The Effect of Recombinant Growth Hormone Treatment in Children with Idiopathic Short Stature and Low Insulin-Like Growth Factor-1 Levels

Objective:

Idiopathic short stature (ISS) constitutes a heterogeneous group of short stature which is not associated with an endocrine or other identifiable cause. Some ISS patients may have varying degrees of insulin-like growth factor-1 (IGF-1) deficiency. Recombinant growth hormone (rGH) treatment has been used by some authors with variable results. Reports on long-term rGH treatment are limited.

Methods:

In this study, 21 slowly growing, non-GH-deficient ISS children who received rGH treatment for 3.62±0.92 years were evaluated at the end of a 5.42±1.67-year follow-up period. The study group included patients with low IGF-1 levels who also responded well to an IGF generation test. The patients were divided into two groups as good responders [height increment >1 standard deviation (SD)] and poor responders (height increment <1 SD) at the end of the follow-up period.

Results:

The height of the patients improved from -3.16±0.46 SD score (SDS) to -1.9±0.66 SDS. At the end of the follow-up period, mean height SDS was -1.72. Eleven of the patients showed a good response to treatment. Clinical parameters were essentially similar in the good responders and the poor responders groups. A female preponderance was noted in the good responders group.

Conclusion:

rGH treatment can safely be used in ISS children. Long-term GH treatment will ameliorate the height deficit and almost 40% of patients may reach their target height.     

The effect of growth hormone treatment on bone mineral density in prepubertal girls with Turner syndrome: a multicentre prospective clinical trial

Background Patients with Turner syndrome (TS) are treated with GH to increase adult height. Although it is well established that GH promotes longitudinal bone growth, the effects of GH treatment on bone density are less clear. Objective To determine how GH treatment affects trabecular bone mineral density (BMD) in girls with TS at prepubertal ages in a prospective multicentre study. Patients and method Twenty‐two patients with TS in the prepubertal period with a mean age of 9·8 ± 2·5 (range 3·6–12·8) years were included in the study. All girls with TS underwent measurement of areal BMD using dual‐energy X‐ray absorptiometry (DXA) to obtain pretreatment anteroposterior (AP) lumbar spine values at L1–L4. Patients received GH (Genotropin) subcutaneously for 1 year at a dose of 0·05 mg/kg/day. Height and weight were measured at 3‐monthly intervals. The AP lumbar spine areal BMD was remeasured using the same technique after 1 year of treatment. Lumbar spine BMD Z‐scores and volumetric BMD (vBMD) Z‐scores were calculated using national standards. Results The height SDS of our cases showed a significant increase with GH therapy. The pretreatment lumbar spine (L1–L4) BMD Z‐score was –1·2 ± 1·2 SD and the vBMD Z‐score was –0·8 ± 1·6 SD. There were no significant changes in these values after 1 year of GH treatment. Prepubertal TS girls more than 11 years of age had lower vBMD Z‐scores (–1·7 ± 1·7 SD) than the girls aged less than 11 (–0·1 ± 1·0 SD) (P < 0·05) at the onset of therapy. No significant changes were observed in these values after 1 year of GH therapy. Conclusions Osteopaenia becomes apparent in prepubertal TS patients as they reach pubertal age. BMD evaluation may be necessary in these prepubertal TS girls at diagnosis. Short‐term GH therapy in these TS patients does not have a significant effect on bone density when measured at a site with a predominance of trabecular bone.     

Neonatal osteofibrous dysplasia associated with pathological tibia fracture: a case report and review of the literature

Osteofibrous dysplasia is a rare and benign disease that originates from the tibia or fibula. The symptoms of osteofibrous dysplasia include painless enlargement and bowing of the tibia and pain occurring in the presence of pathological fracture. Herein a male infant who was admitted with redness and swelling on the right leg and diagnosed as pathological tibia fracture due to left tibia osteofibrous dysplasia on the third day of life was presented. To our knowledge, this is the earliest presentation of osteofibrous dysplasia with a pathological fracture in a neonate. Therefore, it must be suspected in neonatal bone fractures.     

A case of familial Mediterranean fever and polyarteritis nodosa complicated by spontaneous perirenal and subcapsular hepatic hemorrhage requiring multiple arterial embolizations

The association of familial Mediterranean fever (FMF) and polyarteritis nodosa (PAN) has been well established. These patients have been reported to have an overall better prognosis than other PAN patients. Herein we report a patient with FMF and PAN who died of sepsis following a severe course of recurrent bleeding episodes which required multiple embolization attempts. The 39-year-old Turkish male presented with abdominal pain of 1-month duration. He had been diagnosed with FMF at the age of 24. On admission, he had pallor with general ill appearance. Rebound tenderness was obtained in the right upper abdominal quadrant. He had mild anemia, leukocytosis, thrombocytosis, and hypoalbuminemia. On the 2nd day of his admission, he developed hypotension with a rapid decline in hemoglobin level. Abdominal angiography showed multiple aneurysms in the branches of renal arteries, superior mesenteric artery, and hepatic arterial system including left renal infarct, suggesting PAN. He was put on high-dose steroids and oral cyclophosphamide. Despite medical treatment, he developed intense abdominal pain, hypotension, tachycardia, and a rapid fall in hemoglobin on four occasions. Active bleeding sites were embolized in two different angiography sessions. Although the patient experienced no more recurrent bleeding, he died of multiorgan dysfunction syndrome resulting from sepsis 6 weeks after admission. Polyarteritis nodosa associated with FMF may follow a grave course despite immunosuppressive therapy. Arterial embolization should be considered in the presence of bleeding aneurysms in addition to immunosuppressive therapy.     

