A link between psychosocial factors and blood pressure trend in women.

The powerful effect of psychosocial and acculturating influences on population blood pressure trends seems to be confirmed, through longitudinal observations, in the nuns in a secluded order. After initial observation had been made on culture, body form, blood pressure, diet, and other variables in 144 nuns and 138 laywomen, included as a control group, a 32-year follow-up study was undertaken. Most striking were opposite trends noted between the two groups in blood pressure trend. During the follow-up period blood pressure remained remarkably stable among the nuns. None showed a rise in diastolic blood pressure to above 90 mmHg. By contrast the control women showed the expected increase in blood pressure with age. This resulted in a gradually greater difference (Delta>30/15 mmHg) in systolic and diastolic blood pressure between the two groups, which was statistically significant. Fatal and nonfatal events were exceedingly lower in the nuns than in the control women over the follow-up period. It appears reasonable to attribute much of the difference in blood pressure to the different burden in psychosocial factor and to the preserved peaceful lifestyle of the nuns.

The spectrum of cardiovascular abnormalities in autosomal dominant polycystic kidney disease: a 10-year follow-up in a five-generation kindred.

Clinical, electrocardiographic and echocardiographic data were collected in a group of 228 patients with autosomal dominant polycystic kidney disease (PKD) and in another group of 146 unaffected members (NPKD) both comprised in a five-generation kindred followed for 10 years, in order to determine the profile and prevalence of cardiovascular derangement of the genetic disease. A family of 181 members was used as a control. The prevalence of left ventricular hypertrophy in the three groups was 24, 14 and 6% respectively (p less than 0.01); after 10 years it increased up to 35, 26 and 13% respectively (p less than 0.05). The evidence of mitral-valve prolapse was more frequent in PKD and in NPKD group (25 and 20% respectively) than in control subjects (2%) (p less than 0.0001). Mitral incompetence was found in 30, 18 and 8% of those groups respectively (p less than 0.002). The large difference in mitral involvement did not change over time. Tricuspid valve prolapse was detected in 5, 4 and 1% of the three groups, respectively (p less than 0.05). A small increase in frequency was found after 10 years only in polycystic kidney disease patients. Regurgitant aortic lesions were present in higher prevalence in PKD (19%) and NPKD (17%) members than in controls (5%) (p less than 0.001). After 10 years they were 23, 20 and 8%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

"Non-dipper" hypertensive patients and progressive renal insufficiency: a 3-year longitudinal study.

Patients with a blunted or absent nocturnal blood pressure (BP) drop may be subject to increased risk for target organ damage. In this 3-year longitudinal case-control study we tested the hypothesis that an association exists between a reduced or absent night-time fall in BP and a future decline of kidney function in renal hypertensive patients. The case subjects were 48 hypertensives with renal insufficiency, divided into two groups according to the presence (dippers: n 20) or absence (non-dippers: n 28) of a nocturnal diastolic BP decline greater than 10% of daytime values, detected by ambulatory BP monitoring. At the baseline evaluation the two groups did not differ with respect to age, sex, body weight, office systolic and diastolic BP, mean daytime ambulatory BP, creatinine clearance, 24 h proteinuria. In the ambulatory BP profiles over a 3-year follow-up the nocturnal reductions of systolic and diastolic BP in the dippers were 14% and 15%, respectively, vs 7% and 5% in the non-dippers (p = 0.002/0.003). The non-dippers had a faster rate of creatinine clearance decline than the dippers (0.37 +/- 0.26 vs 0.27 +/- 0.09 ml/min/month; p = 0.002). Urinary protein excretion increase was higher in the non-dipper group than in the dipper group (993 +/- 438 vs 691 +/- 222 mg/24 h; p = 0.009). This longitudinal study suggests that the non-dipping pattern of ambulatory BP can be associated with a faster progression of renal insufficiency in renal hypertensives and that a proper nocturnal BP control is an additional aim of antihypertensive therapy.

Sudden death in a child due to rare endocranial neoformation.

