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Biomedical subjects

M Cappa

Publications and source records attributed to M Cappa.

101 records · Page 6Linked to original sources

[Evaluation of hematometric parameters and plasma ferritin levels in patients treated with ferritin-containing preparations].

Ferritin has been administered in immediately bioavailable solution form, and in solid form, with exclusively duodenal bioavailability, to two groups of patients suffering from sideropenic dyserythropoietic anaemia. The results obtained point to a significant stimulating action on erythropoiesis documented by the increase in reticulocytes and red cells. This increase is certainly relatable to the increase of available iron and to its incorporation in the erythrocytes, as demonstrated by the increase in ferritinaemia, sideraemia, haemoglobin and mean corpuscular haemoglocbin concentration. The data obtained showed the bioavailable fluid form to be more active than the solid.

Anemia, Hypochromic↗

A study of growth hormone release in man after oral administration of amino acids.

A study was carried out in 15 male volunteers to evaluate qualitatively the secretion of growth factors following stimulation by oral amino acids. The results showed that oral administration of a combination of two amino acids (1200 mg 1-lysine plus 1200 mg 1-arginine) provoked a release of pituitary somatotropin and insulin. This phenomenon was reproducible and the growth hormone secreted in response to this stimulation had biological activity (as demonstrated by a radioreceptor assay and somatomedin induction). The effect appeared to be specific to the combination of the two amino acids; neither of the amino acids demonstrated appreciable stimulating activity when administered alone, even at the same doses.

Administration, Oral↗

The analgesic effect of calcitonin in humans: studies on the role of opioid peptides.

The possibility that the analgesic action of calcitonin could be due to variations in beta-endorphin levels in both peripheral blood and cerebrospinal fluid was studied. A total of 40 microgram of synthetic salmon calcitonin was injected i. v. in four male volunteers undergoing minor operations under rachianesthesia. Beta-Endorphin was determined in blood and cerebrospinal fluid samples that were collected before and at regular intervals after calcitonin injection. No statistically significant variation in beta-endorphin levels was observed either in peripheral blood or cerebrospinal fluid. Since the injected calcitonin was free of contaminants (demonstrated by both high pressure liquid chromatography and radioimmunoassay of endorphin made on the injected preparation) and since an increase was observed in the immunoreactive calcitonin in cerebrospinal fluid following calcitonin i. v., self-analgesic action may be hypothesized, even though action through prostaglandins or other systems cannot be excluded.

Adult↗

Simpson-Golabi-Behmel syndrome: an X-linked encephalo-tropho-schisis syndrome.

We report on another family with the so-called "gigantism-dysplasia syndrome", an X-linked condition characterized by pre- and postnatal overgrowth, characteristic face with apparent coarseness, dysplastic changes in several tissues, and mild intellectual impairment. This condition has been called the Golabi-Rosen syndrome; however, we agree that is the same entity as that described, in a milder form, by Simpson et al in 1975 and by Behmel et al in 1984. Therefore, we suggest that this entity be designated the Simpson-Golabi-Behmel syndrome. The manifestations in affected individuals suggest that this condition represents an X-linked encephalo-tropho-schisis syndrome.

Abnormalities, Multiple↗

Absolute alcohol in esophageal vein sclerosis.

Absolute alcohol is a potentially optimal agent for sclerotherapy of esophageal varices. It is cheap and readily available. We compared the efficacy and safety of alcohol with those of a commonly used sclerosing agent, polidocanol. The study was planned to include patients with previous bleeding from esophageal varices randomly assigned to one of the two treatments. After the inclusion of the first 11 patients (6 in the polidocanol group and 5 in the alcohol group), however, the trial was interrupted because of serious complications in patients treated with alcohol (four major bleeding episodes and one esophageal stenosis). The two agents were of comparable efficacy in the small sample of patients studied. The complications were related to the presence of iatrogenic esophageal ulcers which were more frequent (100% vs. 30%) and significantly larger (mean, 1.4 cm vs. 0.7 cm, p less than 0.05) in patients treated with alcohol.

Clinical Trials as Topic↗

Auxological evaluation in patients with DiGeorge/velocardiofacial syndrome (deletion 22q11.2 syndrome).

