Salivary gland involvement in hypohidrotic ectodermal dysplasia

Ectodermal dysplasias (EDs) are a group of developmental disorders (more than 100) mainly affecting ectodermal tissues and organs. The X-linked hypohidrotic ED (HED) is the most common form of EDs, involving defects in teeth, sweat glands, and hair. In a few reports, HED has been associated with reduced salivary function. In the present case report, a dramatically reduced salivary fluid and acidic proline rich protein production was identified in a 38-year-old man with HED. Computed tomography was performed, revealing that one submandibular gland and both parotid glands were hypoplastic, whereas the right submandibular gland seemed to be absent. These findings are in line with a general developmental disturbance also involving the salivary glands. As salivary tests are inexpensive and easy to perform, it is suggested to routinely evaluate salivary secretion in persons with HED, to prevent a possible negative impact on oral health.     

Renal thorium deposition associated with transitional cell carcinoma: radiologic demonstration in two patients

Abdominal radiography, excretory urography, retrograde pyelography, and computed tomography were performed in two patients who had undergone retrograde pyelography with thorium dioxide (Thorotrast) approximately 40 years ago. Both patients developed a transitional cell carcinoma due to suburothelial thorium deposition. Typical thorium densities were demonstrated at CT in the peripelvicalyceal area as well as in retroperitoneal lymph nodes. Elderly patients in whom radiographic examination reveals retained Thorotrast in the kidney should be followed up because of the high risk of renal carcinoma.     

The Sequential Relation Between Changes in Catastrophizing and Changes in Posttraumatic Stress Disorder Symptom Severity

Catastrophizing has been discussed as a cognitive precursor to the emergence of posttraumatic stress disorder (PTSD) symptoms following the experience of stressful events. Implicit in cognitive models of PTSD is that treatment-related reductions in catastrophizing should yield reductions in PTSD symptoms. The tenability of this prediction has yet to be tested. The present study investigated the sequential relation between changes in a specific form of catastrophizing—symptom catastrophizing—and changes in PTSD symptom severity in a sample of 73 work-disabled individuals enrolled in a 10-week behavioral activation intervention. Measures of symptom catastrophizing and PTSD symptom severity were completed at pre-, mid-, and posttreatment assessment points. Cross-sectional analyses of pretreatment data revealed that symptom catastrophizing accounted for significant variance in PTSD symptom severity, β = .40, p < .001, sr = .28 (medium effect size), even when controlling for known correlates of symptom catastrophizing, such as pain and depression. Significant reductions in symptom catastrophizing and PTSD symptoms were observed during treatment, with large effect sizes, ds = 1.42 and 0.94, respectively, ps < .001. Cross-lagged analyses revealed that early change in symptom catastrophizing predicted later change in PTSD symptoms; early changes in PTSD symptom severity did not predict later change in symptom catastrophizing. These findings are consistent with the conceptual models that posit a causal relation between catastrophizing and PTSD symptom severity. The clinical implications of the findings are discussed.     

Heterogeneity of human whole blood platelet subpopulations. III. Density-dependent differences in subcellular constituents

Structurally intact platelet cohorts of differing densities can be isolated from normal subjects by the use of isosmolar arabinogalactan density gradients. Using platelets separated in this fashion, we have studied the density-dependent distribution of four subcellular organelles: mitochondria, lysosomes, dense bodies, and alpha granules. Mitochondria, which are not secreted during platelet release, demonstrate a slow decline in monoamine oxidase activity within the gradient. Lysosomal beta-glucuronidase does not vary significantly with platelet density. In contrast, dense body number and endogenous serotonin content decrease significantly with decreasing platelet density, primarily as the result of differences in the number of storage organelles. Platelet factor 4 content declines rapidly in comparison to lysosomal activities (P less than .001 from bottom to top of the gradient); but beta-thromboglobulin, also an alpha granule component, exhibits considerably less change than platelet factor 4 (P less than .001). Thus, specific platelet subcellular constituents have different density distributions. We postulate that these density differences may be due to differential in vivo loss of selective biochemical constituents from unique subcellular compartments.     

Is ASCT With TBI Superior to ASCT Without TBI in Mantle Cell Lymphoma Patients?

BACKGROUND: Impact of total-body irradiation (TBI) in conditioning regimen on outcome for patients with mantle cell lymphoma (MCL) remains unknown. METHODS: Patients with MCL who underwent autologous stem-cell transplantation (ASCT) in our institution were eligible for the present study (n=73). We analyzed the impact of various biologic and clinical parameters, with and without TBI, on patient outcome. RESULTS: All patients presented with chemosensitive disease at transplantation. Median follow-up from ASCT was 37.2 months. One- and three-year overall survival (OS) rates were 90.3% and 74.5%, progression-free survival (PFS) rates were 85% and 59%, respectively. Three-year OS and PFS rates in the non-TBI group versus TBI group were similar: 80% versus 72.5% and 60% versus 57%, respectively. In univariate analysis, the use of TBI did not modify OS or PFS (P=0.93 and P=0.48, respectively). This remains true for patients who underwent ASCT up front. According to multivariate analysis, OS tended to be shorter for patients presenting with high Mantle Cell Lymphoma International Prognostic Index or low hemoglobin level. CONCLUSIONS: Absence of TBI in conditioning regimen modifies neither PFS nor OS. The present retrospective and monocentric analysis shows that transplant patients with MCL remain highly exposed to relapse.     

