Biomedical subjects

Bruno Ranchin

Publications and source records attributed to Bruno Ranchin.

11 recordsLinked to original sources

PubMed2008

Osteosarcoma metastatic to the kidney and iatrogenic hemorrhage.

A 16-year old female presented painful masses in the lumbar region 5 years after the initial diagnosis of a localized osteosarcoma of the tibia. Abdominal X-ray revealed calcified masses. A bone scan confirmed an increased uptake in the renal areas. An ultrasound-guided fine needle biopsy confirmed the diagnosis of metastases. The procedure was complicated by subcapsular hemorrhage and gross hematuria. Renal metastases from osteosarcoma are usually asymptomatic and invisible on abdominal X-rays. The diagnosis is generally established by radionuclide bone scan or abdominal CT-scan. Our observation suggests that histological documentation of these unusual clinical presentations should be carefully weighed against the risk of the procedure.

Amputation, Surgical↗

PubMed2006

Hypertensive crisis, hepatitis B virus and polyarteritis nodosa in a child.

We report on a case of hepatitis B virus-related polyarteritis nodosa (PAN) in a 4-year-old Turkish boy who was first admitted because of severe arterial hypertension. The diagnosis of PAN was provided by conventional renal arteriography and the child was successfully treated with intensive sequential therapy combining short-term prednisone, plasma exchange and interferon-alpha-2b. Nine years later, he had no sign of PAN, normal blood pressure and normal renal function in the absence of any treatment.

Angiography↗

PubMed2006

Primary hyperoxaluria type 1: still challenging!

Primary hyperoxaluria type 1, the most common form of primary hyperoxaluria, is an autosomal recessive disorder caused by a deficiency of the liver-specific enzyme alanine: glyoxylate aminotransferase (AGT). This results in increased synthesis and subsequent urinary excretion of the metabolic end product oxalate and the deposition of insoluble calcium oxalate in the kidney and urinary tract. As glomerular filtration rate (GFR) decreases due to progressive renal involvement, oxalate accumulates and results in systemic oxalosis. Diagnosis is still often delayed. It may be established on the basis of clinical and sonographic findings, urinary oxalate +/- glycolate assessment, DNA analysis and, sometimes, direct AGT activity measurement in liver biopsy tissue. The initiation of conservative measures, based on hydration, citrate and/or phosphate, and pyridoxine, in responsive cases at an early stage to minimize oxalate crystal formation will help to maintain renal function in compliant subjects. Patients with established urolithiasis may benefit from extracorporeal shock-wave lithotripsy and/or JJ stent insertion. Correction of the enzyme defect by liver transplantation should be planned, before systemic oxalosis develops, to optimize outcomes and may be either sequential (biochemical benefit) or simultaneous (immunological benefit) liver-kidney transplantation, depending on facilities and access to cadaveric or living donors. Aggressive dialysis therapies are required to avoid progressive oxalate deposition in established end-stage renal disease (ESRD), and minimization of the time on dialysis will improve both the patient's quality of life and survival.

Child↗

PubMed2006

Prognosis of lupus membranous nephropathy in children.

The occurrence of membranous nephropathy in pediatric series of systemic lupus erythematosus has been reported only rarely, probably due to a very low frequency. One hundred fifty-four children who were seen in 100 French pediatric centers between January 2002 and April 2005 were included. Fifteen (12 girls and three boys) out of the 81 (18.5 %) children with renal involvement presented histological features of membranous nephropathy. Their ages ranged from six to 15 years old (mean=11.3) at the age of SLE diagnosis and 8/15 children were of African origin. Isolated membranous nephropathy was observed in nine patients, of whom five patients displayed a complete recovery following immunosuppressive treatment. Associated proliferative lesions were observed on the first kidney specimen in two patients and in a further renal biopsy in four other patients, leading to a less favorable course of lupus nephropathy.

Adolescent↗

PubMed2006

Four-year follow-up of oral health surveillance in renal transplant children.

