Behcet's disease is associated with increased concentrations of antibodies against phosphatidylserine and ribosomal phosphoproteins.

BACKGROUND: Although Behcet's disease (BD) is classified among the vasculitides laboratory diagnostic does not include regularly autoantibodies associated with vascular manifestations of systemic autoimmune diseases. PATIENTS AND METHODS: Twelve consecutive BD patients were studied for autoantibodies associated with vascular manifestations of systemic autoimmune diseases, HLA frequencies, and possible neurological involvement using neurophysiological methods and MRI. RESULTS: HLA-C*15 and C*16 frequencies were significantly (p < 0.05) higher in the patients compared with a reference population. Immunoglobulin G concentrations of antiphosphatidylserine and antiribosomal phosphoprotein antibodies were significantly elevated in BD patients when compared with healthy controls. CONCLUSIONS: The increased frequencies of HLA-C alleles in BD patients may stress the role of NK cells in the pathogenesis of this disease. Antiphosphatidylserine autoantibodies may in view of their role in apoptosis be involved in the development of vasculitis in BD. Because concentrations of antiphosphatidylserine and antiribosomal phosphoprotein antibodies were increased in BD diagnostic tools of this disease should be extended with humoral parameters associated with vascular manifestations of systemic autoimmune diseases.

Toxicity and excretion of cisplatin in the avian kidney.

Cisplatin is a widely applied antineoplastic drug with significant nephrotoxic potential. The purpose of this study was to define the toxic effect and excretion of cisplatin in the chicken, a species widely applied to the study of tubular transport mechanisms but little used for toxicology studies. Toxicity was assessed by the relative effect of cisplatin on renal clearances of the standard reference substrates inulin and p-aminohippurate (PAH) and by morphologic assessment of the kidneys of cisplatin-treated chickens. The data clearly support close similarities in the pattern of tubular cell damage produced in the chicken versus that reported for rats and human patients. It was further demonstrated that administration of an organic cation reduced Pt accumulation in the kidney and mitigated the toxicity as has been reported for rats. Excretion studies were carried out during unilateral renal portal infusion of cisplatin, and the results indicated that cisplatin does not undergo net tubular secretion as occurs in the rat and human. It can be concluded that, while the pattern of cisplatin-induced nephrotoxicity closely parallels that seen in mammals, the avian kidney exhibits a different pattern of urinary Pt excretion than does the mammalian kidney after cisplatin administration.

Omphalocele associated with umbilical cord allantoic cyst: sonographic evaluation in utero.

The ultrasonographic in utero diagnosis of nine fetal omphaloceles was made between 1978 and 1982. In three patients an allantoic cyst of the umbilical cord was associated with the abdominal wall defect. This association should alert the ultrasonographer to consider the possibility of omphalocele when an apparent cyst of the umbilical cord is observed.

A potential pitfall in the ultrasonographic diagnosis of fetal encephalocele.

Abnormalities of the fetal neural axis may be diagnosed prenatally utilizing ultrasonography. Attempts to increase the sensitivity of ultrasonography in the detection of these anomalies run the risk of increasing the false-positive diagnoses. Since the prenatal diagnosis of neural tube defects significantly affects the clinical management of the pregnancy, a false-positive diagnosis of neural tube anomaly must be avoided. The authors describe several cases of patients with polyhydramnios in which the fetal ear protruded into the amniotic fluid, simulating the appearance of an encephalocele on ultrasonography.

Nutrient foramina in the phalanges of the hands.

The normal distribution and size of nutrient foramina in the terminal and middle phalanges of the fingers, which appear as radiolucencies on hand radiographs, is described. In a study based on the antero-posterior (AP) hand films of 218 patients, we found no evidence that the foramina were more frequently seen in childhood. When age is disregarded in statistical analysis, the foramina are more frequently observed in women than in men. In both sexes foramina are seen most often in the middle phalanges. We have found that the upper limit of normal of foraminal diameters is 1 mm (uncorrected for enlargement) and this permits recognition of enlargement of the foramina which can occur in disorders such as Gaucher's disease, sickle cell anemia and thalassemia.

