Pulse Doses Of Methylprednisolone Research Articles

S2385 Hepatic Artery Vasculitis: Very Unique Presentation of a Single Organ Vasculitis

INTRODUCTION: Vasculitis usually has a systemic involvement. Rare cases of localized vasculitis have been described, but the exclusive involvement of the hepatic artery is exceptionally rare. We report the case of a patient who developed vasculitis of the right and left hepatic arteries as a very rare presentation of single organ vasculitis. CASE DESCRIPTION/METHODS: A 47-year-old Lady with no medical history was admitted with epigastric discomfort for a couple of weeks. The pain was sharp, localized and progressive. She reported bloating, nausea, and episodes of vomiting, but denied, fever, chills, melena, or other complaints. She was afebrile with stable vital signs and normal examination otherwise. There was minimal tenderness on deep palpation of the epigastrium and right upper quadrant without distension, guarding, or rigidity. Complete blood count and comprehensive metabolic panel including liver enzymes were normal. CRP and ESR were both elevated at 9.2 mg/dL and 62 mm/hr, respectively, but amylase and lipase were normal. CT scan showed diffuse mural thickening with luminal narrowing of the hepatic artery extending into the distal celiac trunk and proximal few millimeters of the splenic artery. These findings are most likely due to vasculitis. Tests for for anti-neutrophil cytoplasmic antibodies, ANA, RF, cryoglobulins, antiphospholipid antibodies, hepatitis B and C serology, HIV serology and the complement levels were all uneventful. Given these radiologic findings, elevated sedimentation rate, CRP, and the evaluation for systemic vasculitis, the patient was treated as isolated Hepatic artery vasculitis. She was started on pulse dose methylprednisolone intravenously for 3 days, after which her ESR and CRP normalized and her symptoms resolved. She was discharged home on prednisone which was later tapered off during clinic follow up. DISCUSSION: Vasculitis usually has a systemic involvement. The exclusive involvement of the hepatic artery of localized vasculitis is very rare, that’s why it’s mandatory to go through full work up for systemic vasculitis to be ruled out. Very few cases of localized vasculitis have been described, but the exclusive involvement of the hepatic artery is exceptionally rare. The outcome was favorable with corticosteroid therapy alone.Figure 1.: CT of the abdomen indicates the common hepatic artery with a thickened rind of soft tissue density and mild stranding in the adjacent fat.Figure 2.: Ct abdomen and pelvis coronal section, the red dot represent the area of hepatic artery with a thickening.Figure 3.: Abdomen and pelvis CT scan sagittal section, the red dot represent the area of hepatic artery with a thickening.

Mycoplasma pneumoniae-induced cerebral venous sinus thrombosis with autoimmune hemolytic anemia

Mycoplasma pneumoniae (MP) is a common organism causing pneumonia in school-going children. Although the disease is usually mild, it can rarely lead to severe extrapulmonary complications which may be life-threatening. We hereby report a case of an 8-year-old male child who presented with fever, cough, a maculopapular rash, and difficulty in breathing for 8 days who, after initially receiving treatment as community-acquired pneumonia with synpneumonic effusion, went on to develop severe autoimmune hemolytic anemia and cerebral venous thrombosis with features of vasculitis. With utilization of DNA polymerase chain reaction along with other laboratory parameters, the diagnosis of MP infection was made. The child was treated with oral clarithromycin, pulse dose methylprednisolone, low-molecular-weight heparin, and intravenous immunoglobulin while he also required mechanical ventilation, transfusions, and vasopressor support. He responded to these measures and survived with no neurological sequelae.

A case of hypereosinophilic syndrome with eosinophilic myocarditis secondary to nabumetone

Introduction: Hypereosinophilic syndromes are a group of disorders defined by an absolute eosinophil count greater than 1.5 × 109/L that leads to eosinophil-mediated end-organ damage. Cardiac involvement can range from asymptomatic inflammation of the myocardium to endomyocardial fibrosis and irreversible constrictive cardiomyopathy. Case Report: We present the case of a patient who presented with confusion and was found to have an absolute eosinophil count of 8.08 × 109/L as well as clinical findings consistent with eosinophilic myocarditis. It was determined that her clinical presentation was due to a new medication she had taken, nabumetone. Other causes of hypereosinophilia were ruled out. She improved with two days of pulse dose methylprednisolone and subsequent prednisone taper with complete resolution of her cardiomyopathy and normalization of her absolute eosinophil count. Conclusion: While there are no guidelines available for the treatment of eosinophilic myocarditis, corticosteroids should be considered in patients with evidence of cardiomyopathy in the setting of hypereosinophilia. Hypereosinophilic syndrome is an uncommonly recognized adverse event of non-steroidal anti-inflammatory drug (NSAID) therapy.

