Sarcoidosis and primary biliary cirrhosis

Summary An unusual case of a woman with primary biliary cirrhosis and cutaneous sarcoidosis is described. The factors that allow a specific diagnosis of each condition are presented and the literature pertaining to such complex and unusual cases is presented.This work was supported in part by a grant from the Gastroenterology Medical Research Foundation of Southwestern Pennsylvania.     

Change in renal tubular sodium and water handling during progression of polycystic kidney disease: relationship to atrial natriuretic peptide

Renal tubular sodium and water handling determined by the lithium clearance technique, plasma concentrations of atrial natriuretic peptide (ANP), angiotensin II, aldosterone, arginine vasopressin (AVP), and urinary excretion of prostaglandin E2 (PGE2) were determined both during basal conditions and before and after intravenous sodium loading with a 2.5% sodium chloride solution in patients with polycystic kidney disease (PKD), ten with normal or slightly reduced kidney function (PKDN) and seven with moderately reduced kidney function (PKDR), and in 15 healthy controls. In PKDN tubular function was normal, whereas in PKDR both proximal and distal reabsorption of sodium and water were reduced. Angiotensin II and aldosterone were normal in both groups of patients. During basal conditions ANP was higher in PKDR than in PKDN. PGE2 was significantly higher in PKDR than in PKDN. For all patients significant correlations were found between GFR and both ANP (rho = -0.51, n = 17, P less than 0.05) and PGE2 (rho = -0.53, n = 17, P less than 0.05). It is concluded that renal sodium handling is normal in the early stages of PKD. With deterioration of kidney function both proximal and distal tubular reabsorption of sodium is reduced and the accompanying changes in ANP and PGE2 may be compensatory phenomena counteracting declining glomerular filtration rate.     

Neridronate prevents bone loss in patients receiving androgen deprivation therapy for prostate cancer.

Today, androgen deprivation therapy is a cornerstone of treatment for advanced prostate cancer, although it presents important complications such as osteoporosis. Neridronate, a relatively new bisphosphonate, is able to prevent bone loss in patients with prostate cancer during androgen ablation. INTRODUCTION: Androgen-deprivation therapy (ADT) is a cornerstone of treatment for advanced prostate cancer. This therapy has iatrogenic complications, such as osteoporosis. The aim of our study was to evaluate the efficacy of neridronate, a relatively new bisphosphonate, to prevent bone loss during androgen ablation. MATERIALS AND METHODS: Forty-eight osteoporotic patients with prostate cancer, treated with 3-month depot triptorelina, were enrolled and randomly assigned to two different treatment groups: group A (n = 24) was treated with a daily calcium and cholecalciferol supplement (500 mg of elemental calcium and 400 IU cholecalciferol), and group B (n = 24) received in addition to the same daily calcium and cholecalciferol supplement, 25 mg of neridronate given intramuscularly every month. All patients also received bicalutamide for 4 weeks. Lumbar and femoral BMD was evaluated by DXA at baseline and after 1 year of therapy; moreover, deoxypyridinoline (DPD) and bone alkaline phosphatase (BALP) were determined at the beginning, midway through, and at the end of the study. RESULTS: After 6 and 12 months, whereas patients treated only with calcium and cholecalciferol (group A) showed a marked bone loss, with increased levels of DPD and BALP compared with baseline values, patients treated also with neridronate (group B) had substantially unchanged levels of these markers. After 1 year of treatment, lumbar and total hip BMD decreased significantly in patients treated only with calcium and cholecalciferol (group A), whereas it did not change significantly at any skeletal site in patients treated also with neridronate (group B). No relevant side effects were recorded during our study. CONCLUSIONS: Neridronate is an effective treatment in preventing bone loss in the hip and lumbar spine in men receiving ADT for prostate cancer.     

Outcomes of a pilot occupational therapy wellness program for older adults.

OBJECTIVE: The purpose of this study was to evaluate a pilot occupational therapy wellness program designed to teach elders the importance of participation in meaningful social and community occupations to their quality of life. METHOD: Sixty-five older adults participated in this pilot wellness program held at each of three senior apartment complexes. Measures of health-related quality of life using the SF-36 Health Survey and frequencies of social and community participation from a program-specific intake form were completed by 39 participants before and after the 6-month program. Participants also evaluated components of the program through a satisfaction survey. RESULTS: Scores on the SF-36 Health Survey were significantly higher in vitality, social functioning, and the mental health summary scores following participation in the program. Participants reported an increased frequency of socialization and community participation with an average of 55% participating in at least three or more activities per week before the program to an average of 66% participating after the program. Participants who benefited the most attended more classes, were older, and were nondrivers. Eighty percent of those polled rated the pilot program as good or excellent. CONCLUSION: This pilot study provides additional support for prevention efforts for elders in the community. Wellness programs for seniors may be most effective if targeted to those who are older and nondrivers.     

