Including Steroid Therapy Research Articles

AN ATYPICAL CASE OF INFECTIOUS MONONUCLEOSIS – CASE REPORT

Aims: Infectious mononucleosis, caused by the Epstein-Barr virus (EBV), typically presents with fever, exudative pharyngitis and lymphadenopathy. However, atypical presentations can complicate diagnosis and treatment. The objective of this paper is to illustrate the atypical progression of infectious mononucleosis and to delineate the differential diagnosis that should be considered. Additionally, it outlines the therapeutic approaches for managing symptoms associated with the disease. Case Description: A 25-year-old woman presented at a GP's office with eyelid edema, fever, and significant weight loss. Initial tests and treatments were inconclusive, leading to further investigations. Despite typical treatment for viral infections, the patient’s condition worsened, displaying dyspnoea, acute hepatitis, rash, significantly elevated ESR, hemolytic anemia, leukopenia, and thrombocytopenia. Hospitalization was required for comprehensive care, including steroid therapy and fluid management. Conclusions: This case highlights the diagnostic challenges posed by atypical presentations of infectious mononucleosis and underscores the importance of considering EBV infection in the differential diagnosis. Early recognition and appropriate management are crucial for optimal patient outcomes.

Dipnech: A Rare Cause of Slow-Growing Pulmonary Nodules in a Dyspnoeic Patient with a History of Breast Cancer.

Clinical symptoms of diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) are common to several other respiratory conditions that are found ubiquitously in the community; being aware of this rare condition will help in reaching a diagnosis in a timelier manner.Several potential treatments are described in the literature including steroid therapy, cytotoxic agents and somatostatin analogues, which despite their efficacy have not been demonstrated in studies; however, a small number of case reports such as this one showed an improvement in symptomatology with this treatment.

COVID-19-Associated Cerebellitis: A Case Report and Rehabilitation Outcome.

The COVID-19 pandemic has brought attention to neurological complications, including cerebellitis, characterized by inflammation of the cerebellum. Despite its rare occurrence, cerebellitis has been associated with COVID-19 infection, albeit the pathogenic mechanisms remain unclear. We present the case of a 22-year-old male with acute onset ataxia and dysarthria during a SARS-CoV-2 infection. Diagnostic evaluations ruled out other causes, confirming cerebellitis. Treatment included steroid therapy, vitamin supplementation, physiotherapy, and intravenous immunoglobulins. Rehabilitation focused on enhancing balance, coordination, and daily activities. The patient showed significant improvement in functional abilities, with increased autonomy in daily activities and improved ambulation. Despite persistent mild symptoms, the multidisciplinary rehabilitation approach led to remarkable progress. This case underscores the importance of recognizing and managing neurological complications, such as cerebellitis, in COVID-19 patients. A comprehensive approach combining medical treatment and rehabilitation is essential for optimizing outcomes. Further research is needed to elucidate the pathogenesis and optimal management strategies for such complications.

Role of transduodenal endoscopic ultrasound-guided fine-needle aspiration/biopsy (EUS-FNA/FNB) for diagnosis of retroperitoneal fibrosis (Ormond's disease).

Retroperitoneal fibrosis (RPF), often referred to as Ormond's disease when it is of idiopathic origin, is a rare disease characterized by the presence of inflammatory infiltrates and periaortic masses in the retroperitoneum. For a definite diagnosis, a biopsy and subsequent pathological examination is required. Currently accepted methods for retroperitoneal biopsy include open, laparoscopic, or CT-guided approaches. However, transduodenal endoscopic ultrasound-guided fine-needle aspiration/biopsy (EUS-FNA/FNB) for diagnosis of RPF has attracted only little attention in the literature. We report two male patient cases who presented with leukocytosis, elevated C-reactive protein, and a suspicious retroperitoneal mass of unknown origin on computed tomography. One patient also reported left lower quadrant pain, whereas the other patient suffered from back pain and weight loss. In both patients, idiopathic RPF was successfully diagnosed by using transduodenal EUS-FNA/FNB with 22- and 20-gauge aspiration needles. Histopathology revealed dense lymphocytic infiltrates and fibrosis. The procedures lasted approximately 25 and 20 minutes, respectively, and in both patients no serious adverse events occurred. Treatment included steroid therapy and administration of Azathioprine. We demonstrate that using EUS-FNA/FNB to diagnose RPF is a feasible, fast, and safe method, which should always be considered as a first-line diagnostic modality. Hence, this case report emphasizes that gastrointestinal endoscopists are likely to play an important role in the setting of suspected RPF.