Enlarged phalangeal nutrient foramina in Gaucher disease and beta-thalassemia major.

The posteroanterior hand films of 10 of 15 patients with Gaucher disease demonstrated enlarged nutrient foramina when compared with a control group matched for patient gender and age. Similar findings were also noted in seven of 22 patients with beta-thalassemia major. Enlargement of nutrient foramina in the hands may indicate the existence of a hematologic disorder.

CT-guided aspiration biopsy of the thorax.

Percutaneous needle aspiration biopsy of thoracic lesions was performed under the guidance of computed tomography (CT) in 28 patients. The lesions chosen for CT guidance of needle placement were those not amenable to demonstration by biplane fluoroscopy: lesions in or adjacent to the mediastinum or hila; peripheral lesions; and small or ill-defined lesions, such as cavitary masses. The accuracy of the technique was assessed on the basis of pathological and radiographic confirmation of the cytologic diagnoses. The sensitivity of the technique was 84% (16/19), the specificity was 100% (6/6), and the overall accuracy was 88% (22/25). Inadequate specimens were obtained in two patients, and another patient could not complete the procedure. Pneumothorax, which developed in 17 patients, was remedied by intercostal tube drainage in three. There was one mild episode of hemoptysis, which resolved spontaneously. We recommended that CT-guided aspiration biopsy of thoracic lesions be used when the type, size, or position of the lesion makes fluoroscopic guidance of the needle unreliable.

Absolute ethanol injection of the adrenal artery: hypertensive reaction.

Transcatheter injection of 0.4 ml of absolute ethanol into the adrenal artery was performed in three Rhesus monkeys. The injection produced a mean increase of 60 mmHg in systolic blood pressure and 50 mmHg in diastolic blood pressure within two minutes. Hypertension was accompanied by cardiac arrhythmias (two monkeys) and sinus tachycardia (one monkey). These changes were probably related to an acute catecholamine release. Embolization of the inferior phrenic artery with Gelfoam powder produced only a mild blood pressure elevation in three monkeys (6 mmHg systolic pressure and 10 mmHg diastolic pressure).

Ontogeny of surfactant proteins A and B in human amniotic fluid as indices of fetal lung maturity.

Surfactant proteins A and B (SP-A and SP-B) were measured in human amniotic fluid by ELISA and correlated with lecithin to sphingomyelin ratio (L/S), phosphatidylglycerol (PG), and perinatal outcome. Amniotic fluid SP-A, SP-B, and L/S increased with advancing gestation. SP-A was detected at 19 wk gestation and increased dramatically in the 3rd trimester of pregnancy. SP-B was first detectable at 31 wk gestation and increased significantly to term. SP-A was a more specific predictor of nonrespiratory distress syndrome (RDS) than L/S or SP-B; however, the sensitivity of SP-A in predicting RDS was less than L/S less than 2.0 (26.3 versus 82.3%, respectively). In 209 pregnancies assessed within 48 h of delivery, the sensitivity of SP-B in predicting RDS (nondetectable SP-B) was comparable to the L/S, however, SP-B = 0 was frequently observed in mature infants, limiting its specificity for prediction of RDS. The greatest sensitivity and specificity were achieved with the measurement of L/S less than 2.0 and negative PG, which correctly predicted 100% of the infants with RDS and 94% of those who did not develop the disorder. Measurement of SP-A or SP-B did not improve the prediction of RDS. SP-A, SP-B, and L/S were not affected by infant sex, Apgar score, rupture of membranes, size for gestational age, maternal diabetes, hypertension, or exposure to medications. SP-A, SP-B, and L/S were significantly elevated in amniotic fluid from black mothers. SP-A was significantly elevated in amniotic fluid from mothers who smoked during pregnancy.(ABSTRACT TRUNCATED AT 250 WORDS)

Computed tomography with metrizamide myelography to define the extent of spinal canal block due to tumor.

