Slow Tapering Research Articles

1215

Introduction: Chlorine is an irritant gas that is commonly used for water treatment and as part of household bleach. We present a case of ARDS in a homemaker secondary to household use of chlorine-based bleach. Case Presentation: A 46-year-old African-American woman presented to the emergency room with 1-day history of rapidly progressive dyspnea after using a relatively higher than usual amount of bleach with poor ventilation at home. In the past, she used to have some fatigue after use of this bleach which resolved over few hours. On examination, she was in obvious respiratory distress with tachycardia, tachypnea and oxygen saturation at 79% on ambient air. She had diffuse coarse crackles bilaterally on lung auscultation, but no wheezing. Arterial blood gas analysis showed a pH of 7.41, pCO2 of 37 and pO2 of 66 on FiO2 of 40% (PaO2/FiO2 ratio of 165). CT scan of the chest showed bilateral diffuse interstitial infiltrates and multifocal patchy areas of solid consolidation, both worse in upper lung zones. She was admitted to the intensive care unit and was started on intravenous steroids for presumed acute inhalation lung injury. Her hypoxia and dyspnea improved over the next 2 days and she was discharged on day 4 of hospitalization with a slow prednisone taper starting at 40 mg daily over 1 month and supplemental oxygen at rest and activity. At 1-month follow up, her oxygen saturation was normal at 100% on ambient air. Repeat CT chest at 2-month follow up showed complete resolution of previously seen infiltrates. Discussion: The usual description of accidental chlorine injury is reactive airway dysfunction syndrome, while non-cardiogenic pulmonary edema and ARDS have been reported in association with high levels of exposures such as transportation accidents, industrial or swimming pool spills. Our case is rare as the patient had a near-fatal presentation from household exposure to bleach which she has otherwise been using in the past. Both acute and chronic exposures to chlorine have been associated with airflow obstruction and bronchial airway hyperreactivity (AHR). Our patient did not show any evidence airflow obstruction on spirometry performed 4 weeks after the acute event. A methacholine challenge test was performed and it did not demonstrate any AHR as well. Animal models of high-dose chlorine exposure have shown that much of chlorine damage is oxidative in nature. There is histologic evidence of airway and alveolar epithelial damage and alveolar edema that usually resolves in 10 days. It is likely that genetic susceptibility to oxidative injury determines which patients would present with AHR versus alveolar damage. Conclusion: Household exposure to chlorine-based bleach may present with ARDS in susceptible individuals. Apart from a rare presentation of household chlorine exposure, this case also highlights the importance of detailed exposure history in the context of clinical presentation. Our patient had no recurrence of symptoms after switching to non-chlorine based bleach.

Diagnosis and treatment of pediatric acquired aplastic anemia (AAA): An initial survey of the North American Pediatric Aplastic Anemia Consortium (NAPAAC)

Randomized clinical trials in pediatric aplastic anemia (AA) are rare and data to guide standards of care are scarce. Eighteen pediatric institutions formed the North American Pediatric Aplastic Anemia Consortium to foster collaborative studies in AA. The initial goal of NAPAAC was to survey the diagnostic studies and therapies utilized in AA. Our survey indicates considerable variability among institutions in the diagnosis and treatment of AA. There were areas of general consensus, including the need for a bone marrow evaluation, cytogenetic and specific fluorescent in situ hybridization assays to establish diagnosis and exclude genetic etiologies with many institutions requiring results prior to initiation of immunosuppressive therapy (IST); uniform referral for hematopoietic stem cell transplantation as first line therapy if an HLA-identical sibling is identified; the use of first-line IST containing horse anti-thymocyte globulin and cyclosporine A (CSA) if an HLA-identical sibling donor is not identified; supportive care measures; and slow taper of CSA after response. Areas of controversy included the need for telomere length results prior to IST, the time after IST initiation defining a treatment failure; use of hematopoietic growth factors; the preferred rescue therapy after failure of IST; the use of specific hemoglobin and platelet levels as triggers for transfusion support; the use of prophylactic antibiotics; and follow-up monitoring after completion of treatment. These initial survey results reflect heterogeneity in diagnosis and care amongst pediatric centers and emphasize the need to develop evidence-based diagnosis and treatment approaches in this rare disease.

