HLA haplotype in a patient with systemic lupus erythematosus triggered by hepatitis B vaccine.

AIM: Find an association between hepatitis B vaccine-related systemic lupus erythematosus and HLA. MATERIAL: A 27-year-old woman who developed a lupus nephritis after the administration of hepatitis B vaccine. METHODS: We studied HLA antigen expression on lymphocytes and genomic haplotype. Class I-II HLA antigen typing was performed by the microlymphocytotoxicity test with the standard NIH method, and Class I-II HLA allele typing by polymerase chain reaction, using single-strand oligonucleotide dot-blot kits. RESULTS: The serological haplotype was HLA A24/25, B18 (Bw6)/-, C-/-, DQ7/-, DR11(5)/52. The genomic haplotype was A*2403/2504, B*1825/1825, C*1207/ 1207, DRB1*1102/1132, DRB3*0202/0202, DQA1*0505/0505, DQB1*0301/0301. Then we sought for analogies with haplotypes known to be related to other systemic AID. Since we have found HLA alleles typical both of systemic lupus erythematosus and Sjogren's syndrome, the persistence of ENA-SSA positivity was highly suspicious for a possible overlap syndrome. CONCLUSIONS: Hepatitis B vaccine can potentially trigger both the onset or the exacerbations of several autoimmune disorders, including systemic lupus erythematosus, by reduced immune complex clearance or molecular mimicry. This study represents the first report on the association between hepatitis B vaccine related systemic lupus erythematosus and HLA. Probably autoimmune reactions triggered by vaccines occur only in predisposed subjects, in which antigen presentation influenced by HLA haplotypes leads to the autoimmune cascade. More studies are needed to corroborate our hypothesis. They could disclose new pathways in the field of prevention.

Aerobic exercise and non-alcoholic fatty liver disease: a case report.

Non-alcoholic steatosis (non-alcoholic fatty liver disease [NAFLD]), now considered a metabolic pathway to advanced liver disease, cirrhosis and hepatocellular carcinoma, can also be explained by physical inactivity and increased dietary fat intake. No established treatment exists for this potentially serious disorder. The authors present the case of a 29-year-old man with NALFD who followed a restricted diet and practiced aerobic exercise for 16 weeks. Outcome after a combination therapy of aerobic exercise and diet was good, suggesting that treatment with a restricted diet and physical exercise can improve blood biochemical values in patients with NAFLD. Moderate-intensity aerobic exercise may help to normalize liver enzyme values and the quality of life of patients with fatty liver diseases.

[Management of hyperphosphatemia in patients with chronic kidney disease]. / Il controllo della fosforemia nell'insufficienza renale cronica.

Hyperphosphatemia is a common finding in patients with chronic kidney disease (CKD) undergoing hemodialysis or receiving conservative treatment. Recent papers have reported a link between hyperphosphatemia, soft tissue calcifications, and cardiovascular events responsible for high morbidity and mortality in these patients. Our group identified in salivary phosphate secretion a method to study the phosphate balance in CKD. Moreover, we found that CKD patients on hemodialysis usually drink beverages with a high phosphate content that may increase their serum phosphorus levels. Hyperphosphatemia is currently treated with diet, phosphate-binding drugs, and drugs acting on bone metabolism. Despite such treatment, only half of the patients with end-stage renal disease fall within the K/DOQI guidelines range for serum phosphorus levels. This paper reports positive results obtained with the use of the polymer chitosan as a phosphatebinding chewing gum in CKD patients undergoing periodic hemodialysis.

[Nursing and quality of life in CKD]. / L'infermiere e la qualita' della vita nella CKD.

Quality of life is one of the main targets of modern medicine. This applies specifically to people suffering from chronic diseases, who are frequently hospitalized and receive continuing care for their irreversible condition. Among chronic diseases, end-stage renal disease is extremely interesting because it can be assessed transversely in different settings but also longitudinally over time, thus allowing adequate evaluation of quality of life.

Defense mechanisms in hemodialysis-dependent patients.

