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Biomedical subjects

M Demarche

Publications and source records attributed to M Demarche.

14 recordsLinked to original sources

[Clinical evaluation of 23 children with neuroblastoma. The experience of a single institution].

In this retrospective study, we analyse epidemiology, clinical symptoms and therapeutic results in a group of 23 children with neuroblastoma. Half of them were less than 2 years of age; in 19 of 23, the primitive tumour was abdominal; 35% of them were initially metastatic. The overall survival was 83% at 5 years and the event free survival, 75% at 5 years. Pronostic factors are age, extension of the disease at diagnosis, biologic parameters and genetic study of the neuroblast cells (amplification of N-myc oncogen). Our study shows the deleterious effect of bone lesions.

Abdominal Neoplasms↗

[Retrospective study of childhood lymphomas. Report of 27 children treated at a single institution].

Childhood lymphomas represent a heterogeneous group of disorders that are quite different from adult lymphomas. Over the past three decades, empirical chemotherapeutic management has transformed survival figures, and more recently greater understanding of the biology is offering hope for improved management of resistant disease. We present here the experience of a single institution in the management of 27 childhood lymphomas; epidemiological and clinical characteristics are described as well as survival rates. The median follow up of the patients is 4 years 7 months. The five-year overall survival for the entire group is more than 95 %; the 5-year disease free survival is 91,6 % for Hodgkin's lymphomas, 92,8% for non Hodgkin's lymphomas and 100% for Burkitt diseases. Two relapses have occurred and all of them appeared within the 18 months of the diagnosis. No toxic death has been reported.

Adolescent↗

[Paroxystic abdominal pain in children].

Acute abdominal pain in children is a frequent symptom of a wide spectrum of abdominal and extra-abdominal pathologies. Some of them are more common in specific age groups. Prompt diagnosis and treatment are facilitated by a good knowledge of the etiologies. Although surgery is mandatory in most of the cases of perforation, torsion, ischemia or necrosis, a medical treatment will prevent an unnecessary laparotomy in some circumtances.

Abdominal Pain↗

Bony syngnathia, vertebral segmentation defect, coloboma, microcephaly and mental retardation: confirmation of Dobrow syndrome and review of syndromal syngnathias.

Congenital bony fusion of the maxilla and mandible is a rare condition. Two classifications were previously proposed dealing exclusively with craniofacial malformations. Most of the reported cases to date represent either aglossia-adactylia or hemifacial microsomia syndromes. We report a young girl with bony syngnathia associated with multiple defects (severe microcephaly, coloboma, vertebral segmentation defects), growth and mental delay. This patient is very similar to the patient described by Dobrow in 1983 and confirms the existence of this extremely rare disorder.

Coloboma↗

Sacrococcygeal teratoma: results of a retrospective multicentric study in Belgium and Luxembourg.

Eighteen patients, operated upon for sacrococcygeal teratoma in 7 different centres in Belgium and Luxembourg between 1992 and 1996, were reviewed. From an epidemiological point of view, this series compares very well to others. Although excellent results were obtained, with all patients surviving, some imperfection in diagnosis, timing of delivery and of operation, and in operative technique was observed. Therefore, it is stated that for optimal treatment of sacrococcygeal teratoma to be achieved, these cases should be treated in just a very few centres of neonatal surgery.

Adult↗

Discrepant flow cytometric expression and function of P-glycoprotein in neuroblastic tumors.

BACKGROUND: Patients suffering from neuroblastic tumors are currently being classified into prognostic subsets based on different clinical and biologic features. In this study, a triple-color flow cytometric assay and a functional test were applied to neuroblastoma cell lines and patients with a neuroblastic tumor, and the value of P-glycoprotein expression and function as potential prognostic characteristics, was determined. METHODS: Twenty-two single-cell suspensions prepared from tumors, and neuroblasts from four bone marrow samples were analyzed by triple-color flow cytometry. Neuroblasts were identified by NB84-positivity and absence of CD45. P-glycoprotein expression was evaluated using 4E3 and MRK16 antibodies. Eighteen samples were tested with a functional assay, based on accumulation and retention of rhodamine-123 with and without the inhibitor verapamil. Six neuroblastoma cell lines were also evaluated. RESULTS: P-glycoprotein expression was seen in 18 of 26 patient samples and in three of six cell lines. The highest expression levels were found in low stage neuroblastoma and well-differentiated tumors; whereas the highest activities were found in stage 4 neuroblastoma and the lowest in ganglioneuroblastoma and ganglioneuroma patients. In 10 of 17 samples, concordant results were found between the flow cytometric immunological test and immunocytochemistry. CONCLUSIONS: The described flow cytometric technique is a new, alternative approach to detect P-glycoprotein expression and function in neural crest tumors. Based on the expression level and the activity value, patients can be segregated into different phenotypic groups. In particular, those patients with high P-glycoprotein activity might benefit from treatment regimens containing reversal agents.

