Nephrocalcinosis in Tunisian children.

Background Nephrocalcinosis is rare in children. Its etiologies are multiple. The aim of this study was to analyze the etiology of nephrocalcinosis in Tunisian children. Methods This retrospective study was conducted in the department of pediatrics in Charles Nicolle Hospital during a period of 10 years (2001-2010). Results There were 40 children. The mean age was 3.5 years. The most common signs and symptoms at presentation were growth retardation (42.5%) and hematuria (53.8%). At presentation, renal failure was detected in 70% of patients. The diagnosis of nephrocalcinosis was performed by ultrasonography. The etiology of nephrocalcinosis included primary hyperoxaluria type 1 (65%) and distal renal tubular acidosis (20%). A progression to renal insufficiency was observed in 18 cases. Conclusion Primary oxaluria is the principal cause of nephrocalcinosis; early diagnosis and treatment are mandatory as they help limiting renal function deterioration.

Focal segmental glomerulosclerosis in children.

Background Focal segmental glomerulosclerosis (FSGS) represents 20% of nephrotic syndrome in children. The clinical course and prognosis is heterogeneous in children. The aim of this study was to analyze treatment and outcome of children with FSGS. Methods This retrospective study was conducted in the Department of Pediatrics in Charles Nicolle Hospital during a 15-year period (1996-2010). Results There were 30 children, 16 boys and 14 girls. The mean age was 7 ± 4 years. Nephrotic syndrome was observed in 26 patients, hematuria was noticed in 2 patients and renal insufficiency was detected in 2 patients at presentation. FSGS, not otherwise specified, was the predominant variant. All patients with nephrotic syndrome were treated with steroids. Only three patients responded to it. Twenty one patients were treated with cisclosporin A and this resulted in a 57% complete remission and a 24% partial response. Cyclophosphamide was administered to 6 patients and engendered a 50% complete remission. Six patients were treated with mycophenolate mophetil and showed no response in all cases. Renal insufficiency has been developed in 12 children. Conclusion Results from this study showed that the majority of children with FSGS achieve a high sustained remission rate with ciclosporine A.

Peritoneal dialysis: Experience of the department of pediatrics of the hospital Charles Nicolle of Tunis.

Introduction Peritoneal dialysis (PD) is still the most common modality used in treatment for children with End Stage Renal Disease (ESRD). The objective of this study was to identify the epidemiological, clinical, and microbiological factors affecting the outcome of PD. Methods In this study, we retrospectively reviewed the records of 85 patients who were treated with DP for the last ten years (from January 2004 to December 2013) in the Department of Pediatrics in Charles Nicolle hospital, Tunis. Results The mean duration of PD was 18.1±12 months (3.5-75 months). The average age of PD onset was 9.3±5.7 years (29 days-23 years). The sex ratio was 1.5. In a significant number of cases with ESRD, the primary cause is Congenital Anomalies of the Kidneys and Urinary Tract (CAKUT). Seventy-four of our patients (87%) had been treated with Automated PD. The average time between catheter placement and PD commencement was 3.9±4.6 days. Catheter change was 1.62 (1-5). Sixty-one patients (71.8%) had experienced at least one episode of peritonitis. The most frequently isolated organisms was the Gram-positive bacteria (61%). Survival rates without peritonitis at 12th, 24th and 36th months were 40%, 32% and 18%, respectively. Transition to permanent hemodialysis was required in 66% of patients. Conclusion Considering the important incidence of peritonitis in our patients, it is imperative to establish a targeted primary prevention.

Contribution of ultrasound scans in the first episode of urinary tract infection in children.

Background - Vesicoureteral reflux (VUR) is a common pediatric urologic disorder. After the first urinary tract infection (UTI), imaging studies are recommended, starting with a renal ultrasound (US) and voiding cystourethrography (VCUG). We propose to determine whether abnormalities found on US can help indicate the necessity of VCUG in children after the first urinary tract infection. Methods - A retrospective study included all children admitted with their first episode of urinary tract infection from January 2007 to December 2012. Results - A total of 311 children were included. The median age was 2.5 years, 72.3% were female. VUR Prevalence was 14%.  Forty-four patients were found to have VUR on VCUG, giving a prevalence of 14%. Of these 44 patients, 11 had grade I reflux, 6 had grade II reflux, 3 had grade III reflux, 15had grade IV reflux, and 9 had grade V reflux. Ultrasound findings were positive for VUR in 43 patients, 19 of them had RVU. Twenty five patients had a normal ultrasound but showed VUR on VCUG (11 had grade I reflux, six grade II reflux, three grade III reflux and five grade IV reflux).  The sensitivity and specificity of ultrasound in suggesting VUR were 43% and 91%, respectively. The positive predictive value of ultrasound in suggesting VUR was 44%; the negative predictive value was 91%. Conclusion - Renal ultrasound findings are specific for VUR in children with a first UTI, but no sensitive. Clinicians should consider renal ultrasound results to take decision on whether or not to proceed with a VCUG in the investigation of a first episode UTI in young children.

