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Biomedical subjects

C Manzoni

Publications and source records attributed to C Manzoni.

At least 91 records · Page 5Linked to original sources

The fetus with an abdominal wall defect: management and outcome.

Despite prenatal diagnosis, maternal transport and early perinatal treatment in specialized hospitals, improvement in overall mortality has not been observed among newborns with abdominal wall defects (omphalocele and gastroschisis). A comparison has been made, for both anomalies, between 55 cases treated in the periods 1967-1979 and 30 treated in the periods 1980-1985. In this last group 14 prenatally detected cases were separated from cases discovered at birth. Recent advances in treatment have reduced mortality rates for both of these anomalies. This was especially true for gastroschisis; in omphalocele cases, associated abnormalities appeared to be an unavoidable limiting factor in survival. With prenatal diagnosis a surprising increase in mortality rate was observed among omphalocele cases detected before birth. A possible reason for this is the total number of antenatally diagnosed cases that, with maternal transport, are concentrated in centers where surgical facilities are available. Before introduction of antenatal diagnosis, most of these cases would never have been observed by the pediatric surgeon because of death prior to referral or treatment. Gastroschisis cases may benefit more from antenatal recognition and early treatment because multiple abnormalities or immaturity are not so important limiting factors in survival as in omphalocele cases. Reduced trauma and contamination of exposed viscera, immediate treatment and adequate supportive measures may significantly reduce mortality rate in gastroschisis cases. Operative delivery of fetuses with an abdominal wall defect is controversial. The risk of vaginal route delivery has been over emphasized. Intrapartum rupture of an omphalocelic sac was an extremely rare event before introduction of antenatal diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Abdominal Muscles↗

Prenatal diagnosis and management of some fetal intrathoracic abnormalities.

Nine cases of fetal intrathoracic anomalies detected in utero and followed to birth are reviewed. There were 6 congenital diaphragmatic hernias (CDH), one congenital pleural effusion and two isolated cysts of the lung. All these conditions were potentially responsible for neonatal respiratory distress and received early intensive treatment after maternal transport and delivery had been arranged in a center with thoracic surgical facilities available. The risks of a delayed or missed diagnosis were thus avoided, especially for CDH. Despite intensive, traditional, respiratory support, started in the delivery room, mortality among prenatally detected cases of CDH was paradoxically high (83%), compared to mortality among 7 cases of CDH not detected in utero, referred in the same period to our Institution, and symptomatic within 6 h from birth (63%). With prenatal diagnosis the total number of CDH cases referred to a surgical center before birth increases. Many cases which would never have been treated in the past because of death before referral and treatment for severe pulmonary hypoplasia not compatible with life are thus observed and sometimes treated. Nevertheless, lung development continues to be a determining factor for survival even when intensive treatment at birth is available. Responsiveness to therapy is unpredictable before birth and proposed antenatal treatment is still far from being a realistic option. For the other three newborns, where a pleural effusion and pulmonary cysts were found, prenatal diagnosis helped to start appropriate treatment and to prevent neonatal hypoxia in two of them. In the third case, with an incommunicant, isolated pulmonary cyst, the outcome would have been favourable even without a prenatal diagnosis.

Congenital Abnormalities↗

High density lipoprotein-3 heterogeneity in subjects with the apo-AIMilano variant.

The structure of high density lipoproteins (HDL) isolated from subjects with the AIMilano (AIM) apoprotein variant was studied by the use of the cross-linking reagent dimethylsuberimidate. The HDL2 subfraction is markedly reduced, as compared to control subjects; the HDL3 subfraction, on the other hand, shows a marked heterogeneity, being characterized by at least three particle subpopulations, identified as HDL3-I, HDL3-II, and HDL3-III. The HDL3 fraction purified from the AI Milano subjects eluted as a symmetrical peak from a 6% agarose column, corresponding to a unimodal particle size distribution. The content of the different HDL3 particles, detected by cross-linking, varied widely along the elution profile, the tail of the peak being enriched in anomalous particles of very small size (HDL3-III). Apoprotein compositional studies indicated that these small HDL3-III may be enriched in the AIM monomer, the larger particles containing more AIM-AII complexes and AIM dimers. All the anomalous HDL3 particles are triglyceride enriched, with a decreased cholesterol ester content. They may be an intermediate product in the cholesterol transfer chain between HDL and very low density lipoproteins, or be generated during interconversion of HDL. These particles may have a functional role in tissue cholesterol homeostasis; their unusual compositional changes may help explain the protection of the studied subjects from tissue cholesterol deposition, in spite of the marked decrease of the total HDL fraction.

Amino Acids↗

The problem of subclinical localised paroxysmal rhythmic discharges (psychomotor variant discharges). Report of two cases.

