The cardio-facio-cutaneous (CFC) syndrome and Noonan syndrome: are they the same?

The distinction between the cardio-facio-cutaneous syndrome (CFC) and the Noonan syndrome (NS) has been based on the presence of a characteristic facies, abnormal hair and skin, and sporadic occurrence. However, all reports of the CFC syndrome comment on the similarity between it and NS, and its sporadic nature is now debatable. This report demonstrates the evolution of the clinical phenotype in a patient with the CFC syndrome and prompts us to question the validity of separating CFC from NS.     

Changes in the nuclear uptake and retention of 3H-estrogen in gonadotrophs and lactotrophs as a function of age

A quantitative autoradiographic immunocytochemical study was performed in which the nuclear uptake and retention of 3H-estradiol (3H-E2) by luteinizing hormone (LH) and prolactin (PRL) cells was examined in 19-21-year-old baboons. 3H-E2 concentrating cells were found in all of the three lobes of the pituitary in varying percentages (38.7%, pars distalis; 17.1%, pars intermedia; 6.3%, pars nervosa). Approximately 80% of PRL cells and nearly 100% of LH cells were labeled. A count of the number of silver grains over nuclei revealed a marked variation of the accumulation of 3H-E2 by LH cells and to a lesser extent in PRL cells. These results suggest functional heterogeneity among LH and PRL cells. The present results are discussed in relation to the physiological state of old animals.     

Regulation of T-cell function in lung tissue by pulmonary alveolar macrophages.

We have examined by limit dilution analysis the frequency of several types of DBA/2-specific precursor cells found in the draining lymph nodes of BALB/c mice following anterior chamber or subconjunctival inoculations of P815 tumour cells. Assays for precursors of cytotoxic T cells (pTc) and T-helper cells [interleukin-2 (IL-2)- and IL-4-producing cells] were conducted periodically during a 6-month interval after injection of tumour cells. The results indicate that nodes of both sets of recipients contained primed P815-specific CD8+ pTc that were detectable within 2 weeks of tumour implantation, and persisted throughout the 6-month observation period. Early after tumour inoculation, but not thereafter, these CD8+ cells also secreted Il-2. By contrast, only lymph nodes from mice that received P815 cells into the subconjunctival space contained CD4+ cells that secreted both IL-2 and IL-4; eventually, IL-4-secreting cells formed the vast majority of P815-specific CD4+ cells in these mice. Lymph nodes of mice that received P815 cells in the anterior chamber contained CD4+ T cells that were clonally expanded, and secreted IL-2, but not IL-4. These IL-2-secreting cells proved to be short-lived and were not present 6 months after inoculation. It is proposed that the IL-2- and IL-4-secreting T cells found in lymph nodes of subconjunctival tumour recipients are in vivo homologues of Th0 cells, that these cells can mediate delayed hypersensitivity responses, and that they are the forerunners of, or are themselves, memory T cells. These data indicate that the failure of mice that receive P815 tumour cells in the anterior chamber to display antigen-specific delayed hypersensitivity results from an inability to convert antigen-activated, IL-2-only-secreting CD4+ T cells (pTh) into Th0 cells. These findings also imply that mice with anterior chamber-associated immune deviation (ACAID) fail to develop memory CD4+ T cells.     

Improved method for the isolation of purified mouse peritoneal macrophages

Mouse peritoneal macrophages were cultured for 45 min in medium supplemented with fetal calf serum (FCS) in petri dishes coated overnight with heat-inactivated FCS. After removal of non-adherent cells by washing, adherent cells were detached by a brief incubation in the presence of sub-toxic levels of ethylenediamine tetraacetate (EDTA). Overall peritoneal macrophage recoveries of 90% can be routinely achieved with this method, and full cell viability is maintained.     

Treatment of severe cardiogenic pulmonary edema with continuous positive airway pressure delivered by face mask

BACKGROUND. Severe cardiogenic pulmonary edema is a frequent cause of respiratory failure, and many patients with this condition require endotracheal intubation and mechanical ventilation. We investigated whether continuous positive airway pressure delivered by means of a face mask had physiologic benefit and would reduce the need for intubation and mechanical ventilation. METHODS. We randomly assigned 39 consecutive patients with respiratory failure due to severe cardiogenic pulmonary edema to receive either oxygen alone or oxygen plus continuous positive airway pressure delivered through a face mask. It was not possible to blind the investigators to the assigned treatment. Physiologic measurements were made over the subsequent 24 hours, and the patients were followed to hospital discharge. RESULTS. After 30 minutes, both respiratory rate and arterial carbon dioxide tension had decreased more in the patients who received oxygen plus continuous positive airway pressure. The mean (+/- SD) respiratory rate at 30 minutes decreased from 32 +/- 6 to 33 +/- 9 breaths per minute in the patients receiving oxygen alone and from 35 +/- 8 to 27 +/- 6 breaths per minute in those receiving oxygen plus continuous positive airway pressure (P = 0.008); the arterial carbon dioxide tension decreased from 64 +/- 17 to 62 +/- 14 mm Hg in those receiving oxygen alone and from 58 +/- 8 to 46 +/- 4 mm Hg in those receiving oxygen plus continuous positive airway pressure (P less than 0.001). The patients receiving continuous positive airway pressure also had a greater increase in the arterial pH (oxygen alone, from 7.15 +/- 0.11 to 7.18 +/- 0.18; oxygen plus continuous positive airway pressure, from 7.18 +/- 0.08 to 7.28 +/- 0.06; P less than 0.001) and in the ratio of arterial oxygen tension to the fraction of inspired oxygen (oxygen alone, from 136 +/- 44 to 126 +/- 47; oxygen plus continuous positive airway pressure, from 138 +/- 32 to 206 +/- 126; P = 0.01). After 24 hours, however, there were no significant differences between the two treatment groups in any of these respiratory indexes. Seven (35 percent) of the patients who received oxygen alone but none who received oxygen plus continuous positive airway pressure required intubation and mechanical ventilation (P = 0.005). However, no significant difference was found in in-hospital mortality (oxygen alone, 4 of 20 patients; oxygen plus continuous positive airway pressure, 2 of 19; P = 0.36) or the length of the hospital stay. CONCLUSIONS. Continuous positive airway pressure delivered by face mask in patients with severe cardiogenic pulmonary edema can result in early physiologic improvement and reduce the need for intubation and mechanical ventilation. This short-term study could not establish whether continuous positive airway pressure has any long-term benefit or whether a larger study would have shown a difference in mortality between the treatment groups.     

Developmental regulation of type 1 and type 3 interferon production and risk for infant infections and asthma development

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