Association of empty sella and neuroendocrine disorders in childhood

Empty sella syndrome (ESS) is a multicausal entity. The incidence of primary empty sella syndrome (PESS) in children with neuroendocrine dysfunction is not known. In the pediatric age group, frequency seems to have been underestimated. A total of 117 cases of neuroendocrine disorders, including complete growth hormone deficiency, primary hypothyroidism with pituitary resistance to thyroid hormone, obesity, central precocious puberty, hypothalamic hypogonadism and central diabetes insipidus, have been studied with computed tomography and/or magnetic resonance imaging of sellar region for etiologic evaluation. Twenty-one patients were found to have PESS. We noted a high incidence of PESS in children with neuroendocrine dysfunction (17.9%). Children with neuroendocrine dysfunction should be investigated with respect to PESS, and children with PESS recognized coincidentally should be studied with the particular consideration of subclinical neuroendocrine dysfunction.     

Changes with growth hormone treatment in growth hormone deficient children

A total of 54 previously untreated patients (15 girls, 39 boys) with poor growth due to idiopathic growth hormone deficiency (IGHD) were treated with human growth hormone (hGH), continuously up to 4 years. All of the patients had a peak hGH level which was below 10 ng/mL after at least two pharmacological tests and/or blunted physiologic hGH secretion, and their height was below ?2.5 s.d. for age and gender. After the 1st year of therapy, height velocity (HV) increased significantly when compared with baseline (from 3.18 ±0.76 cm/year to 9.17±1.03 cm/year; P <0.001), declined during the 2nd year and then remained significantly higher than pretreatment HV. When considering improvement in height expressed by height standard deviation score (SDS), during the therapy all of the patients showed a significant gain ± 1.72±1.09 (from ?4.11±0.61 to ?2.21±0.48). The height values were significantly higher than pretreatment, but remained below ?2 s.d. after 4 years of hGH therapy in our patients. Increased height velocity has been sustained, but height improvement after therapy was inversely correlated to height SDS for chronological age of patients at the start of therapy. In conclusion post-treatment height has been shown to be related to height deficit at the beginning of therapy. Therapy was well tolerated with no local or systemic adverse effects or acceleration of bone age.     

Solid-phase synthesis of (tyrosyl-alanyl-glutamyl)n,by segment condensation

(Tyr-Ala-Glu)_n, n= 1–9, were synthesized by segment condensation using the Fmoc/tert-butyl protection strategy and solid-phase techniques. The C-terminal residue was coupled to the resin and the peptides were built out by adding Fmoc-Glu(O-r-Bu)-Tyr(t-Bu)-Ala-OH units. When the desired lengths were reached the peptides were capped with Fmoc-Tyr(t-Bu)-Ala-OH units. Fmoc-Tyr(t-Bu)-Ala-OH and Fmoc-Glu(O-t-Bu)-Tyr(t-Bu)-Ala-OH were synthesized in aqueous solution by the successive addition of N-hydroxysuccinimide esters of Fmoc-Tyr(t-Bu) and Fnioc-Glu(0-t-Bu) to the growing chain. Neither sequential amino acid addition or segment condensation techniques were successful on polystyrene supports. However, the segment condensations were highly successful on kieselguhr-supported polydimethylacrylamide based resins. (Tyr-Ala-Glu)_n, n= 1–9, were tested as inhibitors of the protein [yosine kinase, pp60C^c-src. Inhibition, as measured by IC₅₀ values, increased with increasing size of the peptide.     

QT Duration and Dispersion in Patients with Anomalous Origins of Coronary Arteries

Background: Coronary artery anomalies have been reported to show various symptoms ranging from chest pain and dyspnea to cardio-respiratory arrest and sudden death. In this study, we attempted to assess the changes in QT interval duration and dispersion in anomalous origins of coronary arteries (AOCA).
Methods: Nineteen AOCA patients (mean age: 52 ± 11 years) and 30 healthy control subjects (mean age: 50 ± 12 years) were included in the study. Minimum and maximum corrected QT intervals, and corrected QT dispersion were calculated. The two groups were compared in terms of QT dispersion and QT duration.
Results: There was no difference between the two groups in terms of baseline demographic characteristics. Maximum corrected QT intervals (QTc max), minimum corrected QT intervals (QTc min), and corrected QT dispersion were higher in AOCA patients than controls (452 ± 38 vs 411 ± 25 ms [P = 0.0001], 402 ± 31 vs 383 ± 28 ms [P = 0.048], and 51 ± 30 vs 28 ± 12 ms [P = 0.001], respectively).
Conclusion: In the patients with anomalous origins of coronary arteries, QT dispersion that is an indicator of sudden cardiac death and arrhythmias frequency increased. QTc max, QTc min, and corrected QT dispersion are higher in patients with anomalous origin of the coronary artery than in control subjects.     

Anterior Mitral Valve Length is Associated with Ventricular Tachycardia in Patients with Classical Mitral Valve Prolapse

Background: The aim of this study was to investigate the electrocardiographic and echocardiographic predictors of ventricular tachycardia (VT) in patients with classical mitral valve prolapse (MVP). Methods: Thirty patients (nine men and 21 women; mean age, 41.5 ± 15 years) in sinus rhythm with mitral valve prolapse who had VT in 24‐hour Holter analysis and 30 patients with MVP without VT (eight men and 22 women; mean age, 43 ± 16 years) were included in this study. Transthoracic echocardiography, QT analyses from 12‐lead electrocardiography, and 24‐hour Holter electrocardiogram recordings were performed. Results: Mitral posterior leaflet thickness (0.48 ± 0.03 cm vs 0.43 ± 0,08 cm, P = 0.025), mitral anterior leaflet length (3.2 ± 0.24 cm vs 2.9 ± 0.36, P < 0.001), mitral posterior leaflet length (2.2 ± 0.3 cm vs 1.9 ± 0.35 cm, P = 0.01), left atrium anteroposterior diameter (4.2 ± 0.8 cm vs 3.5 ± 0.5 cm, P = 0.001), and mitral annulus circumference (15.7 ± 1.3 cm vs 14.6 ± 1.6 cm, P = 0.004) were increased significantly in MVP cases with VT. No significant difference was found between the cases with and without VT in terms of frequency‐ and time‐domain analysis. QT dispersion (72 ± 18 ms vs 55 ± 15 ms, P = 0.0002) and corrected QT dispersion (QTcD) (76 ± 18 ms vs 55 ± 15 ms, P = 0.0002) were significantly increased in cases with VT compared with those without VT. Based on logistic regression analysis for MVP cases, in the case of VT, an enhancement in QTcD (P = 0.01) and the mitral anterior leaflet length (P = 0.003) were the independent predictors of VT. Conclusion: Mitral anterior leaflet length and enhanced QTcD are closely related with VT in patients with classical MVP. (PACE 2010; 33:1224–1230)