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Biomedical subjects

A Turconi

Publications and source records attributed to A Turconi.

10 recordsLinked to original sources

Sirolimus in pediatric renal transplantation.

Side effects of calcineurin inhibitors (CNIs) include nephrotoxicity and hypertension. Moreover, children have a higher risk of infections and posttransplantation lymphoproliferative disorders. We retrospectively evaluated the efficacy and safety of Sirolimus (SRL) in 18 patients, who were 10.52 +/- 5.03 years at time of transplantation and received a CNI as the core immunosuppression. The most common indications for starting SRL therapy were chronic allograft nephropathy, Epstein-Barr virus-associated neoplasia, and thrombotic microangiopathy. The patients were converted to SRL at 49.14 +/- 45.9 months posttransplantation. Mean follow-up after the switch to SRL was 13.83 +/- 7.24 months. All patients who began SRL therapy remained on that medication. We observed a significant improvement (P < .05) in glomerular filtration rate assessed using the Schwartz formula at 3 months, which was sustained thereafter. There were no changes in proteinuria, plasma lipids, and platelet number. Although the prevalence of hypertensive patients decreased during follow-up, it was not significant. There was one steroid-sensitive, acute rejection episode. Serious adverse events included 1 death due to a relapse of B lymphoma, 1 sepsis, and 1 pancreatic pseudo-cyst. Adverse events were present in 17% of patients: 3 Herpes Simplex infections, and 1 dose-related lymphedema. Further studies are necessary to assess the impact of adverse events in the pediatric transplant population receiving SRL as immunosuppression.

Child↗

Open-label, multicenter study on the safety, tolerability, and efficacy of Simulect in pediatric renal transplant recipients receiving triple therapy with cyclosporin, mycophenolate, and corticosteroids.

INTRODUCTION: Basiliximab is a monoclonal antibody directed to the interleukin-2 receptor. Several studies have demonstrated both its efficacy and safety. Even with the use of polyclonal antibodies in renal pediatric transplant recipients, the local incidence of steroid-resistant rejections has been close to 10% of the total incidence of acute rejection episodes (AREs). An open, multicenter prospective study was performed to assess the safety tolerability, and efficacy of induction with basiliximab in renal pediatric transplant patients receiving cyclosporine, mycophenolate, and steroids. MATERIALS AND METHODS: Eighteen patients (8 boys) of mean age 11.9 +/- 4.5 years and body weight 32 +/- 15 kg received cadaveric (n = 7) or living (n = 11) donor grafts. Simulect was administered on days 0 and 4. Efficacy was assessed by the incidence of biopsy-proven acute rejection (BPAR). Safety assessment consisted of a description of the adverse events (AEs). RESULTS: Six BPAR (Banff I and II) occurred in 5, (27.7%) children all of which were steroid responsive. Creatinine levels at day 7 and months 3, 6, and 12 were 1.6 +/- 1.5 mg/dL, 1.0 +/- 0.4 mg/dL, 1.0 +/- 0.5 mg/dL, and 1.0 +/- 0.4 mg/dL, respectively. Schwartz calculation at 12 months was 71 +/- 15 mL/1.73 m2 AEs were hypertension (12), anemia (9), abdominal pain (8), metabolic acidosis (8), nausea (7), diarrhea (2), gingival hypertrophy (2), hirsutism (2), lymphocele (2), and infections (15). No deaths, graft losses, PTLDs, or malignancies were observed. CONCLUSIONS: No steroid-resistant AREs, were observed in this pediatric group using basiliximab. The Schwartz calculation at 12 months was 71 +/- 15 mL/min/1.73 m2.

Adrenal Cortex Hormones↗

Is fibrin glue useful in preventing dialysate leakage in children on CAPD? Preliminary results of a prospective randomized study.

Children on continuous ambulatory peritoneal dialysis (CAPD) have a higher incidence of dialysate leakage than patients receiving cycler dialysis. To date, fibrin glue has been used to treat dialysate leakage but not as a method of prevention. Therefore, we conducted a prospective study in which 20 catheters were implanted in 19 children, with each catheter randomly assigned to either the treatment group (1 mL of fibrin glue added to peritoneal cuff suture) or the control group. There was no difference in mean patient age, elapsed time between catheter implantation and first catheter usage, and albumin concentration between the two groups. Dialysate leakage was detected in 4 patients in the control group, 3 of whom underwent early initiation of dialysis, but none of the patients in the treatment group (including 4 with early dialysis) had a similar problem. In summary, these preliminary data suggest that fibrin glue may be of benefit when applied at the time of catheter implantation. Further experience is necessary before this method can be routinely recommended.

Adolescent↗

Thyroid function and serum thyroid binding proteins in prepubertal and pubertal children with chronic renal insufficiency receiving conservative treatment, undergoing hemodialysis, or receiving care after renal transplantation.

