Chronic renal failure and portal hypertension--is portosystemic shunt indicated?

We report two girls with histories of recessive polycystic kidney disease. Both were on maintenance hemodialysis. They had undergone surgical distal portocaval shunt because of portal hypertension. Later, bilateral nephrectomy was performed, and they presented with hepatic encephalopathy (HE) and evolution towards irreversible hepatic coma and death. Portosystemic shunt is the treatment of choice of portal hypertension. The kidney plays a pivotal role in ammonia disposal during portosystemic shunt. Thus, we stress the risk of HE after portosystemic shunt followed by bilateral nephrectomy in patients with end-stage renal failure and suggest that combined liver-kidney transplantation should be considered.

Kidney function in cyclosporine-treated paediatric pulmonary transplant recipients.

BACKGROUND: Lung or heart-lung transplantation is a useful therapy in life-threatening pulmonary disorders during childhood. Cyclosporine A is a major immunosuppressive treatment but has a number of adverse effects including nephrotoxicity. There have been no reports on the long-term evolution of renal function in a large series of paediatric pulmonary transplantation recipients. METHODS: We examined 19 patients followed up for at least 3 years after pulmonary transplantation. The mean time of follow-up was 5.36 years. Kidney function was evaluated by calculation of glomerular filtration rate (GFR) according the Schwartz formula. RESULTS: The GFR was normal before transplantation in all patients. The short-term evolution of GFR was marked by a significant drop during the first and until the 6th month. Then, regardless of the level reached at the end of the 6th month, the GFR remained stable in all patients except one until the end of follow-up. At the end of follow-up, 31% had normal GFR, 57% had mild chronic renal failure, and 5% had advanced renal failure. Hypertension was frequent and associated with renal failure. CONCLUSIONS: Paediatric pulmonary recipients showed evidence of long-term cyclosporine A-associated nephrotoxicity. Most of this toxicity occurred during the first 6 months.

[Iterative fractures in type I primary hyperoxaluria. Report of 2 cases]. / Fractures itératives au cours de l'hyperoxalurie primitive de type I. A propos de 2 cas.

PURPOSE OF THE STUDY: Type I primary hyperoxaluria is a rare autosomal recessive disease linked to a deficit in an hepatic enzyme. The purpose of this study was to analyze orthopedics problems caused by type I primary hyperoxaluria before and after liver and kidney transplantation. MATERIAL AND METHODS: Two cases of children carrying this type I primary hyperoxaluria followed up after liver kidney transplantation are presented and compared to last publications. RESULTS: Combined transplantation progressively corrected osseous lesions and aspect of the stroma. However it did not provide protection against fractures particularly for femoral neck fractures. DISCUSSION: In type I hyperoxaluria overproduction of calcium oxalate causes its accumulation in the whole organism and particularly in bone. Osseous fragility favors pathological fractures. Only combined liverkidney transplantation can save and cure these children. Frequency of this fracture after transplantation indicates preventive plating at first pain, possibly at the same time as transplantation. Kidney transplant failure puts the patient in a "congealed" clinical state where the bone is very rich in oxalate and where the hemodialysis does not eliminate oxalate salts. CONCLUSION: Type I primary hyperoxaluria is a very rare disease. Fractures are very common even after liver and kidney transplantation and especialy femoral neck fractures. We think that preventive plating must be done at first pain. We do not have any explanation for bony weakness after liver-kidney transplantation.

Crescentic glomerulonephritis in hyper IgD syndrome.

The hyperimmunoglobulinemia D syndrome (HIDS) is a well-defined entity resembling familial Mediterranean fever. HIDS is a systemic inflammatory disease associated with stimulation of T-cell-mediated immunity. These patients are at low risk for amyloidosis and are not known to develop nephropathy. We report a boy of Mediterranean ancestry who exhibited typical HIDS and end-stage renal failure. Kidney biopsy revealed pauci-immune crescentic glomerulonephritis (cGN). We hypothesized that the glomerular involvement was secondary to the cytokine network activation observed in HIDS. Thus, cGN should be considered as part of the syndrome, and kidney biopsy should be performed early in the course of the renal disease in patients with HIDS.

[Wilson disease, IgA glomerulonephritis and vascular purpura: an incidental association?]. / Maladie de Wilson, glomérulonéphrite à IgA et purpura vasculaire: une association fortuite?

BACKGROUND: IgA nephropathy may occur in adults with alcoholic cirrhosis. It has also been reported in children with alpha-1-antitrypsin deficiency. A case of IgA nephropathy associated with cirrhosis due to Wilson's disease is reported. CASE REPORT: A 10 year-old girl, was admitted for cirrhosis. She had suffered from ascitis and hematuria since the age of 6 years and vascular purpura since the age of 9 yr 6 mo. At admission, she had atrophic liver cirrhosis, ascitis splenomegaly and petechiae on her legs. There was microcytic anemia due to iron deficiency. The Coombs test was positive. There were hypoalbuminemia (2 g/dl) and polyclonal hyperglobulinemia (IgA 750 mg/dl, IgG 1670 mg/dl and IgM 250 mg/dl). Her serum concentrations of complement factors were normal and no immune complexes were detected in serum. She also showed biological changes due to kidney failure, hematuria and proteinuria. A liver biopsy showed micronodular cirrhosis with numerous plasma cells containing IgA. The search for an etiology showed ceruloplasmin levels of 5 mg/dl, elevated urinary copper excretion (> 150 micrograms/day) and above normal liver copper (255 micrograms/g dry weight); Kayser-Fleischer ring was also seen. The renal biopsy showed membranoproliferative glomerulonephritis, and immunofluorescent microscopy revealed C3 and IgA in the mesangial and subendothelial regions. The patient was given spironolactone and furosemide followed by triethylenetetramine dihydrochloride. CONCLUSIONS: The IgA nephropathy, vascular purpura and elevated serum IgA levels could be due to the liver changes. If so, they should be improved by a liver transplant, although our patient would be a better candidate for combined liver and kidney transplantation.

