Mechanical complications of peritoneal dialysis in Tunisian children.

INTRODUCTION: Peritoneal dialysis (PD) is the method of choice for extra-renal replacement therapy (ERT) for children with end-stage renal disease (ESRD), because of its various advantages. However, it presents different infectious and non-infectious complications, causes of important morbidity and mortality. AIM: To determine the mechanical complications of PD in our center and to identify risk factors of their occurrence. METHODS: We retrospectively collected the records of 99 patients who were treated with PD within the past eleven years in the department of pediatrics of the University Hospital Charles Nicolle of Tunis. Analysis examining possible risque factors were performed using parametric and non-parametric tests. RESULTS: A total of 63 patients had mechanical complications with an incidence of peritoneal dialysis catheter revision of 1 procedure every 38 months. The causes were dominated by catheter migration (27.5%) and obstruction by fibrin or blood clotting (19.8%). A history of peritonitis (p=0.046) and the presence of comorbidity (p=0.008) were the two independent risk factors for catheter revision. Inguinal hernia was noted in six patients. No patient presented with a hydrothorax. Seven patients presented an episode of hemoperitoneum. CONCLUSION: Our results lead us to focus our efforts on preventing peritonitis and controlling morbidity. Prospective studies will enable us to confirm our results.

Alimentary tract duplications in children: report of 16 years' experience.

BACKGROUND: Alimentary tract duplications (ATDs) are a rare condition in children, characterised by a large pathogenic, clinical, and histological polymorphism. Surgical observation and pathologic evaluation of the resected specimens are the only way to confirm the diagnosis. In this study, we want to analyse the anatomical, clinical and therapeutic aspects of this entity. PATIENTS AND METHODS: A total of 12 cases of ATD were diagnosed over a 16-year period at paediatric surgery department. The diagnosis was evoked on clinical and radiological data. Histological study of the resected specimens confirmed the diagnosis in all cases. RESULTS: The mean age of patients at diagnosis was 41 months with a peak of incidence at the 1 st year of life (42%). Out of a total 12 cases, 10 were girls and 2 were boys. Abdominal pain and vomiting were the most frequent presenting features. Ultrasonography, tomodensitometry and magnetic resonance imaging were useful for diagnosis. ATDs were localised on the oesophagus in one case, the stomach in one case, the duodenum in four cases, the ileum in five cases, and the colon in one case. All these duplications were cystic, with three communicating duplications. All patients underwent surgery, and resection procedure was chosen according to duplication type and site. Histological study confirmed the diagnosis in all cases. CONCLUSION: ATDs are a rare condition in children. Diagnosis relies on histology, and treatment can only be by means of surgery. The outcome after surgery is generally favourable. Diagnosis and precocious surgery of ATDs can warn serious complications.

Peptic oesophageal stricture in children: management problems.

BACKGROUND: Peptic oesophageal stricture (PES) is a serious complication of gastroesophageal reflux disease (GERD) in childhood. The treatment of PES is still controversial, ranging from simple oesophageal dilations to resection/anastomosis of the stenotic portion of the oesophagus. In this study, we want to share our experience with 11 children with GERD and PES. PATIENTS AND METHODS: A retrospective review of clinical data obtained from children who underwent dilation and antireflux surgery for PES was performed. RESULTS: A total of 11 patients were diagnosed with PES. The clinical picture was dominated by dysphagia. Barium swallow showed hiatal hernia in nine cases (82%). Oesophageal strictures were located most commonly in the lower third of the oesophagus (91%). Three Children (27%) with PES had a neurologic impairment and patients had a mean duration of symptoms of 20 months (range, 3 month to 6.2 years) before intervention. Children received a median of four dilations (range, 1-21 dilations) for PES. Time to first dilation from age of diagnosis was a mean of 4.5 months (range, 2-14 months). Antireflux surgery was performed in all patients. Post-operatively, seven patients required repeat oesophageal dilation. Patients were followed with serial dilation for a median of 6 years (range, 1-9 years) and only one patient has a continued requirement of oesophageal dilation for PES. CONCLUSION: GERD complicated by PES is an important condition affecting a significant number of children. Early and effective treatment of both stricture and GERD is required to improve the prognosis of this serious condition.

[Enzyme replacement therapy for Gaucher paediatric disease: the only Tunisian experience]. / Traitement enzymatique substitutif de la maladie de Gaucher chez l'enfant: premiere experience Tunisienne.

AIM: We report through the first Tunisian experience with enzyme replacement therapy, the goals and consensus recommendations for treatment and monitoring of paediatric non neuronopathic Gaucher disease. METHODS: Three children with Gaucher disease undergone enzyme replacement therapy with Cerezyme for severe visceral and/or bone involvement. Visceral, hematologic, bone, and growth parameters were assessed initially and under treatment. RESULTS: Two children presented with severe visceral or hematologic picture. One patient had myocardiopathy and primitive portal hypertension and another was diagnosed with cirrhosis related to Gaucher disease. Recurrent avascular necrosis and osteoporosis have justified treatment in another child. All patients received an initial dose of 60U/Kg/2 weeks. We have seen a gradual disappearance of hepatosplenomegaly and a rapid normalisation of hematological parameters in two patients. A resistance to treatment indicated splenectomy in one patient. The improvement in bone mineral density was slower. A significant growth gain was observed in patients with growth retardation. No patient had developed Cerezyme antibodies. CONCLUSION: Despite its effectiveness and safety demonstrated in these children, enzyme replacement therapy remains inaccessible because of its cost for emerging countries. The allogeneic bone marrow is an alternative therapy to encourage and to propose precociously for severe paediatric forms of Gaucher disease.