A regional-based newborn hearing screening program: the Emilia-Romagna model after ten years of legislation.

Background: Hearing loss, occurring in 1-3/1,000 newborns in the well-babies population, is one of the most common congenital diseases, and hearing screening at birth still represents the only means for its early detection. Since 2011 the Emilia Romagna Regional Health Agency has recommended Newborn Hearing Screening for all babies at its birth points and for newborns moving to the region. The aims of this study are to analyze the results of this regional-based Newborn Hearing Screening program and to discuss the impact of the legislative endorsement on the organization. Material and methods: This is an observational retrospective chart study. The recordings of well-babies and babies at Neonatal Intensive Care Units were collected during the period from January 1st 2015 to December 31st 2020. The following data were included: Newborn Hearing Screening coverage, percentage of refer at otoacoustic emissions, prevalence and entity of hearing loss, unilateral/bilateral rate, presence of audiological risk factors. Results: More than 99% of a total of 198,396 newborns underwent the Newborn Hearing Screening test during the period January 1st 2015 to December 31st 2020, with a coverage ranging between 99.6% and 99.9%. Overall, the percentage of confirmed hearing loss cases was about 17-30 % of refer cases, 745 children received a diagnosis of hearing loss (prevalence 3.7/1,000). Considering profound hearing loss cases, these represent 13% of bilateral hearing loss. Conclusion: A regional-based Newborn Hearing Screening program is valuable and cost-effective. In our experience, the centralization of the data system and of the data control is crucial in order to implement its efficiency and effectiveness. Healthcare policies, tracking systems and public awareness are decisive for a successful programme implementation.

The music-related quality of life: Italian validation of MuRQoL into MUSQUAV questionnaire and preliminary data from a cohort of postlingually deafened cochlear implant users.

PURPOSE: Cochlear implant (CI) users do not receive much of the auditory information necessary for an accurate perception of music. This usually entails a dissatisfaction with the music they hear, so that their quality of life may potentially be affected. The main aim of this paper was to translate and validate into Italian an instrument to evaluate these aspects-The Music-Related Quality of Life Questionnaire (MuRQoL)-to help the work of clinicians and therapists. METHODS: The translation of the MuRQoL into "Questionario Musica e Qualità della Vita" (MUSQUAV) was done according to the international guidelines. The translated questionnaire was administered to normal hearing (NH) and CI users adults. Exploratory factor analysis, confirmatory factor analysis and known group method were used to confirm construct validity and applicability of MUSQUAV. RESULTS: We retrieved 225 results. The MUSQUAV questionnaire was acceptable according to the goodness-of-fit indices. The correlation between the items, evaluated using Cronbach's α coefficient, indicates a good internal consistency (> 0.80). The non-parametric Mann-Whitney test showed significant differences in the distinct populations tested. CONCLUSIONS: The MUSQUAV questionnaire is a valid, low-cost and rapid instrument for professional workers in the audiological field, especially useful in the assessment of the patients' perception and musical engagement.

No thyroid abnormalities in patients submitted to cardiac catheterization in the first eighteen months of life.

BACKGROUND: No data are available about the risk of thyroid disturbance after exposure to low-dose radiation due to the use of cardiac catheterization in the first years of life. AIM: To determine the risk of functional and morphological thyroid abnormalities in a homogeneous cohort of patients who underwent diagnostic low-dose radiation for heart catheterization during the first 18 months of life. SUBJECTS AND METHODS: Fifty-five patients, submitted to cardiac catheterization during the first 18 months of life, underwent evaluation of the thyroid function and structure after a median period of 13 yr since the first radiation exposure. Sixty-eight unexposed controls matched for age and sex, underwent the same protocol. Twenty-two patients were then re-evaluated after a median period of 22 yr. RESULTS: Thyroid function resulted normal in both patients and controls. The prevalence of small thyroid nodules and inhomogeneous structures in ultrasound study was not augmented in irradiated patients compared to controls. No thyroid tumors or reduced thyroid volume were observed. CONCLUSIONS: Neither functional nor morphological disorders of the thyroid gland were demonstrated after a period up to 24 yr in patients exposed to diagnostic ionizing radiation for cardiac catheterization during the first 18 months of life.