Vasoactive intestinal peptide (VIP) activation of nuclear protein kinase C in purified nuclei of rat splenocytes

We have examined the actions of vasoactive intestinal peptide (VIP) and certain other known immune modulators on a nuclear pool(s) of protein kinase C (PKC) in isolated rat splenocyte nuclei. Rat splenocyte nuclei pure by enzymatic and electron microscope criteria demonstrated a time- and concentration-dependent activation of nuclear PKC (nPKC) by VIP. A biphasic pattern of three bell-shaped curves was observed with peak phosphorylation at 10⁻¹⁵, 10⁻⁹ and 10⁻⁶M VIP. The phosphorylation of endogenous nuclear substrates was characterized as a PKC-mediated event by use of three known PKC inhibitors, 1-(5-isoquinolinylsulfonyl)-2-methylpiperazine (H-7), sphingosine, and staurosporine, which produced similar phosphate incorporation measurements. Also, this activity was blocked with the addition of a monoclonal antibody to PKC. Inhibitors of the ability of VIP to activate nPKC included somatostatin, 8-bromo-cAMP, peripheral benzodiazepine receptor modulators, and the PKC inhibitors, sphingosine and staurosporine. These data have direct relevance to our knowledge of cell-mediated immunity.     

Early growth delay in diabetic pregnancy: relation to psychomotor development at age 4

Ninety nine consecutive insulin dependent and 101 non-diabetic pregnant women were examined by ultrasonograph to assess early fetal growth. In 42 of the diabetic mothers and three of the non-diabetic mothers the scan showed early intrauterine growth delay. At 4-5 years of age all children available for study were evaluated by the Denver developmental screening test. Only 23 of the 34 children of diabetic mothers with early intrauterine growth delay had normal test scores compared with 46 of the 50 children of diabetic mothers with normal intrauterine growth. The children failed in personal-social development, gross motor development, and particularly in language and speech development. Children of diabetic mothers with normal early fetal growth had scores very similar to those of the children of non-diabetic mothers, of whom 76 of the 86 tested had normal scores. This study suggests that children with a history of growth delay in early diabetic pregnancy should be screened for possible developmental impairment.     

Prediction of severe postoperative complications in patients admitted to neuroanaesthesia

The purpose of this study was to identify factors which will predict the risk of severe postoperative complications in individual patients in a neurosurgical unit. Eleven risk factors were investigated in 363 neurosurgical patients, of whom 40 (11%) developed postoperative complications requiring mechanical ventilation for more than 24 h in an intensive care unit, 16 were found to be severely disabled or in a vegetative state at follow-up 1 month after admission, and 28 patients died. By applying stepwise, logistic regression analysis to the patient's data, we were able to select two significant risk variables, i.e. the Glasgow Coma Scale (GCS) 8 or less preoperatively and emergency anaesthesia. The presence of GCS 8 or less on the consciousness scale was associated with postoperative complications in approximately 40% of the cases. Emergency patients with severe neurological damage had the greatest risk of postoperative complications (93%). Fatal outcome for patients with postoperative complications was seen in 40% of the cases.     

Anesthesia and/or Sedation for Pulsed Dye Laser Therapy

One of the most exciting developments in pediatric dermatology has been the use of the flashlamp-pumped, 585-nm, pulsed dye laser for treatment of vascular birthmarks. In many cases the results are miraculous. The increase in self-esteem and happiness of many children and adolescents has been overwhelming; for some, depression has been lifted, stuttering has ceased, social involvement has increased, and antidepressants have been discontinued. There are many success stories to tell.
Despite the remarkable effects of the pulsed dye laser and the medical and psychosocial indications for its use, the issue of pain control remains significant. We have no perfect outpatient pediatric anesthetic. Most methods carry either some risk or, if not hazardous, often are not very effective for controlling pain. Needless to say, a diversity of opinions exist on how to manage discomfort from this treatment modality. Therefore, we thought it would be useful to share the experiences and opinions of several dermatologists who have extensive experience with the pulsed dye laser.     

Myeloradiculopathy associated with wasp sting

A 12-year-old girl developed a reversible myeloradiculopathy 1 week after a wasp sting. Delayed neurologic hypersensitivity reactions to Hymenopteran stings occur primarily in adults. Reactions involving both the peripheral and central nervous systems are extremely rare and have never been reported in a child. The mechanisms underlying this uncommon reaction may be related to age-dependent differences in immunologic responses.     

Inter- and intraobserver variability in the histopathological diagnosis of medullary carcinoma of the breast,and its prognostic implications

Summary One hundred thirty-one breast carcinomas with medullary features, registered in the Danish Breast Cancer Cooperative Group from 1977–1982, have been histopathologically reviewed by two senior pathologists and classified as typical medullary carcinoma (TMC), atypical medullary carcinoma (AMC), and non-medullary carcinoma (NMC). Diagnostic criteria were based on those put forward by Ridolfiet al. and Fisheret al. The procedure was repeated with an interval of about one year by both pathologists. The diagnostic interobserver agreement was 72% with a Kappa of 0.55. The intraobserver agreement was 77% and 63% with Kappa values of 0.64 and 0.44, respectively. To see whether the observed inter- and intraobserver variability had any prognostic implications, diagnostic subgroups for both pathologists were analyzed with Kaplan Meier plots for recurrence-free survival (RFS) and with log rank tests. In the first evaluation pathologist 1 segregated a group of TMC with a significantly better RFS than for the NMC group, and pathologist 2 segregated a group of TMC with a corresponding strong trend. These findings could not, however, be reproduced in the second evaluation. The study indicates that the criteria of TMC and AMC as proposed by Ridolfiet al. need to be sharpened and simplified in order to reduce inter-and intraobserver variability. Larger studies with a control group of infiltrating ductal carcinomas are mandatory to elucidate the clinical importance of the diagnoses of Typical and Atypical Medullary Carcinoma of the breast.