To outline the specific oral surveillance needs of renal transplant children, we report the 4-year follow-up data of 106 children examined routinely on a 6-month basis and upon request from the medical team or the parents in the interval. Data were recorded for hard and soft dental tissues, including enamel defects carious lesions, gingival status, orthodontic treatment needs, and wisdom teeth eruption. Hard-tissue lesions were noted in 34.9% of the children, caries lesions on the permanent teeth were seen in 15.0% of cases, 18.86% of the patients had orthodontic treatment. Spontaneous examination, upon request for medical reasons, was given for 26 children. Twenty-one demands originated from the parents. Over a 4-year period the attendance of a dental specialist resulted in a significant improvement in the oral health of the renal transplant children.

Adolescent↗

PubMed2005

Neonatal hemolytic uremic syndrome after mother-to-child transmission of Escherichia coli O157.

About 90% of cases of hemorrhagic uremic syndrome (HUS) occur in early childhood and most frequently are preceded by bloody diarrhea due to shiga-like toxin (SLT) producing Escherichia coli. We report a case of a newborn girl presenting with bloody diarrhea on her 7th day of life. Acute renal failure, severe arterial hypertension and hemolytic anemia were detected and prompt peritoneal dialysis and antihypertensive therapy were required. The girl had several episodes of seizures, necessitating intravenous phenobarbital. Transfontanel ultrasonography 48 h after disease onset was normal, whereas, MRI investigation 10 days later revealed severe ischemic lesions with beginning cystic encephalopathy. Renal function recovered and only very moderate tubular dysfunction remained. Serum analysis of factor H, von Willebrand factor protease, homocystinemia, proteins C and S, and antithrombin III were all normal. Mutation analysis of factor V Leiden, factor II, and methyltetrahydrofolate-reductase were normal. E. coli 0157:H7 and SLT 2 were detected in the stool. SLT 2 was also found in the mother's stool. This is the first report of mother-to-child transmission of SLT-producing E. coli.

Escherichia coli Infections↗

PubMed2005

Triamcinolone acetonide: a new management of noncompliance in nephrotic children.

Noncompliance is frequent in children and adolescents with nephrotic syndrome. Once suspected, noncompliance is difficult to confirm and often impossible to avoid. The standard oral glucocorticoid treatment for children has been shown to be efficient and safe. However, a small number of children/parents are noncompliant to the steroid treatment, resulting in multiple relapses. For these patients the use of steroids with prolonged half-life such as triamcinolone acetonide (TA) can be helpful. We studied seven children (six boys, one girl; median age at diagnosis 8.6 years, range 1.8-10.7) receiving conventional steroid treatment for a median of 30 months (8-74) before starting intramuscular (IM) TA treatment. The standard prednisone treatment was replaced by 1 monthly IM injection of TA (1 mg/kg per day oral prednisone replaced by 1 mg/kg per month IM TA). The treatment was tapered off by a reduction of 10-20% of the initial dose per month over 6-8 months. After a mean observation period of 14 months (3-36) the results were evaluated in terms of number of relapses and treatment tolerance. Four children showed a clear decrease in number of relapses (1.8 to 0 per year); in the other three the number of relapses remained stable. Tolerance was excellent (no cataract, no arterial hypertension), and the cushingoid syndrome did not exceed the level experienced under conventional oral steroid therapy. However, growth velocity decreased during the TA treatment and returned to normal after discontinuation of TA. These preliminary results demonstrate that TA may be used in patients of suspected noncompliance in steroid-sensitive patients who respond with a complete remission during TA treatment over the observation period. Patients who do not benefit from the TA can be classified as very probably steroid-dependent. TA seems to be a useful therapeutic strategy in those patients for whom noncompliance is strongly suspected.

Child↗

PubMed2005

Switch from cyclosporine A to mycophenolate mofetil in nephrotic children.

