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Biomedical subjects

G Distefano

Publications and source records attributed to G Distefano.

At least 55 records · Page 3Linked to original sources
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[Physiopathologic and therapeutic aspects of persistent fetal circulation. Review of the literature and personal histological observations].

Persistence of the fetal circulation (PFC) is a syndrome characterized by failure of the cardiocirculatory system to adapt successfully to postnatal life. Its typical feature is persisting right-left shunt across fetal channels which determines cyanosis refractory to oxygen treatment. PFC can simulate cyanotic congenital cardiopathy. It has two forms: a primitive form and secondary one due to various causes especially perinatal asphyxia. Both forms have a common pathogenesis consisting of hypertension of the pulmonary arterial circulation. This article reviews the physiology of the main prenatal and postnatal circulatory characteristics and the factors which regulate the pulmonary circulation. It also reports the latest findings on PFC physiopathology and treatment indicating the prognostic factors and future perspectives.

Arterioles↗
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[A case of diaphragmatic eventration associated with Wolff-Parkinson-White syndrome].

The Authors report a case of diaphragmatic eventration associated with Wolff-Parkinson-White's syndrome. It's difficult to explain this unusual association. It's possible that the presence of cardiac accessory pathways in this particular patient is due to the alteration of the normal anatomical relationship resulting from the diaphragmatic eventration.

Child, Preschool↗
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[Characteristic aspects of the therapy of heart failure in infancy. Review of the literature and personal data].

The treatment of congestive heart failure in view of its pathophysiology can be divided in 3 parts: cardiokinetic therapy, diuretic therapy and vasodilator therapy. For each therapeutic component we report the latest findings and underline the peculiar aspects in the newborn. Furthermore we outline the basis for a rational use of the various types of drugs and indicate the future therapeutic prospectives.

Cardiovascular Agents↗
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[A comparison of the efficacy of aminophylline and doxapram in preventing idiopathic apnea in preterm newborn infants].

Doxapram is an analeptic of the respiratory system that has been used in the last few years for the treatment of idiopathic apnea spells in infants who show resistance to methylxantine. In this study we have compared the efficacy of aminophylline and doxapram for the prevention of idiopathic apnea spells in two groups of preterm infants comparable for gestational age, birthweight and postnatal age. The two drugs resulted to be effective in preventing the spells of apnea in 66% and 60% of the cases respectively. In the cases in which there was a partial or negative response, the association of the two substances resulted in a noticeable reduction of the apnea spells. The positive effect of the association of aminophylline and doxapram is probably due to the different action mechanism on the stimulation of the respiratory system.

Aminophylline↗
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[Juvenile spongiform encephalopathy with unusual pathomorphologic findings].

A 27-year-old man presented disturbances of gait and language, quickly followed by intellectual deterioration, tetraplegia, anarthria and myoclonus. Histological examination of a cerebral biopsy showed not only cortical changes consistent with the diagnosis of Creutzfeldt-Jakob disease, but also many amyloid-plaques with variable morphology. The diagnostic interpretation of the case particularly as to concern his relationship to s.c. Gerstmann-Strüssler syndrome is discussed.

Adult↗
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[A case of Hallermann-Streiff syndrome with rapidly fatal course].

The Hallermann-Streiff syndrome is a rare affection characterized by beaked nose, dyscephaly, hypotrichosis, cataracts, micrognathia and proportionate short stature. The most severe complication in the syndrome is respiratory embarrassment. Narrow air passage with abnormal glottic closure, prenatal growth deficiency, immunodeficiency and sometimes associated cardiovascular anomalies, can predispose these patients to pulmonary infections. The Authors describe a new-born with Hallermann-Streiff syndrome, who, after a brief period of obstructive apnea successfully treated with CPAP, developed a severe pulmonary infection that caused his death at the age of 61 days.

Age Factors↗
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[THe echographic diagnosis of diaphragmatic eventration in newborn infants. 2 cases].

The authors report two cases of neonatal partial diaphragmatic eventration. The diagnosis in both two cases was echographic more than radiographic. The authors underline the role of B-mode echography in the diagnosis and follow-up of diaphragmatic anomalies, especially eventration, and suggest that examination of diaphragm and of its motion should complete any abdominal and thoracic echography.

Diaphragm↗
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[Oral administration of doxapram in preterm neonates with aminophylline-resistant idiopathic apnea crisis].

Doxapram is an analeptic capable of stimulating both central and periferal areas of the respiratory system. During the last few years, intravenous infusion of doxapram has been carried out, with success, for the treatment of idiopathic apnea in preterm infants otherwise unresponsive to methylxantine. Since doxapram has a tendency to precipitate into various solution containing amino acids and calcium gluconate, oral administration has been suggested. The Authors in this study have seen that in 18 preterm infants suffering from idiopathic apnea unresponsive to amynofillina, an oral administration of doxapram at 12 mg/kg/6h resulted in a complete recovery from apnea spells in 66.7% of cases, while 22.2% gave a partial positive response and only 11.1% a negative result. Furthermore the Authors would like to stress that doxapram showed a good tolerance level when administered orally. In fact, no side effects which were previously reported in other publications were presented in the infants studied.

Administration, Oral↗
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[Pena-Shokeir syndrome: report of a case with benign outcome].

Pena-Shokeir syndrome is a rare, often lethal disease, characterized by intrauterine growth retardation and by fetal akinesia or hypokinesia that leads to craniofacial anomalies, limb ankylosis, polyhydramnios and pulmonary hypoplasia. The case that we report had a favourable evolution, although there was at birth a severe respiratory distress. EMG studies revealed deficit of innervation. Contraceptive therapy, wrongly used by the mother in the first period of pregnancy, played, probably, a concomitant role in the pathogenesis of the syndrome.

