Renal USG Research Articles

Balancing the Bleeding Risk & Treatment Related Complications in Immune Thrombocytopenia - an Unmet Need: Real-World Data from India

Background: Primary Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder due to decreased platelet count (PLT). Patients (Pts) with ITP face manifold challenges owing to the multifarious burden of living with the disease and its treatment-related complications. Corticosteroids used as first-line therapy in both adults and children do not improve quality of life or patient-related outcomes. The real-world experience data on the treatment outcomes of ITP pts in India is sparse. Objective: Our study aims to compare and evaluate the treatment outcomes and treatment-related adverse events in newly diagnosed ITP pts treated with corticosteroids (Prednisolone/ Dexamethasone) in terms of initial response at 4 weeks (wks) and sustained response at 3 months from initiation of therapy respectively. Methods: We enrolled 73 pts with new onset ITP, attending the outpatient department of AIIMS Rishikesh, between March 2019 to August 2020. Pre-treatment Complete Blood Count(CBC) Platelet count (PLT), Reticulocyte count, LDH, Liver and Renal Function Tests, PT, APTT, ANA, HIV, HBsAg, HCV, USG Abdomen, and Chest X-Ray were done. Bone marrow aspirate and biopsy (BM) was done in pts >50 yrs or to look for other hematological disorders. BM done in 19pts was normal. All pts had a pretreatment PLT <30 x 109/L. Children (age <12yrs) were treated only in the presence of bleeding. The severity of bleeding was graded as per the WHO bleeding score. Pts were randomized by block randomization and treated with Prednisolone or Dexamethasone. Prednisolone was administered at 1mg/kg/day for 4 wks (age >12yrs) and 2mg/kg/day for 2 wks (age < 12yrs) and tapered gradually over 2 months. Dexamethasone was administered at 40mg/day for 4 days/cycle, for 3 cycles at an interval of 14 days. Pts were followed up every wk for the first 4 wks and monthly thereafter for 3 months with CBC, PLT, and evaluated for any possible bleeding/complications. Response was defined as Complete response (CR) plt ≥ 100 x 109 /L. Response (R) plt ≥ 30 x 109/L and >2 fold increase in plt from baseline, no bleeding. No response (NR) plt < 30 x 109 /L or <2 fold increase in plt from baseline or bleeding. Loss of response (LR) plt < 30 x 109/L or bleeding, from CR or R previously. Results: Of the 73 pts evaluated, 23(32%) were males and 50 (68%) females. The median age was 29 (range 3 to 75years), of which 13 (18%) were pediatric pts (age <12 yrs). 10 (14%) pts had no bleeding at presentation, 63 pts (86%) presented with bleeding, of which 23 pts (45%) had mild bleeding grade 1 -2, 27pts (37%) had moderate bleeding grade 3, and 4 pts had severe bleeding grade 4 including 1 pt with intracerebral bleed. 39 (53%)pts received dexamethasone, and 34 (47%)pts received prednisolone. 68 out of 73 pts (94%) had an initial response at 4 weeks, 54pts (74%) attained CR, and 14(20%) had R. 5pts had NR in whom steroids were discontinued and second-line therapy was started. Sustained response was assessed at 3 months in 58 out of 73pts, 15 were lost to follow-up. An overall sustained response CR and R was seen in 39 pts (67%), 15 (26%) had loss of response LR and 4 (7%) had no response NR. The results are summarised in table 1. 57 (88%) out of 73pts had treatment-related adverse events (TRAE) and 16 (22%) had no TRAE. The TRAE observed in our study include weight gain, infections, acne, fatigue, hyperglycemia, hypertension, dyspepsia, and others (insomnia, proximal myopathy, & central serous retinopathy-CSR). 1pt had proximal myopathy and 1 pt developed CSR. The severity of TRAE was graded as per CTCAE. There was no grade 4 or 5 TRAE. All TRAE resolved spontaneously or with treatment. TRAE is summarised in table 2. No significant difference was noticed between the prednisolone or the dexamethasone group either in terms of treatment outcomes or treatment-related adverse events. Conclusion: Our study shows comparable results between prednisolone and dexamethasone groups in initial and sustained response rates to first-line therapy in newly diagnosed ITP patients but a high incidence 88% of treatment-related adverse events in both groups. A shorter duration of steroids and affordable newer modalities of therapy in ITP is required to improve the overall well-being of ITP patients with lower middle income in India. Disclosures: No relevant conflicts of interest to declare. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

