Primary hyperoxaluria type 1 (PH1) is a rare inherited metabolic disorder in which deficiency of the liver enzyme AGT leads to renal failure and systemic oxalosis. Timely, combined cadaveric liver-kidney transplantation (LKT) is recommended for end-stage renal failure (ESRF) caused by PH1; however, the shortage of cadaveric organs has generated enthusiasm for living-related transplantation in years. Recently, successful sequential LKT from the same living donor has been reported in a child with PH1. We present a sister-to-brother simultaneous LKT in a pediatric patient who suffered from PH1 with ESRF. Twelve months after transplantation, his daily urine oxalate excretion was decreased from 160 mg to 19.5 mg with normal liver and renal allograft functions. In addition to the well-known advantages of living organ transplantation, simultaneous LKT may facilitate early postoperative hemodynamic stability and may induce immunotolerance and allow for low-dose immunosuppression.

Adult↗

PubMed2003

Unilateral ureteroperitoneostomy in the management of hypoproteinemia in nephrotic rats with normal renal function.

Maintenance of serum albumin levels within normal limits is difficult to achieve in nephrotic children with normal renal functions who are unresponsive to specific treatment. One approach in such children is unilateral nephrectomy with rapid progression to renal failure. Peritoneal membrane is permeable to fluids, electrolytes and proteins, and peritoneal space has been used for total parenteral alimentation. Experimental ureteroperitoneostomy has been reported not to cause any significant side effect. The aim of this study was to evaluate the effects of unilateral ureteroperitoneostomy on serum and urine protein levels in rats with adriamycin-induced nephrosis. Adriamycin nephrosis was induced in 45 male Wistar rats. After two weeks, unilateral nephrectomy (Nx), unilateral ureteroperitoneostomy (Up) and sham operated (Sh) groups, each including 15 rats were formed. Serum creatine (S(Cr)) and albumin (S(alb)), and daily urinary protein excretion (U(pro)) were determined before adriamycin injection (week 0), before operations (week 2) and at the end of 6th week in all rats. In addition, percent change in serum albumin (deltaS(alb)) and urine protein levels (deltaU(pro)) between weeks 0-2, 0-6 and 2-6 were calculated for each group (e.g.; deltaS(alb) 0-2 = [S(alb) week 2-S(alb) week 0]/S(alb) week 0 x 100). Then, these parameters were compared within and between the groups. Furthermore, peritoneal tissue samples were obtained from the rats in Sh and Up groups to be examined for pathological changes. S(Cr) did not change within and in between the groups during the study period. S(alb) decreased significantly at weeks 2 and 6 with respect to week 0 in all three groups. In addition, although S(alb) tended to decrease at week 6 with respect to week 2 in all groups, this was significant only in Sh group. U(pro) increased significantly at weeks 2 and 6 with respect to week 0, and at week 6 with respect to week 2 in all groups. However, S(alb) and U(pro) were not different between the three groups at weeks 0.2 and 6. On the other hand, deltaS(alb) and deltaU(pro) were not different between Sh vs. Nx and Nx vs. Up rats, but deltaS(alb) 0-6, deltaS(alb) 2-6 and deltaU(pro) 0-6 were significantly lower in Up group compared to Sh group. Histopathological examination of peritoneal samples revealed significantly higher fibrosis score in Up group compared to Sh group. In conclusion, unilateral ureteroperitoneostomy may one important therapeutic selection in the treatment of intractable nephrotic syndrome. However, peritoneal fibrosis could be a concern for further use of peritoneum in case of end stage renal failure.

Albumins↗

PubMed2003

High altitude and blood pressure in children.

