Neonatal lupus in triplet pregnancy of a patient with undifferentiated connective tissue disease evolving to systemic lupus erythematosus.

Pregnancy in patients suffering from undifferentiated connective tissue disease (UCTD) represents a risk situation for both the mother and the child. SSA/SSB autoantibodies can determine neonatal lupus (NL) in the foetus, regardless of the maternal disease. Furthermore, pregnancy increases the risk of flares and evolution to differentiated connective tissue disease (CTD). We report an uncommon case in which these complications occurred in a mother and in her foetuses. A 37-year-old woman affected by UCTD developed systemic lupus erythematosus (SLE) after her triplet pregnancy. The only manifestation of neonatal lupus we observed in the three newborns was SSA positivity associated with asymptomatic transient neutropenia.

Risk factors for a first thrombotic event in antiphospholipid antibody carriers. A multicentre, retrospective follow-up study.

OBJECTIVES: To asses risk factors for a first thrombotic event in antiphospholipid antibody (aPL) positive carriers and evaluate the efficacy of prophylactic treatments. METHODS: Recruitment criteria were age 18-65 years, no history of thrombosis, positivity for lupus anticoagulant and/or IgG/IgM anticardiolipin antibody (aCL) on > or =2 occasions at least 6 weeks apart. Demographic, laboratory and clinical parameters were collected at enrolment and at the time of the thrombotic event. RESULTS: 370 patients/subjects (mean (SD) age 34 (9.9) years) were analysed retrospectively for a mean (SD) follow-up of 59.3 (45.5) months. Thirty patients (8.1%) developed a first thrombotic event during follow-up. Hypertension and medium/high levels of IgG aCL were identified by multivariate logistic regression analysis as independent risk factors for thrombosis. Thromboprophylaxis during high-risk and long-term periods was significantly protective. CONCLUSIONS: Hypertension or medium/high titres of IgG aCL are risk factors for a first thrombotic event in asymptomatic aPL carriers and primary prophylaxis is protective.

Knowledge of the anomalies of the big central veins reduces the morbidity during the cannulation for hemodialysis: Description of a case of persistent left superior vena cava and revision of literature.

The persistence of a left superior vena cava is the result of a lack of an embryological involution of the left anterior cardinal vein. This anomaly is very rare: about 0.3% of the general population. Its incidence increases remarkably from 3-10% in those patients affected with congenital heart disease. Described herein is a case of persistent left superior vena cava, discovered by chance, following the placement of a central venous catheter for hemodialysis. A chest X-ray in projection back-forward showed the central venous catheter along the left sternal margin simulating a placement in the aorta artery. This clinical picture, as described in the literature, is often accompanied by other anatomical anomalies, in our case, by the congenital agenesis of a solitary pelvic kidney. In agreement with the literature and in contrast with what has been reported recently, we sustain that a central venous catheter placed, for any reason, in the persistent left superior vena cava must be removed immediately because it can induce hyperkinetic arrhythmia and cardiac arrest as in our case. Our case report should be a warning that lack of awareness of the anomalies of the big central veins can cause a rise in morbidity.

[Acute kidney failure and losartan: a recently observed event of antagonists of angiotensin II AT1 receptors]. / Insufficienza renale acuta e losartan: una nuova recente segnalazione degli inibitori dei recettori AT1 dell'angiotensina II.

Losartan is the first of a new category of drug that inhibits angiotensin II (ANG II) AT1 receptors antagonists. This drug lowers blood pressure by inhibiting the activity of ANG II and reduces proteinuria and progression of chronic renal failure (CRF). It seems therefore an extremely interesting drug. Aim of this study is to describe 3 cases of acute renal failure (ARF), occurred during therapy with losartan. None of the patients showed renal arteries stenosis or other predisposing factors for the development of ARF. In conclusion, we want pointed out that losartan could affect renal function in a similar way as angiotensin converting enzyme inhibitors (ACEI). We suggested that use of losartan in risk situations, like old age, preexiting CRF, stenosis of renal arteries, solitary kidney and diuretic therapy, should be carefully monitored as well as that of ACE I.

[Evaluation of urea recirculation and dialysis efficiency of central venous jugular catheters when the venous lumen is used as an arterial lumen]. / Valutazione del ricircolo dell'urea ed efficacia dialitica del catetere giugulare venoso centrale quando il lume venoso è utilizzato come lume arterioso.