Defense mechanisms are automatic psychological processes that protect the individual against anxiety and from the awareness of internal or external dangers or stressors. The influence of defense mechanisms in patients on chronic hemodialysis treatment was studied. There were 53 uremic subjects (37 males and 16 females), aged between 22 and 88 years (mean age 60.11, SD 15.03), on chronic dialysis and 50 healthy subjects as controls have been enrolled in the study. According to the duration of dialysis, uremic patients were divided in two subgroups: 21 patients with less than 5 years and 19 patients with more than 10 years of dialytic treatment. Assessment was conducted using the Defense Mechanisms Inventory DMI. The inventory identifies five defensive styles: turning against the object (TAO), projection (PRO), principalization (PRN), turning against the self (TAS) and reversal (REV). Results showed DMI scores within the normal range both for uremics and controls with significant differences in TAO (t = -3.053, p = 0.003) and REV (t = 5.067, p < 0.0001) between groups. No significant differences in the use of defensive styles related to the duration of dialytic treatment were observed. Besides other psychological features, the assessment of defense mechanisms in patients with chronic and invalidating diseases may contribute to ameliorate the knowledge of the adjustment processes and of the psychological well-being of the patients.

[Evolution of the classification of acute and chronic transplant rejection]. / Evoluzione della classificazione del rigetto acuto e cronico del trapianto.

In the last twenty years, several systems have been proposed to codify renal allograft rejection. The Banff classification for kidney allograft pathology, introduced in 1993, started a new era in the standardization of criteria for rejection and for allowing uniform reporting. This consensus on allograft grading proposed a scheme to guide therapy in transplant patients and to help establish an objective rejection end point in clinical trials. This scheme, modified during 1993-1997 to address many of the criticisms, was substantially improved. Another important system of classification of allograft renal biopsies was the Cooperative Clinical Trials in Transplantation (CCTT) classification which was published in 1997. The aim of this system was to develop a schema that would be practical to implement, easy to describe to unfamiliar personnel, reproducible, with high rates of sensitivity and specificity and clinically informative (predictive of course and/or response to therapy). In March 1997, a fundamental revision of the Banff classification for acute rejection was achieved by a consensus conference for incorporating many of the strengths of the CCTT system. Some of these were the importance of vascular damage (endoarteritis, endothelial activation, fibrinoid necrosis) and interstitial hemorrhage, but not the interstitial infiltrate or tubulitis, which correlated with response to anti-rejection therapy and/or 1 year clinical outcome. The most recent modification concerns the addition of C4d-positive acute humoral rejection and the emphasis on differences between cell-mediated and antibody-mediated rejections. Future refinements of these classifications and findings of new molecular markers of allograft rejection, such as fas-ligand or granzyme-B, will help to improve diagnosis and therapy in renal transplant patients.

Correlation between increased serum and tissue L-carnitine levels and improved muscle symptoms in hemodialyzed patients.

A group of 14 uremic patients on intermittent hemodialysis was admitted to a cross-over double-blind trial in order to evaluate serum and muscle carnitine levels before and after 60 days of L-carnitine oral (2 g/day) treatment. The morphology of muscle fragments was studied by light and electron microscopy. Symptoms (asthenia, cramps) occurring during hemodialysis were also recorded. After L-carnitine treatment the blood and muscle levels of the metabolite increased simultaneously to reduced asthenia and cramps. Morphological examination of the muscle of 13 of 14 patients did not reveal any pre- or posttreatment pathological alterations. The presence of nemaline rods was morphologically diagnosed in only one case and was no longer observed at the second biopsy at 2 months of L-carnitine treatment.

Erectile dysfunction in uremic dialysis patients: diagnostic evaluation in the sildenafil era.

The two words that mean sexual dysfunction, impotence and erectile dysfunction (ED), express two different concepts. Impotence is a general male sexual dysfunction that includes libidinal, orgasmic, and ejaculatory dysfunction. ED is the inability to achieve or maintain an erection sufficient to allow satisfactory sexual intercourse and is part of the general male sexual dysfunction termed impotence that includes libidinal, orgasmic, and ejaculatory dysfunction. Uremic men of different ages report a variety of sexual problems, including sexual hormonal pattern alterations, reduction in or loss of libido, infertility, and impotence, conditioning their well-being status. In evaluating and treating sexual dysfunction, a nephrologist must consider factors involved in its pathogenesis, such as hypothalamic-pituitary-gonadal axis alterations, psychological problems related to chronic disease, secondary hyperparathyroidism, anemia, autonomic neuropathy, derangements in arterial supply or venous outflow, and the normal structure of cavernous body smooth muscle cells. The introduction of sildenafil to treat impotent patients has completely changed the approach to evaluating these subjects because this drug is considered an effective well-tolerated treatment for men with ED. In the past, we proposed an algorithm that gave the opportunity to explore the previously mentioned factors using such instrumental interventions as the nocturnal penile tumescence test, penile echo color Doppler, nervous conduction velocity, and cavernous body biopsy, addressed to prescribe needed surgical or medical interventions. The complexity of the proposed algorithm requires many diagnostic procedures and much time and economic resources to localize the pathological lesions responsible for ED. Because of the new oral drug sildenafil, we propose a new algorithm to test the possibility of obtaining an erection and classify patients as responders or nonresponders to the sildenafil test.

