Growth hormone response following growth hormone releasing hormone injection in thalassemia major: influence of pubertal development

Thirty-five patients with thalassemia major, aged 7 to 21 years, were studied to define the relationship between the pubertal development and the growth-hormone (GH) secretion during sleep, after administration of GH-releasing factor (hpGRF 1-44), and betaxolol-glucagon or arginin-insulin. Pubertal development was classified as being appropriate or delayed for chronological age. GH response to pharmacological stimuli and during sleep was not linked to the pubertal development according to the chronological age. The peak of GH secretion after GHRH injection was significantly delayed in thalassemic patients with retarded puberty. The integrated secretion of GH during the 120-min test was slightly but not significantly reduced in these patients. The prepubertal pattern of GHRH response was restored in the patients receiving substitutive therapy by HCG or testosterone. The alteration of GH response to GHRH in thalassemic patients is likely to be only due to delayed puberty and decreased endogeneous GHRH secretion since it is corrected by androgen or gonadotropin replacement.     

Progressive myoclonic encephalopathy in X-linked hypogamma-globulinemia. Case report, review of the literature and its relationship with progressive encephalopathy in children with A.I.D.S

A 7-year-old boy suffering from X-linked hypogammaglobulinemia and progressive myoclonic encephalopathy is reported. The onset of neurological disturbances is at four years of age with ataxic gait and myoclonic jerks. The EEG shows a progressive slowing of background activity, bilateral diffuse and repetitive, pseudoperiodic, high amplitude slow waves, myoclonic jerks polygraphically documented. The CT-scan shows generalized cerebral atrophy, white matter hypodensity--principally in the frontal regions -, multiple nodular calcifications, also in the basal ganglia. Two years after the onset of neurological signs, the boy is completely bedridden, spastic, dement and blind; the myoclonic jerks persist. Finally the relationship is discussed with both the previously reported patients with the same affection, and with similar progressive encephalopathy in children suffering from A.I.D.S.     

Intracranial and intraspinal hemorrhage following spinal anesthesia

Spinal anesthesia (SA), accounting for more than 50% of regional anesthesias in the spinal region, is generally perceived as simple and safe. Our purpose is to increase awareness of hemorrhagic complications following SA. A 69-year-old male without either coagulation disorders or anticoagulant/antiplatelet therapy developed acute radiculopathy, and severe mental confusion after SA for prostatectomy. CT showed intracranial subarachnoid and intraventricular acute hemorrhage. Cerebral angiography was negative. MRI showed subarachnoid and subdural hematoma in the dorsolumbar spine. Seven-year follow-up showed permanent cognitive and radicular damage. Multiple attempts for SA most likely caused spinal vessels rupture, either directly or indirectly by inducing differential pressure changes between cerebrospinal fluid and intravascular spaces; however, definite mechanisms have not been completely understood. Patients undergoing spinal puncture must report any neurological abnormality, which may result in irreversible damage. Cases of altered consciousness require an extensive neuroradiological evaluation. Proper competency of physicians responsible for spinal puncture is mandatory.     

Über den Einfluss des Berylliums und Seiner Verbindungen auf den Organismus

Zusammenfassung In einer Reihe von Versuchen an 12 Kaninchen hat der Autor das reticuloendotheliale System durch intravenöse Injektionen von kolloidalem Be(OH)₂ sensibilisiert, ohne daß sich weder primäre noch sekundäre toxische — oder irgendwie sonst dem Organismus schädliche Erscheinungen bemerkbar gemacht hätten. Er nimmt daher an, daß diese Substanz für die Erforschung des reticuloendothelialen Systems besonders geeignet ist, vor allem, wenn man es anreizen will, die Oberflächenvergrößerung zu verursachen, durch die dem Organismus größere Abwehrkräfte gegen weitere toxische Schädigungen erwachsen.     

