Prayong Vachvanichsanong - Academia.edu (original) (raw)

Papers by Prayong Vachvanichsanong

Research paper thumbnail of Hyperlipidaemia-induced pancreatitis and infected intra-abdominal cysts in a boy with systemic lupus erythematosus: a case report

Scandinavian Journal of Rheumatology, Jul 24, 2023

Research paper thumbnail of Impaired differential renal function in a child with pheochromocytoma

Journal of Human Hypertension, Jun 2, 2005

We report a girl with extra-adrenal pheochromocytoma complicated with impaired renal function of ... more We report a girl with extra-adrenal pheochromocytoma complicated with impaired renal function of the ipsilateral kidney, discussing aetiologies. A 14-year-old girl presented with uncontrolled hypertension, high urinary vanillylmandelic acid level and a 5 Â 6 cm mass at the right renal hilum. Her blood pressure was under control with propranolol, prazosin, and nifedipine before surgery, and with sodium nitroprusside during surgical intervention. The total tumour removal required reconstruction of the right renal vein. Histopathology confirmed pheochromocytoma. Dimercaptosuccinic acid (DMSA) after surgery showed low uptake of isotope by the right kidney; it was unclear if this was due to the surgery or the tumour causing renal artery stenosis, but fortunately her blood pressure returned to normal thereafter. We recommend obtaining a DMSA in the preoperative evaluation of pheochromocytoma.

Research paper thumbnail of Multi-population genome-wide association study implicates immune and non-immune factors in pediatric steroid-sensitive nephrotic syndrome

Nature Communications, Apr 29, 2023

Pediatric steroid-sensitive nephrotic syndrome (pSSNS) is the most common childhood glomerular di... more Pediatric steroid-sensitive nephrotic syndrome (pSSNS) is the most common childhood glomerular disease. Previous genome-wide association studies (GWAS) identified a risk locus in the HLA Class II region and three additional independent risk loci. But the genetic architecture of pSSNS, and its genetically driven pathobiology, is largely unknown. Here, we conduct a multi-population GWAS meta-analysis in 38,463 participants (2440 cases). We then conduct conditional analyses and population specific GWAS. We discover twelve significant associations-eight from the multi-population meta-analysis (four novel), two from the multi-population conditional analysis (one novel), and two additional novel loci from the European meta-analysis. Fine-mapping implicates specific amino acid haplotypes in HLA-DQA1 and HLA-DQB1 driving the HLA Class II risk locus. Non-HLA loci colocalize with eQTLs of monocytes and numerous T-cell subsets in independent datasets. Colocalization with kidney eQTLs is lacking but overlap with kidney cell open chromatin suggests an uncharacterized disease mechanism in kidney cells. A polygenic risk score (PRS) associates with earlier disease onset. Altogether, these discoveries expand our knowledge of pSSNS genetic architecture across populations and provide cell-specific insights into its molecular drivers. Evaluating these associations in additional cohorts will refine our understanding of population specificity, heterogeneity, and clinical and molecular associations. Pediatric steroid-sensitive nephrotic syndrome (pSSNS) is a rare disease of the glomerular filtration barrier. Its incidence ranges from 1.15-16.9 cases in every 100,000 children, occurring most frequently in South Asian populations 1. pSSNS causes massive proteinuria and increases the risk of thromboembolism, sepsis, and progression to chronic kidney disease (CKD)/end-stage kidney disease (ESKD) 2-7. And those progressing to ESKD have increased odds of recurrent NS in their allograft 8. pSSNS is impactful across the lifespan-31-50% of those affected have relapses in adulthood 9. Much of pSSNS's morbidity is related to side effects of the non-specific immunosuppressants which allow some to achieve remission of their proteinuria 7, 10-17. Despite intensive investigation, there are no known monogenic forms of pSSNS to illuminate its pathobiology. However, we know that immune dysregulation is a major contributor 18,19. But determining causal immune factors via case-control studies of cytokines profiles, cell types, and transcriptomic signatures is challenging. The dynamic responses of the immune system at different disease stages and to various stimuli make it difficult to determine whether observed differences are causal, correlated, or due to independent biological/

Research paper thumbnail of Childhood‐onset systemic lupus erythematosus and immune thrombocytopenia: Prevalence and risk factors

Pediatric Blood & Cancer, May 27, 2021

BackgroundThere are few studies examining the prevalence and clinical risk factors for subsequent... more BackgroundThere are few studies examining the prevalence and clinical risk factors for subsequent systemic lupus erythematosus (SLE) development after long‐term follow‐up in childhood immune thrombocytopenia (ITP). The aims of this study were to evaluate the prevalence and risk factors for subsequent SLE development in childhood ITP.MethodsThe medical records of childhood ITP patients aged under 15 years in a major tertiary care center in Southern Thailand were retrospectively reviewed. The Kaplan–Meier method was used to estimate the cumulative probability of subsequent SLE development after ITP. Logistic regression analysis was used to identify independent risk factors for SLE development.ResultsA total of 473 childhood ITP cases were included in the study. During a mean follow‐up time of 6.1 ± 6.7 years, the prevalence of subsequent SLE development was 2.96%. Older age at ITP diagnosis (odds ratio [OR]: 1.24, 95% CI: 1.07–1.45) and chronic ITP (OR: 24.67, 95% CI: 3.14–100.0) were independent risk factors. The cumulative probabilities of subsequently developing SLE at 5 and 10 years after diagnosis of ITP were 3.8% (95% CI: 1.4–6.2) and 6.5% (95% CI: 2.9‐9.8), respectively.ConclusionOlder age at ITP diagnosis and chronic ITP were risk factors for subsequent SLE developed in childhood ITP.

Research paper thumbnail of Peritoneal Dialysis-Related Peritonitis in Southern Thailand

The International Journal of Artificial Organs, 2008

Objective To evaluate peritonitis in children receiving peritoneal dialysis (PD) in southern Thai... more Objective To evaluate peritonitis in children receiving peritoneal dialysis (PD) in southern Thailand. Patients and Methods We reviewed the records of patients who received PD at the Department of Pediatrics, Prince of Songkla University from January 1994 to December 2003. Results Forty-six patients had PD performed for 228.6 patient-months. Their mean age was 9.5±4.8 years (range 3.0 months-16.7 years). Twenty-eight patients had complications with 61 episodes of peritonitis. The age, sex and causes of renal failure did not display any differences between patients who had or did not have peritonitis (p=0.2, 0.6 and 0.6, respectively). The first peritonitis occurrence was on average at 2.7±4.0 patient-months (median 0.4, range 0–12.5) following catheter insertion, with an average incidence rate of one infection per 3.5±4.8 patient-months (median 1.0, range 0–23.2). The causative agents were both gram-positive and gram-negative bacteria, and fungi at 24%, 44%, and 8% respectively (24%...

