation therapy, assertiveness training, contingency management, a tension-reduction technique and biofeedback training. Overall, HRT is the best-studied and most widely-used technique and there is sufficient experimental evidence to suggest that it is an effective treatment. Most of the other treatments, however, require further investigation to evaluate their efficacy. Specifically, evidence suggests that exposure with response prevention and self-monitoring are effective, and more research is needed to determine the therapeutic value of the other treatments. As most of the studies investigating behavioural treatments for TS are small-sample or single-case studies, larger randomised controlled trials are advocated. PMID:23187152

  17. Diagnosis and treatment of diarrhea-predominant irritable bowel syndrome.

    PubMed

    Lacy, Brian E

    2016-01-01

    Irritable bowel syndrome (IBS) is one of the most common gastrointestinal disorders worldwide. The economic impact of IBS on the health care system is substantial, as is the personal impact on patients. Patients with diarrhea-predominant IBS (IBS-D) comprise a substantial proportion of the overall IBS population. Primary care providers are often the first point of contact for patients with IBS-D and can accurately diagnose IBS after a careful history and examination without extensive diagnostic tests. Several pharmacologic treatments (eg, loperamide, alosetron, and antidepressants) and non-pharmacologic treatments (eg, dietary modification and probiotics) are available for IBS-D, but restrictions on use (eg, alosetron) or the lack of controlled trial data showing reductions in both global and individual IBS-D symptoms (eg, bloating, pain and stool frequency) emphasize the need for alternative treatment options. Two newer medications (eluxadoline and rifaximin) were approved in May 2015 for the treatment of IBS-D, and represent new treatment options for this common gastrointestinal condition. PMID:26929659

  18. Diagnosis and treatment of diarrhea-predominant irritable bowel syndrome

    PubMed Central

    Lacy, Brian E

    2016-01-01

    Irritable bowel syndrome (IBS) is one of the most common gastrointestinal disorders worldwide. The economic impact of IBS on the health care system is substantial, as is the personal impact on patients. Patients with diarrhea-predominant IBS (IBS-D) comprise a substantial proportion of the overall IBS population. Primary care providers are often the first point of contact for patients with IBS-D and can accurately diagnose IBS after a careful history and examination without extensive diagnostic tests. Several pharmacologic treatments (eg, loperamide, alosetron, and antidepressants) and non-pharmacologic treatments (eg, dietary modification and probiotics) are available for IBS-D, but restrictions on use (eg, alosetron) or the lack of controlled trial data showing reductions in both global and individual IBS-D symptoms (eg, bloating, pain and stool frequency) emphasize the need for alternative treatment options. Two newer medications (eluxadoline and rifaximin) were approved in May 2015 for the treatment of IBS-D, and represent new treatment options for this common gastrointestinal condition. PMID:26929659

  19. Colonised and neurasthenic: from the appropriation of a word to the reality of a malaise de civilisation in urban French Vietnam.

    PubMed

    Monnais, Laurence

    2012-01-01

    Neurasthenia remains an important health problem in certain Asian populations, both in Asia as well as in a diasporic context. An anachronistic disease for Western observers, it has become an exotic culture-bound syndrome as well as a somatoform disorder too often hiding much more serious issues of depression. This article approaches this 'problematic' health issue from a historian's point of view and offers a colonial genealogy that will discuss neurasthenia's outline in French Vietnam. By retracing and analysing the different mentions, definitions, and uses of the term neurasthenia in the interwar period, it aims to better understand certain historical realities that might have shaped the local identity and spatiality of this problem (concentrated in colonial cities in which social change and modernity were expressed in their most salient forms), and perhaps even identify reasons that facilitated its post-colonial survival. PMID:23066605

  20. Four treatment strategies for complex regional pain syndrome type 1.

    PubMed

    Lee, Sang Ki; Yang, Dae Suk; Lee, Jae Won; Choy, Won Sik

    2012-06-01

    Complex regional pain syndrome (CRPS) poses a dilemma for many clinicians due to its unknown etiology and largely unsuccessful treatment modalities. The purpose of this study was to compare the clinical results of 4 treatment modalities for CRPS type 1. A total of 59 patients were divided into 4 groups based on treatment modality: group A, an oral nonsteroidal anti-inflammatory drug (NSAID) (n=10); group B, oral gabapentin (n=12); group C, intravenous (IV) 10% mannitol and steroid (n=11); group D, a combination of IV 20% mannitol and steroid with oral gabapentin (n=26). The patients remained under medical supervision after discharge and were evaluated either once a month or once every 2 months until final follow-up at a mean of 8 months. Patients in group A showed improvement in pain level, finger range of motion, swelling, and grip strength, without statistical significance (P=.076, P=.062, P=.312, and P=.804, respectively). Patients in group B showed significant improvement in pain level (P<.001), and patients in group C showed improvement in pain, finger range of motion, and swelling (P=.127), which rendered functional impairment unchanged. In comparison, patients in group D showed recovery of grip strength and improvement in pain level, finger range of motion, and (P<.001, P=.016, P=.031, and P=.047, respectively). Based on these results, a protocol including a combination of IV 20% mannitol and steroid with oral gabapentin is an acceptable and effective treatment for CRPS type 1. PMID:22691654

  1. Current pharmacotherapeutic approaches for the treatment of Tourette syndrome.

    PubMed

    Egolf, A; Coffey, B J

    2014-02-01

    Tourette syndrome is a childhood onset neurodevelopmental disorder characterized by multiple motor and vocal tics. Although many youth experience attenuation or even remission of tics in adolescence and young adulthood, some individuals experience persistent tics which can be debilitating or disabling. The majority of patients also have one or more psychiatric comorbid disorders, such as attention deficit hyperactivity disorder and/or obsessive-compulsive disorder. Treatment is multimodal, including both pharmacotherapy and cognitive behavioral treatment, and requires disentanglement of tics and the comorbid symptoms. Although the only two formally approved medications in the United States are haloperidol and pimozide, these treatments are generally not used as first-line interventions due to their significant potential for adverse effects. The α-adrenoceptor agonists guanfacine and clonidine have an established evidence base for both efficacy and tolerability, and are usually recommended as initial pharmacotherapy. Atypical neuroleptics, such as aripiprazole or risperidone, are typically used if the α-adrenoceptor agonists are ineffective or intolerable. However, many other pharmacological agents reviewed in this manuscript have been studied as treatment alternatives. PMID:24619591

  2. Kleine–Levin syndrome: Etiology, diagnosis, and treatment

    PubMed Central

    Ramdurg, Santosh

    2010-01-01

    Kleine–Levin syndrome (KLS) is a rare sleep disorder mainly affecting teenage boys in which the main features are intermittent hypersomnolence, behavioral and cognitive disturbances, hyperphagia, and in some cases hypersexuality. Each episode is of brief duration varying from a week to 1–2 months and affected people are entirely asymptomatic between episodes. No definite cause has been identified, and no effective treatments are available even though illness is having well-defined clinical features. Multiple relapses occur every few weeks or months, and the condition may last for a decade or more before spontaneous resolution. In this study, PubMed was searched and appropriate articles were reviewed to highlight etiology, clinical features, and management of KLS. On the basis of this knowledge, practical information is offered to help clinicians about how to investigate a case of KLS, and what are the possible treatment modalities available currently for the treatment during an episode and interepisodic period for prophylaxis. Comprehensive research into the etiology, pathophysiology, investigation, and treatments are required to aid the development of disease-specific targeted therapies. PMID:21264130

  3. The chronic syndromes after previous treatment of pituitary tumours.

    PubMed

    Romijn, Johannes A

    2016-09-01

    Ultimately, almost all patients who are appropriately treated for pituitary tumours enter a chronic phase with control or cure of hormonal excess, adequate treatment of pituitary insufficiency and relief of mass effects. This phase is associated with improvement of initial signs and symptoms, but also with the persistent consequences of the initial disease and associated treatments. Pituitary insufficiency is a common denominator in many of these patients, and is associated with a reduction in quality of life, despite adequate endocrine substitution. Hypothalamic dysfunction can be present in patients previously treated for visual impairments caused by large suprasellar adenomas, or craniopharyngiomas. In addition to hypopituitarism, these patients can have multisystem morbidities caused by altered hypothalamic function, including weight gain and disturbed regulation of sleep-wake cycles. Mortality can also be affected. Patients cured of Cushing disease or acromegaly have chronic multisystem morbidities (in the case of Cushing disease, also affecting mortality) caused by irreversible effects of the previous excesses of cortisol in Cushing disease and growth hormone and insulin-like growth factor 1 in acromegaly. In addition to early diagnosis and treatment of pituitary tumours, research should focus on the amenability of these chronic post-treatment syndromes to therapeutic intervention, to improve quality of life and clinical outcomes. PMID:27259177

  4. Myelodysplastic Syndromes in the Elderly: Treatment Options and Personalized Management.

    PubMed

    Burgstaller, Sonja; Wiesinger, Petra; Stauder, Reinhard

    2015-11-01

    Myelodysplastic syndromes (MDS) are typical diseases of the elderly, with a median age of 68-75 years at initial diagnosis. Demographic changes producing an increased proportion of elderly in our societies mean the incidence of MDS will rise dramatically. Considering the increasing number of treatment options, ranging from best supportive care to hematopoietic stem cell transplantation (HSCT), decision making is rather complex in this cohort of patients. Moreover, aspects of the aging process also have to be considered in therapy planning. Treatment of elderly MDS patients is dependent on the patient's individual risk and prognosis. Comorbidities play an essential role as predictors of survival and therapy tolerance. Age-adjusted models and the use of geriatric assessment scores are described as a basis for individualized treatment algorithms. Specific treatment recommendations for the different groups of patients are given. Currently available therapeutic agents, including supportive care, erythropoiesis-stimulating agents (ESAs), immune-modulating agents, hypomethylating agents, and HSCT are described in detail and discussed with a special focus on elderly MDS patients. The inclusion of elderly patients in clinical trials is of utmost importance to obtain data on efficacy and safety in this particular group of patients. Endpoints relevant for the elderly should be integrated, including maintenance of quality of life and functional activities as well as evaluation of use of healthcare resources. PMID:26476843

  5. Dental Treatment of a Child with Pallister-Killian Syndrome

    PubMed Central

    Didinen, Serhan; Atabek, Didem; Kip, Gülay; Patır Münevveroğlu, Aslı; Tulunoğlu, Özlem

    2016-01-01

    The Pallister-Killian syndrome (PKS) is an extremely rare genetic disorder with an incidence estimated around 1/25000. PKS is a multiple congenital anomaly deficit syndrome caused by mosaic tissue limited tetrasomy for chromosome 12p. The presented report is the first confirmed case with PKS in Turkey. This report focuses on the orofacial clinical manifestations of an 6-year-old boy with PKS who was referred to the Department of Paediatric Dentistry clinic, Gazi University. It has been learned that the PKS was diagnosed 1 year after birth. Due to intellectual disability, it was decided to make the dental treatments under moderate sedation. Although significant tongue thrust and anterior open bite were determined, any oral appliances could not be applied because of the 2 epilepsy seizures in the last 2 years. The aim was to treat decayed teeth and set good oral hygiene in the patient's mouth. Still, there is a probability for epilepsy seizures. If epileptic seizures stop permanently, we can apply an oral appliance to block tongue thrust. The patient is now under control. In cases of systemic and oral findings such as PKS, conducting medical and dental approaches together will increase the life quality of patients. PMID:26998367

  6. Tourette Syndrome Associated with Mental Retardation: A Single-Subject Treatment Study with Haloperidol.

    ERIC Educational Resources Information Center

    Rosenquist, Peter B.; And Others

    1997-01-01

    A study of a 35-year-old woman with severe mental retardation and Tourette syndrome examined the efficacy of haloperidol in the treatment of Tourette syndrome. Results indicate that the haloperidol treatment produced significant reduction of all tic topographies. Improvement was also seen in tic severity, hyperactivity, and compulsive behaviors.…

  7. Stuttering Treatment for a School-Age Child with Down Syndrome: A Descriptive Case Report

    ERIC Educational Resources Information Center

    Harasym, Jessica; Langevin, Marilyn

    2012-01-01

    Background: Little is known about optimal treatment approaches and stuttering treatment outcomes for children with Down syndrome. Aims and method: The purpose of this study was to investigate outcomes for a child with Down syndrome who received a combination of fluency shaping therapy and parent delivered contingencies for normally fluent speech,…

  8. Use of krypton laser stimulation in the treatment of dry eye syndrome

    NASA Astrophysics Data System (ADS)

    Kecik, Tadeusz; Switka-Wieclawska, Iwona; Ciszewska, Joanna; Portacha, Lidia

    1991-08-01

    We''d like to present the use of krypton laser stimulation in the treatment of dry eye syndrom. 10 patients with dry eye syndrom were treated with irradiation of the lacrimal gland. Schirmer test and break up time were performed before and after therapy. After 10 days of treatment we observed higher value of secreted tear amount.

  9. Guideline for diagnosis and treatment of subacromial pain syndrome

    PubMed Central

    Diercks, Ron; Bron, Carel; Dorrestijn, Oscar; Meskers, Carel; Naber, René; de Ruiter, Tjerk; Willems, Jaap; Winters, Jan; van der Woude, Henk Jan

    2014-01-01

    Treatment of “subacromial impingement syndrome” of the shoulder has changed drastically in the past decade. The anatomical explanation as “impingement” of the rotator cuff is not sufficient to cover the pathology. “Subacromial pain syndrome”, SAPS, describes the condition better. A working group formed from a number of Dutch specialist societies, joined by the Dutch Orthopedic Association, has produced a guideline based on the available scientific evidence. This resulted in a new outlook for the treatment of subacromial pain syndrome. The important conclusions and advice from this work are as follows: (1) The diagnosis SAPS can only be made using a combination of clinical tests. (2) SAPS should preferably be treated non-operatively. (3) Acute pain should be treated with analgetics if necessary. (4) Subacromial injection with corticosteroids is indicated for persistent or recurrent symptoms. (5) Diagnostic imaging is useful after 6 weeks of symptoms. Ultrasound examination is the recommended imaging, to exclude a rotator cuff rupture. (6) Occupational interventions are useful when complaints persist for longer than 6 weeks. (7) Exercise therapy should be specific and should be of low intensity and high frequency, combining eccentric training, attention to relaxation and posture, and treatment of myofascial trigger points (including stretching of the muscles) may be considered. (8) Strict immobilization and mobilization techniques are not recommended. (9) Tendinosis calcarea can be treated by shockwave (ESWT) or needling under ultrasound guidance (barbotage). (10) Rehabilitation in a specialized unit can be considered in chronic, treatment resistant SAPS, with pain perpetuating behavior. (11) There is no convincing evidence that surgical treatment for SAPS is more effective than conservature management. (12) There is no indication for the surgical treatment of asymptomatic rotator cuff tears. PMID:24847788

  10. Advances in mechanistic understanding and treatment approaches to Tourette syndrome.

    PubMed

    Shprecher, David R; Kious, Brent M; Himle, Michael H

    2015-11-01

    Tourette syndrome (TS) is a childhood onset neurodevelopmental disorder characterized by semi-involuntary, repetitive movements and sounds (motor and phonic tics). Transient tics in childhood are common, and their persistence in TS may be due to failure of maturation of frontal-subcortical circuits mediated by genetic predisposition and environmental factors. Tic improvement by young adult years is common, but its mechanism and predictive factors are unclear. Though tics can often be managed with nonmedical therapies, pharmacotherapy is often used for refractory, severe, or injurious tics but is complicated by side effects and incomplete benefit. This review summarizes the current understanding of TS pathophysiology, current and future treatment options, and recommendations for future research. PMID:26645901

  11. Low-FODMAP Diet for Treatment of Irritable Bowel Syndrome

    PubMed Central

    Magge, Suma

    2012-01-01

    Functional bowel disorders, including irritable bowel syndrome (IBS), are common disorders that have a significant impact on patients’ quality of life. These disorders present major challenges to healthcare providers, as few effective medical therapies are currently available. Recently, there has been increasing interest in dietary therapies for IBS, particularly a diet low in fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAPs). Since ingestion of FODMAPs increases the delivery of readily fermentable substrates and water to the distal small intestine and colon—which results in luminal distention and gas—the reduction of FODMAPs in a patient’s diet may improve functional gastrointestinal symptoms. This paper will review the pathophysiology of IBS and the role of FODMAPs for the treatment of this condition. PMID:24672410

  12. Teduglutide for the treatment of short bowel syndrome.

    PubMed

    Jeppesen, P B

    2013-10-01

    Glucagon-like peptide 2 (GLP-2) decreases gastric and intestinal motility, reduces gastric secretions, promotes intestinal growth and improves post-resection structural and functional adaptation in short bowel syndrome (SBS). Teduglutide, an analogue of GLP-2, has a prolonged half-life and provides intestinotrophic effects with once-daily subcutaneous injection in patients with SBS. This monograph reviews the preclinical and clinical data that provide the scientific rationale for the use of teduglutide in this orphan condition. Teduglutide increases intestinal absorption and diminishes the need for parenteral support in patients with SBS. The adverse event profile is consistent with the underlying disease and the known mechanism of action of teduglutide. Following its positive regulatory review and approval by the European Medicines Agency and the U.S. Food and Drug Administration in 2012, teduglutide has moved from the research setting to clinical practice, offering a new treatment paradigm for this burdensome and potentially life-threatening condition. PMID:24191254

  13. Mifepristone Treatment of Cushing's Syndrome in a Pediatric Patient.

    PubMed

    Banerjee, Ronadip R; Marina, Neyssa; Katznelson, Laurence; Feldman, Brian J

    2015-11-01

    Cushing's syndrome (CS) in the pediatric population is challenging to diagnose and treat. Although next-generation medical therapies are emerging for adults with CS, none are currently approved or used in children. Here we describe the first use of mifepristone, a glucocorticoid receptor antagonist, to treat CS in a pediatric subject. The patient, a 14-year-old girl with an 18-month history of metastatic neuroendocrine carcinoma, suffered from fatigue, profound myopathy, irritability, and depression. She was found to have hypertension, hypokalemia, and worsening control of her preexisting type 1 diabetes. In this report, we detail our clinical evaluation that confirmed CS caused by an ectopic adrenocorticotropic hormone secreting tumor. Surgical and radiation therapies were not pursued because of her poor functional status and limited life expectancy, and medical treatment of CS was indicated for symptom relief. Mifepristone treatment provided rapid improvement in glycemic control, insulin resistance, and hypertension as well as significant diminishment of her myopathy and fatigue. Hypokalemia was managed with an oral potassium replacement and dose escalation of spironolactone; no other significant adverse effects were observed. Despite successful palliation of Cushing's signs and symptoms, the patient died of progression of her cancer. This case demonstrates the safety and efficacy of mifepristone treatment in a pediatric patient with symptomatic, ectopic CS. We conclude that, in appropriate pediatric patients with CS, glucocorticoid receptor antagonism with mifepristone should be considered to control the effects of hypercortisolism and to improve quality of life. PMID:26459648

  14. Efficacy of dry needling for treatment of myofascial pain syndrome.

    PubMed

    Fogelman, Yacov; Kent, John

    2015-01-01

    Myofascial pain is a major cause of musculoskeletal regional pain. Myofascial pain, which is a high-prevalence but eminently treatable condition, is almost universally underdiagnosed by physicians and undertreated by physical therapy modalities. Large numbers of patients can be left suffering in chronic pain for years. Dry needling, also referred to as Intramuscular Stimulation, is a method in the arsenal of pain management which has been known for almost 200 years in Western medicine, yet has been almost completely ignored. With the increase in research in this field over the past two decades, there are many high-quality studies that demonstrate dry needling to be an effective and safe method for the treatment of myofascial pain when diagnosed and treated by adequately-trained physicians or physical therapists. This article provides an overview of recent literature regarding the treatment of myofascial pain syndrome, evidence for the efficacy of dry needling as a central component of its management, and a glimpse at developments in recent imaging methods to aid in the treatment of these problems. PMID:25322743

  15. Treatment and management of ascites and hepatorenal syndrome: an update

    PubMed Central

    Buder, Robert; Kapun, Lisbeth; Voglmayr, Martin

    2015-01-01

    Ascites and renal dysfunction are frequent complications experienced by patients with cirrhosis of the liver. Ascites is the pathologic accumulation of fluid in the peritoneal cavity, and is one of the cardinal signs of portal hypertension. The diagnostic evaluation of ascites involves assessment of its granulocyte count and protein concentration to exclude complications such as infection or malignoma and to allow risk stratification for the development of spontaneous peritonitis. Although sodium restriction and diuretics remain the cornerstone of the management of ascites, many patients require additional therapy when they become refractory to this treatment. In this situation, the treatment of choice is repeated large-volume paracentesis. Alteration in splanchnic hemodynamics is one of the most important changes underlying the development of ascites. Further splanchnic dilation leads to changes in systemic hemodynamics, activating vasopressor agents and leading to decreased renal perfusion. Small alterations in renal function influence the prognosis, which depends on the cause of renal failure. Prerenal failure is evident in about 70% of patients, whereas in about 30% of patients the cause is hepatorenal syndrome (HRS), which is associated with a worse prognosis. Therefore, effective therapy is of great clinical importance. Recent data indicate that use of the new definition of acute kidney injury facilitates the identification and treatment of patients with renal insufficiency more rapidly than use of the current criteria for HRS. In this review article, we evaluate approaches to the management of patients with ascites and HRS. PMID:25729433

  16. Early versus Late Surgical Treatment for Neurogenic Thoracic Outlet Syndrome

    PubMed Central

    Al-Hashel, Jasem Yousef; El Shorbgy, Ashraf Ali M. A.; Elshereef, Rawhia R.

    2013-01-01

    Objectives. To compare the outcome of early surgical intervention versus late surgical treatment in cases of neurogenic thoracic outlet syndrome (NTOS). Design. Prospective study. Settings. Secondary care (Al-Minia University Hospital, Egypt) from 2007 to 2010. Participants. Thirty-five patients of NTOS (25 women and 10 men, aged 20–52 years), were classified into 2 groups. First group (20 patients) was operated within 3 months of the onset and the second group (15 patients) was operated 6 months after physiotherapy. Interventions. All patients were operated via supraclavicular surgical approach. Outcomes Measures. Both groups were evaluated clinically and, neurophysiologically and answered the disabilities of the arm, shoulder, and hand (DASH) questionnaire preoperatively and 6 months after the surgery. Results. Paraesthesia, pain, and sensory nerve action potential (SNAP) of ulnar nerve were significantly improved in group one. Muscle weakness and denervation in electromyography EMG were less frequent in group one. The postoperative DASH score improved in both groups but it was less significant in group two (P < .001 in group 1 and P < .05 in group 2). Conclusions. Surgical treatment of NTOS improves functional disability and stop degeneration of the nerves. Early surgical treatment decreases the occurrence of muscle wasting and denervation of nerves compared to late surgery. PMID:24109518

  17. Pharmacology of opioids in the treatment of chronic pain syndromes.

    PubMed

    Vallejo, Ricardo; Barkin, Robert L; Wang, Victor C

    2011-01-01

    The perpetual pursuit of pain elimination has been constant throughout human history and pervades human cultures. In some ways it is as old as medicine itself. Cultures throughout history have practiced the art of pain management through remedies such as oral ingestion of herbs or techniques believed to have special properties. In fact, even Hippocrates wrote about the practice of trepanation, the cutting of holes in the body to release pain. Current therapies for management of pain include the pervasive utilization of opioids, which have an extensive history, spanning centuries. There is general agreement about the appropriateness of opioids for the treatment of acute and cancer pain, but the long-term use of these drugs for treatment of chronic non-malignant pain remains controversial. The pros and cons regarding these issues are beyond the scope of this review. Instead, the purpose of this review will be directed towards the pharmacology of commonly prescribed opioids in the treatment of various chronic pain syndromes. Opium, derived from the Greek word for "juice," is extracted from the latex sap of the opium poppy (Papaverum somniferum). The juice of the poppy is the source of some 20 different alkaloids of opium. These alkaloids of opioids can be divided into 2 chemical classes: phenanthrenes (morphine, codeine, and thebaine) and benzylisoquinolines (agents that do not interact with opioid receptors). PMID:21785485

  18. Current treatment of antiphospholipid syndrome: lights and shadows.

    PubMed

    Espinosa, Gerard; Cervera, Ricard

    2015-10-01

    For patients with antiphospholipid syndrome (APS), the consensus is to treat those who develop thrombosis with long-term oral anticoagulation therapy and to prevent obstetric manifestations by use of aspirin and heparin. These recommendations are based on data from randomized controlled trials and observational studies. Despite this body of knowledge, areas of uncertainty regarding the management of APS exist where evidence is scarce or nonexistent. In other words, for a subset of patients the course of management is unclear. Some examples are patients with 'seronegative' APS, those who do not fulfil the formal (clinical or serological) classification criteria for definite APS, and those with recurrent thrombotic events despite optimal anticoagulation. Other challenges include the treatment of clinical manifestations not included in the classification criteria, such as haematologic manifestations (thrombocytopenia and haemolytic anaemia), neurologic manifestations (chorea, myelitis and multiple sclerosis-like lesions), and nephropathy and heart valve disease associated with antiphospholipid antibodies (aPL), as well as the possible withdrawal of anticoagulation treatment in selected cases of thrombotic APS in which assays for aPL become persistently negative. This Review focuses on the current recommendations for thrombotic and obstetric manifestations of APS, as well as the management of difficult cases. Some aspects of treatment, such as secondary prophylaxis of venous thrombosis, are based on strong evidence--the 'lights' of APS treatment. Conversely, other areas, such as the treatment of non-criteria manifestations of APS, are based only on expert consensus or common sense and remain the 'shadows' of APS therapy. PMID:26122952

  19. Dural Reduction Surgery: A Treatment Option for Frontotemporal Brain Sagging Syndrome.

    PubMed

    Mostofi, Emily; Schievink, Wouter I; Sim, Valerie L

    2016-07-01

    Frontotemporal brain sagging syndrome is a dementia associated with hypersomnolence, personality changes, and features of intracranial hypotension on magnetic resonance imaging. The literature is sparse with respect to treatment options; many patients simply worsen. We present a case in which this syndrome responded to lumbar dural reduction surgery. Postoperative magnetic resonance imaging indicated normalization of brain sagging and lumbar intrathecal pressure. Although no evidence of cerebrospinal leak was found, extremely thin dura was noted intraoperatively, suggesting that a thin and incompetent dura could result in this low-pressure syndrome. Clinicians who encounter this syndrome should consider dural reduction surgery as a treatment strategy. PMID:26972054

  20. Piriformis Syndrome in Fibromyalgia: Clinical Diagnosis and Successful Treatment

    PubMed Central

    Siddiq, Md Abu Bakar; Khasru, Moshiur Rahman; Rasker, Johannes J.

    2014-01-01

    Piriformis syndrome is an underdiagnosed extraspinal association of sciatica. Patients usually complain of deep seated gluteal pain. In severe cases the clinical features of piriformis syndrome are primarily due to spasm of the piriformis muscle and irritation of the underlying sciatic nerve but this mysterious clinical scenario is also described in lumbar spinal canal stenosis, leg length discrepancy, piriformis myofascial pain syndrome, following vaginal delivery, and anomalous piriformis muscle or sciatic nerve. In this paper, we describe piriformis and fibromyalgia syndrome in a 30-year-old young lady, an often missed diagnosis. We also focus on management of the piriformis syndrome. PMID:25328750

  1. New antiplatelet agents in the treatment of acute coronary syndromes.

    PubMed

    Sabouret, Pierre; Taiel-Sartral, Magali

    2014-03-01

    Effective antagonism of the P2Y12 platelet receptor is central to the treatment of acute coronary syndrome (ACS) patients, especially in the setting of percutaneous coronary intervention and stenting. According to consensus guidelines, early revascularization and intensive antiplatelet therapy are key to reducing the complications that arise from myocardial ischaemia and the recurrence of cardiovascular events. Until recently, clopidogrel was the key P2Y12 antagonist advocated, but due to several limitations as an antiplatelet agent, newer drugs with more predictable, rapid and potent effects have been developed. Prasugrel and ticagrelor are now the recommended first-line agents in patients presenting with non-ST-segment elevation ACS and ST-segment elevation ACS, due to large-scale randomized trials that demonstrated net clinical benefit of these agents over clopidogrel, as stated in the European guidelines. Although no study has directly compared the two agents, analysis of the data to date suggests that certain patient types, such as diabetics, those with ST-segment elevation myocardial infarction or renal failure and the elderly may have a better outcome with one agent over the other. Further studies are needed to confirm these differences and answer pending questions regarding the use of these drugs to optimize efficacy while minimizing adverse events, such as bleeding. The aim of this review is to provide an overview of the current P2Y12 receptor antagonists in the treatment of ACS, with a focus on issues of appropriate agent selection, timing of treatment, bleeding risk and the future role of personalized treatment using platelet function and genetic testing. PMID:24630752

  2. Excited delirium syndrome (ExDS): treatment options and considerations.

    PubMed

    Vilke, Gary M; Bozeman, William P; Dawes, Donald M; Demers, Gerard; Wilson, Michael P

    2012-04-01

    The term Excited Delirium Syndrome (ExDS) has traditionally been used in the forensic literature to describe findings in a subgroup of patients with delirium who suffered lethal consequences from their untreated severe agitation.(1-5) Excited delirium syndrome, also known as agitated delirium, is generally defined as altered mental status and combativeness or aggressiveness. Although the exact signs and symptoms are difficult to define precisely, clinical findings often include many of the following: tolerance to significant pain, rapid breathing, sweating, severe agitation, elevated temperature, delirium, non-compliance or poor awareness to direction from police or medical personnel, lack of fatiguing, unusual or superhuman strength, and inappropriate clothing for the current environment. It has become increasingly recognized that individuals displaying ExDS are at high risk for sudden death, and ExDS therefore represents a true medical emergency. Recently the American College of Emergency Physicians (ACEP) published the findings of a white paper on the topic of ExDS to better find consensus on the issues of definition, diagnosis, and treatment.(6) In so doing, ACEP joined the National Association of Medical Examiners (NAME) in recognizing ExDS as a medical condition. For both paramedics and physicians, the difficulty in diagnosing the underlying cause of ExDS in an individual patient is that the presenting clinical signs and symptoms of ExDS can be produced by a wide variety of clinical disease processes. For example, agitation, combativeness, and altered mental status can be produced by hypoglycemia, thyroid storm, certain kinds of seizures, and these conditions can be difficult to distinguish from those produced by cocaine or methamphetamine intoxication.(7) Prehospital personnel are generally not expected to differentiate between the multiple possible causes of the patient's presentation, but rather simply to recognize that the patient has a medical emergency

  3. Atypical hemolytic uremic syndrome: from diagnosis to treatment.

    PubMed

    Franchini, Massimo

    2015-10-01

    Thrombotic microangiopathy (TMA) is a relatively rare condition but a medical urgency requiring immediate intervention to avoid irreversible organ damage or death. Symptoms on presentation include microangiopathic haemolytic anaemia, thrombocytopenia and organ damage. The most frequent direct causes of TMA are thrombotic thrombocytopenic purpura (TTP) and haemolytic uremic syndrome (HUS). The most common form of HUS is related to Shiga toxin producing Escherichia coli (STEC) infection while approximately 10% of cases are due to dysregulation of the complement pathway (atypical haemolytic uremic syndrome, aHUS). Optimal treatment regimens differ depending on the underlying cause; however, differential diagnosis may be difficult. The most accurate method of diagnosis is based on exclusion and should consider, beyond the symptoms common to TMA, ADAMTS13 activity levels and STEC infection status. For the management of TTP, plasma exchange (PE) is the most important acute intervention and is associated with lower mortality and better outcomes than plasma infusion. In most patients with STEC-HUS, the course of disease is self-limiting although management of acute kidney injury is often required. Until recently, the management of aHUS consisted of early and intensive PE, although this was mostly ineffective in protecting from subsequent organ damage. Eculizumab, an inhibitor of the alternative complement pathway, produces a rapid and sustained inhibition of the TMA process, with significant improvements in long-term clinical outcomes. Due to the significant improvement achieved, eculizumab has subsequently been approved as first-line therapy when an unequivocal diagnosis of aHUS has been made. PMID:25803082

  4. Traditional Chinese Medicine in Treatment of Metabolic Syndrome

    PubMed Central

    Yin, Jun; Zhang, Hanjie; Ye, Jianping

    2008-01-01

    In management of metabolic syndrome, the traditional Chinese medicine (TCM) is an excellent representative in alternative and complementary medicines with a complete theory system and substantial herb remedies. In this article, basic principle of TCM is introduced and 22 traditional Chinese herbs are reviewed for their potential activities in the treatment of metabolic syndrome. Three herbs, ginseng, rhizoma coptidis (berberine, the major active compound) and bitter melon, were discussed in detail on their therapeutic potentials. Ginseng extracts made from root, rootlet, berry and leaf of Panax quinquefolium (American ginseng) and Panax ginseng (Asian ginseng), are proved for anti-hyperglycemia, insulin sensitization, islet protection, anti-obesity and anti-oxidation in many model systems. Energy expenditure is enhanced by ginseng through thermogenesis. Ginseng-specific saponins (ginsenosides) are considered as the major bioactive compounds for the metabolic activities of ginseng. Berberine from rhizoma coptidis is an oral hypoglycemic agent. It also has anti-obesity and anti-dyslipidemia activities. The action mechanism is related to inhibition of mitochondrial function, stimulation of glycolysis, activation of AMPK pathway, suppression of adipogenesis and induction of low-density lipoprotein (LDL) receptor expression. Bitter melon or bitter gourd (Momordica charantia) is able to reduce blood glucose and lipids in both normal and diabetic animals. It may also protect β cells, enhance insulin sensitivity and reduce oxidative stress. Although evidence from animals and humans consistently supports the therapeutic activities of ginseng, berberine and bitter melon, multi-center large-scale clinical trials have not been conducted to evaluate the efficacy and safety of these herbal medicines. PMID:18537696

  5. Cerebral creatine deficiency syndromes: clinical aspects, treatment and pathophysiology.

    PubMed

    Stockler, Sylvia; Schutz, Peter W; Salomons, Gajja S

    2007-01-01

    Cerebral creatine deficiency syndromes (CCDSs) are a group of inborn errors of creatine metabolism comprising two autosomal recessive disorders that affect the biosynthesis of creatine--i.e. arginine:glycine amidinotransferase deficiency (AGAT; MIM 602360) and guanidinoacetate methyltransferase deficiency (GAMT; MIM 601240)--and an X-linked defect that affects the creatine transporter, SLC6A8 deficiency (SLC6A8; MIM 300036). The biochemical hallmarks of these disorders include cerebral creatine deficiency as detected in vivo by 1H magnetic resonance spectroscopy (MRS) of the brain, and specific disturbances in metabolites of creatine metabolism in body fluids. In urine and plasma, abnormal guanidinoacetic acid (GAA) levels are found in AGAT deficiency (reduced GAA) and in GAMT deficiency (increased GAA). In urine of males with SLC6A8 deficiency, an increased creatine/creatinine ratio is detected. The common clinical presentation in CCDS includes mental retardation, expressive speech and language delay, autistic like behaviour and epilepsy. Treatment of the creatine biosynthesis defects has yielded clinical improvement, while for creatine transporter deficiency, successful treatment strategies still need to be discovered. CCDSs may be responsible for a considerable fraction of children and adults affected with mental retardation of unknown etiology. Thus, screening for this group of disorders should be included in the differential diagnosis of this population. In this review, also the importance of CCDSs for the unravelling of the (patho)physiology of cerebral creatine metabolism is discussed. PMID:18652076

  6. Treatment of Dientamoeba fragilis in Patients with Irritable Bowel Syndrome

    PubMed Central

    Engsbro, Anne Line; Stensvold, C. Rune; Nielsen, Henrik V.; Bytzer, Peter

    2012-01-01

    The role of Dientamoeba fragilis in irritable bowel syndrome (IBS) is incompletely known. We aimed to investigate whether eradication of D. fragilis alleviates symptoms in IBS. Twenty-five D. fragilis-positive IBS patients were treated with Metronidazole (MZ) or Tetracycline. The patients were mostly female (89%), and mean age (SD) was 35.1 (8.2) years. Microbiological response, evaluated 2 weeks post-treatment, was observed in 15 of 25 patients (60%), all by MZ. Clinical response, defined as adequate relief of symptoms, was observed in 7 of 22 patients (32%), all by MZ. In a logistic regression analysis, we found no significant association between clinical and microbiological response. This case study did not support our hypothesis of a simple association between D. fragilis and IBS. Some D. fragilis-infections were insufficiently treated by MZ. Further studies into the prevalence and effect of eradication of D. fragilis in IBS and into efficient treatments of D. fragilis are warranted. PMID:23091195

  7. Treatment of irritable bowel syndrome: beyond fiber and antispasmodic agents

    PubMed Central

    Sainsbury, Anita; Ford, Alexander C.

    2011-01-01

    Irritable bowel syndrome (IBS) is a chronic functional disorder of the gastrointestinal tract of unknown etiology. The diagnosis of IBS is made clinically, using symptom-based criteria such as the Manning or Rome criteria. Medical therapy for this condition has traditionally been directed towards symptom relief, using fiber or antispasmodic agents. In recent years, emerging data have confirmed the efficacy of antidepressants, psychological therapies, 5-HT3 antagonists, 5-HT4 agonists, and probiotics in the short-term treatment of IBS, although whether these therapies influence the long-term course of the disease is unknown. Increasing knowledge regarding the pathophysiological mechanisms underlying IBS has resulted in a number of novel molecular treatments, which show promise. These include therapies targeting gastrointestinal mucosal chloride channels and guanylate cyclase-C receptors, as well as highly selective agents influencing serotonergic transmission that, at the time of writing, do not appear to have any severe deleterious effects. In this article we provide a summary of current and emerging therapies in this field. PMID:21694813

  8. Recent advances in pharmacological treatment of irritable bowel syndrome

    PubMed Central

    Lazaraki, Georgia; Chatzimavroudis, Grigoris; Katsinelos, Panagiotis

    2014-01-01

    Irritable bowel syndrome (IBS) is a highly prevalent functional disorder that reduces patients’ quality of life. It is a chronic disorder characterized by abdominal pain or discomfort associated with disordered defecation in the absence of identifiable structural or biochemical abnormalities. IBS imposes a significant economic burden to the healthcare system. Alteration in neurohumoral mechanisms and psychological factors, bacterial overgrowth, genetic factors, gut motility, visceral hypersensitivity, and immune system factors are currently believed to influence the pathogenesis of IBS. It is possible that there is an interaction of one or more of these etiologic factors leading to heterogeneous symptoms of IBS. IBS treatment is predicated upon the patient’s most bothersome symptoms. Despite the wide range of medications and the high prevalence of the disease, to date no completely effective remedy is available. This article reviews the literature from January 2008 to July 2013 on the subject of IBS peripherally acting pharmacological treatment. Drugs are categorized according to their administration for IBS-C, IBS-D or abdominal pain predominant IBS. PMID:25083060

  9. New and Emerging Treatment Options for Irritable Bowel Syndrome.

    PubMed

    Lacy, Brian E; Chey, William D; Lembo, Anthony J

    2015-04-01

    Irritable bowel syndrome (IBS) is a functional gastrointestinal disorder associated with abdominal pain, diarrhea, constipation, or a mix of symptoms. The pathophysiology of IBS is not completely understood but appears to involve genetics, the gut microbiome, immune activation, altered intestinal permeability, and brain-gut interactions. There is no gold standard for diagnosis. Several sets of symptom-based guidelines exist. Treatment strategies for IBS may include both nonpharmacologic and pharmacologic approaches. Lifestyle modifications that aim to improve exercise, sleep, diet, and stress may be warranted. Recent data suggest that a gluten-free diet and a diet low in fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAPs) may benefit some patients. For patients with diarrhea-predominant IBS, treatment options include the synthetic peripheral μ-opioid receptor agonist loperamide, antispasmodic agents, antidepressants, serotonin 5-HT3 antagonists, and the gut-specific antibiotic rifaximin. Ongoing research is evaluating the use of probiotics. For patients with constipation-predominant IBS, therapeutic strategies may include dietary fiber, laxatives, and the prosecretory agents lubiprostone and linaclotide. Research is continuing to optimize the use of available agents and evaluating new approaches to further improve the care of patients with IBS. PMID:26491416

  10. Acute Coronary Syndromes in Women: Recent Treatment Trends and Outcomes

    PubMed Central

    Graham, Garth

    2016-01-01

    In the USA and internationally, women experience farranging differences with respect to acute coronary syndrome (ACS) and myocardial infarction (MI). Women suffer from more comorbidities than men, such as smoking, obesity, hypertension, diabetes, and poor mental health. They some-times exhibit atypical MI presentation symptoms and are overall less likely to present with chest pain. Women are more likely than men to encounter delays between the onset of symptoms and arrival at the hospital or to guideline treatment. The use of various surgical and pharmacological treatments, including revascularization approaches, also differs. Women, on average, have worse outcomes than men following MI, with more complications, higher mortality rates, and poorer recovery. Internationally, outcomes are similar despite various differences in health care and culture in non-US countries. In this review, we detail differences regarding ACS and MI in women, describing their complex correlations and discussing their possible causes. Educational approaches that are tailored to women might help to reduce the incidence of ACS and MI, as well as outcomes following hospitalization. Although outcomes following acute MI have been improving over the years, women may require special consideration in order to see continued improvement. PMID:26884685

  11. New and Emerging Treatment Options for Irritable Bowel Syndrome

    PubMed Central

    Lacy, Brian E.; Chey, William D.; Lembo, Anthony J.

    2015-01-01

    Irritable bowel syndrome (IBS) is a functional gastrointestinal disorder associated with abdominal pain, diarrhea, constipation, or a mix of symptoms. The pathophysiology of IBS is not completely understood but appears to involve genetics, the gut microbiome, immune activation, altered intestinal permeability, and brain-gut interactions. There is no gold standard for diagnosis. Several sets of symptom-based guidelines exist. Treatment strategies for IBS may include both nonpharmacologic and pharmacologic approaches. Lifestyle modifications that aim to improve exercise, sleep, diet, and stress may be warranted. Recent data suggest that a gluten-free diet and a diet low in fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAPs) may benefit some patients. For patients with diarrhea-predominant IBS, treatment options include the synthetic peripheral μ-opioid receptor agonist loperamide, antispasmodic agents, antidepressants, serotonin 5-HT3 antagonists, and the gut-specific antibiotic rifaximin. Ongoing research is evaluating the use of probiotics. For patients with constipation-predominant IBS, therapeutic strategies may include dietary fiber, laxatives, and the prosecretory agents lubiprostone and linaclotide. Research is continuing to optimize the use of available agents and evaluating new approaches to further improve the care of patients with IBS. PMID:26491416

  12. Role of antispasmodics in the treatment of irritable bowel syndrome

    PubMed Central

    Annaházi, Anita; Róka, Richárd; Rosztóczy, András; Wittmann, Tibor

    2014-01-01

    Irritable bowel syndrome (IBS) is a long-lasting, relapsing disorder characterized by abdominal pain/discomfort and altered bowel habits. Intestinal motility impairment and visceral hypersensitivity are the key factors among its multifactorial pathogenesis, both of which require effective treatment. Voltage-gated calcium channels mediate smooth muscle contraction and endocrine secretion and play important roles in neuronal transmission. Antispasmodics are a group of drugs that have been used in the treatment of IBS for decades. Alverine citrate, a spasmolytic, decreases the sensitivity of smooth muscle contractile proteins to calcium, and it is a selective 5-HT1A receptor antagonist. Alverine, in combination with simethicone, has been demonstrated to effectively reduce abdominal pain and discomfort in a large placebo-controlled trial. Mebeverine is a musculotropic agent that potently blocks intestinal peristalsis. Non-placebo-controlled trials have shown positive effects of mebeverine in IBS regarding symptom control; nevertheless, in recent placebo-controlled studies, mebeverine did not exhibit superiority over placebo. Otilonium bromide is poorly absorbed from the GI tract, where it acts locally as an L-type calcium channel blocker, an antimuscarinic and a tachykinin NK2 receptor antagonist. Otilonium has effectively reduced pain and improved defecation alterations in placebo-controlled trials in IBS patients. Pinaverium bromide is also an L-type calcium channel blocker that acts locally in the GI tract. Pinaverium improves motility disorders and consequently reduces stool problems in IBS patients. Phloroglucinol and trimethylphloroglucinol are non-specific antispasmodics that reduced pain in IBS patients in a placebo-controlled trial. Antispasmodics have excellent safety profiles. T-type calcium channel blockers can abolish visceral hypersensitivity in animal models, which makes them potential candidates for the development of novel therapeutic agents in the

  13. Role of antispasmodics in the treatment of irritable bowel syndrome.

    PubMed

    Annaházi, Anita; Róka, Richárd; Rosztóczy, András; Wittmann, Tibor

    2014-05-28

    Irritable bowel syndrome (IBS) is a long-lasting, relapsing disorder characterized by abdominal pain/discomfort and altered bowel habits. Intestinal motility impairment and visceral hypersensitivity are the key factors among its multifactorial pathogenesis, both of which require effective treatment. Voltage-gated calcium channels mediate smooth muscle contraction and endocrine secretion and play important roles in neuronal transmission. Antispasmodics are a group of drugs that have been used in the treatment of IBS for decades. Alverine citrate, a spasmolytic, decreases the sensitivity of smooth muscle contractile proteins to calcium, and it is a selective 5-HT1A receptor antagonist. Alverine, in combination with simethicone, has been demonstrated to effectively reduce abdominal pain and discomfort in a large placebo-controlled trial. Mebeverine is a musculotropic agent that potently blocks intestinal peristalsis. Non-placebo-controlled trials have shown positive effects of mebeverine in IBS regarding symptom control; nevertheless, in recent placebo-controlled studies, mebeverine did not exhibit superiority over placebo. Otilonium bromide is poorly absorbed from the GI tract, where it acts locally as an L-type calcium channel blocker, an antimuscarinic and a tachykinin NK2 receptor antagonist. Otilonium has effectively reduced pain and improved defecation alterations in placebo-controlled trials in IBS patients. Pinaverium bromide is also an L-type calcium channel blocker that acts locally in the GI tract. Pinaverium improves motility disorders and consequently reduces stool problems in IBS patients. Phloroglucinol and trimethylphloroglucinol are non-specific antispasmodics that reduced pain in IBS patients in a placebo-controlled trial. Antispasmodics have excellent safety profiles. T-type calcium channel blockers can abolish visceral hypersensitivity in animal models, which makes them potential candidates for the development of novel therapeutic agents in the

  14. [Frey syndrome secondary to submaxillectomy and botulinic treatment].

    PubMed

    Báez, Alejandra; Paleari, Julieta; Durán, María Nöel; Rudy, Tamara; Califano, Ines; Barbosa, Nicolas; Parera, Ignacio Casas

    2007-01-01

    A case of Frey syndrome (FS) secondary to submaxillar gland exeresis is presented and the results of the treatment with botulinum toxin (BTX) type A. FS is a condition of sweating cheek and preauricular area during realtime as a sequel detected in about 20-60% of patients after parotidectomy. The clinical symptoms include swelling, flushing and hyperhidrosis. The treatment choice for this condition is intracutaneous injection of BTX type A which blocks acetylcholine release at the sweat glands. A 30-year-old man, with thyroid medullar carcinoma diagnosed in 2002 received 6 cicles of cisplatin plus doxorubicin previous to the thyroidectomy with anterolateral neck dissection. During surgery the left ramus marginalis mandibulae was damaged. Two years later the patient referred sweating in submaxillar region during meals. CT scan demonstrated the absence of left submaxillar gland. Minor's test disclosed the affected area and BTX type A was injected (2.5 U/cm2/17 points). A twenty-one-day control showed a 95% reduction of the affected skin area. Persistent efficacy was observed up to one year follow-up time when he was reinjected. The FS, also known as "gustatory hyperhidrosis", was probably first reported by M. Duphenix in 1757. Lucja Frey considered its physiopathology as a disorder of both sympathetic and parasympathetic innervation. In our case the FS was caused by a misdirected regeneration of postganglionic parasympathetic nerve fibers that arrised from the nervus lingualis rami ganglionares of the nervus trigeminus. After nerve injury the colinergic parasympathetic fibers seek out colinergic receptors--sympathetic receptors of the skin--innervating sweat glands and small skin vessels. All previous cases were located at masseter region post-parotidectomy. We have not found any description of FS in the submaxillary region. The self-assessed efficacy of the treatment with a hyperhidrosis disease severity scale revealed a very satisfied patient at 20 months follow

  15. Diagnosis and treatment of acquired von Willebrand syndrome.

    PubMed

    Tiede, Andreas

    2012-12-01

    Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that is characterized by structural or functional alterations in von Willebrand factor (VWF) caused by a range of lymphoproliferative, myeloproliferative, cardiovascular, autoimmune, and other disorders. The pathogenic mechanisms responsible for the VWF abnormalities depend on the underlying condition, but include clearance due to binding of paraproteins, inhibition of VWF, adsorption to the surface of platelets, increased fluid shear stress, and resultant proteolysis or, more rarely, decreased synthesis. The diagnosis and treatment of AVWS are complicated by the need for multiple laboratory tests and the management of bleeding risk in a typically elderly population with serious underlying conditions that predispose towards thrombosis. Recently developed diagnostic algorithms, based on standard laboratory assays, may assist clinicians with the diagnostic workup and help differentiate between AVWS and von Willebrand disease (VWD) types 1 and 2. AVWS should be considered in all patients with new-onset bleeding whenever laboratory findings suggest VWD, particularly in the presence of an AVWS-associated disorder. AVWS testing is also recommended prior to surgery or an intervention with a high risk of bleeding in any individual with an AVWS-associated disorder. Treatment of the underlying condition using immunosuppressants, surgery, or chemotherapy, can lead to remission of AVWS in some individuals and should always be considered. Strategies to prevent and/or treat bleeding episodes should also be in place, including the use of VWF-containing factor VIII concentrates, desmopressin and tranexamic acid. Treatment success will depend largely on the underlying pathogenesis of the disorder. PMID:23439003

  16. Surfactant Treatment Threshold during NCPAP for the Treatment of Preterm Infants with Respiratory Distress Syndrome.

    PubMed

    Dani, Carlo

    2016-08-01

    Although surfactant is the most studied drug in the preterm infant, the best criteria for treatment of preterm infants with respiratory distress syndrome (RDS) with surfactant have been not extensively investigated. We assessed the criteria used for deciding the rescue surfactant treatment of preterm infants with RDS in combination with nasal continuous positive airway pressure as reported by the main recent randomized controlled trials. We evaluated 10 studies and found that the criteria chosen for administering selective surfactant were very heterogeneous, different types and doses of surfactant were used, and this limits their applicability in the clinical practice. In conclusion, although current data seem to suggest that low threshold is better than high threshold, additional studies are necessary to identify the most effective criteria for selective surfactant treatment of preterm infants with RDS. PMID:27120482

  17. Emerging pharmacologic treatment options for fragile X syndrome

    PubMed Central

    Schaefer, Tori L; Davenport, Matthew H; Erickson, Craig A

    2015-01-01

    Fragile X syndrome (FXS) is the most common single gene cause of intellectual disability and autism spectrum disorder. Caused by a silenced fragile X mental retardation 1 gene and the subsequent deficiency in fragile X mental retardation protein, patients with FXS experience a range of physical, behavioral, and intellectual debilitations. The FXS field, as a whole, has recently met with some challenges, as several targeted clinical trials with high expectations of success have failed to elucidate significant improvements in a variety of symptom domains. As new clinical trials in FXS are planned, there has been much discussion about the use of the commonly used clinical outcome measures, as well as study design considerations, patient stratification, and optimal age range for treatment. The evidence that modification of these drug targets and use of these failed compounds would prove to be efficacious in human clinical study were rooted in years of basic and translational research. There are questions arising as to the use of the mouse models for studying FXS treatment development. This issue is twofold: many of the symptom domains and molecular and biochemical changes assessed and indicative of efficacy in mouse model study are not easily amenable to clinical trials in people with FXS because of the intolerability of the testing paradigm or a lack of noninvasive techniques (prepulse inhibition, sensory hypersensitivity, startle reactivity, or electrophysiologic, biochemical, or structural changes in the brain); and capturing subtle yet meaningful changes in symptom domains such as sociability, anxiety, and hyperactivity in human FXS clinical trials is challenging with the currently used measures (typically parent/caregiver rating scales). Clinicians, researchers, and the pharmaceutical industry have all had to take a step back and critically evaluate the way we think about how to best optimize future investigations into pharmacologic FXS treatments. As new clinical

  18. [Interference therapy and radon baths in the combined treatment of patients with reflex cervicobrachial syndromes].

