Implementing a Health Utility Assessment Platform to Acquire Health Utilities in a Hemodialysis Outpatient Setting: Feasibility Study.

BACKGROUND: Patients with end-stage kidney disease (ESKD) wait roughly 4 years for a kidney transplant. A potential way to reduce wait times is using hepatitis C virus (HCV)-viremic kidneys. OBJECTIVE: As preparation for developing a shared decision-making tool to assist patients with ESKD with the decision to accept an HCV-viremic kidney transplant, our initial goal was to assess the feasibility of using The Gambler II, a health utility assessment tool, in an ambulatory dialysis clinic setting. Our secondary goals were to collect health utilities for patients with ESKD and to explore whether the use of race-matched versus race-mismatched exemplars impacted the knowledge gained during the assessment process. METHODS: We used The Gambler II to elicit utilities for the following ESKD-related health states: hemodialysis, kidney transplant with HCV-unexposed kidney, and transplantation with HCV-viremic kidney. We created race exemplar video clips describing these health states and randomly assigned patients into the race-matched or race-mismatched video arms. We obtained utilities for these 3 health states from each patient, and we evaluated knowledge about ESKD and HCV-associated health conditions with pre- and postintervention knowledge assessments. RESULTS: A total of 63 patients with hemodialysis from 4 outpatient Dialysis Center Inc sites completed the study. Mean adjusted standard gamble utilities for hemodialysis, transplant with HCV-unexposed kidney, and transplantation with HCV-viremic kidney were 82.5, 89, and 75.5, respectively. General group knowledge assessment scores improved by 10 points (P<.05) following utility assessment process. The use of race-matched exemplars had little effect on the results of the knowledge assessment of patients. CONCLUSIONS: Using The Gambler II to collect utilities for patients with ESKD in an ambulatory dialysis clinic setting proved feasible. In addition, educational information about health states provided as part of the utility assessment process tool improved patients' knowledge and understanding about ESKD-related health states and implications of organ transplantation with HCV-viremic kidneys. A wide variation in patient health state utilities reinforces the importance of incorporating patients' preferences into decisions regarding use of HCV-viremic kidneys for transplantation.

An interpretation of the neonatal period definition obtained with echocardiographic examination by using change point regression analysis.

BACKGROUND: We had reported on the left ventricular end-diastolic dimension (LVDd) in normal children from the premature/neonatal period to the adolescence period by using two-dimensional echocardiography, and formulated equations to evaluate normal LVDd values by using body height as an index. There was an inflection point at around birth that seemed relevant to the fetal and neonatal periods for the relation of LVDd and body height. METHODS: We aimed to reveal the true inflection point and its meaning by using change point regression analysis. The study group consisted of 421 neonates and infants. The ages at examination ranged from 24 weeks' gestation to 1 year after birth. The subjects' body heights at examination were between 31 and 75 cm. RESULTS: The analysis showed no definite inflection point in height, and a flat bottom was observed between body heights of 48 and 55 cm. The inflection range seemed to mean the duration of the neonatal period, which connects the fetal and infantile periods. CONCLUSION: The results revealed that neonates reach the infantile period slower than usually imagined, and the end of the neonatal period may be at the age when the body height is around 55 cm- in other words, at 2 months after birth. This manuscript might be the first one to consider the definition of the neonatal period using cardiovascular methods.

Costimulatory Effects of an Immunodominant Parasite Antigen Paradoxically Prevent Induction of Optimal CD8 T Cell Protective Immunity.

