Biomedical subjects

T Willems

Publications and source records attributed to T Willems.

25 records · Page 2Linked to original sources

PubMed1997

Lethal femoral-facial syndrome: a case with unusual manifestations.

The femoral-facial syndrome is a very rare syndrome of uncertain inheritance comprising hypoplastic femora, microretrognathia, and a peculiar facies. We report an additional observation detected by ultrasound at 25 weeks and diagnosed at birth. In addition to the malformations usually described in this syndrome, there were heterotopias of the brain, partial agenesis of the corpus callosum, bilobed lungs, intestinal malrotation, and vertebral segmentation defects.

Abnormalities, Multiple↗

PubMed1995

Outcome after right middle lobe syndrome.

The long-term pulmonary consequences of right middle lobe syndrome (RMLS) in childhood are not known. Therefore, outcome was evaluated in 17 children with RMLS diagnosed in early childhood (mean age, 3.3 years; SD, 1.1 year). Mean age at follow-up was 10.1 years (SD, 2.6 years). RMLS was defined as atelectasis of the right middle lobe (RML) of at least 1 month's duration and visible on the lateral view of the chest radiograph as a wedge-shaped density extending from the hilum anteriorly and downward. Seventeen children without personal history of allergy or respiratory tract disease were studied as control group. Five of 17 study group children had ongoing respiratory problems: symptoms of asthma were present in 4 patients, and cylindrical bronchiectasis was present in one patient. Chest radiograph at follow-up was abnormal in six patients. Pulmonary function tests, including mean and SEM for vital capacity (VC) (82% of predicted +/- 7 vs 94% predicted +/- 3), FEV1 (77% of predicted +/- 12 vs 96% of predicted +/- 4) and their ratio (75 +/- 5 vs 85 +/- 3) were significantly lower in patients with ongoing respiratory symptoms than in the control children. The provocative dose causing a 20% decrease in FEV1 (PD20) of methacholine was significantly lower in patients with ongoing symptoms at follow-up than in control children and in patients without symptoms at follow-up (2.8[2.2 to 3.1] vs 4.5[2.2 to 8.8] and 9.2[2.3 to 24] mg/mL; median and P25-75, p < 0.05). Age at initial diagnosis tended to be younger in patients with ongoing symptoms at follow-up (2.3 +/- 0.7 years vs 3.8 +/- 0.4 years; p < 0.08).

Bronchial Provocation Tests↗

PubMed1989

Nucleolar organizer regions in the normal and carcinomatous epithelium of the uterine cervix. A morphometric study.

Nonhistone nucleoproteins associated with the nucleolar organizer regions (NORs), the genes coding for the ribosomal RNA precursor, can be visualized by silver staining. The black dots (AgNORs) appearing on the nuclei are thought to reflect cell differentiation. In this study, AgNORs were counted and their area was measured and compared with the area of the nuclei in normal and carcinomatous cells of the uterine cervix. The number of AgNORs per nucleus was significantly higher in the endocervical than in the basal exocervical epithelium (p less than 0.005) and in the carcinomatous epithelium, either in situ or invasive, than in both normal epithelia (p less than 0.002). Individual AgNORs were significantly smaller in carcinoma in situ than in endocervical epithelium (p less than 0.05) or in invasive carcinoma (p less than 0.01). Significant differences were also found in the total AgNORs area per nucleus between the following groups: basal exocervical versus endocervical epithelium (p less than 0.01), basal exocervical and endocervical epithelium versus invasive carcinoma (p less than 0.001), and in-situ versus invasive carcinoma (p = 0.02). The conclusions are that the number and the total area of AgNORs per nucleus increase with the differentiation of the cell or with its carcinomatous transformation, but no prognostic significance can be drawn so far from our measurements.

Biopsy↗

PubMed1987

Effect on ovulation of surgically induced endometriosis in rabbits.