Nephrotic syndrome in children: risk factors for steroid dependence.

Background - Most patients with idiopathic nephrotic syndrome are steroid-responsive, about 50% relapse and often become steroid-dependent and exposed to long-term steroid complications. The aim of this study was to determine predictive risk factors for steroid dependence using clinical and biological variables present at onset of the disease. It may be useful to adapt the therapeutic strategy. Methods - Retrospective hospital-based cohort study in the department of pediatric nephrology of Charles Nicolle, Tunis, between 2002 and 2012 included 52 children with idiopathic steroid-responsive nephrotic. Results - Risk factors for steroid dependency identified from univariate analysis were season of the first episode (winter or autumn) (p=0.008), hyperalpha2globulinemia>16g/l (p=0.028), hyperbeta2globulinemia >3g/l (p=0.003), hypercholesterolemia>10mmol/l (p=0.001), proteinuria>110mg/kg/day (p=0.05), time to achieve remission>9 days (p=0,0001). Logistic regression revealed that time to first remission and hypercholesterolemia>10mmol/l at first presentation were independent risk factors for steroid dependency. Conclusion - Time to first remission, hypercholesterolemia more than 10mmol/l at first presentation and a respiratory tract infection at first relapse are independent risk factors for steroid dependency.

Mycophenolate mofetil in treatment of childhood steroid-dependent nephrotic syndrome.

OBJECTIVES: To establish the efficacy of mycophenolate mofetil (MMF) in steroid dependent nephrotic syndrome and to determine the predictive factors for a good response. METHODS: retrospective hospital-based cohort study in the department of pediatric of Charles Nicolle hospital, between 2005 and 2012 included 30 children with steroid-dependent nephritic syndrome who were treated with MMF. RESULTS: A total of 30 patients (20 boys and 10 girls) were included. The mean age at the time of diagnosis was 5.45 years and treatment with MMF was performed at a mean age of 10.84 years. Side effects of steroid were found in 17 patients. Four patients had renal impairment (ciclotoxicity). The evolution of the disease was 5.45 years. The average rate of relapse was 1.75 relapses / year. The minimum dose of corticosteroids was 0.74 mg / kg / day. During MMF therapy, the average rate of relapse was 0.45 relapses / year (p<0.0001). The average residual steroid dose was 0.2 mg/kg/ day. Responding patients were younger at the onset of MMF (8.57 versus 12.83, p=0.009), had a short development period (3.75 vs 7.03 years, p=0.05), had not received cyclosporine (p=0.02). CONCLUSION: MMF allows steroid sparing and reduces the number of relapse. It is more effective than the patients are young, with short disease outcome and had not previously been treated with cyclosporine.

Prospective study investigating the influence of nutritional intervention on biochemical profiles in patients with recurrent urolithiasis.

BACKGROUND AND OBJECTIVES: Urolithiasis, commonly known as kidney stones, is a condition significantly impacted by dietary habits. The objective of this study is to evaluate the impact of a tailored dietary plan on the crystalluria and biological parameters of patients with different types of kidney stones over a 3-month period. METHODS AND STUDY DESIGN: We conducted a prospective study of 3 months. The study involved patients with recurrent nephrolithiasis. Alongside the medical consultation, a comprehensive dietary survey was performed to assess the patients' nutritional habits. Urinary parameters, including volume, calcium, oxalate, uric acid, and power of hydrogen (pH), were evaluated both before and after the dietary intervention. RESULTS: 69 patients were involved. There were 17 patients diagnosed with cystine lithiasis, 33 with oxalocalcic lithiasis and 19 with uric lithiasis. After 3 months, only 32 patients revisited for follow-up. There were significant changes (p = 0.002 and 0.04) in urine crystalluria for cystinic and uric lithiasis. For the urinary oxalate variation, there was a significant decrease from T1 (before dietary intervention) to T2 (after dietary intervention), with levels dropping from 0.289 ± 0.10 umol/l to 0.215 ± 0.079 umol/l (p = 0.02).Regarding urinary calcium (calciuria), there was a trend toward a decrease from T1 to T2, although the change was not statistically significant, with levels decreasing from 2.42 ± 1.68 umol/l to 2.14 ± 1.62 umol/l (p = 0.1). CONCLUSIONS: Our research underscores the favorable effects of a tailored and well-balanced diet on both the crystalluria and biological parameters of individuals with recurrent lithiasis.

[Predictive factors of severe Henoch-Schonlein nephritis in children: report of 34 cases]. / Les facteurs préédictifs des atteintes rénales sévères du purpura rhumatoïde chez l'enfant: a propos de 34 cas.

