Liver transplantation in Langerhans' cell histiocytosis (histiocytosis X).

Two children with biopsy-proven LCH underwent successful hepatic transplantation for end-stage liver disease. These patients were thought not to have active LCH disease at the time of transplantation, although one had developed a new osteolytic lesion a few months before the operation and the other had suspicious osteolytic lesions at the time of transplantation. The histologic examination of the excised liver showed features consistent with primary sclerosing cholangitis.

Natural killer cell-dependent mycobacteriostatic and mycobactericidal activity in human macrophages.

Host defense mechanisms against Mycobacterium avium complex (MAC) are poorly understood. Recent evidence suggests the role of NK cells in the host defense against some intracellular pathogens. We investigated whether NK cells play a role in MAC infection.

Orbital exenteration. The reconstructive ladder.

Following orbital exenteration, there is a spectrum of immediate and delayed options for orbital reconstruction. Goals of reconstruction after exenteration include detection of recurrent disease, restoration of boundaries between the orbit and surrounding structures, and optimal aesthetics. Local solutions to problems of the exenterated orbit, such as healing by granulation or application of split-thickness skin grafts, are advantageous for detecting recurrent disease.

A micro system for rigid bony fixation in orbital surgery.

Plates and screws are effective for rigid internal fixation of bone fractures and osteotomies in the midface, orbit, and cranium. We describe our experience with the Luhr Micro System. This is the newest generation of Luhr Systems and is the smallest system available for rigid internal fixation.

Negative association between schizophrenia and rheumatoid arthritis.

Persuasive evidence has accumulated demonstrating a strong negative association between rheumatoid arthritis and schizophrenia at the population level. Explanations for this phenomenon have taken into consideration immunological, biochemical, and genetic factors. In this article, we examine these and other factors in closer detail.

Photic sneeze reflex in nephropathic cystinosis.

Photic induced sneeze is a reflex that occurs in certain individuals after exposure to bright light. Cystinosis is an autosomal recessive inborn error of metabolism in which nonprotein cystine accumulates within lysosomes. The pathognomonic ocular manifestation of cystinosis is corneal crystal deposition.

Immunopathology of vitreous and retinochoroidal biopsy in posterior uveitis.

The authors used immunopathologic techniques to study vitreous and/or retinochoroidal biopsies from 23 patients with posterior uveitis unresponsive to conventional therapy or who had developed significant complications despite therapy. Results indicated that during active uveitis from many causes, T-helper cells predominated in the vitreous and retinochoroidal biopsies. Monocytes were not prominent constituents except in several cases of granulomatous etiology (e.

Seven hundred medicolegal cases in ophthalmology.

Seven hundred medicolegal claims in ophthalmology were reviewed by one ophthalmologist who served as an expert for four decades. The ophthalmologist was personally involved in 620 claims. The 700 cases have been categorized and analyzed.

Gastrointestinal tuberculosis. Report of four cases.

Gastrointestinal tuberculosis is a rare disease in the United States. Correct identification is often delayed because it is not considered early on in the differential diagnosis. Four patients with gastrointestinal tuberculosis and the symptoms, diagnosis, complications, and treatment of the disease are discussed.

Needle revision with and without 5-fluorouracil for the treatment of failed filtering blebs.

Twelve patients with failed or failing filtering blebs were treated by transconjunctival needle revision of the bleb. Seven of these received 5-fluorouracil as an adjunct. Intraocular pressure decreased from 31.

Disc swelling in an adult diabetic patient.

A patient with insulin dependent adult onset diabetes presented with bilateral disc edema and minimal visual dysfunction. Initial work-up excluded an intracranial lesion, and a lumbar puncture revealed a normal opening pressure. The patient developed proliferative retinopathy, for which she received photocoagulation therapy.

Postpartum psychosis with abnormal movements: dopamine supersensitivity unmasked by withdrawal of endogenous estrogens?

The authors describe two cases of postpartum psychosis accompanied by abnormal extrapyramidal movements. The presentation of these cases, as well as research evidence indicating that estrogens influence dopamine receptor function, leads the authors to hypothesize that dopamine receptor supersensitivity may have been induced by prepartum increases in endogenous estrogens and unmasked by postpartum estrogen withdrawal.

Neurologic complications of cardiac transplantation.