Two cases are reported of patients whose EEGs showed localised rhythmic seizure activity in the midtemporal regions of one or both hemispheres, unaccompanied by any clinical symptoms: the patients' histories differed: one was of classic migraine and the other complex partial epilepsy. The frequency and morphology of the paroxysmal anomalies was identical in the waking state and in sleep. The nosographic classification of the phenomenon is discussed with reference to Gibbs' reports regarding the "psychomotor variant type of seizure discharge", to the work of Lipman and Hughes on "rhythmic mid-temporal discharge" and to that of Westmoreland and Klass on the "subclinical rhythmic EEG discharge of adults". But in contrast to the last phenomenon there were no signs pointing to a diffuse cerebrovascular disease. Reports of such a pattern are rare in the European literature and nonexistent in the Italian literature, facts which make an ordinary interpretation of the phenomenon difficult.

Aged↗

Changes in the clinical condition of haemodialysis patients.

The characteristics of the dialytic population have substantially changed over the past 30 years, becoming older and with a greater number of coexisting diseases. The considerable evolution in treatment modalities has lead to a significant increase in the efficacy and tolerability of dialysis. However, physicians have to deal with illnesses in long term dialysis survivors that may be a consequence of inadequate renal replacement therapy rather than of the dialysis procedure per se. Cardiovascular diseases are the leading cause of death and, although many of the risk factors are the same as in the general population (i.e. hypertension), some appear to be specific to CRF (i.e. hyperparathyroidism, anaemia). Age is the most important demographic factor associated with increased mortality. The increasing incidence of ESRD diabetic patients, as well as malnutrition, also contribute to higher mortality in RRT. The therapeutic answer to a worsening in clinical condition is adequate medical care (starting in the conservative phase), with particular attention being given to correcting anaemia, hypertension, volume overload and hyperparathyroidism, and preventing malnutrition. Treatment modalities also play a crucial role. Data suggest that adequate dialytic dose (and possibly time) can reduce morbidity and mortality, and on-line sodium and potassium modelling can improve intradialytic cardiovascular stability and reduce arrhythmias. Long-term treatment with synthetic high-flux membranes may confer some beneficial effect on beta2-m amyloidosis-related morbidity and may also reduce mortality. Family and social support greatly affect the quality of life of the patients. However technologically advanced, no procedure can succeed unless it is performed in the context of humanised health care directed towards patient needs.

Age Factors↗

[Clinical dialysis: new problems and new prospects].

The main problem nephrologists have to face today is the very high patient morbidity and mortality. A number of traditional and non-traditional risk factors have a role; among these anaemia, hypertension, dislipidemia, abnormalities in calcium-phosphate metabolism, hyperhomocysteinemia and endothelial dysfunction. An important innovation in the field of hemodialysis has been the availability of high-permeable and high-flux membranes, characterized by a high biocompatibility and ultrafiltration coefficient. The development of automatic systems to control ultrafiltration has enabled the utilisation of these membranes in the clinical setting (high-flux hemodialysis, hemofiltration, hemodiafiltration). It is common opinion that high-flux membranes can positively influence cardiovascular instability, but this has not been confirmed by clinical trials. Although preliminary data indicated a favorable effect on the correction of anemia in patients treated with high-permeable membranes, randomized trials have not shown a significant effect. Better control of anemia could be possible by means of on-line treatments, given their higher removal of medium- and large molecules and reduced microbiological and pyrogenic contamination of the dialysate. A number of analyses showed a lower incidence of bone cysts and/or carpal tunnel syndrome in patients treated with high-flux membranes compared to low-flux ones. High-flux treatments could reduce morbidity and mortality in hemodialysis patients. However, despite its large sample size, the HEMO Study has not been capable of showing a statistically significant effect of higher dialysis dose and high-flux membranes on survival and morbidity. The MPO study has been expressively designed to do a prospective evaluation of the long-term effect of membrane permeability on clinical outcomes. These results are greatly awaited.

Amyloidosis↗

[Use of dialysate conductivity to obtain neutral sodium balance in chronic hemodialysis patients].

In order to assure a zero sodium balance in hemodialysis patients, attaining 'constant' values of total body water and plasma water sodium concentration at the end of each dialysis session is a basic pre-requisite. This is achieved by matching the ultrafiltration to the inter-dialytic weight gain and by individualizing dialysate sodium concentration at each dialysis session by making use of a kinetic model. Clinical results suggest that the single pool variable volume sodium kinetic model allows the targeted end-dialysis plasma water sodium concentration to be obtained. Nevertheless, this model is not suitable for routine clinical application, because of difficulties in the real-time determination of initial plasma water sodium concentration and 'effective' sodium dialysance. Measuring dialysate conductivity at the inlet and outlet ports of the dialyzer allows the estimation of sodium transfer during dialysis, if the function of concentration versus conductivity is known. If sodium transfer is measured at two different inlet dialysate conductivities, it is possible to determine ionic dialysance and systemic plasma water conductivity, which can be used routinely to apply the single pool sodium kinetic model. Given that ionic dialysance and plasma water conductivity can be measured easily repeatedly and inexpensively at each dialysis session without the need for blood sampling or laboratory determinations, it can be expected that conductivity kinetic models will soon become a part of everyday clinical practice.

Electric Conductivity↗

[One-stage "forced" closure of large congenital defects of the abdominal wall with mechanical ventilation and curarization. Clinical evaluation].