BACKGROUND: Henoch Schonlein Purpura is the most frequent vasculitis in children. Renal involvement is variable. Renal manifestations vary from isolated microscopic hematuria to the association on nephrotic syndrome to nephritic syndrome. AIM: To determine the predictors of severe Henoch - Schönlein nephritis. METHODS: Retrospective study over 15 years (1996-2010) of 34 chidren, with henoch-schonlein nephritis. RESULTS: Renal involvement was determined in 68.7%. Mean age was 7.23 years (3-14 years). Renal manifestations were variable. Moderate renal manifestations were noted in 15 cases. Microscopic hematuria was observed in 23.5% of cases and moderate proteinuria with or without hematuria is noted in 20.5% of cases. Severe nephritis was noted in 18 cases: nephrotic syndrome in 29.5 % and nephrotic syndrome associated to nephritic syndrome in 23.5%. Hypertension without urinary anomalies was observed in one case. In univariate analysis, factor predictive of severe nephritis were: male sex, macroscopic hematuria, biologic inflammatory syndrome and leukocytosis. In multivariate analysis, only the leukocytosis was predictor of severity. CONCLUSION: In our study, only leukocytosis was predictor of severity in henoch-schönlein nephritis.

Systemic lupus erythematosus-related acute pancreatitis: An exceptional form with severe exocrine and endocrine pancreatitic failure in a Tunisian child.

Acute pancreatitis may be the first manifestation in systemic lupus erythematosus or occur during evolution. It is a rare complication, which is often associated with other visceral manifestations. Outcome is usually favorable but can be serious. We report a case of a 17-year-old girl with a past history of systemic lupus erythematosus who developed acute pancreatitis revealed by abdominal pain. Elevated serum amylase and lipase levels and pancreatic enlargement on tomography confirmed the diagnosis. Although high-dose corticosteroid was prescribed, the patient died from a refractory diabetic ketoacidosis.

Infectious-mononucleosis-like exanthema associated with COVID-19 in a child.

Cutaneous manifestations of childhood COVID-19 differ from those of adults. Maculopapular rash is not specific and could be mistaken with other viral exanthema. A nasopharyngeal swab is strongly recommended to confirm the possible COVID-19 diagnosis.

Primary hyperoxaluria in infants.

The infantile form of primary hyperoxaluria type-1 (PH-1) is characterized by a rapid progression to the end-stage renal disease (ESRD) due to both increased oxalate load and reduced glomerular filtration rate. In the literature, data on this form are limited. The purpose of this study is to analyze retrospectively the clinical, biological, and radiological features of children who were diagnosed with PH-1 during the 1(st) year of life. We reviewed the records of all children with PH-1 diagnosed and followed-up at our department between January 1995 and December 2013. Among them, only infants younger than 12 months of age were retrospectively enrolled in the study. Fourteen infants with the median age of two months were enrolled in the study. At diagnosis, 11 patients had ESRD. All patients had nephrocalcinosis and two of them had calculi. The diagnosis was established in nine patients on the basis of the positive family history of PH-1, bilateral nephrocalcinosis, and quantitative crystalluria. In four patients, the diagnosis was made with molecular analysis of DNA. Kidney biopsy contributed to the diagnosis in one patient. During follow-up, two patients were pyridoxine sensitive and preserved renal function. Seven among 11 patients who had ESRD died, four patients are currently undergoing peritoneal dialysis. Children with infantile PH and ESRD are at high risk of early death. Peritoneal dialysis is not a treatment of choice. Combined liver-kidney transplantation is mandatory.

[Etiologies of end-stage renal disease of children in Tunisia]. / Épidémiologie de l'insuffisance rénale terminale de l'enfant en Tunisie.

BACKGROUND: The end-stage renal disease (ESRD) in children has special features in terms of etiologies, therapeutic modalities and access to renal transplantation. In Tunisia, there are no data on the epidemiology of ESRD in children. The aim of our study was to describe epidemiology of ESRD among Tunisian children. METHODS: This retrospective study was conducted in pediatric departments in Charles-Nicolle Hospital, Tunis and Hedi Chaker hospital, Sfax, during a period of 15 years (1st January 1998-31st December 2013). We included children who develop ESRD before the age of 15 years. RESULTS: In total, 166 patients were included. The median duration of follow-up was 48 months. We collected respectively 24 children (14.5%) aged less than 2 years, 24 children (14.5%) aged between 2 and 6 years and 118 children (71%) older than 6 years. The sex ratio was equal to 1.4. The mean incidence was 4.25 cases per million children. The main causes were represented by congenital anomalies of the kidneys and urinary tract (35.5%), hereditary renal disease (31.3%) and glomerular kidney disease (9.6%). All patients were treated in kidney transplant dialysis programs; the main mode of dialysis was represented by peritoneal dialysis, which represented the initial dialysis mode in 81% of cases. The transition to hemodialysis was noted in 43.4% cases. Thirty-eight patients (22.8%) were transplanted. The mortality rate was 27.1%. The leading cause of death was cardiovascular diseases (37.7%) and infections (22.2%). CONCLUSION: The creation of a national registry of kidney disease in Tunisia is necessary for a better knowledge of needs for dialysis and renal transplantation in children.