38 Pregnant with COVID-19 and acute renal insufficiency attended in a hospital in the Amazon region: Case report

Introduction: COVID-19 caused by the infection of the new coronavirus (SARS-COV-2) was recognized by the WHO as a pandemic. With an acute condition, its symptoms are variable and can lead to death. Pregnant women generally have mild symptoms and there is no evidence of vertical transmission, and should receive specialized multidisciplinary assistance,with conditions of safety and isolation. Objective: To report a case of a pregnant woman with COVID-19 and Acute Renal Insufficiency treated at a hospital in the Amazon Region, demonstrating the importance of the correct and early diagnosis to indicate the best therapeutic approach. Methodology: The present work uses a bibliographic research framework, for theoretical basis and to report this case of a pregnant woman with COVID-19 and Acute Renal Insufficiency. Results: DLA, 18 years old, 23s6d, complaints of shortness of breath, weakness, chest pain, vomiting, diarrhea and fever for 3 days, rapid COVID-19 positive test and urinary tract infection. On examination BCF138bpm, FR 19irpm, FC 153bpm, TAX 39.2°C, SAT.O2 96% in room air, hospitalized in respiratory isolation, prescribed azithromycin, ceftriaxone, dexamethasone, acetylcysteine, enoxaparin. Tests urea 85mg/dL and creatinine 3.07mg/dL. On the second day, 131mg/dL urea and 5.01mg/dL creatinine, renal USG: kidneys with increased echogenicity of the parenchyma, slight loss of cortical-medullary relationship, mild bilateral pyelocalytic dilation. Nephrology diagnosed pre-renal insufficiency, maintained oral hydration, potassium replacement, due to hypokalemia (K+ 2.94).She was followed up by obstetrics, medical clinic and nephrology, remaining hemodynamically stable, with antibiotic therapy and improvement of renal function. Hospital discharge on the 9th day for outpatient follow-up. Conclusion: Due to the impact of COVID-19 on pregnancy, differentiated attention is needed from the preventive aspects, diagnosis, management, early treatment and follow-up of these women and their concepts, by a multidisciplinary team to improve the quality of care provided to pregnant women.

Perinatal asphyxia and acute renal failure in neonates: presentation and outcomes

Background: Perinatal asphyxia (PNA) is described by the world health organization as a "failure to begin and sustain breathing at delivery." Acute renal failure (ARF), a well-known consequence of birth asphyxia, has a poor immediate prognosis and can leave survivors with lasting renal impairment. The goal of this study was to find out how often ARF is in cases of PNA and how it relates to the severity of the Apgar score and the grading of hypoxic ischemic encephalopathy.Methods: This study was based on APGAR scores and a renal function evaluation done on day 3 of life on neonates with a gestational age of &gt;34 weeks and signs of birth asphyxia.Results: According to the APGAR score, 46 percent of the 75 infants investigated had mild PNA, whereas 42% and 12% of the babies had moderate and severe PNA. ARF was discovered in 24% of PNA patients, with oliguric renal failure (ORF) in 42% and non-ORF (NORF) in 58%. Blood urea was elevated in all instances with ARF (100%) but only 8% of non-ARF patients. ARF was found in 100% of individuals with severe PNA, but only 28% of patients with mild PNA. ARF was not found in any of the individuals with mild PNA. Only 3 (4%) of asphyxiated babies had abnormal renal USG results.Conclusions: Our findings revealed a substantial link between PNA severity and ARF, with no ARF in mild PNA. The incidence of ARF and the stage of HIE were shown to have a linear association in this study. Only ORF instances resulted in death.

