Vitamin K status in cystic fibrosis

Appearance of PIVKA-II (protein induced by vitamin K absence-II) in serum is a biochemical sign of insufficient vitamin K-dependent carboxylation of prothrombin. Plasma concentrations of PIVKA-II and vitamin K1 were determined in 24 children with cystic fibrosis. Eight were supplemented with vitamin K1. The purpose of the study was to determine the occurrence of vitamin K deficiency in cystic fibrosis and to evaluate the effect of vitamin K supplementation. PIVKA-II was detectable in only one unsupplemented child. In this patient, the concentration of vitamin K1 was below the limit of detection of 60 ng/l. Vitamin K1 levels in the other unsupplemented children were normal (mean 476 ng/l = 1 mmol/l). The supplemented patients showed extremely high levels of vitamin K1 (mean 22445 ng/l = 50 nmol/l). In conclusion, vitamin K deficiency occurs infrequently in cystic fibrosis. Checking the coagulation system is advised, but routine vitamin K supplementation is not recommended. If additional vitamin K is needed, the starting dose should not exceed 1 mg daily.     

Angiotensin-converting enzyme genotype and outcome in pediatric IgA nephropathy

Angiotensin-converting enzyme (ACE) I/D polymorphism has been implicated as a genetic marker for progression of glomerular disease. Studies of ACE genotypes in adults with IgA nephropathy (IgAN) have yielded conflicting results. We performed ACE genotyping on 79 patients with IgAN diagnosed prior to age 18 years who had either progressed to end-stage renal disease (ESRD) or are now more than 5 years post biopsy. Mean follow-up was 14.8 years for those with normal renal function. Forty-three (54.4%) subjects had normal renal function and a normal urinalysis at last evaluation. Sixteen (20%) progressed to ESRD and 1 has chronic renal insufficiency. Kaplan-Meier survival curves for progression to ESRD did not differ significantly for the ACE DD, ID, and II genotype groups (P=0.095, log-rank test). By univariate analysis, presence of hypertension and degree of proteinuria at diagnosis, and unfavorable histology but not ACE genotype, was significantly associated with progression to ESRD. In the Cox proportional hazards model that included grade of proteinuria, the ACE D allele was a significant independent predictor of outcome with a hazard ratio of 2.37 (P=0.031). Our data, while inconclusive, suggest that the ACE D allele may associate with poor outcome in pediatric IgAN.     

Hemodynamic stability during 17 years of the Carpentier-Edwards aortic pericardial bioprosthesis

BACKGROUND: Long-term stability of the hemodynamic performance of commercially available Carpentier-Edwards stented bovine pericardial aortic bioprostheses (Perimount RSR) is unknown. To anticipate the fate of this bioprosthesis, we examined its hemodynamic performance up to 17 years using echocardiographic studies in a Premarket Approval cohort. METHODS: Of 267 patients at four institutions in the Premarket Approval cohort, 85 had a total of 168 echocardiographic studies during a 17-year period of yearly follow-up examinations. These were reviewed and quantified in a core echocardiographic facility. Longitudinal data analysis was used to account for repeated, censored data. RESULTS: Mean transvalvular gradient was inversely related to prosthesis size (p = 0.01), and possibly (p = 0.06) increased somewhat during the first 10 years of follow-up, then stabilized. Effective orifice area was larger in larger valve sizes (p = 0.01), declined somewhat during the first 10 years, and then began to increase again. Ejection fraction declined minimally (p = 0.2). In contrast to the rather stable hemodynamics, aortic regurgitation steadily increased from none to 1 to 2+ (p = 0.005), but rarely (< 10% at 17 years) progressed to 3+ or 4+. CONCLUSIONS: The Carpentier-Edwards aortic pericardial bioprosthesis can be anticipated to have an acceptable long-term transvalvular gradient and effective orifice size that will change trivially up to 17 years after implantation. Mild aortic regurgitation will develop progressively. This anticipated hemodynamic resilience supports continued clinical use of the Perimount Carpentier-Edwards bovine pericardial stented bioprosthesis.     

Endocarditis after mitral valve repair

Background. Native valve endocarditis is frequently managed with antibiotics alone, but prosthetic valve endocarditis usually requires an early operation. What is the best treatment of endocarditis after mitral valve repair?

Methods. From 1986 to 2000, 22 patients were treated for endocarditis affecting a previously repaired mitral valve. Causes of mitral valve dysfunction that led to repair were degenerative (11 patients), ischemic (5 patients), endocarditic (3 patients), rheumatic (2 patients), and functional (1 patient). Endocarditis was active in 21 patients and healed in 1. Interval from initial mitral valve repair to onset of endocarditis ranged from 1 week to 10.3 years (median, 6 months). Pathology included leaflet vegetation (15), annuloplasty vegetation (4), leaflet perforation (5), and abscess (3). Mean follow-up was 3.9 ± 3.3 years.