Pregnancy-related issues in women with multiple sclerosis: an evidence-based review with practical recommendations

Objective To review the current evidence regarding pregnancy-related issues in multiple sclerosis (MS) and to provide recommendations specific for each of them. Research design and methods A systematic review was performed based on a comprehensive literature search. Results MS has no effect on fertility, pregnancy or fetal outcomes, and pregnancies do not affect the long-term disease course and accumulation of disability. There is a potential risk for relapse after use of gonadotropin-releasing hormone agonists during assisted reproduction techniques. At short-term, pregnancy leads to a reduction of relapses during the third trimester, followed by an increased risk of relapses during the first three months postpartum. Pregnancies in MS are not per se high risk pregnancies, and MS does not influence the mode of delivery or anesthesia unless in the presence of significant disability. MRI is not contraindicated during pregnancy; however, gadolinium contrast media should be avoided whenever possible. It is safe to use pulse dose methylprednisolone infusions to manage acute disabling relapses during pregnancy and breastfeeding. However, its use during the first trimester of pregnancy is still controversial. Women with MS should be encouraged to breastfeed with a possible favorable effect of exclusive breastfeeding. Disease-modifying drugs can be classified according to their potential for pregnancy-associated risk and impact on fetal outcome. Interferon beta (IFNβ) and glatiramer acetate (GA) may be continued until pregnancy is confirmed and, after consideration of the individual risk-benefit if continued, during pregnancy. The benefit of continuing natalizumab during the entire pregnancy may outweigh the risk of recurring disease activity, particularly in women with highly active MS. GA and IFNβ are considered safe during breastfeeding. The use of natalizumab during pregnancy or lactation requires monitoring of the newborn. Conclusions This review provides current evidence and recommendations for counseling and management of women with MS preconception, during pregnancy and postpartum.

DIFFUSE ALVEOLAR HEMORRHAGE AS A RARE COMPLICATION OF SWEET SYNDROME

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Sweet’s syndrome (SS) is a rare inflammatory condition, characterized by fever, neutrophilia and skin lesions in the form of papules, nodules, plaques and rarely bullae. It typically occurs in middle age female, with around 70% of cases being idiopathic. While the disease may involve many organ systems, involvement of pulmonary system is rare (1,2). Systemic corticosteroids is the treatment of choice with favorable clinical outcomes. We present a case of sweet syndrome with pulmonary involvement presenting as diffuse alveolar hemorrhage. CASE PRESENTATION: Our patient is a 62 year old female who presented with Acute Hypoxemic respiratory failure secondary to Diffuse Alveolar Hemorrhage (DAH). An extensive Rheumatologic workup failed to indicate a possible etiology, until the skin biopsy returned positive for an intense neutrophilic infiltration of the epidermal layers of skin, consistent with Sweet Syndrome. A Lung biopsy was also negative for any vasculitis changes, while being suggestive of a diagnosis of Sweet Syndrome. She responded well to intravenous and oral corticosteroids, with full resolution of her respiratory and dermatological lesions. She was maintained on oral Prednisone in the outpatient setting with good clinical response. The patient then returned to our emergency department for new skin lesions. Initial physical exam was significant for purpuric papules on the dorsum of the left hand with ecchymosis and scattered bullae. The rest of the physical exam, laboratory investigations and imaging were within normal limits. The patient then developed hemoptysis. A chest x-ray revealed the development of left sided diffuse patchy opacities. The Patient was eventually intubated for acute hypoxemic respiratory failure. A Bronchoscopy revealed active bleeding from all left segmental bronchi. A skin biopsy showed pathological findings consistent with sweet syndrome (see figure), while being negative for vasculitis. Despite Pulse dose Methylprednisolone 1gram daily, and maximal supportive care , the progressed to develop refractory shock and Multi organ Failure, succumbing to her illness within the next 36 hours DISCUSSION: SS also known as acute febrile neutrophilic dermatosis is a rare inflammatory condition. It typically presents in three clinical settings: idiopathic or classic, malignancy associated and drug-induced sweet syndrome. Classic sweet syndrome commonly affects women between ages of 30 to 60, with no racial predilection. It is usually a multi system disease, with pulmonary involvement being rare, with symptoms ranging from flu like symptoms to acute respiratory distress syndrome. Most of patient responds well to prednisone. CONCLUSIONS: Our case highlights the importance of life threatening early recognition of the pulmonary manifestations in SS, and we emphasize the need for early and aggressive corticosteroid therapy to prevent severe respiratory compromise. Reference #1: Cohen PR. Sweet's syndrome--a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007;2:34. Published 2007 Jul 26. https://doi.org/10.1186/1750-1172-2-34 Reference #2: Villarreal-Villarreal CD1, Ocampo-Candiani J1, Villarreal-Martínez A2 Sweet Syndrome: A Review and Update. Actas Dermosifiliogr. 2016 Jun;107(5):369-78. https://doi.org/10.1016/j.ad.2015.12.001. Epub 2016 Jan 2 Reference #3: Vettakkara KMN, Banerjee S, Mittal A, et al. Not so sweet; severe Sweet's syndrome presenting as SIRS and pleural effusion. J Family Med Prim Care. 2018;7(6):1584-1587 DISCLOSURES: No relevant relationships by nargis jilani, source=Web Response No relevant relationships by Ravi Manglani, source=Web Response

