Biomedical subjects
Publications and source records attributed to Riadh Jouini.
Rhabdoid tumour of the kidney is an extremely rare cancer in children, which raises aetiopathogenic, diagnostic and therapeutic problems. Treatment of this tumour is not well defined and its prognosis remains poor despite progress in paediatric oncology. The authors report a new case of neonatal rhabdoid tumour of the kidney, with a rapidly fatal outcome and present a review of the literature.
OBJECTIVE: To define the epidemiological and clinical characteristics of urinary stones in infants, to study the role of stone chemical analysis in the aetiological assessment of urinary stones and to define the various treatment modalities adapted to this age-group. PATIENTS AND METHODS: Between 1984 and 2002, 64 infants (age: 5-24 months) were hospitalised for urinary stones. Urine culture was performed in all patients and metabolic assessment was performed in 24 patients. Physicochemical stone analysis was performed by infrared spectrophotometry in 37 patients. RESULTS: Upper tract and lower tract stones were equally prevalent. Urine culture was positive in 48 cases. The micro-organism most frequently isolated was Proteus mirabilis (19 cases). The metabolic assessment was normal in 15 patients and pathological in 9 patients. Infrared spectrophotometry showed that 17 stones were pure. 60 patients were treated surgically, 2 were treated by endoscopy associated with intracorporeal lithotripsy. One patient was treated medically and another patient passed the stone spontaneously while in hospital. No intraoperative or postoperative complication was observed. No recurrence was observed in this series. The mean follow-up is 16 months (range: 6 months to 94 months). CONCLUSION: The epidemiological profile of urinary stones in infants in Tunisia is situated between that observed in developed countries and that observed in developing countries. In our study, the incidence of metabolic abnormalities appears to be low despite a high rate of consanguinity in Tunisia. This can be largely explained by the absence of an aetiological survey and/or an inadequate survey when it is performed.
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Nephroblastomatosis is a rare disease, considered to be a precursor of Wilms tumour. When it is isolated, recommended treatment consists of chemotherapy followed by surveillance. In the presence of associated Wilms tumour, treatment consists of preoperative chemotherapy followed by partial nephrectomy (when possible), followed by postoperative chemotherapy and surveillance.
Cystic dysplasia of the testis is an exceptional benign tumour resulting from a functional abnormality of the connection between the rete testis and the efferent ductules of the epididymis. The authors report a new case observed in a child. The frequent association with a renal abnormality is highly suggestive. Treatment usually consists of orchidectomy, but can be conservative with long-term surveillance. The prognosis is usually excellent.
Desmoplastic small round cell tumor (DSRCT) correspond to a recent clinicopathological entity, individualized in 1989 into the group of tumors with small round cells. This pathology puts ethiopathogenic, diagnostic, therapeutic and prognosis problems. Indeed, the ethiopathogenic is still unknown, diagnosis is asserted only by immuno-histochimic and cytogenetic study because of the big number of differential diagnoses and the anatomopathologic polymorphism. Its treatment is not well codified and its outcome remains dark in spite of therapeutic progress. The objective of this work is to report a personal observation of a DSRCT and to proceed to a review of the literature to clarify the epidemiological, clinical, paraclinical and therapeutic aspects of this rare tumor.
OBJECTIVE: Psoas abscess is very rare in children and raises problems concerning the clinical and aetiological diagnosis. The authors describe the characteristics of this rare disease and emphasize the role of imaging in the diagnostic approach and therapeutic management. MATERIAL AND METHOD: The authors report a series of 18 cases children aged 11 months to 13 years treated between 1988 and 2000 in the Monastir department of paediatric surgery. The time to diagnosis ranged from 4 days to 1 month. The clinical features comprised fever, abdominal pain and functional impairment of the homolateral lower limb in 17 cases. RESULTS: Ultrasound was performed systematically and established the diagnosis in 17 cases. Computed tomography, performed in 13 cases, confirmed the diagnosis and demonstrated the aetiology in 3 cases. The microorganism was isolated in 16 cases (Staphylococcus aureus in 13 cases). An aetiology was identified in 4 cases: acute appendicitis, Potts disease, sacro-iliitis and perirenal abscess. Treatment comprised systematic antibiotic therapy combined with drainage of the abscess in 16 cases, which was performed surgically in 4 cases and percutaneously in 12 cases. CONCLUSION: The prognosis of psoas abscess in children is generally favourable. CT- or ultrasound-guided percutaneous drainage is a valuable alternative to surgery, which must be reserved for cases of failure of percutaneous drainage.
Xanthogranulomatous pyelonephritis (XGP) is an unusual and rare form of chronic renal suppuration in children, which appear to have a multifactorial pathogenesis. It is usually diffuse, but can remain localized to one part of the kidney, resulting in a misleading pseudoneoplastic appearance raising a problem of differential diagnosis with the other renal masses, particularly Wilms tumour. The authors present a retrospective analysis of three cases of XGP observed in the Monastir department of paediatric surgery. The preoperative diagnosis was pyonephrosis with perirenal abscess in two cases and Wilms tumour in the third case. The diagnosis of XGP was established on histological examination of the operative specimens. The aim of this study is to emphasize the diagnostic difficulties of this disease that, in localized forms, often lead to inappropriate treatment and to establish a treatment regimen for this rare disease.
Mesoblastic nephroma is a rare renal tumour mainly observed in neonates and young infants. The authors report a case in a 5-day-old new-born infant presenting with an isolated abdominal mass occupying the left hypochondrium and flank. Ultrasonography demonstrated a 4 cm heterogeneous mass in the lower pole and middle of the left kidney. CT showed an intensely contrast-enhanced heterogeneous mass. The patient was treated by left radical nephrectomy with an uneventful postoperative course. The outcome was favourable without recurrence with a follow-up of one year. Mesoblastic nephroma is considered to be a benign tumour. The diagnosis is suggested by clinical, ultrasonographic and CT findings. Treatment is based on radical nephrectomy. In the light of this case, the authors discuss the clinical, radiological and therapeutic aspects of this tumour.
Transverse ectopic testis (TET) is a rare form of ectopic testis. The authors report the case of a 2-month-old infant presenting with right inguinoscrotal hernia and ectopic left testis with an impalpable testis. Opening of the hernia sac revealed two testes with two distally fused vasa deferentes. The contralateral testis was easily descended by translocation through the other inguinal canal. A favourable result was obtained with two testes situated in a normal position. In the light of this case, the authors emphasize the clinical and therapeutic features of this anomaly.