Simultaneous pancreas-kidney transplant in two children with hemolytic-uremic syndrome

Simultaneous pancreas-kidney (SPK) transplantation has rarely been performed in the pediatric population. This report describes successful SPK transplantation in a 12-year-old girl and a 14-year-old boy with renal and pancreatic insufficiency secondary to postdiarrheal hemolytic-uremic syndrome. All reported cases of pediatric SPK transplantation are reviewed. SPK transplantation is a feasible option in selected pediatric patients with combined pancreatic and renal insufficiency.     

Plasma exchange in children with hemolytic-uremic syndrome at risk of poor outcome

A 28-year-old male with AIDS and a CD4 cell count of 100/mm3 presented with fever, hepatosplenomegaly, weight loss, and multiple, polypoid, angiomatous lesions on his face. It was determined by means of biopsy that the lesions were due to bacillary angiomatosis. The patient was treated with oral azithromycin (1 g daily as a single dose). Rapid resolution of the skin lesions was noted. After 1 week of therapy, diminution in the size of the liver and spleen was noted. The only significant side effect noted was diarrhea, which was controlled with symptomatic therapy.     

Pathomorphology of pneumococcal infection in children

The Colles' fracture is probably the most common fracture seen in the Emergency Department. Although there are several different methods of reduction and fixation, the goal of any treatment plan is to return the patient to normal function. Depending on the complexity, the Colles' fracture can be successfully treated open or closed. This article reviews the mechanism of injury, assessment, conservative or operative treatment, and rehabilitation of these fractures.     

Adult hemolytic-uremic syndrome. Familial variant

We describe here a family with hemolytic-uremic syndrome culminating in renal failure and severe hypertension in the involved male adults. Members of this family developed a microangiopathic hemolytic anemia, progressive renal failure, the onset of elevation of blood pressure, and an untimely death in young adulthood. One affected family member has survived the initial crisis. The family history presents further evidence for an autosomal dominant pattern of inheritance of hemolytic-uremic syndrome with presentation in adulthood.     

Recurrence of hemolytic-uremic syndrome in renal transplant recipients: a meta-analysis

To identify contributing factors for cheese-associated outbreaks, we reviewed all cheese-associated outbreaks of human illness reported to the Centers for Disease Control and Prevention (CDC) with onsets during 1973 to 1992. The infrequency of large, cheese-associated outbreaks was notable because such outbreaks had been a frequent public health problem before the mid-20th century. Of 32 reported cheese-associated outbreaks, 11 attributed to manufacturing errors caused most of the illnesses and hospitalizations and all 58 deaths. Important factors in these 11 outbreaks were manufacturing cheese with raw or improperly pasteurized milk and postpasteurization contamination. If current Food and Drug Administration sanitary requirements for cheesemaking had been met, these outbreaks would have been preventable. In two outbreaks of Salmonella infections, fewer than 10 Salmonella per 100 g of cheese were detected. In two outbreaks of Brucella infections, efforts to recover the pathogen from the implicated cheese were unsuccessful, emphasizing the inadequacy of end product testing for assuring consumer safety. Curing cheeses kills most bacteria present in cheeses; however, evidence from sources other than the CDC Foodborne Disease Outbreak Surveillance System suggests that curing alone may not be a sufficient pathogen control step to eliminate Salmonella, Listeria, and E. coli O157:H7 from cheese.     

