Body growth in early diagnosed vesicoureteric reflux

Body growth was studied in 32 subjects with vesicoureteric reflux (VUR), diagnosed following the prenatal finding of urinary tract dilatation, who had normal renal filtration function and who received antibacterial prophylaxis by the first few days of life. They were followed for 1–5 years (mean 2.3 years). Most had persistent VUR during the 1st year of life. Body growth performance was compared with that of 94 subjects with VUR diagnosed and treated by us after the neonatal period. During the follow-up period, none of the patients with prenatally detected VUR had a height Z score below –2, nor a weight-for-height index below 90%, and 1 had variations in height Z score ≥1. The difference in the percentage of patients with prenatally detected VUR (1/32) and those with VUR diagnosed and treated after the neonatal period (20/94) who had variations in height Z score ≥1 was significant (P=0.035). Patients with prenatally detected VUR and normal renal filtration function, given antibacterial prophylaxis by the first few days of life, have normal body growth, although VUR still persists. Received: 19 March 1998 / Revised: 10 February 1999 / Accepted: 10 February 1999     

Serum beta2-microglobulin and immunoglobulin levels in young hemodialysis patients

 Amyloidosis is a complication of long-term hemodialysis treatment. The major histological feature of hemodialysis-associated amyloidosis (HAA) is the deposition of amyloid fibrils in the affected lesions, due, in part, to elevated serum β₂-microglobulin (β2M) levels. In vitro studies reveal that serum immunoglobulin light and heavy chains co-deposit with β2M in tissues affected by HAA. Only one study of HAA has been performed in young dialysis patients. We therefore assessed risk factors for HAA in a group (n=30) of young (18.7±0.9 years) patients receiving chronic, uninterrupted hemodialysis using cellulose acetate membranes. All patients initiated dialysis before reaching 18 years of age. The pre-dialysis serum β2M level was 49.7±3.9 mg/l (normal 0–2.4 mg/l). Since serum albumin was normal (4.3±0.1 mg/dl) and serum protein/albumin was elevated (1.7±0.0, normal 1.2–1.5), indicating increased circulating protein, we assayed immunoglobulins in the same patients. The serum immunoglobulin levels (expressed as a percentage of the total level of serum proteins) were elevated (21.3±0.9%, normal 11.1%–21.0%). The Kt/v was 1.37±0.03, suggesting that the high levels of serum β2M and immunoglobulins were not due to inadequate dialysis in these patients. Patients with residual renal function (Kr) did display significantly lower serum levels of β2M (33.2±2.3, P=0.03). Furthermore, improved clearance of β2M correlated with higher values of Kr (r=0.914). In contrast, serum levels of immunoglobulin (22.6±3.7, P=0.5) were unaffected by Kr. In addition, there was no correlation between older age at onset of dialysis and serum levels of either β2M (r=0.107) or immunoglobulins (r=0.321). Finally, the length of time on dialysis had no effect on serum levels of either β2M (r=0.105) or immunoglobulins (r=0.092). Taken together, these results indicate that young hemodialysis patients may be at risk for HAA. Received: 13 January 1998 / Revised: 1 June 1998 / Accepted: 2 June 1998     

Giant abdominopelvic epithelioid angiomyolipoma associated with tuberous sclerosis: Report of a case

(Received for publication on July 16, 1998; accepted on Mar. 11, 1999)     

Emotion-related cerebral asymmetry: hemodynamics measured by functional ultrasound

This study assessed the use of transcranial Doppler ultrasound in detecting selective changes in cerebral blood flow velocity during emotional processes. The role of the respective hemispheres in emotional processing is controversial. Cerebral control of emotional processing has previously been investigated by analysis of patients with unilateral brain damage, experiments with selective stimulation of only one hemisphere, and more recently by imaging techniques measuring local cerebral blood flow. We investigated mean flow velocity continuously and simultaneously in both the right and left middle cerebral arteries (MCAs) in 16 healthy right-handed young subjects at rest and during the performance of three tasks: task 1: 15 slides with nonemotional content; task 2: 15 slides with negative emotional content; task 3: 15 slides with nonemotional content with different content from that in task 1. The three tasks produced significantly different effects on the right and left hemispheres. During the two nonemotional tasks the increase in mean flow velocity over basal values was similar in the two MCAs (task 1: left MCA = 3.27 ± 1.9%; right MCA = 3.63 ± 2.1%; task 3: left MCA = 2.42 ± 0.7%; right MCA = 2.56 ± 1.3%); the negative emotional task was accompanied by a significantly higher increase in the right (11.31 ± 1.6%) than in the left MCA (4.72 ± 3.7%; analysis of variance two-way interaction: side of recording x task, F = 43.6, P < 0.001). These results show the possibility of obtaining specific functional information from bilateral transcranial Doppler ultrasound and suggest the involvement of the right hemisphere in emotional processing. Received: 4 March 1999 Received in revised form: 29 June 1999 Accepted: 5 August 1999     

