Familial fibrocystic pulmonary dysplasia: a detailed family study

Six cases of familial fibrocystic pulmonary dysplasia are described involving five siblings and their father. The clinical findings and radiological features were similar in all six patients although there was some variation in the period of survival following the onset of the disease. In three the diagnosis was confirmed pathologically; the two brothers, who did not have lung biopsies, had disturbances in respiratory function which are considered typical of the impaired diffusion produced by interstitial fibrosis. One hundred and five members of the family were surveyed for evidence of this disease, but no further cases were discovered. Four of the patients had some elevation of their gamma globulin. Immunoelectrophoretic analysis, which was performed on three of the patients, the two healthy siblings, and 16 of their offspring, showed elevated immunoglobulin patterns. This evidence suggests the possibility of an inherited aberration in the immune response in this family.     

Intracerebral hemorrhage in two patients with Down’s syndrome and cerebral amyloid angiopathy

Cerebral amyloid angiopathy (CAA) is an important cause of spontaneous intracerebral hemorrhages in the elderly and is often seen in the brains of patients with Alzheimer’s disease, Down’s syndrome (DS), and hereditary cerebral hemorrhage with amyloidosis of the Dutch type. We report two patients with DS and extensive CAA who died of intracerebral hemorrhage; only two other such case reports exist in the literature. We believe the incidence of such cases is higher than is reported and that the likelihood of hemorrhage in the setting of CAA is independent of the patient’s underlying disease. Received: 27 March 1997 / Revised, accepted: 8 August 1997     

Causes of the Increased Stroke Rate after Carotid Endarterectomy in Patients with Previous Strokes

n = 183) were compared to those who were either asymptomatic or experienced only transient ischemic attacks (TIAs) preoperatively (n= 423). Of the 183 patients who had suffered preoperative strokes, eight patients who experienced perioperative strokes after endarterectomy were compared with 175 who successfully underwent surgery. Patients with a prior stroke had an increased perioperative stroke rate (4.4% versus 1.2%, p= 0.01). They had a significantly higher incidence of hypertension (62.6% versus 47.9%, p < 0.001), cardiac disease (54.7% versus 40.7%, p= 0.001), and positive smoking history (52% versus 40.6%, p= 0.01) than did the asymptomatic/TIA patients. The presence of contralateral total occlusion was also significantly increased (22% versus 10.3%, p < 0.001). Although not statistically significant due to the overall small number of patients who sustained perioperative strokes, the preoperative stroke patients who sustained perioperative strokes had a higher incidence of hypertension (87.5% versus 61.5%) and contralateral total occlusion (37.5% versus 21.3%) than did those who successfully underwent surgery. Patients with both a prior stroke and contralateral total occlusion had a 7.5% perioperative stroke rate. Patients with both a prior stroke and hypertension had a 6.1% perioperative stroke rate. The perioperative strokes in patients with prior strokes were not related to the severity of the prior stroke, the interval between the stroke and surgery, the use of a shunt, or the type of anesthesia employed. Patients who have sustained preoperative strokes have a higher incidence of significant medical illnesses and overall cerebrovascular disease. Hypertension and total occlusion of the contralateral carotid artery appear to be particularly poor prognostic indicators of outcome after endarterectomy in these patients. Patients who have sustained preoperative strokes may be more likely to display clinical neurologic symptoms in response to any form of cerebral ischemia. In this higher risk subgroup, intraoperative and surgeon-dependent factors appear to play less of a role.     

Glial hyaluronate-binding protein in polar spongioblastoma

Glial hyaluronate-binding (GHA) protein is a 60 kDa glycoprotein isolated from human white matter by affinity chromatography on immobilized hyaluronate. It is localized in white matter astrocytes by immunofluorescence with monoclonal antibodies. Amino acid sequences have not revealed similarities with other proteins except cartilage extracellular matrix proteins, the region of similarity being located within the hyaluronate-binding region. Cryostat sections of 13 intracranial neoplasms removed at surgery were tested for the presence of GHA protein by indirect immunofluorescence with monoclonal antibodies. These included seven astrocytomas, one oligodendroglioma, one medulloblastoma and one spinal cord ependymoma. All tumors were negative with the exception of one astrocytoma in which the GHA protein-positive areas had the typical appearance of polar spongioblastoma, i.e. small cells palisading around blood vessels and very delicate glial fibrillary acidic (GFA) protein-positive fibrils. Conversely, neoplastic as well as reactive GFA protein-positive astrocytes were GHA protein-negative. We suggest that polar spongioblastoma derives from a GHA protein-positive glial precursor and pertinent to this suggestion is the observation that the periventricular germinal layer was found GHA protein-positive in a 22-week human fetus.     

Rehabilitation in the elderly

Prevention of disease should be every physician's goal, however, when primary or secondary prevention fail, rehabilitation of the patient becomes an additional goal. Elderly patient can be as successfully rehabilitated as younger patients, but their special needs are most successfully met by a multidisciplinary team. Almost every patient with a disability warrants a trial of rehabilitation aimed toward placing the patient in the least restrictive environment possible. Rehabilitation is especially useful in patients with arthritis, and following stroke, joint replacement, and amputation.     

MR imaging of rat brain glioma: Gd-DTPA versus Gd-DOTA

The enhancement properties of gadolinium diethylenetriaminepentaacetic acid (DTPA) and gadolinium tetraazacyclododecanetetraacetic acid (DOTA) were compared using a rat glioma model. In vitro analysis included the calculation of T1 relaxivity and determination of characteristic curves. Enhancement of the intracerebral glioma was studied in 23 rats approximately 2 weeks after glioma implantation with Gd-DTPA in 12 rats and Gd-DOTA in 11 rats. Six rats were also studied 1 week after implantation. Gd-DTPA exhibited a slightly greater T1 relaxivity in vitro than Gd-DOTA. Enhancement of the glioma was also greater with Gd-DTPA than with Gd-DOTA (P less than .05).     

Effects of enteral and intravenous antimicrobial treatment on survival following intestinal ischemia in rats

One hundred and twenty rats underwent transection of the superior mesenteric artery. The animals were randomly divided into eight groups of 15 animals. Control group 1 and groups 3, 5, and 7 received intravenous normal saline, gentamicin, metronidazole, and gentamicin plus metronidazole, respectively. Control group 2 and groups 4, 6, and 8 received the same compounds enterally. Small and large bowel sections were taken postmortem and a necrosis score was assigned in blinded fashion. Gentamicin did not prolong survival, indicating that gram-negative microbes were not important in this pathology. Longer survival times for animals given either metronidazole or gentamicin plus metronidazole (P less than 0.01) indicate that anaerobes were a causative factor in mortality. During the first 15 hr after ischemia, antibiotics did not change mortality. After 15 hr, enteral administration was superior to intravenous administration in any regimen including metronidazole (P less than 0.01).