Prospective study on the withdrawal and reinstitution of antiepileptic drugs among seizure-free patients in west China

This study explored the relapse rates and risk factors for seizure recurrence after discontinuing antiepileptic drug (AED) therapy among seizure-free patients in west China, and explored whether to reinstitute AED immediately after a single seizure after AED withdrawal. Patients with epilepsy who were seizure-free for at least 2years and decided to gradually stop AED therapy were followed up every 3months for seizure relapse. Patients who experienced their first seizure after drug withdrawal were divided into two groups according to their willingness to reinstitute AED therapy, and were followed up until their second seizure. In the mean 29.35months of follow-up, 37 patients (37/162, 22.8%) suffered at least one seizure after withdrawal. The cumulative probability of seizure recurrence was 16% at 12months and 20.2% at 24months. AED response time >1year and multiple types of seizure were identified as risk factors for seizure recurrence. Eight patients (8/32, 25%) suffered a second seizure within 1year after the first whether or not they reinstituted AED immediately. There were no significant demographic or clinical differences between patients who reinstituted AED therapy and those who did not. The epilepsy recurrence rate after AED withdrawal is relatively low, with a relatively slow tapering process. Patients with long AED response times and/or multiple types of seizures have a higher risk of seizure recurrence. The first seizure after drug withdrawal is not an indication for immediate AED reinstitution, but may be recommended after a second seizure.

Churg-Strauss Syndrome and Persistent Heart Failure

Churg-Strauss syndrome (CSS) is a rare small-vessel vasculitis typically associated with adult-onset asthma, peripheral and tissue hypereosinophilia, migratory pulmonary infiltrates, upper respiratory tract symptoms, and clinical evidence of systemic vasculitis. Cardiac involvement is a well-recognized complication with an estimated prevalence of 60%. Heart disease is associated with poor prognosis, accounting for almost 50% mortality in CSS. We present a case of a 48-year-old woman with CSS complicated by congestive heart failure with left ventricular ejection fraction of 25%, who was initially treated with long course of high-dose steroids without any clinical or echocardiographic improvement. She was referred to our hospital 1 year later and was initiated with cyclophosphamide 2 mg/kg per day and prednisone 60 mg/d followed by slow taper. Subsequently, the patient had remarkable improvement. Patient was then transitioned to azathioprine for 1.5 years with sustained disease remission. It may be difficult to determine myocardial disease activity status versus tissue damage in CSS with prolonged duration of heart failure symptoms. This is the first case report demonstrating that CSS cardiac disease may remain active despite 1 year of corticosteroid therapy, and significant improvement or remission can still be achieved by administering more aggressive cytotoxic immunosuppressive therapy.

Ipilimumab-induced Colitis with Concomitant Clostridium difficile Infection

Introduction: Ipilimumab (Anti-Cytotoxic T lymphocyte-associated antigen (CTLA)-4 antibody)-induced colitis is a rare immune-mediated adverse event. Prompt diagnosis and treatment can prevent life-threatening complications. We report a case of colitis caused by Ipilimumab with concomitant Clostridium difficile (C. diff) infection and successful treatment with systemic corticosteroids. Case Report: A 32-year-old female who was undergoing Ipilimumab therapy post cycle number four for relapse of malignant melanoma presented with bloody diarrhea for two days. The patient denied fever, nausea, and vomiting. Examination revealed a soft but tender abdomen in the left lower quadrant. Stool studies were positive for C. diff toxin. She was treated with PO Vancomycin. Diarrhea slowly improved with recurrent episodes of bright red blood. Repeat C. diff toxin on day 10 was negative. IV Solumedrol 40 mg q8hrs was started with immediate response and complete resolution of hematochezia and diarrhea. Colonoscopy on day 13 showed severe colitis with erythema, edema, and friability with a plethora of large crated ulcers in the left colon, predominantly the splenic flexure. Biopsy showed mucosal ulceration and granulation tissue with rare cryptitis. She remained asymptomatic for the next few days on IV steroids, and was transitioned to 60-mg prednisone with a slow taper of Vancomycin over three weeks. As an outpatient, she was switched to Budesonide with plan for repeat colonoscopy. Discussion: Colonic toxicity is an immune-mediated adverse event caused by a biological agent. With increased use of Ipilimumab to treat malignant melanoma, the incidence of colitis is rising (recorded in about 20% of the patients receiving this treatment). Typical symptoms include abdominal pain and diarrhea, sometimes bloody. Endoscopy can show segmental or diffuse colitis. Biopsies show nonspecific inflammatory infiltrate with predominant T-cells, cryptitis, and abscess formation. Fatal complications (5%) like perforation (<1%) can occur without prompt appropriate treatment. Systemic steroids are the treatment of choice. Limited or no response to steroids in a week should lead to the use of infliximab. Failure of medical treatment can necessitate partial or complete colectomy with diverting ileostomy. Few cases have been reported of concomitant C. diff colitis, which complicates the course. Our patient was initially treated only for C. diff. Lack of sustained response to antibiotics prompted initiation of corticosteroids. Colonoscopy showed significantly deep ulcerations, suggesting that perforation could have occurred without timely steroids. C. diff infection should not preclude use of systemic steroids if immune mediated colitis is suspected.

