Congenital acute myelogenous leukemia presenting as palpable renal masses in a neonate

Our report concerns a neonate who was noted to have palpable kidneys soon after birth. Her clinical and radiologic findings were compatible with the diagnosis of autosomal recessive polycystic kidney disease. The patient eventually died of sepsis-related multiorgan failure, and on autopsy, surprisingly, was found to have leukemic infiltrates in the kidney. Healthcare providers need to be aware that congenital acute myelogenous leukemia, although rare, can present in the newborn period with nephromegaly.     

Pilot study describing the use of pravastatin in pediatric renal transplant recipients

Renal transplant (Tx) recipients frequently develop hypercholesterolemia. Pravastatin (P) has been shown to be effective in adult renal Tx recipients, not only in reducing serum cholesterol, but possibly also in decreasing graft rejection. However, there are no data on the use of P in children following renal transplantation. We conducted a retrospective case-control study evaluating the safety and efficacy of P (10-20 mg/day) in reducing hypercholesterolemia, when used pre-emptively in the post-Tx period in seven children, compared with an historical control (C) group of nine children who had not received P. The two groups were comparable with respect to their demographics and in their pretransplant serum cholesterol. Compared with the C group, the mean serum cholesterol in the P group was lower at 3 months (159 mg/dL vs. 225 mg/dL), 6 months (134 mg/dL vs. 200 mg/dL), 9 months (134 mg/dL vs. 209 mg/dL), and 12 months (125 mg/dL vs. 195 mg/dL) (p < 0.005 for all, Student's two-tailed t-test). At 1 month only 43% of the P group had hypercholesterolemia compared with 67% of the controls; by 12 months this difference was even more significant (0% in the P group vs. 45% in the C group). None of the treated patients developed any adverse reactions. This study demonstrates that the pre-emptive use of P in pediatric renal Tx recipients appears to be effective in significantly reducing serum cholesterol. Whether this effect will translate into improved allograft and patient survival in the long term cannot be predicted at present and will require additional studies to evaluate.     

Life-threatening extrarenal lupus in children despite improvement in serologic findings

Systemic lupus erythematosus (SLE) is an autoimmune disorder with the potential for multiorgan involvement. Serologic tests are helpful in establishing the diagnosis of SLE and predicting disease flares. However, there are few data on the relationship between the onset of new organ involvement and lupus serologies, especially in children. This report details our experience in managing two children with lupus nephritis. Both developed life-threatening extrarenal complications (cerebritis and carditis) soon after receiving high-dose immunosuppressive therapy and despite normalizing serologies. This lack of concordance between serologies and the development of carditis and cerebritis needs to be recognized so that health care professionals treating children with SLE can promptly intensify immunosuppressive medications and avoid life-threatening delays from seeking alternative explanations for symptomatology.     

Use of the intubating laryngeal mask airway in a case of ankylosing spondylitis for coronary artery bypass grafting

The intubating laryngeal mask airway is a modification of the conventional laryngeal mask and is used as a tool for difficult intubation. It can be inserted without placing the head and neck in the Magill position and has been used successfully in cervical spine injuries. Reported is a case of successful insertion of the intubating laryngeal mask and subsequent intubation through it in a patient with ankylosing spondylitis with cervical involvement resulting in a fixed flexion deformity who underwent coronary artery bypass grafting.     

Overview of the evidence for clinical interventions in pediatric dentistry

PURPOSE: The purpose of this report was to describe the quantity of published literature and types of studies supporting the use of 4 pediatric dentistry procedures: (1) ferric sulfate pulpotomy; (2) stainless steel crowns; (3) space maintainers; and (4) atraumatic restorative technique (ART). METHODS: When available, titles and abstracts of reports written in English and published over a 36-year period (1966-2002) concerning these procedures were retrieved from MEDLINE. They were classified using a modified classification scheme that, in addition to the study designs, also considered the 4 dimensions of measuring dental outcomes. RESULTS: The quantity of available literature concerning each dental procedure varied considerably. Even though many reports were published on treatments, only a small proportion of the published literature for each procedure was found to evaluate outcomes, regardless of outcome dimension. Besides outcomes evaluations, studies on techniques, material properties, and review articles comprised a large proportion of the literature. Clinical dimension of outcomes was most commonly studied. Case series and case reports were the most frequently used study designs to report outcomes. CONCLUSIONS: The outcomes-related literature to support some of the commonly performed treatments is limited both in quantity and study types. More reports are needed to develop the evidence base to support the commonly performed procedures in pediatric dental practice. Additional analyses reporting of the literature are also needed to assess internal and external validity of the studies.     

Immunoglobulin e-mediated reactions to corticosteroids

Allergic reactions to steroids are rare, but are becoming more commonly recognized by clinicians. Although many reactions are thought to be IgE mediated, most are adverse drug reactions caused by nonallergic mechanisms, some related to additives in the steroid preparation being administered. Provocative challenge with the suspected offending agent, serologic tests for allergen-specific IgE, and skin testing are the most reliable methods for assessing hypersensitivity to steroids, although none is infallible. Therapeutic options for patients with documented steroid allergy include the use of alternative steroid preparations, desensitization, and, possibly, the use of monoclonal anti-IgE antibodies.     

Thrombotic complications in childhood-onset idiopathic membranous nephropathy

While the most common clinical feature of nephrotic syndrome is generalized edema, patients are at risk of developing other problems, such as bacterial infections, electrolyte abnormalities, and venous thromboses. Adults with membranous nephropathy appear to be at the greatest risk for developing thromboses, especially renal vein thrombosis. However, the same is not true for children with membranous nephropathy. A review of pediatric membranous nephropathy stated that renal vein thrombosis is unrecorded in childhood-onset membranous nephropathy. We present our experience in managing two children with idiopathic membranous nephropathy who developed venous thromboses. To our knowledge, this is the first report of pediatric patients with membranous nephropathy to develop a thromboembolic complication without evidence of predisposing factors or coagulation abnormalities. This report emphasizes the need for appropriate evaluation of patients with membranous nephropathy who develop signs and symptoms suggestive of arterial or venous occlusion in order to avoid missing this potentially life-threatening medical complication.