Persistent hypertension in Thai children: etiologies and outcome.

A retrospective study was done in 66 children (0.21% of all admitted children) below the age of 18 years with persistent hypertension diagnosed at the Department of Pediatrics, Faculty of Medicine Siriraj Hospital from Jan 1999 to Dec 2003. Male to female ratio was 1.4:1 with 54.5% aged between 6-12 years old and 9.1% aged less than 1 year. Hypertension was found to be severe (BP more than the 99th percentile for age, sex and height) in 79.1% but most (78.6%) of the patients did not have symptoms related to hypertension. Chronic headaches were found in 10%, hypertensive encephalopathy in 8.6%, epistaxis in 1.4% and visual disturbance in 1.4%. The most common cause of hypertension was renal parenchymal diseases (62.7%) mainly lupus nephritis (26.9%), idiopathic nephrotic syndrome (16.4%) and chronic renal failure (16.4%). Other causes of hypertension included renovascular diseases (7.5%), drug-induced (7.5%), essential (7.5%), tumors (4.5%), coarctation of aorta (3.0%), bronchopulmonary dysplasia (3.0%), and pheochromocytoma (1.5%). Obesity and overweight (body mass index, BMI more than 25) was found in only 10 patients (15.1%).The proportion of children with BMI more than 25 was not different between essential and secondary hypertension (p = 0.15). Left ventricular hypertrophy was noted in 7.5%, hypertensive retinopathy in 3.0%, and hypertensive encephalopathy in 9.0%. One-third of the patients had normal BP within 1 month and another 47.0% had normal BP within 6 months of diagnosis. One-fifth of the patients also needed surgical intervention for specific underlying diseases. The authors suggest that since a large number of children with hypertension have secondary hypertension, intensive investigation and prompt management should be done in all. Obesity and overweight is not reliable in the differentiation between primary and secondary hypertension. Short term outcome of hypertension is good with medications and surgery in selected cases but long term outcome is still unknown.

The relation of vesicoureteral reflux and renal scarring in childhood urinary tract infection.

OBJECTIVE: Assess the relation of age and sex in vesico ureteral reflux (VUR) and renal scarring and the relation of VUR and renal scarring in childhood urinary tract infection. MATERIAL AND METHOD: A descriptive study of one hundred and twenty-six children who received renal cortical scintigraphy from 1st Jan 2000 to 31st Dec 2004 in the Department of Radiology, Faculty of Medicine Siriraj Hospital, was conducted. Ninety-three (50 males, 43 females) patients were diagnosed with urinary tract infections (UTIs) but only ninety-one of them had renal cortical scintigraphic results available. The male to female ratio was 1.16:1. The mean age of the patients was 4.33 years (SD +/- 4.17, range 7 days-16 years). During the 1st year of life the male to female ratio is 2.6:1. Fever, dysuria, and poor feeding were the most presenting signs and symptoms. Eighty-five (45 males, 40 females) patients received Voiding Cysto Urethro Gram (VCUG). RESULT: The authors did not find the correlation between the age groups and sex with VCUG results on right and left side, respectively (p = 0.856, p = 0.145, p = 0.77, p = 0.75). Ninety-one (49 males, 42 females) patients received DMSA renal scintigraphy. Fifty-two patients (57.1%) had abnormal DMSA renal scan results. However; the authors did not find the correlation between age groups and sex with DMSA renal scan results on the right and left kidneys, respectively. (p = 0.202, p = 0.416, p = 0.511, p = 0.791). The authors compared times of UTIs with and DMSA renal scintigraphy in each side of the kidney. Even though the authors did find the correlation between episodes of UTIs and abnormal DMSA on the left kidneys (p = 0.017), it was not found on the right kidneys (p = 0.081). There were 80 patients who received both VCUG and DMSA renal scintigraphy. The authors found the correlation between severity of VUR and abnormal DMSA results on right and left kidneys (p = 0.001, p = 0.01). CONCLUSION: The authors recommend that all children who have repeated UTI and/or VUR, irrespective of age and sex, should receive DMSA renal scintigraphy to detect renal scarring and follow up future complications.