3281 Management of Acute Rejection in Penile Allotransplantation

3281 Management of Acute Rejection in Penile Allotransplantation

Antibody-mediated pure red cell aplasia after treatment with darbepoetin-alpha postrenal transplantation

Pure red cell aplasia (PRCA) is a rare normochromic, normocytic anemia with a near-to-complete absence of erythroblasts in the bone marrow. Erythropoiesis-stimulating agents have been used widely to improve anemia in patients with chronic kidney disease. We present a case of a 38-year-old woman who was detected to have anemia (hemoglobin: 10 g/dl) and renal dysfunction (creatinine: 3 mg/dl) on a routine health check in December 2016. Her kidney biopsy showed chronic interstitial nephritis. She received intravenous (IV) iron and was started on darbepoetin-alpha. Her hemoglobin after 3 months of treatment was 12 mg/dl. She underwent preemptive one haplomatch renal transplantation on June 2017. She was discharged with serum creatinine of 0.9 mg/dl and hemoglobin of 8 g/dl. She was not on any Erthropoietin stimulating agents (ESA) at discharge. In the 4th week after transplant, her hemoglobin dropped to 7 g/dl, and she was restarted on darbepoetin-alpha 40 μg subcutaneously once a week. In the 6th week posttransplant, her hemoglobin dropped to 6 g/dl. Her peripheral smear showed normocytic normochromic anemia with no evidence of hemolysis. Transferrin saturation was 81%, serum ferritin levels were 1812 ng/ml, and reticulocyte count was 0.1%. Bone marrow biopsy showed suppression of erythroid precursors with myeloid-to-erythroid ratio of 8:1, adequate iron stores, and normal white blood cell and platelet precursors with no dysplastic cells. Paroxysmal Nocturnal hemoglobinuria (PNH) workup; antinuclear antibody; and screening for hepatitis B, hepatitis C, HIV, CMV, and human parvovirus B19 were negative. Anti-erythropoietin (EPO) antibody by enzyme-linked immunosorbent assay was positive. She was diagnosed to have PRCA secondary to anti-EPO antibodies. She was managed with pulse doses of methylprednisolone, adequate dose of tacrolimus, IV immunoglobulin of 125 g (2 g/kg) over 5 days, and two packed red cell transfusions. Darbepoetin-alpha was stopped. At present, her hemoglobin is 12.5 g/dl with serum creatinine of 1 mg/dl on triple immunosuppression with no requirement of ESA.

