Skeletal health in patients with differentiated thyroid carcinoma.

Osteoporosis and fractures are important comorbidities in patients with differentiated thyroid cancer (DTC), with potential negative impact on quality of life and survival. The main determinant of skeletal fragility in DTC is the thyrotropin (TSH)-suppressive therapy, which is commonly recommended to prevent disease's recurrence, especially in patients with structural incomplete response after thyroid surgery and radio-iodine therapy. TSH-suppressive therapy can stimulate bone resorption with consequent bone loss, deterioration of bone microstructure and high risk of fragility fractures. The skeletal effects of TSH-suppressive therapy may be amplified when thyroid cancer cells localize to the skeleton inducing alterations in bone remodelling, impairment of bone structure and further increase in risk of fractures. The management of skeletal fragility in DTC may be challenging, since prediction of fractures is a matter of uncertainty and data on effectiveness and safety of bone-active agents in this clinical setting are still scanty. This review deals with pathophysiological, clinical and therapeutic aspects of skeletal fragility of patients with DTC.

Secondary hyperparathyroidism and thoracic vertebral fractures in heart failure middle-aged patients: a 3-year prospective study.

PURPOSE: Vertebral fractures (VFs) were described in elderly patients with heart failure (HF) whereas their prevalence and determinants in younger HF patients are still unknown. This study aimed at assessing whether secondary hyperparathyroidism (SHPT) may influence the risk of VFs in middle-aged patients with HF. METHODS: 84 patients (44 males, median age 48.5 years, range 43-65) with HF were prospectively evaluated at the baseline and after 36-month follow-up for bone mineral density (BMD) and VFs by quantitative morphometry on chest X-rays. Serum PTH, calcium, 25-hydroxyvitamin D and 24-h-urinary calcium were evaluated at the baseline and every 6-12 months during the study period. RESULTS: At baseline, SHPT, hypovitaminosis D and VFs were found in 43 patients (51.2%), 73 patients (86.9%) and 29 patients (34.5%), respectively. SHPT was associated with VFs at baseline [inverse probability-weighted (ipw) odds ratio (OR) 12.2, p < 0.001]. Patients were treated with vitamin D3 alone (56%), vitamin D3 plus calcium carbonate (21.4%), calcitriol alone (4.8%), bisphosphonates plus vitamin D3 (8.3%) or a combination of bisphosphonates, vitamin D3 and calcium carbonate (9.5%). At the end of follow-up, hypovitaminosis D was corrected in all patients, whereas 19/84 patients (22.6%) had persistent SHPT. During the follow-up, 16 patients developed incident VFs which resulted to be associated with baseline SHPT (ipw OR 55.7, p < 0.001), even after adjusting from BMD change from baseline to follow-up (ipw OR 46.4, p < 0.001). CONCLUSIONS: This study provides a first evidence that SHPT may be a risk factor for VFs in middle-aged patients with HF.

Fungal infections of the central nervous system and paranasal sinuses in onco-haematologic patients. Epidemiological study reporting the diagnostic-therapeutic approach and outcome in 89 cases.