In five patients with complete spinal block due to tumor, CT was used to show the upper level of the block. In each case metrizamide myelography demonstrated the lower level of the block, but not enough metrizamide leaked past the block to allow myelographic identification of the upper level. However, the subarachnoid space both below and above the level of the block was clearly outlined by metrizamide on CT in each case.

Lipid emulsions as contrast agents for hepatic sonography: an experimental study in rabbits.

An ultrasound contrast agent capable of increasing hepatic echogenicity would be useful for the detection of hepatic tumors and metastases. Fatty liver is known to produce increased liver echogenicity. Intravenously administered lipid emulsions are phagocytosed by cells of the reticuloendothelial system the liver with transient hepatic lipid accumulation. We examined the effectiveness of three lipid emulsions of differing particle size as potential ultrasound contrast agents using a rabbit liver model. None of the tested emulsions showed any consistent ability to alter liver echogenicity at maximum tolerable doses. Lipid emulsions do not appear to have potential as contrast agents for ultrasound examination of the liver.

Atypical CT findings in bacterial meningoencephalitis.

Computed tomography has become a valuable imaging modality in the evaluation and management of most intracerebral infections. We report two cases of intracranial infections with atypical CT findings, and attempt to correlate these findings with the pathophysiology.

Phorbol ester inhibits surfactant protein SP-A and SP-B expression.

Effects of the tumor promoter, 12-O-tetradecanoylphorbol-13-acetate (TPA), on expression of pulmonary surfactant proteins, SP-A and SP-B, were determined in a human pulmonary adenocarcinoma cell line (H441-4). TPA decreased cellular SP-A content in association with decreased de novo synthesis of SP-A as assessed by [35S]methionine incorporation. Effects of TPA were time (0-72 h) and dose (IC50 0.5-1.0 nM)-dependent. Phorbol 12,13-dibutyrate (PDBu), and adenosine 5'-O-(3-thiotriphosphate), and 1-oleoyl-2-acetyl-sn-glycerol also decreased SP-A content in these cells. Characteristics of inhibition of SP-A content by PDBu were similar to those of [3H]PDBu binding to H441-4 cells. Inhibitory effects of TPA on SP-A synthesis were associated with concomitant decreases in SP-A mRNA. Expression of a distinct surfactant protein, SP-B, was also markedly decreased after exposure to TPA. SP-A and SP-B mRNA contents decreased more rapidly after treatment with TPA than after actinomycin D. Actinomycin D completely blocked the rapid decrease in SP-A and SP-B mRNAs caused by the phorbol ester, consistent with the concept that the inhibitory effect of TPA on the surfactant protein mRNAs required continued gene transcription and was not mediated solely by changes in SP-A or SP-B transcription. Inhibitory effects of phorbol esters on SP-A and SP-B synthesis support the concept that protein kinase C modulates surfactant protein expression in this cell.

MR imaging of pheochromocytomas.

Magnetic resonance imaging (MRI) of seven patients with pheochromocytomas demonstrable by CT was performed. Magnetic resonance imaging showed all primary pheochromocytomas, as well as metastatic lesions to the thorax, retroperitoneum, and liver. The ability to distinguish pheochromocytomas from surrounding structures without the need of an intravenous contrast agent and to provide direct coronal and sagittal images suggests that MRI may be useful in detecting and localizing these tumors.

Malignant thoracopulmonary small-cell ("Askin") tumor.

The clinical, radiographic, and pathologic features of 10 patients with documented malignant small-cell tumor of the thoracopulmonary region (Askin tumor) were reviewed. The tumor represents a distinct pathologic entity of neuroectodermal origin. Clinically, it presents as a chest-wall mass with or without pain. Its radiographic appearance is that of a soft-tissue mass with or without pleural or rib involvement, often with metastatic disease--to the skeletal system, bone marrow, thorax, and sympathetic chain. Two patients developed metastases to the adrenal gland and liver, one after autologous bone marrow transplantation. The radiologist should be aware of this entity and its pattern of metastatic spread since metastases are treated aggressively.