[Preterm infant follow-up program in Lombardy Region-- Italy]. / Il programma di follow-up del nato pretermine in Regione Lombardia, Italia.

The correct management of preterm babies requires, after discharge, an adequate follow-up program. The plan could be adjusted in relation to economical rises, rooms and staff amount of Neonatal Intensive Care Unit (NICU), varying its length and number of visits for patient for year. A survey was performed in Lombardy Region, Italy, to evaluate the differences among the various follow-up programs and to find a possible common approach suitable by all NICUs. A 23 questions formulated questionnaire, with multiple choice answers, has been sent to the referents of outpatients management of any NICU of the Region. The answers obtained from 13 of the 17 Units interviewed have been evaluated. All NICUs have a follow-up program, including ophthalmological, neurological, cardiac and audiometric evaluations. The plan length vary by 1 to 7 years; annually a mean of 130, and daily a mean of 7.4 patients are visited. At discharge all NICUs provide a clinical report to the family, but it is mailed to the Primary Care Physician just in 54% of cases. The differences founded among the follow-up programs resulted not significant for the preterm management, therefore it is possible to uniform the different plans, improving communication and interaction between NICUs and Primary Care Physicians.

[Chylothorax]. / Il chilotorace.

Chylothorax is the accumulation of chyle in the pleural space. In newborns the congenital form is often prenatal diagnosed, while the late variety originates to damage to the thoracic duct by cardiac surgery, diaphragmatic hernia, etc. Clinical presentation results from the accumulation of pleural fluid and the symptoms depends on the size of the effusion. The treatment needs both medical and surgical care. The pleural cavity should be drained via thoracocentesis, and total parenteral nutrition should be started. Afterward fat-free diet with the addition of medium-chain triglycerides could be initiate. Somatostatin and octreotide have been successfully employed, mainly in post-surgery chylothorax. Surgery should be considered when medical management fails. Some approaches are reported, and thoracic duct ligation, pleurodesis and pleuroperitoneal shunts are the most utilized. The prognosis of chylothorax depends on the etiology, and it is consequence of a variety of treatments that may influence the outcome.

[Umbilical acid-base status of term infants: correlation with delivery mode]. / Equilibrio acido-base sul sangue cordonale di neonati a termine: correlazioni con il tipo di parto.

OBJECTIVE: To determine possible relations between Apgar score, umbilical artery haemogasanalysis and delivery mode. METHODS: 948 babies were considered; preterm infants and charts with incomplete data were excluded. 762 newborns and their respective umbilical artery blood gas parameters at delivery were evaluated. The collected Data were related to mode of delivery: vaginal with and without analgesia, elective caesarean section (CS), CS after labour, emergency CS for non reassuring trace. RESULTS: Mean pH (+/- DS) was 7.26 (+/- 0.08). The comparison vaginal delivery vs. CS was statistically significant (p < 0.0001). Analyzing the CS only, the babies born by emergency CS for non reassuring trace had pH statistically lower (ANOVA p < 0.004) and, in the latter group, there was a statistically difference between Italian newborns and those from other ethnicities (p < 0.02). No difference were found on Apgar score, excluding babies by vaginal delivery with analgesia that showed lower scores both at 1 (p < 0.02) and 5 (p < 0.002) minutes versus newborns without analgesia. CONCLUSIONS: Normal labour causes frequently a mild degree of acidosis, but it is not always related to neonatal asphyxia. The differences found between different delivery modes are, probably, the expression of causes related to the obstetric choice. That is also remarked by the statistically difference found in emergency CS for non reassuring trace in Italian population and in other ethnicities, showing a probably different cultural level, mainly about the "obstetric emergency" poorly understood by the pregnant. In vaginal deliveries the analgesia does not modify the neonatal metabolism, but can be a reason of delayed adaptation of extrauterine life.

Early use of Nasal-BiPAP in two infants with Congenital Central Hypoventilation syndrome.