Psychological treatment of hypnotic-dependent insomnia in a primarily older adult sample

ObjectiveThis study tested cognitive behavior therapy (CBT) in hypnotic-dependent, late middle-age and older adults with insomnia. MethodSeventy volunteers age 50 and older were randomized to CBT plus drug withdrawal, placebo biofeedback (PL) plus drug withdrawal, or drug withdrawal (MED) only. The CBT and PL groups received eight, 45 min weekly treatment sessions. The drug withdrawal protocol comprised slow tapering monitored with about six biweekly, 30 min sessions. Assessment including polysomnography (PSG), sleep diaries, hypnotic consumption, daytime functioning questionnaires, and drug screens collected at baseline, posttreatment, and 1-year follow-up. ResultsOnly the CBT group showed significant sleep diary improvement, sleep onset latency significantly decreased at posttreatment. For all sleep diary measures for all groups, including MED, sleep trended to improvement from baseline to follow-up. Most PSG sleep variables did not significantly change. There were no significant between group differences in medication reduction. Compared to baseline, the three groups decreased hypnotic use at posttreatment, down 84%, and follow-up, down 66%. There was no evidence of withdrawal side-effects. Daytime functioning, including anxiety and depression, improved by posttreatment. Rigorous methodological features, including documentation of strong treatment implementation and the presence of a credible placebo, elevated the confidence due these findings. ConclusionsGradual drug withdrawal was associated with substantial hypnotic reduction at posttreatment and follow-up, and withdrawal side-effects were absent. When supplemented with CBT, participants accrued incremental self-reported, but not PSG, sleep benefits.

Gabapentin Withdrawal: Case Report in an Older Adult and Review of the Literature

Gabapentin Withdrawal: Case Report in an Older Adult and Review of the Literature

Withdrawal of antiepileptic drugs during presurgical video-EEG monitoring: an observational study for evaluation of current practice at a referral center for epilepsy

Withdrawal of antiepileptic drugs (AEDs) before and during video-EEG-monitoring is commonly implemented to reduce time needed to register a sufficient number of seizures during presurgical evaluation. There are, however, few guidelines regarding withdrawal rate and observation time. We performed an observational study including sixty patients admitted to the national Norwegian epilepsy centre and registered tapering of AEDs and their effect on seizure rate and possible complications. The mean daily seizure rate before admission to the EMU was 0.4 (range 0.02-4) increasing to 1.1 (range 0-8) at the EMU. 29 patients (48%) followed a slow tapering rate whereas 31 (52%) had an intermediate tapering rate. There was no significant difference between the patients with a daily seizure rate during LTM of more or <0.7 seizures per day, an increase of seizure frequency from habitual to during LTM of more or <3.3 or 6.9 with regard to rate of tapering (slow vs intermediate) etiology or AED monotherapy vs polytherapy. Twenty-six patients (43%) had a sufficient number of seizures registered within 3 days to conclude regarding the presurgical evaluation. Two patients received escape treatment while 25 patients did have 24 h-seizure-clusters. There was no serious event. Less than 50% of the patients got a sufficient number of seizures for a conclusive result within 3 days. An increase in the registration period could increase the number of successful registrations.

Chronic Constipation

The lack of information about management of chronic constipation in children amidst general physicians has necessitated this review. A literature search in PubMed was conducted with regard to epidemiology, clinical features, investigation and management of chronic constipation in children. English language studies published over the last 20 y were considered and relevant information was extracted. Constipation is a common problem among children; the commonest cause is functional (95 %). An elaborate history and thorough physical examination are only essential things required to make a diagnosis of functional constipation. Management consists of disimpaction, followed by maintenance therapy with oral laxative, dietary modification and toilet training. A regular follow-up with slow tapering of laxative is the must for effective treatment. Early withdrawal of laxative is the commonest cause of recurrence.