To study the effect of endometriosis on follicular rupture, endometrial tissue was autografted to New Zealand White rabbits. Endometrium was surgically implanted into the peritoneal cavity or into the rectus muscle. Human chorionic gonadotropin was administered to induce ovulation. During three subsequent laparotomies, the number of corpora lutea and stigmata were counted. The viability of the implants was demonstrated histologically. Ovaries were removed during the last laparotomy and ovarian serial sections were examined. In rabbits with peritoneal induced endometriosis, the percentage of stigmata/corpora lutea was significantly decreased. Macroscopic study was confirmed by histological examination. Indeed, a high incidence of entrapped oocytes was found in rabbits with peritoneal endometriosis. Extraperitoneal endometriosis had no effect on ovulation. Our data demonstrated that endometriosis induced a failure of follicular rupture. After endometriumectomy, no failure to ovulate was observed, suggesting that the effect of endometriosis on the ovulation disappeared with excision of endometrial implants.

Animals↗

PubMed1987

[Unruptured luteinized follicle syndrome and experimental endometriosis in the female rabbit].

To study the effect of endometriosis on follicular rupture, endometrial tissue was autografted to New Zealand White rabbits. Endometrium was surgically implanted into the peritoneal cavity. Human chorionic gonadotropin was administered to induce ovulation. The viability of the implants was demonstrated histologically during three subsequent laparotomies, the number of corpora lutea and stigmata were counted. Ovaries were then removed and ovarian serial sections were examined. In rabbits with peritoneal induced endometriosis, the percentage of stigmata per corpora lutea was significantly decreased. Macroscopic study was confirmed by histological examination. Indeed, a high incidence of entrapped oocytes was found in rabbits with peritoneal endometriosis. Our data demonstrated that endometriosis induced a failure of follicular rupture.

Animals↗

PubMedDate not supplied

Rhinitis/Bronchopneumonia syndrome in Irish Wolfhounds.

This study describes the clinical, immunologic, genetic, and pathologic features of Irish Wolfhounds with rhinitis/bronchopneumonia syndrome. The dogs examined were from Belgium, The Netherlands, UK, Canada, Germany, and Switzerland. Signs included transient to persistent mucoid or mucopurulent rhinorrhea, cough, and respiratory dyspnea. Radiographic, rhinoscopic, and bronchoscopic findings were variable. Analysis of ciliary ultrastructure was performed in 5 affected dogs, but no characteristic primary ciliary defects (primary ciliary dyskinesia) were detected. Serum and bronchoalveolar lavage fluid (BALF) concentrations of IgA, IgG, and IgM were determined in some affected dogs and clinically normal Irish Wolfhounds. Serum IgA concentration was below the reference range in 5 of 8 affected dogs tested, whereas BALF IgA concentration was above the normal range in 2 affected adult dogs. The CD4 to CD8 lymphocyte subset ratio (CD4:CD8) in peripheral blood was tested in 3 affected dogs and was within the normal range. BALF CD4:CD8 was tested in 1 affected dog and was higher than the normal range. Decreased neutrophil phagocytosis was observed in 1 of the 4 dogs tested. Analysis of pedigrees of the Belgian, Canadian, German, and Swiss dogs revealed common ancestry, suggesting a heritable syndrome.

Animals↗

PubMedDate not supplied

Diagnostic evaluation of mucociliary transport: from symptoms to coordinated ciliary activity after ciliogenesis in culture.

Mucociliary transport is one of the most important local defense mechanisms of the airways, but it is prone to many and frequent acquired abnormalities and to inherited abnormalities. These abnormalities result in basic physiologic disturbances leading to a number of respiratory symptoms and signs. In order to critically evaluate the diagnostic value of parameters of this mucociliary cascade, the results of ciliary investigations in over 500 individuals (controls, acquired, and inherited abnormalities) were reviewed. Ciliary beat frequency, ciliary coordination and ultrastructural abnormalities were measured and evaluated in biopsies and after ciliogenesis in culture. There is a considerable overlap for all investigated parameters in biopsy material of controls, secondary, and primary ciliary dyskinesia. There is not one parameter that is diagnostic for primary ciliary dyskinesia or can be used as an exclusion criterion. After ciliogenesis in culture, cilia are always completely normal except for the inherited abnormalities. Absence of coordinated ciliary activity after ciliogenesis in culture is 100% sensitive and specific for the diagnosis of primary ciliary dyskinesia.

Cilia↗