    PubMed

    Gorbunov, F E; Semenistaia, S V

    1998-01-01

    Patients with cervicobrachialgic syndromes on interference therapy, exercise treatment, massage of the cervical collar region received balneotherapy. 42 of them took water baths, 39 took dry air radon baths. These complexes proved effective in cervicobrachialgic syndromes, the effect being slightly dependent on the clinical symptoms of the disease. PMID:9987978

  19. Cavernous haemangioma with Kasabach-Merritt syndrome: treatment with alpha-interferon.

    PubMed Central

    Ettlinger, J J; Fleming, P J; Joffe, H S; Kennedy, C T

    1996-01-01

    A small number of patients with haemangioma need treatment for serious complications such as Kasabach-Merritt syndrome, cardiac failure and obstruction of the airway. We report on the management of an infant with Kasabach-Merritt syndrome. Images Figure 1 PMID:8709089

  20. Surgical treatment for mitral stenosis in Scheie's syndrome: mucopolysaccharidosis type I-S.

    PubMed

    Kitabayashi, Katsukiyo; Matsumiya, Goro; Ichikawa, Hajime; Matsue, Hajime; Shimamura, Kazuo; Sawa, Yoshiki

    2007-08-01

    Scheie's syndrome is a subtype of mucopolysaccharidosis, which is a rare hereditary disorder of proteoglycan degrading enzymes. Deposition of mucopolysaccharide often causes cardiac disease, especially valvular lesion, but reports of its surgical treatment have been rare. We report a case of 41-year-old woman with Scheie's syndrome who successfully underwent mitral valve replacement for mitral stenosis. PMID:17643657

  1. Side Effects of Minocycline Treatment in Patients with Fragile X Syndrome and Exploration of Outcome Measures

    ERIC Educational Resources Information Center

    Utari, Agustini; Chonchaiya, Weerasak; Rivera, Susan M.; Schneider, Andrea; Hagerman, Randi J.; Faradz, Sultana M. H.; Ethell, Iryna M.; Nguyen, Danh V.

    2010-01-01

    Minocycline can rescue the dendritic spine and synaptic structural abnormalities in the fragile X knock-out mouse. This is a review and preliminary survey to document side effects and potential outcome measures for minocycline use in the treatment of individuals with fragile X syndrome. We surveyed 50 patients with fragile X syndrome who received…

  2. The Efficacy of Social Skills Treatment for Children with Asperger Syndrome

    ERIC Educational Resources Information Center

    Elder, Lisa M.; Caterino, Linda C.; Chao, Janet; Shaknai, Dina; De Simone, Gina

    2006-01-01

    Children with Asperger Syndrome present with significant social skills deficits, which may contribute to clinical problems such as anxiety, depression, and/or other behavioral disorders. This article provides a description of the nature of Asperger Syndrome and provides possible treatment interventions, specifically focusing on the efficacy of…

  3. Treatment of acute thoracic aortic syndromes using endovascular techniques

    PubMed Central

    Uğuz, Emrah; Canyiğit, Murat; Hıdıroğlu, Mete; Şener, Erol

    2016-01-01

    PURPOSE Acute thoracic aortic syndrome (ATAS) is a novel term to define emergency aortic conditions with common clinical features and challenges. Traditional management of ATAS includes surgical replacement of the aorta and is correlated with high perioperative mortality and morbidity. We aimed to evaluate our experience and outcomes in patients presenting with ATAS, managed by endovascular techniques. METHODS This cohort consisted of 31 consecutive patients (24 males; mean age, 57.5±13.81 years; range, 19–84 years) with acute thoracic aortic pathologies who underwent endovascular repair between January 2011 and January 2015. The study was designed as a retrospective analysis of prospectively maintained data. RESULTS Complicated acute type-B aortic dissection was the most common pathology (35.5%). All aortic stent-grafts (n=37) and dissection stents (n=9) were implanted with 100% procedural success. The overall in-hospital mortality was 9.7%. The mean follow-up duration of patients who were alive at 30 days was 25.9±11.49 months (3–53 months). So far, there have been no late deaths after 30 days. CONCLUSION In the high-risk setting of ATAS, endovascular procedures come forward as novel therapeutic strategies with promising results. Endovascular repair of ATAS can be considered as a first-line treatment alternative under emergency conditions with encouraging results, particularly when conventional surgical repair cannot be implemented due to prohibitive comorbidities. PMID:27113420

  4. Treatment strategies for infertile women with polycystic ovary syndrome.

    PubMed

    Vitek, Wendy; Hoeger, Kathleen; Legro, Richard S

    2016-08-01

    Polycystic ovary syndrome (PCOS) is a common reproductive disorder that can be diagnosed when two of the following three criteria are present: menstrual irregularity, hyperandrogenism and polycystic ovaries. Factors such as the individual's body weight influence the severity of the phenotype and risk of metabolic comorbidities. While anovulatory infertility is a common issue among lean and obese reproductive-aged women with PCOS, obesity is associated with resistance to oral ovulation induction agents, lower pregnancy rates and a higher risk of pregnancy complications. Lifestyle modification is recommended as first line therapy among obese women with PCOS in order to optimize their outcomes. Among lean and obese women with PCOS, ovulation induction can be achieved with aromatase inhibitors, selective estrogen receptor modulators, insulin sensitizing agents, gonadotropins and ovarian drilling with varying rates of ovulation, live birth and multiple gestations. Assisted reproductive technologies are reserved for women who do not conceive despite restoration of ovulation or couples with additional factors contributing to their infertility. This review will outline treatment strategies for achieving a healthy pregnancy among lean and obese women with PCOS and infertility. PMID:26765152

  5. Nuts in the prevention and treatment of metabolic syndrome.

    PubMed

    Salas-Salvadó, Jordi; Guasch-Ferré, Marta; Bulló, Mònica; Sabaté, Joan

    2014-07-01

    Nuts are rich in many bioactive compounds that can exert beneficial effects on cardiovascular health. We reviewed the evidence relating nut consumption and the metabolic syndrome (MetS) and its components. Nuts reduce the postprandial glycemic response; however, long-term trials of nuts on insulin resistance and glycemic control in diabetic individuals are inconsistent. Epidemiologic studies have shown that nuts may lower the risk of diabetes incidence in women. Few studies have assessed the association between nuts and abdominal obesity, although an inverse association with body mass index and general obesity has been observed. Limited evidence suggests that nuts have a protective effect on blood pressure and endothelial function. Nuts have a cholesterol-lowering effect, but the relation between nuts and hypertriglyceridemia and high-density lipoprotein cholesterol is not well established. A recent pooled analysis of clinical trials showed that nuts are inversely related to triglyceride concentrations only in subjects with hypertriglyceridemia. An inverse association was found between the frequency of nut consumption and the prevalence and the incidence of MetS. Several trials evaluated the effect of nuts on subjects with MetS and found that they may have benefits in some components. Compared with a low-fat diet, a Mediterranean diet enriched with nuts could be beneficial for MetS management. The protective effects on metabolism could be explained by the modulation of inflammation and oxidation. Further trials are needed to clarify the role of nuts in MetS prevention and treatment. PMID:24898227

  6. Chronic Fatigue Syndrome: Searching for the Cause and Treatment.

    ERIC Educational Resources Information Center

    Eichner, Edward R.

    1989-01-01

    Chronic fatigue syndrome became known nationally in l985 with a pseudoepidemic in a Nevada resort community. Initially and erroneously linked to the Epstein-Barr virus, the cause of this puzzling syndrome and the mind-body connection are areas of controversy and research. (Author/SM)

  7. Unrecognized acute exertional compartment syndrome of the leg and treatment.

    PubMed

    Popovic, Nebojsa; Bottoni, Craig; Cassidy, Charles

    2011-04-01

    Acute-on-chronic exertional compartment syndrome is rare and may be easily missed without a high degree of awareness and clinical suspicion. We report a case of unrecognized acute-on-chronic exertional compartment syndrome in a recreational soccer player. The late sequela of this condition, foot drop, was successfully treated with transfer of the peroneus longus tendon. PMID:21667742

  8. Treatment of abdominal pain in irritable bowel syndrome.

    PubMed

    Vanuytsel, Tim; Tack, Jan F; Boeckxstaens, Guy E

    2014-08-01

    Functional abdominal pain in the context of irritable bowel syndrome (IBS) is a challenging problem for primary care physicians, gastroenterologists and pain specialists. We review the evidence for the current and future non-pharmacological and pharmacological treatment options targeting the central nervous system and the gastrointestinal tract. Cognitive interventions such as cognitive behavioral therapy and hypnotherapy have demonstrated excellent results in IBS patients, but the limited availability and labor-intensive nature limit their routine use in daily practice. In patients who are refractory to first-line therapy, tricyclic antidepressants (TCA) and selective serotonin reuptake inhibitors are both effective to obtain symptomatic relief, but only TCAs have been shown to improve abdominal pain in meta-analyses. A diet low in fermentable carbohydrates and polyols (FODMAP) seems effective in subgroups of patients to reduce abdominal pain, bloating, and to improve the stool pattern. The evidence for fiber is limited and only isphagula may be somewhat beneficial. The efficacy of probiotics is difficult to interpret since several strains in different quantities have been used across studies. Antispasmodics, including peppermint oil, are still considered the first-line treatment for abdominal pain in IBS. Second-line therapies for diarrhea-predominant IBS include the non-absorbable antibiotic rifaximin and the 5HT3 antagonists alosetron and ramosetron, although the use of the former is restricted because of the rare risk of ischemic colitis. In laxative-resistant, constipation-predominant IBS, the chloride-secretion stimulating drugs lubiprostone and linaclotide, a guanylate cyclase C agonist that also has direct analgesic effects, reduce abdominal pain and improve the stool pattern. PMID:24845149

  9. Vincristine treatment in steroid-dependent nephrotic syndrome.

    PubMed

    Kausman, Joshua Yehuda; Yin, Lei; Jones, Colin Lindsay; Johnstone, Lillian; Powell, Harley Robert

    2005-10-01

    Treatment of children with steroid-dependent nephrotic syndrome (SDNS) continues to be a challenge when relapses recur after treatment with cyclophosphamide and side effects or non-compliance make steroids and cyclosporin unsatisfactory. We treated 12 patients with intravenous vincristine for SDNS in a regime of 1-1.5 mg/m2 weekly for 4 weeks then monthly for 4 months. Four of the 5 patients in relapse when commencing vincristine remitted within 2 doses. Comparing relapse frequency in the 12 months before and after vincristine, there was a reduction from 4 to 1.5 (p=0.004) relapses per year. Median sustained remission was 5 months, but 1 frequently relapsing patient remains in remission 4 years after vincristine. Vincristine was also successfully used in 1 or 2 doses at weekly intervals for subsequent relapses in 5 patients. Side effects were minimal in most cases. Abdominal pain occurred in 2 patients who commenced vincristine at 1.5 mg/m2, but resolved when continued at 1 mg/m2. We felt vincristine had a role in a subset of children with challenging SDNS administered as 1 mg/m2 weekly for 4 weeks then 1.5 mg/m2 monthly for 4 months. Vincristine allowed steroid- and cyclosporin-sparing, contributed to long-term remission in some patients, and was especially valuable in children with poor compliance with oral medication. Many patients expressed a preference for a few doses of vincristine rather than a standard course of oral prednisolone or cyclosporin. PMID:15977025

  10. Treatment of carpal tunnel syndrome with alpha-lipoic acid.

    PubMed

    Di Geronimo, G; Caccese, A Fonzone; Caruso, L; Soldati, A; Passaretti, U

    2009-01-01

    Carpal Tunnel Syndrome (CTS) is the most common peripheral mononeuropathy; its symptoms and functional limitations significantly penalize the daily activities and quality of life of many people. While surgery is reserved to most severe cases, the earlier stages of disease may be controlled by a pharmacological treatment aimed to "neuroprotection", i.e. to limiting and correcting the nerve damage. Our study was aimed to compare the efficacy of a fixed association of alpha-lipoic acid (ALA) 600 mg/die and gamma-linolenic acid (GLA) 360 mg/die, and a multivitamin B preparation (Vit B6 150 mg, Vit B1 100 mg, Vit B12 500 microg daily) for 90 days in 112 subjects with moderately severe CTS. Demographic, case-history and treatment efficacy data were collected; the Boston questionnaire was administered and the patients were evaluated by Hi-Ob scale and electro-myography. A significant reduction in both symptoms scores and functional impairment (Boston questionnaire) was observed in ALA/GLA group, while the multivitamin group experienced a slight improvement of symptoms and a deterioration of functional scores. Electromyography showed a statistically significant improvement with ALA/GLA, but not with the multivitamin product. The Hi-Ob scale showed significant efficacy of ALA/GLA in improving symptoms and functional impairment, while in the multivitamin group the improvement was significant, but less marked than in the ALA/GLA group. In conclusion, the fixed association of ALA and GLA proved to be a useful tool and may be proposed for controlling symptoms and improving the evolution of CTS, especially in the earlier stages of disease. PMID:19499849

  11. Autoimmune Syndromes Presenting as a Paraneoplastic Manifestation of Myelodysplastic Syndromes: Clinical Features, Course, Treatment and Outcome.

    PubMed

    Williamson, Bradley T; Foltz, Lynda; Leitch, Heather A

    2016-05-10

    Autoimmune manifestations (AIM) are reported in up to 10-30% of myelodysplastic syndromes (MDS) patients; this association is not well defined. We present herein a retrospective chart review of single center MDS patients for AIM, a case discussion and a literature review. Of 252 MDS patients examined, 11 (4.4%) had AIM around MDS diagnosis. International Prognostic Scoring System scores were: low or intermediate (int)-1 (n=7); int-2 or high (n=4). AIM were: culture negative sepsis (n=7); inflammatory arthritis (n=3); vasculitis (n=4); sweats; pericarditis; polymyalgia rheumatica (n=2 each); mouth ulcers; pulmonary infiltrates; suspicion for Behcet's; polychondritis and undifferentiated (n=1 each). AIM treatment and outcome were: prednisone +/- steroid sparing agents, n=8, ongoing symptoms in 5; azacitidine (n=3), 2 resolved; and observation, n=1, ongoing symptoms. At a median follow up of 13 months, seven patients are alive. In summary, 4.4% of MDS patients presented with concomitant AIM. MDS should remain on the differential diagnosis of patients with inflammatory symptoms. PMID:27499837

  12. Autoimmune Syndromes Presenting as a Paraneoplastic Manifestation of Myelodysplastic Syndromes: Clinical Features, Course, Treatment and Outcome

    PubMed Central

    Williamson, Bradley T.; Foltz, Lynda; Leitch, Heather A.

    2016-01-01

    Autoimmune manifestations (AIM) are reported in up to 10-30% of myelodysplastic syndromes (MDS) patients; this association is not well defined. We present herein a retrospective chart review of single center MDS patients for AIM, a case discussion and a literature review. Of 252 MDS patients examined, 11 (4.4%) had AIM around MDS diagnosis. International Prognostic Scoring System scores were: low or intermediate (int)-1 (n=7); int-2 or high (n=4). AIM were: culture negative sepsis (n=7); inflammatory arthritis (n=3); vasculitis (n=4); sweats; pericarditis; polymyalgia rheumatica (n=2 each); mouth ulcers; pulmonary infiltrates; suspicion for Behcet’s; polychondritis and undifferentiated (n=1 each). AIM treatment and outcome were: prednisone +/- steroid sparing agents, n=8, ongoing symptoms in 5; azacitidine (n=3), 2 resolved; and observation, n=1, ongoing symptoms. At a median follow up of 13 months, seven patients are alive. In summary, 4.4% of MDS patients presented with concomitant AIM. MDS should remain on the differential diagnosis of patients with inflammatory symptoms.

  13. Irritable Bowel Syndrome Treatments Aren't One-Size-Fits-All

    MedlinePlus

    ... Bowel Syndrome Treatments Aren't One-Size-Fits-All Share Tweet Linkedin Pin it More sharing options ... the United States. No one remedy works for all patients, so there’s a great medical need to ...

  14. Holmium laser use in the treatment of selected dry eye syndrome complications

    NASA Astrophysics Data System (ADS)

    Kecik, Dariusz; Kecik, Tadeusz; Kasprzak, Jan; Kecik, Mariusz

    1996-03-01

    The authors present initial results of treatment selected complications of dry eye syndrome with holmium laser. The lacrimal puncta obliteration and coagulation of the corneal ulcer surface were done.

  15. Corticosteroid Injections Versus Manual Physical Therapy for Treatment of the Shoulder Impingement Syndrome

    MedlinePlus

    ... Medicine Summaries for Patients Corticosteroid Injections Versus Manual Physical Therapy for Treatment of the Shoulder Impingement Syndrome The ... Outcome of Subacromial Corticosteroid Injection Compared With Manual Physical Therapy for the Management of the Unilateral Shoulder Impingement ...

  16. Complex regional pain syndrome: observations on diagnosis, treatment and definition of a new subgroup.

    PubMed

    Żyluk, A; Puchalski, P

    2013-07-01

    Several definitions and sets of diagnostic criteria of complex regional pain syndrome have been proposed, but to date none has been accepted completely. This article presents a specific subtype of the disease, called 'chronic, refractory complex regional pain syndrome' which is extremely severe, disabling and resistant to treatment. It also emphasizes difficulties with diagnosing complex regional pain syndrome because of its variable clinical presentation and diagnostic criteria being insufficiently precise. The necessity to distinguish between criteria for clinical use and for scientific purposes is suggested with a proposal of practical guideline for diagnosing acute complex regional pain syndrome. A review of treatments for complex regional pain syndrome is presented, with opinion on their effectiveness: good in an early stage, less well in chronic and generally poor in the chronic, refractory subtype. PMID:23221182

  17. Targeted pharmacological treatment of autism spectrum disorders: fragile X and Rett syndromes

    PubMed Central

    Wang, Hansen; Pati, Sandipan; Pozzo-Miller, Lucas; Doering, Laurie C.

    2015-01-01

    Autism spectrum disorders (ASDs) are genetically and clinically heterogeneous and lack effective medications to treat their core symptoms. Studies of syndromic ASDs caused by single gene mutations have provided insights into the pathophysiology of autism. Fragile X and Rett syndromes belong to the syndromic ASDs in which preclinical studies have identified rational targets for drug therapies focused on correcting underlying neural dysfunction. These preclinical discoveries are increasingly translating into exciting human clinical trials. Since there are significant molecular and neurobiological overlaps among ASDs, targeted treatments developed for fragile X and Rett syndromes may be helpful for autism of different etiologies. Here, we review the targeted pharmacological treatment of fragile X and Rett syndromes and discuss related issues in both preclinical studies and clinical trials of potential therapies for the diseases. PMID:25767435

  18. Crouzon syndrome: Virtual planning of surgical treatment by application of internal distractors.

    PubMed

    Rahimov, Chingiz; Asadov, Ruslan; Hajiyeva, Gunel; Verdiyev, Nazim; Novruzov, Zaur; Farzaliyev, Ismayil

    2016-01-01

    Crouzon syndrome is one of the frequent pathologies within craniosynostosis syndrome. Current progress in computers and biotechnologies allows improving surgical approach and forecasting final result of reconstruction as well. We present a case of successful surgical treatment of Crouzon syndrome, done by application of virtual planning allowing determining "monobloc" features, type of reconstruction and distraction protocol as well. A 20-year-old female had presented with craniofacial deformity. Clinical and radiological investigation revealed Crouzon syndrome. The "monobloc" creation, cranioplasty and internal distractors positioning, direction and schedule of advancement were done according to preoperative virtual planning data achieved by Materialise Mimics Research software. Nine months postoperative functional and esthetic result and radiological findings showed to be reasonable. That application of virtual simulation significantly allows to determine best direction of distraction and improves postoperative outcomes of surgical treatment of Crouzon syndrome. PMID:27563623

  19. Targeted pharmacological treatment of autism spectrum disorders: fragile X and Rett syndromes.

    PubMed

    Wang, Hansen; Pati, Sandipan; Pozzo-Miller, Lucas; Doering, Laurie C

    2015-01-01

    Autism spectrum disorders (ASDs) are genetically and clinically heterogeneous and lack effective medications to treat their core symptoms. Studies of syndromic ASDs caused by single gene mutations have provided insights into the pathophysiology of autism. Fragile X and Rett syndromes belong to the syndromic ASDs in which preclinical studies have identified rational targets for drug therapies focused on correcting underlying neural dysfunction. These preclinical discoveries are increasingly translating into exciting human clinical trials. Since there are significant molecular and neurobiological overlaps among ASDs, targeted treatments developed for fragile X and Rett syndromes may be helpful for autism of different etiologies. Here, we review the targeted pharmacological treatment of fragile X and Rett syndromes and discuss related issues in both preclinical studies and clinical trials of potential therapies for the diseases. PMID:25767435

  20. Crouzon syndrome: Virtual planning of surgical treatment by application of internal distractors

    PubMed Central

    Rahimov, Chingiz; Asadov, Ruslan; Hajiyeva, Gunel; Verdiyev, Nazim; Novruzov, Zaur; Farzaliyev, Ismayil

    2016-01-01

    Crouzon syndrome is one of the frequent pathologies within craniosynostosis syndrome. Current progress in computers and biotechnologies allows improving surgical approach and forecasting final result of reconstruction as well. We present a case of successful surgical treatment of Crouzon syndrome, done by application of virtual planning allowing determining “monobloc” features, type of reconstruction and distraction protocol as well. A 20-year-old female had presented with craniofacial deformity. Clinical and radiological investigation revealed Crouzon syndrome. The “monobloc” creation, cranioplasty and internal distractors positioning, direction and schedule of advancement were done according to preoperative virtual planning data achieved by Materialise Mimics Research software. Nine months postoperative functional and esthetic result and radiological findings showed to be reasonable. That application of virtual simulation significantly allows to determine best direction of distraction and improves postoperative outcomes of surgical treatment of Crouzon syndrome.

  1. Prosthodontic treatment and medical considerations for a patient with Turner syndrome: a clinical report.

    PubMed

    Nguyen, Caroline T; Hofstede, Theresa M

    2012-10-01

    This clinical report describes a multidisciplinary approach in the rehabilitation of a 23-year-old Caucasian woman affected with Turner's syndrome and subsequently diagnosed with T4 Giant cell reparative granuloma of the right maxillary sinus. The surgical treatment included a maxillectomy and infratemporal fossa dissection followed by a free fibula palatal reconstruction, fibula bone graft of the orbital floor, dental implant placement, and prosthodontic rehabilitation. Prosthodontic planning and treatment considerations in an adult patient with Turner Syndrome are discussed. PMID:22672559

  2. Rituximab in the treatment of shrinking lung syndrome in systemic lupus erythematosus.

    PubMed

    Peñacoba Toribio, Patricia; Córica Albani, María Emilia; Mayos Pérez, Mercedes; Rodríguez de la Serna, Arturo

    2014-01-01

    Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. We report the case of a patient with non-responding SLS (neither to glucocorticoids nor immunosupresors), who showed remarkable improvement after the onset of treatment with rituximab. Although there is a little evidence, treatment with rituximab could be proposed in SLS when classical treatment fails. PMID:24315464

  3. Nutritional strategies in the prevention and treatment of metabolic syndrome.

    PubMed

    Feldeisen, Sabrina E; Tucker, Katherine L

    2007-02-01

    The metabolic syndrome (MetS) is a clustering of metabolic abnormalities that increase the risk of developing atherosclerotic cardiovascular disease and type 2 diabetes. The exact etiology remains unclear, but it is known to be a complex interaction between genetic, metabolic, and environmental factors. Among environmental factors, dietary habits are of central importance in the prevention and treatment of this condition. However, there is currently no firm consensus on the most appropriate dietary recommendations. General recommendations include decreasing obesity, increasing physical activity, and consuming an anti-atherogenic diet, and have traditionally focused on low total fat intake. A major problem with the focus on low fat is that high-carbohydrate diets can contribute to increasing triglyceride and decreasing high-density lipoprotein (HDL) concentrations. Low-carbohydrate diets have been popular in recent years. However, such diets are typically higher in saturated fat and lower in fruits, vegetables, and whole grains than national dietary recommendations. More recently the quality of carbohydrate has been studied in relation to MetS, including a focus on dietary fiber and glycemic index. Similarly, there has been a move from limiting total fat to a focus on the quality of the fat, with evidence of beneficial effects of replacing some carbohydrate with monounsaturated fat. Other nutrients examined for possible importance include calcium, vitamin D, and magnesium. Together, the evidence suggests that the components of diet currently recommended as "healthy" are likely also protective against MetS, including low saturated and trans fat (rather than low total fat) and balanced carbohydrate intake rich in dietary fiber, as well as high fruit and vegetable intake (rather than low total carbohydrate); and the inclusion of low-fat dairy foods. Accelerating research on gene-diet interactions is likely to contribute interesting information that may lead to further

  4. Treatment of macroglossia in Beckwith-Wiedemann syndrome.

    PubMed

    Clauser, Luigi; Tieghi, Riccardo; Polito, Jessica

    2006-03-01

    A case of macroglossia caused by Beckwith Wiedemann syndrome is reported. Beckwith-Wiedemann Syndrome is an overgrowth disorder characterized by a constellation of congenital anomalies. The most common manifestations are omphalocele, macroglossia, gigantism, and visceromegaly. When the tongue reaches a huge dimension, clinical symptoms are represented by dysphagia, alterations in speech, difficulty in chewing, obstruction of the upper airways, and psychologic consequences derived from the patient's physical appearance. The authors describe the surgical strategy performed in the reported case. PMID:16633192

  5. MELAS syndrome: Clinical manifestations, pathogenesis, and treatment options.

    PubMed

    El-Hattab, Ayman W; Adesina, Adekunle M; Jones, Jeremy; Scaglia, Fernando

    2015-01-01

    Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is one of the most frequent maternally inherited mitochondrial disorders. MELAS syndrome is a multi-organ disease with broad manifestations including stroke-like episodes, dementia, epilepsy, lactic acidemia, myopathy, recurrent headaches, hearing impairment, diabetes, and short stature. The most common mutation associated with MELAS syndrome is the m.3243A>G mutation in the MT-TL1 gene encoding the mitochondrial tRNA(Leu(UUR)). The m.3243A>G mutation results in impaired mitochondrial translation and protein synthesis including the mitochondrial electron transport chain complex subunits leading to impaired mitochondrial energy production. The inability of dysfunctional mitochondria to generate sufficient energy to meet the needs of various organs results in the multi-organ dysfunction observed in MELAS syndrome. Energy deficiency can also stimulate mitochondrial proliferation in the smooth muscle and endothelial cells of small blood vessels leading to angiopathy and impaired blood perfusion in the microvasculature of several organs. These events will contribute to the complications observed in MELAS syndrome particularly the stroke-like episodes. In addition, nitric oxide deficiency occurs in MELAS syndrome and can contribute to its complications. There is no specific consensus approach for treating MELAS syndrome. Management is largely symptomatic and should involve a multidisciplinary team. Unblinded studies showed that l-arginine therapy improves stroke-like episode symptoms and decreases the frequency and severity of these episodes. Additionally, carnitine and coenzyme Q10 are commonly used in MELAS syndrome without proven efficacy. PMID:26095523

  6. Hemoptysis Complicating Scimitar Syndrome: From Diagnosis to Treatment

    SciTech Connect

    Nedelcu, Cosmina; Carette, Marie-France; Parrot, Antoine; Hammoudi, Nadjib; Marsault, Claude; Khalil, Antoine

    2008-07-15

    We report the case of a 24-year-old patient with known scimitar syndrome presenting with hemoptysis. Multidetector CT angiography showed the scimitar vein draining the right lung to the inferior vena cava associated with right lower lobe bronchopulmonary sequestration. The presence of hemoptysis associated with scimitar syndrome is likely secondary to an anomalous systemic arterial blood supply. We treated this patient successfully with transcatheter occlusion of the anomalous feeding arterial vessels.

  7. Research Thinking of Low-intensity laser For the Treatment of Menopausal Syndrome

    NASA Astrophysics Data System (ADS)

    Chen, G. Z.; Xu, Y. X.; Wang, X. Y.; Liu, S. H.; Li, L. J.

    2011-02-01

    Female climacteric syndrome is a clinical syndrome due to autonomic nerve dysfunction occurring in women during climacteric period, which may affect their physical and mental health. Therefore, how to pass climacteric period for women without any problems, avoid or reduce the occurrence of climacteric syndrome, prevent geriatric diseases and improve life quality is a key issue now for great attention. Looking for a convenient, effective, and safer method without toxic-side effects to control the disease is a modern medical problem. By analyzing the relationship between laser technology and traditional acupuncture and moxibustion, the advantage and the existing problems on acupuncture and moxibustion for the treatment of menopausal syndrome, the application of laser methods for the mechanism research on TCM diagnosis and treatment of menopausal syndrome was discussed. It's pointed out that the laser acupuncture is safe and effective to treat menopausal syndrome. Breakthrough will be achieved from the research of the selection of the acupoint prescription and mechanism of Acupuncture and Moxibustion for the treatment of menopausal syndrome by utilizing the advantage of interdisciplinary intersection. Laser technology will make the development of acupuncture and moxibustion science possess an unprecedented field.

  8. Seizure treatment in Angelman syndrome: A case series from the Angelman Syndrome Clinic at Massachusetts General Hospital.

    PubMed

    Shaaya, Elias A; Grocott, Olivia R; Laing, Olivia; Thibert, Ronald L

    2016-07-01

    Epilepsy is a common feature of Angelman syndrome (~80-90%), with the most common seizure types including myoclonic, atonic, atypical absence, focal, and generalized tonic-clonic. Seizure types are similar among the various genetic subtypes, but epilepsy in those with maternal deletions is more frequent and more refractory to medication. Treatment with older antiepileptic drugs such as valproic acid and clonazepam is effective, but these medications tend to have less favorable side effect profiles in Angelman syndrome compared with those in newer medications. This study aimed to assess the use of newer antiepileptic drug therapies in individuals with Angelman syndrome followed at the Angelman Syndrome Clinic at the Massachusetts General Hospital. Many of the subjects in this study were on valproic acid therapy prior to their initial evaluation and exhibited increased tremor, decreased balance, and/or regression of motor skills, which resolved after tapering off of this medication. Newer antiepileptic drugs such as levetiracetam, lamotrigine, and clobazam, and to a lesser extent topiramate, appeared to be as effective - if not more so - as valproic acid and clonazepam while offering more favorable side effect profiles. The low glycemic index treatment also provided effective seizure control with minimal side effects. The majority of subjects remained on combination therapy with levetiracetam, lamotrigine, and clobazam being the most commonly used medications, indicating a changing trend when compared with prior studies. PMID:27206232

  9. Effectiveness of conservative treatments for the lumbosacral radicular syndrome: a systematic review

    PubMed Central

    Verhagen, Arianne P.; Ostelo, Raymond W. J. G.; van Os, Ton A. G.; Peul, Wilco C.; Koes, Bart W.

    2007-01-01

    Patients with a lumbosacral radicular syndrome are mostly treated conservatively first. The effect of the conservative treatments remains controversial. To assess the effectiveness of conservative treatments of the lumbosacral radicular syndrome (sciatica). Relevant electronic databases and the reference lists of articles up to May 2004 were searched. Randomised clinical trials of all types of conservative treatments for patients with the lumbosacral radicular syndrome selected by two reviewers. Two reviewers independently assessed the methodological quality and the clinical relevance. Because the trials were considered heterogeneous we decided not to perform a meta-analysis but to summarise the results using the rating system of levels of evidence. Thirty trials were included that evaluated injections, traction, physical therapy, bed rest, manipulation, medication, and acupuncture as treatment for the lumbosacral radicular syndrome. Because several trials indicated no evidence of an effect it is not recommended to use corticosteroid injections and traction as treatment option. Whether clinicians should prescribe physical therapy, bed rest, manipulation or medication could not be concluded from this review. At present there is no evidence that one type of treatment is clearly superior to others, including no treatment, for patients with a lumbosacral radicular syndrome. PMID:17415595

  10. The metabolic vascular syndrome - guide to an individualized treatment.

    PubMed

    Hanefeld, Markolf; Pistrosch, Frank; Bornstein, Stefan R; Birkenfeld, Andreas L

    2016-03-01

    In ancient Greek medicine the concept of a distinct syndrome (going together) was used to label 'a group of signs and symptoms' that occur together and 'characterize a particular abnormality and condition'. The (dys)metabolic syndrome is a common cluster of five pre-morbid metabolic-vascular risk factors or diseases associated with increased cardiovascular morbidity, fatty liver disease and risk of cancer. The risk for major complications such as cardiovascular diseases, NASH and some cancers develops along a continuum of risk factors into clinical diseases. Therefore we still include hyperglycemia, visceral obesity, dyslipidemia and hypertension as diagnostic traits in the definition according to the term 'deadly quartet'. From the beginning elevated blood pressure and hyperglycemia were core traits of the metabolic syndrome associated with endothelial dysfunction and increased risk of cardiovascular disease. Thus metabolic and vascular abnormalities are in extricable linked. Therefore it seems reasonable to extend the term to metabolic-vascular syndrome (MVS) to signal the clinical relevance and related risk of multimorbidity. This has important implications for integrated diagnostics and therapeutic approach. According to the definition of a syndrome the rapid global rise in the prevalence of all traits and comorbidities of the MVS is mainly caused by rapid changes in life-style and sociocultural transition resp. with over- and malnutrition, low physical activity and social stress as a common soil. PMID:26956847

  11. Improving the understanding of Takotsubo syndrome: consequences of diagnosis and treatment.

    PubMed

    Tanabe, Yasuhiro; Akashi, Yoshihiro J

    2016-06-01

    Takotsubo syndrome was first described in Japan in 1990. Although it has gained worldwide recognition in the last 25 years, the pathophysiology of Takotsubo syndrome remains controversial. This syndrome is often reported in elderly postmenopausal women suffering from sudden, unexpected emotional and physical stress. Its clinical presentation resembles that of acute myocardial infarction. The prognosis of Takotsubo syndrome was initially considered favorable compared with that of myocardial infarction; however, subsequent studies have demonstrated that the mortality was higher than previously reported. Prompt diagnosis followed by appropriate management for acute complications such as heart failure, arrhythmia, intraventricular thrombus, and left ventricular outflow tract obstruction is required. We discuss the current knowledge on Takotsubo syndrome to facilitate optimal diagnosis and treatment. PMID:26840569

  12. International Guidelines for the Management and Treatment of Morquio A Syndrome

    PubMed Central

    Hendriksz, Christian J; Berger, Kenneth I; Giugliani, Roberto; Harmatz, Paul; Kampmann, Christoph; Mackenzie, William G; Raiman, Julian; Villarreal, Martha Solano; Savarirayan, Ravi

    2015-01-01

    Morquio A syndrome (mucopolysaccharidosis IVA) is a lysosomal storage disorder associated with skeletal and joint abnormalities and significant non-skeletal manifestations including respiratory disease, spinal cord compression, cardiac disease, impaired vision, hearing loss, and dental problems. The clinical presentation, onset, severity and progression rate of clinical manifestations of Morquio A syndrome vary widely between patients. Because of the heterogeneous and progressive nature of the disease, the management of patients with Morquio A syndrome is challenging and requires a multidisciplinary approach, involving an array of specialists. The current paper presents international guidelines for the evaluation, treatment and symptom-based management of Morquio A syndrome. These guidelines were developed during two expert meetings by an international panel of specialists in pediatrics, genetics, orthopedics, pulmonology, cardiology, and anesthesia with extensive experience in managing Morquio A syndrome. © 2014 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals, Inc. PMID:25346323

  13. Treatment of compartment syndrome of the thigh associated with acute renal failure after the Wenchuan earthquake.

    PubMed

    Duan, Xin; Zhang, Kaiwei; Zhong, Gang; Cen, Shiqiang; Huang, Fuguo; Lv, Jingtong; Xiang, Zhou

    2012-04-01

    Compartment syndrome of the thigh is a rare emergency often treated operatively. The purpose of this study was to evaluate the effects of nonoperative treatment for compartment syndrome of the thigh associated with acute renal failure after the 2008 Wenchuan earthquake. Nonoperative treatment, which primarily involves continuous renal replacement therapy, was performed in 6 patients (3 men and 3 women) who presented with compartment syndrome of the thigh associated with acute renal failure. The mean mangled extremity severity score (MESS) and laboratory data regarding renal function were analyzed before and after treatment, and the clinical outcome was evaluated at 17-month follow-up. Laboratory data regarding renal function showed improvements. All 6 patients survived with the affected lower limbs intact after nonoperative treatment. Follow-up revealed active knee range of motion and increased muscle strength, as well as a recovery of sensation. A positive linear correlation was found between MESS and the time required to achieve a reduction in swelling, as well as the time required for the recovery of sensation and knee range of motion (r>0.8; P<.05). Satisfactory clinical outcomes were obtained in patients with compartment syndrome of the thigh associated with acute renal failure.Urine alkalization, electrolyte and water balance, and continuous renal replacement therapy have played an important role in saving lives and extremities. Nonoperative treatment should be considered in the treatment of compartment syndrome of the thigh associated with acute renal failure. PMID:22495847

  14. [Primary "pseudotumor cerebri" syndrome or idiopathic intracranial hypertension: clinical features and treatment].

    PubMed

    Timmermans, G; Scholtes, F; Andris, C; Martin, D

    2015-10-01

    "Pseudotumor cerebri" generally refers to a syndrome associating signs and symptoms of intracranial hypertension, increased cerebrospinal fluid (CSF) pressure and normal CSF composition, without any identifiable intracranial abnormality, particularly by neuroimaging studies. Although the "idiopathic" variant of the syndrome is most common, there are secondary forms where a cause can be found. The term "benign intracranial hypertension" should be abandoned, since permanent visual impairment can complicate the condition. This disaster can be avoided by early recognition and medical or surgical treatment of the disease. This article discusses the terminology, as well as diagnostic and therapeutic aspects of the syndrome. PMID:26727837

  15. Surgical treatment for Scheie's syndrome (mucopolysaccharidosis type I-S): report of two cases.

    PubMed

    Minakata, K; Konishi, Y; Matsumoto, M; Miwa, S

    1998-09-01

    Scheie's syndrome (mucopolysaccharidosis type I-S) is a rare genetic lysosomal storage disease affecting mucopolysaccharide metabolism, and is known to include cardiovascular disease. Surgical treatment was carried out in 2 patients with Scheie's syndrome. Patient 1 was a 56-year-old man with triple-vessel coronary artery disease, who successfully underwent coronary artery bypass grafting. Patient 2 was a 52-year-old man with aortic and mitral valve stenosis, who successfully underwent combined aortic and mitral valve replacement. The literature on Scheie's syndrome associated with valvular and coronary artery disease is also reviewed. PMID:9766711

  16. Reversible Posterior Leukoencephalopathy Syndrome Associated with Treatment for Acute Exacerbation of Ulcerative Colitis.

    PubMed

    Kikuchi, Shinsuke; Orii, Fumika; Maemoto, Atsuo; Ashida, Toshifumi

    2016-01-01

    Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome of varying etiologies with similar neuroimaging findings. This is a case report of a 25-year-old woman who developed typical, neurological symptoms and magnetic resonance imaging abnormalities after treatment for the acute exacerbation of ulcerative colitis (UC), which included blood transfusion, the systemic administration of prednisolone, and the administration of metronidazole. It has been reported that these treatments may contribute to the development of RPLS. RPLS should therefore be considered in the differential diagnosis of UC patients who exhibit impaired consciousness, seizures or visual deficits during treatment. We report a rare case of RPLS in a patient with UC. PMID:26935366

  17. Successful Treatment of Suspected Cannabinoid Hyperemesis Syndrome Using Haloperidol in the Outpatient Setting.

    PubMed

    Jones, Jennifer L; Abernathy, Karen E

    2016-01-01

    Chronic use of cannabis can result in a syndrome of hyperemesis characterized by cyclical vomiting without any other identifiable causes. Cannabinoid hyperemesis syndrome (CHS) is seldom responsive to traditional antiemetic therapies. Despite frequent nausea and vomiting, patients may be reluctant to discontinue use of cannabis. We report a case of severe, refractory CHS with complete resolution of nausea and vomiting after treatment with haloperidol in the outpatient setting. After review of the literature, we believe this is the first reported successful outpatient treatment of CHS and suggests a potential treatment for refractory patients. PMID:27597918

  18. Successful Treatment of Suspected Cannabinoid Hyperemesis Syndrome Using Haloperidol in the Outpatient Setting

    PubMed Central

    Abernathy, Karen E.

    2016-01-01

    Chronic use of cannabis can result in a syndrome of hyperemesis characterized by cyclical vomiting without any other identifiable causes. Cannabinoid hyperemesis syndrome (CHS) is seldom responsive to traditional antiemetic therapies. Despite frequent nausea and vomiting, patients may be reluctant to discontinue use of cannabis. We report a case of severe, refractory CHS with complete resolution of nausea and vomiting after treatment with haloperidol in the outpatient setting. After review of the literature, we believe this is the first reported successful outpatient treatment of CHS and suggests a potential treatment for refractory patients. PMID:27597918

  19. A follow-up study of patients with Dhat syndrome: Treatment pattern, outcome, and reasons for dropout from treatment

    PubMed Central

    Grover, Sandeep; Gupta, Sunil; Avasthi, Ajit

    2016-01-01

    Aim: The aim of this study was to evaluate the treatment pattern and satisfaction with treatment provided to patients with Dhat syndrome. It was also aimed to study the follow-up rates and reasons for dropping out of treatment in patients with Dhat syndrome. Materials and Methods: Sixty-four subjects diagnosed with Dhat syndrome were prospectively contacted to evaluate treatment satisfaction and reason for dropout after 6 months of baseline evaluation. Sociodemographic, clinical details were recorded at initial intake and Sex Knowledge and Attitude Questionnaire was applied. After 6 months, information on treatment received, number of follow-up visits to the clinic and the outcome were extracted from the treatment records. Treatment satisfaction using Patient Satisfaction Questionnaire and reasons for dropping out from treatment were assessed by a telephonic interview. Results: Twenty-three patients were categorized as treatment completers, 14 as early drop-outs and 27 as late drop-out. The mean (standard deviation) number of visits over the period of 6 months was 3.81 (3.06). The outcome at 6 months was no change in 45.3%, improved in 32.8% and recovered in 21.9%. Higher proportion of treatment completers (52.2%) sought psychiatric help on their own compared to those who dropped out early from the treatment (7.1%). Treatment completers had better knowledge, and more positive attitude toward sex compared to late drop-out group. 34.4%of the subjects were fully satisfied with the various components of treatment. Level of satisfaction was highest for treatment completers. The most common reasons given by those who dropped out early were “not able to spare time for consultation” (21.4%) and “not prescribed medications” (21.4%). The most common reason given by those belonging to “late drop-out” group was ‘no improvement with treatment in symptoms of Dhat syndrome (40.7%). Conclusions: Patients with Dhat syndrome frequently drop-out of the treatment network

  20. Toad Intoxication in the Dog by Rhinella marina : The Clinical Syndrome and Current Treatment Recommendations.

    PubMed

    Johnnides, Stephanie; Green, Tiffany; Eubig, Paul

    2016-01-01

    Oral exposure to the secretions of Rhinella marina (formerly Bufo marinus ) can carry a high fatality rate without early and appropriate treatment. In dogs, the clinical syndrome, which is evident almost immediately, manifests in profuse ptyalism along with gastrointestinal, respiratory, and neurologic signs. Severe cardiac arrhythmias develop less frequently. This review will cover the history, toxicology, and clinical syndrome of Rhinella marina intoxication, and will discuss the recommended therapies for stabilization. PMID:27259028

  1. An Overview of Assessment and Treatment of Premenstrual Syndrome.

    ERIC Educational Resources Information Center

    Neimeyer, Greg J.; Kosch, Shae Graham

    1988-01-01

    Notes that premenstrual syndrome (PMS) is a commonly occurring problem that must be dealt with by both the helping and the health professions. Provides a brief update on recent advances in understanding and treating PMS. Emphasizes points of contact among various health professionals in developing an interdisciplinary approach to intervention.…

  2. Recognizing Family Dynamics in the Treatment of Chronic Fatigue Syndrome

    ERIC Educational Resources Information Center

    Sperry, Len

    2012-01-01

    Chronic fatigue syndrome (CFS) is an increasingly common chronic medical condition that affects not only patients but also their families. Because family dynamics, particularly the family life cycle, can and does influence the disease process, those providing counseling to CFS patients and their families would do well to recognize these dynamics.…

  3. Kabuki Syndrome and Anorectal Malformations: Implications for Diagnosis and Treatment

    PubMed Central

    Siminas, Sotirios; Baillie, Colin Tennant; Turnock, Richard

    2015-01-01

    Kabuki syndrome (KS) is a rare genetic condition characterized by a distinctive facies, intellectual disability, growth delay, and a variety of skeletal, visceral, and other anomalies, including anorectal malformations (ARMs). We present two cases of female patients with KS, diagnosed and successfully managed at our institution, one with a perineal fistula and one with a rectovestibular fistula. Our report, along with a literature review, shows that the syndrome is usually associated with “low” anomalies, with a potential for a good prognosis. Management of the anorectal anomaly in patients with KS is not essentially different from that in other nonsyndromic patients, taking into account the frequent association of the syndrome with serious congenital heart disease, which might affect the decision-making and timing of the stages of anorectal reconstruction. The frequent occurrence of learning and feeding difficulties makes establishment of toilet training and bowel management rather more challenging, requiring the expertise of a multidisciplinary team. The finding of ARMs in female patients with other characteristics of KS, although inconstant, could support the clinical suspicion for the syndrome until genetic confirmation is available, and should alert the physician for the potential of severe cardiac defects. PMID:26171318

  4. Carotid Stump Syndrome: Case Report and Endovascular Treatment.

    PubMed

    Dakhoul, Lara Toufic; Tawk, Rabih

    2014-01-01

    Objectives. To highlight the case of a patient with multiple transient ischemic attacks and visual disturbances diagnosed with carotid stump syndrome and managed with endovascular approach. Case Presentation. We present the case of a carotid stump syndrome in an elderly patient found to have moderate left internal carotid artery stenosis in response to an advertisement for carotid screening. After a medical therapeutic approach and a close follow-up, transient ischemic attacks recurred. Computed tomographic angiography showed an occlusion of the left internal carotid artery and the presence of moderate stenosis in the right internal carotid artery, which was treated by endovascular stenting and balloon insertion. One month later, the patient presented with visual disturbances due to the left carotid stump and severe stenosis of the left external carotid artery that was reapproached by endovascular stenting. Conclusion. Considerations should be given to the carotid stump syndrome as a source of emboli for ischemic strokes, and vascular assessment could be used to detect and treat this syndrome. PMID:26425620

  5. Narrowband UVB phototherapy as a novel treatment for Netherton syndrome.

    PubMed

    Kaminska, Edidiong Celestine Ntuen; Ortel, Bernhard; Sharma, Vishakha; Stein, Sarah L

    2012-06-01

    Netherton syndrome (NS) is a rare congenital ichthyosis that is characterized by impaired skin barrier function. Topical medications are cautiously used in NS since toxicity from systemic absorption is a major concern. Narrowband ultraviolet B phototherapy is an alternative therapeutic option that demonstrated its beneficial and practical use in a patient with NS. PMID:22548400

  6. Family Therapy of Terroristic Trauma: Psychological Syndromes and Treatment Strategies.

    ERIC Educational Resources Information Center

    Miller, Laurence

    2003-01-01

    Reviews pertinent literature on terroristic trauma and combines this information with the author's experience in treating adults, children, and family victims and survivors of recent terrorist attacks. Describes the psychological syndromes resulting from terrorism and discusses the relevant individual and family therapy modalities for treating…

  7. [Advances in diagnosis and treatment of hepatopulmonary syndrome].

    PubMed

    Ma, H Y; Li, X

    2016-05-20

    Hepatopulmonary syndrome is a disease with abnormal gas exchange caused by liver diseases, with the feature of abnormal oxygenation caused by intrapulmonary vasodilation. This article introduces the pathogenesis, natural course, clinical manifestations, diagnostic methods, and therapeutic measures of this disease and discusses potential therapeutic measures besides liver transplantation. PMID:27470899

  8. The Dental Needs and Treatment of Patients with Down Syndrome.

    PubMed

    Mubayrik, Azizah Bin

    2016-07-01

    Down syndrome is a common disorder with many oral conditions and systemic manifestations. Dentists need to take a holistic approach including behavioral, oral, and systemic issues. This review of the literature focuses on oral anomalies, systemic interaction, management, and recommendations. PMID:27264854

  9. Nutritional Strategies in the Prevention and Treatment of Metabolic Syndrome

    Technology Transfer Automated Retrieval System (TEKTRAN)

    The metabolic syndrome is a clustering of metabolic abnormalities that increase the risk of developing atherosclerotic cardiovascular disease and type 2 diabetes. The exact etiology remains unclear, but it is known to be a complex interaction between genetic, metabolic and environmental factors. Amo...

  10. Neuropsychological Evaluation in the Diagnosis and Treatment of Tourette's Syndrome

    ERIC Educational Resources Information Center

    Osmon, David C.; Smerz, Jessica M.