Outcome assessment for pyeloplasty in pediatric age group on the basis of ultrasonography

At present, duration and methods of radiographic follow-up after pediatric pyeloplasty are not well defined. We prospectively studied pediatric age group patients to assess outcome for cases of pyeloplasty on the basis of ultrasonography and DTPA scan. : We assessed all cases of PUJ obstruction in age group 0-18 years, who got admitted in LLR hospital, Kanpur and underwent pyeloplasty. Patients were excluded if cause of pyeloplasty was acquired. Preoperatively patients underwent clinical evaluation with history and examination followed by radiological investigations including Renal USG &amp; DTPA scan. All patients were followed up postoperatively at 3, 6, 9 &amp; 12 months. In USG, renal parenchyma thickness, antero-posterior diameter of renal pelvis was assessed. In DTPA scan, differential renal function was assessed. Data of USG and DTPA scan were statistically compared. 32 patients who underwent pyeloplasty at a median age of 4.2 years were studied. Follow-up was done for 1 year. On the basis of our study, DTPA scan is better than renal USG to assess functional outcome after pyeloplasty in pediatric age group up to 6 months during follow-up. But results are comparable for follow-up during 9 &amp; 12 months postoperatively. The results of our study show that in the first 6 months, renal USG is not as much informative as DTPA scan to assess functional outcome after pyeloplasty but after 6 months, renal USG is equally effective as DTPA scan.Our study concludes that for poor resource countries, renal USG can be used to assess functional outcome after pyeloplasty in pediatric age group for post-operative follow-up, instead of costly &amp; scarcely available investigation like Renal DTPA scan.

English

BACKGROUND Renal resistive index (RRI) is a non-invasive marker of renal function, which can be measured by renal USG Doppler. High RI indicates poor prognosis, which has been associated with conditions such as proteinuria, hypertension, chronic kidney disease and renal failure. As nephrotic syndrome is characterised by significant proteinuria and hypoalbuminemia, which may progress to renal failure, it is important to see the correlation of RRI with serum albumin level. The purpose of this study was to assess the relation between renal resistive index and serum albumin in nephrotic syndrome. METHODS USG renal Doppler was done for all the children with nephrotic syndrome and RRI was calculated as [(peak - systolic velocity − end - diastolic velocity) / peak - systolic velocity]. Blood investigations were also done, from which serum albumin values were obtained. RRI was compared with mean serum albumin level. Statistical analysis of data was performed using the computer programme, Statistical Package for Social Sciences (SPSS for windows, version 21.01, Chicago, SPSS Inc.) and Microsoft excel 2010. Results on continuous measurement were presented as mean ± standard deviation and compared using Analysis of Variance (ANOVA). RESULTS The mean serum albumin in the children with nephrotic syndrome in our study was 1.82 ± 0.32 gm / dl. The mean RI value was 0.66 ± 0.14 SD, 0.64 ± 0.15 SD and 0.66 ± 0.12 SD as well as 0.63 ± 0.12 SD, 0.67 ± 0.16 SD and 0.71 ± 0.13 SD on the right and left kidney respectively for each of upper, middle, and lower pole. P value of left kidney and right kidney was 0.0057 and 0.6409 respectively. CONCLUSIONS Lower the mean serum albumin level, higher the range of RRI. KEY WORDS Serum Albumin, Renal Resistive Index, Nephrotic Syndrome, USG Renal Doppler

Prevalence of urinary tract infection in malnourished children aged 6 months to 5 years attending tertiary care centre