The effect of PGI2 and theophylline on the response of platelets subjected to shear stress

A specially designed rotational viscometer was used to investigate the effects of the antiplatelet agent PGI2 in combination with theophylline on the response of human platelets subjected to shear stress. Samples of citrated platelet-rich plasma (PRP) were exposed to shear stress in the viscometer for a period of 5 min at 23 degrees C. The levels of stress studied ranged from 50 to 300 dynes/sq cm. Pretreatment of the platelets with 0.01 microM PGI2 and 500 microM theophylline before exposure to shear stress caused a large reduction in shear-induced platelet aggregation. However, it was also observed that the PGI2-- theophylline pretreatment concomitantly caused a large increase in shear-induced platelet lysis and serotonin release at stress levels equal to or greater than 150 dynes/sq cm. This observed increase in platelet fragility may have important implications for clinical applications of PGI2. The results are discussed and compared to those obtained in prior work in which platelets were pretreated with acetylsalicylic acid or with PGE1.     

Influence of HLA-DP mismatches on primary MLR responses in unrelated HLA-A, B, DR, DQ, Dw identical pairs in allogeneic bone marrow transplantation

The success of bone marrow transplantation relies on class I and class II HLA identity between donor and recipient but until now the impact of DP mismatches on primary mixed lymphocyte reaction (MLR I) responses has not yet been clearly established. HLA-DP typing has been performed by a RFLP method on 10 patients and their 22 selected potential donors (HLA-A, B, DR, DQ, Dw identical according to serological, RFLP and oligotyping methods). HLA-DP-matching was then correlated with MLR I responses obtained (1) in 22 HLA-A, B, DR, DQ, Dw identical donor-recipient pairs (patient series) and (2) in 30 HLA-A, B, DR, DQ, Dw identical donors pairs used as the control series. We showed that MLR I responses involving patients cells were always lower than those involving control cells (p less than 0.02 in case of two DP-mismatches and p less than 0.001 in the case of one DP-mismatch). Moreover, in the control series two or one DP mismatches influenced MLR I responses in 91% and 77% of the cases respectively; however, there was a greater scatter of response values which could be explained by the degree of sequence homology between the DP mismatched alleles. In cases with no DP mismatch, no proliferative response was observed. Overall, these results suggest that the conventional technical conditions of MLR I do not allow detection of mismatches other than the well known HLA specificities.     

The surgical anatomy of the middle nasal meatus

Summary An exact study was made of the middle nasal meatus in 20 anatomical preparations (left and right) from 10 subjects. Some features (the ethmoidal bulla, the uncinate process of the ethmoidal bone, the openings of the maxillary and frontal sinuses) are relatively constant in their arrangement, but very variable in shape and size. An understanding of these structures is now essential for the practice of endonasal microsurgery.

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De l'anatomie médicochirurgicale du méat nasal moyen

Résumé Une série de 20 préparations anatomiques gauche et droite sur 10 sujets a permis une observation précise du méat nasal moyen. Certains reliefs: la bulle ethmoïdale, le processus uncinatus, les méats des sinus maxillaire et frontal sont relativement fixes dans leur disposition, mais très variables dans leur forme et leurs dimensions. Leur connaissance est maintenant indispensable à tout praticien exerçant la microchirurgie endonasale.

     

Perinatal outcomes in inflammatory bowel disease

Objective: To determine whether inflammatory bowel disease (IBD) is associated with increased risk for adverse perinatal outcome.

Methods: A case–control study of 116 singleton pregnancies with IBD compared to 56?398 singleton controls delivered between 1986 and 2001.

Results: Patients with IBD were slightly older (32.8 vs. 30.6 years, p <?0.001), more likely to be Caucasian or Asian than Black or Latino (92% vs. 57%, p <?0.001) and have private health insurance (33% vs. 3%, p <?0.001). IBD was associated with an increased risk for labor induction (32% vs. 24%, p?=?0.002), chorioamnionitis (7% vs. 3%, p?=?0.04) and Cesarean section (32% vs. 22%, p?=?0.007), but there were no differences in neonatal outcomes. Subgroup analysis demonstrated an increased risk for low birth weight (LBW) in the ulcerative colitis group vs. the Crohn's disease group (19% vs. 0%, p?=?0.002). Patients with prior surgery for IBD had a lower incidence of LBW (0% vs. 12%, p?=?0.03). Flares during pregnancy were associated with an increased risk for preterm delivery (27% vs. 8%, p?=?0.02) and LBW (32% vs. 3%, p?=?0.003).

Conclusion: IBD was an independent risk factor for Cesarean section but there was no increase in adverse perinatal outcome. Crohn's disease, prior IBD surgery and quiescent disease were associated with a lower risk for LBW.