BACKGROUND: The dual lumen internal jugular venous catheter has proven to be the most useful temporary vascular access for hemodialysis. According to this evidence it was decided to evaluate urea recirculation rate during hemodialysis performed by dual lumen internal jugular catheter (IJC) under normal condition (R1) and when the arterial lumen of the catheter is used as venous lumen, and the venous lumen as arterial lumen (R2). METHODS: In 71 patients who underwent hemodialysis using a dual lumen IJC, urea recirculation rate was measured during a conventional bicarbonate hemodialysis, under normal condition R1 and during the experimental condition R2. RESULTS: Urea recirculation rate < 5% was achieved for almost all patients under normal condition R1. In the different condition R2, urea recirculation rate increased in all patients, from an average value of 3.7 +/- 1.7 to 5.1 +/- 1.8 p < 0.0001. This increment was expected ut surprisingly low. CONCLUSIONS: In conclusion during hemodialysis with dual lumen IJC efficient treatment can be provided in normal condition and also when the venous lumen is used as arterial lumen.

[Drugs used in the treatment of arterial hypertension in dialysis patients in the Piedmont. What correlations between personal and clinical data can be made from the registry data?]. / I farmaci utilizzati per il trattamento della ipertensione arteriosa nei pazienti in dialisi in Piemonte. Quali correlazioni con i dati anagrafici e clinici sono possibili dai dati del registro?

The therapeutical approach to arterial hypertension in the general population is now relatively well classified, whereas it remains a controversial problem in dialytic patients. The aim of this study was to evaluate the antihypertensive drugs used in dialytic patients in Piedmont and to identify correlations with other personal and clinical data. The authors analysed the data in the Piedmont Dialysis and Transplant Register concerning new patients admitted to dialysis during the period 1990-1995 (2,664 patients at 31/12/1995) and 1,373 patients who began dialysis during the period 1990-1993. A study of the antihypertensive drugs using in single and combined therapy over the five-year period shows major variations in the 45-65 year-old age bracket (increased ACE-inhibitors in single therapy, 15.5-25.6%, increased vasodilators in combined therapy, 15.3-21%). In patients aged > or = 65 years old a slight increase was found in the use of beta-blockers in monotherapy. Antihypertensive drugs at the 1st control (1990-1995 entries) appeared to be stable over the five-year period. From the 1,373 patients who started dialysis in the period 1990-1993, with at least three subsequent controls, the authors selected those hypertensive or normotensive patients receiving ACE-inhibitor therapy (best survival in general population) and compared their survival with that of patients receiving alternative antihypertensive treatment. No significant differences were found. The stability of the antihypertensive drugs taken by these patients over the past 5 years backs the hypothesis of a greater attention paid by nephrologists to the introduction of new drugs, both because of the frequent onset of collateral effects and owing to the special pharmacokinetics present in dialytic patients.

Influence of the hydration state on blood pressure values in a group of patients on regular maintenance hemodialysis.

The pathophysiology of hypertension in patients on renal replacement therapy is not yet clear, and the role of extracellular fluid overload is still a matter of debate. The main problem is the lack of techniques to determine the fluid state. Recently new noninvasive techniques have become available which make it possible to accurately determine the hydration state in these patients. We have studied the influence of the hydration state on interdialytic blood pressure in 45 patients: 21 (46.6%) using antihypertensive medication and 24 (53.4%) without antihypertensive medication. Total body water (TBW) was determined by bioelectrical impedance analysis performed just before a hemodialysis session. The TBW was then related to the fat-free mass calculated by the anthropometric method (aFFM) of Durnin. The hydration state was defined using the following formula: TBW/aFFM 100. Furthermore, for each patient the ideal TBW was calculated according to the Watson formula. The difference between TBW and ideal TBW was considered a further index of the hydration state. Ambulatory blood pressure monitoring was performed by using a Takeda 24200 recorder according to the Korotkoff method during the 24 h before the midweek hemodialysis session. Blood pressure monitoring showed a significant correlation with the hydration state of these patients. In conclusion, the hydration state seems to play a major role in interdialytic blood pressure control.

[Epidemiology and clinical course in hepatitis C-positive patients treated with dialysis in Piemonte. Work group of the RPDT]. / Epidemiologia e comportamento clinico nei pazienti epatite C positivi in dialisi in Piemonte. Gruppo di lavoro dell' RPDT.

The high prevalence of HCV positivity in dialysis patients has recently prompted several studies on this controversial subject. The aim of the study was an evaluation of HCV positivity and of influence on clinical outcome in 2,404 patients on dialysis in Piedmont (Northern Italy Region, about 4,400,000 inhab., 20 Dialysis Centers) at December 1993 (32.7% HCV-positive according to the Regional Registry of Dialysis and Transplantation). As expected, a strong correlation was found with time on dialysis (94.4% in patients with treatment follow-up > or = 20 years) and blood transfusions (41.3% HCV positivity in transfused patients versus 22.6% in not transfused ones; p < 0.0001). Dialysis in a hospital setting is confirmed as at high risk of infection, since HCV positivity was 19.6% in patients always treated by bicarbonate dialysis in hospital versus 8.9% in those treated only by CAPD (start of dialysis in 1992-1993). Despite the high prevalence of HCV positivity, however, death rates for liver disease and for all gastrointestinal causes are very low, regardless of HCV antibody status (1.2% in HCV positive versus 2.3% in HCV negative, NS). Whether this depends upon different clinical features of HCV infection in dialysis patients or merely deflects a relatively short follow-up will be matter of discussion in the future.

