Circulating factors cause proteinuria in parabiotic zebrafish.

Proteinuria can be induced by impairment of any component of the glomerular filtration barrier (GFB). To determine the role of circulating permeability factors on glomerular damage, we developed a parabiosis-based zebrafish model to generate a common circulation between zebrafish larvae. A morpholino-mediated knockdown of a podocyte specific gene (nephronectin) was induced in one zebrafish larva which was then fused to an un-manipulated fish. Notably, proteinuria and glomerular damage were present in the manipulated fish and in the parabiotically-fused partner. Thus, circulating permeability factors may be induced by proteinuria even when an induced podocyte gene dysregulation is the initiating cause.

Characterizing renal involvement in Hermansky-Pudlak Syndrome in a zebrafish model.

Hermansky-Pudlak Syndrome (HPS) is a rare disease caused by mutations in the genes coding for various HPS proteins. HPS proteins are part of multi-subunit complexes involved in the biogenesis of organelles from the lysosomal-endosomal-system. In humans, this syndrome is characterized by the presence of albinism, platelet dysfunction and pulmonary fibrosis. The renal component to the disease remains unstudied and untreated in patients with HPS. Here we demonstrate that in humans, HPS proteins have a high renal expression with active transcription of HPS1, 3, 4 and 5 in human podocyte cell culture, suggesting that impaired function of HPS proteins could directly impact renal function. Therefore, we developed a zebrafish model to study the renal involvement of HPS proteins in proteinuric kidney disease. Remarkably, knockdown of HPS genes in zebrafish causes glomerular injury with edema, proteinuria and structural changes of the glomerular filtration barrier. Moreover, reduced expression of HPS proteins in zebrafish recapitulates other important disease hallmarks, like hypopigmentation and accumulation of intracellular debris characteristic of lysosomal disorders. In conclusion, we present a valid zebrafish model that highlights the previously underestimated relevance of renal disease in HPS. This draws attention to the therapeutic options available to manage this component of the syndrome.

What do parents want for their children who are overweight when visiting the paediatrician?

OBJECTIVE: The objective of this study was to determine whether parental preferences regarding primary care weight-management strategies differ by child age, gender, overweight severity, race/ethnicity or parental agreement that their child is overweight. METHODS: A survey was administered to parents of 2- to 18-year-old children who are overweight at an academic primary-care clinic regarding perception of child overweight, helpful/harmfulness of having the child present during weight discussions, and dietary-advice preferences. Multivariable analyses examined factors associated with preferred weight-management strategies, after adjustment for parent/child characteristics. RESULTS: Eighty-three per cent of parents agreed that a child's presence during weight discussions is helpful/very helpful, 74% that paediatricians should prescribe specific diets, and 55% preferred specific vs. general dietary advice only (N = 219). In multivariable analyses, characteristics associated with helpfulness of child presence included older child age (vs. 2-5 year olds, 6-11 year olds: odds ratio [OR], 4.6; 95% CI, 1.3-16; 12- to 18-year-olds: OR, 23; 95% CI, 4-136), male gender (OR, 5.0; 95% CI, 1.7-10) and obesity (vs. overweight: OR, 2.8; 95% CI, 1.7-12). Characteristics associated with preferring specific diets included Latino race/ethnicity (OR, 5.3; 95% CI, 3-12), older age (vs. 2-5 year olds, 6-11 year olds: OR, 2.8; 95% CI, 1.1-7; 12-18 year olds: OR, 3.7; 95% CI, 1.5-10) and agreement that the child is overweight (OR, 2.3; 95% CI, 1.1-5) and, for specific dietary advice, older age (vs. 2-5 year olds: OR, 2.3; 95% CI, 1.1-5) and agreement that the child is overweight (OR, 2.1; 95% CI, 1.2-4). CONCLUSIONS: Findings suggest that weight-management strategies tailored to child age, gender, over-weight severity, race/ethnicity and parental agreement that their child is overweight may prove useful in improving child weight status.

