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Biomedical subjects

R Riccardi

Publications and source records attributed to R Riccardi.

125 records · Page 7Linked to original sources
PubMedDate not supplied

Carcinoid tumors of the appendix in children: two case reports and review of the literature.

Carcinoid is the most common tumor of the appendix. Reported incidence in pediatric population is 1 per 100,000 per annum. Clinical presentation like acute appendicitis is frequent, but carcinoid tumor can be an incidental finding during surgical procedures other than appendectomy. Size and depth of invasion are important prognostic criteria and tumors larger than 2 cm metastasize more frequently than smaller ones. Simple appendectomy is considered appropriate treatment, while right colectomy is indicated in tumor bigger than 2 cm. The authors report 2 cases of carcinoid tumors of the appendix in children, smaller than 2 cm treated with appendectomy alone, and disease free at follow-up.

Appendiceal Neoplasms↗
PubMedDate not supplied

Microangiopathic hemolytic anemia complicating a postneonatal pertussis pneumonia.

The authors describe a 2-month-old female with microangiopathic hemolytic anemia complicating a pertussis pneumonia. The baby was admitted to the Pediatric Intensive Care Unit for monitoring and treatment. Remission occurred without sequelae, but multiple hemotransfusions were needed in the acute phase. This represents the first reported case of such a complication in childhood.

Anemia, Hemolytic↗
PubMedDate not supplied

Second case of hepatoblastoma in a young patient with Simpson-Golabi-Behmel syndrome.

Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked overgrowth syndrome associated with multiple congenital anomalies caused by a mutant X-linked recessive trait. The authors report on a 14-month-old male patient affected by hepatoblastoma. As far as is known, this is the second patient reported with SGBS and hepatoblastoma. The observations emphasize that an increased risk of neoplasia in SGBS must be kept in mind, especially in young patients.

Genetic Diseases, X-Linked↗
PubMedDate not supplied

Early reactive neutrophil changes in an infant with Kawasaki syndrome.

An infant with Kawasaki syndrome presenting with fever, rash, and a high percentage of neutrophils with toxic granulation is reported. The peripheral blood smears of the patient showed a high proportion of neutrophils with marked vacuoles and toxic granulations. The reactive changes in neutrophils could be helpful in the early detection of the disease, especially in infants younger than 6 months of age who are at high risk for development of coronary artery disease.

Diagnosis, Differential↗
PubMedDate not supplied

Pulmonary hemorrhage complicating a typical hemolytic-uremic syndrome.

We describe a case of pulmonary bleeding and subsequent acute respiratory distress syndrome (ARDS) in a 20-month-old female suffering from a typical postdiarrheal hemolytic-uremic syndrome (HUS). Acute renal failure was treated early by peritoneal dialysis. It is of interest to underline that thrombocytopenia or any coagulative impairment was absent when this complication occurred, and spontaneous diuresis recovery was ongoing. All examinations failed to identify a unique site of bleeding in the main stem bronchi or trachea. Complete renal and clinical recovery was obtained in spite of this very uncommon complication. Intensivists should be aware of this rare and potentially fatal complication of typical (D+)HUS.

Acute Kidney Injury↗
PubMedDate not supplied

Alloimmune neonatal neutropenia treated with rHuG-CSF.

Infants with alloimmune neonatal neutropenia (ANN) may be at risk of life-threatening infection. Various modalities of treatment have been attempted but with differing results. We describe a case treated with rHuG-CSF. The use of rHuG-CSF should be considered in children with ANN especially when associated with life-threatening infection.

Female↗
PubMedDate not supplied

[Neutropenia in childhood].

The discovery of a neutropenia is a common problem in pediatric population. Some children with neutropenia have an uncomplicated clinical course and require no treatment. However, some patients are more severely affected and present a management challenge for the pediatrician and for the hematologist. The clinical approach to these children is directed primarily to understand the etiology of neutropenia in order to define a correct therapeutic behaviour, such as glucocorticoids, intravenous immunoglobulins or, more recently, rhG-CSF. In our article we have reviewed the etiology, the clinical course and the therapeutic options of constitutional and acquired neutropenias.

Child↗
PubMedDate not supplied

Biology of metaiodobenzylguanidine interactions with human neuroblastoma cells.