Invasive fungal infections (IFI) of the Central Nervous System (IFI-CNS) and Paranasal Sinuses (IFI-PS) are rare, life-threatening infections in haematologic patients, and their management remains a challenge despite the availability of new diagnostic techniques and novel antifungal agents. In addition, analyses of large cohorts of patients focusing on these rare IFI are still lacking. Between January 2010 and December 2016, 89 consecutive cases of Proven (53) or Probable (36) IFI-CNS (71/89) and IFI-PS (18/89) were collected in 34 haematological centres. The median age was 40 years (range 5-79); acute leukaemia was the most common underlying disease (69%) and 29% of cases received a previous allogeneic stem cell transplant. Aspergillus spp. were the most common pathogens (69%), followed by mucormycetes (22%), Cryptococcus spp. (4%) and Fusarium spp. (2%). The lung was the primary focus of fungal infection (48% of cases). The nervous system biopsy was performed in 10% of IFI-CNS, and a sinus biopsy was performed in 56% of IFI-PS (P = 0.03). The Galactomannan test on cerebrospinal fluid has been performed in 42% of IFI-CNS (30/71), and it was positive in 67%. Eighty-four pts received a first-line antifungal therapy with Amphotericine B in 58% of cases, Voriconazole in 31% and both in 11%. Moreover, 58% of patients received 2 or more lines of therapy and 38% were treated with a combination of 2 or more antifungal drugs. The median duration of antifungal therapy was 60 days (range 5-835). A surgical intervention was performed in 26% of cases but only 10% of IFI-CNS underwent neurosurgical intervention. The overall response rate to antifungal therapy (complete or partial response) was 57%, and 1-year overall survival was 32% without significant differences between IFI-CNS and IFI-PS. The overall mortality was 69% but the IFI attributable mortality was 33%. Mortality of IFI-CNS/PS remains high but, compared to previous historical data, it seems to be reduced probably due to the availability of newer antifungal drugs. The results arising from this large contemporary cohort of cases may allow a more effective diagnostic and therapeutic management of these very rare IFI complications in haematologic patients.

Endocrine and metabolic complications in children and adolescents with Sickle Cell Disease: an Italian cohort study.

BACKGROUND: Children with Sickle Cell Disease (SCD) show endocrine complications and metabolic alterations. The physiopathology of these conditions is not completely understood: iron overload due to chronic transfusions, ischemic damage, and inflammatory state related to vaso-occlusive crises may be involved. Aims of this study were to evaluate the growth pattern, endocrine complications, and metabolic alterations and to detect the relationship between these conditions and the SCD severity in affected children and adolescents. METHODS: Fifty-two children and adolescents with SCD [38 homozygous sickle hemoglobin (HbSS) and 14 heterozygous sickle hemoglobin (HbSC); age range 3-18 years] were recruited. Anthropometric [height, body mass index (BMI), arm span, sitting height, target height (TH), and pubertal status] and laboratory [blood cell counts, hemolysis indices, metabolic and nutritional status indices and hormonal blood levels] data were evaluated. The SCD severity was defined according to hematological and clinical parameters. RESULTS: Height-SDS adjusted for TH and BMI-SDS were significantly higher in HbSC children than in HbSS ones. Forty-eight out of 52 patients (92%) had at least one metabolic and/or endocrine alteration: insufficiency/deficiency of vitamin D (84.7%), insulin resistance (11.5%), growth hormone deficiency (3.8%), subclinical hypothyroidism (3.8%), and hypogonadism (1.9%). Levels of vitamin D were significantly and negatively correlated with clinical indicators of the SCD severity. Subjects with HbSS genotype show significant lower levels of both insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein 3 than children with HbSC. In the study population IGF-1 values were significantly and positively correlated with Hb and negatively with lactate dehydrogenase. CONCLUSIONS: Metabolic alterations and endocrine complications are very common in children and adolescents with SCD. A regular follow-up is necessary to identify subjects at risk for complications to precociously start an appropriate treatment and to improve the quality of life of SCD patients.

Congenital neuroblastoma with symptoms of epidural compression at birth.

The occurrence of congenital neuroblastoma presenting at birth with symptoms of epidural compression secondary to spinal canal invasion is rare. Almost all cases reported in the literature have survived from the tumor but suffer severe sequelae, with the exception of the 2 most recently described whose birth was anticipated. The 3 cases of this article have been followed for a minimum of 5 years with the aim to describe their definitive late complications. In none of these cases had the routine ultrasound scan performed in third trimester of pregnancy discovered a tumor mass, nor had it shown abnormal fetal movements. All had leg hypotonia detected on the first day of life. In all, both primary and intraspinal tumors responded well to chemotherapy. All survive with motor deficit and severe bladder dysfunction despite early physiotherapy. Scoliosis has developed in the case with the longest follow-up. The description of these patients enforces the importance of early diagnosis of tumor masses in late pregnancy. Neonatologists should be aware of this rare clinical entity and take it into account in the differential diagnosis with other conditions of early-onset hypotonia. On the other hand, obstetric sonologists should be aware of the possibility to detect such rare tumors in late pregnancy, as anticipation of delivery may reduce the risk of late sequelae.