Twice versus thrice weekly administration of intravenous calcitriol in dialysis patients: a randomized prospective trial. Gruppo Italiano di Studio dell'Osteodistrofia Renale.

BACKGROUND: Administration of intravenous (i.v.) calcitriol three times weekly effectively controls the synthesis and secretion of PTH in most uremic patients. Administration of a single dose of 1.25(OH)2D3 reduces synthesis of PTH-mRNA for 6 days in rats. Moreover, it can lower PTH levels for up to 4 days in chronic hemodialysis patients. Therefore, a good response to the administration of i.v. calcitriol two times weekly can be expected. We studied - in a multicenter randomized study in patients with moderate to severe secondary hyperparathyroidism - the effects of the same doses of intravenous calcitriol, administered two or three times weekly. METHODS: Twenty-two hemodialysis patients were randomized into two frequencies of treatment groups: two times (G-2/w) and three times weekly (G-3/w). Both groups were treated with increasing doses of intravenous calcitriol for 3 months (first month 3 microg, second month 4 microg, third month 6 microg weekly). RESULTS: After 12 weeks of therapy with intravenous calcitriol the G-2/w group showed a significant reduction in serum PTH levels (from 821 +/- 392 to 350 +/- 246 pg/ml; mean reduction = 57.4%) comparable to the decrease observed in the G-3/w group (from 632 +/- 116 to 246 +/- 190 pg/ml; mean reduction = 61.2%). Ionized calcium (G-2/w from 1.13 +/-0.10 to 1.14 +/- 0.08 and G-3/w 1.21 +/- 0.13 to 1.26 +/- 0.18 mmol/l) and phosphate levels (G-2/w from 4.99 +/- 1.01 to 5.99 +/- 1.78 and G-3/w 5.31 +/- 0.73 to 5.81 +/- 1.18 mg/dl) did not change significantly and phosphate binders were not modified during the study. CONCLUSION: This study confirms that intravenous calcitriol is an effective therapy for moderate to severe secondary hyperparathyroidism. The administration of two doses per week of intravenous calcitriol is as efficacious as three doses per week in suppressing PTH secretion.

[Blood aluminum in a population of hemodialyzed patients. 1981-1991: a comparison of a decade]. / Alluminiemia in una popolazione di emodializzati. 1981-1991: un decennio a confronto.

The authors report their data on the behaviour of serum aluminium registered at a 10 year interval among a population of haemodialyzed patients. Improved knowledge of the toxic role of aluminium and of the sources of pollution in dialyzed patients seems to relegate the neurologic toxicity of aluminium to a historical aspect.

[A case of Jaffé-Lichtenstein fibrous dysplasia. Etiopathogenetic findings]. / Su di un caso di displasia fibrosa di Jaffé-Lichtenstein. Considerazioni etiopatogenetiche.

The AA. report a case of Jaffé-Lichthenstein Fibrous Dysplasy, a relatively rare syndrome whose aetiopathogenesis is still discussed. Blood laboratory data of the patient and of his family suggest a genetic background on the upon mentioned syndrome.

Subclinical hypercortisol-assessment of bone fragility: experience of single osteoporosis center in Sicily.

