Protein S deficiency. Description of a case associated with chronic inflammatory bowel disease.

Thrombotic disease is one of the most relevant clinical problems for morbility and mortality. We can differentiate congenital and acquired forms. In this short communication we describe 1 case observed by us that seems interesting for the association of a congenital and acquired form [Protein S deficiency and inflammatory bowel disease (IBD)] and for the dramatic events suffered before receiving a complete diagnosis and therapy, indicating the importance of recollection of information from the patients, starting from anamnestic data.

Multiple myeloma in the elderly: clinical features and response to treatment in 113 patients.

BACKGROUND: Considering the conflicting results of the few reports on geriatric MM patients and the increasing relevance of the problem, we analyzed a series of 113 patients over 64 years of age treated with conventional chemotherapy. PATIENTS AND METHODS: The median age was 71 (range 65-92). Stage IA, IIA, IIIA and IIIB patients numbered 28, 33, 45 and 7, respectively. The M component was IgG in 73 patients (65%), IgA in 30 (26%), IgD in 3 (3%), light chain in 5 (4%); no monoclonal component was detected in 2 (2%) cases. Sixty-three patients showed symptomatic skeletal disease. Melphalan/prednisone (MP) was the first-line treatment in 84 patients (74%). Patients were grouped according to age (> 64 < or = 74; > or = 75) in order to carry out analysis. RESULTS: Seventy-eight cases (69%) showed a sizable reduction in the tumor mass; objective and partial response was achieved in 57 (50%) and 21 (19%) patients, respectively. Patients with stage I-II disease fared significantly better than stage III patients (median survival: 70 vs 38 months; p = 0.017). Response to first-line treatment correlated with overall survival; patients with responsive or refractory disease had median survival rates of 64 and 20 months, respectively (p = 0.0001). CONCLUSIONS: Neither patients above nor below 75 years of age showed any difference in presentation features or in response to treatment. These results suggest that advanced age should not be considered a major obstacle to active treatment.

POEMS and Crow-Fukase syndrome: two cases giving rise to more questions about plasma cell dyscrasias.

Two cases of POEMS and Crow-Fukase syndrome are reported. We focused our attention on the problems recently debated in the literature regarding POEMS and osteosclerotic myeloma, the pathogenetic mechanisms of the clinical symptoms in these syndromes and the problems of their classification among plasma cell dyscrasias with polyneuropathy.

Laparoscopic versus conventional appendectomy.

BACKGROUND: The results of recent series suggest remarkable advantages of laparoscopic appendectomy over the conventional open appendectomy. To determine if clear advantages could be established, the charts of all patients admitted to our institution with a presumptive diagnosis of acute appendicitis and subsequent appendectomy were retrospectively reviewed. STUDY DESIGN: From January 1990 through June 1992, there were 300 conventional open appendectomies and 66 laparoscopic appendectomies performed. Data from both groups were compared with respect to anesthesia time, operative time, postoperative morbidity, postoperative pain, time to regular diet, hospitalization period, cost, and return to normal activities. RESULTS: There were no significant differences between the laparoscopic and open appendectomy groups with respect to operative complications, postoperative morbidity, pain medication requirements, and time to regular diet. There were significantly longer anesthesia times, operative times, and operating room costs in the laparoscopic group. For complicated appendicitis, the laparoscopic technique resulted in infectious complications that required readmission in 45.5 percent of the patients. CONCLUSIONS: Laparoscopic appendectomy is a safe alternative to conventional open appendectomy for simple acute appendicitis. However, laparoscopic appendectomy is not superior to the conventional method with regard to operative time, postoperative morbidity, pain medication requirements, time to regular diet, length of stay, cost, or return to normal activity. Laparoscopic appendectomy may be contraindicated in complicated appendicitis (gangrene, perforated with abscess, or peritonitis) due to an increased rate of infectious complications requiring readmission.

[An elevated incidence in the association of diabetes mellitus and Dupuytren's disease]. / Elevata frequenza dell'associazione diabete mellito-malattia di Dupuytren.

The palmar aponeurosis typical of Dupuytren's disease has often been encountered in people with diabetes mellitus. A well-tested technique that is highly sensitive and specific was used to identify the signs of Dupuytren's disease in a group of Diabetic Clinic patients and revealed a statistically significant connection between the two conditions. For this reason, the hands should be examined carefully and systematically during periodic check-ups on diabetic patients.

[Hepato-renal polycystosis, Marfan's syndrome and spina bifida occulta: a complex association. Description of a clinical case]. / Policistosi epato-renale, sindrome di Marfan e spina bifida occulta: una complessa associazione. Descrizione di un caso clinico.

The simultaneous diagnosis of polycystic kidney, Marfan's syndrome and silent spina bifida in a young woman is described. The coexistence of the first two entities has recently been reported as not rare, but the pathogenetic mechanism is not clear. The prognostic importance and the frequency of these two clinical entities make complete assessment of patients with only one of the two malformations mandatory.

Phase III randomized study of fluorouracil, epirubicin, and cyclophosphamide v fluorouracil, doxorubicin, and cyclophosphamide in advanced breast cancer: an Italian multicentre trial.