[Drash syndrome]. / Syndrome de Drash.

The authors report one case of Drash syndrome: association of Wilms' tumor, nephropathy and genital abnormalities. The definition, prognosis and origin of this triad are discussed.

[The role of dialysis in the treatment of terminal renal insufficiency in children]. / Place de la dialyse dans le traitement de l'insuffisance rénale terminale de l'enfant.

In order to analyze the place of dialysis in a hemodialysis/transplantation program, the duration of each treatment modality, mortality rate and quality of inclusion in the social network were studied. Complications which arose during hemodialysis were evaluated by comparing the 1970's and the 1980's. Sixty children with terminal renal failure, aged 3 to 15 years, were entered in a hemodialysis/transplantation program between May 1971 and December 1988. Patients were followed up until December 1989. Among the 47 (78%) survivors at the end of the follow-up period, 25 had a functioning renal transplant and 22 were undergoing dialysis. Among the 13 deaths, 7 occurred during renal transplantation or immediately after loss of the transplant and 6 occurred under dialysis. Mean duration of treatment, including both dialysis and transplantation, was 7 years 11 months. Mean time spent under dialysis was 4 years 9 months. Time spent with a functioning transplant was 3 years 10 months for the 46 transplant recipients. Mean time spent on the transplant waiting list fell from 3 years 6 months before 1980 to 2 years after 1980. Virtually no cases of renal osteodystrophy, acute arterial hypertension or hepatitis B were seen after 1980 as a result of the use of higher-potency vitamin D derivatives, recent antihypertensive drugs including ACE inhibitors, and the Hevac B vaccine. Similarly, safety and patient comfort during dialysis improved substantially, as well as the quality of rehabilitation. Growth remained a significant problem although improvements can be expected to occur in the near future. Hemodialysis is an indispensable complement to transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)

Case report: transthoracic retrograde venous bullet embolization.

The embolism of bullets in the venous system is an uncommon complication of penetrating missile injuries. Retrograde transthoracic venous bullet embolization is exceedingly rare. This report describes embolization of a small-caliber bullet from the left subclavian vein to a branch of the right popliteal vein. The patient was treated successfully without surgery.

The status of rehabilitation, morbidity, and mortality of long-term survivors of pediatric kidney transplants.

The long-term survivors of pediatric renal transplantation show, in our early experience, an excellent status of rehabilitation. The majority of the survivors are enjoying good health and lead active lives. Skin lesions constituted significant morbidity, requiring a better understanding and preventive strategy. Cardiovascular disease appearing in such a young age group is another disturbing problem. The problem of mental health is surprisingly small in these hardy survivors.

[Discordant evolution in monozygotic twins with familial nephropathy]. / Evolution discordante chez deux soeurs jumelles monozygotes atteintes de néphropathie familiale.

Two monozygotic twin sisters presented a familial nephropathy, discovered at 5 years of age. The clinical course was quite different for each patient: end-stage renal failure occurred at 5.5 years of age for one and at 14 years for the other. This difference raises the hypothesis of the role of environmental factors on the clinical expression of an inherited disease.

Nonpenetrating vascular injury to the subclavian artery.

Blunt subclavian artery injury has been uncommonly reported in the literature. Recent encounter with three such injuries prompted us to review our experience over the past 10 years uncovering only one additional case. These four cases and a review of pertinent literature form the basis for this article. Key clinical issues include a high index of suspicion in patients sustaining major blunt deceleration and rotational or direct injuries to the neck, thorax, and/or upper extremities. Prompt diagnosis remains obscured by the presence of severe associated injuries, the treatment of which requires prioritization. Arteriography is invaluable to elucidate injury because prompt vascular control is dictated by various approaches depending on the location. Expeditious surgical repair is indicated to prevent complications of hemorrhage, pseudoaneurysm, thromboembolism, and/or arteriovenous fistula. Long-term results appear to be good with major morbidity related to associated neurologic, soft tissue, and bony injuries.

Use of the Greenfield filter in adolescents for deep vein thrombosis and pulmonary embolism.

Serious venous thromboembolic disease is now recognized more frequently in the pediatric age group. Caval interruption is indicated most commonly for prophylaxis against life-threatening or recurrent pulmonary embolism (PE) when anticoagulation is ineffective or contraindicated. Greenfield vena caval filters have been utilized locally in 415 adult patients with 97% long-term patency and 5% recurrent embolization. Its application in adolescents is reported herein. Standard adult (30-mm) vena caval filters were placed in ten patients, ages 13 to 18. Four filters were required following PE, six were used for deep venous thrombosis (DVT) when anticoagulation was contraindicated, and one was inserted prophylactically. In eight patients, filter insertion was accomplished with local anesthesia, while two underwent filter placement under general anesthesia administered for other procedures. One filter was misplaced into the right renal vein, requiring a second filter insertion. All patients have been followed from 1 to 11 years with yearly vascular duplex imaging and radionuclide venograms documenting caval patency without clinical embolic sequelae. This duplicates the adult experience in safety and efficacy. As recognition of venous thromboembolism becomes more frequent in the pediatric age group, safe caval interruption may be necessary for those excluded from or not responsive to anticoagulation. This technique may be extended to smaller patients with miniaturization of both filter and carrier.