Multidimensional improvement in connective tissue disease-associated interstitial lung disease: Two courses of pulse dose methylprednisolone followed by low-dose prednisone and tacrolimus.

Corticosteroids and immunosuppressive agents are considered mainstays of therapy for connective tissue disease-related interstitial lung disease (CTD-ILD); however, tacrolimus with corticosteroid therapy has not been fully investigated. Our objectives were to examine the multidimensional therapeutic benefit and tolerability of the combined therapy for the initial treatment of patients with CTD-ILD. In this retrospective case series, we identified consecutive CTD-ILD patients treated with tacrolimus plus intravenous (i.v.) methylprednisolone (1000 mg i.v. 3 days a week for 2 weeks) followed by low-dose prednisolone (10 mg/day). We assessed the multidimensional therapeutic benefit and tolerability including lung physiology, exercise capacity, exercise oxygen desaturation, modified Medical Research Council (MMRC) and St George's Respiratory Questionnaire (SGRQ). A total of 26 ILD patients with the underlying CTD diagnoses included 11 with rheumatoid arthritis, 9 with dermatomyositis, 4 with Sjögren's syndrome and 2 others. From baseline to 12 months, the combined therapy significantly improved forced vital capacity (FVC; 77.8% to 94.6%, P < 0.001), diffusing capacity of the lung for carbon monoxide (DLCO ; 66.1% to 75.1%, P < 0.001), 6-min walk distance (6MWD; 530 to 568 m, P = 0.02), lowest oxygen saturation on pulse oximetry (SpO2 ; 85% to 89%, P = 0.01), MMRC (1.3 to 0.8, P = 0.01) and SGRQ (38 to 21, P < 0.001). During the study period, only one patient's therapy was discontinued due to an adverse event and none had a life-threatening adverse event attributed to the combined therapy. In our cohort of CTD-ILD, two courses of pulse dose methylprednisolone therapy followed by prednisone and oral tacrolimus appeared to be well tolerated, and to have multidimensional efficacy.

A case of Hashimotos encephalopathy failed to respond to steroids- A case report

A 52-year-old hypothyroid presented with episodes of seizures in last ten days with altered behavior. CT scan of brain showed cerebral atrophy and MRI of brain revealed non-specific changes. In EEG, there was slow wave pattern. CSF study showed increased cell count with all lymphocytes and raised protein with normal glucose. Anti- TPO Antibody was positive. All relevant investigations like HIV, HBsAg, Anti- HCV Ab, ANA (Hep2 method), ANA profile, P-ANCA, c-ANCA, VGKC (voltage gated potassium channel) Ab, NMDA Receptor/Anti- Glutamate Antibody) Ab, VDRL were negative. She was given pulse doses of methylprednisolone followed by maintenance prednisolone. Then her GCS improved followed by sudden deterioration. Repeat CSF showed marginal decrement of protein and cell count. Then she was given IV Immunoglobulin and patient improved. At discharge her CSF study normalized. Anti-TPO Ab titre came to normal level and EEG normalized. Hashimoto’s encephalopathy(HE) should be suspected in a case of sub-acute encephalopathy with high levels of anti-thyroid antibodies may be with normal thyroid functions. Here the patient did not have improvement on steroids. The patient needed immunoglobulin to improve.

A Successfully Treated Case of Recurrent Focal Segmental Glomerulosclerosis (FSGS) with Plasmapheresis and High dose Methylprednisolone Pulse Therapy

Focal segmental glomerulosclerosis (FSGS) in children, which is a kind of nephrotic syndrome showing steroid resistance, usually progresses to a substantial number of end stage renal disease (ESRD). Although the pathogenesis of primary FSGS is unclear, several recent studies have reported that FSGS is associated with circulating immune factors such as soluble urokinase-type plasminogen activator receptor (suPAR) or anti-CD40 autoantibody. We report a successfully treated case of a 19-year-old female patient who experienced a recurrence of primary FSGS. After the diagnosis of FSGS, the patient progressed to ESRD and received a kidney transplantation (KT). Three days later, recurrence was suspected through proteinuria and hypoalbuminemia. She has been performed plasmapheresis and high dose methylprednisolone pulse therapy and shown remission status without increasing proteinuria for four years after KT. In conclusion, strong immunosuppressive therapy may be helpful for a good prognosis of recurrent FSGS, suppressing several immunologic circulating factors related disease pathogenesis. Key words: Focal segmental glomerulosclerosis (FSGS); End stage renal disease (ESRD); Soluble urokinase-type plasminogen activator receptor (suPAR); Anti-CD40 autoantibody

