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Stable maintenance of 5alpha-reductase activity in long-term subcultures of fibroblasts derived from the foreskin.

BACKGROUND: There is up to a 50-fold variation in control subjects in current assays of 5alpha-reductase activity which makes interpretation difficult. It was therefore attempted in this study to establish an assay method which produced stable 5alpha-reductase activity in long-term subcultured foreskin fibroblasts. METHODS: Foreskin fibroblasts were obtained from three boys with phimosis (control subjects), three patients with Reifenstein syndrome and one patient with 5alpha-reductase deficiency (due to mutation L113P in exon 2 of the SRD5A2 gene). To maximize the number of cells in the DNA synthesis phase, cells were subcultured consistently to approximately 70% confluency. Thawed cells, frozen after the third subculture, were incubated for 24 h with [1beta,2beta-3H] testosterone. 5alpha-Reductase activity was expressed as the sum of formed [3H] 5alpha-reduced metabolites (separated by thin-layer chromatography). RESULTS: The full range of 5alpha-reductase activity in controls and patients with Reifenstein syndrome was 3.44-15.59 pmol/h per mg protein: a 4.53-fold variation. The activity in the patient with 5alpha- reductase deficiency was 0.52 pmol/h per mg protein. CONCLUSION: By the cell culture methods used in this study, which aimed to increase the number of cells in the DNA synthesis phase, foreskin fibroblasts maintained a considerably stable level of 5alpha-reductase activity during long-term subculture. Therefore, this assay method can be used for differential diagnosis of 5alpha-reductase deficiency from other relevant entities.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗

Prophylactic therapy with granisetron in the prevention of vomiting after paediatric surgery. A randomized, double-blind comparison with droperidol and metoclopramide.

The antiemetic efficacy of droperidol, metoclopramide and granisetron was compared with placebo in the reduction of vomiting after paediatric surgery (the extremities; inguinal hernia; and phimosis) during general inhalational anaesthesia. One hundred children, ASA physical status I, 4-10 years of age, were enrolled in a prospectively, randomized, double-blind investigation and assigned to one of four treatment regimens: placebo (saline, n = 25), droperidol (50 micrograms.kg1, n = 25), metoclopramide (0.25 mg.kg-1, n = 25) or granisetron (40 micrograms.kg-1, n = 25). These drugs were administered intravenously (i.v.) after inhalation induction of anaesthesia. A complete response, defined as no emesis and no need for another rescue antiemetic during the first 24 h after anaesthesia, occurred in 60%, 76%, 68% and 88% of patients who had received placebo, droperidol, metoclopramide and granisetron, respectively (P < 0.05; overall Fisher's exact probability test). The incidence of adverse events postoperatively was not different among the treatment groups. In conclusion, granisetron 40 micrograms.kg-1 is a better antiemetic than droperidol and metoclopramide when compared to placebo for the prevention of postoperative emesis in children.

Antiemetics↗

Pretreatment with oral clonidine attenuates cardiovascular responses to tracheal extubation in children.

This study was designed to evaluate the effects of diazepam and clonidine orally given preoperatively on cardiovascular responses to tracheal extubation in children. Fifty children, ASA physical status I, aged 4-10 years, undergoing minor elective surgery (inguinal hernia, phimosis) received orally, in a randomized, double-blind manner, diazepam 0.4 mg.kg-1 or clonidine 4 microgram.kg-1 (n=25 of each). These drugs were administered 105 min before an inhalational induction of anaesthesia. The same standard general anaesthetic technique was employed throughout. The maximum changes in heart rate (HR), systolic blood pressure (SBP) and diastolic blood pressure (DBP) were less in patients who had received clonidine than in those who had received diazepam (HR, 12 vs 24; SBP, 14 vs 26; DBP, 9 vs 16; mean, P < 0.05). In conclusion, compared to diazepam given orally, pretreatment with oral clonidine attenuates haemodynamic changes associated with tracheal extubation in children.

Administration, Oral↗

Penile paraffinoma.

A 40-year-old Caucasian man, originating from Ukraine, but living for the last year and a half in Portugal, presented to our department with a one year history of an irregular penile mass and phimosis. He reluctantly admitted that 8 years previously he had multiple mineral oil injections in the penis, for the purpose of penile enlargement. A diagnosis of penile paraffinoma was made and surgical excision was performed at the urology department. Paraffinoma results from mineral-oil injections: such practice is very rare in the western world nowadays, however, it still is performed in many Eastern European Countries.

