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Biomedical subjects

A Camera

Publications and source records attributed to A Camera.

At least 37 records · Page 2Linked to original sources
PubMed1998

Secondary haematological neoplasm after treatment of adult acute lymphoblastic leukemia: analysis of 1170 adult ALL patients enrolled in the GIMEMA trials. Gruppo Italiano Malattie Ematologiche Maligne dell'Adulto.

Between 1983 and 1994 the incidence of secondary haematological neoplasms (SHM) was evaluated in 1170 new cases of ALL enrolled in the GIMEMA trials. Of the 942 patients who achieved complete remission (CR); seven developed a SHM: four AMLs and three NHLs. The median latency from onset of ALL and of secondary haematological neoplasm was 69 months for AML and 61 months for NHL. Three out of four patients with secondary AML were unresponsive to the new chemotherapy and died, whereas the fourth patient achieved a new CR. Among the three NHL cases, two patients are presently alive in CR, whereas the third patient was refractory to chemotherapy and died. The relative risk of haematological malignancy among the GIMEMA trials population, as compared to that of the Italian Cancer Registries, was 15.25-fold higher, and the actuarial estimated cumulative proportion of ALL patients with a secondary haematological neoplasm at 5 and 10 years were 0.59% and 3.63% respectively. The incidence of adult ALL who developed a SHM, although apparently lower than in the paediatric ALL series, was higher when compared to the normal population. The difference between paediatric and adult ALL is probably due to the lack of craniospinal radiotherapy and to the lower doses of epipodoxiphyllotoxins used in adult trials. The higher percentage of childhood ALL with a prolonged event-free survival could result in an increase of secondary neoplasms in these cases, which suggests that secondary haematological neoplasms in adult ALL patients are real, although rare, events.

Adolescent↗
PubMed1997

Treatment of adults with acute lymphoblastic leukaemia in first bone marrow relapse: results of the ALL R-87 protocol.

Sixty-one adults aged <55 years with acute lymphoblastic leukaemia (ALL) in first bone marrow relapse were enrolled in an Italian cooperative study (ALL R-87 protocol) from 12 GIMEMA Institutions. The treatment programme consisted of: (1) an induction phase with intermediate-dose cytarabine (IDARA-C 1 g/m2, 6 h daily infusion x 6 d) plus idarubicin (IDA; 5 mg/m2/d x 6 d) and prednisone (40 mg/m2/d x 21 d), (2) a consolidation phase followed by (3) bone marrow transplant (BMT). Median first complete remission (CR) duration was 8.5 months (range 1-54 months). 34/61 patients achieved CR (56%); 24 (39%) failed to respond and three (5%) died during induction. Most responders (24 patients) could not enter the BMT programme; 15 relapsed early (median time to relapse 2 months); nine were withdrawn due to toxicity and one died in CR of infection. Nine of the 34 CRs underwent BMT (five autologous and four allogeneic). Three of the four allotransplanted patients are alive in continuous CR at 22, 43 and 63 months; only one of the five who underwent an autologous BMT is alive in CR at 46 months. The estimated disease-free survival (DFS +/- SE) at 36 months was 0.16 +/- 0.08 for all responders. Univariate analysis showed that previous therapy was the only prognostic factor influencing DFS. The estimated probabilities of event-free survival (EFS +/- SE) and survival +/- SE at 37 months were 0.09 +/- 0.04 and 0.10 +/- 0.04, respectively. The EFS was significantly better in patients with a preceding CR > or = 24 months, compared to those with a shorter first remission. Our results confirm the tolerance and efficacy of IDARA-C plus IDA in inducing CR in poor-risk adult ALL. Even though the number of transplanted patients was small, allogeneic BMT seems to give a real opportunity of cure in this category of patients.

Adolescent↗
PubMed1996

In vitro photoinhibition by psoralen and ultraviolet A radiation of human hematopoietic progenitors.

The in vitro sensitivity of human hematopoietic progenitors to PUVA, 8-MOP and UVA alone was investigated. 8-MOP alone at final concentrations of 150, 200, 600 and 1,000 ng/ml did not modify colony growth of circulating and bone marrow erythroid (BFU-E), myeloid (CFU-GM) and immature (CFU-GEMM) hematopoietic progenitors obtained from normal controls. The exposure of the same progenitors to increasing doses of UVA, up to 12 J/cm2, progressively decreased hematopoietic colony growth (with estimated 50% inhibition occurring at about 5 J/cm2). In vitro PUVA treatment (8-MOP 200 ng/ml followed by UVA 5 J/cm2) caused 90% growth inhibition of circulating and bone marrow hematopoietic progenitors. In addition, the treatment completely inhibited the formation of spontaneous erythroid colonies, obtained from 5 polycythemic patients, that are considered to be a marker of this neoplastic disease. PUVA cytotoxicity was assessed by the colorimetric MTT assay. The percentage of cell death after PUVA exposure was 29 +/- 10% for both peripheral and bone marrow mononuclear cells. Our findings indicate that 8-MOP alone is not toxic to hematopoietic progenitors whereas UVA treatment determines in vitro a dose-dependent inhibition of the clonogenic capacity of normal hematopoietic cells. PUVA treatment enhances this effect, causing a quite complete inhibition of hematopoietic progenitors colony formation from normal donors and spontaneous BFU-E colony formation from polycythemic patients.

