Papillary thyroid cancer in thalassaemia.

Thyroid cancer is the most frequent endocrine neoplasm in the general population. Its incidence is 5-10/100,000 per year, with an annual death rate of 0.2-1.2/100,000 in men and 0.4-2.8/100,000 in women. In thalassaemia patients the frequency of this disease is unknown. In this paper we describe five cases of papillary thyroid cancer in thalassaemia patients followed at the Day Hospital for Thalassaemia and Haemoglobinopathies, in Ferrara, Italy. We consider the possible key role of iron as a carcinogenic agent and we also discuss the practical implications of our clinical observations.

Complications and risk factors for severe outcome in children with measles.

OBJECTIVE AND DESIGN: Risk factors for severe measles are poorly investigated in high-income countries. The Italian Society for Paediatric Infectious Diseases conducted a retrospective study in children hospitalised for measles from January 2016 to August 2017 to investigate the risk factors for severe outcome defined by the presence of long-lasting sequelae, need of intensive care or death. RESULTS: Nineteen hospitals enrolled 249 children (median age 14.5 months): 207 (83%) children developed a complication and 3 (1%) died. Neutropaenia was more commonly reported in children with B3-genotype compared with other genotypes (29.5% vs 7.7%, p=0.01). Pancreatitis (adjusted OR [aOR] 9.19, p=0.01) and encephalitis (aOR 7.02, p=0.04) were related to severe outcome in multivariable analysis, as well as C reactive protein (CRP) (aOR 1.1, p=0.028), the increase of which predicted severe outcome (area under the receiver operating characteristic curve 0.67, 95% CI 0.52 to 0.82). CRP values >2 mg/dL were related to higher risk of complications (OR 2.0, 95% CI 1.15 to 3.7, p=0.01) or severe outcome (OR 4.13, 95% CI 1.43 to 11.8, p<0.01). CONCLUSION: The risk of severe outcome in measles is independent of age and underlying conditions, but is related to the development of organ complications and may be predicted by CRP value.

Cardiomyopathy and pericardial effusion in a 7 year-old boy with beta-thalassaemia major, severe primary hypothyroidism and hypoparathyroidism due to iron overload.

The primary cause of cardiac dysfunction in thalassemia is believed to be myocardial iron overload. Besides iron, other factors may play a role in the impairment of myocardial contractility, including prolonged heart tissue hypoxia, pericardial involvement, arrhythmias, endocrine complications and vitamin D deficiency. We present the case of a 7 year-old boy with ?-thalassaemia major and cardiac dysfunction, pericardial effusion and associated endocrinopathies. His serum thyrotropin (TSH) level was increased, and total and free thyroxine (FT4) were low. In addition, biochemical results and serum PTH level were compatible with a diagnosis of hypoparathyroidism. Other laboratory findings were not consistent with rheumatic heart disease, viral myocarditis or autoimmune disease. The child was treated with digoxin, diuretics, oral calcium, vitamin D, L-thyroxine (25 microg daily, which was later gradually increased) and subcutaneous iron chelation therapy (45 mg/kg, six days/week). The patient was discharged from our Unit after 7 days and within 3 months he had appreciable myocardial improvement and disappearance of the pericardial effusion.

Exaggerated TSH response to TRH ("sub-biochemical" hypothyroidism) in prepubertal and adolescent thalassaemic patients with iron overload: prevalence and 20-year natural history.

The severity of thyroid dysfunction in patients with beta-thalassaemia major is variable in different series. An exaggerated thyrotropin response to thyrotropin-releasing-hormone (TRH) has been found in 1 out of 5 beta-thalassaemia major patients. Because it is not well known how many of these patients will develop overt or subclinical hypothyroidism, we reviewed retrospectively the thyroid status (thyroid hormones, TBG, TG basal and TSH peak after TRH test) of 24 children and adolescents with beta-thalassemia major (mean age 12.1+/-3.9 years) on periodic transfusion therapy, regularly followed in our Centre during the last 20 years. As controls we studied 30 normal subjects aged 13.4+/-2.5 years. In our group of 24 thalassaemics an exaggerated TSH response to TRH test was found in 8 (33.3%), 3 of whom developed subclinical or overt hypothyroidism from 3 to 11 years later. TSH peak values correlated directly with ferritin levels, ALT, and compliance index to chelation therapy. Our data suggest that an exaggerated TSH response to TRH test is frequent in beta-thalassemia major, and may evolve into subclinical or overt hypothyroidism as we found in 37.5% of our patients with sub-biochemical hypothyroidism.

Multifocal Skin Tuberculosis: Report of A Case.

Tuberculosis (TB) is a severe problem in underdeveloped countries. Cutaneous TB is rare and often goes unrecognized. We report a Pakistani child with multifocal cutaneous and pulmonary TB. Microbiologic diagnosis was obtained when the abscesses were biopsied. Four-drug therapy produced rapid improvement of the lesions. A high level of suspicion must be maintained when evaluating children from countries at risk.

Risk factors associated with complications/sequelae of acute and subacute haematogenous osteomyelitis: an Italian multicenter study.

BACKGROUND: Acute/subacute haematogenous osteomyelitis (AHOM/SAHOM) are potentially devastating diseases. Updated information about the epidemiology, management and outcome of AHOM/SAHOM is needed to minimize the risk of complications and sequelae. METHODS: A multicenter study was performed to evaluate retrospectively the management and outcome of AHOM/SAHOM in Italy. Data from children aged >1 month, and hospitalized between 2010 and 2016, in 19 pediatric centers, were analyzed. RESULTS: 300 children with AHOM and 98 with SAHOM were included. Median age was 6.0 years (IQR: 2.0-11.0). No clinical difference was observed with the exception of fever at onset (63.0% vs. 42.9%; P < 0.0001), and a more common spinal involvement in SAHOM (6.7% vs 20.4%; P < 0.001). Fifty-Eight Staphylococcus aureus strains were isolated; 5 (8.6%) were MRSA. No Kingella kingae infection was documented. No different risk for complication/sequela was observed between AHOM and SAHOM (38.3% vs. 34.7%; OR:0.85; 95%CI: 0.53-1.38; P = 0.518). Duration and type of antibiotic therapy were not associated with risk of complication/sequelae. CONCLUSION: AHOM and SAHOM displayed some differences, however occurrence and risk factors for complications and sequelae are similar, and the same empiric treatment might be recommended.