Associations between vitamin D, bone mineral density, and the course of inflammatory bowel disease in Polish patients.

There are various factors contributing to the pathogenesis of osteoporosis in inflammatory bowel disease (IBD), including steroid therapy, malnutrition, and vitamin D deficiency. The study aimed to assess the vitamin D level among IBD patients and to investigate the relationship between vitamin D concentration and bone mineral density (BMD). The study participants included 239 adult patients with IBD and a control group of 45 healthy adults. Densitometric measurements of the lumbar spine (L1-L4) and femoral neck (FN) were conducted using dual‑energy X‑ray absorptiometry. All patients completed a questionnaire referring to vitamin D supplementation. Significant differences were observed with regard to the body mass, body mass index, BMD, the Z‑score, and the T‑score of the FN and L1-L4. Only approximately 25% of all participants presented optimal or high concentrations of vitamin D. The research revealed no differences in vitamin D levels with regard to the disease extent and severity among the patients with ulcerative colitis. No differences were observed in terms of the disease localization, behavior, and the patient age at the time of diagnosis in the patients with Crohn disease. Furthermore, no differences were found in BMD, T‑score, and Z‑score of the FN and L1-L4 between the group of patients who supplemented and did not supplement vitamin D. Vitamin D may not be the only factor affecting BMD. Patients with IBD should supplement a higher dose of vitamin D than healthy adults.

Kawasaki-multisystem inflammatory syndrome in children in the delayed period of coronavirus infection (covid-19): modern state of the problem and possible new approaches to treatment (plasmapheresis)

COVID-19 infection usually occurs in children in a mild form, but some of them in a delayed period (one or several weeks after acute infection with COVID-19) may develop a severe inflammatory disease with clinical manifestations similar to toxic shock syndrome (Kawasaki disease), classified as multisystem inflammatory syndrome in children (MISC). It is possible that the syndrome has only a temporary connection with the COVID-19 infection. In the future, new associations of such clinical manifestations with other infectious (or non-infectious) diseases may appear. But currently, all children in the described cohorts with MISC have an association with COVID-19 infection. It is believed that the syndrome is initiated by an excessive adaptive immune response with the formation of autoantibodies. Treatment is based on anti-inflammatory, including steroid therapy, the possible use of intravenous immunoglobulin, aspirin, interleukin 1 and 6 receptor antagonists. The article analyzes current views on Kawasaki-multisystem inflammatory syndrome in children in the delayed period of COVID-19 coronavirus infection in the aspects of diagnosis, pathogenesis, clinical manifestations (with a discussion of foreign and Russian studies) and approaches to therapy and possible prevention, including the possibility of using plasmapheresis in complex therapy.

Acute Invasive Fungal Rhinosinusitis and Coronavirus Disease 2019.

Acute invasive fungal rhinosinusitis (mucormycosis) is a rare, highly fatal disease. This opportunistic fungal infection causes angioinvasion and ischemic tissue necrosis. It mainly affects immunocompromised patients. Since the coronavirus disease 2019 (COVID-19) outbreak, many case reports have described the rhino-orbital-cerebral mucormycosis associated with COVID-19. However, the underlying predisposing factors are unknown. Several factors, other than diabetes, which is the most well-known contributing factor, may be involved in causing this severe fungal infection in COVID-19 patients. These factors may include steroid therapy, which is being used in severely dyspneic patients, the use of broad-spectrum antibiotics that may cause fungal flare-ups, and hospitalization with possible nosocomial infections. In addition, increased serum ferritin levels, possible endothelial damage, and pancreatic islets affection among COVID-19 patients may be implicated. Head and neck surgeons should be aware of the increasing prevalence of craniofacial mucormycosis among COVID-19 patients, as early diagnosis and prompt treatment are essential to improve the outcomes.