Adult↗

[Complete occlusion of the frontal capsule after cataract-operation in a patient with pseudoexfoliation syndrome--a case report and review of literature].

BACKGROUND: Pseudoexfoliation syndrome (PEX) is associated with an increased risk for the development of capsular fibrosis including capsular phimosis. A complete occlusion of the anterior capsular opening is, so far, a rarely reported phenomenon. PATIENT: Here we report the case of a 75-year-old female patient who suffered from a secondary open-angle glaucoma caused by PEX. Three months after an uneventful cataract surgery with capsulorhexis, phakoemulsification and implantation of a posterior chamber lens (PMMA) the anterior capsular opening of her left eye was completely occluded. RESULTS: The patient reported a slow loss of vision (20/40 immediately after cataract surgery to 20/200 at the follow-up visit three months later). The examination revealed a complete closure of the capsulorhexis with thick, central fibrous material. The diameter of the capsulorhexis was extensively diminished. Reopening of the anterior capsular opening utilizing a YAG laser was achieved and visual acuity increased to 20/40. Additionally, the fibrotic, secondary cataract of the posterior capsule was removed, again with a YAG laser, five weeks after the first intervention and, now with a free optical axis, the patient's visual acuity increased further to 20/30. CONCLUSION: The excessive production of fibrosis and the tendency towards a shrinkage of the diameter of the capsulorhexis postoperatively in patients with PEX may lead to a complete occlusion of the capsulorhexis. Even in such extreme cases, the reopening of the anterior and posterior capsule by a YAG laser is possible and, as demonstrated here, leads to a morphologically and functionally satisfying result.

Aged↗

[The pediatric pelvic kidney--a retrospective analysis].

OBJECTIVES: We evaluated children with pelvic kidney and their follow-up according to initial renal function and the appearance of concomitant urological pathologies. PATIENTS AND METHODS: In a retrospective analysis of our case notes, we studied 17 children (8 female, 9 male) who had been referred to our department for further investigation between 1994 and 2002 in whom we found a pelvic ectopic kidney. RESULTS: The mean age of the patients at the initial investigation was 72.5 months (range, 2 - 156 months); 10/17 were referred to our department for further investigation and clarification of a suspected renal agenesis, the remaining 7/17 children presented with urinary tract infection (1/17), nocturnal enuresis (3/17), hypertension (1/17) and phimosis (2/17). The nuclear medicine scan performed at the initial investigation in 14/17 children revealed that the function of the ectopic kidney had been reduced to one-third in contrast to two-thirds for the orthotopic kidney (p = 0.002). Overall global renal clearance was normal in all children. In 8/17 patients, the ongoing control nuclear investigations, on average 26.2 months later, revealed unchanged overall function of the kidney, we did, however, find a slight improvement of the ectopic renal function as compared to initial investigation which was not statistically significant (p = 0.683). In the period of this retrospective analysis, surgical correction of an accompanying pathology was performed in 23.5 % (4/17) of the children (vesico-ureteteric reflux operation in two cases, surgery for pelvic ureteric junction obstruction in one case, and nephroureterectomy in one case of a nonfunctioning-kidney). A left-sided pelvic kidney was seen in 64.7 % (11/17) of cases, a right-sided ectopic kidney in 23.5 % (4/17), a pelvic fused kidney in 11.7 % (2/17), and a solitary left-sided pelvic ectopia with right-sided agenesis in 5.8 % (1/17) of cases. CONCLUSION: In the event of suspected renal agenesis on ultrasonography, the possibility of a pelvic ectopic kidney should always be included in the range of diagnoses. On ultrasonography, the pelvic kidney is best visualized inferior to the iliac vessels, in the presence of a filled bladder. It is more frequently encountered on the left side. Nearly one-thirds of our patients presented with concomitant pathologies and one quarter needed surgical intervention. Although the function of the ectopic kidney alone was reduced by (2/3), the overall renal clearance was normal and remained stable in the course of the observation period.

Age Factors↗

Penile paraffinoma: self-injection with mineral oil.

