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PURPOSE: High dose corticosteroids are an effective tool for rapidly alleviating neurologic symptoms caused by intracranial mass lesions. However, there is concern that preoperative corticosteroids limit the ability to obtain a definitive pathologic diagnosis, particularly if imaging features suggest primary central nervous system lymphoma (PCNSL). METHODS: To explore the impact of preoperative corticosteroids in newly diagnosed PCNSL patients, from 2009 to 2018 treated at our institution. RESULTS: We identified 54 patients; 18 had received corticosteroids prior to biopsy or resection. Only in one case did the patient have a prior non-diagnostic biopsy, requiring a second procedure. The cumulative doses of preoperative dexamethasone ranged from 4 mg to 120 mg (mean 32 mg, median 24 mg), given over 1-14 days (mean 2 days, median 1 day), and the majority had received corticosteroids for only 1-2 days. There was a trend for a larger diameter of lesional T1 contrast enhancement for patients who received steroids (39 mm vs. 34 mm, p = 0.11). In this series of cases with pathologically and clinically proven PCNSL, preoperative corticosteroids had been given in a third of cases, suggesting that they may be given for symptomatic relief without compromising pathologic diagnosis. CONCLUSIONS: Despite the commonly held tenet that preoperative corticosteroids can obscure the pathologic diagnosis in PCNSL, this is likely not the case in the majority of patients who receive a short course preoperatively. Obtaining a second stereotactic scan to confirm continued presence of the lesion prior to tissue sampling may also mitigate these concerns.

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INTRODUCTION: The vast majority of patients presenting with pneumoperitoneum have visceral organ perforation and require urgent diagnostic laparoscopy. Nonsurgical causes are relatively rare and may be attributed to multiple etiologies. CASE PRESENTATION: Here we describe the case of a 38-year-old Caucasian female who presented to the emergency department with three days of cramping, epigastric abdominal pain. Her physical exam was notable for tenderness to palpation in the epigastric area and abdominal and chest X-rays showed free air under the diaphragm. Free air around the porta hepatis was verified on CT scan. Approximately 90% of pneumoperitoneum cases are due to perforation of visceral organs and therefore require operative management. An urgent exploratory laparoscopy revealed no clear source of free air, but postoperatively the patient developed a large volume of watery discharge from her vagina. Subsequent workup revealed a 1 cm vaginal cuff dehiscence which was later repaired with no postoperative complications. CONCLUSION: Although the majority of patients with pneumoperitoneum require urgent exploratory laparoscopy, a careful diagnostic workup may reveal sources of free air that are not related to hollow viscous perforation. Vaginal cuff dehiscence represents a rare yet nonurgent source of pneumoperitoneum. This differential should be considered in light of the possible intra- and postoperative complications of surgery.

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In order to assess the possible effects of sleep states on cerebral haemodynamics in healthy term infants, we measured cerebral oxyhaemoglobin, deoxyhaemoglobin and total haemoglobin concentration using near infrared spectroscopy. Thirty-seven sleep state changes in seventeen infants (gestational age: 37 to 41 4/7 weeks), aged between two and eight days were continuously registrated during 1-3 h. Transcutaneous PaO2, PaCO2, arterial O2 saturation and heart rate were simultaneously recorded and sleep states were clinically defined. There was a close relationship between sleep state changes and changes in total cerebral haemoglobin concentration, which increased from active to quiet sleep and decreased from quiet to active sleep. Changes in total cerebral haemoglobin were due, in the most part, to changes in the cerebral oxyhaemoglobin concentration. In conclusion, sleep states influence the cerebral haemoglobin concentration. Studies on cerebral haemodynamics should take sleep state into account in term newborn infants.

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We report on a patient with EEC/EECUT syndrome and concomitant hypoplasia of the thymus and reduction of T cells in secondary lymphatic organs. The patient was born prematurely at 35 weeks of gestational age and exhibited ectodermal dysplasia, ectrodactyly, cleft palate and urinary tract abnormalities. On the left side, a large ureterocele was present. On the right side, an atretic ureter was found. Both conditions had led to intrauterine hydronephrosis, renal dysplasia, oligohydramnios, pulmonary hypoplasia, and death of the child. Ureteral malformations are thought to be of epithelial origin. Autopsy showed only small rudiments of thymic tissue containing single epithelial cells, but were completely devoid of Hassall corpuscules. Again, this clearly points to an ectodermal defect. Although there was severe reduction of T cells in secondary lymphatic organs, the thymic defect would not have necessarily led to immunological deficiency; perhaps this is the reason that an epithelial defect in the thymus of patients with EEC syndrome has not yet been reported. With regard to an updating of the diagnosis of the EEC/EECUT syndrome, an "EEC/EECUT plus" syndrome is suggested.

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A 44-year-old man was admitted with symptoms compatible with Addison crisis. Abdominal computer tomography revealed extensive bilateral adrenal abscesses. Histoplasma capsulatum was cultured from a needle aspirate. The patient was HIV-seronegative and had no underlying malignancy. He may have acquired the infection during several stays in endemic areas in the United States, South America and Asia. The case was also remarkable for moderate brain atrophy, thrombosis of the portal and splenic veins and liver cirrhosis caused by alpha-1-antitrypsin deficiency (phenotype MZ). The patient recovered fully under substitution of adrenal hormones and antifungal treatment. He received intravenous amphotericin B (75 mg q24h) for 10 days, followed subsequently by oral treatment with itraconazole (400 mg q24h) over several months. Radiologic follow-up 9 and 18 months later showed a pronounced decrease of the inflammatory adrenal lesions.

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The distribution of dianhydrogalactitol-C14 (DAG-C14) which had been administered to rats as an iv bolus injection was studied in plasma and brain tissue. Analysis of samples was carried out by a high-pressure liquid chromatography method which specifically responds to the parent drug. Samples were monitored by both ultraviolet and liquid scintillation spectrometry. Plasma level measurements indicate that intact DAG has a relatively short half-life in plasma (t1/2= 43.7 minutes) and brain (t1/2 = 50.3 minutes). These findings differ significantly from those studies which have measured total radioactivity when monitoring DAG levels; and should be considered in the design of DAG dose regiments. Data from both brain and plasma were consistent with a classic two-compartment open model.

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A high-performance liquid chromatographic method is described for measuring submicrogram quantities of dianhydrogalactitol, a promising anti-neoplastic agent, in plasma. The drug is derivatized directly in plasma with sodium diethyldithiocarbamate to form a bis(dithiocarbamoyl) ester which absorbs UV light at 254 nm (am 17,000). The derivatized product is then extracted quantitatively into chloroform and separated by normal phase chromatography (muBondpak CN column). Dianhydrogalactitol concentration below 50 ng/ml of plasma can be detected in the eluent.

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