Treatment with plasma exchange of a pregnant woman with anti-PP1Pk alloimmunization: A case report.

The histo-blood group antigens P, P1 and Pk are a closely related set of glycosphingolipid structures expressed by red blood cells and other tissues. None of these three characters is expressed on p cells, a null phenotype that arises in the context of homozygous mutation of the A4GALT gene. Subjects with p phenotype spontaneously develop a natural alloantibody named anti-PP1Pk, which is a mixture of IgG and IgM against P1, P and Pk. While anti-P1 is a weak cold antibody with poor clinical significance, anti-P and anti-Pk antibodies are potent haemolysins responsible for severe hemolytic transfusion reactions. The rare anti-PP1Pk alloantibodies are associated with recurrent spontaneous abortion in the first trimester of gestation. P and Pk antigens are expressed at high levels on the placenta and antibodies directed against both these structures are deleterious to placental trophoblasts. Here we describe the use of plasma exchange (PEX) in a nulliparous 39-year-old woman with anti-PP1Pk antibodies and a history of repeated spontaneous early abortions and hypofertility. The patient underwent apheresis starting from the third week throughout the pregnancy and a healthy child was delivered by cesarean section at 35 WG. The newborn required only phototherapy within a few days of life. We can state that an early treatment with the only PEX has proven to be effective and safe in the management of a fetomaternal P-incompatibility caused by a high anti-PP1Pk titer (256).

Celiac disease and school food service in Piedmont Region: Evaluation of gluten-free meal.

INTRODUCTION: The Law 123/2005 recognizes celiac disease as a social disease and so Ministry of Public Health annually allocates specific resources to Regions for managing gluten-free meals in school canteens. Therefore in 2009 Piedmont Region approved a specific project, in collaboration with Food Hygiene and Nutrition Department (SIAN) of several ASL (Local Health Authority), including ASL TO3 as regional leader, and the "Italian Celiac Association - Piedmont and Valle d'Aosta". This project was intended to facilitate the natural integration of celiac people in social life. A retrospective analysis of data has been carried out to assess the management of gluten- free meal of school food services in Piedmont Region in 2010. Furthermore the intervention efficacy has been evaluated comparing the critical points observed in 2010 and 2012. METHODS: The object of the study includes primary and secondary schools that have provided gluten-free food service in Piedmont Region. These school were examined by SIAN staff. (the examination included the check of hygienic aspects and qualitative assessment of the meal). The data were collected using the same checklist throughout the region. All data were included in the unified regional system ("Reteunitaria"). RESULTS: The results show that 29% of the sampled schools (277) are acceptable in all eight sections (supply, storage, process analysis, equipment check, packaging and transport, distribution of meals, self-control plan and qualitative assessment), whereas 71% are inadequate for at least one of the profiles (60% does not perform the qualitative valuation of service) and in 18% of schools three to seven insufficiencies are observed. Correlations between the number of total insufficiencies and the most critical sections of the check list were performed (with lower scores in "good") such as process analysis, distribution of meals, self-control plan and qualitative assessment. The analysis process has achieved a high score in the field of deficiency for at least 3 parameters. Schools with a good self-control plan have a significant correlation with schools suitable for the analysis process, instead schools appeared insufficient in the process analysis have an increased chance of being insufficient also in the distribution of meals. The schools that provide a transported meal (municipalities highly populated, generally) have many differences in distribution of meals respect schools that prepare food in the school kitchen. In fact, 88% of school that provide a transported meal achieved an appropriate score in distribution of meals section and collected fewer failures in overall assessment than the others. 120 structures are included in the indirect comparison between the checklist's sections with criticisms, during years 2010 and 2012: in 2010 32% of schools were recorded acceptable in all of the eight sections of the checklist and in 2012 this percentage rose to 54%. An improvement can be observed in all areas, but a statistical significant result do not turn out. DISCUSSION AND CONCLUSIONS: Data show that carry on the control activities of production of gluten free meal in school canteens would be appropriate. Actions focused on improving the methods of preparing meal without gluten were recommended, especially in under populated municipalities with school kitchen on site. The constant presence of ASL staff in school has promoted important changes: cultural change and about the management of allergy and food intolerance. An improvement can be observed: a transition from a suspicion about "special diet" management to an appropriate and responsible management of meals for children and young people suffering from this specific condition.