We present a 64-year-old patient with a 9-cm firm, irregular penile mass associated with phimosis, erectile dysfunction, and voiding difficulty. After he reluctantly admitted to multiple penile mineral oil self-injections for enlargement, surgical excision was performed. Pathologic examination was consistent with mineral oil granuloma (paraffinoma). Within several weeks after surgery, his erectile dysfunction and voiding complaints resolved. Paraffinomas have been encountered with the use of various oily substances injected for cosmetic purposes. Despite early warnings, these agents continued to be used to treat conditions ranging from hemorrhoids to wrinkles and even baldness. Fortunately, most of these fads have been abandoned by medical professionals, but the complicating lesions have been documented as having lag times as long as 30 years. Complete surgical excision remains the treatment of choice to prevent recurrence. Increased public awareness is needed for the prevention of this physically and psychologically debilitating problem.

Granuloma↗

Penile paraffinoma: self-injection with mineral oil.

We present a 64-year-old patient with a 9-cm firm, irregular penile mass associated with phimosis, erectile dysfunction, and voiding difficulty. After he reluctantly admitted to multiple penile mineral oil self-injections for enlargement, surgical excision was performed. Pathologic examination was consistent with mineral oil granuloma (paraffinoma). Within several weeks after surgery, his erectile dysfunction and voiding complaints resolved. Paraffinomas have been encountered with the use of various oily substances injected for cosmetic purposes. Despite early warnings, these agents continued to be used to treat conditions ranging from hemorrhoids to wrinkles and even baldness. Fortunately, most of these fads have been abandoned by medical professionals, but the complicating lesions have been documented as having lag times as long as 30 years. Complete surgical excision remains the treatment of choice to prevent recurrence. Increased public awareness is needed for the prevention of this physically and psychologically debilitating problem.

Granuloma↗

The circumcision question.

Neonatal circumcision continues to be a controversial subject. The American Academy of Pediatrics has revised its earlier policy, stating that newborn circumcision has potential benefits as well as risks and emphasizing the need to explain these issues to parents considering the procedure so that an informed decision can be made. Compared with circumcised males, uncircumcised males are at greater risk for urinary tract infection, sexually transmitted disease, phimosis, paraphimosis, and balanoposthitis. Complications of circumcision include infection, hemorrhage, and meatal stenosis. Analgesia should be prescribed for the substantial pain associated with the procedure. Alternatives to circumcision include retraction of the foreskin with use of local or general anesthesia. Regular foreskin hygiene is important for all males, whether circumcised or not.

Circumcision, Male↗

Diagnosis and clinical presentation of premalignant lesions of the penis.

Although a considerable number of penile cancers may arise de novo, certain potentially premalignant conditions do exist. We account in some detail for precancerous growths, which may initially be misclassified and not submitted to proper therapy and follow-up. At one end of the spectrum disorders exist that are generally considered as medically benign, such as warty tumors; at the other end growths occur that are highly indicative of being potentially invasive, i.e. giant condylomas, bowenoid papulosis, eythroplasia of Queyrat and Bowen's disease. We also focus on elucidating the clinical behavior of some inflammatory conditions, which may either be of pathogenic significance for squamous cell carcinoma development or give rise to differential diagnostic problems, most importantly lichen sclerosus et atrophicus (balanitis xerotica obliterans). We advocate a vigilant approach for histopathological evaluation whenever any clinical diagnostic uncertainty or therapeutic recalcitrance exists. We also favor the administration of highly active topical therapy against penile chronic inflammatory conditions such as lichen sclerosus et atrophicus, careful clinical follow-up of these cases and surgical treatment of phimosis.

Carcinoma in Situ↗

Is carbon dioxide laser treatment of lichen sclerosus effective in the long run?

OBJECTIVE: To evaluate the long-term results after carbon dioxide laser treatment of lichen sclerosus. MATERIAL AND METHODS: We evaluated 62 consecutive patients with histologically verified lichen sclerosus admitted between 1985 and 1991. All patients were treated with a carbon dioxide laser with an output of 15-20 W and a defocused beam. This treatment vaporized the macroscopically altered area of the glans penis. Phimosis and superficial meatal stenosis were treated at the same session. Fifty-three men were still alive in 2004, and we were able to contact 50 of them (94%). The patients were asked about recurrence of symptoms, any visible penile lesion and recurrence of meatal stenosis. All patients who reported symptoms were invited to visit the outpatient clinic for further examination. RESULTS: The median follow-up time was 14 years. At follow-up, 40 patients (80%) had no local symptoms and no visible lesion. Ten patients had minor symptoms but only two required further treatment. CONCLUSION: Long-term follow-up of carbon dioxide laser treatment of lichen sclerosus shows that the treatment is safe and gives good results.