A case of unexpected death in a six-year-old child, who died after a period of non-specific symptoms and clinical signs, is described. The cause of death was a pilocytic astrocytoma of the pontocerebellar angle, rare with regard to location and histology. The authors have reviewed the literature, which was scanty.

[The neuropathy epidemic in Cuba: eight years of investigation and follow-up]. / La epidemia de neuropatía en Cuba: ocho años de estudio y seguimiento.

INTRODUCTION: The authors describes the past eight years, since an epidemic started in Cuba which mainly affected the nervous system, particularly the optic nerve and the peripheral nerves. It is thought to have been the biggest epidemic involving the nervous system this century, although there may be over-diagnosis. The cause is controversial but is probably nutritional imbalance with additional toxic elements also in some cases. At the present time the continuous, low, notification of new cases means that it is endemic. DEVELOPMENT: In this paper we consider the clinical and electrophysiological aspects characteristic of the disorder, the degree of involvement, evolution, incidence and annual follow-up of cases from 1992 to date and their differential diagnosis. We describe the national programme of healthcare for control of the disease in Cuba, the measures designed to reduce the number of patients, most of the results of clinical and epidemiological studies and the possible causes. We also discuss the most likely physiopathological hypotheses.

[Leber's hereditary optic neuropathy and its possible relation to a recent epidemic in Cuba]. / Neuropatía óptica hereditaria de Leber y su posible relación con la reciente epidemia de Cuba.

INTRODUCTION: This century, the greatest epidemic affecting the nervous system was notified in Cuba seven years ago. At the present time the epidemic continues although to a lesser extent. The clinical findings of the illness were mainly bilateral optic neuropathy sometimes accompanied by other symptoms and peripheral neuropathy. The similarity of the optic form of the illness with Leber's hereditary optic neuropathy, and their common risk factors, were obvious from the beginning. PATIENTS AND METHODS: Statistics from the national reference department of neuro-ophthalmology of the Instituto de Neurologia de Cuba were reviewed. From these it was evident that the number of cases of Leber's hereditary optic neuropathy had increased in recent years, coinciding with the period of epidemic and endemic Cuban neuropathy. Many of these patients had previously been diagnosed as having epidemic optic neuropathy. RESULTS: We describe the characteristics of a group of these patients and discuss the differences and possible relationship between the two conditions. In the case of epidemic optic neuropathy, there is strikingly simultaneous loss of vision, less visual changes with much smaller cecocentral scotomas, loss of ganglion fibres of the retina around the papillomacular bundle, a good response to multivitamin treatment, and increased frequency of association with peripheral sensory neuropathy. CONCLUSION: This analysis reinforces the hypothesis that many patients with Leber's hereditary optic neuropathy, which started at the time of the epidemic, were incorrectly classified as suffering from this, and also perhaps their condition worsened due to the toxic nutritional features common to both conditions.

[Emerging risk factors and prevention of atherosclerosis in uraemia]. / Fattori patogenetici emergenti e prevenzione della cardiovasculopatia aterosclerotica nell'uremia.

A high rate of cardiovascular morbidity and mortality in uremic patients was documented early in the 1970s, and this remained unchanged according to more recent European and U.S. American registry report. In large cross-section studies of uremic patients, traditional cardiovascular risk factors such as hypertension and hypercholesterolemic have been found to have low predictive power, while emerging factors such as markers of inflammation and malnutrition, are strongly correlated with cardiovascular mortality. In addition, retained uremic solute such as b-2-microglobulin, advanced glycosylated end product (AGE), homocysteine, cysteine, sulfate may contribute to the pro-atherogenic milieu of uremia. Exhaled alkanes such as isoprene, increased during and after hemodialysis sessions, have been identified as accelerating factors of the atherosclerotic process. In patients with renal disease, even when GFR is still normal, increased sympathetic activity is demonstrable. The rising catecholamines turnover is a risk factor for cardiovascular disease for the high sensitivity of the heart and the arteries to increased sympthetic activity.

[Clinical aspects (especially nephropathologic) and genetic counseling in tuberous sclerosis. Presentation of a case with polycystic kidney]. / Aspetti clinici (in particolare nefropatologici) e consiglio genetico nella sclerosi tuberosa. Presentazione di un caso esordito come rene policistico.

