Laboratory interpretative comments and guidance: clinical and operative outcomes on moderate to severe hyponatraemia patient management.

AIMS: Hyponatraemia is the most common body fluid disorders but often goes unnoticed. Our laboratory incorporated a standardised procedure to help clinicians detect moderate/severe hyponatraemia. The study aims were to evaluate the outcomes on patient care and clinicians' satisfaction. METHODS: The study, observational and retrospective, included 1839 cases, adult and paediatric patients, with sodium concentration <130 mmol/L. The procedure consisted of interpretative comments in the emergency and core laboratories report and the point-of-care testing blood gas network report. We evaluated hyponatraemia length in two equal periods: before and after the implementation. We conducted a survey addressed to the staff of the clinical settings involved to know their satisfaction. RESULTS: The median hyponatraemia length decreased significantly from 4.95 hours (2.08-16.57) in the first period to 2.17 hours (1.06-5.39) in the second period. The lack of hyponatraemia patients follow-up was significantly less after the procedure implementation. The survey was answered by 92 (60 senior specialists and 32 residents) out of 110 clinicians surveyed. Ninety of them (98%) answered positively. CONCLUSIONS: We have demonstrated the reduction in the time for diagnosing and management by physicians, the higher uniformity in the time required to solve hyponatraemia episodes following our laboratory procedure and the clinicians' satisfaction.

Case Report: Combined Liver-Kidney Transplantation to Correct a Mutation in Complement Factor B in an Atypical Hemolytic Uremic Syndrome Patient.

Pathogenic gain-of-function variants in complement Factor B were identified as causative of atypical Hemolytic Uremic syndrome (aHUS) in 2007. These mutations generate a reduction on the plasma levels of complement C3. A four-month-old boy was diagnosed with hypocomplementemic aHUS in May 2000, and he suffered seven recurrences during the following three years. He developed a severe hypertension which required 6 anti-hypertensive drugs and presented acrocyanosis and several confusional episodes. Plasma infusion or exchange, and immunosuppressive treatments did not improve the clinical evolution, and the patient developed end-stage renal disease at the age of 3 years. Hypertension and vascular symptoms persisted while he was on peritoneal dialysis or hemodialysis, as well as after bilateral nephrectomy. C3 levels remained low, while C4 levels were normal. In 2005, a heterozygous gain-of-function mutation in Factor B (K323E) was found. A combined liver and kidney transplantation (CLKT) was performed in March 2009, since there was not any therapy for complement inhibition in these patients. Kidney and liver functions normalized in the first two weeks, and the C3/C4 ratio immediately after transplantation, indicating that the C3 activation has been corrected. After remaining stable for 4 years, the patient suffered a B-cell non-Hodgkin lymphoma that was cured by chemotherapy and reduction of immunosuppressive drugs. Signs of liver rejection with cholangitis were observed a few months later, and a second liver graft was done 11 years after the CLKT. One year later, the patient maintains normal kidney and liver functions, also C3 and C4 levels are within the normal range. The 12-year follow-up of the patient reveals that, in spite of severe complications, CLKT was an acceptable therapeutic option for this aHUS patient.

Pediatric transplantation in Europe during the COVID-19 pandemic: Early impact on activity and healthcare.

The current pandemic SARS-CoV-2 has required an unusual allocation of resources that can negatively impact chronically ill patients and high-complexity procedures. Across the European Reference Network on Pediatric Transplantation (ERN TransplantChild), we conducted a survey to investigate the impact of the COVID-19 outbreak on pediatric transplant activity and healthcare practices in both solid organ transplantation (SOT) and hematopoietic stem cell transplantation (HSCT). The replies of 30 professionals from 18 centers in Europe were collected. Twelve of 18 centers (67%) showed a reduction in their usual transplant activity. Additionally, outpatient visits have been modified and restricted to selected ones, and the use of telemedicine tools has increased. Additionally, a total of 14 COVID-19 pediatric transplanted patients were identified at the time of the survey, including eight transplant recipients and six candidates for transplantation. Only two moderate-severe cases were reported, both in HSCT setting. These survey results demonstrate the limitations in healthcare resources for pediatric transplantation patients during early stages of this pandemic. COVID-19 disease is a major worldwide challenge for the field of pediatric transplantation, where there will be a need for systematic data collection, encouraging regular discussions to address the long-term consequences for pediatric transplantation candidates, recipients, and their families.

A case-control study to assess the role of polyomavirus in transplant complications: Where do we stand?