We aimed to evaluate the blood pressure of children who had similar demographic characteristics but lived at different altitudes. Blood pressure of the children attending primary schools in Izmir (sea level: n = 425) and Van (altitude: 1725 m, n = 291) were measured by mercurial sphygmomanometer for this study. They were similar with respect to age, sex, weight, height, and BMI. Mean age of the children was 10.51 +/- 0.87 years (range: 9 to 12 years), and 358 (50 percent) of them were female. Mean systolic blood pressure was significantly higher in the children living in Van than in the children living in Izmir (104.72 +/- 11.2 vs. 97.96 +/- 25.5 mmHg, respectively, p < .001). Similarly mean diastolic blood pressure was significantly higher in the children living in Van than in the children living in Izmir (63.98 +/- 9.3 vs. 59.91 +/- 10.0 mmHg, respectively, p < .001). When blood pressure was evaluated with regard to height percentile, the number of children with a blood pressure over 90 percentile were 19 (4.5 percent) and 48 (16.5 percent) for systolic blood pressure, and 25 (5.9 percent) and 37 (12.7 percent) for diastolic blood pressure among the children living in Izmir and Van, respectively (p < .001). Systolic and diastolic blood pressures were found to increase in parallel to the increase in body mass index in children living in Van (r = 0.358, p < .001 and r = 0.235, p < .001, respectively). However, blood pressures were not correlated to body mass index in children living in Izmir. A difference of 1700 m in altitude was associated with higher systolic and diastolic blood pressure levels in children with similar demographic characteristics, and at this altitude, body mass index and blood pressure showed a positive correlation.

Altitude↗

PubMed2002

Hemodialysis vascular access in incident patients in Spain.

Explore the source record for details and available documents.

Catheters, Indwelling↗

PubMed2002

The role of L-carnitine in treatment of a murine model of asthma.

Leukotrienes, one of the mediators of inflammation in asthma, have a strong bronchoconstrictive effect. L-carnitine has been reported to influence respiratory functions. It has also been reported that L-carnitine inhibits leukotriene synthesis. To evaluate the effects of L-carnitine on oxygen saturation, urine leukotriene E4 levels and lung histopathology in a murine model of asthma, high IgE responder BALB/c mice (n = 24) were systemically sensitized to ovalbumin and chronically challenged with low particle mass concentrations of aerosolized ovalbumin, and then they were divided into 3 groups (study groups A, B, and C) each including eight mice. After methacholine-induced bronchoconstriction, the mice in groups A and B were given intraperitoneal L-carnitine (250 and 125 mg/kg, respectively), while the mice in group C were given placebo. Oxygen saturation of the mice was measured by pulse oxymeter before and after methacholine and after L-carnitine/ placebo application. In addition, urine leukotriene E4 levels were measured before asthma development, and 24-h after L-carnitine injection in asthmatic mice. Inflammation in the lung tissues of the sacrificed animals was scored histopathologically to determine the effect of L-carnitine on tissue level. A control group of non-sensitized mice (n = 8) treated with placebo only was used for comparison of urine leukotriene E4 levels and of histopathological parameters. Oxygen saturation of the mice in the study groups tended to decrease after methacholine and to improve after L-carnitine injection, although these changes were not significant at all time points. Urine leukotriene E4 levels of all 3 study groups increased significantly after asthma development. The rate of increment was smallest in the group given the highest L-carnitine dose (group A). Inflammation at the tissue level was also mildest in group A, and severest in the group that was not given carnitine (group C). All of the study groups and the control group differed significantly with respect to inflammation scores. In conclusion, L-carnitine improved oxygen saturation, and decreased urine leukotriene E4 levels and inflammation in lung tissues in the present murine model of asthma.

Animals↗

PubMed2002

The clinical value of urinary N-acetyl-beta-D-glucosaminidase levels in childhood age group.

