Fine needle aspiration cytology of cystic partially differentiated nephroblastoma of the kidney.
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Publications and source records attributed to S Agarwala.
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OBJECTIVE: In this single-center study we reviewed our experience with left atrial myxomas occurring over the past 9 years. METHODS: Sixty-three patients underwent excision of cardiac myxomas between 1995 and 2004. Of these, 56 patients (32 females and 24 males) had left atrial (LA) myxoma. The mean age was 37.80+12.97 years (range 3.5-67 years). Echocardiography was the only diagnostic evaluation done. The preferred approach for resection was right atrial trans-septal. Annual echocardiographic evaluation was undertaken following surgery. Follow-up is current and available in all the survivors (range 4 months-9 years). RESULTS: Clinically 75% of the LA myxomas simulated mitral stenosis. The symptoms were present for 2-6 months before operation. Most (86%) LA myxomas were attached to the fossa ovalis. Few (14%) originated from the LA wall, mitral valve annulus and anterior mitral leaflet. One patient succumbed to low cardiac output and another died of massive embolic stroke following surgery. There were no late deaths. Two patients (3.7%) developed left hemiparesis after operation but recovered completely. There was one (1.9%) recurrence 3 years after surgery. Atrial fibrillation occurred in one patient. Mitral insufficiency which was seen in two (3.7%) patients prior to surgery subsided following excision of the tumor. Postoperatively 94% patients remained without symptoms. CONCLUSION: Owing to the risk of valvular obstruction or embolization early surgery is indicated. Right atrial trans-septal approach is safe and easy. Most patients are asymptomatic following surgery. A yearly follow-up is essential.
We evaluated the results of surgical treatment for hepatoblastoma in infants and children after intensive preoperative chemotherapy, with special reference to histology and extent of liver involvement. The clinical features of 10 children with hepatoblastoma were reviewed regarding response to neoadjuvant chemotherapy, histological subtypes, extent of hepatectomy, operative complications, and prognosis. Response to chemotherapy was measured by volumetric assessment of tumour size by computed tomography scan. Cisplatin and Adriamycin (PLADO regime) up to three cycles markedly reduced the tumour volume on computed tomography (mean regression rate 65.9%); alpha-foetoprotein (AFP) levels also decreased from an initial mean of 16,116.4 ng/ml to 2,050.9 ng/ml. Five patients underwent right hepatectomy, two had right trisegmentectomy, two had left hepatectomy, and one had left trisegmentectomy. Histopathology of resected specimens revealed foetal histology in four patients, poorly differentiated (anaplastic) subtype in three, and mixed histology with mesenchymal components and osteoid formation in three. There was 100% resectability including six unresectable tumours (prechemotherapy). Moreover, hepatic resection tended to be less invasive in patients whose tumours had been much reduced after preoperative chemotherapy. Preoperative administration of cisplatin and Adriamycin reduces the tumour size significantly so that a safe radical hepatectomy can be performed. It also allows early administration of postoperative chemotherapy. Although overall good results were obtained with the current protocol, we also document our experience of unfavourable outcomes in patients with bilobar tumours (despite trisegmentectomy), patients with tumours showing poor response to neoadjuvant chemotherapy, and patients with anaplastic histology. Overall, at a 60-month follow-up we report an 80% survival rate by a combined approach.
