Biomedical subjects
Publications and source records attributed to G Tani.
We report a child affected by Wernicke's encephalopathy (WE), which was unsuspected clinically. MRI suggested the correct diagnosis and prompted appropriate thiamine replacement. WE is a difficult condition to recognise, especially in children, and MRI may be useful in the diagnosis of the disease.
Explore the source record for details and available documents.
Pulmonary blastoma is a rare malignant tumor seen in both adults and children. Approximately only 25% of cases occur in pediatric patients, many of whom affected by a congenital pulmonary cystic lesion. The clinical features, radiological findings and management of a 3-year-old boy affected by a pulmonary blastoma which arose in a congenital cystic adenomatoid malformation are reported, and an extensive review of the literature is also made. Because of the well-known tendency of cystic pulmonary diseases to develop malignancies, authors recommend the surgical excision of these kind of lesion or at least their close radiological follow-up.
Previous studies of contextual interference have shown that practicing several motor skills randomly (high contextual interference) facilitates retention and transfer in comparison to practicing the same tasks in a blocked order (low contextual interference). However, many studies have not supported this phenomenon in motor learning, and some researchers have questioned whether the effect can be tested by using only a few trials on the transfer test. The present study used a different methodological approach in which the number of test trials was increased to assess whether the contextual interference effect is sustained over an extended number of trials in the transfer phase. Undergraduate students (N=32) were randomly allocated to either a blocked or random group. The participants practiced 80 acquisition trials in the dart-throwing task from distances of 300 cm and 420 cm, using two different grips. The transfer test after a 10-min. interval consisted of 40 trials with a new grip at a distance of 360 cm. The results did not support the contextual interference effect since there were no significant differences between groups on transfer. These findings suggest that the contextual interference effect may not be a global learning phenomenon which can be generalized to all learning situations.
Primary laparoscopic repair of Morgagni-Larrey hernia has been described in adult patients but not in children. This is the first report of primary laparoscopic correction in the pediatric age group without using a prosthesis. A Morgagni-Larrey hernia was found incidentally in a 3-year-old-girl. Laparoscopic correction of the defect was performed. After 6 months the patient is doing well. The chest radiograph shows complete resolution of the hernia. The laparoscopic approach allowed repair the hernia with minimal invasiveness. Laparoscopic correction is not difficult except for those hernias in which dense adhesions are present.
The authors refer to the use of the laparoscopic approach in Persistent Mullerian Duct Syndrome, starting from a case of "male vagina" with an anomaly inserted left ductus deferens inside the mullerian persistent duct. This patient was operated on using the laparoscopic technique.
Chronic idiopathic hyperphosphatasia (CIH) is a rare generalised skeletal dysplasia in childhood. The clinical, radiographic and cerebral MR findings in a 5-year-old girl with the severe infantile form of CIH are reported. In spite of cranial enlargement, the intracranial space and the skull base were markedly reduced, the whole brain was compressed and a Chiari I malformation was present. Normal flow in the dural venous sinuses was documented. The patient showed no detectable cranial nerve involvement or hydrocephalus. Cranial MR in this patient enabled us to confirm that CIH involves the skull base and vault.
The cranial MR findings in a patient with Seckel's syndrome are presented. The examination demonstrated osseous anomalies of the face, but, unlike previous reports, the brain and cerebellum were normal. The authors emphasise the importance of further reports on MR findings in patients affected by Seckel's syndrome.
Explore the source record for details and available documents.
The authors describe a case of a 5-years-old girl affected by diffuse oesophageal leiomyomatosis presenting with progressive dysphagia, cyanosis, recurrent pneumonia and retrosternal pain. She also suffered from occasional constipation due to perineal involvement by the disease and had a past history of recurrent microscopic hematuria, suggesting an association with an Alport-like syndrome. Only 24 cases of esophageal leiomyomatosis could be found in the medical literature in children aged less than 14 years, confirming the rarity of the disease. A clinical analysis of all cases reviewed from the literature is made, stressing the importance of an accurate preoperative diagnosis for the choice of proper surgical treatment to avoid recurrence.
Ectopic cervical thymus is rarely considered in the differential diagnosis of cervical masses: this lesion is essentially asymptomatic and generally occupies a position in the neck along the carotid sheath, underneath the sterno-cleido-mastoid muscle. It is supposed that most of these masses arise as a consequence of migration defects during glandular embryogenesis. Ectopic thymus rarely invades contiguous structures but in the literature some cases of malignant transformation of aberrant cervical thymus have been reported. Some non-invasive investigations (MRI, ultrasonography) are useful but accurate diagnosis depends eventually on surgical excision and histologic examination.