Research paper thumbnail of Differences in photoaffinity labeling of DA1 receptors in renal proximal tubules from normotensive rat and SHR

American Journal of Physiology-Renal Physiology, 1995

Renal DA1 dopamine receptors in proximal tubule membranes of normotensive Wistar-Kyoto (WKY) and ... more Renal DA1 dopamine receptors in proximal tubule membranes of normotensive Wistar-Kyoto (WKY) and spontaneously hypertensive rats (SHR) were characterized with the novel D1 dopamine receptor-selective photoaffinity probe, (+/-)-7-[125I]iodo-8-hydroxy-3-methyl-1-(4-azidophenyl)-2,3,4,5- tetrahydro-1H-3-benzazepine ([125I]MAB). Under nonphotolyzing conditions, saturation studies showed that [125I]MAB bound with similar affinity to DA1 dopamine receptors in both WKY [dissociation constant (Kd) = 16.3 nM] and SHR (Kd = 19.5 nM). At photolysis, [125I]MAB was irreversibly incorporated into a single major protein of 74,000 Da in both WKY and SHR. DA1-selective antagonists blocked photolabeling of DA1 sites with similar efficiency and specificity in SHR and WKY. However, under identical assay conditions, dopaminergic agonists were unable to block photoincorporation of [125I]MAB in SHR but not in WKY. This pattern of labeling of DA1 sites by [125I]MAB may suggest the presence of defective ago...

Research paper thumbnail of Multi-population genome-wide association study implicates both immune and non-immune factors in the etiology of pediatric steroid sensitive nephrotic syndrome

ABSTRACTPediatric steroid-sensitive nephrotic syndrome (pSSNS) is the most common childhood glome... more ABSTRACTPediatric steroid-sensitive nephrotic syndrome (pSSNS) is the most common childhood glomerular disease. Previous genome-wide association studies (GWAS) identified a risk locus in the HLA Class II region and three additional signals. But the genetic architecture of pSSNS, and its genetically driven pathobiology, is largely unknown. We conducted a multi-population GWAS meta-analysis in 38,463 participants (2,440 cases) and population specific GWAS, discovering twelve significant associations (eight novel). Fine-mapping implicated specific amino acid haplotypes in HLA-DQA1 and HLA-DQB1 driving the HLA Class II risk signal. Non-HLA loci colocalized with eQTLs of monocytes and numerous T-cell subsets in independent datasets. Colocalization with kidney eQTLs was lacking, but overlap with kidney cell open chromatin suggests an uncharacterized disease mechanism in kidney cells. A polygenic risk score (PRS) associated with earlier disease onset in two independent cohorts. Altogether,...

Research paper thumbnail of Electrolyte Disturbance and Kidney Dysfunction in Dengue Viral Infection

The Southeast Asian journal of tropical medicine and public health, 2015

Dengue virus infection (DVI) is endemic in tropical countries in both children and adults. The cl... more Dengue virus infection (DVI) is endemic in tropical countries in both children and adults. The classical presentation includes fever, hepatomegaly, thrombocytopenia-related bleeding disorders, and plasma leakage. Multi-organ involvement, including kidneys is found in complex cases. Asymptomatic electrolyte disturbances, abnormal urinalysis, and more severe manifestation such as acute kidney injury (AKI) usually indicate kidney involvement. Such manifestations are not rare in DVI, but are often not recognized and can cause the physician to misread the real situation of the patient. The prevalence of electrolyte disturbances or kidney involvement reported in studies varies widely by country and mainly depends on the severity of DVI and age of the patients. The prevalence of DVI-induced AKI ranges from 0.2%-10.0% in children and 2.2%-35.7% in adults. The prevalence among all age groups appears to be increasing in the last decade. Dengue shock syndrome (DSS) has been reported to be an i...

Research paper thumbnail of Acute kidney injury and childhood acute myeloid leukemia

Pediatric Nephrology, 2022

Acute kidney injury (AKI) appears to be particularly common in children with acute myeloid leukem... more Acute kidney injury (AKI) appears to be particularly common in children with acute myeloid leukemia (AML), although the epidemiology data on this patient population is sparse. The objective of this study was to assess the prevalence and factors associated with AKI in childhood AML during chemotherapy treatment. The medical records of 112 children aged under 15 years diagnosed with AML who received chemotherapy in a major tertiary-care referral center in southern Thailand were reviewed. Logistic regression was used to identify factors associated with AKI. Fifty-six (50%) children had AKI events. The median time from AML diagnosis to the first AKI was 29.5 days (interquartile range: 11.0–92.8) and the median follow-up time was 10.9 months (interquartile range: 3.6–31.1). Age at diagnosis ≥ 10 years (OR 2.75, 95% CI 1.09–6.93), glomerular filtration rate < 90 mL/min/1.73 m2 at AML diagnosis (OR 7.58, 95% CI 1.89–30.5), and septic shock (OR 22.0, 95% CI 4.63–104.3) were independently associated with AKI. Childhood AML has a high rate of kidney injury with 50% having AKI. Age ≥ 10 years at diagnosis, impaired kidney function before treatment, and septic shock were strongly associated with AKI. A higher resolution version of the Graphical abstract is available as Supplementary information.