Efficacy and tolerance of acetate free biofiltration: a central Italian multicenter trial.

One hundred and thirteen patients from 51 dialysis units have been treated for 12 months on AFB using a high flux AN69 dialyzer (Filtral 12 or 16-Hospal), a buffer free dialysate (Na+ 139, K+ 2, Ca++ 2 mmol/liter, glucose 1 g/liter) and a reinfusate solution containing 145 mmol/liter HCO3Na. All of them had previously been stabilized on acetate dialysis (30 patients) or bicarbonate dialysis (46 patients) or different dialysis procedures (37 patients). AFB sessions were performed for 3.71 +/- 0.28 hrs, three times a week, at an average blood flow of 308 +/- 8.5 ml/min so as to ensure a stable Kt/V value > 1 (1.16 +/- 0.08). Blood samples were drawn monthly pre- and post-dialysis, for blood electrolytes, calcium, phosphate, chloride, urea, creatinine and uric acid, proteins and lipid profiles, hemochrome. Body weights, blood pressures and adverse side effects were recorded at each session. Using a mean infusion volume of 7.96 +/- 0.61 liter/session, post-dialytic bicarbonate values consistently > 25 mmol/liter (26.33 +/- 2.71) were achieved. Intradialytic occurrence of symptoms was very low at an average rate < 1.5 episodes patient/month. After 12 months of Tx an adequate control of uremia, of electrolytes, of Ca-PO4 balance and of BP was obtained. AFB gets a very high intradialytic tolerance and an excellent depurative capacity which contributes to the well being of the patients.

Responding to threat: hemispheric asymmetries and interhemispheric division of input.

This investigation examined how hemispheric asymmetry and interhemispheric processing contribute to attentional biases toward emotional information. Participants (n = 88) named the color of lateralized squares presented concurrently with neutral, positive, or threatening words. A left-hemisphere advantage in color naming was reduced when distractors were emotional, suggesting right-hemisphere priming by emotional stimuli. Furthermore, the advantage of dividing the word and color across visual fields was increased for emotion words when they were frequently presented, indicating a strategic use of interhemispheric division of labor to reduce the distracting effect of emotional words. Finally, participants with high levels of anxious apprehension were most likely to make use of this interhemispheric processing strategy, supporting a processing efficiency theory of cognitive function in anxiety.

Electrophysiological aspects of nervous conduction in uremia.

Some neurophysiological techniques have been employed in clinical nephrology to record abnormalities of nervous conduction in central and peripheral pathways. The electrical monitoring on the peripheral and central nervous systems has allowed the detection of uremic neural injury, the diagnosis of specific electrophysiological abnormalities, the evaluation of various treatments employed and the identification of those abnormalities that uremia can induce. A group of 156 subjects subdivided into four groups were examined: 100 healthy subjects (64 M, 36 F); 56 patients (21 glomerulonephritis, 14 pyelonephritis, 5 nephrolithiasis, 5 polycystic kidney, 4 nephroangiosclerosis, 7 undetermined) with chronic renal failure treated with a conventional low nitrogen diet (CLND, 0.6 g/kg b.w./d. of proteins), 8 of whom passed from CLND to a very low nitrogen diet supplemented with alpha-keto-analogues; a group of 22 of these 56 underwent a regular dialysis treatment for 12 to 15 hours/weekly for 40.5 +/- 10.2 months. Three patients of the CLND group and 13 patients underwent renal transplantation after a variable period of RDT. In the uremic patients we found different populations of motor unit potentials; a decreased MNCV was found in 35% of the CLND patients, RDT patients had slowed MNCV in 42%. The SNCV was compromised more frequently than the MNCV. An increased duration of evoked potentials was sometimes observed in CLND and RDT patients inducing us to consider this a hallmark of uremic syndrome. The alpha-keto-analogues and HD/HP treated patients showed an improvement in several features.(ABSTRACT TRUNCATED AT 250 WORDS)

[Multilocular cystic nephroma in children. Description of a case]. / Nefroma cistico multiloculare in età pediatrica. Descrizione di un caso.

