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Biomedical subjects

S Zucchini

Publications and source records attributed to S Zucchini.

50 records · Page 3Linked to original sources

Intravascular intrauterine transfusion for severe erythroblastosis fetalis using different techniques.

Over a 3-year period, 44 ultrasound-guided intravascular transfusions were performed between 18 and 32 weeks on 15 patients with severe erythroblastosis fetalis due to Rh immunization. In 4 fetuses, the first transfusion was performed before 20 weeks, in 6 between 20 and 25 weeks and in the remaining 5 between 25 and 31 weeks. Eight of the 15 fetuses were hydropic at the time of referral. Five transfusions were done in the intrahepatic umbilical vein, 6 were simple transfusions via percutaneous umbilical cord puncture, and 33 were partial exchange. There were 4 intrauterine deaths before 26 weeks, despite successfully performed transfusions: 3 of these fetuses were severely hydropic, while in the remaining fetus hydrops had been reversed in utero. Following delivery by cesarean section at 32 weeks of gestation, 1 of the neonates developed respiratory distress syndrome and died 17 h after birth. The overall survival rate was 67% (10 of 15 cases): 4 of the 8 hydropic fetuses (50%) and 6 of the 7 nonhydropic fetuses (83%) were alive at birth and survived the perinatal period. Three of the 5 losses occurred among the first 4 cases, while in the last 11 cases the survival rate increased to 82% (9 of 11).

Blood Transfusion, Intrauterine↗

Somatomedin-C levels related to gestational age, birth weight and day of life.

Capillary blood samples on filter paper were assayed by means of an RIA method (Kit Nichols Institute USA) from 1096 newborns divided into full term, preterm and small-for-date infants. The somatomedin-C (Sm-C) mean value, which did not differ in the three groups, was 0.15 +/- 0.09 IU/ml. One hundred and ten (10%) showed Sm-C disc values less than or equal to 0.075 IU/ml, the minimum value measurable by our method. The day of life and birth weight had a significant influence on Sm-C levels. Gestational age did not have any significant effect. No significant interaction was found among the parameters considered. All the groups presented a progressive increase of Sm-C. Unlike preterm and small-for-date infants, in full term infants the latter increase seemed already to be evident from 5th day of life and reached higher levels from the 7th day of life onwards. In conclusion, Sm-C rates were reduced in the neonatal phase of life, but showed a tendency to rise later.

Age Factors↗

Relationships between growth factors (somatomedin-C and growth hormone) and body development, metabolic control, and retinal changes in children and adolescents with IDDM.

We used the radioimmunoassay (RIA) method to determine somatomedin-C (SmC) basal values in 59 diabetic children and adolescents (20 prepubertal and 39 pubertal subjects; age range 2.75-20.16 yr; duration of diabetes 0.08-15.83 yr) and in 274 control subjects. In comparing diabetic subjects with controls, we considered only those 50 diabetic subjects who were age matched with the controls, i.e., those not over 16 yr chronological age. SmC basal levels in pubertal diabetic patients were no different from those of pubertal age-matched control children, whereas in prepubertal diabetic patients SmC was significantly lower than in the respective control children (P less than .001). No correlation was found between the z score for SmC (i.e., the number of standard deviations each SmC level is from the age- and sex-normalized mean) and duration of disease, velocity standard deviation score, severity of fluoroangiographic retinal changes, basal C-peptide values and HbA1 levels. No differences were encountered in mean SmC and SmC z-score values in the separate groups of poorly, fairly, and well-controlled diabetic children, in the groups with and without residual pancreatic activity, and in the group with and without retinal changes. In 16 of the pubertal diabetics and in 15 pubertal controls, serum glucose, growth hormone (GH), and SmC concentrations were determined during the night. The integrated nocturnal secretion of SmC was no different in diabetics than in controls, whereas the integrated nocturnal secretion of GH was significantly (P less than .025) higher in diabetics than in controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Differences in somatomedin-C between short-normal subjects and those of normal height.

