Health state utilities associated with major clinical events in the context of secondary hyperparathyroidism and chronic kidney disease requiring dialysis.

BACKGROUND: Patients with chronic kidney disease (CKD) and secondary hyperparathyroidism (SHPT) who require dialysis are at increased risk for cardiovascular events and bone fractures. To assist in economic evaluations, this study aimed to estimate the disutility of these events beyond the impact of CKD and SHPT. METHODS: A basic one-year health state was developed describing CKD and SHPT requiring dialysis. Further health states added acute events (cardiovascular events, fractures, and surgical procedures) or chronic post-event effects. Acute health states described a year including an event, and chronic health states described a year subsequent to an event. General population participants in Canada completed time trade-off interviews from which utilities were derived. Pairwise comparisons were made between the basic state and event, and between comparable health states. RESULTS: A total of 199 participants (54.8% female; mean age = 46.3 years) completed interviews. Each health state had ≥130 valuations. The mean (SD) utility of the basic health state was 0.60 (0.34). For acute events, mean utility differences versus the basic state were: myocardial infarction, -0.06; unstable angina, -0.05; peripheral vascular disease (PVD) with amputation, -0.33; PVD without amputation, -0.11; heart failure, -0.14; stroke, -0.30; hip fracture, -0.14; arm fracture, -0.04; parathyroidectomy, +0.02; kidney transplant, +0.06. Disutilities for chronic health states were: stable angina, -0.09; stroke, -0.27; PVD with amputation, -0.30; PVD without amputation, -0.12; heart failure, -0.14. CONCLUSIONS: Cardiovascular events and fractures were associated with lower utility scores, suggesting a perceived decrease in quality of life beyond the impact of CKD and SHPT.

A study of the association of higher parathormone levels with health-related quality of life in hemodialysis patients.

Secondary hyperparathyroidism (SHPT) is associated with poor outcome including mortality, hospitalization, as well as greater healthcare resource utilization and costs in chronic kidney disease (CKD). We hypothesized that SHPT is also associated with poor self reported health-related quality of life (HRQOL) in prevalent hemodialysis (HD) patients. We conducted a case-control study in patients with CKD receiving longterm HD treatment, in six dialysis clinics in Greece. HRQOL was estimated with the KDQOL-SFTM questionnaire, version 1.3, which includes 43 kidney disease targeted items, and 36 items that provide a generic core and an overall health rating item, with a higher score reflecting a more favorable health state. A total of 156 completed the questionnaire, 50 with high parathormone levels (i.e., PTH > 300 pg/ml and or under vitamin D receptor activators, mean: 329 ± 160.9 pg/ml) and 106 with low parathormone levels (PTH < 300 pg/ml, mean: 132.4 ± 69.0 pg/ml) in a 2 : 1 randomization assignment. Patients with high and with low PTH were 62.1 ± 14.9 and 65.9 ± 14.2 y old and the median dialysis vintage time was 31 and 37 months, respectively. There were no significant differences regarding the presence of comorbidities between groups. Patients with high PTH, compared to patients with low PTH, had lower pain component summary (57.6 ± 33.5 vs. 69.2 ± 28.9; p = 0.041) and physical component summary (41.0 ± 23.8 vs. 50.0 ± 20.8; p = 0.031). Both pain component summary and physical component summary differences remained significant after adjustment for age, gender and vintage (p = 0.036 and p = 0.029, respectively). Low PTH patients scored better in 18 out of 23 subscales. In HD patients, SHPT appears to be associated with worse pain component summary score (p = 0.036) and physical component summary score (p = 0.029). Additional studies are needed to verify these associations and to examine whether correction of SHPT can improve HRQOL.

Psychosis-induced vitamin D deficiency with secondary hyperparathyroidism and osteoporotic fractures.

Vitamin D deficiency is common in patients with insufficient sunlight exposure and nutritional deficit in calcium and vitamin D. We present a patient with vitamin D deficiency, secondary hyperparathyroidism and resulting osteoporotic fractures caused or supported by psychosis-induced minimal sunlight exposure and nutritional deficits. To our knowledge, this is the first case where delusional ideas could have led to a vitamin D deficiency, secondary hyperparathyroidism and osteoporotic fractures. Similar clinical cases could be more often, but significantly underdiagnosed, among schizophrenia patients. An appropriate prevention by UV light exposure, food fortification and supplements is normally sufficient for patients at risk.

Is survival enough for quality of life in Sagliker Syndrome-uglifying human face appearances in chronic kidney disease?

BACKGROUND: It is known that secondary hyperparathyroidism (SH) and particularly skeletal changes is a severe condition in chronic kidney disease (CKD). Sagliker syndrome (SS) is a very prominent feature in CKD including uglifying human face appearances, short stature, extremely severe maxillary and mandibulary changes, soft tissues in the mouth, teeth-dental abnormalities, finger tip changes and severe psychological problems. METHODS: In the last 8 years we have confronted 36 extremely incredible SS cases in CKD by performing an international study in Turkey, India, Malaysia, Romania and Egypt. RESULTS: In addition to the uglifying human face appearance, we found extremely severe X-ray and tomographical, pantomographical, histo-pathological changes in the head and whole body. Finally, we compared previous face pictures with recent ones. Just a few years earlier they had been pretty and good-looking young boys and girls. By investigating their history, we understood they had not received proper therapy and were in the late-irreversible period. CONCLUSION: SS is a serious and severe complication of CKD. Late and improper treatment leads to abnormalities throughout skeleton particularly in the skull and face. Changes particularly in children and teens become irreversible-disastrous for appearance and psychological health. Appropriate treatment must begin as early as possible in specialized centers. It is possible that SS patients may survive long-term with dialysis, but with all those particular changes could anyone claim this type of life would continue in an acceptable way without extending their height, correcting all the changes in the skull and face, remodeling new faces and most particularly convincing the patients to deal with all those tragi-dramatic psychological problems?