From February 1983 to January 1985, 497 patients with advanced breast cancer were randomly allocated to receive either epirubicin or doxorubicin in the following combination chemotherapy regimen: fluorouracil (5-FU) 500 mg/m2 intravenous (IV) on days 1 and 8; epirubicin or doxorubicin 50 mg/m2 IV on day 1; cyclophosphamide 500 mg/m2 IV on day 1 (FEC or FAC). Cycles were repeated every 21 days until progression or to cumulative doses of 700 mg/m2 for epirubicin and 550 mg/m2 for doxorubicin. Dose reductions were applied according to the standard criteria. Activity was evaluated in 443 patients (222 in the FEC arm and 221 in the FAC arm). The two experimental groups were comparable in age, performance status, menopausal status, histology, previous treatments, and site of the disease. The overall response rate (complete response and partial response [CR + PR]) was not significantly different: 53.6% for FEC and 56.5% for FAC. The median time to progression was 273 days for FEC and 314 days for FAC; the median survival time was 591 and 613 days, respectively. Leukopenia, anemia, nausea, and vomiting were significantly lower in patients treated with FEC. As for cardiotoxicity, four cases of congestive heart failure (CHF) were recorded among patients treated with FAC while only one was observed in the FEC group. These results indicate that epirubicin in a combination chemotherapy regimen is as active as doxorubicin and is significantly less toxic.

[Ferritin, CEA and TPA as tumor markers in breast neoplasms]. / Ferritina-CEA-TPA come markers neoplastici nelle neoplasie mammarie.

In order to assess the sensitivity and specificity of Ferritin, CEA and TPA as neoplastic markers in breast carcinomas, 91 patients all classified according to the TNM-UICC system were studied in a cancer clinic. The results of the analyses indicate that ferritin is apparently only influenced by the presence of metastatic neoplasias and that greater sensitivity is obtained if all three markers are employed simultaneously.

[Study of blood ferritin in cancer patients and its possible use as an indicator of an unfavorable prognosis]. / Studio della ferritinemia in pazienti neoplastici e sua possibile utilizzazione come indice prognostico sfavorevole.

Ferritinaemia levels were measured in 97 neoplastic patients and compared with the levels found in a healthy control group, in order to discover whether ferritinaemia had any significance as a neoplastic marker. Higher levels were encountered in all neoplastic patients (P less than 0.005) than in the control group. Levels were particularly high in the patients with metastasised tumours (especially breast cancer: P less than 0.001). The highest ferritinaemia levels were found in terminal patients (P less than 0.001).

[Evaluation of blood ferritin in sideropenic anemia and its modifications in the course of divided-dose parenteral iron therapy]. / Valutazione della ferritinemia nell'anemia sideropenica e sue modificazioni in corso di terapia marziale parenterale refratta.

Changes in blood ferritin during divided dose parenteral iron therapy and the importance of ferritin evaluation in iron-deficiency anaemia were investigated in 20 women and 10 men with this diagnosis through withdrawals before and after treatment. In 6 subjects, blood ferritin values enabled the presence of iron deficiency to be ruled out, since they were high at the first control (in agreement with the histological examination of the marrow in the search for iron deposits). In sideropenic males, the difference between values at the time of diagnosis and those of normal controls was significant (p less than 0.001). The absence of this finding in the females may have been due to over-low values in the normal controls. Blood ferritin values during therapy gradually rose until its termination. The conclusion is drawn that at any rate in males the determination of blood ferritin can be a useful aid in the diagnosis of iron-deficiency anaemia, and in the demonstration of normal reserves after treatment.

[Paraneoplastic syndrome of disseminated intravascular coagulation in cardial carcinoma with multiple metastases]. / Sindrome paraneoplastica di coagulazione intravascolare disseminata in carcinoma cardiale con metastasi multiple.

A case of paraneoplastic DIC syndrome (asymptomatic carcinoma of the gastric fundus with multiple metastases) is described. Initially, differential diagnosis hesitated before thrombotic thrombocytopenic purpura (Moschowitz' syndrome), given the presence of grave microangiopathic haemolytic anaemia as a major symptom. The main characteristics of Moschowitz' syndrome and the most frequent causes of DIC are described in the discussion.

Normal range of blood colony-forming cells (CFU-C) in humans: influence of experimental conditions, age, sex, and diurnal variations.

Blood colony-forming cells (CFU-C) and colony-stimulating activity obtained from feeder layers of peripheral blood leucocytes (leucocyte CSA) have been studies in 69 normal subjects by means of semisolid agar culture system. Groups of normal volunteers were selected according to sex and age (20 to 45 and older than 60 years) and the results compared. The mean number of circulating CFU-C was significantly lower in young women (20-45 years old) than in males over 60 years of age, but no differences were found among the other age and sex groups. Leucocyte CSA did not significantly differ among these groups. In 5 young males the blood CFU-C did not show significant variations at 8 AM and at 4 PM of the same day. When the study was repeated in 18 subjects at longer time intervals, the number of colonies showed a maximum fivefold variation. The amount of plasma and polymorphonuclear granulocytes present in our culture system did not inhibit the colony growth. In most cases, double layer cultures grow a higher number of colonies than single layer, but feeder layers of some normal subject seem to inhibit the colony growth.