Parents spend an average of nine hours a day providing palliative care for children at home and need to maintain an average of five life-saving devices.

AIM: This Italian study investigated home-based palliative care for young children and how long it took parents to meet their needs. METHODS: The study population consisted of 33 families with a child under the responsibility of the Veneto Regional Center for Pediatric Palliative Care, northern Italy, who needed medical support in at least two of the following areas: respiratory, feeding, pain and seizures. RESULTS: The children had a mean age of 6.8 ± 4.7 years. We found that 72% of the patients needed medical devices for feeding, 36% had a tracheostomy and 55% were on mechanical ventilatory support. The children needed an average of five different life-supporting medical appliances, and the time taken to provide for their care increased significantly with each additional appliance (p = 0.016). Their most time-consuming daily needs were feeding (174 minutes) and support when they woke up at night (67 minutes). The average daily time that parents spent taking care of their child amounted to eight hours and 54 minutes per day. CONCLUSION: Parents providing palliative care for children with life-limiting diseases spent an average of nine hours a day caring for them each day and had to maintain an average of five medical appliances.

[IMPaCCT: standards of paediatric palliative care]. / IMPaCCT: Standards pädiatrischer Palliativversorgung in Europa.

The needs of children and families living with life-limiting and life-threatening illness are similar across all European countries. Meeting these needs requires a comprehensive and integrative approach, with the input of a skilled multidisciplinary paediatric team. It is essential that the core standards for paediatric palliative care recommended in this document of the European Association for Palliative Care (EAPC) now be implemented across Europe.

Circulating ghrelin level is increased in coeliac disease as in functional dyspepsia and reverts to normal during gluten-free diet.

BACKGROUND: It is controversial whether serum ghrelin concentration is altered in coeliac disease and whether this alteration is related to nutritional impairment or to inflammatory changes of duodenal mucosa. AIM: To investigate clinical and histopathological variables affecting circulating ghrelin in coeliac patients by comparison with dyspeptic patients and with healthy controls. METHODS: We measured serum ghrelin and obtained gastric and duodenal biopsies in 44 coeliac patients before and after 1-year gluten-free diet, in 39 dyspeptic patients and 53 healthy controls. RESULTS: Serum ghrelin concentration was significantly higher in coeliac (531 +/- 29 pg/mL, P < 0.05) and in dyspeptic patients (526 +/- 14 pg/mL, P < 0.01) than in healthy controls (451 +/- 8 pg/mL), and body mass index was significantly lower in coeliac (20 +/- 1) and in dyspeptic patients (20 +/- 1) than in healthy controls (22 +/- 1, P < 0.05). In coeliac patients serum ghrelin concentration was not related to the severity of duodenal lesions. Serum ghrelin reverted to normal (399 +/- 30 pg/mL) and body mass index increased significantly (0.6 +/- 0.1 kg/m(2) increase, P < 0.05) during gluten-free diet despite persistent duodenal lymphocytic infiltration. CONCLUSIONS: Ghrelin concentration is increased and body mass index is decreased in coeliac and in dyspeptic patients irrespective of presence and severity of duodenal inflammation. Nutritional impairment is a key factor in elevating plasma ghrelin levels in coeliac disease.

Survival rate and prognostic factors in patients with intestinal failure.

BACKGROUND: Intestinal failure impairs nutritional status and survival expectance. Though intestinal adaptation and enteral independence may be achieved, artificial nutrition is needed in about half of the patients. AIMS: This study is aimed at assessing the causes of death, survival rate, enteral independence in time, and factors affecting the clinical outcome in a group of patients with intestinal insufficiency. PATIENTS: Sixty-eight patients with intestinal insufficiency, due to major intestinal resection in 60 cases (short bowel syndrome) (remnant intestine length 101-150 cm in 31 cases, 50-100 cm in 23 cases, <50 cm in 6 cases), and due to chronic idiopathic pseudo-obstruction in 8 cases, were enrolled and followed-up for (median) 36 months (25th and 75th percentile in 12 and 60 months, respectively). In 60 short bowel syndrome patients, the main conditions that led to intestinal failure were ischemic bowel (28), major surgery complications or severe adhesions (17), radiation enteritis (10), Chron's disease, intestinal tuberculosis, small bowel lymphoma and trauma (others). METHODS: Seventeen variables age, underlying disorders, length of remnant bowel, type of surgery, hospital stay, type of nutrition (hospital and home) and its variations in time, causes of death, survival rate and time were considered. Statistical analysis was carried out by Mann-Whitney U-test, Pearson chi2, Spearman correlation test, Kaplan-Meyer method and Cox's proportion hazards regression model. RESULTS: At the time of admission to the hospital, none of the patients had nutritional independence, 54 (79.4%) were on parenteral nutrition and 14 (20.6%) were on enteral nutrition. At the time of discharge, 23 (33.8%) patients showed enteral independence, 39 were on home parenteral nutrition, 3 on enteral nutrition + i.v. feeding, 1 on enteral nutrition, and 2 needed oral supplementation with hydroelectrolyte solutions only. After a median value of 36 months, 30 and 2 patients were on home parenteral nutrition and enteral nutrition + i.v. feeding, respectively, 2 on enteral nutrition, 2 on oral supplementation with hydroelectrolyte solutions, and 26 cases reached enteral independence. A significant relationship was detected between the length of remnant bowel and types of nutrition at both admission (r = 0.38; P = 0.001) and discharge (r = 0.48; P = 0.001), parenteral nutrition being more frequent in patients with very short bowel. Twenty-two patients (32.4%) died (4 from newly occurring malignancies), 40 (58.8%) survived, and 6 (8.8%) were lost to the follow-up. Eleven of 22 patients died from conditions related to intestinal failure (8 cases) and/or home parenteral nutrition complications (3 cases). At 12, 24, 36, 48, 60 and 72 months, survival rates were 95.4, 93.3, 88.1, 78.6, 78.6 and 65.5%, respectively, but it was significantly lower for patients with <50 cm of remnant bowel than those with longer residual intestine (P < 0.05), and in patients who started home parenteral nutrition above the age of 45 years (P < 0.02). Survival rate was higher in patients with enteral independence than those with enteral dependence (P < 0.05). Better survival rates were registered in patients with chronic obstructive intestinal pseudo-obstruction and major surgery complications, whereas ischemic bowel and even more radiation enteritis were associated with a lower survival expectance. CONCLUSIONS: Actuarial survival rate of patients with intestinal failure quotes 88 and 78% at 3 and 5 years, respectively. It is influenced by the length of remnant intestine, age at the start of home parenteral nutrition, enteral independence and, to some extent at least, by the primary disorder. Enteral independence can be achieved in time by about 40% of the patients with intestinal insufficiency, but for home parenteral nutrition-dependent cases, intravenous feeding can be stopped in less than one out of five patients during a median 3-year period.