Non-enhanced MR angiography of renal arteries: comparison with contrast-enhanced MR angiography.

BACKGROUND: The main causes of renal artery stenosis (RAS) are atherosclerosis and fibromuscular dysplasia. Despite contrast-enhanced magnetic resonance angiography (CE-MRA) being a safe and reliable method for diagnosis of RAS especially in young individuals, recently it has been possible to adopt innovative technologies that do not require paramagnetic contrast agents. PURPOSE: To assess the accuracy of steady-state free-precession (SSFP) non-contrast-enhanced magnetic resonance angiography (NC-MRA) by using a 1.5 T MR scanner for the detection of renal artery stenosis, in comparison with breath-hold CE-MRA as the reference standard. MATERIAL AND METHODS: Sixty-three patients (33 men, 30 women) with suspected renovascular hypertension (RVHT) were examined by a 1.5T MR scanner; NC-MRA with an electrocardiography (ECG)-gated SSFP sequence was performed in 58.7% (37/63) of patients; in 41.3% (26/63) of patients a respiratory trigger was used in addition to cardiac gating. CE-MRA, with a three-dimensional gradient echo (3D-GRE) T1-weighted sequence, was performed in all patients within the same session. Maximum intensity projection (MIP) image quality, number of renal arteries, and the presence of stenosis were assessed by two observers (independently for NC-MRA and together for CE-MRA). The agreement between NC-MRA and CE-MRA as well as the inter-observer reproducibility were calculated with Bland-Altman plots. RESULTS: MIP image quality was considered better for NC-MRA. NC-MRA identified 143 of 144 (99.3%) arteries detected by CE-MRA (an accessory artery was not identified). Fourteen stenoses were detected by CE-MRA (11 atherosclerotic, 3 dysplastic) with four of 14 (28.5%) significant stenosis. Bland-Altman plot demonstrated an excellent concordance between NC-MRA and CE-MRA; particularly, the reader A evaluated correctly all investigated arteries, while over-estimation of two stenoses occurred for reader B. Regarding NC-MRA, inter-observer agreement was excellent. CONCLUSION: NC-MRA is a valid alternative to CE-MRA for the assessment of renal arteries.

Comparison between MRI with spin-echo echo-planar diffusion-weighted sequence (DWI) and histology in the diagnosis of soft-tissue tumours.

PURPOSE: Our aim was to assess the usefulness of magnetic resonance imaging (MRI) with spin-echo echo-planar diffusion-weighted sequences (SE-EPI-DWI) in the study of primary and secondary soft-tissue tumours by correlating the results of imaging and histology. MATERIAL AND METHODS: We retrospectively studied 23 patients (14 men, 9 women; age range 25-87 years) affected by soft-tissue lesions. The MRI study was performed with baseline and contrast-enhanced SE-T1, proton density/T2-weighted (PD/T2), fat-saturated (FATSAT) DP/T2 and single-shot SE-EPI-DWI (b value 50-400- 800s/mm2) sequences. RESULTS: We identified 7/23 benign lesions (three myxoid, four nonmyxoid) and 16/23 malignant tumours (four myxoid, 12 nonmyxoid) with a mean diameter between 21 mm and 20 cm. Qualitative analysis of DWI showed persistence of high signal intensity for increasing b-values in all malignant tumours. Quantitative DWI analysis of the apparent diffusion coefficient (ADC) maps showed a statistical difference between benign and malignant lesions. CONCLUSIONS: In our experience, DWI with qualitative and quantitative analysis correlated well with histology.

Psychological profile and social behaviour of working adults with mild or moderate hearing loss.