Current diagnosis and treatment of vestibular neuritis: a narrative review.

Vertigo is the sensation of self-motion of the head or body when no self-motion is occurring or the sensation of distorted self-motion during an otherwise normal head movement. Representative peripheral vertigo disorders include benign paroxysmal positional vertigo, Ménière disease, and vestibular neuritis. Vestibular neuritis, also known as vestibular neuronitis, is the third most common peripheral vestibular disorder after benign paroxysmal positional vertigo and Ménière disease. The cause of vestibular neuritis remains unclear. However, a viral infection of the vestibular nerve or ischemia of the anterior vestibular artery is known to cause vestibular neuritis. In addition, recent studies on immune-mediated mechanisms as the cause of vestibular neuritis have been reported. The characteristic clinical features of vestibular neuritis are abrupt true-whirling vertigo lasting for more than 24 hours, and no presence of cochlear symptoms and other neurological symptoms and signs. To accurately diagnose vestibular neuritis, various diagnostic tests such as the head impulse test, bithermal caloric test, and vestibular-evoked myogenic potential test are conducted. Various treatments for vestibular neuritis have been reported, which are largely divided into symptomatic therapy, specific drug therapy, and vestibular rehabilitation therapy. Symptomatic therapies include generalized supportive care and administration of vestibular suppressants and antiemetics. Specific drug therapies include steroid therapy, antiviral therapy, and vasodilator therapy. Vestibular rehabilitation therapies include generalized vestibular and customized vestibular exercises.

The Clinical and Imaging Characteristics Associated With Neurological Sequelae of Pediatric Patients With Acute Necrotizing Encephalopathy.

Background: Acute necrotizing encephalopathy of childhood (ANE) is a rare but rapidly progressing encephalopathy. Importantly, the exact pathogenesis and evidence-based treatment is scarce. Thus, we aimed to identify the clinical, imaging, and therapeutic characteristics that associated with prognosis of pediatric ANE patients.Methods: A retrospective study was conducted on pediatric patients with ANE who were admitted to Wuhan Children's Hospital between January 2014 and September 2019. All cases met the diagnostic criteria for ANE proposed by Mizuguchi in 1997. The clinical information and follow-up data were collected. The prognostic factors were analyzed by trend chi-square test and Goodman–Kruskal gamma test.Results: A total of 41 ANE patients ranging in age from 8.9 to 142 months were included in this study. Seven cases (17%) died, and the other 34 survivors had different degrees of neurological sequelae. Factors tested to be significantly correlated with the severity of neurological sequelae were the intervals from prodromal infection to acute encephalopathy (G = −0.553), conscious disturbance (r = 0.58), endotracheal intubation (r = 0.423), elevation of alanine aminotransferase (r = 0.345), aspartate aminotransferase (r = 0.393), and cerebrospinal fluid protein (r = 0.490). In addition, dynamic magnetic resonance imaging (MRI) evaluation on follow-up revealed that the total numbers of brain lesion location (χ2 = 6.29, P < 0.05), hemorrhage (r = 0.580), cavitation (r = 0.410), and atrophy (r = 0.602) status were significantly correlated with the severity of neurological sequelae, while early steroid therapy (r = −0.127 and 0.212, respectively) and intravenous immunoglobulin (IVIG) (r = 0.111 and −0.023, respectively) within 24 h or within 72 h after onset showed no association.Conclusions: Intervals from prodromal infection to acute encephalopathy (≤1 day), total numbers of brain lesion location (≥3), the recovery duration of hemorrhage and atrophy (>3 months), and the presence of cavitation predict severe neurological sequelae in pediatric patients with ANE. Early treatments, including steroid therapy and IVIG, had no correlation with better outcomes. Further studies are needed to establish a consensus guideline for the management of ANE.

