Laparoscopic retroperitoneal renal and adrenal surgery in children.

OBJECTIVE: To retrospectively assess the use of a retroperitoneal laparoscopic approach for simple nephrectomy and adrenalectomy in children. PATIENTS AND METHODS: All retroperitoneal laparoscopic renal and adrenal procedures carried out in children and completed between 1993 and March 2000 were reviewed retrospectively. Analgesic requirements, hospital stay, complications and blood loss were reviewed. The technique is described in detail. RESULTS: Forty-eight retroperitoneal laparoscopic procedures were completed in 48 patients (mean age 5.5 years, range 0.5-16). The procedures included nephrectomy (22), nephroureterectomy (15), renal biopsy (six), cyst ablation (two) and simple adrenalectomy (three). In all, 11 procedures were undertaken in children aged < 2 years. Forty-one (91%) of the children undergoing renal procedures were discharged in < 24 h. Two patients underwent three adrenalectomies. Two children required conversion to open surgery, one undergoing a right-sided adrenalectomy and one a nephrectomy. The mean operative duration for nephrectomy and nephroureterectomy was 75 min, and for adrenalectomy was 115 min. CONCLUSION: Renal and adrenal surgery in children is a safe and rapid procedure with retroperitoneal laparoscopy. The operative duration for nephrectomy and nephroureterectomy are frequently < 1 h. In addition, laparoscopic surgery offers significant advantages in terms of cosmesis and a quicker recovery.

Nephrotic syndrome associated with Kimura disease.

Kimura disease presents as benign subcutaneous swelling predominantly around the head and neck region. It has a high incidence of renal involvement. However, the pathogenesis of this association remains elusive. Only 2 pediatric cases and 11 adult cases of Kimura disease with renal involvement have been reported in the literature. In recent years many immunopathogenetic features suggesting an underlying T-cell and related cytokine defect have been noted in Kimura disease. We describe a unique case of an Asian boy who presented with nephrotic syndrome resistant to steroid and cytotoxic therapy, and 5 years later developed cervical lymphadenopathy consistent with Kimura disease. We also review the literature, summarizing the presentation, differential diagnosis, incidence of renal disease, prognosis, immunopathogenetic features, and therapy.

Medically resistant neonatal hypertension: revisiting the surgical causes.

OBJECTIVE: To present the importance of searching for the surgical causes of pharmacologically resistant hypertension in the neonatal population. STUDY DESIGN: A case report and discussion are provided. RESULTS: Severe hypertension in the neonatal period is uncommon and almost always has a secondary cause. Although a majority of hypertensive neonates can be successfully managed with medical therapy, some cases are resistant to pharmacological treatment. We report three hypertensive neonates who failed to respond to intensive multidrug therapy. This led to further evaluation and identification of obstructive uropathies in two neonates and renovascular disease that necessitated surgical intervention. Subsequently, all patients had prompt resolution of hypertension and normalization of renal function. All are now off antihypertensive medications and have normal renal function at 12 months of follow-up. CONCLUSION: Our report exemplifies the importance of the consideration of surgical etiologies for differential diagnosis in neonates with severe hypertension that is unresponsive to pharmacological therapy. Early diagnosis and prudent management of these etiologies reduce morbidity and mortality and preserve of renal function.

Applications of the KTP laser in the treatment of posterior urethral valves, ureteroceles, and urethral strictures in the pediatric patient.

PURPOSE: We describes our experience using the potassium titanyl phosphate (KTP)-532 laser in treating posterior urethral valves, ureteroceles, and urethral strictures in the pediatric patient. METHODS: A retrospective chart review was performed from 1987 to 1997 on a total of 33 pediatric patients who underwent retrograde endoscopic treatment for posterior urethral valves (PUV), ureteroceles (UC), and urethral strictures using a KTP-532 laser. RESULTS: Overall, our success rate was excellent in the treatment of valves and ureteroceles. With a mean follow-up of three years in the PUV group, no urethral strictures of micturation abnormalities were seen. The majority of ureteroceles were decompressed and only half of our patients required and additional procedure. Our experience with urethral strictures, however, was not as promising. All of these patients ultimately required open urethral reconstruction. CONCLUSION: The desirable thermal characteristics of the KTP laser, along with minimal complications and the availability of delicate pediatric endoscopic instruments have made this operation optimally suited for treating posterior urethral valves and ureteroceles in infants. However, the advantages for treating urethral strictures in children with the laser still remains to be established.

Retroperitoneal laparoscopic nephrectomy in children.

