It is controversial to test for urinary tract infection (UTI) in patients with unexplained indire... more It is controversial to test for urinary tract infection (UTI) in patients with unexplained indirect hyperbilirubinemia in the first 2 weeks of life. We aimed to study the prevalence and significance of UTIs in such neonates who were requiring phototherapy. Subjects were 2- to 14-day-old neonates with indirect bilirubin levels above phototherapy limit with no other abnormality in their bilirubinaemia-related etiologic workup. UTI was diagnosed by 2 consecutive positive cultures obtained by catheterisation, documenting growth of >10,000 colonies of the same microorganism with consistent antibiograms. The UTI (+) patients were evaluated by renal ultrasonography (US), and some were followed up for possible recurrent UTI. 262 neonates were included in the study. UTI prevalence was 12.2%, and bacteraemia was 6.2% among UTI (+) patients. The two most common pathogens (81.2%) were Escherichiacoli and Klebsiella. pneumonia. All UTI (+) patients had undergone US, revealing 12.5% pelvicalie...
The Turkish Renal Tubular Disorders Working Group aimed to form a patient registry database and g... more The Turkish Renal Tubular Disorders Working Group aimed to form a patient registry database and gathered demographic, clinical, and laboratory data in various hereditary renal tubular disorders (HRTDs). A questionnaire comprising HRTDs was sent to the centers. The cohort was composed of 226 patients (109 girls, 117 boys). The distribution of patients according to HRTD was as follows: 45.6% distal renal tubular acidosis (dRTA), 26.6% proximal RTA (pRTA), 3.5% type IV RTA, 21.7% Bartter's syndrome, and 2.6% Gitelman's syndrome. Cystinosis was the most common cause for renal Fanconi syndrome. Age at diagnosis was between 1 month and 16 years. Overall consanguinity rate was as high as 72%. Rate of affected siblings was 28.5%. pRTA and type IV RTA were more common in males. Most common presenting symptoms were failure to thrive, lack of appetite, and vomiting. Nephropathic cystinosis was the most common HRTD leading to renal failure, followed by dRTA. Hearing loss was present in 23% of patients with dRTA and 6.3% of patients with Bartter's syndrome. No other patient had hearing loss. Convulsions were noted in Bartter's syndrome patients with failure to thrive, especially in those with height below 3%. Polyuria and nephrocalcinosis were more common in dRTA patients with deafness compared with patients without deafness. This data reflected a high number of HRTDs as a result of high consanguinity rate in Turkey. Our data serve as a database of demographic, clinical, and laboratory features of this rare disease group.
INTRODUCTION: Nutcracker syndrome (NCS) is a rare condition caused by the compression of the left... more INTRODUCTION: Nutcracker syndrome (NCS) is a rare condition caused by the compression of the left renal vein between the abdominal aorta and superior mesenteric artery. The purpose of the study was to evaluate our management of NCS. METHODS: Patients were retrospectively reviewed and sex, age, main symptoms at application, physical examination, radiological findings, laboratory examinations, and treatment were recorded. RESULTS: 23 patients (16 girls, 7 boys; age range, 5 to 16) diagnosed with NCS. Microhematuria was detected with 14 (60.8%) patients before diagnosis. And four of 9 (39.1%) were determined after diagnosis. Microhematuria was not detected in 5 (21.7%) of the patients. All patients (100%) had orthostatic proteinuria. 17 (73.9%) patients had mild, 3 (13.0%) patients had moderate and 3 (13.0%) had severe proteinuria. The mean diameter of the left renal vein (LRV) at the aortomesenteric (AM) portions and the hilar were 1.69mm±0.70 and 8.01mm±2.27. The mean angle between t...
It is controversial to test for urinary tract infection (UTI) in patients with unexplained indire... more It is controversial to test for urinary tract infection (UTI) in patients with unexplained indirect hyperbilirubinemia in the first 2 weeks of life. We aimed to study the prevalence and significance of UTIs in such neonates who were requiring phototherapy. Subjects were 2- to 14-day-old neonates with indirect bilirubin levels above phototherapy limit with no other abnormality in their bilirubinaemia-related etiologic workup. UTI was diagnosed by 2 consecutive positive cultures obtained by catheterisation, documenting growth of >10,000 colonies of the same microorganism with consistent antibiograms. The UTI (+) patients were evaluated by renal ultrasonography (US), and some were followed up for possible recurrent UTI. 262 neonates were included in the study. UTI prevalence was 12.2%, and bacteraemia was 6.2% among UTI (+) patients. The two most common pathogens (81.2%) were Escherichiacoli and Klebsiella. pneumonia. All UTI (+) patients had undergone US, revealing 12.5% pelvicalie...
The Turkish Renal Tubular Disorders Working Group aimed to form a patient registry database and g... more The Turkish Renal Tubular Disorders Working Group aimed to form a patient registry database and gathered demographic, clinical, and laboratory data in various hereditary renal tubular disorders (HRTDs). A questionnaire comprising HRTDs was sent to the centers. The cohort was composed of 226 patients (109 girls, 117 boys). The distribution of patients according to HRTD was as follows: 45.6% distal renal tubular acidosis (dRTA), 26.6% proximal RTA (pRTA), 3.5% type IV RTA, 21.7% Bartter's syndrome, and 2.6% Gitelman's syndrome. Cystinosis was the most common cause for renal Fanconi syndrome. Age at diagnosis was between 1 month and 16 years. Overall consanguinity rate was as high as 72%. Rate of affected siblings was 28.5%. pRTA and type IV RTA were more common in males. Most common presenting symptoms were failure to thrive, lack of appetite, and vomiting. Nephropathic cystinosis was the most common HRTD leading to renal failure, followed by dRTA. Hearing loss was present in 23% of patients with dRTA and 6.3% of patients with Bartter's syndrome. No other patient had hearing loss. Convulsions were noted in Bartter's syndrome patients with failure to thrive, especially in those with height below 3%. Polyuria and nephrocalcinosis were more common in dRTA patients with deafness compared with patients without deafness. This data reflected a high number of HRTDs as a result of high consanguinity rate in Turkey. Our data serve as a database of demographic, clinical, and laboratory features of this rare disease group.
INTRODUCTION: Nutcracker syndrome (NCS) is a rare condition caused by the compression of the left... more INTRODUCTION: Nutcracker syndrome (NCS) is a rare condition caused by the compression of the left renal vein between the abdominal aorta and superior mesenteric artery. The purpose of the study was to evaluate our management of NCS. METHODS: Patients were retrospectively reviewed and sex, age, main symptoms at application, physical examination, radiological findings, laboratory examinations, and treatment were recorded. RESULTS: 23 patients (16 girls, 7 boys; age range, 5 to 16) diagnosed with NCS. Microhematuria was detected with 14 (60.8%) patients before diagnosis. And four of 9 (39.1%) were determined after diagnosis. Microhematuria was not detected in 5 (21.7%) of the patients. All patients (100%) had orthostatic proteinuria. 17 (73.9%) patients had mild, 3 (13.0%) patients had moderate and 3 (13.0%) had severe proteinuria. The mean diameter of the left renal vein (LRV) at the aortomesenteric (AM) portions and the hilar were 1.69mm±0.70 and 8.01mm±2.27. The mean angle between t...
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