[Disseminated intravascular coagulation in chickenpox. Report of a case in a child]. / CID in corso di varicella. Descrizione di un caso in un bambino.

The case of a healthy and immunocompetent five-year-old boy, who developed a disseminated intravascular coagulation during chickenpox is described. Disseminated intravascular coagulation manifestations were extremely severe and included macroscopic hematuria, necrotic purpura and cerebrovascular thrombosis. The outcome in this patient was a complete recovery. Nevertheless, the possibility of a seriously complicated course of chickenpox even in low-risk children subgroups suggests that the Varicella-Zoster virus infection should not be underestimated. More accurate information about the impact of chickenpox and its complications on the population is needed, in order to provide a contribution for the debate about the costs associated with this disease and the potential benefits of both the early antiviral therapy and the vaccinal prophylaxis.

[Rapid recovery from optic papillitis secondary to oto-mastoiditis in pediatric age]. / Rapida guarigione di una papillite ottica secondaria ad otomastoidite in paziente pediatrico.

In this paper we report the case of an acute papillitis of the right optic nerve secundary to a mastoiditis and sinusitis of the same site in a 10-year old boy. At onset the child presented a painful movement of the ocular globe, monolateral amaurosis and papillary oedema. The exams have confirmed the correlation among mastoiditis, sinusitis and papillitis. At the same time, we were able to exclude the presence of endocranial tumours and alteration of the other side. The child underwent a steroid therapy with a complete recovery within 30 days.

[The follow-up of a child with a syndrome due to partial deletion of chromosome 3 (p25-pter)]. / Follow-up di un bambino affetto da sindrome da delezione parziale del cromosoma 3 (p25-pter).

A case of del(3) p25-pter syndrome in a four-year-old boy whose clinical manifestations were followed and studied since birth, is described. Diagnosis was made by means of karyotype analysis. The parental chromosomes were normal. So far, only about twenty cases of this syndrome have been described in living individuals. Comparison with previously reported cases confirms that the phenotype exhibits an identifiable pattern of malformation, consisting of pre- and postnatal growth delay, typical craniofacial dysmorphisms and limb abnormalities. No severe visceral anomalies were detected in this patient. Nevertheless, the follow-up revealed a progressive decay of the psychomotor and neurosensory functions.

[Orbital cellulitis. A case report]. / Cellulite orbitaria. Descrizione di un caso clinico.

This study describes the case of a 6 years old child, male, with orbital cellulitis and underlines the importance of an early diagnosis and therapy to avoid severe complications often present in this disease. Swelling and redness of the eyelid, pain and ophthalmoplegia are the first sign of an orbital cellulitis and they require rapid diagnostic procedure such as ultrasound and TC scan of the orbital region to evaluate the integrity of the profound orbital tissues. The child was admitted at the Department of Pediatrics, University "La Sapienza" of Rome and underwent an ultrasound, TC scan and serum exams which demonstrated the elevation of the sedimentation rate, reactive C protein and WBC plus the interesting of the profound orbital tissues. The child was treated with antibiotic and antiinflammatory therapy showing a complete recovery within 7 days. An ultrasound performed 7 days later demonstrated a complete resolution of the inflammatory process. In summary, this study would like to stress the necessity of an early diagnosis and an appropriate therapy in order to avoid the severe complications often present in children with orbital cellulitis.

Liddle's syndrome: a 14-year follow-up of the youngest diagnosed case.

The 14-year follow-up of a female patient with Liddle's syndrome (LS), a rare disease characterized by hypertension, hypokalemic alkalosis, and negligible aldosterone secretion due to renin suppression, is described. The disease was diagnosed at the age of 10 months (youngest identification). The patient was repeatedly investigated during follow-up for plasma renin activity (PRA), plasma aldosterone concentration (PA), serum sodium and potassium (K) concentration, blood pressure (BP), somatic anthropometry, and mental development. Noteworthy results included: persistent low circulating K, PRA, and PA and high BP, coinciding with unauthorized withdrawal of the triamterene therapy. These findings are in keeping with the hypothesis that LS results from a pathogenetic disorder which is not correctable with age. The triamterene therapy was effective in correcting the endocrine and metabolic disorders as well as arterial hypertension, but did not prevent a deficit in mental and physical development. However, the information derived from this study allows further clarification of the clinical picture of the disease.

[Colles' fracture in a girl after fulguration]. / Frattura di Colles in una bambina in seguito a folgorazione.