A Rare Presentation of Acute Rhomboencephalitis and Cervical Myelitis Secondary to Systemic Lupus Erythematosus

SESSION TITLE: Nervous System Disorders in the ICU 2 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Neuroligic manifestations of Lupus requires a prompt diagnosis in initiation of therapy. We present a rare case of Lupus associated with rhomboencephalitis and cervical myelitis. In literature review there has not been a documented case of similar presentation as well as recommendations regarding appropriate treatment protocol. CASE PRESENTATION: This is a case of a 45 year old female with a history of Rheumatoid Arthritis managed on chronic steroids. She was diagnosed four years prior to admission and had abruptly stopped taking her prednisone 6-8 weeks prior to her admission. She was admitted for progressive dizziness, weakness, decreased appetite along with a weight loss of 20 lbs. She reported night sweats, cough with sputum, and episodes of diarrhea. Her initial laboratory workup revealed pancytopenia, mild transaminitis, and UTI. She was started on antibiotics to cover for the UTI and Clostridium difficile colitis. CT imaging only revealed splenomegaly. Bone marrow biopsy was performed due to concern of underlying malignancy which was negative. Over the next few days, the patient developed an unsteady gait and had two falls, along with extremity weakness. MRI brain was emergently done which shows increased signal Flair and T2 sequences involving the posterior right and left pons, midbrain, cerebellar peduncles, and the cervicomedullary cord. She later developed respiratory failure and was intubated for airway protection.The patient was then started on cyclophosphamide and pulse dose methylprednisolone. Amphotericin B was empirically started due to concern of neuro-histoplasmosis. All cultures were negative, autoimmune work up showed hypocomplementemia, positive antinuclear antibody 1:1280, and positive dsDNA. GM-1Ab was also negative. Repeat urinalysis was positive for 8.5 grams proteinuria with + RBC and blood. Due to lack of clinical response, daily plasmapheresis was implemented for a course of 5 days after which the patient clinically improved and was successfully weaned from mechanical ventilation. Repeat MRI imaging showed dramatic reduction of signal abnormality although some involvement was present. DISCUSSION: Although rhomboencephalitis is common in infectious and autoimmune conditions, involvement of brainstem and cervical spinal cord is quite rare, especially with a confirmed diagnosis of Lupus. CONCLUSIONS: Prompt diagnosis and initiation of immunosupressive therapy and plasmapheresis is effective in treating even rare neurologic manifestations of lupus. Reference #1: None DISCLOSURE: The following authors have nothing to disclose: Kunal Patel, Lavanya Srinivasan No Product/Research Disclosure Information

Relationship between Autoimmune Diseases and Imbalances in Helper/Suppressor T-cell Populations after Rituximab-containing Chemotherapyfor Non-Hodgkin Lymphoma

Immunologic abnormalities including autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP) have been described in patients with non-Hodgkin lymphoma. In some cases, anti-red cell or platelet antibodies are produced by lymphoma cells. However, cases of the occurrence of autoimmune diseases without recurrence of lymphomas have been also reported. The relationship between autoimmune diseases with post-bone marrow transplantation and Hodgkin disease may be attributed to immune dysfunctions, particularly those involving T cells. This autoimmune phenomenon may be related to imbalances in helper/suppressor T-cell populations. Imbalances in helper and suppressor T-cell populations have been reported after R-CHOP chemotherapy, and the recovery of serum IgG and CD4+ counts was observed more than 2 years after R-CHOP therapy in patients with B-cell lymphoma. In this report, we present a case of an 87-year-old man who was treated with rituximab-containing chemotherapy and maintained complete remission. However, the immunophenotyping of peripheral blood and bone mallow mononuclear cells revealed a reversed CD4/CD8 ratio. Two years later, he developed ITP. He was treated with intravenous immunoglobulin and eltrobopag, and thrombocytopenia improved. Five years later, he developed pneumonia and sudden Coombs-positive hemolytic anemia caused by autoantibodies against D antigen and thrombocytopenia without the recurrence of lymphoma. He was treated with prednisolone and a pulse dose of methylprednisolone; however, his response to therapy was poor and he subsequently died. We herein report a case who have been showed reversed imbalances in the helper/suppressor T cell populations over 2 years after R-CHOP therapy, developed AIHA and ITP without recurrence of lymphoma. The long-term monitoring of T-cell counts after rituximab containing chemotherapies is important, and careful attention to infection signs.