In paediatric age there may occur the various types of cystic renal tumor also of different clinical meaning; we can count among them shapes openly or potentially deadly, among which Wilms tumor cystic owing to haemorrhages and tumoral necrosis, congenital mesoblastic cystic nephroma, clear cell carcinoma, cystic, partially differentiated nephroblastoma (CPDN) and benign forms such as multilocular cystic nephroma (MLCN), cystic localized dysplasia of the kidney. In some cases, the differential diagnosis is difficult and it becomes only by histology: particularly the differential diagnosis between MLCN and CPDN, indistinguishable on the basis of the imaging and the macroscopic aspect, it is possible only by histology. It is described a case of multicystic lesion in a baby, male, aged twenty-two months, with an indolent mass of left side, screened by US and TC. The US examination has put into evidence the presence of renal with clean outlines of eight cms, formed by multiple small cysts, divided by thin regular septa . These aspects have been confirmed by TC. The histological examination has diagnosed a multilocular cystic nephroma, without atypias. Therefore, the multilocular cystic lesions of the kidney have to be considered potentially malignant and submitted to surgical treatment.

[Food allergy in children. Diagnostic difficulties, doubts and digressions]. / Allergia alimentare nel bambino. Difficoltà diagnostiche, dubbi, divagazioni.

It is very difficult to diagnose an alimentary allergy especially because of: the difference among individuals in their physical, psychic and humoral reactions to the environment; weakness and variability of these reactions during the puberty; limited reliability in the results of certain kind of research (i.e. false positiveness and negativeness, discordance between laboratory results and the results of the challenge, neutralization of diagnostic antigenic extracts and/or the presence in these extracts of lectin, etc.); the bad compliance of some therapies and their restricted efficaciousness, particularly in the little children. Moreover: the superimposition of the clinical manifestations of alimentary allergy and pseudoallergy with those of allergy, the possibility of an association between the alimentary and the respiratory allergy, the acquisition on behalf of the allergic child of other kinds of allergy, false polyallergy, the allergy might change its seats and in consequence also its manifestations. The authors, apart a short account of the different diagnostic methods, lay stress on the challenge as the most reliable. They discuss upon the dose of a nourishment that can be given to the patient without risk, besides they suggest to take into account the type and the seriousness of the clinical manifestations in fixing this dose.

[IgA deficiency in pediatrics]. / Deficit di IgA in pediatria.

To evaluate the IgA deficiency we have considered 368 children, admitted to "Day hospital" for different pathologies: 64 of them had serum IgA levels lower than the normal values for their age according to Ellis and Robbin. The age of the children with this deficiency was included from 4 months up to 8 years: 39 were boys, 25 were girls. In 54 cases the IgA deficiency was "partial" that is: the IgA value found out was lower than the normal for the age, but higher than 5 mg%. In 10 cases the IgA deficiency was "severe" (the IgA value was lower than 5 mg%). Six cases, among the 29 ones with intestinal diseases, had a severe deficiency, whereas among the 22 cases with respiratory diseases 3 had a "severe" deficiency. The 2 patients with urinary disease had a partial deficiency; only one case of the remaining 11 with different pathologies had a "severe" deficiency. Among the carriers of serum IgA deficiency an high incidence of chronic pathology has been found out (83%). Nevertheless the percentage of IgA deficiency extrapolated from the all case histories doesn't differ very much (64 cases among 368 = 17.39%) considering only those patients with chronic relapsing pathology (53 cases among 275 = 18.2%). Therefore it seems that the IgA deficiency could be considered a chronicizing factor in the examinated pathology even if in few cases. Anyway the frequency of IgA deficiency has reached major values (29/54; 45.3%) in the patients with enteric diseases. The frequency of IgA deficiency is smaller but still important in those patients with respiratory diseases (22/64: 34.3%).(ABSTRACT TRUNCATED AT 250 WORDS)

[Short near-fainting in children with or without loss of conscience]. / Malesseri fugaci del bambino con o senza perdita di coscienza.

