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Biomedical subjects

L Boccon-Gibod

Publications and source records attributed to L Boccon-Gibod.

At least 181 records · Page 10Linked to original sources
PubMed1992

[A rare cause of neonatal exudative enteropathy: congenital Langerhans cell histiocytosis (histiocytosis X)].

A case of Langerhans cell histiocytosis is reported in a neonate. Intestinal involvement was especially diffuse and severe, presenting as a protein-losing enteropathy secondary to massive mucosal infiltration by histiocytic cells. The infant died at the age of 3 1/2 months despite therapy with corticosteroids and vinblastine then etoposide and interferon. Such an outcome confirmed the severity of forms with neonatal onset and digestive involvement.

Adrenal Cortex Hormones↗
PubMed1992

Low levels of human immunodeficiency virus replication in the brain tissue of children with severe acquired immunodeficiency syndrome encephalopathy.

The authors examined the autopsy brain samples of nine children infected with human immunodeficiency virus (HIV) at birth by histology, immunologic staining, and in situ hybridization. Surprisingly, although seven of these children presented with typical AIDS encephalopathy, the authors could detect a multifocal HIV infection in the brains of only three of these patients. The authors could not detect any significant HIV replication in the brain of four other children despite severe neurologic disease. However, HIV DNA was detected by polymerase chain reaction (PCR) in the central nervous system (CNS) of all patients. In addition, the authors found associated lesions in the brains of three of these four patients. This study shows that severe AIDS encephalopathy exists in children and therefore might exist in adults with few signs or without any signs of HIV replication or inflammation in the CNS. Understanding the pathogenesis of this neurologic disease and the kinetics of HIV replication in brain tissue of children with AIDS encephalopathy is essential to determine the best therapeutic strategy.

Acquired Immunodeficiency Syndrome↗
PubMed1991

Combined analysis of DNA ploidy index and N-myc genomic content in neuroblastoma.

The aim of the study was to assess, in a group of nonselected patients with neuroblastoma, the prognostic value of both N-myc gene amplification and DNA ploidy index, taking into account potential confounding factors such as age and stage. Of 59 patients studied, 23 were younger than 1 year at diagnosis, 31 presented with stage IV, 10 with stage III, 5 with stage II, 8 with stage I, and 4 with stage IV-S. N-myc genomic content was analyzed by Southern blot hybridization technique and N-myc amplification (greater than or equal to 3 copies/haploid genome) was present in 6 stage IV, 2 stage III, and 1 stage IV-S. The DNA ploidy index was analyzed by flow cytometry. Of the 59 neuroblastomas, 26 were diploid (DNA index, 1) and 33 were aneuploid (DNA index, greater than 1). The majority of the aneuploid tumors (28 of 33) were near-triploid with DNA indexes between 1.25 and 1.68, 4 were near-diploid (DNA index up to 1.18), and 1 was hypotetraploid (DNA index, 1.85). The proportion of near-triploid tumors was significantly greater among patients under 1 year of age and among patients presenting with stages I, II, and IV-S. Interestingly, 0 of 28 near-triploid neuroblastomas exhibited N-myc gene amplification, compared to 9 of 31 in the group of diploid, near-diploid, and hypotetraploid tumors (Fisher's exact test, P less than 0.001). Four factors were significantly related to a high risk of relapse in univariate analysis, i.e., age, stage, DNA index, and N-myc amplification. In multivariate analysis, only N-myc amplification and the DNA index remained significantly associated with a high risk of relapse. The 2-year disease-free survival rate was 94% (95% confidence interval, 77-98%) for patients with near-triploid neuroblastoma, compared to 45 and 11% (95% confidence interval, 32-70 and 4-23%) for patients with diploid or near-diploid tumors, without and with N-myc amplification, respectively. We concluded that the combination of N-myc and DNA index should be included in routine management of neuroblastoma.

Age Factors↗
PubMed1991

Prostate cancer in a 75-year-old man after four decades of testicular androgen deprivation.

In a 75-year-old patient referred for mild obstructive symptoms, physical examination revealed a clinically manifest T2B cancer of the prostate (CaP) involving the right lobe, and bilateral testicular atrophy (TA), right cryptorchidism with TA after orchiopexy at age 10, and left mumps orchitis with secondary atrophy at age 35). Since the age of 35, the patient had a limited sexual life and fathered no children. Transrectal ultrasonography demonstrated a small prostate (21 cm3) with a large hypoechoic zone in the right lobe, an isoechoic left lobe and no sign of capsular/seminal involvement. Three biopsies of the right lobe demonstrated a cancer (Gleason 4-3; score 7) of the right lobe, 3 biopsies of the left lobe showed an atrophic prostate with slight intra-acinar hyperplasia and moderate dysplasia. Morphometric studies showed these findings to be consistent with long-standing androgen deprivation. Serum prostate-specific antigen (PSA) was 8.7 ng/ml, whereas serum testicular androgens were within the castrate value (serum testosterone 0.2 ng/ml, dihydrotestosterone less than 0.05 ng/ml). Serum LH and adrenal androgens were within normal ranges. Bone scan was negative and NMR demonstrated no nodal enlargement. Considering the age of the patient, antiandrogen therapy was given (cyproterone acetate 50 mg t.i.d.). The patient reported marked improvement at 3 and 6 months of follow-up and PSA fell to 1 ng/ml at month 6. However, repeat rectal exams and ultrasonography showed no dramatic modification of the gland. The patient has since been lost to follow-up. Two conclusions may be drawn from this observation. Testicular androgen deprivation at age 35 although leading to prostatic atrophy does not prevent the development of CaP.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1991

Anatomy of the prostate: review of the different models.