In this study, an assessment was made of the global assumption that working adults with a mild to moderate sensorineural hearing loss experience more negative emotional reactions and socio-situational limitations than subjects with no hearing problems and that a deterioration of health-related quality of life on these specific domains would occur. Comparisons between 73 hearing-impaired subjects and 96 controls, well-matched for socio-demographic variables, were performed using the HHIA, MOS 36-Item Short Form Health Survey (SF-36) and SFQ questionnaires scores and revealed that the former experience a higher level of perceived hearing handicap and a deterioration of health-related quality of life while investigating emotional and socio-situational domains than the latter (p < 0.005). While investigating the psychological distress dimension of the hearing-impaired subjects by means of the Symptom Check List (SCL-90-R), it emerged that they are more prone to depression, anxiety, interpersonal sensitivity, and hostility than subjects with no hearing problems (p < 0.05). It is argued that the sensory impairment, with its associated disability, may discourage hearing-impaired individuals from exposing themselves to socially challenging situations, producing isolation that leads to depression, irritability, feelings of inferiority. The same psychological symptoms, on the other hand, can compound and worsen the picture by influencing social behaviour of the affected persons. Further prospective studies are needed to address this issue. Nevertheless, it is concluded that Audiology Services, despite the time and costs involved, should improve their diagnostic ability by exploring more areas of hearing-impaired subjects concerns in order not to overlook their potentially reduced psychosocial well-being.

Validity of the Italian adaptation of the Tinnitus Handicap Inventory; focus on quality of life and psychological distress in tinnitus-sufferers.

The aim of this study was to determine the validity of the Italian translation of the Tinnitus Handicap Inventory (THI) by Newman et al. in order to make this self-report measure of perceived tinnitus handicap available both for clinical and research purposes in our country and to contribute to its cross-cultural validation as a self-report measure of perceived severity of tinnitus. The Italian translation of the Tinnitus Handicap Inventory (THI) was administered to 100 outpatients suffering from chronic tinnitus, aged between 20 and 82 years, who attended the audiological tertiary centres of the University Hospital of Modena and the Regional Hospital of Treviso. No segregation of cases was made on audiometric results; patients suffering from vertigo and neurological diseases were excluded. Pyschoacoustic characteristics of tinnitus (loudness and pitch) were determined and all patients also completed the MOS 36-Item Short Form Health Survey to assess self-perceived quality of life and the Hospital Anxiety and Depression Scale as a measure of self-perceived levels of anxiety and depression. The THI-I showed a robust internal consistency reliability (Cronbach's alpha = 0.91) that was only slightly lower than the original version (Tinnitus Handicap Inventory-US; Cronbach's alpha = 0.93) and its Danish (Cronbach's alpha = 0.93) and Portuguese (Cronbach's alpha = 0.94) translations. Also its two subscales (Functional and Emotional) showed a good internal consistency reliability (Cronbach's alpha = 0.85 and 0.86, respectively). On the other hand, the Catastrophic subscale showed an unacceptable internal consistency reliability as it is too short in length (5 items). A confirmatory factor analysis failed to demonstrate that the 3 subscales of the THI-I correspond to 3 different factors. Close correlations were found between the total score of the Italian translation of the Tinnitus Handicap Inventory and all the subscales of the MOS 36-Item Short Form Health Survey (SF-36) and the Hospital Anxiety and Depression Scale scores indicating a good construct validity. Moreover, these statistically significant correlations (p < 0.005) confirmed that the self-report tinnitus handicap is largely related to psychological distress and a deterioration in the quality of life. On the other hand, it was confirmed that the tinnitus perceived handicap is totally independent (p > 0.05) from its audiometrically-derived measures of loudness and pitch thus supporting previous studies that focused on the importance of non-auditory factors, namely somatic attention, psychological distress and coping strategies, in the generation of tinnitus annoyance. Finally the results of the present study suggest that the THI-I maintains its original validity and should be incorporated, together with other adequate psychometric questionnaires, in the audiological examination of patients suffering from tinnitus and that psychiatric counselling should be recommended for the suspected co-morbidity between tinnitus annoyance and psychological distress.