ATP Binding Cassette Transporter, Subfamily B, Mem↗

Femoral anastomotic aneurysms: pathogenic factors, clinical presentations and treatment. A study of 142 cases.

In this study, the files of 112 patients with a total of 142 femoral anastomotic aneurysms were reviewed. Eighty-five patients (76%) were initially operated upon for obstructive aorto-iliac disease, while the remaining 27 (24%) had abdominal aortic aneurysms repaired. The majority of the patients (104/112) were male and their mean age was 64.5 years (range 45-88). Ninety-three per cent of the subjects were smokers prior to the first operation and 43% continued to smoke at the time of their femoral anastomotic aneurysms operation. The mean delay between the initial surgery and the repair of the femoral anastomotic aneurysms was 74.5 months (range 1-228). The diagnosis was made because of a painless pulsatile mass (91/142), acute leg ischaemia (27/142), a painful pulsatile mass (12/142), haemorrhage (10/142), pseudo-post-phlebitic oedema (1/142) and microemboli of the toes (1/142). The operative mortality was 2.7% (3/112) of which two-thirds were patients with infected grafts. Two subgroups were distinguished: 10 patients with an infected femoral anastomotic aneurysm and 12 patients with recurrent femoral anastomotic aneurysms, 11 with a single recurrence and one with a double recurrence. In the infected group, the time to development of anastomotic aneurysm was shorter than for the group with non-infected femoral anastomotic aneurysms (41 versus 74.5 months) and the operative mortality was 20% (2/10). One patient developed a recurrent femoral anastomotic aneurysm and another was lost to follow-up. Two subsequent deaths occurred, which were unrelated to the femoral anastomotic aneurysms. In the group of recurrent femoral anastomotic aneurysms one patient was lost to follow-up and two patients died, but not as a result of recurrent femoral anastomotic aneurysms. A total of 122 cases underwent interposition of a new prosthetic segment between the proximal prosthesis and the distal artery (89 at the common femoral, 21 at the femoral profundis, eight at the superficial femoral and four at an existing femoro-popliteal graft).

Aged↗

[Importance of long-term follow-up of infants operated of sacro-coccygeal teratoma].

Sacro-coccygeal teratoma is the tumor which is most frequently diagnosed in infants. In spite of the sometimes spectacular appearance of the tumor, the surgical treatment generally permits a definitive cure of the disease without any significant functional or esthetic sequellae. The risk of malignant recurrence, even after removal of an apparently benign mass is not totally excluded and may imply to resort to combined treatment by surgery and chemotherapy. The baby must be carefully followed-up and the serum levels of alpha-foetoprotein (a specific tumor marker), and sometimes beta-HCG must be regularly measured. Local surveillance is mandatory to prevent any loss of time in case of recurrence.

Bone Neoplasms↗

Laparoscopic Nissen fundoplication: detailed analysis of 132 patients.

With the world-wide use of laparoscopy, new applications have been found, particularly in the field of esophageal and gastric surgery. From January 1991 through April 1992, 132 patients underwent laparoscopic Nissen fundoplication. The indication for laparoscopic intervention was symptomatic gastroesophageal reflux disease (GERD) not responding to medical treatment. The preoperative assessment included esophagogastroscopy, barium meal, esophageal manometry, and, in selected cases, 24 h pH studies. There was no operative-related mortality. The overall morbidity was 7.5%. Ninety-eight patients were seen 3 months after surgery and evaluated for control of reflux symptoms. One patient complained of mild recurrence of reflux symptoms; however, endoscopic examination revealed no evidence of recurrent esophagitis. Three complained of occasional dysphagia and two, of mild dysphagia. One individual required reoperation for persistent, severe dysphagia. The excellent results observed so far with this procedure have led us to believe that laparoscopic Nissen fundoplication is the procedure of choice for patients with intractable GERD.

Adolescent↗

[Long-term results in ileo-anal reservoirs in the treatment of ulcero-hemorrhagic rectocolitis].

The authors report long term results of 53 ileo-anal anastomosis for ulcerative colitis. They used a "J" pouch technique with anastomosis to the anal canal after mucosectomy of the lower rectum. An upstream ileostomy should be created and left in place for two to three months. Early morbidity primarily involves pelvic or parietal infectious problems, while late complications comprise pelvic fistulae, obstructive events, and episodes of pouch inflammation. Functional results improve over the first year and remain stable thereafter. The major long term problem remains that of pouch inflammation its treatment, and the understanding of its pathophysiology.

Activities of Daily Living↗