We evaluated basal somatomedin-C (SmC) levels in 98 subjects 2 to 16.6 years of age, with height less than 3rd centile (Tanner), and in 274 healthy controls 2 to 15.8 years, with height greater than 10th centile. Growth-retarded subjects were defined as short-normal when they had normal GH release (greater than 8 ng/ml) in at least one of three tests: arginine, L-dopa, and sleep. In control subjects, there was a significant positive correlation between SmC levels and chronologic age, bone age, and pubertal stage (pubic hair, breast or testicular volume). The same correlations were present in short-normal subjects, but SmC levels were significantly lower than in normal children. The percentage of subjects with very low SmC values (less than or equal to 0.25 IU/ml in those older than 6 years, and less than 0.1 IU/ml in those younger than 6 years) was higher in the short-normal group of children older than 6 years. In growth-retarded subjects, SmC values were significantly higher (P less than 0.005) in subjects with normal GH response in at least one of the two pharmacologic tests, compared with those with normal GH response only during sleep. We conclude that short-normal subjects have, on average, low SmC values, which might indicate insufficient GH release. Therefore, current criteria to define GH deficiency and children needing treatment may be too restrictive.

Adolescent↗

Modifications of metabolic control in type 1 diabetic children and adolescents: experience over the last 20 years.

The aim of this retrospective study was to verify whether the daily number of insulin injections could have affected metabolic control in 181 unselected diabetic patients (age 0.66-14.75 yr at onset of diabetes) followed in our clinic from the 1970s to the 1990s. They were evaluated regularly since onset of disease for a mean follow-up period of 6.8 years. The factor with the greatest effect on HbA1c levels was the year of disease onset, which was negatively correlated with HbA1c independently of the daily number of injections and disease duration. Disease duration showed an effect on metabolic control only in the first 5 years of disease. Daily insulin injections affected metabolic control above all as regards 1 vs 2 or more injections. Regarding the change in insulin regimen from 2 to 3-4 injections, there was an improvement in metabolic control in patients with HbA1c > 9% and a worsening in those with HbA1c < 7%/ After the first 5 years of the disease HbA1c levels were higher in adolescent patients than in both younger and older patients. In conclusion, increasing the daily number of injections does not seem in itself capable of eliciting marked improvement in metabolic control, as in our young diabetic patients in the last decade. Multiple insulin injection therapy seems to be mostly indicated for patients with poor control and for adolescents.

Adolescent↗

Unusual magnetic resonance imaging findings of the sellar region in subjects with hypopituitarism: report of 4 cases.

Out of 323 consecutive growth hormone deficient patients who underwent magnetic resonance imaging (MRI), we describe the clinical and neuroradiological characteristics of four patients in whom MRI revealed unusual pictures of the sellar area. They were selected as unique in their morphological picture and representative of rare conditions. At presentation all subjects had short stature, growth hormone (GH) deficiency and complex phenotypical abnormalities. Patient 1. Female affected by vaginal atresia and sinus urogenitalis, polydactyly and syndactyly with Y-shaped metacarpals. MRI at age 11.2 years revealed normal pituitary, but hypothalamic mass occupying the suprasellar and interpeduncular cistern. The diagnosis of Hall-Pallister syndrome was made. Patients 2 and 3. Two sisters with a history of epilepsy both showing mild intellectual deficiency, midface hypoplasia and ectodermal dysplasia. MRI at age 8 and 12 years respectively displayed in both cases a round hypointensity protruding from the dorsum sellae into a normal pituitary. The diagnosis was sellar spine. Patient 4. Male with a history of postnatal hypoglycemia showing microphallus and clinical features of severe hypopituitarism. Hormonal evaluation at age 8 months confirmed multiple pituitary hormone deficiencies and MRI at age 6 years showed absent anterior lobe, rudimentary stalk and posterior lobe ectopia. The diagnosis was pituitary aplasia. The patients described show that MRI in pituitary dwarfs can reveal unusual intrasellar findings and allow the correct diagnosis of rare syndromes. Our patients also demonstrate the wide variability in the association of hypopituitarism with midline congenital abnormalities and the possible combination with complex syndromes.

Abnormalities, Multiple↗

[Cryptorchidism].