Results. Fifteen patients underwent repeat mitral valve operations with freedom from mitral valve reoperation of 65%, 41%, and 26% at 30 days, 1 year, and 5 years after onset of endocarditis. After a high early hazard, risk of reoperation fell to 10.8% per year. Seven patients, all with a leaflet vegetation, were treated with antibiotics alone. Antibiotics eradicated infection in all; however all had mitral regurgitation 2+ to 4+. Survival was 96%, 74%, and 68% at 30 days, 1 year, and 5 years. Endocarditis recurred in 1 patient (92% free of event).

Conclusions. Most patients that have endocarditis develop after mitral valve repair require reoperation. However if infection is limited to a leaflet, early reoperation may be unnecessary because antibiotics alone can eradicate infection.     

Microwave ablation of atrial fibrillation during mitral valve operations

Although the Cox-Maze III procedure cures atrial fibrillation in the majority of patients, it has not had widespread application. Development of new operations that use alternate energy sources and different lesion sets have caused resurgence in the surgical treatment of atrial fibrillation. Microwave creates lines of conduction block by thermal damage and subsequent scar formation. We describe a rapid and simple technique for microwave ablation of atrial fibrillation in patients having mitral valve operations.     

Reoperative cryopreserved root and ascending aorta replacement for acute aortic prosthetic valve endocarditis

BACKGROUND: Prosthetic aortic valve endocarditis (PVE) is an important complication of aortic valve replacement (AVR) and is a particularly difficult situation after an operation combining AVR with ascending aortic replacement. METHODS: From 1988 through 2000, 27 patients with aortic valve PVE after previous ascending aortic replacement (aortic root replacement in 13, aortic valve replacement with a supracoronary graft in 14) underwent reoperation for aortic root replacement with a cryopreserved aortic allograft and prolonged intravenous antibiotic therapy. All patients were considered to have active PVE (25 with positive cultures); root abscess formation was present in 89% and aortoventricular discontinuity in 41%. RESULTS: One patient (3.7%) died in-hospital, and permanent pacemakers were required in 10 patients (37%). Mean postoperative follow-up interval was 3.9 +/- 3.0 years, and survival at 1, 2, 5, and 7.5 years was 92%, 88%, 70%, and 56%, respectively. One patient underwent reoperation for recurrent PVE 8 months after operation. CONCLUSIONS: Radical debridement of infected prosthetic material and tissue, and allograft aortic root and ascending aorta replacement, combined with intravenous antibiotic therapy, appears to achieve a low hospital mortality and a high degree of freedom from recurrent infection for patients with PVE after AVR and ascending aortic replacement.     

Biodistribution of ultrasmall iron oxide particles in the rat liver

Ferumoxtran, an ultrasmall superparamagnetic iron oxide particle, can be located in several tissue compartments in the liver, namely the extracellular space (blood and interstitium), reticuloendothelial cells, and possibly hepatocytes. To better understand the compartmental distribution of ferumoxtran in the liver, we performed a longitudinal study in the rat using microscopy and magnetic resonance imaging. At light microscopy, no substantial cellular uptake of ferumoxtran was observed before one hour after injection. With a dose of 15 micromol Fe/kg, the number of ferumoxtran particles in the reticuloendothelial cells peaked between one and four hours and with a 150 micromol Fe/kg dose, it peaked between eight and 24 hours. Within hepatocytes, only sparse particles were observed with electron microscopy, at a dose of 150 micromol Fe/kg. Imaging performed up until one hour after ferumoxtran injection showed a significant increase in liver signal intensity on T1-weighted images. These results suggest that ferumoxtran mainly acts as an extracellular agent for at least one hour in the rat and that reticuloendothelial accumulation peaks at later time points. Substantial uptake within hepatocytes did not occur.     

Sexual precocity: sex incidence and aetiology

The aetiology of 197 girls and 16 boys presenting with sexual precocity was reviewed. Ninety one girls and four boys had central precocious puberty (M:F 23:1); a cause was identified in all the boys but in only six girls. All boys with precocious puberty need detailed investigation; in girls investigation should be based on clinical findings, particularly the consonance of puberty.     

Thymic cysts in mediastinal Hodgkin disease

Three cases of proved thymic cysts associated with mediastinal Hodgkin disease are presented. Two illustrate regression of lymphoma with chemotherapy but persistence of thymic cysts. The third case demonstrates a thymic cyst in untreated Hodgkin disease. These cases suggest that such cysts are probably neither coincidental with nor a consequence of therapy but are probably related to initial thymic involvement by Hodgkin disease.