Iatrogenic sclerosing cholangitis following hepatic arterial chemotherapy infusion

A case of common hepatic duct stricture secondary to hepatic artery chemotherapy infusion is described. CT and endoscopic retrograde cholangiopancreatography (ERCP) may be used in concert to differentiate this entity from other causes of jaundice--namely, hepatic replacement by tumor, porta hepatis adenopathy, and chemotherapy hepatotoxicity.     

Origin of spinal arteriovenous malformation and normal cord vasculature from a common segmental artery: angiographic and therapeutic considerations

The dural type of spinal arteriovenous malformation (AVM) can be cured by excision or by embolization of the nidus. The common origin of the blood supply to the malformation and to the cord from the same segmental artery would profoundly affect therapeutic choices. This anatomic situation was encountered in two of nine such lesions. The angiographic appearance and the importance of recognizing this common origin is discussed.     

二仙合剂的降压及其药理研究

二仙合剂可治疗妇女高血压,在腎型高血压狗也有降压效果。本文用5种高血压大白鼠(神經型、腎型、腎上腺燙伤型、睾丸切除型及卵巢切除型)来比較降压作用,并观察性周期、前庭时值及某些加压物质的升压反应。每天灌胃二仙合剂10克/公斤,共2周,神經型、腎型及睾丸切除型的降压面积%分别为-9,-11及-15%,与同型对照組比較,降压“显著”,腎上腺燙伤型及卵巢切除型的降压“不显著”。每天灌服二仙合剂10克/公斤,共7周,对未成熟大白鼠生长及性腺发育无影响。对神經型、腎型及腎上腺燙伤型雌鼠的性周期紊乱亦无糾正作用。二仙合剂能延长腎型高血压鼠的前庭时值,但在其他型则变化不显著。无明显对抗去甲腎上腺素及垂体后叶素的加压反应的作用。     

Study of smell and reproductive organs in a mouse model for CHARGE syndrome

CHARGE syndrome is a multiple congenital anomaly syndrome characterised by Coloboma, Heart defects, Atresia of choanae, Retardation of growth and/or development, Genital hypoplasia, and Ear anomalies often associated with deafness. It is caused by heterozygous mutations in the CHD7 gene and shows a highly variable phenotype. Anosmia and hypogonadotropic hypogonadism occur in the majority of the CHARGE patients, but the underlying pathogenesis is unknown. Therefore, we studied the ability to smell and aspects of the reproductive system (reproductive performance, gonadotropin-releasing hormone (GnRH) neurons and anatomy of testes and uteri) in a mouse model for CHARGE syndrome, the whirligig mouse (Chd7^Whi/+). We showed that Chromodomain Helicase DNA-binding protein 7 (Chd7) is expressed in brain areas involved in olfaction and reproduction during embryonic development. We observed poorer performance in the smell test in adult Chd7^Whi/+ mice, secondary either to olfactory dysfunction or to balance disturbances. Olfactory bulb and reproductive organ abnormalities were observed in a proportion of Chd7^Whi/+ mice. Hypothalamic GnRH neurons were slightly reduced in Chd7^Whi/+ females and reproductive performance was slightly less in Chd7^Whi/+ mice. This study shows that the penetrance of anosmia and hypogonadotropic hypogonadism is lower in Chd7^Whi/+ mice than in CHARGE patients. Interestingly, many phenotypic features of the Chd7 mutation showed incomplete penetrance in our model mice, despite the use of inbred, genetically identical mice. This supports the theory that the extreme variability of the CHARGE phenotype in both humans and mice might be attributed to variations in the fetal microenvironment or to purely stochastic events.     

BRCA1 as tumor suppressor: lord without its RING?

BRCA1 is a tumor suppressor with critical roles in the maintenance of genomic stability. It encodes a large protein with an amino-terminal RING domain that possesses ubiquitin-ligase activity. Given the occurrence of numerous cancer-causing mutations within its RING domain, investigators have long suspected that BRCA1's ubiquitin ligase is important for its tumor suppression and DNA repair activities. Using genetically engineered mouse models, two recent studies shed light on this age-old hypothesis.     

Low-dose deoxycoformycin in the treatment of hairy cell leukemia

Ten patients with progressive hairy cell leukemia were treated with 2'deoxycoformycin (dCF) by intravenous bolus (4 mg/m2) given every other week. All ten patients are evaluable for response and nine of the ten patients have achieved a complete remission. In addition to clearing of hairy cells from the bone marrow, eight patients had resolution of their monocytopenia. Seven of the nine patients remain in unmaintained remission with a median duration of 6.2 months. Two patients have had relapse in the bone marrow alone and continue to have normal peripheral blood counts. They are being followed without treatment. Toxicity was minimal at this low dose with one patient having a mild reversible reduction in creatinine clearance. Four other patients had reversible neutropenia. There were no significant infections associated with treatment. Low-dose deoxycoformycin administered intravenously every other week represents an extremely effective treatment for hairy cell leukemia.