Aspiration: diagnostic contributions from bedside swallowing evaluation and endoscopy.

The aim of this study was to identify which characteristics, collected by bedside swallowing evaluation (BSE) and fiberoptic endoscopic evaluation of swallowing (FEES), are a risk or a protective factor for aspiration. This retrospective study included data on 1577 consecutive patients, collected by BSE and FEES. Bivariate analysis was performed to verify the association of each variable with aspiration (Chi-Square test). The variables associated with aspiration were entered into a multivariate logistic model to quantify this association. Several variables were significantly associated (p < 0.05) with aspiration; cooperation, sensation, laryngeal elevation and direct therapy were found to be protective factors against aspiration. The regression model identified the most variables related with aspiration, among which tracheotomy, material pooling and spillage. Patients able to perform dry swallows were 77% less likely to aspirate (protective factor). Several variables are involved in protection of airways during swallowing. Their interaction, in patients with swallowing disorders, offers the clinician the best means of interpreting BSE and FEES.

Measuring the psychosocial consequences of hearing loss in a working adult population: focus on validity and reliability of the Italian translation of the hearing handicap inventory.

Despite increasing demand for questionnaires for assessing hearing handicap and the effectiveness of some tools across different languages, empirical studies to evaluate the reliability and the validity of translations of original English questionnaires into an Italian version have not been reported in the literature, thus making comparisons of Italian experimental and clinical data across cultures and countries impossible. This study tested the global assumption that the Hearing Handicap Inventory for Adults (HHIA), that is one of the most widely used instruments in English-speaking countries, can be adapted to the Italian language maintaining the reliability and clinical validity of the original version. The English version of this 25-item, self-assessment questionnaire was developed by Newman et al. in 1990 and special emphasis was placed on emotional reaction and social limitations perceived by hearing-impaired subjects and scored separately. This tool was translated into the Italian language by a forward and backward technique, as established by the IQOLA (International Quality of Life Assessment) project. Overall, 94 subjects, aged 18-65 years, with acquired hearing impairment and 104 individuals with no hearing problems, well-matched for socio-demographic variables, were enrolled in the study in a case-control design. Reliability of the Italian version of HHIA was tested by measuring internal consistency and test-retest reproducibility. Validity was assessed by using construct, convergent and discriminant methods. A Cronbach's alpha coefficient near 0.90 confirmed a more than acceptable internal consistency and a highly statistically significant Spearman's correlation coefficient (< 0.005) between scores of the two administrations at an interval of one month documented an excellent stability of the questionnaire over time. Construct validity was demonstrated by a correlation between the severity of hearing loss and the score of questionnaire (< 0.005) and convergent validity was supported by a significant correlation between the scores of the emotional and socio/situational subscales of the HHIA to the analogous subscales of a health-related quality of life questionnaire (MOS 36-Item Short Form Health Survey) (< 0.005). Finally, since hearing-impaired subjects scored significantly higher than controls on HHIA (< 0.005), it clearly emerged that also the Italian version of HHIA differentiates the two populations (those with and those without hearing problems) demonstrating a robust discriminant validity. Given the lack of appropriate measures to assess hearing handicap in Italy, the results achieved in this study, confirm that the HHIA, Italian version, is suitable for both experimental and clinical use.

A mild phenotype of sensorineural hearing loss and palmoplantar keratoderma caused by a novel GJB2 dominant mutation.

Dominant GJB2 mutations are known to cause a syndromic form of sensorineural hearing loss associated with palmo-plantar skin manifestations. We present the genotype/phenotype correlations of a new GJB2 mutation identified in three generations of an Italian family (proband, mother and grandfather) whose members are affected by sensorineural hearing impairment associated with adult-onset palmoplantar keratoderma. In all affected members we identified a new heterozygous GJB2 mutation (c.66G > T, p.Lys22Asn) whose segregation, population frequency and in silico prediction analysis have suggested a pathogenic role. The p.Lys22Asn GJB2 mutation causes a dominant form of hearing loss associated with variable expression of palmoplantar keratoderma, representing a model of full penetrance, with an age-dependent effect on the phenotype.

