Morphologic and immunohistochemical observations in granulomatous glomerulonephritis.

Eight renal biopsies of Wegener's granulomatosis and other vasculitic syndromes with periglomerular granulomatous reactions (granulomatous glomerulonephritis) are studied. Controls consist of 57 biopsies of crescentic and focal necrotizing glomerulonephritis (of various diseases, excluding systemic lupus erythematosus). Both groups have in common the morphologic alterations of inflammatory tuft necrosis, fibrin exudation into the urinary space, crescent formation, and breaks of Bowman's capsule. In the study group, the unique feature of glomeruli with surrounding granulomatous reactions was the presence, within the urinary space, of an exudate of fibrin mixed with immune reactants (immunoglobulins and usually complement) in association with gaps of Bowman's capsule. This contrasts with an exudate of fibrin alone in glomeruli of the control group and other glomeruli of the study group. The study group also had a significantly greater incidence of immune deposits in Bowman's capsule (P less than 0.001). Giant cells may result from the interaction of macrophages of the granuloma with Bowman's capsule that is permeated with immune reactants.     

Renal protection by antihypertensive therapy

The attainment of adequate renal protection requires strict blood pressure control and a diminution of proteinuria or microalbuminuria to values as near from normalcy as possible. It has been considered that by getting the first, the second could be attained at the same price. Recent data have confirmed that renal protection in hypertensive patients, diabetics or not, requires combination therapy that has to include an angiotensin converting enzyme inhibitor or an angiotensin receptor blocker. A calcium channel blocker can be added to this without renal compromise. A diuretic will also be needed in most cases. Proteinuria will diminish with this combination in particular if up-titration of the drug blocking the effects of angiotensin II is performed. The control of other associated risk factors is also required, in particular smoking and lipids.     

Emmonsia crescens infection in a British water vole (Arvicola terrestris).

Emmonsia crescens, a dimorphic fungus of the order Onygenales, is primarily a pathogen of lower animals and rarely humans. Inhaled conidia of E. crescens fail to germinate in the lungs, and instead simply enlarge in lung tissue to become giant adiaspores. We present here the case of fatal Emmonsia crescens infection in a wild-caught British water vole (Arvicola terrestris). Histopathological examination of the animal, which died in captivity, revealed a multifocally extensive granulomatous reaction containing oval adiaspores scattered irregularly throughout the lungs. Mycological examination of fungus cultured from lung tissue and PCR amplification and sequencing of rDNA gene fragments of the cultured organism confirmed the diagnosis of massive infection by E. crescens.     

The effect of increased sodium in the drinking water on right ventricular hypertrophy, right ventricular failure and ascites in broiler chickens

One hundred commercial male broiler chickens were grown to 27 days in four floor pens on a commercial diet containing 0.14% sodium (Na+). From day 6 each pen received different levels of sodium chloride (NaCl) in the drinking water; 0.0%, 0.15% (0.06% Na+), 0.3% (0.12% Na+) and 0.6% (0.24% Na+). Eight chicks from each group were killed at 13, 20 and 27 days and examined for right ventricular hypertrophy (RVH) and right ventricular failure (RVF). By day 27 as little as 0.06% added Na+ caused two cases of RVH and one case of RVF with ascites, typical of the ascites caused by RVF in commercial broilers. RVH, RVF and ascites developed earlier in broilers on higher levels of Na+.     

Phenotype delineation of ZNF462 related syndrome

Zinc finger protein 462 (ZNF462) is a relatively newly discovered vertebrate specific protein with known critical roles in embryonic development in animal models. Two case reports and a case series study have described the phenotype of 10 individuals with ZNF462 loss of function variants. Herein, we present 14 new individuals with loss of function variants to the previous studies to delineate the syndrome of loss of function in ZNF462. Collectively, these 24 individuals present with recurring phenotypes that define a multiple congenital anomaly syndrome. Most have some form of developmental delay (79%) and a minority has autism spectrum disorder (33%). Characteristic facial features include ptosis (83%), down slanting palpebral fissures (58%), exaggerated Cupid's bow/wide philtrum (54%), and arched eyebrows (50%). Metopic ridging or craniosynostosis was found in a third of study participants and feeding problems in half. Other phenotype characteristics include dysgenesis of the corpus callosum in 25% of individuals, hypotonia in half, and structural heart defects in 21%. Using facial analysis technology, a computer algorithm applying deep learning was able to accurately differentiate individuals with ZNF462 loss of function variants from individuals with Noonan syndrome and healthy controls. In summary, we describe a multiple congenital anomaly syndrome associated with haploinsufficiency of ZNF462 that has distinct clinical characteristics and facial features.     

Utility of a multiplex PCR assay for detecting herpesvirus DNA in clinical samples

A multiplex PCR was designed to amplify herpes simplex virus types 1 and 2, cytomegalovirus, and varicella-zoster virus DNA present in a diverse range of clinical material. The susceptibility of these viruses to in vivo inhibition by at least one antiviral drug was an important consideration in their inclusion in the multiplex detection system. An aliquot of equine herpesvirus was introduced into each specimen prior to extraction and served as an indicator of potential inhibitors of the PCR and a detector of suboptimal PCR conditions. Compared to virus isolation and immunofluorescence-based antigen detection, the multiplex assay yielded higher detection rates for all viruses represented in the assay. The turnaround time for performance of the assay was markedly reduced compared to those for the other techniques used to identify these viruses. More than 21,000 tests have been performed using the assay. Overall, the multiplex PCR enabled the detection of substantially increased numbers of herpesviruses, in some cases in specimens or anatomical sites where previously they were rarely if ever identified using traditional detection methods.