Cryptorchidism is a frequent abnormality which affects approximately 1% of children at age 1 year. Many nonscrotal testes are retractile and require no therapy. The pathogenesis remains controversial and involves possible mechanical, dysgenetic or hormonal factors. The latter are consistent with a partial defect in the hypothalamo-pituitary-gonadal axis, which may cause progressive histologic alterations in the testes after the first 6 months of life. Ultrasound is the simplest diagnostic technique (after clinical examination) to identify the testes in the inguinal region, while Magnetic Resonance Imaging can be performed to visualize abdominal testes. Therapy is still a matter of controversy. Hormonal treatments with HCG, LHRH or both should be used as first-intention treatment and their efficacy ranges from 0% to 60% in the various studies. LHRH nasal spray seems less effective if used alone. An early surgical treatment is recommended if the gonad is in the abdomen or close to the internal inguinal ring. The risk of cancer is increased in subjects with a history of cryptorchidism and even includes the contralateral descended testes. Early orchidopexy is not associated with a certain decrease of the risk. Cancer can be prevented by searching for in situ carcinoma with a biopsy performed after puberty. Fertility is impaired mainly in men with a history of bilateral cryptorchidism. There is weak evidence that early orchidopexy may improve fertility rates.

Adolescent↗

[Drug treatment of cryptorchidism].

There is still a debate on the choice of medical or surgical approach for the management of the undescended testis and on their efficacy in preventing long-term complications. HCG has long been used, with various schedules, in the treatment of cryptorchidism. A complete descent occurs in 14-59% of cases, with the highest percentages in older children and the lowest in intra-abdominal testes. The usefulness of intranasally administered LHRH is even more controversial and its potency seems inferior to HCG. In the last decades the surgical intervention has been recommended at progressively earlier ages. Our opinion is that the orchiopexy is inevitable, around age 2 years, if medical treatment has failed and the gonad is located intra-abdominally or high in the inguinal canal. In contrast, if the testis shows a partial response to medical treatment and is close to the scrotum, a second HCG course can be performed around age 4 years.

Administration, Intranasal↗

[Magnetic resonance imaging and hypothalamo-hypophyseal disorders in children: morpho-functional and etiologic aspects].

In recent years magnetic resonance (MR) has improved the diagnostic accuracy of the study of the sellar area. In patients with growth hormone deficiency, particularly when associated with other hormonal defects, MR has identified the picture characterized by posterior lobe ectopia, non-visible stalk, hypoplasia of the anterior lobe and presence of intrasellar CSF (empty sella). The latter is not exclusive of growth hormone deficient patients, but can be found also in children with disorders of puberty. Although a certain relationship between pituitary height and growth hormone secretion has been described, it is usually difficult to find a clear association between pituitary dimensions and function of the gland. The theory supporting the congenital origin of the above mentioned abnormalities, which may be part of midline abnormalities, is currently sustained by most Authors. In diabetes insipidus the posterior lobe may or may not be visible and a thickened stalk has been found in patients with disease duration of less than 2 years. In disorders of puberty morphological abnormalities of the sellar area are rare. In girls with precocious puberty the pathological findings described are hamartoma of the tuber cinereum, empty sella and pituitary height increased for age. There seems to be a certain relationship between gonadotropin secretion and pituitary dimensions.

Adolescent↗

Normal pituitary size in two patients with growth hormone gene deletion.

Somatotrope cells account for almost 50% of the volume of the pituitary gland, which is usually hypoplastic in subjects with growth hormone (GH) deficiency. Magnetic resonance imaging (MRI) was used to describe the sellar area of two siblings affected by GH gene deletion and hence absent GH secretion. Pituitary size and shape were normal in both subjects and there were no other abnormal findings in the sellar area. In particular, pituitary height (5.6 and 5.1 mm) and volume (268 and 229 mm3) in the two patients were within the normal range for age and higher than the usually reported values in GH deficient subjects. Our results suggest that the amount of GH secreted from the pituitary may have little influence on the dimensions of the gland.

Child↗

[Medical therapy of cryptorchidism].

There is still a debate on the choice of medical or surgical approach for the management of the undescended testis and on their efficacy in preventing long-term complications. HCG has long been used, with various schedules, in the treatment of cryptorchidism. A complete descent occurs in 14-59% of cases, with the highest percentages in older children and the lowest in intra-abdominal testes. The usefulness of intranasally administered LHRH is even more controversial and its potency seems inferior to HCG. In the last decades the surgical intervention has been recommended at progressively earlier ages. Our opinion is that the orchiopexy is inevitable, around age 2 years, if medical treatment has failed and the gonad is located intra-abdominally or high in the inguinal canal. In contrast, if the testis shows a partial response to medical treatment and is close to the scrotum, a second HCG course can be performed around age 4 years.

Age Factors↗