Polycythemia as rare secondary direct manifestation of acromegaly: management and single-centre epidemiological data

Polycythemia associated with acromegaly is usually caused by the systemic manifestations of the disease, such as sleep-apnea or concomitant erythropoietin-secreting kidney tumors. The recognition of underlying pathologies requires a thorough diagnostic process. We report a unique case of acromegaly with polycythemia, not caused by commonly described manifestations of the disease, and receding with octreotide therapy. The medical history of 141 acromegalic patients followed by the Endocrinology Unit of the San Martino University Hospital in Genoa has been also reviewed, together with the literature evidence for similar cases. The diagnostic workflow and 2-years follow-up of a 43-years old acromegalic, polycythemic man with a history of past smoking, moderate hypertension, and mental retardation are described. The hematological parameters of our cohort was retrospectively compared with those of a healthy, age/gender-related control group as well. Therapy with octreotide LAR, 20?mg i.m. q28d was begun soon after diagnosis of acromegaly in the polycythemic patient. Haematocrit level, hormonal setting, as well as pituitary tumor size and visual perimetry during treatment were recorded. Octreotide LAR treatment normalized hormonal alterations, as well as hematological parameters. Polycythemia has not recurred after 2?years of therapy. The median hemoglobin and hematocrit levels of the retrospectively analyzed cohort of acromegalic were significantly lower than normal ranges of a healthy, age/sex- related control population. In conclusions, polycythemia can be a direct, albeit rare, secondary manifestation of acromegaly, that must be considered during the diagnostic work-up of acromegalic patients presenting with such disorder.     

Remission of Type 2 Diabetes Mellitus Should Not Be the Foremost Goal after Bariatric Surgery

Background

Remission of type 2 diabetes (T2D) is a desired outcome after bariatric surgery (BS). Even if this goal is not achieved, individuals who do not strictly fulfill remission criteria experience an overall improvement. The aim of this study was to evaluate the metabolic control status in patients considered as diabetes “non-remitters.”

Methods

A retrospective study of 125 patients (59.2 % women) with preoperative diagnosis of T2D who underwent BS in a single center (2006–2011) was conducted. We collected anthropometric and metabolic parameters before surgery and at 1-year follow-up. T2D remission was defined according to the 2009 consensus statement: glycosylated hemoglobin (HbA1c) <6 %, fasting glucose (FG) <100 mg/dLs, and absence of pharmacologic treatment. We evaluated metabolic status of non-remitters, according to the American Diabetes Association's (ADA) target recommendations: HbA1c <7 %, LDL-c <100 mg/dL, triglycerides <150 mg/dL, and HDL-c >40 (male) or >50 mg/dL (female). Statistics: analysis of variance.

Results

Baseline characteristics (mean ± SD): age 53.5?±?9.7 years, BMI 43.5?±?5.6 kg/m², time since diagnosis of T2D 7.7?±?7.9 years, FG 162.0?±?56.3 mg/dL, HbA1c 7.7?±?1.6 %. ADA's target recommendations were present in 12 patients (9.6 %) preoperatively, and in 45 (36.0 %) at 1-year follow-up (p <0.001). Sixty-two (49.6 %) patients did not achieve diabetes remission; 26 (41.9 %) had now diet treatment, 30 (48.4 %) oral medications, and 6 (9.7 %) required insulin. Of the non-remitters, 57 (91.9 %) had HbA1c <7 % and 18 (40.0 %) achieved ADA's target recommendations. There were no differences between remitters and non-remitters in the number of individuals reaching ADA's combined metabolic control.

Conclusions

Although almost 50 % of the patients may not be classified as diabetes remitters, their significant improvement in metabolic control should be regarded as a success, according to most scientific societies' target recommendations.     

Outcome of patients with splanchnic venous thrombosis presenting without overt MPN: A role for the JAK2 V617F mutation re-evaluation

Introduction

Although investigation for JAK2 V617F mutation is recommended in patients presenting with splanchnic venous thrombosis (SVT), no specific clinical advice is given to SVT patients presenting without myeloproliferative neoplasms (MPN) and JAK2 V617F mutation. In MPN-free SVT patients, to investigate the clinical outcome, the clinical impact of re-evaluation for the JAK2 V617F mutation, and relationships with the occurrence and time to diagnosis of MPN.

