Outcomes of pediatric deceased donor kidney transplant in northeast Thailand.

BACKGROUND: Kidney transplantation (KT) is the best therapy in children with end-stage renal disease (ESRD), however, improving long-term graft survival remains challenging. The aim of this study was to determine graft survival and potential risk factors in pediatric patients who undergo deceased donor KT with a steroid-based regimen. METHODS: The medical records of children who underwent their first deceased donor KT in Srinagarind Hospital (Khon Kaen, Thailand) between 2001 and 2020 were reviewed. RESULTS: Seventy-two patients were studied. Male adolescents were the predominant recipients and the majority of donors were young adult males. Non-glomerular disease, particularly hypoplastic/dysplastic kidney disease, was the major cause of ESRD (48.61%). The mean cold ischemic time (CIT) was 18.29 ± 5.29 h. Most of the recipients had more than 4 human leukocyte antigen (HLA) mismatched loci with positive HLA-DR mismatch (52.78%). Induction therapy was administered in 76.74% of recipients. Tacrolimus plus mycophenolate sodium and prednisolone was the most common immunosuppressive maintenance regimen (69.44%). Graft failure occurred in 18 patients, mostly due to graft rejection (50%). Graft survival at 1, 3, and 5 years after KT were 94.40%, 86.25%, and 74.92%, respectively. The only significant risk factor of graft failure in this study was delayed graft function (DGF) (adjusted HR = 3.55; 95%CI: 1.14, 11.12; p = .029). Patient survival at 1, 3, and 5 years was 100%, 98.48%, and 96.19%, respectively. CONCLUSION: The short-term outcomes of pediatric KT from deceased donors were satisfactory; however, prevention of DGF would result in better outcomes.

Clinical manifestations and pathological correlation of immunoglobulin A nephropathy in children.

BACKGROUND: IgA nephropathy in children has various clinical manifestations. Kidney biopsy is a gold standard for diagnosis by using Oxford classification 2016 with few studies about the correlation between clinical and pathology manifestations. This study aims to find these correlations at the time of diagnosis and during short-term follow-up. METHOD: In this retrospective cohort study, 47 pediatric patients who underwent renal biopsy from 2010 to 2021 in Thailand, were included. Oxford classification 2016 has been used to score patients' pathology. Univariate and multivariate associations have been used for correlation between clinical and pathologic parameters. RESULTS: The most common clinical manifestations were microscopic hematuria and proteinuria. There were 68% of children with mesangial hypercellularity (M1), 42% with segmental glomerulosclerosis (S1), 25% with moderate to severe crescent (C1/C2), 23% with endocapillary hypercellularity (E1), and 14% with moderate to a severe tubular atrophy/interstitial fibrosis (T1/T2). Microscopic hematuria was strongly associated with mesangial hypercellularity (M1) OR 7.14 (95%CI 1.83 - 27.88, p-value 0.005) and hypertension was strongly associated with segmental glomerulosclerosis (S1) adjusted OR 7.87 (95%CI 1.65 - 37.59, p-value 0.01). Intensive treatment was used more in the patients with tubular atrophy/interstitial fibrosis lesion on renal biopsy than other lesions from MEST-C scores OR 4.98 (95%CI 1.17-21.24, p-value 0.03). Furthermore, pulse methylprednisolone and cyclophosphamide were used in patients with crescentic lesions significantly than other lesions with OR 15.5 (95%CI 3.16- 75.93, p-value 0.001) and OR 5.75 (95%CI 1.31-25.29, p-value 0.021), respectively. CONCLUSION: Tubular atrophy/interstitial fibrosis and crescent lesions were correlated to intensive treatment in short-term outcomes.

Molecular Diagnosis of Solute Carrier Family 4 Member 1 (SLC4A1) Mutation-Related Autosomal Recessive Distal Renal Tubular Acidosis.

