Role for specific complement phenotypes and deficiencies in the clinical expression of IgA nephropathy

IgA nephropathy, the most commonly occurring type of chronic glomerulonephritis in individuals of European and Asian descent, exhibits marked heterogeneity of clinical signs and ultimate prognosis. Based upon their studies of regional clustering of the ancestors of related patients in eastern Kentucky, the authors have postulated the existence of an inherited disease susceptibility for IgA nephropathy. They examined serum concentrations of individual complement proteins and phenotypes for C3, C4A, C4B, and factor B (Bf) for related and unrelated patients with IgA nephropathy from Kentucky and for patients from the Mid-South region of Tennessee, Mississippi, and Alabama. In these populations, they have described partial complement deficiencies or specific phenotypes which may be associated with the disease. Their findings include the following: (1) partial deficiencies for C2, beta 1H (H), properdin (P), or C4 binding protein (C4BP) in four patients with end-stage renal disease, (2) an association between the C3*F allele with IgA nephropathy in the combined group of unrelated patients from Kentucky and the Mid-South, (3) the occurrence of C4B deficiency in two siblings with IgA nephropathy, and (4) an association between C4A deficiency and poor outcome in patients with IgA nephropathy diagnosed as adults. In addition, the related patients differ from the unrelated patients from Kentucky with respect to frequencies of Bf*F and the BfF (FF + FS + F1F + F1S) phenotype, suggestive of immunogenetic difference between these groups. Important functional differences exist between C4A and C4B isotypes and functional differences are also possible based upon C3 or Bf phenotype.(ABSTRACT TRUNCATED AT 250 WORDS)     

Sternal tuberculosis after open heart surgery

We present a 68-y-old female who had undergone aortocoronary bypass and developed Staphylococcus aureus sternal osteomyelitis. Despite prolonged therapy with different antibiotics and several local debridements and drainage, the wound remained open and suppurative. Only after sternectomy and wide excision of infected costal cartilage was the diagnosis of sternal tuberculosis made, 24 months later. This case illustrates the necessity of a high degree of suspicion to diagnose this very rare entity which coexists with more common causes of sternal osteomyelitis.     

Characterization of Trypanosoma cruzi from Argentina by electrophoretic zymograms.

Polyacrylamide gel electrophoretic patterns for six enzymes in 73 isolates and 38 clones of Trypanosoma cruzi from different areas of Argentina were classified into 12 zymodemes. The isolates were obtained from human patients with acute, chronic or congenital Chagas' disease, vector insects, domestic and sylvatic animals. Two out of 8 isolates cloned were shown to be heterogeneous. Zymodemes 1 and 12 exhibit widespread geographic distribution; isolates belonging to both zymodemes account for 55% of the total analyzed. The other zymodemes are not widely geographically dispersed. Although there is a clear predominance of zymodeme 1 among asymptomatic patients, the data do not show a clear relationship between particular zymodemes and the clinical picture. The results suggest that the sylvatic and domestic transmission cycles overlap. This remarkable heterogeneity of T. cruzi in Argentina supports the possible multiclonal origin of these parasite populations.     

Independent influences upon mother–toddler role reversal: infant–mother attachment disorganization and role reversal in mother's childhood

In role reversal a child takes an inappropriate parental, spousal, or peer role with the caregiver. The study assessed attachment disorganization with mother in infancy in the Strange Situation (Ainsworth, Blehar, Waters, & Wall, 1978 Ainsworth, M. D.S., Blehar, M. C., Waters, E. and Wall, S. 1978. Patterns of attachment: A psychological study of the strange situation, Hillsdale, NJ: Erlbaum.  [Google Scholar]) and role reversal at 2 years old in videotaped mother–child interactions. By closely observing role reversal at this early age, results fill in the picture concerning the link between disorganized infant–mother attachment and controlling role reversal at 6 years old (Main & Cassidy, 1988 Main, M. and Cassidy, J. 1988. Categories of response to reunion with the parent at age 6: Predictable from infant attachment classifications and stable over a 1-month period. Developmental Psychology, 24: 415–426. [Crossref], [Web of Science ®] , [Google Scholar]; Main, Kaplan, & Cassidy, 1985 Main, M., Kaplan, N. and Cassidy, J. C. 1985. “Security in infancy, childhood, and adulthood: A move to the level of representation”. In Monographs of the Society for Research in Child Development Edited by: Bretherton, I. and Waters, E. 50(Serial No. 209, 1–2), 66–104[Crossref] , [Google Scholar]). As hypothesized, infant–mother disorganization significantly predicted mother–toddler role reversal. The study also deepened research that predicted role reversal from parent Adult Attachment Interview (AAI) role reversal assessed before the child was born (Macfie, McElwain, Houts, & Cox, 2005 Macfie, J., McElwain, N. L., Houts, R. M. and Cox, M. J. 2005. Intergenerational transmission of role reversal between parent and child: Dyadic and family systems internal working models. Attachment & Human Development, 7: 51–65. [Taylor & Francis Online], [Web of Science ®] , [Google Scholar]). As hypothesized, mother AAI role reversal with her mother in childhood significantly predicted mother–toddler role reversal over and above infant–mother disorganization. Results are discussed within a developmental psychopathology framework including opportunities for developmentally sensitive interventions.     

