Thirty-year follow-up of superior vena cava-pulmonary artery (Glenn) shunts.

The first superior vena cava-pulmonary artery shunt (Glenn shunt) in our series was performed in February 1958. From then through September 1988, 91 patients have undergone this procedure for a wide variety of congenital defects. We here report follow-up data available on all patients. Ages ranged from 2 days to 46 years (mean 6.8). Diagnoses were as follows: tricuspid atresia, 27; single ventricle, 22; tetralogy of Fallot, 14; D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis, 9; D-transposition, 5; Ebstein's anomaly, 4; pulmonary atresia + intact septum, 4; and others, 6. The hospital mortality rate was 7.7% (one death in the last 53 patients, 1.9%). Five deaths occurred in patients less than 6 months old. There were 20 late deaths (22%) with actuarial survival rates of 84% and 66% at 10 and 20 years, respectively. Pulmonary arteriovenous fistula formation was seen in 18 patients (19.7%), six of whom have undergone therapeutic embolization with improvement in saturation. The prevalence of pulmonary arteriovenous fistula increases with time after shunt. No long-term shunt thrombosis or stricture formation was seen. Fifty percent of shunts were still functioning at 20 years. Palliation was limited because of decrease in blood flow to the contralateral pulmonary artery, collaterals between the inferior and superior venae cavae, and pulmonary arteriovenous fistula formation. Improvement in saturation was obtained in eight otherwise inoperable patients by creation of a right axillary arteriovenous fistula up to 19 years after the Glenn shunt. Three patients had conversion of a Blalock-Taussig shunt to a Glenn shunt with improvement in congestive heart failure. Twenty-six patients have undergone a Fontan procedure with two deaths. Compared with the group having a Fontan procedure without a prior Glenn operation, there was no difference in early or late mortality. Thirty years after a Glenn shunt, the first patient in this series is working full time after having undergone a modified Fontan procedure in 1981. We conclude that the Glenn connection, usually with supplemental procedures to enhance oxygenation, has provided excellent physiologic palliation with low mortality up to 30 years with no late thrombosis or stricture formation. The incidence of pulmonary arteriovenous fistula increases with time and can be effectively treated with embolization. Physiologic repair after the Glenn shunt carries a low mortality. Although currently used infrequently, superior vena cava-pulmonary artery shunting remains a useful method of palliation in selected patients.(ABSTRACT TRUNCATED AT 400 WORDS)     

Percutaneous nephrostomy drainage in the management of neonatal anuria secondary to renal candidiasis

We report a case of anuria in a premature neonate secondary to bilateral ureteropelvic junction obstructions related to Candida bezoars. Percutaneous decompression and drainage of both kidneys contributed significantly to the successful management of renal candidiasis in this patient. A review of the literature is presented.     

Malignant peripheral neuroectodermal tumors in urology

Summary During the past few years, a new tumor type has emerged in the pediatric and adolescent group of cancer patients, which has been designated malignant peripheral neuroectodermal tumor (MPNT). This tumor has some clinical and pathological signs in common with either soft-tissue sarcomas or classic Ewing's sarcoma, but is defined as a distinct entity because of its immunohistological characteristics. The tumor expresses neuronal markers, but the pattern varies: chromogranin, neuron-specific enolase, synaptophysin, protein S-100 and others. MPNT can occur in the urogenital region. The differential diagnosis on clinical grounds must include Ewing's and soft tissue sarcomas, and also Wilms' tumor and its variants. MPNT are often wide spread in the urogenital region when first diagnosed. Response to radiotherapy and combination chemotherapy is limited. Radical surgery is not always possible. The prognosis therefore remains rather poor at this time. A selection of MPNT patients is presented to demonstrate the various problems associated with this diagnosis. MRI and CT of all patients showed large tumors with direct infiltration of the surrounding structures. MRI is the best imaging modality for diagnosis and therapy monitoring in these tumors, because of its high soft-tissue contrast.     

Neutrophil Bactericidal Function in Diabetes Mellitus: Evidence for Association with Blood Glucose Control

Neutrophil bactericidal activity was assessed in patients with type 1 (n=45) and Type 2 diabetes mellitus (n=68) and non-diabetic control subjects (n=40) by measurement of whole blood chemiluminescence. Though chemiluminescence values tended to be highest in the non-diabetic subjects these differences were not statistically significant (mean ± SD) (2.73 ± 1.65 mV (controls), 2.33 ± 1.41 mV (Type 1 diabetes) and 2.38 ± 1.12 mV (Type 2 diabetes), F=1.12, p=0.33). Significant negative correlations were evident, however, in patients with both Type 1 and Type 2 diabetes between chemiluminescence and glycated haemoglobin (r_s=-0.35, p=0.005 (Type 1), r_s=-0.45, p=0.002 (Type 2), fructosamine (r_s=-0.36, p=0.003 (Type 1), r_s=-0.42, p=0.004 (Type 2)), and random blood glucose (r_s=-0.25, p=0.04 (Type 1), r_s=-0.48, p=0.001 (Type 2)). Changes in whole blood chemiluminescence in a further group of 10 patients with Type 2 diabetes mellitus commenced on insulin therapy were followed for 21 days. Serum fructosamine concentrations fell significantly over this time (524 ± 58 μmol l^?1 to 405 ± 47 μmol l^?1, p<0.001), however, although chemiluminescence values tended to rise these changes were not statistically significant (1.01 ± 0.38 mV to 1.60 ± 0.91 mV, S=4.24, df=5, p=0.52). These results suggest that impaired neutrophil bactericidal function is associated with poor blood glucose control. While it is likely that neutrophil bactericidal function will improve as blood glucose control improves, further studies are required both to confirm this and to demonstrate a reduction in the incidence of clinical bacterial infection.     