Materials and Methods

A cohort of non-cirrhotic SVT patients, enrolled at a single centre and prospectively analyzed.

Results

In 121 SVT patients prospectively followed from 1994 to 2012, a MPN was present in 28 (23.1%). Additional 13 patients (10.7%) showed only the JAK2 V617F mutation. During the follow-up, the JAK2 V617F mutation and/or MPN were identified in 8 patients (median time of development: 21 months, range 6-120), whereas 72 remained (MPN and JAK2 V617F)-free until the end of the observation.The mortality rate was higher among patients presenting with MPN and/or the JAK2 V617F mutation than in patients who developed later or remained disease-free (p = 0.032). The thrombosis-free survival was lower in patients with (p = 0.04) or developing later MPN and the JAK2 V617F mutation (p = 0.005) than in patients (MPN and JAK2 V617F)-free. The incidence of bleeding was similar among groups.

Conclusions

MPN with or without circulating positive clones for JAK2 V617F mutation can occur long after a SVT, identifying at risk patients for new thrombotic events. If confirmed in other studies, re-evaluation for JAK2 V617F mutation may be of help in early MPN detection and clinical management of SVT patients.     

Effectiveness of collaborative care for depression in Italy. A randomized controlled trial

Trial design

This was a multicenter cluster-randomized controlled trial.

Participants

A total of 227 patients ≥ 18 years old with a new onset of depressive symptoms who screened positive on the first two items of the Patient Health Questionnaire-9 (PHQ-9) were recruited by primary care physicians (PCPs) of eight health districts of three Italian regions from September 2009 to June 2011.

Intervention

PCPs of the intervention group received a specific collaborative care program including 2 days of intensive training, implementation of a stepped care protocol, depression management toolkit and scheduled meetings with a dedicated consultant psychiatrist.

Objective

The objective was to determine whether a collaborative care program for depression management in primary care leads to higher remission rate than usual PCP care.

Outcomes

Outcome was clinical remission as expressed on PHQ-9 < 5 at 3 months.

Randomization

An independent researcher used computer-generated randomization to assign involved primary care groups to the two alternative arms.

Blinding

PCPs and research personnel were not blinded.

Results

The 223 PCPs enrolled recruited 227 patients (128 in collaborative care arm, 99 in the usual care arm). At 3 months (n= 210), the proportion of patients who achieved remission was higher, though the difference was not statistically significant, in the collaborative care group. The effect size was of 0.11. When considering only patients with minor/major depression, collaborative care appeared to be more effective than usual care (P= .015).

Conclusions

The present intervention for managing depression in primary care, designed to be applicable to the Italian context, appears to be effective and feasible.     

Age assessment based on dental calcification in individuals with Down syndrome

It is important to estimate both chronological age (CA) and maturational age of an individual, in order to perform orthopedic treatment or surgery, and in cases of lost documentation. Use of dental age (DA) for these purposes has been widely studied; however, the literature is scarce with regard to individuals with Down syndrome (DS), a prevalent condition worldwide. In this study the chronology of dental maturation was evaluated by analyzing the DA of individuals with DS based on the Chronological Mineralization Table proposed by Nolla (1960). Thus, second molars were evaluated in 57 panoramic radiographs of male and female individuals with DS, between 5 and 16 years-old. These data were compared with a control group of 191 nonsyndromic individuals of the same age group. Correlation between CA and DA was ascertained using Pearson's correlation coefficient (r), and the difference between these variables was measured using Student's t-test for paired samples and the method proposed by Bland and Altman. The difference between DA and CA was compared between the control and DS groups using Student's t-test for independent samples (α = 0.05). DA was slightly lower than the CA; however, this difference was only significant for females. The difference between DA and CA was not significant between individuals with DS and control group (both genders, p = 0.945; males, p = 0.542; females, p = 0.381). We concluded that dental maturation in individuals with DS occurs similarly to that of nonsyndromic individuals.     