PURPOSE: The study's aim was to assess whether polyomavirus DNAemia screening was associated with different outcomes in patients with positive viremia compared with negative viremia. METHODS: Case-control retrospective study of patients with polyomavirus DNAemia (viremia > 1000 copies/mL) matched 1:1 with controls. Control group consists of the patient who received a transplant immediately before or after each identified case and did have nil viremia. FINDING: Ultimately, 120 cases of BK polyomavirus (BKPyV) were detected and matched with 130 controls. Of these, 54 were adult kidney transplant recipients (KTRs), 43 were pediatric KTRs, and 23 were undergoing hemato-oncologic therapy, of which 20 were undergoing hematopoietic stem cell transplantation. The odds ratio (OR) for overall risk of poorer outcomes in cases versus controls was 16.07 (95% CI: 5.55-46.54). The unfavorable outcome of switching the immunosuppressive drug (ISD) (14/40,35%) was no different from that of those treated with reduced ISD doses (31/71, 43.6%, P = .250). Acute rejection or graft-versus-host disease, previous transplant, and intensity of immunosuppression (4 ISDs plus induction or conditioning) were risk factors for BKPyV-DNAemia (OR: 13.96, 95% CI: 11.25-15.18, P < .001; OR: 6.14, 95% CI: 3.91-8.80, P < .001; OR: 5.53, 95% CI: 3.37-7.30, P < .001, respectively). CONCLUSIONS: Despite viremia screening, dose reduction, and change in therapeutic protocol, patients with positive BKPyV-DNAemia present poorer outcomes and unfavorable results.

Living-donor transplantation after excision of unrecognized renal cancer diagnosed after transplant.

BACKGROUND: Published data on kidneys transplanted after resecting small renal cancers during the transplantation surgery are very rare and, to the best of our knowledge, no pediatric cases have been reported in the literature. CASE-DIAGNOSIS/TREATMENT: Our patient was diagnosed with a bilateral Wilms tumor when he was 15 months old. A total bilateral nephrectomy was required to control the disease. Two years later, a human leukocyte antigen (HLA)-identical living-donor transplant from his father was performed. A small mass in the father's left kidney was diagnosed as an angiomyolipoma during the pretransplant donor evaluation. During the surgery, the mass was excised and the kidney implanted. One week later, the pathological study revealed the mass to be a clear cell renal carcinoma. After joint discussion, the urologic and nephrologic teams and the family decided to maintain the transplant, managing the patient with monotherapy based on rapamycin and close ultrasound control. To date, 8 years after transplantation, no signs of malignancy have been detected, and renal function is normal. CONCLUSION: This is the first reported pediatric case of a living-donor graft with a small renal carcinoma excised in the operating room. No malignancy has been observed in 8 years of follow-up.

Acute renal failure due to bilateral pieloureteral stone impaction in a 10-month-old boy.

Urolithiasis (UL) can present with its classic signs and symptoms, such as flank or abdominal pain and gross hematuria. However, atypical complaints can be more common in younger children. We report here a case of bilateral ureteropelvic junction (UPJ) stones in a 10-month-old boy who only showed nonspecific symptoms at the time of presentation. The initial blood test revealed renal failure (serum creatinine 3.4 mg/dl), hyperkalemia (6.4 mEq/l), hyperphosphoremia (9.4 mEq/l) and mild metabolic acidosis. Medical treatment for electrolyte disorders was started. The ultrasonography revealed impacted stones in both ureteropelvic junctions. A pigtail catheter was placed in each ureter. High urine flow was promptly achieved after the pigtail procedure, and the serum creatinine level dropped quickly from 4.5 to 0.32 mg/dl. Quantitative determination of urinary amino acids by ion exchange chromatography showed high cystine levels of 8.43 mmol/g creatinine. Outpatient follow-up was scheduled every 3 months to monitor patient compliance with potassium citrate. In the first 6 months, the patient underwent three febrile urinary tract infections (UTIs). Since both pigtail catheters were removed, he has been free of UTIs and stones. Our case emphasizes the need for considering UL in infants who complain with unclear signs, because UL can only show nonspecific symptoms in children younger than 1 year old. Since cystinuria can cause loss of renal function due to urinary system obstruction and UTI, an early diagnosis and a close follow-up are the key to achieving the best long-term outcome.

Antierythropoietin antibody-induced pure red cell aplasia: posttransplant evolution.

Pure red cell aplasia is a rare complication of recombinant human erythropoietin (rHuEPO) treatment, which physicians should consider once the more frequent causes of hyporegenerative anemia have been excluded. To our knowledge, no pediatric cases have been described. In our patient, cyclosporin A treatment enabled a reduction in the number of transfusions and the risk of hyperimmunization. After transplantation, our patient's hemoglobin level has remained normal and stable.