[Castleman's disease in childhood: a case report]. / Malattia di castleman in età infantile: case report.

We report a case of Castleman's disease in 3-month old infant with cervical swelling, treated with surgical excision. This case is interesting because of the rarity of this disease in childhood, of the cervical localization and of the complexity of long- term follow-up.

[Proposal for a protocol for the staging of incontinentia pigmenti in pediatric age]. / Proposta di un protocollo per la stadiazione della Incontinentia Pigmenti in età pediatrica.

Incontinentia Pigmenti (IP) is an X-linked dominant disorder of skin with neurologic and ophthalmologic involvement. IP predominantly affects females because the mutations are usually lethal in males in utero. IP is characterized by abnormalities of neuroectodermal tissues. IP is caused by mutations in a gene called NEMO, which is required to activate the NF-kB pathway. We present a diagnostic protocol for IP and a meta-analysis of the clinical spectrum of IP in 82 patients cited by MEDLINE in the European literature from 2000 to 2006.

[Incontinentia pigmenti: case report]. / Incontinentia pigmenti: descrizione di un caso clinico.

IP is an uncommon X-linked dominant disorder (incidence: 1/40.000 newborn). It is caused by mutations in NEMO. It is characterized by cutaneous lesions and dental, ocular, neurologic, nails, hair disorders. The ocular and neurologic sequelae represent the major morbidity in IP. We present a case-report with classical cutaneous features diagnostic for IP. The clinical, ophtalomologic and neurologic examinations revealed no other pathological manifestations.

[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.

An unusual case of non-Langerhans cell histiocytosis.

A 28-month-old boy had firm, red nodules for 4 months that were mostly localized to the face. The eruption was preceded by conjunctivitis, eyelid edema, and swelling of the root of the nose. He also had dyspnea, anisocoria with areflexia, swelling of the parotid glands, and hepatosplenomegaly. A bone marrow aspirate showed 25% eosinophils. A skin biopsy specimen revealed a lymphohistiocytic infiltrate that involved the entire dermis. Most of the cells expressed antimacrophage markers; S-100 was negative. Electron microscopy showed poorly differentiated histiocytes without any specific marker. Steroid therapy induced complete clearing. Two years later, after several cutaneous recurrences, the patient is free of disease. We believe that this case represents a nodular, benign non-Langerhans cell histiocytosis with cutaneous lesions that differ from previously described histiocytopathies.

[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.

[Meningococcal sepsis and DIC in childhood: a report of a clinical case]. / Sepsi meningococcica e C.I.D. in età pediatrica: discussione di un caso clinico.

Disseminated intravascular coagulation (DIC) is a frequent complication of meningococcal sepsis in children. The clinical course variability, the severity of manifestations and the need of an early diagnosis for appropriate treatment, guides us to report a case of meningococcal sepsis and DIC. The patient, male, prematurely born, 11 months years old, presented himself with high fever of sudden onset, malaise, diarrhea, diffuse skin rash with abdominal petechiae, and no clinical evidence of meningitidis. Initial hematochemical findings, peripheral leukocytosis, quantitative and qualitative changes in plasma coagulation factors, liquoral hypocellularity together with the development of signs of meningeal irritation (stiff neck and back) were considered diagnostic clues for meningococcal sepsis associated with DIC. A gram-negative diplococcus was cultured from liquor. Primary goals of the treatment of this life-threatening clinical picture were the elimination of the bacterial component, the correction of clotting disorders and careful control of shock and metabolic acidosis often related with DIC. The patient then received a wide spectrum Cephalosporin, fresh frozen plasma, appropriate electrolyte solutions and eventually heparin, which led to a complete control and resolution of symptomatology.

[Induction of general anesthesia with ketamine for short surgical interventions in pediatric patients with chronic hepatic pathologies]. / L'induzione dell'anestesia generale con ketamina per brevi interventi chirurgici in pazienti pediatrici affetti da patologie epatiche croniche.