PURPOSE: Patients with microdeletion of chromosome 22q11.2 (del22q11) were studied, in order to evaluate auxological parameters and to correlate growth patterns with the presence of main clinical characteristics of the syndrome. METHODS: Between January 1995 and March 2000, auxological parameters (weight, height, head circumference, and bone age) of 73 patients with del22q11 were collected. Five subgroups of patients were distinguished: Group I (37 patients) included patients aged between 0.3 and 4 years, Group II (20 patients) included patients aged between 5 and 10 years, Group III (16 patients) included patients aged between 11 and 16.3 years. The presence or absence of some clinical features was correlated to growth patterns. RESULTS: Weight: in Group I, 5 of 37 (13.5%) patients were below the 3rd percentile, 29 of 37 (78.3%) were below the mean percentile, none was overweight; in Group II, 13 of 20 (65%) patients were between the 10th and the 50th percentiles; in Group III, weight corresponded to the 97th percentile in 5 of 16 (31.2%) patients, and in 2/16 (12.5%) adolescents, the weight measurements were even above the 97th percentile. Height: short stature was detected in 7 of 73 (9.6%) of the total patients; the patients with short stature were all < 10 years old; the height was within the normal age in all adolescent patients. Head circumference: it was below the 3rd percentile in 7 of 73 (9.6%), between the 3rd and the 25th percentiles in 36 of 73 (49.3%) patients, between the 25th and the 75th percentiles in 20 of 73 (27.3%) patients, and between the 75th and the 97th percentiles in 10 of 73 (13.7%) patients. Bone age: mean +/- SD bone age was -0.25 +/- 0.78 years. Comparisons: the only statistically significant correlation was that between the presence of feeding difficulties and underweight. CONCLUSION: Auxological parameters of children with del22q11 are characterized by: (1) weight deficiency in the first years of age, (2) weight normalization in the following years, (3) development of obesity in adolescence, (4) short stature in 10% of the patients, (5) normal height in adolescents, (6) slight delay in bone age in infancy, (7) microcephaly in 10% of the patients.

Adolescent↗

Antisperm antibodies in young boys.

The authors report the results of antisperm antibody tests carried out on a group of 159 pre-pubertal boys suffering from various testicular pathologies. The data show that 21% of the patients' sera showed antibody activity against antigens on the sperm of healthy fertile donors. None of the control sera was ASA positive. Some differences were observed in pathology and age distribution of positivity.

Adolescent↗

The enhancing effect of pyridostigmine on the GH response to GHRH undergoes an accelerated age-related reduction in Down syndrome.

Cholinergic agonists are known to potentiate GHRH-induced GH secretion, probably acting via inhibition of hypothalamic somatostatin release. Their effect is reduced in aging and in patients with Alzheimer's disease. This may be the consequence of age-related cholinergic impairment, which, in turn, could cause somatostatinergic hyperactivity leading to GH hyposecretion. As in Down syndrome (DS) neural alterations have been reported similar to those in aging, including cholinergic impairment, we verified the GH response to GHRH (1 microgram/kg i.v. at 0 min) alone or combined with pyridostigmine (PD), a cholinesterase inhibitor (60 and 120 mg, respectively, in children and adults, orally at -60 min) in 15 DS children (13.5 +/- 0.6 years) and in 11 DS young adults (24.0 +/- 1.2 years). Fifteen normal children (11.9 +/- 0.5 years), 15 normal adults (27.3 +/- 0.9 years) and 16 normal elderly (76.3 +/- 1.5 years) were studied as controls. IGF-I levels showed an age-related reduction both in DS (children vs. adults, mean +/- SEM:354.8 +/- 44.9 vs. 204.4 +/- 29.4 micrograms/l, p < 0.02) and in controls (normal children vs. normal adults vs. normal elderly:281.4 +/- 36.3 vs. 175.4 +/- 11.2 vs. 72.5 +/- 6.6 micrograms/l, p < 0.001). The GH response to GHRH in DS children was higher than in DS adults (areas under curve: 1,197.6 +/- 241.5 vs. 434.4 +/- 83.3 micrograms/l/h, p < 0.01). On the other hand, in normal subjects the GHRH-induced GH rise was similar in children and adults (1,056.2 +/- 128.4 vs. 800.8 +/- 124.5 micrograms/l/h) and both were higher than that in elderly subjects (296.0 +/- 61.0 micrograms/l/h, p < 0.001). PD enhanced the GH response to GHRH both in DS and in normal subjects (p < 0.005). The GH response to PD+GHRH was lower in DS adults than in DS children (1,068.1 +/- 145.7 vs. 1,897.4 +/- 198.8 micrograms/l/h, p < 0.001) as well as in normal elderly subjects with respect to that in normal children and normal adults (832.3 +/- 144.7 vs. 2,172.1 +/- 156.1 and 2,347.6 +/- 322.4 micrograms/l/h, respectively, p < 0.001). The GH response to GHRH alone or combined with PD in DS adults was lower (p < 0.01) than that in normal adults and similar to that in normal elderly subjects. In conclusion, the present data demonstrate that the stimulated GH secretion in DS undergoes an accelerated age-related reduction. They also suggest the existence of a precocious impairment of central cholinergic activity in DS, which, in turn, could cause somatostatinergic hyperactivity and reduced GH secretion.

