Renal medullary carcinoma

Hyperhomocysteinemia is an independent risk factor for coronary artery and cerebrovascular disease, but its significance in the perioperative period is unknown. Nitrous oxide inhibits methionine synthase, which aids in the conversion of homocysteine to methionine. In this prospective, controlled, randomized study, we determined the effect of intraoperative nitrous oxide exposure on postoperative plasma homocysteine concentrations. Twenty ASA physical status I-III patients, aged >18 yr, presenting for elective craniotomy, were randomized to receive general anesthesia with or without nitrous oxide (inspired nitrous oxide >50%). Plasma was sampled before the induction of anesthesia, on arrival in the postanesthesia care unit (PACU) after discontinuation of nitrous oxide, and 24 h after induction. There was a significant increase (22.6+/-11.4 vs 13.0+/-4.7 micromol/L; P = 0.0038 for postoperative versus preinduction values) in plasma homocysteine concentrations in the nitrous oxide group on arrival in the PACU and for 24 h. In the nonnitrous oxide group, mean plasma homocysteine concentrations did not change (9.5+/-1.9 vs 9.8+/-1.6 micromol/L; P = 0.86 for postoperative versus preinduction values). The change in plasma homocysteine concentrations in the nitrous oxide group was significantly different from that in the nonnitrous group (P = 0.0031). We conclude that the use of intraoperative nitrous oxide leads to significant increases in perioperative plasma homocysteine concentrations. IMPLICATIONS: Short-term exposure to nitrous oxide led to significant increases in plasma homocysteine. Further investigations are required to determine the clinical significance of this change.     

Renal carcinoma in adolescents

Renal adenocarcinoma is an infrequent tumor in children. The clinical and radiographic findings in 4 adolescents with such tumors are described. The surgical therapies of these patients and the possible role of adjunctive chemotherapy and radiotherapy are discussed.     

Renal length in sickle cell disease: observations from a cohort study

Renal length has been measured by ultrasound in 237 subjects with homozygous sickle cell (SS) disease, 147 with sickle cell-hemoglobin C (SC) disease, and in 78 age-matched controls with a normal hemoglobin (AA) genotype. As expected, renal length increased with age in all genotypes but mean length was significantly greater in SS disease compared with SC disease (mean difference 4.3 mm after adjustment for height) and significantly greater in both genotypes than in AA controls (SS/AA difference 9.2 mm, SC/AA difference 5.0 mm after adjustment for height). Examination of relationships between renal length and some hematological indices (hemoglobin, fetal hemoglobin, reticulocyte counts, alpha thalassemia status) in SS or SC disease showed only a significant negative correlation with hemoglobin and positive correlation with reticulocyte count in SS disease. Further analysis suggested that the stronger relationship was between renal length and high reticulocyte count. The mechanism of renal enlargement is unknown although glomerular hypertrophy and increased renal blood volume are likely contributors.     

Renal cell carcinoma

This is a report on two cases of mesomelic dysplasia Langer type. This is a rare kind of dwarfism which combines severe dwarfism with predominant shortening of the forearms and lower legs and other morphological abnormalities. The diagnosis can be made by ultrasonography in the early second trimester of the pregnancy.     

Cardiovascular features in adolescents and adults with sickle cell anaemia

Fifty five sickle cell anaemia (SCA) patients at the Kenyatta National Hospital were studied with a view to elucidating their cardiovascular status. Their age range was 13 to 27 years (median 18.9 years). They comprised 27 males and 28 females and their mean haemoglobin concentration was 8.5 +/- 1.4 g/dl. Haemoglobin level of 8.0-9.9 g/dl seen in 30 patients was noted to confer the lowest incidence of exertional dyspnoea and palpitation. Similarly, patients with this haemoglobin level had the lowest mean heart rate. The mean blood pressure was 114.9 +/- 9.9 mmHg systolic and 64.6 +/- 10 mmHg diastolic. Blood pressures, ejection fraction (EF) and differential fibre shortening (%D) were found to be directly related to haemoglobin level, whereas cardio-thoracic index (CTI) and left ventricular dimensions were inversely related to haemoglobin level. Mean echocardiographic measurements were within normal limits and left ventricular functions were found to be normal in 80.9% of the patients indicating that the majority of SCA patients at the Kenyatta National Hospital have good cardiac function.     

Stability and change in the psychological adjustment of mothers of children and adolescents with cystic fibrosis and sickle cell disease

Found moderate stability in the classification of maternal adjustment in two longitudinal studies of mothers of children and adolescents with cystic fibrosis and sickle cell disease. In terms of the transactional stress and coping model, stable poor maternal adjustment was associated with higher levels of appraisal of daily stress and palliative coping and low levels of family supportiveness. With initial levels of maternal adjustment, demographic parameters, and follow-up interval controlled, concurrent levels of daily stress accounted for significant portions of variance in maternal adjustment at follow-up for both illness groups. In addition, illness severity, child psychological adjustment, and family conflict added significant increments to maternal adjustment at follow-up in the cystic fibrosis group. Findings are discussed in terms of a basis for subsequent intervention studies to enhance the adjustment of mothers of children with chronic illness.     

