[Marfan's syndrome. Clinical and biological aspects with particular attention to the existence of a mucopolysacchariduria].
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Biomedical subjects
Publications and source records attributed to G Tortorolo.
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OBJECTIVE: To evaluate the efficacy of recombinant human erythropoietin (rHuEPO) in prevention of late anaemia due to Rh-haemolytic disease in neonates subjected to one or more intrauterine transfusions (IUTs). STUDY DESIGN: Six neonates (GA 28-38 weeks, BW 980-3,360 g), subjected to one or more IUTs for Rh-haemolytic disease, were treated for 3 weeks with rHuEPO (200 U/kg/day, s.c.) after the second week of life to prevent late anaemia and consequently reduce the need for blood transfusions. All treated neonates were supplemented weekly with iron, vitamin E and folinic acid, intramuscularly. RESULTS: Of the 6 patients studied, 4 preterm neonates, after commencement of rHuEPO treatment, showed a decrease in Hct values with persistent reticulocytopenia, and consequent need for one or more transfusions with packed and filtered red cells (PFRC). These 4 neonates had received a greater blood volume with IUTs than the 2 other term neonates, who, after starting rHuEPO treatment, showed an increase in Hct values and in reticulocyte count, with no transfusion requirements after birth (247 +/- 47 vs. 84 +/- 76 ml). CONCLUSIONS: Our results seem to correlate the efficacy of erythropoietin treatment in prevention of late anaemia resulting from Rh-haemolytic disease to the severity of intrauterine anaemia and to gestational age. Erythropoietin, in fact, was less effective in cases of severe intrauterine anaemia requiring a high volume of PFRC; it was also less effective in the preterm babies, because of the simultaneous presence of anaemia of prematurity and other major diseases.
OBJECTIVE: To verify the efficacy of oral iodide therapy in treating a case of early neonatal hyperthyroidism due to maternal Graves' disease. METHODS: We report a case of neonatal hyperthyroidism which occurred in a 2,650-gram, female baby, born at 39 weeks' gestational age (GA) to a 30-year-old mother affected by Graves' disease and treated with thionamides (propylthiouracil) from the 20th week of gestation. A fetal goiter, due to maternal therapy, had been observed by ultrasound scan at 31 and 35 weeks of gestation, with contemporary low cord thyroid hormone levels. Two intra-amniotic injections of levothyroxine were then performed at 34 and 36 weeks of gestation, which led to a significant reduction of fetal goiter and to normalization of cord thyroid hormone levels. The neonatal clinical course was characterized by symptoms of hyperthyroidism from the 2nd to 3rd days of life (irritability, tachycardia, tachypnea, hyperphagia), mostly during feeding. Oral treatment with potassium iodide (KI, 8 mg x 3 times a day) was started at 23 days of life. RESULTS: Treatment with KI led to a significant reduction of neonatal clinical symptoms and to a normalization of hormone levels within 4 days of therapy. The treatment was discontinued in 13th week of life because of neonatal well-being and normal hormone levels. CONCLUSIONS: We believe that KI therapy is effective in treating neonatal hyperthyroidism and does not cause suppression of neonatal thyroid activity, which is possible using antithyroid drugs like thionamides.
PURPOSE: To define standard values of blood flow velocities and indices in the ophthalmic and central retinal arteries in the neonatal period. METHODS: Forty-two healthy full-term neonates comprised the study population. A color Doppler with mechanical sector probe was used for measuring blood flow velocity in the ophthalmic and central retinal arteries. Systolic, end diastolic, and mean-enveloped velocities were measured, and the resistance index and pulsatility index were calculated. RESULTS: Ophthalmic artery Doppler velocities were similar on the first and third days of life, but increased significantly on the fifth and seventh days of life; resistance index significantly increased during the first week of life, whereas pulsatility index did not change significantly. Doppler velocities of the central retinal artery were similar on the first and third days; they show a delayed increase compared to the ophthalmic artery. Central retinal artery blood flow velocities increased significantly from the third to seventh postnatal day. Resistance index also increased between the first two days and on the fifth and seventh postnatal days, while pulsatility index did not change. CONCLUSION: These data constitute a starting point for studying the possible relationship between eye circulation and pathogenesis of retinopathy of prematurity.