OBJECTIVE: To evaluate the incidence, types and the effect on outcome of associated anomalies in neonates with anorectal malformations (ARM). METHODS: This retrospective study was carried out on all neonates with ARM admitted to the neonatal surgical intensive care unit (NSICU) from 1998 through 2003. RESULTS: Of the 754 neonates admitted to the NSICU during the study period of 6 years, there were 124 (16.4%) neonates with anorectal malformations. Of these 110 were included in the study. 73 % were male and 27% female. 86% of these were high ARM (HARM) while only 14% were low ARM (LARM). Associated anomalies were seen in 68% of patients. The incidence was 72% for HARM and 50% for LARM. The major associated anomalies consisted of esophageal (13%), gastrointestinal (GIT) (11%), genitourinary (GUT) (32%), skeletal (26%), cardiac (33%) and miscellaneous 26%. The overall survival rate was 84% (82% for HARM and 94% for LARM). The survival among those with associated esophageal anomalies was 43%, GIT 67%, GUT 80%, cardiac 61%, skeletal 76% and miscellaneous 79% respectively. This difference in survival was significant only for those with esophageal (p=0.004) and cardiac anomalies (p=0.0026). The survival rates among those with one, two or more than two organ systems involved with associated anomalies were 88%, 82% and 58% respectively. This difference was significant only for more than two organ systems involvement (p=0.003). CONCLUSION: Associated anomalies are common in neonates with ARM, the incidence being similar for HARM and LARM. The survival depends upon the number and severity of associated anomalies both in patients with LARM and HARM. Neonates with more number of organ systems involved have a poorer survival specially when associated with esophageal and cardiac anomalies. All neonates with ARM merit a meticulous search for associated anomalies so that the management can be tailored for each baby.
AIMS: In an era, in which valve bladder is recognised as perhaps the single most important determinant of long-term outcome in patients with posterior urethral valves (PUV), an insight into the etiopathogenesis and management of valve bladders is warranted. The present study was designed to evaluate bladder dysfunction in PUV and to assess the response to imipramine in these patients, both subjectively and objectively, by serial urodynamic studies (UDS). METHODS: From 1998-2001, 30 patients with PUV who had documented bladder dysfunction on UDS were studied. Patients with non-compliant or unstable bladders were treated with imipramine (1.5 - 2 mg/kg). All the patients in the present study were 5 years or older and hence old enough to be toilet trained. Assessment of continence and side effects of the drug was done after 3 months and repeat UDS were done at 3-6 months, 1 and 2 years. RESULTS: On the basis of initial treatment, the patients were divided into 2 groups; a fulguration group (n = 10, 33.3 %) and a vesicostomy group (n = 20, 66.6 %). Symptomatic voiding dysfunction was present in 27 of the 30 patients (90 %). Two patterns of urodynamic abnormalities were noted in the present study; 1) unstable bladders with single or multiple uninhibited contractions (18/30 patients, 60 %), and 2) small capacity, hypocompliant, hypertonic bladder (12/30 patients, 40 %). Post imipramine therapy significant symptomatic improvement was noted in 16/30 patients. On serial UDS, there was a 18-20 % increase in maximum cystometric capacity (MCC) and 30-35 % increase in pressure specific bladder volume (PSBV) following one year of imipramine therapy in 16/30 patients and 11/30 patients, respectively. 4 patients failed to show any improvement in MCC and PSBV with imipramine, they had been initially diverted with vesicostomy and later required augmentation cystoplasty. CONCLUSION: Unstable bladders and those with marginal bladder capacity and compliance showed the best response to imipramine therapy. Fibrotic, small capacity, hypertonic bladders are less responsive to imipramine. However, a trial of imipramine therapy is still warranted in these patients, as only 4/12 (33.3 %) patients with fibrotic hypertonic bladders failed to show any response and ultimately required augmentation cystoplasty. Imipramine qualifies as an effective and cheap drug for valve bladders.
We highlight the spectrum of scintigraphic findings likely to be encountered in patients with ectopic gastric mucosa with illustrative cases for each such finding. 11 children (aged 4 months to 120 months, all males) underwent (99)Tc(m)-pertechnetate scintigraphic evaluation for ectopic gastric mucosa. Functioning ectopic gastric mucosa was detected in Meckel's diverticulae in three patients, in small bowel duplications in four, in a gastric duplication in one, and in intrathoracic foregut duplication cysts in three. Ectopic functioning gastric mucosa in Meckel's diverticulum, and gastric duplication is visualized simultaneously with the stomach while in intestinal duplications tracer activity can be visualized in the dynamic sequence and even before gastric tracer visualization. In the three patients with intrathoracic duplication cysts, the functioning ectopic gastric mucosa was evident only in the delayed (99)Tc(m)-pertechnetate images, much later than the visualization of stomach activity. Therefore, acquisition of delayed images are useful when the initial images are equivocal or negative in children with intrathoracic foregut duplication cysts. In addition, we suggest a hypothesis of a possible mechanism for the uptake of pertechnetate in ectopic gastric mucosa in some patients with intrathoracic forget duplication cysts. In conclusion, a variety of scintigraphic patterns may be found in patients with ectopic gastric mucosa undergoing (99)Tc(m)-pertechnetate scintigraphy depending upon the location and size of the ectopic tissue.
