Search

During a multicentre study of chronic hepatitis in childhood diagnosed by biopsy, the spectrum of the disease has been evaluated in 196 consecutive patients, including 157 from Northern Italy and 39 from Southern Italy. Only 31% of patients in the former group and 27% in the latter were symptomatic when first seen: the majority of cases being seen after familial screenings for hepatitis B virus (HBV) markers or during intercurrent infections, thus suggesting that the frequency of chronic hepatitis in childhood might be largely underestimated in our area. In Southern and Northern Italy 83% of symptomatic and 95% of asymptomatic patients were hepatitis B surface antigen (HBsAg) positive in serum; only 15 (8.3%) of these children were born to mothers known to be HBsAg positive at delivery, but a high circulation of HBV was found in their families: in fact more than 65% of household contacts in Northern Italy and more than 90% in Southern Italy had serological evidence of past or ongoing HBV infection. These data indicate that, although familial screenings for HBV could have enhanced the percentage of HBsAg positive asymptomatic cases, chronic hepatitis in Italian children is mainly caused by HBV infection acquired in the familial setting through horizontal transmission. Such findings also emphasise the importance of mass vaccination of infants as the most effective means to prevent chronic type B hepatitis in childhood in our area. Among HBsAg positive children 55% had histological features of chronic active hepatitis and 85% were hepatitis Be antigen (HBeAg) positive in serum. Anti-HBe positive hepatitis was significantly more frequent in Southern than in Northern Italy in parallel with the significantly higher prevalence (17%) of hepatitis delta virus infection in that area. Of the 16 HBsAg negative cases included in the study three had autoimmune hepatitis, three Wilson's disease, one alpha1 antitrypsin deficiency, and nine had cryptogenic hepatitis, often associated to mild liver lesions resembling those seen in our adult patients with chronic non-A, non-B hepatitis unrelated to percutaneous exposure. [ABSTRACT FROM PUBLISHER]

ABSTRACT- During a prospective follow-up study of 65 children with chronic hepatitis B, the HBcAg expression pattern in the liver was investigated, by immunofluorescence, in relation to biochemical and histological features and to the evolution of the disease. HBcAg was detected in 50 (77%) cases, with a diffuse pattern of distribution (more than 60% positive nuclei) in 20 cases, and with a focal pattern (less than 40% positive nuclei) in 30 cases. The diffuse pattern significantly prevailed in younger children, while the focal pattern was more frequent in children beyond 10 years of age. Transaminase levels were higher and histological features of activity were more frequent in patients with the focal pattern than in those with a diffuse pattern. Twenty-five hepatitis B e antigen (HBeAg) positive children, all initially HBcAg positive, were followed prospectively for 24 months. Seroconversion to anti-HBe, with subsequent normalization of transaminases, occurred in 72% of cases with a focal pattern, but only in 21% of those with a diffuse HBcAg pattern. In four patients transition from the diffuse to the focal pattern was observed during follow-up. These results suggest that, in children with chronic hepatitis B, the diffuse HBcAg pattern in the liver might reflect an earlier phase of infection. In HBeAg positive cases the focal HBcAg pattern may represent a prognostic marker of short-term favourable evolution of the disease. [ABSTRACT FROM AUTHOR]

Nine children born to HBsAg positive mothers, who became chronic HBsAg carriers with associated liver disease, were followed for five to 10 years. Five children with active hepatitis or active cirrhosis at presentation achieved complete remission within six years, while three HBeAg positive patients with minimal histological lesions remained unchanged. [ABSTRACT FROM AUTHOR]

A 3 year old boy who had chronic active hepatitis type B with features of ongoing liver damage and active virus replication, developed acute membranous glomerulonephritis two years after the clinical onset of liver disease, when both hepatitis B e antigen and antibody were detectable in serum. After withdrawal of short term steroid treatment and resolution of hepatitis B virus replication, both glomerulonephritis and chronic hepatitis went into remission. Some months later hepatitis B surface antigen was no longer found in serum. [ABSTRACT FROM AUTHOR]

