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.     

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     

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.     

Lymphokine Production in Ty Lymphoproliferative Disorders

We have studied five patients with chronic lymphocytosis consisting of large granular lymphocytes (LGL). The increased numbers of LGL in these patients had little or no natural killer activity, mediated antibody-dependent cellular cytotoxicity, and were induced to kill tumour lines after culture for 3 days with interleukin 2 (IL-2). Patients' LGL showed considerable reactivity with HNK-1 and AB8.28 monoclonal antibodies (MoAb), whereas positivity for OKM1 and N901 was found in only two subjects, and only one patient reacted with B73.1. No appreciable reactivity has been found with anti-Tac MoAb in the four patients tested. In the absence of stimulation, the patients' LGL produced no IL-2 and only minimal amounts of IL-1 and interferon (IFN). On stimulation with lipopolysaccharides (for IL-1) or phytohaemagglutinin A (PHA) (for IL-2 and IFN), they produced IL-1 and IFN in amounts similar to those produced by normal lymphocytes, but only modest levels of IL-2. These results indicated that proliferating LGL, like normal LGL, have a secretory capacity. The lack of constitutive lymphokine production, the lack of Tac receptor expression, and the defect in IL-2 production after PHA stimulation do not support the hypothesis of an autocrine proliferation sustained by a known growth factor.     

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.