OBJECTIVE: To study the efficacy of alendronate, in the treatment of avascular necrosis (AVN) of the hip. METHODS: Sixty patients with AVN of the hip (100 hips with AVN) were studied. The follow-up period ranged from 3 months to 5 yr. The most common cause of AVN was steroids. Parameters studied were walking time, standing time, pain and disability on a visual analogue scale (VAS), range of motion of the hip, X-ray and MRI of the hip. All patients were treated with alendronate 10 mg/day (or 70 mg/week) along with 500-1000 mg of daily calcium and vitamin D supplements, and were advised to avoid weight-bearing. NSAIDs and analgesics were permitted as needed and were recorded. RESULTS: Forty-one patients (71 AVN hips) with AVN have been followed up for a minimum of 1 yr, 24 patients (42 AVN hips) for 2 yr and 21 patients (37 AVN hips) for more than 2 yr (average 37 month). Fourteen patients have been followed up for less than 1 yr (3-9 months). Significant reduction in pain and disability scores (P < 0.001) and significant increase in standing and walking time (P < 0.001) were observed. All hip movements improved at 1 yr (P value 0.000-0.009) with an insignificant decline after that (P > 0.001). Radiologically, the hips either stabilized in the same grade or progressed by one grade. MRI showed a decrease in marrow oedema in most cases at the 1-yr follow-up. Six patients (10 hips) required surgery and there were two (three hips) dropouts. The drug was well tolerated and there was a reduction in NSAID requirement. CONCLUSION: Alendronate reduces pain, improves function and retards AVN progression. Early surgical intervention can be avoided in most patients.
The effect of chlorpyrifos (CPF) and its metabolite, chlorpyrifos-oxon (CPO), on multidrug resistance-1 (MDR1) gene expression and efflux transporter function in Caco-2 cells was determined. The effect of CPF and CPO on gene expression in Caco-2 cells was tested as a function of time using RT-PCR and competitive PCR (compPCR) techniques. The RT-PCR results depicted a maximal effect of CPF exposure on MDR1 expression at 8 h, which decreased at 24 h. Studies with CPO displayed an initial increase in expression at 4 h only. The compPCR assays were conducted with the CPF-treated group to quantify the changes in gene expression levels. The compPCR data confirmed and quantitated the results from the time-course study using semiquantitative RT-PCR. In addition to the gene expression studies, changes in efflux transporter function were investigated using Caco-2 cells grown on semipermeable membranes in Transwell plates. The permeability of verapamil was determined in cells treated for 8 h with CPF. Efflux ratios demonstrated that verapamil was effluxed at a higher rate from the CPF-treated cells as compared to the control group, confirming the inductive action of CPF on transporter function. These results suggest that CPF has the potential to modulate the bioavailability of drugs via changes in expression and function of membrane efflux transporters.
Between 1991 and 1998, 28 out of 356 neonates with esophageal atresia and tracheoesophageal fistula (EATEF) required esophageal substitution. As only 8 returned (28%) for the esophageal replacement after initial esophagostomy and gastrostomy at our center, we were prompted to offer single-stage esophageal replacement when the primary repair had either failed or was not found feasible. Twelve full-term neonates (mean birth weight 2.32 kg) with EATEF who underwent esophageal replacement by gastric pull-up between 1998 and 2000 were reviewed. The indications were: major leak after primary repair (n=9); pure EA (n=2); and EATEF with a very wide gap (n=1). The average ages at presentation and gastric pull-up were 6.0 and 8.5 days, respectively. The patients were evaluated for gastric transit by a colloid radiopharmaceutical, for duodenogastric reflux (DGR) by hepatic immunodiacetic acid (HIDA) scan, and for gastric clearance and transit by contrast studies. Three patients had minimal leaks from the neck site, all of which healed well. Follow-up with nuclear scans and contrast studies to evaluate gastric emptying revealed obstruction in 1 case and DGR in 25% of cases. There were 2 deaths (16%), 1 due to complex congenital cardiac disease and the other due to septicemia. In view of the acceptable morbidity, mortality, and functional outcome following gastric pull-up, we recommend this procedure if it becomes inevitable in the neonatal period.
