Primary focal segmental glomerulosclerosis in children: epidemiology, clinical presentation and prognosis.

INTRODUCTION: Focal segmental glomerulosclerosis is a histopathological entity. AIM: To analyze the epidemiological, clinical and histological profile of primary focal segmental glomerulosclerosis in children, as well as their prognostic factors. METHODS: This was a retrospective cross-sectional study over a period of 20 years (2001-2020), conducted in the Department of Pediatrics at Charles Nicolle Hospital in Tunis, which included children followed for primary focal segmental glomerulosclerosis. RESULTS: There were 35 children, 19 boys and 16 girls. The median age was 4.5 years. Nephrotic syndrome was seen in 88% of patients. Macroscopic hematuria was found in 4 cases, hypertension in 8 cases and renal failure in 7 cases at presentation. The most common variant was the not otherwise specified variant (77.1%). Steroid-sensitive nephrotic syndrome was observed in 71% of cases, and steroid-resistance in 29% of cases. Treatment with cyclosporine was indicated in 23 patients with complete remission rate of 56.5%. 42,8% children had progressed to chronic kidney disease, including an end-stage renal disease in 11.5% of cases. Only the presence of a family history of kidney disease was found as a predictive factor of progression to chronic kidney disease and end-stage renal disease. Renal survival rates were estimated at 100% at 3 years, 85% at 5 years and 73% at 10 years. CONCLUSION: Identification of patients at high risk for chronic kidney disease progression, such as those with a family history of kidney disease or those who have failed to respond to corticosteroids, would allow therapeutic adjustments.

The practice of the mental image tool for the training of hand hygiene with Hydro-alcoholics solutions: randomized trial.

INTRODUCTION: Mental image (or mental practice), a psychological representation of a task to be carried out, is a technique that could enhance skills in several areas areas including medicine. OBJECTIVE: To evaluate the practice of the mental image tool for the training of hand hygiene with Hydro-alcoholics solutions among students of DCEM2. METHODS: Randomized trial including DCEM2 students in Neonatology and Pediatrics at Charles Nicolle Hospital during the same period. Group1: group training in the mental image, Group 2 of reference. The assessment of student achievement was evaluated in terms of obtained scores. RESULTS: The total number of students was 37. The overall score in group 1 was 17.17 ± 3.82 versus 11.58 ± 4.05 in group 2, p <10 -3. The friction duration of hands in group 1 was 30.56 S ± 4.52S versus 24 ± 5.17 in group 2, p <10-3. CONCLUSION: Mental practice may be a time- and cost-effective strategy that improves hand hygiene with Hydro-alcoholics solutions.

[Post-streptococcal glomerulonephritis in the south of Tunisia: A 12-year retrospective review]. / La glomérulonéphrite aiguë post-streptococcique de l'enfant dans le sud tunisien : étude rétrospective de 12 ans.

AIM: Post-streptococcal glomerulonephritis (PSGN) is a frequent cause of acute nephritis in children. This study aimed to describe the epidemiology, clinical characteristics and outcomes of PSGN and look for predictor's factors of severity. METHODS: A 12-year retrospective review of case notes and laboratory data was conducted at a department of pediatrics, pediatric emergency and intensive care, Hedi Chaker Hospital. RESULTS: One hundred seventy eight children were treated for PSGN with a mean age of 7.6 ans±3.43 ans. One hundred and forty-two patients (80%) had a history of a recent upper respiratory tract or skin infection. Streptococcal pharyngitis was the most common cause, identified in 113 patients (67.6%). Macroscopic hematuria and edema were noted in 135 (75.8%) and 114 cases (64%) respectively. Hypertension was present in 55 patients (31%). Oliguria was noted in 30 children (16.8%). Sixty-six subjects (37%) developed acute renal impairment (creatinine≥70 micromoles/L). No correlation was demonstrated between acute renal impairment and age, sex, triggering infection, anemia and white blood cell count. Creatinine greater than 56.35 micromoles/L was associated with a high risk of developing high blood pressure. The mean length of admission was 5.8 days±4.44. Only one subject has ongoing renal dysfunction. CONCLUSION: PSGN remains a common nephropathy in our region. The detection and effective treatment of any infection that may be involved can reduce the incidence of this disease.