The citalopram challenge test in patients with major depression and in healthy controls

Neuroendocrine challenge tests in depressed patients have revealed a blunted hormonal reaction to serotonergic stimuli. In the present study, citalopram was chosen as the serotonergic agent for neuroendocrine stimulation. Compared to earlier challenge agents, citalopram has the advantage of serotonergic selectivity, its application is well tolerated and the possibility of intravenous application reduces pharmacokinetic interference. Sixteen patients suffering from an acute episode of major depression and 16 healthy controls underwent the stimulation procedure with 20 mg of citalopram and placebo. Whereas significant differences in the secretion of prolactin and cortisol between citalopram and placebo challenge were observed in the control group, no differences were found in the group of depressed patients. Comparison of depressed patients and controls showed a significantly blunted prolactin secretion in patients. Differences in cortisol secretion following serotonergic stimulation with citalopram did not become significant. The stimulation procedure was well tolerated in all subjects, although a higher number of side effects was observed in the control group. The amount of side effects did not correlate with the hormone responses. These results are in line with the hypothesis of serotonergic hypofunction in depressed patients. In conclusion, the 20-mg citalopram challenge test is thought to be a promising tool for further investigation of serotonergic function in psychiatric illness.     

Screening for mutations in candidate genes for hypospadias

Hypospadias, a condition with a frontally placed urethral orifice on the penis, is the most common malformation in males. During fetal development several components are necessary for normal male genital development. Testosterone and dihydrotestosterone act via the androgen receptor but a defective receptor function results in different degrees of genital malformations. Testosterone-5α-reductase converts testosterone to dihydrotestosterone, which is crucial for normal differentiation, and a total lack of this enzyme results, in syndromes with hypospadias. The Wilms' tumour 1 (WT1) gene is expressed in the fetal gonad and genital malformations can occur due to WT1 gene mutations. These genes are therefore strong candidate genes for hypospadias. We have analysed 35 boys with hypopadias and one girl diagnosed as with complete androgen insensitivity syndrome, using exon by exon polymerace chain reaction (PCR) amplification of the AR, WT1 and 5α-reductase genes and screened for point mutations and performed subsequent DNA sequencing. No mutations in any of these genes were found in the 26 patients with isolated hypospadias. Two patients with severe hypospadias with cryptorchidism were found to carry mutations in the androgen receptor gene. Also the girl with clinically diagnosed complete androgen insensitivity was found to be homozygous for a splice mutation in the 5α-reductase gene. In summary, mutations in the WT1, AR and 5α-reductase genes are not common causes of isolated hypospadias. Received: 1 October 1997 / Accepted: 4 May 1998     

Full-Face Laser Resurfacing and Rhytidectomy

The Ultrapulse CO₂ laser (Coherent Inc., Palo Alto, CA, USA) was used in 239 patients, from March 1996 to July 1998, for full-face laser resurfacing. In 106 (43%) of these patients rhytidectomy was performed in the same procedure. All patients submitted to laser resurfacing were prepared for 1 to 2 months beforehand with retinoic acid and hydroquinone. The procedures were done under local anesthesia controlled by an anesthesiologist. A clear film dressing impregnated with silicone gel (Silon TSR; Bio-Med Sciences, Bethlehem, PA, USA) was used for 6 to 7 days and complete healing was observed in 7 to 10 days. Complications were exclusively dermatologic, without relation to surgery. No necrosis of the cutaneous flap was observed. Skin biopsies of 10 consecutive patients undergoing the combined procedures revealed no vascular impairment to the dermis. The patients were able to resume their activities 2 weeks after the procedure.     

