Synovial cyst in juvenile idiopathic arthritis.

Small synovial cysts are a common manifestation of juvenile idiopathic arthritis; large brachial cysts, however, are a rare sign of the disease and they must be differentiated from other soft tissue swelling which are not related to articular involvement. We describe the case of three children with juvenile idiopathic arthritis who came to our attention with large synovial cysts. Ultrasonographic examination and MRI were performed in all cases, showing the real nature of the swelling and the connection to the joint. In all cases, swelling reduced and then disappeared with control of disease activity; in two cases, they reappeared in coincidence with a severe relapse of juvenile idiopathic arthritis. Brachial swellings represent a diagnostic challenge because they can be the clinical expression of a variety of diseases. In children with juvenile idiopathic arthritis who present with a sudden swelling of the upper arm, synovial cysts must be considered in the diagnostic workout, because they are a possible rare manifestation of juvenile idiopathic arthritis.

Altered cytokine and acute phase response protein levels in the blood of children with Downs syndrome: relationship with dementia of Alzheimer's type.

Downs syndrome (DS) subjects are at high risk of developing Alzheimer's disease (AD). Patients with AD often show altered levels of some immune molecules in their peripheral blood which correlate with cognitive impairment. However, whether the altered peripheral immune phenotype is a late and secondary phenomenon associated with dementia or an early impairment linked to mechanisms controlling neurodegeneration of the central nervous system (CNS) is still an unanswered question. Here we studied immune molecules in the blood of non demented children with DS to investigate whether altered peripheral immune phenotype could be present in these subjects without dementia, many years before the presentation of clinical signs of cognitive deterioration. Plasma levels of interleukin-6 (IL-6) and soluble IL-6 receptor (sIL-6R) were significantly higher in DS than in control children. Plasma levels of soluble intercellular adhesion molecule-3 (sICAM-3), soluble vascular cell adhesion molecule-1 (sVCAM-1) and C reactive protein (CRP) were also increased in DS. The increase of IL-6 and CRP from DS children was similar to that found in elderly patients with clinical AD. Peripheral altered immune phenotype in healthy young subjects with DS might be an early sign of CNS alterations leading many years later to cognitive deterioration and dementia.

[CVS-cardiac thrombosis related in a child with an intracranial germinoma]. / Trombosi intracardiaca grave in paziente affetta da germinoma intracranico portatrice di CVC.

We report a case of a CVC infection with a grave cardiac thrombosis in a child afflicted with intracranial functioning germinoma. During the second chemotherapeutic cycle we found a positive blood culture and a partial CVC occlusion; echocardiography showed a 2 cm diameter interatrial thrombus. After removal of the CVC a voluminous thrombus in the right atrium persisted. The profibrinolytic factor's concentration was normal, and so we preferred to use aspirin, the most common antiplatelet agent, combined with antibiotic and antimycotis therapy. This treatment allowed us to observe the disappearance of the thrombus by 25 days.

[Treatment of iron deficiency in infancy with iron-acetyl transferrin]. / II trattamento della carenza marziale delle prime epoche di vita con ferro-acetil-transferrina.

In twenty eight patients with iron deficiency the efficacy of iron-acetil-transferrin treatment (2-3 mg/kg/die) has been evaluated from the changes of the following variables: RBC and reticulocyte count, Hb concentration, MCV, MCH, serum ferritin, serum iron, TIBC, and ZnPP. These variables were assessed before and after three months of treatment in all patients, and after three months from the end of the treatment in thirteen patients. At the end of the treatment there was a significant increase of RBC count, Hb concentration, MCV, MCH, serum ferritin, serum iron, and TIBC, a significant decrease of ZnPP, while reticulocyte count remained essentially unchanged. After three months from the end of the treatment only serum ferritin and ZnPP underwent an additional significant increase and decrease, respectively. In twenty-six patients serum ferritin values returned to normal. The changes of RBC and reticulocyte count, Hb concentration, MCV, serum iron, and TIBC were larger the lower the initial values, suggesting that the efficacy of the treatment is greater the more serious the iron deficiency.