Detection of an anti-RhD antibody 2 years after sensitization in a patient who had undergone an allogeneic BMT.

We describe an HLA matched bone marrow transplantation with minor ABO incompatibility and RhD mismatch (donor RhD negative and recipient RhD positive). GVHD appeared on day +96 and therapy with steroid and cyclosporin was started. When GVHD disappeared and immunosuppressive therapy was stopped (2 years after BMT), an anti-RhD antibody was detected in the patient's serum. The delayed appearance of this antibody may have been associated with the prolonged immunosuppression that was required for treatment of the patient's GVHD.

Spontaneous resolution of cystic hygroma in a 46,XX normal female.

We report a case of nuchal cystic hygroma with spontaneous resolution detected by ultrasound examination at 13 weeks' gestation. Fetal karyotype and amniotic fluid alpha-fetoprotein levels were normal. Extreme caution in evaluating this situation is stressed.

Dexamethasone in the treatment of bronchopulmonary dysplasia.

Sixteen chronically ventilator-dependent newborns with BPD were treated with one or more cycles of dexamethasone (0.5 mg/kg/day). In 11 cases extubation was possible during the therapy period. Ventilatory parameters were lowered in 3 other newborns. FIO2, respiratory rate, PIP, and PEEP, assessed before and after dexamethasone administration, decreased in a statistically significant way. Our data confirm the utility of dexamethasone in the extubation in chronically ventilated infants with BPD.

Periventricular intraparenchymal cystic lesions: critical determinant of neurodevelopmental outcome in preterm infants.

During a four-year period, 154 surviving preterm infants of 32 weeks gestation or less were prospectively examined by cerebral ultrasound for periventricular-intraparenchymal cystic lesions (IPCL) subsequent to ischemic and/or haemorrhagic damage. Neurological and developmental outcome was assessed with examinations at 0, 3, 6, 12, 18, 24, 36, 48 months of age corrected for prematurity. Twenty-four (15.5%) patients were found to have IPCL changes at ultrasound. In 8 cases, a porencephalic cyst subsequent to grade IV IVH (Papile's classification) was found; all had cerebral palsy and severe developmental deficit was present in 4. Diffuse bilateral PVL was found in 8 cases: 1 was not evaluable, 7 developed cerebral palsy; the developmental delay was severe in 4, moderate in 2 patients, and only 1 was normal. Four patients had localized bilateral PVL: 3 patients had mild diplegia and 1 was normal; the developmental outcome was normal only in 1 case, 1 had a severe cognitive delay, and 2 were moderate. In the remaining 4 cases, the ultrasound showed a monolateral localized PVL: 1 patient had mild diplegia and moderate cognitive delay, 3 were normal. - This study confirms the important role of the ultrasonographic diagnosis of IPCL in preterm infants to foresee later neurodevelopmental outcome. Extensive parenchymal lesions were strongly associated with major neurodevelopmental handicaps, while localized and small lesions were correlated with more favorable neurological as well as developmental prognosis.

Bronchopulmonary dysplasia: a longitudinal study of 20 cases.