Nephrotoxicity is a well-known adverse effect of cyclosporine A (CyA) treatment in children with steroid-dependent (SD) and steroid-resistant (SR) nephrotic syndrome (NS). We analyzed nine children (age: 3.3-15.7 years, two girls) with SD or SR NS who experienced a significant decrease in their GFR under CyA treatment as measured by inulin clearance (C(IN)). Mycophenolate mofetil (MMF) was introduced progressively until doses of 1 g/1.73 m(2) twice daily were reached. CyA treatment was stopped after introduction of MMF and oral steroids were reduced if possible. After a median follow up of 261 days, no adverse effects of MMF such as diarrhea or hematological anomalies occurred in our patients. After switching from CyA to MMF, those children with SD NS remained in remission without proteinuria and those with SR NS did not show any significant changes in their residual proteinuria. The serum protein level did not change significantly in any of the children analyzed. GFR increased from a mean of 76.9+/-4.8 to 119.9+/-5.9 mL/1.73 m(2) per min (P<0.001). Oral steroid treatment could be reduced from a median [range] prednisone dose of 0.85 [0.26-2.94] mg/kg/d pre-MMF to 0.29 [0-1.1] mg/kg per day (P=0.026), and blood pressure decreased moderately after CyA withdrawal, but the difference did not reach statistical significance. We conclude that a switch from CyA to MMF seems to be safe for children with SDNS and SRNS in terms of side effects as well as disease control, at least in the short term. Interruption of CyA treatment lead to rapid amelioration of kidney function in these children, often associated with steroid sparing, which may lead to additional benefit for growth velocity, blood pressure and physical appearance.

Adolescent↗

PubMed2005

[Renal transplantation techniques in children with impaired patency of the inferior vena cava or iliac vein].

INTRODUCTION: Congenital or acquired disorders of patency of the inferior vena cava (IVC) or iliac veins have been considered for a long time to be an almost insurmountable technical obstacle in paediatric renal transplantation. The authors report their experience based on 7 transplantations performed in children with a disorder of venous patency, in whom renal transplantation was able to be performed. More generally, they also discuss the available technical options based on a review of the literature. MATERIAL AND METHOD: From April 1987 to January 2002, renal transplantation was performed in 7 children with congenital or acquired abnormality of the iliac veins or IVC in our unit. All children underwent radiological assessment by venous Doppler ultrasound before transplantation. The contribution of radiological examinations to the diagnosis, the surgical technique performed, postoperative complications, patient survival and graft survival as well as long-term renal function were studied retrospectively. RESULTS: Four of the 7 transplanted children presented a congenital anomaly of the IVC and 3 had acquired thrombosis. In 5 out of 7 cases, the diagnosis was established before transplantation by Doppler ultrasound (completed by cavography in one case). The transplant renal vein was anastomosed to the iliocaval system in the majority of cases and the portal system had to be used in one case. With a mean follow-up of 94 months, all children have a functioning graft with a mean serum creatinine of 88 micromol/l. CONCLUSIONS: Disorders of patency of the IVC or iliac veins in children waiting for renal transplantation are rare, but these abnormalities can cause problems for the surgical team and compromise graft survival. Radiological assessment of patency of the venous network must help to demonstrated these abnormalities in order to optimally adapt the transplantation technique, which ensures very acceptable graft survival rates.

Adolescent↗

PubMed2002

Chronic parvovirus B19 infection in a pediatric lung transplanted patient.

In immunocompromised patients, clinical manifestations of human parvovirus B19 (PVB19) infection are mostly reported as acute or chronic hematological disorders. Recently, PVB19 infection has been associated with nonhematological symptoms. Four years after lung transplantation, a 9-year-old girl developed a severe anemia with reticulocytopenia requiring blood transfusion. PVB19 DNA was found by polymerase chain reaction in blood. Blood marrow aspiration revealed typical features of PVB19 infection. She was successfully treated with high dose of i.v. Ig. Then, she exhibited recurrent nonregenerative anemia requiring another course of i.v. Ig. PVB19 DNA has been persisted in blood with no specific immune response. At the same time, she suffered from several lung infection syndromes with no microorganism found except PVB19 DNA. Recurrent mild renal dysfunction was noticed with no other explanation than PVB19 infection. This report indicates that pediatric transplanted patients are at risk of chronic PVB19 infection, which can be associated with lung and/or renal disorders.

Adult↗

PubMed2002

Transient hyperphosphatasemia after organ transplantation in children.

Transient, isolated hyperphosphatasemia is a rare, benign condition of childhood. Few cases have been described in transplant patients. We report six cases: three after liver transplantation and three after kidney transplantation. Such a phenomenon was found to be as benign after organ transplantation as it is in healthy children. Hence, an isolated increase in the serum alkaline phosphatase level following transplantation should not be of concern in this population of patients.

Alkaline Phosphatase↗