Cochlear involvement in Familial Mediterranean Fever: a new feature of an old disease

Objectives

In this study we first aimed to assess the cochlear functions in children with Familial Mediterranean Fever. The second aim was to investigate the correlation between the hearing levels and some clinical features of Familial Mediterranean Fever including the duration of the disease, age at onset, genetic analysis and colchicine use.

Methods

Thirty-four children with Familial Mediterranean Fever and 27 age matched children were included in the study. Following otologic examination, all children underwent audiometric evaluation, including Pure Tone Average measurements and Distortion Product Otoaoustic Emission testing. Audiological results of the two groups were compared and correlation between the audiologic status and clinical parameters of the disease like the duration of disease, age at onset, mutations and colchicine treatment were studied.

Results

Pure tone audiometry hearing levels were within normal levels in both groups. Hearing thresholds of Familial Mediterranean Fever patients were found to be increased at frequencies 8000, 10,000, 12,500 and 16,000 (p < 0.05). In otoacoustic emission evaluation, distortion products and signal-noise ratio of FMF children were lower in the tested frequencies, from 1400 Hz to 4000 Hz (p < 0.05). Interaction of the disease duration and age of disease onset was found to predict hearing levels, distortion products and signal-noise ratios of children with Familial Mediterranean Fever (F value = 2.034; p = 0.033).

Conclusions

To our knowledge this is the first study demonstrating cochlear involvement in children with Familial Mediterranean Fever which showed increased hearing thresholds at higher frequencies in audiometry together with decreased distortion products and signal-noise ratios demonstrated by distortion product otoacoustic emission testing. Similar studies must be carried out on adult patients to see if a clinical hearing impairment develops. The possible mechanisms that cause cochlear involvement and the effect of colchicine treatment on cochlear functions must be enlightened.     

The role of ATP in non-adrenergic sympathetic vascular control of the nasal mucosa in anaesthetized cats and dogs.

1. In anaesthetized cats and dogs, local intra-arterial injection of noradrenaline and alpha, beta-methylene adenosine 5'-triphosphate (mATP) reduced both nasal arterial blood flow and nasal mucosal volume (a measure of capacitance vessel function). The responses to mATP were not modified by pretreatment with the adrenoceptor antagonists phentolamine and propranolol or the purinoceptor antagonist suramin. The vascular effects of noradrenaline were not altered by suramin, but were virtually abolished by adrenoceptor antagonists. 2. After adrenoceptor blockade, frequency-dependent reductions in nasal arterial blood flow with sympathetic nerve stimulation were reduced by 25 and 39% in cats and dogs, respectively; whereas the volume response was reduced by 56% in cats and 54% in dogs. The remaining non-adrenergic sympathetic nerve-evoked vascular responses were not influenced by suramin. 3. During desensitization to mATP induced by local intra-arterial infusion for 5 min, the remaining non-adrenergic nasal blood flow and volume responses to sympathetic nerve stimulation were reduced in the dog but not in the cat. 4. It is suggested that both adrenergic and non-adrenergic mechanisms are involved in the sympathetic control of the nasal mucosa vascular bed of both species. Since desensitization to mATP markedly reduces the remaining non-adrenergic nasal vasoconstriction evoked by sympathetic nerve stimulation in the dog, ATP is a possible sympathetic mediator in the nasal vascular bed in this species.     

Prenatally detected ureteropelvic junction obstruction: clinical features and associated urologic abnormalities

Urologic congenital anomalies associated with ureteropelvic obstruction (UPJO) have been previously characterized; however, less data are available regarding these associations in a prenatally diagnosed population. A retrospective study was conducted to evaluate significant clinical features and urological anomalies associated with prenatally diagnosed UPJO. The records of 143 children with prenatally diagnosed hydronephrosis secondary to UPJO were retrospectively reviewed. The gender, side of obstruction, degree of hydronephrosis, associated clinical features, and urological anomalies were noted. Hundred and forty-three children (M/F = 2.7) with a total of 198 affected renal units (RU) presenting with unilateral (61%) or bilateral (39%) UPJO were enrolled. In cases of unilateral obstruction, the left side was affected in 60 children (68%). The grade of hydronephrosis was Grade 1 in 56 RU (28%), Grade 2 in 51 RU (26%), Grade 3 in 50 RU (25%) and Grade 4 in 41 RU (21%). Associated clinical features included prematurity (n = 7, 4.9%), twinning (n = 5, 3.5%) and presentation with renal failure (RF) (n = 2). Excluding contralateral UPJO, other urologic anomalies were encountered in 29 patients (20.3%). Associated vesicoureteral reflux (VUR) was encountered in 11 patients (7.7%, M/F = 2.7). Pyeloplasty was required more often in children with associated VUR (54.5 vs. 18.2%) (P = 0.01). Contralateral multicystic dysplastic kidney (MCDK) was encountered in six patients (M/F = 2), one of whom presented with RF. One child carried the diagnosis of Schinzel–Giedion syndrome (SGS), demonstrating severe developmental and neurological disorders and bilateral hydronephrosis. The more frequent occurrence of UPJO in males with predominantly left-sided location, association with VUR and MCDK, and increased frequency of bilaterality in our prenatally diagnosed patients were similar to historical reports. In addition, prematurity and twinning were independently associated with UPJO. The higher rate of pyeloplasty in patients with associated reflux warrants further investigation.     