Pacemaker and ICD oversensing induced by movements near the MRI scanner bore.

PURPOSE: The effect of the movement near the MRI scanner bore for people with a pacemaker (PM) or an implantable cardioverter defibrillator (ICD) is experimentally evaluated and discussed. METHODS: The authors performed in vitro measurements on a saline-filled human-shaped phantom (male, 170 cm height), equipped first with an MR-conditional PM (bicameral configuration, DDD programming), then with an MR-conditional ICD (biventricular configuration, detection algorithms enable but shock delivery disable). Both the devices were able to transmit in real-time the detected cardiac activity (electrograms) while moving the phantom around the MRI scanner. The phantom was also equipped with an accelerometer and a magnetic field probe to measure the angular velocity and the magnetic field variation during the experiment. Unipolar versus bipolar sensing mode and maximum sensitivity versus nominal settings were tested. RESULTS: The sensing functions of the PM and ICD systems began to react to motion induced electromagnetic interference starting at an angular velocity as low as 2 rad/s (|dB/dT| = 2 T/s). The motion induced EMI in PM and ICD systems was interpreted as sensed intrinsic heartbeats which resulted in inappropriate pacing inhibition and arrhythmia classification. At the maximum speed of about 6 rad/s (|dB/dT| = 3 T/s), the induced EMI affected classification of ectopic beats and two episodes of VF were inappropriately recorded. CONCLUSIONS: These results demonstrate that motion in and around an MR scanner can induce EMI significant enough to be misinterpreted by implanted PMs and ICDs leading to inappropriate changes in therapy. These findings highlight that PM or ICDs, including MR-conditional systems should not enter the MRI room, except in case of an examination under specified conditions.

Bilateral Gasser's ganglion sarcoidosis: diagnosis, treatment and unsolved questions.

Magnetic resonance imaging is currently the best means for confirming clinical suspicion of neurosarcoidosis as well as being useful in the follow-up of corticosteroid-treated patients. We report the case of a 34 year old male presenting suspected Heerfordt's syndrome with concentric facial hypesthesia. Mediastinal and parotid sarcoidosis was diagnosed and magnetic resonance imaging showed bilateral sarcoid involvement of Gasser's ganglion cisternae (such involvement was not revealed by computed tomography). The patient received corticosteroid therapy, with a clinical and radiological improvement. Magnetic resonance imaging showed disappearance of Gasser's ganglion lesions despite the persistence of mild facial hypesthesia. This case is noteworthy for its extremely rare lesion site. Post-treatment discrepancy between the clinical picture and imaging results is probably due to low MRI resolution threshold. 18-FDG positron emission tomography imaging might perhaps overcome the limits of magnetic resonance imaging.

Repeated visually-guided saccades improves postural control in patients with vestibular disorders.

One of the most recent and promising theoretical hypotheses for compensation of persistent asymmetry of dynamic vestibulo-ocular gain is sensory substitution. As a switch between oculomotor and vestibulo-ocular systems, saccadic eye movements are engaged in humans to compensate the angular displacement of the head towards the labyrinthine defective side thus preserving the foveal fixation of the target. This study focused on the possibility that saccadic eye movements might also compensate for the impaired vestibulo-spinal reflexes and force the postural system to a more effective control on upright stance and verified whether this sway-stabilizing effect could be applied to patients with vestibular disorders and balance dysfunction. In the first experiment, 27 patients with unilateral labyrinthine hypofunction, 24 patients with central vestibular disorders and 24 healthy volunteers were evaluated by static posturography in 3 different visual conditions: (a) eye open with fixation of a steady target, (b) eye closed, and (c) while performing horizontal visually-guided saccades. The percentage of individuals with a decreased body sway area during the oculomotor task was found to be higher in labyrinthine-defective patients as compared to those with central vestibular disorders and controls. In the second experiment, 46 patients with vestibular disorders both of central and peripheral origin, whose postural control improved by eye-tracking, as assessed by posturography, were later submitted to 12 consecutive training sessions based on repeated visually-guided saccades. Both the saccadic performances and postural control improved in all patients but a more pronounced effect was observed in those with peripheral vestibular disorders. Outcome of this rehabilitation technique was also corroborated by a general reduction of the perceived overall impairment from balance disorders as tested by a specific questionnaire.

