Red urine, updated for the nephrologist: a case report.

BACKGROUND: Sickle cell trait is not completely benign, and some renal complications can occur. The baseline rate of admission for gross hematuria in normal males carrying the sickle cell trait is 2%. CASE PRESENTATION: A 35-year-old non-smoking African man experienced a 2-week history of painless, profuse and persistent gross hematuria. Laboratory tests showed normal renal function, hematuria and mild proteinuria. Abdominal ultrasonography and computed tomography angiography revealed no renal abnormalities; the bladder appeared pristine under cystoscopy. The diagnosis of sickle cell trait associated with gross hematuria was made using hemoglobin electrophoresis; renal biopsy and its complications were avoided. Urine was clear after 2 weeks of oral hydration and gamma epsilon-aminocaproic acid. CONCLUSION: Hemoglobin electrophoresis should be performed in cases of gross hematuria. Coupled with other non-invasive evaluation, this could avoid renal biopsy and its associated complications.

Sickled erythrocytes in urine as a clue to the diagnosis of sickle cell trait.

Sickled erythrocytes in patients of sickle cell trait with microscopic hematuria have rarely been reported so far. A 30-year-old female underwent delivery of a healthy full-term baby by cesarean section. However, postcesarean, she had pain in abdomen and fever, for which she was advised blood and urine examination. The hemogram suggested mild leukocytosis with neutrophilia and the urine showed red blood cells, some of which were sickled. The patient was advised hemoglobin electrophoresis which suggested sickle cell trait (Hb-AS). We conclude that sickled erythrocytes should not be ignored in a sample of urine as it may serve as an important clue to the diagnosis of sickle cell trait or disease.

Renal Medullary Carcinoma and Sickle Cell Trait: A Push for Early Diagnosis and Intervention Report of Two Cases.

Renal medullary carcinoma (RMC) is a rare but highly aggressive neoplasm that primarily affects young African Americans with sickle cell trait. Most patients present with macroscopic hematuria and have metastases at diagnosis. Chemotherapy, biologics directed against the more common renal cell carcinomas and radiation have all shown limited efficacy in treating patients with advanced RMC. We report two patients with RMC. Both had Stage IV disease. One underwent radical nephrectomy followed by radiation and biologic drug therapy but died five months later; the other underwent multiple cycles of chemotherapy plus anti-angiogenesis treatment but died 15 months after diagnosis. Review of the literature suggests that early diagnosis and surgical intervention while the tumor is confined to the kidney offer the best prospect for long term survival. Since newborn screening for sickle cell is now mandated in the US, the at-risk population for RMC could be identified and followed by yearly urine dipstick testing for microscopic hematuria. Those who test positive can be further evaluated to rule out RMC.

[Evaluation of erythrocyte deformability in subjects with sickle cell trait during a soccer game: effect of hydration ad libitum]. / Évaluation de la déformabilité érythrocytaire des sujets porteurs de trait drépanocytaire au cours d'un match de football : effet de l'hydratation ad libitum.

This study compared erythrocyte changes between a group of subjects with sickle cell trait (SCT) and controls (subjects without hemoglobinopathy) during a soccer game in two conditions: with and without hydration. Erythrocyte deformability of subjects was assessed by the coefficient of erythrocyte rigidity (Tk) which was calculated before and after football match. Our results showed a significant increase in erythrocyte rigidity (Tk) in SCT at the end of physical activities without hydration; however when water was provided ad libitum their Tk decreased significantly, reaching values of controls. And adequate hydration is recommended in subjects with sickle cell trait during and after exercise.

Renal tubular dysfunction in children with sickle cell haemoglobinopathy.

AIM: Children with sickle cell disease (SCD) are remarkably more prone than others to renal dysfunction. The kidneys, as one of the systemic long-term hazards in SCD, may be affected by both the haemodynamic changes of chronic anaemia as well as by the consequences of vaso-occlusion. The aim of this study was to evaluate the proximal tubular function in a group of Saudi children with established SCD. METHODS: This study was conducted in Al-Khafji Joint Operations (KJO) Hospital, in Saudi Arabia during the period from June 2011 to August 2012. Thirty-four children: Group I (18 males and 16 females) with SCD (HBSS) and 27 children: Group II (17 males and 10 females) with sickle cell trait (HBAS) were evaluated for urinary excretion of retinol binding protein (RBP) and - Beta 2 microglobulin (ß2 MG). RESULTS: Group I patients showed a significantly impaired urinary concentrating ability compared to that of Group II (417 ± 94 mOsm/kg vs 581 ± 165 mOsm/kg). The urinary excretions of RBP and ß2-microglobulin were significantly higher in Group I than in Group II. The values were 762.01 ± 124.20 µg/L and 841.84 ± 389.02 µg/L versus 198.12 ± 42.24 µg/L and 298.3 ± 38.11 µg/L, respectively. CONCLUSION: Significant proximal tubular dysfunction was a feature in the SCD group, indicated by high urinary RBP and ß2-microglobulin excretion. Assessing the urinary excretion of these low molecular weight proteins in children with sickle cell disease at different points of diagnosis may add key clinical information to the follow up of renal tubular function in patients with SCD.

