HbSC hemoglobinopathy suspected by chest x-ray and red blood cell morphology.

Thorax scan was performed for elucidation of a pulmonary problem in a Nigerian immigrant. The aspect of the vertebrae suggested sickle cell disease, of course without specification of the genotype. Routine hematological tests seemed compatible with an HbSC disease, showing typical laboratory features, namely a significant proportion of hyperchromic RBC, corresponding to secondary, non hereditary spherocytosis, presence of numerous target cells and occasional HbC crystals on Pappenheim stained blood films. The diagnosis of HbSC disease was confirmed by HPLC, iso-electric focusing and citrate agar electrophoresis of hemoglobin and by reverse phase HPLC of globin-chains. This case illustrates the importance of screening for hemoglobin anomalies as it is performed in a multiethnic country such as the Grand Duchy of Luxembourg

Risk factors for hyperlipidemia in long-term pediatric renal transplant recipients.

Hyperlipidemia (HL) is a common problem in adult renal transplant (TP) recipients, contributing to an increased risk of cardiovascular disease and chronic TP nephropathy. There are multiple causes of HL post renal TP in adult patients, including pre TP HL, immunosuppressive agents, renal dysfunction, hypoalbuminemia secondary to nephrotic syndrome, obesity, and conditions that lead to end-stage renal disease (ESRD). We evaluated the incidence and risk factors of HL in 62 pediatric renal TP recipients (15.4+/-4.2 years, range-3.0-22.3 years) with long-term (6.7+/-3.1 years) functioning [glomerular filtration rate (GFR) 66.7+/-23.2 ml/min per 1.73 m(2)] allografts. The mean serum cholesterol (C) level was 205. 5+/-43.6 mg/dl. Thirty-two patients (51.6%) exhibited elevated serum C levels. The mean serum triglyceride (TG) level was 157.3+/-88.4 mg/dl. Serum TG levels were elevated in 32 patients (51.6%). In patients with elevated serum levels of either C or TG, the mean low-density lipoprotein level (LDL) was 138.6+/-44.1 mg/dl (normal <130 mg/dl) and the high-density lipoprotein (HDL) level 54.6+/-15.9 mg/dl (normal>34 mg/dl). Of those patients studied, 45.5% had high LDL levels, whereas 9.1% exhibited low HDL levels. The two risk factors for elevated serum C levels in our patient population were pre-TP HL and increased years since TP. The only risk factor for elevated serum TG levels was reduced GFR. A family history of HL had a significant deleterious impact upon serum levels of C (P=0.01), but did not affect serum TG levels (P=0.7). Years on dialysis prior to TP, history of prior TP, gender, body mass index, and disease leading to ESRD had no influence upon the development of post-TP HL. We conclude that post-renal TP HL is a significant problem in pediatric renal TP recipients.

Peritoneal dialysis catheter infections in children after renal transplantation: choosing the time of removal.

As a foreign body, the peritoneal dialysis (PD) catheter represents a potential source of infection, particularly for immunosuppressed renal transplant patients. A retrospective study was therefore undertaken to compare the risks and benefits of our policy of removing PD catheters at 3 months following renal transplant, which was established to allow for early re-initiation of dialysis. Between 1984 and 1990, 43 renal transplants were performed in 35 children who had been receiving maintenance PD. During the 1st month post transplantation, the PD catheter was used in 25 patients (58%) because of acute rejection or primary allograft non-function. Thirty-one patients were eventually discharged with functioning allografts and a PD catheter in place. Of them, 43% developed a catheter-related infection within the next 2 months, a period during which PD was not performed. Potential contributing factors included a history of catheter-related infection prior to transplantation, use of high-dose methylprednisolone to treat acute rejection, and the type of maintenance immunosuppression prescribed; conversely, the use of prophylactic antibiotics appeared to decrease this risk. This study established the potential need for the catheter during the first few weeks, but because of the infection risk of 43% by 3 months post transplantation, our protocol was revised to include catheter removal at the time of hospital discharge. From 1990 until the end of 1992, an additional 19 PD recipients underwent transplantation. In this group, catheters were used during the 1st month in 6 children (32%). Fifteen patients were discharged with a functioning allograft and only 1 patient returned to PD at 12 months post transplant. It is concluded that PD catheters represent an additional source of infection following transplantation and should be removed at the time of hospital discharge, after which the likelihood of use is low.