The Authors report an update relative to the dietetic prevention strategies in the high allergic risk subject, as proposed from more recent literature. The babies with a familiar history of atopia are defined as population with allergic risk. The Authors examine the role of early exposure to cow's milk formulas and maternal diet during breast-feeding as risk factors for allergic symptoms in such babies. Moreover, they examine the indications for hydrolisated milk (partial and extensive) formulas and soy milk formulas use, as reported in published Meta-analysis and official statements of several Scientific Associations. They conclude that beyond the undoubted preventive role of exclusive breast-feeding in the first 4-6 month after birth, and of the extensively hydrolisated formulas, there are many concerns about the role for partially hydrolisated formulas and soy formulas. The Authors claim for multicentric methodologically correct trials in order to clear the controversies.
OBJECTIVES: The aim of the study was to compare a group of very low birth-weight infants feeded with a preterm formula with two other groups feeded with human milk and two different fortifiers. METHODS: 30 preterm newborns with birth-weight < 1.500 g, admitted to the Neonatal Intensive Care Unit (NICU) of Department of Neonatology of Catholic University of Rome were randomized for three different feeding groups: total enteral nutrition with HM fortified with Enfamil Human Milk fortifier or with Eoprotin, compared to a group feeded with Similac 24 preterm formula. Statistical analysis was performed using the two-way analysis of variance (ANOVA). RESULTS: During the study and at the end we found a growth rate for weight, cranial circumference and lenght similar to the fetal standard growth rate in the third trimester of pregnancy in all the three groups. Fortified HM was well tolerated. No pathologic value of the biochemical parameters studied was found. Higher level of serum phosphorus in spite of significantly lower intakes of phosphorus occurred in fortified HM feeded neonates, as if there was a better availability of this nutrient in HM. CONCLUSIONS: This study demonstrates the role of fortified HM as a good alternative to the preterm formula.
The aim of this study is to investigate if nasogastric feeding may provide an adequate caloric intake and a good growth in preterm infants. One hundred and thirty-one newborns with gestational age less than or equal to 33 weeks, admitted to the Neonatal Unit of the Catholic University of Rome over a period of three years, were included in the study. Infants were divided according to birth weight in four groups: the first includes 22 neonates weighing less than or equal to 1000 g; the second 60 newborns with birth weight of 1001-1500 g; the third includes 36 prematures weighing 1501-2000 g; the fourth group 19 neonates with birth weight greater than 2000 grams. Body weight was measured daily and head circumference weekly for all the study period (60-90 days). Mean postnatal weight loss was greater in the lowest birth weight group (13.2% of the birth weight) as compared to the other three groups (8%-9%). Birth weight was regained at 18th day of age in the newborns of the first group and in the second week of age in the other three groups. A caloric intake greater than 100 Kcal/Kg/day was achieved in the second week, ranging between the 8th day (forth group) and 14th day (first group). The achievement of full enteral feeding was inversely related to the birth weight.(ABSTRACT TRUNCATED AT 250 WORDS)
The authors studied the effects of different kinds of phototherapy in 186 newborns with a gestational age less than 33 weeks and weighing less than 2500 g. A control group of 60 infants was compared with a group of 31 infants submitted to photoprophylaxis, a group of 54 infants treated with day light, and a group of 41 infants treated with day light + special blue light. As far as the serum bilirubin variations in the first week, the maximum levels attained and the number of exchange transfusions are concerned, phototherapy always proved effective in reducing potentially neurotoxic serum bilirubin concentrations. Among the infants subjected to treatment, those treated with the day light showed a significant smaller decrease of serum bilirubin compared to those treated with the day light + special blue light, even if levels above 15 mg/dl were found in a similar percentage. Only 3.2% of the babies treated with photoprophylaxis showed serum bilirubin values above 15 mg/dl. The authors conclude that prophylaxis seems to be preferable to other phototherapeutic schedules used in the treatment of hyperbilirubinemia in low gestational age infants.