Chronic Alcoholic Hallucinosis Masquerading as Schizophrenia

To the Editor: Alcoholic hallucinosis is a well-known phenomenon consisting of vivid auditory hallucinations that begin immediately following the reduction or cessation of alcohol consumption.1 The estimated prevalence of alcohol hallucinosis is 7.4% among alcohol-dependent inpatients,2 a subset of whom will develop chronic hallucinations that resemble paranoid schizophrenia.3 Here, we present a case of chronic alcohol hallucinosis previously diagnosed and treated as schizophrenia. Case report. Mr A, a 32-year-old man, was admitted recently to the inpatient psychiatry ward after several visits to the outpatient psychiatry service. Mr A complained of auditory pseudohallucinations that began in adolescence. These were described as 3 adult male voices with origin inside the head that are ego-dystonic and frequently attempt to persuade him to hurt himself. His hallucinatory experiences were identified during an inpatient stay for alcohol rehabilitation about 5 years earlier at another center. At the time of discharge, he was given the diagnosis of schizophrenia (DSM-IV-TR criteria) and was started on clozapine treatment. Mr A began drinking alcohol to excess at the age of 14 years, with self-report ranging from 7 to 48 standard drinks per day. Mr A smokes tobacco daily and cannabis occasionally and has been a heavy user of several other substances. Previously known treated diseases were unremarkable and stable. Mr A’s family history is significant for psychiatric illness, including mood disorders, substance use disorders, and suicide. There was no evidence of thought disorder. Results of physical examination and broad laboratory screens were unremarkable. Findings of noncontrast magnetic resonance imaging were similarly unremarkable. Outcomes of neuropsychological testing were remarkable for poor verbal memory, both immediate and delayed. A previous psychological report was obtained and noted third- to fifth-grade capabilities in reading, spelling, mathematics, and verbal comprehension as well as strong visuospatial ability. Clozapine therapy was ceased by slow taper. Mr A was monitored closely but was unable to notice any subjective changes. The nursing and medical staff were similarly unable to note any objective changes. Mr A was discharged 16 days following the cessation of clozapine therapy. He will be assessed for a therapeutic trial of a newer neuroleptic agent such as risperidone or lurasidone and anticraving medications such as acamprosate. Here, we present a unique case of what we believe to be persistent alcoholic hallucinosis occurring for many years. Our patient was initially diagnosed with schizophrenia; however, upon further inspection, we do not believe this to be the case. He has insight into his illness and has no evidence of thought disorder or negative symptoms, and his illness has not run the typical course of schizophrenia. Furthermore, he lacks the characteristic memory loss and confabulation typical of Korsakoff’s psychosis. Misdiagnosis resulted in exposing the patient to the risks of superfluous pharmacotherapy, in this case, metabolic syndrome, cardiac arrhythmias, and blood dyscrasia. We suggest that alcoholic hallucinosis should be suspected in patients who present with auditory hallucinations and significant alcohol use history and who are refractory to best-practice pharmacotherapy for schizophrenia.

AB0749 Immuglobuline G4 (IGG4) deposit disease and orbital pseudotumor as first manifestations of wegener’s granulomatosis (WG) in child

BackgroundWG is rare severe systemic disease characterized by necrotizing granulomatous vaculitis that primary affects the upper and lower respiratory tracts and kidneys. Immunoglobulin G4 (IgG4)-related disease is an evolving concept...

25 years after Prozac

Twenty-fi ve years ago, Eli Lilly’s Prozac (fl uoxetine) became the fi rst selective serotonin reuptake inhibitor (SSRI) to hit the market, where it quickly gained popularity for the treatment of depression. Soon, the drug was earning more than a billion dollars in revenues—at a time when the average American’s income was around $25,000. A quarter of a century later, treatments for depression are no more effective, but they are safer. Much has happened since the advent of fl uoxetine, but as many as half of patients still do not receive relief in the idealized situations studied in clinical trials. Why was fl uoxetine so successful? According to John Markowitz, PharmD, Professor of Pharmacy at the