Guillain-Barré syndrome (GBS) may be complicated by severe hypertension (HT) and in turns severe HT can occur with neurological damage mimicking a GBS, so that underlying causes should be investigated. We describe a case of a 62-year-old woman presented to the emergency department for hypertensive crisis with symmetric flaccid paralysis, hypotonia and hyporeflexia of both upper and lower limbs. Brain computed tomography, magnetic resonance imaging and lumbar puncture were normal. Laboratory investigations revealed severe hypokalemia, renal failure, liver impairment, rabdomyolysis, metabolic alkalosis, and low plasma renin and aldosterone levels. Continuous potassium replacement led to complete clinical resolution. A detailed history revealed chronic intake of 250 g/day black liquorice. Hypokalaemic muscle weakness may simulate a GBS. When serum potassium level falls below 2.5 mmol/l, rhabdomyolysis may occur. In this clinical case, an apparent mineralocorticoid excess syndrome was induced by chronic ingestion of liquorice. This latter contains the glycyrrhetic acid that inhibits the enzyme 11-β-hydroxysteroid dehydrogenase enzyme type-2 leading an aldosterone-like effect and causing hypertension, hypokalemia, metabolic alkalosis and low renin values. The clinical presentation is similar to that observed in the primary aldosteronism, but in this syndrome plasma aldosterone levels are low rather than elevated as in primary aldosteronism. Liquorice-induced hypertension with severe hypokalemia and rhabdomyolysis is a rare condition and the initial presentation with acute muscle paralysis is still more unusual. Before performing instrumental examinations in middle-aged peoples with hypertension crisis and neurological impairment, a detailed clinical history is mandatory.

Introduction: Resistant hypertension is a clinical condition in which blood pressure (BP) control is not achieved under a pharmacological therapy including a diuretic and at least two additional antihypertensive drug classes., Aim: To discuss an unusual presentation of uncontrolled hypertension despite multiple anti-hypertensive medications., Methods and Results: A 46-year-old woman presented with resistant hypertension (HT) and with a long history of polydipsia, polyuria, weight loss and psychiatric symptoms (sudden onset of personality disorder with free anxiety, negativism and asthenia) unsuccessfully treated with antidepressant drugs. Tests for secondary HT showed a marked increase of serum renin and aldosterone both in clinostatic (342 pg/ml and 907 pmol/l, respectively) and orthostatic posture (351 pg/ml and 2845 pmol/l, respectively), hypokalemia (2.9 mmol/l) and macroalbuminuria (431 mg/day). Diagnostic examinations also revealed a focal stenosis of approximately 70 % of the proximal right renal artery with post-stenotic dilation. After percutaneous balloon angioplasty and stent implantation, BP was normalized with 5 mg/day amlodipine and psychiatric symptoms suddenly disappeared., Conclusions: Psychopathological symptoms are rare at the onset of hyperaldosteronism, and their aetiology is not well defined. A proper diagnostic and therapeutic process is mandatory in order to get the recommended therapeutic targets in short-midterm improving long-term prognosis. We also suggest not considering depressed or treat with antidepressant agents a young hypertensive subject with uncontrolled hypertension despite multiple anti-hypertensive medications without having ruled out a secondary form of hypertension.

Pheochromocytoma (PH) and paraganglioma (PG) are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and the sympathetic ganglia, respectively. Although are unusual cause of hypertension (HT) accounting for at most 0.1-0.2 % of cases, they may lead to severe and potentially lethal hypertensive crisis due to the effects of the released catecholamines. However, both PH and PG may be asymptomatic as ~30 % of subjects are normotensive or have orthostatic hypotension and in these cases the 24 h ambulatory blood pressure (BP) monitoring is an important toll to diagnose and treat HT. HT treatment may be difficult when PH or PG occurs in pregnancy or in the elderly subjects and in these cases a multidisciplinary team is required. When surgical excision is mandatory the perioperative management requires the administration of selective α1-adrenergic blocking agents (i.e., doxazosin, prazosin or terazosin) followed by a β-adrenergic blockade (i.e., propranolol, atenolol). This latter should never be started first because blockade of vasodilatory peripheral β-adrenergic receptors with unopposed α-adrenergic receptor stimulation can lead to a further elevation of BP. Although labetalol is traditionally considered the ideal agent due to its α- and β-adrenergic antagonism, experimental studies do not support its use in this clinical setting. As second regimen, the administration of vasodilators as calcium channel blockers (i.e., nicardipine, nifedipine) may be required to control BP. Oral and sublingual short-acting nifedipine are potentially dangerous in patients with hypertensive emergencies and are not recommend. The latest evidences into the diagnosis and treatment of hypertensive crisis due to PH and PG are reviewed here.

