Cross-sectional echocardiography with pulsed and continuous wave Doppler in the management of ventricular septal defects.

We designed this study in order to evaluate those cross-sectional echocardiographic projections of most value in the diagnosis of ventricular septal defects and to compare the techniques of cross-sectional and Doppler echocardiography in these lesions. We studied 71 cases with ventricular septal defects confirmed by cross-sectional and/or pulsed Doppler echocardiography. The defect was imaged by cross-sectional echocardiography in 49 patients but not imaged in 22. In the group of 49 patients, except two with pulmonary hypertension, pulsed Doppler enabled us to detect a left-to-right shunt at ventricular level. In the second group of 22 patients, a positive pulsed Doppler signal was detected in the ventricles although no defect was visualized. Pulsed Doppler examination supplemented the information detectable from cross-sectional echocardiography in small defects; in the diagnosis of multiple septal defects; in the presence of aortic valve regurgitation in doubly committed and subarterial defects; in those having residual shunts after surgical correction; and in those with tricuspid valve regurgitation in the setting of perimembranous defects. Continuous wave Doppler cannot always be reliably employed in the evaluation of transventricular pressure gradient because of a failure to align with the jet in the presence of poor signals. The sub-costal oblique projections and the introduction of the right oblique sub-costal view proved, in our hands, to be the most important tools for identifying and classifying the various types of ventricular septal defect.

Nifedipine treatment of Raynaud's phenomenon in a paediatric age.

Success in the treatment of juvenile Raynaud's phenomenon has been obtained with nifedipine at 10-13 mg/day in a 6-year-old boy and an 8-year-old girl.

The outcome of Wilms' tumor in infants. Italy 1970-79.

Thirty-four infants under 1 year of age with Wilms' tumor were diagnosed and treated in 14 Italian pediatric oncology units during 1970-79. The 3-year survival rates decreased with higher group unilateral tumors: 95% in group I Wilms' tumor, 75% in group II and 20% in group III. The survival rates for children with group I and II Wilms' tumor were similar for those who were treated with surgery and chemotherapy and those who also received postoperative radiotherapy. During 1975-79 fewer patients with group I Wilms' tumor received radiotherapy (1 of 11) than during 1970-74 (4 of 6, p less than 0.05). All these children are alive at this writing.

Changes in blood pressure reactivity and 24-hour blood pressure profile occurring at puberty.

To evaluate blood pressure reactivity in children and its changes in adolescents, the acute pressor response to a video-game stress test and the noninvasive ambulatory blood pressure monitoring were evaluated in 62 healthy children divided into three age groups. Basal blood pressure values were measured according to the NIH Task Force. With baseline measures and body mass index controlled for, analysis of covariance showed that the video game provoked significant and incremental cardiovascular reactivity across the games in adolescents when compared with the two other groups of children. The same group of children showed also a significantly higher systolic ambulatory pressure during the daytime, whereas no significant difference was observed by basal BP measurement. In conclusion an increased reactivity to external stimuli was observed in adolescents, and this pattern was strictly associated with a higher daily blood pressure.

[An open non-comparative study of the efficacy and tolerability of sultamicillin in the therapy of respiratory infections in childhood]. / Studio aperto non comparativo dell'efficacia e tollerabilità della sultamicillina nella terapia delle infezioni respiratorie in età pediatrica.

Forty-eight children (25 males + 23 females), mean age 3.5 years +/- 2.6 (range 1-11), were treated for the following respiratory infections: pharyngotonsillitis (9), bronchitis (18), bronchopneumonia (14), asthmatic bronchitis (4) and pneumonia (3). The average duration of treatment was 5.3 +/- 2.0 days (range 3-13). Sultamicillin was administered at the dose of 50 mg/kg/day. Patients with fever experienced a defervescence on the second day of therapy. Forty-six children (96%) showed a good clinical response. The tolerability of the drug was excellent or good in 93.8% of the cases.

[Accidents in childhood. From epidemiology to prevention]. / Gli incidenti in età evolutiva. Dall'epidemiologia alla prevenzione.

