Frequency and localization of congenital anomalies of the middle and inner ears: a human temporal bone histopathological study

This study investigated congenital anomalies occurring in the middle and the inner ears, with particular attention to their features, localizations, and frequencies. One hundred human temporal bones obtained from 73 individuals, aged 31 gestational weeks to 39 years, each of whom had anomalies of the middle ear and/or inner ear, were used for this study. The temporal bones had been removed at autopsy, fixed, dehydrated, embedded in celloidin, and sectioned horizontally or vertically at 20 microns. Every 10th horizontal section or every 20th vertical section was stained with hematoxylin and eosin, mounted and studied under a light microscope. In the middle ear the structure most often found to be anomalous was the facial nerve; in the inner ear it was the lateral semicircular canal. The implications of the anomalies observed are discussed as they relate to fetal development, dysfunction of the ear, and clinical interpretation of diagnostic radiological studies.     

Activation of vascular protein kinase C-beta inhibits Akt-dependent endothelial nitric oxide synthase function in obesity-associated insulin resistance

Activation of protein kinase C (PKC) in vascular tissue is associated with endothelial dysfunction and insulin resistance. However, the effect of vascular PKC activation on insulin-stimulated endothelial nitric oxide (NO) synthase (eNOS) regulation has not been characterized in obesity-associated insulin resistance. Diacylglycerol (DAG) concentration and PKC activity were increased in the aorta of Zucker fatty compared with Zucker lean rats. Insulin-stimulated increases in Akt phosphorylation and cGMP concentration (a measure of NO bioavailability) after euglycemic-hyperinsulinemic clamp were blunted in the aorta of fatty compared with lean rats but were partly normalized after 2 weeks of treatment with the PKCbeta inhibitor ruboxistaurin (LY333531). In endothelial cell culture, overexpression of PKCbeta1 and -beta2, but not PKCalpha, -delta, or -zeta, decreased insulin-stimulated Akt phosphorylation and eNOS expression. Overexpression of PKCbeta1 and -beta2, but not PKCalpha or -delta, also decreased Akt phosphorylation stimulated by vascular endothelial growth factor (VEGF). In microvessels isolated from transgenic mice overexpressing PKCbeta2 only in vascular cells, Akt phosphorylation stimulated by insulin was decreased compared with wild-type mice. Thus, activation of PKCbeta in endothelial cells and vascular tissue inhibits Akt activation by insulin and VEGF, inhibits Akt-dependent eNOS regulation by insulin, and causes endothelial dysfunction in obesity-associated insulin resistance.     

Large goiter and multiple rib tumors

We report an interesting case of a 47-yr-old who had a large goiter and multiple rib tumors. The patient was initially suspected of having thyroid cancer, which had metastasized on the ribs, based on imaging studies. However, laboratory tests revealed a high level of ionized calcium and parathyroid hormone (PTH). The large goiter was diagnosed as having parathyroid tumors owing to the high level of PTH in the tissue fluid. The biopsy specimen from a rib tumor was diagnosed as containing brown tumors associated with primary hyperparathyroidism (PHP). The patient also had prolactinoma and pancreatic gastrinoma. Her daughter had both prolactinoma and PHP, and her brother and her father had PHP. Thus, the patient was diagnosed as having multiple endocrine neoplasia type 1.     

Treatment satisfaction,willingness to pay and quality of life in Japanese patients with psoriasis

There is a range of psoriasis treatments available, from topical applications to biologic therapy, with corresponding cost variations. The efficacy of each treatment is usually evaluated by objective measures such as the Psoriasis Area and Severity Index (PASI) or subjective measures such as the Dermatology Life Quality Index (DLQI). However, the social and economic impacts of psoriasis, including cost‐effectiveness, have not been assessed in Japan. The EuroQol 5‐Dimension (EQ‐5D) is a generic instrument used worldwide to calculate quality‐adjusted life years, on which calculations of treatment cost‐effectiveness are based. We conducted a pilot study to determine the cost‐effectiveness of psoriasis treatment in Japan. We administered a questionnaire to 133 patients with psoriasis (105 men and 28 women) who visited four university hospitals in Fukuoka Prefecture. The questionnaire covered medical costs, satisfaction and willingness to pay (WTP), and we investigated the relationships between these items. PASI was evaluated by physicians. More participants indicated satisfaction with treatment in the group paying less than ¥5000/month. WTP, PASI and EQ‐5D showed little correlation. However, the DLQI and EQ‐5D showed a moderate correlation (r = 0.472). WTP seemed more dependent on participants' economic backgrounds. We found that it was difficult to reflect the PASI with the EQ‐5D. However, the DLQI may be used to estimate the cost–benefit relationship in patients with psoriasis. This is the first study to evaluate the EQ‐5D in patients with psoriasis in Japan.     

Diminished visceral adipose tissue in cancer cachexia

To estimate the relationship between the visceral adipose tissue (AT) area and cancer cachexia, 13 cachectic patients (7 males, 6 females; age 65.2 ± 11.0 years; body mass index 20.8 ± 4.1 kg/m²) were examined by computed tomography (CT) scanning. Cachectic cancer patients who had a 10% decrease of body weight and died within 6 months because of gastrointestinal carcinoma had a significantly smaller visceral AT area than control subjects (mean ± sd: 43.9 ± 42.2 cm² vs. 93.4 ± 56.0 cm², P < 0.05, P=0.014). Otherwise, there were no significant differences between the visceral AT areas of cachectic cancer patients and those of cancer patients with resectable tumors treated by curative operation (mean ± sd: 68.8 ± 57.7 cm²) (NS, P=0.206). There was, however, a tendency for cachectic cancer patients to have a smaller visceral AT area than those with resectable tumors. This result suggests that the visceral AT area is not preserved in the cachectic state associated with cancer. © 1994 Wiley-Liss, Inc.     

Phosphorus Deficiency Syndrome in Two Very Low Birthweight Infants

Biochemical and radiographic abnormalities linked with phosphorus deficiency syndrome (PDS) developed in two very low birthweight (VLBW) infants fed exclusively with human milk. By increasing phosphorus (P) and calcium (Ca) intakes with the introduction of a specialized premature formula, osteopenia or rickets, as well as hypophosphatemia, hypo-phosphaturia and increased serum alkaline phosphatase levels improved in both patients. We speculated that all VLBW infants who are being fed exclusively with human milk should be monitored for PDS and that if PDS develops, supplementation of human milk with both P and Ca appears to be necessary for its treatment.