Arterial hypertension (HT) is age dependent and, with the prolongation of life expectancy, affects more and more elderly people. In the elderly, HT is a risk factor for organ damage and cardiovascular (CV) events. Both pharmacologic and nonpharmacologic reduction of blood pressure (BP) is associated with a corresponding decrease in systolic-diastolic or isolated systolic HT. Clinical trials have shown that BP lowering is associated with a decrease in stroke and other CV events. Therefore, BP reduction per se appears more important than a particular class of antihypertensive drugs. The benefit of antihypertensive treatment has been confirmed up to the age of 80 years, remaining unclear in the octogenarians. The benefit in lowering diastolic BP between 80 and 90 mmHg is well established, while that of lowering systolic BP below 140 mmHg requires further confirmations.

This review describes the therapeutic approach of endocrine arterial hypertension in clinical practice. In mineralocorticoid-related hypertension, adrenalectomy is the treatment of choice for aldosterone-producing adenomas and monolateral primary aldosteronism, whereas pharmacologic blood pressure (BP) control is indicated for the other forms of primary aldosteronism such as bilateral adrenal hyperplasia. Spironolactone is the drug of choice, but intolerable side effects limit its use; amiloride or eplerenone are a valid alternative. If BP remains uncontrolled, angiotensin converting enzyme inhibitors (ACE-I), angiotensin II receptor antagonists (AII-RA) and calcium channel blockers (CCB) may be added. Hypertension accompanying Cushing's syndrome can be approached with surgery, but antihypertensive treatment both pre- and postoperative is required as well. Eplerenone, AII-RA and ACE-I are indicated, while peroxisome proliferator activated receptor upsilon agonists may help for the insulin resistance syndrome. Drugs that suppress steroidogenesis should be used with care because of their serious side effects. Subjects with catecholamine-dependent hypertension due to a neuroendocrine neoplasm need to undergo preoperative alpha-adrenergic blockade with phenoxybenzamine or doxazozine. When adequate alpha-adrenergic blockade is achieved, beta-adrenergic blockade with low dose propranolol may be added. If target BP is not achieved, CCB and/or metyrosine are indicated. Laparoscopic adrenalectomy is the procedure of choice for solitary intra-adrenal neoplasms <8 cm. Acute hypertensive crises that may occur before or during surgery should be treated intravenously with sodium nitroprusside, phentolamine, nicardipine or labetalol. For malignant neoplasms, chemo- and radiopharmaceutical therapy may be considered.

Endocrine arterial hypertension (EAH) a condition in which hormone excess results in clinically significant hypertension is a rare cause of hypertension. However in the last years its prevalence has increased, mostly due to the improvement of diagnostic work-up. In clinical practice, hypertensive subjects with suspicion of EAH currently undergo hormonal screening of the renin-aldosterone and catecholamines and glucocorticoids excess. This paper reviews current understanding for earlier recognition of the main forms of EAH and discusses screening laboratory methods and localization techniques that have enhanced the clinician's ability to make the diagnosis of EAH. Primary aldosteronism (PA) has recently been recognised as the most frequent cause of EAH. The aldosterone to renin ratio (ARR) is a highly recommended screening test for PA. When ARR is increased, confirmatory tests as saline infusion or fludrocortisone suppression are required. Differential diagnosis of PA requires adrenal gland imaging by computed tomography (CT) or magnetic resonance imaging (MRI), biochemical testing of the aldosterone response to posture, and selective adrenal venous sampling to differentiate unilateral aldosterone-producing adenoma from bilateral hyperplasia. Hypertension is frequently found in endogenous Cushing's Syndrome (CS). Twenty-four-hour urinary free cortisol measurement is the gold standard for the diagnosis of CS, but it must be confirmed by the overnight dexamethasone suppression test. CT and MRI are the primary imaging studies to perform, while scintigraphy is a useful confirmatory method. The most specific and sensitive diagnostic test for catecholamine-producing neoplasms is determination of urinary metanephrine levels; the neoplasms can be located by CT, MRI and metaiodo-benzylguanidine scintigraphy.