In the present study we describe the case of a 11 years old child, female, who was accidentally fulgurated by the left arm and after has presented an episode of pallor, shaking, confusion, throbbing and tingle of the arms. For this reason she was admitted in our Department of Pediatrics, University of Rome "La Sapienza". The child presented also a moderate pain at the left wrist. For this reason was performed a wrist X Ray which showed an incomplete fracture of the distal extremity of the radial diaphysis, with a small ulnar infraction of the same side. Life parameters and the ECG were normal so as the blood exams, in particular the CPK and the LDH (in fact, they can be indication of muscular necrosis). In summary the Authors with this study would like to remark the necessity of to suspect a fracture in the place of admittance of the electric current, even if not immediately appear clean signs of oedema, pain and functional impotence.

[Pseudotumor cerebri: a pediatric case report]. / Pseudotumor cerebri: descrizione di un caso pediatrico.

We describe a case of pseudotumor cerebri (PTC) in a five years old child. Pseudotumor cerebri is a not very common syndrome in the pediatric age. The incidence rate in about 1:100.000. The prognosis of PTC is usually good. Sometimes however, PTC could be complicated by eye diseases such as reduction of visual field. Our case is a child with diplopia and with a bilateral oedema of the papilla. The diagnosis of PTC was possible after the physical examination and the instrumental exams (EEG, brain NMR and others). During the staying in the hospital the child performed a therapy with betamethasone and acetazolamide; the diplopia improved and the bilateral oedema of the papilla started to be less evident. The child was discharged in good health and with a planned ocular follow-up. We think that the Pediatrician must considered the possibility of the presence of a PTC; that is an important syndrome not only for the Neurologist.

[Anterior scalenus syndrome in pediatric age. Report of a case]. / Sindrome dello scaleno anteriore in età pediatrica. Descrizione di un caso.

This paper aims to describe a case of a twelve-year-old girl affected by "scalenus syndrome" and who, at its outbreak, suffered great pain, cyanosis hyperhydrosis in the affected arm. The patient's follow-up took place at the Children's Hospital of the University of Rome "La Sapienza". The diagnosis, already clear from the anamnesis as well as from both the onset of the symptoms and the general conditions of the patient herself, was then confirmed by a local MR-angiography (MRA), the only test that gave us a positive result. No medical treatment was given the girl; she was only recommended to rest and avoid putting too much weight on the scapular girdle. The outcome of our study is mainly represented by the singling out and recognition of a syndrome which on the one hand is rare, but on the other is gaining more and more social significance, especially because of the so frequent use of often too heavy school bags, weighing on the scapular girdle. The description of this case, therefore, aims also to awake not only medical people, but also teachers and parents to the problem.

[Left pneumonectomy in a child with congenital bronchiectasis]. / Pneumonectomia sinistra in un bambino affetto da bronchiectasie congenite.

A case of diffuse bronchiectasis concerning an entire lung in an eight-year-old boy is described. The disease in this case was widespread in the entire left lung and was considered as consequent to a defect in the development of the left bronchial wall. Because of the frequent respiratory infections, occurring since birth, and given the good anatomical and functional conditions of the right lung, we recommended the surgical excision of the sick lung. After one year, the operation showed good results with regard to respiratory functions. The functioning of the remaining pulmonary parenchyma is quite satisfactory and allows the boy an excellent quality of life.

[The interpretation of a negative intradermal reaction to tuberculin in a case of tuberculous lymphadenitis]. / Interpretazione di una intradermoreazione alla tubercolina negativa in un caso di linfadenite tubercolare.

This work aims at describing a particular case of tuberculous lymphadenitis with negative tuberculin skin test in a five-year child who has afterwards developed Hodgkin disease. BK presence is initially demonstrated through the examination of a first bioptic sample of a lymph-node, then confirmed by the presence in the serum of mycobacterial antigens on the peripheral lymphocytes and monocytes. Nine months later the second bioptic sample puts in evidence the presence of Reed-Sternberg cells. The follow-up of our patient took place at the Pediatric Department of the University of Rome "La Sapienza" and lasted an overall period of twenty days. The child was hospitalized twice and underwent a large number of radiological and laboratory tests. The therapy consisted only of medical specific treatment. Our study results lead us to evaluate the presence of immunological suppressive mechanisms in some particular tuberculosis cases, justifying the tuberculin skin-test negativity. When Hodgkin's disease has turned up, it has been too difficult to outline a precise temporal sequence of events, we could only advance hypothesis.

[Budd-Chiari syndrome in children: case report]. / Sindrome di Budd-Chiari in età pediatrica. Descrizione di un caso.

This work aims at describing a particular case of Budd-Chiari syndrome in a ten-year-old girl. In our patient, the subjective and objective features of this disease, which is already rare during childhood, are associated with a well-assessed polyposis in the blind intestine (intestinum caecum). In our opinion, a possible connection between these two pathological events, a connection unheard of in the literature so far, is an interesting issue to be dealt with in this case report. The follow-up of our patient took place at the Pediatric Hospital of the University of Rome "La Sapienza" and lasted an overall period of 6 months. The girl was hospitalized twice and she underwent a large number of radiological and laboratory tests. The therapy consisted only of medical treatment and support measures. Liver transplant is regarded as the only chance to obtain a full recovery, but it has not yet been performed on our patient. It is by comparing those researches carried out on similar cases with the outcome of our personal study, that we come to the conclusion that, as for our patient, the Budd-Chiari syndrome can be classified as an idiopathic syndrome. Moreover, the fact that this syndrome and the polyposis of caecum occur at the same time in a patient can probably be considered as a coincidence.