Management of community-acquired pneumonia by trained family general practitioners.

SETTING: Community-acquired pneumonia (CAP) is a respiratory health disease with a high prevalence in the general population. Family general practitioners (GPs) can play an important role in CAP management by reducing unnecessary hospital admissions and, consequently, national health costs. OBJECTIVE: To assess CAP management by trained GPs. DESIGN: A course in CAP management, including a risk classification method based only on clinical criteria, was developed within the framework of an educational programme. GPs who participated in the programme (n = 220) were asked to collect data on their CAP patients. RESULTS: GPs (n = 94, response rate 42.7%) provided information on 370 patients (50% males, aged 18-93 years). The numbers of patients judged to be at low, moderate and high risk were 81%, 13% and 6%, respectively. The admission rate was 19.5%. All home-treated patients had good clinical outcomes. Home treatment was based on quinolones (62%), beta-lactams (23%) and macrolides (15%). The attributable economic mean cost of antibiotic home treatment was euro 96 per episode (standard deviation 71, range 17-445). CONCLUSIONS: The good outcomes suggest that GPs managed their CAP patients well, adhering to the content of the CAP management course. The risk evaluation of patients admitted to hospital, based exclusively on clinical elements, was consistent with more complex classification.

Early detection of biochemically occult autonomous thyroid nodules.

OBJECTIVE: Autonomously functioning thyroid areas may be associated with subclinical or overt hyperthyroidism, but may exist even in the presence of normal TSH. This study was aimed at comparing the rate of autonomously functioning areas and their cardiac sequelae in patients with nodular goitre studied with the usual and a novel approach. DESIGN AND METHODS: In total 490 adult outpatients with thyroid nodular goitre, living in a mild iodine-deficient area, were selected in our referral centre for thyroid diseases from 2009 to 2014 on the basis of a suspicion of thyroid functional autonomy. They were divided in three groups according to a non-conventional approach (excessive response to thyroxine treatment: group 1) or conventional approach (low/normal TSH with clinical suspicion or low TSH: groups 2 and 3). All patients of the study with the suspicion of thyroid functional autonomy underwent thyroid scan with radioactive iodine (I131) uptake (RAIU). RESULTS: The percentage of confirmed thyroid functional autonomy was 319/490, being significantly higher in group 3 than in groups 1 and 2 (81.5 vs 64.7 vs 52.6%; chi-square P < 0.0001). However, the diagnosis with non-conventional approach was made at a significant earlier age (P < 0.0001). Cardiac arrhythmias as well as atrial fibrillation were similarly detected by conventional and non-conventional approaches (chi-square test: P = 0.2537; P = 0.8425). CONCLUSIONS: The hyper-responsiveness to thyroxine treatment should induce the suspicion of thyroid functional autonomy at an early stage, allowing to detect autonomous functioning areas in apparently euthyroid patients.

Individually-tailored thyroxine requirement in the same patients before and after thyroidectomy: a longitudinal study.

