Proteomic evaluation of GCF in the development of pregnancy related periodontal disease: a pilot clinical study.

OBJECTIVE: This pilot study analyzed the possible changes of periodontal disease status in female patients during the period following pregnancy. Both clinical and laboratory data were collected and analyzed. PATIENTS AND METHODS: A non-randomized controlled clinical trial was conducted by the Periodontal Department of the Dental Clinic in collaboration with the Pediatrics Department, at Fondazione Policlinico Universitario A. Gemelli, Rome, Italy. Ten female patients, who completed the pregnancy without complications, were enrolled in this research protocol forming the experimental group. During the first post-partum days, gingival crevicular fluid (GCF) samples were collected and analyzed with high-performance liquid chromatography associated with high-resolution mass spectrometry (HPLC ESI MS); periodontal parameters as pocket depth (PD), full mouth plaque score (FMPS) and full mouth bleeding score (FMBS) were recorded, and a professional oral hygiene session was performed. The same protocol was applied after three months with the same patients forming the recall group. A control group was created in order to compare the results with GCF samples from 10 not pregnant fertile women. RESULTS: Student's t-test has been used to evaluate the statistical significance of the collected data. Mean levels of PD decreased from 3.75 mm ± 1.2 mm after pregnancy to 2.88 mm ± 0.85 mm at three months post-partum (p<0.01). Mean value of FMPS and FMBS decreased from 21.8% ± 1.35% and 34.27% ± 1.5% after pregnancy to 13% ± 2.81% and 17.55% ± 2.84% at three months post-partum, respectively (p<0.05). The concentration of each analyzed peptide has changed in relation to the general improvement of the periodontal status at three months post-partum. CONCLUSIONS: Pregnancy may be associated with an increased risk of periodontal disease. Both clinical and laboratory data have demonstrated that a professional oral hygiene session can affect the course of pregnancy inducing periodontal diseases allowing a faster healing and restitutio ab integrum.

Propofol/alfentanil and propofol/ketamine procedural sedation in children with acute lymphoblastic leukaemia: safety, efficacy and their correlation with pain neuromediator expression.

Invasive procedures, such as the lumbar puncture, can cause anxiety and pain in children undergoing treatment for acute lymphoblastic leukaemia (ALL). We investigated the safety and efficacy of two different protocols for analgo-sedation in 20 children with ALL undergoing lumbar puncture. We have conducted a prospective, cross-over study. Protocol A was composed of an association between propofol and alfentanil. Protocol B consisted in the combination of propofol and ketamine. We also evaluated the levels of nerve growth factor, substance P and enkephalins in the cerebrospinal fluid of these patients. All patients showed a satisfactory sedation and analgesia. We found a statistically significant difference of vital parameters between protocol A and protocol B, while there were no significant differences between sedation scores and the other parameters evaluated. Patients in protocol A showed a higher incidence of major side effects, such as respiratory depression. Pain neuromediator levels did not show any statistical difference between the two groups. This study shows that both protocols are effective to obtain a good sedation and analgesia in children with ALL undergoing lumbar puncture, but the association between propofol and ketamine appears to be safer due to the lower incidence of side effects.

Defective platelet responsiveness to thrombin and protease-activated receptors agonists in a novel case of gray platelet syndrome: correlation between the platelet defect and the alpha-granule content in the patient and four relatives.

BACKGROUND: We report a novel case of gray platelet syndrome (GPS). A 14-year-old boy had bleeding diathesis, mild thrombocytopenia, giant platelets with severe defect of alpha-granule secretory proteins, myelofibrosis and splenomegaly. METHODS AND RESULTS: Platelet function studies showed a marked reduction of aggregation and Ca(2+) mobilization by thrombin, protease-activated receptor 1 (PAR1)-activating peptide (AP) and PAR4-AP, PAR1 expression at 55% of normal levels, and a more than two hundred fold reduction of in vitro whole-blood thromboxane B(2) (TXB(2)) production. Sequencing of coding regions of the PAR1 gene failed to show abnormalities. This patient was initially classified as a sporadic case of GPS, as electron microscopy failed to identify giant platelets and/or alpha-granule deficiency in his relatives. However, further studies on the father and three other relatives showed a relative lack of platelet alpha-granule proteins by immunofluorescence microscopy, a defective platelet response to PAR4-AP, and severely reduced in vitro whole-blood TXB(2) production. On this basis, we suggest that in this family, GPS was transmitted in a dominant fashion with highly variable penetrance. CONCLUSIONS: Our study suggests that current diagnostic criteria fail to identify some patients with a mild GPS phenotype and that such patients might be identified by the methods cited above. It also better characterizes the pathogenesis of defective platelet responses to thrombin, and raises interesting questions on the correlation between abnormal PAR function and the lack of alpha-granule content in GPS.