Choosing the correct treatment for acute aortic type B dissection.

Acute type B aortic dissection is a life threatening disease process, which remains a clinical dilemma despite advances in technology, surgical technique and postoperative management. The variability of presenting symptoms, lack of a consensus on indications for treatment and differing opinions about the optimal timing for repair have added to the management confusion. Medical management has been the standard of care for acute uncomplicated type B dissection. Surgical repair and endovascular intervention are reserved for those who present with, or subsequently develop, dissection-related complications. Complicated dissections occur in 25% of cases and may include organ malperfusion, aortic rupture, periaortic hematoma, and uncontrolled hypertension. In the past decade thoracic endovascular aortic repair (TEVAR) has gained widespread acceptance as the modality of choice for the treatment of complicated type B dissection. This transition is representative of advances in technology, physician experience with aortic endografts and lower morbidity and mortality rates associated with TEVAR. The best medical therapy remains the standard of care for uncomplicated dissection, however this strategy fails to prevent long-term aortic-related morbidity and mortality. Recent data suggest that early TEVAR lowers aortic-related events and improves long-term aortic specific survival by covering the entry tear, promoting false lumen thrombosis and inducing aortic wall remodeling. The paucity of supporting data has created controversy surrounding the optimal treatment strategy for acute type B dissection. Nonetheless, recent healthcare trends show a paradigm shift towards the utilization of early TEVAR in acute type B dissection.

Nimodipine in otolaryngology: from past evidence to clinical perspectives.

As L-type voltage-gated calcium channels (VGCCs) control Ca(2+) influx and depolarisation of cardiac and vascular smooth muscle, they represent a specific therapeutic target for calcium channel blockers (CCBs), which are approved and widely used to treat hypertension, myocardial ischaemia and arrhythmias. L-type currents also play a role in calcium entry in the sensory cells of the inner ear. In hair cells of both cochlea and labyrinth, calcium cytoplasmic influx is the first physiological process that activates complex intracellular enzymatic reactions resulting in neurotransmitter release. Excessive calcium ion entry into sensory cells, as a consequence of L-VGCCs malfunction is responsible for over-activation of phospholipase A2 and C, protein kinase II and C, nitric oxide synthase and both endonucleases and depolymerases, which can cause membrane damage and cellular death if the cytoplasmic buffering capacity is overcome. Nimodipine, a highly lipophilic 1-4 dihydropyridine that easily crosses the brain-blood barrier, is generally used to reduce the severity of neurological deficits resulting from vasospasm in patients with subarachnoid haemorrhage. Moreover, due to its selective blocking activity on L-channel calcium currents, nimodipine is also suggested to be an effective countermeasure for cochlear and vestibular dysfunctions known as channelopathies. Indeed, experimental data in amphibians and mammalians indicate that nimodipine has a stronger efficacy than other CCBs (aminopyridine, nifedipine) on voltage-dependent whole-cell currents within hair cells at rest and it is the only agent that is also effective during their mechanically induced depolarisation. In humans, the efficacy of nimodipine is documented in the medical management of peripheral vestibular vertigo, sensorineural hearing loss and tinnitus, even in a pathology as complex as Ménière's disease. Nimodipine is also considered useful in the prophylaxis of damage to the facial and cochlear nerves caused by ablative surgery of cerebellopontine tumours; it has been recently hypothesised to accelerate functional recovery of recurrent nerve lesions during thyroid cancer surgery. Further trials with adequate study design are needed to test the efficacy of nimodipine in the treatment of vertigo due to cerebrovascular disease and vestibular migraine.