Abnormalities, Drug-Induced↗
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[Variations of blood bilirubin levels in the newborn with and without retinopathy of prematurity (ROP)].

Retinopathy of prematurity (ROP) is a multifactorial disease where production of free radicals is a pathogenic factor. Bilirubin is regarded today as the most powerful antioxidant substance "in vitro". To test such effect "in vivo" we studied 219 premature infants, admitted to our Neonatal Intensive Case Unit from April 1991 to October 1992, evaluating their serum bilirubin levels from day two to seven and mean bilirubin level of first week for each child. We also calculated the mean rate of daily increase of bilirubin. Our results show that bilirubin parameters considered are higher in neonates which will develop ROP of every stage than in the control ones. These results therefore do not support the concept that bilirubin could have a role in the prevention of ROP.

Bilirubin↗
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[Heart involvement in progressive spinal muscular atrophy. A review of the literature and case histories in childhood].

There are few cardiological studies in progressive spinal muscular atrophy and mainly concern subjects affected by the juvenile form (Kugelberg-Welander disease). The presence of a cardiomyopathy has been reported in these patients but the cardiac involvement is often secondary to the chronic respiratory insufficiency typical of the disease. We performed a retrospective study in our Institute on 43 patients, age range 3 months to 3 years, 37 of which presented type I (Werdnig-Hoffmann disease) and 6 type II (intermediate form) of the disease. No clinical nor instrumental signs of cardiomyopathy were observed. However, ECG revealed signs of right ventricular overload in 37.3% of the patients, probably provoked by pulmonary hypertension due to respiration anomalies. The authors underline the importance of correct respiratory assistance to prevent onset of cardiological alterations.

Cardiomyopathies↗
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[A rare cause of a continuous murmur. Our experience with a newborn infant with a congenital coronary fistula].

Congenital coronary artery fistulas are very rare. The most common connect one of the two coronary arteries with the right cardiac chambers, in particular the right ventricle. So, a left to right shunt appears; furthermore ischemic problems are possible. Notwithstanding in the pediatric age subset the patients are usually free of symptoms. The most important clinical sign is a continuous murmur in the precordium. We report a case of a newborn with a fistula between the right coronary artery and the right ventricle. He presented with the typical murmur; ECG and chest-ray were considered as normal. Echocardiography did not do the diagnosis, and the baby was submitted to a cardiac catheterization. There was a mild left to right shunt (QP/QS = 1.5/1), without pulmonary hypertension. The diagnosis was done by selective coronary angiography. We decided not to close the fistula, and the baby was discharged. Three months later he is still well. In this discussion we consider indications for surgery, and hypothesize a wider application in the future of transcutaneous embolization.

Arteriovenous Fistula↗
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[Epidemiological,and physiopathological findings in retinopathy of prematurity: the authors' personal cases].

The authors present a retrospective epidemiological study on retinopathy of prematurity (ROP), performed on 738 newborns with a gestational age of 34 +/- 2.41 weeks and birth weight of 1971 +/- 351 g. They excluded all cases with severe respiratory distress and other pathological conditions. First stage of ROP shows no correlation with gestational age and birth weight, whereas second and third stages of ROP show an inverse correlation with the above mentioned factors, particularly birth weight. Among neonates with the same gestational age, the incidence of ROP is higher in those "small for date". Regarding the pathogenesis of ROP, the authors outline the importance of perinatal hypoxia.

Birth Weight↗
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[Hypertrophic cardiomyopathy (probably secondary) in an infant with Stickler's syndrome. The physiopathological aspects].

Stickler syndrome is a hereditary connective tissue disease transmitted by AD pattern. It is characterized by peculiar facies with bone and ocular anomalies. Heart anomalies (except mitral valve prolapse) are an uncommon features that may be associated. The authors report on a 4 months patient affected by this syndrome who had hypertrophic cardiomyopathy. Pathogenetic mechanism of this anomaly is discussed.

Abnormalities, Multiple↗
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Evaluation of transdermal theophylline pharmacokinetics in neonates.

Theophylline may be administered by several routes, but problems are associated with neonatal dosing. The transdermal route may provide a safer and noninvasive method of administration, yet produce therapeutic concentrations in a consistent and reliable manner. To study the feasibility of this in the apnea of prematurity, stable neonates were administered a subtherapeutic transdermal dose for 24 hours in order to assess pharmacokinetics and bioavailability. This was followed with routine intravenous theophylline therapy according to institutional policy. Six of nine neonates had detectable serum theophylline concentrations that increased slowly after patch application. Mean (+/- SD) maximum serum concentration was 2.4 +/- 1.3 micrograms/ml, mean time to maximum serum concentration was 22 +/- 8.2 hours, and mean latency period was 8.0 +/- 4.9 hours. Mean total amount of theophylline delivered to the skin was 18.6 +/- 4.1 mg. Mean fractional absorption at 30 hours was 0.25 +/- 0.12. These data demonstrate that it is possible to produce systemic theophylline concentrations with a transdermal patch in preterm infants sufficient to study pharmacokinetics and bioavailability, and that transdermal delivery of therapeutic doses is technologically feasible.

Administration, Cutaneous↗
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[Kawasaki disease in Sicily: description of the first case with giant coronary aneurysm].

A case of Kawasaki disease with early development of giant coronary artery aneurysm is reported. The delay in the diagnosis and consequently in the use of correct therapy has conditioned the outcome of the illness. We don't know the predictive factors of cardiac complications, but steroid treatment in our patient probably had an important role in the cardiac damage. The use of echocardiography in the evaluation of coronary aneurysm has confirmed the sensitivity, specificity and predictive value of this technique in detecting cardiac lesions.

Coronary Aneurysm↗