Primary immunodeficiencies : Report of 33 Pediatric Tunisian cases.

Background Primary immunodeficiencies (PID) are a group of heterogeneous and relatively rare diseases. Aim to determine the clinical characteristics, outcome and genetic data of primary immunodeficiencies in pediatrics patients. Methods A retrospective, descriptive and multicentered study, enrolling 33 children presenting a PID in Tunis, during a period of 22 years (1991-2012). Resultats a masculine predominance has been noticed with a sex ratio at 2,3. Consanguinity was found in 71% of family cases. History of early infant deaths was found in 42% of cases. The media age of diagnosis was of 1 year 2 months. The median diagnosis delay was of 11 months and 1/2. Most frenquently observed PID were combined immunodeficiency (36%), mostly severe combined immunodeficiency (SCID) (21%), followed by congenial defects of phagocyte function (33%), mostly chronic granulomatosis disease (21%). Antibody defects were found in 21% of cases. Most frequently observed out comes were lung infections (66%) recurrent oral thrush (57%) and diarrhea (42%). Most important complications were severe infections and bronchiectasis. 30% of patients were dead by the end of the study. A molecular characterization was performed in 33% of patients, and an antenatal diagnosis was performed in 10% of cases. Conclusion The PID are a group of disease with variable expressions and etiologies. Their frequency remains understimated in Tunisia, and their management, difficult and insufficient. We suggest the establishment of systematic genetic consulting visit, the creation of a national registry and developing bone marrow transplantation in children in Tunisia.

[Acute tubular necrosis in kidney transplantation]. / La nécrose tubulaire aiguë en transplantation rénale.

BACKGROUND: The acute tubular necrosis (ATN) is common after kidney transplantation. Acute tubular necrosis (ATN) is multifactorial and represents one of the main causes of the delayed graft function. Its impact on graft and patients survival is documented. AIMS: To study the prevalence of the ATN in kidney transplanted patients, the acute rejection rate and their impact on the graft and the patient survival. METHODS: We retrospectively studied the frequency of ATN, its causes and its impact on patient and graft survival in 255 kidney transplanted patients between 1986-2006. RESULTS: Thirty-nine patients had ATN (15.29%). They are 25 men and 14 women with mean age of 30.1 ± 12.6 years (8-61) followed for an average of 98 ± 61.76 months. The majority was treated by hemodialysis (79.48%) and half of them were transplanted from kidney of deceased donor. All patients received anti lymphocyte serum and the majority anticalcineurins (69.23%). The outcome was favorable in 26 patients (66.66%) with recovery of diuresis and normalization of renal function after 6 weeks on average. An acute rejection was diagnosed in 21 patients (53.48%). The mean creatinine at 1, 5 and 10 years was 135.3, 159.9 and 121.4 µmol / l. Eight patients had creatinine ² 130 µmol / l at 10 years. Ten patients died from infectious and cardiovascular causes. By comparing the 2 groups ATN + and ATN - we found a statistically significant correlation between ATN and cold ischemia (10 ± 10.9 vs 1.2 ± 4.7 hours, p <0.0001) and the interval between the start of dialysis and transplantation (42.18 ± 38.44 vs. 31.1 ± 25.2 months, p= 0.02). No statistical correlation was found between the ATN and gender, age of recipient and donor, warm ischemia, acute rejection, chronic rejection and graft and patient survival at 1, 5 and 10 years. CONCLUSION: The ATN is more common among transplanted patients from deceased donors. It had good evolution in the majority of cases and it's correlated to cold ischemia and duration of dialysis. Finally, it has no impact on patients and graft survival.