[131I]Metaiodobenzylguanidine (131I-MIBG) is selectively taken up and stored by tumors derived from the neural crest and is utilized in the diagnosis and treatment of neuroblastoma (NB). Variable MIBG uptake has been observed, although the underlying mechanisms are not known. We have studied the uptake kinetics of 125I-MIBG and uptake characteristics of NB cell clones with different phenotypes (SH-SY5Y and SH-EP1, the neuroblastic and the substrate-adherent sublines of SK-N-SH respectively, BE(2)-M17 and LA-N-1n with neuroblastic phenotype). We have been able to correlate the MIBG uptake with the neuroblastic phenotype: a specific uptake system satisfying all the characteristics of the neuronal uptake-1 (temperature dependency, sodium dependency, high affinity, saturability and imipramine sensitivity) was observed in all the neuroblastic sublines. In contrast, MIBG accumulation was a passive diffusion phenomenon in the substrate-adherent clone SH-EP1. In addition, terminal neuronal differentiation induced in SH-SY5Y by retinoic acid caused a marked increase of the uptake and retention of MIBG. Our findings may be pertinent to an understanding of the variability of the MIBG uptake in vivo.

3-Iodobenzylguanidine↗
PubMedDate not supplied

The use of [131I]metaiodobenzylguanidine in the treatment of neuroblastoma after conventional therapy.

Eleven cases of neuroblastoma (10 males and 1 female; 9 aged 1-13 years, and two aged 17 and 38 years, respectively) ten of which were refractory to chemotherapy, were submitted to treatment with [131I]metaiodobenzylguanidine (131I-MIBG). The therapeutic procedure consisted essentially of single doses (2.6-9.5 GBq) of 131I-MIBG mostly split into two parts, administered by slow i.v. infusion and given in several therapeutic courses, usually at 1-2 month intervals. The treatment resulted in: 1 complete response, 1 partial response, 1 minor response, 4 stabilized diseases and 2 progressive diseases (two patients were not evaluable due to rapid progression of the disease). Pain relief was observed in all cases and particularly in four patients who suffered severe tumor pain. The major side-effects recorded were: hypertensive crises over a 6-day period in one case, fever lasting a few days in another and bone marrow depression in two intensively pretreated patients. A slight hematologic toxicity was observed, however, in almost all cases.

3-Iodobenzylguanidine↗
PubMedDate not supplied

[131I]metaiodobenzylguanidine in neuroblastoma patients at diagnosis.

Treatment of resistant neuroblastoma with high dosage [131I]metaiodobenzylguanidine (131I-MIBG) appears effective since encouraging results have been obtained so far even in patients with very advanced, intensively pre-treated disease. We have already reported a stage III NB patient treated at diagnosis, who is at present in complete remission with a 4-year follow-up. To further explore the potential role of this new drug in untreated patients, we administered radionuclide to two children with stage III neuroblastoma. Both cases received 131I-MIBG at relatively low doses, and showed a significant reduction of the tumor mass and, interestingly enough, no evidence of 131I-MIBG uptake of a tracer dose in the remaining tumor. Particularly in case 1, the permanence and subsequent progression of the part of the tumor mass without 131I-MIBG uptake, after therapeutic doses of 131I-MIBG which apparently destroyed the 131I-MIBG-positive cell population, clearly suggest heterogeneity at diagnosis, with a dual neuroblastoma cell population, one with 131I-MIBG uptake and the other without. Aside from the biological implications of the heterogeneous MIBG uptake in neuroblastoma at diagnosis, our findings suggest that in stage III neuroblastoma patients even a relatively small dose of 131I-MIBG administered at diagnosis is sufficient to either completely destroy the primary tumor, as reported by our group, or to destroy that part of the tumor which shows 131I-MIBG uptake (as in the present cases), without any significant hematologic toxicity. Furthermore, a single course of 131I-MIBG at the dosage employed here does not appear to jeopardize the subsequent use of chemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)

3-Iodobenzylguanidine↗
PubMedDate not supplied

[Relapse modalities in medulloblastoma].

We evaluated the modality of relapse and progression of medulloblastoma in 45 patients observed between 1981-1991. Children aged over 2 years were treated with surgery and postoperative radio-chemotherapy; among 12 children younger than 2 years, 8 were treated with surgery and chemotherapy and 4 with postoperative irradiation of remaining tumor. Cerebrospinal fluid shunting system was placed in 32 patients (71.1%). Patients were organized into three groups: group I (25 cases) = total tumor removal; group II (11 cases) = subtotal tumor removal; group III (9 cases) = partial tumor removal. 22 children died (48.8%: 10 of group I; 7 of group II; 5 of group III) at variable time interval from the operation during the following period. Among 23 alive patients, 3 are surviving with recurrence and progression of disease (all of group I), 20 are disease-free (44.4%: 12 of group I; 4 of group II; 4 of group III). Average postoperative follow-up period: 6 years for group I/II and 2 years for group III. Even if disease-free children are those treated by total-subtotal surgical removal (while local relapse occurs principally in patients treated by partial surgical resection: 44.4% of local relapse vs 12% of group I and 27% of group II), extensive surgery does not exclude possibility of metastatic localization in cerebrospinal axis, that occurs in 1/5-1/6 of all the patients without differences in each group. CSF shunting system did not cause extraneural metastases in our patients. We obtained long term survival rates or apparent recovery in almost 50% of cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