OBJECTIVE: Thyroxine (T4) requirement after total thyroidectomy for differentiated thyroid carcinoma (DTC) is a debated issue. As most of the studies in the area have been retrospective and/or performed with heterogeneous therapeutic approaches, we designed our study to determine T4 requirement in the same patients and treatment settings, before and after total thyroidectomy. DESIGN, PATIENTS AND METHODS: This was a longitudinal study including 23 goitrous patients treated with T4 in an individually tailored fashion. All patients exhibited a stable TSH (median TSH = 0.28 mU/l) at a stable T4 dose for at least 1 year before surgery (median T4 dose = 1.50 µg/kg per day). The patients underwent total thyroidectomy based on cancer suspicion or compressive symptoms. Eventually diagnosed as having DTC (pT1b-pT2N0) and following surgical and radiometabolic treatment, they were treated with the same pre-surgical doses of T4. RESULTS: Three months after surgery,using the same pre-surgical dose, median TSH increased up to 5.38 mU/l (P<0.0001) and so the T4 dose had to be increased (median T4 dose = 1.95 µg/kg per day; +30%; P < 0.0001). Once divided by patients' age, we observed that, after thyroidectomy and maintaining the same pre-surgical dose, serum TSH significantly increased both in younger and in older patients (median TSH = 4.57 and 6.11 mU/l respectively). Serum TSH was restored to the pre-surgical level by increasing the dose up to 1.95 and 1.77 µg/kg per day (+25 and +21%) respectively. CONCLUSIONS: Following the same treatment regimen, a thyroidectomized patient requires one-third higher therapeutic T4 dose than before surgery. Despite this increase, the dose of T4 needed in our patients remains significantly lower than that previously described in athyreotic patients.

Quantitation of HIV-1 p24 antibody expressed as relative binding capacity p24 antigen (p24 RBC) in infants born to HIV-1 seropositive mothers: correlation of this serological marker with the HIV-1 maternal antibody course and p24 antigenemia detection.

The antibody content to HIV-1 p24 Ag expressed as relative binding capacity to the target antigen (p24 RBC) was retrospectively quantified in serum samples from 20 HIV-1-uninfected infants born to HIV-1 seropositive mothers. p24 RBC values quantified at birth were included either in a low (0-20%) or high (80-100%) range of values, classified as group A (11 infants) and group B (9 infants), respectively. The course of maternal antibodies to HIV-1 antigens p17, p24, p31, gp41, p51, p66, gp120, and gp160 was studied in each group. A substantial difference in the amount and subsequently in the decline of maternal antibodies to gag proteins p17, p24, and p55 and to pol proteins p51 and p66 was observed in the two infant groups in contrast with a similar content and decline of the remaining antibodies. In 7 HIV-1-infected infants of whom 4 resembled infant group A and 3 infant group B for p24 RBC values, a relationship appeared between p24 antibody decline and p24 antigenemia detection.

Intrinsic secretory characteristics of luteinizing hormone and prolactin episodic release during pubertal development.

The intrinsic characteristics of LH and prolactin (PRL) episodic secretion were evaluated in a group of 18 children (8M and 10F). The children were divided into two groups according to the Tanner stage: Group A (Tanner < or = 1, N = 7, 3M and 4F, 6-10 years of age) and group B (Tanner 2-3, N = 11, 5M and 6F, 9-11 years of age). A pulsatility study of 4 h, sampling every 10 min, was carried out in all children. LH and PRL plasma levels were assayed by IFMA and RIA respectively. LH and PRL secretory episodes were then identified on plasma determinations using the program DETECT. Instantaneous secretory rates (ISR) were then computed for both LH and PRL using the specific algorithm within the DETECT program. Plasma LH levels were different between the two groups of children. Group A children showed undetectable LH plasma levels (below the minimal detectable dose of 0.1 mIU/ml), while group B demonstrated LH plasma levels in the normal range of values for age and sexual development (1.5 +/- 0.3 mIU/ml, mean +/- SEM). LH pulse frequency for group B was 3.2 +/- 0.4 peaks/4 h. No significant differences in mean plasma PRL levels, pulse frequency and pulse amplitude were observed between the two groups of children. Computation of ISR for LH (group B only) and PRL (both groups) identified the intrinsic episodic characteristics of the two hormones. No significant differences in LH and PRL pulse frequencies were observed when comparing the results estimated on ISR with those estimated on plasma concentrations.(ABSTRACT TRUNCATED AT 250 WORDS)

Adhesion mechanisms of human lens epithelial cells on 4 intraocular lens materials.