Postprandial glucose variability in type 1 diabetes: The individual matters beyond the meal.

AIM: To explore intraindividual (between-meals) and interindividual (between-subjects) variability of postprandial glucose response (PGR) in type 1 diabetes (T1DM). METHODS: Data were taken from five cross-over trials in 61 subjects with T1DM on insulin pump wherein the effects of different dietary components or the intraindividual-variability of PGR to the same meal were evaluated by CGM. Predictors (type of meal or nutrient composition) of early (iAUC0-3h), late (iAUC3-6h), total (iAUC0-6h), and time-course of postprandial blood glucose changes (iAUC3-6hminus0-3h) were evaluated using two mixed-effect linear regression models considering the patient's identification number as random-effect. RESULTS: High-glycemic-index (HGI) and low-glycemic-index meals were the best positive and negative predictors of glucose iAUC0-3h, respectively. A Low-Fat-HGI meal significantly predicted iAUC3-6hminus0-3h (Estimate 3268; p = 0.017). Among nutrients, dietary fiber was the only significant negative predictor of iAUC0-3h (Estimate -550; p < 0.001) and iAUC0-6h (Estimate -742; p = 0.01) and positive predictor of iAUC3-6hminus0-3h (Estimate 336; p = 0.043). For all models, the random-effect patient was statistically significant (p < 0.001 by ANOVA). CONCLUSION: Beyond the meal characteristics (including glycemic index, fat and fiber content), individual traits significantly influence PGR. Specific interindividual factors should be further identified to properly predict glucose response to meals with different composition in individuals with T1DM.

Intradialytic changes of plasma amino acid levels: effect of hemodiafiltration with endogenous reinfusion versus acetate-free biofiltration.

During hemodialysis, amino acids (AA) are lost in the ultrafiltrate with consequent modification of their plasma profile. The aim of this cross-sectional study was to evaluate intradialytic changes of plasma AA levels during a single session of hemodiafiltration with endogenous reinfusion (HFR) versus acetate-free biofiltration (AFB). 48 patients chronically treated with HFR or AFB were matched 1:1 for age, gender, Kt/V and diabetes. Blood samples were collected at the beginning and the end of dialysis. Baseline plasma levels (µmol/l) of total AA (3,176 ± 722), essential AA (889 ± 221), and branched chain AA (459 ± 140) levels in HFR were similar to those in AFB (3,399 ± 621, 938 ± 277, and 463 ± 71, respectively). Plasma intradialytic AA levels did not change in HFR, while in AFB there was a reduction by about 25%. In conclusion, as compared with AFB, HFR has a sparing effect on AA loss due to the lack of adsorption by cartridge and to their complete reinfusion in blood.

BKV reactivation in renal transplant recipients: diagnostic and therapeutic strategy--case reports.

The Polyomaviridae family includes several viruses that are ubiquitous with specific host spectra. The human polyoma viruses BK and JC were discovered in 1971. Following primary infection, transmitted by the respiratory and probably the oral route, BK remains latent in uroepithelial cells, in B lymphocytes, or in other tissues (spleen, brain). Reactivation with asymptomatic viruria may occur in both immunocompetent subjects and immunocompromised patients. In renal transplant recipients, BKV replication may cause tubulointerstitial nephropathy (BKVAN) with increasing prevalence rates--1% in 1995, 8% in 2007--leading to the loss of the transplanted organ in 30% to 80% of cases. With the availability of diagnostic programs (decoy cells in urine, amplification of viral DNA by polymerase chain reaction (PCR) on serum and urine, real time (RT)-PCR test for mRNA VP1 urine (mRNA-VP1), and renal biopsy accompanied by reduction in immunosuppression, administration of leflunomide, cidofovir (after hydration), and N-acetylcysteine, as well as immunoglobulin by intravenous injection (IVIg), the incidence of renal loss caused by BKVAN infection has been reduced by 10% to 80%. In this study, we have described 12 patients: 6 treated with tacrolimus (FK), mycophenolate mofetil (MMF), and steroids, and 6 treated with cyclosporine or with mTOR inhibitors. Two patients from the first group showed BKVAN about 3 months posttransplantation. Early diagnosis and therapeutic intervention (cidofovir + IVIg) led to reduction in the viral load, with improvement and stabilization in renal function. Considering the high positive predictive value (98%) of mRNA VP1, it should be possible to avoid renal biopsy. The level of immunosuppression--rather than the immunosuppressive drug itself (FK and MMF)--seemed to be associated with BKV reactivation.