Effects of P-wave dispersion on atrial fibrillation in patients with acute anterior wall myocardial infarction

Backround: P‐wave dispersion (P dispersion), defined as the difference between the maximum and the minimum P‐wave duration (P minimum), and maximum P‐wave duration (P maximum) have been used to evaluate the discontinuous propagation of sinus impulse and the prolongation of atrial conduction time respectively. The aim of this study was to investigate whether early assessment of P dispersion predicts paroxysmal atrial fibrillation (AF) in patients with acute anterior wall myocardial infarction (MI). Methods: We prospectively evaluated 147 consecutive patients (45 women, 102 men; aged 55 ± 9 years) with a first acute anterior wall MI. All patients were evaluated by echocardiography to measure the left atrial diameter and left ventricular ejection fraction (LVEF). Electrocardiography was recorded from all patients on admission and every day during hospitalization. Results: AF occurred in 25 patients. In 122 patients, AF did not occur. P maximum was found to be significantly higher in patients with AF than in patients without AF (115 ± 17.3 ms vs 101 ± 14.7 ms, P = 0.001 ). P dispersion also was significantly higher in patients with AF than in patients without AF (50 ± 12.5 ms vs 43 ± 10.1 ms, P = 0.01 ). There was no significant difference between the two groups in P minimum (64 ± 12.5 ms vs 59 ± 11.7 ms, P = 0.057 ). The echocardiographically left atrial diameters were not significantly higher in the patients with AF than those without (25 ± 3.38 mm and 23 ± 3.36 mm , respectively, P = 0.76 ). LVEF was found to be significantly different in the patients who developed AF and in those who did not (37.96 ± 6.18% vs 47.70 ± 6.01%, P = 0.0001 ). Conclusions: Although P maximum and P dispersion are significant predictive factors of AF in patients with acute anterior wall MI in the univariate analysis, on the basis of multivariate analysis, only age and LVEF were independent predictive parameters for AF.     

Are Reciprocal Changes a Consequence of “Ischemia at a Distance” or Merely a Benign Electrical Phenomenon? A Pulsed‐Wave Tissue Doppler Echocardiographic Study

Objectives: The aim of the present study was to investigate whether ST segment depression in precordial leads at the time of acute inferior myocardial infarction represents a reciprocal change rather than concurrent anterior wall ischemia on the surface electrocardiography. Background: The mechanism of reciprocal ST segment depression during acute myocardial infarction is controversial. “Ischemia at a distance” or a benign electrical phenomenon has been implicated in numerous reports. Pulsed‐wave tissue Doppler (PWTD) echocardiography can be used to examine the regional diastolic motion of the left ventricular myocardial wall and may allow the detection of ischemic segments. Methods: We evaluated regional myocardial ischemia using PWTD echocardiography in 48 patients with a first inferior wall myocardial infarction. The left ventricle was divided into 16 segments. PWTD echocardiographic velocities were obtained from each left ventricular segments. Results: Reciprocal ST segment depression was present in 35 patients (Group 1) but not in the remaining 13 patients (Group 2). There were no significant differences between groups 1 and 2 with respect to systolic (S) (7.4 ± 1.1 vs 6.8 ± 0.9 cm/s; P > 0.05), early (E) (10.5 ± 2 vs 9.4 ± 1.2 cm/s; P > 0.05), and late (A) (9.5 ± 3.2 vs 8.5 ± 2.3 cm/s; P > 0.05) diastolic waves peak velocities, E/A ratio 1.1 ± 0.2 vs 1.1 ± 0.1; P > 0.05), Ewave deceleration time (DT) (92 ± 17 vs 101 ± 16 ms; P > 0.05) and regional relaxation time (RT) (82 ± 19 vs 93 ± 21 ms; P > 0.05) in anterior wall (basal levels), which correspond to reciprocal ST segment depression on electrocardiography. According to E/A ratio detected by PWTD echocardiography in anterior wall and anterior septum, patients with reciprocal ST segment depression were also divided into two groups: Group A, with E/A ratio > 1; Group B, with E/A ratio < 1. Among the 35 patients with reciprocal ST segment depression, anterior wall ischemia was present in 10 patients and absent in 25 patients, whereas anterior septal ischemia was present 12 patients and absent in 23 patients. Conclusions: Reciprocal ST segment depression during the early phases of inferior infarction is an electrical reflection of primary ST segment elevation in the area of infarction.