Serum levels of bilirubin and of other four enzymes known to be elevated in liver diseases were measured preoperatively and controlled on the first and fourth postoperative day in 14 children with chronic liver disease and anesthetised for liver biopsy using Ketamine (7 patients, group A) or other drugs (group B). Serum levels of gamma GT, Ph. Alk, direct and indirect bilirubin show neither significant variations nor differences between the two groups of patients. SGOT and SGPT basal levels were similar in the two groups and were rather two-fold the normal values. At the first postoperative day, in group B only SGOT levels increased significantly. Moreover, their increase over the basal levels monitored at the first day was significantly lower in the group of patients anesthetised with Ketamine than in group B. In group A, SGPT values were constant showing a small, non significant decrease, while in group B, above levels present a significant increase at the postoperative day and a significant difference between the two groups. At the fourth day, SGOT and SGPT differences versus basal values were not significant. Our experience shows that - as known by normal subjects - Ketamine has no hepatic toxicity in pediatrics patients affected by chronic liver disease, too. Finally, our study suggests that Ketamine must be used in hepatopath children whenever they have to be submitted to total anesthesia.

[Effectiveness of piperacillin in childhood infections]. / Efficacia della piperacillina nelle infezioni dell'età pediatrica.

Piperacillin is a semisynthetic penicillin with a broad-spectrum: in particular it is active against Gram positive, Gram negative, aerobic and anaerobic bacteria often resistant to other antibiotics of the same group. We report a trial carried out on 38 children with severe and moderate infections treated with piperacillin. We have considered ESR, all leucocytes count and body temperature. No severe side effects have been reported. Piperacillin must be used as a first choice in pediatric age, mostly in case of mixed bacterial infections and in immunocompromised patients.

[Long-term development of chronic hepatitis caused by virus B in childhood]. / L'evoluzione a distanza dell'epatite cronica da virus B in età pediatrica.

Ten children with chronic hepatitis B have been followed for at least 3 years. All patients received 2 liver biopsies, the first after 1 year of continuous liver disfunction and HBsAg positivity, and the second 2 years after, that showed a mild improvement of the histologic lesions in 4 cases of chronic persistent hepatitis (CPH) and in 1 case of chronic lobular hepatitis (CLH). Three children out of 5 with chronic active hepatitis (CAN) changed to CPH and the other 2 remained unchanged without any evidence of cirrhosis. No patient received any drug for the treatment of hepatitis.

[Sylvatic rabies: a pediatric problem?]. / La rabbia silvestre: un problema pediatrico?

Rabies epizooty has been recently reported from several Italian provinces: Bolzano, Udine, Belluno, Brescia, sondrio, Varese, Como and Bergamo. The rate of spread of the infection through wild animals has been estimated in 50 Km/year. We have reviewed the fundamental characters or rabies virus, the pathogenesis and clinical manifestations of disease. Furthermore the fundamental guidelines of passive and active immunization will be stressed with spread emphasis to the pre-exposition active immunization in children-living in high risk areas.

[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.

[Intra-familial transmission of HBV infection]. / La diffusione intrafamiliare dell'infezione da HBV.

We investigated 63 household contacts of 29 children positive for serologic markers of hepatitis B virus, in order to assess the intrafamiliar spread of HBV infection. 40 relatives of 15 children affected by chronic hepatitis B (Group I) and 23 relatives of 14 patients affected by hemophilia (Group II) were studied. HBV markers (HBsAg, anti-HBs, anti-HBc, HBeAg, anti-HBe) were tested in all the subjects by RIA. Positive markers were found in 39 (62%) of the subjects; the combined prevalence for all HBV markers was 75% in the Group I, and 39% in the Group II. The higher prevalence rate in Group I can be explained by the possible previous infection of another member of the family, whereas in Group II the child is almost surely the first infected subject. Besides, subjects of Group I have probably been in contact with HBV infected children a longer time, compared to subjects in Group II. Our data suggest that household contacts of children with HBV infection are at high risk and should therefore be immunized against HBV.