Human herpesvirus 8 in Italian HIV-seronegative patients with Kaposi sarcoma.

OBJECTIVE: To evaluate the prevalence of human herpesvirus 8 (HHV-8) DNA detection in a large series of human immunodeficiency virus-seronegative patients with and without Kaposi sarcoma (KS) from the central and southern regions of Italy where classic KS is prevalent. DESIGN: Samples of lesional, peripheral unaffected, and distant normal skin and peripheral blood mononuclear cells (PBMCs) from 33 patients with KS and PBMCs from 42 control subjects were analyzed using single and nested polymerase chain reaction techniques for the presence of HHV-8 DNA. PATIENTS: A total of 33 patients with KS not related to acquired immunodeficiency syndrome (26 patients with classic KS and 7 patients with iatrogenic KS) were studied. Furthermore, 2 control groups were enrolled. The first group consisted of 13 healthy volunteers, the second of 29 patients affected by different dermatological diseases. RESULTS: Human herpesvirus 8 sequences were found in 100% of lesional and perilesional specimens, in 33% of the distant normal skin samples, and in 69.6% of the PBMCs from patients with KS. A possible correlation between HHV-8 DNA in PBMCs and the clinical stage of the disease was observed. Moreover, the prevalence of viral DNA in PBMCs from the total control group was 23.8%. No viral DNA was detected in tissue biopsy specimens taken from the control group. CONCLUSIONS: Our data suggest that HHV-8 could be a widespread virus, at least in Mediterranean regions where KS is more prevalent, such as southern and central Italy. As with other herpesviruses, it may be present lifelong in latent form somewhere in the body and may contribute to the pathogenesis of KS when other predisposing conditions are present.

Immunohistochemical study of the early histopathologic changes occurring in trauma-injured skin of psoriatic patients.

In the present study we have investigated the early histopathologic as changes occurring in the Koebner reaction induced by traumatic injury in uninvolved skin of 23 psoriatic and 7 non-psoriatic control patients. A punch biopsy of the injured area was performed after 2-3 (15 cases) or 7 days (8 cases). As a trauma, instead of the classic sellotape stripping, needle scarification was used. A peculiar histological feature of the skin biopsies of 13/23 psoriatic patients (56%) was a keratinocyte hyperplasia leading to a "papillary" projection into the upper dermis, just beneath the scarification. The papillary projection was associated with the expression of intercellular adhesion molecule-1 (ICAM-1) in the keratinocytes of 9/13 cases (70%) and with the presence of peri-papillary aggregates of CD68+ cells in 10/13 cases. In the upper dermis, tenascin was markedly expressed in 12/13 cases. Moreover, in one third of the cases, just beneath the scarification, there was reabsorption of the epidermal basal membrane as documented by a marked reduction of collagen type IV and laminin content. These histopathological alterations were detected in 6/15 psoriatic patients whose skin biopsy was taken 2-3 days after scarification, in 7/8 after 7 days, and in only 1/7 non psoriatic controls. Our results indicate that needle scarification can be a suitable method to study the early events occurring in trauma injured psoriatic skin.

[Cystic fibrosis of the pancreas. Historical notes, sweat test, and neonatal screening]. / Fibrosi cistica del pancreas. Cenni storici, test del sudore e screening neonatale.

Chronologic milestones in cystic fibrosis (CF) are reported. The value of the sweat test and the most recent advances on neonatal CF screening are described.