Research paper thumbnail of Extended-spectrum beta-lactamase Escherichia coli and Klebsiella pneumoniae urinary tract infections

Epidemiology and Infection, 2020

The prevalence of extended-spectrum beta-lactamase (ESBL)-producing Escherichia coli and Klebsiel... more The prevalence of extended-spectrum beta-lactamase (ESBL)-producing Escherichia coli and Klebsiella pneumoniae urinary tract infections (UTIs) is increasing worldwide. We investigated the prevalence, clinical findings, impact and risk factors of ESBL E. coli/K. pneumoniae UTI through a retrospective review of the medical records of children with UTI aged <15 years admitted to Prince of Songkla University Hospital, Thailand over 10 years (2004–2013). Thirty-seven boys and 46 girls had ESBL-positive isolates in 102 UTI episodes, compared with 85 boys and 103 girls with non-ESBL isolates in 222 UTI episodes. The age of presentation and gender were not significantly different between the two groups. The prevalence of ESBL rose between 2004 and 2008 before plateauing at around 30–40% per year, with a significant difference between first and recurrent UTI episodes of 27.3% and 46.5%, respectively (P = 0.003). Fever prior to UTI diagnosis was found in 78.4% of episodes in the non-ESBL g...

Research paper thumbnail of A 30-year retrospective study on causes of death in childhood-onset systemic lupus erythematosus in a tertiary care centre in Southern Thailand

Clinical and experimental rheumatology, 2019

OBJECTIVES We set out to determine the causes of death in childhood-onset systemic lupus erythema... more OBJECTIVES We set out to determine the causes of death in childhood-onset systemic lupus erythematosus (cSLE). METHODS The medical records of children aged <18 years who were diagnosed with SLE from 1985 to 2016 in the Division of Nephrology, Department of Paediatrics, Faculty of Medicine, Prince of Songkla University, Thailand, were reviewed. RESULTS There was a total of 331 patients, 272 girls and 59 boys, of whom 77 (23.3%) died, 28.6% within the first year after diagnosis. Only 29 medical records of the 77 confirmed-death patients were available for evaluation of cause of death; 7 boys and 22 girls, with a mean age at presentation of 10.9±3.1 years. The mean follow-up duration was 4.6±3.7 (range 0.2-12.6) years. The major cause of death was sepsis (n=13 patients with 15 identified organisms, which were Acinetobacter baumannii (9), Escherichia coli (3), Candida albicans (2) and Aspergillosis (1)), followed by acute respiratory distress syndrome (ARDS) (6), severe heart conditi...

Research paper thumbnail of Extended - spectrum beta - lactamase E. coli and K. pneumoniae urinary tract infections

Background: The prevalences of extended-spectrum beta-lactamase (ESBL) Escherichia coli (E. coli)... more Background: The prevalences of extended-spectrum beta-lactamase (ESBL) Escherichia coli (E. coli) and Klebsiella pneumoniae (K. pneumoniae) urinary tract infections (UTI) in children are increasing worldwide. We aimed to investigate the prevalence, clinical findings, impact and risk factors of ESBL E. coli / K. pneumoniae UTI. Methods: The medical records of children with UTI aged <15 years admitted to Prince of Songkla University Hospital were reviewed. Results: Theirty-seven boys and 46 girls had ESBL in 102 UTI episodes; 85 boys and 103 girls had non-ESBL in all of their 222 UTI episodes. The median age at presentation was 1.5 (0.7-4.8) years for the ESBL group and 1.3 (0.6-3.9) for the non-ESBL group (p=0.2). Age and gender were not significantly different between the two groups. The prevalence of ESBL rose between 2004 and 2008 before plateauing at around 30-40% per year. The prevalences in first and recurrent UTI were 27.3% and 46.5%, respectively (p=0.003). Fever prior to UTI diagnosis was found in 78.4% of episodes in the non-ESBL group and 61.8% of episodes in the ESBL group (p=0.003). Multivariate analysis, children without fever (OR=2.14, 95% CI: 1.23-3.74) and those with recurrent UTI (OR=2.67, 95% CI: 1.37-5.19) were more likely to have ESBL UTI. The presence of CAKUT had no effect on ESBL UTI. Conclusions: ESBL was found in one-third of E. coli/K. pneumoniae UTI episodes. No clinical condition nor imaging study could predict ESBL. Recurrent UTI was the only independent risk factor.

Research paper thumbnail of Childhood‐onset systemic lupus erythematosus and immune thrombocytopenia: Prevalence and risk factors

Pediatric Blood & Cancer, 2021

There are few studies examining the prevalence and clinical risk factors for subsequent systemic ... more There are few studies examining the prevalence and clinical risk factors for subsequent systemic lupus erythematosus (SLE) development after long‐term follow‐up in childhood immune thrombocytopenia (ITP). The aims of this study were to evaluate the prevalence and risk factors for subsequent SLE development in childhood ITP.

Research paper thumbnail of PS10:187 Causes of death in childhood-onset systemic lupus erythematosus in a tertiary care centre, southern thailand

Poster session 10: Difficult cases, 2018

infectious or malignant aetiology, although the histological findings are typically non-specific ... more infectious or malignant aetiology, although the histological findings are typically non-specific for a diagnosis of lupusrelated pericarditis. Pericardectomy is likely to be required for definitive treatment.

Research paper thumbnail of Improvement of survival rates in the last decade in Thai childhood-onset systemic lupus erythematosus

Pediatric Rheumatology, 2018

Background: Morbidity and mortality in childhood onset systemic lupus erythematosus (SLE) is more... more Background: Morbidity and mortality in childhood onset systemic lupus erythematosus (SLE) is more severe than adult onset SLE. Long-term follow up is needed to determine the prognosis. The objectives of this study are to describe the mortality of childhood SLE in a single tertiary care centre over three decades, compare trends in survival over time, and determine predictors for survival. Methods: We retrospectively reviewed the medical records of children aged < 18 years who were diagnosed with SLE at the Department of Pediatrics, Songklanagarind Hospital, Thailand, from 1985 to 2016. Results: There were 331 children (272 girls, 59 boys) with a mean age at presentation of 11.5 ± 2.6 years. The mean follow-up duration was 7.0 ± 5.0 (range 1-28) years, 77 children (23.3%) died, 28.6% within the first year after diagnosis. The overall mortality rate was 3.3 per 100 person-years. Survival rates at 1, 5 and 10 years were 93.4%, 83.1% and 72.6%, respectively. Ten-year survival rates for the children diagnosed in the decades 1985-1996, 1997-2006 and 2007-2016 were 67.4%, 63.4% and 82.8%, respectively (p < 0.001). Boys had worse survival than girls (hazard ratio = 2.3, 95% CI: 1.4-3.7) even after adjusting for decade of diagnosis. Lupus nephritis (LN) class IV had similar survival compared to LN classes II/III/V combined (hazard ratio = 1.0, 95% CI: 0.6-1.7). Conclusion: In our setting, the survival rate of childhood onset SLE has improved during the past 10 years, but mortality is still high compared to developed countries, particularly in boys.