2740 IgG₄-Related Disease: A Gastric Mass With an IgG4 Surprise

INTRODUCTION: IgG₄-related disease is a rare autoimmune syndrome that causes fibrosis of glands, most commonly of the pancreas and biliary tract1,3. The overall prevalence is unknown, with an estimated less than 1:100,000 of the general population typically found in middle-aged males2,3. Diagnosis is made with IgG₄ levels at least 2x the upper limit of normal and dense lymphoplasmacytic infiltrate, however, it may be seen with normal IgG₄ levels6. Although rare, it can present similar to GI malignancies and should be considered in the differential for patients with autoimmune disease1,3,4,5. Here we present one of those unusual cases. CASE DESCRIPTION/METHODS: A 65-year-old lady with PMH of DM2 and Sjogren's Syndrome (SS) who presented to the hospital with intractable nausea, vomiting, and failure to thrive for over 1 month. The physical exam, labs that included CMP, LFTs, and serology was unremarkable; previous EGD revealed diffuse thickening of the gastric submucosa. Biopsy via open gastrectomy revealed chronic inflammation with multiple IgG₄ plasma cells and no signs of lymphoma, suspicious for IgG₄-RD. After the partial gastrectomy, serum Na levels were 162, which improved with DDAVP and was later confirmed to be diabetes insipidus (DI). Further workup and imaging were negative and baseline IgG and IgG₄ levels were 1178 and 61, respectively. Treatment included steroid therapy and DDAVP which improved her symptoms and preceded an uneventful hospital course. DISCUSSION: IgG₄-RD is a disease with numerous presentations based on the organs involved. It presents as a GI-specific illness due to its affinity for the pancreas and biliary tract1,2,3. As seen in our patient, although rare, it may also affect the stomach, form mass lesions and cause symptomatology similar to a GI malignancy3,5. In patients with autoimmune diseases, where GI malignancy is considered, it is reasonable to include IgG₄-RD in the differential. IgG₄-RD may mimic symptoms of SS, and therefore case-patients with SS should also be considered for IgG₄-RD. This patient also experienced DI which may be a manifestation of IgG₄-RD, as the syndrome may affect the pituitary4. This case is one of the few instances reported to have pituitary involvement after the onset of multiorgan involvement4. Like other autoimmune disorders, IgG₄-RD is responsive to steroid therapy or rituximab. Therefore, a timely diagnosis can help slow down or prevent symptomatic manifestations, as seen in the case. Clinicians should be aware of this clinical presentation.

Low Incidence of Allergic Fungal Rhinosinusitis in Japanese Patients.

Background:Allergic fungal rhinosinusitis (AFRS) is a noninvasive fungal disease of the sinuses with a very high recurrence rate. A very small number of Japanese cases have been reported.Material and methods:The subjects were 6 patients with AFRS out of 429 patients who underwent endoscopic sinus surgery at Kagawa Rosai Hospital between December 2011 and November 2017. We retrospectively examined the clinical features and outcomes of these 6 patients.Results:The incidence of AFRS was 1.4% (6/429). Allergic fungal rhinosinusitis was unilateral in 5 cases and bilateral in 1. Computed tomography revealed hyperdense areas representing allergic mucin, but no patient exhibited bone erosion. Magnetic resonance imaging showed hypointense or no signal regions at the locations of allergic mucin. Postoperatively, 1 patient developed recurrence. Because the recurrent patient had no significant symptoms, he refused further surgery and received drug therapy. Preoperative eosinophil counts and total IgE levels were elevated in all patients; postoperatively, both remained high in the patient who developed recurrence. Postoperative treatments included steroid therapy and nasal irrigation.Conclusions:Allergic fungal rhinosinusitis is less prevalent in Japan than in Western nations. Peripheral blood eosinophil and serum IgE values may be used as the biomarkers.Significance:Allergic fungal rhinosinusitis is prone to recurrence. Postoperative treatment including steroid therapy is important in the management of AFRS.