PURPOSE: To evaluate lens epithelial cell (LEC) adhesion on different intraocular lens (IOL) materials with particular attention to the distribution of proteins located in the focal contacts. SETTING: Center of Biotechnological and Clinical Research in Ophthalmology, University of Bologna, Italy. METHODS: The IOL materials tested were poly(methyl methacrylate) (PMMA), heparin-surface-modified PMMA (HSM PMMA), polyHEMA, and silicone. Primary cultures of human LECs were established from human anterior capsules obtained during cataract surgery. The mean number of cells attached per square millimeter was calculated for each material after 24 and 72 hours. Transmission electron microscopy and immunocytochemical analysis were performed to detect the proteins actin, vinculin, and talin. RESULTS: Mean adhesiveness of human LECs increased over time with PMMA and decreased with the other materials. At 72 hours, mean LECs ranged from 54.8 cells/mm2 +/- 12.8 (SD) on PMMA to 2.1 +/- 0.7 cells/mm2 on silicone. The means for HSM PMMA and polyHEMA fell in between. The cytoskeletal proteins were arranged to produce focal contacts in only the LECs cultured on PMMA. The LECs cultured on polyHEMA, HSM PMMA, and silicone attached but failed to develop focal contacts or stress fibers. CONCLUSION: This study confirms the multifactorial pathogenesis of posterior capsule opacification and suggests its incidence will be reduced by improving surgical techniques and using IOL surfaces that discourage cell adhesion.

Primary hypergammaglobulinemic purpura associated with IgG2 deficiency. A case report.

Hypergammaglobulinemic purpura is a rare disease in children. We report a case of a 12 year-old girl with a history of frequent infections. We found the presence of IgG2 deficiency despite polyclonal hypergammaglobulinemia. An IgG subclass determination should be obtained in every child with polyclonal hypergammaglobulinemia and features of immunodeficiency.

Ultrastructural and immunohistochemical study on the effect of topical cyclosporin A in the rabbit eye.

The effect of topically administered cyclosporin A (CyA) was evaluated in this experimental study by means of transmission and scanning electron microscopy, and immunohistochemistry. CyA dissolved in sterile olive oil was injected in the retrobulbar area and in the subconjunctival space, or administered as eyedrops in both non-grafted and corneal xenografted rabbit eyes. The vehicle rather than the drug was thought to be responsible for the inflammatory reaction observed in the conjunctiva of the injected eyes as well as for the surface epithelial defects developing in the cornea of the topically administered eyes. CyA was found to be effective in prolonging the survival of the xenografts after 1 month follow-up. The immunohistochemistry indirectly demonstrated the CyA effectiveness in impairing the T-cell function, and this is, to our knowledge, the first documented report on this subject. We conclude that another type of vehicle for the drug has to be introduced and that the administration of CyA as eyedrops is the best route to provide fast diffusion and to avoid adverse side effects.

Reduced spinal bone density in young women with amenorrhoea.