OBJECTIVE: The abnormalities reported in some thyroid function tests in children with renal disease could be adaptive phenomena, shared by a variety of other nonthyroidal illnesses, or could reflect hypothyroidism. STUDY DESIGN: To answer this question, we studied thyroid function and serum thyroid binding proteins in 36 prepubertal and 23 pubertal patients with renal disease receiving three different therapies: conservative treatment, hemodialysis, and care after renal transplantation. RESULTS: During prepuberty, the serum concentration thyroxine binding globulin (mean +/- SE) in the three groups of patients (294 +/- 18, 303 +/- 18, and 323 +/- 16 nmol/L, respectively) was significantly lower than in prepubertal control subjects (451 +/- 71 nmol/L). Only in prepubertal patients after renal transplantation (3583 +/- 573 nmol/L) were serum thyroxine binding prealbumin values lower than in respective control subjects (5999 +/- 908 nmol/L). The serum total thyroxine concentration in the three groups of patients (108 +/- 41.9, 121 +/- 5.7, and 123 +/- 5.5 nmol/L, respectively) was significantly lower than in prepubertal control subjects (149 +/- 10 nmol/L), whereas serum free thyroxine and serum albumin-bound thyroxine concentrations were similar to those in control subjects. The serum total triiodothyronine level in the three groups of patients (2.29 +/- 0.82, 2.13 +/- 0.13, and 2.01 +/- 0.20 nmol/L respectively) was significantly lower than in prepubertal control subjects (3.04 +/- 0.24 nmol/L), whereas serum levels of free triiodothyronine and serum albumin-bound triiodothyronine were similar to those in prepubertal control subjects. During puberty, serum thyroxine binding globulin and serum thyroxine binding prealbumin levels in the three groups of patients were not statistically different from those in pubertal control subjects (309 +/- 47 and 4950 +/- 1230 nmol/L, respectively). Serum levels of total thyroxine, free thyroxine, albumin-bound thyroxine, total triiodothyronine, free triiodothyronine, and albumin-bound triiodothyronine were similar to those in pubertal control subjects except for pubertal patients undergoing hemodialysis. In all clinical groups the basal serum thyrotropin concentration was similar to those in respective control subjects. The frequency of goiter was increased in patients undergoing hemodialysis, probably as a result of iodide washout with dialysis. CONCLUSION: Children and adolescents with chronic renal insufficiency or endstage renal disease or after renal transplantation do not have a primary abnormality of thyroid function and therefore are not candidates for thyroid hormone treatment.

Adolescent↗

Cognitive impairment in Duchenne muscular dystrophy.

Cognitive function and dystrophin gene mutations were investigated in 50 DMD patients (mean age 11.1 yr; range 3.5-20.3). General intelligence assessment showed 31% of patients with Wechsler full intelligence quotient (FIQ) lower than 75 (normal values: 100 +/- 14), and only 24% with appropriate FIQ level. Modal distribution of Wechsler verbal, performance, and FIQs, and Raven IQs was normal. Verbal IQ was more affected than performance IQ (PIQ) only in the younger group of subjects. Low PIQ correlated with the presence of macroglossia, detected in 13 out of 50 patients. Impairment of productive language was of non-dysphasic nature and correlated with defects of short-term memory, which was also affected in non-verbal skills. DMD patients shared the same spectrum of neuropsychological defects, regardless of whether they were or were not mentally retarded. The proportion of patients with dystrophin gene deletions was 64%. No statistically significant correlations were found between genetic data and psychometric assessment. Finally, (18F)-fluorodeoxyglucose positron emission tomography studies demonstrated cerebellar hypometabolism in all the DMD patients examined and variable involvement of associative cortical areas. These findings suggest a possible role of the cerebral and cerebellar hypometabolism in the cognitive impairment of DMD.

Adolescent↗

[Treatment of anemia in children undergoing chronic hemodialysis with recombinant human erythropoietin].

Anemia associated to chronic renal failure (CRF) is a very frequent disorder. Twenty five per cent of adult patients under hemodialysis require periodical transfusions to maintain acceptable quality of life. This anemia is due mainly to a relative deficit of erythropoietin (EPO). Thanks to recombinant DNA techniques, EPO availability has made it possible to treat this population with the hormone. Most of the reported experience has been obtained from adult patients and literature on children is scarce. For this reason, a controlled prospective trial on 18 patients (9 males and 9 females) with a mean age of 12.4 years (range 7-17) was conducted, evaluating hematimetric response, safety of treatment and effect on quality of life after one year of treatment. Seventeen patients could be evaluated; mean follow-up was of 365 days (180-323). Treatment started with an administration scheme of 25 U/kg/dose, i.v. route, three times weekly, and dose was corrected according to the hematimetric response. Target hemoglobin was set in 10 g%. Mean dose required to reach target was 101.5 +/- 37.7 U/kg/dose (50-200) three times weekly and time elapsed was of 223.3 days (175-355). Only one patient did not achieve target hemoglobin value with the maximal dose planned (200 U/kg/dose). Bone marrow biopsy in this patient showed alpha widespread fibrosis secondary to hyperparathyroidism caused by CRF. Direct correlation was found between the required rHuEPO dose and basal levels of serum alkaline phosphatase (Table 2). Results obtained in status performance on six patients showed significant increase in all the variables under analysis (Table 4).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Hypothalamic-pituitary-gonadal function in prepubertal boys and girls with chronic renal failure.

