Spontaneous perforation of the extrahepatic bile duct in infancy: report of two cases and literature review.
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Bile Duct Diseases↗
Biomedical subjects
A Nouri
Publications and source records attributed to A Nouri.
[Intussusception owing to pathologic lead points in children: report of 27 cases].
OBJECTIVE: Intussusception owing to pathologic lead points is a challenging condition for pediatric surgeons. The aim of this study was to review the particularities of clinical presentation, the place of morphologic investigation in depicting the presence of an organic lesion and the management of secondary intussusception. PATIENTS AND METHODS: The authors report a series of 27 patients treated from 1986 to 2004, for secondary intussusception. RESULTS: Nineteen boys and 8 girls, aged from 45 days to 11 years (mean age: 40 months) presented with secondary intussusception: Meckel's diverticulum (13 cases); lymphoma (8 cases); intestinal duplication (3 cases); heterotopic pancreas (2 cases); intestinal polyp (1 case). All patients were operated upon after failure of hydrostatic reduction. An intestinal resection with an end to end anastomosis was done for 26 patients. The biopsy of a large abdominal mass after an easy reduction of the intussusception was performed in 1 case. Chemotherapy was started at the sixth postoperative day for the 8 children having lymphoma. Two of them died during therapy. For the 25 others, the postoperative course was uneventful with a mean follow-up of 4 years. COMMENTARY: The improvement of the management and the prognosis of secondary intussusception requires an early diagnosis. Morphologic examination must not be limited to the diagnostic of intussusception but must aim at searching a lead point. The reduction of this particular form is based exclusively on surgery.
Child↗
[Morgagni hernia in infancy: report of 7 cases].
BACKGROUND: Morgagni or retrosternal hernia is a rare entity. It's representing almost 4% of all types of congenital diaphragmatic hernias in children. POPULATION AND METHODS: The data concerning 7 infants with Morgagni hernias (MH) have been retrospectively reviewed by the authors. RESULTS: Their ages at presentation ranged from 7 months to 11 years. There were 4 males and 3 females. The majority of patients had repeated chest infections. The diagnosis was made on chest radiograph and barium enema. All patients were operated through the abdomen (5 upper midline, 2 laparoscopic approach). The hernia sacs were excised and the defects repaired in all patients. DISCUSSION: Clinical awareness, early diagnosis and surgical treatment especially with laparoscopic correction, are important factors. CONCLUSION: Surgical correction is needed for asymptomatic MH in children to obviate the risk of complications and because the treatment is easy.
Bronchopneumonia↗
[Congenital esophageal stenosis due to tracheobronchial remnants: report of 2 cases and literature review].
Congenital esophageal stenosis due to tracheobronchial remnants is a rare malformation whose diagnosis may be difficult. It is characterised by the abnormal presence of congenital tissue of tracheal origin in the esophageal wall, which is responsible for the narrowing of the esophagus. We report 2 cases whose treatment was surgical after failure of esophageal dilations. The presence of tracheal-bronchial tissue was confirmed by histological examination of the operative piece. Outcome was favourable and the final result was excellent. Recently, endoscopic ultrasonography has been proved useful in the diagnosis of congenital esophageal stenosis due to tracheobronchial remnants by showing the presence of cartilage, which explains the failure of dilation. The high rate of perforation in these cases is due to brutal fragmentation of the cartilaginous rings. Surgical resection of esophageal stenosis with the tracheobronchial tissue appears the only treatment susceptible to completely suppress the stenosis and its consequences.
Bronchi↗
Genetic differences between Tunisian camel and sheep strains of the cestode Echinococcus granulosus revealed by SSCP.
Ovine and dromedary Echinococcus granulosus isolates from Tunisia were identified as G1 and G6 strains based on polymorphism of the mitochondrial cytochrome C oxydase CO1. Single strand conformation polymorphism (SSCP) was used in order to examine the genetic variation within and between Tunisian G1 and G6 strains and to estimate the extent of selfing. The dromedary isolates are genetically distinct from sheep isolates (high value of genetic variation between populations: Fst= 0.46). No significant deficiency in heterozygotes was found in sheep isolates, whereas heterozygote deficiency (suggesting selfing) was found in a limited number of camel isolates.
Animals↗
Molecular evidence of ovine (G1) and camel (G6) strains of Echinococcus granulosus in Tunisia and putative role of cattle in human contamination.