Adolescent↗

McCune-Albright syndrome: a longitudinal clinical study of 32 patients.

We report the diagnostic clinical features and their long term evolution in 32 patients with McCune-Albright syndrome. Patient data are made up of two periods: the first, classified as personal history, is from birth until the time when the diagnosis of McCune-Albright syndrome was made; the second, classified as clinical observation, is from the first observation until the end of follow up. The total duration of these two periods was 9.6+/-2.9 yr; mean age at first observation was 5.7 yr (range 0.7-11 yr). The probability of manifesting main clinical signs according to age was calculated: almost all had skin dysplasia at birth, 50% probability of peripheral precocious puberty in females at 4 years and 50% of bone dysplasia at 8 years of age were found. Other clinical signs had diagnostic relevance when preceding the main signs leading to diagnosis of McCune-Albright syndrome even without specific genetic investigation. The most important clinical manifestations have different evolutions: skin lesions increase in dimensions according to body growth; precocious puberty in females evolves rapidly but periods of regression can be seen in some patients; bone dysplasia in most patients evolves with an increase both in the number of affected bones and in the severity of lesions.

Child↗

[Retroperitoneal liposarcoma].

Retroperitoneal soft-tissue sarcomas are a heterogeneous group of rare and peculiar mesenchymal tumors. They are locally invasive and have a peak incidence in the fifth decade of life. They account for 0.1-0.2% of all solid tumors and 15% of all soft-tissue tumors. Liposarcomas are usually large and occur most frequently in the lower extremities, in the retroperitoneal, perineal and mesenteric region. In the retroperitoneum they grow slowly due to the ability of the abdominal cavity to accommodate these slowly expanding masses. They don't produce symptoms until they are very large and have invaded local tissues. The case of a 61-year old man with a retroperitoneal liposarcoma is reported. The tumor was discovered due to the association of abdominal mass, weight loss and persistent fever. The fever, especially, is present due to a wide tumor necrosis. The diagnosis was suggested by computed tomography. Normally, the interval between start of symptoms and diagnosis is included within three weeks and one year. Surgical complete resection of the mass with splenectomy and local postoperative radiotherapy were performed. The weight of the mass was 8.56 kilograms and the pathological evaluation showed a pleomorphic highly undifferentiated liposarcoma. This histological type normally presents many tumor giant cells, some of which have the features of lipoblasts. The single most important prognostic factor in patients with soft-tissue sarcomas is the histologic grade of the primary lesion. In the last AJCC Staging System the grades are assigned from grade 1 (well differentiated) to grade 3 (poorly differentiated). The present case is grade 3. In the treatment of sarcoma of the retroperitoneum or genitourinary tract, the conventional chemotherapy does not seem effective, while radiotherapy has a little improvement on survival. Local recurrences are frequent, especially in the first three years, often in the absence of distant metastases. When the tumor recurs locally, the best therapy is still to remove the mass. Sometimes, two or more operations may be necessary for the patient. Generally, the prognosis is poor with overall 5-year survival of 15-50%. The patient was admitted in our division 4 months after the first operation with poor medical condition. The patient died nine months after surgery.

Follow-Up Studies↗