Aniridia associated with ptosis in three generations of the same family

Aniridia is a spectrum of abnormalities of the iris anatomy, which range from a total absence of the iris to a mild stromal hypoplasia with normal pupil. Aniridia associated with ptosis in three generations of the same family is described. The cases fit the autosomal dominant familial aniridia (AN1) type, a genetic form of congenital aniridia characterised by isolated ocular defects.     

Burkitt's lymphoma presenting as obstructive jaundice

A 12-year-old boy presented with a 5-month history of yellowness of the eyes, progressive painless abdominal swelling and weight loss. Physical examination revealed a grossly wasted child with marked jaundice and non-tender hepatomegaly. Liver function tests and abdominal ultrasound suggested obstructive liver disease. Tissue biopsy at laparotomy showed histological findings consistent with Burkitt's lymphoma. He was commenced on cytotoxic chemotherapy and, after two courses, the jaundice disappeared and he remained well. Although ante-mortem presentation of Burkitt's lymphoma as hepatic disease is rare, this condition should be included in the differential diagnosis of a child with obstructive jaundice.     

A case of primary Addison's disease with hyperemesis gravidarum and successful pregnancy

We followed up a pregnant woman with Addison's disease diagnosed before conception. She presented with hyperemesis gravidarum. Throughout pregnancy, she received prednisone and the basic disease did not deteriorate during pregnancy. She was delivered by caesarean section due to breech presentation. The fetal prognosis was good.     

N-Acetylcysteine for Preventing Pump-Induced Oxidoinflammatory Response During Cardiopulmonary Bypass

Purpose To investigate the effect of N-acetylcysteine on preventing pump-induced oxidoinflammatory response during cardiopulmonary bypass (CPB).Methods Forty patients undergoing coronary artery bypass grafting (CABG) were randomly divided into a study group (n = 20), given 50mgkg^–1 N-acetylcysteine intravenously for 3 days, and a control group (n = 20) given saline. Serum samples were collected for measurement of myeloperoxidase (MPO), malondialdehyde (MDA), interleukin-6, ₁-acid glycoprotein (AAGP), and C-reactive protein (CRP) during surgery and postoperatively.Results The MPO and MDA values showed a similar pattern during and after CPB in the study group, with significantly less variance than in the control group. Interleukin-6 showed similar patterns in the two groups, but the data from 30min after the start of CPB and from 6h post-CPB were significantly different. The AAGP and CRP values were both elevated during CPB in the two groups without a significant difference, but 6 and 24h post-CPB, the values were significantly higher in the control group than in the study group.Conclusions N-Acetylcysteine decreased pump-induced oxidoinflammatory response during CPB, suggesting that it could be a novel therapy for assisting in the prevention of CBP-induced oxidoinflammatory damage.     

Effects of long-term passive smoking on the diameter of glomeruli in rats: Histopathological evaluation

BACKGROUND AND AIMS: Long-term smoking increases the risk of renal diseases and it also causes the progression of the diseases. The aim of this study was to evaluate the effects of smoking on glomeruli in healthy rats exposed to long-term passive smoking for a short period of time after birth. METHODS: Sixty-five rats were randomly assigned into four groups. Groups 1 (18 males) and 2 (18 females) were exposed to smoke, whereas groups 3 (15 males) and 4 (14 females) were not exposed to smoke. Overall, 130 renal units were evaluated, and diameters of 10 different glomeruli were measured. The renal and bodyweights of the rats were measured. RESULTS: The diameter of the glomeruli was 95.51 +/- 9.25 and 100.91 +/- 4.06 microm in groups 1 and 3, respectively (P < 0.05). It was 97.32 +/- 4.06 and 98.86 +/- 6.25 microm in groups 2 and 4, respectively (P > 0.05). Irrespective of the sex, the diameter of the glomeruli was 96.42 +/- 7.15 microm in smokers and 99.92 +/- 5.56 microm in non-smokers (P < 0.05). The renal weights of the male rats affected by smoke were significantly lower than that of those that were not affected by smoke (P < 0.05). Bodyweights of male rats affected by smoke were significantly lower that of those not affected (P < 0.05). There was a weak correlation between the diameter of the glomeruli and the bodyweights of male rats affected by smoke (r = 0.474, P < 0.05). CONCLUSION: We conclude that passive smoking might have a negative affect on renal morphometry and body growth.     

The flurbiprofen derivatives HCT1026 and HCT1027 inhibit bone resorption by a mechanism independent of COX inhibition and nitric oxide production

Prostaglandins and nitric oxide both modulate bone resorption and bone formation. We previously reported that a nitrosylated derivative of flurbiprofen, termed HCT1026, exerted inhibitory effects on osteoclastic bone resorption, which could not be reproduced by combining the parent compound with nitric oxide (NO) donors. The aim of this study was to investigate the mechanism by which HCT1026 inhibits bone resorption. We compared the effects of flurbiprofen and HCT1026 on osteoclast and osteoblast activity with those of HCT1027--an analogue of HCT1026, which lacks an NO-donating moiety. We found that HCT1026 and HCT1027 inhibited bone resorption in interleukin (IL)-1-stimulated murine osteoblast-bone marrow cocultures, with half-maximal effects (IC50) at 20 +/- 5 microM for HCT1026 and 25 +/- 6 microM for HCT1027 compared with 399 +/- 25 microM for flurbiprofen (P < 0.0001). These differences were unrelated to cyclooxygenase (COX) inhibition since HCT1026 and HCT1027 were about seven to eight times less potent than flurbiprofen at inhibiting COX-1 activity and half as potent at inhibiting COX-2 activity. Further studies showed that HCT1026 and HCT1027 activated caspase-3 in rabbit osteoclasts and promoted osteoclast apoptosis, as assessed by nuclear morphology and TUNEL assays. We conclude that HCT1026 and HCT1027 inhibit osteoclast formation and activity by a mechanism that is independent of NO production and COX inhibition. This raises the possibility that both compounds interact with a novel molecular target expressed on osteoclasts to promote apoptosis and inhibit bone resorption. This demonstrates that HCT1026 and derivatives could represent a novel class of antiresorptive drugs with therapeutic value in the treatment of bone diseases associated with accelerated bone loss due to osteoclast activation.