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M Bosio

Publications and source records attributed to M Bosio.

At least 19 recordsLinked to original sources
PubMed2002

Emergency system prospective performance evaluation for cardiac arrest in Lombardia, an Italian region.

BACKGROUND: The aim of this research is to evaluate quality of out-of-hospital medical services in our country, using performance indicators and a new computerised database. METHODS: (a) EXPERIMENTAL DESIGN: Data were collected prospectively in three emergency dispatch centres for 90 days. Follow-up was evaluated at 1 day and 1 month after the event. This paper presents data on the cardiac arrest cohort only. (b) SETTING: Three emergency dispatch centres in Lombardia. (c) PATIENTS: One hundred and seventy-eight patients in non-traumatic cardiac arrest were enrolled. (d) INTERVENTIONS: None. The study was observational only. RESULTS: Mean interval between phone call and arrival on scene was 8.5+/-3.5 min. BLS manoeuvres were carried out from bystanders only in 15% of the cohort; this was associated with significant mortality reduction (85.7 versus 95.8%, chi(2) P<0.05). One hundred and thirty-three patients (75%) received assistance from BLS crews while only 45 patients (25%) were assisted by ALS medical personel, with a significant mortality reduction (ALS deaths 86.7%, BLS deaths 97%). Total 24 h survival was 9% and survival at 1 month declined to 6.17%. CONCLUSIONS: Quality monitoring produces objective information on interventions and outcomes. Only with this information, is it possible to implement improvement programmes that are planned according to the data presented.

Aged↗
PubMed2002

Detection of posterior urethral valves with voiding cystourethrosonography with echo contrast.

PURPOSE: Cystosonography with echo contrast is an imaging modality developed to avoid x-ray exposure during detection of vesicoureteral reflux. The main role of cystosonography has been limited to screening for reflux in the female and for followup of both sexes. In males the radiographic voiding cystourethrogram is still considered the gold standard for urethral evaluation. We determined whether cystosonography during the voiding phase (voiding cystourethrosonography) can adequately visualize the male urethra and differentiate the normal from the obstructed urethra. MATERIALS AND METHODS: 100 males underwent cystosonography with echo contrast to detect vesicoureteral reflux. During the voiding phase the urethra was studied with ultrasound. The transperineal sagittal approach was chosen because the bladder neck and proximal part of the urethra were better visualized with the probe maintained coaxial to these structures. In every case in which ultrasound urethral imaging was considered abnormal a radiographic voiding cystourethrogram was performed for comparison, and the urethra was directly observed with cystourethroscopy. All patients with normal urethral imaging have been followed for 12 to 54 months (mean 32). RESULTS: Voiding cystourethrosonography visualized the voiding phase in all patients studied with the transperineal ultrasound approach. Of the 100 boys 8 were correctly diagnosed with posterior urethral valves. None of those with normal ultrasound imaging showed clinical signs suggestive of urethral obstruction and remained asymptomatic. CONCLUSIONS: The male posterior urethra can be effectively visualized with voiding cystourethrosonography, which can differentiate normal from the pathological obstructive urethral findings. This new approach can be recommended as the primary imaging modality to detect vesicoureteral reflux for both sexes, limiting the role of conventional fluoroscopic voiding cystourethrogram only to confirmation of a pathological finding in the male patient with a suspected obstructed urethra.

Child↗
PubMed1999

A familial syndrome due to Arg648Stop mutation in the X-linked renal chloride channel gene.

We describe a familial syndrome in two brothers who were investigated after the casual discovery of tubular proteinuria in their 1st month of life. During a follow-up of 20 and 11 years, respectively, the two children grew well and were asymptomatic, but developed the same biochemical abnormalities, i.e., tubular proteinuria and hyperphosphaturia, progressive decrease in serum phosphorus below the normal values for age, and an increase in serum 1,25-dihydroxyvitamin D levels over normal values. Moreover, hyperabsorptive hypercalciuria and systemic osteopenia developed and progressively worsened. In both children, at a different age, medullary nephrocalcinosis appeared. The oldest boy suffered a progressive decrease in urinary concentration ability and in glomerular filtration rate. Oral phosphate supplementation led to reversal of all biochemical abnormalities, with the exception of decreased phosphate tubular reabsorption and tubular proteinuria. With long-term phosphate supplementation, a normal bone mass was reached, while progression of nephrocalcinosis was arrested and impairment of renal function was slowed down. In a family study (siblings and parents), the only detectable abnormality was microglobinuria in the mother, thus suggesting a X-linked inheritance of this disorder. In the two probands a mutation within the renal chloride channel gene (CLCN5) was discovered.