PURPOSE: We report our experience with retroperitoneal laparoscopic nephrectomy and nephroureterectomy in children, and describe our surgical technique. MATERIALS AND METHODS: Five and 15 children 9 months to 17 years old underwent nephrectomy with cystoscopy plus intravesical ureteral stump fulguration for ureteral ablation and nephrectomy only, respectively. Surgical indications were unilateral multicystic dysplastic kidney in 8 cases (parental preference for surgery), a refluxing, chronic pyelonephritic kidney in 5, renal vascular hypertension in 2, and hydronephrosis and chronic pyelonephritis in 5, including 3 in whom a nephrostomy tube was placed percutaneously before laparoscopic nephrectomy. Access was obtained by a 10 mm. incision made posterior to the anterosuperior iliac spine with dissection into the retroperitoneal space and trochar placement. Two and sometimes 3 additional 5 mm. ports were placed retroperitoneally. RESULTS: Average operative time was 1 hour 42 minutes. The most recent cases were performed in less than 1 hour and in 3 nephrectomy only required 30 minutes. All but 1 procedure were completed laparoscopically. One case was converted to open surgery secondary to obscured visibility due to bleeding. Blood loss in all cases was less than 30 cc (average 5 to 10). A total of 13 children were discharged home immediately postoperatively. Five children underwent concomitant procedures, including contralateral ureteroneocystotomy in 4, circumcision in 1 and cystoscopic fulguration of the ureteral stump in 5. Those who underwent ureteral reimplantation were hospitalized for 48 hours. One patient remained hospitalized for 3 days due to fever of unknown origin and 2 were admitted to the hospital for 23-hour observation. All children returned to full activity within 1 week of surgery. Analgesia consisted of 1 dose of ketorolac, bupivacaine injections at the incisional sites at the completion of the procedure, and acetaminophen postoperatively. CONCLUSIONS: As confirmed by parent questionnaire, patient satisfaction was excellent.

Effects of conventional peritoneal dialysates on leukocyte adhesion and CD11b, CD18 and CD14 expression.

Bacterial peritonitis is the most important complication of peritoneal dialysis (PD), limiting its widespread application. Conventional glucose-based peritoneal dialysates (G-PDS) depress oxygen consumption, chemiluminescence, superoxide production, phagocytosis, bacterial killing and actin polymerization in neutrophils (PMN) in vitro. Expression of adhesion receptors is critical to leukocyte activation, adhesion, migration and phagocytosis. The effects of G-PDS on basal and stimulated leukocyte adhesion molecule expression and leukocyte adhering capacity is unknown. We examined the effect of a five minutes incubation of whole blood in either HEPES-buffered saline or G-PDS containing 1.5% (83 mM), 2.5% (139 mM) or 4.25% (236 mM) glucose, at pH = 5.2, and pH = 7.4. PMN intracellular pH was measured spectrofluorometrically. Leukocyte CD11b, CD18 and CD14 were measured by flow cytometry using monoclonal antibodies in otherwise unstimulated cells or 60 minutes after lipopolysaccharide (LPS) stimulation. In addition, leukocyte adhering capacity to nylon wool was tested. In an attempt to dissect the effect of high glucose concentrations from that of the attendant hyperosmolality, the experiments were repeated with dialysates in which glucose was substituted by sodium chloride (NaCl-PDS) to attain identical osmolalities. G-PDS, as well as the mixtures of spent and fresh G-PDS, significantly depressed the basal PMN expression of adhesion receptors CD11b and CD18 and monocyte expression of CD14, and substantially mitigated the LPS-mediated up-regulation of CD11b and CD18. Likewise, G-PDS significantly inhibited leukocyte adhering capacity without affecting cell viability. Similar results were observed with NaCl-PDS. The observed abnormalities were primarily osmolality-dependent, and largely intra- and extracellular pH-independent. Impaired adhesion receptor expression and cell adhesion capacity shown here reveal another dimension of the G-PDS-induced leukocyte abnormalities.

Childhood AIDS nephropathy: a 10-year experience.