Background: Infections are more common in malnourished children, as result of impaired immunity. Unexplained fever and failure to thrive are the common presenting features of urinary tract infection (UTI) in children. In malnourished children, UTI is mostly asymptomatic and is leading to pyelonephritis and renal scarring. The study was done with the aim to evaluate and find out the prevalence of urinary tract infection in malnourished children between 6 months to 5 year and to find out the causative organism and antibiotic sensitivity pattern.Methods: A total of 180 cases of malnourished children were enrolled and clean catch midstream urine sample was collected for urine culture, according to WHO criteria of malnutrition. Children with urinary tract abnormality were excluded from the study. Renal USG was done in all urine culture positive cases.Results: Total of 174 cases was present after exclusion. Of 174 children, 27 (15.5%) children were having UTI. In this study, 37% of children are asymptomatic. E. coli is the commonest organism causing UTI 16 (59%). Other organism are Klebsiella pneumonia 4 (14.9%), Proteus mirabilis 3 (11.1%), and Pseudomonas 3 (11.1%). The order of antimicrobial sensitivity pattern was amikacin (100%)&gt; ciprofloxacin (81.4%)&gt; cefotaxime (7%). Other common drugs have developed resistance to these organisms.Conclusions: Our observations conclude that malnourished children with fever of unknown origin are at risk of UTI. Hence, urine analysis and culture tests are to be done to all malnutrition cases for assisting to diagnose the bacterial infection and providing the appropriate treatment.

C4d 100% Positive in Renal Biopsy of Patient with Acute Graft Dysfunction Successfully Managed with Bortezomib, Clinical Case

Introduction 57-year-old male patients with renal insufficiency secondary to hypertensive disease. It has as background medical hemotransfusion positive four globular units and reative panel of negative antibodies, zero%. Materials and Methods The patient received renal transplantation from cadaveric donor, cold ischemia of 24 hours, delayed function of the graft requiring three sessions of hemodialysis. received induction treatment based on basiliximab 40 mg, methylprednisolone 3 grams, without surgical complications, his maintenance treatment was mycophenolate mofetil 500 mg three times a day, Cyclosporine 100 mg twice daily, cyclosporine (C0) concentration: 153 ng / mL, and 200 mg of prednisone daily that was subsequently tapered. The patient received antiviral prophylaxis consisted of 450 mg of acyclovir three times a week and trimethoprim-sulfamethoxazole 160/800 mg three times a week. The patient, two months after the transplant, presented lymphocele and was drained and sclerosed. The cratinin went to 1.5 mg%. Four months after the transplant, presented urinary tract infection without creatinin problems or sepsis, he was managed with ciprofloxacin 500 mg every 12 hours for 7 days. Subsequently, the patient presented elevated creatinine at 2.2 mg%, renal USG without obstructive data and it was decided to perform a renal graft biopsy. Results The diagnosis was kidney rejection mediated by antibodies with positive C4d in all the peritubular capillaries of the sample.the treatment was methylprednisolone 3 gr, plasmapheresis three sessions of 1.5 volums treated on day 1, 3, 5, bortezomib (1.3 mg / m2) on days 1, 4, 8, and 11, rabbit antithymocyte globulin: (1.5mg / kg / day) on days 9, 10, 11. At discharge the serum creatinine fell to 1.7 mg%,asymptomatic without infectious complaints. Conclusions Our patient had risk factors for antibody-mediated rejection, it was successfully managed with a complete scheme of inmusupresion, it is very important to give treatment in the different phases regarding the production of antibodies.