A promising immunosuppressive strategy of cyclosporine alternately combined with levamisole is highly effective for moderate aplastic anemia

The appropriate management of patients with moderate aplastic anemia (mAA) remains to be unclear and controversial. A cohort of 118 patients with mAA received a novel immunosuppressive strategy of cyclosporine alternately combined with levamisole (CSA and LMS regimen), which included 42 newly diagnosed and 76 chronic (disease duration >6 months) cases. CSA and LMS regimen was orally administrated with the initial dose of CSA 3 mg/kg per day in adults or 5 mg/kg per day in children, and LMS 150 mg per day in adults or 2.5 mg/kg per day in children, continued for 12 more months after achieving maximal hematologic response, followed by a slow tapering. The overall response rates were of 100 and 86.8 % for newly diagnosed and chronic group, respectively. The 24-month progression-free survival were 95.2 % (95 % confidence intervals [CI], 85.9-100 %) and 93.6 % (95 % CI, 86.9-100 %) for newly diagnosed and chronic group, respectively (P = 0.50). The 2-year event-free survival for the patients in newly diagnosed group (86.6 %; 95 % CI, 70.4-100 %) was superior to that in chronic group (57.0 %; 95 % CI, 43.5-70.4 %, P = 0.001). To date, 11 patients relapsed and no patients evolved to clonal disorders. Thus, CSA and LMS regimen represents a promising immunosuppressive strategy for mAA.

Methadone at tapered doses for the management of opioid withdrawal.

The evidence of tapered methadone's efficacy in managing opioid withdrawal has been systematically evaluated in the previous version of this review that needs to be updated To evaluate the effectiveness of tapered methadone compared with other detoxification treatments and placebo in managing opioid withdrawal on completion of detoxification and relapse rate. We searched: Cochrane Central Register of Controlled Trials (The Cochrane Library 2012, Issue 4), PubMed (January 1966 to May 2012), EMBASE (January 1988 to May 2012), CINAHL (2003- December 2007), PsycINFO (January 1985 to December 2004), reference lists of articles. All randomised controlled trials that focused on the use of tapered methadone versus all other pharmacological detoxification treatments or placebo for the treatment of opiate withdrawal. Two review authors assessed the included studies. Any doubts about how to rate the studies were resolved by discussion with a third review author. Study quality was assessed according to the criteria indicated in the Cochrane Handbook for Systematic Reviews of Interventions. Twenty-three trials involving 2467 people were included. Comparing methadone versus any other pharmacological treatment, we observed no clinical difference between the two treatments in terms of completion of treatment, 16 studies 1381 participants, risk ratio (RR) 1.08 (95% confidence interval (CI) 0.97 to 1.21); number of participants abstinent at follow-up, three studies, 386 participants RR 0.98 (95% CI 0.70 to 1.37); degree of discomfort for withdrawal symptoms and adverse events, although it was impossible to pool data for the last two outcomes. These results were confirmed also when we considered the single comparisons: methadone with: adrenergic agonists (11 studies), other opioid agonists (eight studies), anxiolytic (two studies), paiduyangsheng (one study). Comparing methadone with placebo (two studies) more severe withdrawal and more drop-outs were found in the placebo group. The results indicate that the medications used in the included studies are similar in terms of overall effectiveness, although symptoms experienced by participants differed according to the medication used and the program adopted. Data from literature are hardly comparable; programs vary widely with regard to the assessment of outcome measures, impairing the application of meta-analysis. The studies included in this review confirm that slow tapering with temporary substitution of long- acting opioids, can reduce withdrawal severity. Nevertheless, the majority of patients relapsed to heroin use.

Prednisone on the threshold of rational use in the treatment of rheumatoid arthritis

