Initial experience with laparoscopic adrenal surgery in children: is endoscopic surgery recommended and safe for the treatment of adrenocortical neoplasms?

Through a review of 83 cases reported in literature, including our experience of two successful right laparoscopic adrenalectomies performed in a 3-year-old girl for androgen-secreting adenoma and in a 9-year-old male for pheochromocitoma, we have anaIyzed the indications, the techniques and the results of laparoscopic or retroperitoneoscopic adrenalectomy in children. Nineteen pheochromocytomas, 18 neuroblastomas, 15 adenomas, 12 ganglioneuromas, 9 hyperplasias, 2 carcinomas, 1 teratoma and 1 adrenogenital syndrome have been treated with excellent results (follow-up 1 month to 4 years) with no evidence of recurrence. The age range is from 1 month to 16 years with an equal female/male ratio. The tumor size range from 10 mm to 80 mm with approximately a mean of 40 mm. Sixty-three laparoscopies of which 54 lateral and 9 anterior have been performed versus 14 posterior retroperitoneoscopies. Regarding right adrenalectomy, 29 were laparoscopic and only 2 retroperitoneoscopic. With regards to left adrenalectomy, 23 were laparoscopic and 9 retroperitoneoscopic. Looking at bilateral lesions, these tumors were approached laparoscopically in 6 cases alternating both sides and 1 retroperitoneoscopically (this case was already operated on the other side). The operative time ranged from 25 minutes in newborn to 320 minutes in bilateral cases with an average of 120 minutes. Conversion rate is higher in retroperitoneoscopy (30%) (4 cases out of 13), than laparoscopy (6%) (4 cases out of 63). Specifically, for right retroperitoneoscopic adrenalectomy conversion rate was 100% (2 cases out 2), for left retroperitoneoscopic adrenalectomy 22.2% (2 cases out 9), for right laparoscopic adrenalectomy 6.8% (2 cases out of 29) and for left laparoscopic adrenalectomy 8.6% (2 cases out of 23). Only in 1 case were blood transfusions requested. Hospital stays ranged from 35 hours to 17 days with an average of 4 days. In conclusion the indications of endoscopic adrenalectomy in children are not different from those of traditional surgery and the well-known advantages of laparoscopic adrenal surgery should be applied to pediatric patients. It seems that there is no age and tumor size limits for a well-trained surgical team. Lateral transperitoneal approach is the most utilized with the child positioned in 90-degree flank decubitus. Laparoscopy is undoubtly preferred for right adrenalectomy (93% of cases), while for left adrenalectomy retroperitoneoscopy has been used in 39% of the cases. Considering the conversion rate and on the basis of our experience with adults, we recommend laparoscopic adrenalectomy for both right and left adrenal lesions, but we think that the pediatric surgeon should feel free to choose the approach in which he/she is more skilled. However, the best surgical result will be achieved if the pediatric and adult surgeon collaborate with their different experiences. Lastly, we suggest the use of new technological devices such as Ultracision Harmonic Scalpel which was a critical factor in our two successful right adrenalectomies.

Pseudomonas aeruginosa infection in cystic fibrosis caused by an epidemic metallo-ß-lactamase-producing clone with a heterogeneous carbapenem resistance phenotype.

An epidemic IMP-13 metallo-ß-lactamase (MBL)-producing Pseudomonas aeruginosa clone, causing infections and even large outbreaks in Italian critical care settings, was detected in a young cystic fibrosis patient. In this patient, the chronic infection was sustained by distinct clonal sub-populations of the MBL-producing P. aeruginosa clone, either susceptible or resistant to carbapenems. These findings underscore the importance of infection prevention practices in cystic fibrosis settings and pose an important diagnostic and therapeutic challenge.

The costs of treatment of early and chronic Pseudomonas aeruginosa infection in cystic fibrosis patients.

