Preconception use of pegvisomant alone or as combination therapy for acromegaly: a case series and review of the literature.

PURPOSE: Pegvisomant (PEG) is an effective therapy for acromegaly. Its safety in women seeking fertility and during pregnancy has been scarcely reported. METHODS: A retrospective chart review was performed in three patients with acromegaly who received PEG while attempting to conceive. Published studies regarding this topic were analyzed. RESULTS: Four pregnancies in three women with acromegaly are reported. In the first patient, PEG was withdrawn three days before embryo transfer in her first pregnancy and 2 weeks prior to transfer in the second pregnancy. Each transfer resulted in a healthy full-term newborn. In the second and third patients, PEG was withdrawn at diagnosis of pregnancy. No fetal complications occurred during gestations which resulted in three full-term newborns (one single and one twin pregnancy). No abnormalities in development were found in the five live births described. Few cases of pregnancies in women exposed to PEG have been reported and therefore safety cannot be clearly established. In this series, all four pregnancies had good outcomes with discontinuation of the drug before or at first knowledge of conception. A review of the literature reveals no evident drug-related abnormalities in the offspring, even in the few women with continued use of PEG throughout pregnancy. CONCLUSION: Preconception therapy with PEG resulted in successful fertility outcomes. Although few cases have been reported, these four pregnancies with PEG use prior to or at the time of conception were not associated with significant maternal or fetal complications. More studies are needed to establish the safety of PEG preconception.

Retinal nerve fibre layer thickness is reduced in metabolic syndrome.

AIMS: To investigate retinal nerve fibre layer (RNFL) thickness in people with metabolic syndrome (MetS) and healthy controls. METHODS: A cross-sectional study was performed from March 2014 to January 2016. All participants underwent anthropometric and serological biochemical measurements, ophthalmological examination, and spectral-domain optical coherence tomography (SD-OCT). Individuals with elevated intraocular pressure, glaucoma, diabetic retinopathy and other ocular disorders were excluded. T-test, Chi square and general linear models were used to analyse the data. RESULTS: In total, 278 eyes from 139 participants were investigated [median (interquartile range) age: 37 (32-43) years]. RNFL thickness was lower in the nasal superior (107.8 ± 19.5µm) and temporal superior (135.7 ± 18.9µm) sectors in MetS group compared with the control group (114.6 ± 22.4 µm, P = 0.013 and 140.7 ± 18.2 µm, P = 0.027, respectively). After multiple adjustments for age, gender and the side of the examined [right (OD)/left (OS)] eye, MetS was independently associated with a lower RFNL thickness in the nasal superior (ß = 0.20, P = 0.009) and temporal superior (ß = 0.14, P = 0.048) sectors. RNFL thickness was significantly reduced in participants with higher numbers of metabolic abnormalities, independent of age, gender and the side of the examined eye (P = 0.043). CONCLUSION: Our findings demonstrate that MetS is independently associated with reduced RNFL thickness, suggesting that neurodegeneration is implicated in pathogenesis of MetS.

Crocus sativus L. versus Citalopram in the Treatment of Major Depressive Disorder with Anxious Distress: A Double-Blind, Controlled Clinical Trial.

Introduction: Saffron (Crocus sativus L.) has demonstrated antidepressant effects in clinical studies and extensive anxiolytic effects in experimental animal models. Methods: 66 patients with major depressive disorder accompanied by anxious distress were randomly assigned to receive either saffron (30 mg/day) or citalopram (40 mg/day) for 6 weeks. Hamilton Rating Scale for Depression (HAM-D) and Hamilton Rating Scale for Anxiety (HAM-A) were used to assess treatment effect during the trial. Results: 60 participants finished the study. Patients who received either saffron or citalopram showed significant improvement in scores of the Hamilton Rating Scale for Depression (P-value<0.001 in both groups) and Hamilton Rating Scale for Anxiety (P-value<0.001 in both groups). Comparison of score changes between the 2 trial arms showed no significant difference (P-value=0.984). Frequency of side effects was not significantly different between the 2 groups. Discussion: The present study indicates saffron as a potential efficacious and tolerable treatment for major depressive disorder with anxious distress.

Primary Antibody Deficiency in a Tertiary Referral Hospital: A 30-Year Experiment.

BACKGROUND: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. OBJECTIVES: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. MATERIALS AND METHODS: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children's Medical Center, Tehran, Iran. RESULTS: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. CONCLUSIONS: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively.

Circulating levels of fibroblast growth factor 21 in early-stage diabetic kidney disease.