Dynamic endocrine testing and magnetic resonance imaging in the long-term follow-up of childhood langerhans cell histiocytosis.

Children treated for Langerhans cell histiocytosis (LCH) are at risk for short and long term endocrine sequelae, but biological predictors of specific deficits are not well defined. We evaluated the frequency and progression of LCH-related endocrine deficits during long term follow-up and assessed the ability of dynamic endocrine testing to identify patients at risk for late anterior or posterior pituitary hormone dysfunction. The 17 patients (5 males and 12 females) were followed a median of 10 yr after diagnosis of single system (n = 6) or multisystem (n = 11) disease. Study evaluations, performed a median of 4.1 yr after the diagnosis, comprised pituitary hormone responses to the appropriate challenge, 7-h water deprivation test, 3% hypertonic saline infusion, and magnetic resonance imaging (MRI). The six patients with GH deficiency at the time of evaluation had a significantly lower GH response to GHRH than the other patients [median peak, 7.3 vs. 21.5 micrograms/L (P = 0.03); median area under the curve, 4.7 vs. 13.5 micrograms/L (P = 0.03)]; levels in the latter group did not differ significantly from those in 20 age- and sex-matched controls with constitutional or familial short stature. Two patients who had GH responses to GHRH of 20.6 and 23 ng/mL at 2.8 and 9.5 yr of age developed GH deficiency at 6.5 and 11.2 yr of age, respectively. The TSH response to TRH was less than 10 mU/L in three patients, two of whom later developed central hypothyroidism. ACTH and cortisol responses to CRF, and PRL responses to TRH were normal in all cases, and LH and FSH responses to GnRH were compatible with pubertal stage. Abnormalities in arginine vasopressin responses to water deprivation or hypertonic saline infusion were seen only in four patients who had preexisting diabetes insipidus (DI); one patient who later developed DI had normal findings. On standard MRI, posterior pituitary hyperintensity was absent only in the patients with DI. Pituitary stalk thickening was seen in seven patients, including three who did not have DI and had normal arginine vasopressin responses. Delayed posterior and anterior enhancement on dynamic MRI was present in two patients, both of whom later developed central hypothyroidism. Patients with single system disease had a lower 5-yr probability of LCH reactivation (41% vs. 83% for those with multisystem disease; P = 0.21) and a significantly lower risk of endocrine dysfunction (P = 0.007). In this series, dynamic evaluation of pituitary function was not a useful predictor of late endocrine sequelae, with the possible exception of the progressively decreasing TSH response to TRH. Similarly, a standard MRI was not predictive, although dynamic imaging may be informative regarding evolving pituitary hormone deficiency.

Correlation between magnetic resonance imaging of posterior pituitary and neurohypophyseal function in children with diabetes insipidus.

The posterior pituitary lobe and stalk were studied by magnetic resonance imaging in 20 children with diabetes insipidus of different origins: primary familial autosomal dominant (n = 2) or idiopathic (n = 2), and secondary to craniopharyngioma (n = 6, resected in 5), to Langerhans cell histiocytosis (n = 5), to excessive water intake (dipsogenic; n = 3), to renal vasopressin insensitivity (n = 1), and to osmoreceptor dysfunction (n = 1). Of the four children with primary diabetes insipidus, the posterior bright signal was recognizable in two with the familial autosomal dominant form and one with the idiopathic form; in the latter, the pituitary stalk was thin, while it was normal in the first two patients; no posterior hyperintense signal with enlarged and gadolinium-enhanced pituitary stalk was observed in the fourth. The posterior hyperintense signal was absent without evidence of ectopic posterior pituitary tissue regeneration in five children with surgically removed craniopharyngioma and was doubtful in the child with unresected craniopharyngioma; the stalk was unrecognizable in all patients. In the five children with Langherans cell histiocytosis, the posterior bright signal was absent, while the stalk was normal in two and unexpectedly enlarged in three (uniformly in two and mainly at the level of median eminence and hypothalamus in one). All five patients with dipsogenic or nephrogenic diabetes insipidus or osmoreceptor dysfunction had normal images of posterior pituitary lobe and stalk. Normal posterior pituitary bright signal and stalk were found in all 25 healthy control children. Plasma vasopressin was undetectable in all patients except in nephrogenic one, in the child with osmoreceptor dysfunction, and in two of three dipsogenic children, the third mimicking partial neurogenic diabetes insipidus.(ABSTRACT TRUNCATED AT 400 WORDS)

