Even mild forms of HSP nephritis risk significant long-term proteinuria. Very early introduction of ACEi/ARB may improve the long-term outcome independent of histological lesions.

Cardiovascular complications are the main cause of death in end-stage renal failure in adult patients, but those complications start in childhood. Renal transplantation (RT) seems to reduce or even reverse certain abnormalities seen in dialyzed patients. Since RT seems to correct metabolic abnormalities that play a role in cardiovascular disease, aortic pulse wave velocity (APWV) was used to evaluate aortic stiffness before and after RT. We included 15 children on chronic hemodialysis (HD), aged 11.1 +/- 4.8 years and dialysis duration was 12.9 +/- 7.4 months. APWV was performed every 6 months before RT and 6 months after. There was no significant difference in APWV (6.1 +/- 1.3 m/s vs 6.5 +/- 1.4 m/s) and augmentation index (AI) on HD and 6 months after RT. APWV pre-transplant was not correlated with time on HD, but increased with age (p = 0.016). No correlation between APWV pre-/post-transplant and other HD parameters or calcineurin inhibitor exposure were found. Only graft function was inversely correlated with APWV post-transplant (p = 0.02). In conclusion, aortic stiffness seems to remain stable before and 6 months after pediatric RT. Graft function was inversely correlated with APWV. Differences in vessel structure among children of the same age group and increase in aortic stiffness with age may jeopardize data interpretation.

Sirs, Patients with nephrotic syndrome (NS) are at risk of developing pneumococcal infections, such as peritonitis, pneumonia ± pleural effusion, and meningitis [1], with the reported incidence of such infections population being 3-5% per year [1]. There is currently no consensus on how to prevent severe episodes of pneumococcal infections in NS children. Anti-pneumococcal vaccine is given to such patients in many centers, but the fear of relapse induced by the vaccine still causes many pediatricians to hesitate in prescribing this treatment.We have recently reported that early treatment with 23-valent INS pneumococcal vaccine (23PPV) at disease onset (in patients receiving high-dose steroids and with nephrotic proteinuria) induces a high antibody response [2]. An approximately tenfold increase in PPV23 antibody level at 1 month (M1) post-vaccination was followed by a decline for up to 6 months (M6) post-vaccination and stabilization at 12 months (M12) post-vaccination. This led us to question of whether the M12 levels remain stable or continue to decline until 36 months (M36) post-vaccination. We also investigated the impact of the number of relapses, immunosuppressive treatment, and patient age on the development of antibody levels [3].Here, we report 23PPV specific antibody levels at M12 and M36 post-vaccination.This was a prospective study on long-term anti-PPV23 antibody levels after the early PPV23 vaccination of patients at the onset of NS. All patients who were included in our first study [2] (investigation of initial antibody response to PPV23) were considered for inclusion in this study. All patients received a unique dose of PPV23 either at disease onset when on high-dose steroids or in remission when on low-dose steroids every other day. Those children who had received 7-valent anti-PV during their first 2 years of life or who had proven invasive pneumococcal infection at disease onset were not included in the study.We compared patients who received PPV23 at disease onset (group 1) with those who received the vaccine when in remission and/or receiving low-dose alternate-day steroids (group 2). From 2004 until 2006, all children >2 years presenting with a first manifestation of idiopathic NS (group 1) were considered for inclusion. Once NS had been diagnosed, global 23-valent PV antibody levels were determined on the same day, and treatment with oral prednisone was started at 60 mg/m 2 per day, with a maximum dose of 60 mg/day. Once the oral steroid treatment started, the patients received one injection of 23-valent anti-PV (Pneumo 23; Merck, West Point, PA) 0.5 ml.Group 2 consisted of all children seen in our out-patient department with a NS in remission who were either receiving low-dose alternate-day prednisone therapy (≤ 15 mg/m 2 ) every other day or were without steroids, with or without other immunomodulatory treatments.Twenty-one patients (five girls) were included in the study. Eight patients received the vaccine when in remission on low-dose steroids (group A) and 13 received the vaccine at d...

Systemic Lupus Erythematosus (SLE) is a chronic multisystem autoimmune disease. Serositis occurs in 16% of SLE patients, and while cardiac tamponade and acute peritonitis with ascites can occur during the course of the disease, they are rare as the first presentation. A 25-year-old woman presented to the emergency department in Tishreen Hospital with complaints of dyspnea, fever, chills, and chest and abdominal pain. Two months prior, she suffered from musculoskeletal pain, fatigue, anorexia, weight loss of about 15 kg, severe hair loss, and recurrent oral aphthous. On clinical examination, the patient was pale and tired with dyspnea and pitting edema (grade 3–4). Pericardiocentesis was emergently performed because there were signs of cardiac tamponade. Three days later, the patient developed an acute surgical abdomen due to acute peritonitis and ascites. Later, the patient was diagnosed with SLE after excluding malignant and infectious diseases. Consequently, methylprednisolone pulses, azathioprine, and hydroxychloroquine 200 mg/day were introduced immediately. The clinical status of the patient dramatically improved, and three months later, the patient was symptom-free with normal laboratory tests. In conclusion, although cardiac tamponade and acute surgical abdomen because of acute peritonitis and ascites as the initial presentation of SLE are very rare, they can occur coincidently.

Patients on tacrolimus had less acute rejection episodes detected on protocol biopsies 3 months after transplant. Protocol biopsies seem to play an important role in the detection of subclinical rejection in patients on CsA.

Methods: We report a case of 4 years' child presenting with a hematuria with a remote history of abdominal trauma whom US suspected Right kidney Nephroblastoma. She received neoadjuvant chemotherapy and the histopathological findings revealed a well-organised hematoma which should be managed conservatively. Results: This is a 4years old girl who presented with hematuria at the hospital with a history of falling down on a root of the tree three years back. The investigation done including abdominal ultrasound (US) revealed right renal mass suggestive of Nephroblastoma of the right kidney. The patient was sent for 6 weeks of neoadjuvant chemotherapy at the specialized cancer center. After receiving preoperative chemotherapy, she underwent right nephrectomy which was sent in our histopathology department for pathologic diagnosis and staging. The sample were processed and microscopy revealed a well encapsulated old organized hematoma (Photomicrograph.1 below). Conclusions: A medical personnel should consider the patient's history while taking care of Wilms' tumor patient for better management, this will also reduce unnecessary treatment which cause some adverse reactions.

Background Characterization of the molecular basis of primary hyperoxaluria type 1 (PH-1) in Syria has been accomplished through the analysis of 90 unrelated chromosomes from 45 Syrians patients with PH-1 from different regions. Methods Alanine glyoxylate aminotransferase (AGXT) gene mutations have been analyzed by using molecular detection methods based on the direct DNA sequencing for all exons of the AGXT gene. Results Seventeen pathogenic mutations were detected in our patients. Six mutations were novels. The three most frequent mutations were c.33_34insC (p.Lys12fs) in Exon 1, c.584 T < G; p.Met195Arg in exon 5 and c.1007 T > A (p.Val336Asp) in exon 10, with a frequency of 33.3%, 12.2%, and 11.1%, respectively. Conclusion DNA sequencing used in this study can offer a useful method to investigate the mutations in Syrian PH-1 patients, and could offer an accurate tool for prenatal diagnosis and genetic counseling.

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