N-acetyl-beta-D-glucosaminidase is a high molecular-weight lysosomal enzyme found in many tissues of the body. It cannot pass into glomerular ultrafiltrate due to its high molecular weight. However, this enzyme shows high activity in renal proximal tubular cells, and leaks into the tubular fluid as the ultrafiltrate passes through proximal tubules. When proximal tubular cells are injured due to to any disease process including glomerular proteinuria, nephrolithiasis, hyperglycemia, interstitial nephritis, transplant rejection or nephrotoxic agents such as antibiotics, antiepileptics, or radiocontrast agents, its urine level increases and thus is used as a reflection of proximal tubular cell necrosis. However, the clinical use of urinary N-acetyl-beta-D-glucosaminidase determination is limited in childhood because of certain technical problems. In addition, the urinary level of this enzyme changes with the maturational level of proximal tubular cells. Thus, difficulties are involved in assessing normal urine levels of this enzyme for age. On the other hand, successive measurements of urinary N-acetyl-beta-D-glucosaminidase during the longitudinal follow-up of the patients may enhance its clinical use as an indicator of ongoing tubular injury.

Acetylglucosaminidase↗

PubMed2002

Intestinal urea metabolism: could the bacteria involved in nitrogen cycle lead to reutilization of intestinal urea nitrogen in uremic rabbits?

We aimed to evaluate the effect of bacteria involved in the nitrogen cycle on the reutilization of intestinal urea nitrogen in uremic rabbits. New Zealand white rabbits were made uremic via bilateral nephrectomy. Study and control rabbits were given live and heat-inactivated bacteria through their jejunostomies. After they were injected with 99mTc biurea intravenously, serial serum and stool levels of labeled nitrogen were assessed by instant thin-layer chromatography, and the change in the labeled-nitrogen level was determined. The serum labeled-nitrogen level increased significantly in the study group (r=0.990); however, this level decreased in the control group (r=0.662). Furthermore, the labeled-nitrogen level in the stool samples increased throughout the study in the control rabbits, but it decreased after the 6th hour in the study group. In conclusion, the results of this study suggest that when the bacterial flora of the intestinal system is changed to include bacteria involved in the nitrogen cycle in uremic rabbits, the intraintestinal and systemic nitrogen metabolisms could both be altered in favor of positive nitrogen balance.

Animals↗

PubMedDate not supplied

Immune complex type crescentic glomerulonephritis and ANCA-positivity in a nine-year-old girl.

We report a nine-year-old girl who presented with the clinical and laboratory findings of rapidly progressive glomerulonephritis. She was found to be positive for both pANCA and cANCA. However, renal histopathology revealed immune complex type of crescentic glomerulonephritis. Thus, although testing for ANCA is an important tool in the prediction of the subtype of crescentic glomerulonephritis, a renal biopsy is still required to establish the diagnosis.

Antibodies, Antineutrophil Cytoplasmic↗

PubMedDate not supplied

Horseshoe kidney with mesangioproliferative glomerulonephritis and goiter.

Horseshoe kidney is a relatively common renal anomaly with which many structural and developmental anomalies have been shown to be associated. However, there are only a few case reports regarding the association of membranous glomerulonephritis and focal sclerosing glomerulonephritis in patients with horseshoe kidneys. We report a girl who was evaluated for hematuria and proteinuria, and found to have horseshoe kidney. Renal biopsy demonstrated mesangioproliferative glomerulonephritis. She also had simple diffuse goiter.

Anti-Bacterial Agents↗

PubMedDate not supplied

Sarcoidosis with an uncommon presentation: apropos of a case.

A 17-year-old male presenting with chronic renal failure whose supporting clinical manifestations of the disease had appeared independently over a four-year period is reported. The renal biopsy specimen of the patient revealed tubulointerstitial nephritis and membranous glomerulonephritis. He never had hilar adenopathy, but maculopapular rashes, erythema nodosum, arthritis, chronic lymphocytopenia, hepatomegaly, splenomegaly, and lymphadenomegaly had been observed at different periods over four years. The presence of non-caseating granulomatous lesions in the liver biopsy accompanying uveitis verified the diagnosis of sarcoidosis. Low dose steroid was applied to this hepatitis-C carrier, and uveitis was suppressed. No recurrence has been observed in two-year follow-up.

Adolescent↗