Evaluation of ceftazidime in the treatment of 80 infectious episodes in compromised children.

The efficacy of ceftazidime in the treatment of infections in compromised children was evaluated in 80 such episodes occurring in 64 patients with various underlying diseases. Among the patients treated, 9 were newborns with severe neonatal distress, 21 were children with cancer and neutropenia, 8 were surgical patients, 22 had cystic fibrosis and 4 were suffering from meningitis. The following types of infections were treated: 19 bacteriologically documented and 8 possible septicemias (the latter only in newborns and neutropenic cancer patients); 2 severe upper respiratory tract infections in cancer patients; 8 soft tissue or skin infections; 1 cholangitis; 1 pneumonia; 1 osteomyelitis; 1 mediastinitis; 35 infectious exacerbations of underlying pulmonary disease in cystic fibrosis patients; and 4 meningitides. In almost all cases ceftazidime was administered intravenously in combination with an aminoglycoside. In 2 cases it was also given intrathecally or intraventricularly. Bacteriological documentation was achieved in 70 out of 80 episodes. A successful outcome was obtained in 79% of the cases with slight and statistically nonsignificant differences between groups of patients with different etiological patterns in terms of prevalence of gram-positive microorganisms. Tolerance of the treatment was uniformly good, only one patient showing a mild, transient transaminase elevation.

[Advantages of a permanent venous access in children treated for cancer. Preliminary results]. / Vantaggi di un accesso venoso permanente in bambini trattati per malattie oncologiche. Risultati preliminari.

Current therapy for children with cancer includes a variety of invasive procedures many of which require repeated venous access over a considerable period of time. Such procedures are poorly tolerated by children and by their veins. Recently it has become possible to undertake the majority of such procedures by means of permanent indwelling silastic catheters improving the quality of life of the children and their parents and increasing the scope of therapeutic intervention. In the period July '83 - August '84 we have used 46 of these catheters in 45 children with malignant disease, 12 with acute myeloid leukaemia, 12 with neuroblastoma, 7 with B cell leukaemia-lymphoma, 6 with rhabdomyosarcomas, 2 with Ewing's Sarcoma, 2 with Wilms' tumor and 1 case each of Hodgkin's disease, teratocarcinoma, osteosarcoma and juvenile chronic myeloid leukaemia. The children's ages ranged from 2 months to 14 years; 22 were male and 23 female. The catheters were inserted under general anaesthesia (duration 20-40 minutes) usually without difficulty, except for a single patient in whom no suitable vein could be found. No complications connected with the placement of the catheter were observed. Subsequent management of the catheter was initially complicated and time-consuming, but was subsequently simplified so that acceptance by parents, children and nursing staff was eventually excellent. The duration of use of 46 catheters ranges from 7 to 350+ days; 24 catheters are presently in use at 30-350+ days from insertion. Eight children died as a result of disease progression and two of sepsis with the catheter in place.(ABSTRACT TRUNCATED AT 250 WORDS)

Multiple endocrine neoplasia type 2b (MEN 2b). Report of 2 pediatric cases.

Two new sporadic cases of multiple endocrine neoplasia type 2b (MEN 2b) are described. Both patients were diagnosed in pediatric age and presented the characteristic features of the syndrome (facies "sui generis", Marfanoid habitus, mucosal neuromas, history of chronic gastrointestinal disturbances) and developed medullary thyroid carcinoma (MTC). In the former case, metastatic neck adenopathies were the first sign by which the disease was recognized. Antitumor treatment consisted of total thyroidectomy, cervical node dissection, administration of I131 and neck irradiation. This patient is alive and well 20 months from diagnosis, still having high serum levels of thyrocalcitonin (TC). In the latter case, the syndrome was diagnosed on clinical grounds before the development of a MTC. The patient was then strictly followed-up and thyroidectomy performed only when serum TC levels rose to abnormally high values: no tumor spread was documented at that time. She is alive and well 4 years from diagnosis.--Early recognition of MEN 2b syndrome is necessary in order to detect and properly treat MTC.

[Ewing's sarcoma. Results of treatment in 16 consecutive cases]. / Il sarcoma di Ewing. Risultati del trattamento in 16 casi consecutivi.

In the period January 1974-August 1981, 16 previously untreated cases of Ewing's Sarcoma have been diagnosed at the Giannina Gaslini Children's Hospital Genova. Eight were male, eight female. Median age at diagnosis was 11 years. Two patients presented with a unique metastatic lesion, in the right lung and in an illiac lymph node, respectively. Fourteen patients have been initially treated with local radiotherapy (dosages ranging form 4,800 to 6,600 rads) in association with antiblastic polichemotherapy utilizing 4 drugs (Adriamycin, Actinomycin D, Vincristine, Cyclophosphamide). The Rosen et al.'s T-2 protocol was adopted, modifying the initial phase in order to give more weight to Adriamycin and reduce the toxic effects related to radio-chemotherapy combination. Two patients bearing a costal primary were immediately treated with a more complex and aggressive chemotherapy (T-6 Protocol), followed by local irradiation (in one case preceded by surgical ablation) and then chemotherapy again (T-2 protocol, second phase) for 10 months. Treatment determined a fast subjective relief in the 13 symptomatic patients. All 16 cases achieved a status of complete remission. Four of them subsequently relapsed: locally in two, in distant sites in the remaining 2. All 4 died 12-27 months form diagnosis. Twelve patients are presently alive without evidence of disease at 3-92 months (median 37 months) following diagnosis. Treatment has caused early and delayed toxicity in all cases. However, the entity of these complications varied considerably from one patient to an other. Age at diagnosis and site of primary tumor were the factors most relevant in this respect.

[Cystic lymphangioma of the spleen (author's transl)]. / Linfoangioma cistico della milza.

The spleen is the least common intra-abdominal site for the development of cystic disease. A lymphoangioma of the spleen was first reported in 1885 by Fink; since that time 47 cases of lymphoangioma or lymphoangectasia have been reported. This report concern a case of cystic lymphoangioma of the spleen consisting of a large splenic cyst with associated multiple small subcapsular cysts and lymphangectasia. The diagnosis and treatment of splenic cysts are discussed and a new classification of splenic cysts is proposed.

[Diminished in vitro colony forming capacity of bone marrow cells in a case of chromosome 8 trisomy (mosaicism): criteria for "high risk" pre-leukemia syndrome]. / Diminuita capacita' clonate in vitro dele cellule midollari in un caso di trisomia dell'otto (mosaico): criterio di "alto rischio" ? sindrome pre-leucemica?

A case has been described of Trisomy 8 mosaicism Syndrome. At onset the child presented with hyporegenerative anemia; the study of colony forming capacity in vitro (CFU) by Bone Marrow (B) and Peripheral Blood (PB) showed an abnormal colony formation by myeloid and erythroid progenitor cells. No immunological defects were discovered. The in vitro colony formation appears to have a definite role in the identification of patients who may be at higher risk of developing leukemia. The importance of 8 chromosome for hematopoiesis control is discussed.