Efficacy and safety of pidotimod in the treatment of recurrent respiratory infections in children.

The activity of pidotimod ((R)-3-[(S)-(5-oxo-2-pyrrolidinyl) carbonyl]-thiazolidine-4-carboxylic acid, PGT/1A, CAS 121808-62-6) was evaluated in a double-blind, placebo-controlled, randomized, multicentre trial, on 120 pediatric patients affected by recurrent respiratory infections. The clinical course of acute infections was favourable both in placebo and in treatment group, but recovery was quicker with pidotimod than with placebo. Antibiotic therapy and time of hospitalization were shorter in the patients taking pidotimod, and main symptomatic parameters (pharyngalgia, dysphagia, mucous membrane inflammation, adenopathy, anorexia) receded quickly. In patients receiving the drug as well as in placebo group changes in laboratory parameters, indicating recovery from the acute infectious events, were observed. A significant trend to normalization of the immune response, evidenced by chemotaxis and leukocyte phagocytosis index, was found only in patients treated with pidotimod. A significant decrease in the risk of relapses was observed in patients treated with pidotimod (35%), as well as a reduction of hospitalization (86%) and a decreased antibiotic therapy (47%). If a relapse occurred, the response of treated patients was quicker (fever, antibiotic therapy, hospitalization). These findings allow to correlate the individual immune response activation to the resistance to recurrent infections and also to a better response to therapy in case of clinically relevant disease. No side effects were observed. Only in 12 patients (5 pidotimod, 7 placebo) mild reactions were observed, but they were attributed to concomitant antibiotic treatment or other factors. No alterations in main laboratory parameters were seen.(ABSTRACT TRUNCATED AT 250 WORDS)

[Clinical improvement induced by chemotherapy and social rehabilitation in a preterminal case of brain stem neoplasm: the objective and instrumental aspects]. / Miglioramento clinico indotto dalla chemioterapia e riabilitazione sociale in un caso preterminale di neoplasia tronco-encefalica: aspetti obiettivi e strumentali.

A sixteen-years-old boy with a brain-stem neoplasia inducing coma was treated with chemotherapeutic agents. It resulted in dramatic clinical and instrumental improvement; radiologic findings were evaluated, showing decreasing dimension of intracranial mass. Brain-stem auditory evoked potentials were highly sensitive. We think that chemotherapy can be considered among the options a physician can offer to such a patient explaining informed consensus.

[Iron deficiency in children: which is the correct therapy?]. / Carenza marziale in età pediatrica: quale la corretta terapia?

Iron is essential for human metabolism. Under normal circumstances its homeostasis is strictly kept by absorption and excretion through genitourinary, gastrointestinal tracts and skin losses. In several systemic disorders, dietary iron is insufficient to keep such a dynamic balance: development of iron deficiency may be due to increased requirements, decreased intestinal absorption, inadequate dietary uptake. Low birth weight newborns, children and adolescents are at increased risk for developing iron deficiency. Although clinical aspects may vary, hematochemical findings show a three-step gradual progression. In a first step iron deficiency is diagnosed by serum ferritin level which will be under 10-20/micrograms/ml showing a depletion of total body iron stores. In a second step progressing iron deficiency will be assessed by lowered serum iron and increased unsaturated serum transferrin, serum iron bound to transferrin and erythrocyte protoporphyrin IX. Scanty clinical signs are still available. In a third step while clinical findings show a complete features of iron deficiency anemia (weakness, fatigue, palpitations, etc.), laboratory findings show morphologic alterations in red cells (hypochromia and microcytemia), together with the aforementioned disorders in ferrokinetics. Iron deficiency anemia responds very effectively to treatment due a correct etiological diagnosis, crucial to a through therapy tending to first eliminating the causes of it. Prophylaxis against iron deficiency anemia is required in prematurely born and low birth weight infants because of doubled iron requirements. After the second month of life diet is supplemented with 2-4 mg/kg/die of ferrous iron orally along the first year of life.