Trypanosoma cruzi infection is controlled but not eliminated by host immunity. The T. cruzi trans-sialidase (TS) gene superfamily encodes immunodominant protective antigens, but expression of altered peptide ligands by different TS genes has been hypothesized to promote immunoevasion. We molecularly defined TS epitopes to determine their importance for protection versus parasite persistence. Peptide-pulsed dendritic cell vaccination experiments demonstrated that one pair of immunodominant CD4+ and CD8+ TS peptides alone can induce protective immunity (100% survival post-lethal parasite challenge). TS DNA vaccines have been shown by us (and others) to protect BALB/c mice against T. cruzi challenge. We generated a new TS vaccine in which the immunodominant TS CD8+ epitope MHC anchoring positions were mutated, rendering the mutant TS vaccine incapable of inducing immunity to the immunodominant CD8 epitope. Immunization of mice with wild type (WT) and mutant TS vaccines demonstrated that vaccines encoding enzymatically active protein and the immunodominant CD8+ T cell epitope enhance subdominant pathogen-specific CD8+ T cell responses. More specifically, CD8+ T cells from WT TS DNA vaccinated mice were responsive to 14 predicted CD8+ TS epitopes, while T cells from mutant TS DNA vaccinated mice were responsive to just one of these 14 predicted TS epitopes. Molecular and structural biology studies revealed that this novel costimulatory mechanism involves CD45 signaling triggered by enzymatically active TS. This enhancing effect on subdominant T cells negatively regulates protective immunity. Using peptide-pulsed DC vaccination experiments, we have shown that vaccines inducing both immunodominant and subdominant epitope responses were significantly less protective than vaccines inducing only immunodominant-specific responses. These results have important implications for T. cruzi vaccine development. Of broader significance, we demonstrate that increasing breadth of T cell epitope responses induced by vaccination is not always advantageous for host immunity.

Echocardiographic measurements of cardiac dimensions correlate better with body length than with body weight or body surface area.

Looking after children means caring for very small infants up to adult-sized adolescents, with weights ranging from 500 g to more than 100 kg and heights ranging from 25 to more than 200 cm. The available echocardiographic reference data were drawn from a small sample, which did not include preterm infants. Most authors have used body weight or body surface area to predict left ventricular dimensions. The current authors had the impression that body length would be a better surrogate parameter than body weight or body surface area. They analyzed their echocardiographic database retrospectively. The analysis included all available echocardiographic data from 6 June 2001 to 15 December 2011 from their echocardiographic database. The authors included 12,086 of 26,325 subjects documented as patients with normal hearts in their analysis by the examining the pediatric cardiologist. For their analysis, they selected body weight, length, age, and aortic and pulmonary valve diameter in two-dimensional echocardiography and left ventricular dimension in M-mode. They found good correlation between echocardiographic dimensions and body surface area, body weight, and body length. The analysis showed a complex relationship between echocardiographic measurements and body weight and body surface area, whereas body length showed a linear relationship. This makes prediction of echo parameters more reliable. According to this retrospective analysis, body length is a better parameter for evaluating echocardiographic measurements than body weight or body surface area and should therefore be used in daily practice.

[Pulsatile tumor on the left side of the thorax after heart surgery]. / Pulsierender Tumor auf der linken Thoraxseite nach Herzoperation.

HISTORY AND ADMISSION FINDINGS: In a 67-year-old woman a large haematoma developed on the left side of the chest after two sternotomies because of an aortic valve and aorta ascendens replacement for aortic type A dissection. After a few weeks, a pulsating mass remained over the 6th left parasternal intercostal space. A consultant general surgeon punctured the lesion and aspirated bright red blood. The patient was transferred for further diagnostic procedures to the cardiology department. INVESTIGATIONS: Ultrasound examination of the pulsating mass showed a 35 mm echo-free cavity with turbulent flow in the color Doppler. To clarify the inflow into this perfused cavity magnetic resonance imaging (MRI) with angiography of the thorax vessels was performed. The cavity was found to be a perfused pseudoaneurysm originating from the native left mammary artery. The aneurysm had a 6 mm long neck and a diameter of 35 mm. An interventional closure was planned. TREATMENT AND COURSE: With access via the right groin a 6 French LIMA guiding catheter was inserted into the origin of the left internal mammary artery. With a 0.014 inch Balance Middleweight coronary guide wire it was possible to engage the pseudoaneurysm. Over the wire, a tracker 18 infusion catheter was pushed down and placed in the pseudoaneurysm. The closure was performed by insertion of a 0.018 inch 3 cm/4 mm Hilal Microcoil into the aneurysm neck. The mammary artery was anatomically and functionally unaffected. After the deposit of a coil, the inflow was stopped. An ultrasound showed a blood coagulation in the pseudoaneurysm, which decreased in size over time. CONCLUSION: After sternotomy a pseudoaneurysm of the left internal mammary artery had developed. As a noninvasive imaging modalitiy the magnetic resonance angiography showed well the exact flow into the aneurysm and allowed the planning of the intervention. The closure was achieved with a microcoil with preservation of the native internal mammary artery.

