Understanding the factors that influence patient satisfaction with ambulance services.

The quality of ambulance services has an immense impact on patients' future well-being and quality of life. Patient satisfaction is one of the key metrics for evaluating the quality of this service. Yet, the patient satisfaction measurement may be limited in its ability to accurately reflect this service quality, and even reflect factors beyond the patient experiences. We analyze 10 years of survey data to reveal a number of factors that systematically bias ambulance satisfaction ratings. Taking into account these biases provides more robust comparison of ambulance performance over time or across different jurisdictions.

Late onset of severe graft-versus-host disease in a pediatric liver transplant recipient.

We report the management of a patient with the late onset of chronic graft-versus-host disease (GVHD) after orthotopic liver transplantation. GVHD is a rare complication of solid organ transplants that usually presents early after transplantation and is fatal in the majority of cases. Our patient differs from the typical patient with GVHD in that the onset of her disease was very late. Although most treatment to date consisted of an increase in immunosuppressive therapy, our patient showed an excellent response to a reduction. This resulted in the abatement of the symptoms of GVHD and the preservation of her allograft function.

Monotherapy with cyclosporine for chronic immunosuppression in pediatric liver transplant recipients.

Children who survive liver transplantation (LT) suffer the adverse effects of life-long immunosuppression. In an attempt to minimize these effects, we have instituted a program of tapering immunosuppression, resulting in chronic monotherapy for children after LT. Seventy-three children ages 4 months to 19 years received LT between January 1987 and December 1992. Patient survival was 85% (62/73), with graft survival of 73%, at one year. Triple therapy with prednisone, cyclosporine, and azathioprine begun at transplant was tapered as follows: 1-2 mg/kg prednisone at discharge was reduced by .2 mg/kg every 2 months until a .2 mg/kg total was reached. Alternate-day steroids (.2 mg/kg) were begun at 1 year and discontinued at 1.5 years. AZA (1 mg/kg) was begun posttransplant and discontinued after any serious viral illness or by 1 year. Currently 37 survivors are > 18 months post-LT and were considered candidates for monotherapy. Monotherapy was attempted in 28 (76%), and 25 of these remain on monotherapy an average of 2 years later. All have normal liver function. After monotherapy and alternate-day steroids were achieved, 66% of children < 5th percentile for height at the time of transplant improved to greater than the 5th percentile. There were 3 (11%) patients who rejected while on monotherapy an average of 1.15 years after it was started. These patients had the following predisposing factors that decreased cyclosporine levels and led to rejection: common bile duct stricture, chronic and intermittent antibiotic administration for urinary tract infection, and noncompliance. In the 9 potential candidates not tapered to monotherapy, 6 have had recurrent acute or chronic rejection; 2 of these now receive FK506. We conclude that the majority of stable pediatric LT recipients may be safely tapered to chronic cyclosporine monotherapy. Increased growth is a major benefit of decreased steroid dosing in these children. Cyclosporine absorption and adequate levels are crucial for success of this approach.

Absorption characteristics of a microemulsion formulation of cyclosporine in de novo pediatric liver transplant recipients.

In pediatric liver transplant recipients, oral cyclosporine (CsA) therapy may be complicated by impaired or delayed absorption during the initial weeks posttransplant. Neoral (NL) is a microemulsion preconcentrate formulation of CsA expected to increase the rate and extent of absorption of CsA and have less pharmacokinetic variability. The absolute bioavailability (F) of CsA from NL was compared with that of the currently marketed Sandimmune (SM) formulation in a double-blind, crossover study conducted in 9 pediatric liver transplant recipients (age 6 months to 11 years) between 8 and 20 days posttransplant. After determination of CsA pharmacokinetics for a steady-state intravenous dose, patients were randomized to receive a single oral dose of NL or SM in period I and the alternative formulation in period II. Clearance (Clt) and volume of distribution (Vss) values (mean +/- s.d.) calculated from the i.v. dose were similar to that previously reported for pediatric patients (Clt = 12.0 +/- 1.3 ml/min/kg; Vss = 2.2 +/- 0.2 L/kg). Mean F (+/- SD) for NL was significantly higher than SM (NL = 37.6 +/- 14.6%; SM = 24.7 +/- 8.0%; P = 0.05). Although not reaching statistical significance, the observed maximum blood concentration (Cmax) was higher, and the time to Cmax (Tmax) was shorter for NL in 8 or 9 patients. There were no significant correlations between age and any pharmacokinetic parameter for the group as a whole--however, there were statistically significant correlations between age and F for NL (r = 0.87; P = 0.02), and for age and Vss (r = 0.91; P = 0.01) for the 6 patients aged 2 years or less. In this pediatric liver transplant population, Neoral demonstrated improved absorption (% increase in F) compared to Sandimmune. In liver transplant recipients aged 2 years or less, absorption of Neoral may be a function of age and/or bowel length.