[Children with hemorrhagic diathesis: correct diagnostic and therapeutic approach]. / Il bambino con diatesi emorragica: corretta impostazione diagnostica e terapeutica.

Bleeding defects are of great interest in pediatrics since the prevalence of congenital forms and the early appearance of acquired ones. The pathology itself and the therapy indeed can often interfere with the growing-up patients. Bleeding defects have been identified with an heterogeneous group of clinical disease that differs from one another in etiology, pathogenesis, epidemiology and incidence in population. Bleeding diathesis is the common symptom: bleeding tendency may be mild, moderate or severe, localized or generalized, cutaneous or mucosal, superficial or deep. Bleeding disorders may be classified as a) defects in the primary haemostasis, which include quantitative and qualitative abnormalities of platelets and vascular disorders and b) defects in secondary haemostasis, which include intravascular disorders (blood coagulation). Careful history and clinical examination are essential in diagnosis of bleeding disorders. History of patient should be taken a) to differentiate acquired from congenital disease and to know the way of hereditary transmission (family history); b) to know exactly the disease's start and the mutual relation with former or accompanying disease; c) mutual relation with drugs token. Subsequently a careful physical examination should be done. A specific hemorrhagic diathesis has been seen with a deficiency of primary or secondary haemostasis. A deficient or late haemostatic plug in small vessels can cause superficial, interstitial bleeding that may be intracutaneous or intramucosal and is called purpura. In coagulation factor deficiency the haemostatic plug cannot be consolidated by fibrin: spontaneous hematomas, hemarthrosis and ecchymoses often occurs. The initial laboratory work up for screening patients with bleeding disorders should include first step tests to differentiate bleeding disorders for bone-marrow malignancies; from virus infections carrying screening of major viruses and from hepatic diseases. Second step laboratory examination includes a) platelet count or estimation of platelet number on blood smear; b) bleeding time to test small vessel integrity and platelet function; c) aPTT, PT, AP to measure clotting activity; d) fibrinogen determination. With this battery of screening test it is usually possible to determine the general area of the defect (abnormalities of platelets number or function or congenital defect of one or more clotting factors activity). Acute idiopathic thrombocytopenic purpura is the most common bleeding disorders in childhood. Usually no therapy may be required no matter platelet count. Patients with a significant hemorrhagic tendency are treated either with prednisone (2 mg/kg orally in divided daily doses) for a period of 2 weeks or with a 5 days course of special polyvalent intact immunoglobulin (400 mg/kg/die) for intravenous use.(ABSTRACT TRUNCATED AT 400 WORDS)

Hepatitis B vaccination of 113 hemophiliacs: lower antibody response in anti-LAV/HTLV-III-positive patients.

One-hundred thirteen adults and children with hemophilia or other congenital bleeding disorders were vaccinated against the hepatitis B virus. Each patient was given three subcutaneous injections of the vaccine at monthly intervals and then a fourth booster dose 14 months after the first. The vaccine was highly immunogenic, since 111 of 113 patients (98%) produced anti-HBs (10 mIU/ml or more). After the first three vaccine doses and after the booster dose, ten anti-LAV/HTLV-III-positive hemophiliacs produced anti-HBs but had a lower average titer than anti-LAV/HTLV-III-negative hemophiliacs. Of the 23 patients treated with concentrates in the 15 month postvaccination period only, none acquired HBV infection. Of the 50 patients treated with concentrates also in the 6 month prevaccination period, one developed hepatitis B. In summary, the vaccine was highly immunogenic in both children and adults with hemophilia; anti-LAV/HTLV-III-positive patients responded to the vaccine, but the average anti-HBs response was lower; no case of hepatitis B occurred in patients treated with concentrates only in the postvaccination period.

[Sudden infant death syndrome in Italy. A multidisciplinary approach]. / La sindrome della morte improvvisa del lattante in Italia. Un approccio multidisciplinare.