Tuberous sclerosis (TS) represents a relatively frequent inherited disorder of the skin and neurological tissues. Defects of other organs may also be present, but subjects differ significantly in their individual involvement. Usually, white leaf - shaped macules, even though most subtle, are the first precocious sign of the disease in young patients. Other signs tend to appear when the patient grows older. Visceral disorders include renal angiomyolipomata; clinical behavior of these solid tumors is almost always benign. Sometimes the renal lesions present themselves as polycystic kidneys, and may be the earliest sign and the only manifestation of TS, such as the case here described. This unusual form of renal involvement may be a severe potential complication of TS for the possible blood hypertension, recurrent urinary sepsis and chronic renal failure. It is of the utmost importance to search for the classical stigmata of TS in any patient who has cystic renal enlargement as only apparent abnormality. Careful inquiry into the family history cannot be overemphasized. The authors believe that, failing availability of adequate therapy for TS, the role in genetic counseling is to provide as much informations as possible to enable the involved family to make an intelligent decision about future children.

[Anemia and hepatosplenomegaly: search for the protozoon?]. / Anemia ed epatosplenomegalia: cercare il protozoo?

An eight-months-old infant presenting with anemia, hepatosplenomegaly, hypoalbuminemia and polyclonal hypergammaglobulinemia was finally found to be affected by visceral leishmaniasis, after having suspected oncohaematologic problems. Diagnosis was confirmed by bone marrow aspiration. Complete recovery was achieved with antimonial N-methylglucamine (Glucantim) administered IM.

[Report of a case of celiac disease associated with transient erythroblastopenia in pediatric age]. / Descrizione di un caso di malattia celiaca associata ad eritroblastopenia transitoria in età pediatrica.

Authors reports a rare case of association between two pathologies that, even if apparently very different, are both immunologically determined: celiac disease (CD) and transient erythroblastopenia of childhood (TEC). Such an association may be less rare commonly thought and this may be true for other autoimmune diseases, too. Clinical and pathological observations, together with etio-pathogenetic hypotheses, are discussed.

[Trisomy 18 (Edwards syndrome): two case reports]. / La trisomia 18 (sindrome di Edwards): considerazioni su due casi.

Edwards (or trisomy 18) syndrome, with a frequency of about 1 over 8000 births, is commonly characterized by a very short survival; those rare cases reported with ages of 12 months or more, always were demonstrated to be chromosomic mosaicisms. Two affected children with completely different outcomes are here described; the first with a classic short-life evolution, in opposition to the second with long survival, the last a good example of all those various biological, psychological and social support interventions in these circumstances, very rare to match with, for this appears to be a trisomy 18 without mosaicism.

Time-course study of blood pressure over a 20-year period in congenital blindness.

In a prospective study blood pressure was recorded for 20 years in 132 white patients with congenital blindness and in 138 white control schoolchildren. The subjects for both groups were consecutively enrolled without any selection parameters. During the study period a similar proportion of blind patients and controls reported identical consumption of tobacco (above the age of 14 years) and of oral contraceptives (above 21 years) among women. Mean 24-h urinary sodium excretion, collected randomly in both groups, did not differ between the blind patients and the controls. During the 20-year period systolic and diastolic blood pressure rose with age in the control group as expected and much less in blind patients; after 14-17 years the difference was statistically significant. The mean slope of the regression line (beta coefficient) for systolic blood pressure versus age was 1.143 in the blind patients and 1.794 in the controls (P less than 0.001), and for diastolic blood pressure 0.908 in the patients and 1.353 in the school children (P less than 0.001). The mean weight and body mass index increased more in the blind patients than in the controls (difference 5.9 and 2.8 kg, respectively). The results support the hypothesis that low visual and cognitive stress levels determine a lower level of cardiovascular reactivity with ageing.

[Experience with the use of a computerized data processing program on solutions for parenteral nutrition of neonates]. / Esperienza dell'impiego di un "foglio elettronico" computerizzato per l'elaborazione delle soluzioni per nutrizione parenterale nei neonati.

