Guillain-Barré syndrome in childhood. Characteristic course and therapeutic possibilities]

The incidence of the GBS in childhood is significantly lower than in adults. The clinical course may be equally severe, 10-25% of the affected children requiring artificial ventilation. Besides respiratory insufficiency, cardial arrhythmias are a major threat to the patients' lives. However, eventually more than 90% of the children recover completely. Controlled studies on the efficacy of therapeutic measures have been performed only in adults. To children their results should be transferred with caution. Steroids are of no value in the acute disease. However, they should be tried when progression for more than 4 weeks suggests the diagnosis of CIDP. Plasmapheresis is beneficial in CIDP and severe GBS; in GBS the time for weaning from the respirator and for achieving independent ambulation is significantly reduced. Recent studies have demonstrated that intravenous immunoglobulins can be equally effective. However, the administration of immunoglobulins in less severe cases should at present be reserved to a controlled study.     

Cytokines and their receptors networks: biology and therapeutic possibilities

Cytokinins are biological proteins which permit the transmission of signals from one cell to another. These proteins are secreted by groups of specialized cells and are fixed on specific receptors on the surface of responding cells. However, since receptors may be widely distributed, and since the activation of a cell usually permits the release of other cytokinins and a succession of reactions, cytokinins have pleiotropic effects. Cloning of the genes encoding for these cytokinins and their obtention on a large scale by genetic recombination techniques have allowed a better understanding of their biological characteristics and their use in therapy. This cytokinin network is particularly wide in the hematopoietic and in the immune systems. In the present study, we describe the biological properties of the main interleukins and hematopoietic cell growth factors, together with their therapeutic applications, particularly in hemato-oncology.     

Non-epileptic sleep disorders (somnambulism) in epilepsy. Diagnostic and therapeutic possibilities]

The case history of a 15 1/2-year-old boy is presented who suffers from screaming fits during the night and epilepsia. The problem has existed since he was eight. He lives alone with his mother in a "partner-like" relationship. The previous diagnosis, namely epilepsy, has masked any possible psychodynamic element. Our diagnostic instruments were: 1. Standardized diagnostics with DSM III-R; 2. psychoanalytically oriented psychodiagnostics; 3. long term EEG-video-monitoring, which eventually succeeded in differentiating his multiple symptoms. Using these methods we were able to differentiate a sleep disorder (somnambulism) from his grandmal epilepsy. We changed his anticonvulsive pharmacological therapy and introduced an individual psychotherapy ("Katathymes Bilderleben"). This kind of psychotherapy is applied for the first time as a therapy for somnambulism. By using this therapeutic concept we cured our patient from his symptoms.     

Giant pigmented nevi: Clinical, histopathologic, and therapeutic considerations

Eighty pediatric patients with giant pigmented nevi more than 20 cm in their greatest diameter are reported. The incidence was 1 in 4150 general pediatric outpatients. The mode of inheritance of giant pigmented nevi is probably multifactorial; four second-degree relatives of our patients also had large nevi, and there was a 2:1 female predominance. Satellite nevi were present in 74% and nevi in mucous membranes in 31% of the patients. Eighty-six percent of nevi were pigmented and hairy. Benign nodules were observed in 19% of the patients and plexiform overgrowths in 6%. Nevi extensively involving the extremities resulted in reduced growth of the affected limb. Electroencephalograms showed abnormalities in 20% of the patients with giant pigmented nevi involving the head and upper portion of the trunk. Malignant transformation appeared in four patients and was fatal in three of them. Management consisted of observation only in 49%, surgery in 27.5%, chemical peel in 21%, and dermabrasion in 2.5% of the patients. The mean follow-up was 4.7 years.     