Adult↗

Peyronie's disease as a complication of chronic graft versus host disease.

Apart from one report of phimosis, involvement of the penis has not been reported as a complication of chronic GVHD. We report a patient with recurrent chronic GVHD who developed skin discoloration of the penile shaft, together with erectile dysfunction consistent with Peyronie's disease. Histological features were consistent with sclerodermatous change. These features suggest that the penis may be a target organ in chronic GVHD.

Adolescent↗

A sporadic case of delayed implantation after in-vitro fertilization in the human?

A possible case of delayed implantation after in-vitro fertilization (IVF) is described. The patient was sterilized in 1981, and made fertile again by tubal anastomosis in 1988. In 1990 and 1992 the patient had two right-sided tubal pregnancies, the first was treated with prostaglandin instillation, the second with salpingectomy. In connection with the salpingectomy in 1992, the left tube was observed to be constricted in the middle part and with phimosis of the ostium. In 1994 three IVF embryos were transferred, but 15 days after the transfer, serum human chorionic gonadotrophin (HCG) was negative (< 10 IU/ml). Seven weeks after the embryo transfer, menstruation was still missing, and the serum HCG was now positive (329 IU/ml). Subsequent ultrasound scans were compatible with an intrauterine pregnancy, progressing normally, but 5 weeks delayed compared with the oocyte aspiration. The pregnancy was successfully carried to term. Such a long delay in detection of HCG, in association with a normal pregnancy, has not been described in the literature before.

Adult↗

Effects of ondansetron in the prevention of postoperative nausea and vomiting in children.

BACKGROUND: Postoperative nausea and vomiting (PONV) is a commonly observed adverse effect of general anesthesia. Recently, ondansetron, a new serotonin3 (5-hydroxytryptamine3) receptor antagonist was shown to be effective in the prophylaxis and prevention of chemotherapy-induced nausea and vomiting in children and adults as well as of PONV in adults. The aim of the current study was to evaluate the capacity of ondansetron to prevent PONV in pediatric patients. METHODS: Two hundred children (132 boys and 68 girls) 2-10 yr of age received general inhalational anesthesia for surgical procedures (the extremities; ear, nose, and throat; inguinal hernia and phimosis; and dentistry) of an expected duration of less than 90 min. This study was divided into two phases: prophylaxis and rescue treatment. For prophylaxis, patients were randomly assigned to two groups: one group received an intravenous injection of 0.1 mg/kg ondansetron, and the other group received a placebo before surgical incision under double-blind conditions. For rescue treatment, only placebo patients were included; as a rescue medication they received an intravenous injection of 0.1 mg/kg ondansetron or 0.02 mg/kg droperidol according to a prestudy randomization under double-blind conditions. Incidence and severity of PONV (PONV score 0 = no nausea and no retching; 1 = complaining of sickness and retching; 2 = vomiting one or two time in 30 min; 3 = vomiting more than two times in 30 min) was recorded over a 4-h period in the postanesthesia care unit. Within 72 h of the procedure, a follow-up nurse interviewed the parents for late-onset nausea in the children. RESULTS: With regard to prophylaxis, 10% of patients receiving ondansetron had PONV during the 4-h observation period versus 40% of those receiving placebo (P < 0.001). The incidence of vomiting alone (PONV score > or = 2) was 5% and 25%, respectively (P < 0.001). There were no significant differences between ondansetron and droperidol in the treatment of PONV. However, at the end of the 4-h period, ondansetron patients were less sedated than were patients who had received droperidol (P < 0.01). Interviews with parents could be performed for 143 of 200 children (76 ondansetron and 67 placebo). Twenty-four children (15 ondansetron and 9 placebo) showed late-onset PONV after the 4-h observation period but within 24 h of the procedure (19.7% vs. 13.4%; P not significant). CONCLUSIONS: Ondansetron is effective in the prevention of PONV in pediatric patients for the first 4 h after general anesthesia. Lower sedation scores with ondansetron compared with droperidol may be an advantage, especially in ambulatory surgery. However, the incidence of late-onset PONV (> 4-24 h) was not influenced by prophylactic treatment with one dose of ondansetron preoperatively.

Anesthesia, General↗

Surgical treatment of balanitis xerotica obliterans.