Difficult-to-treat nephrotic syndrome: management and outcome.

A retrospective study was performed in 68 patients diagnosed as having idiopathic nephrotic syndrome with steroid-dependent, steroid-resistant or frequent relapse subtypes at the Department of Pediatrics, Siriraj Hospital during Jan 1996-Dec 2004. Male to female ratio was 3.3:1 and mean age (+/- SD) was 8.4 +/- 3.5 years. Mean follow up time (+/- SD) was 47.4 +/- 30.5 months. Renal biopsy was done in 60 patients, showing IgM nephropathy in 73.3%. Fifty-four patients (79.4%) received cyclophosphamide at a dose (+/- SD) of 2.2 +/- 0.5 mg/kg/d for 11.6 +/- 3.4 weeks. Negative proteinuria at 1 year was found in 70% and prednisolone was discontinued in 52%. Leucopenia was found in 9.2%. At last follow up, 34% of the patients were still in remission. Enalapril was prescribed in 50 patients for 12.4 +/- 10.0 months. Thirty-six patients also received cyclophosphamide. Remission at 1 year was achieved in 66% and prednisolone discontinued in 28%. Twelve patients (24%) were still in remission at last follow up. The results of 3 regimens: cyclophosphamide, enalapril, and cyclophosphamide plus enalapril were compared using chi-square test. Remission was significantly better in cyclophosphamide group (p = 0.014). Dipyridamole was prescribed in 14 patients due to thrombocytosis. Only 2 of 14 patients achieved remission although 11 patients received cyclophosphamide plus enalapril, and another 2 patients received only cyclophosphamide. Complications included hypertension (44%), cataract (40%), glaucoma (15%), short stature (17.6%), and obesity (5.9%). Recurrent infection was found in 69%, including dental caries (16.29%), urinary tract infection (14.7%), intestinal parasitic infestration (10.3%), respiratory tract infection (8.8%), and skin infection (7.4%). Chronic renal failure was found in 3 patients and portal vein thrombosis was found in 1 patient. We suggest that cyclophosphamide should be used as first line drug in difficult-to-treat nephrotic syndrome patients. Enalapril may be beneficial in some patients. Thrombocytosis may be associated with poor response to both medications. Difficult-to-treat patients also need long-term follow up and surveillance for complications due to disease and/or treatment.

Pediatric systemic lupus erythematosus in Siriraj Hospital.

A descriptive study of one hundred and one pediatric patients with systemic lupus erythematosus treated between July 1985 and March 2003 in Department of Pediatrics, Faculty of Medicine Siriraj Hospital was conducted. According to existing database, there were a total of 181 patients, 101 of them (55.8%) had available data for review. The female to male ratio was 6.2:1. The mean and median ages of onset were 9.7 +/- 2.8 and 10 +/- 2.2 years, respectively (range 4-14 years). The clinical presentations were renal involvement in 87 patients (86.2%), skin and mucocutaneous involvement 77 patients (76.3%), hematological abnormalities 74 patients (73.4%), musculoskeletal involvement 32 patients (31.7%), prolongedfever 24 patients (23.8%), neuropsychiatric symptoms 21 patients (20.8%), gastrointestinal involvement 20 patients (19.8%), cardiac involvement 14 patients (13.9%), lymphadenopathy 13 patients (12.9%), and pulmonary involvement 7 patients (6.9%). The most common renal, skin and mucocutaneous, and hematological manifestations were proteinuria, malar rash, and anemia, respectively. Lupus nephritis with WHO class IV was the most common histopathological finding of the initial renal biopsies. The most common neuropsychiatric, gastrointestinal, cardiac, and pulmonary involvements were seizure, hepatomegaly, pericarditis, and pleuritis, respectively. Ninety-two percent of patients reported as having significant ANA positive results using rat liver tissue as a substrate. Sixty-six out of 94 patients (70.2%) had positive test result of Anti-dsDNA. In conclusion, the age at onset, clinical manifestations and laboratory investigation results of SLE in children at Siriraj Hospital are comparable to other studies in the Country and also to other Asian and Western studies.