Interstitial 13q14 deletions detected in the karyotype and translocations with concomitant deletion at 13q14 in chronic lymphocytic leukemia: Different genetic mechanisms but equivalent poorer clinical outcome

Deletion of 13q14 as the sole abnormality is a good prognostic marker in chronic lymphocytic leukemia (CLL). Nonetheless, the prognostic value of reciprocal 13q14 translocations [t(13q)] with related 13q losses has not been fully elucidated. We described clinical and biological characteristics of 25 CLL patients with t(13q), and compared with 62 patients carrying interstitial del(13q) by conventional G‐banding cytogenetics (CGC) [i‐del(13q)] and 295 patients with del(13q) only detected by fluorescence in situ hybridization (FISH) [F‐del(13q)]. Besides from the CLL FISH panel (D13S319, CEP12, ATM, TP53), we studied RB1 deletions in all t(13q) cases and a representative group of i‐del(13q) and F‐del(13q). We analyzed NOTCH1, SF3B1, and MYD88 mutations in t(13q) cases by Sanger sequencing. In all, 25 distinct t(13q) were described. All these cases showed D13S319 deletion while 32% also lost RB1. The median percentage of 13q‐deleted nuclei did not differ from i‐del(13q) patients (73% vs. 64%), but both were significantly higher than F‐del(13q) (52%, P < 0.001). Moreover, t(13q) patients showed an increased incidence of biallelic del(13q) (52% vs. 11.3% and 14.9%, P < 0.001) and higher rates of concomitant 17p deletion (37.5% vs. 8.6% and 7.2%, P < 0.001). RB1 involvement was significantly higher in the i‐del(13q) group (79%, P < 0.001). Two t(13q) patients (11.8%) carried NOTCH1 mutations. Time to first treatment in t(13q) and i‐del(13q) was shorter than F‐del(13q) (67, 44, and 137 months, P = 0.029), and preserved significance in the multivariate analysis. In conclusion, t(13q) and del(13q) patients detected by CGC constitute a subgroup within the 13q‐deleted CLL patients associated with a worse clinical outcome. © 2014 Wiley Periodicals, Inc.     

Variations in non-pharmacological anti-infective measures in childhood leukemia - results of an international survey

Background Standardization in clinical practice may lead to improved outcomes. Unfortunately, little is known about the variability of non-pharmacological anti-infective measures in children with cancer. DESIGN AND METHODS: A web-based survey assessed institutional recommendations regarding restrictions of social contacts, pets and food and instructions on wearing face masks in public for children with standard- risk acute lymphoblastic leuk emia and acute myeloid leukemia during intensive chemotherapy. RESULTS: A total of 336 institutions in 27 countries responded to the survey (range, 1-76 institutions per country; overall response rate 61%). Most institutions recommend that patients with acute myeloid leukemia avoid indoor public places and daycare, kindergarten and school, whereas recommendations for patients with acute lymphoblastic leukemia differ considerably by institution. In terms of restrictions related to pets, there was a wide variability between institutions for both acute lymphoblastic and acute myeloid leukemia patients. Most, but not all institutions do not allow children with either acute lymphoblastic or acute myeloid leukemia to eat raw meat, raw seafood or unpasteurized milk. Whereas most institutions do not routinely recommend that patients with acute lymphoblastic leukemia wear face masks in public, advice on this matter varies for patients with acute myeloid leukemia. Conclusions The survey demonstrates that there is a wide variation in recommendations on non-pharmacological anti-infective measures between different institutions, countries and continents. This information may be used to encourage harmonization of supportive care practices and future clinical trials.     

Periodontal Disease,Hypertension, and Blood Pressure Among Older Adults in Puerto Rico

Background: Current scientific evidence addressing the relationship between periodontitis and hypertension is limited to studies producing inconsistent results. Methods: All participants of an ongoing representative cohort of Puerto Rican elderly who were ≥70 years old and residing in the San Juan metropolitan area were invited to this cross‐sectional study. Periodontal probing depth (PD) and attachment loss (AL) were summarized using the Centers for Disease Control and Prevention and the American Academy of Periodontology definition for severe periodontitis (≥2 teeth with AL ≥6 mm and ≥1 tooth with PD ≥5 mm). Three repeated blood pressure (BP) measurements taken were averaged using a standardized auscultatory method. Information on hypertension history, use of antihypertensive medications, and potential confounders (age, sex, smoking, heavy and binge drinking, diabetes, use of preventive dental services, flossing, body mass index, consumption of fruits, vegetables, whole wheat bread, and high‐fiber cereal) was collected during in‐person interviews. High BP was defined as average systolic BP ≥140 mm Hg or diastolic ≥90 mm Hg. Multivariate logistic regression models were used to study the relationship between severe periodontitis, hypertension history, and high BP. Results: The study population comprised 182 adults. In multivariate analysis, there was no association between severe periodontitis and hypertension history (odds ratio [OR] = 0.99; 95% confidence interval [CI]: 0.40 to 2.48). Severe periodontitis was associated with high BP, with OR of 2.93 (95% CI: 1.25 to 6.84), after adjusting for age, sex, smoking, and binge drinking. This association was stronger when restricted to those with hypertension or taking antihypertensive medications: OR = 4.20 (95% CI: 1.28 to 13.80). Conclusion: The results of this study suggest that periodontitis may contribute to poor BP control among older adults.     

Reconstrucción del conducto biliar mediante tubos tridimensionales de colágeno

Introduction

In recent years, with widespread laparoscopic cholecystectomy and liver transplantation, complications involving the biliary system are increasing. All current techniques have a high risk of recurrence or high-morbidity.

Material and methods

A 3-dimensional collagen bile duct modified with agarose hydrogel was developed to substitute the affected extrahepatic bile duct. It was used in 40 guinea pigs and the histology and physiology was studied at 4 weeks, 3 and 6 months after transplantation.

Conclusions

The graft shows to have a high potential in applications to treat hepatobiliary diseases which require surgery.