Accidents are still the first cause of death in childhood with the exception of the first year of life. Perhaps the most important barrier in their prevention is in the term "accident" itself: the concept that an accident is an unpredictable and so unpreventable event, not an injury with a specific epidemiology that can be watched, studied, solved. The pediatrician plays, a key-role in accident's prevention: as epidemiologist, expert of growth and development, child's advocate, teacher and promoter of researchers, educational campaigns, policies. So he can no more say: "Accidents are not a medical problem"; on the contrary it is the time to know accident's epidemiology, apply the "safety equations" in the real life and be finally "accidentologists".

[Economic and social aspects of pediatric dialysis in Italy]. / Aspetti economici e sociali della dialisi pediatrica in Italia.

Rarely in italian medical journals have been discussed the social and economic problems related to dialysis much less the ones related pediatric dialysis. On the contrary, we believe that these problems hold a great importance due to their obvious consequences on the family structure, society and most of all on the psychophysical development of the affected child. Present structures of pediatric centers in Italy have been studied along with the available facilities, their spreading over the national territory and consequent transport problems. We also provided data regarding social and scholastic rehabilitation of the little patients as well as the effects of followed treatments on the family economy. Through these we can say that at the moment in Italy the 50% only of the children are treated in pediatric centers, which are still unequally distributed with a major concentration in the North, followed by the South and the Center Italy as last. However, it is important to notice that in every Center the child is seen as an individual and many efforts are done to reach his complete welfare. This purpose justify the presence, besides the specialized medical and nursing staff, of many dietitian, psychologist, teachers, play teachers, social workers. Results of a good recovery are evident in the sphere of the little patients, at school as at home, even if it is still difficult to evaluate a following complete integration in the work world. Still far away from solution is transplant problem in Italy: centers are insufficient and not perfectly working; patients suffer long waiting-lists; which causes to find the solution of their problem abroad.

[Schoenlein-Henoch syndrome. Renal involvement and prognosis]. / La sindrome di Schoenlein-Henoch. Interessamento renale e prognosi.

We have studied the incidence of renal involvement, the severity and the clinical course of nephropathy in 83 children, 47 males and 36 females, aged from 2 to 13 years, who were under observation for SSH at the II Pediatric Clinic, Florence University. In 72.3% of cases, we have not observed any sign of renal involvement, at least within 6 months of onset of the syndrome. In 16.3% we have observed persistent urinary abnormalities: these findings have returned to normal within 1 year in 13 children and within 3 years in one child. In 10.8% of cases, a nephropathy has appeared, in all of the cases within 3 months of onset. Only one case has developed renal failure 5 years after onset. We can conclude that a good correlation exists between clinical manifestations of this disease and histopathologic changes; that therapy is of little value in modifying the clinical course; that renal failure is a rare occurrence.

[Azlocillin in the treatment of pulmonary infections in patients with cystic fibrosis: plasma concentrations and therapeutic indications]. / L'azlocillina nel trattamento delle infezioni polmonari dei pazienti affetti da fibrosi cistica: concentrazioni plasmatiche e indicazioni terapeutiche.

Azlocillin plasma concentrations have been studied in 10 cystic fibrosis patients suffering from chronic pulmonary infections with Pseudomonas aeruginosa. Patients were given single i.v. doses of 100 e 200 mg/kg body weight as intravenous infusion over 30 minutes. Azlocillin plasma levels have been assayed by a rapid, sensitive and precise high performance liquid chromatographic method. After the dose of 100 mg/kg body weight concentrations of azlocillin decreased below the therapeutic concentrations after three hours; dose of 200 mg/kg was followed by plasma concentrations in the therapeutically desirable range during the 6-8 hours study period. The pharmacokinetic analysis offers further evidence of the dose-dependent nature of azlocillin elimination. Higher dosage of 200 mg/kg body weight and monitoring of plasma drug levels are recommended in the therapy of patients with cystic fibrosis.

[Evaluation and treatment of the enuretic child: eight years' experience]. / Valutazione e trattamento del bambino enuretico: l'esperienza di otto anni.