Evaluation of platelet turnover by flow cytometry

The number of circulating newly produced platelets depends on the thrombopoietic capacity of bone marrow as well as platelet removal from the bloodstream. Flow cytometric analysis with thiazole orange (TO), a fluorescent dye that crosses platelet membranes and binds intracellular RNA, has been used to measure circulating reticulated platelets (RPs) with high RNA content as an index of platelet turnover. We first assessed the specificity of TO flow cytometry and then applied this method in the diagnosis of thrombocytopenia caused by impaired platelet production or increased destruction. We also explored the utility of TO flow cytometry to predict thrombocytopoiesis after chemotherapy-induced bone marrow aplasia. Venous blood, anticoagulated with K₂EDTA, was incubated with 0.6?µg/ml TO plus an anti-GPIIIa monoclonal antibody. The mean percentage of RPs in control subjects (n?=?23) was 6.13?±?3.09%. RPs were 10.41?±?9.02% in patients (n?=?10) with hematological malignancies during aplasia induced by chemotherapy and a significant increase in RPs (35.45?±?6.11%) was seen in the recovery phase. In 10 patients with idiopathic thrombocytopenic purpura, the percentage of TO positive platelets was 67.81?±?18.79 (P?<?0.001 vs. controls). In patients with thrombocytopenia associated with hepatic cirrhosis (n?=?21; 21.04?±?16.21%, P?<?0.001 vs. controls) or systemic lupus erythematosus (n?=?6, 29.08?±?15.57%; P?<?0.001 vs. controls) increases in TO-stained platelets were also observed. Measurement of TO positive platelets may be a reliable tool for the laboratory identification of platelet disorders, with a higher sensitivity than measurement of platelet volume. Measurement of RPs may also prove useful to recognize the underlying pathogenetic mechanisms in thrombocytopenia.     

Two patients with tumours presenting as inguino-scrotal masses

Inguinal masses in children may indicate different conditions and pathologies, ranging from congenital anomalies to neoplasms. Although inguinal hernias represent the majority 'masses' in the inguinal canal, there are other rare lesions that may occur. For this reason, irreducible masses shall always receive an appropriate preoperative diagnosis. In this report, we describe two interesting cases of inguinal masses in children first diagnosed as irreducible inguinal hernia. Appropriate investigations before surgery and local surgical excision are therefore recommended.     

Spontaneous pneumothorax caused by lung metastasis of sarcoma of the thigh

Relapsing spontaneous pneumothorax can be the first manifestation of pulmonary metastases of soft tissues sarcomas. Standard imaging techniques and computed tomography may not be able to detect small malignant cystic lesion or to distinguish between them and benign bollous lesions. We report the case of a 33 year-old male who, in the past, underwent surgical treatment for a synovial sarcoma of the inferior limb. The patient was admitted to our hospital because of right spontaneous pneumothorax; both chest x-ray and CT scan didn't detect any metastatic pulmonary lesion. A few days after the discharge the patient was readmitted because of relapsed pneumothorax; high-definition CT of the chest revealed a pulmonary cystic lesion that was resected thoracoscopically. Histological examination revealed a pulmonary metastases of synovial sarcoma.     

Association of osteopetrosis and vitamin D-resistant rickets.

The following report concerns a case of malignant osteopetrosis associated with hypocalcemic rickets unresponsive to vitamin D. Parathyroid hormone (PTH) and Calcitonin (CT) secretions were studied in basal conditions and under calcium gluconate infusion, before and after high doses of vitamin D. Basal values (PTH: 690 pg Eq/ml; CT: 560 pg/ml) were found to be much higher than in five control subjects of the same age group, even after vitamin D therapy (PTH: 990 pg Eq/ml; CT:450 pg/ml). Like rickets, PTH and CT secretions do not seem, therefore, to be notably influenced by vitamin D therapy.     

Transient focal leukoencephalopathy following intraventricular methotrexate and cytarabine

A 14-year-old boy, suffering from acute lymphoblastic leukemia with meningeal involvement, was treated with intraventricular methotrexate and cytosine arabinoside, administered via an Ommaya reservoir (OR). Three months later, right occipital headache, vomiting, and lethargy appeared. Cerebrospinal fluid specimens showed increased proteins and a right frontal slow-wave focus was evident on the EEG recording. The computed tomography scan revealed white matter hypodensity within the right frontal and rolandic regions. After injection of medium contrast, an abscesslike hyperdensity appeared, surrounding both a well-placed cannula tip and the right frontal horn of the lateral ventricle. Brain swelling and shift signs were also evident. Nine cases of focal methotrexate leukoencephalopathy have been previously reported, and in six of these there was a misplaced OR cannula tip. The focal meihotrexate leukoencephalopathy seems to be related to the neurotoxicity of the drugs administered, and may also exist with a well-placed OR cannula tip. Immediate removal of the catheter may be associated with a benign evolution.