Calcineurin activity in children with renal transplants receiving cyclosporine.

BACKGROUND: The major immunosuppressive effect of cyclosporine is through the inhibition of calcineurin, an enzyme important in the activation of T lymphocytes. In children, neither calcineurin activity nor its inhibition by cyclosporine (CsA) has been investigated. METHODS: Calcineurin activity, was measured in stable pediatric renal transplant patients, with healthy children used as controls. Whole blood CsA concentrations were measured by monoclonal radioimmunoassay. Simultaneous calcineurin and CsA levels were measured before and 1, 2, 3.5, 5, and 12 hr after their routine morning CsA dose. RESULTS: Calcineurin activity was approximately 50% inhibited at trough blood concentrations (148 microg/L); moreover, inhibition increased as CsA concentrations rose and declined as concentrations fell. Maximum calcineurin inhibition was about 70% at concentrations of about 431 microg/L. Linear regression analysis revealed a significant correlation between mean CsA blood concentration and the mean degree of inhibition of calcineurin activity (P=0.005, one-tailed). CONCLUSION: We conclude that inhibition of calcineurin activity by CsA in pediatric renal transplant recipients correlates with CsA blood concentrations.

New findings in posterior amorphous corneal dystrophy.

Eight members of a family spanning five generations were found to have posterior amorphous corneal dystrophy. This rare abnormality was noted in both its centroperipheral and peripheral forms. The following five heretofore unrecognized features were noted: (1) hyperopia, (2) flattened corneal topography, (3) anterior iris surface and stromal abnormalities, (4) fine iris processes extending to Schwalbe's line for 360 degrees, and (5) extension of the opacity to the limbus. The presence of this dystrophy in a 6-month-old family member further suggests the possibility that it is a congenital abnormality.

Transsclerally sutured intraocular lenses in penetrating keratoplasty.

We reviewed the charts of 114 consecutive patients who underwent penetrating keratoplasty with transscleral fixation of a posterior chamber intraocular lens. Two patients died within three months of follow-up and were excluded from the study. In the remaining 112 patients, follow-up ranged from four to 47 months (mean, 17.2 months). Postoperative visual acuity improved in 95 patients (85%), remained the same in 13 patients (11.5%), and worsened in four patients (3.5%). In 71 patients with at least one year of follow-up, best-corrected visual acuity was 20/40 or better in 17 patients (24%), 20/50 to 20/80 in 25 patients (35%), 20/100 to 20/400 in 17 patients (24%), and counting fingers or worse in 12 patients (17%). Problems with lens decentration, tilt, dislocation, or scleral suture-related infections were minimal. Glaucoma and cystoid macular edema were the most common causes of decreased visual acuity. Four patients (3.6%) developed intraoperative choroidal detachments. Three patients (2.7%) developed rhegmatogenous retinal detachments early in the postoperative course.

Aspergillus keratitis after radial keratotomy.

PURPOSE/METHODS: A case of severe Aspergillus keratitis after radial keratotomy manifested as a discrete midstromal infiltrate along a radial incision. Despite aggressive treatment with topical amphotericin B and oral itraconazole, the patient required a therapeutic penetrating keratoplasty. RESULTS/CONCLUSIONS: Histopathologic examination disclosed a corneal ulcer with numerous septate, branching hyphae throughout the stroma, and marked stromal necrosis. Aspergillus species should be included among the microbial pathogens responsible for infectious keratitis after radial keratotomy. If infectious keratitis is suspected, comprehensive cultures for bacteria and fungi should be obtained.

Postkeratoplasty crystalline deposits mimicking bacterial infectious crystalline keratopathy.

Three corneal transplant patients developed white, crystalline, anterior stromal lesions in their grafts while receiving long-term topical corticosteroid therapy. A presumed diagnosis of infectious crystalline keratopathy was made in each case. However, histologic examination of the lesions failed to show gram-positive cocci. A fungal infiltrate was present in the first two patients, and calcium deposits produced the lesion in the third patient.