Urinary cysteinyl leukotriene E4 significantly increases during pain in children and adults with sickle cell disease.

Baseline level of the cysteinyl leukotriene (CysLT), leukotriene E4 (LTE4), is associated with an increased pain rate in children and adults with sickle cell disease (SCD). To provide additional evidence for a role of CysLTs in the pathogenesis of vaso-occlusion, we tested the hypothesis that LTE4 levels will increase within an individual during painful episodes compared to baseline. In a cohort of 19 children and adults with SCD, median LTE4 levels increased from 82.36 pg/mg creatinine at baseline to 162.81 pg/mg creatinine during a painful episode (P < 0.001). These data further support a contribution of CysLTs to the process of vaso-occlusion.

Bacteriuria in pregnant women with sickle cell trait.

OBJECTIVE: The purpose of this study was to compare the following outcome variables in pregnant patients with sickle cell trait and matched pregnant control patients: asymptomatic bacteriuria, acute cystitis, urinary pathogens that were present, and pyelonephritis. STUDY DESIGN: This was a retrospective cohort study that was conducted at a university clinic. Pregnant patients with sickle cell trait (n = 455) were matched with control patients (n = 448) for race, age, gestational age at entry into prenatal care, and number of prenatal visits. RESULTS: Women with sickle cell trait received urine testing significantly more often. There was no difference in the incidence of positive urine cultures, urinary pathogens, or asymptomatic bacteriuria among the comparison groups. Sickle cell trait carriers had significantly higher rates of pyelonephritis, but many affected patients had risk factors, such as previous pyelonephritis or noncompliance with therapy. CONCLUSION: Sickle cell trait carriers were no more susceptible to acute cystitis and asymptomatic bacteriuria than were the control patients. On the basis of these data, we outline recommendations for urinary screening and pyelonephritis prevention in pregnant patients with sickle cell trait.

Serum and urinary amino acid levels in sickle cell disease.

Neonates with sickle cell disease (SCD) are of normal size at birth in terms of height and weight. However, by the sixth month of life their growth begins to lag significantly behind that of non-sicklers. We hypothesize that such growth retardation could be explained, at least in part, by the increased excretion of free amino acids in the urine of children with SCD. It is well established that in SCD there are abnormalities in the proximal tubules where amino acids are reabsorbed. We collected serum and urine samples from 13 patients with SCD (age range, 10 months to 14 years), and 17 age-and gender-matched controls, and analysed these specimens for free amino acids and creatinine. The SCD population was less well nourished than the controls, as evidenced by the lower serum prealbumin levels in the former group (91.3 v. 127 mg/l, P = 0.01). The serum concentrations of all of the essential amino acids were significantly reduced (21-47 per cent, P < 0.01) in the SCD subjects, as were those of most of the non-essential amino acids (exceptions: alanine, glutamic acid, proline). The urine concentrations of seven of the essential amino acids (indexed to creatinine) were increased in the SCD children. The greatest difference in urinary amino acid excretion was seen with methionine; the SCD subjects excreted 3.6-fold more methionine than the controls. These data indicate that reduced levels of serum amino acids resulting from increased urinary loss of these amino acids in children with SCD could contribute to the decreased growth rates one sees in children with this genetically inherited hematologic disorder.

Penicillin prophylaxis in children with sickle cell disease in Brent.