Research paper thumbnail of What Did We Find From Imaging Studies in Childhood Urinary Tract Infection and Which Studies Are Mandatory?

Urology, 2018

OBJECTIVE To evaluate the imaging results of childhood urinary tract infection (UTI) in our setti... more OBJECTIVE To evaluate the imaging results of childhood urinary tract infection (UTI) in our setting, and examine if it would be appropriate to apply the recent guideline changes regarding imaging studies as routine practice in Thailand. METHODS Medical records of children with UTI aged 0-15 years were reviewed, with focus on renal ultrasound (RUS), cystogram, and 99mTc dimercaptosuccinic acid (DMSA) renal scan results to determine congenital anomalies of the kidney and urinary tract (CAKUT) and renal damage. Mild CAKUT was defined as primary vesicoureteral reflux grades I-III or isolated hydronephrosis, and all other abnormalities were defined as severe CAKUT. RESULTS A total of 142 boys and 129 girls had at least 1 imaging study after UTI. Their median (interquartile range) age was 1.0 (0.5-2.7) year: 0.7 and 1.4 years for boys and girls, respectively (P = .006). A total of 262 children had an RUS performed, of which 99 (37.8%) were abnormal. Cystograms were performed in 221 children, from which 83 (37.6%) CAKUTs were detected, and 108 children had a DMSA performed, of which 53 (49.1%) were abnormal. Overall, CAKUTs were detected in 148 (54.6%) children, of which 43 were severe. RUS together with cystogram provided higher sensitivity (100% vs 88.9%) and specificity (53.8% vs 42.4%) to detect severe CAKUT than RUS together with DMSA. CONCLUSION A CAKUT was detected in more than half of the children with first UTI, with one-third having severe CAKUT. In our setting, RUS combined with cystogram is still the most reliable way to detect potentially harmful post-UTI problems, and the new western guidelines are not appropriate.

Research paper thumbnail of OC-48 Survival of childhood onset sle in a single centre

Oral Communications, 2017

Introduction Systemic lupus erythematosus (SLE), one of the most common connective tissue disease... more Introduction Systemic lupus erythematosus (SLE), one of the most common connective tissue diseases in children, causes high morbidity and mortality. Childhood onset SLE is more severe than adult onset SLE. Advances in medical technology and management have improved SLE outcomes; however there has been no recent study to investigate long-term outcomes, including gender-specific ones, in a large single group of children. Objective To describe the mortality of childhood SLE in a single tertiary care centre in southern Thailand over a 30 year period, compare trends in survival over time and determine predictors for survival. Methods We retrospectively reviewed the medical records of children (aged<15 years) who were diagnosed with SLE at the Department of Paediatrics, Songklanagarind Hospital, from 1985–2014 and were followed up for at least 2 years. Gender-specific mortality and survival rates were compared across three decades, and survival rates compared for different LN classifications. Results The study included 322 children (263 girls, 59 boys) having a mean age at presentation of 11.5±2.6 years. During a mean follow-up of 6.9±5.1 (range 2–28) years, 77 children (23.9%) died, 28.6% within the first year. Renal biopsy results in 249 children (77.3%) showed LN classes I, II, III, IV, and V in 10, 68, 11, 138, and 20 children, respectively. The overall mortality rate was 3.5 per 100 person years. Survival rates at 1, 5 and 10 years were 93.1%, 82.4% and 71.6%, respectively. Ten-year survival rates for children diagnosed in the decades 1985–1994, 1995–2004 and 2005–2014 were 67.4%, 63.2% and 80.6%, respectively (p<0.001). Boys had worse survival than girls (hazard ratio=2.3, 95% CI: 1.4–3.7) even after adjusting for decade of diagnosis. There was no difference in survival among the LN classes (p=0.99). Conclusion In our setting, the survival rate of childhood onset SLE has improved over the past 10 years, but mortality is still high compared to developed countries, particularly in boys. Deaths occurred within the first year of presentation more than in subsequent years. Severity of lupus nephritis did not determine survival.

Research paper thumbnail of Diffuse

proliferative glomerulonephritis does not determine the worst outcome in childhood onset lupus ne... more proliferative glomerulonephritis does not determine the worst outcome in childhood onset lupus nephritis: a 23-year experience in a single centre

Research paper thumbnail of In Reply: Re

The Journal of Urology, 1995

Research paper thumbnail of FRI0359 Intravenous cyclophosphamide combined therapy in severe lupus nephritis: Immediate and long-term results

Annals of the Rheumatic Diseases, 2012

Background Intravenous cyclophosphamide (IVCY) has been used to treat severe lupus nephritis (LN)... more Background Intravenous cyclophosphamide (IVCY) has been used to treat severe lupus nephritis (LN) for many years. Because of the wide variety of manifestations of the condition and the long-term nature of the disease, outcomes vary widely. Objectives To evaluate and compare the immediate and long-term results of IVCY in pediatric onset severe LN and between patients with normal and abnormal renal function. Patients and Methods The records of patients aged <18 years who attended the Department of Pediatrics, Prince of Songkla University, diagnosed with severe LN, and who were given a 36-month IVCY course, were reviewed. Results 108 patients were studied with a mean age of 12.6±2.7 years and mean follow up time of 5.7±4.3 years. 48 patients completed the 36-month IVCY course, 29 did not complete the course, and 31 were ongoing at the time of analysis. There were 36 patients with abnormal renal function at the start of therapy and 72 with normal renal function. Abnormal renal function patients had a higher mortality rate than normal renal function patients (13/36 vs 10/72, p-value<0.001). Both groups responded well initially to treatment; proteinuria reduced to normal levels after 1 and 2 courses in the normal and abnormal renal function groups, respectively, while creatinine clearance returned to normal levels after 8 courses in patients with abnormal renal function. Conclusions Three years of IVCY treatment reduced proteinuria in both normal and abnormal renal function patients; however the mortality rate was higher in patients with abnormal renal function. Disclosure of Interest None Declared