Drusen Formation in Type II Membranoproliferative Glomerulonephritis after Renal Transplantation

Abstract Type II membranoproliferative glomerulonephritis (MPGN) is a systemic disease that almost invariably recurs in renal allografts. This is a case of a 45-year-old woman with biopsy proven type II MPGN that led to renal failure 10 years after diagnosis. During the fifth month after cadaveric transplantation she was treated with pulse doses of methylprednisolone owing to acute T-cell mediated rejection without pathohistological signs of type II MPGN recurrence. One month later the patient was hospitalized due to acute bilateral vision deterioration. Ophthalmoscopy showed bilateral, multifocal drusen, concentrating in the posterior pole, and exudative ablation of the retinal pigment epithelium. Ocular coherence tomography (OCT) revealed focal retinal pigment epithelial elevation and detachments. The patient was treated with methylprednisolone (1 mg/kg) for 3 days. Therapy led to regression of exudation and flattening of the pigment epithelial detachments with discrete subjective visual improvement. Type II MPGN almost invariably recurs and leads to graft failure in 50% of cases. Our patient had evident chronic eye changes due to type II MPGN leaving allograft function intact during the first year of follow-up. Considering these potentially devastating effects of the disease, type II MPGN patients should be observed carefully from both the renal and eye point of view, because the severity of ocular changes, like in our case, is not always in line with allograft function.

36.: A rare case of central nervous system vasculitis requiring infliximab to achieve remission

Granulomatous vasculitis of the central nervous system (CNS) is an uncommon illness characterised by sporadic small to medium sized vessel involvement of the meninges, brain and spinal cord. It presents with CNS dysfunction and is often difficult to diagnose due to a wide range of differential diagnoses and occasionally non-definitive investigations. The prognosis is poor unless long term remission is achieved via immunosuppression. We illustrate a rare case of granulomatous vasculitis of the CNS where the patient failed conventional first and second line immunosuppressive therapies, eventually achieving remission using the tumour necrosis factor (TNF)-α blocker, infliximab. A previously well 34-year-old man first presented in 2009 with ataxia and nausea, accompanied by lethargy/malaise. Initial MRI of the brain revealed diffuse signal abnormality within the white matter of the cerebellum as well as a patchy, diffuse nodular leptomeningeal enhancement in the posterior fossa. Cerebrospinal fluid examination was abnormal with elevated protein of 1.7 g/L, approximately 100 mononuclear cells, polymerase chain reaction was negative for tuberculosis and herpes simplex virus, and glucose/cytology was unremarkable. Screening tests for systemic vasculitis were unremarkable including an angiotensin-converting enzyme level of 16. Formal angiogram showed no evidence of vasculitis, however a leptomeningeal biopsy revealed a granulomatous vasculitis with no specific diagnostic features. Typical therapies such as cyclophosphamide, pulse dose methylprednisolone, methotrexate, azathioprine, mycophenolate and rituximab all failed. Remission was eventually achieved with a combination of infliximab and oral prednisolone. This case adds further to a small number of case reports where TNF-α blockade has been used effectively to treat multi drug resistant vasculitis of the CNS. Despite the fact that randomised control trial data is lacking in this subject due to the low incidence of the disease and difficulties in diagnosis, TNF blockade appears a viable option in treatment resistant cases.