Of 20 patients with bronchopulmonary dysplasia (BPD), 17 survived (85%) and were followed prospectively up to one year post-term. Lower respiratory tract infections occurred in 12 patients (70.5%), and in 7 (41%) at least one hospital admission was required. At one year post-term follow-up, 9 patient (52.9%) continued to present respiratory symptoms, and 5 out of 13 (38.4%) radiographic changes. Six cases (35%) presented chronic cor pulmonale. Only 6 children (35%) showed normal growth, while the others showed deficits in one or more growth parameters. Cerebral palsy occurred in 41% of the children; 3 cases of severe tetraparesis and 4 of moderately severe palsy (hemiparesis or diplegia). Developmental quotient (DQ) was less than 70 in 6 cases (35%), from 70-90 in 4 (23.5%), and greater than 90 in 7 (41%). The severe neurodevelopmental outcomes were significantly correlated with the presence of important neonatal cerebral pathology (3-4 degrees IVH or periventricular leukomalacia). Retinopathy due to prematurity was diagnosed in 7 patients (41%), and in 5 it progressed to retrolental fibroplasia. Ten cases (58.8%) showed strabism, of which 8 had previous eye background involvement. Hearing deficit was not detected in any patient. Esthetic and functional sequelae consisted of scalp eschar (3 cases), post-thoracotomy scar (1 case), pleural drainage scars (3 cases), nasal deformity due to prolonged intubation (1 case), laryngeal stenosis (1 case), and post-tracheostomy stenosis (1 case).(ABSTRACT TRUNCATED AT 250 WORDS)

Gram-negative septicemia in patients with hematologic malignancies.

The clinical records of 66 consecutive episodes of Gram-negative bacteremia occurring in 60 patients with hematologic malignancies during a 66-month period were reviewed to assess the major prognostic factors. The bacteremia-related mortality was 53%. Overall, Pseudomonas aeruginosa (54%) and Escherichia coli (24%) were the predominant isolates (fatality rate 78 and 31% respectively). The majority of patients (58/66) were granulocytopenic (PMN less than 1000/microliters). Among the 18 patients whose circulating granulocytes increased by one log10 or to above 1000/mmc during therapy, the fatality rate was 39%, as opposed to 70% in the 40 patients without such an increase. Pneumonia-associated bacteremia (56%) had a high fatality rate (73%) compared to isolated bacteremias (27%). Septic shock and inappropriate antibiotic therapy accounted for the highest mortality. Our data suggest that Pseudomonas etiology, persistent neutropenia, associated pneumonia, septic shock and inappropriate antibiotic therapy account for a bad prognosis in Gram-negative bacteremia in hematologic malignancies.

[Neonatal cystic lymphangioma]. / Linfangioma cistico neonatale.

The A.A. reports a case of cystic congenital linphangioma of the face and neck, recently observed on the Neonatal Intensive Care Unit. The therapeutic approach was very difficult and required three times a surgical menagement because of successively relapses. The control and normalization of the compressive and infective complications due to the massa and its removal were hard too. In spite of some aesthetical problems, the results may be considered satisfactory. Our case enphasizes the intrinsec, complex, therapeutical difficulties of the cystic linphangiomas.

Empiric therapy of infections in hematologic malignancies: a prospective, randomized trial.

Patients with hematologic malignancies were randomly assigned to receive cefuroxime (group A) or tobramycin plus ampicillin (Group B) during 86 febrile episodes. In both regimens carbenicillin was added during neutropenia (71% of all episodes: groups C and D). The most common type of infection was pneumonia (48% alone; 72% with other sites involved), which accounted for a high fatality rate (15%); the highest rate occurred during septicemia with pneumonia (50%). The overall response rate to initial therapy was 63% without significant differences among the four regimens. The worst prognosis was observed in neutropenic patients without granulocyte recovery. When initial and cross-over trials were combined, there were favorable outcomes in 90% of all cases. Cefuroxime alone seems to be as effective as tobramycin plus ampicillin in the treatment of infections in hematologic malignancies. No side effects could be attributed to the cefuroxime-containing regimens.

Evaluation of the scintigraphic technique for detecting bone metastases in 100 breast cancer patients.

In an attempt to evaluate the utility of bone scintigraphy for the diagnosis and follow-up of skeletal metastases, radioisotopic scan (total body) by 99mTc has been performed on 100 patients suffering from breast cancer in various stages. The radiographic control followed the scan on the basis of clinical or scintigraphic suspicion of bone lesions. Twenty-one patients showed metastases by scan, and in all but 2 the radiography was concordantly positive; in 22 subjects scintigraphy and radiology demonstrated non-malignant lesions. Both scan and radiology were used to explore 169 metastatic localizations, with concordant results in 45% of the sites; however, 7% of the sites with discordant results became positive for metastasis by radiology some months after the scan positivity. The false-negative results were more frequent in the radiographic study (27%) than with the scintigraphic scan (21%). The latter technique was unable to detect osteoblastic lesions in particular. The scintigraphic test also gave reliable results in the follow-up procedure, frequently anticipating the radiological modifications of metastatic lesions. In conclusion, bone scan often appears to be more accurate and preocious than the roentgengraphic test in the detection and the follow-up of skeletal metastatic lesions; it suggests the utility of the bone scan as a preliminary test, which allows a more rational use of the radiology.