Safety and efficacy of fentanyl administered by patient controlled analgesia in children with cancer pain.

BACKGROUND: Pain is the most common discomfort experienced by children with cancer and occurs in almost 89% of patients in an advanced stage of the disease. It is most often not adequately treated because of inexperience and unfounded fears of analgesic treatment. In adults, patient controlled analgesia (PCA) is widely administered, while in children with moderate to severe cancer pain its use is still under evaluation for safety and efficacy. GOALS OF WORK: To evaluate the efficacy and safety of fentanyl administered by PCA in children with cancer pain. MATERIALS AND METHODS: Eighteen children (range 6 to 15 years) with moderate to severe pain were enrolled and treated with fentanyl by PCA plus background infusion (BI) (BI of 1 microg/kg/h with booster doses of 1 microg/kg by intravenous route). To evaluate efficacy and safety of the analgesic treatment, different subjective and objective parameters were monitored at 4-h intervals. In addition, patients' satisfaction was assessed by a questionnaire at the end of the treatment. MAIN RESULTS: All children experienced a good degree of analgesia and did not require any other analgesic drug during the treatment. Both subjective and objective parameters improved after starting pain-relieving treatment and no major side effects occurred. The questionnaire administered to the children showed a high grade of satisfaction. CONCLUSIONS: PCA plus BI with fentanyl administered by intravenous route is a safe and efficacious method for analgesia in children with moderate to severe cancer pain. Our policy of fentanyl-treatment did not show any major side effects.

Successful treatment with oral valganciclovir in immunocompetent infant with gastrointestinal manifestations of cytomegalovirus infection.

A 3-month-old male infant was admitted to hospital with anemia. Follow-up controls revealed the presence of specific cytomegalovirus (CMV) antibodies. Virus was isolated from urine, blood, and saliva. At 7 months of age, he presented with melena. Polymerase chain reaction (PCR) of biopsy samples from the duodenum was positive for CMV. Anemia resolved after starting antiviral therapy with oral valganciclovir.

Chronic idiopathic thrombocytopenic purpura in children: case reports of spontaneous recovery without splenectomy.

Children with chronic idiopathic thrombocytopenic purpura generally show a favorable outcome with a high spontaneous recovery rate even many years after the initial diagnosis. In this retrospective study, 5 out of 12 children with chronic ITP achieved a spontaneous recovery. A careful follow-up appears to be adequate for most of the patients, reserving splenectomy to the rare severely affected patients.

Phase II trial of temozolomide in children with recurrent high-grade glioma.

PURPOSE: The objective of the study was to evaluate the efficacy and toxicity of Temozolomide (TMZ) administered for 5 consecutive days in three daily dosing in children with recurrent or refractory high-grade glioma. PATIENTS AND METHODS: Twenty-four patients with a median age of 10.5 years were enrolled onto this open-label, multicenter, phase II study. The patients were previously treated with surgical resection (17 of 24), radiotherapy (19 of 24) and chemotherapy (18 of 24). Therapy was administered orally three times a day for 5 consecutive days at the dose of 200 mg/m(2)/dx5 for chemotherapy naive patients. In patients heavily pretreated with chemotherapy the starting dose was of 150 mg/m(2)/dx5. RESULTS: A total of 95 cycles were administered. The median progression free-survival (PFS) was 3 months for the entire group while disease stabilization was obtained in 7 patients (29.1%), all with supratentorial tumors. No CR or PR was observed. TMZ treatment showed a limited toxicity. Thrombocytopenia was the most common hematological adverse effect. Our data suggest a marginal activity of TMZ in children with recurrent high-grade glioma.

The lumbar puncture in pediatric oncology.

In pediatric oncology, LPs are frequently performed for diagnostic and therapeutic purposes. A LP procedure may be helpful in diagnosing many diseases and disorders. In addition, a LP may be performed therapeutically, to inject medications directly into the spinal canal. Intrathecal administration of antineoplastic drugs allows to bypass the selective filter of BBB and to achieve significant concentrations of the antineoplastic agents in CSF reducing the likelihood of systemic toxicity. Lumbar puncture is generally well tolerated but might be characterized by several disadvantages and risks.

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.

Intrathecal chemotherapy with antineoplastic agents in children.