Anti-gbm Rapidly Progressive Glomerulonephritis (Syndroma Goodpasture): A Case Report

Abstract Goodpasture syndrome is a severe illness caused by the formation of antibodies to the glomerular basement membrane and alveolus with consequential damage to renal and pulmonary function. With current therapy, long-term survival is more than 50%. Before, the mortality was higher than 90%. In our patient, the disease began as dysuria, continued as anaemic syndrome, and ended with the development of end-stage renal failure. Immunosuppressive therapy with pulse doses of methylprednisolone and cyclophosphamide has put the disease into remission, but the permanent impairment of renal function remained. Early diagnosis of Goodpasture syndrome helps preserve renal function and improves patients’ survival. In patients who achieve remission, a kidney transplant can be considered. Currently, our patient is awaiting transplantation

Practical Applications of New Frontiers in Risk and Asset Allocation

<h3>Objective:</h3> To explore various post covid immune mediatedneurological manifestations in children <h3>Background:</h3> The neurological manifestation following a SARS-CoV2 infection is varied and till now there are only few studies reported regarding the same. Our study aimed to identify the varied spectrum of neurological manifestation following SARS-CoV2 infection. <h3>Design/Methods:</h3> Retrospective data were collected from May 2021 to September 2021, including all children aged from 1month to 18 years of age who presented to our pediatric emergency or OPD (a tertiary care center from western Rajasthan, India) with the neurological manifestation with history of COVID-19 infection or exposure and positive SARS-CoV-2 serology. Those who are RT PCR positive were excluded. The neurological manifestations were further categorized in a pre-designed proforma. <h3>Results:</h3> Case records of the 18 children who fulfilled the criteria were included in the study, among them 7 (38.8%) were male and 11 (61.1%) were female. Predominant presentation in our study group was seizures (6/18) and Gullian Barre Syndrome (5/18). Other manifestations included stroke (2/18), ADEM (1/18), MS (1/18), LETM (1/18), Autoimmune encephalitis[NMDAR](2/18). In our study group, 13/18 (72.2%) required immunomodulatory therapy either IVIG or high dose methylprednisolone pulse therapy. Steroids were used upfront in patients with elevated inflammatory markers. Cerebrovascular complications in children were less common compared to adults. Most of the children had favourable outcomes except for one mortality in our cohort. <h3>Conclusions:</h3> Delayed complications following SARS-CoV2 infection with varied manifestations are seen in children. A temporal correlation between the COVID 19 infection and the increasing number of neurological cases after the second wave was noted. Steroids are beneficial while treating such patients. Testing for SARS-CoV2 serology during the pandemic can give a clue to the underlying etiology. However, further studies are required to understand the CNS effects of SARS-CoV2 infection in children. <b>Disclosure:</b> Dr. Saini has nothing to disclose. Dr. Krishna has nothing to disclose. Sarbesh Tiwari has nothing to disclose. Dr. Khera has nothing to disclose. Dr. KUMAR has nothing to disclose. Dr. Goyal has nothing to disclose. Dr. Choudhary has nothing to disclose. Prof. Singh has nothing to disclose.

Tacrolimus-related adverse effects in liver transplant recipients: its association with trough concentrations.

Tacrolimus is an important immunosuppressant administered to patients following liver transplantation (LT), with a recommended trough concentration of 8 to 11 ng/mL to prevent allograft rejection. We retrospectively examined our data to identify the tacrolimus trough concentration that combined efficacy with minimal adverse effects. The case records of LT recipients, who were nondiabetic, nonhypertensive, and with normal renal parameters prior to LT were retrospectively examined for acute cellular rejection (ACR) episodes and three major adverse effects of tacrolimus, i.e. neurotoxicity, nephrotoxicity, and new onset diabetes mellitus (NODM). Thirty-two LT recipients fulfilled the criteria for the study. The mean (±SD) tacrolimus level for the 290 troughs (after 10 days) was 8.5 ± 3.8 ng/mL. At 10 days, 1 month, 3 months, and 6 months, the trough values were 7.3 ± 2.9, 9.7 ± 3.4, 7.9 ± 3.3, and 7.6 ± 2.6 ng/mL, respectively. The mean time taken for stabilization of the blood pressure and biochemical parameters was 7 ± 2 days. Overall, a trough window with the least adverse effect was 7 to 7.9 ng/mL. Neurotoxicity was least in the trough range 5 to <8 ng/mL. Symptoms included headache in four, tremors in three, seizure in one, confusion and psychosis in two, and combination in three. Nephrotoxicity was least in trough 8 to <11 ng/mL. One patient progressed to chronic kidney disease at 6 months. NODM was present in 11 % to 18 % across the various trough range, including the extremes (mean trough level, 8.4 ± 4.4 ng/dL). At 6 months, five recipients were on treatment for NODM. Three recipients developed ACR, two within the first month and one at 7 weeks. The trough levels were 8.5, 9, 15.2 ng/mL, respectively. All recovered with three pulse doses of methylprednisolone. Tacrolimus concentration of 5 to <8 ng/mL was associated with least overall toxicity, neurotoxicity, and ACR.