National Surveillance System and Hib meningitis incidence in Italy

In 1994, the Italian Ministry of Health implemented a National Surveillance System to obtain data on the incidence of bacterial meningitis and its causative agents, including Haemophilus influenzae type b (Hib). As a consequence, case reporting of Hib meningitis is increasing year by year; in 1996, there were 126 notifications, of which 73% were in children under 2 years of age. Although underreporting still exists, parallel prospective or retrospective epidemiological surveys conducted in some Italian Regions allowed for partial correction of the incidence of Hib meningitis (up to 18.5/100,000 population in 1994).     

Long-term results of silicone expander for moderate and severe Brown syndrome (Brown syndrome “plus”)

Background: The treatment of Brown syndrome has been undergoing an evolution toward more effective procedures with fewer operative interventions. Dr Kenneth Wright has introduced a procedure of superior oblique muscle tenotomy with a silicone expander to reduce the incidence of overcorrection. Methods: There was a retrospective study of 20 eyes of 19 consecutive patients with moderate or severe Brown syndrome (Brown syndrome “plus”). Follow-up ranged from 12 to 72 months. The expander, which varies 6 to 10 mm in length, was placed in all patients in the tenotomized superior oblique muscle tendon 5 mm nasal to the nasal border of the superior rectus muscle using 7-0 or 8-0 Prolene suture without violating the inner layer of the intermuscular septum. The intermuscular septum was closed over the silicone expander. Results: One hundred percent of patients had resolution of the down shoot in adduction and some or full ability to elevate the eye in adduction. Twenty percent of patients required reoperation (12.5% using 5-8 mm expanders) for overcorrection. Restriction of downgaze was not seen postoperatively. Patients often show an undercorrection 1 to 6 months postoperatively and improve or occasionally overcorrect at 1 to 2 years postoperatively. One patient with a 10-mm expander extruded the implant. Discussion: Placement of a 5- to 8-mm silicone expander in the tenotomized superior oblique muscle tendon is an effective means of correcting Brown syndrome with a low rate of reoperation. Initial undercorrection should not discourage the surgeon because improvement may continue for up to 3 years. The goal of treatment should be to convert a moderate or severe Brown syndrome (Brown syndrome plus) to a mild Brown syndrome (“true” Brown syndrome). Conclusion: This technique reduces the need for either simultaneous or subsequent inferior oblique muscle weakening and represents an advance in the treatment of Brown syndrome. (J AAPOS 1999;3:328-32)     

Stabilization of rat serum proteins following oral administration of fish oil

The mechanism of action of fish oil (FO), currently used in different chronic inflammatory conditions such as rheumatoid arthritis (RA), is not completely understood, although it is thought that it could alter the metabolism of endogenous autacoids. In addition, we hypothesized that the known capability of fatty acids (FA) of stabilizing serum albumin and perhaps other proteins, may be of pharmacological relevance considering that it is shared by other anti-rheumatic agents (e.g. nonsteroidal antiinflammatory drugs). Thus, we studied the effect of oral administration of FO and corn oil (CO), a vegetable oil with a different composition, on the stability of rat serum proteins, evaluated by a classical in vitro method based on heat-induced protein denaturation. FO, and, to a lower extent, CO inhibited heat-induced denaturation of rat serum (RS): based on the inhibitory activity (EC50) of the major fatty acids against heat-induced denaturation of RS in vitro, it was possible to speculate that in vivo effects of palmitic acid (C16:0) and eicosapentaenoic acid (EPA, C20:5, n-3) may be more relevant than that of linolenic acid (C18:2). To better investigate this phenomenon, we extracted albumin from the serum of animals treated or not with FO with a one-step affinity chromatography technique, obtaining high purity rat serum albumin preparations (RSA-CTRL and RSA-FO), as judged by SDS-PAGE with Coomassie blue staining. When these RSA preparations were heated at 70 degrees C for 30 min, it was noted that RSA-FO was much more stable than RSA-CTRL, presumably due to higher number of long chain fatty acids (FA) such as palmitic acid or EPA. In conclusion, we provided evidences that oral administration of FO in the rat stabilizes serum albumin, due to an increase in the number of protein bound long chain fatty acids (e.g. palmitic acid and EPA). We speculate that the stabilization of serum albumin and perhaps other proteins could prevent changes of antigenicity due to protein denaturation and glycosylation, which may trigger pathological autoimmune responses, suggesting that this action may be involved in the mode of action of FO in RA and other chronic inflammatory diseases.