von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome

BACKGROUND: Thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome are severe microvascular disorders of platelet clumping with similar signs and symptoms. Unusually large multimers of von Willebrand factor, capable of agglutinating circulating platelets under high shear stress, occur in the two conditions. We investigated the prevalence of von Willebrand factor-cleaving protease deficiency in patients with familial and nonfamilial forms of these disorders. METHODS: Plasma samples were obtained from 53 patients with thrombotic thrombocytopenic purpura or hemolytic-uremic syndrome. Von Willebrand factor-cleaving protease was assayed in diluted plasma samples with purified normal von Willebrand factor as the substrate. The extent of the degradation of von Willebrand factor was assessed by electrophoresis in sodium dodecyl sulfate-agarose gels and immunoblotting. To determine whether an inhibitor of von Willebrand factor-cleaving protease was present, we measured the protease activity in normal plasma after incubation with plasma from the patients. RESULTS: We examined 30 patients with thrombotic thrombocytopenic purpura and 23 patients with the hemolytic-uremic syndrome. Of 24 patients with nonfamilial thrombotic thrombocytopenic purpura, 20 had severe and 4 had moderate protease deficiency during an acute event. An inhibitor found in 20 of these patients was shown to be IgG in five of five tested plasma samples. Of 13 patients with nonfamilial hemolytic-uremic syndrome, 11 had normal levels of activity of von Willebrand factor-cleaving protease during the acute episode, whereas in 2 patients, the activity was slightly decreased. All 6 patients with familial thrombotic thrombocytopenic purpura lacked von Willebrand factor-cleaving protease activity but had no inhibitor, whereas all 10 patients with familial hemolytic-uremic syndrome had normal protease activity. In vitro proteolytic degradation of von Willebrand factor by the protease was studied in 5 patients with familial and 7 patients with nonfamilial hemolytic-uremic syndrome and was normal in all 12 patients. CONCLUSIONS: Nonfamilial thrombotic thrombocytopenic purpura is due to an inhibitor of von Willebrand factor-cleaving protease, whereas the familial form seems to be caused by a constitutional deficiency of the protease. Patients with the hemolyticuremic syndrome do not have a deficiency of von Willebrand factor-cleaving protease or a defect in von Willebrand factor that leads to its resistance to protease.     

Long-term neurological sequelae of hemolytic-uremic syndrome: a preliminary report

Seven patients with hemolytic-uremic syndrome who had major neurological symptoms during the acute illness were neurologically and cognitively evaluated prospectively several years after recovery from the illness. Four patients showed evidence of subtle neurological sequelae, including posturing, clumsiness, poor fine-motor coordination, hyperactivity, and distractibility. Psychoeducational evaluation of all seven subjects revealed mean scores within the average range in cognitive abilities, academic achievement, single word receptive vocabulary, visual/motor planning, overall adaptive functioning, and hyperactivity. The lapse of time (minimum of 7 years) between the acute illness and the psychometric evaluation could have been responsible for our normal results.     

Anticardiolipin antibodies in classic pediatric hemolytic-uremic syndrome: a possible pathogenic role

BACKGROUND: Leptin, an adipose tissue-derived signalling factor encoded by the obese gene has been shown to be present as a 16-kDa protein in the blood of mice and humans. Resistance to leptin occurs in human obesity. Leptin has also been shown to associate with plasma insulin concentrations and there is currently considerable debate about the potential link between insulin resistance and resistance to leptin. In non-pregnant individuals, circulating leptin concentrations associate strongly with both total body fat mass and body mass index (BMI). In normal human pregnancy, the maternal fat stores increase to a peak in the late second trimester, before declining towards term as fat stores are mobilized to support the rapidly growing fetus. Insulin resistance increases during late pregnancy and is believed to be further enhanced in pregnancies complicated by pre-eclampsia. The aim of this study was to examine if leptin levels were altered in pregnancy and, if so, whether the pattern of change in circulating leptin related to previously established changes in fasting insulin concentrations or fat mass. METHODS: We measured third trimester plasma leptin concentrations in 12 uncomplicated pregnant women, nine women with pre-eclampsia matched for age and booking BMI, and 18 non-pregnant women similarly matched. We also examined the longitudinal course of leptin concentrations occurring throughout gestation (from 10 weeks gestation and at five week intervals thereafter), in five normal pregnancies and two women with gestational-onset diabetes. RESULTS: Leptin concentrations were significantly higher in the normal pregnant women (37.1 microg/L, [15.4-117.0], geometric mean, [range]; p=0.049), and women with pre-eclampsia (45.3 microg/L, [21.3-98.4]; p=0.001), than in non-pregnant controls (17.85 microg/L, [1.3-36.5]), however, there was no significant difference between uncomplicated and pre-eclamptic pregnancies (p=0.22). On examination of the longitudinal course of leptin concentrations occurring throughout gestation, in all seven women plasma leptin concentrations initially increased relative to booking (10 weeks) concentrations, but did so by varying amounts (ranging between 30-233%). Significantly, however, in all seven women plasma leptin concentrations peaked at around 20-30 weeks of gestation before declining towards term. CONCLUSION: On the basis of these observations, we postulate that plasma leptin levels increase significantly in human pregnancies and that the pattern of change in circulating leptin parallels the process of fat accumulation and mobilization.     

