[An unusual case of amelia of the left lower extremity associated with homolateral renal agenesis].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to A Fichera.
Explore the source record for details and available documents.
The objective of this study was to prospectively assess the long-term functional results after restorative proctectomy with coloanal anastomosis for rectal cancer. Thirty consecutive patients (18 males; mean age 59.6 +/- 9.8 years, range 40 to 75 years) underwent proctectomy with coloanal anastomosis for rectal cancer between January 1990 and March 1997. Cancers were located between 5 and 12 cm from the anal verge. Differences existed in the administration of adjuvant therapy and in the kind of anastomotic reconstruction. An 8 cm colonic J-pouch was fashioned in 11 patients. The coloanal anastomosis was protected by a diverting loop ileostomy in 22 patients. All patients were evaluated using a prospective patient-completed protocol to record daily bowel activity over a 1-week period at 3, 6, and 12 months, and yearly thereafter. Mean follow-up extends to 55.5 +/- 27 months (range 7 to 117 months). There were no perioperative deaths. Four patients (13.3%) developed a clinically evident anastomotic dehiscence. Overall, stool frequency decreased from 4.4 +/- 2.5 bowel movements per day at 3 months to 3.0 +/- 2.8 bowel movements per day at 5 years. Patients with a J-pouch had a lower stool frequency in comparison to patients with an end-to-end coloanal anastomosis during the entire study period (from 3.2 +/- 2.2 vs. 3.9 +/- 2.7 bowel movements per day at 6 months to 2.8 +/- 1.9 vs. 3.4 +/- 4.0 bowel movements per day at 5 years; no statistical significance). The percentage of continent patients increased from 50% at 6 months to 75% at 5 years; the percentage of patients with incontinence for solid stool and with frequent incontinence ($7 episodes per week) decreased from 35.7% at 6 months to 12.5% at 5 years. The influence of the type of anastomosis, dehiscence, protective stoma, J-pouch, radiation therapy, and gender was evaluated with univariate analysis. Although there was no statistically significant correlation between any of these variables and the development of incontinence, when incontinence occurred, a history of anastomotic dehiscence increased the number of episodes of incontinence per week and the percentage of episodes of incontinence for solid stools at 6 months, 2 years, and 5 years (P < 0.05 and P < 0.001, respectively); the use of preoperative radiation therapy increased the number of episodes of incontinence per week at 6 months, 1 year, 2 years, and 5 years (P < 0.01) and the percentage of episodes of incontinence for solid stools at 3 and 6 months and 1 and 2 years (P < 0.04); and the presence of a J-pouch increased the number of episodes of incontinence per week at 1 and 2 years (P < 0.03 and 0.005, respectively) and the percentage of episodes of incontinence for solid stools at 2, 3, and 4 years (P < 0.05). These data suggest that the functional results after proctectomy with coloanal anastomosis improve at least over the course of the first 5 postoperative years. Furthermore, when incontinence develops, its severity is made worse by the occurrence of an anastomotic dehiscence, the use of preoperative radiation therapy, and the presence of a J-pouch.
The authors describe a two months aged patient affected by cow's milk protein intolerance (CMPI) with serious haemorrhagic manifestations. As blood coagulative laboratory findings demonstrated a prolongation of P.T. and P.T.T. with a marked reduction of vitamin K-dependent factors only, the authors believe these bleeding manifestations secondary to a case of late haemorrhagic disease of the newborn. Vitamin K treatment determined a rapid normalization of haemorrhagic symptoms and laboratory clotting tests, without any alteration of these ones during the patient's follow-up too. The authors suggest that blood coagulative pattern must be investigated in all CMPI cases, especially in the ones with a precocious onset of clinical symptoms. In the cases with vitamin K-dependent factors deficiency the treatment is immediately necessary, while in other cases a daily dietary supplementation or a vitamin K weekly or monthly injection could be enough in order to prevent any further vitamin K-dependent factors deficiency.
Impairment of esophageal motor function is well recognized in connective tissue disease. We have investigated esophageal function, by manometric studies, presence of symptoms of esophageal involvement and antibodies pattern, in 18 female patients affected by systemic lupus erythematosus (SLE). Esophageal manometry showed motor abnormalities in 72.3% of the patients, especially hypokinetic abnormalities (hypotony of lower esophageal sphincter pressure, low amplitude or alterations of peristaltic waves) or, rarely, an increase of amplitude of peristaltic contractions. No significant correlation were found between antinuclear antibodies, esophageal symptoms and manometric findings. Hypoperistalsis or aperistalsis, may be due to an inflammatory reaction in the esophageal muscles or to an ischemic vasculitic damage of Auerbach plexus. High amplitude of peristaltic esophageal waves may be due to an early stage of reflux esophagitis: we have found gastro-esophageal reflux symptoms in more than half of our patients.