Treatment of diabetic autonomic neuropathy with an aldose reductase inhibitor

To evaluate the effects of the aldose reductase inhibitor Ponalrestat (Statil) on diabetic autonomic neuropathy, a double-blind placebo controlled trial was carried out on a group of 34 diabetic patients with documented cardiac autonomic neuropathy. After a 4-week, placebo run-in period, patients were randomised for treatment with 600 mg Statil or placebo for another 24 weeks. Moreover, the reliability of the autonomic nerve function tests was investigated by comparing the results at onset and at week 4. Fifteen patients treated with Statil and 12 with placebo completed the study. Neither symptom scores nor cardiovascular reflexes, pupil reflexes and skin vasomotor reflexes improved after Statil therapy, which led us to conclude that Statil is not effective in the treatment of diabetic autonomic neuropathy. Reliability coefficients for cardiovascular reflexes and pupil reflex showed high values, ranging from 60% to 80%. Therefore these methods are recommended in future therapy trials.     

Sexually transmitted diseases control in developing countries.

Sexually transmitted diseases (STDs) are a major public health problem now compounded by the advent of AIDS and HIV infection. The size of the problem represented by STDs and HIV is unknown however it is estimated that there are 333 million new cases of STD per annum and currently 15-20 million people infected worldwide with HIV. Control programmes for STDs must prevent the acquisition of STDs, their complications and sequelae and interrupt and reduce transmission. They can also reduce the incidence of HIV infection. Such programmes must place emphasis on health education, condom usage, altering health seeking behaviour and providing case management. The syndromic approach currently offers the most realistic, and cost effective, way in which to treat patients.     

Alteration in the pattern of macrophage subtypes in chronic osteomyelitis compared with acute joint infection

Summary Macrophage subtypes were detected in cryostat sections of biopsies from patients with chronic osteomyelitis, acute joint infections and normal bone marrow, using monoclonal antibodies against different macrophage populations. The resident macrophage subtype 25F9, the glucocorticoid-inducible macrophage RM 3/1 and the inflammatory type 27E10 were found in abundance in acute infections. They were also present in tissue sections of uninflamed bone marrow. By contrast, in about 50% of the biopsies from patients with chronic osteomyelitis a reduced number of macrophage subtypes, or even the lack of one or more macrophage subpopulations was found. The unusual absence of macrophage phenotypes seems to be restricted to the area of osteomyelitis because in the tissues of inflamed sinuses in these patients, the macrophage subtypes were present. These findings suggest a disturbance at the level of the macrophages which may contribute to the persistence of the inflammatory process in osteomyelitis.

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Résumé L'utilisation d'anticorps monoclonaux contre différentes sous-populations de macrophages a permis de mettre en évidence, à l'examen de coupes cryostatiques, des sous-types de macrophages. Ces derniers ont été retrouvés dans les moelles osseuses normales comme dans les biopsies provenant de malades atteints d'ostéomyélite chronique et d'arthrite aiguë. Le sous-type résident 25F9, le macrophage glycocorticoïde inductible RM 3/1 et le sous-type inflammatoire 27E10 ont été trouvés en abondance dans les infections aiguës. Ils étaient également présents dans des coupes de moelle osseuse non inflammatoire. Inversement dans près de 50% des cas le nombre de ces sous-populations était considérablement diminué, et même certaines d'entre elles avaient complétement disparu, sur les pièces biopsiques d'ostéomyélite chronique. L'absence inhabituelle de certains types de macrophages semble être limitée à la zone ostéitique car on les retrouve dans le tissu inflammatoire des fistules. Cette étude semble montrer que l'atteinte de ces sous-populations serait responsable de la chronicité de l'ostéomyélite.

     

Erotomania in a brain-damaged male

A 31-year-old male presented to a neuropsychiatric service for the first time with an erotomatic delusion among other symptoms. He had suffered significant brain damage several times in the past and experienced a recent loss. After extensive evaluation, a trial of haloperidol resulted in some improvement. However, the erotomanic delusion remained unchanged.     

Relation between toxicity and carcinogenesis in the kidney: an heuristic hypothesis

Cellular toxicity and cellular carcinogenesis are closely linked. In the kidney, this relationship has been emphasized by the recent discovery of a number of putatively non-mutagenic chemicals that result in acute and chronic toxicity and ultimately in carcinogenesis, especially in the male rat. Many, but not all such compounds, result in renal PTE phagolysosomal overload. At the same time, known metabolites of other carcinogens, e.g., HCBD and FBPA, result in acute renal injury and/or necrosis, followed by chronic tubular disease, interstitial nephritis, and ultimately carcinogenesis. A series of cell mechanisms have been suggested that lead from acute cell injury to altered control of cell division. These mechanisms appear to involve ion deregulation, (especially [Ca2+]i) resulting from a variety of continued injuries, (e.g., oxidative stress from inflammatory cells) and ultimately leading to altered gene expression.