Gastro-oesophageal reflux (GOR) is a major cause of morbidity in children who undergo surgical repair for oesophageal atresia with tracheo-oesophageal fistula (OA/TOF). We performed a retrospective analysis to determine the incidence of GOR on radionuclide scintigraphy in symptomatic and asymptomatic OA/TOF patients in the first post-operative year. A total of 124 patients (74 males, 50 females), with a mean age of 3.5 months (range, 20 days to 12 months), were studied. Of these 124 patients, 67 were symptomatic and 57 were asymptomatic. On radionuclide scintigraphy, 73 patients (48 symptomatic and 25 asymptomatic) had reflux. Of the 48 symptomatic patients with scintigraphic studies positive for reflux, 79.2% (38) had proximal reflux and 20.8% (10) had distal reflux, whereas, of the 57 asymptomatic patients, 48% (12) had proximal reflux and 52% (13) had distal reflux. There was a significantly higher incidence of GOR in symptomatic children than in asymptomatic children (P<0.01). In particular, there was a significantly higher incidence of proximal GOR in symptomatic children than in asymptomatic children (P<0.001). In conclusion, the severity and incidence of GOR were significantly higher in symptomatic than asymptomatic OA/TOF patients in their first post-operative year. Scintigraphic evidence of proximal reflux correlates with the presence of symptomatic GOR.
Bladder function in patients with posterior urethral valves (PUV) has an immense impact on long-term continence and renal function. Bladder dysfunction was corelated with the initial surgical treatment in 67 patients with PUV treated between 1985 and 2000. Age at presentation, current age, duration of follow-up, initial surgical treatment (diversion or valve fulguration), trends of renal function tests, voiding disturbances, and changes in the upper tracts were recorded. Urodynamic studies were done in all patients to determine urine flow rates, residual volume, maximal cystometric capacity (MCC), bladder compliance, involuntary detrusor activity, and pressure-specific bladder volume (PSBV) at 30 cm water. The patients were divided into three groups depending on the initial treatment: fulguration (n = 38), vesicostomy (n = 25), and ureterostomy (n = 4). At the time of this study voiding symptoms persisted in 45 patients. Mean percent MCC (% MCC) was 62%, 96%, and 100% of normal in the vesicostomy, fulguration, and ureterostomy groups, respectively (P = 0.002). Large-capacity bladders were seen in 10.9% of patients, mostly in pubertal and post-pubertal boys who were treated initially by either fulguration or ureterostomy; vesicostomy adversely affected bladder capacity and compliance (P = 0.007). PSBV was decreased in 48% of patients in the vesicostomy group and was significantly lower in the other groups (P = 0.01). Mean percent PSBV was 75%, 95%, and 96% of normal in the vesicostomy, fulguration, and ureterostomy groups, respectively. Uninhibited contractions were present in 21 patients (14 in the vesicostomy group) (P = 0.01). The highest incidence of upper-tract deterioration was seen with %MCC below 60% of normal (P = 0.001). The predominant urodynamic patterns were: (1) fulgurated group: good-capacity, compliant bladder; (2) vesicostomy group: small-capacity, hyperreflexic bladder; and (3) ureterostomy group: good capacity, compliant bladder. Primary valve ablation is associated with better bladder function than vesicostomy and should be the treatment of choice in PUV. Also, vesicostomy and ureterostomy have distinctly different effects on bladder function.