AIM: Hypercortisolism is known to cause osteoporosis. Some evidence suggests that osteoporotic fractures may be the presenting manifestations of otherwise-asymptomatic hypercortisolism. The aim of our research was to investigate the prevalence of subclinical hypercortisolism (SH) in postmenopausal women evaluated for bone fragility. PATIENTS AND METHODS: One hundred consecutive postmenopausal women attending the Osteoporosis Centre in the Department of Internal Medicine of the University of Messina (Messina, Italy), for the first time, were screened and a total of 50 patients (age 58±5 years) were studied. Hypercortisolism was diagnosed by unsuppressed serum cortisol levels after 2 day low dose dexamethasone suppression test. RESULTS: Among the 50 postmenopausal women studied, 3 had SH. This prevalence was 6%. The three patients with SH had a normal bone mineral density (BMD) at lumbar spine and were osteopenic at femoral neck, and presented one or more vertebral fractures at spinal radiography. CONCLUSIONS: Physicians should always consider SH among the causes of bone fragility, especially in individuals with vertebral fractures and the presence of an only slightly reduced BMD.

Thyroid metastases from clear cell renal carcinoma 18 years after nephrectomy.

Single or multiple thyroid metastases from extra-thyroid primary tumors are reported to be rare. Malignancies that metastasize to the thyroid include cancers originating from lung, breast and kidney. We report our experience with a case of thyroid metastases, which were detected 18 years after curative kidney surgery for renal cell carcinoma. After 18 years, the patients noted the sudden appearance of a lump in the neck. Ultrasonography showed the presence of a multinodular goiter, all nodules being "cold" at scintiscan. Total thyroidectomy was performed; histology of all nodules revealed a metastatic thyroid cancer from renal cell carcinoma. The patient was still alive and in good health 16 months after thyroidectomy. History of patients with thyroid nodules should include inquiring about extra-thyroid malignancies, especially renal cell carcinoma, that may have been diagnosed even many years earlier. As a corollary, follow-up of such patients should include periodic thyroid exploration or at least a physical examination.

The discovery of nephrouroameba: was it real or not?

In 1938 Procaccini showed scientific interest in a new kind of ameba, and called it "nephrouroameba" from which the disease "nephrouroamebiasis" is derived. He wrote a paper titled "La Nefrouroamoebiasi" thus describing its history, the biopathogenetic evolutionary cycle of the protozoon, its therapeutic, epidemiological, anatomo-biological, diagnostic, cultural, biological and morphological features. Between 1934 and 1939, Procaccini had the opportunity to follow many patients belonging to a group of Italian soldiers serving in the Eastern Italian Army in Ethiopia. At that time he was responsible for the biopathological laboratory. After a short preclinical stage of fatigue, patients suffering from nephrouroamebiasis showed a nephrotic syndrome with gross hematuria. The symptoms ceased within a few days but residual microhematuria, albuminuria and urine casts persisted for many months. After microscopic observation, he reproduced some protozoons and classified them as a kind of ameba. Critical analysis of his report leads to the morphological identification of Trichomonas, thus excluding his classification as nephrouroamebas.

Involvement of interleukin-18 in patients on maintenance haemodialysis.

Maintenance dialysis induces a clinical state of immunodeficiency. The pathway of circulating T cells from haemodialyzed patients is changed and characterized by an increase of Th1 cells. The unbalanced T helper differentiation derives from an altered regulation of interleukin-12 (IL-12), which represents an important inducer of Th1. IL-18 is a pro-inflammatory cytokine expressed by a variety of cell types that is structurally related to the Th1 family and shares biological properties with IL-12 as the promotion of Th1 responses. To explain the involvement of IL-18 in the typical disorders of dialysis, we analyzed IL-18 serum levels in a group of haemodialyzed patients. We enrolled 16 patients on chronic haemodialysis (HD) treatment for end-stage renal failure and 16 healthy volunteers as the control group. IL-18 levels were assessed by immunoenzymatic methods (detection limit was <12.5 pg/ml). HD patients strongly showed higher IL-18 serum levels compared to healthy donors (508.47 +/- 314.39 vs. 193.44 +/- 56.33 pg/ml, p < 0.005). Moreover, IL-18 levels in HD directly correlated to dialytic age (Rho = 0.544, p = 0.0419) and indirectly to Kt/V (Rho = 0.703, p = 0.0086). Our data represent the first evidence of the relation between IL-18 serum levels and HD. In the light of our results, we think that the unbalanced T helper differentiation may depend, at least in part, on an abnormality in the IL-18 production.