Short near-faints with or without loss of consciousness have different etiologies and pathogenesis and, according to these, they recur with various frequency. This subject was reported on several publications and here is again examined with the contribute of personal experience and the description of distinct cases. The following causes were examined: vagal hypertonia, digestive troubles (gastroesophageal reflux), metabolic troubles (cyclic ketosis, hypoglycemia and hypocalcemia with peculiar clinic manifestations). Among the neurological causes were examined: epilepsy, benign paroxysmal vertigos and, particularly, the so called breath-holding spells and the migraine, about that were reported a few examples of "migraine accompagnée". Among the cardiovascular causes (ischaemic cerebral crisis), -particular attention was dedicated to orthostatic hypotension syncope. A short hint about hysteria ends the exposition of psychical causes of short near-faints.

Action of glutamic acid and of some glutamate analogues on the molluscan central neurones.

The effects of L-glutamic acid and of some glutamate analogues have been studied on the central nervous system of the snail Heobania vermiculata, using conventional electrophysiological techniques. The glutamate H-response had the mean equilibrium value of -(57 +/- 4) mV and was associated with a Cl- conductance change. The D-response to glutamate application showed an involvement of sodium ions. Aspartate was agonist of glutamate action and displayed similar equilibrium value of the H-response, whereas quisqualate H-response was 'non-invertible'.

Lipids in the progression of chronic renal failure.

Lipid disturbances have been linked to the progression of chronic renal disease. We examined 52 patients with a creatinine clearance (CCr) of 38.5 +/- 7.9 ml/min due to various nephropathies, on free diet. Bimonthly, over a 12-month period, we assessed: serum creatinine (Cr); CCr; daily urinary urea excretion; urinary protein excretion per unit of residual renal function (UProt/CCr); total, HDL, VLDL and LDL cholesterol; triglycerides; Apo A, Apo B. Chronic renal failure was progressive in 22 patients with a slope of 1/Cr-0.00358 +/- 0.00247, stable in 30 with a slope of 0.00420 +/- 0.00285. Lipid parameters did not differ significantly between the two groups but for the lower Apo A and Apo A/Apo B ratio values in the progressive group. Overall slope inversely correlated with basal CCr; in the progressive patients the slope correlated with the percentage variation of UProt/CCr and only partially with the altered Apo profile.

The brainstem auditory evoked responses in Alport's syndrome.

Alport's syndrome is a "hereditary nephritis" associated with structural defects of basement membranes in kidneys, ears and eyes, with variable clinical expression. As the acoustic abnormalities are often subclinical, we studied the brainstem auditory evoked responses (ABR) and tonal audiometry in 12 patients (7 males, 5 females, mean age 32.50 +/- 16.70 years) with histologically documented renal lesions consistent with Alport's syndrome. ABR were used to document the altered acoustic-nerve conduction and transmission typical of the early stage of Alport's syndrome. In 11 patients (seven males, four females), we found bilateral delayed latency (I dx: 2.50 +/- 0.80 msec; III dx: 4.24 +/- 0.68 msec; V dx: 7.02 +/- 0.94 msec) and altered waveform I, III, V by ABR. By audiometry eight patients (six males, two females) showed a loss of medium-high tones, and this group included the patients with a negative ABR study. Therefore, the ABR is abnormal in Alport's syndrome; it may be positive in patients with normal tonal audiometry, usually all cochlear functions are grossly intact; the acoustic-nerve lesions in Alport's syndrome may precede the cochlear involvement and clinical hearing loss; the electrophysiological analysis does not identify the particular nervous structures involved in the acoustic pathways.