AIMS/PURPOSE: Fibroblast growth factor 21 (FGF21), a hepatoadipokine with pleiotropic metabolic regulatory actions, is emerging as a novel biomarker of progressive nephropathy. We sought to evaluate circulating FGF21 and its association with clinical and biochemical characteristics as well as the urinary albumin excretion (UAE) rates in a population of patients with type 2 diabetes (T2D) with or without microalbuminuria and their matched healthy controls. METHODS: Cross-sectionally, 130 consecutive individuals comprising patients with T2D with (n = 44) or without (n = 44) microalbuminuria and their healthy controls (n = 42) were recruited for analysis. Various demographic, clinical and biochemical parameters were assessed. RESULTS: Serum FGF21 levels were significantly elevated in patients with microalbuminuria [median (interquartile range, IQR): 269.50 (188.50) pg/mL] compared to their normoalbuminuric peers with T2D [median (IQR): 103.50 (75.75) pg/mL] and nondiabetic people [median (IQR): 99.00 (126.75) pg/mL]. While serum FGF21, diastolic blood pressure and duration of diabetes mellitus (DDM) were independently associated with microalbuminuria in the baseline logistic regression model, FGF21 and DDM emerged as significant correlates in the multivariate adjusted model (OR for FGF21 = 1.060, 95% CI = 1.011-1.110, P < .016). CONCLUSIONS: Serum FGF21 level is strongly associated with early-stage diabetic kidney disease in the high-risk population of patients with T2D (particularly with circulating FGF21 values rising above 181 pg/mL). The association of serum FGF21 with subclinical stages of diabetic nephropathy may unearth perspectives on early detection and prevention of the advanced stages of chronic diabetes microvascular complications through effective FGF21-targeted therapy.

Primary antibody deficiency in a tertiary referral hospital: a 30-year experiment

Background: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. Objectives: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. Materials and Methods: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children’s Medical Center, Tehran, Iran. Results: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. Conclusions: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively (AU)

Antecedentes: Las inmunodeficiencias humorales primarias (PAD) es el grupo más frecuente de inmunodeficiencias primarias (IDP), y engloba un amplio espectro de características clínicas, que van desde los pacientes con infecciones graves y recurrentes a los casos asintomáticos. Objetivos: El presente estudio se realizó para evaluar y comparar los datos demográficos y clínicos de los tipos más comunes de PAD. Materiales y Métodos: Se revisaron retrospectivamente, las historias clínicas de todos los pacientes con PAD con un diagnóstico confirmado de: inmunodeficiencia variable común (CVID), síndrome de hiper IgM (HIgM), deficiencia selectiva de IgA (SIgAD),y de agammaglobulinemia ligada al cromosoma X (XLA), que fueron diagnosticados durante los últimos 30 años, en el Centro Médico de Niños, Teherán, Irán. Resultados: Se incluyeron en este estudio un total de 280 casos de PAD, englobando 125 pacientes con CVID, 32 HIgM, 63 SIgAD, y 60 pacientes con XLA. La mediana (rango) de edad al inicio de la enfermedad en la CVID, HIgM, SIgAD y XLA fue: 2 (0-46), 0,91 (0-9), 1 (0-26) y 1 (0-10) años, respectivamente. Las infecciones gastrointestinales fueron más frecuentes en los pacientes con CVID, mientras que las infecciones del sistema nervioso central lo fueron en la XLA. Las complicaciones autoinmunes fueron más prevalentes en los pacientes con HIgM, los tumores malignos en las CVID y las enfermedades alérgicas en las SIgAD. La tasa de mortalidad de CVID, HIgM y XLA fue 27,2%, 28,1% y 25%, respectivamente. No hubo mortalidad en el grupo de pacientes con SIgAD. Conclusiones: Los pacientes con SIgAD tuvieron el mejor pronóstico. Aunque todos los pacientes con PAD deben ser controlados estrechamente para evitar las complicaciones infecciosas, se debe prestar especial atención a la aparición de enfermedades malignas y autoinmunes en los pacientes con CVID y HIgM, respectivamente (AU)

The differential effect of the level of spinal cord stimulation on patients with advanced peripheral vascular disease in the lower limbs.

Percutaneous spinal cord stimulation (SCS) (Medtronic model 3487A PISCES-Quad lead) was carried out in 10 patients with rest pain from advanced peripheral vascular disease of the lower limb, who were unsuitable for conventional treatment. Trial stimulation ranged from 1-20 weeks and was associated with pain relief in nine of the patients. Claudication distance was improved in six patients. Trophic lesions improved in one patient with small artery disease. Spinal cord stimulation did not reverse the course of acute gangrenous lesions. The distal arterial pressure measured by Doppler Ankle/Brachial Pressure Index, (ABPI), showed no change. The capillary blood flow and skin temperature of both feet, measured, respectively, by Laser Doppler flowmetry and skin thermistor, showed a tendency to decrease when the stimulation was at the higher level, above T10, compared with an increase when the stimulation was at the lower level T12. Transcutaneous oxygen tension monitoring of the symptomatic foot showed an increase in four out of five patients. Pain relief was not dependent on circulatory changes, but it was more significant when the circulatory changes showed an impressive increase in the blood flow. The mechanism of these circulatory changes is probably by modulation of the sympathetic nervous system. Recognition of the optimal sitting of SCS may be critical in the clinical use of this technique, which seems to be a valuable option in the treatment of patients with advanced peripheral vascular disease (PVD).