Applicazione dell'elastosonografia nel follow up del paziente con uropatia malformativa

Applicazione dell'elastosonografia nel follow up del paziente con uropatia malformativa. Il reflusso vescico-ureterale (VUR), l\u2019uropatia malformativa pi\uf9 frequente in et\ue0 pediatrica, si definisce come il flusso retrogrado di urina dalla vescica nell\u2019uretere ed \ue8 frequentemente associato a un danno renale congenito o acquisito. Attualmente la metodica Imagign considerata il Gold Standard per valutare la funzione del parenchima renale \ue8 la scintigrafia renale statica. L\u2019elastosonografia \ue8 una modalit\ue0 imaging basata sulla forza d\u2019impulso delle radiofrequenze (Acoustic Radiation Force Impulse, ARFI) che fornisce informazioni sulle propriet\ue0 elastiche dei tessuti, sotto forma di dati numerici confrontabili. Questo studio preliminare si propone di valutare lo stato di fibrosi renale (correlato verosimilmente a una perdita di elasticit\ue0 del parenchima) in un gruppo di 28 bambini (17 maschi, 11 femmine; et\ue0 media 12+/-3 anni) con un reflusso primitivo (18 casi) o secondario (10 casi) e una storia di infezioni delle vie urinarie. I dati ottenuti nella nostra popolazione sono stati confrontati con quelli di 16 soggetti sani, comparabili per sesso ed et\ue0. I valori elastosonografici misurati nei reni patologici si sono rivelati pi\uf9 elevati rispetto a quelli misurati nei reni non affetti (176\ub145 versus 94\ub12; p<0.001). E\u2019 molto interessante che nei casi di VUR monolaterale, sia nel rene \u2018affetto\u2019 (152\ub140; p<0.001) sia nel rene \u2018controlaterale sano\u2019 (124\ub116; p<0.05), i valori elastosonografici erano pi\uf9 elevati rispetto ai controlli sani. E\u2019 stata infine dimostrata una correlazione statisticamente significativa tra le misure elastosonografiche e i valori plasmatici di cistatina c (r=0,434; P< 0.05). I nostri risultati suggeriscono che l\u2019elastosonografia pu\uf2 essere una nuova modalit\ue0 per valutare il grado di compromissione del parenchima renale in bambini con reflusso vescico ureterale e malattia renale cronica e dovrebbe essere inserita nel follow up di questi pazienti.Virtual Touch Tissue Quantification: a new approach to assess renal stiffness in children with vesicoureteral reflux Vesicoureteral reflux (VUR) is the backup of urine from the bladder into the ureteres frequently associated with congenital or acquired renal damage. The preferred method for visualizing functioning renal parenchyma is 99Tc-DMSA renal scintigraphy. Virtual Touch tissue quantification is an implementation of ultrasound Acoustic Radiation Force Impulse (ARFI) imaging that provides numerical measurements (wave velocity values) of tissue stiffness and the degree of renal fibrosis. The aim of the present preliminary study was to assess renal fibrosis by Virtual Touch tissue quantification in a group of 28 children (17 males, 11 females; age 12 \ub1 3 years) with primary (n\ub0 18) or secondary VUR (n\ub010) and history of urinary tract infection. The data obtained in our children were compared with data obtained on 16 healthy children matched for sex and age. The wave velocity values of pathological kidneys were significantly more elevated than values of non affected kidneys (176\ub145 versus 94\ub12; p<0.001). Is very interesting that in cases of unilateral VUR we found wave velocity values, either in the affected kidney (152\ub140; p<0.001) or in the controlateral \u2018healthy\u2019 kidney (124\ub116; p<0.05), higher than values in healthy controls. Moreover we found a significant correlation between the elastosonographic measures and the plasmatic cystatin C (r=0,434; P< 0.05). The present preliminary study suggests that Virtual Touch tissue quantification might be an innovative and promising technique to assess tissue stiffness and fibrosis in children with VUR and chronic renal disease. A long-term follow-up might aid to monitor the evolution of renal damage

Case Report: Long-Term Tolvaptan Treatment in a Child With SIADH and Suprasellar Arachnoid Cyst