AIM: To reduce the problems caused by prolonged artificial ventilation in babies with Congenital Central Hypoventilation syndrome (CCHS). METHODS: Two term infants with CCHS, weighing 4030 g and 3100 g, respectively, at the beginning of treatment and aged 53 and 31 d, respectively, were successfully ventilated with a Nasal Bilevel Positive Airway Pressure (N-BiPAP) device. RESULTS: In the first patient the tcPO2 recordings (mean +/- SD) during sleep were 46 +/- 12 mmHg before using N-BiPAP and 58 +/- 13 mmHg after using the device, while those for tcPCO2 were 75 +/- 9 mmHg and 49 +/- 11 mmHg, respectively. In the second patient tcPO2 during sleep was 42 +/- 3 mmHg before, and 55 +/- 5 after N-BiPAP, and for tcPCO2 the recordings were 119 +/- 24 mmHg and 55 +/- 6 mmHg, respectively, showing a significant improvement. One infant had persistent gastro-oesophageal reflux, and frontal skin abrasion caused by the face mask. Nevertheless, these complications did not necessitate the discontinuation of N-BiPAP ventilation, thus precluding prolonged use of intubation and tracheotomy. CONCLUSION: In infants with CCHS, early use of non-invasive, positive-pressure ventilation with N-BiPAP, in association with careful monitoring, can decrease problems caused by prolonged intubation and tracheotomy.

Autoimmune haemolytic anaemia in a newborn infant.

The case is reported of an infant with autoimmune haemolytic anaemia of perinatal onset. Combined treatment with steroids and cyclosporin was necessary to improve haemolysis and reduce the high transfusion requirements. Treatment was discontinued at 13 months of age. The child was healthy at the follow up at 24 and 36 months of age.

[Pneumothorax during nasal-CPAP: a predictable complication?]. / Pneumotorace in corso di CPAP nasale. E una complicanza prevedibile?

OBJECTIVE: Pneumothorax (PNX) is a relatively common complication of nasal-CPAP (N-CPAP). Aim of the study was to identify prognostic factors of its onset. METHODS: Seventy-seven newborns, admitted from January to December 2002 to the Neonatal Intensive Care Unit of Brescia, who were treated with N-CPAP with Infant Flow System as first intention, were included. Gestational age and birth weight were (mean +/- SD) 33.7 +/- 3.02 weeks and 2.047 +/- 684 grams, respectively. Infants were put on N-CPAP at 2.7 +/- 4.1 hours of life. The duration of treatment was 27.7 +/- 27.7 hours. RESULTS: Fifty-one neonates improved and N-CPAP was discontinued, 26 worsened and required intubation and mechanical ventilation. Eight of them developed PNX (10,3%). No significant differences were found among the three groups (improved, worsened without PNX and worsened with PNX) concerning mode of delivery, gestational age, birth weight and blood gases. The patients with PNX needed a FiO2 28% higher than the initial value after 12 hours of treatment, and 46% higher at 24 hours (p = 0,017). At diagnosis, FiO2 was 53,5% higher than the initial value (p = 0,005). CONCLUSION: A 40% increase of FiO2, during the first 24 hours of N-CPAP may represent an useful marker to identify the infants at high risk of developing a pneumothorax.

Practical applications of monitoring respiratory mechanics in newborn.

The authors evaluate the use of data supplied by common systems used to calculate respiratory mechanics in newborns in order to analyse the impact on the management of patients requiring assisted ventilation. Over the past few years, the sale of respiratory monitors that are easier to use and less complicated to manage, as well as being less expensive, has meant that nearly every Neonatal ICU can now determine, in real time, both the causes of respiratory insufficiency and the conditions of the newborn lung during mechanical ventilation to ensure improved adaptation of ventilatory support. Leaving aside a discussion of respiratory physiopathology and the principles regulating pulmonary ventilation, the authors focus on the practical effect that data normally supplied by this type of apparatus (airway pressure, inspiration and expiration airflow, expiration minute volume calculated for each respiratory act, tidal volume, system leaks and dynamic compliance) have on the ventilatory "setting" chosen by the clinician or on the diagnosis of some commonly found situations during neonatal mechanical ventilation. The experience described concerns the impression of clinicians, skilled in the management of patients receiving conventional artificial ventilation but not so attracted by respiratory physiopathology, regarding the value of such instruments. The unanimous positive opinion confirms the thesis whereby respiratory monitoring can certainly help the neonatologist make a real time evaluation of both the causes leading to respiratory failure and the effect on the lung of any therapeutic decisions.