Mesangial involvement in hemolytic-uremic syndrome. A light and electron microscopic study

Electron microscopic analysis of the mesangial injury in the hemolytic-uremic syndrome was performed in 10 patients. Proteinaceous material similar to that found in the subendothelial region was also seen focally in the mesangium altering the matrix and imparting a reticular appearance. This degenerative process was associated with reparative changes in the glomerular tuft. Many of the mesangial cells were hypertrophied and demonstrated phagocytic activity and peripheral extension of their cytoplasmic processes. Mitotic figures in endothelial as well as mesangial cells were regarded as evidence of a reparative process. Severe mesangial insudation of material containing fibrinogen derivatives resulted in segmental tuft necrosis with almost complete replacement and destruction of the mesangial matrix. On some occasions, a break of the glomerular basement membrane was accompanied by the escape of intraluminal contents into the urinary space, leading to crescentic epithelial cell proliferation.     

Doppler studies in normal kidneys of healthy children

Oral malodor (halitosis) is a common concern in Western society. As with other human perceptions, emotional as well as cognitive variables play a major role in one's sensation and complaint. To study factors potentially associated with the complaint of oral malodor, periodontal and psychological evaluations were carried out on 38 subjects (66% female, mean age 43 years) with a complaint of oral malodor. Subjects underwent evaluation of their periodontal status, odor evaluation by an odor judge, and psychopathological symptom survey by means of the SCL-90 questionnaire. The patient's self-rating of oral odor was significantly higher than the evaluation of an objective odor judge and was not associated with their periodontal status. The SCL-90 profile of subjects was relatively higher than that of an age- and gender-matched reference group of dental patients. The results suggest that the complaint of oral malodor may be related to psychopathological symptoms as recorded by the SCL-90 questionnaire.     

Nephrological radiodiagnosis in children with healthy kidneys and in children with pyelonephritis. I. Determination of kidney size in children with healthy kidneys

This paper, the third of three articles, discusses the basic techniques of administering local anesthetics to the scalp, face, and neck.     

Quantitative SPECT uptake of 99mTc-dimercaptosuccinic acid by the kidneys in children

Patients with nonvalvular atrial fibrillation (AF) have an increased risk of stroke, but the absolute rate of stroke varies widely depending on coexistent vascular disease. We assessed the stroke rate and predictive value of two published schemes for stroke risk stratification in a population-derived cohort of 259 elderly people with nonvalvular AF followed for a median of 5.3 years. The rate of ischemic stroke was 2.8% per year (95% confidence interval [CI] 1.9, 3.9). Thirty-one percent were predicted to be at low risk, and their stroke rate was 1.7% per year (95% CI 0.6, 3.8). Many people with AF in this population-derived cohort had relatively low rates of stroke. Further studies to reliably stratify stroke risk in patients with AF are needed.     

Inflammatory mediators in Escherichia coli O157:H7 hemorrhagic colitis and hemolytic-uremic syndrome

BACKGROUND: Recent experimental data suggest that the inflammatory response of the host to verotoxin and/or lipopolysaccharides of Escherichia coli is involved in the pathophysiology of verotoxin-producing E. coli (VTEC) infections. METHODS: We measured the circulating concentrations of cytokines [TNF-alpha, interleukin (IL)-1-beta, IL-1 receptor antagonist (Ra), IL-6, IL-8, IL-10] and soluble leukocyte adhesion molecules (L-selectin, P-selectin, E-selectin, intracellular cell adhesion molecule-1, vascular cell adhesion molecule-1) by sandwich enzyme-linked immunosorbent assay among (1) normal controls (n = 12), (2) disease controls with hemorrhagic colitis (HC) not associated with VTEC infections (n = 57), (3) patients with uncomplicated HC caused by E. coli O157:H7 (n = 30), and (4) children with hemolytic-uremic syndrome (HUS) (n = 28). Patients with HUS were matched with children who presented an uncomplicated HC caused by E. coli O157:H7 for the time interval elapsed between the onset of HC and that of blood sample collection. RESULTS: Concentrations of TNF-alpha and IL-1-beta were undetectable. Children with HUS were characterized by increased amounts of IL-6 and IL-8, lower values of soluble L-selectin as well as increased levels of IL-10 and IL-1Ra. The circulating concentrations of IL-1Ra were higher among children with O157:H7 HC who subsequently developed HUS. CONCLUSIONS: Increased pro- and antiinflammatory cytokine responses are produced by the host during the development of HUS among children with VTEC infections. Further studies are needed to determine their relative contribution to the pathophysiology of classic HUS.