Treatment of chronic portal-systemic encephalopathy with lactose in lactase-deficient patients

A controlled cross-over clinical comparison of lactose (50 g twice a day) versus neomycin (3 g/day) plus milk of magnesia, was carried out in ten cirrhotic patients with chronic portal-systemic encephalopathy and documented lactase deficiency. Serial semiquantitative assessments were done including: mental state, asterixis, number connection test, electroencephalogram, and blood ammonia levels. No patient developed deep coma while ingesting either lactose or neomycin plus milk of magnesia. However, a significant improvement of mental state, asterixis, number connection tests, and electroencephalograms was evident during lactose therapy. Apart from mild diarrhea and bloating, no severe side effects were noticeable during lactose treatment. Based on these results, we propose lactose as a valuable alternate treatment of portal-systemic encephalopathy in lactase-deficient populations.With technical assistance of Arabella Briones, BS, and Sofía Gil, BS.     

Advances in pheochromocytoma management in the era of laparoscopy

PURPOSE: To understand the advances that were made in the management of pheochromocytoma since laparoscopy was initiated at our institution. MATERIALS AND METHODS: Data for all patients who underwent surgical procedure for adrenal diseases had been recorded prospectively since September 2000, when laparoscopy was routinely initiated at our institution; all patients with a diagnosis of pheochromocytoma up to December 2005 had their data assessed (group 1). Charts for all patients with a diagnosis of pheochromocytoma who underwent surgery at our institution from 1990 to 1995 (group 2) were reviewed, and the data were compared with data from patients in group 1. RESULTS: In group 1, 24 patients with pheochromocytoma underwent 26 procedures at our institution, including five patients with extra-adrenal tumor and one patient with bilateral tumor; 18 adrenalectomies (one bilateral) were performed, and two extra-adrenal tumors were removed using a laparoscopic approach. In group 2, ten open adrenalectomies were performed in 9 patients (1 bilateral). The patients who underwent laparoscopy for either adrenal or extra-adrenal tumor had shorter operative times and hospital stay and less bleeding. CONCLUSIONS: Pheochromocytoma is a complex disease with potentially severe complications. Laparoscopic adrenalectomy can be safely performed for removal of either adrenal or extra-adrenal tumors. Patients spend a shorter time in the critical care unit after the procedure.     

RET/PTC rearrangement is prevalent in follicular Hürthle cell carcinomas

de Vries M M, Celestino R, Castro P, Eloy C, Máximo V, van der Wal J E, Plukker J T M, Links T P, Hofstra R M W, Sobrinho‐Simões M & Soares P
(2012) Histopathology  61, 833–843 RET/PTC rearrangement is prevalent in follicular Hürthle cell carcinomas Aims: The molecular alterations underlying follicular Hürthle cell carcinomas (FHCCs) are largely unknown. In an attempt to clarify this issue, we analysed a series of Hürthle cell tumours for the presence of RET/PTC and PAX8/PPARG rearrangements and BRAF, HRAS and NRAS mutations. Methods and results: We investigated a series of 20 follicular Hürthle cell tumours [17 FHCCs and three follicular Hürthle cell adenomas (FHCAs)]. RET/PTC rearrangements were found in 33% of FHCAs and in 38% of FHCCs. All RET/PTC‐positive FHCCs had a solid pattern of growth. PAX8/PPARG rearrangement was present in 27% of the FHCCs which displayed, in most cases, a follicular architecture. NRAS mutation was detected in one FHCC. An FHCC with a solid/microfollicular growth pattern scored positive for both RET/PTC and PAX8/PPARG rearrangement. Conclusions: Our study has shown a significant association between RET/PTC rearrangements and FHCCs with a solid growth pattern, thus raising the possibility of using tyrosine kinase inhibitors for the treatment of patients with FHCCs, which are often refractory to radioiodine treatment.     

Nonpharmacological treatment of lower urinary tract dysfunction using biofeedback and transcutaneous electrical stimulation: a pilot study

OBJECTIVE

To report a series of children with lower urinary tract dysfunction (LUTD) whose urge syndrome was treated by electrical stimulation, and their voiding dysfunction by biofeedback; none of the children were using anticholinergic drugs during treatment.

PATIENTS AND METHODS

In all, 36 children who presented with symptoms of urinary urgency and/or daily incontinence completed the treatment and were prospectively evaluated. The mean (range) follow‐up was 13.8 (4–24) months, and their mean age 7 (3–14) years, 17 children were aged <5 years. The children were divided into two groups: group 1, with urge syndrome treated with superficial parasacral electrical stimulation, and group 2, with voiding dysfunction, treated with biofeedback.

