Deflazacort Versus Prednisolone: Randomized Controlled Trial in Treatment of Children With Idiopathic Nephrotic Syndrome

Background:

Corticosteroids are the main therapy of nephrotic syndrome and goal of corticosteroid therapy is to obtain maximum clinical benefit with minimum adverse effects. Children are more vulnerable to side effects of corticosteroids related to growth and adrenal suppression, so a search for an alternative steroid with fewer side-effects is underway. Deflazacort is an oxazoline derivative and preliminary data suggest reduced osteoporosis, lesser growth retardation and weight gain with deflazacort.

Objectives:

This study was done to compare the effectiveness and safety of deflazacort in idiopathic nephrotic syndrome.

Patients and Methods:

Twenty five children with age between 2 to 12 years, with idiopathic nephrotic syndrome were enrolled. They were randomly assigned to receive deflazacort (Group A, n = 12) or prednisolone (Group B, n = 13) and were followed up for six months.

Results:

All children of group A and 11 of group B had remission. Two children from group B were steroid resistant. Mean time taken to induce remission was significantly (P = 0.012) less in group A (10.25 ± 2.41 days) than group B (12.55 ± 1.44 days). One patient in group A had relapse on follow up as compared to 3 in group B (P = 0.58). Statistically significant difference (P = 0.03) in change in mean height was found between group A (2.13 ± 0.50cm) and B (1.44 ± 0.45 cm), with group B gaining less height.

Conclusions:

Remission rate in both groups was comparable although time taken to induce remission was shorter in deflazacort group and there was a significant difference in change of mean height on follow up with prednisolone group gaining lesser height.     

Spontaneous choledochal cyst rupture in pregnancy with concomitant chronic pancreatitis

Choledochal cysts are rare cystic transformations of the biliary tree that are increasingly diagnosed in adult patients. We report here a case of spontaneous rupture of a choledochal cyst in a pregnant young lady with chronic pancreatitis.     

Laparoscopic pyeloplasty compared with open pyeloplasty in children

BACKGROUND AND PURPOSE: Open dismembered pyeloplasty by a retroperitoneal approach remains the reference standard for correcting ureteropelvic junction (UPJ) obstruction in children. We prospectively compared the results of laparoscopic and open pyeloplasty in children with UPJ obstruction. PATIENTS AND METHODS: During the period April 2003 to March 2005, 15 children underwent laparoscopic pyeloplasty and 14 children underwent open pyeloplasty. The two groups were similar in age and mean weight. We prospectively analyzed and compared operative time, postoperative pain and activity, complications, and hospital stay in the two groups. The mean follow-up was 23 and 24 months in the laparoscopy and open surgery group, respectively. RESULTS: The mean operative time was significantly shorter in the open surgery group (159 +/- 21.39 [SD] v 214 +/- 32.26 minutes; P = 5.874 x 10(-6)). Postoperative discomfort/pain on day 7 was significantly less in the laparoscopic group, and the mean hospital stay was significantly shorter (P = 0.018019). CONCLUSIONS: The benefits of laparoscopic pyeloplasty include a high reproducible success rate comparable to the results achieved by open pyeloplasty. Our results indicate minimal morbidity such as pain and a quick return to normal activities. The hospital stay is significantly reduced, although the operative times are long compared with open pyeloplasty.     

Cystoscopy-assisted laparoscopic partial cystectomy

BACKGROUND AND PURPOSE: Laparoscopic partial cystectomy is performed in selected patients with isolated diseases, such as bladder endometriosis, pheochromocytoma, leiomyoma, and malignant bladder tumors. Laparoscopic partial cystectomy is indicated for a solitary bladder tumor that is distant from the bladder neck, the ureteral orifices, and the trigone, to allow a resection margin of 1 to 2 cm. We report our experience with cystoscopy-assisted laparoscopic partial cystectomy. MATERIALS AND METHODS: The bladder was mobilized adequately by laparoscopy. Intraoperative cystoscopy was performed Cystoscopic guidance was used for the initial cystotomy. Further excision of the bladder tumor with a safety margin of 1.5 to 2 cm was performed under laparoscopic vision. RESULTS: Three patients underwent cystoscopy-assisted laparoscopic partial cystectomy. Cystoscopy aided in planning a proper and adequate safety margin around the tumor as well as helped in marking the initial cystotomy. CONCLUSIONS: Cystoscopic assistance during laparoscopic partial cystectomy helps to properly place the initial cystotomy as well aids in planning the safety margin around the tumor. It is safe, easy, and does not add to increased operative time or morbidity.     

Concomitant choroid plexus papillomas involving the third and fourth ventricles: A case report and review of the literature

Choroid plexus papillomas (CPP) are histopathologically benign and rare central nervous system (CNS) neoplasms arising from the epithelium of the choroid plexus. The most common site of presentation of these lesions is in the fourth ventricle in adults and lateral ventricles in children. Third ventricular CPP are uncommon. In this study, we present a case of a 66-year-old woman with complaints of progressive confusion, lethargy, and weakness who was found to have concomitant third and fourth ventricular masses on imaging studies. The patient underwent a biopsy of the third ventricular mass. The biopsy was followed by staged resections of the fourth and third ventricular masses, respectively. Pathology from the biopsy and both resections was benign CPP. Multifocal concomitant CPP is rare. Concomitant CPPs may be secondary to mere coincidental tumor occurrence or to biologic seeding of cerebrospinal fluid (CSF) from a primary CPP despite otherwise benign histopathology. The primary treatment for CPP is surgical resection. Post-operative chemotherapy or radiation for CPP is of controversial benefit.     