Child↗
PubMed1998

Cystosonography with echocontrast: a new imaging modality to detect vesicoureteric reflux in children.

BACKGROUND: Conventionally, vesicoureteric reflux (VUR) is assessed by fluoroscopic micturating cystourethrography (MCU) or direct or indirect radionuclide cystography (RNC). There is preliminary experience with contrast-enhanced sonography for the detection of VUR. OBJECTIVE: To evaluate the usefulness of contrast-enhanced ultrasonography using galactose suspension as an echogenic contrast medium. MATERIALS AND METHODS: In this study a galactose suspension was instilled into the bladder in 58 neonates, infants and children along with normal saline to detect the presence and the severity of VUR through the enhanced US signal. The results were compared with those of MCU and RNC. One ml/kg of echocontrast in the neonatal age group, and 0.5 ml/kg thereafter, were slowly instilled through a 4-F catheter after preliminary supine and prone urinary tract US. Indications for the investigation were antenatally diagnosed pyelectasis (21 cases) and pyelonephritis (37 cases). In 38 patients echocontrast cystosonography (ECS) was performed before, and in 20 patients after, MCU or RNC. RESULTS: ECS detected 76 refluxing units in 50 patients. In eight patients, no VUR was shown, and none of these developed a urinary tract infection in 18 months of follow-up. In 43 patients, MCU or RNC detected 62 refluxing units, while in 15 no VUR was shown. Taking MCU as the gold standard and using the same grading scale, the sensitivity of ECS was 100 %. No side effects were observed. Several urinary tract abnormalities were detected by ECS. The male urethra was studied by ECS, both by retrograde infusion and during micturition. CONCLUSIONS: ECS is a promising imaging technique for detecting and grading VUR without exposing patients to ionising radiation.

Child↗
PubMed1998

Congenital reflux nephropathy: a follow-up of 108 cases diagnosed perinatally.

OBJECTIVE: To determine: (i) the proportion of vesicoureteric reflux (VUR) associated with congenital renal damage and whether it can be severe enough to cause renal impairment from birth: (ii) to evaluate the distribution of males and females affected; and (iii) to describe the course of congenital damage in the first years of life. PATIENTS AND METHODS: A total of 108 children (76 male and 32 female, M:F 2.3:1), whose VUR was diagnosed before any infection, were followed from birth for a mean (range) of 4.3 (1-10) years. Renal damage was defined by serum creatinine concentration, creatinine clearance and renal imaging (ultrasonography and renal scintigraphy) performed within the first month of life and periodically thereafter. RESULTS: Of the 108 children, 58 had bilateral and 50 unilateral reflux (total number of refluxing units, 166). High-grade VUR (grade > or = 4) was found in 96 (58%) refluxing renal units (RRUs). Males had a prevalence of bilateral severe (> or = grade 4) reflux (M:F 5.2:1), while in those wit unilateral VUR, the M:F ration was 1.5:1. At birth, mild to moderate damage was present in 56 (36%) RRUs and only associated with VUR of grade > or = 3. Bilateral reflux of grade > or = 4 was associated with congenital moderate/severe renal failure in nine neonates (seven males). In infants with grade > or = 4 VUR who underwent surgical correction, VUR resolved in 92% of cases. In infants with VUR of grade > or = 4 followed medically, the reflux spontaneously resolved in 42% and ameliorated in 16% after 18 months. Serial renal scans during the follow-up showed no progression of renal damage. CONCLUSIONS: VUR diagnosed at birth on prenatal ultrasonography is associated with congenital damage, with males affected more often than females. The damage involves both kidneys in a consistent proportion and is an important cause of chronic renal impairment from birth. It does not progress in the first years of life if infections are prevented. It is suggested that males with this condition may constitute a major group at risk of developing chronic renal failure in later life.

