Conventional Steroid Research Articles

Comparison of conventional and low dose steroid in the treatment of PFAPA syndrome: Preliminary study

BackgroundSteroids have been widely used to relief symptoms in the patients with PFAPA syndrome. ObjectivesThis study was constructed to show the effectiveness of low-dose steroid therapy in patients diagnosed with PFAPA syndrome. Methods41 patients (86 febrile attacks) who were diagnosed using the criteria suggested by Thomas et al. were involved in the study. The cases were classified into two groups and the selection of patients in groups was made randomly. Twenty patients received prednisolone at a dose of 2mg/kg/day (first group: 40 attacks) and 21 patients received a dose of 0.5mg/kg/day (second group: 46 attacks). The effectiveness of the treatment was especially determined by the time needed to reduce the fever and the effect on the duration between the two attacks. The patients were re-examined 24hours later, after a steroid treatment. ResultsThe patients who were in the first group received 2mg/kg/day dose of prednisolone and their fever was dramatically decreased in 6–8hours (7.6±0.9hours). The second group received 0.5mg/kg/day dose and 19 of these patients’ fever was decreased in 8–12hours. Two patients whose temperature did not decrease, received another dose of prednisolone 24hours after the first dose and their fever was reduced 12hours after the second dose (11.3±6.4hours). A comparison of the rate of fever reduction and the interval between the attacks (Group I: 5.11±1.01 week and Group II: 5.2±1.13 week) in the two groups did not show any statistical significance (p=0.104). ConclusionLow-dose steroid treatment is as effective as normal dose in PFAPA syndrome but there is need to study with a larger group.

Tratamiento del rechazo agudo hiperinmune con plasmaféresis y Rituximab. Nuestra Experiencia

El trasplante renal consiste en la extracción de un riñón de un individuo sano y su colocación en un paciente con insuficiencia renal crónica terminal1. El rechazo inmunológico es la principal complicación tras el trasplante. Se denomina rechazo al conjunto de manifestaciones clínicas, funcionales, anatomopatológicas, que pueden aparecer en un receptor de trasplante renal, como consecuencia de la activación del sistema inmune contra el injerto. El rechazo puede aparecer desde el momento de revascularización del órgano hasta años más tarde2. La incidencia del rechazo dependerá esencialmente de dos factores: las características del receptor y el tratamiento inmunosupresor utilizado. En general se acepta que un 30-40% de los pacientes desarrollan algún episodio de rechazo en el trasplante inmediato (3 primeros meses)1. En la mayoría de los protocolos se utilizan como tratamiento convencional bolos de metil-prednisolona a dosis que varían entre 250 y 1.000mg/día, durante 3-4 días. Con esta pauta se consigue respuesta entre el 60-70% de los casos. En el resto de los casos se habla de rechazo corticoresistente2. La Plasmaferesis ha sido recientemente introducida en el tratamiento del rechazo agudo del trasplante renal resistente al tratamiento convencional con esteroides3. La aféresis puede definirse como una modalidad terapéutica que consiste en la extracción de un volumen determinado de plasma (de 2 a 5 litros) con el fin de eliminar aquellos patógenos considerados responsables de una enfermedad o bien sus manifestaciones clínicas. El recambio plasmático mediante filtración a través de membranas se realiza mediante el montaje de un circuito extracorpóreo.

Clinical Experiences of Transforaminal Balloon Decompression for Patients with Spinal Stenosis

Lumbar spinal stenosis is a commonly treated with epidural injections of local anesthetics and corticosteroids, however, these therapies may relieve leg pain for weeks to months but do not influence functional status. Furthermore, the majority of patients report no substantial symptom change over the repeated treatment. Utilizing balloon catheters, we successfully treated with three patients who complained persistent symptoms despite repeated conventional steroid injections. Our results suggest that transforaminal decompression using a balloon catheter may have potential in the nonsurgical treatment of spinal stenosis by modifying the underlying pathophysiology.