To identify possible differences between empty sella in children and adults we studied 43 subjects (age 13.6 +/- 5.4 years, range 4.1-27 years) with hypothalamic-pituitary disorders and empty sella at magnetic resonance imaging. Pituitary function, presence of non-endocrine symptoms, perinatal history, sellar volume, pituitary height, midline or intrasellar anatomical abnormalities were evaluated. Twenty subjects had isolated growth hormone deficiency, 17 multiple pituitary hormone deficiency and 6 puberty disorders (3 precocious puberty, 2 idiopathic delayed puberty, 1 Kallmann syndrome). The group with multiple pituitary hormone deficiency had a higher percentage of subjects with complete empty sella, i.e. pituitary height < 2 mm (p = 0.016), or intrasellar anatomical abnormalities (p = 0.0002) than the other groups. The subjects with puberty disorders had a mean sellar volume higher than the other groups (p < 0.05). Apart from pituitary dysfunction, symptoms of the empty sella syndrome were infrequent (9.3% of cases) in our subjects. The age of our subjects, the frequent association between empty sella and pituitary dwarfism and the non-enlarged sellae suggest a different aetiology, perhaps congenital, for empty sella in our subjects. As in adults, empty sella may be associated with both pituitary hypo- and hyperfunction.
Explore the source record for details and available documents.
OBJECTIVE: To evaluate clinical, hormonal, and auxologic features in a group of girls with central precocious puberty during and after long-term treatment with luteinizing hormone-releasing hormone analogue nasal spray. DESIGN: Clinical survey, before-after trial. SETTING: Pediatric Clinic, Endocrinological Center, University of Bologna (Italy). PATIENTS: Forty-one girls with central precocious puberty were treated for 28 months (range, 12 to 60 months); 25 of them discontinued therapy at a mean chronological age of 10.0 +/- 0.9 years and were followed up for 25 months (range, 6 to 50 months). Twelve patients achieved adult height. INTERVENTION: Buserelin acetate (D-Ser [TBU] LHRH A1-9EA) nasal spray; 1800 micrograms/d subdivided into six intranasal administrations of 300 micrograms each. MEASUREMENTS/MAIN RESULTS: Basal follicle-stimulating hormone, peak gonadotropin values (fluoro-immunoenzymatic method, Eurogenetics, Tessenderlo, Belgium; sensitivity was 0.5 IU/L for luteinizing hormone and 1 IU/L for follicle-stimulating hormone), and estradiol (radioimmunoassay method, DPC Kit, Los Angeles, Calif; sensitivity was 11.01 pmol/L) were significantly suppressed (P < .0001) as of the third month of treatment and increased significantly (P < .01) to pretreatment levels 6 months after discontinuation of therapy. Uterine and mean ovarian volumes, which were stable throughout treatment, appeared significantly higher (P < .05) than before treatment at only 6 months after stopping therapy. In patients with more advanced bone age (according to Greulich and Pyle) at onset of treatment, we observed a more significant improvement of SD score for height (Tanner). Mean adult height in our patients was 159.5 +/- 6.1 cm, and the variables that were significantly associated with final height were height age/bone age ratio at onset and target height (according to Tanner). CONCLUSIONS: Long-term buserelin treatment administered nasally is effective because of completeness of inhibition, quick reversibility after treatment is stopped, and lack of side effects. Its auxologic results are different depending on bone age advancement at onset, which represents a predictor of "therapeutic success." Further follow-up would be useful.
