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

[Henoch Schonlein nephrites in children: clinical features and outcome: about 34 cases]

To describe epidemiological, clinical, histological aspects, treatment and outcome of Henoch-Sch"nlein nephrits in children. A retrospective study was conducted on medical data of 34 patients with Henoch-Sch"nlein nephritis From January 1, 1996 to December 31, 2010 in the Pediatric Department of Charles Nicolle Hospital. Nephritis occured in 68,7%. The average patient age was 7 years 2 months and sex ratio was 0,6. Microscopic hematuria was noted in 23,5%, moderate proteinuria with or without hematuria was observed in 20,5%. Nephrotic syndrome was noted in 29,5%; nephritic syndrome was associated to nephritic syndrome in 23,5%. Isolated hypertension was noted in one patient. Follow-up was 2 years 6 months. Remission was noted in 26 cases [76,4%], minor urinary abnormalities in 7 cases [20,5%] and renal active disease in one case. Relapse was observed in 6 cases. Our study was characterized by the predominance of severe renal manifestations and low grade histological aspects as well as favourable outcome in most 90% of cases

[Thromboembolic complications of childhood nephrotic syndrome]

Vascular thrombosis in the childhood nephrotic syndrome is a rare event. It poses major diagnostic and therapeutic problems. The prognosis depends on early diagnosis and on precocious anticoagulation. The risk of extension of thrombosis and pulmonary embolism is real. To consider the major thrombotic events associated with childhood nephrotic syndrome and to establish an appropriate preventive approach based on objective clinical and laboratory parameters. This is a retrospective study of all cases of patients suffering from idiopathic nephrotic syndrome, during a period of 20 years, starting from January 1990 to December 2009. We selected six patients with vascular thrombosis. The diagnosis was confirmed by appropriate radiological investigation. Six cases of vascular thrombosis were identified among 260 cases of nephrotic syndrome collected during the period study. Patients are divided into five boys and one girl. The mean age was 13.3 years. The localisation of thrombosis is venous in all cases. One patient presented a massively fatal pulmonary embolism. Sinovenous thrombosis has been objectified in 3 patients who were all treated with a good therapeutic response. Vascular thrombosis remains one of the most serious complications of nephrotic syndrome in children. Early diagnosis and precocious anticoagulation are essential for preventing the extension of thrombosis

[Primary psoas abscesses of the psoas muscle in children]

Primary psoas abscesses of the psoas muscle are relatively rare in childhood and can determine problems of diagnosis and therapeutic assumption. To demonstrate that ultrasonography is an excellent means in diagnosis and treatment of psoas abscess in children limiting the use of CT more costly and radiant and the surgery that is decaying. Over a 15 years period [January 1995-december 2009], 16 children with psoas abscess were studied retrospectively. The diagnosis gait was based on questioning, clinical examination and imaging techniques and, in first sight, ultrasonography. Median age of our patients was 6.4 years [extremes 18 months-14 years] and mean delay of evolution was 12 days. Fever and lumbar pain were both constants, associated with another signs like painful boitery, psoitis, mictional burns and lumbar mass. The ultrasonography made in 16 patients allowed to the diagnosis in all cases. The tomodensitometry, made in 3 patients, confirmed the ultrasonography. Echo guided percutaneous drainage, made in 14 cases, was successful in 12. The examination of pus showed S Aureus in 11 cases and E coli in the other. Ultrasonography is a very important means in the primary psoas abscess in children. In addition to give diagnosis, this technique allows the drainage and the cure of the collection, limiting the open surgery to some particular cases

Primary hyperoxaluria type 1 in Tunisian children

Primary hyperoxaliuria type 1 is an autosomal-recessive disorder characterized by increasing urinary excretion of calcium oxalate, recurrent urolithiasis, nephrocalcinosis, and accumulation of insoluble oxalate throughout the body. This inborn error of metabolism appears to be a common cause of end stage renal disease in Tunisia. To review the clinical, biological and radiological futures of primary hyperoxaluria type 1 and to correlate these aspects with the development of end-stage renal disease. we retrospectively reviewed 44 children with Primary hyperoxaliuria type I who were treated in our department during a period of 15 years between 1995 and 2009. The diagnosis was established by quantitative urinary oxalate excretion. In patient with renal impairment, the diagnosis was made by infrared spectroscopy of stone or by renal biopsy. Male to female ratio was 1.2. The median age at diagnosis was 5.75 years. About 43% of those were diagnosed before the age of 5 years. Initial symptoms were dominated by uraemia. Four patients were asymptomatic and diagnosed by sibling screening of known patients. Nephrocalcinosis was present in all patients. It is cortical in 34%, medullary in 32% and global in 34%. At diagnosis, twelve children were in end-stage renal disease [27%]. Pyridoxine response, which is defined by a reduction in urine oxalate excretion of 60% or more, was found in 27%. In the majority of patients, the clinical expression of Primary hyperoxaliuria type 1 is characterized by nephrocalcinosis, urolithiasis and renal failure. Pyridoxine sensitivity is associated with better outcome

Histopathological spectrum of childhood idiopathic steroid-resistant nephrotic syndrome in Tunisia