Advantages of primary fascial closure of abdominal wall defects are mainly in reducing the number of staged procedures with related complications and the need of multiple operation. Nevertheless correction of large defects still remains a challenge to pediatric surgeon. Postoperative paralysis and mechanical ventilation after intraoperative milking of intestinal content and abdominal muscles stretching have been reported to reduce the risks of "forced" primary closure. A series of 64 Omphalocele and Gastroschisis has been reviewed. Associated anomalies are still the main cause of mortality among Omphalocele. Prematurity plays a secondary role on survival of Gastroschisis cases; deaths were mainly due to sepsis. Primary respiratory insufficiency affected a large number of Giant Omphalocele cases (larger than 5 cm with herniated liver) and was associated to a restricted chest structure. All these cases died in the first weeks of life. Primary closure with or without postoperative paralysis and mechanical ventilation showed to reduce in a significant way the postoperative complication rate compared to staged procedures. Mortality and hospital stay were not significantly influenced by different kinds of surgical treatment among Omphalocele. Associated anomalies are an unavoidable limiting factor to survival. Among Giant Omphalocele the use of aggressive primary fascial closure with ventilatory support showed in our hands to be a safe procedure provided that a preoperative selection of cases on the basis of chest X-ray and blood gases has been made.

Abdominal Muscles↗

[Scintigraphic diagnosis of Meckel's diverticulum using Tc-99m-pertechnetate].

Meckel's diverticulum (MD) is a well-known cause of lower gastrointestinal hemorrhage, particularly in the pediatric age group. The radionuclide 99mTc-pertechnetate has been found useful in visualizing MD which contain gastric mucosa. This test is predicated of the affinity of the isotope for the parietal cells of the gastric glands. In an attempt to evaluate the sensibility and specificity of pertechnetate, the clinical data and scintigrams of 27 patients with suspected MD were reviewed. The scintigram correctly identified MD before operation in 6 of 7 patients. The false negative study occurred in a MD without gastric mucosa.

Adolescent↗

[Dumbbell neurogenic tumors in children].

Dumbbell neurogenic tumours are unusual neoplasms in the pediatric age group. Six cases all in children under 10 years of age, have been reviewed with respect to diagnosis, management and results. Neurologic deficits have been found in 4 patients only; in the other 2 cases the tumours were occasionally discovered. Myelograms, sometimes associated with CT scan, allowed a correct diagnosis in all the patients. Initial treatment consisted in all the cases in laminectomy or laminotomy with total excision of the extradural tumour. The primary paravertebral tumours have been removed in one patient during the same neurosurgical operation, and in other three patients at a second stage. Two patients did not require the second operation: one recovered after chemotherapy and the other died few days after the first surgical procedure. Five patients are still alive: two, who had the tumour occasionally discovered, are free from disease; among the other 3 patients, presenting with neurological symptoms, 1 is free from disease without neurological symptoms and 2 have still long lasting sequelae. The authors emphasize the role of early diagnosis to prevent neurologic abnormalities and to achieve better results.

Child↗

[Natural history of fetal uropathies. Experience in 40 cases].

Appropriate management of fetal uropathies depends on accuracy in assessing the severity of urinary tract obstruction and renal damage, and in predicting the potential for recovery after surgical correction. A review of 40 fetuses aged between 20 an 39 weeks referred to us for prenatal counseling for a suspected anomaly of the urinary tract, has been made. Reliability of our diagnostic resources has been retrospectively evaluated on the basis of clinical, ultrasonographic and radiological postnatal data. Conservative approach has been adopted in all our cases. None intrauterine surgery was attempted; pre term delivery was advised only in two cases. Our results confirm poor prognosis of fetuses with marked decrease of amniotic fluid within the 24th week of gestational age. After this date the amount of amniotic fluid alone may not be a completely reliable prognostic factor. Ultrasonographic aspect of fetal kidneys and, in some occasions, chemical analysis of fetal urines may accomplish our prenatal study and help to predict the degree of neonatal renal function and potential of survival.

Female↗

[Transient idiopathic hyperphosphatasemia in infancy. Presentation of a case].

One case of a 13-months-old-female infant with transient idiopathic hyperphosphatasemia is described. This syndrome is characterized by: 1) increased serum alkaline phosphatase activity not associate with an organic disease; 2) normalization of the enzyme activity within 12 weeks. Familial hyperphosphatasemia, a permanens disease, is also excluded because of ALP normal values in both parents. Rickets, hepatic and biliary diseases are excluded by clinical, radiologic and laboratory data.

Alkaline Phosphatase↗

[Hepato-diaphragmatic interposition of the colon (Chilaiditi syndrome)].

The authors describe a Chilaiditi's Syndrome which they observed and supervised for two years. They consider its clinical picture, the essential elements for the diagnosis, the evolution and the therapy. In accordance with the pediatric literature the resolution was spontaneous, and this fact confirms the opportuneness of a therapy of wait, also to avoid an unnecessary surgical operation.

Abnormalities, Multiple↗