Research paper thumbnail of Hyperlipidaemia-induced pancreatitis and infected intra-abdominal cysts in a boy with systemic lupus erythematosus: a case report

Scandinavian Journal of Rheumatology, Jul 24, 2023

Research paper thumbnail of Impaired differential renal function in a child with pheochromocytoma

Journal of Human Hypertension, Jun 2, 2005

We report a girl with extra-adrenal pheochromocytoma complicated with impaired renal function of ... more We report a girl with extra-adrenal pheochromocytoma complicated with impaired renal function of the ipsilateral kidney, discussing aetiologies. A 14-year-old girl presented with uncontrolled hypertension, high urinary vanillylmandelic acid level and a 5 Â 6 cm mass at the right renal hilum. Her blood pressure was under control with propranolol, prazosin, and nifedipine before surgery, and with sodium nitroprusside during surgical intervention. The total tumour removal required reconstruction of the right renal vein. Histopathology confirmed pheochromocytoma. Dimercaptosuccinic acid (DMSA) after surgery showed low uptake of isotope by the right kidney; it was unclear if this was due to the surgery or the tumour causing renal artery stenosis, but fortunately her blood pressure returned to normal thereafter. We recommend obtaining a DMSA in the preoperative evaluation of pheochromocytoma.

Research paper thumbnail of Multi-population genome-wide association study implicates immune and non-immune factors in pediatric steroid-sensitive nephrotic syndrome

Nature Communications, Apr 29, 2023

Pediatric steroid-sensitive nephrotic syndrome (pSSNS) is the most common childhood glomerular di... more Pediatric steroid-sensitive nephrotic syndrome (pSSNS) is the most common childhood glomerular disease. Previous genome-wide association studies (GWAS) identified a risk locus in the HLA Class II region and three additional independent risk loci. But the genetic architecture of pSSNS, and its genetically driven pathobiology, is largely unknown. Here, we conduct a multi-population GWAS meta-analysis in 38,463 participants (2440 cases). We then conduct conditional analyses and population specific GWAS. We discover twelve significant associations-eight from the multi-population meta-analysis (four novel), two from the multi-population conditional analysis (one novel), and two additional novel loci from the European meta-analysis. Fine-mapping implicates specific amino acid haplotypes in HLA-DQA1 and HLA-DQB1 driving the HLA Class II risk locus. Non-HLA loci colocalize with eQTLs of monocytes and numerous T-cell subsets in independent datasets. Colocalization with kidney eQTLs is lacking but overlap with kidney cell open chromatin suggests an uncharacterized disease mechanism in kidney cells. A polygenic risk score (PRS) associates with earlier disease onset. Altogether, these discoveries expand our knowledge of pSSNS genetic architecture across populations and provide cell-specific insights into its molecular drivers. Evaluating these associations in additional cohorts will refine our understanding of population specificity, heterogeneity, and clinical and molecular associations. Pediatric steroid-sensitive nephrotic syndrome (pSSNS) is a rare disease of the glomerular filtration barrier. Its incidence ranges from 1.15-16.9 cases in every 100,000 children, occurring most frequently in South Asian populations 1. pSSNS causes massive proteinuria and increases the risk of thromboembolism, sepsis, and progression to chronic kidney disease (CKD)/end-stage kidney disease (ESKD) 2-7. And those progressing to ESKD have increased odds of recurrent NS in their allograft 8. pSSNS is impactful across the lifespan-31-50% of those affected have relapses in adulthood 9. Much of pSSNS's morbidity is related to side effects of the non-specific immunosuppressants which allow some to achieve remission of their proteinuria 7, 10-17. Despite intensive investigation, there are no known monogenic forms of pSSNS to illuminate its pathobiology. However, we know that immune dysregulation is a major contributor 18,19. But determining causal immune factors via case-control studies of cytokines profiles, cell types, and transcriptomic signatures is challenging. The dynamic responses of the immune system at different disease stages and to various stimuli make it difficult to determine whether observed differences are causal, correlated, or due to independent biological/

Research paper thumbnail of Childhood‐onset systemic lupus erythematosus and immune thrombocytopenia: Prevalence and risk factors

Pediatric Blood & Cancer, May 27, 2021

BackgroundThere are few studies examining the prevalence and clinical risk factors for subsequent... more BackgroundThere are few studies examining the prevalence and clinical risk factors for subsequent systemic lupus erythematosus (SLE) development after long‐term follow‐up in childhood immune thrombocytopenia (ITP). The aims of this study were to evaluate the prevalence and risk factors for subsequent SLE development in childhood ITP.MethodsThe medical records of childhood ITP patients aged under 15 years in a major tertiary care center in Southern Thailand were retrospectively reviewed. The Kaplan–Meier method was used to estimate the cumulative probability of subsequent SLE development after ITP. Logistic regression analysis was used to identify independent risk factors for SLE development.ResultsA total of 473 childhood ITP cases were included in the study. During a mean follow‐up time of 6.1 ± 6.7 years, the prevalence of subsequent SLE development was 2.96%. Older age at ITP diagnosis (odds ratio [OR]: 1.24, 95% CI: 1.07–1.45) and chronic ITP (OR: 24.67, 95% CI: 3.14–100.0) were independent risk factors. The cumulative probabilities of subsequently developing SLE at 5 and 10 years after diagnosis of ITP were 3.8% (95% CI: 1.4–6.2) and 6.5% (95% CI: 2.9‐9.8), respectively.ConclusionOlder age at ITP diagnosis and chronic ITP were risk factors for subsequent SLE developed in childhood ITP.

Research paper thumbnail of Peritoneal Dialysis-Related Peritonitis in Southern Thailand

The International Journal of Artificial Organs, 2008

Objective To evaluate peritonitis in children receiving peritoneal dialysis (PD) in southern Thai... more Objective To evaluate peritonitis in children receiving peritoneal dialysis (PD) in southern Thailand. Patients and Methods We reviewed the records of patients who received PD at the Department of Pediatrics, Prince of Songkla University from January 1994 to December 2003. Results Forty-six patients had PD performed for 228.6 patient-months. Their mean age was 9.5±4.8 years (range 3.0 months-16.7 years). Twenty-eight patients had complications with 61 episodes of peritonitis. The age, sex and causes of renal failure did not display any differences between patients who had or did not have peritonitis (p=0.2, 0.6 and 0.6, respectively). The first peritonitis occurrence was on average at 2.7±4.0 patient-months (median 0.4, range 0–12.5) following catheter insertion, with an average incidence rate of one infection per 3.5±4.8 patient-months (median 1.0, range 0–23.2). The causative agents were both gram-positive and gram-negative bacteria, and fungi at 24%, 44%, and 8% respectively (24%...