BACKGROUND: The preparation of a PN (parenteral nutrition) solution in neonatal and paediatric Departments is an operation of both critical importance and great difficulty (need of time to do it; complex calculations in order, for example, to subtract takes of nutrients from other sources; etc.). MATERIALS AND METHODS: With the aim to speed such a proceeding, the authors (from a 1000 deliveries/year NICU) developed a computerised system for the preparation of PN mixtures, based on a common and easily available "spreadsheet". RESULTS: The results of its application on a population of preterm babies in which parenteral and enteral (minimal enteral nutrition etc.) feedings were contemporarily provided, are illustrated. Advantages by using this software are the lower cost if compared with other dedicated softwares on sale and its great simplicity and versatility. The program is easy to use eve if different feeding plans are required; the data obtained at the end of the study demonstrated the effectiveness of the software in correctly calculate food components dosages. CONCLUSIONS: Using producer's data on feedings composition and up-to-date parenteral nutrition schedules, a reliable composition of the obtained PN mixtures was eventually achieved, as demonstrated by growth and blood samples.

[Phakomatosis in childhood. Clinical and instrumental observations of 11 cases]. / Le facomatosi in età evolutiva. Osservazione clinica e strumentale di undici casi.

Taking the institution of the Italian Neuroectodermosis Register (Fois, April 1987) as a starting point, the Authors reviewed their own case-records, including six cases of neurofibromatosis and five cases of tuberous sclerosis, observed during the period 1982/88, and stress the need for early diagnosis (unfortunately often neglected), indispensable both for an effective prevention of any pathological involvement and for precise genetic counselling, susceptible of further improvements thanks to more and more advanced diagnostic techniques.

[Feto-maternal transfusion. Description of a case]. / Trasfusione feto-materna. Descrizione di un caso.

Feto-maternal transfusion is an uncommon cause of neonatal anemia due to the passage of fetal blood in maternal circulation, in an amount greater than 150-180 ml. The onset of anemia may be acute or chronic in relation to the speed of blood loss. The case that we report has intermediate features between the acute and chronic form.

Circadian blood pressure changes in patients with chronic renal insufficiency: a prospective study.

Circadian blood pressure (BP) rhythm was prospectively studied by ambulatory 24-h monitoring in normotensive (n = 27) and hypertensive (n = 41) patients with stable progression of chronic renal insufficiency, and in matched control groups (healthy subjects: n = 28 and patients with essential hypertension: n = 47) without renal disease. The follow-up period lasted 24 months. The renal patients showed a disturbance in the 24-h profile of BP, with significantly blunted nocturnal pressure reduction as compared with the respective control groups (p < 0.01 and p < 0.001, respectively). In addition to the rearrangement of circadian rhythm, the normotensive and hypertensive renal patients displayed a wider distribution of systolic and diastolic BP values and a greater nocturnal variability. Among the normotensive and hypertensive patients with chronic renal insufficiency, a significant correlation was found between the decline in creatinine clearance over the 24-month period and the average nighttime diastolic BP (r = 0.526; p < 0.01 and r = 0.613; p = 0.001, respectively) and nocturnal diastolic fall (r = 0.612; p < 0.001 and r = 0.496; p < 0.01, respectively). These data offer support for the view that renal normotensive patients are exposed to a relative hypertension at nighttime and that renal hypertensive subjects can be underestimated in their hypertensive status if the measurement of BP is confined to daytime. In both groups, nocturnal BP overload can accelerate the progression rate of renal insufficiency.

Blood pressure in nuns in a secluded order: A 30-year follow-up.

