Steroidogenic acute regulatory protein expression in the normal human brain and intracranial tumors

In this study we found the expression of steroidogenic acute regulatory protein (StAR) mRNA and protein in both the normal human brain and intracranial tumors, using RT-PCR and immunohistochemistry. StAR mRNA is expressed at a very low level in the white matter of the normal human brain, but in tumoral tissues StAR mRNA was specifically expressed in oligodendrogliomas and malignant glial tumors. StAR-positive cells were also detected in the normal human brains and gliomas; its frequency and density were higher in glioma tissue. These findings suggest that StAR expression might be correlated with the growth of glial tumors.     

Idiopathic normal pressure hydrocephalus predominantly with prolonged fever and hyponatremia

Described is a patient with hydrocephalus and a gait disorder with associated prolonged fever and hyponatremia. The authors made a diagnosis of normal pressure hydrocephalus (NPH) and performed a ventriculoperitoneal shunt, which improved the gait disturbance, accompanied by resolution of the fever and hyponatremia.     

Role of K(+) channels in frequency regulation of spontaneous action potentials in rat pituitary GH(3) cells

The frequency of spontaneous action potentials (SAP) is important in the regulation of hormone secretion. The decrease in K(+) conductance is known as a primary mechanism for increasing SAP frequency. To investigate the nature of K(+) channels that contribute to the frequency regulation of the SAP in rat clonal pituitary GH(3) cells, the effect of various K(+) channel blockers on the SAP and membrane currents were recorded using the patch-clamp technique. A classical inward rectifying K(+) channel blocker, Cs(+) (5 mM), caused an increase in firing frequency and depolarization in after-hyperpolarization (AHP) voltage. An ETHER-A-GO-GO(ERG) type K(+) channel blocker, E-4031 (5 microM), caused no significant effect on the SAP. Tetraethylammonium (TEA, 10 mM) decreased firing frequency and increased the duration of SAP. These effects were not changed by the presence of high concentration of Ca(2+) buffer (10 mM EGTA or BAPTA) in pipette solutions. In voltage-clamp experiments, Cs(+) and E-4031 did not affect outwardly rectifying K(+) currents, but significantly inhibited inwardly rectifying K(+) currents recorded in isotonic K(+) solution. However, the kinetics of Cs(+)-sensitive current and E-4031-sensitive current were distinctive: the time to peak was more immediate and the decay rate was slower in Cs(+)-sensitive current than in E-4031-sensitive current. These results imply that Cs(+) and E-4031 inhibit the distinct components of inwardly rectifying K(+) currents, and that the contribution of the Cs(+)-sensitive current can be immediate on repolarization and can last more effectively over pacemaking potential range than E-4031-sensitive current.     

A novel compound heterozygous mutation in the CYP17 (P450 17alpha-hydroxylase) gene leading to 17alpha-hydroxylase/17,20-lyase deficiency

Mutations in the CYP17 gene impair steroid biosynthesis in the adrenals and gonads and often cause 17alpha-hydroxylase/17,20-lyase deficiency, leading to amenorrhea, sexual infantilism, and hypokalemic low aldosterone hypertension. Several CYP17 mutations resulting in 17alpha-hydroxylase/17,20-lyase deficiency have been reported previously. In the present study, we found a novel CYP17 mutation from the molecular analysis of a Korean patient with primary amenorrhea with a 46,XX karyotype, and hypokalemic hypertension. We sequenced all 8 exons of the CYP17 gene that were amplified from patient's genomic DNA using polymerase chain reaction (PCR) and found a compound heterozygous mutation in the CYP17 structural gene; a 1-base deletion and a 1-base transversion (TAC-->AA) at codon 329, leading to the production of a truncated protein (1-417 amino acids), and a 3-base deletion (TCC, either 350-351 or 351-352 codon) in the other allele. Restriction enzyme digestion analysis of patient's and parental DNA showed that the 1-base deletion and the 3-base deletion are inherited from mother and father, respectively. Here we conclude that these novel compound heterozygous mutations might account for the patient's clinical manifestations of 17alpha-hydroxylase/17,20-lyase deficiency.     

Metastatic hepatocellular carcinoma of gingiva mimicking pyogenic granuloma

We report a case of metastatic hepatocellular carcinoma of gingiva mimicking pyogenic granuloma. To our knowledge, no such reports have been published in the literature of dermatology. Gingival metastatic lesions are of interest both because they are easily mistaken for several benign lesions and because they may be the first sign of an undiscovered malignancy.     

Species identification and strain differentiation of dermatophyte fungi using polymerase chain reaction amplification and restriction enzyme analysis

BACKGROUND: Standard biochemical tests, microscopy, colony characteristics, and mating tests have conventionally been used for the identification of dermatophytes species, but these methods of identification are costly, time-consuming, and require special skills. OBJECTIVE: Our purpose was to identify a method that enables rapid species identification and strain differentiation of dermatophyte fungi. METHODS: We chose 4 restriction enzymes (BsYiI, DdeI, HinfI, and MvaI) that could produce different fragment patterns after enzyme digestion according to species or strain. We performed enzyme digestions after polymerase chain reaction amplification of internal transcribed spacer region and identified different restriction fragment length polymorphisms (RFLP) according to species and strains. RESULTS: All the species included in this study could be easily differentiated using any combination of 2 different restriction enzymes except Trichophyton rubrum and T raubitschekii, which produced identical digestion patterns after all 4 restriction enzyme digestions. In the case of T mentagrophytes, MvaI and DdeI each produced 2 distinct RFLP patterns. CONCLUSION: This study showed that internal transcribed spacer region analysis using polymerase chain reaction-RFLP through DdeI and MvaI is useful for rapid identification of the majority of dermatophytes species. However, there were 2 different band patterns by DdeI and MvaI restriction enzyme digestion and no correlations between morphologic types and RFLP patterns in T mentagrophytes.     

Acute interstitial nephritis due to nicergoline (Sermion)

We report a case of acute interstitial nephritis (AIN) due to nicergoline (Sermion). A 50-year-old patient admitted to our hospital for fever and acute renal failure. Before admission, he had been taking nicergoline and bendazac lysine due to retinal vein occlusion at ophthalmologic department. Thereafter, he experienced intermittent fever and skin rash. On admission, clinical symptoms (i.e. arthralgia and fever) and laboratory findings (i.e. eosinophilia and renal failure) suggested AIN, and which was confirmed by pathologic findings on renal biopsy. A lymphocyte transformation test demonstrated a positive result against nicergoline. Treatment was consisted of withdrawal of nicergoline and intravenous methylprednisolone, and his renal function was completely recovered. To our knowledge, this is the first report of nicergoline-associated AIN.     

Modified extracardiac Fontan in a patient with separate hepatic venous drainage

We describe an alternative technique to the extracardiac Fontan procedure in a patient with a univentricular heart, in which the inferior caval vein and the hepatic vein drained separately into the common atrium and the intraatrial orifice of the hepatic vein was adjacent to the opening of the left lower pulmonary vein.