PPARγLigands Regulate Noncontractile and Contractile Functions of Airway Smooth Muscle: Implications for Asthma Therapy

In asthma, the increase in airway smooth muscle (ASM) can contribute to inflammation, airway wall remodeling and airway hyperresponsiveness (AHR). Targetting peroxisome proliferator-activated receptor γ (PPARγ), a receptor upregulated in ASM in asthmatic airways, may provide a novel approach to regulate these contributions. This review summarises experimental evidence that PPARγ ligands, such as rosiglitazone (RGZ) and pioglitazone (PGZ), inhibit proliferation and inflammatory cytokine production from ASM in vitro. In addition, inhaled administration of these ligands reduces inflammatory cell infiltration and airway remodelling in mouse models of allergen-induced airways disease. PPARγ ligands can also regulate ASM contractility, with acute treatment eliciting relaxation of mouse trachea in vitro through a PPARγ-independent mechanism. Chronic treatment can protect against the loss of bronchodilator sensitivity to β 2-adrenoceptor agonists and inhibit the development of AHR associated with exposure to nicotine in utero or following allergen challenge. Of particular interest, a small clinical trial has shown that oral RGZ treatment improves lung function in smokers with asthma, a group that is generally unresponsive to conventional steroid treatment. These combined findings support further investigation of the potential for PPARγ agonists to target the noncontractile and contractile functions of ASM to improve outcomes for patients with poorly controlled asthma.

P5-13-19: Borderline Estrogen and Progesterone Receptor Expression and Efficacy of Anti-Estrogen Therapy Analyzed by Subpopulation Treatment Effect Pattern Plot Analysis.

Abstract Background: The American Society of Clinical Oncology (ASCO) and the College of American Pathologists (CAP) recently recommended that estrogen receptor (ER) and progesterone receptor (PR) values be considered positive in the evaluation of breast cancer specimens if there are at least 1% positive tumor nuclei by immunohistochemisty (IHC) scoring. This change constitutes a lower cutoff value than the 5% or 10% widely used in the past and increases the number of patients that can now be treated with anti-estrogen therapy, a therapy which carries significant treatment efficacy and a favorable toxicity profile. Despite the new recommendation however, it remains unclear if patients whose tumors display borderline ER and/or PR expression truly benefit from anti-estrogen therapy. Methods: We examined a total of 608 patient samples from the OHSU Knight Cancer Institute Breast Cancer Tissue Repository for which we have quantitative ER and PR information and their clinical outcome. Of those, 282 patients did not receive any systemic treatment and 165 received anti-estrogen therapy (the majority tamoxifen). Cytosolic steroid hormone receptors were quantitated by conventional steroid binding assay and Scatchard plot analysis or by enzyme-linked immunoassay system. For immunoassays, human estrogen receptor (ER) and progesterone receptor (PR) kits were obtained from Abbott Laboratories and assays were conducted according to manufacturer's specification. We performed subpopulation treatment effect pattern plot (STEPP) analysis to explore the presence of interaction between treatment effects and quantitative ER and PR levels. Clinical outcome used in this study includes 5-year overall survival and 5-year breast cancer free interval. Results: Multi-variate analysis finds neither ER nor PR expression adjusted by treatment status is associated with either overall survival (P= 0.20, median follow up of 63 months) or breast cancer free interval (P= 0.82, median follow up of 60 months). For both ER and PR, there is no interaction between treatment outcome (5 year survival probability) and levels of hormone receptor expression based on Kaplan-Meier estimate (P=0.36 and P= 0.65, respectively). Patients with borderline ER expression appear to benefit from anti-estrogen therapy while patients with borderline PR expression appear to have worse outcome with anti-estrogen therapy. Conclusions: Though descriptive in nature, our STEPP analysis provides support for the recommendation by ASCO and CAP to lower the cutoff for ER positivity in breast cancer specimens. There is overall no interaction between the level of hormone receptor and treatment benefit for all patients from low ER to high ER. Therefore, treatment will benefit all patients including those with low ER levels. Extending hormone therapies to the group of patients whose tumors express borderline PR values, however does not seem to confer therapeutic benefit. More study is needed to determine the benefits of offering anti-estrogen therapy to patients whose tumors express low PR. Citation Information: Cancer Res 2011;71(24 Suppl):Abstract nr P5-13-19.