Renal cell carcinoma in a horseshoe kidney: report of two cases

We report 2 patients with renal cell carcinoma in a horseshoe kidney. A renal tumor was diagnosed during evaluation of right humeral metastatic cancer of unknown origin in case 1 (64-year-old male) and gross hematuria in case 2 (54-year-old male). Both patients underwent radical nephrectomy with division of isthmus and retroperitoneal lymph node dissection. The tumors were staged pathologically as pT2bpN0pV0pM1(oss) and pT3pN0pV1bM0, respectively. Only 27 cases of renal cell carcinoma developed in a horseshoe kidney, including the present two cases, have been reported in the Japanese literature. The blood supply to the horseshoe kidney and its evaluation by preoperative angiography were discussed.     

Elevated resting energy expenditure in adolescents with sickle cell anemia

OBJECTIVE: To examine further the role of the amygdala in the recognition of facial expression in adolescents. METHOD: Twelve healthy adolescents were studied using functional magnetic resonance imaging technology during a task of facial affect recognition and a visual control task. RESULTS: All subjects demonstrated a significant increase in signal intensity in the amygdala for the facial expression recognition task. CONCLUSIONS: The data are consistent with previous work in healthy adult subjects implicating the amygdala as essential for the recognition of fearful facial expression.     

Renal cell carcinoma in a patient with Beckwith-Wiedemann syndrome

We report the case of a patient with Beckwith-Wiedemann syndrome (BWS) who developed renal cell carcinoma (RCC). At birth, this patient presented with macroglossia, diastasis recti, mild gigantism, hepatomegaly and hypoglycemia, and the diagnosis of BWS was made. At 22 months, an intrapelvic rhabdomyosarcoma was detected and resected. At 37 months, computed tomography (CT) demonstrated a small mass with high attenuation in the right kidney, which was surgically confirmed to be RCC.     

Psychological adjustment of mothers of children and adolescents with sickle cell disease: the role of stress, coping methods, and family functioning

Assessed the psychological adjustment of 78 mothers of children and adolescents (7-17 years of age) with sickle cell disease. Support was provided for a transactional stress and coping model in delineating the processes associated with maternal adjustment. In particular, poor maternal adjustment was associated with use of palliative coping methods and high levels of stress related to daily hassles. Variables of the model accounted for 55% of the variance in maternal psychological distress.     

Renal medullary cystic disease: assessment by MRI

Cat-scratch disease is a self-limited condition commonly causing a benign chronic lymphadenopathy in children. Osteolytic lesions are a rare complication, but have been previously reported. We report a case of a solitary osteolytic lesion of the skull whose clinical, radiographic and pathological features were initially interpreted as being consistent with Histiocytosis X. Subsequently, positive serological titers for Bartonella, a history of a cat-scratch antecedent to the onset of clinical symptoms and review of the original histopathology confirmed the diagnosis of cat-scratch disease. We reviewed the English language literature on osteolytic lesions associated with cat-scratch disease and compare the current case with those previously reported.     

Vascular cell adhesion molecule-1 is involved in mediating hypoxia-induced sickle red blood cell adherence to endothelium: potential role in sickle cell disease

We investigated the effects of hypoxia on red blood cell (RBC)-endothelial cell (EC) adherence and the potential mechanism(s) involved in mediating this effect. We report that hypoxia significantly increased sickle RBC adherence to aortic EC when compared with the normoxia controls. However, hypoxia had no effect on the adherence of normal RBCs. In additional studies, we found that the least dense sickle RBCs containing CD36+ and VLA-4+ reticulocytes were involved in hypoxia-induced adherence. We next evaluated the effects of hypoxia on the expression of EC surface receptors involved in RBC adherence to macrovascular ECs, including vascular cell adhesion molecule-1 (VCAM-1), intracellular adhesion molecule-1 (ICAM-1), and the vitronectin receptor (VnR). Hypoxia upregulated the expression of both VCAM-1 and ICAM-1, whereas no effect on VnR was noted. Potential involvement of VCAM-1 and ICAM-1 in mediating hypoxia-induced sickle RBC-EC adhesion was next investigated using monoclonal antibodies against these receptors. Whereas anti-VCAM-1 had no effect on basal adherence, it inhibited hypoxia-induced sickle RBC adherence in a concentration-dependent manner, with 50% to 75% inhibition noted at 10 to 60 micrograms/mL antibody (n = 6, P < .05 to P < .01). Anti-ICAM-1 (10 to 60 micrograms/mL, n = 8) had no effect on either basal or hypoxia-induced adherence. As noted in the bovine aortic ECs, hypoxia stimulated the adherence of sickle RBCs to human retinal capillary ECs, and this response appeared to be mediated via mechanisms similar to those observed with macro-endothelium, ie, via the adhesive receptor combination VCAM-1-VLA-4. Our studies show that hypoxia enhances sickle RBC adhesion to both macrovascular and human microvascular ECs via the adhesive receptor VCAM-1. Our findings are of interest because hypoxia is an integral part of the pathophysiology of the vaso-occlusive phenomenon in sickle cell anemia.     