Between 1984 and 1989, orthotopic cardiac transplantations were done in 90 patients from 10 to 65 years of age for end-stage, refractory congestive cardiomyopathy. Two patients had had ischemic strokes 5 months and 18 years, respectively, before transplantation. Six patients (7%) suffered acute neurologic events perioperatively.

Can lifestyle changes reverse coronary heart disease? The Lifestyle Heart Trial.

In a prospective, randomised, controlled trial to determine whether comprehensive lifestyle changes affect coronary atherosclerosis after 1 year, 28 patients were assigned to an experimental group (low-fat vegetarian diet, stopping smoking, stress management training, and moderate exercise) and 20 to a usual-care control group. 195 coronary artery lesions were analysed by quantitative coronary angiography. The average percentage diameter stenosis regressed from 40.

M wave changes with temperature in amyotrophic lateral sclerosis and disorders of neuromuscular transmission.

Local cooling of the muscle resulted in significant increases in M wave surface areas in patients with ALS, myasthenia gravis, the Lambert-Eaton myasthenic syndrome, and also in controls. The most striking increases were seen in patients with early ALS who had minimal lower motor neuron involvement and/or little defect on neuromuscular transmission and patients with the myasthenic syndrome. Patients with myasthenia gravis had intermediate increases between these groups and the controls; there was a larger increase in M wave surface area in myasthenia gravis compared with controls but this could be accounted for by improvement in neuromuscular transmission.

Comparison of right ventricular and biventricular circulatory support in a porcine model of right heart failure.

The effects of right ventricular (RVAD) and biventricular assist devices (BVAD) in an acute porcine model of right heart ischemic failure produced by occluding the right coronary artery for 2 min (RCAO) were compared. Right and left ventricular pressures were measured with Millar transducers and respective septal-to-free wall dimensions (RVSFWD, LVSFWD) with ultrasonic crystals. RCAO alone resulted in significant right heart failure, marked by a 36 +/- 5% reduction in cardiac output (pulmonary artery flow) and a 54 +/- 16% reduction in RV stroke work.

Effect of intraocular aspiration of sodium hyaluronate on postoperative intraocular pressure.

A prospective, randomized, masked study was conducted to evaluate whether intraocular aspiration of sodium hyaluronate used in cataract surgery influenced postoperative intraocular pressure (IOP). Ninety-nine patients (105 eyes) underwent uncomplicated extracapsular extractions with posterior chamber intraocular lens implantation using 1% sodium hyaluronate (AMVISC). In 53 eyes, sodium hyaluronate was aspirated from the anterior chamber prior to wound closure.

Recombinant granulocyte-macrophage colony-stimulating factor activates human macrophages to inhibit growth or kill Mycobacterium avium complex.

Organisms belonging to the Mycobacterium avium complex (MAC) are associated with life-threatening bacteremia in patients with the acquired immunodeficiency syndrome (AIDS). As these organisms survive within macrophages, we examined the ability of recombinant human granulocyte-monocyte colony-stimulating factor (GM-CSF) to activate human monocyte-derived macrophages to inhibit the intracellular growth or kill the most mouse-virulent MAC strain in our collection that belongs to serotype 1. While unstimulated cells did not inhibit intracellular growth of MAC, macrophages activated by GM-CSF (10-10(4) U/ml) inhibited or killed up to 58 +/- 5% of the initial inoculum.

Immediate breast reconstruction with deepithelialized TRAM flaps: techniques for improving breast reconstruction.

In selected patients with lower quadrant breast masses, large breasts, and sufficient abdominal tissue, standard techniques for breast reconstruction can be modified to improve overall results. The transverse abdominal island flap can be deepithelialized and mobilized to reconstruct unilateral or bilateral defects. Furthermore, skin markings prior to mastectomy that conform to a modified Wise pattern will allow for more aesthetic positioning of eventual scars.

Preliminary correlations of clinical outcome with in vitro chemosensitivity of second passage human breast cancer cells.

These studies describe the clinical correlations of 63 in vitro chemosensitivity assays on breast cancer cells after short-term monolayer culture. Forty-five of the assays were single agent correlations. Based on cut-off values determined empirically, the test accurately predicted resistance for 36 of 41 patients (88%) who did not respond to the drug.