    2005-01-01

    The neurobiological basis of Tourettes syndrome is reviewed for the purpose of presenting a clinically relevant account of the neuropsychology of the disorder for the clinician who is behaviorally oriented. The neuropathology and neuropsychological deficits typically found in Tourettes are reviewed, and a neuropsychological test battery is…

  11. Clozapine Treatment of Psychosis Associated with Velo-Cardio-Facial Syndrome: Benefits and Risks

    ERIC Educational Resources Information Center

    Gladston, S.; Clarke, D. J.

    2005-01-01

    Clozapine is licensed for the treatment of psychotic illnesses resistant to other antipsychotic medications. Velo-cardio-facial syndrome (VCFS) is associated with a vulnerability to psychotic illness that may be resistant to treatment with conventional typical and atypical antipsychotics. A 32-year-old man with intellectual disability (ID) and a…

  12. Oral Health Condition and Treatment Needs of a Group of Nigerian Individuals with Down Syndrome

    ERIC Educational Resources Information Center

    Oredugba, Folakemi A.

    2007-01-01

    Objective: This study was carried out to determine the oral health condition and treatment needs of a group of individuals with Down syndrome in Nigeria. Method: Participants were examined for oral hygiene status, dental caries, malocclusion, hypoplasia, missing teeth, crowding and treatment needs. Findings were compared with controls across age…

  13. Treatment Options by Stage (Mycosis Fungoides and the Sezary Syndrome)

    MedlinePlus

    ... following PDQ summaries: Adult Non-Hodgkin Lymphoma Treatment Skin Cancer Treatment Melanoma Treatment Kaposi Sarcoma Treatment A sign of ... or laser that directs UVB radiation at the skin. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of ...

  14. Treatment Option Overview (Mycosis Fungoides and the Sezary Syndrome)

    MedlinePlus

    ... following PDQ summaries: Adult Non-Hodgkin Lymphoma Treatment Skin Cancer Treatment Melanoma Treatment Kaposi Sarcoma Treatment A sign of ... or laser that directs UVB radiation at the skin. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of ...

  15. Treatment Options for Recurrent Mycosis Fungoides and the Sezary Syndrome

    MedlinePlus

    ... following PDQ summaries: Adult Non-Hodgkin Lymphoma Treatment Skin Cancer Treatment Melanoma Treatment Kaposi Sarcoma Treatment A sign of ... or laser that directs UVB radiation at the skin. Chemotherapy Chemotherapy is a cancer treatment that uses drugs to stop the growth of ...

  16. Efficacy of Percutaneous Adhesiolysis in the Treatment of Lumbar Post Surgery Syndrome

    PubMed Central

    Manchikanti, Laxmaiah; Manchikanti, Kavita N.; Gharibo, Christopher G.; Kaye, Alan D.

    2016-01-01

    Context Lumbar post-surgery syndrome is common and often results in chronic, persistent pain and disability, which can lead to multiple interventions. After failure of conservative treatment, either surgical treatment or a nonsurgical modality of treatment such as epidural injections, percutaneous adhesiolysis is often contemplated in managing lumbar post surgery syndrome. Recent guidelines and systematic reviews have reached different conclusions about the level of evidence for the efficacy of epidural injections and percutaneous adhesiolysis in managing lumbar post surgery syndrome. The objective of this systematic review was to determine the efficacy of all 3 percutaneous adhesiolysis anatomical approaches (caudal, interlaminar, and transforaminal) in treating lumbar post-surgery syndrome. Evidence Acquisition Data Sources: A literature search was performed from 1966 through October 2014 utilizing multiple databases. Study Selection: A systematic review of randomized trials published from 1966 through October 2014 of all types of epidural injections and percutaneous adhesiolysis in managing lumbar post-surgery syndrome was performed including methodological quality assessment utilizing Cochrane review criteria, Interventional Pain Management Techniques–Quality Appraisal of Reliability and Risk of Bias Assessment (IPM–QRB), and grading of evidence using 5 levels of evidence ranging from Level I to Level V. Data Extraction: The search strategy emphasized post-surgery syndrome and related pathologies treated with percutaneous adhesiolysis procedures. Results The search criteria yielded 16 manuscripts on percutaneous adhesiolysis assessing post-surgery syndrome. Of these, only 4 randomized trials met inclusion criteria for methodological quality assessment, 3 of them were of high quality; and the fourth manuscript was of low quality. Based on these 3 randomized controlled trials, 2 of them with one-day procedure and one with a 3-day procedure, the level of

  17. [Acute respiratory distress syndrome: definitions, mechanisms and treatment].

    PubMed

    Urso, Domenico Lorenzo

    2006-01-01

    Acute respiratory distress syndrome is a secondary acute respiratory insufficiency caused by an inflammatory syndrome which is characterized by an increased of permeability pulmonary edema, associated with many other clinic anomalies, radiological and pathophysiological not directly caused by, but with which it could coexist, a left atrial hypertension. The illness, characterized by refractory hypoxemia, recognizes several causes, which have direct or indirect harm on the cells of the membrane alveolus-capillary. In spite of the improvements in the therapeutic approach, during these last 40 years, represented by the aid of the mechanical ventilation and the use of selective pulmonary vasodilators, this condition is life threatening and often lethal: 90% of mortality rate amongst those older than 65 years. PMID:16913178

  18. [Invasive diagnosis, transcatheter and surgical treatment of acute coronary syndromes].

    PubMed

    Fabián, J; Hricák, V; Fridrich, V; Fischer, V

    1998-01-01

    On the basis of long-term personal experiences and critical evaluation of the present literatury sources authors described the role of invasive diagnostic methods and transcathetral and cardiosurgical possibilities in the recognition and therapy of acute coronary syndromes. These techniques are, and in the forthcoming year shall be available only in specialized institutions. The paper describes the indication for these aggressive techniques as well as their limitations and complications. The goal of the presented article is to inform both the cardiological and frequently broad physicians' societies about the possibilities of diaventional cardiology and cardiosurgery which will be gradually more applied in the care of the patients with acute coronary syndromes. (Ref. 39, Tab. 2, Fig. 3.) PMID:9919748

  19. Experience in the use of clobazam in the treatment of Lennox–Gastaut syndrome

    PubMed Central

    Purcarin, Gabriela

    2014-01-01

    Clobazam is a 1,5-benzodiazepine used successfully worldwide since the 1970s as an anxiolytic and antiepileptic drug. Since its recent Food and Drug Administration (FDA) approval in the United States in 2011 as adjunctive treatment for Lennox–Gastaut syndrome, it has continued to show sustained efficacy and a better safety and tolerability profile compared with other benzodiazepines. The two randomized, controlled studies that led to the US FDA approval, as well as the follow-up multicenter, open-label study of clobazam, showed ≥50% seizure reduction for more than 50% of Lennox–Gastaut syndrome patients, while none of the other FDA-approved treatments for LGS have demonstrated efficacy rates better than 50%. Clobazam appears to have a safe profile and sustained effectiveness over the first 3 years of use in LGS and other epilepsy syndromes with intractable seizures, which makes it a viable long-term treatment option. PMID:24790647

  20. Standard and Novel Treatment Options for Metabolic Syndrome and Diabetes Mellitus

    PubMed Central

    Groves, Elliott M; Yu, Katherine; Wong, Nathan D.; Malik, Shaista

    2014-01-01

    Type II Diabetes and metabolic syndrome are two intertwined conditions that are critical to the healthcare landscape in the United States and abroad. Patients with either diabetes or metabolic syndrome can have a dramatically increased risk of developing cardiovascular disease. Numerous treatment options have existed for some time, which include non-pharmacologic and pharmacologic therapies. Additionally, within the last decade a multiple of novel treatment options have emerged for the management of hyperglycemia in particular. By targeting novel pathways beyond the secretion and supply of insulin, these new therapeutics provide a valuable adjunctive to the currently available therapies for diabetes and metabolic syndrome. Here we discuss the current guideline driven usage of standard therapies with some novel indications. In addition we discuss the novel therapies for the treatment of hyperglycemia, their mechanisms of action and appropriate therapeutic indications. PMID:24234946

  1. Work-Related Carpal Tunnel Syndrome: Diagnosis and Treatment Guideline.

    PubMed

    Franklin, Gary M; Friedman, Andrew S

    2015-08-01

    Carpal tunnel syndrome is the most common entrapment neuropathy, and its risk of occurrence in the presence of repetitive, forceful angular hand movements, or vibration, is common. It is critical to make the diagnosis based on appropriate clinical history and findings and with corroborating electrodiagnostic studies. Conservative management should be undertaken with the goal of maintaining employment; surgical decompression can be highly effective, particularly if undertaken early on. PMID:26231963

  2. Pisa syndrome in Parkinson's disease and parkinsonism: clinical features, pathophysiology, and treatment.

    PubMed

    Barone, Paolo; Santangelo, Gabriella; Amboni, Marianna; Pellecchia, Maria Teresa; Vitale, Carmine

    2016-09-01

    Pisa syndrome is defined as a reversible lateral bending of the trunk with a tendency to lean to one side. It is a frequent and often disabling complication of Parkinson's disease, and has also been described in several atypical forms of parkinsonism and in neurodegenerative and psychiatric disorders after drug exposure and surgical procedures. Although no consistent diagnostic criteria for Pisa syndrome are available, most investigations have adopted an arbitrary cutoff of at least 10° of lateral flexion for the diagnosis of the syndrome. Pathophysiological mechanisms underlying Pisa syndrome have not been fully explained. One hypothesis emphasises central mechanisms, whereby Pisa syndrome is thought to be caused by alterations in sensory-motor integration pathways; by contrast, a peripheral hypothesis emphasises the role of anatomical changes in the musculoskeletal system. Furthermore, several drugs are reported to induce Pisa syndrome, including antiparkinsonian drugs. As Pisa syndrome might be reversible, clinicians need to be able to recognise this condition early to enable prompt management. Nevertheless, further research is needed to determine optimum treatment strategies. PMID:27571158

  3. Syndromic treatment of gonococcal and chlamydial infections in women seeking primary care for the genital discharge syndrome: decision-making.

    PubMed Central

    Behets, F. M.; Miller, W. C.; Cohen, M. S.

    2001-01-01

    The syndromic treatment of gonococcal and chlamydial infections in women seeking primary care in clinics where resources are scarce, as recommended by WHO and implemented in many developing countries, necessitates a balance to be struck between overtreatment and undertreatment. The present paper identifies factors that are relevant to the selection of specific strategies for syndromic treatment in the above circumstances. Among them are the general aspects of decision-making and caveats concerning the rational decision-making approach. The positive and negative implications are outlined of providing or withholding treatment following a specific algorithm with a given accuracy to detect infection, i.e. sensitivity, specificity and predictive values. Other decision-making considerations that are identified are related to implementation and include the stability of risk factors with regard to time, space and the implementer, acceptability by stakeholders, and environmental constraints. There is a need to consider empirically developed treatment algorithms as a basis for policy discourse, to be evaluated together with the evidence, alternatives and arguments by the stakeholders. PMID:11731816

  4. Effectiveness of therapeutic physical exercise in the treatment of patellofemoral pain syndrome: a systematic review

    PubMed Central

    Alba-Martín, Pablo; Gallego-Izquierdo, T; Plaza-Manzano, Gustavo; Romero-Franco, Natalia; Núñez-Nagy, Susana; Pecos-Martín, Daniel

    2015-01-01

    [Purpose] The aim of this study was to analyze the effectiveness of conservative treatment of patellofemoral pain syndrome with physical exercise. [Subjects and Methods] A computer-based review conducted of four databases (PubMed, the Cochrane Library, PEDro, and the University Library) was completed based on the inclusion criteria of patellofemoral pain syndrome patients treated with physical exercise methods and examination with self-reported pain and/or functional questionnaires. [Results] The findings of ten clinical trials of moderate to high quality were evaluated to determine the effectiveness of physical exercise as conservative management for patellofemoral pain syndrome. [Conclusion] The intervention programs that were most effective in relieving pain and improving function in patellofemoral pain syndrome included proprioceptive neuromuscular facilitation stretching and strengthening exercises for the hip external rotator and abductor muscles and knee extensor muscles. PMID:26311988

  5. The treatment of myofascial pain-dysfunction syndrome using the biofeedback principle.

    PubMed

    Clarke, N G; Kardachi, B J

    1977-10-01

    Facial pain is a relatively common sequel to bruxism and the biofeedback principle was used on seven subjects experiencing this syndrome. The results obtained were satisfactory and support the concept that the etiology of the M.P.D. syndrome is psychophysiological. This study showed that biofeedback is both a logical and appropriate form of treatment. However, the result with subject 7 indicates that not all patients are willing to wear the equipment but conselling and empathy probably form an equally satisfactory form of treatment. PMID:269245

  6. Treatments for Infertility Resulting from Polycystic Ovary Syndrome (PCOS)

    MedlinePlus

    ... Information Clinical Trials Resources and Publications Treatments for Infertility Resulting from PCOS Skip sharing on social media ... reason for these problems. Before beginning treatment for infertility possibly related to PCOS, be sure that your ...

  7. Diagnosis and Treatment of Obstructive Sleep Apnea Syndrome in Children.

    PubMed

    Tsubomatsu, Chieko; Shintani, Tomoko; Abe, Ayumi; Yajima, Ryoto; Takahashi, Nozomi; Ito, Fumie; Takano, Kenichi; Himi, Tetsuo

    2016-01-01

    Sleep is important for children pertaining to their physical and mental growth. Obstructive sleep apnea syndrome (OSAS) in children has been shown to have different effects as compared to OSAS in adults, including deficits in cognition and neuropsychological functions, hyperactivity, ADHD, behavior problems, aggressive behavior, learning problems and nocturnal enuresis. Hypertrophy of the adenoids and tonsils is a major cause of OSAS in children; therefore, adenotonsillectomy may decrease the effects of OSAS pertaining to physical and mental growth. It is important to accurately diagnose and appropriately treat OSAS in children to prevent OSAS in their adulthood. PMID:27115764

  8. Short QT Syndrome – Review of Diagnosis and Treatment

    PubMed Central

    Schimpf, Rainer; Borggrefe, Martin

    2014-01-01

    Short QT syndrome (SQTS) is an inherited cardiac channelopathy characterised by an abnormally short QT interval and increased risk for atrial and ventricular arrhythmias. Diagnosis is based on the evaluation of symptoms (syncope or cardiac arrest), family history and electrocardiogram (ECG) findings. Mutations of cardiac ion channels responsible for the repolarisation orchestrate electrical heterogeneity during the action potential and provide substrate for triggering and maintaining of tachyarrhythmias. Due to the malignant natural history of SQTS, implantable cardioverter defibrillator (ICD) is the first-line therapy in affected patients. This review summarises current data and addresses the genetic basis and clinical features of SQTS. PMID:26835070

  9. Wiskott–Aldrich syndrome: diagnosis, current management, and emerging treatments

    PubMed Central

    Buchbinder, David; Nugent, Diane J; Fillipovich, Alexandra H

    2014-01-01

    Wiskott–Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency disorder characterized by the triad of eczema, thrombocytopenia, and severe and often recurrent infections. Despite the rarity of this disorder, our understanding of the molecular and cellular pathogenesis of WAS has continued to increase. Advances in the use of diagnostic tools, the provision of supportive care, and improvements in allogeneic hematopoietic stem cell transplantation have significantly reduced the morbidity and mortality associated with this disorder. Exciting advancements in the care of patients with WAS have also occurred, including the successful application of autologous gene-modified hematopoietic stem cell transplantation. PMID:24817816

  10. Early Treatment of Severe Acute Respiratory Distress Syndrome.

    PubMed

    Przybysz, Thomas M; Heffner, Alan C

    2016-02-01

    Acute respiratory distress syndrome (ARDS) is defined by acute diffuse inflammatory lung injury invoked by a variety of systemic or pulmonary insults. Despite medical progress in management, mortality remains 27% to 45%. Patients with ARDS should be managed with low tidal volume ventilation. Permissive hypercapnea is well tolerated. Conservative fluid strategy can reduce ventilator and hospital days in patients without shock. Prone positioning and neuromuscular blockers reduce mortality in some patients. Early management of ARDS is relevant to emergency medicine. Identifying ARDS patients who should be transferred to an extracorporeal membrane oxygenation center is an important task for emergency providers. PMID:26614238

  11. Therapeutic plasma exchange as treatment for propofol infusion syndrome.

    PubMed

    Levin, Phillip D; Levin, Valentin; Weissman, Charles; Sprung, Charles L; Rund, Deborah

    2015-10-01

    Propofol infusion syndrome (PRIS), a rare complication of propofol sedation, is associated with high mortality. There is no specific therapy. A 16-year-old with head injury and status epilepticus is described. Three days after seizure resolution, whilst receiving propofol, he developed severe lactic acidosis, rhabdomyolysis, and hemodynamic instability. Suspected PRIS was treated with a single session of therapeutic plasma exchange (TPE). This was associated with immediate improvement in hemodynamic status, resolution of lactic acidosis within 24 h, normalization of CPK over 10 days, and a subsequent full recovery. TPE is suggested as a novel therapy for PRIS. PMID:25619501

  12. Recording and treatment of premenstrual syndrome in UK general practice: a retrospective cohort study

    PubMed Central

    Sammon, Cormac J; Nazareth, Irwin; Petersen, Irene

    2016-01-01

    Objectives To investigate the rate of recording of premenstrual syndrome diagnoses in UK primary care and describe pharmacological treatments initiated following a premenstrual syndrome (PMS) diagnosis. Design Retrospective cohort study. Setting UK primary care. Participants Women registered with a practice contributing to The Health Improvement Network primary care database between 1995 and 2013. Primary and secondary outcome measures The primary outcome was the rate of first premenstrual syndrome records per 1000 person years, stratified by calendar year and age. The secondary outcome was the proportions of women with a premenstrual syndrome record prescribed a selective serotonin reuptake inhibitor, progestogen, oestrogen, combined oral contraceptive, progestin only contraceptive, gonadotrophin-releasing hormone, danazol and vitamin B6. Results The rate of recording of premenstrual syndrome diagnoses decreased over calendar time from 8.43 in 1995 to 1.72 in 2013. Of the 38 614 women without treatment in the 6 months prior to diagnosis, 54% received a potentially premenstrual syndrome-related prescription on the day of their first PMS record while 77% received a prescription in the 24 months after. Between 1995 and 1999, the majority of women were prescribed progestogens (23%) or vitamin B6 (20%) on the day of their first PMS record; after 1999, these figures fell to 3% for progestogen and vitamin B6 with the majority of women instead being prescribed a selective serotonin reuptake inhibitor (28%) or combined oral contraceptive (17%). Conclusions Recording of premenstrual syndrome diagnoses in UK primary care has declined substantially over time and preferred prescription treatment has changed from progestogen to selective serotonin reuptake inhibitor and combined oral contraceptives. PMID:26993623

  13. Effective Methylphenidate Treatment of an Adult Aspergers Syndrome and a Comorbid ADHD: A Clinical Investigation with fMRI

    ERIC Educational Resources Information Center

    Roy, Mandy; Dillo, Wolfgang; Bessling, Svenja; Emrich, Hinderk M.; Ohlmeier, Martin D.

    2009-01-01

    Objective: Aspergers Syndrome can present as comorbid with attention-deficit/hyperactivity disorder (ADHD). Very few cases of the assessment and treatment of this comorbidity in adulthood are described in the research literature. Method: A 26-year-old patient as suffering from ADHD in combination with Aspergers Syndrome is diagnosed. Treatment is…

  14. Teduglutide for the treatment of short bowel syndrome.

    PubMed

    Vipperla, Kishore; O'Keefe, Stephen J

    2011-12-01

    Extensive resection of the intestine impairs its absorptive capacity and results in short bowel syndrome when the nutritional equilibrium is compromised. The remnant intestine adapts structurally to compensate, but nutritional autonomy cannot be achieved in patients with intestinal failure, requiring intravenous fluids and parenteral nutrition (PN) for sustenance of life. PN is expensive and associated with serious complications. Efforts to minimize or eliminate the need for PN heralded research focusing on the therapeutic utility of intrinsic gut factors involved in the postresection adaptation process. With the breakthrough recognition of the intestinotrophic properties of glucagon-like peptide-2, teduglutide, a recombinant analogue of glucagon-like peptide-2, is being investigated as a promising hope to mitigate the requirement of PN. Clinical studies to date have demonstrated a desirable benefit-to-risk profile in regards to its safety and efficacy. If approved for marketing, it will be the first of its class in short bowel syndrome management, offering an innovative therapeutic modality for this debilitating condition. PMID:22017694

  15. Advances in Positive Airway Pressure Treatment Modalities for Hypoventilation Syndromes

    PubMed Central

    Combs, Dan; Shetty, Safal; Parthasarathy, Sairam

    2014-01-01

    SYNOPSIS Rationale Positive airway pressure therapy for hypoventilation syndromes can significantly improve health-related quality of life (HR-QOL), healthcare costs, and even mortality. The sleep-disordered breathing in such individuals are quite complex and require sophisticated devices with algorithms that are designed to accurately detect and effectively treat respiratory events that includes hypoventilation, upper airway obstruction, lower airway obstruction, central apneas and central hypopneas and reduce the work of breathing while maintaining breathing comfort. Objectives The therapeutic physiological rationale for the various advanced PAP modalities and the details about the principles of operation and technology implementation are provided here. Conclusions The physiological rationale for advanced PAP modalities is sound considering the complexity of sleep-disordered breathing in patients with hypoventilation syndromes. Although such devices are increasingly used in clinical practice, the supporting clinical evidence – specifically comparative-effectiveness studies in real-life conditions -- needs to be performed. Moreover, there is much opportunity for further refining these devices that include the ability of the device to reliably monitor gas-exchange, sleep-wakefulness state, and for reducing variability in device efficacy due to provider-selected device-settings. PMID:25346650

  16. [Treatment of nephrotic syndrome: immuno- or rather podocyte therapy?].

    PubMed

    Lewko, Barbara

    2016-01-01

    Nephrotic syndrome (NS) is a group of clinical symptoms resulting from massive proteinuria caused by impairment of the glomerular filtration barrier. The filtration barrier comprises glomerular basement membrane with endothelial cells lining its inner side and a podocyte monolayer covering its outer aspect. As well as forming part of the glomerular filter, podocytes also regulate synthesis of other components of the filtration barrier. Therefore, integrity of these cells is crucial for maintaining the normal ultrafiltration function. The pathogenesis of idiopathic nephrotic syndrome (INS) was proposed to be associated with autoimmunity and appearance in the circulation of a still unknown protein permeability factor (PF) inducing changes in the glomerular filtration barrier. Several candidate PFs have been identified to date, and current results indicate that podocytes are target cells for all of them. Changes in podocyte structure and functions induced by these factors are typical for changes observed in patients with nephrotic proteinuria. Most pharmacotherapeutic approaches in NS are based on various immunosuppressive agents and are targeted toward minimizing proteinuria. It appears, however, that these drugs not only target the cells of the immune system but also act directly on podocytes. Thus, it can be concluded that detailed studies on mechanisms regulating podocyte functions as well as designing drugs to protect these cells are required for effective therapy of NS. PMID:27180964

  17. Novel association of rectal evacuation disorder and rumination syndrome: Diagnosis, comorbidities, and treatment

    PubMed Central

    Vijayvargiya, Priya; Iturrino, Johanna; Shin, Andrea; Vazquez-Roque, Maria; Katzka, David A; Snuggerud, Jill R; Seime, Richard J

    2014-01-01

    Background Patients with disorders of gastrointestinal function may undergo unnecessary treatment if misdiagnosed as motility disorders. Objective To report on clinical features, medical, surgical, and psychiatric comorbidities, and prior treatments of a patient cohort diagnosed concurrently with nonpsychogenic rumination syndrome and pelvic floor dysfunction (also termed rectal evacuation disorder). Methods From a consecutive series (1994–2013) of 438 outpatients with rectal evacuation disorders in the practice of a single gastroenterologist at a tertiary care centre, 57 adolescents or adults were diagnosed with concomitant rumination syndrome. All underwent formal psychological assessment or completed validated questionnaires. Results All 57 patients (95% female) fulfilled Rome III criteria for rumination syndrome; rectal evacuation disorder was confirmed by testing of anal sphincter pressures and rectal balloon evacuation. Prior to diagnosis, most patients underwent multiple medical and surgical treatments (gastrostomy, gastric fundoplication, other gastric surgery, ileostomy, colectomy) for their symptoms. Psychological comorbidity was identified in 93% of patients. Patients were managed predominantly with psychological and behavioural approaches: diaphragmatic breathing for rumination and biofeedback retraining for pelvic floor dysfunction. Conclusions Awareness of concomitant rectal evacuation disorder and rumination syndrome and prompt identification of psychological comorbidity are keys to instituting behavioural and psychological methods to avoid unnecessary treatment. PMID:24724013

  18. B-complex vitamins in the prophylaxis and treatment of Wernicke-Korsakoff syndrome.

    PubMed

    Cook, C C; Thomson, A D

    Wernicke-Korsakoff syndrome, which is associated with high morbidity and mortality, is a more common neuropsychiatric sequela of alcohol misuse than is widely realized. It is easily prevented and treated with parenteral B-complex vitamins, although this treatment is widely under-utilized. PMID:9274684

  19. Treatments for Fragile X Syndrome: A Closer Look at the Data

    ERIC Educational Resources Information Center

    Hall, Scott S.

    2009-01-01

    Research into the determinants and developmental course of fragile X syndrome (FXS) has made remarkable progress over the last 25 years. However, treatments to ameliorate the symptoms of FXS have been less forthcoming. While there is optimism in the field that the pace of intervention research is quickening, there has been a bias toward…

  20. Habituation of Premonitory Sensations during Exposure and Response Prevention Treatment in Tourette's Syndrome

    ERIC Educational Resources Information Center

    Verdellen, Cara W. J.; Hoogduin, Cees A. L.; Kato, Bernet S.; Keijsers, Ger P. J.; Cath, Danielle C.; Hoijtink, Herbert B.

    2008-01-01

    Exposure to premonitory sensations and response prevention of tics (ER) has been shown to be a promising new treatment for Tourette's syndrome (TS). The present study tested the hypothesis that habituation to unpleasant premonitory sensations associated with the tic is an underlying mechanism of change in ER. Patients rated the severity of…

  1. Psychological Treatments for Irritable Bowel Syndrome: A Systematic Review and Meta-Analysis

    ERIC Educational Resources Information Center

    Lackner, Jeffrey M.; Mesmer, Christina; Morley, Stephen; Dowzer, Clare; Hamilton, Simon

    2004-01-01

    This study conducted a systematic review to assess the quality of existing literature on psychological treatments for irritable bowel syndrome and to quantify the evidence for their efficacy. Three independent reviewers (2 from England, 1 from the United States) coded the quality of 32 studies, 17 of which provided data suitable for meta-analysis.…

  2. Treatment of a Down's Syndrome Patient for Hyperthyroidism With Radioactive Iodine

    PubMed Central

    Nibhanupudy, J. Rao; Streeter, O. E.; King, G. C.; Mahan, J.; Talley, G.; Lander, C.; Ashayeri, E.

    1986-01-01

    A Down's syndrome patient was hospitalized for evaluation of vomiting, abdominal pain, and a history of weight loss. A subsequent workup revealed that she had hyperthyroidism. The treatment of choice was radioactive iodine therapy. The patient had a history of consistent nausea and incontinence for urine and feces. Special problems posed by the patient and radiation safety are discussed. PMID:2936892

  3. Evaluation of a Behavioral Treatment Package to Reduce Sleep Problems in Children with Angelman Syndrome

    ERIC Educational Resources Information Center

    Allen, Keith D.; Kuhn, Brett R.; DeHaai, Kristi A.; Wallace, Dustin P.

    2013-01-01

    The purpose of this investigation was to evaluate the effectiveness of a behavioral treatment package to reduce chronic sleep problems in children with Angelman Syndrome. Participants were five children, 2-11 years-of-age. Parents maintained sleep diaries to record sleep and disruptive nighttime behaviors. Actigraphy was added to provide…

  4. Gilles de la Tourette syndrome: the complexities of phenotype and treatment.

    PubMed

    Robertson, Mary M

    2011-02-01

    Tourette syndrome is a chronic motor and vocal tic disorder, which is common (1%). The aetiology is complex (mostly genetic) and 90% of people have co-morbid psychiatric disorders and reduced quality of life. Management includes reassurance, education, behavioural treatments and medications for tics and psychopathology. PMID:21378617

  5. Effect of Fungicide Seed Treatments on Fusarium virguliforme and Sudden Death Syndrome of Soybean

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Sudden death syndrome (SDS) is a yield reducing disease increasing in prevalence across soybean producing states. Recent research indicates the SDS pathogen, Fusarium virguliforme, can infect as early as initial radicle emergence. This suggests fungicide seed treatments could offer some protection a...

  6. Psychotherapeutic Treatment in Combination with Relaxation in a Flotation Tank: Effects on "Burn-Out Syndrome"

    ERIC Educational Resources Information Center

    Kjellgren, Anette; Buhrkall, Hanne; Norlander, Torsten

    2010-01-01

    The focus of this study was to investigate experiences gained from treatment combining relaxation in flotation tank with psychotherapy for sufferers from "burn-out syndrome". Six people participated in a ten week program. They were all interviewed; the data were analyzed using the Empirical Phenomenological Psychological method. Five themes…

  7. [The post-discectomy syndrome: clinical and electroneuromyographic characteristics and methods of treatment].

    PubMed

    Musaev, A V; Guseĭnova, S G; Musaeva, I R

    2008-01-01

    The data of the Azerbaijan Neurosurgical Center, including 2618 case-reports of patients operated on for low back discal hernia between 1997 and 2002, have been analyzed. The retrospective analysis of the data reveals that 26,4% of patients need further restorative treatment due to the presence of various neurological disturbances: pain syndromes of different intensity, motor deficits (pareses), sensory disorders and functional disorders of pelvic organs. The retrospective analysis of the data reveals that 26,4% of patients need further restorative treatment due to the presence of various neurological disturbances: pain syndromes of different intensity, motor deficits (pareses), sensory disorders and functional disorders of pelvic organs. Along with these data, the results of our own clinical and neurophysiological study of 110 patients have been summarized as well. Along with these data, the results of our own clinical and neurophysiological study of 110 patients have been summarized as well. A high effectiveness of electrostimulation and naphthalan therapy alone and in combination with massage and medical gymnastics has been revealed. A high effectiveness of electrostimulation and naphthalan therapy alone and in combination with massage and medical gymnastics has been revealed. Electroneuromyographic data revealing the positive dynamics as a result of the treatment of patients with the post-discectomy syndrome are presented. Electroneuromyographic data revealing the positive dynamics as a result of the treatment of patients with the post-discectomy syndrome are presented. PMID:18689008

  8. Transitions in endocrinology: treatment of Turner's syndrome during transition.

    PubMed

    Gawlik, Aneta; Malecka-Tendera, Ewa

    2014-02-01

    Transition in health care for young patients with Turner's syndrome (TS) should be perceived as a staged but uninterrupted process starting in adolescence and moving into adulthood. As a condition associated with high risk of short stature, cardiovascular diseases, ovarian failure, hearing loss and hypothyroidism, TS requires the attention of a multidisciplinary team. In this review paper, we systematically searched the relevant literature from the last decade to discuss the array of problems faced by TS patients and to outline their optimal management during the time of transfer to adult service. The literature search identified 233 potentially relevant articles of which 114 were analysed. The analysis confirmed that all medical problems present during childhood should also be followed in adult life. Additionally, screening for hypertension, diabetes mellitus, dyslipidaemia, and osteoporosis is needed. After discharge from the paediatric clinic, there is still a long way to go. PMID:24225028

  9. Inflammation in irritable bowel syndrome: Myth or new treatment target?

    PubMed Central

    Sinagra, Emanuele; Pompei, Giancarlo; Tomasello, Giovanni; Cappello, Francesco; Morreale, Gaetano Cristian; Amvrosiadis, Georgios; Rossi, Francesca; Lo Monte, Attilio Ignazio; Rizzo, Aroldo Gabriele; Raimondo, Dario

    2016-01-01

    Low-grade intestinal inflammation plays a key role in the pathophysiology of irritable bowel syndrome (IBS), and this role is likely to be multifactorial. The aim of this review was to summarize the evidence on the spectrum of mucosal inflammation in IBS, highlighting the relationship of this inflammation to the pathophysiology of IBS and its connection to clinical practice. We carried out a bibliographic search in Medline and the Cochrane Library for the period of January 1966 to December 2014, focusing on publications describing an interaction between inflammation and IBS. Several evidences demonstrate microscopic and molecular abnormalities in IBS patients. Understanding the mechanisms underlying low-grade inflammation in IBS may help to design clinical trials to test the efficacy and safety of drugs that target this pathophysiologic mechanism. PMID:26900287

  10. Endovascular treatment of multiple aneurysms complicating Cogan syndrome.

    PubMed

    Angiletta, Domenico; Wiesel, Paola; Pulli, Raffaele; Marinazzo, Davide; Bortone, Alessandro Santo; Regina, Guido

    2015-02-01

    To report the use of endografts to manage multiple aneurysms due to Cogan syndrome (CS). A 38-year-old woman with descending thoracic aorta and right common carotid artery aneurysms due to CS was treated with endovascular grafts. After 4 years, angio computed tomography scan demonstrated complete exclusion of the aneurysms with no signs of endoleak, whereas echo color Doppler showed patency of the carotid graft, no signs of restenosis, no progression of the disease in the landing zones, and complete aneurysm exclusion. Endovascular repair seems to have favorable long-term outcomes and should be considered a viable alternative to surgery in unfit for open surgery patients, even if they are young, and when the aneurysm size and location would pose a higher risk of perioperative and postoperative complications after an open surgical procedure. PMID:25462550

  11. Standardized hypnosis treatment for irritable bowel syndrome: the North Carolina protocol.

    PubMed

    Palsson, Olafur S

    2006-01-01

    The North Carolina protocol is a seven-session hypnosis-treatment approach for irritable bowel syndrome that is unique in that the entire course of treatment is designed for verbatim delivery. The protocol has been tested in two published research studies and found to benefit more than 80% of patients. This article describes the development, content, and testing of the protocol, and how it is used in clinical practice. PMID:16316883

  12. [Transcatheter delivery of recombinant adenovirus vector containing exogenous aquaporin gene in treatment of Sjögren's syndrome].

    PubMed

    Hong, H E; Jieqiong, Zhang; Yan, Fan; Xiaoshuang, Sun; Yuhao, Zhu

    2016-05-25

    Sjögren's syndrome is a kind of autoimmune disease, whose main clinical symptoms are dry mouth, dry eye and chronic parotid glandular inflammation. The conservative treatments include artificial tears or saliva,oral administration of corticosteroids,and immunosuppressantsl with limited effectiveness. Along with the development of molecular biology, vast attentions are being paid to researches on gene therapy for Sjögren's syndrome, hopefully to bring gospel to patients with Sjögren's syndrome. This article reviews the recent research progresses on transcatheter delivery of recombinant adenovirus vector with aquaporin gene in experimental treatment of Sjögren's syndrome. PMID:27045247

  13. Interdisciplinary Treatment of Maladaptive Behaviors Associated with Postural Orthostatic Tachycardia Syndrome (POTS): A Case Report.

    PubMed

    Bruce, Barbara K; Weiss, Karen E; Harrison, Tracy E; Allman, Daniel A; Petersen, Matthew A; Luedkte, Connie A; Fischer, Philip R

    2016-06-01

    The prevalence of postural orthostatic tachycardia syndrome (POTS) in adolescents and young adults has been increasing during the past decade. Despite this increase, documentation regarding treatment of these patients is just beginning to emerge. In addition, despite a call for a multidisciplinary or interdisciplinary approach, no studies have examined the efficacy of such an approach to treatment. This paper describes a case study of a 19-year-old male with debilitating POTS seen at a tertiary clinic for evaluation and subsequent intensive interdisciplinary treatment. The treatment approach is described and outcomes are presented. PMID:26538160

  14. Randomized Multicenter Feasibility Trial of Myofascial Physical Therapy for Treatment of Urologic Chronic Pelvic Pain Syndrome

    PubMed Central

    FitzGerald, Mary P; Anderson, Rodney U; Potts, Jeannette; Payne, Christopher K; Peters, Kenneth M; Clemens, J Quentin; Kotarinos, Rhonda; Fraser, Laura; Cosby, Annamarie; Fortman, Carole; Neville, Cynthia; Badillo, Suzanne; Odabachian, Lisa; Sanfield, Anna; O’Dougherty, Betsy; Halle-Podell, Rick; Cen, Liyi; Chuai, Shannon; Landis, J Richard; Kusek, John W; Nyberg, Leroy M

    2010-01-01

    Objectives To determine the feasibility of conducting a randomized clinical trial designed to compare two methods of manual therapy (myofascial physical therapy (MPT) and global therapeutic massage (GTM)) among patients with urologic chronic pelvic pain syndromes. Materials and Methods Our goal was to recruit 48 subjects with chronic prostatitis/chronic pelvic pain syndrome or interstitial cystitis/painful bladder syndrome at six clinical centers. Eligible patients were randomized to either MPT or GTM and were scheduled to receive up to 10 weekly treatments, each 1 hour in duration. Criteria to assess feasibility included adherence of therapists to prescribed therapeutic protocol as determined by records of treatment, adverse events which occurred during study treatment, and rate of response to therapy as assessed by the Patient Global Response Assessment (GRA). Primary outcome analysis compared response rates between treatment arms using Mantel-Haenszel methods. Results Twenty-three (49%) men and 24 (51%) women were randomized over a six month period. Twenty-four (51%) patients were randomized to GTM, 23 (49%) to MPT; 44 (94%) patients completed the study. Therapist adherence to the treatment protocols was excellent. The GRA response rate of 57% in the MPT group was significantly higher than the rate of 21% in the GTM treatment group (p=0.03). Conclusions The goals to judge feasibility of conducting a full-scale trial of physical therapy methods were met. The preliminary findings of a beneficial effect of MPT warrants further study. PMID:19535099

  15. A Comprehensive Review of Treatment Options for Premenstrual Syndrome and Premenstrual Dysphoric Disorder.

    PubMed

    Maharaj, Shalini; Trevino, Kenneth

    2015-09-01

    Premenstrual dysphoric disorder (PMDD) is a severe form of premenstrual syndrome that involves a combination of emotional and physical symptoms that result in significant functional impairment. Because of the debilitating nature of PMDD, multiple treatment options have been considered. This review provides a comprehensive overview of these therapeutic regimens to help health care professionals provide adequate treatment for PMDD and premenstrual syndrome. The treatments that are reviewed are organized into the following categories: psychiatric, anovulatory, supplements, herbal, nonpharmacological, and other. Selective serotonin reuptake inhibitors have been established as the first-line treatment for PMDD. Although luteal phase or continuous dosing can be used, additional research is needed to more thoroughly compare the efficacies and differential symptom response of continuous, semi-intermittent, luteal phase, and symptoms-onset dosing. The psychiatric medications venlafaxine, duloxetine, alprazolam, and buspirone have also been found to be useful treatments for PMDD. Various anovulatory-related treatments have demonstrated efficacy; however, the use of some of these treatments remains limited due to potential side effects and/or the availability of cheaper alternatives. Although a variety of supplement and herbal-related treatments have been proposed, with some warranting further research, at this time only calcium supplementation has demonstrated a consistent therapeutic benefit. In conclusion, serotoninergic antidepressants have been established as the first-line treatment option for PMDD; however, there are a variety of additional treatment options that should be considered if a patient fails to achieve an adequate therapeutic response with a selective serotonin reuptake inhibitor. PMID:26352222

  16. Vasculitis complicating granulocyte colony stimulating factor treatment of leukopenia and infection in Felty's syndrome.

    PubMed

    Farhey, Y D; Herman, J H

    1995-06-01

    Recombinant myeloid growth factors have been increasingly used in recent years to combat induced and disease associated neutropenia. Their application in the management of Felty's syndrome with intercurrent infection has raised concern that resultant neutrophilia and activation of a diverse array of polymorphonuclear cell functions may have an adverse effect on the rheumatoid disease process. We describe a patient with Felty's syndrome receiving short term treatment with recombinant human granulocyte colony stimulating factor (GCSF), who then developed acute renal failure in conjunction with leukocytoclastic vasculitis and presumptive gout. We address the issue of "adding fuel to the fire" and review reported implications of GCSF in induction of vasculitis. PMID:7545756

  17. Treatment of Complex Regional Pain Syndrome (CRPS) using low dose naltrexone (LDN).

    PubMed

    Chopra, Pradeep; Cooper, Mark S

    2013-06-01

    Complex Regional Pain Syndrome (CRPS) is a neuropathic pain syndrome, which involves glial activation and central sensitization in the central nervous system. Here, we describe positive outcomes of two CRPS patients, after they were treated with low-dose naltrexone (a glial attenuator), in combination with other CRPS therapies. Prominent CRPS symptoms remitted in these two patients, including dystonic spasms and fixed dystonia (respectively), following treatment with low-dose naltrexone (LDN). LDN, which is known to antagonize the Toll-like Receptor 4 pathway and attenuate activated microglia, was utilized in these patients after conventional CRPS pharmacotherapy failed to suppress their recalcitrant CRPS symptoms. PMID:23546884

  18. Percutaneous treatment of Lutembacher syndrome in a case with difficult mitral valve crossing.

    PubMed

    Bhambhani, Anupam; Somanath, H S

    2012-03-01

    Most cases of combination congenital cardiac anomalies are treated with open-heart surgeries because the coexisting anomalies change the cardiac anatomy in an adverse way, making catheter manipulations complex. Lutembacher syndrome is a combination of acquired mitral stenosis and congenital ostium secundum atrial septal defect. The large defect in the septum makes an Inoue balloon catheter unstable, which provides excessive space for free floatation of the catheter, making its passage into the left ventricle difficult by Inoue technique. We present a case of elective definitive percutaneous treatment of Lutembacher syndrome, discussing the technical difficulties faced in mitral valve crossing and reviewing the possible strategies to improve chances of success. PMID:22388316

  19. Arthroscopic treatment of painful Sinding-Larsen-Johansson syndrome in a professional handball player.

    PubMed

    Kajetanek, C; Thaunat, M; Guimaraes, T; Carnesecchi, O; Daggett, M; Sonnery-Cottet, B

    2016-09-01

    Sinding-Larsen-Johansson (SLJ) syndrome is a type of osteochondrosis of the distal pole of the patella most often caused by repeated microtrauma. Here, we describe the case of a professional athlete with painful SLJ syndrome treated arthroscopically. A 29-year-old male professional handball player presented with anterior knee pain that persisted after 4 months of an eccentric rehabilitation protocol and platelet-rich plasma injections. Despite this conservative treatment, the patient could not participate in his sport. The SLJ lesion was excised arthroscopically, which led to complete disappearance of symptoms and return to competitive sports after 5 months. PMID:27450859

  20. [Diagnostics and treatment of Wernicke-Korsakoff syndrome patients with an alcohol abuse].

    PubMed

    Nilsson, Maria; Sonne, Charlotte

    2013-04-01

    Wernicke-Korsakoff syndrome is a condition with high morbidity and mortality and occurs as a consequence of thiamine deficiency. Clinical symptoms are often ambiguous and post-mortem examinations show that the syndrome is underdiagnosed and probably undertreated. There is sparse clinical evidence concerning optimal dosage and duration of treatment. This article reviews the current literature and concludes that all patients with a history of alcohol abuse should be treated with high dosage IV thiamine for an extended period of time, albeit further research is needed. PMID:23582065

  1. Pharmacologic studies in Bartter's syndrome: opposite effects of treatment with antikaliuretic and antiprostaglandin drugs. Part I.

    PubMed

    Simatupang, T A; Radó, J P; Boer, P; Geyskes, G G; Vos, J; Dorhout Mees, E J

    1978-01-01

    The effect of antikaliuretic agents (spironolactone, amiloride) and antiprostaglandin agents (indomethacin, ibuprofen) on plasma renin activity and potassium metabolism was studied in a patient with familial Bartter's syndrome. The decrease in renal potassium clearance in response to antikaliuretic treatment was only of a temporary nature, in contrast to the marked potassium retention seen after indomethacin therapy. Plasma renin activity showed a consistent increase after kaliuretics and a marked decrease in response to antiprostaglandin agents. The opposite effect of the drugs on plasma renin activity, due to their different sites of action on the pathophysiologic chain of events in Bartter's syndrome, given an explanation for the contrasting therapeutic results. PMID:631965

  2. Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version.

    PubMed

    Masaki, Yasufumi; Kawabata, Hiroshi; Takai, Kazue; Kojima, Masaru; Tsukamoto, Norifumi; Ishigaki, Yasuhito; Kurose, Nozomu; Ide, Makoto; Murakami, Jun; Nara, Kenji; Yamamoto, Hiroshi; Ozawa, Yoko; Takahashi, Hidekazu; Miura, Katsuhiro; Miyauchi, Tsutomu; Yoshida, Shinichirou; Momoi, Akihito; Awano, Nobuyasu; Ikushima, Soichiro; Ohta, Yasunori; Furuta, Natsue; Fujimoto, Shino; Kawanami, Haruka; Sakai, Tomoyuki; Kawanami, Takafumi; Fujita, Yoshimasa; Fukushima, Toshihiro; Nakamura, Shigeo; Kinoshita, Tomohiro; Aoki, Sadao

    2016-06-01

    TAFRO syndrome is a systemic inflammatory disorder characterized by thrombocytopenia, anasarca including pleural effusion and ascites, fever, renal insufficiency, and organomegaly including hepatosplenomegaly and lymphadenopathy. Its onset may be acute or sub-acute, but its etiology is undetermined. Although several clinical and pathological characteristics of TAFRO syndrome resemble those of multicentric Castleman disease (MCD), other specific features can differentiate between them. Some TAFRO syndrome patients have been successfully treated with glucocorticoids and/or immunosuppressants, including cyclosporin A, tocilizumab and rituximab, whereas others are refractory to treatment, and eventually succumb to the disease. Early and reliable diagnoses and early treatments with appropriate agents are essential to enhancing patient survival. The present article reports the 2015 updated diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, as formulated by Japanese research teams. These criteria and classification have been applied and retrospectively validated on clinicopathologic data of 28 patients with this and similar conditions (e.g. MCD with serositis and thrombocytopenia). PMID:27084250

  3. Mandibular advancement splints for the treatment of sleep apnea syndrome.

    PubMed

    Sutherland, K; Cistulli, P

    2011-01-01

    Oral devices, in particular Mandibular Advancement Splints (MAS), which hold the mandible in a protruded position during sleep, are increasingly used for the treatment of Obstructive Sleep Apnoea (OSA). These devices can be effective in treating OSA across a range of severity. Complete resolution of OSA (Apnoea-Hypopnoea Index [AHI] reduced <5/hr) with use of an MAS occurs in around 40% of patients. Overall two thirds of patients experience some clinical benefit (≥50% AHI reduction AHI) however others will not objectively respond to this form of treatment, despite improvement in symptoms. Although MAS are less efficacious in reducing polysomnographic indices of OSA than the standard treatment, Continuous Positive Airway Pressure (CPAP), improvements in health outcomes appear to be comparable. Therefore, the superiority of CPAP in improving oxygen desaturations and reducing AHI may be extenuated by its low compliance, resulting in both treatments having similar effectiveness in clinical practice. MAS are now recommended as a first line treatment for mild to moderate OSA, as well as in more severe patients who are unable to tolerate or refuse CPAP. Success with MAS treatment has been associated with factors such as female gender, younger age, supine-dependent OSA, lower BMI, smaller neck circumference and craniofacial factors, however a reliable, validated method for prediction in the clinical setting has yet to be established. MAS are well tolerated, however short-term side effects are common although generally minor and transient. Long-term dental changes are for the most part subclinical, but can be problematic for a minority of patients. MAS are a dental-based treatment for a medical sleep disorder and, as such, an interdisciplinary care model is considered important for the attainment of optimal patient outcomes. PMID:21956677

  4. Treatment of recurrent toxic shock syndrome with oral contraceptive agents.

    PubMed

    McIvor, M E; Levin, M L

    1982-09-01

    The case of a 20-year old woman who was hospitalized for toxic shock syndrome (TSS) for 4 consecutive months despite use of conventional therapy for prevention of recurrence is described. Following discharge after the 4th episode she was started on oral contraceptives (OCs), and her next 8 menstrual cycles were uneventful despite continued use of tampons. She since discontinued use of tampons and remains asymptomatic. TSS recurrences occur in 30-64% of patients and can follow the initial episode by up to 41 months. The experience of this patient demonstrates the limitations of using antistaphyloccal antibiotics at the end of each menstrual cycle but suggests that hormonal manipulation may be an effective alternative therapy for TSS recurrences. Epidemiological studies show TSS patients use OCs less frequently than controls. Whether OCs suppress the vaginal flora, as some evidence suggests, or make conditions less favorable for the development of TSS in some other way is unknown. OC therapy for recurrences of TSS should receive further study. PMID:7144265

  5. Novel nutraceutic therapies for the treatment of metabolic syndrome.

    PubMed

    Martínez-Abundis, Esperanza; Méndez-Del Villar, Miriam; Pérez-Rubio, Karina G; Zuñiga, Laura Y; Cortez-Navarrete, Marisol; Ramírez-Rodriguez, Alejandra; González-Ortiz, Manuel

    2016-04-10

    Nutraceutic therapies such as berberine, bitter melon, Gymnema sylvestre, Irvingia gabonensis, resveratrol and ursolic acid have been shown to help control metabolic syndrome (MetS). The effect of berberine on glucose and lipid metabolism, hypertension, obesity and MetS has been evaluated in animal models and humans. Most clinical trials involving bitter melon have been conducted to evaluate its effect on glucose metabolism; nevertheless, some studies have reported favorable effects on lipids and blood pressure although there is little information about its effect on body weight. Gymnema sylvestre helps to decrease body weight and blood sugar levels; however, there is limited information on dyslipidemia and hypertension. Clinical trials of Irvingia gabonensis have shown important effects decreasing glucose and cholesterol concentrations as well decreasing body weight. Resveratrol acts through different mechanisms to decrease blood pressure, lipids, glucose and weight, showing its effects on the population with MetS. Finally, there is evidence of positive effects with ursolic acid in in vitro and in vivo studies on glucose and lipid metabolism and on body weight and visceral fat. Therefore, a review of the beneficial effects and limitations of the above-mentioned nutraceutic therapies is presented. PMID:27076875

  6. Novel nutraceutic therapies for the treatment of metabolic syndrome

    PubMed Central

    Martínez-Abundis, Esperanza; Méndez-del Villar, Miriam; Pérez-Rubio, Karina G; Zuñiga, Laura Y; Cortez-Navarrete, Marisol; Ramírez-Rodriguez, Alejandra; González-Ortiz, Manuel

    2016-01-01

    Nutraceutic therapies such as berberine, bitter melon, Gymnema sylvestre, Irvingia gabonensis, resveratrol and ursolic acid have been shown to help control metabolic syndrome (MetS). The effect of berberine on glucose and lipid metabolism, hypertension, obesity and MetS has been evaluated in animal models and humans. Most clinical trials involving bitter melon have been conducted to evaluate its effect on glucose metabolism; nevertheless, some studies have reported favorable effects on lipids and blood pressure although there is little information about its effect on body weight. Gymnema sylvestre helps to decrease body weight and blood sugar levels; however, there is limited information on dyslipidemia and hypertension. Clinical trials of Irvingia gabonensis have shown important effects decreasing glucose and cholesterol concentrations as well decreasing body weight. Resveratrol acts through different mechanisms to decrease blood pressure, lipids, glucose and weight, showing its effects on the population with MetS. Finally, there is evidence of positive effects with ursolic acid in in vitro and in vivo studies on glucose and lipid metabolism and on body weight and visceral fat. Therefore, a review of the beneficial effects and limitations of the above-mentioned nutraceutic therapies is presented. PMID:27076875

  7. Ehlers-danlos syndrome in orthopaedics: etiology, diagnosis, and treatment implications.

    PubMed

    Shirley, Eric D; Demaio, Marlene; Bodurtha, Joanne

    2012-09-01

    Ehlers-Danlos syndrome is a heterogeneous connective tissue condition characterized by varying degrees of skin hyperextensibility, joint hypermobility, and vascular fragility. Joint dislocations, musculoskeletal pain, atrophic scars, easy bleeding, vessel/viscera rupture, severe scoliosis, and obstetric complications may occur. These manifestations are secondary to abnormal collagen, with specific molecular defects in types I, III, and V collagen; they may also be related to tenascin-X, which has been identified in some patients. Ehlers-Danlos syndrome has been classified into 6 types, with variable degrees of joint instability, skin hyperextensibility, wound healing difficulty, and vascular fragility. Diagnosis begins with recognition of the signs and symptoms of global hypermobility and referring appropriate patients for genetic consultation. It is important to accurately identify patients with Ehlers-Danlos syndrome to initiate appropriate musculoskeletal treatment, optimize anesthetic and postoperative management, perform appropriate vascular screening, and help families address their concerns with other families and advocacy groups. PMID:23016112

  8. Habilitational treatment of a child with fetal alcohol syndrome: case report.

    PubMed

    Matijević, Valentina; Bartolović, Jelena; Crnković, Maja; Kosicek, Tena; Barisić, Irma

    2014-03-01

    Fetal alcohol syndrome is defined by a triad of symptoms such as facial dysmorphology, prenatal and postnatal growth deficiency, and central nervous system dysfunction. It is the result of teratogenic effects of alcohol consumption in pregnancy. The prevalence of fetal alcohol syndrome is 1 to 3 per 1000 live births. From the neurological point of view, there is a possibility of the central nervous system dysfunction. Structural disjunctions are the consequences of fine and gross motor dysfunction, oculomotor dysfunction, and difficulties in sensorimotor integration. From the functional point view, there are complex cognitive disorders and behavioral disorders, attention disorders and impulse control disorders, learning difficulties, and social communication and perception difficulties. This paper presents a case study of a boy diagnosed with fetal alcohol syndrome at the age of four, monitored by a team of experts including a physiatrist and neuropediatrician. The boy is also included in polyvalent habilitation treatment provided by a speech therapist, rehabilitator and psychologist. PMID:24974670

  9. Successful treatment of reactive airways dysfunction syndrome by high-dose vitamin D.

    PubMed

    Varney, Veronica A; Evans, Jane; Bansal, Amolak S

    2011-01-01

    Reactive airways dysfunction syndrome is a controversial and poorly understood condition produced by inhalational injury from gas, vapors, or fumes. The symptoms mimic asthma, but appear unresponsive to asthma treatments. If symptoms persist for more than 6 months, there is a risk that they can become chronic. For these cases, effective treatments are lacking and quality of life is poor. We describe the first use of high-dose vitamin D in a patient with this condition, who fulfilled the 1995 American College of Chest Physicians criteria for this syndrome. The patient we describe presented an extremely difficult management problem and was refractory to conventional treatments, but responded to high-dose oral vitamin D supplements. PMID:22034572

  10. Successful Treatment of Lower Limb Complex Regional Pain Syndrome following Three Weeks of Hyperbaric Oxygen Therapy.