Congenital nasolacrimal duct obstruction: therapeutic management

PURPOSE: Congenital nasolacrimal duct obstruction is a common condition, and its diagnosis must be based more on regular in-time epiphora than mattering. The purpose of this study is to better define the diagnostic criteria and therapeutic strategy. METHODS: We have retrospectively evaluated 1563 subjects treated from 1990-1997 at the Pediatric Ophthalmological Service of Modena University Eye Clinic. The mean age at first examination was 5.7 months. According to symptoms and age, patients were treated with antibiotic therapy, office probing, or general anesthesia probing. Recovery was confirmed only after a 3-month symptom-free period. RESULTS: More than 29% of patients recovered spontaneously or with topical antibiotic therapy. Office probing reduced the number of patients who needed a general anesthesia probing (from 62.97% from 1990-1993 to 33.61% from 1994-1997). In the period from 1994-1997, we changed the technique and especially the age of patients, obtaining a clearing of the obstruction in about 86% of cases compared with a 39% rate in the earlier period. Since 1994, in cases of general anesthesia probing, we have used a midazolam and ketamine anesthesiologic technique that has reduced risks and increased parental satisfaction and cost-effectiveness. Also, our data show that age at first attempt highly influenced probing failure rate. CONCLUSION: If the first ophthalmologic examination is precocious and correctly timed, the possibility of curing the patient using the simplest method is increased. Better results can be obtained with cooperation among pediatricians, ophthalmologists, and anesthesiologists.     

Cerebral palsy, new therapeutic possibilities

OBJECTIVE: To provide pediatrician with updated information about diagnoses and treatment of cerebral palsy. This articles aims at supplying pediatricians with tools that will help them diagnose and treat cerebral palsy. SOURCES: Non-systematic review of literature combined with personal experience at the Neurology Unit of Pediatrics Service of Hospital de Clínicas de Porto Alegre - Universidade Federal do Rio Grande do Sul. SUMMARY OF THE FINDINGS: The cerebral palsy diagnosis and treatment are based on multidisciplinary clinical exam, EEG, CT and MR. CONCLUSIONS: Pediatricians are the first physician that see the patient with cerebral palsy. Thus, they should be able to diagnose an treat it.     

Communication in the trisomy 21 patient: observation and therapeutic possibilities

The behaviour of 4- to 6-year-old children with Down syndrome was studied by audio-visual means during individual or group stimulation sessions. From these observations, it is concluded that children with Down syndrome have a rich preverbal communication, but poor child-to-child interchanges. Thus, when taking care of children with Down syndrome, it is necessary to accentuate individual stimulation and take into account the affective drive of the child towards the adult.     

Congenital lobar emphysema: Differential diagnosis and therapeutic approach

Background: Congenital lobar emphysema (CLE) is a rare anomaly of lung development that usually presents in the neonatal period with respirator distress and pulmonary lobar hyperinflation. It is commonly confused with pneumothorax. The aim of the present paper was to review the authors’ experience in order to emphasize the importance of differential diagnosis with pneumothorax. Methods: Children with CLE treatment at Department of Thoracic Surgery, Dicle University School of Medicine, Turkey, between January 1993 and June 2004, were reviewed. Results: Ten children consisting of six boys and four girls (age range, 6 h–12 months) had CLE. Major presenting symptoms were tachypnea(n = 100%) and respiratory distress in (n = 80%). On chest radiograph, emphysema was seen in all patients, and shift‐herniation to the opposite lung, atelectasis were observed. Computed tomography was performed in all patients, which indicated emphysema in the affected lobes in all cases. Pulmonary perfusion scan was performed in two patients, showing loss of perfusion in the affected lobe. The most common affected lobe was the left upper lobe (50%). In the present series, three patients were mistakenly diagnosed as pneumothorax and intercostal drains were inserted in the emergency department. Eight patients underwent lobectomy, and postoperative course was uneventful. Two patients were followed conservatively. Emphysema was detected in all pathological specimens. One patient was lost to follow up. Mean follow‐up duration of all patients was 26.8 ± 29.24 months (range, 1–89 months). Conclusions: CLE is established on combined clinical, radiological and scintigraphic imaging. Surgical excision of the affected lobe is the appropriate treatment. Particularly, differential diagnosis should be made between CLE and pneumothorax.     

Congenital cystic lung disease: diagnostic and therapeutic considerations

Congenital lung cysts such as congenital cystic adenomatoid malformation, pulmonary sequestration, congenital lobar emphysema, and bronchogenic cysts are rare but fascinating anomalies of lung development. While there are many similarities in terms of their presenting features, there are particular differences between the diagnostic groups that are important to highlight, especially in relationship to approaches to imaging and long-term outcome. A case of each entity is presented with an emphasis on the contemporary approach to diagnostic investigations and therapeutic options.