Follicular dendritic cell tumor of the mediastinum: expression of fractalkine and SDF-1α as mast cell chemoattractants

Follicular dendritic cell tumor (FDCT) is a rare tumor mainly located in laterocervical lymph nodes. We report one case of mediastinal FDCT associated with a history of bullous skin disease and clinically obvious immunosuppression. This tumor was characterized by heavy mast cell infiltration. Mast cells were in close relationship with tumor cells as demonstrated by ultrastructural examination and their presence are probably related with the strong expression of mast cell chemoattractants as fraktalkine and stromal cell-derived factor-1α by tumor cells. The long follow-up period of more than 17 years allowed to us assess the relatively indolent evolution of this tumor characterized by three slowly growing local recurrences without metastasis.     

Hyponatremia in neurological patients: cerebral salt wasting versus inappropriate antidiuretic hormone secretion

Objective To assess whether hyponatremia in acute neurological patients is associated with the syndrome of inappropriate antidiuretic hormone secretion (SIADH) or with the cerebral salt-wasting syndrome (CSWS). Design Clinical, controlled, prospective study. Setting Department of intensive care of a tertiary care academic hospital. Patients Forty acute neurological patients with hyponatremia suggesting SIADH or CSWS (20) or with normonatremia (20). Interventions None. Measurements and main results Measurement of clinical and biological variables. Measurement of blood, plasma, and red blood cell volumes to discriminate SIADH and CSWS. Renal, adrenal and thyroid functions were normal in all patients. Average blood, plasma, and red blood cell volumes were 54, 37 and 17 ml/kg in control patients and 54, 37 and 18 ml/kg in hyponatremic patients, respectively. Conclusions The adequate blood volumes in hyponatremic patients confirm the diagnosis of SIADH and do not support the concept of CSWS. Electronic supplementary material The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Late ear sequelae in cleft palate patients

This retrospective study looks at the incidence and nature of ear disease in 50 adolescent patients who had cleft palates repaired in infancy. Half of these patients had a history of grommet insertion. We found that most patients had normal hearing (81%) and middle-ear pressures (86%), although about half had tympanic membrane abnormalities. Grommet insertion did not result in better long-term hearing in this study but was strongly associated with tympanosclerosis. Cleft type did not influence the degree of ear disease although more patients with complete clefts had a history of repeated grommet insertion. Otitis media with effusion is almost universal in cleft palate infants and may influence later language, speech and educational development. At the time of palatal repair grommets should be inserted to improve hearing in these infants.     

Systemic oxygen extraction can be improved during repeated episodes of cardiac tamponade

We used a tamponade model to study the relationship between oxygen uptake (VO₂) and oxygen delivery (Do₂) during successive, reversible decreases in blood flow. In 7 pentobarbital-anesthetized and mechanically ventilated dogs, a catheter was introduced via a left thoracotomy into the pericardium to inject and to withdraw saline. Each experiment consisted of three steps. First, cardiac output was reduced by successive pericardial fluid injections until 4 to 6 data points were obtained in the dependent region of the o₂/ o₂ plot (step 1). Second, cardiac output was restored by progressive withdrawal of pericardial fluid (step 2). Third, cardiac output was lowered again by reinjection of fluid into the pericardium until death (step 3). Expired gases were collected for determination of ₂. In each animal, critical o₂ ( o₂crit), below which ₂ became o₂ dependent, was determined from a plot of ₂ versus o₂. When releasing tamponade, ₂ was restored to baseline. For the 3 steps, o₂crit were 10.5 ±- 2.2 mL/kg/min in step 1, 9.8 ±- 1.8 mL/kg/min in step 2, and 8.3 ±- 1.9 mL/kg/min in step 3 (P < .01 v step 1; P < .05 v step 2, respectively). There was no significant difference in o₂2 at o₂crit for the three steps. Hence, critical oxygen extraction ratio (ERo₂crit) increased from 60% ±- 12% in step 1 to 64% ± 11 % in step 2 (not significant) and to 73% ±- 12% in step 3 (P < .01). The ₂/ o₂ dependency slope was also steeper in step 3 than in step 1 (0.77 ± 0.31 v 0.54 ±- 0.20, P < .05). A progressive decrease in arterial and in mixed venous pH was observed during the experiment. We conclude that a decrease in ₂ associated with an acute reduction in blood flow can be readily reversible. When the procedure is repeated, a progressive increase in oxygen extraction capabilities is observed. This reversible tamponade model is potentially suitable to induce several hypoxic episodes in the same animal.     

