The aim of the study was to evaluate the craniofacial morphology in Caucasian patients with sickle cell disease (SCD) by comparing them with a healthy group paired for gender and age, by means of lateral cephalometric radiographs. Thirty-six Sicilian patients with SCD (17 females and 19 males), including 14 beta(s)beta(s) (mean age 28 +/- 5.9 years), 13 beta(s)beta(0 th) (mean age 27.5 +/- 8 years), and nine beta(s)beta(+th) (mean age 32.8 +/- 9.9 years) were examined. The control group consisted of 36 subjects (mean age 28.9 +/- 8 years) without recognized haematological abnormalities. The means and standard deviations were calculated for each cephalometric variable. A two-sample t-test was used to compare the means between the study and control groups. One-way analysis of variance and Dunnet's multiple comparison test were used in order to analyse the differences between the control group and the subgroups divided according to genotype. The level of significance used was P<0.05. The cephalometric findings indicated a posterior rotation of the mandible and a tendency towards a vertical pattern (clockwise), with lower (P=0.000) and total (P=0.002) face heights increased in comparison with the control sample. These findings were more pronounced in subjects with SCD (beta(s)beta(s)). In all patients, there was a significantly greater maxillary incisor proclination than in the control group. The upper first molar position to the PTV line was significantly increased but only in patients with compound heterozygosis beta(s)beta(th). The SCD patients did not exhibit the craniofacial abnormalities noted in black American patients with SCD; the craniofacial features observed, reflecting the degree of clinical expression of SCD in Sicilian patients, were of moderate severity.

Background Following diagnosis, children with cancer suddenly find themselves in an unknown world where unfamiliar adults make all the important decisions. Children typically experience increasing levels of anxiety with repeated invasive procedures and do not adapt to the discomfort. The aim of the present study is to explore the possibility of asking children directly about their medical support preferences during invasive procedures. Procedure Each patient was offered a choice of medical support on the day of the procedure, specifically general anesthesia (GA), conscious sedation (CS), or nothing. An ad hoc assessment tool was prepared in order to measure child discomfort before, during, and after each procedure, and caregiver adequacy was measured. Both instruments were completed at each procedure by the attending psychologist. Results We monitored 247 consecutive invasive procedures in 85 children and found that children in the 4 to 7 year age group showed significantly higher distress levels. GA was chosen 66 times (26.7%), CS was chosen 97 times (39.3%), and nothing was chosen 5 times and exclusively by adolescents. The child did not choose in 79 procedures (32%). The selection of medical support differed between age groups and distress level was reduced at succeeding procedures. Conclusions Offering children the choice of medical support during invasive procedures allows for tailored support based on individual needs and is an effective modality to return active control to young patients, limiting the emotional trauma of cancer and treatment.

Children with acute lymphoblastic leukemia (ALL), the most common childhood malignancy, usually present at diagnosis with signs of bone marrow failure. Pallor, fatigue, anemia, fever, neutropenia, and thrombocytopenia are frequent signs. Skeletal manifestations on imaging studies are found in 20-36% of cases [1,2]. These include metaphyseal bands, periosteal new bone formations, lytic lesions, osteosclerosis, osteopenia, and mixed osteolysis and sclerosis [1][2][3]. Generalized osteopenia and vertebral complications have, however, reported in ALL [4]. Our experience with such a child is of interest.She was an 8-year-old female, who was admitted to our Center because of anemia, leukopenia, and thrombocytopenia. Three months earlier, she had experienced a sharp back pain and for this reason was admitted to a local hospital. Radiography and computed tomography of her spine showed collapse of L2, and diffuse severe osteoporosis. Laboratory data were normal, except for the blood sedimentation rate (62 mm/hr). A working diagnosis of juvenile rheumatoid arthritis was entertained, and a plaster cast was applied for a month. When it was removed, the child continued to suffer from diffuse pain and had difficulty in walking. For this reason, physiotherapy was administered for 2 months but her general condition worsened; diffuse bone pain forced her to stay in bed. Because of an altered blood cell count (hemoglobin 8.5 g%; white blood cell 3,000/mm 3 ; platelets 130,000/mm 3 ), the child was transferred to our Center. She was pale, had a very restricted range of motion in her lumbar spine and pain on motion forced her to remain supine. Diffusely enlarged lymph nodes, and both hepato-and splenomegaly were present. Leukemia was suspected and a FAB L1 ALL was confirmed by bone marrow examination. Serum levels of parathormone, calcium, phosphorus, and magnesium were normal, while the calcitonin was 54.4 ng/ml (normal range: 3.1-13.7 ng/ml). The 24-hr calciuria and phosphaturia values were 92 mg/24 hr (normal range: 100-300 mg/24 hr) and 234.6 mg/24 hr (normal range: 400-1,300 mg/24 hr), respectively. Renal and liver function findings were normal. Total-body X-ray films showed lumbar kyphosis and marked rarefaction of trabeculae and spongiosa of the vertebral bodies. The bodies of T4, T7, T9, T10, and L1-4 were collapsed indicating previous fractures (see Fig. 1). A corset was applied and physiotherapy was ruled out. Chemotherapy was started; during the induction phase, prednisone was preferred to dexamethasone, which is known to be more toxic to bone metabolism. Supplementation with oral calcium and vitamin D was ruled out. A prompt clinical and laboratory response followed. DISCUSSIONOsteoporosis in children at the onset of ALL probably has a multifactorial basis. Direct ALL involvement or an indirect effect through osteoclast activating factor, produced by lymphoblasts have been proposed [5]. Low concentrations of activated vitamin D3 and osteocalsin have been considered as possible causes of these alterations in bone metaboli...

Natural killer (NK) cell lymphomas are rare in the USA and Europe but more common in Asia and Central America although very rare among children. We report a case of Epstein-Barr virus-positive NK lymphoma/leukemia, that showed peculiar features represented by a very long clinical course with a significant interval between the first clinical signs and the diagnosis, detection of neoplastic cells in the peripheral blood but not in the bone marrow, and good response to treatment and clinical outcome.

Background Spinal cord compression (SCC) is an uncommon, severe complication of Hodgkin lymphoma (HL), occurring in 0.2% of cases at the onset and in 6% during disease progression. We present a teenager with SCC with clinical onset of HL; her pre-existing neurological abnormalities covered the presence of an epidural mass, which could have misled us. Case presentation A 13-year-old girl presented with a three-month history of lower back pain and degrading ability to walk. She suffered from a chronic gait disorder due to her preterm birth. A magnetic resonance imaging of the spine revealed an epidural mass causing collapse of twelfth thoracic vertebra and thus compression and displacement of the spinal cord. Histological examination with immunohistochemical analysis of the epidural mass demonstrated a classic-type Hodgkin lymphoma. Early pathology-specific treatment allowed to avoid urgent surgery, achieve survival and restore of neurological function. Conclusions Children and adolescents with back pain and neurological abnormalities should be prioritized to avoid diagnostic delay resulting in potential loss of neurological function. SCC requires a prompt radiological assessment and an expert multidisciplinary management.