Several computed tomographic scan studies have described empty sellae in children with hypothalamic-pituitary disorders. Magnetic resonance imaging, however, is a more precise technique for visualizing the intrasellar content, such as the stalk and pituitary lobes. Using magnetic resonance imaging, we studied 339 children and adolescents (mean age +/- SD, 12.7 +/- 4.5 yr) with possible hypothalamic-pituitary disorders to ascertain the frequency of primary empty sella and examine its relationships with other intrasellar abnormalities, pituitary function, and adverse perinatal events. One hundred and ninety-three patients had isolated GH deficiency, 43 had multiple pituitary hormone deficiency, 10 had diabetes insipidus, 17 had hypogonadotropic hypogonadism, 5 had idiopathic delayed puberty, 47 had precocious puberty, and 24 had other hypothalamic pituitary disorders of hyperfunction. One tenth (10.9%) of the patients (37 cases) had empty sella, with a marked variation of incidences among the disorders listed above. A statistically higher frequency of subjects with empty sellae was found only in patients with multiple pituitary hormone deficiency. Patients with and without empty sellae were not different in regard to age or sex. The incidence of empty sella in the various groups of patients was as follows: isolated GH deficiency, 8.8% (17 cases); multiple pituitary hormone deficiency, 34.9% (15 cases); hypogonadotropic hypogonadism, 5.9% (1 case); idiopathic delayed puberty, 40% (2 cases); and precocious puberty, 4.2% (2 cases). No patients with isolated diabetes insipidus or other hypothalamic-pituitary disorders had empty sellae. In the patients with empty sellae, abnormalities of the stalk or posterior lobe were found in 1 patient with isolated GH deficiency (5.9%), 13 patients with multiple pituitary hormone deficiency (86.7%), and no patients with puberty disorders. Likewise, adverse perinatal events were found only in 1 patient with isolated GH deficiency and 9 patients with multiple pituitary hormone deficiency. These findings suggest that empty sella is not rare in children and adolescents evaluated for hypothalamic-pituitary disorders, particularly if there is multiple pituitary hormone deficiency. Empty sella can be found regardless of abnormalities of the stalk and posterior lobe, and adverse perinatal events do not seem to be the primary etiological factor. Empty sella is usually associated with pituitary hypofunction, but it can be found in patients with hyperfunction of the hypothalamic-pituitary-gonadal axis.
An epidemiological analysis of deaths occurring while taking a bath was carried out in Tochigi Prefecture. Data concerning the numbers of deaths were obtained from the Tochigi Central Police Office. Long-term assessments from 1978 to 1992 were completed for a total number of 1,348 persons (793 men and 555 women). The annual number of deaths during taking a bath increased gradually from 43 in 1978 to 140 in 1992. Death occurred 1.43 times more frequently in males than in females. The number during summer was only one eighth that of the winter peak. In addition, the increase in mortality was proportional to age. The mortality figures per 100,000 were, 10 for 40-49 age group, 31 for 50-59, 79 for 60-69, 251 for 70-79 and 469 for those 80 and over. It is concluded that the number of deaths occurring while taking a bath has increased gradually in those 80 or over.
A 67 year old woman with rheumatoid arthritis was admitted to hospital in acute renal failure. Her clinical features included increasing dyspnoea and oedema, and a computed tomogram of the abdomen showed a large mass in the retroperitoneum. Twenty six days later, she died, and a post mortem examination was carried out. The histological changes of the mass indicated B cell lymphoma of diffuse large cell type, with a reactive proliferation of erythrophagocytosing histiocytes. Immunocytochemical studies showed that the histiocytes were positive for CD-68 and lysozyme, but negative for S-100 protein. Such neoplastic B cell proliferation accompanied by activation of benign looking histiocytes with erythrophagocytosis is very rare.
The reliability of ultrasonography (US) was investigated in the diagnosis of gastroesophageal reflux in the pediatric patient. Therefore, specificity and sensitivity of US were compared with those of a 24-hour pH monitoring. A hundred and twenty-nine patients were examined; their age ranged 38 days to 14 years (56 were under and 73 over 2 years old). Since these patients presented with gastroesophageal symptoms, they underwent both pH monitoring and US. The results of the two methods were in agreement in 79.8% of the cases. Notably, in the age group ranging 0 to 2 years, US exhibited greater sensitivity (87% vs. 78.8%) and slightly lower specificity (76.5% vs. 81.1%) than in the age group ranging 2 to 14 years. The greater sensitivity in the 0-2 age group can be explained by physiological factors (which are typical of the first months of life) as well as by technical factors. Therefore, in infants, US should be combined with pH monitoring to identify the cases which are unquestionably pathological. The high specificity in the children over 2 years of age could justify the use of US alone in this group of patients for both diagnosis and follow-up, while pH monitoring might be used only in the most severe and/or complicated cases. The results suggest that, in the two age groups considered, abdominal US can be used not only as the diagnostic method of choice in the study of gastroesophageal reflux, but also as an extremely useful tool during follow-up when therapeutic monitoring is also needed.