The aim of cystic fibrosis (CF) care is to improve both the life expectancy and quality of life of patients. However, rising costs and limited resources of health services must be taken into account. There are many different antibiotic strategies for therapy of Pseudomonas aeruginosa infection in CF patients. In this 5-year retrospective study we found that the cost of treatment of initial infection is considerably lower than the cost of treating chronic P. aeruginosa infections. The percentage distribution of costs of antibiotic treatment in relationship to the administration route was considerably different between outpatients and inpatients. We observed an increase in antibiotic costs with the age of the patient and the decrease in FEV(1)values. The implementation of early eradication treatment, in addition to decreasing the prevalence of patients chronically infected by P. aeruginosa, might also bring about a notable decrease in costs.

An overview of international literature from cystic fibrosis registries: 1. Mortality and survival studies in cystic fibrosis.

Patient registries are organized systems of data collection for scientific, clinical or health strategy purposes. Aims of our review were to document scientific literature based on data and information from cystic fibrosis (CF) registries; to understand which clinical problems have been addressed and for which of these the studies concerned have correctly answered the questions raised (i.e. a methodological critique) and to identify clinical issues in need of further investigation. The review included primary studies starting from a formally constituted CF registry of at least national level, using data from the registry to evaluate research hypotheses. This article is an overview of the research undertaken, focusing in detail on the issues of mortality and survival. The studies considered here focused mainly or secondarily on survival in CF, the aim being to ascertain an improving trend, identify any prognostic factors and, in some cases, attempt to provide a predictive model of survival.

Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis.

Antibiotic strategies against Pseudomonas aeruginosa infection in cystic fibrosis (CF) patients should consider the natural history of the P. aeruginosa infection, ranging from the first isolation of the germ in the airways to isolation at every microbiological culture, and the patient's clinical condition. Antibiotic treatment against P. aeruginosa given at the time of first isolation may prevent or delay chronic infection. The period of intermittent colonization can be considered the time before the development of mucoid P. aeruginosa phenotype. The optimal treatment strategy in this stage remains unclear in terms of agents used and duration of treatment. To treat acute exacerbation, the authors suggest using intravenous administration of two different classes of antibiotics. Maintenance antibiotics are administered to slow the decline in pulmonary function for P. aeruginosa chronic infection. The meaning of maintenance therapy has changed over time, beginning from intravenous quarterly anti-Pseudomonas antibiotics, irrespective of symptoms, to other strategies such as oral macrolides, ciprofloxacin or inhaled antibiotics (tobramycin and colistin). Aerosol delivery can provide a high concentration at the desired site with minimal absorption and therefore low risk of toxicity. There is scientific evidence that antibiotics are clinically effective in CF patients. Antibiotic selection should be based on periodic isolation and identification of pathogens and antimicrobial susceptibility.

Bone mineral density in cystic fibrosis patients under the age of 18 years.

AIM: The increase in life expectancy of cystic fibrosis (CF) patients has brought about a rise in new clinical problems in these patients, such as a decrease in bone mineral density (BMD). The cause of diminished BMD in CF is multi-factorial. METHODS: The aim of this cross-sectional study, conducted on 39 CF patients under the age of 18 years, was to evaluate the degree of bone mineralization and the prevalence of low BMD in these patients during a follow-up at the Cystic Fibrosis Regional Center of Tuscany, using a dual energy X-ray absorptiometry (DXA) scan, and to then study the factors correlated with low BMD. RESULTS: Areas BMD values (g/cm2) and Z-score values were determined. Eighteen patients (46%) out of the our sample had decreased BMD, while 21 patients (54%) had normal values. A statistically significant association was found between BMD Z-score values and pancreatic insufficiency, BMI<5th percentile and DeltaF508 homozygosis. Subjects treated with oral steroid therapy had a 3.9 times greater risk of developing osteoporosis compared to non-treated subjects (95% C.I.: 1.07-22.6; R.R. 4.9). An association was found between BMD Z-score values and FEV1 values (r=0.29; P=0.06), physical activity total score values (r=0.22; P=0.19) and the Chrispin-Norman chest radiographic score (r=-0.31; P=0.06). CONCLUSION: Early identification of reduced bone mass values would permit early intervention to prevent the development of osteoporosis. Maintaining pulmonary function, guaranteeing optimal nutritional status, following an adequate program of physical activity and controlling steroid intake could maintain BMD over time.