Study of clinical etiological and radiological profile of UTI cases

Background: Fever and significant bacteriuria and pyuria in children with undocumented sources of infections must be presumed to be symptoms of pyelonephritis, an invasive infection of the renal parenchyma requiring prompt treatment. The present study is undertaken to study of clinical, etiological and radiological profile of children with culture positive UTI.Methods: This study is being performed on children with culture positive Urinary tract infection admitted or attending OPD at department of Paediatrics during the study duration were included in the present study.Results: The highest incidence of UTI in present study was found to be 52.25% in the age group of children below 2 years. E-coli (75%) was the commonest organism found in urine culture in all age groups. Sensitivity, negative predictive value and positive predictive value of renal ultrasonography in detecting VUR was very poor, supporting the impression that renal USG is deficient in accurately excluding VUR. VUR are common in Culture positive UTI, milder grade are more common which are more likely to be missed by renal USG, thus stressing the need of MCU in children of culture positive UTI.Conclusions: Urine examination and culture and sensitivity must be a part and partial in evaluating child with fever without focus. As the scope of USG in detecting VUR is low, MCU must be done in all cases of UTI -5years irrespective of USG finding.

Obstructive Uropathy in Children – An Update

Obstructive nephropathy is a structural or functional hindrance of normal urine flow, sometimes leading to renal dysfunction. Urinary tract obstruction can result from congenital (anatomic) lesion or can be caused by trauma, neoplasia, calculi, inflammation or surgical procedures, although most childhood obstructive lesions are congenital.The clinical features in most of the patients are due to consequences of the obstruction2. Obstruction of the urinary tract generally causeshydronephrosis, which is typically asymptomatic in its early phase. Renal USG gives information about urinary tract dilatation, renal cortical thickness, calyx size, diameter of pelvis, ureter, bladder thickness, tumor &amp; calculi and doppler USG for evaluation of aberrentvessles. Once obstructive nephropathy has been identified therapy focuses on the rapid restoration of normal urine flow either by medical or surgical intervention.Bangladesh J Child Health 2017; VOL 41 (2) :117-124

Role of Renal Doppler Flowmetry in Patients with Altered Renal Function Tests

Objectives: (i) Study was done to know the sensitivity of renal ultrasound with Doppler studies in patient with altered renal function test. (ii) To study the sonomorphological characteristics in grayscale ultrasound and renal Doppler flowmetry in altered renal function test. Material and Methods: A hospital based cross sectional study of 83 patients were conducted over a period of 1 year from June 2013 to May 2014 in the Deptt. Radio Daignosis, Assam Medical College & Hospital Dibrugarh. Approved was obtained from Institutional ethics review committees, AMCH, Dibrugarh. All the patients are subjected to grey scale USG of abdomen and Renal Doppler study. Grey scale USG done with 2.5-5 MHz curvilinear transducer and 10 MHz linear transducer. Both colour Doppler and pulsed wave Doppler mode were done by using 2.5-5 MHz convex array probe. Result: For the analysis of the study, group is subdivided into four subgroups e.g. (i) Chronic kidney disease (CKD) (ii) Acute kidney Injury (AKI) (iii) Glomerular dieases – Nephritic Syndrome (NeS) & Nephrotic Syndrome (NS) (iv) Lupus Nephritis (LN). Among the 93 patients CKD 68 (73%), AKI – 13(12.9%) Nes- 3(3.2%), NS- 6 (6.4%), LN – 4 (4.3%). Renal length are reduced 19%, increased in 16%. Renal cortical echoes are increased in 66.6%. In Renal Doppler RIm is increased 72 (77%) patients. PSVa were normal in 82.7% and increased 17% patients. Conclusion: Renal Doppler USG can delineate altered renal function tests in medical renal disease. USG features like increased renal cortical echotexture, increased and decreased renal size, irregular renal surface are the useful indicators for altered renal function. Keyword: USG, Renal Doppler flowmetry, altered Renal Function.