: Suprasellar arachnoid cysts represent a rare occurrence in the pediatric population and usually cause symptoms related to mass effect and can occasionally cause endocrine dysfunctions. The association between SAC and the syndrome of inappropriate antidiuretic hormone (SIADH) in the pediatric population has rarely been described previously. In most cases, SIADH is temporary and resolves by treating the underlying cause. The first-line treatment consists of fluid restriction in asymptomatic children. Oral urea and demeclocycline are other effective treatment options. Vaptans are a new class of medication for the management of SIADH. These agents are a nonpeptide vasopressin V2 receptor antagonist that selectively antagonizes the antidiuretic effect of AVP, resulting in excretion of diluted urine or "aquaresis." Their efficacy has been shown in adult patients with euvolemic or hypervolemic hyponatremia. However, evidence is lacking in pediatric patients with SIADH. We report the case of a 9-year-old female child with a SAC, who underwent endoscopic fenestration at the age of 2 years. After surgery she developed chronic hyponatremia due to SIADH. Hyponatremia was refractory to treatment with fluid restriction, oral sodium, and urea. In order to normalize serum sodium levels, tolvaptan treatment was started on a compassionate-use basis; 24-48 h later serum sodium levels returned to normal. To date, tolvaptan has been used regularly for 6 years with no side effects occurring during the treatment period. This is the first case of a child with chronic SIADH secondary to SAC successfully treated with tolvaptan. Further studies are needed to demonstrate its usefulness on a broader case series

Renal elasticity quantification by acoustic radiation force impulse in children born preterm: preliminary results

An emerging hypothesis from the recent literature describes how specific adverse factors related to growth retardation and low birth weight might influence renal development during fetal life and the insurgence of relevant pathologies in adulthood. Preterm births and related perinatal events can affect the mechanical proprieties of kidneys in childhood. We retrieved the laboratory medical records of 3 children born preterm, all in good condition, and investigated the elastic properties of their kidneys using the acoustic radiation force impulse (ARFI) technique. Shear wave velocity (SWV) obtained by the ARFI technique depends on the elasticity of a parenchymatous tissue. Medical records of case patient 1 showed a shorter right kidney (5th percentile), a greater protein/creatinine ratio and higher \u3b11- microglobulin in the urine spot if compared with controls. Patients 2 and 3 had unremarkable laboratory results. Moreover, compared with the same results of healthy fullterm normal children obtained from a previous study of ours, we observed higher SWV values (m/s) for the left kidney in patients 1 and 3; patient 2 had lower SWV values in both kidneys. The altered SWV values, measured in these children born preterm, may be correlated with a possible underlying renal pathology (for instance, disruption of the renal histology). Altered SWV values are generally observed also in 2 out of 3 children with a history of normal laboratory markers of renal function. Further studies are needed on larger cohorts of patients

Bioestimulantes aplicados às sementes e folhas de mudas de maracujazeiro-amarelo

O objetivo deste trabalho foi avaliar o efeito de dois produtos com ação bioestimulante, Acadian® e Biozyme TS®, na emergência, no crescimento e em parâmetros morfofisiológicos de mudas de maracujazeiro-amarelo. Foram executados dois experimentos de outubro a dezembro de 2012. No primeiro, em Curitiba – PR, a aplicação dos produtos foi feita por imersão de sementes e a avaliação até os 40 dias após a semeadura. No segundo, em Pinhais, PR, a aplicação foi feita por pulverização foliar e a avaliação realizada até 60 dias após a semeadura. As concentrações testadas foram: 0, 2, 4 e 6 mL.L-1 em solução aquosa. O aumento da concentração dos produtos via semente incrementou linearmente a matéria seca da parte aérea das mudas, mas não houve efeito na emergência, altura, número de folhas e no crescimento radicular. A aplicação via foliar até a concentração de 3,15 mL.L-1 aumentou o teor de clorofila nas folhas. Também houve aumento da quantidade de raízes grossas com o aumento da concentração dos produtos, porém o comprimento total das raízes e os parâmetros da parte aérea não foram afetados. Como conclusão, a aplicação de Biozyme TS® e de Acadian® às sementes, até as concentrações de 6 mL.L-1, aumenta a matéria seca da parte aérea de plântulas de maracujazeiro-amarelo. A aplicação via foliar aumenta a quantidade de raízes grossas, e nas concentrações de 3,15 mL.L-1 promove o maior teor relativo de clorofila nas folhas

Renal function and volume of infants born with a very low birth-weight: a preliminary cross-sectional study.