OBJECTIVE: To assess compliance with oral penicillin prophylaxis in children with sickle cell disease and identify possible reasons for poor compliance. DESIGN: Closed questionnaires given to parents of children with sickle cell disease and general practitioners in Brent. Urine samples from 23 children were tested for penicillin. SETTING: Paediatric haematology clinic, Central Middlesex Hospital, and general practices in Brent. SUBJECTS: 50 children (aged less than or equal to 16) attending clinic with sickle cell disease over six months (33 HbSS, 12 HbSC, five HbS beta thalassaemia). 30 general practitioners: 15 with the greatest number of patients with sickle cell disease on the Brent register; 15 selected randomly from family practitioner committee's list. MAIN OUTCOME MEASURES: Reported compliance with and awareness of importance of penicillin prophylaxis. Results of urine tests for penicillin. RESULTS: 31 parents claimed that their children received penicillin every day and 19 that they received it most days (greater than or equal to 5 days a week). Penicillin was detected in only 10 of 23 urine samples tested. Parents and doctors seemed not to appreciate the importance of treatment: only eight parents were aware of the risk of death if penicillin were discontinued, and 16 doctors were unaware that regular penicillin prophylaxis prevents pneumococcal septicaemia and death in these children. CONCLUSIONS: Education for families with children with sickle cell disease must be improved. Specialised information and training are needed for doctors working in areas with a high prevalence of the disorder.

Urea kinetics in adults with homozygous sickle cell disease.

The kinetics of urea metabolism were measured in four adults with homozygous sickle cell disease (HbSS). On a dietary intake of 1.2 to 2.7 g protein/kg/d the rate of urea production was 188 to 277 mg nitrogen/kg/d. A relatively small proportion of the urea was excreted in the urine (40 per cent), with a high fixed rate of hydrolysis in the bowel, 145 mg nitrogen/kg/d. Although 50 per cent of the nitrogen from hydrolysed urea was resynthesized to urea, and a further 10 per cent may have been lost in the stool, it is estimated that 58 mg nitrogen/kg/d was available for synthetic metabolic activity. Urea kinetics in sickle cell disease subjects are markedly different from normals, and this may be a reflection of the metabolic demands for increased red cell synthesis.

Urea kinetics in adults with homozygous sickle cell disease

The kinetics of urea metabolism were measured in four adults with homozyguous sickle cell disease (HbSS). On a dietary intake of 1.2 to 2.7g protein /kg/d. A relatively small proportion of the urea was excreted in the urine (40 per cent), with a high fixed rate of hydrolysis in the bowel, 145 mg nitrogen /kg/d. Although 50 per cent of the nitrogen from hydrolysed urea was resynthesized to urea, and a further 10 per cent may have been lost in the stool, it is estimated that 58 mg nitrogen /kg/d was available for synthetic metabolic activity. Urea kinetics in sickle cell disease subjects are markedly different from normals, and this may be a reflection of the metabolic demands for increased red cell synthesis. (AU)

The effect of sodium intake on zinc excretion in patients with sickle cell anemia.

Urinary zinc excretion is known to be elevated in subjects with sickle cell anemia. Sodium intake has been suggested to influence zinc excretion in normal subjects. In order to assess the effect of sodium on zinc excretion in subjects with sickle cell anemia, urinary zinc excretion was measured in thirteen children and adolescents with sickle cell anemia on both a high (140 mEq/day) and low (20 mEq/day) sodium intake. Urinary zinc excretion was elevated on both diets. The mean urinary zinc excretion on the high sodium diet (775 +/- 238 micrograms/24 h) was significantly lower (P less than .005) than that on the low sodium diet (947 +/- 344 micrograms/24 h). The zinc excretion did not correlate with calcium or magnesium excretion or aldosterone secretion rates or plasma renin activity. Although elevated, the urinary zinc excretion in patients with sickle cell anemia is still significantly lowered by increasing sodium intake.

The effect of bicarbonate and distilled water on sickle cell trait hematuria and in vitro studies on the interaction of osmolality and pH on erythrocyte sickling in sickle cell trait.

The effect of intravenously administered distilled water was examined alone and during alkalization in a patient with gross hematuria associated with the sickle cell trait. On each of 4 occasions hematuria ceased promptly after the infusion of distilled water. Bicarbonate therapy also consistently decreased hematuria. In vitro studies on erythrocytes from another patient with sickle cell trait and hematuria demonstrated that slight increases in urinary pH similar to those that occur in the urine during alkalization can reverse or prevent erythrocyte sickling in the sicle cell trait. If patients with the sickle cell trait are hydrated adequately and have a good rate of urine flow distilled water can be given intravenously with virtually no danger of acute tubular necrosis secondary to erythrocyte hemolysis.