Posttraumatic Spinal Cord Injury without Radiographic Abnormality.

“Spinal Cord Injury without Radiographic Abnormality” (SCIWORA) is a term that denotes objective clinical signs of posttraumatic spinal cord injury without evidence of fracture or malalignment on plain radiographs and computed tomography (CT) of the spine. SCIWORA is most commonly seen in children with a predilection for the cervical spinal cord due to the increased mobility of the cervical spine, the inherent ligamentous laxity, and the large head-to-body ratio during childhood. However, SCIWORA can also be seen in adults and, in rare cases, the thoracolumbar spinal cord can be affected too. Magnetic resonance imaging (MRI) has become a valuable diagnostic tool in patients with SCIWORA because of its superior ability to identify soft tissue lesions such as cord edema, hematomas and transections, and discoligamentous injuries that may not be visualized in plain radiographs and CT. The mainstay of treatment in patients with SCIWORA is nonoperative management including steroid therapy, immobilization, and avoidance of activities that may increase the risk of exacerbation or recurrent injury. Although the role of operative treatment in SCIWORA can be controversial, surgical alternatives such as decompression and fusion should be considered in selected patients with clinical and MRI evidence of persistent spinal cord compression and instability.

Multicentre phase II study of nivolumab in Japanese patients with advanced or recurrent non-squamous non-small cell lung cancer

ObjectiveNivolumab is a fully human IgG4 programmed cell death 1 immune checkpoint inhibitor monoclonal antibody approved for the treatment of non-small cell lung cancer (NSCLC). The aim of this study was to evaluate the safety and efficacy of nivolumab in Japanese patients with advanced or recurrent non-squamous NSCLC.MethodsIn this multicentre phase II study, patients with advanced or recurrent non-squamous NSCLC, which had progressed after platinum-containing chemotherapy, were treated with nivolumab 3 mg/kg, intravenously every 2 weeks until progressive disease or unacceptable toxicity was observed. The primary end point was independent radiology review committee (IRC) assessed overall response rate (ORR) and the secondary endpoints included ORR (investigator assessed), progression-free survival (PFS), overall survival (OS), duration of response, time to response, best overall response, and safety.Results76 patients were enrolled across 19 sites in Japan. The ORR (IRC assessed) was 22.4% (95% CI 14.5% to 32.9%). The median PFS and OS were 2.8 months (95% CI 1.4 to 3.4) and 17.1 months (95% CI 13.3 to 23.0), respectively. The OS rate at 1 year was 68.0% (95% CI 56.2% to 77.3%). Current/former smokers were more responsive to treatment than non-smokers (ORR 29.1% vs 4.8%). Patients with epidermal growth factor receptor (EGFR) mutation wild type/unknown showed higher ORR compared with EGFR mutation-positive patients (ORR 28.6% vs 5.0%) and programmed cell death ligand-1 (PD-L1) expression was likely associated with higher ORR, longer PFS and OS. Treatment-related adverse events of grade 3 or higher were reported in 17 patients; these events resolved or were resolving with appropriate treatment including steroid therapy or discontinuation of nivolumab.ConclusionsNivolumab was well tolerated and showed clinical efficacy in Japanese patients with non-squamous NSCLC progressed after platinum-containing chemotherapy, especially in those with a history of smoking, wild type/unknown EGFR mutation status or positive PD-L1 expression.Trial registration numberJapicCTI-132073.