Systemic lupus erythematosus in Thai children: clinicopathologic findings and outcome in 82 patients.

OBJECTIVES: To define the patterns of clinicopathologic findings and to identify the risk factors for renal failure and mortality of childhood-onset systemic lupus erythematosus (SLE) in Thailand. MATERIAL AND METHOD: The study is a retrospective analysis of clinical manifestations, laboratory data, and pathologic findings, treatment modalities, and outcome of 82 patients with biopsy-proven lupus nephritis (LN) with disease onset between I January 1987 and 31 December 1997. All children developed these first manifestations at the age 13 years or under RESULTS: Sixty-four (789%) patients were females and eighteen (22%) were males (ratio female/male = 3.5:1). The patients were followed for a mean period of 53.6 months (range 1 -141). The mean age at disease onset was 9.2 years (range 2-12.6). Class-IV LN, observed in 40 (48.8%) patients, was the most frequent histopathology on initial renal biopsy. Less frequent findings were class-II (30.5%), V (14.6%), I (3.7%) and III (2.4%) LN. Based on the renal histopathology and clinical presentations, patients were treated with corticosteroids alone or in combination with azathioprine or with intravenous cyclophosphamide (CYC). Methylprednisolone pulses were given in patients with clinically more severe disease. Follow-up biopsies, performed in 12 patients, showed no change in 4 patients, and were progressive in 8 patients. On final clinical evaluation, 20 patients died, 65% died from serious infections, 15% from cardiopulmonary complications, and 10% from end stage renal disease. As the whole group, survival rates were 89% and 74% at 12 and 60 months, respectively. The 5-year patient survival in class-II, class-IV and class- VLN patients were 83%, 67% and 64%, respectively. Within the group of class-IV LN, the 5-year survival in the patients treated with intravenous CYC was significantly better than those receiving prednisolone with or without azathioprine. Five-year kidney survival rates from the time of diagnosis to the endpoints of terminal renal failure were 94% for the whole group, and 100%, 96%, 91% in the class- V, class-II, and class-IV group, respectively. Initial presence of hypertension, hematuria, renal insufficiency were independent factors significantly associated with lower patient survival probabilities. There was no association of either patient and kidney survival with gender, age, cytopenia, and autoantibody level. CONCLUSIONS: Infectious complications were the most common cause of morbidity and mortality in our pediatric patients with SLE. The immunosuppressive agents used to treat SLE seemed to be a major contribution to the patient survival. With judicious use of corticosteroid, intravenous CYC in severe SLE showed superior efficacy over oral prednisolone with or without azathioprine.

Henoch-schonlein purpura in children: A 17-year experience.

A descriptive study of one hundred and five Henoch-Schonlein purpura (HSP) patients (57 males, 48 females) treated during 1987-2003 in Department of Pediatrics, Faculty of Medicine Siriraj Hospital, was conducted. The male to female ratio was 1.2:1. The mean age of the patients was 7.1 years (range 2-15). Most patients lived in Bangkok and the central region of Thailand. HSP most commonly occurs in the rainy season. Clinical manifestations were rash (100%), arthralgia (61.9%), arthritis (25.7%), abdominal pain (66.7%), gastrointestinal bleeding (14.3%: stool occult blood 11.4% and hematemesis or melena 2.9%), nephritis (37.1%: microscopic hematuria 29.5%, gross hematuria 7.6%), proteinuria (23.8%), nephritic syndrome (1%), and hypertension (1%). The most characteristic rash was purpura, mainly on the lower extremities. Arthritis and/or arthralgia commonly affected feet ad ankles. The abdominal pain was commonly localized at epigastrium and umbilical area. Gastrointestinal complications included upper gastrointestinal bleeding, 34.3% developed recurrent symptoms including abdominal pain, nephritis, and rash mostly occurring within the first 3 months after the initial resolution (range 2 days-9.6 years). The mainstay of management was supportive care. The patients with severe abdominal pain received prednisolone. Prednisolone and cyclophosphamide were only given to serve nephritis patient with a good outcome.