Three hundred and seventy-two cysts coming from 50 humans, 166 cattle, 153 sheep and 3 camels were collected in order to establish some epidemiological molecular information in Tunisia for the first time. The analysis by PCR-RFLP of ITS1 sequence showed that all the human, ovine and bovine cysts were due to the common sheep strain of Echinococcus granulosus. The sequencing of the CO1 gene of 37 isolates confirm the G1 genotype of this strain. For seven of these isolates, we found the mutation C56T which is present in the three principal intermediate hosts: human (three cysts), cattle (three cysts) and sheep (one cyst). With regard to the G1 genotype, we identified three other point mutations. The camel strain G6 is uniquely found in the three camels isolates and not in the other intermediate hosts analysed. The fertility of the bovine cyst represents 48% that means that this host is involved in a bovine-dog cycle and consequently represents a reservoir of sheep strain in Tunisia. Our results confirm the importance of the prophylaxis measures in order to disrupt the cycle of transmission sheep-dog in Tunisia. Nevertheless, the supervision of bovine infection should be reinforced because this intermediate host may constitute an important link with the human contamination.
Animals↗
[Imaging of pulmonary hydatid cyst in children].
PURPOSE: We present the various imaging features of pediatric pulmonary hydatidosis based upon a series of 232 cases. The importance of chest radiographs and thoraco-abdominal ultrasound is emphasized. PATIENTS AND METHODS: A retrospective study of 232 children with pulmonary hydatidosis is presented. The 232 cases (130 boys and 102 girls, age range: 18 months to 14 years) were studied between January 1982 and December 2001. Chest radiographs were available in all cases, chest ultrasound in 156 cases and abdominal ultrasound in 212 cases. CT was only performed in 40 cases. Surgery was performed for all patients. Diagnosis was confirmed with serology tests and/or pathological study of resected cysts. RESULT: The total number of pulmonary cysts detected on chest radiographs was 344. The right lung was more frequently affected than the left (208 lesions). A simple cyst was the most commonly observed lesion (220 cysts). Complicated forms were noted in 118 cases. 180 cysts were observed at ultrasound. A simple cyst presenting as an anechoic mass was noted in 140 cases. In 38 cases, the lesion was heterogeneous. In two lesions endocystic germinal membranes were detected. CT visualized 50 lesions. Thirty three lesions were complicated with endocystic floating or collapsed membranes. Bronchiectasis was observed in seven cases. CONCLUSION: Chest radiographs and thoraco-abdominal ultrasound are very useful for the diagnosis of pulmonary hydatidosis and evaluation of lesion extension. CT is useful for diagnosis of atypical or complicated lesions and to detect bronchiectasis.
Adolescent↗
[Omental lipoblastoma: a case report].
Lipoblastoma is a rare benign neoplasm of foetal fat tissue. To our knowledge, less than ten cases of omental lipoblastoma have been reported in the literature. We present a new case of omental lipoblastoma in a 3-month old girl. The role of CT in the diagnosis and surgery planning of this tumor will be discussed.
Child, Preschool↗
Genetic relationships between sheep, cattle and human Echinococcus infection in Tunisia.
Allozyme variation at seven polymorphic loci (GPI, EST, MDH, MPI, DIA, PEP, PGM) was studied to examine genetic variation within and between sheep, cattle and human populations of Echinococcus granulosus in Tunisia. A high degree of genetic similarity was shown between the cysts of the three host origins. Nevertheless, whereas, the ovine and human samples were highly similar, the cattle samples were slightly different genetically. We conclude that humans are mostly infected by parasites originating from sheep liver. The intense deficiency in heterozygotes was partly artefactual (Wahlund effect) and partly due to self-fertilisation.
Adolescent↗
[Radiological quiz of the month: Morgagni hernia: a case report].
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Child↗
[Urethral duplication in boys: report of 4 cases].
Urethral duplication is a rare anomaly, affecting mainly boys. The clinical presentation varies because of the different anatomical patterns of this abnormality. We report 4 cases of urethral duplication in children. An epispadiac duplication was present in 1 case, hypospadiac duplication in 1 case, Y-duplication in 1 case and a congenital prepubic sinus in the last case. The pre-operative probe was based on urethrocystography and fistulography. The surgical management included excision of the duplicated urethra in 3 cases. Transurethral incision of the intra-urethral septum was performed in the case of hypospadiac duplication. The characteristics of male urethral duplication are reviewed.
Child↗
[Malignant renal tumors before one year of age. Experience of a North African pediatric surgery service].