    PubMed

    Katznelson, Rita; Segal, Shira C; Clarke, Hance

    2016-01-01

    Hyperbaric oxygen therapy (HBOT) is a treatment that delivers 100% oxygen at increased atmospheric pressures. The efficacy of HBOT for treating pain has been described in various animal pain models and may have clinical efficacy in the treatment of human chronic pain syndromes. We present our experience with posttraumatic Complex Regional Pain Syndrome (CRPS) type 2 in a patient who underwent 15 sessions of HBOT. A 41-year-old male with one-year history of CRPS of left foot followed by left ankle fracture demonstrated less pain, decreased swelling, less allodynia, and improvement in skin color and range of motion of the lower limb after 3 weeks of HBOT. Patient was back to work for the first time in over a year. HBOT may be considered as a valuable therapeutic tool in the treatment of long-standing CRPS. PMID:27445607

  11. Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome – effective treatment with tocilizumab

    PubMed Central

    Kwiatkowska, Małgorzata; Jednacz, Ewa

    2015-01-01

    A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented. In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5th year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy's condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

  12. Successful Treatment of Lower Limb Complex Regional Pain Syndrome following Three Weeks of Hyperbaric Oxygen Therapy

    PubMed Central

    Katznelson, Rita; Segal, Shira C.; Clarke, Hance

    2016-01-01

    Hyperbaric oxygen therapy (HBOT) is a treatment that delivers 100% oxygen at increased atmospheric pressures. The efficacy of HBOT for treating pain has been described in various animal pain models and may have clinical efficacy in the treatment of human chronic pain syndromes. We present our experience with posttraumatic Complex Regional Pain Syndrome (CRPS) type 2 in a patient who underwent 15 sessions of HBOT. A 41-year-old male with one-year history of CRPS of left foot followed by left ankle fracture demonstrated less pain, decreased swelling, less allodynia, and improvement in skin color and range of motion of the lower limb after 3 weeks of HBOT. Patient was back to work for the first time in over a year. HBOT may be considered as a valuable therapeutic tool in the treatment of long-standing CRPS. PMID:27445607

  13. [ZHANG Tangfa's characteristics of acupuncture academic ideology and clinical treatment of syndrome differentiation].

    PubMed

    Zhang, Hongxing

    2015-10-01

    Through collecting and sorting of works, literature and medical cases regarding professor ZHANG Tangfa, it is found that his acupuncture academic ideology and clinical treatment of syndrome differentiation can be summarized as: tracing the source and paying attention to basic theory, especially the meridian theory and conception vessel and governor vessel; focusing on acupuncture manipulation and emphasizing acupuncture basic skills; highly valuing treating spirit, acquiring and maintaining needling sensation; underlining "three differentiations" that is consisted of syndrome differentiation, disease differentiation and meridian differentiation to guide the clinical prescriptions of acupoints; exploring and ingenious use of scalp acupuncture; being concerned on research of difficult and complicated diseases; advocating comparative studies to optimize the clinical treatment plan; proposing the combination of Chinese and western medicine, including diagnosis, treatment and basic theory, to improve the clinical therapeutic effects of acupuncture. PMID:26790219

  14. Ovarian drilling for surgical treatment of polycystic ovarian syndrome: a comprehensive review.

    PubMed

    Fernandez, Hervé; Morin-Surruca, Michèle; Torre, Antoine; Faivre, Erika; Deffieux, Xavier; Gervaise, Amélie

    2011-06-01

    This systematic literature review is intended to clarify and evaluate the results obtained by ovarian drilling as surgical treatment for polycystic ovarian syndrome (PCOS). Four databases were consulted (Medline at the National Library of Medicine, USA; Cochrane Library, UK; National Guideline Clearinghouse, USA; and the Health Technology Assessment Database, Sweden) and searched for 'polycystic ovary syndrome' plus 'drilling' in the title or abstract. The assessment criteria used to define the efficacy of the procedure were the rates of ovulation, clinical pregnancy and early miscarriage. Alternatives to surgical ovarian drilling were evaluated. This search produced 147 references, 81 of which met the selection criteria. This review of infertility management in women with PCOS indicates that ovarian drilling is a second-line treatment when treatment with clomiphene citrate fails to lead to conception. The benefits of ovarian drilling are that it does not induce either hyperstimulation syndrome or multiple pregnancies. It is concluded that ovarian drilling is an option in the management of female infertility associated with PCOS, especially as a second-line treatment after the failure of clomiphene citrate treatment. PMID:21511534

  15. Prevention and treatment of Wernicke-Korsakoff syndrome.

    PubMed

    Cook, C C

    2000-01-01

    Wernicke's encephalopathy (WE) is both common and associated with high morbidity and mortality and yet there is evidence that appropriate and effective prophylaxis and treatment are often not given. Effective treatment and prophylaxis may only be achieved by use of parenteral vitamin supplements, since oral supplements are not absorbed in significant amounts. Although there are rare anaphylactoid reactions associated with the use of parenteral thiamine preparations, the risks and consequences of inadequate prophylaxis and treatment, in appropriately targeted groups of patients, are far greater. It is therefore proposed that all in-patient alcohol withdrawal should be covered by prophylactic use of parenteral thiamine, that there should be a low threshold for making a presumptive diagnosis of WE, and that there is a need for guidelines to assist physicians in appropriate management of this common clinical problem. PMID:11304070

  16. Vascular endothelial growth factor as a predictive marker for POEMS syndrome treatment response: retrospective cohort study

    PubMed Central

    Misawa, S; Sato, Y; Katayama, K; Hanaoka, H; Sawai, S; Beppu, M; Nomura, F; Shibuya, K; Sekiguchi, Y; Iwai, Y; Watanabe, K; Amino, H; Ohwada, C; Takeuchi, M; Sakaida, E; Nakaseko, C; Kuwabara, S

    2015-01-01

    Objective POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome is a rare multisystem disease characterised by plasma cell dyscrasia and overproduction of vascular endothelial growth factor (VEGF). VEGF is assumed to be useful in monitoring disease activity, because VEGF levels usually decrease after treatment. However, there is no study to investigate whether the extent of decrease in VEGF correlates with clinical outcome. We tested the predictive efficacy of serum VEGF levels in POEMS syndrome. Method This was an institutional review board approved retrospective observational cohort study of 20 patients with POEMS monitored regularly for more than 12 months (median follow-up, 87 months) after treatment onset using our prospectively accumulated database of POEMS from 1999 to 2015. Patients were treated by autologous peripheral blood stem cell transplantation or thalidomide administration. Serum VEGF was measured by ELISA. Outcome measures included clinical and laboratory findings and relapse-free survival. Results Serum VEGF levels decreased rapidly after treatment, and stabilised by 6 months post treatment. Patients with normalised serum VEGF levels (<1040 pg/mL) at 6 months showed prolonged relapse-free survival (HR=12.81, 95% CI 2.691 to 90.96; p=0.0001) and greater later clinical improvement. The rate of serum VEGF reduction over the first 6 months post treatment correlated with increased grip strength, serum albumin levels, and compound muscle action potential amplitudes at 12 months. Conclusions Serum VEGF level at 6 months post treatment is a predicative biomarker for disease activity and prognosis in POEMS syndrome. Serum VEGF could be used as a surrogate endpoint for relapse-free survival or clinical or laboratory improvement of POEMS syndrome for clinical trials. PMID:26560063

  17. Single injection of platelet-rich plasma as a novel treatment of carpal tunnel syndrome

    PubMed Central

    Malahias, Michael Alexander; Johnson, Elizabeth O.; Babis, George C.; Nikolaou, Vasileios S.

    2015-01-01

    Both in vitro and in vivo experiments have confirmed that platelet-rich plasma has therapeutic effects on many neuropathies, but its effects on carpal tunnel syndrome remain poorly understood. We aimed to investigate whether single injection of platelet-rich plasma can improve the clinical symptoms of carpal tunnel syndrome. Fourteen patients presenting with median nerve injury who had suffered from mild carpal tunnel syndrome for over 3 months were included in this study. Under ultrasound guidance, 1–2 mL of platelet-rich plasma was injected into the region around the median nerve at the proximal edge of the carpal tunnel. At 1 month after single injection of platelet-rich plasma, Visual Analogue Scale results showed that pain almost disappeared in eight patients and it was obviously alleviated in three patients. Simultaneously, the disabilities of the arm, shoulder and hand questionnaire showed that upper limb function was obviously improved. In addition, no ultrasonographic manifestation of the carpal tunnel syndrome was found in five patients during ultrasonographic measurement of the width of the median nerve. During 3-month follow-up, the pain was not greatly alleviated in three patients. These findings show very encouraging mid-term outcomes regarding use of platelet-rich plasma for the treatment of carpal tunnel syndrome. PMID:26807124

  18. Evaluation of drug treatment in irritable bowel syndrome

    PubMed Central

    Talley, Nicholas J

    2003-01-01

    The irritable bowel syndrome (IBS) remains a therapeutic challenge in part because of the limited understanding of the pathophysiology. The placebo response rate varies in randomized controlled trials from 20 to 70%, and can persist for up to at least 1 year. It is contentious whether dietary fibre and bulking agents relieve the symptoms of IBS; constipation probably improves. Anticholinergic and antispasmodic agents are of questionable benefit in IBS despite positive meta-analyses of poor quality trials. A meta-analysis concluded that the tricyclic antidepressants were superior to placebo in IBS, although the individual trial results were variable. Selective serotonin reuptake inhibitors are of uncertain benefit. Laxatives are used for constipation but probably poorly control the IBS symptom complex. Loperamide is superior to placebo in improvement of diarrhoea but not abdominal pain in IBS. Tegaserod is a well- tolerated aminoguanidine indole derivative of serotonin that is a partial 5HT4–receptor agonist with prokinetic properties; a therapeutic gain over placebo of 5% to 15% has been observed in constipation-predominant IBS in females. Alosetron is a 5HT3-receptor antagonist that is efficacious in females with diarrhoea-predominant IBS, with a 12% to 17% therapeutic gain; the risk of ischaemic colitis is 1 in 350, with very severe constipation occurring in about 1 in 1000. Optimizing study design remains a challenge in IBS. New visceral analgesic and motility modifying agents, as well as anti-inflammatory agents are in trials, and hopefully additional efficacious therapeutic options for patients with IBS will soon emerge. PMID:12968980

  19. Targeted Treatments in Autism and Fragile X Syndrome

    ERIC Educational Resources Information Center

    Gurkan, C. Kagan; Hagerman, Randi J.

    2012-01-01

    Autism is a neurodevelopmental disorder consisting of a constellation of symptoms that sometimes occur as part of a complex disorder characterized by impairments in social interaction, communication and behavioral domains. It is a highly disabling disorder and there is a need for treatment targeting the core symptoms. Although autism is accepted…

  20. Long-term treatment of irritable bowel syndrome: results of a randomized controlled trial.

    PubMed

    Misra, S P; Thorat, V K; Sachdev, G K; Anand, B S

    1989-10-01

    To examine the long-term management of irritable bowel syndrome we conducted a two-part controlled therapeutic trial on 28 patients who had recovered completely after four to six weeks of treatment with ispaghula husk and propantheline. In part I patients were randomly divided into two groups. Group A received a placebo capsule while Group B continued with treatment as before. After six months the response to treatment was assessed according to a scoring system. The overall relapse rate in Group B was 46 per cent compared to 82 per cent in group A. With continued treatment patients in Group B became asymptomatic from the fourth month while patients in Group A continued to deteriorate. In part II, patients who had relapsed whilst on placebo received active treatment. Six of the seven who agreed to continue with the study became asymptomatic within four weeks. However, all the patients who were asymptomatic while on active treatment relapsed on discontinuation and again recovered on reinstitution of active treatment. We conclude that irritable bowel syndrome is a chronic relapsing disorder and that treatment with a combination of ispaghula husk and propantheline is effective, both in relieving symptoms and in the maintenance of remission. PMID:2697886

  1. [New method for treatment of chronic blepharitis associated with dry eye syndrome].

    PubMed

    Safonova, T N; Zabegaĭlo, A O; Fedorov, A A; Lukisheva, O V

    2014-01-01

    A new combined method for treatment of chronic blepharitis associated with dry eye syndrome is developed. A total of 25 patients were enrolled. The treatment implied the use of artificial tears, Restasis, and Blephasteam spectacle frame. The course included 20 procedures on daily basis. The monitoring period was 1 year. A pronounced anti-inflammatory, reparative, and moistening effect as well as stabilization of the precorneal film was achieved in short time. Owing to the combined approach, negative side effects (such as burning, conjunctival irritation, and fluctuating vision) were minimized. Repeated treatment courses maintained the positive therapeutic effect and prolongated the remission. PMID:24684070

  2. A Review of Thoracic Outlet Syndrome and the Possible Role of Botulinum Toxin in the Treatment of This Syndrome

    PubMed Central

    Foley, Jacqueline Mary; Finlayson, Heather; Travlos, Andrew

    2012-01-01

    The objective of this paper is to discuss the classification, diagnosis, pathophysiology and management of Thoracic outlet syndrome (TOS). Thoracic outlet syndrome (TOS) is a complex entity that is characterized by different neurovascular signs and symptoms involving the upper limb. TOS is defined as upper extremity symptoms due to compression of the neurovascular bundle in the area of the neck just above the first rib. Compression is thought to occur at one or more of the three anatomical compartments: the interscalene triangle, the costoclavicular space and the retropectoralis minor spaces. The clinical presentation can include both neurogenic and vascular symptoms. TOS can be difficult to diagnose because there is no standardized objective test that can be used and the clinician must rely on history and several positive findings on physical exam. The medial antebrachial cutaneous nerve conduction may be a sensitive way to detect pathology in the lower trunks of the brachial plexus which is promising for future research. Treatment options continue to be conservative and surgical. However, for those who have failed physical therapy there is research to suggest that botulinum toxin may help with symptom relief. However, given that there has been conflicting evidence, further research is required using randomized controlled trials. PMID:23202313

  3. [Prevention and treatment of the complications of polycystic ovarian syndrome--the significance of evidence-based, interdisciplinary management].

    PubMed

    Gődény, Sándor; Csenteri, Orsolya Karola

    2015-12-13

    Polycystic ovary syndrome is the most common hormonal and metabolic disorder likely to affect women. The syndrome is often associated with obesity, hyperinsulinemia and adversely affects endocrine, metabolic, and cardiovascular health. The complex feature of the syndrome requires an interdisciplinary approach to treatment, where cooperation of paediatrician, internist, gynaecologist, endocrinologist, dermatologist, psychologist and oncologist is essential. The prevention and the treatment should be based on the best available evidence. This should include physical examination, laboratory tests for hormones, serum insulin, glucose, lipids, in addition patient's preferences should be considered, too. To maximise health gain of polycystic ovarian syndrome, adequate, effective, efficient and safe treatment is necessary. This article summarises the highest available evidence provided by meta-analyses and systematic reviews of the prevention of metabolic and cardiovascular complications of the syndrome, and discusses the relevant evidence published in the literature. PMID:26639643

  4. [Treatment of Poland syndrome thorax deformity with the lipomodeling technique: about ten cases].

    PubMed

    La Marca, S; Delay, E; Toussoun, G; Ho Quoc, C; Sinna, R

    2013-02-01

    The correct management, with the classic techniques, of the thoracic deformity in Poland's syndrome is difficult, with often insatisfactory results. The current surgical treatment involves the use of prothetic material and/or different flaps with their own complications and scares. The experience of our team with fat grafting (we named lipomodeling) in breast reconstruction helped us to propose the correction of the thoracic and mammary deformity by repeated fat transfer sessions. Fat grafting is commonly used in our team since 1998 in various indication of breast surgery. We reviewed retrospectively our ten first cases of thoracic deformity in Poland's syndrome treated with only fat grafting. Patients had repeated procedures until obtaining a satisfactory result. The fat was harvested from the thigh, buttocks, and abdomen. There were young patients with a mean age of 16years old (from 12 to 24). The mean follow-up was 51months. An average of 2.9 procedures (1 to 5) with 255cm(3) of fat injection at each procedure was needed to obtain symetry. Hundred percent of the patients were satisfied. No complication was noted. As reported, the reconstruction of the thoracic deformity and the mammary shape can be obtained by fat grafting. The absence of a flap donor site sequelae and the absence an implant allow this technique to be simple, reproductible, and without great complication. These criteria match well the surgical management of this deformity, which is mainly aesthetic. Moreover, the secondary benefit of liposuction of disgracious steatomery helps the acceptance of the procedure. Therefore in our hands, fat grafting to the breast (lipomodeling) is now our first choice treatment in thoracic Poland syndrome deformity. Given the rarity of this syndrome, we recommend a treatment by an operator who makes the learning curve of lipomodeling, and who often deals with Poland syndrome. PMID:23153632

  5. Successful use of infliximab in the treatment of Reiter's syndrome: a case report and discussion.

    PubMed

    Gill, Himmat; Majithia, Vikas

    2008-01-01

    Reiter's syndrome is one of the reactive forms of seronegative spondyloarthropathies. Various therapies used in the management of Reiter's syndrome are nonsteroidal antiinflammatory drugs (NSAIDs), antibiotics, and disease-modifying antirheumatic drugs (DMARDs) such as sulfasalazine (SSZ) or methotrexate (MTX). There is only one case report of successful treatment of Reiter's syndrome with tumor necrosis factor-alpha (TNF-alpha) blockers in human immunodeficiency virus (HIV) patient (Gaylis N, 2003, J Rheumatol 30(2):407-411 Feb). We hereby report a case of Reiter's syndrome treated successfully with infliximab, an anti-TNF-alpha chimeric monoclonal antibody. A 28-year-old white male presented with painful swelling of right elbow and ankle joints, urethritis. and lesions involving skin of soles of feet and penis. Detailed work-up of sexually transmitted diseases (STDs), HIV, and systemic etiology were negative. Despite aggressive treatment with antibiotics, NSAIDS, prednisone, and MTX for 3 months, he had persistent synovitis and worsening of skin lesions. He was then treated with infliximab 200 mg intravenously at weeks 0, 2, 6, and 14 weeks which resulted in complete resolution of arthritis and skin lesions within 6 weeks of infliximab therapy. PMID:17643185

  6. Signal transduction inhibitors in treatment of myelodysplastic syndromes.

    PubMed

    Bachegowda, Lohith; Gligich, Oleg; Mantzaris, Ionnis; Schinke, Carolina; Wyville, Dale; Carrillo, Tatiana; Braunschweig, Ira; Steidl, Ulrich; Verma, Amit

    2013-01-01

    Myelodysplastic syndromes (MDS) are a group of hematologic disorders characterized by ineffective hematopoiesis that results in reduced blood counts. Although MDS can transform into leukemia, most of the morbidity experienced by these patients is due to chronically low blood counts. Conventional cytotoxic agents used to treat MDS have yielded some encouraging results but are characterized by many adverse effects in the predominantly elderly patient population. Targeted interventions aimed at reversing the bone marrow failure and increasing the peripheral blood counts would be advantageous in this cohort of patients. Studies have demonstrated over-activated signaling of myelo-suppressive cytokines such as TGF-β, TNF-α and Interferons in MDS hematopoietic stem cells. Targeting these signaling cascades could be potentially therapeutic in MDS. The p38 MAP kinase pathway, which is constitutively activated in MDS, is an example of cytokine stimulated kinase that promotes aberrant apoptosis of stem and progenitor cells in MDS. ARRY-614 and SCIO-469 are p38 MAPK inhibitors that have been used in clinical trials and have shown activity in a subset of MDS patients. TGF-β signaling has been therapeutically targeted by small molecule inhibitor of the TGF-β receptor kinase, LY-2157299, with encouraging preclinical results. Apart from TGF-β receptor kinase inhibition, members of TGF-β super family and BMP ligands have also been targeted by ligand trap compounds like Sotatercept (ACE-011) and ACE-536. The multikinase inhibitor, ON-01910.Na (Rigosertib) has demonstrated early signs of efficacy in reducing the percentage of leukemic blasts and is in advanced stages of clinical testing. Temsirolimus, Deforolimus and other mTOR inhibitors are being tested in clinical trials and have shown preclinical efficacy in CMML. EGF receptor inhibitors, Erlotinib and Gefitinib have shown efficacy in small trials that may be related to off target effects. Cell cycle regulator inhibitors

  7. Signal transduction inhibitors in treatment of myelodysplastic syndromes

    PubMed Central

    2013-01-01

    Myelodysplastic syndromes (MDS) are a group of hematologic disorders characterized by ineffective hematopoiesis that results in reduced blood counts. Although MDS can transform into leukemia, most of the morbidity experienced by these patients is due to chronically low blood counts. Conventional cytotoxic agents used to treat MDS have yielded some encouraging results but are characterized by many adverse effects in the predominantly elderly patient population. Targeted interventions aimed at reversing the bone marrow failure and increasing the peripheral blood counts would be advantageous in this cohort of patients. Studies have demonstrated over-activated signaling of myelo-suppressive cytokines such as TGF-β, TNF-α and Interferons in MDS hematopoietic stem cells. Targeting these signaling cascades could be potentially therapeutic in MDS. The p38 MAP kinase pathway, which is constitutively activated in MDS, is an example of cytokine stimulated kinase that promotes aberrant apoptosis of stem and progenitor cells in MDS. ARRY-614 and SCIO-469 are p38 MAPK inhibitors that have been used in clinical trials and have shown activity in a subset of MDS patients. TGF-β signaling has been therapeutically targeted by small molecule inhibitor of the TGF-β receptor kinase, LY-2157299, with encouraging preclinical results. Apart from TGF-β receptor kinase inhibition, members of TGF-β super family and BMP ligands have also been targeted by ligand trap compounds like Sotatercept (ACE-011) and ACE-536. The multikinase inhibitor, ON-01910.Na (Rigosertib) has demonstrated early signs of efficacy in reducing the percentage of leukemic blasts and is in advanced stages of clinical testing. Temsirolimus, Deforolimus and other mTOR inhibitors are being tested in clinical trials and have shown preclinical efficacy in CMML. EGF receptor inhibitors, Erlotinib and Gefitinib have shown efficacy in small trials that may be related to off target effects. Cell cycle regulator inhibitors

  8. The usefulness of biliopancreatic diversion/Scopinaro operation in treatment of patients with Prader-Willi syndrome.

    PubMed

    Michalik, Maciej; Frask, Agata; Lech, Pawel; Zdrojewski, Michal; Doboszynska, Anna

    2015-07-01

    Prader-Willi syndrome (PWS) is the most common form of obesity with a genetic basis. The short expected survival time due to numerous accompanying diseases and their complications is the reason for research on the maximally efficient method of treatment of obesity in this syndrome. Undertaken attempts of conservative treatment, for example with somatostatin, are ineffective. It seems that the only effective treatment of obesity in this syndrome may be surgical. In this article we present 2 cases of patients with PWS who underwent surgery consisting of biliopancreatic diversion (BPD)/Scopinaro procedure. The BPD/Scopinaro operation in selected cases of disciplined patients with a co-operative family, which we find of key importance, can be considered as one option of treatment of this syndrome in patients with prior neglect of conservative treatment. PMID:26240637

  9. The usefulness of biliopancreatic diversion/Scopinaro operation in treatment of patients with Prader-Willi syndrome

    PubMed Central

    Michalik, Maciej; Frask, Agata; Zdrojewski, Michal; Doboszynska, Anna

    2015-01-01

    Prader-Willi syndrome (PWS) is the most common form of obesity with a genetic basis. The short expected survival time due to numerous accompanying diseases and their complications is the reason for research on the maximally efficient method of treatment of obesity in this syndrome. Undertaken attempts of conservative treatment, for example with somatostatin, are ineffective. It seems that the only effective treatment of obesity in this syndrome may be surgical. In this article we present 2 cases of patients with PWS who underwent surgery consisting of biliopancreatic diversion (BPD)/Scopinaro procedure. The BPD/Scopinaro operation in selected cases of disciplined patients with a co-operative family, which we find of key importance, can be considered as one option of treatment of this syndrome in patients with prior neglect of conservative treatment. PMID:26240637

  10. Updates and Future Horizons on the Understanding, Diagnosis, and Treatment of Sturge-Weber Syndrome Brain Involvement

    ERIC Educational Resources Information Center

    Lo, Warren; Marchuk, Douglas A.; Ball, Karen L.; Juhasz, Csaba; Jordan, Lori C.; Ewen, Joshua B.; Comi, Anne

    2012-01-01

    Aim: To review recent developments in the understanding, diagnosis, and treatment of Sturge-Weber syndrome (SWS). Method: Members of the Brain Vascular Malformation Consortium Sturge-Weber Syndrome National Workgroup contributed their expertise to review the literature and present promising directions for research. Results: The increasing number…

  11. Combined orthodontic and periodontic treatment in a child with Papillon Lefèvre syndrome

    PubMed Central

    AlSarheed, Maha A.; Al-Sehaibany, Fares S.

    2015-01-01

    A 9-year-old girl with Papillon-Lefèvre syndrome (PLS) was treated orthodontically 24 months after the start of mechanical and antibiotic therapy in adjunct with periodontal treatment every 6 weeks. After achieving stable periodontal conditions, orthodontic treatment was commenced to correct the teeth position, facial profile, and maxillary protraction. Following the combination therapy and a failure to detect Actinobacillus actinomycetemcomitans from any site in the oral cavity, orthodontic treatment with a fixed appliance was performed aside from creating space for eruption of permanent teeth. We found that combined periodontal and orthodontic treatment of PLS may be successful with a complex interdisciplinary regimen and close follow up. This is a 2-year follow-up case report of a girl with PLS. Orthodontic and periodontic therapy were offered using combined treatments of orthodontic and periodontal with the benefit of prosthodontic consultation, resulting in a treatment plan. PMID:26219452

  12. Recommendations on chronic constipation (including constipation associated with irritable bowel syndrome) treatment

    PubMed Central

    Paré, Pierre; Bridges, Ronald; Champion, Malcolm C; Ganguli, Subhas C; Gray, James R; Irvine, E Jan; Plourde, Victor; Poitras, Pierre; Turnbull, Geoffrey K; Moayyedi, Paul; Flook, Nigel; Collins, Stephen M

    2007-01-01

    While chronic constipation (CC) has a high prevalence in primary care, there are no existing treatment recommendations to guide health care professionals. To address this, a consensus group of 10 gastroenterologists was formed to develop treatment recommendations. Although constipation may occur as a result of organic disease, the present paper addresses only the management of primary CC or constipation associated with irritable bowel syndrome. The final consensus group was assembled and the recommendations were created following the exact process outlined by the Canadian Association of Gastroenterology for the following areas: epidemiology, quality of life and threshold for treatment; definitions and diagnostic criteria; lifestyle changes; bulking agents and stool softeners; osmotic agents; prokinetics; stimulant laxatives; suppositories; enemas; other drugs; biofeedback and behavioural approaches; surgery; and probiotics. A treatment algorithm was developed by the group for CC and constipation associated with irritable bowel syndrome. Where possible, an evidence-based approach and expert opinions were used to develop the statements in areas with insufficient evidence. The nature of the underlying pathophysiology for constipation is often unclear, and it can be tricky for physicians to decide on an appropriate treatment strategy for the individual patient. The myriad of treatment options available to Canadian physicians can be confusing; thus, the main aim of the recommendations and treatment algorithm is to optimize the approach in clinical care based on available evidence. PMID:17464377

  13. Treatment of infertility in women with polycystic ovary syndrome: approach to clinical practice

    PubMed Central

    Melo, Anderson Sanches; Ferriani, Rui Alberto; Navarro, Paula Andrea

    2015-01-01

    Polycystic ovary syndrome represents 80% of anovulatory infertility cases. Treatment initially includes preconception guidelines, such as lifestyle changes (weight loss), folic acid therapy to prevent the risk of fetal neural tube defects and halting the consumption of tobacco and alcohol. The first-line pharmacological treatment for inducing ovulation consists of a clomiphene citrate treatment for timed intercourse. The second-line pharmacological treatment includes the administration of exogenous gonadotropins or laparoscopic ovarian surgery (ovarian drilling). Ovulation induction using clomiphene citrate or gonadotropins is effective with cumulative live birth rates of approximately 70%. Ovarian drilling should be performed when laparoscopy is indicated; this procedure is typically effective in approximately 50% of cases. Finally, a high-complexity reproduction treatment (in vitro fertilization or intracytoplasmic sperm injection) is the third-line treatment and is recommended when the previous interventions fail. This option is also the first choice in cases of bilateral tubal occlusion or semen alterations that impair the occurrence of natural pregnancy. Evidence for the routine use of metformin in infertility treatment of anovulatory women with polycystic ovary syndrome is not available. Aromatase inhibitors are promising and longer term studies are necessary to prove their safety. PMID:26602525

  14. Treatment of infertility in women with polycystic ovary syndrome: approach to clinical practice.

    PubMed

    Melo, Anderson Sanches; Ferriani, Rui Alberto; Navarro, Paula Andrea

    2015-11-01

    Polycystic ovary syndrome represents 80% of anovulatory infertility cases. Treatment initially includes preconception guidelines, such as lifestyle changes (weight loss), folic acid therapy to prevent the risk of fetal neural tube defects and halting the consumption of tobacco and alcohol. The first-line pharmacological treatment for inducing ovulation consists of a clomiphene citrate treatment for timed intercourse. The second-line pharmacological treatment includes the administration of exogenous gonadotropins or laparoscopic ovarian surgery (ovarian drilling). Ovulation induction using clomiphene citrate or gonadotropins is effective with cumulative live birth rates of approximately 70%. Ovarian drilling should be performed when laparoscopy is indicated; this procedure is typically effective in approximately 50% of cases. Finally, a high-complexity reproduction treatment (in vitro fertilization or intracytoplasmic sperm injection) is the third-line treatment and is recommended when the previous interventions fail. This option is also the first choice in cases of bilateral tubal occlusion or semen alterations that impair the occurrence of natural pregnancy. Evidence for the routine use of metformin in infertility treatment of anovulatory women with polycystic ovary syndrome is not available. Aromatase inhibitors are promising and longer term studies are necessary to prove their safety. PMID:26602525

  15. The thrower's elbow: arthroscopic treatment of valgus extension overload syndrome.

    PubMed

    O'Holleran, James D; Altchek, David W

    2006-02-01

    Injury to the medial collateral ligament of the elbow (MCL) can be a career-threatening injury for an overhead athlete without appropriate diagnosis and treatment. It has been considered separately from other athletic injuries due to the unique constellation of pathology that results from repetitive overhead throwing. The past decade has witnessed tremendous gains in understanding of the complex interplay between the dynamic and static stabilizers of the athlete's elbow. Likewise, the necessity to treat these problems in a minimally invasive manner has driven the development of sophisticated techniques and instrumentation for elbow arthroscopy. MCL injuries, ulnar neuritis, valgus extension overload with osteophyte formation and posteromedial impingement, flexor pronator strain, medial epicondyle pathology, and osteochondritis dissecans (OCD) of the capitellum have all been described as sequelae of the overhead throwing motion. In addition, loose body formation, bony spur formation, and capsular contracture can all be present in conjunction with these problems or as isolated entities. Not all pathology in the thrower's elbow is amenable to arthroscopic treatment; however, the clinician must be familiar with all of these problems in order to form a comprehensive differential diagnosis for an athlete presenting with elbow pain, and he or she must be comfortable with the variety of open and arthroscopic treatments available to best serve the patient. An understanding of the anatomy and biomechanics of the thrower's elbow is critical to the care of this population. The preoperative evaluation should focus on a thorough history and physical examination, as well as on specific diagnostic imaging modalities. Arthroscopic setup, including anesthesia, patient positioning, and portal choices will be discussed. Operative techniques in the anterior and posterior compartments will be reviewed, as well as postoperative rehabilitation and surgical results. Lastly, complications

  16. Successful Endovascular Treatment of Iliac Vein Compression (May-Thurner) Syndrome in a Pediatric Patient

    SciTech Connect

    Oguzkurt, Levent Tercan, Fahri; Sener, Mesut

    2006-06-15

    A 10-year-old boy presented to our clinic with left lower extremity swelling present for 1 year with deterioration of symptoms during the prior month. Laboratory investigation for deep vein thrombosis was negative. Venography and computed tomography scan of the pelvis showed compression of the left common iliac vein by the right common iliac artery. A diagnosis of iliac vein compression syndrome was made. After venography, endovascular treatment was planned. The stenosis did not respond to balloon dilatation and a 12 mm Wallstent was placed with successful outcome. The patient's symptoms improved but did not resolve completely, probably due to a chronically occluded left superficial femoral vein that did not respond to endovascular recanalization. To the best of our knowledge, this is the first case of successful endovascular treatment of iliac vein compression syndrome with stent placement in a pediatric patient.

  17. Barraquer-Simons syndrome: a unique patient's perspective on diagnosis, disease progression and recontouring treatment.

    PubMed

    Heidemann, Lene Nyhøj; Thomsen, Jørn Bo; Sørensen, Jens Ahm

    2016-01-01

    This case report describes a female patient diagnosed with Barraquer-Simons syndrome, a rare form of acquired partial lipodystrophy characterised by symmetrical loss of adipose tissue from face, neck, upper extremities and the trunk with onset in early childhood. Initial symptoms were seen at the age of 8 years. Our patient did not show signs of renal impairment and this may be associated with the syndrome. Treatment of lipoatrophy in these patients is limited to cosmetic restoration, and autologous fat grafting has shown sustained positive effects with no or very little loss of volume at follow-ups. Furthermore, the treatment has resulted in considerable improvements in her quality of life and daily functioning. She has not experienced any adverse effects. Accurate and early diagnosis is important, and clinicians should consider early intervention for these patients. Autologous fat grafting is recommended as a safe procedure. PMID:27402657

  18. [Advances in the treatment of chronic prostatitis/chronic pelvic pain syndrome].

    PubMed

    Yang, Ming-Gen; Zhao, Xiao-Kun

    2008-12-01

    So far the etiology of chronic prostatitis (PC) and particularly chronic pelvic pain syndrome (CPPS) remains to be elucidated. According to recent epidemiologic data, CP is the most common urological disease in men below 50 years and occurs in 2.5%-16.0% of the world population. Since the 1990s, researchers of many countries have carried out deeper, more extensive and larger scaled studies than ever before on the etiology, diagnosis and treatment of the disease, with the sponsorship and coordination of such international institutions as the International Prostatitis Collaborative Network (IPCN), the Chronic Prostatitis Collaborative Research Network of the National Institute of Health (NIH-CPCRN) and so on. As prevalent as multiple sclerosis, CPPS is the most common yet most poorly understood "prostatitis syndrome". This article reviews the progress in the studies of the treatment of CPPS, explores the main problems and ventures the prospects for the development in this field. PMID:19157239

  19. From bladder to systemic syndrome: concept and treatment evolution of interstitial cystitis

    PubMed Central

    Dinis, Sara; de Oliveira, Joana Tavares; Pinto, Rui; Cruz, Francisco; Buffington, CA Tony; Dinis, Paulo

    2015-01-01

    Interstitial cystitis, presently known as bladder pain syndrome, has been recognized for over a century but is still far from being understood. Its etiology is unknown and the syndrome probably harbors different diseases. Autoimmune dysfunction, urothelial leakage, infection, central and peripheral nervous system dysfunction, genetic disease, childhood trauma/abuse, and subsequent stress response system dysregulation might be implicated. Management is slowly evolving from a solo act by the end-organ specialist to a team approach based on new typing and phenotyping of the disease. However, oral and invasive treatments are still largely aimed at the bladder and are based on currently proposed pathophysiologic mechanisms. Future research will better define the disease, permitting individualization of treatment. PMID:26229509

  20. Chiari malformation and central sleep apnea syndrome: efficacy of treatment with adaptive servo-ventilation*

    PubMed Central

    do Vale, Jorge Marques; Silva, Eloísa; Pereira, Isabel Gil; Marques, Catarina; Sanchez-Serrano, Amparo; Torres, António Simões

    2014-01-01

    The Chiari malformation type I (CM-I) has been associated with sleep-disordered breathing, especially central sleep apnea syndrome. We report the case of a 44-year-old female with CM-I who was referred to our sleep laboratory for suspected sleep apnea. The patient had undergone decompressive surgery 3 years prior. An arterial blood gas analysis showed hypercapnia. Polysomnography showed a respiratory disturbance index of 108 events/h, and all were central apnea events. Treatment with adaptive servo-ventilation was initiated, and central apnea was resolved. This report demonstrates the efficacy of servo-ventilation in the treatment of central sleep apnea syndrome associated with alveolar hypoventilation in a CM-I patient with a history of decompressive surgery. PMID:25410846

  1. Advances in the Treatment of Syndromic Midface Hypoplasia Using Monobloc and Facial Bipartition Distraction Osteogenesis

    PubMed Central

    Kumar, Anand R.; Steinbacher, Derek

    2014-01-01

    Midface hypoplasia or retrusion remains a persistent feature of syndromic craniosynostosis years after successful treatment of the cranium. Although expansion of the cranial vault in infancy by traditional fronto-orbital advancement, posterior expansion, or both, can treat the immediate intracranial constriction, midface hypoplasia and its stigmata of exorbitism, sleep apnea, central face concavity, and malocclusion remain suboptimally treated. Initial enthusiasm for the procedures was tempered due to a high rate of infectious complications; timing and indications for surgery continue to stir controversy. During the last decade renewed interest with the monobloc and facial bipartition procedure using distraction osteogenesis with either an internal or external distraction system has decreased morbidity significantly. These procedures have re-emerged as powerful and comprehensive tools in the treatment of syndromic midface hypoplasia. PMID:26417208

  2. Advances in the diagnosis and treatment of tumor necrosis factor receptor-associated periodic syndrome.

    PubMed

    Aguado-Gil, L; Irarrazaval-Armendáriz, I; Pretel-Irazabal, M

    2013-09-01

    Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a rare autosomal dominant disease included in the group of autoinflammatory syndromes. It is characterized by recurrent episodes of fever and inflammation in different regions of the body. The main clinical manifestations are myalgia, migratory erythematous rash, periorbital edema, and abdominal pain. The diagnosis is reached using gene analysis and prognosis depends on the appearance of amyloidosis secondary to the recurrent episodes of inflammation. Tumor necrosis factor inhibitors and corticosteroids are the most widely used treatments. In recent years, significant advances have been made in the diagnosis and treatment of TRAPS, thanks to a better understanding of its pathogenesis. Dermatologists must be aware that the skin manifestations of TRAPS are particularly important, as they are often diagnostic. PMID:23891452

  3. Treatment of the Guillain-Barré syndrome by plasma exchange.

    PubMed Central

    Kennard, C; Newland, A C; Ridley, A

    1982-01-01

    Twelve consecutive patients with Guillain-Barré syndrome were treated with plasma exchange. Examination two weeks after treatment was commenced showed that three had not improved. Five showed only a minimal improvement, which was considered to be compatible with the natural history of the disease. The remaining four patients showed a more substantial improvement which could have been related to the plasma exchange. PMID:7131022

  4. Corrective Neuromuscular Approach to the Treatment of Iliotibial Band Friction Syndrome: A Case Report

    PubMed Central

    Pettitt, Robert; Dolski, Angela

    2000-01-01

    Objective: To describe the evaluation and treatment process for inappropriate functional patterns of neuromuscular activity within the scope of an iliotibial band friction syndrome protocol. Background: Runners with iliotibial band friction syndrome are frequently fitted with orthotic devices to restrict excessive midfoot or rearfoot, or both, motions during the stance phase. These devices may fail to yield favorable results when underlying neuromuscular factors are associated with functional iliotibial band tightening. Differential Diagnosis: Distal biceps femoris tendinitis, popliteal tendinitis, lateral meniscus lesion. Treatment: The athlete's physical examination revealed several patterns of inappropriate neuromuscular activity attributed partly to the prolonged daily wear of beach-type sandals. Modifications of casual footwear and a temporary reduction in training volume were recommended initially to prevent exacerbation of the athlete's condition. Stretching, massage, and soft tissue mobilization were administered in accordance with the athlete's specific needs. The protocol included progressions of nonweightbearing and weightbearing therapeutic exercises. Neuromuscular electric stimulation was incorporated into the protocol to re-educate the role of the first ray within the stance phase of the athlete's walking gait. Uniqueness: Upon stationary examination, this athlete presented with normal lumbar and lower extremity postures. Gait analysis, however, revealed inappropriate dorsiflexion of the great toe during ambulation. Further, the athlete's performances on a series of tests to assess neuromuscular function were substandard. This athlete's response to previous treatment and unique physical findings required a corrective neuromuscular approach that deviates from iliotibial band friction syndrome protocols advocating the use of orthotics. Conclusions: While the role of any single treatment in the athlete's recovery remains unknown, it seems that a

  5. Surgical treatment of scoliosis in Smith-Magenis syndrome: a case report

    PubMed Central

    2010-01-01

    Introduction Smith-Magenis syndrome is a rare genetic condition associated with scoliosis in approximately 30% of cases. There is limited information in the literature on the treatment of scoliosis and the surgical outcome in patients with this condition. Characteristic features of the syndrome, such as the presence of congenital heart and renal disease, inherent immunodeficiency, as well as severe behavioural disorders may complicate the surgical treatment of patients. Case presentation We present the case of an 11-year-old British Caucasian girl with Smith-Magenis syndrome who developed a severe, progressive thoracic and lumbar scoliosis measuring 85° and 80°, respectively. She had no cardiac or renal anomalies. Brace treatment was unsuccessful to prevent deterioration of the scoliosis. Both curves were rigid on supine maximum side-bending and traction radiographs. Our patient underwent a posterior spinal arthrodesis with pedicle screw/hook and rod instrumentation and autologous iliac crest graft, supplemented by allograft bone. She had an uneventful postoperative course other than the development of a small wound dehiscence which required resuturing with no signs of a wound infection. A good correction of both scoliotic curvatures to 45° and 40° and a balanced spine in both the coronal and sagittal planes was achieved. Follow-up to skeletal maturity (4 years post-surgery) showed no loss of deformity correction, no detected pseudarthrosis and a good clinical outcome. Conclusion Patients with Smith-Magenis syndrome can develop a severe scoliosis that may require surgical treatment. Congenital cardiac and renal disease, immunodeficiency and severe behavioural problems can affect the surgical outcome following spinal arthrodesis and need to be taken into consideration. Our case demonstrates that surgical correction of the deformity can be performed safely on this group of patients, with a good outcome and an uncomplicated postoperative course. PMID:20181043

  6. Propranolol and prednisolone combination for the treatment of segmental haemangioma in PHACES syndrome.

    PubMed

    Gnarra, M; Solman, L; Harper, J; Batul Syed, S

    2015-07-01

    Posterior fossa malformations-haemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe syndrome (also known as PHACES syndrome) is a rare neurocutaneous disorder. Children presenting with these manifestations need careful ophthalmological, cardiac and neurological assessment. They may have one or more of these extracutaneous manifestations, the most common being cerebral and cardiovascular anomalies. There is controversy about treating these children with propranolol especially if they have cerebrovascular involvement with narrow, dysplastic or absent blood vessels. The concern with propranolol is that hypotension may lead to reduced cerebral blood flow and neurological consequences. Prior to propranolol the systemic treatment for haemangiomas was prednisolone and then the concern was the opposite, namely hypertension. Our proposal was whether a combination of these two drugs would provide a safer and faster recovery. We report three retrospective cases of PHACES syndrome, each of whom received treatment with a combination of propranolol and prednisolone: two children were started on prednisolone and propranolol was added because the haemangiomas failed to respond adequately; the third child was started on propranolol and developed peripheral ischaemia and ulceration necessitating a reduction in dose addition of a low dose of prednisolone. All three patients, who failed on the one treatment, responded well to combination therapy without any significant complications. These outcomes suggest that for some patients with PHACES syndrome the use of combination treatment with propranolol and prednisolone could be advantageous, potentially allowing for the introduction of low doses of each with an enhanced combined effect. The doses can be increased gradually depending on the magnetic resonance imaging findings. PMID:25639889

  7. Growth hormone treatment in a patient with Hurler-Scheie syndrome.

    PubMed

    Rogers, Douglas G; Nasomyont, Nat

    2014-09-01

    A female patient with known Hurler-Scheie syndrome, who underwent hematopoietic cell transplantation, presented with growth retardation and delayed puberty. She started growth hormone (GH) treatment at age 12.33 years, resulting in significantly improved linear growth and predicted adult height. We describe details of her clinical course and literature review of growth pattern as well as GH use in patients with mucopolysaccharidosis I. PMID:24825081

  8. Iodide iontophoresis as a treatment for dry eye syndrome

    PubMed Central

    Horwath-Winter, J; Schmut, O; Haller-Schober, E-M; Gruber, A; Rieger, G

    2005-01-01

    Background/aims: Among the causes related to the development or perpetuation and aggravation of dry eye disease, oxidative reactions may have a role in the pathogenesis of this disorder. Antioxidants, such as iodide, have shown a strong effect in preventing the oxidative damage to constituents of the anterior part of the eye. In this clinical trial the effectiveness of iodide iontophoresis and iodide application without current in moderate to severe dry eye patients was compared. Methods: 16 patients were treated with iodide iontophoresis and 12 patients with iodide application without current for 10 days. Subjective improvement, frequency of artificial tear application, tear function parameters (break up time, Schirmer test without local anaesthesia), vital staining (fluorescein and rose bengal staining) as well as impression cytology of the bulbar conjunctiva were evaluated before treatment, 1 week, 1 month, and 3 months after treatment. Results: A reduction in subjective symptoms, frequency of artificial tear substitute application, and an improvement in certain tear film and ocular surface factors could be observed in both groups. A stronger positive influence was seen after application of iodide with current (iontophoresis), as observed in a distinct improvement in break up time, fluorescein and rose bengal staining, and in a longer duration of this effect compared with the non-current group. No significant change in Schirmer test results and impression cytology were observed in both groups. Conclusions: Iodide iontophoresis has been demonstrated to be a safe and well tolerated method of improving subjective and objective dry eye factors in patients with ocular surface disease. PMID:15615744

  9. Natural history, symptoms and treatment of the narcoleptic syndrome.

    PubMed

    Parkes, J D; Baraitser, M; Marsden, C D; Asselman, P

    1975-11-01

    This study describes the clinical features, natural history and treatment of 100 patients with narcolepsy. Over half had one or more affected relatives. Symptoms commenced in adolescence or early adult life in most patients, and remissions were uncommon. Narcolepsy occurred several times each day, often in unusual circumstances and sometimes with little warning. The mean total sleep time of narco-leptics was a little over 9 hours in each 24 hour period, as compared with under 8 in normal subjects. Cataplexy occurred in 93 patients, most commonly when subjects were tired. Attacks were similar in nature to physiological weakness with laughter, although other sudden sensory or emotional stimuli did not cause paralysis of voluntary movement nor loss of muscle tone in normal subjects. Half these patients had frequent dreams before the onset of proper sleep, and 62 had sleep paralysis. This was often frightening, with feelings of suffocation, accompanied by dreams, and of uncertain length. A minority of patients with narcolepsy had muscle aches and jerks before sleep, double vision or loss of focus during cataplexy, went sleep-walking by day, and had daytime hallucinations. Amphetamines had been given to 71 patients for periods of up to 33 years with adequate, but rarely complete, control of narcolepsy. Side effects were common and almost half these patients became tolerant, needing higher dosage to control symptoms. Three patients had a cerebrovascular accident whilst taking amphetamines. Imipramine or clomipramine had ben given in combination with amphetamines to 33 patients for periods of up to 6 years with considerable improvement in both cataplexy and sleep paralysis, and few side effects. Sustained or paroxysmal hypertension as a result of amphetamines or combined treatment did not occur. PMID:19899267

  10. Water drinking as a treatment for orthostatic syndromes

    NASA Technical Reports Server (NTRS)

    Shannon, John R.; Diedrich, Andre; Biaggioni, Italo; Tank, Jens; Robertson, Rose Marie; Robertson, David; Jordan, Jens

    2002-01-01

    PURPOSE: Water drinking increases blood pressure in a substantial proportion of patients who have severe orthostatic hypotension due to autonomic failure. We tested the hypothesis that water drinking can be used as a practical treatment for patients with orthostatic and postprandial hypotension, as well as those with orthostatic tachycardia. SUBJECTS AND METHODS: We studied the effect of drinking water on seated and standing blood pressure and heart rate in 11 patients who had severe orthostatic hypotension due to autonomic failure and in 9 patients who had orthostatic tachycardia due to idiopathic orthostatic intolerance. We also tested the effect of water drinking on postprandial hypotension in 7 patients who had autonomic failure. Patients drank 480 mL of tap water at room temperature in less than 5 minutes. RESULTS: In patients with autonomic failure, mean (+/- SD) blood pressure after 1 minute of standing was 83 +/- 6/53 +/- 3.4 mm Hg at baseline, which increased to 114 +/- 30/66 +/- 18 mm Hg (P <0.01) 35 minutes after drinking. After a meal, blood pressure decreased by 43 +/- 36/20 +/- 13 mm Hg without water drinking, compared with 22 +/- 10/12 +/- 5 mm Hg with drinking (P <0.001). In patients with idiopathic orthostatic intolerance, water drinking attenuated orthostatic tachycardia (123 +/- 23 beats per minute) at baseline to 108 +/- 21 beats per minute after water drinking ( P <0.001). CONCLUSION: Water drinking elicits a rapid pressor response in patients with autonomic failure and can be used to treat orthostatic and postprandial hypotension. Water drinking moderately reduces orthostatic tachycardia in patients with idiopathic orthostatic intolerance. Thus, water drinking may serve as an adjunctive treatment in patients with impaired orthostatic tolerance.