To assess the influence of diminished oestrogen production on bone density, we studied 23 amenorrhoeic women and 20 controls (age range 16-29 years) divided into four groups: group 1: 6 patients with idopathic hypogonadotrophic hypogonadism with primary amenorrhoea (IHH); group 2: 5 patients with delayed puberty owing to thalassaemia major (TM); group 3: 12 patients with secondary hypothalamic amenorrhoea (HA); group 4: 20 women with normal menses (controls). Secondary sexual characteristics had developed in all except the women with TM. Groups 1 and 2 had never menstruated and group 3 had been amenorrhoeic for 6 months to 3 years. The control group was studied during the follicular phase of the cycle. None of the patients were taking oestrogens at the time of observation. Plasma concentrations were determined for 17 beta-oestradiol (E2), deidroepiandrosterone sulphate (DHEA-S), cortisol (F), prolactin (PRL), thyroid hormones (T3 and T4), and gonadotrophins (LH and FSH). Spinal bone mineral density (BMD g/cm2) was assessed by dual photon absorbiometry. BMD (mean +/- 1SD) was reduced in the patients (group 2: 0.920 +/- 0.95; group 1: 0.980 +/- 0.94; and group 3: 1.037 +/- 0.75) as compared with the controls (1.290 +/- 0.95) (P less than 0.01). In the three groups of patients, plasma E2 levels were lower than 50 pg/ml and were positively correlated with the BMD. As expected, plasma gonadotrophin levels were highly and significantly reduced (P less than 0.01) in the patients, compared with that of the controls. These results suggest that reduced spinal BMD in hypogonadic women may be related to the lack of oestrogenic influence on bone metabolism.(ABSTRACT TRUNCATED AT 250 WORDS)

Interferon alpha in the therapy of conjunctival lymphoma in an HIV+ patient.

The authors evaluated the efficacy of interferon-alpha used intralesionally in the therapy of low-grade conjunctival non-Hodgkin lymphoma in an HIV-positive patient. The patient received one intralesional injection of 1,000,000 U of interferon-alpha three times a week for a total of twelve doses. The remission of conjunctival involvement was seen three months from the end of therapy. The intralesional use of interferon-alpha can be considered an efficacious therapy for low-grade conjunctival non-Hodgkin lymphoma.

Frequent replacement and conventional daily wear soft contact lens symptomatic patients: tear film and ocular surface changes.

We analyzed tear film changes, ocular surface tissue alterations and inflammatory conditions in Frequent Replacement Soft (FRS) Contact Lens (CL) or Conventional Soft (CS)-CL wearers suffering from eye discomfort, by applying complementary diagnostic tests. Our data suggest the following conclusions: i) eye discomfort symptoms begin after 1-3 years of successful wear, mainly in FRS-CL wearers; ii) tear film stability is affected by CL wear, mainly in FRS-CL wearers; iii) CL wear produces a subclinical moderate inflammation based on mononuclear cell recruitment in 3 out of 4 patients of both types of lenses; iv) CL wear induces dry eye conditions, more pronounced in FRS-CL users; v) discomfort symptoms, presence of subclinical inflammation, degree of dryness are not directly related to a clinical presentation of intolerance, mainly in FRS-CL wearers; vi) CL wear-related discomfort symptoms should never be underestimated, since a hidden inflammation or dryness can be present beyond an apparently good clinical picture.

Quality of generated diagnosis related groups in Italian Respiratory Intermediate Care Units.

BACKGROUND: To date we lack official data on tipology of Diagnosis Related Groups (DRGs) and their quality in Italian Respiratory Intermediate Care Units (RICUs). AIM: The objective of the study was to collect data on the activity of 26 Italian RICUs and to evaluate the quality of the DRGs generated. METHODS: The primary and secondary diseases, the procedures carried out and their coding using the ICD9 system (valid Italy until 2000) were collected from the discharge forms of patients admitted to RICUs. To obtain the DRG, these codes were automatically recoded in the ICD9-CM classification system by Grouper 10. Afterwards, the same diseases and procedures were directly processed by the ICD9-CM classification system. Finally, in order to evaluate the quality of care, the DRGs generated by the ICD9 classification system were compared to DRGs generated by the ICD9-CM classification system. RESULTS: The average weight of the patients cared for in an Italian RICU was 2.05 using the ICD9 classification system and 2.53 using the ICD9-CM classification system. Some non-complicated DRGs (80-97) or non specific DRGs (101-102) were set to zero; others, like DRG 87 appear due to the ability of the ICD9-CM classification system to recognise and accept the fifth digit of the Respiratory Failure code (518.81). The difference in terms of DRG scores generated by the two codification systems was 360.5 DRG points in favour of ICD9-CM. More than 1 million Euro of reimbursements have been lost, as the average national reimbursement for each DRG score is Euro 2,943.80. CONCLUSION: Severe pulmonary diseases determined the case mix of patients cared for in the Italian RICUs during the observed period. The Italian RICUs offer high quality assistance and are characterised by high mean weight per treated patient. However, the activity has been under-estimated due to the low sensitivity of the ICD9 classification system used in the recognition of the real disease and in the correct generation of relative DRG. The ICD9 classification system penalised the recognition of respiratory failure in particular.