Tuning structural parameters for the optimization of drug delivery performance of cyclodextrin-based nanosponges

Introduction: In light of the recent development of new soft materials, nanostructured self-assembled systems have attracted attention in a variety of technological fields of high social impact. Cyclodextrin nanosponges (CDNS) represent a new and highly versatile class of cross-linked cyclodextrin (CD)-based nanoporous polymers. Their intriguing properties, including safety, biodegradability, negligible toxicity, marked swelling behavior, superior inclusion capability with respect to native CD, are the bases for potential for applications in drug delivery, tissue engineering and regenerative medicine.

Areas covered: We report on the state-of-art concerning a detailed characterization of structural and dynamical features of CDNS explored by the combined use of different and complementary techniques, such as Fourier transform infrared absorption in attenuated total reflectance geometry (FTIR-ATR) and Raman spectroscopies, and high resolution magic angle spinning (HR-MAS) NMR spectroscopy. The ambitious objective is to furnish an exhaustive survey of the role played by hydrophobic and hydrophilic groups within the cross-linked network, in dry and swollen states, in determining the macroscopic functional features of CDNS.

Expert opinion: The reported results may significantly contribute in the rational design and optimization of new stimuli-responsive systems exhibiting tunable inclusion/release properties, adapted to the therapeutic demands of pathology.     

Prolonged distal motor latency of median nerve does not improve diagnostic accuracy for CIDP

Journal of Neurology - Compression of the median nerve at the carpal tunnel can give demyelinating features and result in distal motor latency (DML) prolongation fulfilling the EFNS/PNS...     

ALS Cognitive Behavioral Screen-Phone Version (ALS-CBS™-PhV): norms,psychometrics, and diagnostics in an Italian population sample

Neurological Sciences - Up to 50% of motor neuron disease (MND) patients show neuropsychological deficits which negatively affect prognosis and care. However, disability-related logistical issues...     

Validation of the DYALS (dysphagia in amyotrophic lateral sclerosis) questionnaire for the evaluation of dysphagia in ALS patients

Neurological Sciences - Dysphagia is a common symptom during the trajectory of ALS, and it can significantly impact on the quality of life and prognosis of patients. Nowadays, no specific tool for...     

Remote cerebellar hemorrhage after spinal procedures (part 2): a systematic review

A remote cerebellar hemorrhage (RCH) is a spontaneous bleeding in the posterior fossa, which can be rarely observed as a complication of spine surgery. As well as for RCH reported after supratentorial procedures, it shows a characteristic bleeding pattern defined “zebra sign”. Nowadays, RCH pathophysiology still remains unknown. We performed a comprehensive review, collecting all cases of RCH after spine surgery reported in literature in order to identify the procedures most frequently associated with RCH and the possible risk factors. We assessed percentages of incidence and 95 % confidence interval of all demographic, neuroradiological, and clinical features. Univariate and multivariate analyses were used to evaluate their association with outcome. We included 44 articles reporting 57 patients with mean age of 57.6?±?13.9 years and a male/female ratio of 23/34. A RCH was more frequently reported as a complication of decompressive procedures for spinal canal stenosis, particularly when associated with instrumented fusion, followed by spinal tumor debulking and disc herniation removal. In the majority of cases, RCH occurrence was characterized by progressive impairment of consciousness, whereas some patients complained non-specific symptoms. Coagulation disorders, hypertension, and placement of postoperative subfascial drainages were the most frequently reported risk factors. The occurrence of intraoperative dural lesions was described in about 93 % of patients. Zebra sign was the most common bleeding pattern (about 43 % of cases) followed by parenchymal hematoma (37.5 %) and mixed hemorrhage (about 20 %). Impairment of consciousness at clinical onset and intake of anticoagulants/antiplatelets appeared associated with poor outcome at univariate analysis. However, more than 75 % of patients showed a good outcome and a RCH often appeared as a benign and self-limiting condition, which usually did not require surgical treatment, but only prolonged clinical surveillance, unless of the occurrence of complications.