RESULTS

In group 1, the mean (range) number of electrical stimulation sessions was 13.1 (4–20). Of the 19 children treated, 12 had a complete clinical improvement, six a significant improvement, and one a mild improvement. In group 2, the mean (range) number of biofeedback sessions was 6 (4–14). Of the 17 children treated, there was complete improvement of symptoms in 10, significant improvement in two and mild improvement in five. Six children who had no resolution of symptoms after biofeedback had salvage therapy with electrical stimulation, after which four had complete improvement of symptoms, and two a 90% and 40% improvement, respectively. Taking the two groups together, after treatment, four children developed isolated episodes of urinary tract infection. Of 21 children with nocturnal enuresis, bed‐wetting continued in 13 (62%) after treatment.

CONCLUSION

In this short‐term follow‐up, the nonpharmacological treatment of voiding dysfunction using biofeedback, and of urge syndrome by electrical stimulation, was effective for treating LUTD in children.

     

The Macedo-Malone antegrade continence enema procedure: early experience

PURPOSE: The successful treatment of fecal incontinence can dramatically improve the quality of life of affected children. The introduction of the Malone antegrade continence enema provides the opportunity to manage previously resistant cases. However, using the to create this catheterizable channel is not always possible, and the duration of these antegrade enemas is a source of concern for the patients. We describe a new approach to create left continent colonic access to shorten the duration of these enemas, and report the experience gained from the first 9 cases managed at our institution. MATERIALS AND METHODS: During a 5-year period 9 patients underwent a Macedo-Malone antegrade continence enema at our institution. Incontinence was associated with myelomeningocele in 7 patients and anorectal malformation in 2. The antegrade continence enema procedure is begun by isolating a 2 cm flap in a tenia on the left colon (spleen flexure). A 12Fr silicone Foley catheter is placed on the mucosal surface of the flap to allow tubularization of the plate with interrupted polyglycolic acid 3-zero transverse sutures, creating an efferent tubular conduit. Antegrade colonic washouts were started 2 weeks after surgery with saline solution or tap water in all patients. RESULTS: Followup of our 9 cases ranged from 8 to 33 months (average 20.7). Enema volume varied from 250 to 800 ml, with administration taking from 45 to 60 minutes, and colonic evacuation occurred within 30 to 60 minutes of enema administration. Of the 9 patients 8 were completely continent and 1 was partially continent. Four patients experienced difficulty with catheterization initially because of stenosis of the stomal track. The affected stomas were dilated, which was successful in 1 case. Three patients subsequently required stomal revision. CONCLUSIONS: The Macedo-Malone procedure is a relatively straightforward operative approach providing an effective washout technique that is acceptable to parents and children.     

The tunica vaginalis dorsal graft urethroplasty: experimental study in rabbits

PURPOSE: We created an experimental model of urethral defect and then repaired it using a tunica vaginalis graft applied on the dorsal surface of the urethra. We studied the histological and radiological characteristics of free tunica vaginalis graft urethroplasty. MATERIALS AND METHODS: In 20 New Zealand rabbits a dorsal urethral defect was created by excising a portion of the dorsal urethral surface. The tunica vaginalis graft was placed dorsally over the corpora cavernosa and tied with 4 interrupted sutures. The mucosal margin of the urethral defect was sutured to the graft using 6-zero polydioxanone sutures in continuous fashion. The animals were divided into 4 equal groups and were sacrificed 14 days, and 4, 8 and 12 weeks after surgery, respectively. A retrograde urethrogram was done at autopsy. The penis was sent for histological analysis and an experienced pathologist evaluated the severity of acute and chronic inflammation, foreign body reaction and scar formation. RESULTS: There were no deaths related to the procedure and no intraoperative complications. All rabbits voided spontaneously after surgery. Retrograde urethrograms showed no fistula or stricture. As time after surgery increased, the signs of inflammation response disappeared, and the orientation of collagen fibrils and smooth muscle fascicles resembled that of a normal urethra. The mesothelial lining of the tunica vaginalis gradually became replaced by a more stratified epithelial lining, similar to the urothelial lining of the native urethra. CONCLUSIONS: In the current study we noted that a tunica vaginalis graft placed dorsally can be a successful urethral substitute in the animal model.