Volumetric Modulated Arc Therapy (VMAT) make a difference in retro-orbital irradiation treatment of patients with bilateral Graves’ ophthalmopathy. Comparative analysis of dosimetric parameters from different radiation techniques

BackgroundGraves’ ophthalmopathy is the commonest extrathyroidal manifestation of Graves’ disease. Treatment options include steroid therapy, corrective/decompressive surgery, radiation therapy or combination of these approaches. AimOur purpose was to investigate if retro-orbital irradiation with Volumetric Modulated Arc Therapy (VMAT) yielded better target coverage and dose sparing to adjacent normal structures compared to 3-Dimensional Conformal Radiotherapy (3DCRT) and Lateral Opposing Conformed Fields (LOCF). MethodsFourteen consecutive patients diagnosed with bilateral Graves’ ophthalmopathy were prospectively recruited into this study from August 2012 until August 2014. An individual VMAT, 3DCRT and LOF plan was created for each patient. Conformity Index (CI), Homogeneity Index (HI) and other dosimetric parameters of the targets and organs-at-risk (OAR) were analyzed in all 28 orbits compared between the different techniques. ResultsCI generated by VMAT was superior to that produced by 3DCRT(p<.001) and LOF (p<.001). As expected, 3DCRT was also superior to LOF (p=.007). Regarding the OARs sparing dose (lens, globes, retina and lacrimal glands), VMAT showed a significant benefit when compared with 3DCRT and LOCF, with no differences between the two latter techniques. ConclusionsVMAT should be preferred over 3DCRT and LOF for bilateral Graves’ ophthalmopathy treatment.

Ischemic Gastritis

To report a case series of ischemic gastritis and discuss its etiology, management, and associated mortality according to our results and the published English literature. Ischemic gastritis is rare, given the rich blood supply of the stomach. It has been reported in isolated case reports and small case series. Most cases are vascular in origin and associated with a high mortality. Pathology databases from 3 hospitals affiliated with the University of Minnesota Medical School were searched for cases of ischemic gastritis in the last 10 years. Patients' demographics, clinical course, and 1-month and 1-year mortalities were collected from electronic medical records. A total of 12 patients were identified (age range, 32.1 to 83.2), the largest series reported to date. The presenting symptom was gastrointestinal bleeding (8), abdominal pain (2), nausea (1), and symptomatic anemia (1). The etiology included postinterventional radiology embolization (2), hemodynamic changes in the setting of celiac axis stenosis (2), vasculitis (1), systemic hypotension (1), and unknown (6). Treatment included steroid therapy, revascularization by interventional radiology, surgery, or supportive treatment. Thirty-day and 1-year mortalities were 33% and 41%, respectively. Ischemic gastritis is rare, but associated with a high mortality. Evaluation for treatable etiologies should be sought and corrected if present.

Expression of Extracellular Matrix-Remodeling Proteins Is Altered in Vaginal Tissue of Premenopausal Women With Severe Pelvic Organ Prolapse

The molecular etiology of pelvic organ prolapse (POP) is complex and not well understood. We compared the expression/activity of extracellular matrix (ECM)-processing (procollagen I N-proteinase/ a disintegrin and metalloproteinase with thrombospondin motifs [ADAMTS]-2,-3,-14) and ECM-degrading (matrix metalloproteinase [MMP]-1, -2, -7, -8, -9, -12) enzymes and their natural tissue inhibitors (tissue inhibitors of metalloproteinase [TIMP]-1,-2,-3,-4) in vaginal tissues from premenopausal women with advanced POP (POP-Q stage ≥ 3) and asymptomatic controls (POP-Q = 0). We sampled the anterior vaginal wall of 36 premenopausal women (17 patients with POP and 19 controls) undergoing total hysterectomy. Exclusion criteria include steroid therapy, malignancy, previous pelvic surgery, and connective tissue diseases. Total RNAs and proteins were quantified by real-time polymerase chain reaction, immunoblotting, and Luminex assay; MMPs activity was analyzed by zymography and tissue localization by immunohistochemistry. The MMP-2 gelatinase activity as well as expression of 58-kDa isoform of ADAMTS-2 was upregulated in patients with POP, irrespective of menstrual phase status, secretory or proliferative, when compared to controls (P < .05). The TIMP-1-4 gene and TIMP-1 protein expression were significantly (P < .05) reduced, whereas protein expression of MMP-12 (pro and active forms) was significantly increased in vaginal biopsies of patients with POP in the proliferative phase of the menstrual cycle compared to corresponding controls. Analyses of MMP-12, TIMP-1, and ADAMTS-2 tissue immunostaining indicate similar localization in the vaginal specimens from control and patients with POP. Expression of ECM-remodeling proteins is altered in the vagina of premenopausal patients with severe POP. We speculate that dysregulation of MMP/TIMP complexes and ADAMTS-2 proteins may cause connective tissue defects, which result in weakened vaginal wall support and POP development.