[Outcome of rapidly progressive glomerulonephritis post-streptococcal disease in children]. / Profil évolutif de la glomérulonéphrite rapidement progressive post-infectieuse de l'enfant.

BACKGROUND: Rapidly progressive glomerulonephritis is a rare form of postinfectious glomerulonephritis. The aim of this study was to describe the outcome of our patients with severe post-streptococcal glomerulonephritis. METHODS: This retrospective study was conducted in the department of pediatrics in Charles-Nicolle Hospital during a period of 13 years (1997-2009). RESULTS: Twenty-seven children were identified. The mean age was 8.7 years. All patients presented renal failure at presentation. The mean serum creatinine at presentation was 376.9 µmol/L. Six patients presented nephrotic syndrome. Twenty-six children had renal biopsies. Renal biopsies showed crescents in 24 cases. Eighteen children received pulse dose of corticosteroids (66.6%) and 6 children (22%) received pulse dose of corticosteroids and cyclophosphamide. Eleven patients required dialysis. At last follow-up, 22 patients (81.5%) had normal kidney function, 2 had renal dysfunction and 3 reached end stage renal disease. The only significant determinant for renal survival was the supportive dialysis (P=0.015). CONCLUSION: Rapidly progressive glomerulonephritis is uncommon. There have been significant advancements in supportive, as well as specific therapy, but the outcome continues to be poor.

[Peritonitis in pediatric patients receiving peritoneal dialysis]. / Péritonite infectieuse sur cathéter de dialyse péritonéale chez l'enfant.

BACKGROUND: Peritonitis on catheter of dialysis represents the most frequent complication of the peritoneal dialysis (PD) in the pediatric population. It remains a significant cause of morbidity and mortality. In this study, we investigated the risk factors for peritonitis in children. METHODS: In this study, we retrospectively collected the records of 85 patients who were treated with PD within the past ten years in the service of pediatrics of the University Hospital Charles-Nicolle of Tunis. RESULTS: Peritonitis rate was 0.75 episode per patient-year. Notably, peritonitis caused by Gram-positive organisms were more common. Analysis of infection risk revealed three significant independent factors: the poor weight (P=0.0045), the non-automated PD (P=0.02) and the short delay from catheter insertion to starting PD (P=0.02). The early onset peritonitis was significantly associated with frequent peritonitis episodes (P=0.0008). The mean duration between the first and second episode of peritonitis was significantly shorter than between PD commencement and the first episode of peritonitis. We revealed a significant association between Gram-negative peritonitis and the presence of ureterostomy (0.018) and between Gram-positive peritonitis and the presence of exit-site and tunnel infections (0.02). Transition to permanent hemodialysis was needed in many children but no death occurred in patients with peritonitis. CONCLUSION: Considering the important incidence of peritonitis in our patients, it is imperative to establish a targeted primary prevention. Nutritional care must be provided to children to avoid poor weight. The automated dialysis has to be the modality of choice.

Acute pancreatitis: a rare complication in a patient with senior loken syndrome.

INTRODUCTION: Senior-Loken syndrome is a rare entity that combines familial nephronophthisisand retinal dystrophy. It has an autosomal recessive inheritance pattern and is characterized by a chronic tubulointerstitial nephritis that progress to terminal renal failure during the first or second decades of life. Systemic associations of this syndrome include sensorineural hearing loss, liver fibrosis or cerebral vermis hypoplasia. Acute pancreatitis has not been previously reported in this syndrome. CASE REPORT: This is a 28- years-old patient who was diagnosed to have Senior Loken syndrome at the age of 10 years because of renal impairment and tapetoretinal degeneration and was later started on regular hemodialysis. He had no family history of renal disease, hypertriglyceridemia or cholelithiasis. He presented to our center complaining of acute abdominal pain and vomiting. He had abdominal tenderness without guarding. Investigations revealed a lipase level of 3856 I U/l and an abdominal CT scan showed features of acute pancreatitis. The abdominal ultrasound showed no biliary tree malformations or gallstone obstruction. He had no history of recent drug intake or alcohol consumption and his serum triglyceride level was normal. A diagnosis of moderate acute pancreatitis was made and the patient was managed conservatively with good outcome. CONCLUSION: Taking into consideration the uncertainty about the presence of liver fibrosis and the fact that imaging may have missed a passing gallstone, this case may indicate another rare systemic complication of Senior-Loken syndrome.