[Evaluation of the nutritional status and growth of children with chronic HBsAg-positive hepatitis]. / Valutazione dello stato nutrizionale e dell'accrescimento in bambini con epatite cronica HBsAg positiva.

Chronic hepatitis in children seems to have a better prognosis than in adults, with a discrete trend to spontaneous remission. Actually biopsy and liver enzymes are mandatory for a correct follow up of the disease, while few authors are interested in the assessment of growth and nutritional status. We think that an auxological approach is one of the main points in every chronic disease of the pediatric age, chiefly when some anabolic step might be affected. Our first result in a cross sectional study of growth and nutritional status in a selected group of untreated HBsAg+ chronic hepatitis children are as follows. According to enzyme values in the range of 16-171 UI/1 SGOT all patients are growing very well with a good-discrete nutritional status. Though anthropometric proteic nutritional status (muscle circumference) was in the range of normal distribution, we were able to show a moderate correlation between SGOT and muscle circumference (r = -0,50). Our impression is that some patients with a worse hepatic damage can be exposed at risk of proteic malnutrition which is a negative element in the prognosis of every chronic disease. Anthropometric auxology can detect degrees of proteic malnutrition, and can follow its development. So we recommend routine auxological assessment in all pediatric patients with chronic hepatitis.

[Biliary atresia: pathogenetic considerations emerging from the histopathologic analysis of the liver parenchyma]. / L'atresia biliare: considerazioni patogenetiche emergenti dall'analisi istopatologica del parenchima epatico.

Nine patients had been surgically treated for extrahepatic biliary atresia from 1974 to 1982. The analysis of the hepatic specimens obtained during the surgical exploration revealed a wide spectrum of histopathologic lesions. In 3 patients we found severe Giant-cell transformation, while in the other 6 it was not possible to ascribe the histopathologic picture to a univocal pathogenesis. Comparing our results to the reports of other Authors we believe biliary atresia can not be explained by a single pathologic mechanism. The obstruction of the extrahepatic bile ducts might be the common morphologic denominator of different pathologic entities, from each of them it depends a different evolution of the hepatic lesions and a different survival after Kasai's operation.

["Follow-up milk": general principles and evaluation of the nutritional status of 100 subjects fed a liquid transitional formula]. / Latti di seguito: principi generali e valutazione dello stato nutrizionale in 100 soggetti alimentati con una formula di transizione liquida.

Many authors have pointed that precocious weaning expose infants to serious risks as hypernutrition, obesity, adverse reactions to foods, hypernatremia, dental caries, emotional problems as anorexia or bulimia, so that actually weaning is delayed after 6th month of age. Going on with the "adapted" formula is a relative nonsense because "adapted" milks have low protein and calcium contents so that they are not adequate to cover estimated and advisable intakes of 4-6 month baby unless feeding unusual higher volumes. On the other side "fresh milk" can not be considered a nutritional "chance", owing its low values of EFA, iron, vitamins, getting worse when fresh milk is diluted. So, recently was born a new milk formula "the follow up milk", on covering nutritional requirement for infants after 4 months of age. Someone is still critical about a follow up milk, also if ESPGAN in 1981 has confirmed its value in the infant feeding. Our work dealed on physical and biochemical nutritional assessment of 100 infants fed a new "liquid follow up formula" (Transilat). Nutritional assessment was performed with the following parameters: daily changes in weight according Fomon standards, plasmatic iron, cholesterol, transferrin, calcium, total proteins, hemoglobin concentration; all data are related to literature values for age. Results show that infants fed (Transilat) are growing well; nutritional data from biochemical point of view discovered any form of minimal or sporadic malnutrition. The follow up milk is a good nutritional "chance" after 4th month of age, instead of fresh cow milk; some infant with clinical problem needing a delayed introduction of cow milk can benefit of follow up milk also in older ages.