Laparoscopic extravesical detrusorraphy, a minimally invasive treatment option for vesicoureteral reflux: A single centre experience

Laparoscopy in pediatric patients offers more benefits than was earlier presumed and these widely reported benefits significantly outweigh any concerns regarding the technical difficulties. Laparoscopic correction of vesicoureteral reflux aims to duplicate the excellent results of open surgery while at the same time reducing perioperative morbidity and analgesic requirements, improving cosmesis and shortening hospital stay. To share our experience of laparoscopic extravesical detrusorraphy, highlight our technical modification of intraoperative minimal "atraumatic" ureteric handling of the ureter, which we hypothesize may decrease ureteral complications, and report our results. This was a retrospective chart review of 76 toilet-trained children (98 refluxing units), in the age group of 3-16 years, with Grade I-IV reflux, who underwent laparoscopic detrusorraphy from June 2006 to January 2014. A ureteric catheter is inserted into the refluxing ureter and is tied to the Foleys to drain into a common bag. A three port technique is used. During ureteral dissection, a vascular sling in the form of a Rumel loop is used for atraumatic handling of the ureter. A detrusor tunnel is created with hook electrocautery. A stay suture is later passed through the abdominal wall and slings around the dissected ureter, which helps in holding the ureter approximated against the mucosal trough during detrusorraphy. Detrusor fibers are approximated with 5-0 Vicryl. No drain is placed and the Foley and ureteric catheter(s) are removed after 24 h. Intravenous ketorolac is given every 6 h for the first 24 h. Oral paracetamol is used for analgesia after the first 24 h. Adequate bladder emptying is ensured by assessment of post void residual urine before discharge. Renal USG alone is performed 2 weeks post operatively and repeated after 3 months along with a VCUG (voiding cystourethrography). Success was defined as absence of reflux in the follow-up VCUG done at 3 months. Mean operative time was 102 ± 26.5 min for unilateral detrusorraphy and 165 ± 18 min for bilateral extravesical detrusorraphy. The mean duration of hospital stay was 1.5 ± 1.7 days. There was one case of urinary retention that was managed with temporary recatheterization. There were no cases of ureteral ischemia, obstruction, hematuria or bladder spasms. Surgery was successful in 97.9% of the refluxing units (96/98). In two patients with grade IV reflux, there was downgrading to grade II on VCUG done at 3 months' follow-up. The reflux resolved at 8 and 14 months' follow-up, respectively. Our technique of atraumatic handling of the ureter, initially with the help of a vascular sling and later with the help of a stay suture passed percutaneously through the abdominal wall, resulted in no ureteric injuries. The postoperative morbidity of this procedure is low because the bladder is not opened, the ureter is not transected, no new UVJ is created and there is no need for placement of a drain. The risk of postoperative bowel adhesions is low as the ureter is dissected out through a narrow peritoneal window, which is again extraperitonealized at the end of the procedure (see figure). The postoperative complications of gross hematuria and bladder spasms, which may be especially encountered in patients undergoing laparoscopic Cohen's, were not seen in our case series. Laparoscopic extravesical detrusorraphy provides a minimally invasive treatment option for treatment of unilateral/bilateral grade I-IV vesicoureteral reflux. The postoperative morbidity is low and the success rate is favorable. Our technical modification of a "vascular sling" around the ureter facilitates atraumatic ureteric handling, which may reduce distal ureteral complications like ureteral ischemia and obstruction.