AIM: The aim of our study was to compare the function and volumes of kidneys of very low birth-weight (VLBW) and of extremely low birth-weight (ELBW) infants at pre-school ages.PATIENTS AND METHODS: We did a revision of the neonatal records of infants born in our hospital that weighed &lt; or =1500 g at birth. The children were divided into two groups according to their weight at birth: ELBW (&lt;1000 g) and VLBW (1000-1500 g). At the age of 5.7 +/- 1.4 years, the children underwent clinical, laboratory and ultrasound renal assessments.RESULTS: Sixty-nine children fulfilled the requirements for the study. The rate of neonatal treatment with aminoglycosides was higher in ELBW preterms. Renal function parameters, i.e. estimated glomerular filtration rate and albuminuria, did not differ between the two groups of children. Urinary alpha1-microglobulin excretion was significantly higher and kidneys were significantly smaller in the ELBW group than in the VLBW group.CONCLUSION: No impairment or differences in renal parameters were found in pre-school children born ELBW compared with those born with VLBW, except for differences in kidney volume, renal cortical thickness and urinary alpha1-microglobulin excretion. Thus, patients born with ELBW would require a longer follow-up period

A complex pheotype in a girl with a novel heterozygous missense variant (p.Ile56Phe) of the GNAS gene

Background: GNAS is a complex gene that encodes Gsα, a signaling protein that triggers a complex network of pathways. Heterozygous inactivating mutations in Gsα-coding GNAS exons cause hormonal resistance; on the contrary, activating mutations in Gsα result in constitutive cAMP stimulation. Recent research has described a clinical condition characterized by both gain and loss of Gsα function, due to a heterozygous de novo variant of the maternal GNAS allele. Patients and methods: We describe a girl with a complex combination of clinical signs and a new heterozygous GNAS variant. For the molecular analysis of GNAS gene, DNA samples of the proband and her parents were extracted from their peripheral blood samples. In silico analysis was performed to predict the possible in vivo effect of the detected novel genetic variant. The activity of Gsα protein was in vitro analyzed from samples of erythrocyte membranes, recovered from heparinized blood samples. Results: We found a new heterozygous missense c.166A &gt; T-(p.Ile56Phe) GNAS variant in exon 2, inherited from the mother that determined a reduced activity of 50% of Gsα protein function. The analysis of her parents showed a 20-25% reduction in Gsα protein activity in the mother and a normal function in the father. Clinically our patient presented a multisystemic disorder characterized by hyponatremia compatible with a nephrogenic syndrome of inappropriate antidiuresis, subclinical hyperthyroidism, subclinical hypercortisolism, precocious thelarche and pubarche and congenital bone abnormalities. Conclusions: This is the first time that the new variant c.166A &gt; T (p.Ile56Phe) on exon 2 of GNAS gene, originated on maternal allele, has been described as probable cause of a multisystemic disorder. Although the mutation is associated with a reduced activity of the function of Gsα protein, this unusual phenotype on the contrary suggests a mild functional gain

Prenatal diagnosis of a rare form of congenital mid-ureteral stricture: a case report and literature revisited

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Therapy for children with henoch-schonlein purpura nephritis: a systematic review

Although severe kidney involvement in children with Henoch-Shonlein purpura (HSP) is rarer than that in adults, morbidity should not be underevaluated and follow-up is mandatory. Some drugs are introduced as well-defined treatment options, others can be promising therapeutic alternatives. Therapy of HSP nephritis in children can range from simply steroids to combined immunosuppressant treatments. The prophylactic treatment for renal complication of patients with HSP has been sometimes suggested, but with conflicting results and ultimately not clearly proven. The treatment of overt HSP nephritis includes steroids and other immunosuppressant drugs. Methylprednisolone pulse therapy and prednisone per os are tested drugs. These steroids could be used in combination with other immunosuppressant drugs, such as cyclosporin A and cyclophosphamide. Unfortunately, of these two drugs, only cyclophosphamide is demonstrated as effective in a recent randomized controlled trial. However, since there are insufficient data and unstructured study designs, ACE-I, azathioprine, mycophenolate mofetil, and urokinase need to be more tested in childhood HSP nephritis. In addition to drugs, other techniques are used to treat the severe form of nephritis. Of these, in a multicenter study, plasmapheresis demonstrated efficacy in delaying the progression of kidney disease. However, no convincing studies have been made to date concerning either intravenous immunoglobulin, factor XIII administration, antioxidant vitamin E, and fish oil to treat HSP nephritis

Therapy for Children with Henoch-Schonlein Purpura Nephritis: A Systematic Review

Although severe kidney involvement in children with Henoch-Shonlein purpura (HSP) is rarer than that in adults, morbidity should not be underevaluated and follow-up is mandatory. Some drugs are introduced as well-defined treatment options, others can be promising therapeutic alternatives