Current understanding of the anatomic subdivision of the adult prostate gland is secondary to two major studies successively published by Gil Vernet in 1953 and by McNeal in 1968. In Gil Vernet's report, glandular tissue consists of a caudal gland, a cranial gland and a intermediate gland. These 3 glandular areas were defined according to the location of their glandular openings into the urethra. In McNeal's report, the urethra is taken as the primary anatomic reference point. The four glandular areas are best visualized by examination of the selected coronal plane of sections along the proximal and distal prostatic urethra. The peripheral zone is located posterolaterally, the central zone is located at the base, and the 2 lobes of the transition zone are located along the proximal urethra. Fibromuscular regions are concentrated anteromedially. Most of the anatomic regions are similar between the two models, however, in McNeal's model, accuracies concerning the boundaries of the central zone and transition zone ultimately make this model superior for the study of both the physiology and pathology of the prostate.

Humans↗
PubMed1991

Benign prostatic hyperplasia: surgical versus nonsurgical treatment. A critical review.

With the advent of new treatments for benign prostatic hyperplasia (BPH), the urologist must objectively assess the results of new and traditional therapeutic options. This requires three sets of data-not all of which may be readily available-including a precise knowledge of the natural history of BPH; an objective assessment of the results of surgery, both open and transurethral; and an accurate estimate of objective and subjective results of medical treatment based on randomized studies with a placebo arm. The two types of medical treatment available are hormonal and neuropharmacologic manipulation. Hormonal treatment, using agents such as luteinizing hormone-releasing hormone agonists or antiandrogens, reduces the volume of the epithelial component of the prostate, but urodynamic improvement does not always parallel volumetric reduction. Neuropharmacologic treatment using alpha-blockers alone or in combination with anticholinergic agents has no effect on prostatic volume, but reduces the tone of the muscular component.

Androgen Antagonists↗
PubMed1991

[The correlation between the level of prostate-specific antigen and prostate gland volume in the early diagnosis of prostatic cancer].

One hundred and eighty six patients (Pts) presenting with either an abnormal per rectum examination (P.R. suggestive of prostatic cancer (P.Ca) (excluding stage T3) or a prostatic specific antigen (P.S.A.) level greater than or equal to 2.5 ng/ml (radioimmunoassay) were submitted to transrectal ultrasonography (T.R.U.) using a 7 MHz transducer. The prostatic volume (Vol.P) was systematically calculated during T.R.U. and correlated with the P.S.A. level. Six systematic multiple ultrasound-guided biopsies (S.M.U.B.) were performed in the 2 prostatic lobes (3 per lobe) regardless of the result of T.R.U. In the 111 patients in whom the S.M.U.B. were negative, a highly significant correlation (p < 0.001) was observed between the serum P.S.A. level (y) and the prostatic volume (Vol.P): y = (4.13 +/- 0.15 Vol.P) +/- 8.43. In the 75 Pts with P.Ca, the P.S.A./Vol.P correlation was significantly different (p < 0.05) from the straight line of regression with the previous standard deviation. A similar difference in correlation (p < 0.05) was observed in the sub-group of 14 Pts with P.Ca despite normal P.R. and T.R.U. These results suggest: 1) that there is a correlation between the Vol.P measured by T.R.U. and the P.S.A. level in patients with negative S.M.U.B. 2) that the absence of correlation is highly suspicious of P.Ca and requires S.M.U.B. 3) that there is a 15% incidence of P.Ca in the sub-group of Pts with normal P.R. and T.R.U. which are only detected by the raised P.S.A. not correlated with the volume of the prostate.

Biopsy↗
PubMed1991

[Mediastino-pulmonary sarcoidosis in children. Clinical study, analysis of data of bronchoalveolar fluid lavage and respiratory function tests, therapeutic trends].

A series of 27 children (mean age: 12 yrs, 5 mos.) presenting with thoracic sarcoidosis is reported. This series, collected from 1961 to 1988 shows the rarity of the disease at that age. However the low rate of asymptomatic forms (22%) suggests that the frequency of the disease is underestimated, as it is not diagnosed. The histological proof is necessary for the diagnosis. When peripheral lesions available for biopsy are lacking, a liver needle biopsy is helpful (93% of positivity). This study shows the frequency of multivisceral types, the intensity of the macrophagic and lymphocytic alveolitis. The therapeutic indications depend on the comparison of the radiological stage, the results of pulmonary function tests (PFT), those of the bronchoalveolar lavages (BAL) and of the serum granulomatous activity markers, especially concerning angiotensin converting enzyme (ACE). When present at the beginning of evolution, several risk factors lead to use a corticosteroid treatment: age of onset before 4 years, multivisceral involvement, presence of functional pulmonary signs, delayed diagnosis and onset of treatment, impaired respiratory function (especially concerning the alveolo-capillary diffusion), PMN cells greater than or equal to 2% in the initial BAL, and IgG proteins greater than 4 SD. Thus sarcoidosis in children differs from that seen in adults as it has a more marked evolutive tendency and leaves severe sequelae in one third of patients.