Survival trends in elderly patients with glioblastoma multiforme: resective surgery, radiation, and chemotherapy

BACKGROUND: It is appropriate to investigate and to determine survival trends following glioblastoma multiforme treatment using resective surgery, radiation therapy, and/or chemotherapy in patients aged 59 years and higher. METHODS: We retrospectively reviewed 30 elderly patients (> or =59 years old) who were treated for histopathologically confirmed glioblastoma multiforme at our tertiary care institution from 1990 through 2002. All patients were treated with steroids. In addition, 22 patients underwent resective surgery (RS), 17 patients underwent radiation therapy (RT), and 10 patients underwent chemotherapy (C). Many patients underwent these treatments in various combinations: 6 underwent biopsy only, 7 RS only, 6 RS+RT only, and 9 RS+RT+C. For each case, pre-treatment Karnofsky performance scores (KPS), tumor location, presenting symptoms and signs, associated surgical morbidity, and pre-existing medical conditions were also recorded. Patients were categorized into one of four treatment subgroups: Biopsy only, RS only, RS+RT, and RT+RS+C. For each of these subgroups, pretreatment KPS and post-treatment survival were compared. RESULTS: Post-treatment survival following biopsy only was 3.2 +/- 0.8 months (mean +/- SE); RS 2.2 +/- 0.5; RS+RT 5.5 +/- 1.2; RS+RT+C 13.6 +/- 2.1. A longer survival trend was noted for the RS+RT versus RS group (two-tailed unpaired t test, p = 0.02;), as well as the RS+RT+C group, which showed consistently higher survival in comparison to most of the other groups (p = 0.0021, 0.00039, 0.013 vs. the biopsy only, RS only, and RS+RT groups, respectively). No significant difference was found in KPS, comparing all individual groups versus each other (p > or = 0.06). Remarkably, 6 patients survived over 14 months (range, 14.1-22.7 months), all of which received RS+RT+C. CONCLUSIONS: This study suggests a significant improvement in elderly patients treated with the combination of resective surgery, radiation therapy, and chemotherapy, rather than either treatment alone or other combination. This significant improvement does not appear to be biased by pretreatment KPS, as mean KPS values did not significantly differ between any of these groups. However, a greater number of patients in each group must be considered to achieve the power to make more definitive treatment guidelines.     

Laparoscopic dismembered pyeloplasty in children

PURPOSE: Ureteropelvic junction obstruction remains the most common obstructive uropathy in children. Although laparoscopic dismembered pyeloplasty was described in a child in 1993, there have been few reports of laparoscopic Anderson-Hynes dismembered pyeloplasty in children. We report on a series of children who underwent laparoscopic Anderson-Hynes dismembered pyeloplasty. MATERIALS AND METHODS: The diagnosis of ureteropelvic junction obstruction was firmly established in all patients based on history, clinical examination, renal sonography and scintigraphy. Laparoscopic Anderson-Hynes pyeloplasty was performed using either 3 or 4 ports. Children were followed for urinary tract infection, and renogram was repeated at 3 months. RESULTS: A total of 16 children 5 months to 11 years old underwent laparoscopic Anderson-Hynes pyeloplasty between July 2002 and December 2003. No major intraoperative or postoperative complications were noted. One child with horseshoe kidney had development of fever and tenderness on the operated side on postoperative day 4. A percutaneous nephrostomy tube was placed and was removed 2 weeks later. CONCLUSIONS: Laparoscopic Anderson-Hynes pyeloplasty in children is too new to assess long-term outcome adequately. However, our study reveals improved outcome in the short term in the form of improved hydronephrosis and improved glomerular filtration rate on renal scan, and resolution of symptoms in all children.     

Intracranial fungal granuloma: analysis of 40 patients and review of the literature