[Orbital cellulitis in pediatric age. Report of a case and review of the literature]. / Cellulite orbitaria in età pediatrica. Descrizione di un caso e rassegna della letteratura.

The authors report about 2-year-old female affected by orbital cellulitis. The most recent advances about physiopathology, diagnosis and therapy of orbital cellulitis are described.

Justifying and tracking BMET training.

We have outlined the importance of BMET training and how it is a crucial investment for not only the health care facility but also clinical engineering and individual BMETs. Training methods and funding mechanisms are also important aspects of the continuing education process. Being aware of the alternatives helps to achieve the goals of the clinical engineering program. We have also demonstrated management techniques of acquiring training, evaluating courses, and keeping statistics. Courstat manages statistics of training intensity among BMETs. This kind of tool provides managers with an overall picture of departmental expertise.

Risk-oriented maintenance.

Responding to information has become the most critical aspect of technology management. With ROMSYS we gain significant ability to exercise this ideology. Accessing reports in real-time provides instant knowledge of any aspect concerning equipment support. This kind of resource can give management the confidence of having a system that virtually takes care of itself. Managers can spend their time making informed decisions rather than wasting it searching for information. The philosophy of doing only what needs to be done, when it needs to be done, becomes reality when systems such as PM Interval Adjustment, PM Index, and % PM Effectiveness are utilized. These elements of ROMSYS also prevent the tying up of expensive expertise (biomedical equipment technicians) in mundane activities that are primarily ineffective. The system helps to enhance the pride of work among BMETs by elevating the importance of the PM inspection. The health care facility stands to gain from a system that monitors itself and continues to adjust to improve the process. This is the fundamental principle of total quality management, where the goal is to improve patient outcome while being cost-effective.

Correction of posterior crossbite.

Posterior teeth exhibiting a transverse discrepancy are said to be in crossbite. This abnormal bucco-lingual relationship is usually viewed as affecting one side, but it is really a bilateral problem. There is a multifactorial etiology for this. Early diagnosis by the treating dentist is essential for proper treatment. If not detected early, surgically assisted rapid palatal expansion is needed to correct the transverse discrepancy. Through a series of osteotomies, along with orthodontic appliance therapy, it can be corrected. On such case is presented here. The three main objectives of this treatment are to correct the posterior crossbite; to reduce crowding, and to form a stable occlusion. As shown by this case example, surgically assisted rapid palatal expansion is a safe and effective means of correcting posterior crossbites.

[Hemodiafiltration with endogenous reinfusion in chronic inflammation: a possible therapeutic alternative?]. / L'impiego dell' Emodiafiltrazione con reinfusione endogena (HFR) nell'infiammazione cronica idiopatica: una possibile alternativa terapeutica?

The patients under maintenance haemodialysis (HD) continue to have an unacceptably excess of mortality compared to general population, that may be explained by high prevalence of inflammation that significantly influences the survival of these patients. Indeed, chronic inflammation is very common in HD and it may cause malnutrition and progression of atherosclerotic disease by several pathogenetic mechanisms triggered by pro-inflammatory cytokines. Currently no pharmacological intervention is specifically targeted the idiopathic chronic inflammation. Hemodiafiltration with endogenous reinfusion (HFR) is a dialysis technique, highly biocompatible, that combines three depurative mechanisms: diffusion, convection and absorption. The ultrafiltrate is obtained from convective section of dialyzer (convection). It is regenerated by passing through the adsorbent macro-porous synthetic resin cartridge (absorption) and then it is reinfused into the second section of the filter (diffusion). This resin cartridge is able to absorb cytokines and other uremic toxins, whereas allows to pass nutrients and antioxidants, as amino acids and vitamins, with a consequent decrement of inflammation and oxidative stress. These characteristics suggest the use of HFR in HD patients affected by overt and idiopathic chronic inflammation. In these patients, we observed that the switching from Bic-HD to HFR allowed an improvement of inflammatory as testified by a significant decrement of serum levels of CRP IL-6, IL-1 and TNF- and a significant increase of albumin and pre-albumin. Whether these favorable effects may modify the outcomes of these high-risk patients, needs to be confirmed by studies ad-hoc.