Enuresis is a common functional problem among children which is defined as a complete involuntary voiding of urine at an age which control should be present. Bed wetting generally resolves with increasing age, but the restriction in social life and the psychological secondary problems, so frequent in older patients, justify an appropriate treatment of the problem in the child over seven. At children's Hospital of Florence University an enuresis service exists since 1983, and during these years 541 children applied to the structure. 326 children completed the treatment, among these there were 202 boy and 124 girls with age between 6 and 19. All the patients have been initially helped only with conversation (motivational counseling) and 76 among them (23% of the whole) obtained permanent cure. The remaining 250 children were treated with the conditioning alarm system, always associated to periodic conversation, urine stop exercises and other psychological support (token economy, etc). There were 161 boys and 89 girls: 220 children had nocturnal primary enuresis and 30 secondary. The family history was positive in 77%. The results obtained of this kind of treatment after a follow-up of 6 months, were permanent recovery in 211 children (84%) and failure in 39 patients (16% of the cases). There have been 35 relaxes. Regarding the sex, no significant difference was noted. These positive results with the conditioning devices favor the view that the etiology of primary enuresis is mainly biologic. The bell alarm represents the most effective treatment for nocturnal enuresis included more than seven.(ABSTRACT TRUNCATED AT 250 WORDS)

[Influence of iron metabolism on the efficacy of r-HuEPO (recombinant human erythropoietin) treatment of anemia in children on hemodialysis]. / Influenza del metabolismo del ferro sull'efficacia del trattamento dell'anemia con r-HuEPO (recombined human erythropoietin) in pazienti embodializzati in età pediatrica.

Recombinant Human Erythropoietin (r-HuEPO) is efficient in the treatment of anaemia in terminal renal failure under dialysis. Five pediatric patients, who were under periodic hemodialysis, were treated and the interaction between the metabolism of iron and the response to r-HuEPO was studied in particular. In two patients it was noticed that a significant reduction of hematic ferritin levels occurred, while an efficient erythropoietic activity was maintained. On the contrary, three patients showed iron deficiency characterized by a reduced percentage of total transferrin saturation in the plasma, in the presence of high levels of ferritin in the blood. Also discovered was a missing increase or even a fall of the hemoglobin values that were obtained till now. In these cases, the increase of the hormone dose didn't lead to an improvement, that could only be obtained by the oral or parenteral administration of iron. The Authors in conclusion affirm that iron deficiency is the first cause to be searched for and to be corrected in the presence of missing hemoglobin increase even with adequate doses of r-HuEPO.

[Arthropathies related to HLA-B27]. / Artropatie correlate all'HLA B27.

Spondyloarthropathies represent an important problem within the field of chronic childhood arthropathies. Nosology and differential diagnosis are yet unclear. It is important to distinguish spondyloarthropathies from JCA because biological aspects of affected patients, clinical findings, extraarticular manifestations and prognosis are very different. Ankylosing spondyloarthritis is the prototype of spondyloarthropathies: at the beginning, axial involvement is rare; it may develop during the following years or it may not occur. Enthesopathy is an important finding of spondyloarthropathies. Diseases with joint involvement, HLA B27 related, as ankylosing spondyloarthritis, psoriatic arthritis. Reiter's syndrome or arthritis associated with chronic bowel disease, enter the chapter of spondyloarthropathies. Children with familial history of spondyloarthropathies showing enthesopathy, "sausage fingers" and with the presence of HLA B27, may be classified in the group of spondyloarthropathies. Children with a chronic arthritis with pauciarticular onset, B27 positive, without any sign and finding spondyloarthropathies, should be classified as JCA from the beginning. A follow up of children affected with chronic arthritis is fundamental for a more correct classification of the disease.

[Pulmonary calcification in vitamin D poisoning in an infant]. / Calcificazioni polmonari in corso di intossicazione da vitamina D in un bambino.

We describe the case of a child aged 11 months with vitamin D intoxication and hypercalcemia, who developed acute renal failure and dyspnea. Chest X-rays showed interstitial changes compatible with either pulmonary alveolar proteinosis or pulmonary edema. The hypercalcemia suggested the possibility of metastatic calcifications of the lung. This hypothesis was subsequently confirmed by the progressive disappearance of pulmonary findings as calcemic levels returned to normal values... Our report emphasize the opportunity of studying the respiratory system in each patient with hypercalcemia, whichever the etiology may be.