  11. Scapular-focused treatment in patients with shoulder impingement syndrome: a randomized clinical trial.

    PubMed

    Struyf, F; Nijs, J; Mollekens, S; Jeurissen, I; Truijen, S; Mottram, S; Meeusen, R

    2013-01-01

    The purpose of this clinical trial is to compare the effectiveness of a scapular-focused treatment with a control therapy in patients with shoulder impingement syndrome. Therefore, a randomized clinical trial with a blinded assessor was used in 22 patients with shoulder impingement syndrome. The primary outcome measures included self-reported shoulder disability and pain. Next, patients were evaluated regarding scapular positioning and shoulder muscle strength. The scapular-focused treatment included stretching and scapular motor control training. The control therapy included stretching, muscle friction, and eccentric rotator cuff training. Main outcome measures were the shoulder disability questionnaire, diagnostic tests for shoulder impingement syndrome, clinical tests for scapular positioning, shoulder pain (visual analog scale; VAS), and muscle strength. A large clinically important treatment effect in favor of scapular motor control training was found in self-reported disability (Cohen's d = 0.93, p = 0.025), and a moderate to large clinically important improvement in pain during the Neer test, Hawkins test, and empty can test (Cohen's d 0.76, 1.04, and 0.92, respectively). In addition, the experimental group demonstrated a moderate (Cohen's d = 0.67) improvement in self-experienced pain at rest (VAS), whereas the control group did not change. The effects were maintained at three months follow-up. PMID:23053685

  12. Transcatheter Splenic Artery Occlusion for Treatment of Splenic Artery Steal Syndrome After Orthotopic Liver Transplantation

    SciTech Connect

    Uflacker, Renan; Selby, J. Bayne; Chavin, Kenneth; Rogers, Jeffrey; Baliga, Prabhakar

    2002-08-15

    Purpose: To review some aspects of the problem of splenic artery steal syndrome as cause of ischemia in transplanted livers and treatment by selective splenic artery occlusion. Materials and Methods: Eleven liver transplant patients from a group of 350 patients, nine men and two women,ranging in age from 40 years to 61 years (mean 52 years), presented with biochemical evidences of liver ischemia and failure, ranging from one to 60 days following orthotopic liver transplantation. Diagnosis of splenic artery steal syndrome was suspected by elevated enzymes, Doppler ultrasound and confirmed by celiac angiogram. Patients with confirmed hepatic artery thrombosis before angiography were excluded from the study. Embolization with Gianturco coils was performed. Results: All patients were treated by splenic artery embolization with Gianturco coils. The 11 patients improved clinically within 24 hours of the procedure with significant change in the biochemical and clinical parameters. Followup ranged from one month to two years. One of the 11 patient initially improved, but developed hepatic artery thrombosis within 24 hours of the embolic treatment,requiring surgical repair. Conclusion: Splenicartery steal syndrome following liver transplantation surgery can be diagnosed by celiac angiography, and effectively treated by splenic artery embolization with coils. Embolization is one of the treatments available, it is minimally invasive, and leads to immediate clinical improvement. Hepatic artery thrombosis is a possible complication of the procedure.

  13. Guidelines for diagnosis and treatment of Hunter Syndrome for clinicians in Latin America

    PubMed Central

    Giugliani, Roberto; Villarreal, Martha Luz Solano; Valdez, C. Araceli Arellano; Hawilou, Antonieta Mahfoud; Guelbert, Norberto; Garzón, Luz Norela Correa; Martins, Ana Maria; Acosta, Angelina; Cabello, Juan Francisco; Lemes, Aída; Santos, Mara Lucia Schmitz Ferreira; Amartino, Hernán

    2014-01-01

    This review aims to provide clinicians in Latin America with the most current information on the clinical aspects, diagnosis, and management of Hunter syndrome, a serious and progressive disease for which specific treatment is available. Hunter syndrome is a genetic disorder where iduronate-2-sulfatase (I2S), an enzyme that degrades glycosaminoglycans, is absent or deficient. Clinical manifestations vary widely in severity and involve multiple organs and tissues. An attenuated and a severe phenotype are recognized depending on the degree of cognitive impairment. Early diagnosis is vital for disease management. Clinical signs common to children with Hunter syndrome include inguinal hernia, frequent ear and respiratory infections, facial dysmorphisms, macrocephaly, bone dysplasia, short stature, sleep apnea, and behavior problems. Diagnosis is based on screening urinary glycosaminoglycans and confirmation by measuring I2S activity and analyzing I2S gene mutations. Idursulfase (recombinant I2S) (Elaprase®, Shire) enzyme replacement therapy (ERT), designed to address the underlying enzyme deficiency, is approved treatment and improves walking capacity and respiratory function, and reduces spleen and liver size and urinary glycosaminoglycan levels. Additional measures, responding to the multi-organ manifestations, such as abdominal/inguinal hernia repair, carpal tunnel surgery, and cardiac valve replacement, should also be considered. Investigational treatment options such as intrathecal ERT are active areas of research, and bone marrow transplantation is in clinical practice. Communication among care providers, social workers, patients and families is essential to inform and guide their decisions, establish realistic expectations, and assess patients’ responses. PMID:25071396

  14. Cost-effectiveness of implantable cardioverter-defibrillators in Brugada syndrome treatment.

    PubMed

    Wang, Kai; Yamauchi, Kazunobu; Li, Ping; Kato, Hiroki; Kobayashi, Makoto; Kato, Ken; Shimizu, Yoshiyuki

    2008-02-01

    Brugada syndrome is characterized by an ST-segment elevation in the right precordial ECG leads and a high incidence of sudden death in patients with structurally normal hearts. Some trials have demonstrated that the cost-effectiveness of ICD implantation treatment in patients with structurally abnormal hearts is more favorable than that of control treatment. We used Treeage pro 2005 to estimate costs and survival among the Brugada syndrome patients who received either an ICD or were treated by control therapy of Ito-blocking properties (quinidine) or beta-blockers (propranolol). In conclusion, our analysis suggests that prophylactic implantation of an ICD has good cost-effectiveness in patients with Brugada syndrome who are at high risk of sudden death. ICD treatment has shown a cost-effectiveness ratio below $9591 per QALY gained from trials of defibrillator vs beta-blockers for Unexplained Death in Thailand (DEBUT). The control therapy of quinidine may be a good choice for patients who are infants or living in developing countries. PMID:18333406

  15. Guidelines for diagnosis and treatment of Hunter Syndrome for clinicians in Latin America.

    PubMed

    Giugliani, Roberto; Villarreal, Martha Luz Solano; Valdez, C Araceli Arellano; Hawilou, Antonieta Mahfoud; Guelbert, Norberto; Garzón, Luz Norela Correa; Martins, Ana Maria; Acosta, Angelina; Cabello, Juan Francisco; Lemes, Aída; Santos, Mara Lucia Schmitz Ferreira; Amartino, Hernán

    2014-06-01

    This review aims to provide clinicians in Latin America with the most current information on the clinical aspects, diagnosis, and management of Hunter syndrome, a serious and progressive disease for which specific treatment is available. Hunter syndrome is a genetic disorder where iduronate-2-sulfatase (I2S), an enzyme that degrades glycosaminoglycans, is absent or deficient. Clinical manifestations vary widely in severity and involve multiple organs and tissues. An attenuated and a severe phenotype are recognized depending on the degree of cognitive impairment. Early diagnosis is vital for disease management. Clinical signs common to children with Hunter syndrome include inguinal hernia, frequent ear and respiratory infections, facial dysmorphisms, macrocephaly, bone dysplasia, short stature, sleep apnea, and behavior problems. Diagnosis is based on screening urinary glycosaminoglycans and confirmation by measuring I2S activity and analyzing I2S gene mutations. Idursulfase (recombinant I2S) (Elaprase(®), Shire) enzyme replacement therapy (ERT), designed to address the underlying enzyme deficiency, is approved treatment and improves walking capacity and respiratory function, and reduces spleen and liver size and urinary glycosaminoglycan levels. Additional measures, responding to the multi-organ manifestations, such as abdominal/inguinal hernia repair, carpal tunnel surgery, and cardiac valve replacement, should also be considered. Investigational treatment options such as intrathecal ERT are active areas of research, and bone marrow transplantation is in clinical practice. Communication among care providers, social workers, patients and families is essential to inform and guide their decisions, establish realistic expectations, and assess patients' responses. PMID:25071396

  16. Pulsed radiofrequency treatment of complex regional pain syndrome: A case series

    PubMed Central

    Djuric, Vlad

    2014-01-01

    BACKGROUND: Various forms of sympathetic chain neurolysis (sympathectomy) have, at one time or another, held promise as effective treatment options for complex regional pain syndrome (CRPS). Complications, such as worsening pain and the development of new pain syndromes, have prevented sympathectomy from emerging as a standard intervention. In an effort to avoid poor outcomes associated with neurolysis, pulsed radiofrequency (PRF) has been proposed as a potential treatment alternative for a number of chronic neuropathic pain states, including some forms of CRPS. METHODS: The present report describes three cases in which patients diagnosed with lower extremity CRPS type I obtained substantial and lasting intervals of pain relief following PRF of the lumbar sympathetic chain. Over a period of four years, 14 fluoroscopically guided procedures using PRF lesioning of the lumbar sympathetic chain at L2, L3 and L4 were performed in three individuals with CRPS type I of the lower limb. Outcome measures included pre- and post-treatment self-reported pain and medication requirements. RESULTS: Substantial pain relief (>50%) was achieved in 91.7% of PRF applications at three months and 83.3% at six months, with some treatments resulting in persistent relief well beyond 12 months. Medication use decreased to a comparable degree, with discontinuation of opiates after all but three treatments. CONCLUSIONS: PRF lesioning of the lumbar sympathetic chain can be an effective treatment for patients with CRPS type I of the lower extremity, with the potential to provide ≥6 months of substantial pain relief. PMID:24945285

  17. [Treatment outcome using prednisone in corticosteroid-responsive primary nephrotic syndrome in children].

    PubMed

    Brumariu, O; Cucer, Florentina; Munteanu, Mihaela; Haliţchi, Codruţa; Müller, R; Russu, R

    2005-01-01

    In children, the nephrotic syndrome is usually corticoid-responsive; approximately 70% of patients experience relapses, frequently triggered by infections. Our paper presents the results obtained using a 4 month prednisone regimen. This retrospective study included 83 children afflicted with nephrotic syndrome over a 10 year span. We analyzed: age at diagnosis, boys/girls ratio, response to corticoid treatment - after one month of prednisone and at the completion of the treatment course, number of relapses and their frequency, complications of prednisone treatment. The median age at diagnosis was 4.8 years, males predominating M:F = 1.5:1. Complete response after 4 weeks of prednisone therapy was noted in 98.79% of cases. We had 116 episodes of relapses during the first year of follow-up, occurring in 67.4% of children (27.9% were frequent relapsers, 11.62% subsequently became corticoid-dependent). Late relapses, after the first year, occurred in 32.55% of cases. We noted mostly mild adverse effects of the prednisone treatment: occurrence of infections during therapy (16.27%), cushingoid facies (37.2%), hirsutism (4.6%), high blood pressure (4.65%), stretch marks (2.32%). In conclusion, the 4 month prednisone treatment regimen is efficient in inducing and maintaining a remission. The incidence of relapses is 32.55%, comparable to the figure cited in larger studies. Serious adverse effects are significantly lower with this regimen compared to other corticoid treatment schemes. Key wo PMID:16607738

  18. Abdominal compartment syndrome – the prevention and treatment of possible lethal complications following hip arthroscopy: a case report

    PubMed Central

    2014-01-01

    Introduction Intra-abdominal hypertension and abdominal compartment syndrome have been increasingly recognized as a hip arthroscopy complication over the past decade. In the absence of consensus definitions and treatment guidelines, the diagnosis and management of intra-abdominal hypertension and abdominal compartment syndrome remains variable from institution to institution. Case presentation We report the occurrence of the extravasation of fluid into the abdomen during arthroscopic treatment of femoroacetabular impingement combined with resection of trochanteric bursa and our management of the condition in a 55-year old Caucasian woman. Conclusions We present an algorithm of treatment of abdominal compartment syndrome, as a hip arthroscopy complication, according to the consensus definitions and recommendations of the World Society of the Abdominal Compartment Syndrome. In the algorithm options, we have included paracentesis and percutaneous catheter decompression as the main point of treatment. Our algorithm will have a broader clinical impact on orthopedic surgery, anesthesiology and emergency medicine. PMID:25394557

  19. Oral iloprost as a treatment for Raynaud's syndrome: a double blind multicentre placebo controlled study.

    PubMed Central

    Belch, J J; Capell, H A; Cooke, E D; Kirby, J D; Lau, C S; Madhok, R; Murphy, E; Steinberg, M

    1995-01-01

    OBJECTIVE--To compare the efficacy, tolerance and safety of 50-150 micrograms orally administered iloprost given twice a day versus placebo in patients with Raynaud's syndrome. METHODS--The study was multicentre (n = 3), double blind and placebo controlled. Sixty three patients who had eight or more vasospastic attacks per week were enrolled. After a one week run-in period, all patients received either iloprost or placebo treatment to a maximum tolerated dose of 150 micrograms twice a day for 10 days. Diary cards assessed the duration and severity of the vasospastic attacks. Side effects were monitored by direct questioning. A global assessment of treatment efficacy was made by the patient at the end of treatment and two weeks later. RESULTS--Patient opinion tended to favour iloprost at the end of the 10 day treatment phase (p = 0.09) and this was significant at day 24 (the follow up visit) (p = 0.011). Although the duration and severity of attacks tended to decrease in the iloprost treated group, these results tended not to reach statistical significance (for severity p = 0.06 at end of treatment, p = 0.09 on day 24). CONCLUSION--Iloprost administered intravenously has been shown to be of benefit in the treatment of the Raynaud's syndrome associated with systemic sclerosis, but this route of administration is inconvenient. This study evaluated the use of iloprost administered orally to patients with Raynaud's syndrome. Patient documented improvement was significantly improved by iloprost. Diary card analysis showed a trend in favour of iloprost, but these results did not reach statistical significance. PMID:7538285

  20. Treatment of Myoclonus-Dystonia Syndrome with Tetrabenazine

    PubMed Central

    Luciano, Angelo Y.; Jinnah, H. A.; Pfeiffer, Ronald F.; Truong, Daniel D.; Nance, Martha A.; LeDoux, Mark S.

    2014-01-01

    Background Many cases of myoclonus-dystonia (M-D) are due to mutations in SGCE (DYT11). For the majority of patients, myoclonus is relatively more severe than dystonia and can lead to significant functional disability. Deep brain stimulation has been chosen as a treatment option in some patients given that M-D often responds poorly to oral pharmacotherapy. Methods Two siblings with M-D due to the same SGCE deletion mutation were evaluated with the Global Dystonia Rating Scale (GDRS), Fahn-Marsden Rating Scale (FM) and Unified Myoclonus Rating Scale (UMRS) on and off tetrabenazine. Results Both subjects showed marked improvement in myoclonus and mild-to-moderate improvement in dystonia with tetrabenazine. In addition, the response to tetrabenazine has been sustained for years. Conclusions A therapeutic trial of tetrabenazine should be considered in patients with M-D, especially before consideration of deep brain stimulation. An adequately powered multi-center, double-blind study of tetrabenazine will be required to determine the relative contributions of tetrabenazine therapy to myoclonus, dystonia, quality of life, and activities of daily living in patients with M-D. PMID:25406829

  1. Treatment and re-characterization of mouse obstructive genitourinary syndrome.

    PubMed

    Molina-Cimadevila, María Jesús; García-Robles, Tomás; Muñoz-Mediavilla, Clara; Brito-Casillas, Yeray; Wägner, Ana M; Rey, Paloma; Sanchez, Alicia

    2016-05-20

    We aimed to characterize and to explore a treatment for a condition in which male mice exhibited a solid bulge in the preputial area and an inability to breed. Twenty-seven mice from several animal housing institutions in Spain were included in this study for microbiological and pathological characterization of this condition. The condition mostly affected breeding animals and was associated with the C57BL/6J genetic background. A solid, yellowish-white substance was found inside the prepuce, which displaced the penis cranially, preventing its externalization and limiting the animal's capacity to breed. This pattern was almost identical to that of post-coital vaginal plugs, suggesting that the blocking substance originated from ejaculate. Opposite to what was suggested in previous publications, the penis was completely intact in all of the cases, with no signs of mutilation or wounds. Based on our findings, we developed a surgical technique to clear the prepuce and recover breeding performance, which we tested in 15 other mice with the condition. We eliminated the blocking substance and recurrence of the condition by surgically opening the prepuce, and most of the animals recovered fertility. PMID:27203264

  2. Metabolic syndrome - the consequence of lifelong treatment of bipolar affective disorder.

    PubMed

    Dadić-Hero, Elizabeta; Ruzić, Klementina; Grahovac, Tanja; Petranović, Duska; Graovac, Mirjana; Palijan, Tija Zarković

    2010-06-01

    Mood disturbances are characteristic and dominant feature of Mood disorders. Bipolar Affective Disorder (BAD) is a mood disorder which occurs equally in both sexes. BAD may occur in co morbidity with other mental diseases and disorders such as: Anorexia Nervosa, Bulimia Nervosa, Attention Deficit, Panic Disorder and Social Phobia. However, medical disorders (one or more) can also coexist with BAD. Metabolic syndrome is a combination of metabolic disorders that increase the risk of developing cardiovascular disease. A 61-year old female patient has been receiving continuous and systematic psychiatric treatment for Bipolar Affective Disorder for the last 39 years. The first episode was a depressive one and it occurred after a child delivery. Seventeen years ago the patient developed diabetes (diabetes type II), and twelve years ago arterial hypertension was diagnosed. High cholesterol and triglyceride levels as well as weight gain were objective findings. During the last nine years she has been treated for lower leg ulcer. Since metabolic syndrome includes abdominal obesity, hypertension, diabetes mellitus, increased cholesterol and serum triglyceride levels, the aforesaid patient can be diagnosed with Metabolic Syndrome. When treating Bipolar Affective Disorder, the antipsychotic drug choice should be careful and aware of its side-effects in order to avoid the development or aggravation of metabolic syndrome. PMID:20562789

  3. Metabolic Syndrome Screening and Assertive Community Treatment: A Quality Improvement Study.

    PubMed

    Castillo, Enrico G; Rosati, Justin; Williams, Caroline; Pessin, Neil; Lindy, David C

    2015-01-01

    Metabolic syndrome defines a collection of cardiometabolic illnesses that predict risk for poor physical health and early death and is highly prevalent among those with serious mental illness. Despite recommendations for routine monitoring, those with serious mental illness frequently do not receive physical health screenings. We conducted a quality improvement (QI) project to increase rates of metabolic syndrome screening in three New York City Assertive Community Treatment (ACT) teams. The project, conducted from December 2010 to May 2011, involved educational sessions for staff and consumers and a systematic screening protocol. We collected complete metabolic syndrome screening measurements for 71% of participating ACT consumers. We found metabolic risk to be nearly universal among participants, with over half diagnosed with metabolic syndrome. We also found high rates of previously undiagnosed hypertension, diabetes, and dyslipidemia. We describe the resources and obstacles we encountered in our QI project to make systematic metabolic screening a routine part of ACT care. This QI project suggests that ACT teams can take a leadership role in screening their consumers for physical health issues, aligning with recent policy trends to better integrate behavioral health and primary care services. PMID:26282669

  4. Pierre Robin sequence: Subdivision, data, theories, and treatment - Part 2: Syndromic and nonsyndromic Pierre Robin sequence

    PubMed Central

    Bütow, Kurt-W; Morkel, Jean A.; Naidoo, Sharan; Zwahlen, Roger Arthur

    2016-01-01

    Context: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in the scientific literature, relating to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch-up growth, midfacial hyperplasia, and the early management. Aims of Part 2: Contribute to the sparse scientific knowledge about pathogenesis and involved genetics. Subjects and Methods: An analysis of this large database was conducted focusing on genetic involvement, family history, and the incidence of additional syndromes. Results: Beside of differences related to clinical signs of dyspnea, feeding problems and mortality rates, various concomitant syndromes, and genetic abnormalities were found in cases of Fairbairn–Robin triad (FRT) and Siebold–Robin sequence (SRS), in addition to differences in relation to clinical signs of dyspnea, feeding problems, and mortality rates. Conclusion: Multiple FRT cases presented with various concomitant syndromes and genetic abnormalities, but only one type occurred in two SRS cases. The latter presented a significantly different mortality rate when compared to the FRT subgroup.

  5. Radial tunnel syndrome caused by ganglion cyst: treatment by arthroscopic cyst decompression.

    PubMed

    Mileti, Joseph; Largacha, Mauricio; O'Driscoll, Shawn W

    2004-05-01

    Compressive neuropathies of the radial nerve at the elbow can lead to one of 2 clinical entities. Posterior interosseous syndrome is primarily a motor deficiency of the posterior interosseous nerve, and radial tunnel syndrome presents as pain along the radial tunnel and extensor muscle mass. The radial nerve can be compressed at a number of sites around the elbow. In addition, numerous mass lesions reported in the literature can cause compressive neuropathy of the radial nerve at the elbow. Standard surgical management for persistent radial tunnel syndrome that is refractory to nonsurgical treatment is open decompression of the radial nerve. Cysts occurring in other joints are commonly treated arthroscopically. Supraglenoid cysts of the shoulder, meniscal cysts in the knee, and dorsal wrist ganglia are routinely treated with arthroscopic decompression or excision with management of the underlying etiology of the cyst. We present a case of radial tunnel syndrome caused by a ganglion cyst of the proximal radioulnar joint that was treated using arthroscopic excision of the cyst and decompression of the radial nerve. PMID:15122155

  6. Safinamide for the treatment of Parkinson's disease, epilepsy and restless legs syndrome.

    PubMed

    Chazot, Paul L

    2007-07-01

    Merck Serono SA (formerly Serono), under license from Newron Pharmaceuticals SpA (following its acquisition of the rights from Pharmacia and Upjohn AB [now Pfizer Inc]), is developing the oral alpha-aminoamide derivative of milacemide, safinamide, a monoamine oxidase-B and glutamate release inhibitor, for the potential treatment of Parkinson's disease, epilepsy and restless legs syndrome. In March 2007, plans to develop the agent for the potential treatment of other cognitive disorders, such as Alzheimer's disease, were being finalized and testing was expected to begin before the end of that year. PMID:17659477

  7. Diagnosis and treatment of polycystic ovary syndrome (PCOS): an interview with Richard Legro.

    PubMed

    Legro, Richard

    2015-01-01

    In this podcast, we talk to Professor Richard Legro about the recommendations for the diagnosis and treatment of polycystic ovary syndrome (PCOS) based on clinical practice guidelines and discuss the challenges of diagnosis PCOS at specific age groups. The controversies associated with treatment of PCOS, including therapies for infertility as this is a problem commonly observed in PCOS subjects, are highlighted together with future directions on the topic. The podcast for this interview is available at. http://www.biomedcentral.com/content/supplementary/s12916-015-0299-2-s1.mp3. PMID:25879641

  8. Alternative treatment for open bite Class III malocclusion in a child with Williams-Beuren syndrome.

    PubMed

    Vieira, Giovanni Modesto; Franco, Eduardo Jacomino; da Rocha, Denise Falcão Pinheiro; de Oliveira, Laudimar Alves; Amorim, Rivadávio Fernandes Batista

    2015-01-01

    Williams-Beuren syndrome (WBS) is a rare genetic condition that affects approximately 1 in every 20,000 - 50,000 live births. WBS children have specific skeletal deformities, dental malformations and rare lingual muscle dysfunction. The need for orthodontic and orthognathic therapy has arisen and has been considered a real clinical challenge even for experienced professionals, once it requires a complex and individualized treatment plan. This study reports a case of orthopedic expansion of the maxilla, in which a modified facial mask was used for protraction of the maxillary complex associated with clockwise rotation of the maxilla. In addition, special considerations about treatment time and orthopedic outcomes are discussed. PMID:25741831

  9. Alternative treatment for open bite Class III malocclusion in a child with Williams-Beuren syndrome

    PubMed Central

    Vieira, Giovanni Modesto; Franco, Eduardo Jacomino; da Rocha, Denise Falcão Pinheiro; de Oliveira, Laudimar Alves; Amorim, Rivadávio Fernandes Batista

    2015-01-01

    Williams-Beuren syndrome (WBS) is a rare genetic condition that affects approximately 1 in every 20,000 - 50,000 live births. WBS children have specific skeletal deformities, dental malformations and rare lingual muscle dysfunction. The need for orthodontic and orthognathic therapy has arisen and has been considered a real clinical challenge even for experienced professionals, once it requires a complex and individualized treatment plan. This study reports a case of orthopedic expansion of the maxilla, in which a modified facial mask was used for protraction of the maxillary complex associated with clockwise rotation of the maxilla. In addition, special considerations about treatment time and orthopedic outcomes are discussed. PMID:25741831

  10. Role of "old" pharmacological agents in the treatment of Cushing's syndrome.

    PubMed

    Ambrogio, A G; Cavagnini, F

    2016-09-01

    Despite recent advances in the management of endogenous Cushing's syndrome (CS), its treatment remains a challenge. When surgery has been unsuccessful or unfeasible as well in case of recurrence, the "old" pharmacological agents represent an important alternative for both ACTH-dependent and independent hypercortisolism. Especially in the latter, the advent of novel molecules directly targeting ACTH secretion has not outweighed the "old" drugs, which continue to be largely employed and have recently undergone a reappraisal. This review provides a survey of the "old" pharmacological agents in the treatment of CS. PMID:27086313

  11. Cardiac vagal activation by adrenocorticotropic hormone treatment in infants with West syndrome.

    PubMed

    Hattori, Ayako; Hayano, Junichiro; Fujimoto, Shinji; Ando, Naoki; Mizuno, Kumiko; Kamei, Michi; Kobayashi, Satoru; Ishikawa, Tatsuya; Togari, Hajime

    2007-02-01

    West syndrome (WS) is a generalized epileptic syndrome of infancy and early childhood with various etiologies, and consists of a triad of infantile spasm, arrest or regress of psychomotor development and specific electroencephalogram (EEG) pattern of hypsarrhythmia. WS had been believed to be refractory, but recent evidence supports effectiveness of adrenocorticotropic hormone (ACTH) treatment. The ACTH treatment, however, has a problem that it is often accompanied by adverse autonomic symptoms. We therefore examined heart rate variability (HRV) for assessing cardiac autonomic functions in WS and prospectively observed the changes during ACTH treatment. We studied 15 patients with WS and 9 age-matched controls during sleep (EEG stage 2). Compared with controls, the patients with WS were greater in the low-frequency component (LF) of HRV, an index reflecting sympatho-vagal interaction (p = 0.02), but were comparable for high-frequency component (HF) and LF-to-HF ratio (LF/HF), indices reflecting cardiac vagal activity and sympathetic predominance, respectively. During ACTH treatment, heart rate decreased (p < 0.01), LF and HF increased (p < 0.01), and LF/HF did not differ significantly. These results indicate that WS might be accompanied by autonomic changes and that ACTH treatment enhances parasympathetic function and causes bradycardia. PMID:17287597

  12. Modulation of the GABAergic pathway for the treatment of fragile X syndrome

    PubMed Central

    Lozano, Reymundo; Hare, Emma B; Hagerman, Randi J

    2014-01-01

    Fragile X syndrome (FXS) is the most common genetic cause of intellectual disability and the most common single-gene cause of autism. It is caused by mutations on the fragile X mental retardation gene (FMR1) and lack of fragile X mental retardation protein, which in turn, leads to decreased inhibition of translation of many synaptic proteins. The metabotropic glutamate receptor (mGluR) hypothesis states that the neurological deficits in individuals with FXS are due mainly to downstream consequences of overstimulation of the mGluR pathway. The main efforts have focused on mGluR5 targeted treatments; however, investigation on the gamma-aminobutyric acid (GABA) system and its potential as a targeted treatment is less emphasized. The fragile X mouse models (Fmr1-knock out) show decreased GABA subunit receptors, decreased synthesis of GABA, increased catabolism of GABA, and overall decreased GABAergic input in many regions of the brain. Consequences of the reduced GABAergic input in FXS include oversensitivity to sensory stimuli, seizures, and anxiety. Deficits in the GABA receptors in different regions of the brain are associated with behavioral and attentional processing deficits linked to anxiety and autistic behaviors. The understanding of the neurobiology of FXS has led to the development of targeted treatments for the core behavioral features of FXS, which include social deficits, inattention, and anxiety. These symptoms are also observed in individuals with autism and other neurodevelopmental disorders, therefore the targeted treatments for FXS are leading the way in the treatment of other neurodevelopmental syndromes and autism. The GABAergic system in FXS represents a target for new treatments. Herein, we discuss the animal and human trials of GABAergic treatment in FXS. Arbaclofen and ganaxolone have been used in individuals with FXS. Other potential GABAergic treatments, such as riluzole, gaboxadol, tiagabine, and vigabatrin, will be also discussed. Further

  13. Which patients do not recover from shoulder impingement syndrome, either with operative treatment or with nonoperative treatment?

    PubMed Central

    Ketola, Saara; Lehtinen, Janne; Rousi, Timo; Nissinen, Maunu; Huhtala, Heini; Arnala, Ilkka

    2015-01-01

    Background and purpose — Shoulder impingement syndrome is common, but treatment is controversial. Arthroscopic acromioplasty is popular even though its efficacy is unknown. In this study, we analyzed stage-II shoulder impingement patients in subgroups to identify those who would benefit from the operation. Patients and methods — In a previous randomized study, 140 patients were either treated with a supervised exercise program or with arthroscopic acromioplasty followed by a similar exercise program. The patients were followed up at 2 and 5 years after randomization. Self-reported pain was used as the primary outcome measure. Results — Both treatment groups had less pain at 2 and 5 years, and this was similar in both groups. Duration of symptoms, marital status (single), long periods of sick leave, and lack of professional education appeared to increase the risk of persistent pain despite the treatment. Patients with impingement with radiological acromioclavicular (AC) joint degeneration also had more pain. The patients in the exercise group who later wanted operative treatment and had it did not get better after the operation. Interpretation — The natural course probably plays a substantial role in the outcome. Based on our findings, it is difficult to recommend arthroscopic acromioplasty for any specific subgroup. Regarding operative treatment, however, a concomitant AC joint resection might be recommended if there are signs of AC joint degeneration. Even more challenging for the development of a treatment algorithm is the finding that patients who do not recover after nonoperative treatment should not be operated either. PMID:25809315

  14. Tic Disorders and Tourette Syndrome: Current Concepts of Etiology and Treatment in Children and Adolescents.

    PubMed

    Tagwerker Gloor, Friederike; Walitza, Susanne

    2016-04-01

    Tic disorders (TD), including chronic/persistent TD (CTD) and Tourette syndrome, have been described and studied for many years. Within the last two decades, intensified study efforts led to more specific assumptions about genesis and influences of both hereditary and environmental factors. TD in children and adolescents are very often accompanied by attention-deficit/hyperactivity disorders and obsessive-compulsive disorders (OCD) as comorbid disorders. Comorbidities are aggravating factors concerning prognosis and treatment opportunities. Therefore, etiological considerations and treatment strategies have to take associated psychiatric disorders into account. Treatment approaches are symptom targeted and include behavioral treatments and/or medication and show positive outcomes concerning tic symptomatology, global functioning, and associated psychopathology. This review presents an update of the research, definitions, and classification according to ICD-10 and DSM-5 and summarizes the diagnostic procedures and most effective clinical strategies. PMID:26829367

  15. An unfortunate challenge: Ketogenic diet for the treatment of Lennox-Gastaut syndrome in tyrosinemia type 1.

    PubMed

    De Lucia, Silvana; Pichard, Samia; Ilea, Adina; Greneche, Marie-Odile; François, Laurent; Delanoë, Catherine; Schiff, Manuel; Auvin, Stéphane

    2016-07-01

    The ketogenic diet is an evidence-based treatment for resistant epilepsy including Lennox-Gastaut syndrome. This diet is based on low carbohydrate-high fat intakes. Dietary treatment is also therapeutic for inborn errors of metabolism such as aminoacdiopathies. We report a child with both Lennox-Gastaut syndrome and tyrosinemia type 1. This epilepsy syndrome resulted form a porencephalic cyst secondary to brain abscesses that occurred during the management of malnutrition due to untreated tyrosinemia type 1. We used a ketogenic diet as treatment for Lennox-Gastaut syndrome taking into account dietary requirements for tyrosinemia type 1. The patient was transiently responder during a 6-month period. This report illustrates that ketogenic diet remains a therapeutic option even when additional dietary requirements are needed. PMID:27052529

  16. A new interdisciplinary treatment strategy versus usual medical care for the treatment of subacromial impingement syndrome: a randomized controlled trial

    PubMed Central

    Dorrestijn, Oscar; Stevens, Martin; Diercks, Ron L; van der Meer, Klaas; Winters, Jan C

    2007-01-01

    Background Subacromial impingement syndrome (SIS) is the most frequently recorded shoulder disorder. When conservative treatment of SIS fails, a subacromial decompression is warranted. However, the best moment of referral for surgery is not well defined. Both early and late referrals have disadvantages – unnecessary operations and smaller improvements in shoulder function, respectively. This paper describes the design of a new interdisciplinary treatment strategy for SIS (TRANSIT), which comprises rules to treat SIS in primary care and a well-defined moment of referral for surgery. Methods/Design The effectiveness of an arthroscopic subacromial decompression versus usual medical care will be evaluated in a randomized controlled trial (RCT). Patients are eligible for inclusion when experiencing a recurrence of SIS within one year after a first episode of SIS which was successfully treated with a subacromial corticosteroid injection. After inclusion they will receive injection treatment again by their general practitioner. When, after this treatment, there is a second recurrence within a year post-injection, the participants will be randomized to either an arthroscopic subacromial decompression (intervention group) or continuation of usual medical care (control group). The latter will be performed by a general practitioner according to the Dutch National Guidelines for Shoulder Problems. At inclusion, at randomization and three, six and 12 months post-randomization an outcome assessment will take place. The primary outcome measure is the patient-reported Shoulder Disability Questionnaire. The secondary outcome measures include both disease-specific and generic measures, and an economic evaluation. Treatment effects will be compared for all measurement points by using a GLM repeated measures analyses. Discussion The rationale and design of an RCT comparing arthroscopic subacromial decompression with usual medical care for subacromial impingement syndrome are

  17. Successful Multimodality Endoscopic Treatment of Gastric Outlet Obstruction Caused by an Impacted Gallstone (Bouveret's Syndrome)

    PubMed Central

    Rogart, Jason N.; Perkal, Melissa; Nagar, Anil

    2008-01-01

    Bouveret's syndrome is a rare condition of gastric outlet obstruction resulting from the migration of a gallstone through a choledochoduodenal fistula. Due to the large size of these stones and the difficult location in which they become impacted, endoscopic treatment is unsuccessful and most patients require surgery. We report the case of an elderly male who presented with nausea and hematemesis, and was found on CT scan and endoscopy to have an obstructing gallstone in his duodenal bulb. After several endoscopic sessions and the use of multiple instruments including a Holmium: YAG laser and electrohydraulic lithotripter, fragmentation and endoscopic removal of the stone were successful. We believe this to be the first case of Bouveret's syndrome successfully treated by endoscopy alone in the United States. We describe the difficulties encountered which necessitated varied and innovative therapeutic techniques. PMID:18493330

  18. Growth Charts for Prader-Willi Syndrome During Growth Hormone Treatment.

    PubMed

    Butler, Merlin G; Lee, Jaehoon; Cox, Devin M; Manzardo, Ann M; Gold, June-Anne; Miller, Jennifer L; Roof, Elizabeth; Dykens, Elisabeth; Kimonis, Virginia; Driscoll, Daniel J

    2016-09-01

    The purpose of the current study was to develop syndrome-specific standardized growth curves for growth hormone-treated Prader-Willi syndrome (PWS) individuals aged 0 to 18 years. Anthropometric growth-related measures were obtained on 171 subjects with PWS who were treated with growth hormone for at least 40% of their lifespan. They had no history of scoliosis. PWS standardized growth curves were developed for 7 percentile ranges using the LMS method for weight, height, head circumference, weight/length, and BMI along with normative 3rd, 50th, and 97th percentiles plotted using control data from the literature and growth databases. Percentiles were plotted on growth charts for comparison purposes. Growth hormone treatment appears to normalize stature and markedly improves weight in PWS compared with standardized curves for non-growth hormone-treated PWS individuals. Growth chart implications and recommended usage are discussed. PMID:26842920

  19. Refractory Pigmentation Associated with Laugier-Hunziker Syndrome following Er:YAG Laser Treatment

    PubMed Central

    Ergun, Sertan; Saruhanoğlu, Alp; Migliari, Dante-Antonio; Maden, Ilay; Tanyeri, Hakkı

    2013-01-01

    The present report describes a case of Laugier-Hunziker syndrome (LHS), a rare benign condition. A patient with LHS develops acquired melanotic pigmentation of the lips and buccal mucosa, often with pigmentation of the nails occurring. No systemic symptoms are associated with this syndrome. Normally, no treatment is required for this condition, unless for aesthetic reason, mainly due to pigmentation on the lip mucosa. We present a case of LHS, 37-year-old female, whose pigmentations on her lip and in the oral cavity were treated with an Er:YAG laser. At the postoperative 12th month followup, the lesions recurred. The effects of any surgical attempt to treat pigmentations associated with LHS were discussed. PMID:24367727

  20. Exertional Compartment Syndrome of the Medial Foot Compartment: Diagnosis and Treatment.

    PubMed

    Izadi, Faye E; Richie Jr, Douglas H

    2014-06-25

    Abstract Exertional compartment syndrome (ECS) in the foot is rarely reported and often confused with plantar fasciitis as a cause of arch pain in the running athlete. We describe a case involving a 19 year old competitive collegiate runner who developed a chronic case of bilateral medial arch pain during training, which was initially diagnosed as plantar fasciitis but failed to respond to conventional treatment. After symptoms began to suggest exertional compartment syndrome, the diagnosis was confirmed by measuring an elevated resting pressure in the medial compartment of both feet. The patient underwent a bilateral medial compartment fasciotomy, which allowed a full return to activity, and has remained pain free after a one year follow up. PMID:24963970

  1. Exertional compartment syndrome of the medial foot compartment--diagnosis and treatment: a case report.

    PubMed

    Izadi, Faye E; Richie, Douglas H

    2014-07-01

    Exertional compartment syndrome in the foot is rarely reported and often confused with plantar fasciitis as a cause of arch pain in the running athlete. We describe a case involving a 19-year-old competitive collegiate runner who developed a chronic case of bilateral medial arch pain during training, which was initially diagnosed as plantar fasciitis but failed to respond to conventional treatment. After symptoms began to suggest exertional compartment syndrome, the diagnosis was confirmed by measuring an elevated resting pressure in the medial compartment of both feet. The patient underwent a bilateral medial compartment fasciotomy, which allowed a full return to activity, and has remained pain free after a 1-year follow-up. PMID:25076087

  2. Endoscopic Sinus Surgery for Treatment of Kartagener Syndrome: A Case Report

    PubMed Central

    Tang, Xinghua; Zou, Jian; Liu, Shixi

    2013-01-01

    Background: Kartagener syndrome (KS) is a rare congenital disease characterised by a clinical triad of symptoms: situs inversus, chronic rhinosinusitis, and bronchiectasis. Although congenital ciliary defect is recognised as the main cause of this syndrome, it remains difficult to treat the associated airway infection. Case Report: A 17-year-old female patient presented with repeated refractory airway infection. She also had bronchiectasis and situs inversus. Electron microscopic evaluation of her nasal mucosa revealed ciliary defect and confirmed the diagnosis of KS. She underwent functional endoscopic sinus surgery (FESS) followed by long-term postoperative debridement of the sinonasal cavity. This treatment reduced chronic rhinosinusitis and protected against subsequent airway infection in a 7-year follow-up. Conclusion: FESS is effective for relieving both chronic rhinosinusitis and lung infection of KS in the long term. PMID:25207108

  3. Case study: a minimally invasive approach to the treatment of Klippel-Trenaunay syndrome.

    PubMed

    Latessa, Victoria; Frasier, Krista

    2007-12-01

    Klippel-Trenaunay syndrome (KTS) is a congenital developmental disorder characterized by port wine stain, venous abnormalities, soft tissue, and bony deformities of the affected extremity. It is usually diagnosed in early childhood and has many long-term sequelae. Patients not only have physical health problems but also must learn to cope with psychosocial factors that will affect their self-esteem and interpersonal relationships. This article describes the syndrome of KTS and the minimally invasive techniques used in the treatment of superficial varicosities in patients with reasonably mild KTS with an intact deep venous system. Treating the varicosities relatively early to avoid the long-term complications of chronic venous insufficiency may improve the quality of life, maintain limb function, and decrease the risk of long-term venous complications. PMID:18036494

  4. Ethnomedical syndromes and treatment-seeking behavior among Mayan refugees in Chiapas, Mexico.

    PubMed

    Smith, Bryce D; Sabin, Miriam; Berlin, Elois Ann; Nackerud, Larry

    2009-09-01

    This survey investigated the prevalence of ethnomedical syndromes and examined treatments and treatment-seeking in Mayan Guatemalans living in United Nations High Commissioner for Refugee (UNHCR) camps in Chiapas, Mexico. Methods included a rapid ethnographic assessment to refine survey methods and inform the cross-sectional survey, which also examined mental health outcomes; 183 households were approached for interview, representing an estimated 1,546 residents in five refugee camps and 93% of all households. One adult per household (N = 170) was interviewed regarding his or her health; an additional 9 adults in three surveyed households participated and were included in this analysis; of the 179 participants, 95 primary child-care providers also answered a children's health questionnaire for their children. Results indicated that ethnomedical syndromes were common in this sample, with 59% of adults and 48.4% of children having experienced susto (fright condition) and 34.1% of adults reporting ataques de nervios (nervous attacks); both conditions were significantly associated with symptoms consistent with posttraumatic stress disorder, anxiety and depression and are mental health conditions recognized by the American Psychiatric Association. Combining healthcare provider and indigenous treatments such as physician prescribed medication (65%), medicinal plants (65.7%), and limpias (spiritual cleansings) (40.6%) was reported. Most participants (86%) sought routine medical treatment from UNHCR trained health promoters in their camp. Assessing ethnomedical health is important for informing mental health programs among this population. PMID:19526330

  5. Combined endothelin a blockade and chlorthalidone treatment in a rat model of metabolic syndrome.

    PubMed

    Jin, Chunhua; Jeon, Yejoo; Kleven, Daniel T; Pollock, Jennifer S; White, John J; Pollock, David M

    2014-11-01

    Experiments determined whether the combination of endothelin A (ETA) receptor antagonist [ABT-627, atrasentan; (2R,3R,4S)-4-(1,3-benzodioxol-5-yl)-1-[2-(dibutylamino)-2-oxoethyl]-2-(4-methoxyphenyl)pyrrolidine-3-carboxylic acid] and a thiazide diuretic (chlorthalidone) would be more effective at lowering blood pressure and reducing renal injury in a rodent model of metabolic syndrome compared with either treatment alone. Male Dahl salt-sensitive rats were fed a high-fat (36% fat), high-salt (4% NaCl) diet for 4 weeks. Separate groups of rats were then treated with vehicle (control), ABT-627 (ABT; 5 mg/kg per day, in drinking water), chlorthalidone (CLTD; 5 mg/kg per day, in drinking water), or both ABT plus CLTD. Mean arterial pressure (MAP) was recorded continuously by telemetry. After 4 weeks, both ABT and CLTD severely attenuated the development of hypertension, whereas the combination further reduced MAP compared with ABT alone. All treatments prevented proteinuria. CLTD and ABT plus CLTD significantly reduced nephrin (a podocyte injury marker) and kidney injury molecule-1 (a tubulointerstitial injury marker) excretion. ABT, with or without CLTD, significantly reduced plasma 8-oxo-2'-deoxyguanosine, a measure of DNA oxidation, whereas CLTD alone had no effect. All treatments suppressed the number of ED1(+) cells (macrophages) in the kidney. Plasma tumor necrosis factor receptors 1 and 2 were reduced only in the combined ABT and CLTD group. These results suggest that ABT and CLTD have antihypertensive and renal-protective effects in a model of metabolic syndrome that are maximally effective when both drugs are administered together. The findings support the hypothesis that combined ETA antagonist and diuretic treatment may provide therapeutic benefit for individuals with metabolic syndrome consuming a Western diet. PMID:25189702

  6. Social Support and Treatment Outcome in Alcohol Dependence Syndrome in Armed Forces

    PubMed Central

    Chauhan, Vinay Singh; Azad, Sudip

    2015-01-01

    Introduction Social factors play vital role in unfolding of alcohol use disorders in any given population. Several factors beyond the confines of treatment settings influence treatment outcome in alcohol dependence syndrome. Social support has positive effect in treatment outcome of alcohol dependence syndrome. This has not been much studied in India in past. Therefore we decided to study the perception of social support in cases of alcohol dependence syndrome admitted in a busy hospital in armed forces. Aim The aim was to study the perception of social support across relapsed and abstinent group and see if it reached any statistical proportion and also to see if any socio-demographic variables also affected perception of social support. Materials and Methods Fifty five consecutive male patients of alcohol dependent syndrome without a co-morbid neurological/psychiatric diagnosis were assessed for their perception of social support after taking informed consent. They were explained the procedure and their alcoholic milestones were recorded in specially designed pro-forma. Subjects were then divided in abstinent and relapsed group. Subsequently they were assessed for their perception of social support by administering Social provision scale and Social support questionnaire. Statistical Analysis Data were tabulated and statistically analysed by using chi square test, Mann Whitney U-Test and Rank ANOVA test where applicable p-value <.05 was taken as significant. Results Results indicated that perception of social support across abstinent (n=18) and relapsed (n= 37) group reached significant statistical proportion as measured by social provision scale and social support questionnaire. Duration of use, dependence and family history of alcoholism did not influence perception of social support across patient population. There was inverse relationship between patients with alcohol related problem and their perception of social support. Professional and qualified soldiers

  7. [Automatic positive airway pressure in titration and treatment of the obstructive sleep apnea syndrome].

    PubMed

    Randerath, W J

    2007-04-01

    Although continuous airway pressure therapy (CPAP) represents the standard treatment for obstructive sleep apnea syndrome (OSAS) auto-adjusting CPAP (APAP) devices were developed which adapt the treatment pressure to the actual requirement of the patients. The aim of automatic CPAP therapy is to improve the patients' acceptance of positive pressure treatment. The devices react to respiratory flow, flattening of the inspiratory flow contour, snoring, generator speed or the upper airway impedance. In recent years several studies showed that auto CPAP effectively treats respiratory disturbances, improves sleep profile and the self-assessment of the patients equally as good as the gold standard constant CPAP. Moreover, APAP reduces the treatment pressure substantially. Although an improvement of the patient's compliance has not consistently been proven, most patients prefer APAP versus constant CPAP. APAP devices use different algorithms depending on the primary purpose of the application. Therefore, a clear distinction between automatic titration and treatment is of major relevance. While titration devices aim at the finding of one single pressure which is fixed to a constant CPAP device, automatic treatment means the chronic use of APAP at home for optimal adaptation of the treatment pressure to the actual requirements of the patient. A high constant CPAP level, huge pressure variability, insufficient compliance with constant CPAP may be indications for APAP treatment. The main reason for automatic titration is the standardisation of the initiation process. PMID:17455137

  8. Morquio syndrome

    MedlinePlus

    ... to have children and who have a family history of Morquio syndrome. Counseling is also recommended for families who have a child with Morquio syndrome to help them understand the condition and possible treatments. Prenatal testing is available.