PF4 levels and platelets counts were studied in a group of 15 term newborn infants before treatment and after 24-48-72 and 96 hours of phototherapy and in a control group of 10 babies. Unlike data found by other AA. in vitro and in preterm infants, our values show only minimal, not statistically significant, differences in PF4 levels and platelets counts between the two groups. The AA. believe that in term infants, with adequate weight for gestational age, proper phototherapy treatment does not cause any change in platelet function, owing to the thicker and more mature skin and to the better bone marrow compensation typical of term versus preterm infants.
Coagulation abnormalities with and without haemorrhagic manifestations have been frequently reported in newborn-infants affected by hypoxia. Particularly in postmature-infants and in those ones with acute asphyxia at birth, respiratory distress syndrome (RDS), intra-uterine growth retardation (IUGR) and cyanotic congenital heart disease (CCHD). A reduction of synthesis or a consumption of blood coagulation factors are the main causes of these abnormalities. The anomalies of platelet number and of their function, of haemostasis global tests, of coagulation factors and physiologic inhibitors levels, of fibrinogenesis and fibrinolysis are examined, including authors' studies and a review of literature too. The authors think platelet count, PT, PTT, fibrinogen, factor V and VIII, and PDF determinations are necessary laboratory investigations for newborn-infants with RDS or acute asphyxia for about the first week of life, because of the risk of consumption coagulopathy. In the other hypoxic newborns (IUGR, CCHD, postmature infants) platelets count, PT, PTT and serum PDF determinations could be enough in order to value any coagulation abnormalities presence.
The Authors carried out a study on 37 untreated coeliac children to investigate the behaviour of K-dependent factors after vitamin K administration. The children were randomized into two groups: 22 children receiving a single dose of 10 mg i.m. of Phytonadione (Konakion, Roche) on the initial day of GFD and 15 children who did not receive vitamin K administration. PT, PTT and clotting activity of Factors II, VII, IX, X were determined before the treatment and/or GFD, and after 24 hours, 7 and 15 days. The results demonstrated that vitamin K administration determined a rapid increase in clotting activity of all K-dependent factors after 24 hours. These values remained normal after 7 and 15 days, except for Factor II, which slightly decreased on the 7th day. On the contrary, the children not treated, had levels similar to those of acute stage. After 7 days these values showed a slight increase and reached normal limits on the 15th day. No significant changes were seen in either PT or PTT in the two groups. They were constantly prolonged, reaching normal limits on the 15th day. These results indicate that the vitamin K deficiency, not only seems constant in children with CD, but also seems responsible for the haemocoagulative deficit of the K-dependent factors. After GFD when intestinal absorption is regained, all parameters returned to normal. The Authors concluded that K-dependent factors can be used as short-term indexes of improved intestinal absorption and that the coeliac children with severely compromised nutritional status can be treated with vitamin K (10 mg bolus).
The authors report a newborn infant affected by terminal transverse hemimelia of the right lower limb, associated with symmetric malformations of the hands, simulating the "amniotic bands syndrome". The symmetric hand anomalies and the presence of slight malformations of the left lower limb lead us to think that the etiology of this case is genetic, probably related to a new mutation.
The authors have studied the correlations between serum levels of calcitonin and the degree of oxygenation assessed by means of transcutaneous pO2 and pCO2 and capillary pH in 40 term newborns of adequate birth weight. Highly significant correlations (P < 0.001) were found at the 24th hour of life between calcitonin levels and the asphyxia parametres and between the latter measured at the 12th or the 24th hour and calcitonin levels found respectively at the 24th or the 48th hour. Similar correlations were found subdividing the studied newborns with regard to the type of delivery. We conclude that the severity of neonatal asphyxia is indeed the main determining factor of the magnitude of the calcitonin hyperincretion.
In this report the Authors describe the case of a 3-years-old boy with a septate gallbladder and chronic abdominal pain: an uncommon clinical condition at this age. Diagnosis was confirmed by ultrasound scan and oral cholecistogram. As congenital gallbladder abnormalities may cause, although rarely, chronic abdominal pain, also in the first years of the life, they must be included in the differential diagnosis.