SAT0474 Predictors of Bone Loss in Men Treated with Androgen Deprivation Therapy for Prostate Cancer: an Observational Study

BackgroundAndrogen deprivation therapy (ADT) is the mainstay of treatment for non-metastatic prostate cancer1. It is known to have adverse effects including fatigue, anaemia, a decrease in lean body mass as...

A Case of Cronkhite-Canada Syndrome with Esophageal Candidiasis

Cronkhite-Cadana syndrome is a rare non-familial disease. This syndrome is characterized by multiple hamartomatous polyps on the entire gastrointestinal tract except esophagus, nail dystrophy, alopecia and hyperpigmentation. Taste disturbance, abdominal pain, diarrhea and weight loss are common symptoms of it. The pathogenesis and causes of Cronkhite-Canada syndrome remain unknown until now. Although various treatment strategies including steroid therapy have been tried, their prognosis is poor. We report a 68 years old man who were diagnosed Cronkhite-Canada syndrome with esophageal candidiasis. After using combination of steroids and anti-fungal drugs, both Cronkhite-Canada syndrome and esophageal candidiasis were cured. (Korean J Helicobacter Up Gastrointest Res 2012;12:183-187)

Autoimmune Inner Ear Disease- A Clinical Viewpoint

Recent developments in medicine have given us a better insight into a group of disorders known as autoimmune diseases. In particular, advances have occurred in our understanding of the Autoimmune Inner Ear Disease (AIED). In this article, the authors review the different postulated theories in the pathogenesis of this disease. The clinical presentation, the available para-clinical diagnostic tools, and the important differential diagnoses will be summarized. The management methods, including steroid therapy, immunosuppressive medications, other biological agents and intra-tympanic injections, will be addressed. Cochlear implantation as a final solution to the advanced stages of the disease, causing total deafness, will also be discussed.

Behavior profile of children with nephrotic syndrome

Background:Nephrotic syndrome, a primarily paediatric disease, is associated with a high relapse rate. Studies have reported behavioral and psychological difficulties in children with nephrotic syndrome, their caregivers and siblings, a factor that is likely to influence the overall outcome of the disease in an adverse manner. In clinical practice, however, the psychosocial aspects of care may be overlooked in the pressure to treat the disease process, unless their importance is stressed by appropriate evidence.Objectives:The study aims to assess the prevalence of behavior abnormalities in children with nephrotic syndrome attending the renal clinic of a state medical college in eastern India and to compare this with the prevalence in a control group of school children without any detectable physical illness. It also aims to explore the relationship between sociodemographic, disease, and treatment related variables and behavioral abnormalities in the nephrotic syndrome group.Materials and Methods:We assessed the prevalence of behavior abnormalities in 50 consecutive children with nephrotic syndrome attending the renal clinic of a state medical college and 51 school children as controls using the Developmental Psychopathology Checklist (DPCL). We also assessed the statistical association between sociodemographic, disease and treatment related variables and behavior profile in the nephrotic children group.Results:Prevalence of behavior disturbance in children with nephrotic syndrome was 68%, significantly higher than that in the control group (21.6%). The behavior abnormalities found in the nephrotic syndrome group were hyperkinesis, obsessive compulsive neurosis, conduct disorder, and emotional disorder, in that order. Frequency of relapse and low socioeconomic status showed significant association with presence of behavior disturbance in the nephrotic syndrome group. This association persisted even after adjusting for other sociodemographic, disease, and treatment related variables, including steroid therapy.