English

Abdominal migraine is a migraine variant, causing chronic idiopathic recurrent abdominal pain in 4-15% of children. It is usually seen between the ages of seven to twelve years and is more common in girls, with peak prevalence at the age of ten years. We report a 3 year old girl suffering from recurrent abdominal pain since 1½ years of age, who underwent extensive investigations as well as diagnostic laparotomy with appendectomy, and was ultimately diagnosed to have abdominal migraine. She responded well to the prophylactic drug Flunarizine. CASE REPORT: A three year old girl was referred with the complaint of episodes of severe abdominal pain since one and half years. It was dull aching, predominantly per umbilical, usually in the evening, intermittent, lasting for 1 to 2 hours, getting relieved on its own. Initially, there were 1 to 2 episodes per week and then the frequency increased to 2 episodes every alternate day. During the attack the child had anorexia, nausea, irritability and insomnia. There was no history of vomiting, loose stools or fever. She was completely symptom free between two episodes. There was no history of Koch's or Koch's contact and she had gained adequate weight with no developmental delay. There was no history of migraine in either parent. The patient was being investigated since one and half year. Complete blood count, liver function test, renal function test, urine, stool routine, X-ray abdomen, USG Abdomen, CT abdomen and barium meal study were all within normal limits. She did not respond to antacids and analgesics during the acute episodes and had received anthelminthic and antibiotics on multiple occasions. She had also undergone diagnostic laparoscopy with appendectomy four months prior to presenting to us with persisting complaints even after the surgery. On examination, the patient was conscious, alert, active and cooperative. Her vital parameters and anthropometry were within normal limits. There was no pallor, icterus, cyanosis, clubbing, lead lines, lymphadenopathy or edema. Systemic examination did not reveal any abnormality apart from the operative scar mark on the abdomen. A differential diagnosis of lead poisoning, acute intermittent porphyria and abdominal migraine were considered and the patient was investigated for the same. Blood lead level was 5mcg/dl. Urine porphobilinogen was negative. EEG was suggestive of epileptiform activity with spike pattern over right hemisphere and the diagnosis of abdominal migraine was made. During the ward stay she had a severe attack of abdominal pain and she was loaded with injection valproic acid at 20 mg/kg. In view of no response in 24 hours, she was started on oral flunarazine to which she responded. She was advised to continue tablet Flunarizine 2.5 mg twice daily and valproic acid was omitted. She is asymptomatic for the past one year.

Protective effect of preserved bladder-filling sensation on upper urinary tract in patients with spinal cord injury.

In this study the possible preventive effect of bladder-filling sensation (BFS) on upper urinary tract deterioration (UUTD) in spinal cord injury (SCI) patients was investigated. Eighty-four (60 males, 24 females) patients with SCI who underwent neurourological assessment and urodynamic examinations because of neurogenic lower urinary tract dysfunction and were prescribed appropriate bladder management and were under follow-up and whose disease duration was at least 3 years were included in the study. Bladder-filling sensation was categorised as "absent", "partially preserved" or "preserved", and as "normal" (preserved) and "abnormal" (partially preserved, absent). Presence of hydronephrosis, renal stones, and chronic pyelonephritis in any renal USG or IVP performed during the follow-up period were accepted as criteria of UUTD. The frequencies of UUTD for BFS subgroups were determined and compared. Mean age and mean disease duration were 38.4 ± 13.0 years and 72.2 ± 36.2 months. The frequency of UUTD was 31/84 (36.9 %) in the study population. The frequencies of UUTD were 0/8 (0 %), 22/53 (41.5 %) and 9/23 (39.1 %) for "preserved" "partially preserved" and "absent" BFS subgroups, respectively (p = 0.074). There was a statistically significant difference between normal BFS [0/8 (0 %)] and abnormal BFS [31/76 (40.8 %)] subgroups (p = 0.024). According to the results of our study preserved (normal) bladder-filling sensation has protective effect on upper urinary tract in patients with SCI.

A Rare Cause of Secondary Amyloidosis: Common Variable Immunodeficiency Disease

The common variable immunodeficiency disease (CVID) is the most common symptomatic primary antibody deficiency. It is the most frequently observed cause of panhypogammaglobulinemia in adults. Here, we present a case of systemic amyloidosis that developed secondary to the common variable immunodeficiency disease causing recurrent infections in a young female patient. A 24-year-old female patient, who was under treatment at the gynecology and obstetrics clinic for pelvic inflammatory disease, was referred to our clinic when she was observed to have swellings in her legs, hands, and face. She had proteinuria at a rate of 3.5 gr/day, and her serum albumin was 1.5 gr/dl. The levels of immunoglobulins are IgG: 138 mg/dl, IgA: 22,6 mg/dl, and IgM: 16,8 mg/dl. The renal USG revealed that the kidneys were observed to be enlarged. Since the patient had recurrent infections, hypogammaglobulinemia, nephrotic range proteinuria, and enlarged kidneys in the renal USG, she was thought to have type AA amyloidosis and therefore underwent a renal biopsy. The kidney biopsy revealed amyloid (+). So the patient was diagnosed with AA type of amyloidosis secondary to common variable immunodeficiency disease. A treatment regimen (an ACE inhibitor and a statin) with monthly administration of intravenous immunoglobulin was started.