Research paper thumbnail of Differences in photoaffinity labeling of DA1 receptors in renal proximal tubules from normotensive rat and SHR

American Journal of Physiology-Renal Physiology, 1995

Renal DA1 dopamine receptors in proximal tubule membranes of normotensive Wistar-Kyoto (WKY) and ... more Renal DA1 dopamine receptors in proximal tubule membranes of normotensive Wistar-Kyoto (WKY) and spontaneously hypertensive rats (SHR) were characterized with the novel D1 dopamine receptor-selective photoaffinity probe, (+/-)-7-[125I]iodo-8-hydroxy-3-methyl-1-(4-azidophenyl)-2,3,4,5- tetrahydro-1H-3-benzazepine ([125I]MAB). Under nonphotolyzing conditions, saturation studies showed that [125I]MAB bound with similar affinity to DA1 dopamine receptors in both WKY [dissociation constant (Kd) = 16.3 nM] and SHR (Kd = 19.5 nM). At photolysis, [125I]MAB was irreversibly incorporated into a single major protein of 74,000 Da in both WKY and SHR. DA1-selective antagonists blocked photolabeling of DA1 sites with similar efficiency and specificity in SHR and WKY. However, under identical assay conditions, dopaminergic agonists were unable to block photoincorporation of [125I]MAB in SHR but not in WKY. This pattern of labeling of DA1 sites by [125I]MAB may suggest the presence of defective ago...

Research paper thumbnail of Multi-population genome-wide association study implicates both immune and non-immune factors in the etiology of pediatric steroid sensitive nephrotic syndrome

ABSTRACTPediatric steroid-sensitive nephrotic syndrome (pSSNS) is the most common childhood glome... more ABSTRACTPediatric steroid-sensitive nephrotic syndrome (pSSNS) is the most common childhood glomerular disease. Previous genome-wide association studies (GWAS) identified a risk locus in the HLA Class II region and three additional signals. But the genetic architecture of pSSNS, and its genetically driven pathobiology, is largely unknown. We conducted a multi-population GWAS meta-analysis in 38,463 participants (2,440 cases) and population specific GWAS, discovering twelve significant associations (eight novel). Fine-mapping implicated specific amino acid haplotypes in HLA-DQA1 and HLA-DQB1 driving the HLA Class II risk signal. Non-HLA loci colocalized with eQTLs of monocytes and numerous T-cell subsets in independent datasets. Colocalization with kidney eQTLs was lacking, but overlap with kidney cell open chromatin suggests an uncharacterized disease mechanism in kidney cells. A polygenic risk score (PRS) associated with earlier disease onset in two independent cohorts. Altogether,...

Research paper thumbnail of Electrolyte Disturbance and Kidney Dysfunction in Dengue Viral Infection

The Southeast Asian journal of tropical medicine and public health, 2015

Dengue virus infection (DVI) is endemic in tropical countries in both children and adults. The cl... more Dengue virus infection (DVI) is endemic in tropical countries in both children and adults. The classical presentation includes fever, hepatomegaly, thrombocytopenia-related bleeding disorders, and plasma leakage. Multi-organ involvement, including kidneys is found in complex cases. Asymptomatic electrolyte disturbances, abnormal urinalysis, and more severe manifestation such as acute kidney injury (AKI) usually indicate kidney involvement. Such manifestations are not rare in DVI, but are often not recognized and can cause the physician to misread the real situation of the patient. The prevalence of electrolyte disturbances or kidney involvement reported in studies varies widely by country and mainly depends on the severity of DVI and age of the patients. The prevalence of DVI-induced AKI ranges from 0.2%-10.0% in children and 2.2%-35.7% in adults. The prevalence among all age groups appears to be increasing in the last decade. Dengue shock syndrome (DSS) has been reported to be an i...

Research paper thumbnail of Acute kidney injury and childhood acute myeloid leukemia

Pediatric Nephrology, 2022

Acute kidney injury (AKI) appears to be particularly common in children with acute myeloid leukem... more Acute kidney injury (AKI) appears to be particularly common in children with acute myeloid leukemia (AML), although the epidemiology data on this patient population is sparse. The objective of this study was to assess the prevalence and factors associated with AKI in childhood AML during chemotherapy treatment. The medical records of 112 children aged under 15 years diagnosed with AML who received chemotherapy in a major tertiary-care referral center in southern Thailand were reviewed. Logistic regression was used to identify factors associated with AKI. Fifty-six (50%) children had AKI events. The median time from AML diagnosis to the first AKI was 29.5 days (interquartile range: 11.0–92.8) and the median follow-up time was 10.9 months (interquartile range: 3.6–31.1). Age at diagnosis ≥ 10 years (OR 2.75, 95% CI 1.09–6.93), glomerular filtration rate < 90 mL/min/1.73 m2 at AML diagnosis (OR 7.58, 95% CI 1.89–30.5), and septic shock (OR 22.0, 95% CI 4.63–104.3) were independently associated with AKI. Childhood AML has a high rate of kidney injury with 50% having AKI. Age ≥ 10 years at diagnosis, impaired kidney function before treatment, and septic shock were strongly associated with AKI. A higher resolution version of the Graphical abstract is available as Supplementary information.