Treatment of gastroesophageal reflux with nissen fundoplication and gastrostomy tube insertion in infantile pompe's disease.

In infantile Pompe's disease, enzyme replacement therapy (ERT) has been shown to reverse cardiomyopathy, improve skeletal muscle strength, and prolong survival. We report on five patients in whom complications related to gastroesophageal reflux (GER) resulted in deterioration of their clinical status despite initial improvement under ERT. Surgical antireflux therapy, performed in four, yielded positive results in two. Three patients experienced severe aspirations related to GER and underwent fundoplication and gastrostomy subsequently. Two did not regain former motor functions and deceased shortly thereafter, while one slowly recuperated and is in a stable state at age 53 months. In a further patient, severe GER prompted fundoplication at age 17 months. No aspirations occurred until the girl deceased probably due to cardiac arrest 20 months later. These cases suggest that infants with Pompe's disease under ERT may benefit from timely performed fundoplication and gastric tube placement.

[Blood pressure difference between upper arm and thigh, and aortic stiffness in healthy subjects and in patients after coarcectomy]. / Blutdruckdifferenz zwischen Oberarm und Oberschenkel und Aortensteifheit bei Herzgesunden und nach korrigierter Aortenisthmusstenose.

BACKGROUND: The blood pressure difference between the right arm and the legs is often used as an estimate of a possible gradient across a coarctation or recoarctation aortae. We wanted to test the reliability of this hypotheses while estimating the local stiffness of the aortae ascendens and abdominalis. PATIENTS: We examined 50 healthy children and adolescents as well as 50 patients of a similar age after repair of an coarctation aortae. There was no relevant recoarctation on echocardiography or magnet resonance tomography. METHODS: We measured in all patients the blood pressure by oscillometry three times on the right upper arm and thigh. At the same time we measured the systolic and diastolic diameter of the aorta before the branching of the truncus brachiocephalicus and the branching of the truncus coeliacus. The local stiffness was calculated, using the stiffness index b, from the aortic diameter and the corresponding blood pressure. RESULTS: The systolic blood pressure difference showed in healthy subjects and patients after coarctation a wide range (about 60 mm Hg). The diastolic and mean blood pressure showed a slightly smaller range. There was no significant difference in this respect between the two groups. The stiffness index beta was elevated after coarcectomy in the aorta ascendens compared to healthy subjects. The local stiffness of the abdominal aortae were similar in both groups and showed a similar increase with advancing age. DISCUSSION: The blood pressure difference between the upper arm and thigh showed a wide range. Therefore is the blood pressure difference an unreliable tool to estimate the severity of a re-coarctation. The local stiffness of the aorta ascendens was elevated after coarctation and implied at least a partial loss of the Windkessel. The local stiffness in the aorta abdominalis was normal after coarctation repair.

Dependence on a respiratory ventilator due to an atrial septal defect.

An atrial septal defect is commonly thought of as a benign cardiac lesion especially in infancy. The haemodynamic consequences for the lungs, nonetheless, can be comparable to that produced by patency of the arterial duct. In a preterm boy, this lesion led to the development of broncho-pulmonary dysplasia, and dependence on a respiratory ventilator. He could be extubated shortly after surgical closure of the septal defect.

Reduced frequency of occlusion of aorto-pulmonary shunts in infants receiving aspirin.