The authors assessed the prevalence of pulmonary arterial hypertension (PAH) in patients with hyperthyroidism and evaluated the response to treatment of the thyrotoxicosis. They assessed the pulmonary artery systolic pressure (PASP) at rest (estimated by echocardiography) in 23 consecutive patients diagnosed with hyperthyroidism due to Graves' disease or toxic multinodular goiter. Twelve of 23 patients (52%) did not show antithyroglobulin and antithyroperoxidase antibodies. Seventeen patients were followed up for at least 9 months after achieving a stable euthyroid status. Fifteen (65%) patients demonstrated PAH at admission. Four patients were lost to follow-up; therefore they were able to evaluate 17 patients serially with echocardiography. Sixteen patients normalized their PASP value: 13 after methimazole, 2 after total thyroidectomy, and 1 after (131)I treatment. In 1 patient no significant change in PASP was observed. This patient experienced an acute myocardial reinfarction during follow-up. They found a higher prevalence than that previously reported in observational studies. In addition, they demonstrated that the PAH reverses after correction of hyperthyroidism. Elevated PASP at rest on echocardiography may be considered a frequent finding of thyrotoxicosis. Moreover, the data seem not to support an autoimmune pathogenesis for PAH.

We reported here the data on minimally invasive radio-guided parathyroidectomy (MIRP) in a large group of 253 patients enrolled from the whole series of 355 consecutive patients affected by primary hyperparathyroidism (P-HPT) referred to our center. On the basis of preoperative imaging including Sestamibi scintigraphy and neck ultrasound (US), 263 patients (74% of the whole series) with evidence of a solitary parathyroid adenoma (PA) and a normal thyroid gland were addressed to MIRP and in 253 (96%) of them this minimally invasive neck exploration was successfully performed. The MIRP protocol developed in our center consisted of a very low 1 mCi Sestamibi injection in the operating room a few minutes before the start of intervention, thus minimizing the radiation exposure dose to the patient and personnel. No major intraoperative complication was recorded in patients treated by MIRP and only a transient hypocalcemia in 8.5% of cases. The mean duration time for MIRP was 35 min and the mean hospital stay 1.2 days. Local anesthesia was also performed in 62 patients, 54 of whom were elderly patients with concomitant invalidating diseases contraindicating general anesthesia. No HPT relapse was observed during subsequent follow-up. The gamma probe was used also during bilateral neck exploration in the group of 92 patients excluded from MIRP. The most frequent cause of exclusion from MIRP in our series was the presence of concomitant Sestamibi avid thyroid nodules (68.5% of cases) that can give false positive results at radio-guided surgery. In conclusion, MIRP is an effective treatment in patients with a high likelihood of a solitary PA and a normal thyroid gland at scintigraphy and US so that an accurate preoperative localizing imaging is required for MIRP. A low 1 mCi Sestamibi dose appears sufficient to perform MIRP. Patients with concomitant Sestamibi avid thyroid nodules should be excluded from MIRP.

Secondary pulmonary hypertension (sPH) may develop as a result of few systemic diseases as endocrine diseases, HIV infection, collagen diseases, liver and hematological disorders. In this review we discuss the role of systemic diseases in inducing PH starting from a pathological classification.

Retroperitoneal fibrosis is a rare disease of uncertain pathogenesis. However, its possible association with several immunopathologic conditions, the possibility of systemic involvement by the fibrous process, the presence of various autoantibodies, and the frequent response to immunosuppressive treatment all support an autoimmune pathogenesis. Riedel's thyroiditis is a rare disease the pathogenesis of which is also thought to be immune-mediated based on its optimal response to steroids; Riedel's thyroiditis is also frequently reported in association with retroperitoneal fibrosis. We describe here two cases of autoimmune thyroid disease associated with retroperitoneal fibrosis, the first with features of primary myxedema, the second of primary thyrotoxicosis. Histology of retroperitoneal fibrosis is documented and it is compatible with an immunopathologic condition. Thus, these two cases add further support to the hypothesis of an autoimmune pathogenesis of retroperitoneal fibrosis and indicate the importance of carefully monitoring for the development of other autoimmune disorders, i.e., of the thyroid gland, in patients with retroperitoneal fibrosis.