Pulse steroids as induction therapy for children with ulcerative colitis

Corticosteroids therapy, classically the first-line treatment for ulcerative colitis (UC), often causes serious side-effects. Theoretically, pulse steroid therapy where high doses are given for a shorter period may have maximal beneficial effects and minimal side-effects as induction therapy for UC. We have therefore retrospectively compared induction therapy using pulse steroids with conventional steroid treatment for children and adolescents with moderate-to-severe UC. We utilized conventional steroid treatment (prednisolone 1-1.5 mg/kg/day) as an induction treatment in 17 UC patients between 1985 and 2006. Alternatively we used a 3-day megadose pulse steroid therapy (methylprednisolone intravenously 20-30 mg/kg/day, max. 1000 mg/day) in 20 UC patients from 1993 to 2006. Pulse steroid therapy successfully induced rapid remission in UC patients with moderate-to-severe disease compared with conventional treatment (13.2 days vs 25.1 days; P < 0.05). The amelioration of Pediatric Ulcerative Colitis Activity Index score between before and 1 week after pulse steroid therapy was significantly more than that of conventional treatment (P < 0.01). No serious adverse effects were observed in the patients treated with pulse steroid therapy. However, the rate of the relapse episodes during the next 12 months after pulse steroid therapy was not significantly different from that after conventional treatment. These findings suggest that pulse steroid therapy is an option to be considered in children with moderate-to-severe UC.

Radiation Dose Exposure for Lumbar Spine Epidural Steroid Injections: A Comparison of Conventional Fluoroscopy Data and CT Fluoroscopy Techniques

The purpose of this article is to compare the radiation dose of conventional fluoroscopy-guided lumbar epidural steroid injections (ESIs) and CT fluoroscopy (CTF)-guided lumbar ESI using both clinical data and anthropomorphic phantoms. We performed a retrospective review of dose parameters for 14 conventional fluoroscopy ESI procedures performed by one proceduralist and 42 CTF-guided ESIs performed by three proceduralists (14 each). By use of imaging techniques similar to those for our clinical cohorts, a commercially available anthropomorphic male phantom with metal oxide semiconductor field effect transistor detectors was scanned to obtain absorbed organ doses for conventional fluoroscopy-guided and CTF-guided ESIs. Effective dose (ED) was calculated from measured organ doses. The mean conventional fluoroscopy time for ESI was 37 seconds, and the mean procedural CTF time was 4.7 seconds. Calculated ED for conventional fluoroscopy was 0.85 mSv compared with 0.45 mSv for CTF. The greatest contribution to the radiation dose from CTF-guided ESI came from the planning lumbar spine CT scan, which had an ED of 2.90 mSv when z-axis ranged from L2 to S1. This resulted in a total ED for CTF-guided ESI (lumbar spine CT scan plus CTF) of 3.35 mSv. The ED for the CTF-guided ESI was almost half that of conventional fluoroscopy because of the shorter fluoroscopy time. However, the overall radiation dose for CTF-guided ESIs can be up to four times higher when a full diagnostic lumbar CT scan is performed as part of the procedure. Radiation dose reduction for CTF-guided ESI is best achieved by minimizing the dose from the preliminary planning lumbar spine CT scan.

Oral Leukotrienes Receptor Antagonist as Supplmentary Treatment in Patients With Chronic Sarcoidosis

PURPOSE: Sarcoidosis is granulomatous disease of unknown etiology and unpredictable course. It is characterized by the existence of granulomatous inflammation and in some patients with the variety of lung function impairments. Unfortunately, conventional steroid therapy is not only sufficient for their improvement. In addition to the conventional immunosuppressive therapy, some patients need the supplementary treatment in order to achieve the improvement of their lung function. The purpose of this study is to demonstrate the effects of montelukast an oral leukotrienes receptor antagonist, in the therapy of sarcoidosis patients with lung function impairments and parenchymal lesions (stage 2 and 3 of the lung disease).

Very early steroid withdrawal or complete avoidance for kidney transplant recipients: a systematic review

The safety and efficacy of early steroid withdrawal or avoidance in patients receiving a kidney transplant (KT) are controversial. We performed a systematic review and a meta-analysis of the randomized controlled studies about steroid avoidance or withdrawal after a few days in patients receiving a KT and treated with antibody induction and cyclosporine (CsA) or tacrolimus (Tac) plus mycophenolate mofetil (MMF) (nine available studies and 1934 participants). Death and graft loss (including or excluding death with function) were similar in steroid avoidance and control patients, with no differences between CsA and Tac studies. After steroid avoidance, acute rejection was more frequent than conventional steroid use in CsA trials [risk ratios (RR) 1.59, 95% confidence intervals (95% CI) 1.01-2.49] but not when Tac was used (RR 1.06, 95% CI 0.79-1.42). Steroid avoidance was associated with less frequent new-onset diabetes mellitus, but this decrease was only evident with CsA (RR 0.54, 95% CI 0.30-0.98), whereas this difference was not significant analysing Tac studies (RR 0.75, 95% CI 0.32-1.77). Despite this trend, the corresponding interaction tests were not statistically significant (P = 0.140 and P = 0.535, for acute rejection and new-onset diabetes mellitus, respectively). Serum creatinine, creatinine clearance, mean blood pressure, serum cholesterol and serum triglycerides were similar in both groups. Steroid avoidance or early withdrawal within the first 2 weeks is safe in KT recipients receiving induction with anti-interleukin-2 receptor antibodies or thymoglobulin and a drug regimen based on calcineurin inhibitor and MMF. However, the real benefits remain unclear.