OBJECTIVE: Infants with severely reduced pulmonary perfusion due to complex congenital cardiac malformations are in need of an improved flow of blood to the lungs. One option for treatment is to construct a systemic-to-pulmonary arterial shunt. Although such shunts have been used since 1945, their spontaneous occlusion remains a major problem in the long-term. DESIGN: We studied all infants in whom a systemic-to-pulmonary arterial shunt had been constructed using a Gore-Tex tube graft between December 1989 and March 1996. PATIENTS: Of 46 infants undergoing construction of a shunt, 7 (15%) died within 30 days of surgery. The shunts had to be taken down in 2 infants. Thus, 37 infants were included in the study. All but three infants received Aspirin. Aspirin was discontinued on the personal decision of individual physicians. Of 22 infants, 3 never received Aspirin, and in 19 it was stopped well before undertaking subsequent surgery. Aspirin was administered continuously to 15 infants until further surgery. RESULTS: Those in whom Aspirin was discontinued, or not given, and those receiving Aspirin until further surgery, were comparable concerning their age, time of follow-up, severity of the cardiac lesions, and size and type of shunt. Partial or complete occlusion of the shunt occurred in 2 of 15 (13%) infants taking Aspirin, but was seen in 12 of 22 (54%) infants in whom Aspirin was discontinued. Of these, 3 died due to acute occlusion of the shunt. CONCLUSIONS: Aspirin reduced effectively the rate of occlusion of systemic-to-pulmonary arterial shunts, and should be continued as long as the shunt is in place.

Sarcoidosis associated with interferon-alpha therapy for chronic hepatitis C.

BACKGROUND/AIMS: Pulmonary side effects of interferon-alpha therapy of chronic hepatitis C seem to be rare. So far, only two cases of sarcoidosis in association with interferon-alpha treatment of chronic hepatitis C have been described. METHODS/CASES: We report on three patients who were treated with recombinant interferon-alpha2a for chronic hepatitis C, two of them in combination with ribavirin. These patients developed pulmonary sarcoidosis 12, 20 and 21 weeks, respectively, after beginning interferon therapy, one patient with Löfgren's syndrome. In one patient sarcoidosis emerged only after discontinuation of interferon therapy because of treatment failure. Clinical symptoms of sarcoidosis in the three patients were suggestive of side effects of interferon-alpha. Interferon therapy was discontinued and spontaneous remission was observed in all three cases 5, 6, and 8 months, respectively, after the onset of symptoms. CONCLUSION: The occurrence of sarcoidosis in association with interferon-alpha therapy for chronic hepatitis C may have been underestimated so far. This could be due to the fact that symptoms of sarcoidosis and common side effects of interferon are similar, and sarcoidosis may occur after the end of interferon therapy. We hypothesize that interferon-alpha as a potent stimulator for T-helper 1 (Th1) immune responses may trigger the compartmentalized Th1 reaction that has been shown to take place in sarcoidosis.

[Arterial hypertension and blood pressure profile in patients with Williams-Beuren syndrome]. / Arterielle Hypertension und Blutdruckprofil bei Patienten mit Williams-Beuren-Syndrom.

The prevalence of hypertension and the diurnal blood pressure pattern were investigated in patients with Williams-Beuren syndrome (WBS) by blood pressure measurements in 142 children, adolescents, and young adults (female n = 62, male n = 80; median age 6.5 years (0.1-34.3 years)) and evaluation of ambulatory blood pressure data from 45 patients (female n = 21, male n = 24; median age 7.8 years (1-23.8 years)). Measurements revealed systolic hypertension in 46.5% of 142 patients, diastolic hypertension occurred in 36.6% (i.e. actual pressure > 95 percentile). According to the ambulatory data 42.2% of 45 patients had hypertension (mean arterial pressure > normal + 2SD). The nocturnal decline of the blood pressure was normal in hypertensive patients but reduced in normotensives (p < 0.01 vs normals). Males were more often hypertensive than females (46% vs 38%). Hypertensives had a higher body mass index than normotensives (19.5 vs 16.6 kg/m2, p < 0.05). In normo- and hypertensive WBS patients mean heart rates were elevated during day- and nighttime (p < 0.02 vs normals) the latter due to a reduced nocturnal decline. The prevalence of hypertension in WBS patients amounts to about 40%, thus being four- to eight-fold in comparison to healthy young adults or children. The diurnal blood pressure pattern and the elevated heart rates indicate that an increased arterial stiffness due to the vascular disease in the WBS and augmented sympathetic activity might play a role in the genesis of hypertension. Thus, effective antihypertensive treatment is likely to become difficult. From our experience beta-blocking agents are often successful in hypertensive WBS patients.