Monitoring of central venous dual-lumen catheter placement in haemodialysis: improvement of a technique for the practising nephrologist.

Mismanagement in the placement of central venous catheter (CVC) may occur in up to 20% of cases. The catheter can be inadvertently placed in the contralateral brachiocephalic vein, the ipsi or contralateral internal jugular vein, and usually a thoracic radiograph is necessary to evaluate its location. We propose a technique first described by Serafini et al. to establish the position of a CVC by endocavitary electrocardiography (EC-ECG) and its employment in a large number of uraemic patients requiring haemodialysis. This technique uses the tip of the CVC as reference lead in a standard electrocardiograph. The best employment of this technique has been obtained by echotomographic visualization of the internal jugular vein executed just before transcutaneous puncture of the vessel. For 13 months we have successfully applied this technique in CVC placement in 81 patients requiring haemodialysis. In our opinion this method is a safe and simple technique that avoids the need for thoracic radiographs and time lost waiting for radiographs that prolong the start of the haemodialysis session. According to our experience, we confirm that the EC-ECG technique provides a method for ensuring compliance with Food and Drug Administration guidelines regarding catheter tip location in uraemic patients.

Monitoring of central venous dual-lumen catheter placement in haemodialysis: improvement of a technique for the practising nephrologist.

Mismanagement in the placement of central venous catheter (CVC) may occur in up to 20% of cases. The catheter can be inadvertently placed in the contralateral brachiocephalic vein, the ipsi or contralateral internal jugular vein, and usually a thoracic radiograph is necessary to evaluate its location. We propose a technique first described by Serafini et al. to establish the position of a CVC by endocavitary electrocardiography (EC-ECG) and its employment in a large number of uraemic patients requiring haemodialysis. This technique uses the tip of the CVC as reference lead in a standard electrocardiograph. The best employment of this technique has been obtained by echotomographic visualization of the internal jugular vein executed just before transcutaneous puncture of the vessel. For 13 months we have successfully applied this technique in CVC placement in 81 patients requiring haemodialysis. In our opinion this method is a safe and simple technique that avoids the need for thoracic radiographs and time lost waiting for radiographs that prolong the start of the haemodialysis session. According to our experience, we confirm that the EC-ECG technique provides a method for ensuring compliance with Food and Drug Administration guidelines regarding catheter tip location in uraemic patients.

Longitudinal assessment of body composition in continuous ambulatory peritoneal dialysis patients using bioelectric impedance and anthropometric measurements.

We assessed the utility of bioelectric impedance analysis (BIA) and anthropometry for longitudinal evaluation of body composition in continuous ambulatory peritoneal dialysis (CAPD) patients. Eleven subjects were studied at the beginning of CAPD and again at regular intervals during the first 6 months of treatment. The significant weight gain that occurs in our patients is mainly due to a rise in total body water (TBW), as measured by BIA, during the first weeks of CAPD, and later on due to a body fat increase. Anthropometry seems more reliable than BIA in the evaluation of body mass, because the latter is derived from TBW in BIA. Therefore, any change in TBW that occurs in a CAPD patient necessarily causes a similar change in the fat-free mass. In our experience, only the combined use of both anthropometry and BIA allows a proper assessment of body composition in patients on CAPD.

[Arterial hypertension secondary to Conn's disease: an infrequent pathology in nephrology. Report of a clinical case]. / Ipertensione arteriosa secondaria a morbo di Conn: una patologia di non frequente osservazione nefrologica. Presentazione di un caso clinico.

In this work we describe a case of Conn's syndrome caused by a suprarenal adenoma in a sixty-one year female. The patient had come to our observation because of severe hypertension and hypokalemia. Primary aldosteronism resulting from the secretion of excessive amounts of aldosterone caused by autonomous hyperfunction of the adrenal cortex usually by a solitary adenoma. In most series of unselected patients, it is found in fewer than 0.5% of hypertensives. In our study we demonstrated the circadian changes of arterial blood pressure but we failed to demonstrate the presence of hypertrophic cardiomyopathy as recently emphasized in the literature. Regardless of its rarity, primary aldosteronism is a fascinating disease, protean in its manifestations, logical in its pathophysiology.