A Center for the Sudden Infant Death Syndrome has been established in 1981. Active research involves clinical prospective studies and basic research, mostly aimed at testing our cardiac theory for SIDS. The bereaved families enter in a follow-up program with specific psychological support. At risk babies are evaluated for cardio-respiratory abnormalities and repeatedly controlled. Parents of victims are in the process of forming a group with the objective to support and inform the families of new victims. This is the first attempt to approach systematically the SIDS problem in Italy.

[Thermographic monitoring of the evolution and therapy of chronic synovitis and cysts in hemophiliacs]. / Monitoraggio termografico dell'evoluzione e della terapia nella sinovite cronica e nella ciste degli emofili.

The authors have studied the evolution of chronic synovitis and of cysts in hemophilic patients, through measurement of the articular circumference or of the cyst. To obtain this, they have used a folding rule and liquid cristal strips, that have the characteristic, that they change colours from braunish to orange, from greenish to bluish according to the thermic gradient revealed. The authors have studied 21 hemophilic patients, affected from chronic synovitis for a total of 23 articulations and 5 patients with cyst. All patients have undergone several controls reporting dates revealed at 2,6 and 12 months from beginning of disease. The authors conclude, underlining the usefulness of this simple methodic that permits: - a reliable valutation of the therapeutic (profilatic) scheme applied to every single patient and gives a prognostic precision; - distinguishing, in fact, the recovery from the clinical recovery of the synovitis in order to avoid the appearance of unpleasant relapses.

[Idiopathic pulmonary hemosiderosis: report of a case with a favorable response to cyclophophamide therapy]. / Emosiderosi polmonare idiopatica: descrizione di un caso clinico con favorevole risposta alla terapia con ciclofosfamide.

A 13-years old boy who presented a severe degree of iron-deficiency anemia and diffuse parenchimal infiltrates on the chest roentgenogram is reported. The clinical picture and the presence of hemosiderin laden macrophages in bronchial washing suggest Idiopathic Pulmonary Hemosiderosis (I.P.H.): open lung biopsy confirmed the diagnosis. Immunofluorescence studies showed no deposition of IgG, IgA, IgM and B1C in the lung. The most striking abnormality observed at electron microscopy was hemosiderin deposition in the alveolar-capillary basement membrane. One year after cyclophosphamide therapy was both in complete hematologic and pulmonary remission.

[Infantile angiodysplasias: diagnosis and therapy]. / Le angiodisplasie infantili: diagnosi e terapia.

Three cases of angiodysplasias are reported. There are some problems still open concerning diagnostic and therapeutic approach. In this paper we try to correlate localisation, time of onset, size, aspect, histological features, evolution, side effects and hemodynamic problems with diagnosis, prognosis and therapy of childhood angiodysplasias mainly concerning cavernous, intracranial, visceral and lymphatic angiomas.

[Intestinal lymphangiectasis: late complication of transportation of the great vessels corrected by the Mustard technic. A clinical case]. / Linfangectasia intestinale: complicanza tardiva di una trasposizione dei grossi vasi arteriosi corretta con tecnica di Mustard. Caso clinico.

The Great Arteries Transposition corrected by Mustard's repair is often complicated with inferior vena cava obstruction with consequent intestinal lymphangiectasia and protein-losing enteropathy. We report a case of a 9 years old boy who underwent Mustard's repair and presented diarrhoea, abdominal pain, hypoproteinemia and ascites. The fecal elimination of 51Cr-labeled albumin was remarkable increased and the intestinal biopsy showed a noteworthy lymphangiectasia due to an hemodynamic overload. Therefore subjects with Great Arteries Transposition corrected by Mustard's repair should be valued in the post-surgical follow-up by 51Cr-labeled albumin fecal elimination and by intestinal biopsy, already sensitive in asymptomatic phase.

Leukaemic hypopyon in acute lymphoblastic leukaemia after interruption of treatment.

A 7-year-old girl was successfully treated for acute lymphoblastic leukaemia, and remained in remission after treatment had been completed in 3 years. Four months after cessation of treatment, iridocyclitis with hypopyon developed in one eye. Exudate from the anterior chamber contained numerous lymphoblasts. Local radiotherapy led to complete resolution of the ocular lesions, and the patient remains well 22 months later.