Comparison of conventional dose steroid treatment and high dose steroid treatment as run-in regime for splenectomy in immune thrombocytopenic purpura (ITP)

Background Splenectomy is a treatment procedure for ITP which is being used for more that 50 years and is still considered as the best option for the treatment of the condition. The facts that frequent monitoring is not required and that medical treatment is not indicated following splenectomy in most cases are among the major advantages of the procedure. Aim of the study To compare the conventional dose steroid and high dose steroid treatments as run-in regime for splenectomy in patients diagnosed with ITP. Methods The conventional dose steroid (1 mg/kg, n = 20) and high dose steroid treatments (30 mg/kg, n = 30) were applied to 50 cases with ITP between 1998 and 2008 in our clinic. Results: High dose steroid produced higher platelet count on days 3, 5, and 7 compared to conventional dose steroid. There were no differences between the groups in terms of adverse effects. Treatments were discontinued after the operation by rapidly reducing the dose. Postoperative values regarding response to splenectomy were comparable in both groups. Conclusions Both higher and earlier responses obtained with high dose steroid may be significant in reducing hospitalization period of patients and eliminating life-threatening platelet values within the shortest time possible.

Clinical Outcomes of 174 Nasopharyngeal Carcinoma Patients With Radiation-Induced Temporal Lobe Necrosis

To retrospectively study the clinical outcomes of nasopharyngeal carcinoma patients with radiation-induced temporal lobe necrosis (TLN) treated with steroids, surgery, or observation only. We performed a retrospective analysis of 174 consecutive patients diagnosed with TLN between 1990 and 2008. Before 1998, symptomatic patients were treated with oral steroids, while asymptomatic patients were treated conservatively. After 1998, most symptomatic and asymptomatic patients with a large volume of necrosis were treated by intravenously pulsed-steroid therapy with a standardized protocol. We examined factors affecting grade 4 complication-free survival and overall survival. Outcomes of the three treatment groups, those receiving conservative treatment, those receiving oral steroid, and those receiving intravenous pulse steroid, were compared. The mean follow-up time was 115 months. Rates of grade 4 complication-free survival at 2 years and at 5 years after diagnosis of TLN were 72.2% and 54.1%, respectively. The 2-year and 5-year overall survival rates were 57.5% and 35.4%, respectively. Multivariate analysis revealed that being symptomatic at diagnosis (relative risk [RR], 4.5; p = 0.0001), re-irradiation of the nasopharynx (NP) (RR, 1.56; p = 0.008), salvage brachytherapy to the NP (RR, 1.75; p = 0.012), and a short latency period before the diagnosis of TLN (RR, 0.96, p < 0.0001) were independent prognosticators of poor grade 4 complication-free survival. Patients with all four factors had a 100% risk of developing grade 4 complications within 5 years; whereas if no factor was present, the risk was 12.5%. Intravenous pulse steroid therapy was associated with a higher clinical response rate compared with conventional steroid therapy (p < 0.0001); however, it did not affect complication-free survival in multivariate analysis. TLN patients with good prognosticators could be observed without active treatment. Although treatment with intravenously pulsed steroid was associated with better clinical response than conventional steroid delivery, it did not affect the complication-free survival rate of TLN patients.