The powerful effect of psychosocial and acculturating influences on population blood pressure trends seems to be confirmed, through longitudinal observations, in the nuns in a secluded order. After initial observations had been made on culture, body form, blood pressure, diet, and other variables in 144 nuns and 138 lay women, included as a control group, a 30-year follow-up study was undertaken. Most striking were opposite trends noted between the two groups in blood pressure trend. During the follow-up period, blood pressure remained remarkably stable among the nuns. None showed an increase in diastolic blood pressure over 90 mm Hg. By contrast, the control women showed the expected increase in blood pressure with age. This resulted in a gradually greater difference (delta>30/15 mm Hg) in systolic and diastolic blood pressure between the two groups, which was statistically significant. In addition, cardiovascular morbidity and mortality, expressed as the outcome of fatal and nonfatal events, were different in the two groups. They were significantly more common in the lay women than in the nuns. Comparisons between survival curves were statistically significant (p = 0.0043 for fatal events; p = 0.0056 for nonfatal events) between the two groups. In conclusion, it seems reasonable to attribute much of the difference in blood pressure and cardiovascular events, to the different burden in psychosocial factor and to the preserved peaceful lifestyle of the nuns.

Antibodies against hepatitis C virus in hemodialysis patients in the central Italian region of Umbria: evaluation of some risk factors.

The epidemiology of non-A, non-B hepatitis (NANBH) is still incomplete. To define the prevalence of antibodies against the main causative agent of NANBH, the hepatitis C virus (HCV) and the role of some risk factors, we tested sera from 269 patients on chronic dialysis at the hemodialysis units in our region in central Italy. We utilized the recently developed serological assay. Twenty-nine hemodialysis patients (13.3%) and 3 peritoneal dialysis patients (4.8%) were anti-HCV positive. Of these, 13 (40.6%) had antibodies to hepatitis B core antigen (anti-HBc) indicating prior hepatitis B infection. The anti-HCV seropositive patients had been on dialysis longer than the seronegative ones; they had received more transfusions than the others but without a significant difference. The prevalence rate of anti-HCV was statistically significantly higher among hemodialysis patients utilizing the same dialysis equipment for the previous 12 months.

Blood pressure trend and cardiovascular events in nuns in a secluded order: a 30-year follow-up study.

30-year data are presented on blood pressure and cardiovascular morbidity and mortality for 144 nuns living in a secluded order in six nunnerlie in Umbria, central Italy and 138 lay women from the same region. There were no significant differences at baseline regarding age, blood pressure, body mass index, race, ethnic background, menarche, family history of hypertension or 24-hour urinary sodium excretion. None of the women were smokers and none took birth control pills nor did they use estrogen replacement therapy. During the observation period blood pressure remained remarkably stable among the nuns. None showed a rise in diastolic blood pressure to above 90 mm Hg. On the contrary the lay women showed the expected rise in blood pressure with age. This resulted in a gradually greater difference (delta > 30/15 mm Hg) in blood pressure between the two groups, which was statistically significant. There were 31 fatal and 69 non-fatal cardiovascular events during the 30 years of follow-up. These were significantly more common in the lay women, 10 vs. 21 fatal and 21 vs. 48 non-fatal in the nuns and lay women respectively. It appears reasonable to assume that the difference in psychosocial stress is the main underlying factor for the observed findings.

Volatile alkanes and increased concentrations of isoprene in exhaled air during hemodialysis.

In this study we examined breath volatile hydrocarbon concentrations in exhaled air of hemodialysis patients. We assessed both C(2)-C(5) alkanes - among them ethane and pentane the production of which in man is essentially due to the action free radicals exert on polyunsaturated fatty acids - and isoprene, an unsaturated hydrocarbon the biosynthesis and biological effects of which are the subject of controversy and mounting interest. Twenty patients were studied. Evaluation was performed intrapatient in the breath of patients with chronic renal failure, before and after dialysis (20 patients) and, in the same cases, during hemodialytic treatment (10 patients). Breath concentrations of these volatile hydrocarbons, determined before dialysis, were not different from those of normal subjects. Dialysis did not modify the levels of the C(2)-C(5) saturated hydrocarbons ethane, propane, butane and pentane. Instead, there was a marked increase in isoprene in all patients (basal values rose by a mean of 270%). Since isoprene was not present in the fluids or filters used for dialysis and there were only traces in the ambient air, the isoprene must have been produced endogenously during hemodialysis. As no situation has previously been reported to increase endogenous production of isoprene in humans, patients in hemodialysis offer a unique opportunity to investigate in depth the medical, biological and toxicological aspects of isoprene.