This is a review of the evolution of the use prednisone in the treatment of rheumatoid arthritis (RA). Cortisone was introduced in 1949 and shortly thereafter, the Mayo investigators found that low divided doses with slow tapering were effective and caused fewer side effects. In 1959, a British double blind 2 year study of prednisolone treatment in early RA demonstrated effectiveness and reduced bony erosions. This experience was lost over time and empiricism and efforts to reduce side effects dominated practice for the next 35 years. Since 1995, a number of controlled studies of low single daily doses of prednisone in early RA have been reported by European investigators. They have shown clinical improvement, reduced bony erosions, augmentation of the effect of dmards and few side effects. During the last 25 years, the molecular actions of glucocorticoids have been elucidated. The time relationship of the dose to the biologic and clinical effects has been established. As a result of the information on the diurnal effect of glucocorticoids and the documentation of the effect occurring 5-6 hours after the dose and dissipating by 24 hours, a delayed release preparation of prednisone has been developed. With the rediscovery of the effectiveness of low single daily morning dose of prednisone in early RA by controlled studies and the demonstration of the onset and duration of the clinical effect of low dose of prednisone, it is now possible to use low doses of prednisone rationally and effectively in the treatment of RA. It remains to be determined whether a single morning, single evening or a twice a day low dose is the most effective and safe. It is doubtful if the new delayed release prednisone is any more effective than the usual immediate release prednisone if given at the same time.

Skin Graft-versus-host Disease Following Autologous Stem Cell Transplantation for Multiple Myeloma

Graft-versus-host disease (GVHD) is a common complication of allogeneic stem cell transplantation (allo-SCT). However, a similar syndrome has been reported in autologous stem cell transplantation (ASCT) as well. The target organs of GVHD in ASCT are the skin, liver and gastrointestinal (GI) tract, which are consistent with those in allo-SCT. Histologic findings from the skin and the mucosa of the GI tract also show similar features. Here we describe a case of autologous GVHD involving the skin of a patient who underwent ASCT for multiple myeloma. In this patient, the response to a total prednisone dose of 0.5 mg/kg/day was unsatisfactory, and the patient required more intensive and prolonged immunosuppressive therapy with slow tapering.

Hypereosinophilic syndrome induced cystitis mimicking bladder tumor

Hypereosinophilic syndrome induced cystitis mimicking bladder tumor

Autoimmune Enteropathy and Bullous Pemphigoid

Introduction: Autoimmune enteropathy (AIE) is a rare condition characterized by intractable diarrhea, malabsorption, villous atrophy and enterocyte autoantibodies. A predisposition to autoimmune diseases has been reported in association with AIE. We report the first case of a patient with AIE presenting with bullous pemphigoid. Case Report: A 67-year-old man with recently diagnosed bullous pemphigoid was referred for evaluation of chronic, large volume, watery diarrhea and a 30 lb weight loss. A gluten free diet and empiric courses of metronidazole, tetracycline and rifaxamin failed to improve the diarrhea. He denied abdominal pain, nausea, vomiting and fever. He had no allergies. His medications included esomeprazole 40 mg daily, prednisone 10 mg three times daily, olmesartan-hydrochlorothiazide 20/12.5 mg daily and cetirizine 10 mg daily. Physical examination was unremarkable except for large bullous lesions on the trunk and upper extremity. Laboratory investigations revealed low serum immunoglobulins. IgA was 78 mg/dl (82-453), IgG was 26 mg/dl (46-304) and IgM was 563 mg/dl (751-1560). Stool studies and serology for HIV, HLA-DQ 2 and HLA-DQ 8 were negative. Colonoscopy to the terminal ileum was normal, and random biopsies were negative for microscopic colitis. Esophagogastroduodenosocopy revealed erythema in the duodenum, and small bowel biopsies showed severe villous blunting, intraepithelial lymphocytes, increased crypt apoptosis and focal active inflammation. Anti-enterocyte antibodies were present on immunohistochemical stains. The bullous pemphigoid and diarrhea resolved with prednisone 60 mg daily after two weeks of therapy; however, diarrhea recurred with a steroid taper. Prednisone was restarted at 40 mg daily in addition to 6-Mercaptopurine 1.5/mg/kg daily. A slow steroid taper was performed over 3 months, and at 6 months follow-up, he is without diarrhea or skin lesions. Discussion: Chronic diarrhea, malabsorption, villous atrophy and enterocyte autoantibodies in the absence of celiac disease or a primary immunodeficiency are hallmarks of AIE. Predisposition to autoimmunity is well described in the literature; however, there are no reported cases of an association with bullous pemphigoid. Little is known regarding the epidemiology, natural history and therapeutic options for AIE. Treatment is primarily guided by anecdotal experience, and diarrhea tends to be unremitting without aggressive immunosuppresion. Steroid dependent patients require additional immunomodulators for induction and maintenance of remission. Infliximab, tacrolimus, azathioprine and cyclophosphamide have been used with varying success.