Comparison of steroid pulse therapy and conventional oral steroid therapy as initial treatment for autoimmune pancreatitis

The efficacy of oral steroid therapy for autoimmune pancreatitis (AIP) is well known, and oral prednisolone treatment is most usually commenced at 30-40 mg/day, but there have been few reports about comparative studies of oral steroid therapy and steroid pulse therapy as the initial treatment for AIP. We studied the clinical course and image findings to estimate the utility of steroid pulse therapy for AIP, comparing it with oral steroid therapy. Laboratory and image findings were assessed retrospectively in 11 patients who received steroid pulse therapy, and the findings were compared to those in 10 patients who received conventional oral steroid therapy. Change in pancreatic size showed no significant difference between the therapies after 2 weeks of treatment. Significant improvement of lower bile duct strictures after 2 weeks of treatment and that of immunoglobulin values within 6 months were shown with both therapies. However, steroid pulse therapy showed significant improvement of γ-guanosine triphosphate (GTP) in 2 weeks and of alanine aminotransferase (ALT) in 2 and 8 weeks, compared with oral steroid therapy. Moreover, there was one patient in whom the lower bile duct stricture was not improved by oral steroid therapy, but it did show improvement with steroid pulse therapy. Initial steroid pulse therapy is a beneficial alternative to oral steroid therapy for the improvement of bile duct lesions. In future, the accumulation of a larger number of patients receiving steroid pulse therapy is needed, and prospective studies will be required.

Treatment of colitis with a commensal gut bacterium engineered to secrete human tgf-β1 under the control of dietary xylan

While cytokine therapy and the use of immunosuppressive cytokines such as transforming growth factor-β (TGF-β) offer great potential for the treatment of inflammatory bowel disease (IBD), issues concerning formulation, stability in vivo, delivery to target tissues, and potential toxicity need to be addressed. In consideration of these problems we engineered the human commensal bacterium Bacteroides ovatus for the controlled in situ delivery of TGF-β(1) and treatment of colitis. Sequence encoding the human tgf-β1 gene was cloned downstream of the xylanase promoter in the xylan operon of B. ovatus by homologous recombination. Resulting recombinants (BO-TGF) were tested for TGF-β production in the presence and absence of polysaccharide xylan in vitro and in vivo, and used to treat experimental murine colitis. Clinical and pathological scores were used to assess the effectiveness of therapy. Colonic inflammatory markers including inflammatory cytokine expression were assessed by colorimetric assay and real-time polymerase chain reaction (PCR). BO-TGF secreted high levels of biologically active dimeric TGF-β in vitro and in vivo in a xylan-controlled manner. Administration of xylan in drinking water to BO-TGF-treated mice resulted in a significant clinical improvement of colitis, accelerating healing of damaged colonic epithelium, reducing inflammatory cell infiltration, reducing expression of proinflammatory cytokines, and promoting production of mucin-rich goblet cells in colonic crypts. These beneficial effects are comparable and in most cases superior to that achieved by conventional steroid therapy. This novel drug delivery system has potential for the targeted and controlled delivery of TGF-β(1) and other immunotherapeutic agents for the long-term management of various bowel disorders.

Early Transtympanic Steroid Injection in Patients with ‘Poor Prognosis’ Idiopathic Sensorineural Sudden Hearing Loss

Objective: The present trial is aimed to evaluate the effectiveness of early transtympanic steroid injection in patients with ‘poor prognosis’ idiopathic sensorineural sudden hearing loss (ISSHL). Methods: Seventy-six patients, diagnosed as having ISSHL with ‘poor prognosis’, were randomized into a transtympanic group and a control group. Thirty-nine patients received conventional steroid treatment and 37 patients received 4 courses of additional transtympanic injections of methylprednisolone 7 days after conventional steroid treatment. Results: In the transtympanic group 45.9% of the patients had at least 15 dB improvement in pure tone average compared with 20.5% of the patients in the control group at the 8-week follow-up audiogram. In the transtympanic group 43.2% of the patients had an improvement of ≧15% in speech discrimination, compared with 17.9% of the patients in the control group 8 weeks after the treatment. The differences between the 2 groups were statistically significantly. Conclusion: The outcome was better for the patients with ‘poor prognosis’ ISSHL if the transtympanic steroid therapy could be applied early.

Low-dose human chorionic gonadotropin versus estradiol/progesterone luteal phase support in gonadotropin-releasing hormone agonist–triggered assisted reproductive technique cycles: understanding a new approach

It remains unclear how GnRH agonist (GnRHa) triggering affects the luteal phase, so we investigated the luteal phase after GnRHa triggering, supported with conventional E(2)/P with or without low-dose hCG. E(2)/P support, compared with low-dose hCG, induced a shorter luteal phase (11.2 ± 1.1 vs. 15.0 ± 1.6 days) and fewer subjective complaints (0 vs. 42%), whereas hCG caused more free fluid accumulation and enlarged ovaries than E(2)/P alone. Steroids and low-dose hCG differentially affected corpus luteum function, ovarian size, free fluid accumulation, and patient comfort.

