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Biomedical subjects

A Luisiri

Publications and source records attributed to A Luisiri.

At least 19 recordsLinked to original sources

Prospective assessment of pituitary size and shape on MR imaging after suppressive hormonal therapy in central precocious puberty.

OBJECTIVE: The diagnostic significance of an enlarged pituitary gland regarding both shape and size parameters on MR imaging has previously been demonstrated in children with central precocious puberty. This study was designed to assess changes in these parameters following successful suppressive therapy of central precocious puberty with the gonadotropin-releasing hormone (GnRH) analogue. MATERIALS AND METHODS: Twelve girls (mean age 7.3 years) with central precocious puberty were prospectively enrolled in our study protocol. Sagittal and coronal MR images of the pituitary region were obtained in all patients before treatment and after at least 6 months of GnRH analogue therapy (mean 18.0 months). Parameters measured included pituitary gland height, length, width, sagittal cross-sectional area, and volume. RESULTS: All patients had excellent clinical response to treatment with arrest of secondary sexual development, normalization of serum estradiol levels, and complete obliteration of the LH response to diagnostic GnRH stimulation. No significant change occurred in any pituitary size or shape parameter following GnRH analogue therapy. CONCLUSION: Favorable clinical response to GnRH analogue therapy in central precocious puberty is not accompanied by significant a change in pituitary gland size and shape.

Antineoplastic Agents, Hormonal↗

Cyclosphosphamide/prednisone for combination immunosuppression and therapy of lymphoproliferative disease.

Post-transplant lymphoproliferative disease (PTLD) is a well-known complication of solid organ transplantation. While this disorder can often be controlled by decreasing immunosuppression, it is frequently difficult to control the resultant rejection. This case exemplifies this dilemma. To solve this problem, cyclosphosphamide (600 mg/m2) and prednisone (2 mg/kg times 5 days) were given every 3 weeks to simultaneously treat PTLD and provide immunosuppression.

Cyclophosphamide↗

Bone marrow transplantation for the treatment of alpha-mannosidosis.

We report successful bone marrow transplantation in a child with a severe form of alpha-mannosidosis, type I. There was complete resolution of the recurrent sinopulmonary disease and organomegaly, improvement in the bony disease, and stabilization of neurocognitive function.

Bone Marrow Transplantation↗

A fatal overdose of arginine hydrochloride.

CASE REPORT: Arginine hydrochloride is used both diagnostically to test for growth hormone deficiency and therapeutically for treatment of metabolic alkalosis. We describe a 21-month-old girl who developed cardiopulmonary arrest following an accidental overdose of arginine hydrochloride. The patient developed acute metabolic acidosis and transient, but severe, hyponatremia. Thirty-six hours after successful resuscitation, she developed fatal central pontine and extrapontine myelinolysis. Unlike previous reports of arginine-toxicity, our patient showed no evidence of hyperkalemia. This case illustrates a previously unreported mechanism of arginine hydrochloride toxicity.

Acidosis↗

MR imaging diagnosis of central precocious puberty: importance of changes in the shape and size of the pituitary gland.

OBJECTIVE: Central precocious puberty occurs as a result of premature pituitary stimulation and increased secretion of gonadotropins. The aims of this study were to analyze MR imaging findings in the pituitary glands of children with central precocious puberty compared with matched control subjects, to define MR imaging-derived variables useful in the diagnosis of central precocious puberty, and to correlate MR imaging-derived variables with the hormonal profile and other imaging and clinical findings. MATERIALS AND METHODS: Twenty-six children with central precocious puberty (two boys and 24 girls) were divided into two subgroups according to MR imaging findings: idiopathic (21 patients) and nonidiopathic (five patients: three hypothalamic hamartomas, one pineal tumor, one empty sella syndrome). The control group consisted of 17 normal age- and sex-matched children (two boys, 15 girls). Analyzed parameters included pituitary height, length, width, midsagittal cross-sectional area, calculated volume, and shape. The shape was assessed by a pituitary grading system and two other shape indexes (length-to-height and length-to-width ratios). Pituitary grade was defined by the concavity of the upper pituitary surface (grade 1 = marked concavity, grade 2 = mild concavity, grade 3 = flat, grade 4 = mild convexity, grade 5 = marked convexity). RESULTS: Pituitary grade showed a highly significant difference among groups (p < .001). Area, height, and length-to-height ratio were significantly different (p < .05), whereas length, width, length-to-width ratio, and volume were not. There was no significant difference in any of the variables compared between idiopathic and nonidiopathic groups. When selected variables (pituitary grade, area, height, length) in the central precocious puberty group were stratified by bone age and findings on pelvic sonograms, patients with advanced bone age had a significantly higher pituitary grade (p < .01) and had a tendency toward a greater pituitary length. Pituitary size and shape correlated with the hormonal profile. CONCLUSION: Change in pituitary grade is the most helpful variable for the diagnosis of central precocious puberty in a prepubertal child. A high pituitary grade (4 or above) is highly predictive of central precocious puberty, with the highest specificity and positive predictive value, but with low sensitivity. The use of combinations of high pituitary grade with two other positive findings (height and area greater than 1 SD from the respective means in the control group) improves the sensitivity, specificity, and predictive value of MR imaging in the diagnosis of central precocious puberty.

Analysis of Variance↗

Paroxysmal nocturnal hemoglobinuria associated with venous thrombosis and papillary endothelial hyperplasia presenting as ulcerated duodenal mass.

Paroxysmal nocturnal hemoglobinuria is an acquired clonal expansion of bone marrow stem cells that are deficient in the decay-accelerating factor, which is a complement regulatory glycoprotein (CD55), as well as in the membrane inhibitor of reactive lysis (CD59) and the C8-binding protein. These proteins are deficient on the membranes of red blood cells, granulocytes, monocytes, and platelets. The disorder is associated with intermittent hemolytic anemia, hemoglobinuria, infection, a tendency toward bone marrow aplasia, and venous thromboses. The thromboses, on resolution, may give rise to endothelial proliferation that may cause ischemia and ulceration, or, alternatively, the thromboses may cause ulceration leading to a granulation tissue response with exaggerated endothelial proliferation. We report a second case of paroxysmal nocturnal hemoglobinuria that presented roentgenographically as an ulcerated circumferential duodenal mass secondary to venous thrombosis accompanied by florid papillary endothelial hyperplasia. We also review the literature concerning this phenomenon.

Adolescent↗

Symptomatic cerebral swelling complicating diabetic ketoacidosis documented by intraventricular pressure monitoring: survival without neurologic sequela.

A six-year-old boy developed symptomatic cerebral swelling four hours after the initiation of treatment for newly diagnosed diabetes mellitus complicated by ketoacidosis. Ventriculostomy documented intracranial pressure over a two-day period. Increased intracranial pressure unresponsive to controlled hyperventilation and sedation was treated by administering a diuretic and by drainage via a ventriculostomy. Intracranial pressure monitoring was a useful adjunct in management of this rare, but often lethal, complication of diabetes mellitus. One year later, both school performance and the results of a neurologic examination were normal.

Brain Edema↗