Pathologic characteristics of pediatric intracranial pilocytic astrocytomas and their impact on outcome in 3 countries: a multi-institutional study

Pilocytic astrocytoma (PA) is one of the most common glial neoplasms in the pediatric population, and its gross total resection can be curative. Treatment of partially resected or recurrent tumors is challenging, and the factors associated with increased recurrence risk are not well defined. Identification of favorable and unfavorable factors can lead to a better understanding and management of patients with PA. We studied the pathologic characteristics of 116 intracranial PAs from 4 institutions representing 3 distinct geographic regions to identify factors that may be associated with biological behavior. The study included 65 boys and 51 girls with a median age of 6 years. Median follow-up for all patients was 101 months, during which time 38 patients had recurrence. Progression-free and overall survivals were better in patients who underwent gross total resection. We were not able to identify any differences in pathologic and molecular markers among the 4 institutions from 3 different countries. However, progression-free survival varied significantly among institutions. Sox-2 was the most prevalent stem cell marker in PA, and many tumors showed synaptophysin positivity. BRAF immunostaining was not useful in determining BRAF duplication. BRAF duplication was more typical of posterior fossa tumors. There was a strong correlation between BRAF duplication and pERK immunostaining, suggesting that the RAF/MEK/ERK pathway is active in these tumors. This finding has significant implications given its role in oncogen-induced senescence and possible influence on treatment decisions of subtotally resected tumors.     

Image-directed color Doppler ultrasonography of kidney in systemic lupus nephritis

We evaluated intrarenal arterial waveforms with image-directed color Doppler ultrasonography in 21 patients with systemic lupus erythematosus (SLE). The systolic–diastolic ratio, resistive index, and pulsatility index were compared with serum creatinine levels, creatinine clearance, and quantitation of urinary protein excretion, as well as with histopathologic scores of specimens obtained from 9 patients who underwent renal biopsy. Doppler parameters were in the normal ranges in all patients, without showing significant correlation with any of the histopathologic scores or laboratory parameters except creatinine levels. We conclude that image-directed color Doppler ultrasonography is of no practical value in the evaluation of lupus nephritis during the early stages of the disease. © 1995 John Wiley & Sons, Inc.     

Sellar-Parasellar Brown Tumor: Case Report and Review of Literature

Brown tumors are the end stage of primary or secondary hyperparathyroidism. Such lesions are rare because hyperparathyroidism is now usually diagnosed and treated before they develop. Skeletal bone is most frequently involved, whereas sellar-parasellar involvement is extremely rare. We report a patient with a brown tumor involving the sellar-parasellar region associated with primary hyperparathyroidism. The patient's clinical history and biochemical and radiological tests were used for the diagnosis. Excision of the sellar-parasellar mass enabled histopathological confirmation and surgical decompression of neural structures. After the transsphenoidal surgery, excision of a parathyroid adenoma normalized the patient's metabolic status. Brown tumors are rarely associated with complications but occasionally may compress neural structures such as the optic nerve as they enlarge.     

Stroke in childhood: experience in Istanbul, Turkey

Our objective was to characterize the etiologic factors and outcome for stroke in children. We retrospectively reviewed the charts of patients between the ages of 40 days and 94 months (36.5 +/- 23.7 months) with stroke seen at Istanbul Medical Faculty, Department of Pediatrics between January 1995 and December 2003. We found 79 cases of stroke: 57 ischemic and 22 hemorrhagic strokes. Seventeen children had vitamin K deficiency dependent hemorrhage. In 14 children stroke occurred as a complication of cardiac disease, 7 had moyamoya disease, 3 had protein C deficiency, 2 had thalassemia, 2 had hyperhomocysteinemia (methylene tetrahydrofolate reductase gene mutation), 2 were heterozygote for factor V Leiden, 3 had Down's syndrome, 1 was diagnosed with antiphospholipid syndrome, 1 had glycogen storage disease, and in 28 children no underlying cause could be found. Multiple risk factors were found in 4 children. The outcome in all 79 stroke patients was as follows: asymptomatic 60%; symptomatic epilepsy or persistent neurologic deficit 37%; death 3%; and recurrent stroke 5%. Thus, an underlying cause for stroke was identified in 65% of the children in the study group; 40% of the children either died or suffered motor and/or cognitive sequelae.     

Comparison of two oral contraceptive forms containing cyproterone acetate and drospirenone in the treatment of patients with polycystic ovary syndrome: a randomized clinical trial

Purpose

To compare the effects of combined oral contraceptives (OCs) containing cyproterone acetate and drospirenone in the treatment of polycystic ovary syndrome (PCOS).

Methods

Fifty-two patients with PCOS were randomized in two groups: group A (n = 26) received 0.035 mg ethinyl estradiol + 2 mg cyproterone acetate and group B (n = 26) received 0.03 mg ethinyl estradiol + 3 mg drospirenone-containing OCs for 12 months. Baseline clinical features including body mass index, waist to hip ratio (WHR), and modified Ferriman-Gallwey (mFG) score were noted. Baseline biochemical parameters included androgen profile, carbohydrate metabolism, lipid profile, and oxidative stress. The percentages of changes for all parameters were compared.

Results

The groups were comparable regarding the baseline characteristics. WHR decreased significantly from baseline (?4 % [?31 to 35]) in group B when compared to group A (0 % [?11 to 14]) (P = 0.033). The total mFG score decreased significantly from baseline (?35 % [?71 to 10]) in group A when compared to group B (?18 % [?72 to 30]) (P = 0.035). Changes in androgen hormone profile were comparable except DHEA-SO₄ (?32 % [?53 to 15] in group B vs. ?10 % [?49 to 63] in group A; P = 0.046). The effects of the drugs were similar regarding carbohydrate metabolism, lipid profile, and oxidative stress parameters.

Conclusions

Cyproterone acetate containing OCs seem to be more effective to treat clinical hirsutism in patients with PCOS after 12 months of treatment.