Percutaneous renal biopsy in children.

The authors studied the percutaneous renal biopsies performed in the Department of Pediatrics, Siriraj Hospital from January 2000 to March 2001 in order to evaluate the safety and benefit of the procedure. Eighty-five patients (90 episodes) were included in the study, aged 7.8+/-3.7 year (range 16 months to 16 years), with a male to female ratio of 1.2:1. Nephrotic syndrome (42.3%) and systemic lupus erythematosus (23.5%) were the two most common indications for biopsy. The kidney was localized by ultrasound prior to the procedure in nearly all cases (97.7%). Premedication with Ketamine was adequate in most patients (91.1%). A modified 13 G Vim-Silverman needle was used to obtain 1-4 biopsy cores. The mean number of glomeruli obtained was 44.0+/-29.9, with failure to obtain renal tissue in 6 episodes (6.6%). Percutaneous biopsy was performed twice in one patient without success and the patient eventually underwent an open biopsy. The most common complication was hematuria (74.4%), of these, gross hematuria was found in 23.3 per cent. Blood transfusion was needed in 2 patients, one of them also needed embolization to control bleeding. Transient hypotension occurred in 1 patient. Transient hypertension occurred in 6 episodes (6.6%). Muscle twitching occurred in 2 episodes and was treated with diazepam intravenously. Hypertension and muscle twitching only occurred in those who received ketamine. The Clinical Benefit Score was 2 (information yielding a definite diagnosis and/or prognosis, alternatively allowing a change in, or support of, therapy) in 89.4 per cent. It was concluded that the present practice of renal biopsy is safe, with high clinical benefit score. It remains to be studied whether an ultrasound guidance biopsy with a newer biopsy device will lower the incidence of complications even further.

Hyponatremia, polyuria-polydipsia and renovascular hypertension in a child: An unusual clinical manifestation.

We reported a 3 1/2 years old child presented with history of sudden onset of polyuria-polydipsia with severe hypertension (210/90 mmHg). His initial laboratory work up revealed hyponatremia, marked natriuresis, kaliuresis, and nephrotic range of proteinuria. His echocardiogram showed evidence of moderate to severe left ventricular hypertrophy compatible with long standing hypertension. Captopril renoscintigraphy (Tc99m-EC) suggested grade III right renal artery stenosis. Renal angiography revealed total occlusion of his right renal artery with collateral artery from the left renal artery. The lesion was not suitable for balloon angioplasty. He continued to have severe hypertension despite being treated by five different anti-hypertensive medications. Right nephrectomy was performed. During the recovery period his blood pressure and plasma electrolyte returned to normal. Normal excretion of sodium and protein were also seen in his urine. We suggest that renovascular hypertension should be considered and promptly investigated in a child presented with hypertension association with hyponatremia, hypokalemia, and polyuria-polydipsia.

Persistent hypertension in Thai children.

We reviewed the records of 36 children with persistent hypertension who were admitted to the Department of Paediatrics, Siriraj Hospital between 1987 and 1993. The male to female ratio was 1.4:1, and mean age at diagnosis was 7.6 years. The cause of hypertension in 16 children (44.44 percent) was renal parenchymal disease, while four children (11.11 percent) had renovascular disease, five chil-dren (13.9 percent) had cardiovascular disease and eight children (22 percent) had primary hypertension. Miscellaneous causes were found in three (one adrenal gland tumor, one Wilms’ tumor and one unknown). Hypertension was controlled by medication alone in 70 oercent and combined medical and surgical treatment in 20 percent of patients. Hydralazine, furosemide and propranolol were most common drugs used. Five patients had hypertensive crises. Five patients (13.9 percent) died; the cauese of death were multifactorial and were not directly related to hypertension.