[Experience with the use of a computerized data processing program on solutions for parenteral nutrition of neonates]. / Esperienza dell'impiego di un "foglio elettronico" computerizzato per l'elaborazione delle soluzioni per nutrizione parenterale nei neonati.

BACKGROUND: The preparation of a PN (parenteral nutrition) solution in neonatal and paediatric Departments is an operation of both critical importance and great difficulty (need of time to do it; complex calculations in order, for example, to subtract takes of nutrients from other sources; etc.). MATERIALS AND METHODS: With the aim to speed such a proceeding, the authors (from a 1000 deliveries/year NICU) developed a computerised system for the preparation of PN mixtures, based on a common and easily available "spreadsheet". RESULTS: The results of its application on a population of preterm babies in which parenteral and enteral (minimal enteral nutrition etc.) feedings were contemporarily provided, are illustrated. Advantages by using this software are the lower cost if compared with other dedicated softwares on sale and its great simplicity and versatility. The program is easy to use eve if different feeding plans are required; the data obtained at the end of the study demonstrated the effectiveness of the software in correctly calculate food components dosages. CONCLUSIONS: Using producer's data on feedings composition and up-to-date parenteral nutrition schedules, a reliable composition of the obtained PN mixtures was eventually achieved, as demonstrated by growth and blood samples.

[Toxocariasis: a case report]. / Toxocariasi: descrizione di un caso.

The Authors present a case of infestation by Toxocara canis occurring in a eighteen months' child. A first time he was hospitalised for wheezing, reported hypereosinophilia and a thoracic X-ray with a soften congestion in the right lung's base. Two months later he was admitted to hospital for the persistence of high level of eosinocyte. The clinical tests and the patient's history explain the first symptoms like the passage of the Toxocara's larvae in the lungs, but don't justify the anomalous behaviour of eosinocytes. In fact the hypereosinophilia was present two months before the admission in our Department, but two tests, executed during the first stay in hospital, didn't show abnormality. A few months after, another blood's sample showed an high level of eosinocytes and, in the same time, a new control of thoracic X-ray, didn't show any change in the image.

[The Schönlein-Henoch syndrome with severe multisystemic involvement]. / Sindrome di Schönlein-Henoch con severo interessamento multisistemico.

A case of Henoch-Schönlein purpura (HSP) characterized by several unusual complications and exceedingly prolonged course is reported. A 6-year-old boy, hospitalized with a typical clinical picture of HSP, developed after a few days a severe gastrointestinal vasculitis leading to digestive hemorrhages and food intolerance. The treatment included continuous gastrointestinal infusion, parenteral nutrition, and corticosteroids during the exacerbations of symptoms. Intestinal ultrasonography showed diffuse submucous edema and widespread mural hematomas; hydrops of the gallbladder and edematous swelling of the pancreas were also detected. A gastro-duodenoscopy revealed diffuse and severe mucosal vasculitis with ulcers and petechial lesions. One week after admission the occurrence of weight gain, hyponatremia, and hypoprotidemia in the absence of proteinuria suggested a protein-losing enteropathy. The elevation of serum and urine amylases was consistent with pancreatic involvement. The course was characterized by recurrent exacerbations of gastrointestinal manifestations; symptoms subsided slowly to such a degree that oral feeding could be gradually restarted only seven weeks after admission. The use of high-frequency intestinal ultrasonography proved useful and sensitive in monitoring the evolution of intestinal involvement. During hospitalization the child complained of acute painful scrotal swelling. Ultrasonography showed scrotal hemorrhage and testicular swelling but no signs of testicular torsion, thus helping to avoid surgical exploration. In the recovery phase the child complained of colicky abdominal pain with vomiting. A plain abdominal roentgenogram showed bilateral staghorn pelvic urolithiasis. This finding was confirmed by an intravenous pyelography which also revealed a bilateral pyeloureteritis with edema of the ureteral wall and partial stenosis of the lumen.(ABSTRACT TRUNCATED AT 250 WORDS)