Research paper thumbnail of Extended-spectrum beta-lactamase Escherichia coli and Klebsiella pneumoniae urinary tract infections

Epidemiology and Infection, 2020

The prevalence of extended-spectrum beta-lactamase (ESBL)-producing Escherichia coli and Klebsiel... more The prevalence of extended-spectrum beta-lactamase (ESBL)-producing Escherichia coli and Klebsiella pneumoniae urinary tract infections (UTIs) is increasing worldwide. We investigated the prevalence, clinical findings, impact and risk factors of ESBL E. coli/K. pneumoniae UTI through a retrospective review of the medical records of children with UTI aged <15 years admitted to Prince of Songkla University Hospital, Thailand over 10 years (2004–2013). Thirty-seven boys and 46 girls had ESBL-positive isolates in 102 UTI episodes, compared with 85 boys and 103 girls with non-ESBL isolates in 222 UTI episodes. The age of presentation and gender were not significantly different between the two groups. The prevalence of ESBL rose between 2004 and 2008 before plateauing at around 30–40% per year, with a significant difference between first and recurrent UTI episodes of 27.3% and 46.5%, respectively (P = 0.003). Fever prior to UTI diagnosis was found in 78.4% of episodes in the non-ESBL g...

Research paper thumbnail of A 30-year retrospective study on causes of death in childhood-onset systemic lupus erythematosus in a tertiary care centre in Southern Thailand

Clinical and experimental rheumatology, 2019

OBJECTIVES We set out to determine the causes of death in childhood-onset systemic lupus erythema... more OBJECTIVES We set out to determine the causes of death in childhood-onset systemic lupus erythematosus (cSLE). METHODS The medical records of children aged <18 years who were diagnosed with SLE from 1985 to 2016 in the Division of Nephrology, Department of Paediatrics, Faculty of Medicine, Prince of Songkla University, Thailand, were reviewed. RESULTS There was a total of 331 patients, 272 girls and 59 boys, of whom 77 (23.3%) died, 28.6% within the first year after diagnosis. Only 29 medical records of the 77 confirmed-death patients were available for evaluation of cause of death; 7 boys and 22 girls, with a mean age at presentation of 10.9±3.1 years. The mean follow-up duration was 4.6±3.7 (range 0.2-12.6) years. The major cause of death was sepsis (n=13 patients with 15 identified organisms, which were Acinetobacter baumannii (9), Escherichia coli (3), Candida albicans (2) and Aspergillosis (1)), followed by acute respiratory distress syndrome (ARDS) (6), severe heart conditi...

Research paper thumbnail of Extended - spectrum beta - lactamase E. coli and K. pneumoniae urinary tract infections

Background: The prevalences of extended-spectrum beta-lactamase (ESBL) Escherichia coli (E. coli)... more Background: The prevalences of extended-spectrum beta-lactamase (ESBL) Escherichia coli (E. coli) and Klebsiella pneumoniae (K. pneumoniae) urinary tract infections (UTI) in children are increasing worldwide. We aimed to investigate the prevalence, clinical findings, impact and risk factors of ESBL E. coli / K. pneumoniae UTI. Methods: The medical records of children with UTI aged <15 years admitted to Prince of Songkla University Hospital were reviewed. Results: Theirty-seven boys and 46 girls had ESBL in 102 UTI episodes; 85 boys and 103 girls had non-ESBL in all of their 222 UTI episodes. The median age at presentation was 1.5 (0.7-4.8) years for the ESBL group and 1.3 (0.6-3.9) for the non-ESBL group (p=0.2). Age and gender were not significantly different between the two groups. The prevalence of ESBL rose between 2004 and 2008 before plateauing at around 30-40% per year. The prevalences in first and recurrent UTI were 27.3% and 46.5%, respectively (p=0.003). Fever prior to UTI diagnosis was found in 78.4% of episodes in the non-ESBL group and 61.8% of episodes in the ESBL group (p=0.003). Multivariate analysis, children without fever (OR=2.14, 95% CI: 1.23-3.74) and those with recurrent UTI (OR=2.67, 95% CI: 1.37-5.19) were more likely to have ESBL UTI. The presence of CAKUT had no effect on ESBL UTI. Conclusions: ESBL was found in one-third of E. coli/K. pneumoniae UTI episodes. No clinical condition nor imaging study could predict ESBL. Recurrent UTI was the only independent risk factor.

Research paper thumbnail of Childhood‐onset systemic lupus erythematosus and immune thrombocytopenia: Prevalence and risk factors

Pediatric Blood & Cancer, 2021

There are few studies examining the prevalence and clinical risk factors for subsequent systemic ... more There are few studies examining the prevalence and clinical risk factors for subsequent systemic lupus erythematosus (SLE) development after long‐term follow‐up in childhood immune thrombocytopenia (ITP). The aims of this study were to evaluate the prevalence and risk factors for subsequent SLE development in childhood ITP.

Research paper thumbnail of PS10:187 Causes of death in childhood-onset systemic lupus erythematosus in a tertiary care centre, southern thailand

Poster session 10: Difficult cases, 2018

infectious or malignant aetiology, although the histological findings are typically non-specific ... more infectious or malignant aetiology, although the histological findings are typically non-specific for a diagnosis of lupusrelated pericarditis. Pericardectomy is likely to be required for definitive treatment.

Research paper thumbnail of Improvement of survival rates in the last decade in Thai childhood-onset systemic lupus erythematosus

Pediatric Rheumatology, 2018

Background: Morbidity and mortality in childhood onset systemic lupus erythematosus (SLE) is more... more Background: Morbidity and mortality in childhood onset systemic lupus erythematosus (SLE) is more severe than adult onset SLE. Long-term follow up is needed to determine the prognosis. The objectives of this study are to describe the mortality of childhood SLE in a single tertiary care centre over three decades, compare trends in survival over time, and determine predictors for survival. Methods: We retrospectively reviewed the medical records of children aged < 18 years who were diagnosed with SLE at the Department of Pediatrics, Songklanagarind Hospital, Thailand, from 1985 to 2016. Results: There were 331 children (272 girls, 59 boys) with a mean age at presentation of 11.5 ± 2.6 years. The mean follow-up duration was 7.0 ± 5.0 (range 1-28) years, 77 children (23.3%) died, 28.6% within the first year after diagnosis. The overall mortality rate was 3.3 per 100 person-years. Survival rates at 1, 5 and 10 years were 93.4%, 83.1% and 72.6%, respectively. Ten-year survival rates for the children diagnosed in the decades 1985-1996, 1997-2006 and 2007-2016 were 67.4%, 63.4% and 82.8%, respectively (p < 0.001). Boys had worse survival than girls (hazard ratio = 2.3, 95% CI: 1.4-3.7) even after adjusting for decade of diagnosis. Lupus nephritis (LN) class IV had similar survival compared to LN classes II/III/V combined (hazard ratio = 1.0, 95% CI: 0.6-1.7). Conclusion: In our setting, the survival rate of childhood onset SLE has improved during the past 10 years, but mortality is still high compared to developed countries, particularly in boys.