Balanitis xerotica obliterans, or kraurosis penis, is a chronic progressive scleroatrophic process of the penis, prepuce, and urethral meatus. This syndrome is due to lichen sclerosus et atrophicus of the genital region. We have observed 32 patients, whose ages ranged from 24 to 78, with different clinical and pathologic findings. Clinical symptomatology consisted of painful erection with secondary impotence, burning, itching, and urinary disorders. The treatment in the early stages is pharmacologic; stenosis of the meatus, phimosis, scar adhesions, fissures, and erosions of glans and prepuce prescribe a surgical treatment. We have performed modified circumcision, meatotomy and meatoplasty, removal of the scleroatrophic tract and subsequent grafting. The functional results were satisfactory.

Adult↗

Balanitis xerotica obliterans in boys.

PURPOSE: Balanitis xerotica obliterans (BXO) is a chronic dermatitis of unknown etiology most often involving the glans and prepuce but sometimes extending into the urethra. We report our 10-year experience with BXO in pediatric patients. MATERIALS AND METHODS: Our pathology database was queried for all tissue diagnoses of BXO from 1992 to 2002. Available charts were reviewed and patient presentation, clinical and referral history, operative procedure(s) and postoperative course were recorded. RESULTS: A total of 41 patients had a tissue confirmed diagnosis of BXO. Median patient age was 10.6 years. Of the patients 85% were 8 to 13 years old and all had referrals available for review. The most common referral diagnoses were phimosis (52%), balanitis (13%) and buried penis (10%). No patient had the diagnosis of BXO at referral. Of the patients 19 (46%) underwent curative circumcision or redo circumcision and had no recurrence at a mean followup of 12.5 months (range 1 to 57). A total of 11 patients (27%) had BXO involvement of the meatus and underwent circumcision combined with meatotomy or meatoplasty. Nine patients (22%) required extensive plastic operation(s) of the penis, including buccal mucosa grafts in 2. CONCLUSIONS: The incidence of BXO in pediatric patients may be higher than previously reported, with the diagnosis rarely made by pediatricians. Our study demonstrates that older patients, those with BXO involvement of the meatus and those with a history of surgery for BXO tend to have a more severe and morbid clinical course.

Balanitis↗

Orbitoblepharophimosis syndrome: a 16-year perspective.

The orbitoblepharophimosis syndrome is a congenital malformation of the orbitopalpebral region. It is an autosomal-dominant condition typified by palpebral and orbital phimosis, ptosis, epicanthus inversus with telecanthus, and enophthalmia. It has three forms: minor, major, and extreme. It is a rare malformation affecting both sexes. The gene responsible is 3q21-24. Surgical treatment involves three to four operations: orbital remodeling by burring and grafting (intraorbital and extraorbital), epicanthus correction, and ptosis operation. Results varied depending on the severity of the form and the quality of the tissues. The authors present a series of 50 patients who were treated for this syndrome over the past 16 years.

Adolescent↗

Correction of blepharophimosis with silicone implant suspensor.

BACKGROUND: Blepharophimosis is a rare autosomal-dominant syndrome involving the periorbital region. It is characterized mainly by eyelid ptosis, palpebral phimosis, telecanthus, and epicanthus inversus. METHODS: Ten patients ranging in age from 2 to 22 years who presented with blepharophimosis were retrospectively studied over the past 14 years; they had an average follow-up of 7 years and 5 months. They were treated at the Division of Plastic Surgery of the Faculty of Medicine, University of São Paulo. Eight patients presented with the complete form, including epicanthus, telecanthus, and severe palpebral ptosis, and two presented with the incomplete form. The epicanthi and telecanthi were corrected with Mustardé's technique, and the ptosis was treated by frontalis suspension with a soft, preformed silicone device called the silicone implant suspensor. RESULTS: The results were classified as good, fair, and poor, according to the postoperative position of the upper eyelid in relation to the scleral limbus, medial canthus position, and residual epicanthal fold. The results were assessed as good in seven cases and fair in three. CONCLUSIONS: The silicone eyelid suspensor was effective in improving the patients' difficult condition. It was a better alternative than the use of fascia lata, because it permitted an easier readjustment of the eyelid margin and it offered the possibility of creating a well-defined palpebral fold, which is usually absent in this group. The results were maintained with long-term follow-up. The silicone suspensor was safe and long-term results were good in both children and adults.

Adolescent↗