Cardiovascular risk factors in renal transplant patients after switch from standard tacrolimus to prolonged-release tacrolimus.

Cardiovascular (CV) diseases are the leading cause of death after renal transplantation. Renal transplant patients present CV risk factors that correlate with renal function and the use of immunosuppressive drugs. Noncompliance with immunosuppressive therapy after organ transplantation increases the incidence of rejection, graft loss, and patient death. A simple posology regimen is the best way to promote compliance with prescribed therapy. To meet this need, a new formulation of tacrolimus that is suitable for once-daily administration, is now available on the market: prolonged-release tacrolimus (Fkpr). We analyzed changes in CV risk factors observed in renal transplant patients after transition from standard tacrolimus (Fk) to Fkpr and the rate of patients with the investigated parameters within the normal ranges before and after conversion. The study enrolled 40 Caucasian renal transplant patients (26 men and 14 women) who were being followed at our posttransplantation day hospital clinics. After a varying time interval after transplantation, patients on treatment with tacrolimus, mycophenolate + mofetil (MMF), and steroid entered a 12-month observation period. Thereafter, they were switched to Fkpr, also in association with MMF and steroid, and were observed for a further 12-month period. The following parameters were tested in all patients: creatinine, creatinine clearance, insulin resistance, total cholesterol, HDL cholesterol, LDL cholesterol, triglycerides, uric acid, homocysteine, and urine magnesium. The switch from Fk to Fkpr showed an improvement of the parameters investigated. Moreover, the proportion of patients with normal laboratory values after the transition from Fk to Fkpr was noted either to increase or to remain stable at the improved levels observed during therapy with Fk. Immunosuppressive treatment with Fkpr represents an even better option than Fk for renal transplant patients, because by reducing CV risk factors it favorably affects the long-term outcomes for graft and patient.

Immunosuppressive agents and bone disease in renal transplant patients with hypercalcemia.

Renal transplantation is the definitive treatment for many metabolic abnormalities of uremic patients, although it is only partially effective for renal osteodystrophy, which may interact with posttransplant renal osteopathy. Osteopenic-osteoporotic syndrome represents, together with fractures secondary to osteoporosis and osteonecrosis, the bone complication most related to renal transplantation. Several factors contribute to the pathogenesis of posttransplantation osteoporosis, particularly immunosuppressive treatment. In this study, we evaluated the prevalence of factors related to posttransplant renal osteopathy and the clinical impact of immunosuppressive protocols. We studied 24 renal transplant recipients with hypercalcemia. Glomerular filtration rate was >50 mL/min. Mean age, time on dialysis, and time from transplantation were 49.6, 5.4, and 6.9 years, respectively. We evaluated serum and urine calcium and phosphorus, calcitonin, parathormone, bone-specific alkaline phosphatase, osteocalcin, urine deoxypyridinoline, telopeptide of type 1 procollagen, 1,25-(OH)(2) and 25-OH vitamin D, parathyroid ultrasound, and computerized bone mineralometry. The combination of sirolimus and steroids resulted in the most disadvantageous outcomes regarding alkaline phosphatase and mineralometry. Calcineurin inhibitors did not significantly influence bone metabolism markers; mycophenolate mofetil evidenced no effect on bone. According to the literature, steroids account for the abnormalities found in our patients and in severe osteopenia. Several factors may contribute to the development of osteoporosis and fractures in transplantation patients, although they are overcome by the prominent effect of steroids. In patients at high risk of osteoporosis, steroid-free therapy should be considered. Everolimus is indicated for diseases with bone loss. Combined therapy with everolimus and mycophenolic acid without cyclosporine and steroids, seemed to be particularly indicated. Prophylactic treatments should be commenced early. No single marker was useful to diagnose posttransplant renal osteopathy. The definitive diagnosis should be made by bone biopsy during transplantation, and noninvasive procedures, such as densitometry and evaluation of biologic markers, may be useful during follow-up.