Hearing outcomes of daily intratympanic dexamethasone alone as a primary treatment modality for ISSHL

Objective Intratympanic (IT) dexamethasone offers a good method for avoiding systemic side effects in patients with sudden idiopathic sensorineural hearing loss (ISSHL). However, in most studies, IT was used as a salvage treatment for patients whose hearing failed to improve with the initial systemic treatment, or as an addition to conventional oral steroid protocols. Therefore, we investigated the outcomes of IT steroid administration as a primary treatment modality for ISSHL. Study Design Randomized controlled study. Setting Tertiary referral center. Subjects and Methods Two treatment methods, IT dexamethasone or oral prednisolone, were randomly assigned to 63 participants. A group of 32 patients (IT group) with ISSHL received IT dexamethasone once a day for eight days. A separate group of 31 patients (oral group), received oral prednisolone, also for eight days. We compared the hearing outcomes in the two groups. Results We have found that the outcomes for patients treated with IT dexamethasone as a primary treatment modality for the management of ISSHL presented no difference in pure-tone averages or hearing recovery rate compared with patients treated with oral steroids. However, differences were noted for hearing thresholds according to frequency: the threshold improvement at low frequencies (250, 500, and 1000 Hz) was not statistically significant between groups; at high frequencies, particularly 4000 and 8000 Hz, the threshold improvement was statistically higher in the oral group than in the IT group. Conclusion IT dexamethasone might be a good primary treatment method for ISSHL; but, for the management of ISSHL, we should keep in mind the differences in hearing outcomes with regard to frequency.

Eficacia y seguridad de tacrolimus oral para el tratamiento de la enfermedad inflamatoria intestinal pediátrica

Background In certain clinical situations, such as acute and severe episodes of ulcerative colitis (UC) or Crohn's Disease (CD), that do not respond to conventional intravenous steroid treatment, we need potent, fast-acting drugs to induce clinical remission and avoid surgery. Objectives To evaluate the efficacy and safety of oral tacrolimus treatment of acute and severe UC or CD to induce their remission, and also to assess its efficacy in delaying or avoiding surgery. Material and methods We present a retrospective study that included all patients under 18 years of age with acute and severe bouts of CD (colonic or ileocolonic location) or UC who were treated with oral tacrolimus at our institution from January 1998 to December 2007. Results We included a total of 8 patients (4 males and 4 females), 6 presented with UC and 2 had CD. The mean age of our patients at the start of the treatment was 11.8 years (range 2.75–16.58 y) and the mean time from diagnosis to the start of tacrolimus therapy was 4 months (range 1–96 m). An initial response was obtained in 50% of patients. Plasma trough levels of tacrolimus remained between 5–11 ng/ml. Six of the eight patients (75%) required surgery. In one patient with UC and in another with CD, surgery was avoided. In 2 of the 6 patients with UC, surgery was postponed beyond 6 months. Conclusions Tacrolimus is useful in inducing clinical remission in patients with acute and severe bouts of UC or CD, and so can avoid or delay the surgery; it may also be used as a bridging agent until the new maintenance therapy with other immunosuppressants is effective.

Ketogenic diet in the treatment of refractory continuous spikes and waves during slow sleep

To evaluate the effect of the ketogenic diet on electroclinical characteristics and cognitive function in children with continuous spikes and waves during slow sleep (CSWS). Five children (four boys, one girl) aged between 8 and 13 years with CSWS refractory to conventional antiepileptic drugs (AEDs), including levetiracetam, and steroids were included. The prospective electroclinical assessment was performed prior to the ketogenic diet and once every 6 months post initiation during the 2-year period. All children underwent neuropsychological testing prior to the ketogenic diet and four of the children again 12 months after the diet's introduction. In case 4 the testing has been performed after 7 months and the diet was withdrawn after 9 months because of the lack of efficacy and the parent's wishes. In two patients the cognitive functions were also evaluated after 24 months since the diet's initiation. During the period on the ketogenic diet the concomitant AED treatment was unchanged. Electrographic evaluation after 24 months on the ketogenic diet showed CSWS resolution in one patient, mild decrease of the spike-wave index in one, and lack of response in three patients. The ketogenic diet did not influence the neuropsychological outcome, and intelligence quotient (IQ) scores remained low at the end of the follow-up period. However, in two patients an improvement in attention and behavior was demonstrated. This is the first study evaluating the efficacy of the ketogenic diet in children with CSWS. Five presented cases were refractory to AEDs and steroids. Only one case responded with complete CSWS disappearance; in one the effect of the ketogenic diet was partial and intermittent, whereas in three patients no response has been observed. These results show that the ketogenic diet did not appear to influence the neuropsychological outcome; however, the absence of a control group makes it impossible to conclude with certainty.