In children, renal biopsy is routinely required in the management of idiopathic steroid-resistant nephrotic syndrome particularly prior to starting nephrotoxic immunosuppressive agents. To investigate the correlations between the results of initial renal biopsy in Tunisian children with idiopathic steroid-resistant nephrotic syndrome and the subsequent response to cyclosporine-prednisolone combination. We conducted a retrospective study of children with idiopathic steroid-resistant nephrotic syndrome over the period 2002- 2009. Data on clinico-biological features, histological diagnosis and response to cyclosporine-prednisolone were collected. Thirty patients were enrolled, of whom 16 had focal segmental glomerulosclerosis, eight had minimal change disease and six had diffuse mesangial proliferation. Complete Remission was achieved in 15 patients [50%]. Nine patients [30%] went into partial remission. Only six patients presented no response [20%]. No statistically significant relationship between the different pathological types and the response to CsA-prednisone was found. In our study, two important facts were noted: 1] the predominant histopathological subtype was the focal segmental glomerulosclerosis; 2] a high remission rate was achieved in our patients using a combined cyclosporine-prednisolone treatment regimen. This response is not dependent on the histological type

Vascular complications after pediatric kidney transplantation

Vascular complications, especially those including the renal vein, remain a major cause of lost graft. To evaluate retrospectively the incidence and management of vascular complications after pediatric renal transplantation and to assess possible risk factors and their effects on patient and graft. A total of 82 consecutive renal transplants were performed in 79 patients at a single institution. The diagnosis of vascular complications was suspected in the presence of suggestive symptoms and confirmed by Doppler ultrasound and if necessary by a computed tomographic angiography. Urgent exploration was performed in all suspected cases. There were seven vascular complications [8,5%], including renal vein thrombosis in four patients, renal artery stenosis in one, and sural thrombophlebitis in two. The thrombosis of the graft vein which is the main complication occurred at mean 24 hours after renal transplantation. All these patients needed transplant nephrectomy after thrombosis event. In the remaining cases, the outcome was favorable even for the patient with transplant renal artery stenosis. Vascular complications are common and serious events affecting patient and graft survivals. A perfect surgical technique and rigorous radiological monitoring may result in decreased incidence and severity of these complications

[Posterior uretral valves, about a series of 44 cases]

To explore the physical symptoms and radiological findings for the diagnosis of posterior urethral valves [PUV], to point of the therapeutic snags depending on gravity and delay of lesions. We reviewed the records of 44 patients with PUV. Mean age: 2 years 1/2 [range 1 day-13 years]. More than 75% of patients were less than 2 years old. In utero diagnosis was made in 8 patients [14%]. After birth, the diagnosis was based on urological signs [as frequent as diagnosis is made belatedly] and extra urological signs. Cystourethrography [CUG] showed posterior urethral dilation in all cases. Ultrasonography [US] showed abnormalities in 30 cases and intravenous urography [IVU], made in 21 cases, was normal in appearance in 7 of them. At diagnosis, it was a renal failure in 36 patients and 8 of them had a terminal renal deficiency. Urethroscopy made in 40 patients, showed PUV in all cases. There were 29 types I valves, 3 type II valves, 5 type III and 3 unclassifiables cases. Treatment was endoscopic in 41 cases and by lamination in the 3 others. Mean follow up is 9 years [range16months-19 years] and 19 patients have terminal renal failure from which 3 are deceased. PUV are dangerous obstructive uropathies in boy whom end at terminal renal failure in more than 33% of cases. Precocious diagnosis and early ablation of PUV are able to limit the complications inherent to this pathology

[Uropathies and nephropathies in the Bardet-Biedl in child]

The Bardet-Biedl syndrome is an autosomal recessive disease, characterized by obesity, retinal degeneration, hypegenitalism in men, polydactylism and on often moderate mental retardation. With these cardinal features, others clinical findings [secondary features] including diabetes, congenital heart defects, hypertension or syndactyly can be seen. Renal involvement is almost constant, but varies from a moderate impairment of the tubular functions to chronic renal failure caused by malformative uropathy or glomerulopathy. Report a new cases. We report 6 patients with Bardet-Biedel syndrome who had renal involvement. Three patients had cystic dysplasia, one patient an increased fractional sodium excretion, one other a vesico-ureteral reflux and the last patient developed end-stage renal failure following acute post streptococcal glomerulonephritis. We insist on precocious diagnosis and multidisciplinary treatment of these renal lesions, to avoid or, at least, to slow down the evolution to the terminal renal failure, essential prognosis factor. Renal involvement, is considered as a major criteria predicting high morbidity and mortality during Bardet-Diedl disease

[Urinary tract infections in children following renal transplantation: a single-center experience]

Urinary tract infection [UTI] is the most common complication after kidney transplantation and represents a potential life-threatening risk for the immuocompromised child. The aim of this report is to determinate incidence, risk factors, microbilogic features and evaluate the impact of this complication on graft outcome and patient mortality. We performed a retrospective cohort study reviewing the medical records of 17 children from 38 who received a renal transplant in our center between January 1992 and June 2008 and who present an urinary tract infection. All patients received Lich-Gregoire implantation and insertion of double-J stunt. Antibioprophylaxis was not systematic. After a mean period of 6 years, 9 children [5 boys+4 girl] developed early UTI [during the first month after transplantation] and 5 [3boys + 2 girl] had late UTI. Three patients [2boys+1girl] with an indeterminate nephropathy developed early and late UTI. Causal agents are: E.Coli, Klebsiella Pneumoniae and Candida albicans. The further voiding cystourethrography showed a vesico-ureteral reflux on graft in 5 cases. Among the 17 patients, 4 lost their graft and are actually on haemodialysis. The urinary tract infection represents the major complication after renal transplantation. Diagnosis ant treatment must be made early to avoid the loss of the graft