Bone disease in primary biliary cirrhosis: Lack of association with distal renal tubular acidosis

Background and Aims: Primary biliary cirrhosis (PBC) might be complicated by osteoporosis, whose etiology remains unknown but seems to be multifactorial. Prevalence rates of 30% to 60% for distal renal tubular acidosis (DRTA) have been reported in PBC patients, generally as incomplete DRTA. Although it is undisputed that a reduced bone mineral density (BMD) is the expected outcome among patients who have been suffering from longstanding chronic metabolic acidosis, it is unclear if incomplete DRTA is also associated with metabolic bone disease in PBC patients. The present study was undertaken to compare the BMD of PBC patients with and without DRTA.
Methods: The BMD of 23 PBC patients (11 with DRTA and 12 without), all with normal clearance of creatinine, was assessed by dual energy radiograph absorptiometry. The diagnosis of DRTA was made if the urine pH was above 5.4 in all samples after the oral acid overload, showing tubular inability to acidify urine in the presence of test-induced systemic metabolic acidosis.
Results: Densitometric signs of osteoporosis were found in 82% of DRTA cases and in 83% of patients without DRTA (difference not significant). There were no significant differences in BMD measurement, T and Z scores of patients with and without DRTA.
Conclusions: The present study could not support a correlation between the presence of DRTA and the bone loss observed in PBC patients.     

URODYNAMIC AND CLINICAL EVIDENCE OF ACUTE INHIBITORY EFFECTS OF INTRAVESICAL NOCICEPTIN/ORPHANIN FQ ON DETRUSOR OVERACTIVITY IN HUMANS: A PILOT STUDY

PURPOSE: Management of neurogenic incontinence is complex and available treatments are not satisfactory. Nociceptin/orphanin FQ, a recently discovered neuropeptide, has been reported to inhibit the voiding reflex in the rat. These experimental results prompted us to investigate the urodynamic and clinical effects of intravesical instillation of nociceptin/orphanin FQ in humans. MATERIAL AND METHODS: Our study involved 5 normal subjects (group 1) with a mean age of 40.4 years (range 21 to 54) and 9 patients (group 2) 40.4 years (24 to 54). All patients in group 2 presented with detrusor hyperreflexia refractory to standard therapy. They were invited to undergo a filling cystometrogram with saline solution and after 30 minutes, a new one with a solution containing 1 microM. nociceptin/orphanin FQ. The urodynamic parameters that were recorded included bladder capacity, volume threshold for the appearance of detrusor hyperreflexia and maximum bladder pressure. Clinical and urodynamic followup was performed after 15 days. The data were statistically analyzed with 1-way analysis of variance followed by the Dunnett test for multiple comparison considered statistically significant with p <0.05. RESULTS: Intravesical instillation of 1 microM. nociceptin/orphanin FQ in group 1 did not produce significant functional changes. This infusion in group 2 produced a statistically significant increase in mean bladder capacity and volume threshold for the appearance of detrusor hyperreflexia from 164 plus or minus standard deviation (SD) 84 to 301 +/- 118 and 93 plus or minus SD 41 to 231 +/- 104 ml. (p <0.05, respectively). Mean maximum bladder pressure decreased from 79 plus or minus SD 25 to 54 +/- 44 cm. water but was not statistically significant (p = 0.19). After 15 days an absence of clinical improvement was noticed in group 2, and the urodynamic control did not show any significant changes compared to the values before nociceptin/orphanin FQ treatment. No severe symptomatic reactions were observed during infusion of 1 microM. nociceptin/orphanin FQ. CONCLUSIONS: Our results demonstrate that nociceptin/orphanin FQ is able to elicit a robust inhibitory effect on voiding reflex in group 2 but not 1. The ideal dosage, route of administration of nociceptin/orphanin FQ and treatment interval are not yet established.     