Role of tonsillectomy in the management of IgA nephropathy

Background :The tonsils represent a site of local immunity where immunoglobulin-bearing cells are capable of producing IgA. The tonsils have been considered the foci of abnormal IgA production in IgAN, thus, a relationship is suggested between the tonsillar immune response and the pathogenesis of IgAN. IgAN, then, is considered a tonsillar focal infection. Patients and methods :This study was done on 15 patients their ages ranged between 15 and 35 (10 males and 5 females). All patients were already diagnosed by renal biopsy-proven IgA nephropathy by the nephrologists and under medical treatment by conventional steroid. The diagnosis of IgA nephropathy was based on histologic assessment of percutaneous renal biopsy tissue. The main problem in all patients is that development of gross haematoria, deterioration of urinary findings and deterioration of the general condition of the patient after any acute attack of upper respiratory tract infection especially acute tonsillitis. Tonsillectomy was done after one month from controlling the acute infection. Postoperative follow up for all patients was done at 1st, 3rd, 6th, 12th month by clinical examination and laboratory investigations Results :The gross haematoria was completely stopped in all patients after tonsillectomy except two patients one of them was complicated by hypertension and the other one was complicated by nephritic syndrome. Also there was marked reduction in proteinuria, microscopic haematoria, marked reduction in the serum IgA concentration, urinary abnormalities disappeared and also improvement of renal function for the patients with serum creatinin less than 2mg/dl while in the two patients with serum creatinin more than 2mg/dL there was no much improvement after tonsillectomy with more deterioration of renal function of both patients with the development of chronic renal failure in the patient with nephritic syndrome 9 month postoperative Conclusion :Tonsillectomy is effective in improving renal function ,urinary symptoms, gross haematoria and decreasing the level of IgA in patients with IgAN if it is done in mild to moderate cases with serum creatinin less than 2mg/dL while has no much efficacy on moderate to severe cases with serum creatinin more than 2mg/dL or complicated cases.

Role of tonsillectomy in the management of IgA nephropathy

Background :The tonsils represent a site of local immunity where immunoglobulin-bearing cells are capable of producing IgA. The tonsils have been considered the foci of abnormal IgA production in IgAN, thus, a relationship is suggested between the tonsillar immune response and the pathogenesis of IgAN. IgAN, then, is considered a tonsillar focal infection. Patients and methods :This study was done on 15 patients their ages ranged between 15 and 35 (10 males and 5 females). All patients were already diagnosed by renal biopsy-proven IgA nephropathy by the nephrologists and under medical treatment by conventional steroid. The diagnosis of IgA nephropathy was based on histologic assessment of percutaneous renal biopsy tissue. The main problem in all patients is that development of gross haematoria, deterioration of urinary findings and deterioration of the general condition of the patient after any acute attack of upper respiratory tract infection especially acute tonsillitis. Tonsillectomy was done after one month from controlling the acute infection. Postoperative follow up for all patients was done at 1st, 3rd, 6th, 12th month by clinical examination and laboratory investigations Results :The gross haematoria was completely stopped in all patients after tonsillectomy except two patients one of them was complicated by hypertension and the other one was complicated by nephritic syndrome. Also there was marked reduction in proteinuria, microscopic haematoria, marked reduction in the serum IgA concentration, urinary abnormalities disappeared and also improvement of renal function for the patients with serum creatinin less than 2mg/dl while in the two patients with serum creatinin more than 2mg/dL there was no much improvement after tonsillectomy with more deterioration of renal function of both patients with the development of chronic renal failure in the patient with nephritic syndrome 9 month postoperative Conclusion :Tonsillectomy is effective in improving renal function ,urinary symptoms, gross haematoria and decreasing the level of IgA in patients with IgAN if it is done in mild to moderate cases with serum creatinin less than 2mg/dL while has no much efficacy on moderate to severe cases with serum creatinin more than 2mg/dL or complicated cases.