In 120 nursing mothers, the quantity of milk suckled was evaluated during the first days of the puerperium. Measurements of the milk intake were based on the difference between the weights of the neonate before and after each feeding. It was noted that, as the parity of the mother increased, the mean quantity of breast milk suckled/24 hours increased on the 1st, 2nd and 3rd day of the puerperium, as well as during the last 24 hours prior to discharge from the hospital (3rd or 4th post-partum day). Given the importance of a satisfactory flow of breast milk during the first days of the puerperium in the successful initiation of the breast-feeding experience, the authors would like to emphasize the increased risk of failure in the primipara and to suggest that greater attention be given to the effect of maternal parity in programs which promote breast-feeding.
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The authors have evaluated the influence of some obstetric events (type and duration of labour and method of delivery) and some pharmacological factors (oxytocic drugs during labour, local infiltration, duration of general anaesthesia, oxytocic drugs and/or ergot derivatives during puerperium) on frequency of breast-feeding at the time of discharge from hospital. About 57% of 356 newborns were breast-fed (wholly or prevalently) at discharge from the "A. Gemelli" University Hospital in Rome, during two months period (march-april 1981). The results showed that all those conditions requiring medical and paramedical action, either instrumental or pharmacological (such as: induced labour, prolonged labour, operative delivery either vaginal or abdominal, particularly in emergency; use of local infiltration; use of oxytocic drugs during labour, particularly for induction; prolonged general anaesthesia in caesarean section) may have an unfavorable effect, nearly always significant, on breast-feeding. According with these results, the Authors suggest the following measures for breast-feeding promotional programme: modification of neonatal routine care to promote an earlier mother-infant interaction during lying-in; limitation of avoidable obstetric procedures and of drugs giving to mothers; a better understanding of pharmacokinetics, both in mothers (placentar passage of drugs and through milk) and in newborns during labour, delivery and puerperium.
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Neonatal hyperibilirubinaemia is a real problem for its possible repercussion on the psychomotor development, mainly in low birth weight infants. The Authors studied the physiologic course of bilirubinaemia in 513 low birth weight newborns and then related it to gestational age, birth weight and intrauterine growth. Results obtained show that neonatal hyperbilirubinaemia is strictly depending on gestational age, while both the birth weight and the intrauterine growth have no significant influence on its course. Certainly the very low birth weight infants run the higher risk of Kernicterus and brain injury due to hyperbilirubinaemia; they need therefore a quicker therapeutic approach, though the treatment of physiologic hyperbilirubinaemia must always be planned on the basis of gestational age.
Phototherapy is largely used in the management of neonatal hyperbilirubinaemia, but its safety is very far from being proved. The Authors studied the effectiveness of daylight in low-birth-weight newborns. The results obtained show that the reduction of bilirubin induced by phototherapy is related with gestational age and intra-uterine growth. The very low-birth-weight infants, at higher risk of kernicterus, as they are more responsives to phototherapy, as well they are, probably, more susceptibles to side-effects. The Authors think that phototherapy should be planned on the basis of such observations.
We have compared three different fluorescent light sources (true light, blue light, true light + blue light) for phototherapy in 155 low birth weight newborn babies affected with non haemolytic hyperbilirubinaemia. Our data suggest that blue light is significantly more effective than true light. Surprisingly the association of true light with blue light is significantly more effective than both true light and blue light separately. The greater effectiveness associated with the better tolerance of true light + blue light respect to blue light only, makes it preferable than phototherapy regimen, even if more extensive study on its effectiveness and on short and long term side effects are need.