Total body irradiation–high-dose cytosine arabinoside and melphalan followed by allogeneic bone marrow transplantation from HLA-identical siblings in the treatment of children with acute lymphoblastic leukaemia after relapse while receiving chemotherapy: a Société Française de Greffe de Moelle study

We investigated the use of a new conditioning regimen followed by allogeneic bone marrow transplantation (BMT) for treating children with acute lymphoblastic leukaemia (ALL) after relapse within 6 months of the completion of therapy. One hundred and sixteen children with acute lymphoblastic leukaemia in second or subsequent complete remission (CR) underwent allogeneic bone marrow transplantation from HLA-identical siblings after a preparative regimen comprising total body irradiation (TBI), high-dose cytosine arabinoside and melphalan (TAM regimen). The Kaplan-Meier product-limit estimate (mean ± SE) of disease-free survival (DFS) at 7 years was 59.5 ± 9% (95% confidence interval). The estimated chance of relapse was 22.5 ± 15% with a median follow-up of 88.5 months (range 51–132). 26 patients (22.4%) died with no evidence of recurrent leukaemia, mainly from interstitial pneumonitis, veno-occlusive disease or acute graft-versus-host disease (GVHD). Three factors significantly affected DFS: acute GVHD, site of relapse and, for children in second remission after a marrow relapse, the disease status at the time of transplantation. The DFS were 59.02 ± 12.6%, 37.5 ± 19.8% and 77.4 ± 15% among patients in CR2 after a marrow relapse, in CR3 or in untreated partial marrow relapse, and in CR2 after an isolated CNS relapse, respectively. The lowest DFS was seen in children with acute GVHD grades 3–4. Two significant factors were associated with relapse: the marrow status at the time of transplantation and chronic GVHD. The relapse rate was lower among children in CR2 or with chronic GVHD. We conclude that transplantation after the TAM regimen is an effective therapy for this population with acceptable toxicity, particularly for children in second remission after a very early marrow relapse, or those with early isolated CNS involvement.     

Enhanced removal of cholesterol from macrophage foam cells to serum from type IV hypertriglyceridemic subjects

Hypertriglyceridemia being an independent cardiovascular risk factor, we have compared the potential of sera from asymptomatic hypertriglyceridemic (HTG) type IIb, type IV or normolipidemic (NLP) subjects to promote both fractional cholesterol efflux and cellular cholesterol mass changes using macrophage foam cells. The J774 cells loaded with cholesterol by incubation with acetylated LDL were incubated in the absence or presence of cAMP to upregulate ABCA1 (ATP binding cassette transporter A1) and then incubated for 24 h with 1% serum. Compared with NLP, type IV sera exhibited a major increase in ABCA1-dependent efflux while type IIb sera exhibited a moderate but not significant increased ABCA1-mediated efflux. Moreover, positive correlations were established between ABCA1-dependent efflux and the serum preβ-HDL or TG concentrations. The major finding was that the sera from type IV induced higher total cholesterol and cholesteryl ester mass depletions from ABCA1-expressing cells compared with other groups. Moreover, negative correlations were obtained between total cholesterol or cholesteryl ester mass changes and serum preβ-HDL levels. In conclusion, we demonstrated for the first time that the serum preβ-HDL present in high proportions in type IV HTG subjects are not only responsible for higher cholesterol efflux potential but also for increased abilities to promote net removal of cholesterol from macrophage foam cells.     

Effect on blood lipids of two daily servings of Camembert cheese. An intervention trial in mildly hypercholesterolemic subjects

As a concentrated source of saturated fat, cheese consumption is considered to be associated with increased cholesterolemia and generally forbidden in dietary guidelines for adults with hypercholesterolemia. The aim of this study was to evaluate the impact of saturated fatty acids on lipid parameters and blood pressure with regards to different types of dairy products: Camembert and full-fat yoghurt. One-hundred and fifty-nine moderate hypercholesterolemic subjects without treatment were instructed to consume two full-fat yoghurts (2?×?125?g) per day for 3 weeks (run-in period) and then for a further period of 5 weeks, either two full-fat yoghurts or two 30?g servings of Camembert cheese per day. We observed that over the 5-week daily consumption of two servings of Camembert cheese, blood pressure and serum lipids did not change in moderate hypercholesterolemic subjects. These results suggest that fermented cheese such as Camembert could be consumed daily without affecting serum lipids or blood pressure.     

Lessons from 30 years of selective screening for tetrahydrobiopterin deficiency

In addition to being hyperphenylalaninemic, patients lacking tetrahydrobiopterin (BH4) are deficient in the neurotransmitters whose synthesis depends on the normal activity of tetrahydrobiopterin-dependent tyrosine and tryptophan hydroxylases. Consequently, these patients have to be rapidly recognized among hyperphenylalaninemic babies, since they need specific and early substitutive therapy. Since 1980, BH4 metabolism has been investigated in 2,186 hyperphenylalaninemic babies, using HPLC measurement of pteridines in urine to recognize tetrahydrobiopterin synthesis deficiency (GTP cyclohydrolase and PTPS deficiency) and direct DHPR assay in dried blood samples to recognize DHPR deficiency. A total of 73 tetrahydrobiopterin deficient patients have been detected. Considering the group of neonates born in France (1,342), out of the 32 BH4 deficient patients which have been detected, only 8 were from caucasian families. The lessons from that experience are: (1) tests on blood and urine collected on filter paper cards commend itself by their convenience and simplicity, and samples can be collected on the first visit of the screened infants to the out-patient clinic; and (2) the preconceaved idea that newborns with moderate elevation of blood phenylalanine are false positives of the screening or mild forms of hyperphenylalaninemia explains that a significant number of cases were investigated after 1 month of age; however, in half of BH4-deficient babies, blood phenylalanine was below 10 mg/dl (0.6 mmol/l).