Hypothalamic-pituitary-gonadal function was evaluated in 24 prepubertal children with chronic renal failure (CRF). Among the 17 boys, 5 were receiving conservative treatment and four long-term dialysis. Another eight boys were studied 6 months to 3.3 years after renal transplantation; their ages ranged from 5 years 8 months to 15 1/2 years. Among the girls, two patients were receiving conservative treatment and five long-term dialysis; their ages ranged from 3 1/2 years to 11 years 2 months. In boys with CRF, but not in those after transplantation, mean serum follicle-stimulating hormone 60 minutes after administration of gonadotropin releasing hormone (GnRH) was lower than in 18 control prepubertal boys (mean +/- SD: 2.53 +/- 1.34 vs 6.25 +/- 2.84 IU/L, respectively; p < 0.01). Testosterone steroidogenic capacity after 1 week of stimulation with human chorionic gonadotropin and androgen sensitivity (percentage of decrease of serum sex hormone-binding globulin 1 week after intramuscular administration of testosterone enanthate) were normal. In girls, no difference between those with CRF and a control group of 19 girls was found after intravenous administration of GnRH. However, after intramuscular administration of GnRH agonist, serum follicle-stimulating hormone concentration was lower in girls with CRF than in control girls (p < 0.02); six of seven control girls had an increase of serum estradiol to more than 55 pmol/L, whereas three of seven girls with CRF had no response, and serum follicle-stimulating hormone failed to increase after GnRH agonist therapy in two of these patients. We conclude that hypothalamic-pituitary function is not normal in some prepubertal boys and girls with CRF, particularly in those with low serum albumin concentrations. On the other hand, testicular and ovarian steroidogenic capacity is not impaired, and the biologic response to androgens in boys is preserved.

Adolescent↗

[Iron deficiency in children: which is the correct therapy?].

Iron is essential for human metabolism. Under normal circumstances its homeostasis is strictly kept by absorption and excretion through genitourinary, gastrointestinal tracts and skin losses. In several systemic disorders, dietary iron is insufficient to keep such a dynamic balance: development of iron deficiency may be due to increased requirements, decreased intestinal absorption, inadequate dietary uptake. Low birth weight newborns, children and adolescents are at increased risk for developing iron deficiency. Although clinical aspects may vary, hematochemical findings show a three-step gradual progression. In a first step iron deficiency is diagnosed by serum ferritin level which will be under 10-20/micrograms/ml showing a depletion of total body iron stores. In a second step progressing iron deficiency will be assessed by lowered serum iron and increased unsaturated serum transferrin, serum iron bound to transferrin and erythrocyte protoporphyrin IX. Scanty clinical signs are still available. In a third step while clinical findings show a complete features of iron deficiency anemia (weakness, fatigue, palpitations, etc.), laboratory findings show morphologic alterations in red cells (hypochromia and microcytemia), together with the aforementioned disorders in ferrokinetics. Iron deficiency anemia responds very effectively to treatment due a correct etiological diagnosis, crucial to a through therapy tending to first eliminating the causes of it. Prophylaxis against iron deficiency anemia is required in prematurely born and low birth weight infants because of doubled iron requirements. After the second month of life diet is supplemented with 2-4 mg/kg/die of ferrous iron orally along the first year of life.

Adolescent↗

[Meningococcal sepsis and DIC in childhood: a report of a clinical case].

Disseminated intravascular coagulation (DIC) is a frequent complication of meningococcal sepsis in children. The clinical course variability, the severity of manifestations and the need of an early diagnosis for appropriate treatment, guides us to report a case of meningococcal sepsis and DIC. The patient, male, prematurely born, 11 months years old, presented himself with high fever of sudden onset, malaise, diarrhea, diffuse skin rash with abdominal petechiae, and no clinical evidence of meningitidis. Initial hematochemical findings, peripheral leukocytosis, quantitative and qualitative changes in plasma coagulation factors, liquoral hypocellularity together with the development of signs of meningeal irritation (stiff neck and back) were considered diagnostic clues for meningococcal sepsis associated with DIC. A gram-negative diplococcus was cultured from liquor. Primary goals of the treatment of this life-threatening clinical picture were the elimination of the bacterial component, the correction of clotting disorders and careful control of shock and metabolic acidosis often related with DIC. The patient then received a wide spectrum Cephalosporin, fresh frozen plasma, appropriate electrolyte solutions and eventually heparin, which led to a complete control and resolution of symptomatology.

Acidosis↗