[Care of oncologic child and his family: viewpoint of the pediatrician]. / L'assistenza al bambino oncologico e alla sua famiglia, vista dal pediatra.

The authors report their considerations on the care of the oncologic childhood and their families. Various psychologic aspects are discussed, first of all the child and his cancer, how the parents and siblings face the disease, death and after. Finally they outline the role of pediatrician and the hospital staff.

[Mucha-Habermann disease. Description of a case in childhood]. / La malattia di Mucha-Habermann. Descrizione di un caso in età pediatrica.

The authors report a case of Mucha-Habermann disease in childhood. Mucha-Habermann disease is not a very well known, though not infrequent, disease. It is characterized by recurrent erythematous-papular-vesicular skin lesions associated with arthralgia or arthritis or large joints. Prognosis is generally favourable although an evolution towards Pityriasis Lichenoides Chronica and/or Mycosis Fungoides is possible. There are not specific laboratory findings for this form. Diagnosis is essentially based on histology showing an immunopathogenetic vasculitis. At the present time there is not a safe therapy for the disease; there are however indications for the use of Erythromycin and we followed these in our therapy with positive results.

[Role of viruses in acute gastroenteritis]. / Inquadramento etiopatogenetico delle enteriti acute virali.

In a two years screening carried out on Florentine children we confirmed that Rotaviruses are the most important ethiological agents of acute gastroenteritis in infants less than two years old, irrespective of sex. We had evidence that Rotavirus diarrhea does not occur with the same incidence every year. Rotavirus gastroenteritis is characterized by profuse diarrhea, vomiting, fever and often by respiratory symptoms. Severe complications have not been observed.

[Osteodystrophy in children with chronic renal insufficiency in dialysis therapy]. / Osteodistrofia nel bambino con insufficienza renale cronica in trattamento dialitico.

One of the most important complication of patients with chronic renal failure is osteodystrophy. This causes skeletal deformities, growth failure, bone pain and decreased physical activity. Osteodystrophy is more frequent among children than uraemic adults. In fact, 50-80% of children with chronic renal failure may occur in metabolic bone disease and the incidence tends to be higher in those children who have been in uraemic state for a long time before starting chronic haemodialysis. Osteodystrophy is a result of: 1) lesions of rickets; 2) lesions of osteitis fibrosa: 3) osteosclerosis. In contrast to adult, metastatic calcifications are virtually never observed in uraemic children. Hyperphosphoraemia, that is secondary to the reduction of G.F.R., may be the principal responsible of hyperparathyroidism that is the main cause of osteodystrophy. Hyperparathyroidism is also maintained and increased by deficit of 1,25(OH)2D3 which is responsible for lesions of rickets. Haemodialysis may markedly improve osteitis fibrosa and it is efficacious in reversing the mineral defect. Dialysate calcium concentration should be maintained at approximately 3,5 mEq/l. In this case we can raise serum calcium. On the contrary dialysate has to be lacking in phosphorus to correct hyperphosphoraemia. It must be noted that we have to prepare a dialysate with deionized water lacking in aluminum to avoid encephalopathy compliance.

[Juvenile rheumatoid arthritis with pauciarticular onset and HLA-A, B, DR in Italian children]. / Artrite reumatoide giovanile ad esordio pauciarticolare e HLA-A, B, DR in bambini italiani.

Juvenile Rheumatoid Arthritis is a heterogeneous disease currently divided into different subtypes based on clinical characteristics. Significant alterations in frequencies of HLA-A, B and DR antigens have been described previously in children with pauciarticular onset Juvenile Rheumatoid Arthritis. We report the results of a study on 42 italian children with pauciarticular onset Juvenile Rheumatoid arthritis that partially confirm the literature data.

[Juvenile rheumatoid arthritis]. / Artrite reumatoide giovanile.

Juvenile rheumatoid arthritis (JRA) is the most common rheumatic disease in children. It is one of the more frequent chronic illnesses of childhood and an important cause of disability. Authors review the literature about JRA.