Lymphokine-induced phagocytosis in angiocentric immunoproliferative lesions (AIL) and malignant lymphoma arising in AIL

A factor that augmented the phagocytosis of IgG-coated ox red blood cells by the human monocyte/macrophage line U937 was identified in cell culture supernatants from two of two patients with angiocentric peripheral T cell lymphomas, three of three patients with angiocentric immunoproliferative lesions that were not frankly malignant, and one of two patients with T lymphoblastic malignancies. The factor was not present in supernatants derived from 14 nonangiocentric peripheral T cell lymphomas of other histologic types nor in ten cases of B cell lymphoma and two cases of Hodgkin's disease. A similar factor was present in the supernatants of concanavalin A (Con A)-stimulated normal peripheral blood mononuclear cells and in the supernatants of IL-2- dependent T cell lines derived from normal peripheral blood. The factor had an apparent mol wt of greater than 50,000 daltons, was heat labile (100 degrees C for two minutes), and stable at pH 2.0. Its stimulation of phagocytosis was independent of any increase in number of Fc receptors. Thus, this factor is probably not gamma-interferon. This factor may play a pathogenetic role in the hemophagocytic syndromes associated with certain T cell malignancies and immunodeficient states.     

Spontaneous bacterial peritonitis in fulminant hepatic failure

Ascites may be associated with fulminant hepatic failure (FHF), but spontaneous bacterial peritonitis (SBP) is an extremely rare complication. We report on two patients with FHF who developed SBP. One patient died and the other recovered.     

In vitro and in vivo selectin-blocking activities of sulfated lipids and sulfated sialyl compounds

There is accumulating evidence that sulfated lipids, sulfated oligosaccharides and other sulfated compounds are reactive with selectins in a manner that interferes with selectin interactions with their natural ligands. In the report we describe the ability of sulfated lipids (sulfatides and gangliosides) and multimeric forms of sulfated sialic acid to block binding of P- and E-selectin-Ig to neutrophils. The in vivo ability of these compounds to block lung injury in rats following i.v. infusion of purified cobra venom factor (CVF), which induces injury that is L- and P-selectin dependent, was also determined as well as effects on recruitment of neutrophils, as measured by lung myeloperoxidase. There was a significant correlation between the ability of sulfated lipids and sialyl compounds to interfere in vitro with P-selectin-Ig binding to neutrophils and to protect against P-selectin-dependent acute lung injury induced by CVF. The biological effects of these sulfated compounds were also associated with diminished accumulation of neutrophils. The protective effects of these compounds may be linked to their ability to interfere with P- selectin binding to counter-receptors on neutrophils.      

Surgical division of craniopagi

The world's surgical experience on the division of craniopagi is reviewed, and a new case is reported. The problems, including ethical considerations, that must be addressed in selecting patients for operation and in preparing for the procedure are discussed in detail. Hemodynamic stability and the problem of intraoperative gravitational distortion of the brain are addressed. The importance of careful planning is stressed. Survival of the 31 craniopagi after operation is discussed in relation to types of junction.     

Astigmatism in unilateral coronal synostosis: incidence and laterality.

PURPOSE: Unilateral coronal synostosis alters orbital development and can be associated with strabismus, oblique muscle dysfunction, astigmatism, and amblyopia. While the ocular effects are most dramatic on the side of the fusion, the shape of the contralateral orbit is also abnormal and the effect of these changes on bilateral visual development has not been fully described. The purpose of this study was to analyze the incidence and laterality of astigmatism in children with unilateral coronal synostosis. METHODS: We retrospectively reviewed the charts of 39 consecutive patients with unilateral coronal synostosis. Recorded data included the following: patient demographics, visual acuity, cycloplegic refraction, ocular motility, procedures for craniofacial and strabismus correction, and presence and treatment of amblyopia at each visit. RESULTS: Twenty-one of 39 patients (54%) had 1.00 D or more astigmatism in at least one eye at their last recorded refraction. Sixteen of the 21 (76%) had aniso-astigmatism of 1.00 D or more. Of the 16, 14 (88%) had the greater degree of astigmatism in the contralateral eye. Fifteen of the 39 studied (38%) had amblyopia and, in 12 of these 15 patients (80%), the amblyopic eye was contralateral to the synostosis. Nineteen of 39 patients (49%) had greater ipsilateral fundus excyclotorsion, whereas only 1 of 39 (3%) had greater contralateral excyclotorsion. CONCLUSIONS: Patients with unilateral coronal synostosis are at risk for developing a greater degree of astigmatism in the eye contralateral to the synostosis. Ocular disturbances in the contralateral eye, especially anisometropic amblyopia, must be considered when treating patients with this type of craniosynostosis.     

Correction of the bilateral cleft lip nasal deformity: evolution of a surgical concept.

The origin of the nasal deformity of a bilateral complete cleft lip is both primary (deformation/malformation) and secondary (postoperative distortion). This is an interim report of a personal evolution from staged correction of the bilateral cleft nasal deformity to synchronous repair of the nose and the lip and premaxillary-maxillary clefts. The anatomic concept is that, because of the malpositioned alar cartilages, the columella only appears to be short in an infant with bilateral cleft lip. The technical stratagems to model the nose are: (1) alignment of the premaxilla and (2) anatomic placement of the alar cartilages with sculpturing of the overlying soft tissue.     

A retrospective analysis of growth of the constructed condyle-ramus in children with hemifacial microsomia

A retrospective analysis of changes in costochondral rib grafts used to construct the condyle-ramus in children with hemifacial microsomia (HFM) was made. The mean age at surgical correction was 6.5 years, and the average follow-up was 4.5 years. Direct measurements were made on panoramic radiographs. The condyle-ramus length was expressed as a percentage change comparing the constructed with the normal side. During the first 2 postoperative years, there was either no change or a slight decrease in the length of the rib graft. After 2 years, however, the costochondral graft elongated at a slow, irregular rate. The mode change was 11 percent over the postoperative study period. In four patients who exhibited rapid growth of the normal condyleramus (greater than the mean change of 0.94 cm), the constructed side failed to keep pace. In another group of four patients who exhibited moderate elongation of the normal side, the grafted side grew commensurately or demonstrated greater than normal percentage change in length. There was no correlation between the initial size of the costochondral graft, age at time of operation, or presenting type of mandibular deformity. These findings are discussed in terms of the intrinsic growth and the functional matrix theories of mandibular development.