Child↗
PubMed1997

Characterisation of renal chloride channel, CLCN5, mutations in hypercalciuric nephrolithiasis (kidney stones) disorders.

Mutations of the renal-specific chloride channel (CLCN5) gene, which is located on chromosome Xp11.22, are associated with hypercalciuric nephrolithiasis (kidney stones) in the Northern European and Japanese populations. CLCN5 encodes a 746 amino acid channel (CLC-5) that has approximately 12 transmembrane domains, and heterologous expression of wild-type CLC-5 in Xenopus oocytes has yielded outwardly rectifying chloride currents that were markedly reduced or abolished by these mutations. In order to assess further the structural and functional relationships of this recently cloned chloride channel, additional CLCN5 mutations have been identified in five unrelated families with this disorder. Three of these mutations were missense (G57V, G512R and E527D), one was a nonsense (R648Stop) and one was an insertion (30:H insertion). In addition, two of the mutations (30:H insertion and E527D) were demonstrated to be de novo, and the G57V and E527D mutations were identified in families of Afro-American and Indian origin, respectively. The G57V and 30:H insertion mutations represent the first CLCN5 mutations to be identified in the N-terminus region, and the R648Stop mutation, which has been observed previously in an unrelated family, suggests that this codon may be particularly prone to mutations. Heterologous expression of the mutations resulted in a marked reduction or abolition of the chloride currents, thereby establishing their functional importance. These results help to elucidate further the structure-function relationships of this renal chloride channel.

Adult↗
PubMed1996

[Psychogenic urinary retention in childhood. A severe case treated by an integrated global approach].

Micturating dysfunctions with urinary retention and with diurnal and nocturnal enuresis in children sometimes have a psychogenic genesis. They can appear during the period of development of complete control of micturition. A late recognition of this condition makes the prognosis worse, since high pressure and infections in the urinary tract can cause end-stage renal failure. Here we describe a dramatic case of a 3 year-old boy affected by a psychogenic urine and faecal retention with recurrent pyelonephritis, that was favourably treated for five years by an integrated approach involving clinicians, psychologist, educational and social operators and adoptive parents.

Child, Preschool↗
PubMed1994

Postpheochromocytoma erythropoietin-dependent erythrocytosis. A postischemic mechanism?

PURPOSE: Erythropoietin-dependent pure erythrocytosis (EDPE) is a rare disorder caused by idiopathic hypererythropoietinemia. We describe a 13-year-old girl who developed an EDPE-like erythrocytosis after removal of an adrenal pheochromocytoma. PATIENTS AND METHODS: As occurs in EDPE, this post-pheochromocytoma erythrocytosis was associated with a high serum erythropoietin (s-Epo) level that maintained physiological regulation. RESULTS: Phlebotomies produced a three- to sixfold increase of s-Epo, and a 6-week course of theophylline caused a decrease of both s-Epo and hemoglobin. CONCLUSIONS: We hypothesize that the intense and prolonged pheochromocytoma-induced renal ischemia before surgery could be the cause of this unique case of erythrocytosis.

Adolescent↗
PubMed1993

[The Schönlein-Henoch syndrome with severe multisystemic involvement].

A case of Henoch-Schönlein purpura (HSP) characterized by several unusual complications and exceedingly prolonged course is reported. A 6-year-old boy, hospitalized with a typical clinical picture of HSP, developed after a few days a severe gastrointestinal vasculitis leading to digestive hemorrhages and food intolerance. The treatment included continuous gastrointestinal infusion, parenteral nutrition, and corticosteroids during the exacerbations of symptoms. Intestinal ultrasonography showed diffuse submucous edema and widespread mural hematomas; hydrops of the gallbladder and edematous swelling of the pancreas were also detected. A gastro-duodenoscopy revealed diffuse and severe mucosal vasculitis with ulcers and petechial lesions. One week after admission the occurrence of weight gain, hyponatremia, and hypoprotidemia in the absence of proteinuria suggested a protein-losing enteropathy. The elevation of serum and urine amylases was consistent with pancreatic involvement. The course was characterized by recurrent exacerbations of gastrointestinal manifestations; symptoms subsided slowly to such a degree that oral feeding could be gradually restarted only seven weeks after admission. The use of high-frequency intestinal ultrasonography proved useful and sensitive in monitoring the evolution of intestinal involvement. During hospitalization the child complained of acute painful scrotal swelling. Ultrasonography showed scrotal hemorrhage and testicular swelling but no signs of testicular torsion, thus helping to avoid surgical exploration. In the recovery phase the child complained of colicky abdominal pain with vomiting. A plain abdominal roentgenogram showed bilateral staghorn pelvic urolithiasis. This finding was confirmed by an intravenous pyelography which also revealed a bilateral pyeloureteritis with edema of the ureteral wall and partial stenosis of the lumen.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗
PubMed1992