Urinary tract infection in Thai children.

A retrospective analysis was made of the hospital records of 122 children below 12 years of age who presented at the Dept of Pediatrics, Siriraj Hospital, May 1989 to April 1993 with first episodes of urinary tract infection (UTI). The male to female ratio was 1:1.3. The largest group of patients were under 1 year of age (23%). In young infants signs and symptoms were frequently non-specific. Urinary symptoms, were found in only 18%. Older children had more specific symptoms i.e. pollakiuria (24%), gross hematuria (22.3%) and dysuria (21.3%). Urine culture was done before antibiotics were given in 101 patients (83%) with positive results in 52 (51.5%) of them. E. coli was the most common organism identified (59.6%). Responses to empirical antibiotics were good with parenteral ampicillin (78.6%), gentamicin (83.3%), and ampicillin combined with gentamicin (90.9%). Cotrimoxazole orally was used with a response of 63%. Radiological investigations of the KUB were performed in 54 patients and abnormalities found in 25 (46.3%) in which hydronephrosis was the most common (13 patients). Vesicoureteric reflux (VUR) was found in 7 patients. The highest prevalence of KUB anomalies and VUR was found in the under 1-year-old group. Mean duration of follow up was 7.5 months. Recurrent infection was found in 18 patients, 2 of them were on antibiotics prophylaxis at the time of infection. Hypertension was also noted in 2 patients. The authors suggest that radiologic evaluations should be done in all children with first episodes of UTI, especially if they are younger than one year. Long term follow up is also recommended to prevent recurrent UTI and complications which may lead to permanent renal damage.

End-stage renal failure in children with autosomal dominant polycystic kidney disease.

Clinical presentaiton of autosomal dominant polycystic kidney disease in childhood is uncommon. When present, it is most commonly abdominal mass. Some children may present with hypertension, hematuria, or abdominal pain without enlargement kidneys but renal failure is rare and has never been reported in Thailand. We report a 10-tear-old Thai boy with end-stage renal failure due to Autosomal dominant polycystic kidney disease diagnosed by renal autopsy.

Primary hyperoxaluria: Cases report.

Primary hyperoxaluria is a rare disease characterized by recurrent calcium oxalate nephrolithiasis and nephrocalcinosis. The most diagnostic laboratory finding if an increased amount of urinary oxalate. The inheritance is presumed to be autosomal recessive but autosomal dominant has been repoeted. Here are reported cases of a family: a boy, his brother, and his father. The boy, the most severely affected died at the age of 8 years. His 6-year-old brother, treated by 6 episodes of Extracorporeal Piezo Electric Lithotripsy (EPL); followed with high doses of pyridoxine and thiazides orally, is still alive with normal renal function. His father was treated by 6 episodes of EPI but without medication. The authors suggest that investigations for metabolic causes should be done in children with positive family history of nephrolithiasis.

Multiple pterygium syndrome: Report of three cases from Siriraj Hospital.

Multiple pterygium syndromes is a rare syndrome, only 48 cases have been reported in the literatures. The significant features are comprised of, growth and development retardation, webbing of neck, axillae, antecubital, popliteal, and antecrural joint or area, with disfiguration of external genitalia, interphalangeal joint webbing and club foot. The abnormality greatly interferes with normal joint function and leaves the patient in severely disabled conditions. Three patients reported were seen at Siriraj Hospital between 1981-1987. The first case is still alive, 10 years of age. The other two died at the age of one month due to aspiration and congestive heart failure. Pathological studies were done on both cases.