UNLABELLED: The malignant tumours of the kidney are not very frequent during the first year of life and pose diagnostic and therapeutic problems. The aim of this work is to make an analysis of the epidemiologic, clinical and anatomo-pathological characteristics of these tumours during the first year of life and a development on the therapeutic methods and their results. MATERIAL AND METHODS: [corrected] This is a retrospective study of 8 observations of malignant tumours of the kidney whose first symptomatology appeared during the first year of life. RESULTS: The malignant tumours of the kidney observed before the one year age constituted 18% of the tumours of the kidney in the child. A female prevalence was noted with a sex-ratio of 0.6. The assessment of extension found cutaneous (one case), ganglionic (one case) and pulmonary (two cases) metastases. First chemotherapy was prescribed to five patients. The surgery consisted on a widened nephrectomy in seven cases and a tumorectomy for an infant presenting a nephroblastomatosis. The tumour corresponded to a nephroblastoma in seven cases and a rhabdoide tumour in the last case. Mortality was high (50%) caused by the toxicity of chemotherapy in three cases and an advanced stage of cancer in one case. CONCLUSION: Renal tumoral pathology occurring in infants less than one year of age poses true etiologic and therapeutic problems. The high frequency of the nephroblastoma and the absence of benign tumour in our series encourage us to evocate more often the malignant renal tumours and to practice per cutaneous biopsies in case of diagnostic doubt.
Africa, Northern↗
Balloon dilatation of anastomotic strictures secondary to surgical repair of oesophageal atresia.
The purpose of this study was to evaluate the efficiency and safety of oesophageal balloon dilatation in strictures secondary to surgical treatment of oesophageal atresia in 25 children. Patients comprised 15 males and 10 females, aged 1-36 months. Median age was 4 months (interquartile range (IQR)=19). The strictures were more than 50% of oesophageal lumen and the delay from surgical treatment to balloon dilatation varied from 1 month to 36 months. Associated gastroesophageal reflux was noted in 15 patients. All procedures were performed under sedation using fluoroscopic guidance. Balloons of increasing diameter, 4-20 mm were used. Water soluble contrast swallow was performed after each dilatation session. A total of 115 balloon dilatation sessions were performed with a range of 1-14 procedures per patient (median 4 dilatations, IQR=4.5). Dilatation relieved the stricture in all patients over a follow-up period varying from 4 months to 33 months. The best results were noted in children under 6 months, who needed two or few dilatation sessions, with relative risk (RR) of 0.52 and 95% confidence interval of 0.29-0.92. The presence of associated gastroesophageal reflux indicated a high risk (RR of 12, p<0.001) for undergoing more than two balloon dilatation sessions. The only serious complications observed were two cases of oesophageal perforation, which were treated conservatively. Fluoroscopically guided balloon dilatation is a safe and effective treatment in the management of strictures secondary to surgical repair of oesophageal atresia, especially when started early (within 6 months of surgery) and not associated with gastroesophageal reflux.
Catheterization↗
[Fetal rhabomyomatous nephroblastoma. Report of 2 cases and review of the literature].
OBJECTIVE: Fetal rhabdomyomatous nephroblastoma is a particular and very rare histologic variety of nephroblastoma. The aim of this work is to study the principal clinic, therapeutic and evolutive characteristics of the fetal rhabdomyomatous nephroblastoma through two personal cases and a review of the literature. PATIENTS AND METHODS: This is a retrospective study of two observations of fetal rhabdomyomatous nephroblastoma treated in the pediatric surgery departement of Monastir (Tunisia) among 47 cases of nephroblastoma. The diagnosis was confirmed in the two cases by the histologic examination. RESULTS: The two patients were a six and a sixteen months old boy and girl. They were admitted for a voluminous mass occupying the left half-abdomen. The radiologic and biologic explorations load, in the two cases, to the diagnosis of left nephroblastoma. After a first chemotherapy that did not induce a reduction of the tumoral volume, a widened left nephrectomy was performed for the two patients. The histologic examination of the two pieces of nephrectomy concluded to a fetal rhabdomyomatous nephroblastoma with existence in the second case of an extension of the lesions to the renal pelvis and ureter in the form of a pseudo-botryoïde tumor. The tumor was classified stage I in the first case and stage II N0 in the second. The treatment was completed by an adapted post operative chemotherapy according to the SIOP 9 protocol. The two patients are currently in complete remission with an overview of six years and half. CONCLUSION: The fetal rhabdomyomatous nephroblastoma is a special histologic form of nephroblastoma that is characterized by the paucity of pulmonary metastasis, the absence of response to chemotherapy and the possibility of tumoral extension in the renal pelvis and ureter. His prognosis is similar to the classical nephroblastoma.
Female↗
Truncated forms of the dual function human ASCT2 neutral amino acid transporter/retroviral receptor are translationally initiated at multiple alternative CUG and GUG codons.