Short and long-term effects of reserpine on the concentration of 1-(4-hydroxy-3-methoxyphenyl)-ethane-1,2-diol (MOPEG-SO4) in the brain of the rat.

1 Reserpine (1.25 mg/kg i.p.) induced an increase (172% of controls) in the concentration of 1-(4-hydroxy-3-methoxyphenyl)-ethane-1,2-diol sulphate (MOPEG-SO(4)) in rat brain and a decrease in the noradrenaline (NA) concentration to 50% of controls 2 h after injection. At this time the MOPEG-SO(4)/NA ratio was 0.28. Thereafter the MOPEG-SO(4) concentration declined and the NA concentration decreased further to 28% of control.2 Higher doses of reserpine (2.5 and 5 mg/kg i.p.) did not induce a larger increase in the concentration of MOPEG-SO(4).3 While a second dose of reserpine (1.25 mg/kg i.p.) given 24 h after the first did not increase the MOPEG-SO(4) concentration, amphetamine (5.0 mg/kg i.p.) administration or electrical stimulation significantly increased the concentration of MOPEG-SO(4).4 NA and MOPEG-SO(4) concentrations were examined during 5 days after a single dose of reserpine (1.25 mg/kg i.p.). While the concentration of NA started to return towards normal after 24 h, that of MOPEG-SO(4) remained at approximately 70% of controls during the entire period.5 The probenecid-induced accumulation rate of MOPEG-SO(4) was significantly lower 3 and 4 days after reserpine and returned to the control value on the fifth day. At this time the concentration of NA had reached 50% of the control value.6 These experiments indicate that MOPEG-SO(4) is not the major metabolite of NA during the initial phase of reserpine-induced NA release. Reserpine acts on the storage pool while amphetamine (like electrical stimulation) acts on the functional pool. During the first phase of post-drug recovery, there is a clear decrease in NA output which appears to be regulated by the concentration of NA in the storage pool.

Evolution of childhood central diabetes insipidus into panhypopituitarism with a large hypothalamic mass: is 'lymphocytic infundibuloneurohypophysitis' in children a different entity?

We report on a 15-year-old girl who had presented with acute onset central diabetes insipidus at the age of 8 years; this was followed by growth failure due to acquired growth hormone deficiency. Initial magnetic resonance imaging showed a uniformly enlarged pituitary stalk and absence of posterior pituitary hyperintensity. Frequent patient examination and magnetic resonance imaging gave unchanged results until after 5 years a large hypothalamic mass and panhypopituitarism were found. Dynamic magnetic resonance imaging documented hypothalamic-pituitary vasculopathy. Histopathological examination revealed perivascular inflammatory lymphoplasmic infiltrates with no granulomatosis or necrosis and negative staining for S-100 protein, suggesting autoimmune inflammatory disease (lymphocytic infundibuloneurohypophysitis?). The response to glucocorticoid pulses (30 mg/kg per day for 3 days i.v.) was favorable. the hypothalamic mass being halved and partial anterior pituitary function recovery maintained for 2 years after the start of treatment. We suggest that long-term surveillance is needed for isolated and chronic thickening of the pituitary stalk and that dynamic magnetic resonance imaging can contribute to the demonstration of hypothalamic-pituitary vascular impairment associated with local vasculitis.