Background: Nondiabetic patients with advanced coronary artery disease (CAD) were assessed for lipid peroxidation, LDL modifications and insulin action. Twenty-four patients and 10 normal controls were studied., Methods: Insulin tolerance test (Kitt), glucose, insulin lipoproteins, electronegatively charged, modified, low density lipoproteins (LDL-) and the thiobarbituric acid reactivity (TBARS), as an index of lipid peroxidation, were determined., Results: No difference was observed in insulin action (determined by insulin tolerance test) between patients with CAD (3.31 +/- 0.28%/min; range 0.73-6.13) and normal controls (3.59 +/- 0.42; range 1.76-6.06). The percentage of modified, electronegative LDL (LDL -) was higher in patients with CAD (0.5 +/- 0.48%; range 1.3-9.2) than that of controls (2.80 +/- 0.33; range 1.00-4.00; p = 0.013). TBARS were significantly (P = 0.043) higher in CAD patients (3.49 +/- 0.17 nmol/ml; range 2.4-5.5) than normal controls (1.47 +/- 0.12; range 1.07-2.10). A significantly negative correlation was observed between Kitt and TBARS (r= - 0.48; p = 0.016), and a significant (r = 0.46; p = 0.022) positive correlation was observed between plasma glucose and TBARS. On the contrary no correlation has been observed between LDL- and TBARS., Conclusions: We conclude that in patients with advanced coronary artery disease: A) there are increased circulating levels of modified low density lipoprotein; B) there is evidence of increased lipid peroxidation. This latter process is significantly influenced by the degree of insulin action.

We evaluated the prevalence and the clinical features of liver cirrhosis associated with chronic hepatitis B virus (HBV) infection in a prospective study of 292 consecutive children who were chronic HBsAg carriers with increased aminotransferase activity. Liver histologic changes at presentation were consistent with cirrhosis in 10 (3.4%) patients (100% boys, mean age 4.0 +/- 3.3 years). In none of the remaining children, including 166 with histologic evidence of chronic active hepatitis, did the condition progress to cirrhosis during an observation period of 1 to 10 years. This lack of progression suggests that cirrhosis is an early complication of chronic HBV disease in some patients. A higher prevalence of delta infection and increased incidence of blood transfusions were observed in patients with cirrhosis, supporting the hypothesis that superinfection with delta or non-A, non-B agents may play a synergistic role. Eight of 10 patients had histologic features of disease activity at presentation, although only two had symptoms. During follow-up, persistence of disease activity was observed only in the three delta antigen-positive patients. None of the patients with inactive cirrhosis have developed signs of liver failure or portal hypertension.

During a seven-year survey of acute symptomatic viral hepatitis in Padua (Northern Italy), the epidemiological features of hepatitis A were evaluated in 207 consecutive patients (120 males, mean age 22.7 +/- 11.4 years). The annual attack rate of the disease decreased significantly (p less than 0.05) between 1978 and 1979 (0.11/1000 inhabitants) and 1981 and 1984 (0.04-0.03/1000 inhabitants), mainly due to its declining prevalence in the pediatric age. In parallel with the shifting of hepatitis A towards adulthood, single sources of infection, mainly associated with adult life-style such as foreign travel and raw shellfish ingestion, have become more and more prominent. The spread of drug abuse has not influenced the epidemiology of hepatitis A in our area.

During a prospective study of acute symptomatic viral hepatitis, started in 1978, 664 consecutive adult patients, including 223 drug abusers, fulfilled the diagnostic criteria (anti-HBc IgM positivity) for acute type B hepatitis. In order to evaluate the outcome of the disease, 443 patients were followed for up to 12 months after the onset. 2.4% of the infections became chronic; the rate did not significantly differ between drug addicts and non-drug abusers, suggesting that chronic hepatitis is a rare complication of acute symptomatic hepatitis type B. Ongoing liver damage after clearance of HBsAg from serum was observed in drug abusers only (14% of the cases). Clinical, biochemical and virological features of the acute phase in patients with ongoing infection were compared with those of uncomplicated cases. Anicteric hepatitis and lower transaminase values were significantly (p less than 0.05) associated to a chronic evolution of the disease, as well as a higher prevalence of HBV-DNA, DNA polymerase and HBcAg positivity in serum. Testing HBV-DNA and DNA polymerase early in the course of the infection appeared to be of high predictive value for the subsequent outcome of the illness.