Necrotizing keratitis caused by Capnocytophaga ochracea.

We studied three cases of Capnocytophaga keratitis that demonstrated stromal necrosis and a ring infiltrate. In all cases, the keratitis occurred in a previously diseased or traumatized cornea. One patient was treated with chronic antiamoebic therapy for presumed Acanthamoeba keratitis. Two cases resulted in corneal perforation. Laboratory isolation was difficult because of slow, fastidious growth. Capnocytophaga is not uniformly sensitive to commonly used topical antibiotics such as the cephalosporins and aminoglycosides, but may respond to treatment with topical clindamycin.

Acanthamoeba keratitis associated with disposable contact lenses.

Two patients developed Acanthamoeba keratitis associated with the use of disposable extended-wear hydrogel contact lenses. Both patients removed, irrigated, and reinserted the contact lenses without disinfecting them. One patient wore the lenses on a daily basis, rinsed the lenses in tap water, stored them overnight, and discarded them weekly. Both infections were treated successfully. In a third patient, Acanthamoeba species was cultured from two pairs of disposable lenses that had been stored in cases rinsed with well water. Potential benefits from disposable contact lens wear are negated when patients do not comply with a continuous wearing schedule.

Effects of carbamazepine on cyclosporine metabolism in pediatric renal transplant recipients.

This study documents a pharmacokinetic interaction between carbamazepine and cyclosporine (CsA) in pediatric renal transplant recipients. Noncompartmental steady-state CsA pharmacokinetics were determined in three pediatric renal transplant recipients who were receiving both CsA and carbamazepine as long-term therapy (carbamazepine group) and in three matched renal transplant subjects who were not receiving carbamazepine (control group). Even though the mean daily dosage of CsA was consistently higher in the carbamazepine group than in the control group (16.2 mg/kg/24 hrs vs 10.8 mg/kg/24 hrs, respectively), the predose trough CsA blood concentrations were significantly lower in the carbamazepine group (57 ng/ml vs 162 ng/ml, respectively; p = 0.0023). Mean average steady-state blood concentrations of CsA (Cav) per mg of CsA administered were less than 50% in the carbamazepine group compared with the control group. This reflects either an induction of CsA hepatic metabolism or a reduced systemic bioavailability (possible induction of pre-hepatic metabolism) by concurrent use of carbamazepine.

Endophthalmitis after radial keratotomy enhancement.

A 45-year-old man developed endophthalmitis after a radial keratotomy (RK) enhancement. He developed severe intraocular inflammation, hypopyon, and dense vitreous membranes 4 days after the enhancement surgery. Cultures of the corneal wound yielded a heavy growth of Streptococcus viridans. The inflammation subsided after treatment with intraocular, intravenous, and topical antibiotics. The patient subsequently developed a cataract and retinal detachment. This case demonstrates the risk of endophthalmitis after RK enhancement.

Infectious keratitis after photorefractive keratectomy in a comanaged setting.

A 48-year-old man had simultaneous bilateral photorefractive keratectomy (PRK). The surgeon who performed the PRK did not see the patient in follow-up, and there was confusion regarding the comanaging doctor. Therefore, the patient was not examined immediately postoperatively. Several days later, he was hospitalized for an unrelated, painful orthopedic problem and heavily sedated. Seven days after the PRK, an ophthalmologist was consulted for ocular irritation and discharge. Examination showed bilateral, purulent conjunctivitis and severe infectious keratitis in the left eye. The patient was treated with periocular and topical antibiotics. Corneal cultures yielded Staphylococcus aureus. The keratitis resolved slowly, leaving the patient with hand motion visual acuity. A corneal transplant and cataract extraction was performed 15 months later, resulting in a best corrected visual acuity of 20/400 because of glaucomatous optic nerve damage. Severe infectious keratitis may occur after PRK. Poor communication between the surgeon, comanaging doctor, and patient may result in treatment delay.

Early- versus late-onset infectious keratitis after radial and astigmatic keratotomy: clinical spectrum in a referral practice.