To assess whether symptomatic unilateral ureteropelvic junction obstruction (SUUPJO) affects somatic growth and, if so, the parameters associated with it, 61 children (54 boys and 7 girls) who underwent pyeloplasty for SUUPJO without any other associated urological abnormalities were retrospectively studied. Height was compared with standard growth charts and was considered to be affected if it was below 2.00 Z-score. Such children were considered group B and the rest group A. Mean (+/-SD) age at presentation and mean (+/-SD) split renal function (SRF) (%) of the affected kidney were 6.0 +/- 4.0 years and 27.3 +/- 13.2, respectively, for the entire group. Somatic growth was affected in 16 (12 boys, 4 girls) children (26.2%). Urinary tract infection (UTI) was the presenting symptom in 11 (69%) and 5 (11%) children in groups B and A, respectively. Impaired somatic growth had no association with age at presentation or SRF, but a significant association (P < 0.001) was found with UTI. The mean post-surgery height percentile (2.92 +/- 4.85) over a mean follow-up of 3.37 +/- 1.86 years was significantly (P < 0.005) better compared with pre-surgery height percentile (0.67 +/- 0.96) in group B, indicating catch-up growth after surgery. In SUUPJO, somatic growth is affected. Presentation with UTI has a significant association, and height significantly improves after surgery in these patients.
We describe a variety of acute necrotizing enteritis that is endemic to the Indian subcontinent. During the period 1992-1998, 18 cases of acute jejunoileitis (AJI) were managed. Only those in whom the diagnosis was confirmed at laparotomy have been included in this study. The most common symptoms were abdominal pain (100%), fever (77%), and blood in the stool (100%). A stricture following conservative treatment was present in 1 case. The mean age at presentation was 6.5 years (range 6 months-12 years); the male:female ratio was 1.2:1. All cases were seen during May-October. Routine investigations and X-ray films were nonspecific. Stool cultures did not show any clostridia, shigella, or salmonella. The jejunum was involved most commonly; 28% of patients did not require a bowel resection. Only 1 child presented with shock; the mortality was less than 5%. Short-bowel syndrome resulted in 1 patient due to extensive disease. The histopathologic features that were characteristic of the disease were patchy transmural mucosal necrosis extending centrifugally with submucosal edema, interstitial hemorrhage, type III hypersensitivity reaction, and extensive neovascularization. This type of AJI seen in South/Southeast Asia does not seem to be due a to bacterial infective etiology; immune mediation is suggested. The milder forms can be confused with dysentery. Mild forms of the disease can be managed conservatively, but carry the risk of developing strictures. This disease should be suspected in cases of prolonged dysentery during the summer and autumn months.
The correlation between urinary tract infection (UTI), vesicoureteric reflux (VUR) and renal scarring was studied in 89 patients (177 renal units; 1 solitary kidney) during the period 1997-2000. There were 63 males and 26 females; ages ranged from neonates to 14 years. UTI was diagnosed on the basis of a positive urine culture, VUR was diagnosed and graded by micturating cystourethrogram (MCU), and renal scarring was assessed by technetium 99 m Tc-dimercaptosuccinic acid (DMSA) scan. Ultrasonography (US) was done to evaluate renal tract dilatation and other structural abnormalities. A follow up DMSA scan was performed approximately 6 months after the initial scan. VUR was present in 106 of the 171 renal units in which it was studied and absent in 65 units. The majority of the VUR was grade V. Renal scars were seen in 90 of 177 renal units at presentation and in 72 of the 163 renal units studied at follow-up. Some information was lacking in 31 patients; hence, the correlation between UTI, VUR, and renal scarring was done in 58 patients. The majority of the suspected scars at presentation were not seen at follow-up, but most of the established scars persisted. Only 2 renal units showed scars for the first time on follow-up. On US, approximately 50% of normal kidneys showed either suspicious or established scars on DMSA scan, and patients with bilateral abnormality on US showed renal scars. Renal scars were seen in 15 of 23 children without VUR, 17 of 18 with unilateral VUR, and 16 of 17 with bilateral VUR. Thus, there is a cause-and-effect relationship between UTI and renal scarring that is made worse by VUR. DMSA scans have been shown to be the most reliable method of assessing renal scarring, and an abnormal US scan showing upper-tract dilatation or a structural abnormality may have a predictive value in the detection of renal scarring.