Cell Survival↗
PubMed1996

[Usefulness of computerized dynamic capillaroscopy in the monitoring of treatment of progressive systemic sclerosis with synthetic prostacyclin].

The aim of this study was to evaluate the variations of blood flow velocity in the skin microcirculation before and after vasoactive drugs' administration. Eight patients affected by progressive systemic sclerosis have been treated with synthesis prostacyclin for six hours during a period of fifteen days at the standard dosage of 0.5-2 ng/kg/min. We used computerized dynamic capillaroscopy with Capiflow technique to evaluate cutaneous microcirculation before and after the treatment. The Capiflow technique is a new videophotometric technique to study and quantify the diameter, density and velocity blood flow in the nutritional skin capillary. The quantitative analysis of blood flow velocity detected an increase, statistically significant, at the end of the treatment. The statistic analysis was performed by "t Student" test.

Adult↗
PubMed1996

Relapse of acute lymphoblastic leukemia during pregnancy.

A young woman had a late relapse from acute lymphoblastic leukemia during her first pregnancy at the 17th week of gestation and decided not to interrupt the pregnancy. A partial remission was obtained with mild chemotherapy (vincristine and prednisone) in a month, then the treatment was intensified, but only partial remission was achieved. A cesarean section was performed at the end of the 7th month, with the delivery of a normal male infant. The patient died of refractory leukemia 9 months later. The baby is now 3 years old with no medical problems. This case suggests that: a) pregnancy might be associated with an increased risk of relapse in women in continuous complete remission from leukemia; b) chemotherapy, even intensive, can be safely administered to pregnant women without damaging the fetus, after the first trimester.

Adult↗
PubMed1995

F-syndrome (F-form of acro-pectoro-vertebral dysplasia): report on a second family.

We report on a father and daughter in the second known family affected with F-syndrome. The first family, with 8 affected members, was reported by Grosse et al. [1969: BD:OAS V (3):48-63]. F-syndrome, an autosomal-dominant trait, is mainly characterized by acral defects that may also involve the sternum and the lumbosacral spine. Synostoses between capitate and hamate, and between talus and navicular, are invariably present; other carpal and tarsal bones are sometimes incorporated into the fusion. The hand malformation is principally a malformation of the first 2 rays. In our patients, the short and malformed thumb was webbed with the index finger, which was radially deviated with duplication of the middle and distal phalanges. In the feet, polydactyly and severe metatarsal and toe anomalies were present. The father had a prominent sternum with pectus excavatum, whereas the daughter had no sternal deformity. Both of them had a mild failure of fusion of posterior arch L5 and/or S1.

Abnormalities, Multiple↗
PubMed1995

Synpolydactyly (type II syndactyly) with aplasia/hypoplasia of the middle phalanges of the toes: report on a family with eight affected members in four generations.

We describe a new family with synpolydactyly (syndactyly type II) with 8 affected members in 4 generations. Aplasia/hypoplasia of the middle phalanges of the toes was also noted. In our opinion, this anomaly represents a frequent manifestation of synpolydactyly. No other major skeletal or extraskeletal malformations were present.

Adult↗
PubMed1995

Sertraline treatment for premature ejaculation.

This study was designed to examine the efficacy and safety of sertraline as a treatment for premature ejaculation. Fifty-two heterosexual male patients with self-reported premature ejaculation were randomly assigned to receive either sertraline or placebo. After a 1-week placebo washout period, the dose was titrated during treatment weeks 1 to 3 from 50 to 200 mg of sertraline per day until clinical response was optimal or dose-limiting adverse experiences emerged. Medication was maintained at this level through week 8. The primary outcome measures used to assess efficacy included patient assessment of time to ejaculation (from penetration), number of successful attempts at intercourse, and incidence of ejaculation during foreplay. Sertraline treatment produced significant improvements relative to placebo in time to ejaculation and number of successful attempts at intercourse, as well as overall clinical judgements of improvement. Medication was well tolerated by most patients. Because sertraline therapy caused significant prolongation of time to ejaculation, this agent may be useful as a treatment for selected patients with premature ejaculation.

1-Naphthylamine↗
PubMed1994

Pitfalls of genetic counselling in brachydactyly type C.