  9. Current trends in the treatment of polycystic ovary syndrome with desire for children

    PubMed Central

    Sastre, Margalida E; Prat, Maria O; Checa, Miguel Angel; Carreras, Ramon C

    2009-01-01

    Polycystic ovary syndrome (PCOS), one of the most frequent endocrine diseases, affects approximately 5%–10% of women of childbearing age and constitutes the most common cause of female sterility regardless of the need or not for treatment, a change in lifestyle is essential for the treatment to work and ovulation to be restored. Obesity is the principal reason for modifying lifestyle since its reduction improves ovulation and the capacity for pregnancy and lowers the risk of miscarriage and later complications that may occur during pregnancy (gestational diabetes, pre-eclampsia, etc). When lifestyle modification is not sufficient, the first step in ovulation induction is clomiphene citrate. The second-step recommendation is either exogenous gonadotrophins or laparoscopic ovarian surgery. Recommended third-line treatment is in vitro fertilization. Metformin use in PCOS should be restricted to women with glucose intolerance. PMID:19536311

  10. Hypoplastic left heart syndrome – a review of supportive percutaneous treatment

    PubMed Central

    Moszura, Tomasz; Dryżek, Paweł

    2014-01-01

    Due to the complex anatomical and haemodynamic consequences of hypoplastic left heart syndrome (HLHS), patients with the condition require multistage surgical and supportive interventional treatment. Percutaneous interventions may be required between each stage of surgical palliation, sometimes simultaneously with surgery as hybrid interventions, or after completion of multistage treatment. Recent advances in the field of interventional cardiology, including new devices and techniques, have significantly contributed to improving results of multistage HLHS palliation. Knowledge of the potential interventional options as well as the limitation of percutaneous interventions will enable the creation of safe and effective treatment protocols in this highly challenging group of patients. In this comprehensive review we discuss the types, goals, and potential complications of transcatheter interventions in patients with HLHS. PMID:25489307

  11. Effects of gonadotropin and testosterone treatments on plasma leptin levels in male patients with idiopathic hypogonadotropic hypogonadism and Klinefelter's syndrome.

    PubMed

    Ozata, M; Ozisik, G; Caglayan, S; Yesilova, Z; Bingöl, N; Saglam, M; Turan, M; Beyhan, Z

    1998-05-01

    Since little is known about the effects of gonadotropin and testosterone treatment on leptin levels in male hypogonadism, we determined fasting plasma leptin levels before and 3 months after treatment in 21 patients with idiopathic hypogonadotropic hypogonadism (IHH), 16 patients with Klinefelter's syndrome and 20 male controls. Patients with IHH were treated with hCG/human menopausal gonadotropin, whereas patients with Klinefelter's syndrome received T treatment. Plasma leptin levels were measured by an RIA with a sensitivity of 0.5 microg/L. Mean leptin levels in patients with IHH before treatment (9.23+/-4.09 microg/L) were not significantly different from those in patients with Klinefelter's syndrome (7.29+/-5.05 microg/L; z=-1.41; P=0.15). Leptin levels in both IHH and Klinefelter's syndrome groups were, however, significantly higher than in the normal men (3.91+/-1.67 microg/L) (P<0.001 and P<0.01, respectively). Mean leptin levels did not change significantly 3 months after the initiation of gonadotropin (11.6+/-6.44 microg/L) or T (8.32+/-5.17 microg/L) treatment in either IHH or Klinefelter's syndrome. Our study demonstrated that mean plasma leptin levels are not influenced by short-term gonadotropin or T treatment in male hypogonadism. PMID:9660087

  12. Factorial study of moxibustion in treatment of diarrhea-predominant irritable bowel syndrome

    PubMed Central

    Zhao, Ji-Meng; Wu, Lu-Yi; Liu, Hui-Rong; Hu, Hong-Yi; Wang, Jia-Ying; Huang, Ren-Jia; Shi, Yin; Tao, Shan-Ping; Gao, Qiang; Zhou, Ci-Li; Qi, Li; Ma, Xiao-Peng; Wu, Huan-Gan

    2014-01-01

    AIM: To identify an appropriate therapeutic regimen for using aconite cake-separated moxibustion to treat diarrhea-predominant irritable bowel syndrome (D-IBS). METHODS: A factorial design was employed to examine the two factors of moxibustion frequency and number of cones. The two tested frequencies were three or six moxibustion sessions per week, and the two tested doses were one or two cones per treatment. A total of 166 D-IBS patients were randomly divided into four treatment groups, which included each combination of the examined frequencies and doses. The bilateral Tianshu acupoints (ST25) and the Qihai acupoint (RN6) were selected for aconite cake-separated moxibustion. Each patient received two courses of treatment, and each course had a duration of 2 wk. For each group, the scores on the Birmingham irritable bowel syndrome (IBS) symptom questionnaire, the IBS Quality of Life scale, the Self-Rating Depression Scale (SDS), the Self-Rating Anxiety Scale (SAS), the Hamilton Depression (HAMD) scale, and the Hamilton Anxiety (HAMA) scale were determined before treatment, after the first course of treatment, and after the second course of treatment. RESULTS: The symptom, quality of life, SDS, SAS, HAMD, and HAMA scores of the patients in all 4 aconite cake-separated moxibustion groups were significantly lower after the first and second courses of treatment than before treatment (P < 0.001 for all). The symptom, quality of life, SDS, SAS, HAMD, and HAMA scores of the patients in all four aconite cake-separated moxibustion groups were significantly lower after the second course of treatment than after the first course of treatment (P < 0.001 for all). Between-group comparisons after the second course of treatment revealed that the symptom scores for group 1 (1 cone, 3 treatments/wk) and group 3 (2 cones, 3 treatments/wk) were significantly lower than that for group 2 (1 cone, 6 treatments/wk) (5.55 ± 5.05 vs 10.45 ± 6.61, P < 0.001; 5.65 ± 4.00 vs 10.45 ± 6

  13. Phelan-McDermid Syndrome and SHANK3: Implications for Treatment.

    PubMed

    Costales, Jesse L; Kolevzon, Alexander

    2015-07-01

    Phelan-McDermid syndrome (PMS), also called 22q13.3 deletion syndrome, is a neurodevelopmental disorder characterized by global developmental delay, intellectual disability, severe speech delays, poor motor tone and function, and autism spectrum disorder (ASD). Although the overall prevalence of PMS is unknown, there have been at least 1200 cases reported worldwide, according to the Phelan-McDermid Syndrome Foundation. PMS is now considered to be a relatively common cause of ASD and intellectual disability, accounting for between 0.5% and 2.0% of cases. The cause of PMS has been isolated to loss of function of one copy of SHANK3, which codes for a master scaffolding protein found in the postsynaptic density of excitatory synapses. Reduced expression of SH3 and multiple ankyrin repeat domains 3 (SHANK3) leads to reduced numbers of dendrites, and impaired synaptic transmission and plasticity. Recent mouse and human neuronal models of PMS have led to important opportunities to develop novel therapeutics, and at least 2 clinical trials are underway, one in the USA, and one in the Netherlands. The SHANK3 pathway may also be relevant to other forms of ASD, and many of the single-gene causes of ASD identified to date appear to converge on several common molecular pathways that underlie synaptic neurotransmission. As a result, treatments developed for PMS may also affect other forms of ASD. PMID:25894671

  14. Low-dose fenfluramine in the treatment of neurologic disorders: experience in Dravet syndrome

    PubMed Central

    Schoonjans, An-Sofie; Lagae, Lieven; Ceulemans, Berten

    2015-01-01

    In this paper, we review the experience with fenfluramine in epileptic and other paroxysmal disorders. Since the best available data are from the treatment of Dravet syndrome, we will focus primarily on this condition. Originally fenfluramine was launched as an anorectic agent. As early as 1985, seizure reduction in children could be demonstrated in a few cases with photosensitive, self-induced epilepsy. Hereafter, a small study was launched in patients with self-induced epilepsy. Results showed a significant seizure reduction, and review of the patient data showed that 5 of the 12 patients had Dravet syndrome. During that observation period, fenfluramine was withdrawn from the market because of cardiovascular side effects associated with prescribing higher doses in combination with phentermine for weight loss. In March 2002, a Belgian Royal Decree was issued permitting further study of fenfluramine in pediatric patients with intractable epilepsy. In 2011 under the Royal Decree, a prospective study of patients with Dravet syndrome treated with low-dose fenfluramine was initiated and is currently ongoing. The initial results are promising in terms of reduction of seizure frequency and overall tolerability. PMID:26600876

  15. Surgical treatment of ectopic adrenocorticotropic hormone syndrome with intra-thoracic tumor

    PubMed Central

    Zhou, Xiang; Hang, Junbiao; Che, Jiaming; Chen, Zhongyuan; Qiu, Weicheng; Ren, Jian; Yang, Xiaoqing; Xiang, Jie

    2016-01-01

    Background The study was to review the clinical manifestations and laboratory examinations of ectopic adrenocorticotropic hormone (ACTH) syndrome, and to analyze the efficacy of surgical treatment. Methods The clinical data, surgical therapy, and outcome of 23 cases of ectopic ACTH syndrome accompanied by intra-thoracic tumors were reviewed. The tumors were removed from all the patients according to the principles of radical resection. Results The tumors were confirmed as associated with ectopic ACTH secretion in 19 cases. Hyperglycemia and hypokalemia were recovered, while plasma cortisol, plasma ACTH and 24-hour urinary free cortisol (UFC) levels were significantly reduced after surgery in these 19 cases. Recurrences of the disease were found in six cases during following-up, and five of them died. Conclusions The thoracic cavity should be a focus in routine examinations of patients with symptoms of Cushing’s syndrome (CS), because ectopic ACTH-producing tumors are commonly found in bronchus/lung and mediastinum. Despite the incidence of the pulmonary nodule secondary to opportunistic infection in some cases, surgery is still the first choice if the tumor is localized. The surgical procedure should be performed according to the principles in resection of lung cancer and mediastinal tumor. The surgical efficacy is significant for short-term periods; however, the recurrence of the disease in long-term periods is in great part related to distal metastasis or relapse of the tumor. PMID:27162663

  16. Evaluation of surgical treatment of carpal tunnel syndrome using local anesthesia☆

    PubMed Central

    Barros, Marco Felipe Francisco Honorato; da Rocha Luz Júnior, Aurimar; Roncaglio, Bruno; Queiróz Júnior, Célio Pinheiro; Tribst, Marcelo Fernandes

    2015-01-01

    Objective To evaluate the results and complications from surgical treatment of carpal tunnel syndrome by means of an open route, using a local anesthesia technique comprising use of a solution of lidocaine, epinephrine and sodium bicarbonate. Material and methods This was a cohort study conducted through evaluating the medical files of 16 patients who underwent open surgery to treat carpal tunnel syndrome, with use of local anesthesia consisting of 20 mL of 1% lidocaine, adrenaline at 1:100,000 and 2 mL of sodium bicarbonate. The DASH scores before the operation and six months after the operation were evaluated. Comparisons were made regarding the intensity of pain at the time of applying the anesthetic and during the surgical procedure, and in relation to other types of procedure. Results The DASH score improved from 65.17 to 16.53 six months after the operation (p < 0.01). In relation to the anesthesia, 75% of the patients reported that this technique was better than or the same as venous puncture and 81% reported that it was better than a dental procedure. Intraoperative pain occurred in two cases. There were no occurrences of ischemia. Conclusion Use of local anesthesia for surgically treating carpal tunnel syndrome is effective for performing the procedure and for the final result. PMID:26962490

  17. Obesity, metabolic syndrome and diabetes mellitus after renal transplantation: prevention and treatment.

    PubMed

    Wissing, Karl Martin; Pipeleers, Lissa

    2014-04-01

    The prevalence of the metabolic syndrome in dialysis patients is high and further increases after transplantation due to weight gain and the detrimental metabolic effects of immunosuppressive drugs. Corticosteroids cause insulin resistance, hyperlipidemia, abnormal glucose metabolism and arterial hypertension. The calcineurin inhibitor tacrolimus is diabetogenic by inhibiting insulin secretion, whereas cyclosporine causes hypertension and increases cholesterol levels. Mtor antagonists are responsible for hyperlipidemia and abnormal glucose metabolism by mechanisms that also implicate insulin resistance. The metabolic syndrome in transplant recipients has numerous detrimental effects such as increasing the risk of new onset diabetes, cardiovascular disease events and patient death. In addition, it has also been linked with accelerated loss of graft function, proteinuria and ultimately graft loss. Prevention and management of the metabolic syndrome are based on increasing physical activity, promotion of weight loss and control of cardiovascular risk factors. Bariatric surgery before or after renal transplantation in patients with body mass index >35 kg/m(2) is an option but its long term effects on graft and patient survival have not been investigated. Steroid withdrawal and replacement of tacrolimus with cyclosporine facilitate control of diabetes, whereas replacement of cyclosporine and mtor antagonists can improve hyperlipidemia. The new costimulation inhibitor belatacept has potent immunosuppressive properties without metabolic adverse effects and will be an important component of immunosuppressive regimens with better metabolic risk profile. Medical treatment of cardiovascular risk factors has to take potential drug interactions with immunosuppressive medication and drug accumulation due to renal insufficiency into account. PMID:24507957

  18. Alport syndrome: its effects on the glomerular filtration barrier and implications for future treatment

    PubMed Central

    Savige, Judy

    2014-01-01

    The glomerular filtration barrier comprises a fenestrated capillary endothelium, glomerular basement membrane and podocyte slit diaphragm. Over the past decade we have come to realise that permselectivity depends on size and not necessarily charge, that the molecular sieve depends on the podocyte contractile apparatus and is highly dynamic, and that protein uptake by proximal tubular epithelial cells stimulates signalling and the production of transcription factors and inflammatory mediators. Alport syndrome is the second commonest monogenic cause of renal failure after autosomal dominant polycystic kidney disease. Eighty per cent of patients have X-linked disease caused by mutations in the COL4A5 gene. Most of these result in the replacement of the collagen IV α3α4α5 network with the α1α1α2 heterotrimer. Affected membranes also have ectopic laminin and increased matrix metalloproteinase levels, which makes them more susceptible to proteolysis. Mechanical stress, due to the less elastic membrane and hypertension, interferes with integrin-mediated podocyte–GBM adhesion. Proteinuria occurs when urinary levels exceed tubular reabsorption rates, and initiates tubulointerstitial fibrosis. The glomerular mesangial cells produce increased TGFβ and CTGF which also contribute to glomerulosclerosis. Currently there is no specific therapy for Alport syndrome. However treatment with angiotensin converting enzyme (ACE) inhibitors delays renal failure progression by reducing intraglomerular hypertension, proteinuria, and fibrosis. Our greater understanding of the mechanisms underlying the GBM changes and their consequences in Alport syndrome have provided us with further novel therapeutic targets. PMID:25107927

  19. Oral findings and dental treatment in a child with Williams-Beuren syndrome.

    PubMed

    Torres, Carolina Paes; Valadares, Gleice; Martins, Mariana Izabella; Borsatto, Maria Cristina; Díaz-Serrano, Kranya Victoria; de Queiroz, Alexandra Mussolino

    2015-01-01

    Williams-Beuren syndrome (WBS), also known as Williams syndrome, is a rare congenital disorder involving cardiovascular problems, mental retardation, distinctive facial features and tooth anomalies. It is caused by the submicroscopic deletion of 1.5 to 1.8 Mb on chromosome 7q11.23. This paper reports the dental care to a 7-year-old child with WBS syndrome. The interview also revealed visual impairment, sensorineural hearing loss, hyperacusis, photophobia and hoarse voice. The intraoral clinical examination showed anterior open bite, tongue thrusting, excessive interdental spacing, enamel hypomineralization of the incisors, hypoplasia and caries lesions. The dental treatment included: modulating sessions to control aversion to noises, the photophobia, and the dental fear and anxiety because of his reduced visual acuity; oral hygiene instructions, dietary and daily use of a 0.05% sodium fluoride mouthwash; the permanent mandibular left first molar was treated endodontically, and maxillary and mandibular first molars were restored with amalgam. Due to the patient's heart defect, a prophylactic antibiotic regimen was prescribed prior to the dental procedures. This patient has been followed up for 4 years and this case report underscores the importance of early dental evaluation and counseling for parents of WBS patients. PMID:26200160

  20. Low-dose fenfluramine in the treatment of neurologic disorders: experience in Dravet syndrome.

    PubMed

    Schoonjans, An-Sofie; Lagae, Lieven; Ceulemans, Berten

    2015-11-01

    In this paper, we review the experience with fenfluramine in epileptic and other paroxysmal disorders. Since the best available data are from the treatment of Dravet syndrome, we will focus primarily on this condition. Originally fenfluramine was launched as an anorectic agent. As early as 1985, seizure reduction in children could be demonstrated in a few cases with photosensitive, self-induced epilepsy. Hereafter, a small study was launched in patients with self-induced epilepsy. Results showed a significant seizure reduction, and review of the patient data showed that 5 of the 12 patients had Dravet syndrome. During that observation period, fenfluramine was withdrawn from the market because of cardiovascular side effects associated with prescribing higher doses in combination with phentermine for weight loss. In March 2002, a Belgian Royal Decree was issued permitting further study of fenfluramine in pediatric patients with intractable epilepsy. In 2011 under the Royal Decree, a prospective study of patients with Dravet syndrome treated with low-dose fenfluramine was initiated and is currently ongoing. The initial results are promising in terms of reduction of seizure frequency and overall tolerability. PMID:26600876

  1. Treatments and technologies in the rehabilitation of apraxia and action disorganisation syndrome: A review

    PubMed Central

    Worthington, Andrew

    2016-01-01

    BACKGROUND: Apraxia and Action Disorganisation Syndrome are characterised by an inability to use tools and carry out ordered sequences of movements in the absence of motor or sensory impairment. To date treatment for these complex but debilitating conditions has received little attention. OBJECTIVES: To provide an overview of apraxia and action disorganisation syndrome and its treatment, providing a state of the art summary for practitioners including likely future therapeutic directions. METHOD: Review of apraxia literature and treatment studies collated from internet searches involving MEDLINE, PubMed, PyscINFO and Google Scholar as well as the author’s own catalogue. RESULTS: Evidence for current restitution and compensatory approaches is critically reviewed, with limited evidence to date in support of either method. Strategy training is the most promising intervention type with no support for sensory and exploratory interventions, practice effects only for direct task-specific training, and modest support for gestural training. CONCLUSIONS: Larger controlled studies are needed but evidence is sufficient to indicate certain approaches over others. Advances in assistive technology have not translated into mainstream therapy but future interventions are likely to require a model-based approach which embraces current technologies in order to provide a more accessible, effective and cost-efficient approach to rehabilitation. PMID:27314872

  2. Repurposing of Clinically Developed Drugs for Treatment of Middle East Respiratory Syndrome Coronavirus Infection

    PubMed Central

    Dyall, Julie; Coleman, Christopher M.; Hart, Brit J.; Venkataraman, Thiagarajan; Holbrook, Michael R.; Kindrachuk, Jason; Johnson, Reed F.; Olinger, Gene G.; Jahrling, Peter B.; Laidlaw, Monique; Johansen, Lisa M.; Lear-Rooney, Calli M.; Glass, Pamela J.; Hensley, Lisa E.

    2014-01-01

    Outbreaks of emerging infections present health professionals with the unique challenge of trying to select appropriate pharmacologic treatments in the clinic with little time available for drug testing and development. Typically, clinicians are left with general supportive care and often untested convalescent-phase plasma as available treatment options. Repurposing of approved pharmaceutical drugs for new indications presents an attractive alternative to clinicians, researchers, public health agencies, drug developers, and funding agencies. Given the development times and manufacturing requirements for new products, repurposing of existing drugs is likely the only solution for outbreaks due to emerging viruses. In the studies described here, a library of 290 compounds was screened for antiviral activity against Middle East respiratory syndrome coronavirus (MERS-CoV) and severe acute respiratory syndrome coronavirus (SARS-CoV). Selection of compounds for inclusion in the library was dependent on current or previous FDA approval or advanced clinical development. Some drugs that had a well-defined cellular pathway as target were included. In total, 27 compounds with activity against both MERS-CoV and SARS-CoV were identified. The compounds belong to 13 different classes of pharmaceuticals, including inhibitors of estrogen receptors used for cancer treatment and inhibitors of dopamine receptor used as antipsychotics. The drugs identified in these screens provide new targets for in vivo studies as well as incorporation into ongoing clinical studies. PMID:24841273

  3. Traditional Chinese medicine valuably augments therapeutic options in the treatment of climacteric syndrome.

    PubMed

    Eisenhardt, Sarah; Fleckenstein, Johannes

    2016-07-01

    Climacteric syndrome refers to recurring symptoms such as hot flashes, chills, headache, irritability and depression. This is usually experienced by menopausal women and can be related to a hormonal reorganization in the hypothalamic-pituitary-gonadal axis. In Traditional Chinese Medicine, originating 1000s of years ago, above-mentioned symptoms can be interpreted on the basis of the philosophic diagnostic concepts, such as the imbalance of Yin and Yang, the Zang-Fu and Basic substances (e.g. Qi, Blood and Essence). These concepts postulate balance and harmonization as the principle aim of a treatment. In this context, it is not astounding that one of the most prominent ancient textbooks dating back to 500-200 BC, Huang di Neijing: The Yellow Emperor's Classic of Internal Medicine gives already first instructions for diagnosis and therapy of climacteric symptoms. For therapy, traditional Chinese medicine comprises five treatment principles: Chinese herbal medicine, TuiNa (a Chinese form of manual therapy), nutrition, activity (e.g. QiGong) and acupuncture (being the most widespread form of treatment used in Europe). This review provides an easy access to the concepts of traditional Chinese medicine particularly regarding to climacteric syndrome and also focuses on current scientific evidence. PMID:27040419

  4. Bone microarchitecture in Rett syndrome and treatment with teriparatide: a case report.

    PubMed

    Zanchetta, M B; Scioscia, M F; Zanchetta, J R

    2016-09-01

    We present the case of a 28-year-old female Rett syndrome patient with low bone mass and a recent fracture who was successfully treated with teriparatide. Bone mineral density and microarchitecture substantially improved after treatment. Rett syndrome (RTT), an X-linked progressive neuro-developmental disorder caused by mutations in the methyl-CpG-binding 2 (MECP2) gene, has been consistently associated with low bone mass. Consequently, patients with RTT are at increased risk of skeletal fractures. Teriparatide is a bone-forming agent for the treatment of osteoporosis that has demonstrated its effectiveness in increasing bone strength and reducing the risk of fractures in postmenopausal women, but, recently, its positive action has also been reported in premenopausal women. We present the case of a 28-year-old female RTT patient with low bone mass and a recent fracture who was successfully treated with teriparatide. Both bone mass measured by DXA and microarchitecture assessed by high resolution peripheral computed tomography (HR pQCT) were substantially improved after treatment. PMID:27068223

  5. Medical Treatment of Aortic Aneurysms in Marfan Syndrome and other Heritable Conditions

    PubMed Central

    Jost, Christine H. Attenhofer; Greutmann, Matthias; Connolly, Heidi M.; Weber, Roland; Rohrbach, Marianne; Oxenius, Angela; Kretschmar, Oliver; Luscher, Thomas F.; Matyas, Gabor

    2014-01-01

    Thoracic aortic aneurysms can be triggered by genetic disorders such as Marfan syndrome (MFS) and related aortic diseases as well as by inflammatory disorders such as giant cell arteritis or atherosclerosis. In all these conditions, cardiovascular risk factors, such as systemic arterial hypertension, may contribute to faster rate of aneurysm progression. Optimal medical management to prevent progressive aortic dilatation and aortic dissection is unknown. β-blockers have been the mainstay of medical treatment for many years despite limited evidence of beneficial effects. Recently, losartan, an angiotensin II type I receptor antagonist (ARB), has shown promising results in a mouse model of MFS and subsequently in humans with MFS and hence is increasingly used. Several ongoing trials comparing losartan to β-blockers and/or placebo will better define the role of ARBs in the near future. In addition, other medications, such as statins and tetracyclines have demonstrated potential benefit in experimental aortic aneurysm studies. Given the advances in our understanding of molecular mechanisms triggering aortic dilatation and dissection, individualized management tailored to the underlying genetic defect may be on the horizon of individualized medicine. We anticipate that ongoing research will address the question whether such genotype/pathogenesis-driven treatments can replace current phenotype/syndrome-driven strategies and whether other forms of aortopathies should be treated similarly. In this work, we review currently used and promising medical treatment options for patients with heritable aortic aneurysmal disorders. PMID:24527681

  6. Obstructive sleep apnea syndrome in children: controversies in diagnosis and treatment.

    PubMed

    Rosen, Carol L

    2004-02-01

    Obstructive sleep apnea syndrome (OSAS) is a common, under-recognized condition in childhood with significant morbidities if undiagnosed and untreated. The American Academy of Pediatrics recently issued a clinical practice guideline for the diagnosis and management of childhood OSAS. It was accompanied by a comprehensive evidence-based technical report that summarized the available literature supporting the guideline. The current review highlights areas of controversy and uncertainty that limit the development of more definitive standards of practice, updates the reader to several newer publications relevant to diagnosis and treatment of childhood OSAS, and identifies future directions for clinical research. PMID:15008587

  7. The History of Treatment of Twin-to-Twin Transfusion Syndrome.

    PubMed

    Glennon, Chelsea L; Shemer, Scott A; Palma-Dias, Ricardo; Umstad, Mark P

    2016-06-01

    Historical suggestions of twin-to-twin transfusion syndrome (TTTS) date back to the early 17th century. Placental anastomoses were first reported in 1687; however, it was Schatz who first identified their importance in 1875. He recognized 'the area of transfusion' within the 'villous district' of the placenta, which he named the 'third circulation'. This article describes how the management of TTTS has evolved as we have gained a more sophisticated understanding and appreciation of the complex vascular anastomoses that exist in monochorionic twin placentae. Currently, fetosopic laser occlusion is the preferred treatment option for TTTS. PMID:27203604

  8. Assessment and Surgical Treatment of Calcinosis of the Shoulder Associated with CREST Syndrome

    PubMed Central

    Breusch, S. J.

    2016-01-01

    We report an unusual case of a 65-year-old lady with CREST syndrome with multiple upper and lower limb calcinosis, who presented with severe shoulder pain and stiffness, with widespread intra- and extra-articular calcinosis, which was refractory to conservative measures. We were able to identify the main cause of her symptoms through serial diagnostic injections as calcific biceps tendinosis. We will discuss her assessment and surgical management and the pathophysiology and various treatment modalities for managing the soft tissue calcinosis in rheumatological diseases. PMID:27437158

  9. Hematologic Response to Vorinostat Treatment in Relapsed Myeloid Leukemia of Down Syndrome.

    PubMed

    Scheer, Carina; Kratz, Christian; Witt, Olaf; Creutzig, Ursula; Reinhardt, Dirk; Klusmann, Jan-Henning

    2016-09-01

    Children with Down syndrome are at high risk to develop myeloid leukemia (ML-DS). Despite their excellent prognosis, children with ML-DS particularly suffer from severe therapy-related toxicities and for relapsed ML-DS the cure rates are very poor. Here we report the clinical course of one child with ML-DS treated with the histone deacetylase (HDAC) inhibitor vorinostat (suberoylanilide hydroxamic acid) after second relapse. The child had previously received conventional chemotherapy and stem cell transplantation, yet showed a remarkable clinical and hematologic response. Thus, HDAC inhibitor may represent an effective class of drugs for the treatment of ML-DS. PMID:27191354

  10. Disseminated nocardiosis in a patient on infliximab and methylprednisolone for treatment-resistant Sweet's syndrome.

    PubMed

    Drone, Elizabeth R; McCrory, Allison L; Lane, Natalie; Fiala, Katherine

    2014-07-01

    A 62-year-old white man with a 10-year history of treatment-refractory Sweet's syndrome was admitted to the hospital with the onset of purpuric lesions. Methylprednisolone and infliximab were administered. Our patient developed disseminated Nocardia infection and eventually succumbed. Opportunistic infections such as Nocardia have been associated with infliximab and other tumour necrosis factor (TNF)-α inhibitors. The astute clinician should be aware of the risk of rare opportunistic infections, particularly in patients on TNF-α inhibitors and systemic corticosteroids. PMID:25165648

  11. Extracorporeal Photopheresis in the Treatment of Mycosis Fungoides and Sézary Syndrome.

    PubMed

    Zic, John A

    2015-10-01

    Extracorporeal photopheresis (ECP) is an immunomodulating procedure that leads to an expansion of peripheral blood dendritic cell populations and an enhanced TH1 immune response in cutaneous T-cell lymphoma (CTCL). Because of its excellent side effect profile and moderate efficacy, ECP is considered first-line therapy for erythrodermic mycosis fungoides (MF) and Sézary syndrome. Patients with a measurable but low blood tumor burden are most likely to respond to ECP, and the addition of adjunctive immunostimulatory agents may also increase response rates. There may be a role for ECP in the treatment of refractory early stage MF, but data are limited. PMID:26433848

  12. Acute myeloid leukaemia after treatment for acute lymphoblastic leukaemia in girl with Bloom syndrome

    PubMed Central

    Adams, Madeleine; Jenney, Meriel; Lazarou, Laz; White, Rhian; Birdsall, Sanda; Staab, Timo; Schindler, Detlev; Meyer, Stefan

    2014-01-01

    Bloom syndrome (BS) is an inherited genomic instability disorder caused by disruption of the BLM helicase and confers an extreme cancer predisposition. Here we report on a girl with BS who developed acute lymphoblastic leukaemia (ALL) at age nine, and treatment-related acute myeloid leukaemia (t-AML) aged 12. She was compound heterozygous for the novel BLM frameshift deletion c.1624delG and the previously described c.3415C>T nonsense mutation. Two haematological malignancies in a child with BS imply a fundamental role for BLM for normal haematopoiesis, in particular in the presence of genotoxic stress. PMID:24932421

  13. Acute myeloid leukaemia after treatment for acute lymphoblastic leukaemia in girl with Bloom syndrome.

    PubMed

    Adams, Madeleine; Jenney, Meriel; Lazarou, Laz; White, Rhian; Birdsall, Sanda; Staab, Timo; Schindler, Detlev; Meyer, Stefan

    2013-09-18

    Bloom syndrome (BS) is an inherited genomic instability disorder caused by disruption of the BLM helicase and confers an extreme cancer predisposition. Here we report on a girl with BS who developed acute lymphoblastic leukaemia (ALL) at age nine, and treatment-related acute myeloid leukaemia (t-AML) aged 12. She was compound heterozygous for the novel BLM frameshift deletion c.1624delG and the previously described c.3415C>T nonsense mutation. Two haematological malignancies in a child with BS imply a fundamental role for BLM for normal haematopoiesis, in particular in the presence of genotoxic stress. PMID:24932421

  14. Intrinsic brain networks normalize with treatment in pediatric complex regional pain syndrome

    PubMed Central

    Becerra, Lino; Sava, Simona; Simons, Laura E.; Drosos, Athena M.; Sethna, Navil; Berde, Charles; Lebel, Alyssa A.; Borsook, David

    2014-01-01

    Pediatric complex regional pain syndrome (P-CRPS) offers a unique model of chronic neuropathic pain as it either resolves spontaneously or through therapeutic interventions in most patients. Here we evaluated brain changes in well-characterized children and adolescents with P-CRPS by measuring resting state networks before and following a brief (median = 3 weeks) but intensive physical and psychological treatment program, and compared them to matched healthy controls. Differences in intrinsic brain networks were observed in P-CRPS compared to controls before treatment (disease state) with the most prominent differences in the fronto-parietal, salience, default mode, central executive, and sensorimotor networks. Following treatment, behavioral measures demonstrated a reduction of symptoms and improvement of physical state (pain levels and motor functioning). Correlation of network connectivities with spontaneous pain measures pre- and post-treatment indicated concomitant reductions in connectivity in salience, central executive, default mode and sensorimotor networks (treatment effects). These results suggest a rapid alteration in global brain networks with treatment and provide a venue to assess brain changes in CRPS pre- and post-treatment, and to evaluate therapeutic effects. PMID:25379449

  15. Effects of long-term treatment on brain volume in patients with obstructive sleep apnea syndrome.

    PubMed

    Kim, Hosung; Joo, EunYeon; Suh, Sooyeon; Kim, Jae-Hun; Kim, Sung Tae; Hong, Seung Bong

    2016-01-01

    We assessed structural brain damage in obstructive sleep apnea syndrome (OSA) patients (21 males) and the effects of long-term continuous positive airway pressure (CPAP) treatment (18.2 ± 12.4 months; 8-44 months) on brain structures and investigated the relationship between severity of OSA and effects of treatment. Using deformation-based morphometry to measure local volume changes, we identified widespread neocortical and cerebellar atrophy in untreated patients compared to controls (59 males; Cohen's D = 0.6; FDR < 0.05). Analysis of longitudinally scanned magnetic resonance imaging (MRI) scans both before and after treatment showed increased brain volume following treatment (FDR < 0.05). Volume increase was correlated with longer treatment in the cortical areas that largely overlapped with the initial atrophy. The areas overlying the hippocampal dentate gyrus and the cerebellar dentate nucleus displayed a volume increase after treatment. Patients with very severe OSA (AHI > 64) presented with prefrontal atrophy and displayed an additional volume increase in this area following treatment. Higher impairment of working memory in patients prior to treatment correlated with prefrontal volume increase after treatment. The large overlap between the initial brain damage and the extent of recovery after treatment suggests partial recovery of nonpermanent structural damage. Volume increases in the dentate gyrus and the dentate nucleus possibly likely indicate compensatory neurogenesis in response to diminishing oxidative stress. Such changes in other brain structures may explain gliosis, dendritic volume increase, or inflammation. This study provides neuroimaging evidence that revealed the positive effects of long-term CPAP treatment in patients with OSA. PMID:26503297

  16. Endovascular Treatment of Bilateral Pulmonary Artery Stenoses and Superior Vena Cava Syndrome in a Patient with Advanced Mediastinal Fibrosis

    PubMed Central

    Kuban, Joshua D.; Ramanathan, Rohit; Whigham, Cliff J.

    2016-01-01

    Vascular stenosis is a relatively uncommon and often fatal sequela of mediastinal fibrosis. There are very few reports in the medical literature of endovascular treatment for concomitant bilateral pulmonary artery stenoses and superior vena cava syndrome. We report the endovascular treatment of these conditions in a 54-year-old man, and the long-term outcome. PMID:27303243

  17. Long-Term, Multimodal Treatment of a Child with Asperger's Syndrome and Comorbid Disruptive Behavior Problems: A Case Illustration

    ERIC Educational Resources Information Center

    Wymbs, Brian T.; Robb, Jessica A.; Chronis, Andrea M.; Massetti, Greta M.; Fabiano, Gregory A.; Arnold, Frances W.; Brice, Anne-Christina; Gnagy, Elizabeth M.; Pelham, William E., Jr.; Burrows-MacLean, Lisa; Hoffman, Martin T.

    2005-01-01

    Despite Asperger's Syndrome (AS) becoming a widely recognized disorder on the pervasive developmental spectrum, surprisingly few studies have assessed the utility of psychosocial and/or pharmacological treatments for children with AS. Further, studies have not examined the effects of treatment on disruptive behavior problems commonly exhibited by…

  18. A Day-Hospital Approach to Treatment of Pediatric Complex Regional Pain Syndrome: Initial Functional Outcomes

    PubMed Central

    Logan, Deirdre E.; Carpino, Elizabeth A.; Chiang, Gloria; Condon, Marianne; Firn, Emily; Gaughan, Veronica J.; Hogan, Melinda, P.T.; Leslie, David S.; Olson, Katie, P.T.; Sager, Susan; Sethna, Navil; Simons, Laura E.; Zurakowski, David; Berde, Charles B.

    2013-01-01

    Objectives To examine clinical outcomes of an interdisciplinary day hospital treatment program (comprised of physical, occupational, and cognitive-behavioral therapies with medical and nursing services) for pediatric complex regional pain syndrome (CRPS). Methods The study is a longitudinal case series of consecutive patients treated in a day hospital pediatric pain rehabilitation program. Participants were 56 children and adolescents ages 8–18 years (median = 14 years) with CRPS spectrum conditions who failed to progress sufficiently with a previous outpatient and/or inpatient treatments. Patients participated in daily physical therapy, occupational therapy and psychological treatment and received nursing and medical care as necessary. The model places equal emphasis on physical and cognitive-behavioral approaches to pain management. Median duration of stay was 3 weeks. Outcome measures included assessments of physical, occupational, and psychological functioning at program admission, discharge, and at post-treatment follow-up at a median of 10 months post-discharge. Scores at discharge and follow-up were compared with measures on admission by Wilcoxon tests, paired t tests, or ANOVA as appropriate, with corrections for multiple comparisons. Results Outcomes demonstrate clinically and statistically significant improvements from admission to discharge in pain intensity (p<0.001), functional disability (p<0.001), subjective report of limb function (p<0.001), timed running (p<0.001) occupational performance (p<0.001), medication use (p<0.01), use of assistive devices (p<0.001), and emotional functioning (anxiety, p<0.001; depression, p<0.01). Functional gains were maintained or further improved at follow-up. Discussion A day-hospital interdisciplinary rehabilitation approach appears effective in reducing disability and improving physical and emotional functioning and occupational performance among children and adolescents with complex regional pain syndromes that

  19. Emergency Dental Treatment of a Patient With Sturge-Weber Syndrome.

    PubMed

    de Oliveira, Maria Cecília Querido; Maia, Vanessa Nobre; Franco, Juliana Bertoldi; de Melo Peres, Maria Paula Siqueira

    2015-06-01

    The Sturge-Weber (SWS) syndrome is a rare condition with congenital capillary malformations. Hemorrhages may occur on dental treatment, which can have a dramatic effect on a patient's vital sign. The aim of the present brief clinical study was to briefly report a case of a female patient with SWS who underwent an endodontic treatment. A 25-year-old patient (C.O.B.S.) with SWS and vascular malformation in cervicofacial right region was admitted to the dental clinic with pulsatile pain in tooth 16. An emergency dental treatment with cavity preparation and access to root canals was performed with intraligamentary and intrapulpal anesthesia with 3% prilocain 3% with felypressin 0.03 UI/mL because the tooth was located in the region of the vascular malformation, and any surgical treatment could lead to hemorrhages. After 7 days, the patient was recalled with the absence of painful symptoms. The endodontic treatment in patients with SWS is feasible, and safety should be an alternative to surgical treatments. PMID:26080241

  20. Interventional Treatment of Abdominal Compartment Syndrome during Severe Acute Pancreatitis: Current Status and Historical Perspective

    PubMed Central

    Radenkovic, Dejan V.; Johnson, Colin D.; Milic, Natasa; Gregoric, Pavle; Ivancevic, Nenad; Bezmarevic, Mihailo; Bilanovic, Dragoljub; Cijan, Vladimir; Antic, Andrija; Bajec, Djordje

    2016-01-01

    Abdominal compartment syndrome (ACS) in patients with severe acute pancreatitis (SAP) is a marker of severe disease. It occurs as combination of inflammation of retroperitoneum, visceral edema, ascites, acute peripancreatic fluid collections, paralytic ileus, and aggressive fluid resuscitation. The frequency of ACS in SAP may be rising due to more aggressive fluid resuscitation, a trend towards conservative treatment, and attempts to use a minimally invasive approach. There remains uncertainty about the most appropriate surgical technique for the treatment of ACS in SAP. Some unresolved questions remain including medical treatment, indications, timing, and interventional techniques. This review will focus on interventional treatment of this serious condition. First line therapy is conservative treatment aiming to decrease IAP and to restore organ dysfunction. If nonoperative measures are not effective, early abdominal decompression is mandatory. Midline laparostomy seems to be method of choice. Since it carries significant morbidity we need randomized studies to establish firm advantages over other described techniques. After ACS resolves efforts should be made to achieve early primary fascia closure. Additional data are necessary to resolve uncertainties regarding ideal timing and indication for operative treatment. PMID:26839539

  1. Interventional Treatment of Abdominal Compartment Syndrome during Severe Acute Pancreatitis: Current Status and Historical Perspective.

    PubMed

    Radenkovic, Dejan V; Johnson, Colin D; Milic, Natasa; Gregoric, Pavle; Ivancevic, Nenad; Bezmarevic, Mihailo; Bilanovic, Dragoljub; Cijan, Vladimir; Antic, Andrija; Bajec, Djordje

    2016-01-01

    Abdominal compartment syndrome (ACS) in patients with severe acute pancreatitis (SAP) is a marker of severe disease. It occurs as combination of inflammation of retroperitoneum, visceral edema, ascites, acute peripancreatic fluid collections, paralytic ileus, and aggressive fluid resuscitation. The frequency of ACS in SAP may be rising due to more aggressive fluid resuscitation, a trend towards conservative treatment, and attempts to use a minimally invasive approach. There remains uncertainty about the most appropriate surgical technique for the treatment of ACS in SAP. Some unresolved questions remain including medical treatment, indications, timing, and interventional techniques. This review will focus on interventional treatment of this serious condition. First line therapy is conservative treatment aiming to decrease IAP and to restore organ dysfunction. If nonoperative measures are not effective, early abdominal decompression is mandatory. Midline laparostomy seems to be method of choice. Since it carries significant morbidity we need randomized studies to establish firm advantages over other described techniques. After ACS resolves efforts should be made to achieve early primary fascia closure. Additional data are necessary to resolve uncertainties regarding ideal timing and indication for operative treatment. PMID:26839539

  2. The evolution and treatment of Korsakoff's syndrome: out of sight, out of mind?

    PubMed

    Thomson, A D; Guerrini, Irene; Marshall, E Jane

    2012-06-01

    Wernicke's Encephalopathy is an acute neuro-psychiatric condition caused by an insufficient supply of thiamine (Vitamin B1) to the brain. If undiagnosed or inadequately treated, it is likely to proceed to Korsakoff's Syndrome. Wernicke's Encephalopathy can result from dietary deficiency alone and this form is usually successfully treated, with little chance of Korsakoff's Syndrome supervening. On the other hand, thiamine deficiency associated with alcohol misuse/dependence may require up to 1 gram of thiamine IV in the first 24 hours to be treated successfully. The reasons for this difference in treatment will be discussed. Thiamine diphosphate acts as a co-factor for a number of thiamine-dependent enzymes. Thiamine deficiency leads to a reduction in the activity of these enzymes, and this leads to alterations in mitochondrial activity, impairment of oxidative metabolism, decreased energy status and eventually selective neuronal death. The damage caused by the combination of thiamine deficiency and alcohol metabolism probably interferes with adequate thiamine transport at a number of sites in the body, including the blood-brain barrier, as well as causing damage to the apoenzymes which then require higher concentrations of thiamine to work normally. The accumulated damage is likely to render the use of oral thiamine therapeutically inadequate since the body is unable to produce high enough concentrations of thiamine in the blood to traverse the blood-brain barrier. Some individuals are probably genetically predisposed to develop Wernicke's. Long before individuals with alcohol misuse or dependence develop Wernicke's Encephalopathy the neurons and other cells of the body are functioning sub-optimally because of the inadequate supply of thiamine and the neurotoxic effect of alcohol. This relative deficiency initiates a series of pathological changes which accumulate and further interfere with the supply of thiamine and its utilisation at a time when the requirements

  3. A Controlled Study of Alprostadil Liposomal Preparation in the Treatment of Blue Toe Syndrome.

    PubMed

    Xi, Hai-Lin; Li, Rui; Tian, Zhi-Long; Feng, Su; Jia, Gao-Lei

    2015-05-01

    The aim of this study is to examine the efficacy of alprostadil liposomal preparation in the treatment of blue toe syndrome. As many as 32 patients with blue toe syndrome were randomized into the test group and a control group. Patients out of the test group were treated with alprostadil liposomal preparation, while those out of the control group received placebo administration. Inter-group comparisons were conducted for the post-therapeutic changes of microcirculation and improvements of clinical symptoms. In the test group, there were eight subjects with marked response (50.0 %), six subjects with partial response (37.5 %), and two subjects with no response (12.5 %), with the overall response rate of 87.5 %. In the control group, there were three cases (18.8 %), one case (6.4 %), and 12 cases (75 %), respectively, with the overall response rate of 25.0 %. The inter-group difference of response was statistically significant (Χ (2) = 12.987, P = 0.002 < 0.05). In the test group, there was one case of administration site redness which could be resolved spontaneously. No other adverse drug reactions were reported. No any complaints were reported for the control group. The inter-group difference of nail-fold microcirculation was not statistically significant (P > 0.05). The post-therapeutic points of nail-fold microcirculation in the test group decreased significantly (P < 0.05), but no significant changes were observed for the control group (P > 0.05). The post-therapeutic waveform changes of photoelectric plethysmography were significant for the test group in comparison to the control. The safety and efficacy of alprostadil liposomal preparation have been demonstrated in the treatment of blue toe syndrome. PMID:25534490

  4. Endoscopic treatment for esophageal varices complicated by Isaacs' syndrome involving difficulty with conventional sedation.

    PubMed

    Suzuki, Yuhei; Yamazaki, Yuichi; Hashizume, Hiroaki; Kobayashi, Takeshi; Ohyama, Tatsuya; Horiguchi, Norio; Sato, Ken; Kakizaki, Satoru; Kusano, Motoyasu; Yamada, Masanobu

    2016-02-01

    A 54-year-old male consulted a local doctor with a chief complaint of systemic convulsions and muscle stiffness and was diagnosed with Isaacs' syndrome based on positive findings for antibodies against voltage-gated potassium channels in 2009. He subsequently experienced repeated hematemesis in 2013, at which time he was taken to our hospital by ambulance. Emergent endoscopy revealed esophageal varices with spurting bleeding. The bleeding was stopped with urgent endoscopic variceal ligation. Three days later, the patient developed sudden dyspnea with stridor during inspiration under sedation with an intravenous injection of low-dose flunitrazepam prior to receiving additional treatment and was aroused with intravenous flumazenil, after which his dyspnea immediately improved. Dyspnea may be induced by muscle cramps associated with Isaacs' syndrome exacerbated by sedation. Endoscopic variceal ligation was performed safely using multiple ligation devices in an awake state following pre-medication with hydroxyzine, without sudden dyspnea. Endoscopists should be cautious of the use of sedatives in patients with diseases associated with muscle twitching or stiffness, as in the current case. In addition, it is necessary to administer endoscopic treatment in an awake state or under conscious sedation in patients with a high risk of dyspnea. PMID:26862027

  5. Effect of Treatment with Ginger on the Severity of Premenstrual Syndrome Symptoms

    PubMed Central

    Khayat, Samira; Kheirkhah, Masoomeh; Behboodi Moghadam, Zahra; Fanaei, Hamed; Kasaeian, Amir; Javadimehr, Mani

    2014-01-01

    Premenstrual syndrome (PMS) is a common disorder. Although the etiology of PMS is not clear, to relieve from this syndrome different methods are recommended. One of them is use of medicinal herbs. This study was carried out to evaluate effects of ginger on severity of symptoms of PMS. This study was a clinical trial, double-blinded work, and participants were randomly allocated to intervention (n = 35) and control (n = 35) groups. To determine persons suffering from PMS, participants completed daily record scale questionnaire for two consecutive cycles. After identification, each participant received two ginger capsules daily from seven days before menstruation to three days after menstruation for three cycles and they recorded severity of the symptoms by daily record scale questionnaire. Data before intervention were compared with date 1, 2, and 3 months after intervention. Before intervention, there were no significant differences between the mean scores of PMS symptoms in the two groups, but after 1, 2, and 3 months of treatment, there was a significant difference between the two groups (P < 0.0001). Based on the results of this study, maybe ginger is effective in the reduction of severity of mood and physical and behavioral symptoms of PMS and we suggest ginger as treatment for PMS. PMID:24944825

  6. Favorable Impact of Growth Hormone Treatment on Cholesterol Levels in Turner Syndrome

    PubMed Central

    Kohno, Hitoshi; Igarashi, Yutaka; Ozono, Keiichi; Ohyama, Kenji; Ogawa, Masamichi; Osada, Hisao; Onigata, Kazumichi; Kanzaki, Susumu; Seino, Yoshiki; Takahashi, Hiroaki; Tajima, Toshihiro; Tachibana, Katsuhiko; Tanaka, Hiroyuki; Nishi, Yoshikazu; Hasegawa, Tomonobu; Fujieda, Kenji; Fujita, Keinosuke; Horikawa, Reiko; Yokoya, Susumu; Yorifuji, Toru; Tanaka, Toshiaki

    2012-01-01

    Background: Patients with Turner syndrome (TS) are prone to having metabolic abnormalities, such as obesity, dyslipidemia, hypertension, hyperinsulinemia and type 2 diabetes mellitus, resulting in increased risks of developing atherosclerotic diseases. Objective: To determine the effect of growth hormone (GH) therapy on serum cholesterol levels in prepubertal girls with TS enrolled in the Turner syndrome Research Collaboration (TRC) in Japan. Patients and methods: Eighty-one girls with TS were enrolled in the TRC, and their total cholesterol (TC) levels before GH therapy were compared with reported levels of healthy school-aged Japanese girls. TC levels after 1, 2 and 3 yr of GH treatment were available for 28 of the 81 patients with TS. GH was administered by daily subcutaneous injections, 6 or 7 times/wk, with a weekly dose of 0.35 mg/kg body weight. Results: Baseline TC levels revealed an age-related increase in TS that was in contrast to healthy girls showing unchanged levels. During GH therapy, TC decreased significantly after 1 yr of GH treatment and remained low thereafter. Conclusions: Girls with untreated TS showed an age-related increase in TC that was a striking contrast to healthy girls, who showed unchanged levels. GH therapy in girls with TS brought about a favorable change in TC that indicates the beneficial impact of GH on atherogenic risk. PMID:23926408

  7. Intravesical Liposome and Antisense Treatment for Detrusor Overactivity and Interstitial Cystitis/Painful Bladder Syndrome

    PubMed Central

    Kashyap, Mahendra P.; Kawamorita, Naoki; Yoshizawa, Tsuyoshi; Chancellor, Michael

    2014-01-01

    Purpose. The following review focuses on the recent advancements in intravesical drug delivery, which brings added benefit to the therapy of detrusor overactivity and interstitial cystitis/painful bladder syndrome (IC/PBS). Results. Intravesical route is a preferred route of administration for restricting the action of extremely potent drugs like DMSO for patients of interstitial cystitis/painful bladder syndrome (IC/PBS) and botulinum toxin for detrusor overactivity. Patients who are either refractory to oral treatment or need to mitigate the adverse effects encountered with conventional routes of administration also chose this route. Its usefulness in some cases can be limited by vehicle (carrier) toxicity or short duration of action. Efforts have been underway to overcome these limitations by developing liposome platform for intravesical delivery of biotechnological products including antisense oligonucleotides. Conclusions. Adoption of forward-thinking approaches can achieve advancements in drug delivery systems targeted to future improvement in pharmacotherapy of bladder diseases. Latest developments in the field of nanotechnology can bring this mode of therapy from second line of treatment for refractory cases to the forefront of disease management. PMID:24527221

  8. Current diagnostics and treatment of the cubital tunnel syndrome in Austria

    PubMed Central

    Harder, Kristina; Diehm, Jens; Fassola, Isabella; Al khaled, Nesrin; Doll, Dietrich; Dunda, Sebastian E.; Krapohl, Björn Dirk

    2016-01-01

    According to the vote of the Austrian Society for Surgery of the Hand (ÖGH) an investigation to collect data on the current state of the treatment of cubital tunnel syndrome was initiated. Over one year a total of 875 patients with cubital tunnel syndrome were operated in Austria, this means an incidence of this nerve entrapment of 0.011%. Most of the operations were done by trauma surgeons (287; 33%). For diagnosis most of the centers rely on clinical symptoms, electroneurophysiology, and elbow X-ray. 40% of the institutions regard conservative therapy as useless and not indicated. If conservative treatment modalities are applied, physiotherapy (97%), non-steroidal anti-inflammatory medication (77%), and glucocorticoid injections (30%) are primarily used. In case of simple nerve entrapment most of the surgeons (72%) prefer simple nerve decompression. If there is additional pathology subcutaneous cubital nerve transposition is recommended (62%). Endoscopic techniques are only use by 3% of the surgeons. In the postoperative care, physiotherapy is favored in 51%, whereas 24% do not judge any postoperative care as beneficial. The three most often encountered complications were incomplete remission, scar contracture and hypertrophy, and postoperative bleeding. PMID:26816669

  9. Pharmacological approach to the treatment of long and short QT syndromes.

    PubMed

    Patel, Chinmay; Antzelevitch, Charles

    2008-04-01

    Inherited channelopathies have received increasing attention in recent years. The past decade has witnessed impressive progress in our understanding of the molecular and cellular basis of arrhythmogenesis associated with inherited channelopathies. An imbalance in ionic forces induced by these channelopathies affects the duration of ventricular repolarization and amplifies the intrinsic electrical heterogeneity of the myocardium, creating an arrhythmogenic milieu. Today, many of the channelopathies have been linked to mutations in specific genes encoding either components of ion channels or membrane or regulatory proteins. Many of the channelopathies are genetically heterogeneous with a variable degree of expression of the disease. Defining the molecular basis of channelopathies can have a profound impact on patient management, particularly in cases in which genotype-specific pharmacotherapy is available. The long QT syndrome (LQTS) is one of the first identified and most studied channelopathies where abnormal prolongation of ventricular repolarization predisposes an individual to life threatening ventricular arrhythmia called Torsade de Pointes. On the other hand of the spectrum, molecular defects favoring premature repolarization lead to Short QT syndrome (SQTS), a recently described inherited channelopathy. Both of these channelopathies are associated with a high risk of sudden cardiac death due to malignant ventricular arrhythmia. Whereas pharmacological therapy is first line treatment for LQTS, defibrillators are considered as primary treatment for SQTS. This review provides a comprehensive review of the molecular genetics, clinical features, genotype-phenotype correlations and genotype-specific approach to pharmacotherapy of these two mirror-image channelopathies, SQTS and LQTS. PMID:18378319

  10. Aerosolized GLP-1 for treatment of diabetes mellitus and irritable bowel syndrome.

    PubMed

    Siekmeier, Rüdiger; Hofmann, Thomas; Scheuch, Gerhard; Pokorski, Mieczyslaw

    2015-01-01

    Diabetes is a global burden and the prevalence of the disease, in particular diabetes mellitus type 2 is rapidly increasing worldwide. After introduction of insulin into clinical therapy about 90 years ago a major number of pharmaceuticals has been developed for treatment of diabetes mellitus type 2. One of these, the incretin glucagon-like peptide 1 (GLP-1), like insulin, needs subcutaneous administration causing inconvenience to patients. However, administration of GLP-1 plays also a role for treatment of irritable bowel syndrome (IBS). To improve patient convenience inhaled insulin (Exubera(®)) was developed and approved but failed market acceptance some years ago. Recently, another inhalative insulin (Afrezza(®)) received market approval and GLP-1 may serve as another candidate drug for inhalative administration. This review analyzes the current literature investigating alternative administration of GLP-1 and GLP-1 analogs focusing on inhalation. Several formulations for inhalative administration of GLP-1 and analogs were investigated in animal studies, whereas there are only few clinical data. However, feasibility of GLP-1 inhalation has been shown and should be further investigated as such type of drug administration may serve for improvement of therapy in patients with diabetes mellitus or irritable bowel syndrome. PMID:25427821

  11. Capsaicin and the Treatment of Vulvar Vestibulitis Syndrome: A Valuable Alternative?

    PubMed Central

    Murina, Filippo; Radici, Gianluigi; Bianco, Vanda

    2004-01-01

    Objective To assess the efficacy of topical capsaicin in the treatment of vulvar vestibulitis syndrome. Study Design Thirty-three consecutive women referred for vulvar vestibulitis syndrome were treated with topical capsaicin 0.05 %. The capsaicin cream was applied twice a day for 30 days, then once a Day for 30 days, and finally 2 times a week for 4 months. Results In 19 patients (59%), improvement of symptoms was recorded, but no complete remission was observed. Symptoms recurred in all patients after the use of capsaicin cream was discontinued. A return to a twice-weekly topical application of the cream resulted in the improvement of symptoms. Severe burning was reported as the only side effect by all the patients. Conclusion Response to treatment was only partial, possibly due to the concentration of the compound being too low, or to the need for more frequent than daily applications. The therapeutic role of capsaicin should hence be confined to a last-choice medical approach. PMID:15775875

  12. [Surgical treatment for ureteropelvic junction syndrome in infants and children (report of 73 cases)].