PURPOSE: To compare the clinical characteristics of early- versus late-onset keratitis after radial keratotomy (RK) and astigmatic keratotomy (AK). SETTING: Referral subspecialty practice. METHODS: This retrospective review comprised 19 patients with infectious keratitis after RK and AK. Early- versus late-onset groups were analyzed for predisposing conditions; infiltrate location, size, and depth; microbiologic data; and final visual outcome. RESULTS: Ten patients in the early-onset group developed keratitis within a mean of 7.4 days after surgery (range 3 to 14 days). Nine patients in the late-onset group developed keratitis a mean of 5.4 years after surgery (range 1.5 to 15.0 years). Staphylococcus aureus was the predominant organism in the early-onset group and Pseudomonas aeruginosa in the late-onset group. In the early-onset group, most infiltrates occurred in the paracentral aspect of the RK incision and extended to the middle or posterior stroma. In the late-onset group, most infiltrates occurred in the peripheral portion of the RK incision and were localized to the superficial stroma. A hypopyon was present in 7 of 10 ulcers in the early group and in 1 of 9 in the late group. Two patients in the early group developed endophthalmitis. Most patients in the late-onset group had incisional pseudocysts; 2 had other risk factors for keratitis. Final visual acuity was 20/40 or better in 7 of 10 patients in the early group and in 8 of 9 patients in the late group. CONCLUSIONS: Early-onset corneal ulcers after incisional refractive keratotomy were usually paracentral and deep, whereas late-onset ulcers were usually peripheral and superficial. Despite the predominance of Staphylococcus and Pseudomonas in the early- and late-onset groups, respectively, a variety of organisms may be responsible for infections in keratotomy incisions.

Unusual causes of giant papillary conjunctivitis.

We report two unusual causes of giant papillary conjunctivitis (GPC). One patient developed GPC secondary to and elevated filtering bleb. Another patient developed focal GPC secondary to elevated calcific plaques (band keratopathy). The first patient's symptoms improved after treatment with sodium cromolyn drops. GPC resolved in the second patient after superficial keratectomy was performed. These cases represent two unusual causes of GPC and emphasize the role of mechanical trauma as a causative factor.

Corynebacterium striatus keratitis.

We report a case of diphtheroid keratitis that occurred in a compromised cornea. Corneal cultures yielded heavy growth of Corynebacterium striatus on blood and chocolate agar. The infection responded slowly to treatment with topical fortified cefazolin and fortified tobramycin. This case demonstrates that diphtheroids are capable of causing ocular infections and should not routinely be dismissed as contaminants or commensals.

Endogenous Listeria monocytogenes endophthalmitis presenting as keratouveitis.

We report a case of Listeria monocytogenes endophthalmitis that presented as a recalcitrant keratouveitis in a nonimmunocompromized patient. L. monocytogenes was recovered from the patient's aqueous, vitreous, and two of three blood cultures. He was treated with topical, subconjunctival, and systemic antibiotics, but the visual outcome was poor, possibly because of late diagnosis. A review of previous cases of L. monocytogenes endophthalmitis demonstrates unique features of this infection: (a) presentation as an anterior uveitis or keratouveitis with elevated intraocular pressure, (b) endogenous origin, and (c) frequent occurrence in nonimmunocompromized patients.

Value of plasmapheresis in hepatic encephalopathy.

Plasmapheresis is used for treating the complications of liver failure. We performed plasmapheresis on 6 children with hepatic encephalopathy resulting from acute hepatic failure and prospectively assessed its effects on neurologic and electrophysiologic (electroencephalography and evoked potentials) function. Clinical improvement was observed in 3 of 6 patients; changes in the serum ammonia value or the results of initial electrophysiologic tests did not predict the patient response. Two patients underwent transplantation after neurologic improvement was produced by plasmapheresis; however, despite plasmapheresis, 4 patients progressed to brain death. Our data demonstrate that plasmapheresis may transiently improve the encephalopathy of acute hepatic failure but is not curative alone. Therefore, plasmapheresis may be a useful adjunct in the treatment of liver failure, potentially improving the pretransplantation status of the patient.

Pediatric liver transplantation without red blood cell transfusion.

The authors report the first case of orthotopic liver transplantation in a pediatric patient in whom packed red blood cell transfusion was not required. This has obvious advantages in avoiding the infectious complications of transfusion. Our patient required 3 U of fresh frozen plasma for treatment of preexisting coagulopathy. However, it is possible that, with future advances in transfusion therapy, synthetic coagulation factors may be provided in which there is no risk of infectious complications, and therefore a truly "bloodless" orthotopic liver transplant can be performed.