Cerebral infarction in a child. A case report.

Cerebral infarcts in children are rather rare and in most cases no precise etiology is established. The authors describe a case of cryptogenetic cerebral infarction in a 9-year-old boy. The child presented an acute onset of hemiplegia in the right arm and leg, central facial palsy, dysarthria and steppage. The infarction was proved by Computed Tomography (CT) and Magnetic Resonance Imaging (MRI). Laboratory and instrumental studies rule out all known causes of brain infarction. The only possible etiopathogenetic hypothesis was a varicella arteritis which occurred 45 days before the clinical manifestation.

Acute hepatitis in childhood: virological, immunological and clinical aspects.

Virological, immunological and clinical findings in 7 previously healthy children, aged 18 months to 11 years, with viral hepatitis are reported. Asymptomatic and fully recovering, although protracted, hepatitis B was diagnosed by chance in a 1 1/2 year-old boy. Anicteric and short-term hepatitis occurred in three children with Epstein-Barr virus infection, concomitantly with typical mononucleosis syndrome. On the contrary, cytomegalovirus (CMV)-associated hepatitis was severe and protracted in two children, and fatal in a 4-year-old girl, whose main autoptic finding was submassive hepatic necrosis. Therefore, our study showed that acute viral hepatitis in non-immunocompromised children is generally self-limited and that CMV hepatitis is more frequent and severe than commonly believed.

Role of genetic variability in neonatal jaundice. A prospective study on full-term, blood group-compatible infants.

A series of genetic, developmental and environmental variables have been analyzed in a prospective sample of full-term newborn babies, compatible with their mothers in the major blood group systems, in order to attempt an evaluation of the effect of these variables on serum bilirubin level during the first few days of life. Three genetic factors (PGM1, ACP1 and ADA) and three non-genetic variables (rise of bilirubin level during the first day of life, a mother with a history of previous abortion, and use of alcoholic beverages by the mother) have a significant predictive value for the separation of newborns with clinically relevant jaundice from other infants.

[The efficacy of cyclophosphamide bolus therapy in a girl with SLE with a nephritic onset]. / Efficacia della terapia con ciclofosfamide in boli in una bambina affetta da LES ad esordio nefritico.

A case of glomerulonephritis as the initial clinical manifestation of SLE in a child is reported. Treatment with oral prednisone did not produce beneficial results either worth respect to the symptoms or the laboratory data. The association with intravenous Cyclophosphamide led to rapid improvement in clinical and serological patterns. Moreover this association has reduced the risk of end-stage renal failure with few serious complications, and no other side-effects.

[Usefulness of long-term monitoring of neonatal arterial pressure]. / Utilità del monitoraggio, a lungo termine, della pressione arteriosa neonatale.

30 newborns were studied, whose systolic arterial pressure has been controlled every 30 minutes 48 hours long, with oscillometric automatic technique. The resulting average of oscillation was 45.1 +/- 12.8 mmHg for systolic pressure and 36 +/- 11.4 mmHg for diastolic pressure. Wide pressure oscillations, you can find in some newborns, could be related with the development of a future pressure hypertension, and therefore a long term pressure monitoring would turn to a real useful test to single out future hypertensive subjects.

[A case of juvenile rheumatoid arthritis treated with thymopentin]. / Un caso di artrite reumatoide infantile (ARI) trattato con timopentina.

The case of a ten year old girl with JRA presenting a systemic onset and resistant to non-steroid therapy is described. After one course of thymopentin the patient responded satisfactorily. After ten months from the beginning of treatment and a third course she is still doing well. The authors comment the modalities of therapy.

Chronobiology of blood pressure in childhood: implications for tracking.

Chronobiology, in its methodological evolution, developed data series analyses paying particular attention to blood pressure (BP), because of the importance of this biorhythmic variable for assessing the risk of developing hypertension. An example of the potentiality of the chronobiologic procedures is given in the present report which deals with the inferential analysis of the BP 24-h patterns in 3-12 year-old children. By using the chronobiologic methodology, time-qualified standards for BP are calculated. Rhythmometric parameters for circadian rhythm of systolic and diastolic components of BP are also computed. Data presented are a tangible outcome for emphasizing the introduction of chronobiology in epidemiology and pediatrics in order to optimize the primary prevention and care of hypertension taking as reference the chronobiologic standards of BP.