Research paper thumbnail of What Did We Find From Imaging Studies in Childhood Urinary Tract Infection and Which Studies Are Mandatory?

Urology, 2018

OBJECTIVE To evaluate the imaging results of childhood urinary tract infection (UTI) in our setti... more OBJECTIVE To evaluate the imaging results of childhood urinary tract infection (UTI) in our setting, and examine if it would be appropriate to apply the recent guideline changes regarding imaging studies as routine practice in Thailand. METHODS Medical records of children with UTI aged 0-15 years were reviewed, with focus on renal ultrasound (RUS), cystogram, and 99mTc dimercaptosuccinic acid (DMSA) renal scan results to determine congenital anomalies of the kidney and urinary tract (CAKUT) and renal damage. Mild CAKUT was defined as primary vesicoureteral reflux grades I-III or isolated hydronephrosis, and all other abnormalities were defined as severe CAKUT. RESULTS A total of 142 boys and 129 girls had at least 1 imaging study after UTI. Their median (interquartile range) age was 1.0 (0.5-2.7) year: 0.7 and 1.4 years for boys and girls, respectively (P = .006). A total of 262 children had an RUS performed, of which 99 (37.8%) were abnormal. Cystograms were performed in 221 children, from which 83 (37.6%) CAKUTs were detected, and 108 children had a DMSA performed, of which 53 (49.1%) were abnormal. Overall, CAKUTs were detected in 148 (54.6%) children, of which 43 were severe. RUS together with cystogram provided higher sensitivity (100% vs 88.9%) and specificity (53.8% vs 42.4%) to detect severe CAKUT than RUS together with DMSA. CONCLUSION A CAKUT was detected in more than half of the children with first UTI, with one-third having severe CAKUT. In our setting, RUS combined with cystogram is still the most reliable way to detect potentially harmful post-UTI problems, and the new western guidelines are not appropriate.

Research paper thumbnail of OC-48 Survival of childhood onset sle in a single centre

Oral Communications, 2017

Introduction Systemic lupus erythematosus (SLE), one of the most common connective tissue disease... more Introduction Systemic lupus erythematosus (SLE), one of the most common connective tissue diseases in children, causes high morbidity and mortality. Childhood onset SLE is more severe than adult onset SLE. Advances in medical technology and management have improved SLE outcomes; however there has been no recent study to investigate long-term outcomes, including gender-specific ones, in a large single group of children. Objective To describe the mortality of childhood SLE in a single tertiary care centre in southern Thailand over a 30 year period, compare trends in survival over time and determine predictors for survival. Methods We retrospectively reviewed the medical records of children (aged<15 years) who were diagnosed with SLE at the Department of Paediatrics, Songklanagarind Hospital, from 1985–2014 and were followed up for at least 2 years. Gender-specific mortality and survival rates were compared across three decades, and survival rates compared for different LN classifications. Results The study included 322 children (263 girls, 59 boys) having a mean age at presentation of 11.5±2.6 years. During a mean follow-up of 6.9±5.1 (range 2–28) years, 77 children (23.9%) died, 28.6% within the first year. Renal biopsy results in 249 children (77.3%) showed LN classes I, II, III, IV, and V in 10, 68, 11, 138, and 20 children, respectively. The overall mortality rate was 3.5 per 100 person years. Survival rates at 1, 5 and 10 years were 93.1%, 82.4% and 71.6%, respectively. Ten-year survival rates for children diagnosed in the decades 1985–1994, 1995–2004 and 2005–2014 were 67.4%, 63.2% and 80.6%, respectively (p<0.001). Boys had worse survival than girls (hazard ratio=2.3, 95% CI: 1.4–3.7) even after adjusting for decade of diagnosis. There was no difference in survival among the LN classes (p=0.99). Conclusion In our setting, the survival rate of childhood onset SLE has improved over the past 10 years, but mortality is still high compared to developed countries, particularly in boys. Deaths occurred within the first year of presentation more than in subsequent years. Severity of lupus nephritis did not determine survival.

Research paper thumbnail of Diffuse

proliferative glomerulonephritis does not determine the worst outcome in childhood onset lupus ne... more proliferative glomerulonephritis does not determine the worst outcome in childhood onset lupus nephritis: a 23-year experience in a single centre

Research paper thumbnail of In Reply: Re

The Journal of Urology, 1995

Research paper thumbnail of FRI0359 Intravenous cyclophosphamide combined therapy in severe lupus nephritis: Immediate and long-term results

Annals of the Rheumatic Diseases, 2012

Background Intravenous cyclophosphamide (IVCY) has been used to treat severe lupus nephritis (LN)... more Background Intravenous cyclophosphamide (IVCY) has been used to treat severe lupus nephritis (LN) for many years. Because of the wide variety of manifestations of the condition and the long-term nature of the disease, outcomes vary widely. Objectives To evaluate and compare the immediate and long-term results of IVCY in pediatric onset severe LN and between patients with normal and abnormal renal function. Patients and Methods The records of patients aged <18 years who attended the Department of Pediatrics, Prince of Songkla University, diagnosed with severe LN, and who were given a 36-month IVCY course, were reviewed. Results 108 patients were studied with a mean age of 12.6±2.7 years and mean follow up time of 5.7±4.3 years. 48 patients completed the 36-month IVCY course, 29 did not complete the course, and 31 were ongoing at the time of analysis. There were 36 patients with abnormal renal function at the start of therapy and 72 with normal renal function. Abnormal renal function patients had a higher mortality rate than normal renal function patients (13/36 vs 10/72, p-value<0.001). Both groups responded well initially to treatment; proteinuria reduced to normal levels after 1 and 2 courses in the normal and abnormal renal function groups, respectively, while creatinine clearance returned to normal levels after 8 courses in patients with abnormal renal function. Conclusions Three years of IVCY treatment reduced proteinuria in both normal and abnormal renal function patients; however the mortality rate was higher in patients with abnormal renal function. Disclosure of Interest None Declared