Intrathecal chemotherapy with antineoplastic agents is mainly utilised in children with leukaemia and lymphoma, and in selected brain tumours. In these diseases, intrathecal use is restricted to methotrexate (MTX), cytosine arabinoside (Ara-C) and corticosteroids. A number of other agents are, at the present time, under evaluation. Intrathecal MTX administered sequentially with systemic high dose MTX infusion prolongs therapeutic cerebral spinal fluid (CSF) levels of the drug. Prolonged therapeutic CSF levels can also be achieved by giving repeated small intrathecal doses of MTX over an extended period in selected patients, with an implanted Ommaya reservoir. In the CSF, the metabolic inactivation of Ara-C is significantly lower than in plasma with a CSF clearance similar to the rate of CSF bulk flow. A slow-release formulation of Ara-C may be given intrathecally, resulting in a prolonged cytotoxic concentration in the CSF. CNS relapse and neurotoxicity in patients with acute lymphoblastic leukaemia, especially younger children, may be reduced by using age-related dosing of intrathecal MTX and Ara-C. Hydrocortisone is used in combination with MTX and Ara-C for so-called 'triple intrathecal chemotherapy' in the treatment of meningeal leukaemia. Intrathecal thiotepa does not appear to be advantageous over systemic administration in patients with brain and meningeal leukaemia. Monoclonal antibodies, reactive with tumour-associated antigens, can be used as delivery systems for chemotherapeutic agents and radionuclides. However, the development of this new approach is currently under evaluation in larger clinical studies. Neurological adverse effects may be expected with intrathecal chemotherapy and are increased by high dose systemic therapy, concomitant cranial radiotherapy or meningeal infiltration by neoplastic cells. Inadvertant intrathecal administration of antineoplastic agents that are indicated for systemic administration only, is dangerous and may result in a fatal outcome.

Immune tolerance by intermittent factor VIII boluses in two high responder hemophilia A patients.

Immune tolerance (IT) in hemophilia A patients with anti-factor VIII antibodies is generally based on daily factor VIII administrations. Here we report the preliminary results of an immune tolerance regimen based on recombinant high-dose (400 U/kg) factor VIII boluses administered at 48-hour intervals. Two high responder hemophilia A patients aged 2 and 3 years received this treatment without the need of permanent venous access. In both cases the IT regimen caused an anamnestic response of less than three weeks' duration and an antibody reduction to less than 5 Bethesda units was achieved in about 8-10 weeks. In the child with a more prolonged follow-up the inhibitor became undetectable after four months and factor VIII recovery at 6 months was > 85%. This intermittent high-dose regimen seems to be effective in rapidly inducing immune tolerance and seems particularly suitable for very young children in whom it may be useful to avoid the risks and to reduce the psychological burden of permanent venous access.

Management of idiopathic thrombocytopenic purpura in children: a single institution experience.

Idiopathic thrombocytopenic purpura (ITP) is a relatively common hematologic disease in childhood. ITP is often self-limiting and is characterized by a good clinical outcome. About 10-20% of cases can have a chronic course. In our retrospective analysis we have evaluated 45 patients affected by ITP from January '92 to December '97. Thirty-seven patients (82%) met the criteria of acute ITP and 8 (18%) had chronic ITP. Patients were stratified into 3 categories based up on the type of treatment received: no treatment, steroids, steroids and IVIG. In our series children treated with oral prednisone showed a slightly faster recovery in the first days from treatment. We suggest the use of steroids in children with low platelet count and signs and symptoms of bleeding.

[Rhabdoid tumor of the kidney: a rare malignant neoplasm of infancy. A case report and review of the literature]. / Tumore rabdoide del rene: una rara neoplasia maligna infantile. Descrizione di un caso e revisione della letteratura.

Rhabdoid tumor of the kidney (RTK) is a quite rare malignant neoplasm of early childhood. It has a very unfavourable prognosis, since it tends to give early metastases and shows a poor response to chemotherapy regimens. We report a case of an infant with RTK, who had a rapidly progressive course. Based upon our case and the review of the literature, we would like to stress the importance of a differential diagnosis with another kidney cancer, namely Wilms tumor, which is more frequent and has by far a better prognosis.

[Intracranial hemorrhage in congenital factor II deficiency]. / Emorragia endocranica nel deficit congenito del fattore II.

A case of congenital defect of factor II is reported. It concerns a newborn with a not traumatic haematoma due to congenital hypoprothrombinaemia, which is rarely described in scientific literature.