BACKGROUND: Two common mutations of the solute carrier family 4 member 1 (SLC4A1) gene, namely, Southeast Asian ovalocytosis (SAO) and band 3 Bangkok 1 (G701D), cause autosomal recessive distal renal tubular acidosis (AR dRTA) in ethnic Southeast Asian populations. In this study, we applied the high-resolution melting (HRM) method for screening of AR dRTA associated with SLC4A1 mutations in 10 new patients with unknown cause(s) of AR dRTA. METHODS: We analyzed SAO and G701D mutations in the patients and their family members using HRM. The results were confirmed by polymerase chain reaction-restriction fragment-length polymorphism (PCR-RFLP) and DNA sequencing techniques. RESULTS: All patients carried homozygous G701D mutation, whereas their family members had heterozygous G701D or homozygous wild-type. CONCLUSIONS: Homozygous G701D is a common cause of AR dRTA in ethnic Thai pediatric populations. HRM can be used as a rapid screening method for common SLC4A1 mutations that cause AR dRTA in Southeast Asian and other populations.

Lupus Anticoagulant-hypoprothrombinemia Syndrome (LAC-HPS) in Children With Systemic Lupus Erythematosus: Report of 3 Cases.

Lupus anticoagulant, also known as lupus antibody, is generally associated with thrombosis rather than bleeding events. Lupus anticoagulant-hypoprothrombinemia syndrome in children is rather rare but can lead to mild to life-threatening bleeding. Here, we report 3 cases of lupus anticoagulant-hypoprothrombinemia syndrome associated with systemic lupus erythematosus. They initially presented with mucocutaneous bleedings, and subsequently developed other symptoms fulfilling the laboratory criteria for systemic lupus erythematosus. Case 2 and 3 had significant epistaxis and intracerebral hemorrhage responded to systemic corticosteroid along with fresh frozen plasma. Three cases demonstrated acquired hypoprothrombinemia with no correction of mixing studies. Case 1 had low factor X level, which has never been reported previously. In all 3 cases, their coagulogram returned to normal level after corticosteroid treatment.

Enamel defect and gingival enlargement in pediatric patients with kidney disease at Srinagarind Hospital, Khon Kaen University, Thailand.

BACKGROUND: Although many complications from kidney disease therapy can be prevented or effectively treated, oral health problems are nevertheless a consequence. OBJECTIVE: The objective of this study was to explore the prevalence of enamel defect and gingival enlargement in pediatric patients with kidney disease at Srinagarind Hospital, Khon Kaen University, Thailand. MATERIAL AND METHOD: This cross-sectional study was conducted between January and August 2013, at SrinagarindHospital, Khon Kaen University. Ninety-seven pediatric patients with kidney disease were allowed by their parents to participate in this study. Data were collected from medical records, questionnaires and oral examination records. The enamel defect was recorded using the Developmental Defects of Enamel Index. Gingival enlargement was recorded using the GingivalEnlargement Index. An oral examination was conducted using a mouth mirror, explorer and periodontal probe. RESULTS: The average age of the pediatric patients with kidney disease was 11.53+3.7years (range, 4-17). The majority of subjects were able to (a) come for an appointment (97.9%), (b) take medication according to the medical directions (93.8%) and (c) avoid inappropriatefoodsfor those suffering kidney disease (84.5%). The prevalence of enamel defect was 27.8%. The most common enamel defects were demarcated opacities (13.4%) or diffuse opacities (9.3%). The prevalence ofgingival enlargement was 16.5%. CONCLUSION: This study revealed that the prevalence ofenamel defect was 27.8% and the prevalence ofgingival enlargement was 16.5%.

Risk factors and outcome of atypical acute post-streptococcal glomerulonephritis in pediatrics.

We conducted this study to identify the clinical features and risk factors for atypical acute post-streptococcal glomerulonephritis (APSGN). Thirty-five cases of atypical APSGN treated at Srinagarind Hospital during 2002-2009 were compared with 27 typical cases. The clinical symptoms, anti-streptococcal antibody titers, and laboratory data at the first hospital visit were compared between the two groups. A marked elevation in anti-streptolysin O (ASO) titer was seen more commonly in the atypical APSGN group than in the typical APSGN group (p=0.025). Significantly more patients in the atypical APSGN group had a high urine specific gravity, hematuria and pyuria than patients in the typical APSGN group (p<0.01, p<0.031, and p<0.046, respectively). A high ASO titer, high urine specific gravity, severe hematuria and pyuria early in the illness were suggestive of a higher risk for an atypical presentation.

Disseminated histoplasmosis in a renal transplant child.

Disseminated histoplasmosis has occasionally been documented in solid organ transplant patients in some endemic areas. Early diagnosis and treatment are associated with good outcomes. In this report the authors describe the clinical characteristics and natural history of undiagnosed disseminated histoplasmosis in a child who underwent a cadaveric renal transplant at a tertiary healthcare center in northeastern Thailand.