Horner syndrome as a manifestation of an exacerbation of Takayasu’s arteritis

Takayasu’s arteritis is a chronic, granulomatous, large vessel vasculitis affecting the aorta and its large branches, and occurring most commonly in young women. It is the third most frequently diagnosed vasculitis in childhood. Generally, symptoms reflect ischemia to an affected organ or limb. A small percentage of patients develop neurologic symptoms such as visual disturbances, transient ischemic attacks (TIA’s) or stroke. Horner syndrome is an unusual manifestation of Takayasu’s, with only a single reported case of an adult patient in whom Horner syndrome was the presenting manifestation of Takayasu’s arteritis. We present a 14 year old with a history of Takayasu’s arteritis who developed Horner syndrome during the course of the illness. Results Case report A 14 year old female was diagnosed at the age of 11 years with Takayasu’s arteritis after developing signs and symptoms of acute congestive heart failure while receiving fluid resuscitation for dehydration. She had a history of mild fatigue, decreased endurance, and symptoms of claudication. She was found to have complete occlusion of the superior mesenteric artery and involvement of the celiac, splenic, hepatic, right subclavian, and right renal arteries along with cardiomyopathy. She was treated with prednisone, methotrexate and infliximab with improvement in her symptoms, and stabilization of her cardiomyopathy without signs of progressive vasculitis on serial magnetic resonance imaging studies (MRI/MRA). Shewas in stable condition two years after her diagnosis, treated with infliximab every 6 weeks and methotrexate, when she developed left shoulder pain and left conjunctival injection. On ophthalmologic exam, she was noted to have episcleritis of the left eye as well as ptosis of her left upper eyelid and miosis of her left pupil without anhidrosis (Horner syndrome). An MRI/MRA of her brain and neck revealed stable narrowing of her right subclavian but no masses, no new aneurysms or stenoses, and no evidence of new overt inflammation. Her ESR was 16 mm/h and CRP 0.6 mg/dL (prior baseline 15-17 mm/h and 0.3-0.6 mg/dL, respectively). Given the lack of anhidrosis, her lesion was localized to the superior cervical ganglion. In the absence of other causes, and with the concurrent development of episcleritis, the Horner syndrome was presumed secondary to an exacerbation of vasculitis. Prednisone was started at 40mg orally once daily, followed by a slow taper, and infliximab and methotrexate were continued. Within 1 week the episcleritis resolved, and within 3 weeks, the ptosis and miosis had nearly completely resolved. Conclusion

Hepatitis C viral infection after liver transplantation.

Hepatitis C virus (HCV) is a leading cause of cirrhosis and hepatocellular carcinoma (HCC) and is the most common in- dication for liver transplantation (LT) in the United States and Europe. In contrast to other indications for LT, HCV re- currence after LT is nearly universal. Death and allograft fail- ure (much of it due to HCV recurrence) are more common in this population versus HCVrecipients 1 (Fig. 1). The nat- ural history of HCV disease is accelerated in the posttrans- plant setting and leads to cirrhosis in 10% to 25% of these patients within 5 to 10 years. 2 Once cirrhosis has developed, complications are common: the rate of decompensated dis- ease is greater than 40% within 1 year, and the survival rate 1 year after decompensation is less than 50%. 2 The delicate immune balance required to prevent both rejection and rapid HCV recurrence remains elusive, and measures to prevent and control recurrent HCV with antiviral therapy have been disappointing. In the future, strategies for eradicating the vi- rus in pre-LT and post-LT settings with novel therapeutics will be crucial to improving outcomes. nosuppression in recent years has been implicated in the lack of improvements in the posttransplant outcomes of HCV patients. In addition, the conclusive diagnosis of rejec- tion in the setting of HCV remains challenging. The treat- ment of acute rejection has been associated with diminished survival in HCV þ recipients but not in HCVrecipients. 7 Because the impact of immunosuppression on HCV recur- rence is most pronounced when high-intensity regimens are used to combat acute rejection, 8-11 mild acute rejection is now treated frequently via the modulation of the baseline immunosuppression without bolus steroids or anti-lympho- cyte antibodies. A recent study also suggested a negative impact of rapid fluctuations in immunosuppression as patients with slow prednisone tapering experienced a lower incidence of severe HCV recurrence at 1 year in comparison with historical controls (29% versus 48%). 12 Steroid-free reg-