A familial case of brachydactyly type C is presented in which hand radiographs of the proposita's parents was considered normal. The metacarpophalangeal profile pattern of the mother's hands demonstrated minimal manifestations typical of brachydactyly type C.

Adult↗
PubMed1994

Sponastrime dysplasia: report on a male patient.

We report on a boy in the fourth family known to be affected with sponastrime dysplasia. The clinical and radiological features are presented. In this patient, the ossification delay was severe. The pattern of inheritance of this condition is discussed.

Bone Diseases, Developmental↗
PubMed1994

Treatment of primary refractory and relapsed acute lymphoblastic leukaemia in children and adults: the GIMEMA/AIEOP experience. Gruppo Italiano Malattie Ematologiche Maligne dell'Adulto. Associazione Italiana Ematologìa ed Ocologia Pediatrica.

One hundred and forty-seven patients aged < 55 years with advanced acute lymphoblastic leukaemia (ALL) were enrolled in an Italian cooperative study (ALL R-87). This protocol consists of an induction phase with idarubicin (IDA) plus intermediate-dose cytarabine (IDARA-C), followed by a consolidation phase and bone marrow transplant (BMT). Complete remission (CR) was achieved in 97/147 patients (66%) with a CR rate of 77% in children versus 51% in adults (P < 0.01). 48 responders (50%) underwent BMT. Probability of event-free survival (EFS +/- SE) was 10.2 +/- 3.1% at 56 months. EFS was 14.3 +/- 4.51% at 56 months for children versus 3.8 +/- 3.41% at 37 months for adults (P < 0.0001). Among patients treated in first relapse, EFS was 14.2 +/-7.79% for patients with CR > 18 months verus 6.6 +/- 3.17% for those with CR < 18 months (P < 0.0001). Projected disease-free survival (DFS +/- SE) was 15.4 +/- 4.61% at 55 months for all responders and 43.3 +/- 14.34% at 52 months for allografted patients. Projected overall probability of survival +/- SE for all patients was 18.8 +/- 4.13% at 56 months. This study confirms the efficacy of IDA plus IDARA-C in poor-risk. ALL patients. A more intensive post-remission therapy or alternative approach must be designed to improve long-term results.

Adolescent↗
PubMed1994

New X linked spondyloepimetaphyseal dysplasia: report on eight affected males in the same family.

We report on a probably new form of spondyloepimetaphyseal dysplasia (SEMD) with an X linked inheritance pattern. Eight males were affected in the same family. We were able to examine three adult patients and we studied the skeletal radiological aspect of one of these patients at 2 years 6 months and at 9 years of age. The main clinical features are severe short trunked dwarfism, brachydactyly, normal facies, and normal intelligence. Radiologically, the diaphyses of all the long bones are short and broad. The epiphyses of the distal portion of the femora and those of the proximal and distal portions of the tibia are embedded in their metaphyses and there is marked narrowing of the intercondylar groove. There is moderate platyspondyly. Several vertebrae show an anterior tongue in infancy and severe irregularities of the upper and lower surfaces are present in adulthood. The 11th or 12th thoracic vertebra is wedge shaped. The pelvis is narrow. The distal ulnae and fibulae are disproportionately long. The hands show radial deviation and brachydactyly is present in the hands and feet. This X linked SEMD was not detectable at birth.

Child↗
PubMed1994

[Isolated macrodactyly or extremely circumscribed Proteus syndrome?].

We report on two unrelated female patients with unilateral macrodactyly of the 1st and 2nd toes and of the 3rd, 4th, and 5th toes, respectively. In addition, they had a local plantar soft tissue lump and radiographically abnormally broad phalanges of the affected toes. These clinical manifestations may represent either an isolated macrodactyly or an extremely localized form of Proteus syndrome.

Abnormalities, Multiple↗
PubMed1994

[Oculodentodigital dysplasia: report of 2 familial cases].

We describe a father and his child with bilateral syndactyly of fingers IV and V and with pinched nose, hypoplastic alae nasi and thin anteverted nares. The patients also showed a small nodule on the tongue tip. Both had no ocular or dental anomalies. The clinical features of our patients resemble those of the patients described by Brueton et al. The hypothesis that the oculodentodigital dysplasia may belong to a contiguous gene spectrum could be confirmed.

Abnormalities, Multiple↗
PubMed1994

[Oromandibular-limb hypogenesia spectrum: report of a case of lower ankylogloxia ++ associated with bilateral apodia].

We report on a newborn with ankyloglossia inferior, micrognathia, and bilateral apodia. The tongue was small and adherent to the floor of the mouth. It has been proposed that oromandibular-limb hypogenesis sequences are due to an interruption of embrionic blood supply. Interruption or reduction of uterine artery blood flow appear to be pharmacologically or physically induced. In our case, there was no prenatal history of chorionic villus sampling, of drug or teratogens exposure, and of intrauterine infections.

Abnormalities, Multiple↗