Psychological adjustment of children with sickle cell disease: Stability and change over a 10-month period.

Rates of poor psychological adjustment of children with sickle cell disease remained relatively constant over initial and follow-up assessment points. 50 children (aged 7–12 yrs) and their mothers completed the initial protocol. 30 children completed the follow-up plus 5 additional children and their mothers. These Ss were aged 7–24 yrs at follow-up. With initial levels of adjustment controlled, children's strategies for coping with pain accounted for a significant increment in child-reported symptoms and mother-reported internalizing behavior problems at follow-up beyond the contribution of illness and demographic parameters and follow-up interval. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Renal medullary circulation: morphological characteristics of vessels and their organization

The blood vessels of the renal medulla have several particular features. The supplying vessels of the renal medulla are the efferent arterioles of the juxtamedullary glomeruli. Thus, the blood supply is entirely postglomerular. The distribution of the blood within the renal medulla is effected by the descending vasa recta. Descending and ascending vasa recta form the vascular bundles. Descending vasa recta leave the bundles at any level of the medulla to feed the adjacent capillaries; the longest descending vasa recta reach the papillary tip. The capillary plexus are differently developed. A dense capillary plexus is present only in the inner stripe of the outer medulla. In the inner medulla the capillary plexuses are poorly developed. At these sites ascending recta contribute to the capillarisation. The ascending vasa recta originate from capillaries at any level of the medulla and ascend without joining together towards the intrarenal veins at the cortico-medullary border. They are capillary vessels with wide lumina. Within the outer stripe of the outer medulla these vessels are very narrowly associated with the tubules; interstitial spaces are very poorly developed at this site. These relationships are interpreted as a possible weak point of the medullary circulation in pathological situations.     

Mortality of children with sickle cell disease in a pediatric department in Central Africa

BACKGROUND: Sickle cell disease is a serious public health problem in Gabon with a relatively high mortality rate. PATIENTS AND METHODS: Charts of 23 children (nine boys, 14 girls) who died of complications from sickle cell anemia in the department of pediatrics of Owendo Pediatric Hospital (Libreville, Gabon), from January 1, 1990 through December 31, 1992, were analysed retrospectively. RESULTS: Approximately two-thirds of the children (60.9%) were under 5 years of age. The great majority of patients were from low socio-economic standard families. Of 319 deaths observed during the study period, 23 were due to sickle cell disease-associated complications, for an overall mortality rate of 7.2% and a related mortality of 3.6%. Commonest causes of deaths were severe anemia (11 cases, i.e., 47.8%), which affected predominantly the younger patients between 6 months and 5 years (eight cases), infections (30.4%) and blood transfusion complications (21.7%). CONCLUSION: To decrease these mortality rates, appropriate health supervision and well-designed preventive strategies are needed.     

Nephrotic syndrome in Nigerian children with homozygous sickle cell disease

Two hundred and seventy two children with the nephrotic syndrome were seen and followed up at the Paediatric Renal Unit of the University of Nigeria Teaching Hospital, Enugu over 12 1/2 year period, between June 1983 and December 1995. Of these, there were nine (3.3%) children with homozygous sickle cell disease (SS). This latter group had a mean age of 9.6 +/- 3.2 years, male to female ratio of 1:2 and serum cholesterol of 3.13 +/- 1.48 mmol/l at the time of diagnosis. The mortality rate was 55.6% in these sicklers, death occurring within one and a half to five years of diagnosis. Causes of death were attributable to sickle cell disease in 60%, renal diseases in 20%, and other causes in 20%. Renal biopsy in two of the sicklers showed membrano-proliferative glomerulonephritis (MPGN) and focal glomerulosclerosis (FGS) respectively. Homozygous sickle cell disease does seem to predispose to the development of the nephrotic syndrome and those that do develop nephrotic syndrome exhibit some special characteristics, when compared to non-sicklers with nephrotic syndrome. These include older age of onset of the nephrotic syndrome, reverse sex ratios, lower serum cholesterol, higher mortality rate and sickle cell complications rather than chronic renal failure as the major cause of death.