Oxandrolone in constitutional delay of growth: analysis of the growth patterns up to final stature.

In order to evaluate the effects of low-dosage, 6-12 months course of oxandrolone treatment in constitutional delay of growth, we compared the growth responses on treatment, the pattern of sexual development and pubertal growth events, up to final stature of 11 prepubertal boys, aged 10.6-14.1 yr, with those of 11 prepubertal, age-matched untreated controls. Treatment caused a significant increase of height velocity, from 4 to 9 cm/yr, and a significant acceleration of bone maturation, without affecting the timing of onset of puberty, the progression of sexual development or the onset of pubertal growth spurt. On the other hand, oxandrolone induced an earlier skeletal growth arrest but did not affect the expected final height. Treated boys showed an adult stature not significantly different from that of control subjects. Our data suggest that 6 months-1 year, low dosage oxandrolone treatment in constitutionally delayed growth has no significant effect on the pattern of pubertal growth, nor on the rate of sexual maturation or on final height.

Precocious puberty: auxological criteria discriminating different forms.

It may be possible to recognize different forms of precocious puberty at the first evaluation. In a group of 26 sexually precocious girls we used Bayley-Pinneau predicted adult height (P.A.H.) to discriminate patients with 'poor' or 'good' height prognosis. Patients with evidence of impaired height prognosis (P.A.H. < -1 SDS) (Group 1) were immediately treated with LH-RH analogs, while patients with unimpaired height prognosis (P.A.H. > -1 SDS) (Group 2) were followed without therapy. Two yr of treatment significantly improved P.A.H. in Group 1 patients, from a mean of -1.68 +/- 0.4 to a mean of -0.57 +/- 0.6 (SDS) (p < 0.01). After the 2 yr observation period, Group 2 patients showed no significant variation of P.A.H. (from a mean of 0.45 +/- 0.8 to a mean of 0.33 +/- 0.6). The retrospective analysis of the growth pattern changes in the two Groups seems to indicate that LH-RH agonist treatment improves height potential in girls with initial poor height prognosis and that girls with initial good height prognosis maintain an unimpaired growth potential.

Usefulness of early morning urine estrone-3-glucuronide assay in the monitoring ovarian secretory function in precocious puberty.

To evaluate the usefulness of the urinary estrone-3-glucuronide (EI-3-G) in the monitoring of the ovarian function in girls, we studied 11 girls with idiopathic central precocious puberty (ICPP) treated with LHRH analogs (LHRHa) for 2-5 years. Plasma LH, FSH, 17-beta-Estradiol (E2) levels, early morning urine (EMU) E1-3-G concentrations, were assessed before and 3, 6, 12 months after the onset of treatment. As expected, mean basal plasma LH, FSH and E2 concentrations, as well as mean basal EMU E1-3-G levels were significantly (p < 0.01) higher in patients studied than in normal, age matched, prepubertal controls. Three out of the 11 sexually advanced girls showed undetectable (< 15 pg/ml) basal plasma E2 values. On the contrary, in each patient studied, individual basal E1-3-G levels were higher than in normal age-matched prepubertal girls. LHRHa treatment significantly suppressed both basal and peak stimulated plasma gonadotropins, plasma E2 and EMU E1-3-G. However, while serum E2 levels were below the assay detection limit, not allowing to assess the degree of gonadal suppression, E1-3-G urinary concentrations were detectable in each subject treated, in the range of the normal prepubertal values. EMU E1-3-G determination seems to be a very sensitive and reliable approach to the monitoring of the effectiveness of LHRHa treatment in sexually advanced girls, allowing to detect very low estrogen concentrations and to achieve the desired ovarian suppression.