Peritonitis in children receiving continuous ambulatory peritoneal dialysis in northeast Thailand.

OBJECTIVE: To clarify the peritonitis rate and to characterize the differences between the peritonitis and non-peritonitis group in ESRD children using continuous ambulatory peritoneal dialysis (CAPD) in Khon Kaen, Thailand. MATERIAL AND METHOD: The authors reviewed the medical records of ESRD children under 15 years old at the time of PD catheter placement, who received CAPD in Srinagarind Hospital, Faculty of Medicine, Khon Kaen University between 1994 and 2007. RESULTS: Eighteen male andfifteen female patients were identified Their mean age at the time of PD catheter placement was 11.48 +/- 3.12 years (range, 3.52-15.67). Twenty patients (11 male and 9female) were complicated with 47 episodes of peritonitis during 400.44 patient-months. The peritonitis rate was one episode every 8.52 patient-months. The three most frequent clinical presentations were cloudy effluent (78.72%), abdominal pain (76.60%) and fever (63.83%). Negative effluent culture was 42.55%. Staphylococcus aureus and Enterococcus species were the two most frequent causative organisms. The dialysis duration and serum creatinine level were significantly different between the peritonitis and non-peritonitis groups. CONCLUSION: Peritonitis frequently occurred in ESRD children treated by CAPD in Northeast Thailand, particularly from gram-positive organisms. The dialysis duration and serum creatinine level at the time of catheter placement were associated with PD peritonitis.

Pediatric renal transplantation: a single-center experience in northeast Thailand.

OBJECTIVE: To clarify the demographic data, outcomes and complications of renal transplantation in children at Srinagarind (university) Hospital. MATERIAL AND METHOD: The authors reviewed the medical records of children with end-stage renal disease (ESRD) who received renal transplantation at Srinagarind Hospital, Khon Kaen, between August 2001 and July 2008. RESULTS: Eight male and seven female patients were identified Their mean age was 12.8 +/- 3.2 years (range, 5.0-17.6). The major cause of ESRD was a congenital anomaly of the kidneys (53%). All of the children received cadaveric transplantations and none received induction therapy. Triple immunosuppressive drugs comprising cyclosporine, prednisolone and mycophenolate mofetil were administered to 12 patients. Tacrolimus, instead of cyclosporine, was given to three patients who had received a renal transplant since January 2008. The median follow-up time was 15 months (3 to 82 months). The most frequent complication was urinary tract infection (40%). Acute graft loss was found in one patient (6.7%) due to graft infarction. Other complications included herpes viral infection, chronic rejection, acute rejection, severe gingival hyperplasia, myopathy, lymphocele and transitional cell carcinoma of the bladder. Two patients returned to dialysis due to graft infarction and chronic rejection, respectively. The mean serum creatinine at the last follow-up of the remaining cases was 1.2 +/- 0.5 mg/dL (range, 0.6-2.3). All of the patients survived. The 1- and 5-year graft survival rates were 93.3% and 86.7%, respectively. CONCLUSION: The present study demonstrates the potential for successful outcomes of pediatric renal transplantation in this resource-limited area.

Complications of peritoneal dialysis in children with Eagle-Barrett syndrome.

Eagle Barrett syndrome (EBS) is characterized by the triad of abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism. Approximately 25% of patients with EBS progress to end-stage renal disease. It is speculated that the abdominal muscular defects in EBS pose technical problems in achieving successful peritoneal dialysis (PD). In this retrospective analysis, we reviewed the medical records of EBS and non-EBS PD patients cared for at Rainbow Babies and Children's Hospital from 1985 to 2002; 5 EBS and 9 non-EBS patients were analyzed. PD duration, total complication rates, and catheter usage rates in the two groups were not significantly different. The two most frequent complications were peritonitis and catheter mechanical malfunction during 103 patient-months in EBS patients and 296 patient-months in non-EBS patients. Peritonitis occurred 1 episode every 20.6 patient-months and 14.8 patient-months in EBS and non-EBS patients, respectively. The time from PD initiation to onset of any complication, including first peritonitis, was not significantly different in the two groups. Although the age at PD initiation was significantly different between the groups, there was no correlation between age at onset of PD and complication rates or time to first complication. Despite their abdominal muscle defects, EBS patients do not have more-frequent PD complications.