International study on Sagliker syndrome and uglifying human face appearance in severe and late secondary hyperparathyroidism in chronic kidney disease patients.

OBJECTIVE: It is known that skeletal changes due to secondary hyperparathyroidism (SH) can be severe in chronic kidney disease (CKD). Recently described Sagliker syndrome (SS) is a very striking and prominent feature of SH in CKD, including an uglifying appearance to the face, short stature, extremely severe maxillary and mandibulary changes, soft tissue in the mouth, teeth/dental abnormalities, fingertip changes, knee and scapula deformities, hearing abnormalities, and neurological and, more important, severe psychological problems. DESIGN, SETTING, PATIENTS: In the past 8 years, we have encountered 40 cases of SS in SH and CKD by performing an international study in Turkey, India, Romania, Egypt, Maleysia, Tunis, and China. RESULTS: The medical history of these patients showed that they did not receive proper therapy. Changes, particularly in children and teenagers, become irreversible, which was disastrous for the patients both aesthetically and psychologically. CONCLUSION: Treatment must begin early and be the appropriate treatment given in centers with sophisticated skills. Otherwise, the inability to correct all the changes in the skull and face, to remodel a new face, to extending the height, and, most important, to convince the patients to face the dramatic psychological problems can be catastrophic for those patients.

Neuropsychological function in relation to serum parathyroid hormone and serum 25-hydroxyvitamin D levels. The Tromsø study.

There are receptors for parathyroid hormone (PTH) and 1,25-dihydroxyvitamin D in the brain, and there are clinical and experimental data indicating that PTH and vitamin D may affect cerebral function. In the present study 21 subjects who both in the 5th Tromsø study and at a follow-up examination fulfilled criteria for secondary hyperparathyroidism (SHPT) without renal failure (serum calcium < 2.40 mmol/L, serum PTH > 6.4 pmol/L, and normal serum creatinine) and 63 control subjects were compared with tests for cognitive and emotional function. Those in the SHPT group had significantly impaired performance in 3 of 14 cognitive tests (Digit span forward, Stroop test part 1 and 2, and Word association test (FAS)) as compared with the controls, and also had a significantly higher depression score at the Beck Depression Inventory (BDI) (items 1-13). In a multiple linear regression model, a high serum PTH level was significantly associated with low performance at the Digit span forward, Stroop test part 1 and 2, and Digit Symbol tests. A low level of serum 25-hydroxyvitamin D was significantly associated with a high depression score. In conclusion, a deranged calcium metabolism appears to be associated with impaired function in several tests of neuropsychological function.

Cognitive function in dialysis patients.

There are few detailed studies of cognitive function in dialysis patients. However, appreciating the prevalence and risk factors for cognitive impairment is important because cognitive impairment may decrease an individual's quality of life, increase resource utilization, and result in suboptimal medical care because of difficulty following caregiver recommendations. Cognitive impairment also is likely to become more of a problem as the dialysis population ages. In this review, we argue that cerebrovascular disease is an important cause of cognitive impairment in dialysis patients and discuss risk factors specific for vascular disease, as well as other factors that may influence cognitive function. We describe the structural brain abnormalities frequently seen in dialysis patients and the specific neurocognitive changes noted in prior studies. We explore potential measures to reduce cognitive impairment in this population. We conclude that additional research is needed in this area.

A prospective surgical outcome study assessing the impact of parathyroidectomy on symptoms in patients with secondary and tertiary hyperparathyroidism.

BACKGROUND: To assess the impact of surgery on preoperative symptoms in secondary (2 degrees ) and tertiary (3 degrees ) hyperparathyroidism (HPT) compared with primary (1 degrees ) HPT. METHODS: Twenty-two patients with 2 degrees HPT and 10 with 3 degrees HPT were enrolled. Age-matched patients, 32 with 1 degrees HPT and 32 with thyroid disease were enrolled for comparison. An outcome questionnaire documented symptoms expressed as the median symptom index score (MSIS) preoperatively and at days 7 and 3 and 12 months postoperatively. RESULTS: Preoperatively, the MSIS for the groups with 3 degrees, 2 degrees, and 1 degrees HPT and thyroid disease was 225, 572, 372, and 146, indicating that patients with HPT were more symptomatic than those in the thyroid group (P<.05). Patients with 1 degrees HPT had a decrease in their MSIS at day 7 (195, P<.05) and at 3 and 12 months (159 and 156). Patients with 3 degrees HPT also had a decrease in their MSIS over time. Patients with 2 degrees HPT had a decrease in their MSIS at day 7 (469, P<.05); however, they remained more symptomatic at 3 and 12 months (410 and 355). CONCLUSIONS: Parathyroidectomy reduces many of the preoperative symptoms in HPT. Patients with 1 degrees and 3 degrees HPT have a similar resolution of their symptoms. Patients with 2 degrees HPT have an improvement in many of their symptoms, although they remain more symptomatic at 1 year.