OBJECTIVE: To describe the characteristics of patients diagnosed with intracranial fungal granuloma (IFG) in the largest reported series to date (to our knowledge). METHODS: A 22-year retrospective, multi-institutional review of 40 patients, aged 16 to 62 years (mean, 40.2 years), was performed in patients with histopathologically confirmed IFG. The variables were symptoms/signs at presentation, predisposing factors, location of granuloma, involvement of paranasal sinuses, diagnostic studies including blood and urine cultures, surgical procedures performed, specific organism identified, treatment, and prognosis. Plain x-rays, computed tomography, and/or magnetic resonance imaging scans were performed. RESULTS: Predominant symptoms included headache (83%), vomiting (65%), proptosis (48%), and visual disturbances (48%). Other symptoms were fever, nasal congestion, and seizures (7 [18%]). Common signs included papilledema (12 [30%]), with cranial neuropathy (I, III/IV/VI, and V in 4, 7, and 2 patients, respectively), hemiparesis (3), and meningismus (3). Predisposing factors were diabetes (16 [40%]), tuberculosis (7 [18%]), and immunocompromise related to renal transplant (2), non-Hodgkin's lymphoma (1), and human immunodeficiency virus (1). Location was primarily frontal (10 [25%]), with anterior cranial fossa involved in 8 (20%) patients; 6 (15%) patients had sellar/parasellar involvement. Eighteen (40%) had paranasal sinus involvement. Twenty-nine patients underwent craniotomy for resection, with 11 undergoing biopsy (of which 3 were transsphenoidally approached). Histopathology revealed aspergilloma (25 [63%]), mucormycosis (7 [18%]), cryptococcoma (3), cladosporidium (3), Bipolaris hawaiiensis (1), and Candida species(1). Microbiological analysis of the specimen was positive in 28 (60%) patients. All patients were treated with amphotericin B, fluconazole, and/or flucytosine. Only 26 patients completed amphotericin B therapy (due to nephrotoxicity). Mortality was 63%, most commonly due to meningoencephalitis (16 [36%]). CONCLUSIONS: High index of suspicion of IFG should exist for the following groups: (1) immunocompromised patients with intracranial lesions and (2) diabetic patients with intracranial and rhinocerebral mass lesions. Early diagnosis, surgical decompression, and a complete course of promptly initiated antifungal therapy are associated with better prognosis.     

Ninth cranial nerve stimulation for epilepsy control. Part 1: efficacy in an animal model

OBJECTIVE: To characterize the effects of stimulation of the nerve of Hering (HN; cranial nerve nine) in controlling seizure activity using a canine model. METHODS: Using penicillin applied topically to a region of the cerebral cortex, 16 seizure-type continuous epileptiform discharges were generated. Ten specimens of HN (five left-sided and five right-sided) were dissected from the cervical region in five dogs and stimulated at varying parameters to determine the effects in controlling epileptiform activity. Electroencephalography (using a multielectrode array), electrocardiography and other vital signs were continuously monitored for side effects. RESULTS: Resolution of continuous epileptiform activity following stimulation was found in 12 of 16 trials (75%); no spontaneous resolution was noted in the absence of stimulation, and stimulation significantly shortened seizure duration (p < 0.05). Mean epileptiform activity duration was 139 s prior to stimulation (range 1-432 s), with a mean poststimulation delay of 17 s until resolution and a mean interictal time of 399 s. Two specimens became free of seizure activity for the duration of our study (p < 0.001). No significant side effects (such as the potentially life-threatening cardiac problems seen with right-sided cervical vagal nerve stimulation) were found with stimulation of either left- or right-sided HN. Stimulation of other regional nerves (e.g. twelfth cranial nerve, nerves of the cervical plexus) failed to yield similar control of epileptiform activity. CONCLUSIONS: The results of this pilot study suggest that stimulation of the HN can successfully control focal seizure activity in the majority of cases. Pending further study, stimulation of the HN may have a role in the management of patients suffering from medically and otherwise surgically refractory epilepsy.     

Discernment of adipose versus nervous tissue: a novel adjunct solution in lipomyelomeningocele surgery

OBJECTIVE: To determine a solution capable of discerning adipose versus nervous tissue, to aid in surgical separation of the adipose tissue which appears to be visually indistinguishable from nervous tissue in lipomyelomeningoceles (LMMs). METHODS: The following solutes (in normal saline) were investigated, both at 25 and 37 degrees C: beta-carotene, vitamin D, vitamin E, lecithin, hydrogen peroxide, lipase, protease, hyaluronidase, partially purified collagenase, purified collagenase, trypsin, trypsin plus purified collagenase and non-solute-containing saline (control). Each solution was applied to a pediatric lipoma to determine gross effects over a period of approximately 30 min. If a solution appeared to affect the adipose tissue grossly, studies of functional in vivo sensory evoked and spontaneous potentials using that particular solution were conducted upon sheep spinal cord, nerve roots, dura and peripheral nerve. Additionally, histological studies were conducted to determine the effect of that solution upon adipose tissue, spinal cord, myelin, dura and nerve roots. RESULTS: Of all solutions investigated, partially purified collagenase type 1 (T1C; Lot M0M4322, Code CLS-1, Worthington Biochemical Corporation, Lakewood, N.J., USA) at 37 degrees C was the most successful in grossly altering the consistency and appearance of adipose tissue. This change was more apparent over 20-30 min following application of the solution to the adipose tissue. Solutions not containing T1C did not show appreciable results; purified collagenase plus trypsin did not appear comparable or superior to T1C. No significant histological or functional change was noted when comparing the spinal cord, nerve rootlets, myelin, dura or peripheral nerve from the T1C-treated group versus normal (untreated) control groups. CONCLUSION: T1C appears to be a potentially effective solution for application during LMM surgery in the acute setting, and such use of an adjunct solution may significantly aid in the safe surgical resection of LMMs. Pending further research, this technique may be applied for other indications which require discernment or alteration of adipose versus nervous tissue.