Bone age in children with nocturnal enuresis

It is widely believed that nocturnal enuresis is caused by a hereditary delay in maturation of the various organ systems. In this study, growth and bone age were investigated in enuretic children. There was a significant bone age lag in the enuretic group compared to the control subjects of similar age (8.15 ± 1.56 years vs 9.45 ± 2.17 years, p < 0.05). It has been suggested that skeletal maturation also are retarded in nocturnal enuresis; and, it may be caused by the delayed maturation in regulatory functions of the central nervous system This revised version was published online in September 2006 with corrections to the Cover Date.     

The hateful patient revisited: Relevance for 21st century medicine

While the practice of medicine has changed over the years, including technological advances, access to medical information, and the narrowing of socio-economic and educational gaps between the clinician and his/her patients, the importance of the doctor-patient relationship has not diminished over time. This can be a very rewarding interaction. However, many physicians experience a great deal of anger, inadequacy and frustration, and much of the actual practice of medicine may become a burden rather than a source of satisfaction. Physicians may encounter a subset of patients who engender strong negative feelings, despair and even downright malice. An understanding of the "hateful patient" can therefore be very informative to the physician. Several categories of such patients may be described, and sensitivity to the phenomenon will lead to improved physician well-being, less self-destructive patient behavior and a lower risk of litigation. Several factors may assist the 21st century physician in managing the "hateful patient" in an empathic manner and in making some sense of why the patient has resorted to negative response patterns. Ultimately, a failure to consider these issues will result in poorer medical care and, no less important, reduced satisfaction of both patients and doctors. The intention of this article is to revisit the concept and to place it in the context of contemporary medical practice.     

The Effects of Melatonin on Angiogenesis and Wound Healing

Purpose The pineal gland hormone melatonin is a well-known neoroendocrine hormone. In addition to its immunomodulator effect, it also has a positive effect on monocyte, cytokine, and fibroblast proliferations, which also influence angiogenesis. This study investigated the effects of melatonin hormone on angiogenesis in wound healing on 100 Wistar-Albino rats.Methods The rats were divided into two groups. Melatonin dissolved in 0.9% NaCl was administered to the study group in a dose of 0.4mg/kg/rat per day (0.25cc/rat per day), and 0.9% NaCl to the control group in a dose of 0.25cc/rat per day. Incisions 5cm in length were made on the back skin of the rats and the wounds were closed with a skin stapler. Excisional biopsies from healing tissues were taken on the 3rd, 7th, 10th, 14th, and 21st postoperative days. Angiogenesis was evaluated in healing tissues by light and electron microscopy and by hydroxyproline level measurements.Results The commencement of neovascularization and a significant increase (P 0.05) in the number of vessels were observed at all stages of the study group but not in the control group. The tissue hydroxyproline levels were also higher in the study group than in the control group.Conclusions Melatonin may have a positive effect on both angiogenesis and wound healing.The preliminary results of this study have been published in the Turkish Journal of Surgery     

Diagnosis and Surgical Treatment of Morgagni Hernia: Report of Three Cases

Morgagni hernia is a rare congenital disorder, which is usually asymptomatic, but may cause respiratory or gastrointestinal symptoms. We reviewed the clinical findings of three patients with a Morgagni hernia, diagnosed and treated in our department between 1997 and 2000. The Morgagni hernia caused various symptoms in all three patients and surgery was performed via posterolateral thoracotomy in two, and via laparatomy in one. The hernial defect was closed by primary suturing in two patients and by synthetic mesh in one. All three patients had an uneventful postoperative recovery. We believe that the transthoracic approach is an effective way of repairing Morgagni hernia. Received: October 19, 2001 / Accepted: July 2, 2002 RID="*" ID="*" Reprint requests to: I.C. Kurkcuoglu, Ataturk Universitesi Tip Fakultesi, Gogus Cerrahisi ABD, 25240 Erzurum, Turkey     

Influence of Cardiovascular Risk Factors on the Outcome of Coronary Artery Bypass Surgery

Purpose. To assess the impact of gender, age, and other cardiovascular risk factors on the outcomes of patients undergoing coronary artery bypass grafting (CABG).Methods. A total of 5?067 consecutive patients undergoing isolated CABG between 1995 and 2000 were divided into the age groups: 25–49 years, 50–59 years, 60–69 years, and 70–84 years. Data on patient age, gender, smoking, serum cholesterol, blood pressure, body mass index, diabetes, family history, morbid obesity, and renal failure were retrospectively analyzed.Results. The percentage of women aged >60 years undergoing CABG was higher than the percentage of men aged >60 years (45.6% vs 36.6%). Most of the cardiovascular risk factors, except for smoking, were favorable in women (P < 0.001). The in-hospital mortality was 2.0% in women and 1.7% in men (P = 0.409). CABG was performed on significantly more men than women, accounting for 80.7% and 19.3% of the 5?067 patients, respectively (P < 0.001). However, the incidence increased remarkably in women aged >60 years.Conclusions. The risks of CABG may vary to some degree in accordance with the major cardiovascular risk factors. The risk of operative mortality was independent of gender in this study.