Adolescent↗
PubMed1991

[Value of voiding ultrasonography combined with urodynamic studies in the assessment of vesical sphincter dyssynergy].

Fifteen paraplegic patients all presenting with vesico-sphincteric dyssynergia underwent, between January and September 1990, a urodynamic and electromyographic examination combined with pre-voiding or voiding transrectal ultrasonography. The ultrasound apparatus used was a Siemens Sonoline SL1 with a MHz linear intracavitary probe giving a strictly longitudinal plane of section. The urodynamic apparatus used was a Wiest 6000 with a Böhler 7 F urethral catheter and an electromyography needle-electrode implanted in the striated sphincter. This type of ultrasonography provided a precise and dynamic image of the bladder neck, prostatic urethra and external striated sphincter during the phases of filling and voiding. Spastic contractions of the striated sphincter during detrusor contraction were observed in 8 patients with an intermittent and jerky urinary stream. In 7 patients, the striated sphincter remained closed during detrusor contraction and only opened briefly as soon as detrusor contraction decreased, allowing only a weak and transient flow. By allowing the direct visualisation of the sphincteric obstruction during voiding, dynamic transrectal ultrasonography clearly confirmed the diagnosis of vesico-sphincteric dyssynergia. In contrast with classical voiding cystourethrography, this is a non-invasive, inexpensive and, most importantly, repeatable technique, as it does not require any irradiation. It is therefore suitable for drug evaluation trials, particularly of alpha-blockers and to assess one of the many treatments proposed in vesico-sphincteric dyssynergia.

Adult↗
PubMed1991

["Clam" enteroplasty in the treatment of unstable or low compliance bladders].

The treatment of urinary incontinence due to unstable bladder remains difficult. In the case of failure of medical or physiotherapeutic treatments, clam enterocystoplasty represents a major and important therapeutic possibility. The operation consists of frontal section of the bladder to form a bivalve followed by interposition, between the two valves, of a detubed ileal graft in order to increase the functional vesical capacity and to interrupt the transmission of detrusor contraction waves from one valve to the other. 10 patients (7 men and 3 women) suffering from urinary incontinence due to neurogenic unstable bladder (4 cases: 3 meningoceles, 1 operated cauda equina neuroma) or to another cause (6 cases, including one sequela of radiotherapy for prostatic cancer and 5 cases of apparently primary urinary incontinence) underwent enterocystoplasty combined, in the case of neurogenic incontinence (2 cases simultaneously and 2 cases previously), with the insertion of an AMS 800 sphincter. No major complication was observed. 9 patients are continent and 1 had to be reoperated to undergo an augmentation enterocystoplasty (failure of the initial operation due to radiation changes of the bladder), 3 retain a post-voiding residual of about 200 ml but not requiring self catheterisation due to the absence of any repercussions on the upper urinary tract. Lastly, urodynamic studies demonstrated a spectacular increase in compliance and functional capacity of the bladder together with a reduction of the amplitude of intravesical pressure peaks.

Adolescent↗
PubMed1991

[Treatment of male urinary incontinence with the AMS 800 sphincter prosthesis: 45 cases].

Urinary incontinence due to sphincter incompetence constitutes a major source of disability. The AMS 800 artificial urinary sphincter allows durable restoration of satisfactory continence in patients who are often unresponsive to any other forms of treatment. 45 men underwent implantation of a peribulbar urethral (40) or pericervical (5) artificial sphincter over a 5-year period. Incontinence was secondary to prostatic surgery in the majority of cases (33). The technique, which is now well defined, consisted of urinary drainage via a bladder catheter for 8 days and activation of the sphincter after 1 month. The mean follow-up is 30 months. 36 (80%) of the patients are continent, including 27 (60%) without revision and 9 (20%) underwent unsuccessful revision resulting in partial or complete removal or permanent inactivation of the prosthesis (sepsis, urethral erosion, defective prosthesis). Strict patient selection, a rigorous implantation technique and standardised management of any incidents are key elements in the success of this procedure.

Adolescent↗
PubMed1991

[Multiple condylomata of the urethra and bladder disclosing HIV infection].

The authors report the case of a 26 year old man, in whom recurrent hematuria revealed the diagnosis of urethral condylomata with bladder extension, and HIV serology positive. This case reminds us that it is essential to search for immunodeficiency in every case of extensive or recurrent condylomata. The analysis of such cases in the literature allows discussion of the various clinical courses according to the Human Papilloma Virus types found in condylomatous tissues.

Adult↗