    PubMed

    Michel, A; Monod, P; Descotes, J L; Rambeaud, J J; Bourdat, G; Faure, G

    1991-12-01

    between 1982 and 1990, 73 children were operated for pyeloureteric junction syndrome (76 pyeloplasties). The authors distinguish between a group of 45 infants operated before the age of 2 years and a group of 35 children operated after the age of 2 years. 40% of these cases had been detected antenatally, which modified the management and treatment of these infants, operated at about the age of 6 months (mean: 6.6 months). Treatment was conservative (one nephrectomy out of the 41 infants operated: 2.4%). Pyeloplasty was performed according to the Anderson-Hynes-Küss resection-anastomosis technique under an operating magnifying glass. Early surgery offers the best chance of recovery. Urinary tract drainage was not systematic in these infants (50% of cases) and did not affect the long term results. Early complications and surgical revisions (3.9%) were not more frequent in the infants operated earlier. The definitive results were good in 88.5% of cases, while 7.2% of cases required further surveillance. Renal parenchymal lesions (4.3%) were related more to pyelonephritis than to failure of surgery. Overall, a conservative surgical approach is recommended, except in minor syndromes which require rigorous surveillance. PMID:1844746

  13. Current diagnostics and treatment of the cubital tunnel syndrome in Austria.

    PubMed

    Harder, Kristina; Diehm, Jens; Fassola, Isabella; Al Khaled, Nesrin; Doll, Dietrich; Dunda, Sebastian E; Krapohl, Björn Dirk

    2016-01-01

    According to the vote of the Austrian Society for Surgery of the Hand (ÖGH) an investigation to collect data on the current state of the treatment of cubital tunnel syndrome was initiated. Over one year a total of 875 patients with cubital tunnel syndrome were operated in Austria, this means an incidence of this nerve entrapment of 0.011%. Most of the operations were done by trauma surgeons (287; 33%). For diagnosis most of the centers rely on clinical symptoms, electroneurophysiology, and elbow X-ray. 40% of the institutions regard conservative therapy as useless and not indicated. If conservative treatment modalities are applied, physiotherapy (97%), non-steroidal anti-inflammatory medication (77%), and glucocorticoid injections (30%) are primarily used. In case of simple nerve entrapment most of the surgeons (72%) prefer simple nerve decompression. If there is additional pathology subcutaneous cubital nerve transposition is recommended (62%). Endoscopic techniques are only use by 3% of the surgeons. In the postoperative care, physiotherapy is favored in 51%, whereas 24% do not judge any postoperative care as beneficial. The three most often encountered complications were incomplete remission, scar contracture and hypertrophy, and postoperative bleeding. PMID:26816669

  14. Single portal endoscopic treatment for chronic exertional compartment syndrome of the forearm.

    PubMed

    Pozzi, Alessandro; Pivato, Giorgio; Kask, Kristo; Susini, Francesca; Pegoli, Loris

    2014-09-01

    Chronic exertional compartment syndrome of the forearm is an unusual disease not commonly found in the daily practice of a hand surgeon. This condition is quite rare in the general population but occurs more frequently among musicians and athletes, with the highest incidence found in professional motorcycle drivers. It is mainly because of a critical augmentation of the extracellular pressure of the forearm compartments. The diagnosis is mainly clinical, based on stress dynamic tests and intracompartmental pressure measurements. Traditionally, the treatment of this disease has revolved around trigger activity suspension. In the case of professional athletes, this solution cannot be considered and thus the standard surgical treatment consists of an open forearm fasciotomy. This procedure usually requires a lengthy operation period and has a long recovery time before patients can resume their regular activity. Different surgical endoscopic solutions with mini-open techniques have been proposed to shorten this time and reduce the incision size. The aim of this study was to present a new technique for endoscopic-assisted fasciotomy of the forearm in chronic exertional compartment syndrome using a single mini-incision. Four surgical procedures were performed in 3 patients. They were all treated at our center for this condition, and in one case the disease was found on both sides. PMID:24977494

  15. Medical management of failed back surgery syndrome in Europe: evaluation modalities and treatment proposals.

    PubMed

    Durand, G; Girodon, J; Debiais, F

    2015-03-01

    Failed back surgery syndrome (FBSS) is defined as persistent pain more than 3 months after any form of spinal surgery. Due to its multifactorial origin, FBSS is often difficult to treat. In this context of failed back surgery, a very thorough assessment must be conducted concerning the site and characteristics of the pain (nociceptive or neuropathic), its mode of onset (presence or absence of pain-free intervals), and its impact on the patient's work and social life. Physical examination must exclude a non-spinal cause for the pain. MRI is the imaging modality of choice in this disease, but is often difficult to interpret, as MR signals are modified for 6 months after the operation. Scar tissue, which can be distinguished from recurrent disc hernia by its gadolinium enhancement, is present even in asymptomatic patients. After having eliminated infection and sacroiliac or posterior facet joint disease, the main aetiologies investigated are foraminal stenosis, degenerative disc disease, recurrent disc hernia, and non-union of spinal fusion; sometimes patients only experience persistent neuropathic pain. The treatment of failed back surgery syndrome with a predominant neuropathic component is based on the use of analgesics, especially antiepileptics, antidepressants or transcutaneous electrical stimulation. Epidural spinal infiltration should be considered as second-line treatment in view of the risk of serious neurological complications. Management must be based on a global, multidisciplinary approach with identification of any cognitive or behavioural disorders in combination with an appropriate functional rehabilitation programme. PMID:25676909

  16. Restless legs syndrome and pregnancy: prevalence, possible pathophysiological mechanisms and treatment.

    PubMed

    Gupta, R; Dhyani, M; Kendzerska, T; Pandi-Perumal, S R; BaHammam, A S; Srivanitchapoom, P; Pandey, S; Hallett, M

    2016-05-01

    Restless legs syndrome (RLS) is a common sleep disorder that may be associated with pregnancy. Studies have found that the prevalence of RLS among pregnant women ranged from 10 to 34%. Typically, there is complete remission of symptoms soon after parturition; however, in some patients, they may continue postpartum. RLS has been shown to be associated with a number of complications in pregnancy including preeclampsia and increased incidence of Cesarean sections. Although multiple hypotheses have been proposed to explain this association, each individual hypothesis cannot completely explain the whole pathogenesis. Present understanding suggests that a strong family history, low serum iron and ferritin level, and high estrogen level during pregnancy might play important roles. Vitamin D deficiency and calcium metabolism may also play a role. Medical treatment of RLS during pregnancy is difficult and challenging considering the risks to mother and fetus. However, in some cases, the disease may be severe enough to require treatment. PMID:26482928

  17. INCREASED RISK OF OCCURRENCE OF NEUROLEPTIC MALIGNANT SYNDROME ON COMBINED TREATMENT WITH LITHIUM AND NEUROLEPTIC

    PubMed Central

    Alexander, P. John; Thomas, Ranji Mathai

    1997-01-01

    Whether there is an increased risk of occurrence of neuroleptic malignant syndrome (NMS) on combined treatment with lithium and neuroleptic is a controversial issue. Patients seen in a general psychiatry unit of a university hospital in India were prospectively screened for NMS over a 2 year period. Diagnosis of NMS was made on operational criteria and the details of treatment at the time of occurrence of NMS were collected systematically. Eight cases of NMS were identified during the period of the study, out of which 5 (62.5%). were taking lithium and a neuroleptic together at the time of occurrence of NMS. The high prevalence of patients on lithium and neuroleptic concomitantly in our sample of NMS, and the similar findings in many of the earlier prospective studies, makes it possible to speculate whether there is an association between combined use of lithium and neuroleptic and occurrence of NMS. Findings are discussed. PMID:21584083

  18. Errorless compliance training: success-focused behavioral treatment of children with Asperger syndrome.

    PubMed

    Ducharme, Joseph M; Sanjuan, Elena; Drain, Tammy

    2007-05-01

    Errorless compliance training is a noncoercive, success-focused approach to treatment of problem behavior in children. The intervention involves graduated exposure of a child to increasingly more challenging requests at a slow enough rate to ensure that noncompliance rarely occurs, providing parents with many opportunities to reinforce cooperative responses and rendering punishment unnecessary. The authors evaluated this approach with three boys with characteristics of Asperger syndrome. Mothers first delivered a range of requests to their children and recorded child responses. For each child, the authors calculated compliance probability for all requests and categorized them into four probability levels, from those yielding high compliance (Level 1) to those that commonly led to opposition (Level 4). Treatment began with delivery of Level 1 requests. Requests from Levels 2 through 4 were faded in sequentially over several weeks. All three children demonstrated substantial generalized improvement in compliance. PMID:17438346

  19. The Chinese approach to complementary and alternative medicine treatment for interstitial cystitis/bladder pain syndrome.

    PubMed

    Pang, Ran; Ali, Abdullah

    2015-12-01

    Management of interstitial cystitis/bladder pain syndrome (IC/BPS) remains a challenge due to poor understanding on its etiology. Complementary and alternative medicine (CAM), as an optional treatment, has been widely used, because no definitive conventional therapy is available. The different domain of CAM provides miscellaneous treatments for IC/BPS, which mainly include dietary modification, nutraceuticals, bladder training, biofeedback, yoga, massage, physical therapy, Qigong, traditional Chinese medicine and acupuncture. Clinical evidence has shown that each therapy can certainly benefit a portion of IC/BPS patients. However, the target patient group of each therapy has not been well studied and randomized, controlled trials are needed to further confirm the efficacy and reliability of CAM on managing IC/BPS. Despite these limitations, CAM therapeutic characteristics including non-invasive and effectiveness for specific patients allow clinicians and patients to realize multimodal and individualized therapy for IC/BPS. PMID:26816867

  20. The Chinese approach to complementary and alternative medicine treatment for interstitial cystitis/bladder pain syndrome

    PubMed Central

    Ali, Abdullah

    2015-01-01

    Management of interstitial cystitis/bladder pain syndrome (IC/BPS) remains a challenge due to poor understanding on its etiology. Complementary and alternative medicine (CAM), as an optional treatment, has been widely used, because no definitive conventional therapy is available. The different domain of CAM provides miscellaneous treatments for IC/BPS, which mainly include dietary modification, nutraceuticals, bladder training, biofeedback, yoga, massage, physical therapy, Qigong, traditional Chinese medicine and acupuncture. Clinical evidence has shown that each therapy can certainly benefit a portion of IC/BPS patients. However, the target patient group of each therapy has not been well studied and randomized, controlled trials are needed to further confirm the efficacy and reliability of CAM on managing IC/BPS. Despite these limitations, CAM therapeutic characteristics including non-invasive and effectiveness for specific patients allow clinicians and patients to realize multimodal and individualized therapy for IC/BPS. PMID:26816867

  1. [Complex health service for treatment of patients with diabetic foot syndrome].

    PubMed

    Kosul'nikov, S O; Tarnopol'skyĭ, S O; Besedyn, A M; Karpenko, S I

    2014-11-01

    Complex service for treatment for patients, suffering diabetic foot syndrome, was created on the base of Dnipropetrovskiy Regional Clinical Hospital with the objective for application of high technologies. Creation of such a service have permitted to improve the results of the patients treatment. Enhancement of the performance rate of small operations on the foot have permitted to increase by 34.4% the quantity of patients, in whom a supportive function of the foot was preserved, and to reduce the conduction rate of the lower extremity amputation on a hip level, preserving a knee joint, by 39.5%, what is very important for the prosthesis. Widespread propaganda among population and primary link of the health service permit to reveal and eliminate complications of diabetes mellitus on earlier stages. PMID:25675744

  2. A controlled evaluation of a homoeopathic preparation in the treatment of influenza-like syndromes.

    PubMed Central

    Ferley, J P; Zmirou, D; D'Adhemar, D; Balducci, F

    1989-01-01

    1. A controlled clinical trial was conducted to assess the effectiveness of a homoeopathic preparation in the treatment of influenza-like syndromes. 2. 237 cases received the test drug and 241 were assigned to placebo. Patients recorded their rectal temperature twice a day, and the presence or absence of five cardinal symptoms (headache, stiffness, lumbar and articular pain, shivers) along with cough, coryza and fatigue. 3. Recovery was defined as a rectal temperature less than 37.5 degrees C and complete resolution of the five cardinal symptoms. 4. The proportion of cases who recovered within 48 h of treatment was greater among the active drug group than among the placebo group (17.1% against 10.3%, P = 0.03). 5. The result cannot be explained given our present state of knowledge, but it calls for further rigorously designed clinical studies. PMID:2655683

  3. Treatment disparities in acute coronary syndromes, heart failure, and kidney disease.

    PubMed

    McCullough, Peter A; Maynard, Robert C

    2011-01-01

    It has been consistently observed that patients with renal dysfunction have more premature, severe, complicated, and fatal cardiovascular disease than age- and sex-matched individuals with normal renal function. There have been 4 major explanations for this finding: (1) positive confounding by third variables associated with chronic kidney disease (CKD), including diabetes mellitus and hypertension; (2) therapeutic nihilism or lesser use of beneficial therapies in CKD; (3) greater toxicities of therapies, such as bleeding from anticoagulants or contrast-induced kidney injury; (4) biological factors which result directly from CKD that work to promote and accelerate cardiovascular disease. In this paper, we focus on the issue of treatment disparities or therapeutic nihilism and its contribution to poor outcomes in the setting of acute coronary syndromes and acutely decompensated heart failure. This issue is important because if we can overcome barriers to the utilization of beneficial treatments, then clinical outcomes should improve over time. PMID:21625092

  4. Prevention and treatment of the post-thrombotic syndrome and of the chronic thromboembolic pulmonary hypertension.

    PubMed

    Pesavento, Raffaele; Prandoni, Paolo

    2015-02-01

    Post-thrombotic syndrome (PTS) and chronic thromboembolic pulmonary hypertension (CTEPH) are late complications of venous thromboembolism. The purpose of this review is to present and discuss recently published studies that have improved our knowledge of PTS and CTEPH. The current understanding of the pathophysiology of PTS and CTEPH is discussed as well as the importance of chronic residual venous thrombosis, some polymorphisms of plasminogen activator inhibitor-1, and the current concept of misguided thrombus resolution. The surprising finding that elastic compression stockings may not be effective in preventing PTS and the novel medical treatment in CTEPH are discussed in detail. Novel direct oral anticoagulants show potential for prevention of PTS. No firm conclusions can be drawn on the efficacy of elastic stockings. Novel treatments of CTEPH for inoperable patients and those with persistent pulmonary hypertension after surgery have become available and further research on wider indication for their use is urgently needed. PMID:25577951

  5. Rifaximin for the treatment of diarrhea-predominant irritable bowel syndrome.

    PubMed

    Kane, John S; Ford, Alexander C

    2016-04-01

    Irritable bowel syndrome (IBS) is a chronic, functional bowel disorder characterized by abdominal pain or discomfort and altered bowel habit. The pathophysiology is unclear, but may include altered gut motility, visceral hypersensitivity, abnormal central pain processing, chronic low-grade intestinal inflammation, or disturbances in the gut microbiome. These etiological mechanisms, alongside environmental factors such as stress and anxiety, vary between individuals and represent potential targets for treatment. Rifaximin is a poorly absorbed oral antibiotic proposed to act on the gut microenvironment, used in the treatment of travelers' diarrhea and hepatic encephalopathy. Clinical trials suggest the drug can reduce global IBS symptoms and improve bloating, abdominal pain, and stool consistency in some patients with non-constipated IBS, leading to Food and Drug Administration approval in the United States. This article considers the pharmacology of rifaximin, the evidence for its use in IBS, and the safety and tolerability of the drug. PMID:26753693

  6. A new effective method in the treatment of severe ovarian hyperstimulation syndrome

    PubMed Central

    Zhang, Qingrui; Xia, Liangbin; Gao, Gao

    2012-01-01

    Background: Ovarian hyperstimulation syndrome (OHSS) is a recognized complication of ovulation induction, occurring in 1-10% of IVF and embryo transfer cycles. While mild OHSS is of no clinical relevance, severe OHSS is a life threatening complication. However, the efficacy of prevalent treatments appeared to be limited. We developed a continuous autotransfusion system with an ultrafiltration instrument for reinfusion the protein of concentrated ascites for the treatment of severe OHSS. Objective: To study the efficacy and safety of using a continuous autotransfusion system for the treatment of severe OHSS. Materials and Methods: 69 patients with severe OHSS who were treated with controlled ovarian hyperstimulation due to infertility from February 2002 to August 2010 in our reproductive center were divided into two groups. One group treated with continuous autotransfusion system with an ultrafiltration instrument which infused the protein of concentrated ascites, called ultrafiltration group, another group were treated with simple-albumin supplement, called albumin group. Several examinational results and adverse effect were compared between the two groups. Results: The volume of urine output after 72h in ultrafiltration group was more than that in albumin group, the waist circumference and body weight in ultrafiltration group were lower than those in albumin group after 72h. The serum creatinine levels after 72h in ultrafiltration group was still significantly lower than that in albumin group (p<0.05). The ultrafiltration group rarely showed adverse effect compared with albumin group. Conclusion: Autotransfusion of protein in concentrated ascites for the treatment of severe ovarian hyperstimulation syndrome was effective and safe. PMID:25246931

  7. Aspects of the non-pharmacological treatment of irritable bowel syndrome.

    PubMed

    Eriksson, Elsa Maria; Andrén, Kristina Ingrid; Kurlberg, Göran Karl; Eriksson, Henry Ture

    2015-10-28

    Irritable bowel syndrome (IBS) is one of the most commonly diagnosed gastrointestinal conditions. It represents a significant healthcare burden and remains a clinical challenge. Over the years IBS has been described from a variety of different perspectives; from a strict illness of the gastrointestinal tract (medical model) to a more complex multi-symptomatic disorder of the brain-gut axis (biopsychosocial/psychosomatic model). In this article we present aspects of the pathophysiology and the non-pharmacological treatment of IBS based on current knowledge. Effects of conditioned stress and/or traumatic influences on the emotional system (top-down) as well as effects on the intestine through stressors, infection, inflammation, food and dysbiosis (bottom-up) can affect brain-gut communication and result in dysregulation of the autonomic nervous system (ANS), playing an important role in the pathophysiology of IBS. Conditioned stress together with dysregulation of the autonomic nervous system and the emotional system may involve reactions in which the distress inside the body is not recognized due to low body awareness. This may explain why patients have difficulty identifying their symptoms despite dysfunction in muscle tension, movement patterns, and posture and biochemical functions in addition to gastrointestinal symptoms. IBS shares many features with other idiopathic conditions, such as fibromyalgia, chronic fatigue syndrome and somatoform disorders. The key to effective treatment is a thorough examination, including a gastroenterological examination to exclude other diseases along with an assessment of body awareness by a body-mind therapist. The literature suggests that early interdisciplinary diagnostic co-operation between gastroenterologists and body-mind therapists is necessary. Re-establishing balance in the ANS is an important component of IBS treatment. This article discusses the current knowledge of body-mind treatment, addressing the topic from a

  8. Dental Treatment Considerations for Children with Complex Medical Histories: A Case of Townes-Brock Syndrome.

    PubMed

    Elkaiali, Lujayn; Ratliff, Katelin; Oueis, Hassan

    2016-01-01

    It is common for oral health and dental care to be considered a lesser priority for children with complex medical histories than other aspects of their health care. Often, these patients are at a high risk for caries and infection due to poor oral health practices at home, special or restricted diets, and no early establishment of a dental home for routine dental care. Unfortunately, many of these patients present to their first dental visits with caries and require aggressive treatment, such as extractions instead of pulp therapy, or crowns instead of fillings, due to their high caries risk and the difficulty in safely managing them medically during treatment. A unique example of this occurred at the Children's Hospital of Michigan, where a patient with Townes-Brock syndrome (TBS) presented to the dental clinic with advanced caries. TBS is a rare autosomal dominant disorder characterized by major findings such as anomalies of the external ear, imperforate anus, renal malformations, and malformations of the hand. Like many medically complex cases, dental anomalies are not a direct consequence of TBS; however, due to the necessity of high calorie and high sugar feeding supplementation, many of these patients are at high risk for advanced dental caries. Due to this high caries risk, a more aggressive treatment plan is necessary to minimize the risk of recurrent decay and infection. It is critical to stress that even if the disease, syndrome, etc., of a patient does not have inherent dental consequences, it is imperative for regular dental care to be part of the comprehensive treatment plan for these patients. This includes the establishment of a dental home at a young age and proper oral health education of the patient's caregivers and their physicians. In the case of the patient with TBS, recommendations for daily brushing, especially after high sugar feedings was stressed, as well as the reduction of any other sweets within the diet. PMID:26882646

  9. Aspects of the non-pharmacological treatment of irritable bowel syndrome

    PubMed Central

    Eriksson, Elsa Maria; Andrén, Kristina Ingrid; Kurlberg, Göran Karl; Eriksson, Henry Ture

    2015-01-01

    Irritable bowel syndrome (IBS) is one of the most commonly diagnosed gastrointestinal conditions. It represents a significant healthcare burden and remains a clinical challenge. Over the years IBS has been described from a variety of different perspectives; from a strict illness of the gastrointestinal tract (medical model) to a more complex multi-symptomatic disorder of the brain-gut axis (biopsychosocial/psychosomatic model). In this article we present aspects of the pathophysiology and the non-pharmacological treatment of IBS based on current knowledge. Effects of conditioned stress and/or traumatic influences on the emotional system (top-down) as well as effects on the intestine through stressors, infection, inflammation, food and dysbiosis (bottom-up) can affect brain-gut communication and result in dysregulation of the autonomic nervous system (ANS), playing an important role in the pathophysiology of IBS. Conditioned stress together with dysregulation of the autonomic nervous system and the emotional system may involve reactions in which the distress inside the body is not recognized due to low body awareness. This may explain why patients have difficulty identifying their symptoms despite dysfunction in muscle tension, movement patterns, and posture and biochemical functions in addition to gastrointestinal symptoms. IBS shares many features with other idiopathic conditions, such as fibromyalgia, chronic fatigue syndrome and somatoform disorders. The key to effective treatment is a thorough examination, including a gastroenterological examination to exclude other diseases along with an assessment of body awareness by a body-mind therapist. The literature suggests that early interdisciplinary diagnostic co-operation between gastroenterologists and body-mind therapists is necessary. Re-establishing balance in the ANS is an important component of IBS treatment. This article discusses the current knowledge of body-mind treatment, addressing the topic from a

  10. Beneficial Effects of Long-Term Growth Hormone Treatment on Adaptive Functioning in Infants With Prader-Willi Syndrome.

    PubMed

    Lo, Sin T; Festen, Dederieke A M; Tummers-de Lind van Wijngaarden, Roderick F A; Collin, Philippe J L; Hokken-Koelega, Anita C S

    2015-07-01

    The aim of this study was to investigate the effect of growth hormone treatment on adaptive functioning in children with Prader-Willi syndrome. Vineland Adaptive Behavior Scale (VABS) was assessed during a randomized controlled trial (RCT) and after 7 years of growth hormone treatment. In the RCT, 75 children (42 infants and 33 prepubertal children) with Prader-Willi syndrome were included. Subsequently, 53 children were treated with long-term growth hormone. Our study demonstrates a marked delay in adaptive functioning in infants and children with Prader-Willi syndrome, which was associated with older age and lower intelligence. Results of the repeated measurements show that the earlier growth hormone treatment was started during infancy, the better the adaptive skills were on the long-term. PMID:26161469

  11. Treatment results of high dose cabergoline as an adjuvant therapy in six patients with established severe ovarian hyper stimulation syndrome

    PubMed Central

    Saharkhiz, Nasrin; Akbari Sene, Azadeh; Salehpour, Saghar; Tamimi, Maryam; Vasheghani Farahani, Masoumeh; Sheibani, Kourosh

    2014-01-01

    Background: The beneficial role of cabergoline as a prophylactic agent to prevent ovarian hyper stimulation syndrome (OHSS) among high-risk patients has been demonstrated in previous studies. But data for its role as a treatment for established severe OHSS is still limited. We represent the treatment results of high dose oral cabergoline in management of six patients after the syndrome is established. Case: High-dose oral cabergoline (1 mg daily for eight days) was prescribed as an adjuvant to symptomatic treatment for six hospitalized patients with established severe OHSS following infertility treatment cycles. In two cases OHSS resolved rapidly despite the occurrence of ongoing pregnancy. Conclusion: Considering the treatment outcomes of our patients, high dose cabergoline did not eliminate the need for traditional treatments, but it was a relatively effective and safe therapy in management of established severe OHSS, and prevented the increase in its severity following the occurrence of pregnancy. PMID:25469130

  12. Treatment of Tourette syndrome with delta-9-tetrahydrocannabinol (delta 9-THC): no influence on neuropsychological performance.

    PubMed

    Müller-Vahl, Kirsten R; Prevedel, Heidrun; Theloe, Karen; Kolbe, Hans; Emrich, Hinderk M; Schneider, Udo

    2003-02-01

    Previous studies provide evidence that marijuana (Cannabis sativa) and delta-9-tetrahydrocannabinol (Delta(9)-THC), the major psychoactive ingredient of marijuana, respectively, are effective in the treatment of tics and behavioral problems in Tourette syndrome (TS). It, therefore, has been speculated that the central cannabinoid receptor system might be involved in TS pathology. However, in healthy marijuana users there is an ongoing debate as to whether the use of cannabis causes acute and/or long-term cognitive deficits. In this randomized double-blind placebo-controlled study, we investigated the effect of a treatment with up to 10 mg Delta(9)-THC over a 6-week period on neuropsychological performance in 24 patients suffering from TS. During medication and immediately as well as 5-6 weeks after withdrawal of Delta(9)-THC treatment, no detrimental effect was seen on learning curve, interference, recall and recognition of word lists, immediate visual memory span, and divided attention. Measuring immediate verbal memory span, we even found a trend towards a significant improvement during and after treatment. Results from this study corroborate previous data suggesting that in patients suffering from TS, treatment with Delta(9)-THC causes neither acute nor long-term cognitive deficits. Larger and longer-duration controlled studies are recommended to provide more information on the adverse effect profile of THC in patients suffering from TS. PMID:12589392

  13. The Responsive Amygdala: Treatment-induced Alterations in Functional Connectivity in Pediatric Complex Regional Pain Syndrome

    PubMed Central

    Simons, LE; Pielech, M; Erpelding, N; Linnman, C; Moulton, E; Sava, S; Lebel, A; Serrano, P; Sethna, N; Berde, C; Becerra, L; Borsook, D

    2014-01-01

    The amygdala is a key brain region with efferent and afferent neural connections that involve complex behaviors such as pain, reward, fear and anxiety. This study evaluated resting state functional connectivity of the amygdala with cortical and subcortical regions in a group of chronic pain patients (pediatric complex regional pain syndrome) with age-gender matched controls before and after intensive physical-biobehavioral pain treatment. Our main findings include (1) enhanced functional connectivity from the amygdala to multiple cortical, subcortical, and cerebellar regions in patients compared to controls, with differences predominantly in the left amygdala in the pre-treated condition (disease state); (2) dampened hyperconnectivity from the left amygdala to the motor cortex, parietal lobe, and cingulate cortex after intensive pain rehabilitation treatment within patients with nominal differences observed among healthy controls from Time 1 to Time 2 (treatment effects); (3) functional connectivity to several regions key to fear circuitry (prefrontal cortex, bilateral middle temporal lobe, bilateral cingulate, hippocampus) correlated with higher pain-related fear scores and (4) decreases in pain-related fear associated with decreased connectivity between the amygdala and the motor and somatosensory cortex, cingulate, and frontal areas. Our data suggest that there are rapid changes in amygdala connectivity following an aggressive treatment program in children with chronic pain and intrinsic amygdala functional connectivity activity serving as a potential indicator of treatment response. PMID:24861582

  14. Discontinuation of eculizumab maintenance treatment for atypical hemolytic uremic syndrome: a report of 10 cases.

    PubMed

    Ardissino, Gianluigi; Testa, Sara; Possenti, Ilaria; Tel, Francesca; Paglialonga, Fabio; Salardi, Stefania; Tedeschi, Silvana; Belingheri, Mirco; Cugno, Massimo

    2014-10-01

    Atypical hemolytic uremic syndrome (aHUS) is a life-threatening thrombotic microangiopathy, and as many as 70% of patients with aHUS have mutations in the genes encoding complement regulatory proteins. Eculizumab, a humanized recombinant monoclonal antibody targeting C5, has been used successfully in patients with aHUS since 2009. The standard maintenance treatment requires life-long eculizumab therapy, but the possibility of discontinuation has not yet been tested systematically. We report the safety of discontinuing eculizumab treatment in 10 patients who stopped treatment with the aim of minimizing the risk of adverse reactions, reducing the risk of meningitis, and improving quality of life while also reducing the considerable treatment costs. Disease activity was monitored closely at home by means of urine dipstick testing for hemoglobin. During the cumulative observation period of 95 months, 3 of the 10 patients experienced relapse within 6 weeks of discontinuation, but then immediately resumed treatment and completely recovered. Our experience supports the possibility of discontinuing eculizumab therapy with strict home monitoring for early signs of relapse in patients with aHUS who achieve stable remission. PMID:24656451

  15. Generalized annular granuloma associated with crowned dens syndrome, which resolved with colchicine treatment.

    PubMed

    Cozzani, E; Basso, D; Cimmino, M A; Larosa, M; Burlando, M; Rongioletti, F; Drago, F; Parodi, A

    2016-08-01

    Granuloma annulare (GA) is a chronic, benign, and usually self-limiting cutaneous inflammatory disease, typically characterized by small, localized, skin-coloured papules that are usually asymptomatic or mildly pruriginous. Its aetiopathogenesis is still unknown and treatments are rarely effective. Generally, 50-70% of localized GA cases are self-limiting and show spontaneous resolution after 1-2 years, whereas disseminated GA is less likely to disappear without treatment. Treatment of generalized GA is usually based on single case reports, and only a few studies involving large case series have been published. We present the case of a patient affected by generalized GA, which resolved after colchicine treatment used for concomitant crowned dens syndrome due to calcium pyrophosphate deposition disease (CPPD). Colchicine may have worked by a direct action on GA or, alternatively, by controlling CPPD, as a possible trigger. As the low-dosage colchicine treatment was well tolerated by our patient, this could be easily used in the management of GA. However, further studies are needed to confirm the action of colchicine on GA. PMID:27335228

  16. Does Pramipexole Treatment Improve Headache in Patients with Concomitant Migraine and Restless Legs Syndrome?

    PubMed Central

    Suzuki, Keisuke; Suzuki, Shiho; Miyamoto, Masayuki; Miyamoto, Tomoyuki; Numao, Ayaka; Watanabe, Yuka; Takashima, Ryotaro; Hirata, Koichi

    2013-01-01

    Background Recent studies have suggested a strong link between migraines and restless legs syndrome (RLS). It is possible that these disorders share a dopaminergic dysfunction in the hypothalamic A11 nucleus that contributes to this association. However, there have been no clinical studies to evaluate the effect of dopaminergic treatment on migraine symptoms in patients with concomitant migraines and RLS. Methods We present an illustrative patient with concomitant RLS and migraine who showed improvement in her headache frequency and RLS symptoms following immediate-release pramipexole (P-IR) treatment and provide review results from the medical records of patients who experienced both migraines and RLS in our previous cross-sectional study. Results Ten patients (nine patients from the previously completed single-center study) received P-IR treatment were included in the study. RLS symptoms improved markedly in all of the subjects. Five out of the 10 patients (50%) reported improvement in migraine headaches. Of these five patients, four (80%) had reported morning headaches before P-IR treatment. Discussion Our results indicate that the identification of RLS in migraine patients is clinically significant and that dopaminergic treatment may improve both migraines, particularly morning headache (80% improvement in this study), and RLS symptoms. However, further clinical studies are warranted to verify our results. PMID:24116342

  17. Endovascular Treatment of Malignant Superior Vena Cava Syndrome: Results and Predictive Factors of Clinical Efficacy

    SciTech Connect

    Fagedet, Dorothee; Thony, Frederic; Timsit, Jean-Francois; Rodiere, Mathieu; Monnin-Bares, Valerie; Ferretti, Gilbert R.; Vesin, Aurelien; Moro-Sibilot, Denis

    2013-02-15

    To demonstrate the effectiveness of endovascular treatment (EVT) with self-expandable bare stents for malignant superior vena cava syndrome (SVCS) and to analyze predictive factors of EVT efficacy. Retrospective review of the 164 patients with malignant SVCS treated with EVT in our hospital from August 1992 to December 2007 and followed until February 2009. Endovascular treatment includes angioplasty before and after stent placement. We used self-expandable bare stents. We studied results of this treatment and looked for predictive factors of clinical efficacy, recurrence, and complications by statistical analysis. Endovascular treatment was clinically successful in 95% of cases, with an acceptable rate of early mortality (2.4%). Thrombosis of the superior vena cava was the only independent factor for EVT failure. The use of stents over 16 mm in diameter was a predictive factor for complications (P = 0.008). Twenty-one complications (12.8%) occurred during the follow-up period. Relapse occurred in 36 patients (21.9%), with effective restenting in 75% of cases. Recurrence of SVCS was significantly increased in cases of occlusion (P = 0.01), initial associated thrombosis (P = 0.006), or use of steel stents (P = 0.004). Long-term anticoagulant therapy did not influence the risk of recurrence or complications. In malignancy, EVT with self-expandable bare stents is an effective SVCS therapy. These results prompt us to propose treatment with stents earlier in the clinical course of patients with SVCS and to avoid dilatation greater than 16 mm.

  18. Therapeutic effect of pulsed electromagnetic field in conservative treatment of subacromial impingement syndrome.

    PubMed

    Aktas, Ilknur; Akgun, Kenan; Cakmak, Bahar

    2007-08-01

    Subacromial impingement syndrome (SIS) is a frequent cause of shoulder pain. Our purpose in this double-blinded, randomized, and controlled study was to demonstrate whether the pulsed electromagnetic field (PEMF) provides additional benefit when used with other conservative treatment modalities in acute phase rehabilitation program of SIS. Forty-six patients with unilateral shoulder pain who had been diagnosed as having SIS were included in this trial. The cases were randomly separated into two groups. All cases received a treatment program for 3 weeks consisting of Codman's pendulum exercises and subsequent cold pack gel application on shoulders with pain 5 times a day, restriction of daily activities that require the hands to be used over the head, and meloxicam tablet 15 mg daily. One group was given PEMF; the other group was given sham PEMF daily, 25 min per session, 5 days per week for 3 weeks. Shoulder pain during rest and activity and which causes disturbance of sleep was evaluated using a visual analogue scale, and total Constant score investigated shoulder function. Daily living activities were evaluated by shoulder disability questionnaire. Results were assessed before and after treatment. When compared with the baseline values, significant improvements in all these variables were observed at the end of the treatment in both groups (p<0.05). No significant difference between treatments was observed for any of these variables (p>0.05). There is no convincing evidence that electromagnetic therapy is of additional benefit in acute phase rehabilitation program of SIS. PMID:17086382

  19. The responsive amygdala: treatment-induced alterations in functional connectivity in pediatric complex regional pain syndrome.

    PubMed

    Simons, L E; Pielech, M; Erpelding, N; Linnman, C; Moulton, E; Sava, S; Lebel, A; Serrano, P; Sethna, N; Berde, C; Becerra, L; Borsook, D

    2014-09-01

    The amygdala is a key brain region with efferent and afferent neural connections that involve complex behaviors such as pain, reward, fear, and anxiety. This study evaluated resting state functional connectivity of the amygdala with cortical and subcortical regions in a group of chronic pain patients (pediatric complex regional pain syndrome) with age-sex matched control subjects before and after intensive physical-biobehavioral pain treatment. Our main findings include (1) enhanced functional connectivity from the amygdala to multiple cortical, subcortical, and cerebellar regions in patients compared with control subjects, with differences predominantly in the left amygdala in the pretreated condition (disease state); (2) dampened hyperconnectivity from the left amygdala to the motor cortex, parietal lobe, and cingulate cortex after intensive pain rehabilitation treatment within patients with nominal differences observed among healthy control subjects from time 1 to time 2 (treatment effects); (3) functional connectivity to several regions key to fear circuitry (prefrontal cortex, bilateral middle temporal lobe, bilateral cingulate, hippocampus) correlated with higher pain-related fear scores; and (4) decreases in pain-related fear associated with decreased connectivity between the amygdala and the motor and somatosensory cortex, cingulate, and frontal areas. Our data suggest that there are rapid changes in amygdala connectivity after an aggressive treatment program in children with chronic pain and intrinsic amygdala functional connectivity activity serving as a potential indicator of treatment response. PMID:24861582

  20. Pharmacogenomic considerations in the treatment of the pediatric cardiomyopathy called Barth syndrome.

    PubMed

    Malhotra, Ashim; Kahlon, Parmbir; Donoho, Timothy; Doyle, Ian C

    2014-01-01

    Barth syndrome (BTHS) is a genetic, X-linked, rare but often fatal, pediatric skeletal- and cardiomyopathy occurring due to mutations in the tafazzin gene (TAZ). TAZ encodes a transacylase involved in phospholipid biosynthesis, also called tafazzin, which is responsible for remodeling the inner mitochondrial membrane phospholipid, cardiolipin (CL). Tafazzin mutations lead to compositional alterations in CL molecular species, causing extensive mitochondrial aberrations and ultrastructural muscle damage. There are no specific treatments or cure for BTHS. Current therapy is largely palliative and aimed at treatment of organ-specific complications during disease progression. Polypharmacy frequently occurs during treatment and may lead to severe adverse events. Adverse reactions may originate from exogenous factors such as the inadvertent co-administration of contraindicated drugs. Theoretically, endogenous factors such as polymorphic variations in genes encoding drug metabolizing enzymes may also precipitate fatal toxicity. Investigation of the consequences of pharmacogenomic variations on BTHS therapy is lacking. To our knowledge, this review presents the first examination of the possible sources of pharmacogenomic variations that may affect BTHS therapy. We also explore BTHSspecific patents for possible treatment options. The patents discussed suggest innovative strategies for treatment, including feeding linoleic acid to patients to overcome compositional CL deficiency; or the use of 2S,4R ketoconazole formulations to augment CL levels; or the delivery of mitochondrial stabilizing cargo. Future research directions are also discussed. PMID:25185984

  1. Efficacy of high frequency ultrasound in postoperative evaluation of carpal tunnel syndrome treatment

    PubMed Central

    Urbanik, Andrzej

    2016-01-01

    Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy and a frequent cause of sick leave because of work-related hand overload. The main treatment is operation. Aim The aim of the study is to assess the usefulness of high frequency ultrasound in the postoperative evaluation of CTS treatment efficacy. Material and methods Sixty-two patients (50 women and 12 men aged 28–70, mean age 55.2) underwent surgical treatment of CTS. Ultrasound examinations of the wrist in all carpal tunnel sufferers were performed 3 months after the procedure with the use of a high frequency broadband linear array transducer (6–18 MHz, using 18 MHz band) of MyLab 70/Esaote. On the basis of the collected data, the author has performed multiple analyses to confirm the usefulness of ultrasound imaging for postoperative evaluation of CTS treatment efficacy. Results Among all 62 patients, 3 months after surgical median nerve decompression: in 40 patients, CTS symptoms subsided completely, and sonographic evaluation did not show median nerve entrapment signs; in 9 patients, CTS symptoms persisted or exacerbated, and ultrasound proved nerve compression revealing preserved flexor retinaculum fibers; in 13 patients, scar tissue symptoms occurred, and in 5 of them CTS did not subside completely (although ultrasound showed no signs of compression). Conclusions Ultrasound imaging with the use of a high frequency transducer is a valuable diagnostic tool for postoperative assessment of CTS treatment efficacy. PMID:27103999

  2. Treatment of pyoderma gangrenosum with thalidomide in a myelodysplastic syndrome case

    PubMed Central

    Malkan, Umit Yavuz; Gunes, Gursel; Eliacik, Eylem; Haznedaroglu, Ibrahim Celalettin

    2016-01-01

    Thalidomide may be used as a treatment option for pyoderma gangrenosum (PG) and myelodysplastic syndrome (MDS). Herein, we aimed to report a patient who was treated well with thalidomide and whose diagnosis was PG with MDS. A 61-year-old man with painless ecchymotic lesions in his right upper extremity was admitted to the hospital in Isparta, Turkey, in January 2015. The lesions were diagnosed as PG. In his anamnesis, it was found that he was diagnosed with MDS 6 years ago and had been treated with cyclosporine at 2×100 mg for 5 years, which was stopped in January 2015. Aspiration from liver lesion revealed the presence of Mycobacterium tuberculosis, so antituberculosis treatment was started. Bone marrow investigation revealed MDS-refractory anemia with excess blasts (7%). For lesions in bilateral upper extremities, thalidomide treatment was started at 50 mg/d. After 1 month from the initiation of thalidomide treatment, the lesions in upper extremities had disappeared. In the literature, there are some reports of patients with PG who were successfully treated with thalidomide. Our patient is a complicated case who simultaneously has MDS, PG, and tuberculosis infection. The reason for thalidomide usage in our patient was the need of immune modulation without immune suppression. Our patient has tolerated the drug well, and excellent response was obtained after 1 month of initiation of thalidomide treatment. To conclude, thalidomide is a very effective drug acting as an immune modulator, which is useful in the clinical management of both MDS and PG. PMID:27051318

  3. Treatment of pyoderma gangrenosum with thalidomide in a myelodysplastic syndrome case.

    PubMed

    Malkan, Umit Yavuz; Gunes, Gursel; Eliacik, Eylem; Haznedaroglu, Ibrahim Celalettin

    2016-01-01

    Thalidomide may be used as a treatment option for pyoderma gangrenosum (PG) and myelodysplastic syndrome (MDS). Herein, we aimed to report a patient who was treated well with thalidomide and whose diagnosis was PG with MDS. A 61-year-old man with painless ecchymotic lesions in his right upper extremity was admitted to the hospital in Isparta, Turkey, in January 2015. The lesions were diagnosed as PG. In his anamnesis, it was found that he was diagnosed with MDS 6 years ago and had been treated with cyclosporine at 2×100 mg for 5 years, which was stopped in January 2015. Aspiration from liver lesion revealed the presence of Mycobacterium tuberculosis, so antituberculosis treatment was started. Bone marrow investigation revealed MDS-refractory anemia with excess blasts (7%). For lesions in bilateral upper extremities, thalidomide treatment was started at 50 mg/d. After 1 month from the initiation of thalidomide treatment, the lesions in upper extremities had disappeared. In the literature, there are some reports of patients with PG who were successfully treated with thalidomide. Our patient is a complicated case who simultaneously has MDS, PG, and tuberculosis infection. The reason for thalidomide usage in our patient was the need of immune modulation without immune suppression. Our patient has tolerated the drug well, and excellent response was obtained after 1 month of initiation of thalidomide treatment. To conclude, thalidomide is a very effective drug acting as an immune modulator, which is useful in the clinical management of both MDS and PG. PMID:27051318

  4. Oxidative Inactivation of Liver Mitochondria in High Fructose Diet-Induced Metabolic Syndrome in Rats: Effect of Glycyrrhizin Treatment.

    PubMed

    Sil, Rajarshi; Chakraborti, Abhay Sankar

    2016-09-01

    Metabolic syndrome is a serious health problem in the present world. Glycyrrhizin, a triterpenoid saponin of licorice (Glycyrrhiza glabra) root, has been reported to ameliorate the primary complications and hepatocellular damage in rats with the syndrome. In this study, we have explored metabolic syndrome-induced changes in liver mitochondrial function and effect of glycyrrhizin against the changes. Metabolic syndrome was induced in rats by high fructose (60%) diet for 6 weeks. The rats were then treated with glycyrrhizin (50 mg/kg body weight) by single intra-peritoneal injection. After 2 weeks of the treatment, the rats were sacrificed to collect liver tissue. Elevated mitochondrial ROS, lipid peroxidation and protein carbonyl, and decreased reduced glutathione content indicated oxidative stress in metabolic syndrome. Loss of mitochondrial inner membrane cardiolipin was observed. Mitochondrial complex I activity did not change but complex IV activity decreased significantly. Mitochondrial MTT reduction ability, membrane potential, phosphate utilisation and oxygen consumption decreased in metabolic syndrome. Reduced mitochondrial aconitase activity and increased aconitase carbonyl content suggested oxidative damage of the enzyme. Elevated Fe(2+) ion level in mitochondria might be associated with increased ROS generation in metabolic syndrome. Glycyrrhizin effectively attenuated mitochondrial oxidative stress and aconitase degradation, and improved electron transport chain activity. Copyright © 2016 John Wiley & Sons, Ltd. PMID:27255442

  5. Practical Aspects of Botulinum Toxin-A Treatment in Patients With Overactive Bladder Syndrome.

    PubMed

    Liao, Chun-Hou; Kuo, Hann-Chorng

    2015-12-01

    Intravesical onabotulinumtoxinA (BoNT-A) injection is an effective treatment for overactive bladder syndrome (OAB) that is refractory to antimuscarinics. An injectable dose of 100 U has been suggested to achieve the optimal balance of benefit and safety in patients with OAB. BoNT-A (total volume of 10 mL) was administered as evenly distributed intradetrusor injections (5 U) across 20 sites approximately 1 cm apart (0.5 mL per site) using a flexible or rigid cystoscope. Treatment with BoNT-A was generally well tolerated by most patients, and most treatment-related adverse events were localized to the urinary tract. The prevalence of OAB increases with age, and elderly patients are more vulnerable to complications. The short-term efficacy of intravesical BoNT-A injection for refractory OAB with no treatment-related complications in the elderly population has been documented. Frail elderly patients can experience the same treatment results, such as significantly improved urgent urinary incontinence and quality of life, as young and nonfrail elderly patients with 100-U BoNT-A injections. However, increased risk of larger postvoid residual (PVR) urine and lower long-term success rates were noted in frail elderly patients; around 11% had acute urinary retention, while 60% had PVR urine volume >150 mL after treatment. In addition, intravesical injection of BoNT-A effectively decreased urgency symptoms in elderly patients with OAB and central nervous system lesions. The adverse effects were acceptable, while the long-term effects were comparable to those in patients with OAB without central nervous system lesions. Nonetheless, the possibility of longstanding urinary retention and chronic catheterization in this vulnerable population requires careful evaluation before treatment with intravesical BoNT-A. In conclusion, the current findings indicate that intravesical BoNT-A is an effective and safe treatment for OAB in elderly patients. PMID:26739175

  6. Practical Aspects of Botulinum Toxin-A Treatment in Patients With Overactive Bladder Syndrome

    PubMed Central

    2015-01-01

    Intravesical onabotulinumtoxinA (BoNT-A) injection is an effective treatment for overactive bladder syndrome (OAB) that is refractory to antimuscarinics. An injectable dose of 100 U has been suggested to achieve the optimal balance of benefit and safety in patients with OAB. BoNT-A (total volume of 10 mL) was administered as evenly distributed intradetrusor injections (5 U) across 20 sites approximately 1 cm apart (0.5 mL per site) using a flexible or rigid cystoscope. Treatment with BoNT-A was generally well tolerated by most patients, and most treatment-related adverse events were localized to the urinary tract. The prevalence of OAB increases with age, and elderly patients are more vulnerable to complications. The short-term efficacy of intravesical BoNT-A injection for refractory OAB with no treatment-related complications in the elderly population has been documented. Frail elderly patients can experience the same treatment results, such as significantly improved urgent urinary incontinence and quality of life, as young and nonfrail elderly patients with 100-U BoNT-A injections. However, increased risk of larger postvoid residual (PVR) urine and lower long-term success rates were noted in frail elderly patients; around 11% had acute urinary retention, while 60% had PVR urine volume >150 mL after treatment. In addition, intravesical injection of BoNT-A effectively decreased urgency symptoms in elderly patients with OAB and central nervous system lesions. The adverse effects were acceptable, while the long-term effects were comparable to those in patients with OAB without central nervous system lesions. Nonetheless, the possibility of longstanding urinary retention and chronic catheterization in this vulnerable population requires careful evaluation before treatment with intravesical BoNT-A. In conclusion, the current findings indicate that intravesical BoNT-A is an effective and safe treatment for OAB in elderly patients. PMID:26739175

  7. Treatment of neuroblastoma in congenital central hypoventilation syndrome with a PHOX2B polyalanine repeat expansion mutation: New twist on a neurocristopathy syndrome.