Fertility is decreased in rats with experimentally-created unilateral undescended testis (UUDT). Although light microscopy has shown changes in the UDT, it has consistently failed to demonstrate any significant damage in the contralateral descended testis (CDT) of these animals in previous experimental studies utilizing the same model. To study the ultrastructural details of the CDT and the ipsilateral UDT using transmission electron microscopy (TEM) in rats with experimentally-created UUDT, in Wistar albino rat pups UUDT was experimentally created in accordance with the model described previously. Ten rats with UUDT were compared with 10 control rats. The UDT and CDT were harvested at 135 days of age and sections from specimens studied by TEM for changes in the seminiferous tubules, Leydig cells, and extracellular matrix. All observations were graded and analyzed for statistical significance. TEM of the testes of control rats was normal, while both the ipsilateral UDT and the CDT showed significant changes. Significant changes in the CDT included thickening of the basement membrane ( P=0.001), a decrease in spermatogenic cell lines ( P=0.02), vacuolation ( P=0.0001) and other features of degeneration ( P=0.02), single cell apoptotic figures ( P=0.02), and an increase in Sertoli-cell numbers ( P=0.01), size ( P=0.01), and endoplasmic reticulum ( P=0.01). Another significant feature was the occurrence of morphologic sperm abnormalities in the form of tail denudation ( P=0.02). The surgically-created UDT showed changes of gross degeneration and atrophy. TEM studies thus revealed gross ultrastructural damage to the UDT and similar subtle but significant changes in the CDT. These may explain the decrease in fertility in rats with UUDT.
A good bladder capacity and adequate outlet resistance determine success after staged reconstruction of bladder exstrophy (BE). Augmentation cystoplasty (AC) is an established salvage procedure to treat the small, noncompliant bladders of some of these children. In a series of 89 patients with BE treated over the last 12 years, 19 underwent detubularized augmentation colocystoplasty (ACC) as an adjunctive procedure. Nine underwent ACC at the time of bladder-neck reconstruction (BNR) for small bladder capacity and poor compliance; 10 underwent ACC as a secondary procedure after BNR for persistent urinary incontinence or poor bladder compliance and upper-tract deterioration. The follow-up period ranged between 6 months and 12 years (mean 41 months). Complications included symptomatic urinary-tract infection in 4 patients, recurrent epididymo-orchitis in 2, calculi in 3, colonic anastomotic dehiscence in 1, bladder-patch fistula in 2, and secondary coloureteric-junction obstruction in 1. There was no postoperative reservoir perforation or mortality. The upper tract remained normal or stable in all patients. Nine of the 19 patients are dry both day and night; 4 others are dry during the day but have occasional nocturnal wetting. Three patients have nocturnal wetting with stress incontinence and 2 remain incontinent. Seven patients can void effectively using abdominal contractions and 12 require clean intermittent catherization to ensure complete voiding. The indications and results of AC in BE from other series are reviewed. AC is an important and safe adjunctive procedure in a subset of BE patients with small and poorly compliant bladders. Despite the known complications, more liberal use of AC in BE is warranted.
OBJECTIVE: To assess the role of imipramine therapy after bladder neck reconstruction in patients with bladder exstrophy. PATIENTS AND METHODS: Seventeen children with bladder exstrophy who achieved partial continence after bladder neck reconstruction received imipramine (1.5-2 mg/kg body weight) for a mean duration of 9.5 months. Assessment before and after therapy included an objective classification of the continence status and artificial slow-fill cystometry. RESULTS: Eleven of the 17 patients had a good clinical response to imipramine, with an increase in the continent period to > 2 h, and in nocturnal continence. The urodynamic findings showed a significant improvement in the capacity, end-fill pressure, uninhibited contractions and '20 below' capacity. Only minor side-effects of the drug were reported. CONCLUSION: Imipramine has a role in patients who achieve partial continence after reconstruction, and who have a moderately small capacity bladder with poor compliance and uninhibited contractions.
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