A case of GH deficiency and beta-thalassemia.
A 23-year-old male patient, who suffers from beta-thalassemia major, came to us for an endocrine-metabolic evaluation. Medical history showed a diagnosis of heart disease with heart failure since the age of 16, type 1 diabetes mellitus diagnosed at the age of 18, treated with an intensive insulin therapy with a poor glycometabolic control. Patient performed regular blood transfusions and iron chelation with deferasirox. An echocardiogram revealed an enlarged left ventricle. Patient had undergone a comprehensive study of buoyancy both basal and hormone-stimulated and it was therefore carried out a diagnosis of GH deficiency and hypogonadotropic hypogonadism. A recombinant GH replacement therapy was then prescribed. After six months of therapy, the patient reported a net improvement of asthenic symptoms. Physical examination showed a reduction in abdominal adiposity in waist and an increase of 5 cm in stature. Laboratory tests showed an amelioration of glycometabolic control, such as to justify a reduction in daily insulin dose. The stature observed was thought appropriate to begin the administration of testosterone. Moreover, the cardiological framework showed a reduction of left ventricular dilatation, good ventricular motility, global minimum persistent tricuspid but not mitral regurgitation and no alteration on ECG.
Division of Special Pediatric Hematology Diagnostics, Hereditary Anemias-Rare Diseases, Department of Pediatrics and Child Neuropsychiatry, Sapienza University of Rome, Policlinico Umberto I, Rome, Italy.
M P
MP
Smacchia
V
V
Mercuri
L
L
Antonetti
G
G
Bassotti
T
T
D'Amico
D
D
Pietrobono
P
P
Gargiulo
D001247:Asthenia / Q000209:etiology*; D001803:Blood Transfusion; D002317:Cardiovascular Agents / Q000627:therapeutic use; D015913:Chelation Therapy; D003131:Combined Modality Therapy; D003922:Diabetes Mellitus, Type 1 / Q000188:drug therapy / Q000209:etiology* / Q000378:metabolism; D004392:Dwarfism / Q000188:drug therapy / Q000209:etiology; D013006:Growth Hormone / Q000627:therapeutic use; D006333:Heart Failure / Q000188:drug therapy / Q000209:etiology*; D019382:Human Growth Hormone / Q000172:deficiency*; D006801:Humans; D007006:Hypogonadism / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D007328:Insulin / Q000008:administration & dosage / Q000627:therapeutic use; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000209:etiology*; D008297:Male; D008944:Mitral Valve Insufficiency / Q000209:etiology; D013739:Testosterone / Q000627:therapeutic use; D014262:Tricuspid Valve Insufficiency / Q000209:etiology; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications* / Q000188:drug therapy / Q000628:therapy
D002317:Cardiovascular Agents; D007328:Insulin; D007502:Iron Chelating Agents; D019382:Human Growth Hormone; D013739:Testosterone; D013006:Growth Hormone