    PubMed

    Armstrong, Amy E; Weese-Mayer, Debra E; Mian, Amir; Maris, John M; Batra, Vandana; Gosiengfiao, Yasmin; Reichek, Jennifer; Madonna, Mary Beth; Bush, Jonathan W; Shore, Richard M; Walterhouse, David O

    2015-11-01

    Neuroblastoma in patients with congenital central hypoventilation syndrome (CCHS) as part of a neurocristopathy syndrome is a rare finding and has only been associated with paired-like homeobox 2b (PHOX2B) non-polyalanine-repeat-expansion mutations. To the best of our knowledge, we report the first case of a child with CCHS and Hirschsprung disease who had a PHOX2B polyalanine-repeat-expansion mutation (PARM) (genotype 20/33) and developed high-risk neuroblastoma. We further describe his treatment including chemotherapy and therapeutic I(131) -metaiodobenzylguanidine. This case highlights the need to consider neuroblastoma in patients with CCHS and the longest PHOX2B PARMs and to individualize treatment based on co-morbidities. PMID:26011159

  8. Sanfilippo syndrome

    MedlinePlus

    ... for families who have a child with Sanfilippo syndrome, to help them understand the condition and possible treatments. Prenatal testing is available. Alternative Names MPS III References Pyeritz ...

  9. Simultaneous Open Surgical Treatment of Aortic Coral Reef and Leriche Syndrome: Case Report and Literature Review.

    PubMed

    Pranteda, Chiara; Menna, Danilo; Capoccia, Laura; Sirignano, Pasqualino; Mansour, Wassim; Speziale, Francesco

    2016-04-01

    The coral reef aorta (CRA) is a rare syndrome commonly referred to a distribution of calcified plaques in the visceral part of the aorta. Because those plaques can cause malperfusion of the lower limbs, visceral ischemia or renovascular hypertension, surgical treatment is recommended. Transaortic endarterectomy is accepted as a standard repair and it is often performed through an extensive thoracoabdominal approach. CRA has been reported in association with polidistrectual atherosclerotic disease, such as Leriche syndrome. When these 2 conditions coexist, surgical invasivity increases raising several issues concerning the type of surgical access and the revascularization techniques. We report the case of a patient with CRA and Leriche syndrome treated by simultaneous aortic endarterectomy and aortibifemoral bypass at our institution. Intervention was performed through left lumbotomy at 10th intercostal space extended by a left pararectal abdominal incision with section of 11th rib. Through extraperitoneal access visceral vessels were isolated. Aortic cross-clamping was performed at supraceliac and infrarenal levels and a longitudinal arteriotomy was performed on the posterolateral wall of visceral aorta for an overall 4-cm extension. Aortic endarterectomy was then performed and complete plaque excision was easily achieved. Superior mesenteric artery angioplasty was then performed by a DeBakey dilator, gaining an optimal backflow. The aortotomy was then closed with running 3-0 polypropylene suture. Subsequently, through a transperitoneal access an aortobi-femoral bypass was performed by a Dacron knitted graft. Postoperative course was uneventful. At a 6-month follow-up, the patient is in good clinical condition with normal patency of visceral vessels. PMID:26806247

  10. Recombinant human leptin treatment in genetic lipodystrophic syndromes: the long-term Spanish experience.

    PubMed

    Araujo-Vilar, David; Sánchez-Iglesias, Sofía; Guillín-Amarelle, Cristina; Castro, Ana; Lage, Mary; Pazos, Marcos; Rial, José Manuel; Blasco, Javier; Guillén-Navarro, Encarna; Domingo-Jiménez, Rosario; del Campo, María Ruiz; González-Méndez, Blanca; Casanueva, Felipe F

    2015-05-01

    Lipodystrophies are a group of diseases mainly characterized by a loss of adipose tissue and frequently associated with insulin resistance, hypertriglyceridemia, and hepatic steatosis. In uncommon lipodystrophies, these complications frequently are difficult to control with conventional therapeutic approaches. This retrospective study addressed the effectiveness of recombinant methionyl leptin (metreleptin) for improving glucose metabolism, lipid profile, and hepatic steatosis in patients with genetic lipodystrophic syndromes. We studied nine patients (five females and four males) with genetic lipodystrophies [seven with Berardinelli-Seip syndrome, one with atypical progeroid syndrome, and one with type 2 familial partial lipodystrophy (FPLD)]. Six patients were children under age 9 years, and all patients had baseline triglycerides levels >2.26 mmol/L and hepatic steatosis; six had poorly controlled diabetes mellitus. Metreleptin was self-administered subcutaneously daily at a final dose that ranged between 0.05 and 0.24 mg/(kg day) [median: 0.08 mg/(kg day)] according to the body weight. The duration of treatment ranged from 9 months to 5 years, 9 months (median: 3 years). Plasma glucose, hemoglobin A1c (Hb A1c), lipid profile, plasma insulin and leptin, and hepatic enzymes were evaluated at baseline and at least every 6 months. Except for the patient with FPLD, metreleptin replacement significantly improved metabolic control (Hb A1c: from 10.4 to 7.1 %, p < 0.05). Plasma triglycerides were reduced 76 % on average, and hepatic enzymes decreased more than 65 %. This study extends knowledge about metreleptin replacement in genetic lipodystrophies, bearing out its effectiveness for long periods of time. PMID:25367549

  11. Nephrotic syndrome in dogs: clinical features and evidence-based treatment considerations.

    PubMed

    Klosterman, Emily S; Pressler, Barrak M

    2011-08-01

    Nephrotic syndrome (NS), defined as the concurrent presence of hypoalbuminemia, proteinuria, hyperlipidemia, and fluid accumulation in interstitial spaces and/or body cavities, is a rare complication of glomerular disease in dogs, cats, and people. Affected animals frequently have markedly abnormal urine protein:creatinine ratios because of urinary loss of large amounts of protein; however, hypoalbuminemia-associated decreased plasma oncotic pressure is insufficient to explain fluid extravasation in most laboratory models, and, instead, either aberrant renal tubule retention of sodium with resultant increase in hydrostatic pressure or a systemic increase in vascular permeability may be the primary defects responsible for development of NS. Factors associated with NS in people (including "nephrotic-range" serum albumin concentration and urine protein concentration, and particular glomerular disease subtypes) have been assumed previously to also be important in dogs, although descriptions were limited to those patients included in case series of glomerular disease, and sporadic case reports. However, case-control comparison of larger cohorts of dogs with nephrotic versus nonnephrotic glomerular disease more recently suggests that predisposing factors and concurrent clinicopathologic abnormalities differ from those typically encountered in people with nephrotic syndrome, although case progression and negative effect on patient outcome are similar. This article briefly reviews major current theories and supporting evidence on the pathogenesis of NS, followed by an overview on the clinical features of this syndrome in dogs with glomerular disease. The authors also offer evidence-based and experience-based treatment recommendations that are based on minimizing the suspected dysregulation of the renin-angiotensin-aldosterone axis in affected dogs. PMID:21782144

  12. Pharmaceutical Approach for the Diagnosis and Treatment of Malignant Syndrome: A Case Study.

    PubMed

    Kameda, Keisuke; Watarai, Kazuhiro; Takahashi, Kenji; Shoko, Tomohisa; Koinuma, Masayoshi; Ikushima, Goro

    2016-01-01

    Since 2012, Matsudo City Hospital has increased the number of pharmacists stationed in the ward on weekday mornings at the emergency care center, the intensive care unit (ICU) and the high care unit (HCU). Multidisciplinary joint meetings and joint conferences are conducted in the emergency care center, and patient and drug information is shared. A 20-year-old man was transferred to our hospital after a traffic accident. He was diagnosed with subarachnoid hemorrhage and brain contusion. He exhibited violent movement and intense restlessness. He was sedated with a continuous intravenous infusion of 5 mg/h midazolam and 20 μg/h fentanyl, with intubation. Propofol was also used intermittently. The midazolam infusion was concluded on day 5 of hospitalization. However, his restlessness recurred so an intravenous drip infusion of 150 mg/h haloperidol was administered. On the 7th day, he developed a high-grade fever, muscle rigidity, perspiration, and leukocytosis, and malignant syndrome or malignant hyperthermia was suspected. For malignant syndrome treatment, he received an intravenous drip infusion of 60 mg dantrolene, followed by the combined oral administration of 100 mg/d dantrolene and 7.5 mg/d bromocriptine. Considering various pharmacological effects, we selected an intravenous drip infusion of 25 mg hydroxyzine hydrochloride as the drug to alleviate restlessness. The patient's course continued without recurrence of malignant syndrome; his symptoms improved because of pharmaceutical care with an awareness of patient benefits through clinical and laboratory findings, consultation with the attending physician, presentation of information on causative and therapeutic drugs, and coordinated planning of a prescription design. PMID:27252070

  13. Medical treatment for an adult patient with eisenmenger syndrome. A case report.

    PubMed

    Yao, Atsushi

    2015-01-01

    Previous studies examining the use of pulmonary arterial hypertension (PAH) drugs in patients with Eisenmenger syndrome (ES) have shown that it may have beneficial effects in some patients with ES; however, experience with additional cases is necessary to confirm its efficacy and appropriate clinical use. We herein report our experience of an adult patient with ES who benefitted from treatment with PAH drugs. A 32-year-old Japanese man with severe ES induced by a ventricular septal defect associated with Down syndrome began treatment with bosentan at 62.5 mg. Eleven months later, he was admitted for tadalafil (40 mg) add-on therapy because his 6-minute walking distance and brain natriuretic peptide (BNP) level had not improved and his hepatic enzyme levels had increased. However, marked hypotension developed, and the tadalafil dose was decreased. His BNP level subsequently increased, so the bosentan dose was increased to 125 mg. The bosentan was then abruptly stopped because of a low platelet count and high liver enzyme levels. Ambrisentan was then administered for these side effects, but because severe dyspnea developed, the bosentan was started again at 62.5 mg. This resulted in immediate clinical improvement. The patient was finally switched to ambrisentan (5 mg), which was well tolerated. The findings in this particular case show that although it should be used with caution, bosentan may be beneficial in select patients with ES. In addition, ambrisentan may be considered as first-line treatment in some patients as long as liver enzymes and platelets are carefully monitored. PMID:25787798

  14. Effect of extracorporeal shock wave therapy on the treatment of patients with carpal tunnel syndrome

    PubMed Central

    Vahdatpour, Babak; Kiyani, Abolghasem; Dehghan, Farnaz

    2016-01-01

    Background: The carpal tunnel syndrome (CTS) is the most common neuropathy. The aim of this study was to evaluate the effect of a new and noninvasive treatment including extracorporeal shock wave therapy (ESWT) in the treatment of CTS. Materials and Methods: This study is a clinical trial conducted on 60 patients with moderate CTS in selected health centers of Isfahan Medical University from November 2014 to April 2015. Patients with CTS were randomly divided into two groups. Conservative treatment including wrist splint at night for 3 months, consumption of nonsteroidal anti-inflammatory drugs for 2 weeks, and oral consumption of Vitamin B1 for a month was recommended for both groups. The first group was treated with ESWT, one session per week for 4 weeks. Focus probe with 0.05, 0.07, 0.1, and 0.15 energy and shock numbers 800, 900, 1000, and 1100 were used from the first session to the fourth, respectively. The evaluated parameters were assessed before treatment and after 3 and 6 months. Data were analyzed using SPSS version 19, Student’s t-test, and Chi-square test. Results: All parameters were significantly decreased in the ESWT group after 3 months. These results remained almost constant after 6 months compared with 3 months after treatment. However, only two parameters considerably improved after 3 months of treatment in the control group. The entire indexes in the control group implicated the regression of results in long-term period. Conclusion: It is recommended to use ESWT as a conservative treatment in patients with CTS. PMID:27563630

  15. Treatments for shoulder impingement syndrome: a PRISMA systematic review and network meta-analysis.

    PubMed

    Dong, Wei; Goost, Hans; Lin, Xiang-Bo; Burger, Christof; Paul, Christian; Wang, Zeng-Li; Zhang, Tian-Yi; Jiang, Zhi-Chao; Welle, Kristian; Kabir, Koroush

    2015-03-01

    Many treatments for shoulder impingement syndrome (SIS) are available in clinical practice; some of which have already been compared with other treatments by various investigators. However, a comprehensive treatment comparison is lacking. Several widely used electronic databases were searched for eligible studies. The outcome measurements were the pain score and the Constant-Murley score (CMS). Direct comparisons were performed using the conventional pair-wise meta-analysis method, while a network meta-analysis based on the Bayesian model was used to calculate the results of all potentially possible comparisons and rank probabilities. Included in the meta-analysis procedure were 33 randomized controlled trials involving 2300 patients. Good agreement was demonstrated between the results of the pair-wise meta-analyses and the network meta-analyses. Regarding nonoperative treatments, with respect to the pain score, combined treatments composed of exercise and other therapies tended to yield better effects than single-intervention therapies. Localized drug injections that were combined with exercise showed better treatment effects than any other treatments, whereas worse effects were observed when such injections were used alone. Regarding the CMS, most combined treatments based on exercise also demonstrated better effects than exercise alone. Regarding surgical treatments, according to the pain score and the CMS, arthroscopic subacromial decompression (ASD) together with treatments derived from it, such as ASD combined with radiofrequency and arthroscopic bursectomy, showed better effects than open subacromial decompression (OSD) and OSD combined with the injection of platelet-leukocyte gel. Exercise therapy also demonstrated good performance. Results for inconsistency, sensitivity analysis, and meta-regression all supported the robustness and reliability of these network meta-analyses. Exercise and other exercise-based therapies, such as kinesio taping, specific

  16. Frequent difficulties in the treatment of restless legs syndrome - case report and literature review.

    PubMed

    Narowska, Dominika; Bożek, Milena; Krysiak, Katarzyna; Antczak, Jakub; Holka-Pokorska, Justyna; Jernajczyk, Wojciech; Wichniak, Adam

    2015-01-01

    Restless legs syndrome (RLS) is one of the most common sleep disorders. The purpose of this paper is a case description of the patient suffering from RLS, concurrent with numerous clinical problems. In our patient, during long-term therapy with a dopamine agonist (ropinirole), the phenomenon of the augmentation, defined as an increase in the severity of the RLS symptoms, was observed. The quality of life of the patient was significantly deteriorated. Due to the augmentation of RLS symptoms the dopaminergic drug was gradually withdrawn, and the gabapentin as a second-line drug for the treatment of RLS was introduced. Because of the large increase of both insomnia and RLS symptoms during the reduction of ropinirole dose, clonazepam was temporarily introduced. In addition, in the neurological assessment of the distal parts of the lower limb sensory disturbances of vibration were found. The neurographic study confirmed axonal neuropathy of the sural nerves, which explained an incomplete response to dopaminergic medications. However, gabapentin treatment in the dose recommended in neuropathies was impossible due to bothersome side effects. Another important issue in the treatment of the patient were depressive symptoms and the fact that the majority of used antidepressants (mirtazapine, mianserin, tricyclic antidepressants) increase the severity of RLS. Among antidepressants recommended for the treatment of depression in patients with RLS (such as bupropion, moclobemide, reboxetine, tianeptine and agomelatine) only agomelatine exhibits promoting sleep properties. Because of the concomitant insomnia, this drug was applied in our patient. PMID:26688843

  17. Clinical efficacy of Ayurveda treatment regimen on Subfertility with Poly Cystic Ovarian Syndrome (PCOS)

    PubMed Central

    Dayani Siriwardene, S. A.; Karunathilaka, L. P. A; Kodituwakku, N. D.; Karunarathne, Y. A. U. D.

    2010-01-01

    Poly Cystic Ovarian Syndrome (PCOS) is the most common endocrinopathy in women of reproductive age, resulting from insulin resistance and the compensatory hyperinsulinemia. This results in adverse effect on multiple organ systems and may result in alteration in serum lipids, anovulation, abnormal uterine bleeding and infertility. According to Ayurvedic view PCOS can be correlated with Aarthava Kshaya. It was revealed that most of subfertility patients who were presented Osuki Ayurveda Centre suffered from the PCOS. Therefore the present study was carried out for the clinical evaluation of the efficacy of Ayurveda treatment regimen on subfertility with PCOS. Total 40 patients were selected by using purposive sampling method. According to the Ayurveda theories of Shodhana, Shamana and Tarpana, the treatment was conducted in 3 stages for the duration of 6 months. The response to the treatment was recorded and therapeutic effects were evaluated by symptomatic relief and through Trans Vaginal Scan and LH, FSH hormone levels. The results revealed that, subfertility due to PCOS can be cured successfully by using this Ayurveda treatment regimen. PMID:22131680

  18. Salbutamol and ephedrine in the treatment of severe AChR deficiency syndromes

    PubMed Central

    Rodríguez Cruz, Pedro M.; Palace, Jacqueline; Ramjattan, Hayley; Jayawant, Sandeep; Robb, Stephanie A.

    2015-01-01

    Objective: To evaluate the response to salbutamol and ephedrine in the treatment of congenital myasthenic syndromes due to CHRNE mutations causing severe acetylcholine receptor (AChR) deficiency. Methods: A cohort study of 6 patients with severe AChR deficiency, symptomatic despite optimal therapy with anticholinesterase and 3,4-diaminopyridine, were analyzed for their response to the addition of salbutamol or ephedrine to their medication. Baseline quantitative myasthenia gravis (QMG) (severity) scores were worse than 15 of 39. Patients were assessed in clinic with QMG and mobility scores. Pretreatment and 6- to 8-month follow-up scores were evaluated. Results: All 6 patients tolerated treatment well and reported no side effects. There was a strong positive response to treatment over the 6- to 8-month assessment period with significant improvement in QMG (p = 0.027) and mobility scores. The analysis of subcomponents of the QMG score revealed marked improvement in upper (p = 0.028) and lower (p = 0.028) limb raise times. All patients reported enhanced activities of daily living at 6 to 8 months. Conclusions: Oral salbutamol and ephedrine appear to be effective treatments in severe cases of AChR deficiency on pyridostigmine. They are well tolerated and improvement in strength can be dramatic. Classification of evidence: This study provides Class IV evidence that salbutamol or ephedrine improves muscle strength in patients with congenital myasthenia from severe AChR deficiency. PMID:26296515

  19. Transcatheter Ovarian Vein Embolization Using Coils for the Treatment of Pelvic Congestion Syndrome

    SciTech Connect

    Kwon, Se Hwan; Oh, Joo Hyeong Ko, Kyung Ran; Park, Ho Chul; Huh, Joo Yup

    2007-07-15

    Purpose. To evaluate the therapeutic effectiveness of ovarian vein embolization using coils for pelvic congestion syndrome (PCS), a common cause of chronic pelvic pain in multiparous women. Methods. Between November 1998 and June 2005, 67 patients were diagnosed with PCS and underwent ovarian vein coil embolization. Through medical records and telephone interviews, the pre-embolization pain level and post-embolization pain control were assessed. In addition, in those cases where pain persisted after embolization or where patients were dissatisfied with the procedure, additional treatments and subsequent changes in pain scores were also analyzed. Evaluation after coil embolization was performed within 3-6 months (n = 3), 6 months to 1 year (n 7), 1-2 years (n = 13), 2-3 years (n = 7), 3-4 years (n = 7), 4-5 years (n 13), or 5-6 years (n = 17). Results. Among a total of 67 patients, 82% (55/67) experienced pain reduction after coil embolization, were satisfied with the procedure, and did not pursue any further treatment. Twelve patients (18%, 12/67) responded that their pain level had not changed, or had become more severe. Among them, 9 patients were treated surgically and the remaining 3 patients remained under continuous drug therapy. Conclusion. Ovarian vein embolization using coils is a safe and effective therapeutic method for treatment of PCS. It is thought that surgical treatment should be considered in cases where embolization proves ineffective.

  20. Glycemic index treatment using Japanese foods in a girl with Lennox-Gastaut syndrome.

    PubMed

    Kumada, Tomohiro; Hiejima, Ikuko; Nozaki, Fumihito; Hayashi, Anri; Fujii, Tatsuya

    2013-05-01

    We introduced a low glycemic index treatment using Japanese ethnic foods to a 13-year-old girl with Lennox-Gastaut syndrome caused by tuberous sclerosis complex. She had previously refused the modified Atkins diet within 2 weeks of diet treatment because of its restrictiveness. The low glycemic index treatment was implemented by limiting the daily carbohydrate intake to 50 g of foods with a glycemic index of less than 50 relative to that of glucose, which included udon, soba, and unpolished Japonica rice with natto. One month after the initiation of the diet therapy, the clusters of tonic seizures for 30 to 60 minutes during sleep were reduced from two or three times per week to once or twice per month, and the frequent myoclonic seizures in the awake state disappeared. She has been on the diet therapy for more than 1 year, and the efficacy of the diet has been sustained. Low glycemic index treatment should be considered for patients with medication-resistant epilepsy who cannot tolerate restrictive diet therapies. Japanese ethnic foods can be used for this diet therapy. PMID:23583057

  1. Treatment guidelines and prognosis of immune reconstitution inflammatory syndrome patients: a review.

    PubMed

    Murthy, Anup R; Marulappa, Rekha; Hegde, Usha; Kappadi, Damodhar; Ambikathanaya, U K; Nair, Priyanka

    2015-04-01

    Immune reconstitution inflammatory syndrome (IRIS) is an "unmasking" or paradoxical worsening of a pre-existing infection after commencement of highly active antiretroviral therapy (HAART) in human immunodeficiency virus (HIV) - infected patients. The use of HAART in the management of HIV patients restores immune responses against pathogens however in few patients, the reconstituted immune system leads to IRIS. As the treatment protocols are not standardized for IRIS, this leads to short-term morbidity or in some cases also mortality. Therefore, treatment in these patients is a huge challenge and further more research regarding the immunopathogenesis, diagnosis and management of IRIS should be well thought-out. To understand the immunopathogenesis of IRIS it will be difficult to elucidate the intrinsic dynamics of immune cells after initiation of HAART but, there are few biomarkers which help to predict or diagnose IRIS and develop specific treatment, following initiation of HIV therapy. This review is an attempt to put light on those patients with IRIS with treatment guidelines for the management of the progression of it. PMID:25954081

  2. Calcium channel blockers and Alzheimer's disease: potential relevance in treatment strategies of metabolic syndrome.

    PubMed

    Goodison, William V; Frisardi, Vincenza; Kehoe, Patrick G

    2012-01-01

    Midlife hypertension is a risk factor for late onset Alzheimer's disease (AD) and it is one of the components of metabolic syndrome (MetS). Observational studies and some cardiovascular disease-related clinical trials suggest that antihypertensive treatment reduced the incidence and progression of AD. Calcium channel blockers (CCBs), one of the more commonly used treatments for hypertension, target voltage-gated calcium channels (VGCCs) which are found on neurons in the brain where calcium regulation is very important in both learning and memory. Amyloid-β (Aβ) peptide, one of the main pathological hallmarks of AD, causes increases to intracellular calcium via VGCCs, which in turn leads to further increases in Aβ production. Memantine, a current treatment used in AD, exerts some of its beneficial effects by blocking calcium entry into neurons. We explore the possibility of whether CCBs acting in the brain may delay the onset and progression of AD and thus may inform treatment regimes in people with MetS. PMID:22377784

  3. Classification, treatment and outcomes of a patient with lumbar extension syndrome.

    PubMed

    Harris-Hayes, Marcie; Van Dillen, Linda R; Sahrmann, Shirley A

    2005-01-01

    The purpose of the current report is to describe the classification, treatment, and outcomes of a patient with lumbar extension syndrome. The patient was a 40-year-old female with an 18-month history of mechanical low back pain (LBP). The patient reported a history of daily, intermittent pain (mean intensity of 9/10) that limited her ability to sit, stand, walk, and sleep, as well as perform work-related activities. Symptom-provoking movement and alignment impairments associated with the direction of lumbar extension were identified and modification of these impairments consistently resulted in a decrease in pain. Treatment was provided in 3 sessions over a 2-month period. Priority of treatment was to train the patient to restrict lumbar extension-related alignments and movements during symptom-provoking functional activities. Exercises to address the extension-related impairments also were prescribed. The primary change in outcome was a decrease in the mean intensity (2 months: 2/10; 6 months: 1/10) and frequency of pain (2 months: decreased pain with standing and walking; 6 months: additional decrease with sitting, standing and walking). She also reported a decreased duration and number of LBP episodes. Classification directed treatment resulted in improvement in short and long term impairment and functional-level outcomes. PMID:16389699

  4. Review of the Treatment of Restless Legs Syndrome: Focus on Gabapentin Enacarbil

    PubMed Central

    Burke, Rachel A.; Faulkner, Michele A.

    2012-01-01

    The FDA approved gabapentin enacarbil in 2011 as the first non-dopaminergic agent for the treatment of restless legs syndrome (RLS) symptoms. Although gabapentin enacarbil is a pro-drug of gabapentin, its pharmacokinetics differ. Absorption of gabapentin enacarbil is more predictable, and inter-patient variability in bioavailability is lower than that of gabapentin. Studies have demonstrated superiority of gabapentin enacarbil compared to placebo. Comparisons to currently available RLS treatments are lacking, but clinical trials demonstrate comparable improvement in RLS symptoms to the dopamine agonists ropinirole and pramipexole, which are usually considered first-line therapy for daily RLS symptoms. Gabapentin enacarbil was well tolerated in clinical trials. The role of the drug in RLS treatment remains undefined, although it will likely be used as an alternative for refractory RLS when other treatments have failed. Additionally, gabapentin enacarbil may be recommended for patients with daily RLS symptoms that are less intense or are associated with pain as an alternative to dopamine agonists. PMID:23650473

  5. Alternative Treatment in Prostate Pain Syndrome Based on Iranian Traditional Medicine

    PubMed Central

    Latifi, Seied Amirhossein; Kamalinejad, Mohammad; Minaiee, Bagher; Bahrami, Mohsen; Gooran, Shahram; Nikbakht Nasrabadi, Alireza

    2014-01-01

    Introduction: Unknown etiology and pathophysiology of prostate pain syndrome (PPS) has led to a lack of proper and competent treatment in modern medicine. According to the guidelines of European Association of Urology (EAU), use of complementary treatments is recommended for PPS. In this preliminary study, analyzing the signs and symptoms of PPS from the viewpoint of Iranian traditional medicine (ITM) was helpful in selecting the appropriate alternative treatment. Case Presentation: Two male patients diagnosed with PPS were evaluated and treated according to the ITM. Each patient took 15 mL oxymel 45 minutes after lunch and dinner. For each patient, four clinical visits were made with one week intervals and the validated Farsi version of international prostate symptom score (IPSS) and numeric pain rating score (NPRS) were completed for them. Conclusions: Considering the fact that other major pathological causes are ruled out, many of the symptoms and signs observed in these patients were similar to those associated with flatulency-related diseases in ITM. Selecting treatment with oxymel was based on this view and led to improvements in the digestive and urinary symptoms according to Farsi version of the IPSS and NPRS. PMID:25237573

  6. Personalized treatment of Sézary syndrome by targeting a novel CTLA4:CD28 fusion

    PubMed Central

    Sekulic, Aleksandar; Liang, Winnie S; Tembe, Waibhav; Izatt, Tyler; Kruglyak, Semyon; Kiefer, Jeffrey A; Cuyugan, Lori; Zismann, Victoria; Legendre, Christophe; Pittelkow, Mark R; Gohmann, John J; De Castro, Fernando R; Trent, Jeffrey; Carpten, John; Craig, David W; McDaniel, Timothy K

    2015-01-01

    Matching molecularly targeted therapies with cancer subtype-specific gene mutations is revolutionizing oncology care. However, for rare cancers this approach is problematic due to the often poor understanding of the disease's natural history and phenotypic heterogeneity, making treatment of these cancers a particularly unmet medical need in clinical oncology. Advanced Sézary syndrome (SS), an aggressive, exceedingly rare variant of cutaneous T-cell lymphoma (CTCL) is a prototypical example of a rare cancer. Through whole genome and RNA sequencing (RNA-seq) of a SS patient's tumor we discovered a highly expressed gene fusion between CTLA4 (cytotoxic T lymphocyte antigen 4) and CD28 (cluster of differentiation 28), predicting a novel stimulatory molecule on the surface of tumor T cells. Treatment with the CTLA4 inhibitor ipilimumab resulted in a rapid clinical response. Our findings suggest a novel driver mechanism for SS, and cancer in general, and exemplify an emerging model of cancer treatment using exploratory genomic analysis to identify a personally targeted treatment option when conventional therapies are exhausted. PMID:25802883

  7. Metabolic syndrome, dyslipidemia, hypertension and type 2 diabetes in youth: from diagnosis to treatment

    PubMed Central

    2010-01-01

    Overweight and obesity in youth is a worldwide public health problem. Overweight and obesity in childhood and adolescents have a substantial effect upon many systems, resulting in clinical conditions such as metabolic syndrome, early atherosclerosis, dyslipidemia, hypertension and type 2 diabetes (T2D). Obesity and the type of body fat distribution are still the core aspects of insulin resistance and seem to be the physiopathologic links common to metabolic syndrome, cardiovascular disease and T2D. The earlier the appearance of the clustering of risk factors and the higher the time of exposure, the greater will be the chance of developing coronary disease with a more severe endpoint. The age when the event may occur seems to be related to the presence and aggregation of risk factors throughout life. The treatment in this age-group is non pharmacological and aims at promoting changes in lifestyle. However, pharmacological treatments are indicated in special situations. The major goals in dietary treatments are not only limited to weight loss, but also to an improvement in the quality of life. Modification of risk factors associated to comorbidities, personal satisfaction of the child or adolescent and trying to establish healthy life habits from an early age are also important. There is a continuous debate on the best possible exercise to do, for children or adolescents, in order to lose weight. The prescription of physical activity to children and adolescents requires extensive integrated work among multidisciplinary teams, patients and their families, in order to reach therapeutic success. The most important conclusion drawn from this symposium was that if the growing prevalence of overweight and obesity continues at this pace, the result will be a population of children and adolescents with metabolic syndrome. This would lead to high mortality rates in young adults, changing the current increasing trend of worldwide longevity. Government actions and a better

  8. Directed Therapy: An Approach to the Improved Treatment of Exfoliation syndrome

    PubMed Central

    Angelilli, Allison; Ritch, Robert

    2009-01-01

    Exfoliation syndrome (XFS) is an age-related, generalized disorder of the extracellular matrix characterized by the production and progressive accumulation of a fibrillar extracellular material in many ocular tissues and is the most common identifiable cause of open-angle glaucoma worldwide. Exfoliation syndrome plays an etiologic role in open-angle glaucoma, angle-closure glaucoma, cataract, and retinal vein occlusion. It is accompanied by an increase in serious complications at the time of cataract extraction, such as zonular dialysis, capsular rupture, and vitreous loss. It is associated systemically with an increasing number of vascular disorders, hearing loss, and Alzheimer's disease. Exfoliation syndrome appears to be a disease of elastic tissue microfibrils. Directed therapy simply means devising specific treatments for specific diseases. There was little incentive to attempt to distinguish between various open-angle glaucomas if the treatments were essentially the same. However, this view also prevented the application of directed therapy in those instances in which such was available and applicable. Pilocarpine has multiple beneficial actions in eyes with XFS. Not only does it lower IOP, but by increasing aqueous outflow, it should enable the trabecular meshwork to clear more rapidly, and by limiting pupillary movement, should slow the progression of the disease. Theoretically, miotics should be the first line of treatment. Pilocarpine 2% q.h.s. can provide sufficient limitation of pupillary mobility without causing these side effects. In 2007, two common single nucleotide polymorphisms in the coding region of the lysyl oxidase-like 1 (LOXL1) gene located on chromosome 15 were specifically associated with XFS and XFG. LOXL1 is a member of the lysyl oxidase family of enzymes, which are essential for the formation, stabilization, maintenance, and remodelling of elastic fibers and prevent age-related loss of elasticity of tissues. LOXL1 protein is a major

  9. Burning Mouth Syndrome due to Television Moans, an Enigma for Oral Physician: Treatment with Counseling.

    PubMed

    Gupta, Deepak; Sheikh, Soheyl; Pallagatti, Shambulingappa; Kasariya, Kartikaya; Buttan, Amit; Gupta, Maqul

    2014-01-01

    Burning mouth syndrome (BMS) is a relatively common disease that can severely affect the quality of life of the patient. It causes chronic orofacial pain or oral burning sensation even in the absence of any detectable organic cause. The etiology of BMS is complex and multifactorial. It has been associated with menopause, trigger events and even genetic polymorphisms. Although its etiology remains unclear, there is still much evidence that psychological elements like stress, anxiety or depression do play a significant role. There are several studies in the literature which only report the association of BMS with psychological factors. But to the best of our knowledge, there is no such case reported in the literature which has actually highlighted the management of such a case with psychogenic elements involved. In this case report, apart from discussing the role of psychological factors, the treatment of BMS with emphasis on counseling is also emphasized. Further, it is of interest to know that such patients with psychologically induced burning mouth syndrome have to be evaluated to their deepest details. Even their commonly overlooked gestures and habits like watching a particular television soap opera may be involved in their disease process. It can be concluded that psychological counseling in general dental practice can provide an effective cure for chronic oral burning sensation with psychological factors involved. PMID:25093058

  10. Assessment of Decisional Conflict about the Treatment of carpal tunnel syndrome, Comparing Patients and Physicians

    PubMed Central

    Hageman, Michiel GJS.; Bossen, Jeroen K.; Neuhaus, Valentin; Mudgal, Chaitanya S.; Ring, David

    2016-01-01

    Background: As part of the process of developing a decision aid for carpal tunnel syndrome (CTS) according to the Ottawa Decision Support Framework, we were interested in the level of ‘decisional conflict’ of hand surgeons and patients with CTS. This study addresses the null hypothesis that there is no difference between surgeon and patient decisional conflict with respect to test and treatment options for CTS. Secondary analyses assess the impact of patient and physician demographics and the strength of the patient-physician relationship on decisional conflict. Methods: One-hundred-twenty-three observers of the Science of Variation Group (SOVG) and 84 patients with carpal tunnel syndrome completed a survey regarding the Decisional Conflict Scale. Patients also filled out the Pain Self-efficacy Questionnaire (PSEQ) and the Patient Doctor Relationship Questionnaire (PDRQ-9). Results: On average, patients had significantly greater decision conflict and scored higher on most subscales of the decisional conflict scale than hand surgeons. Factors associated with greater decision conflict were specific hand surgeon, less self-efficacy (confidence that one can achieve one’s goals in spite of pain), and higher PDRQ (relationship between patient and doctor). Surgeons from Europe have--on average--significantly more decision conflict than surgeons in the United States of America. Conclusions: Patients with CTS have more decision conflict than hand surgeons. Decision aids might help narrow this gap in decisional conflict. PMID:27200394

  11. Statins: Do they have potential in the treatment of polycystic ovary syndrome?

    PubMed Central

    Kodaman, Pinar H.; Duleba, Antoni J.

    2010-01-01

    Many women of reproductive age are affected by polycystic ovary syndrome (PCOS), a heterogeneous endocrinopathy characterized by androgen excess, chronic oligo-anovulation and/or polycystic ovarian morphology. In addition, PCOS is often associated with insulin resistance, systemic inflammation and oxidative stress which, on one hand, lead to endothelial dysfunction and dyslipidemia with subsequent cardiovascular sequelae and, on the other hand, to hyperplasia of the ovarian theca compartment with resultant hyperandrogenism and anovulation. While traditionally statins have been used to treat dyslipidemia by blocking HMG-CoA reductase, the rate limiting step in cholesterol biosynthesis; in fact, they possess pleiotropic actions, resulting in antioxidant, anti-inflammatory and anti-proliferative effects. Statins offer a novel therapeutic approach to PCOS in that they address the dyslipidemia associated with the syndrome, as well as hyperandrogenism/hyperandrogenemia. These actions may be due to an inhibition of the effects of systemic inflammation and insulin resistance/hyperinsulinemia. Evidence to date, both in vitro and in vivo, suggests that statins have potential in the treatment of PCOS; however, further clinical trials are needed before they can be considered a standard of care in the medical management of this common endocrinopathy. PMID:18181091

  12. Burning Mouth Syndrome due to Television Moans, an Enigma for Oral Physician: Treatment with Counseling

    PubMed Central

    Gupta, Deepak; Sheikh, Soheyl; Pallagatti, Shambulingappa; Kasariya, Kartikaya; Buttan, Amit; Gupta, Maqul

    2014-01-01

    Burning mouth syndrome (BMS) is a relatively common disease that can severely affect the quality of life of the patient. It causes chronic orofacial pain or oral burning sensation even in the absence of any detectable organic cause. The etiology of BMS is complex and multifactorial. It has been associated with menopause, trigger events and even genetic polymorphisms. Although its etiology remains unclear, there is still much evidence that psychological elements like stress, anxiety or depression do play a significant role. There are several studies in the literature which only report the association of BMS with psychological factors. But to the best of our knowledge, there is no such case reported in the literature which has actually highlighted the management of such a case with psychogenic elements involved. In this case report, apart from discussing the role of psychological factors, the treatment of BMS with emphasis on counseling is also emphasized. Further, it is of interest to know that such patients with psychologically induced burning mouth syndrome have to be evaluated to their deepest details. Even their commonly overlooked gestures and habits like watching a particular television soap opera may be involved in their disease process. It can be concluded that psychological counseling in general dental practice can provide an effective cure for chronic oral burning sensation with psychological factors involved. PMID:25093058

  13. Twenty years of vasoplegic syndrome treatment in heart surgery. Methylene blue revised

    PubMed Central

    Evora, Paulo Roberto Barbosa; Alves, Lafaiete; Ferreira, Cesar Augusto; Menardi, Antônio Carlos; Bassetto, Solange; Rodrigues, Alfredo José; Scorzoni, Adilson; Vicente, Walter Vilella de Andrade

    2015-01-01

    Objective This study was conducted to reassess the concepts established over the past 20 years, in particular in the last 5 years, about the use of methylene blue in the treatment of vasoplegic syndrome in cardiac surgery. Methods A wide literature review was carried out using the data extracted from: MEDLINE, SCOPUS and ISI WEB OF SCIENCE. Results The reassessed and reaffirmed concepts were 1) MB is safe in the recommended doses (the lethal dose is 40 mg/kg); 2) MB does not cause endothelial dysfunction; 3) The MB effect appears in cases of NO up-regulation; 4) MB is not a vasoconstrictor, by blocking the cGMP pathway it releases the cAMP pathway, facilitating the norepinephrine vasoconstrictor effect; 5) The most used dosage is 2 mg/kg as IV bolus, followed by the same continuous infusion because plasma concentrations sharply decrease in the first 40 minutes; and 6) There is a possible "window of opportunity" for MB's effectiveness. In the last five years, major challenges were: 1) Observations about side effects; 2) The need for prophylactic and therapeutic guidelines, and; 3) The need for the establishment of the MB therapeutic window in humans. Conclusion MB action to treat vasoplegic syndrome is time-dependent. Therefore, the great challenge is the need, for the establishment the MB therapeutic window in humans. This would be the first step towards a systematic guideline to be followed by possible multicenter studies. PMID:25859872

  14. Treatment of anterior tarsal tunnel syndrome through an endoscopic or open technique.

    PubMed

    Yassin, Mustafa; Garti, Avraham; Weissbrot, Moshe; Heller, Eyal; Robinson, Dror

    2015-09-01

    Anterior tarsal tunnel syndrome is often underdiagnosed, due to lack of clinical awareness and vague clinical presentation. Most often patients complain of pain located to the dorsum of the foot. The present study is a consecutive series of 13 patients treated according to a fixed protocol followed for a minimum of 24 months. A total of 12/13 cases presented with a bulge in the anterior part of the ankle or the dorsal foot and Tinel's sign was positive over it. Only half had decreased sensation. Surgical technique was either endoscopic or open. Endoscopy is preferable when compression is due to an osteophyte (4/13) or an isolated ganglion 2/13). In other cases presenting with synovitis (5/13) or unknown etiology (2/13) performing open surgery was deemed as safer. The American Orthopedic Foot and Ankle Society (AOFAS) hindfoot scores improved from an average of 55 ± 8 to 83 ± 11 at 12 months after surgery and 88 ± 10 at 24 months after surgery. The anterior tarsal tunnel syndrome accounts for approximately 5% of cases complaining of feet numbness, which undergo electromyographic and nerve conduction testing. Reports in the scientific literature are scarce, perhaps due to underdiagnosis, while it is amenable to surgical management. Clinical diagnosis supported by imaging studies demonstrated osteophytes, ganglions or localized synovitis. Endoscopic treatment can be performed safely provided a clear-cut single compressing element is identified. PMID:26209470

  15. Halogravity traction in the preoperative treatment of scoliosis in twins with Marfan syndrome.

    PubMed

    Cimic, Mislav; Crnogaca, Kresimir; Vrdoljak, Ozren; Bicanic, Goran

    2015-01-01

    We report on the influence of the duration of halogravity traction for achieving curve correction in monozygotic twins with Marfan syndrome who underwent posterior spinal fusion. Review of the medical charts and standard radiograph analysis of twin girls treated at our department was performed. Halogravity traction with a four-pin skull construct was applied for 3 weeks in twin A and for 2 weeks in twin B with a maximum of 20% body weight used. Both were on a 24-hours-day halogravity traction regime. Achieved thoracic curve correction after halogravity traction was 31% in twin A and 18% in twin B. Although less curve correction after traction was achieved in twin B, this had no significant implications on final postoperative curve correction. Halogravity traction can be a useful tool in the preoperative treatment of scoliosis in patients with Marfan syndrome if applied for 3 weeks. In order to avoid complications, we propose that lower weights be used with a starting weight of 1.5 kg increased by 1 kg daily until 20% body weight is reached. PMID:26032703

  16. Advances in the Pathogenesis, Diagnosis and Treatment of Bow Hunter's Syndrome: A Comprehensive Review of the Literature.

    PubMed

    Duan, Guangxin; Xu, Jiaping; Shi, Jijun; Cao, Yongjun

    2016-06-01

    Bow hunter's syndrome (BHS), also known as rotational vertebral artery (VA) occlusion syndrome, is a rare yet treatable type of symptomatic vertebrobasilar insufficiency resulting from mechanical occlusion or stenosis of the VA during head and neck rotation or extension. The symptoms of BHS range from transient vertigo to posterior circulation stroke. The underlying pathology is dynamic stenosis or compression of the VA by abnormal bony structures with neck rotation or extension in many cases, such as osteophyte, disc herniation, cervical spondylosis, tendinous bands or tumors. Imaging approaches, such as Doppler sonography, computed tomography and angiography, as well as magnetic resonance imaging and angiography, are widely used in the diagnosis and evaluation of this syndrome. Digital subtraction angiography with head rotation remains the gold standard diagnostic method. Conservative management, surgery and endovascular procedures are the three major treatment methods for BHS, whereas some symptomatic patients may need operative treatment including surgery and endovascular procedures when conservative management is not adequate. PMID:27610119

  17. Advances in the Pathogenesis, Diagnosis and Treatment of Bow Hunter's Syndrome: A Comprehensive Review of the Literature

    PubMed Central

    Duan, Guangxin; Xu, Jiaping; Shi, Jijun; Cao, Yongjun

    2016-01-01

    Bow hunter's syndrome (BHS), also known as rotational vertebral artery (VA) occlusion syndrome, is a rare yet treatable type of symptomatic vertebrobasilar insufficiency resulting from mechanical occlusion or stenosis of the VA during head and neck rotation or extension. The symptoms of BHS range from transient vertigo to posterior circulation stroke. The underlying pathology is dynamic stenosis or compression of the VA by abnormal bony structures with neck rotation or extension in many cases, such as osteophyte, disc herniation, cervical spondylosis, tendinous bands or tumors. Imaging approaches, such as Doppler sonography, computed tomography and angiography, as well as magnetic resonance imaging and angiography, are widely used in the diagnosis and evaluation of this syndrome. Digital subtraction angiography with head rotation remains the gold standard diagnostic method. Conservative management, surgery and endovascular procedures are the three major treatment methods for BHS, whereas some symptomatic patients may need operative treatment including surgery and endovascular procedures when conservative management is not adequate. PMID:27610119

  18. Clinical presentation and chiropractic treatment of Tietze syndrome: A 34-year-old female with left-sided chest pain

    PubMed Central

    Gijsbers, Eefje; Knaap, Simone F.C.

    2011-01-01

    Objective The purpose of this case report is to describe the clinical presentation and chiropractic management of Tietze syndrome. Clinical Features A 34-year-old woman presented with unexplained left-sided chest pain. Electrocardiogram and radiographs were taken at a medical emergency department to rule out cardiovascular and pulmonary causes, and pain medication did not relieve her pain. Physical examination showed tenderness on palpation and swelling of the second and third chondrosternal joints, as well as thoracic joint dysfunction. Heart and lung pathology was ruled out, and chondrosternal joint swelling was present, Tietze syndrome was diagnosed. Intervention and Outcome A treatment plan aimed at restoring normal thoracic and rib joint movement and decreasing inflammation of the chondrosternal joints resulted in lower pain levels. Treatment consisted of diversified high-velocity, low-amplitude chiropractic manipulation; activator technique; and cryotherapy. Conclusion Chiropractic management of Tietze syndrome was successful in reducing pain levels in this patient's case. PMID:22027210

  19. Circadian transcriptome analysis in human fibroblasts from Hunter syndrome and impact of iduronate-2-sulfatase treatment

    PubMed Central

    2013-01-01

    Background Hunter syndrome (HS) is a lysosomal storage disease caused by iduronate-2-sulfatase (IDS) deficiency and loss of ability to break down and recycle the glycosaminoglycans, heparan and dermatan sulfate, leading to impairment of cellular processes and cell death. Cell activities and functioning of intracellular organelles are controlled by the clock genes (CGs), driving the rhythmic expression of clock controlled genes (CCGs). We aimed to evaluate the expression of CGs and downstream CCGs in HS, before and after enzyme replacement treatment with IDS. Methods The expression levels of CGs and CCGs were evaluated by a whole transcriptome analysis through Next Generation Sequencing in normal primary human fibroblasts and fibroblasts of patients affected by HS before and 24 h/144 h after IDS treatment. The time related expression of CGs after synchronization by serum shock was also evaluated by qRT-PCR before and after 24 hours of IDS treatment. Results In HS fibroblasts we found altered expression of several CGs and CCGs, with dynamic changes 24 h and 144 h after IDS treatment. A semantic hypergraph-based analysis highlighted five gene clusters significantly associated to important biological processes or pathways, and five genes, AHR, HIF1A, CRY1, ITGA5 and EIF2B3, proven to be central players in these pathways. After synchronization by serum shock and 24 h treatment with IDS the expression of ARNTL2 at 10 h (p = 0.036), PER1 at 4 h (p = 0.019), PER2 at 10 h (p = 0.041) and 16 h (p = 0.043) changed in HS fibroblasts. Conclusion CG and CCG expression is altered in HS fibroblasts and IDS treatment determines dynamic modifications, suggesting a direct involvement of the CG machinery in the physiopathology of cellular derangements that characterize HS. PMID:24083598

  20. Novel pharmacotherapeutic strategies for treatment of opioid-induced neonatal abstinence syndrome

    PubMed Central

    McLemore, Gabrielle L.; Lewis, Tamorah; Jones, Catherine H.; Gauda, Estelle B.

    2014-01-01

    Summary The non-medical use of prescription drugs, in general, and opioids, in particular, is a national epidemic, resulting in enormous addiction rates, healthcare expenditures, and overdose deaths. Prescription opioids are overly prescribed, illegally trafficked, and frequently abused, all of which have created a new opioid addiction pathway, adding to the number of opioid-dependent newborns requiring treatment for neonatal abstinence syndrome (NAS), and contributing to challenges in effective care in maternal and fetal/neonatal (M-F/N) medicine. The standard of care for illicit or prescription opioid dependence during pregnancy is opioid agonist (methadone or buprenorphine) substitution therapy, which are also frequently abused. The next generation of pharmacotherapies for the treatment of illicit or prescription opioid addiction in the M-F/N interactional dyad must take into consideration the interplay between genetic, epigenetic, and environmental factors. Addiction to illicit drugs during pregnancy presents unique challenges to effectively treat the mother, and the developing fetus and infant after delivery. New pharmacotherapies should be safe to the developing fetus, effective in treating the physical and psychological consequences of addiction in the mother, and reduce the incidence and severity of NAS in the infant after birth. More pharmacotherapeutic options should be available to the physician such that a more individualized rather than a one-drug/strategy-fits-all approach can be used. A myriad of new and exciting pharmacotherapeutic strategies for the treatment of opioid dependence and addiction are on the horizon. This review focuses on such three strategies: (i) pharmacotherapeutic targeting of the serotoninergic system; (ii) mixed opioid immunotherapeutics (vaccines); (iii) pharmacogenomics as a therapeutic strategy to insure personalized care. We review and discuss how these strategies may offer additional treatment modalities for the treatment