The objective of this study was to define the demographic, immunologic, and clinical characteristics of children with acquired immunodeficiency syndrome (AIDS) and AIDS nephropathy, and contrast this with the existing adult data. Data from 62 pediatric patients with AIDS who were treated at SUNY Health Science Center, Brooklyn, New York, between 1983 and 1993 were analyzed. Human immunodeficiency virus (HIV) infection was acquired during the neonatal period by vertical transmission (n = 60) or blood transfusion (n = 2). All children with AIDS who exhibited clinical nephropathy died (n = 16), with mean survival of 55.3 months. In contrast, 32 of 56 AIDS patients (70%) who did not manifest nephropathy were alive at the end of the study period. Patients with nephropathy were noted to have significantly lower CD4+ lymphocyte counts than those without nephropathy. These observations suggest that the predominant renal lesion in pediatric patients who acquired HIV infection during the perinatal period is focal segmental glomerulosclerosis, although a variety of other histological lesions were present. As in adults, the survival in children is dismal following the onset of clinical renal disease. In contrast to the adult population in whom multiple risk factors can potentially contribute to AIDS-associated nephropathy, occurrence of nephropathy in children with vertical HIV transmission provides convincing evidence for the pathogenetic role of HIV infection.

Focal segmental glomerulosclerosis in a child with acute lymphoblastic leukemia.

A 13-year-old boy was found to have acute lymphoblastic leukemia 4 months after the diagnosis of nephrotic syndrome was made. Renal biopsy revealed focal segmental glomerulosclerosis (FSGS). While his leukemia remains in remission 7+ years from diagnosis, he requires chronic hemodialysis due to renal failure. The multiple immunosuppressive antileukemia agents he received did not appear to affect the progression of his renal disease.

Strategies for optimizing growth in children with kidney transplants.

Children with a well-functioning graft continue to show growth retardation even with low-dose prednisone. We have attempted to utilize the steroid-sparing effect of cyclosporine by discontinuing prednisone after graft stabilization. Since 1983, 53 children have received cyclosporine as primary immunosuppressant for renal graft maintenance. The children, aged 6 months to 18 years, received 60 transplants. One-year and four-year patient survival for cadaveric transplants was 91% and 91%, compared with 96% and 96% for living related transplants. One-year and four-year graft survivals were 82% and 65% for cadaveric transplants (n = 25), compared with 91% and 63% for living related transplants (n = 35). Of 53 patients, 23 were able to discontinue prednisone and be maintained on monodrug cyclosporine therapy, and 21 of the 53 patients had growth hormone measured using L-dopa stimulation. In patients receiving more than 5 mg of prednisone daily, growth hormone levels were lower than normal (less than 10 ng/ml). Of 15 patients who had discontinued prednisone for more than 6 months, 13 showed accelerated growth by improvement in their standard deviation scores. In 4 pubescent children with growth retardation and need for maintenance prednisone, accelerated growth occurred following growth hormone administration for 3-6 months. Based on these data we suggest that (1) discontinuation of even very small doses of prednisone may be essential for normalizing growth hormone response to L-dopa and (2) further studies are needed to exploit the growth stimulation effect of recombinant growth hormone in transplanted children.

Long-term outcome of one haploidentical live related donor transplantation in children.

UNLABELLED: We report on 69 children, 6 months to 18 years old, who received a one-haploidentical live related donor transplantation from 11/72 through 4/89. Thirty patients were maternal and 22 were paternal recipients; 23 were Black, 27 Caucasian, 17 Hispanic, and 1 each Oriental and Arabic. The original diagnoses in these patients were aplastic/dysplastic kidneys 13%, obstructive uropathy 30%, focal segmental glomerulosclerosis (FSGS) 23% and lupus nephritis 10%. RESULTS: 1-, 5-, 10- and 15-year actuarial patient and graft survivals were 98, 84, 84, 84% and 94, 70, 41, 30% respectively. Actual 5-year graft survival of paternal recipients was better than maternal recipients (p less than 0.05). No differences were noted comparing actuarial graft survival between Blacks, Caucasians and Hispanics. 1/16 FSGS and 1/7 lupus nephritis patients had recurrence of their original disease. Posttransplant therapy in 37 patients consisted of azathioprine and prednisone while 32 patients received ciclosporin A (CSA) and prednisone. At transplantation, CSA and prednisone patients were younger, i.e., 10.7 years (range 0.5-18) compared to 13.3 years (range 4-18) and (p less than 0.03) in azathioprine and prednisone patients. The 5-year actuarial graft survival of azathioprine and prednisone patients was 57% compared to 76% in CSA and prednisone patients. 16/32 CSA and prednisone patients had their prednisone discontinued for a mean duration of 18 months (3-54 months). Ten of these 16 patients were prepubertal and had their growth hormone levels measured. All had an adequate level (greater than or equal to 10 ng/ml) poststimulation. Five of the 10 patients were less than 6 years of age and exhibited accelerated growth.(ABSTRACT TRUNCATED AT 250 WORDS)