Frequency of urinary tract infection in pediatric liver transplantation candidates

An increased frequency of infections has been reported in patients with chronic liver disease. The tendency of patients in this population to acquire UTI is not completely understood. We aimed at investigating the incidence of UTI in children with cirrhosis, before liver transplantation. Twenty-six children (9 girls, 17 boys; mean age, 7.66 +/- 5.73 yr) with chronic liver disease who had undergone liver transplantation between 2002 and 2004 were included. On admission for liver transplantation, patients were examined for presence of UTI. Serum biochemistry, complete blood cell count, urinalysis and culture, glomerular filtration rate, and abdominal ultrasonography were performed prior to liver transplantation. Ten of 26 patients (38.5%) were found to have symptomatic UTI. Urine cultures revealed E. coli in five (50%), Klebsiella pneumoniae in three (30%), Enterococcus faecalis in one (10%), and Enterobacter aeruginosa in one (10%) patient(s), respectively, as etiologic factors. The etiologies of chronic liver disease in our patients with UTI were BA in five, PFIC in three, Wilson's disease in one, and alpha-1 antitrypsin deficiency in one patient. We found a significantly greater number of UTIs in patients with biliary atresia than in those without biliary atresia (p < 0.05). The mean age of the patients with UTI was 2.75 +/- 3.49 yr, which was significantly lower than in those without UTI (9.75 +/- 4.86 yr, p < 0.05). Levels for white blood cells, thrombocytes, ALT, and alkaline phosphatase were significantly higher in patients with UTI than in those without UTI. There were no significant differences between the groups with regard to serum albumin, bilirubin, AST, GGT, BUN, or creatinine levels, glomerular filtration rate, duration of disease, and PELD scores. In patients with bacteriuria, renal USG revealed normal findings in all, but except one patient who had pelvicalyceal dilatation. Scintigraphic findings demonstrated acute pyelonephritis in six (60%) patients with UTI. VCUG demonstrated vesicoureteral reflux in two patients. In conclusion, symptomatic UTI is common in children with cirrhosis. It occurs more frequently in patients with biliary atresia than it does in patients with other types of chronic liver disease. In febrile children with chronic liver disease, UTI should be considered in the differential diagnosis.

In Hydronephrosis Less than 10 % Kidney Function is not an Indication for Nephrectomy in Children

To reduce the incidence of nephrectomy or hydronephrosis in children. From September 1998 to October 2000, we treated 58 patients with hydronephrosis; their ages ranged from 35 days to 11 years (mean age 4 years 7 months). All patients were subjected to a DTPA renogram with split function. In 12 patients (study group), kidney function was less than 10 % (range 0 - 10 %). Initially, nephrostomy was carried out in all 12 patients followed by Anderson-Hyne's pyeloplasty after 4 - 6 weeks. Postoperatively renal USG, urine r/m/e & c/s (routine and microscopic examination and culture and sensitivity test), blood urea, serum creatinine were assessed and DMSA scan and DTPA renogram with split functions were carried out in all patients. In the study group, all 12 patients showed improvement of renal function (more than 10 %) after nephrostomy and in all of them pyeloplasty was subsequently carried out within 4 - 6 weeks. There were no significant pre-, peri- or postoperative complications. Contrary to common practice we do not recommend nephrectomy for hydronephrotic kidneys which show < 10 % of renal function on renogram. The renal functional status improves significantly after a preliminary nephrostomy, thus avoiding the need for a straightforward nephrectomy in children along with all the possible long-term effects of a single kidney.