The sodium-dependent neutral amino acid transporter type 2 (ASCT2) was recently identified as a cell surface receptor for endogenously inherited retroviruses of cats, baboons, and humans as well as for horizontally transmitted type-D simian retroviruses. By functional cloning, we obtained 10 full-length 2.9-kilobase pair (kbp) cDNAs and two smaller identical 2.1-kbp cDNAs that conferred susceptibility to these viruses. Compared with the 2.9-kbp cDNA, the 2.1-kbp cDNA contains exonic deletions in its 3' noncoding region and a 627-bp 5' truncation that eliminates sequences encoding the amino-terminal portion of the full-length ASCT2 protein. Although expression of the truncated mRNA caused enhanced amino acid transport and viral receptor activities, the AUG codon nearest to its 5' end is flanked by nucleotides that are incompatible with translational initiation and the next in-frame AUG codon is far downstream toward the end of the protein coding sequence. Interestingly, the 5' region of the truncated ASCT2 mRNA contains a closely linked series of CUG(Leu) and GUG(Val) codons in optimal consensus contexts for translational initiation. By deletion and site-directed mutagenesis, cell-free translation, and analyses of epitope-tagged ASCT2 proteins synthesized intracellularly, we determined that the truncated mRNA encodes multiple ASCT2 isoforms with distinct amino termini that are translationally initiated by a leaky scanning mechanism at these CUG and GUG codons. Although the full-length ASCT2 mRNA contains a 5'-situated AUG initiation codon, a significant degree of leaky scanning also occurred in its translation. ASCT2 isoforms with relatively short truncations were active in both amino acid transport and viral reception, whereas an isoform with a 79-amino acid truncation that lacked the first transmembrane sequence was active only in viral reception. We conclude that ASCT2 isoforms with truncated amino termini are synthesized in mammalian cells by a leaky scanning mechanism that employs multiple alternative CUG and GUG initiation codons.
Amino Acid Sequence↗
[Esophageal duplication in children. Report of 7 cases].
UNLABELLED: Esophageal duplications represent 10 to 20% of esophageal malformations and 15 to 20% of digestive duplications. METHODS: The authors report a series of seven cases observed between 1985 and 1999. RESULTS: The sex ratio was 2.5. The diagnosis was made before the age of six months in five cases. The clinical presentation included respiratory signs in six cases and digestive signs in five cases. The diagnosis was made on the association of a mediastinal mass behind vertebral malformation in five cases. The duplication was cystic in five cases and tubular in two. A perforation in the pleura occurred in one case of tubular duplication. The intervention consisted of total excision of the duplication in four cases and subtotal resection in two cases. For the seventh patient, who presented a perforation of the duplication in the pleura, the operation comprised a thoracic drainage associated to feeding gastrostomy. The histologic examination of the six operative specimens confirmed the diagnosis of esophageal duplication. Six patients survived and have favorable evolution with an average follow-up of four years. DISCUSSION: Esophageal duplications represent 15 to 20% of digestive duplications. Their clinical presentations are variable. Their diagnosis is often made before the age of two years, when a posterior mediastinal mass is associated with vertebral malformations. Apart from the tubular form, which is rare, diagnosis is confirmed by the histologic study of the operative specimen. CONCLUSION: Esophageal duplications are rare and often pose a problem of positive diagnosis. Their treatment is surgical and should be undertaken before the appearance of complications.
Child, Preschool↗
[Pneumatic dilatation of caustic esophageal stenosis in children. Report of five cases].
The ingestion of caustic substances is a frequent accident in the child. The treatment of caustic strictures of the esophagus, which constitute the most frightening complication, varies from conservative treatment to esophageal replacement. We report the preliminary results of a prospective work established since July 1998 and aimed at evaluating the role of pneumatic dilatation in the treatment of these injuries. STUDIED POPULATION: The series included five boys with an average age of six years. Two children were neurologically handicapped. RESULTS: The caustic product was soda (three cases), a dishwasher solution (one case) and bleach (one case). Dysphagia was constant and concerned the semi-fluids in four cases. The stricture was extended over an average length of 4 cm. The total number of dilatations was 36, with an average of seven sessions/patient. No complication was noted. The result was good in four cases. The only failure was noted in a child who presented a gastroesophageal reflux secondary to the scarring phenomena. CONCLUSION: Pneumatic dilatation constitute a therapeutic mean whose results are attested. The best treatment remains, however, preventive: it is the setting out of reach of the child the dangerous products.
Burns, Chemical↗
Intrahepatic spontaneous portosystemic venous shunt: value of color and power Doppler sonography.
Spontaneous portosystemic venous shunts (SPVSs) within the hepatic parenchyma are rare. Fewer than 50 cases have been reported, and most of them were diagnosed by angiography. We present a case of SPVS diagnosed by color Doppler sonography in a 5-year-old boy admitted for bleeding due to rupture of esophageal varices. Conventional color and power Doppler sonography as well as CT showed a large shunt between the posterior branch of the right portal vein and the inferior vena cava. We believe that accurate diagnosis and follow-up of SPVS can be done with color Doppler sonography without resorting to angiography.
Child, Preschool↗