The Effects of Two Alkaloids Derived from Vinca Rosea on the Malignant Cells of Hodgkin's Disease, Lymphosarcoma and Acute Leukemia in Vivo

Hodgkin’s disease, lymphosarcoma, and acute leukemia have been studiedafter treatment with the Vinca rosea alkaloids (vinblastine and vincristine), inorder to demonstrate and evaluate the mitosis-arresting effects of the twodrugs on the malignant cells. Histologic sections, lymph node and bone marrow aspirations were performed immediately before and 24 hours after theintravenous administration of the drugs in 15 cases of Hodgkin’s disease, 12 oflymphoblastic lymphosarcoma, and 12 of acute leukemia, besides other miscellaneous cases.

In Hodgkin’s disease aspirates and sections showed a clear-cut metaphasearrest in the post-VLB specimens, chiefly affecting the pre-Sternberg or Hodgkin cells. This effect, besides corroborating the fundamentally stathmokineticmechanism of VLB in Hodgkin’s disease, was considered an additional factorin confirming the widely proposed conception that these cells represent thefundamentally proliferating and malignant tissue of this disease.

In the acute leukemias and lymphoblastosarcomas, cytomorphologic andquantitative studies demonstrated that the oncolytic effects correlated wellwith the magnitude of metaphasic blockade. It is postulated that only activelyproliferating cells—the so-called "growth fraction"—are the target for thesealkaloids.

Submitted on January 30, 1966 Accepted on June 26, 1966     

ALCOHOL DEHYDROGENASE FROM HUMAN STOMACH: VARIABILITY IN NORMAL MUCOSA AND EFFECT OF AGE, GENDER, ADH3 PHENOTYPE AND GASTRIC REGION

Alcohol dehydrogenase (ADH) has been analysed in 36 endoscopicbiopsies of normal gastric body and/or antrum mucosa, from 31individuals with an age between 17 and 79 years. Oesophageal,duodenal and oral mucosa specimens have been also examined.Stomach mucosa contains three isozyme types: the     

Successful Radiofrequency Ablation of an Accessory Pathway During Pregnancy

The preexcitation syndrome is a rare entity during pregnancy. We present a 20-week pregnant patient with Wolff-Parkinson-White syndrome and recurrent episodes of tachycardia with hemodynamic compromise refractory to medical treatment that required electrical cardioversion several times. Due to the poor evolution we performed a successful radiofrequency ablation of a right posteroseptal accessory pathway using 70 seconds of total fluoroscopy time without complications. We consider this is an alternative and safer treatment in those cases in which the tachyarrhythmias compromise the hemodynamic state during pregnancy.     

Implications of gluten exposure period,CD clinical forms,and HLA typing in the association between celiac disease and dental enamel defects in children. A case–control study

International Journal of Paediatric Dentistry 2010; 20: 119–124 Background. The association between coeliac disease (CD) and dental enamel defects (DED) is well known. Aim. The aim of this study was to investigate the prevalence of DED in children with CD and to specifically find the association of DED and gluten exposure period, CD clinical forms, HLA class II haplotype. Design. This study was designed as a matched case–control study: 250 children were enrolled (125 coeliac children – 79 female and 46 male, 7.2 ± 2.8 years and 125 healthy children). Data about age at CD diagnosis, CD clinical form, and HLA haplotype were recorded. Results. Dental enamel defects were detected in 58 coeliac subjects (46.4%) against seven (5.6%) controls (P < 0.005). We found an association between DED and gluten exposure period, as among CD subjects the mean age at CD diagnosis was significantly (P = 0.0004) higher in the group with DED (3.41 ± 1.27) than without DED (1.26 ± 0.7). DED resulted more frequent (100%) in atypical and silent CD forms than in the typical one (30.93%). The presence of HLA DR 52‐53 and DQ7antigens significantly increased the risk of DED (P = 0.0017) in coeliac children. Conclusions. Our results confirmed a possible correlation between HLA antigens and DED.