Anti-GBM rapidly progressive glomerulonephritis (Syndroma Goodpasture): A case report

Goodpasture syndrome is a severe illness caused by the formation of antibodies to the glomerular basement membrane and alveolus with consequential damage to renal and pulmonary function. With current therapy, long-term survival is more than 50%. Before, the mortality was higher than 90%. In our patient, the disease began as dysuria, continued as anaemic syndrome, and ended with the development of end-stage renal failure. Immunosuppressive therapy with pulse doses of methylprednisolone and cyclophosphamide has put the disease into remission, but the permanent impairment of renal function remained. Early diagnosis of Goodpasture syndrome helps preserve renal function and improves patients’ survival. In patients who achieve remission, a kidney transplant can be considered. Currently, our patient is awaiting transplantation

Tacrolimus related adverse effects in liver transplant recipients: Its association with trough concentrations

Background and Aims: Tacrolimus is an important immunosuppressant used in post liver transplantation (LT). A tacrolimus trough of 8 to 11 ng/mL is considered to be ideal for LT recipients to prevent allograft rejection. The aim of the study was to propose a suitable tacrolimus trough concentration, with minimal adverse effects. Methods: The study group consisted of LT recipients, who were non diabetic, non hypertensive and with normal renal parameters prior to LT. All patients were on standard immunosuppressive regimen. The study retrospectively looked into the acute cellular rejection (ACR) episodes amongst the LT recipients and three major adverse effects of tacrolimus i.e. neurotoxicity, nephrotoxicity and new onset diabetes mellitus (NODM). Results: There were 32 LT recipients who fulfilled the criteria for the study. The mean tacrolimus trough level for the 290 troughs (after 10 days) was 8.5±3.8 ng/mL. At 10 days, one month, three months and six months, the trough values were 7.3±2.9, 9.7±3.4, 7.9±3.3 and 7.6±2.6 ng/mL respectively. The mean time taken for stabilization of the blood pressure, and biochemical parameters was 7 + 2 days. Overall, a trough window with the least adverse effect was 7 to 7.9 ng/mL. Neurotoxicity was least in the trough range 5 to <8 ng/mL. Symptoms included headache in 4, tremors in 3, seizure in one and confusion and psychosis in 2 and combination in 3. Nephrotoxicity was least in trough 8 to 11 <ng/mL. One patient progressed to chronic kidney disease at 6 months. NODM was present in 11% to 18% across the various trough range, including the extremes (mean trough level 8.4 ± 4.4 ng/dL). At 6 months, 5 recipients were on treatment for NODM. Three recipients developed ACR: 2 within the first month and one at 7 weeks; the trough levels were 8.5 ng/mL, 9 ng / mL, 15.2 ng/mL respectively. All recovered with three pulse doses of methylprednisolone. Conclusions: Tacrolimus concentration of 5 to < 8 ng/mL is optimal for minimizing neurotoxicity and ACR and a trough of 8 to < 11 for nephrotoxicity.

Anti HBs antibody level in response to hepatitis B immunoglobulin (HBIG) prophylaxis in liver transplant recipients

S 21 ANNUAL CONFERENCE—2013 Lver ra n sp la n ta tio n including the extremes (mean trough level 8.4 4.4 ng/ dL). At 6 months, 5 recipients were on treatment for NODM. Three recipients developed ACR: 2 within the first month and one at 7 weeks; the trough levels were 8.5 ng/ mL, 9 ng / mL, 15.2 ng/mL respectively. All recovered with three pulse doses of methylprednisolone. Conclusions: Tacrolimus concentration of 5 to 10 kg on mean follow up of 30 months. Conclusion: Pediatric LDLT is well established in India with results comparable to the best centers in world.