[Color Doppler ultrasonography in retinitis pigmentosa. Preliminary study]. / Echo-Doppler couleur dans la rétinopathie pigmentaire. Etude préliminaire.

PURPOSE: Retinitis pigmentosa is a bilateral retinal degeneration. The primary disorder is still debated. METHODS: We performed a prospective investigation of the ocular circulation directly by color Doppler imaging (CDI). A total of 28 eyes of 14 patients (8 men and 6 women, affected with retinitis pigmentosa) were recruited for this study. For each case were evaluated protosystolic velocity and the resistive index of the ophthalmic artery, central retinal artery, posterior ciliary arteries and choroid. These values, furthermore, have been compared with a control group. RESULTS: The results of the CDI in the group of RP and in the CG were: in the OA: PSV 31.177 +/- 5.119 cm/sec vs 36.700 +/- 3.152 cm/sec (p < 0.007); RI 0.713 +/- 0.058 vs 0.717 +/- 0.019 (p < 0.0839); in the CRA PSV 7.075 +/- 1.611 cm/sec vs 12.710 +/- 2.795 cm/sec (p < 0.001); RI 0.560 +/- 0.062 vs 0.550 +/- 0.051 (p < 0.234); in the PCA: PSV 8.569 +/- 3.408 cm/sec vs 14.100 +/- 2.571 cm/sec (p < 0.001) with RI 0.634 +/- 0.090 vs 0.681 +/- 0.045 (p < 0.145). In the CHO: PSV 12.312 +/- 2.327 cm/sec vs 16.170 +/- 1.846 cm/sec (p < 0.001) with RI 0.581 +/- 0.072 vs 0.638 +/- 0.050 (p < 0.065). CONCLUSION: Our results suggest that in the affected eyes there is a statistically significant reduction in blood flow in ophthalmic and ciliary arteries. These data offer new views on the retinitis cause of pigmentosa and possible therapeutics to be studied.

[Use of high-dose intravenous immunoglobulins in pediatric hematology]. / L'uso di immunoglobuline endovena ad alte dosi in ematologia pediatrica.

High-dose intravenous immunoglobulin play a critical role in a lot of pediatric hematologic diseases. In our experience we studied the effects of IVIG treatment in 63 children. They all tolerated IVIG preparations in every infusions; no trouble caused the interruption of treatment. The efficacy is evident in immunomediated diseases, most of all ITP, and controlling septic episodes in immunocompromised patients (ALL, AIDS, marrow bone transplantation). An important problem is the cost of preparation; but we may consider that this treatment, for example in ITP, reduces hospitalization for children, necessity of platelets' transfusions, use of steroids and, therefore, their collateral effects. Hence, we assert that the social cost of the management of ITP disease is not higher in children treated with IVIG. Furthermore, short hospitalization for IVIG therapy contributes to accept this disease. It should be advisable to make controlled studies to define with more accuracy the role of this preparation and the modality of its use in the different clinical conditions in which it is employed.

[Hypochromic hyposideremic anemia as the first symptom of gastroesophageal reflux]. / Anemia ipocromica iposideremica come primo sintomo di reflusso gastroesofageo.

In a five months infant is described by authors iron deficiency anaemia as early symptom of gastroesophageal reflux undiagnosed previously. Laboratory evaluation excluded any haematological pathology; thereafter clinical suspicion was confirmed by radiological exams and oesophageal PH-monitoring.