Publications by authors named "Cavagnaro F"

Article Synopsis
  • * A 3-month-old girl with suspected milk protein allergy showed signs of medullary NC during an ultrasound, and follow-up imaging at 18 months indicated progression of the condition along with genetic analysis revealing PKHD1 variants.
  • * While kidney function remained normal until age 7, the increasing incidence of NC in newborns emphasizes the need for early diagnosis and careful clinical monitoring to prevent complications such as kidney failure.
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Nuclear factor kappa-B subunit 2 (NF-κB2/p100/p52), encoded by (MIM: 164012) belongs to the NF-κB family of transcription factors that play a critical role in inflammation, immunity, cell proliferation, differentiation and survival. Heterozygous C-terminal mutations in have been associated with early-onset common variable immunodeficiency (CVID), central adrenal insufficiency and ectodermal dysplasia. Only two previously reported cases have documented decreased natural killer (NK) cell cytotoxicity, and little is known about the role of NF-κB2 in NK cell maturation and function.

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Unlabelled: Henoch-Schönlein purpura (HSP) or IgA Vasculitis is the most common childhood vasculitis. The classic tetrad of signs and symptoms include palpable purpura, arthralgia, abdominal pain and renal disease. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered.

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Pyomyositis (PM) is an uncommon pyogenic infection of skeletal muscle and, when not properly treated, it can progress to a high-risk clinical situation with high mortality. Because it usually has a subacute presentation, diagnosis is often delayed. We present two cases of PM of the paraspinal muscles in healthy children and discuss the current state of knowledge of this disease.

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Urinary tract infection is a frequent event in children. The correct diagnosis, management and study has been involved in major controversies in the last decade as a result of better design of scientific studies and evidence-based medicine in this area. A significant amount of our knowledge must be subjected to critical analysis because of new information available.

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Extracorporeal membrane oxygenation (ECMO) is a therapy that ensures adequate tissue oxygen delivery in patients suffering cardiac and/or respiratory failure that are unresponsive to conventional therapy. During ECMO, it is common to see a decrease in urine output that may be associated with acute renal failure. In this context, continuous renal replacement therapy (CRRT) should be considered.

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Isolated preauricular tags (IPT) are considered minor malformations whereas nephrourological anomalies (NUA) are considered major malformations. Their incidences fluctuate between 5 and 10 per 1,000 and 1-3 per 100 live births, respectively. There is contradictory evidence regarding the incidence of NUA in infants with IPT.

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Background: Hemolytic uremic syndrome (HUS) is one of the main causes of acute renal failure in the Chilean pediatric population.

Aim: To report the features of patients with HUS, admitted to the pediatric ward of a clinical hospital.

Material And Methods: Retrospective review of medical records of patients admitted with the diagnosis of HUS between 1995 and 2002.

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Amebiasis secondary to Entamoeba histolytica (E. histolytica) continues to be a major source of morbidity and mortality worldwide, mainly in developing countries. An association between amebic dysentery and hemolytic uremic syndrome (HUS) has been mentioned, but a cause-and-effect relationship has never been confirmed.

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The urinary tract infection (UTI) is one of the major causes of bacterial infection in children. Therefore an opportune diagnosis, an adequate treatment and a close follow up will prevent chronic renal damage. In this brief article UTI clinical characteristics and recommendations for diagnosis and treatment according to children age are reviewed.

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Background: Congenital hepatic fibrosis (CHF) is an autosomic dominant disease that has been associated with polycystic kidney disease.

Aim: To describe the medical management of 5 children with CHF and to evaluate the presence and extension of the associated renal disease.

Patients And Methods: Retrospective review of the medical charts of 5 children with CHF, aged 2 to 14 years.

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Renovascular hypertension (RVH) is responsible for 10% of arterial hypertension in children. The early diagnosis of RVH permits specific treatment leading to the cure of hypertension and avoidance of parenchymal damage. Captopril renal scintigraphy (CRS) provides information on the renovascular cause of the arterial hypertension.

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Background: The early and accurate diagnosis of obstructive uropathy in the newborn, prevents secondary complications and kidney damage.

Aim: To study the usefulness of Tc99M MAG3 diuretic renogram in newborns with hydronephrosis.

Material And Methods: Forty newborns, aged 1 to 30 days, with hydronephrosis, were studied.

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Background: In children, urinary tract infection (UTI) is a very common disease, and can cause permanent kidney damage.

Aim: To determine risk factors for permanent kidney damage, in children with UTI.

Patients And Methods: In 337 children with UTI (237 female, mean age 4.

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Background: Close to one half of patients with hemolytic uremic syndrome (HUS) will require a dialytic therapy, mainly peritoneal dialysis (PD). In some cases, PD may have relative or absolute contraindications, usually when HUS is associated to severe intraabdominal complications.

Aim: To report the results of continuous hemofiltration use, in children with abdominal complications of HUS.

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Anti neutrophil cytoplasmic antibodies are associated to vasculitis and crescentic glomerulonephritis in adults. However, this association has been seldom reported in children. We report two girls aged 12 and 15 years old with ANCA + glomerulonephritis.

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Idiopathic acute renal failure (IARF) is an uncommon but severe complication in children with relapsing nephrotic syndrome and may require long-term dialytic support until recovery of renal function takes place. Due to limited understanding of the pathophysiology of IARF, specific guidelines for its prevention and therapy have not been developed. Among triggering factors, peritonitis was present in half of all pediatric patients with this complication described in the English literature over the past 15 years.

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Unlabelled: 99mTc-DMSA late static planar imaging or SPECT is being used for the investigation of focal acute pyelonephritis (APN), especially in children with urinary tract infection (UTI). Diuretic 99mTc-mercaptoacetyltriglycine (MAG3) dynamic scintirenography has been applied in the evaluation of kidney function and structure, frequently to exclude obstruction. However, in children and adults with a clinical picture of APN, diuretic MAG3 scintigraphy with zero time injection of furosemide (MAG3-F0) was observed to display focal parenchymal abnormalities; regional dysfunction (focal parenchymal decrease in early uptake; slow filling in and prolonged late retention of activity); or, less frequently, fixed defects.

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A 2-year-old boy had a severe cytomegalovirus (CMV) infection with multi-organ involvement, while on maintenance therapy for acute lymphoblastic leukemia. The patient was treated with intravenous gancyclovir, with a marked improvement in his clinical status, with the exception of a progressive deterioration of the renal function. He also developed hemolytic anemia and thrombocytopenia, suggesting a diagnosis of atypical hemolytic uremic syndrome (HUS).

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The Nephrology Branch of the Chilean Pediatric Society has greatly influenced the development of government health plans regarding the management and care of patients under 18 years with chronic renal failure (CRF). In order to assess the status of children with CRF in Chile up to 1996, a questionnaire was sent to all pediatric nephrologists in charge of those children. The total sample was of 227 patients under 18 years, giving a national prevalence of 42.

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Infection of the upper respiratory tract is a major cause of morbidity and mortality in patients with lymphoproliferative syndromes and multiple myeloma. Nebulizations with IgA tested in a randomized double blind trial to evaluate its efficacy to prevent respiratory infections in patients with lymphoproliferative syndromes and multiple myeloma. Forty nine patients were evaluated (chronic lymphocytic leukaemia, 22; multiple myeloma, 11; lymphoma, 8; HCL, 6; Waldenström and lymphoepiteliod tymoma, 1 patient each) were randomized to receive nebulizations every 12 hours during 3 months of IgA or placebo.

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