[Cardiac rhabdomyoma in tuberous sclerosis. A report of five cases and review of the literature].

Five children with tuberous sclerosis (TS), a polysystemic disease, had cardiac tumors detected by B-mode echocardiography. In a fetus, sonography performed at the eighth month showed the presence of a cardiac mass and of polycystic kidneys: the diagnosis of TS was confirmed postnatally because of the presence of calcified cerebral nodules. A newborn dichorial twin had paroxysmal tachycardia at ten days. B-mode echocardiography showed the presence of seven intracardiac tumors, and cerebral CT the presence of multifocal periventricular calcifications. An absence in a nine month old baby prompted an electrocardiogram that proved abnormal; a B-mode echocardiography showed large apical cardiac tumor; again cerebral CT showed periventricular calcified nodules. Two girls, 9 and 10 y.o. respectively, affected by TS, with normal electrocardiograms, both presented a small, asymptomatic, intracardiac mass demonstrated sonographically. In recent years, prenatal sonography and B-mode echocardiography in patients with TS demonstrated with increasing frequency the association of cardiac tumors (rhabdomyomas) with TS. Tumor detection often precedes the appearance of the cutaneous and neurological signs typical of the disease. This paper emphasizes the role of echocardiography in detecting cardiac tumors, as an early sign for the diagnosis of tuberous sclerosis, and to depict the natural history of cardiac rhabdomyoma with its variable clinical presentation and prognosis.

Child↗
PubMed1990

Intravenous urography with iopamidol in children with reflux and obstructive nephropathy: effects on glomerular and tubular functions and the renin-angiotensin-aldosterone system.

Twenty-seven children [2 with chronic renal failure (CRF)] with reflux or obstructive nephropathy underwent intravenous urography with iopamidol 370, a nonionic contrast medium 1 (CM), osmolality 796 mosmol/kg, for renal growth evaluation. Mean iopamidol dosing was 1.69 ml/kg (range 1.22-2.42); the 2 children with CRF received 2 and 2.42 ml/kg respectively. One hour after infusion a significant decrease in haematocrit, haemoglobin, plasma sodium (Na+), chloride (Cl-), renin activity and aldosterone was observed, consistent with a possible plasma volume expansion due to the slightly hypertonic CM. At the same time there was a significant increase in fractional excretion of Na+, Cl- and potassium, probably due to the haemodynamic effects and tubular response to a substance acting as on osmotic diuretic. The -24 to +48 h monitoring of albuminuria, beta-2-microglobulin excretion, and in 4 children excretion of N-acetyl-beta-glucosaminidase and alanine-aminopeptidase did not show any relevant nephrotoxicity. No untoward effect of clinical relevance was observed.

Adolescent↗
PubMed1984

Hypertension from ipsilateral renin hypersecretion following kidney upper pole compression by the spleen.

A unique case of renovascular hypertension in an asymptomatic, microhematuric 61/2-year-old girl is presented. Abdominal ultrasonographic (UT) and computed tomographic (CT) studies, splenic and renal radionuclide evaluations revealed that a 'boomerang'-shaped spleen, anterior and medial to the left kidney, compressed and flattened its upper pole. Arteriography excluded stenosis of the renal arterial vessels. Renal vein renin ratio of 1.78 and contralateral/caval ratio of 1.13 eventually demonstrated that hypertension was caused by a lateralized renin hypersecretion from the compressed upper pole through left superior and central renal veins. Overactivity of the renin/angiotensin system was presumably due to ischemia of the renal parenchyma extrinsically compressed by the spleen.

Child↗