Equinococosis hidatidica pulmonar. / Pulmonary hydatid echinococcosis

Se presentan 43 casos de quistes hidatidicos de pulmon intervenidos en el Hospital Zonal de Esquel desde el 1o. de julio de 1977 hasta el 31 de diciembre de 1981. La incidencia de la localizacion pulmonar con respecto al total fue del 33%. Se destaca la importancia del examen catastral radiologico y seroinmunologico. Se recomiendan las tecnicas quirurgicas conservadoras que tratan la periquistica. Se comunican 2 casos poco frecuentes: 1 quiste hidatidico de pleura parietal y un quiste multivesicular del pulmon

Equinococosis hidatidica pulmonar. / Pulmonary hydatid echinococcosis

Se presentan 43 casos de quistes hidatidicos de pulmon intervenidos en el Hospital Zonal de Esquel desde el 1o. de julio de 1977 hasta el 31 de diciembre de 1981. La incidencia de la localizacion pulmonar con respecto al total fue del 33%. Se destaca la importancia del examen catastral radiologico y seroinmunologico. Se recomiendan las tecnicas quirurgicas conservadoras que tratan la periquistica. Se comunican 2 casos poco frecuentes: 1 quiste hidatidico de pleura parietal y un quiste multivesicular del pulmon

Genetic and hormonal characterization of cryptic 21-hydroxylase deficiency.

Cryptic 21-hydroxylase deficiency has been previously described in asymptomatic family members of patients with classical congenital adrenal hyperplasia (CAH). These family members were detected by high baseline 17-hydroxyprogesterone levels found in the course of family studies. The hormonal responses to ACTH of the family members with cryptic 21-hydroxylase deficiency were determined and compared to the responses of patients with CAH, patients with acquired adrenal hyperplasia, family members predicted to be heterozygous for CAH, family members predicted to be unaffected, and the general population. The ACTH-stimulated levels of 17-hydroxyprogesterone and delta 4-androstenedione in the cryptic family members were elevated above the level of the general population or family members heterozygous for classical CAH, but below that of patients with CAH. The hormonal profile of patients with cryptic 21-hydroxylase deficiency is similar to that of patients with acquired adrenal hyperplasia. The response of family members heterozygous for the cryptic gene (21-OH CRYPTIC/21-OH NORMAL) was indistinguishable from that of family members heterozygous for the classical CAH gene (21-OH CAH/21-OH NORMAL). These studies support our previous proposal that patients with cryptic 21-hydroxylase deficiency are genetic compounds, having one gene for a severe enzyme deficiency and one gene for a mild 21-hydroxylase deficiency. Thus, the 21-hydroxylase genotype in cryptic 21-hydroxylase deficiency is 21-OH CAH/21-OH CRYPTIC.

HLA genotypes and HLA-linked genetic markers in Italian patients with classical 21-hydroxylase deficiency.

HLA genotype and HLA-linked marker data for 40 unrelated patients from central Italy and 2 unrelated patients from Sardinia with congenital adrenal hyperplasia due to 21-hydroxylase deficiency (21-OH-def) were analyzed. The results confirm that the HLA-linked 21-OH-def gene is associated with several different HLA determinants and complete HLA haplotypes, although the only determinant with significantly increased frequency was the complement C2 allele C2B. The HLA antigens B8 and DR3 were found in significantly decreased frequencies. The haplotype A3, Cw6, Bw47, BfF, DR7, which is exceptionally rare in the general population but which has been found in many other 21-OH-def patients from diverse geographical origins, was also found in one of the Italian patients. This and other HLA haplotype associations found among the Italian patients may represent mutations that have occurred on HLA haplotypes with genetic linkage disequilibrium or, alternatively, may represent mutations that have not yet had time to become randomly associated with different HLA complex determinants. The marked negative associations with B8 and DR3 could, however, result from an interaction between the gene products of the HLA complex and the 21-OH-def phenotype.

Cryptic 21-hydroxylase deficiency in families of patients with classical congenital adrenal hyperplasia.

Serum androgens and 17-hydroxyprogesterone concentrations and HLA genotypes were determined in 124 families of patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH). In 8 pedigrees, we discovered 16 pubertal or postpubertal family members of either sex who had biochemical evidence of 21-hydroxylase deficiency but were without clinical symptoms of excess virilism, amenorrhea, or infertility. We designated these family members as individuals with cryptic 21-hydroxylase deficiency. Within each generation, the family members with cryptic 21-hydroxylase deficiency were HLA identical. It is proposed that these family members are genetic compounds, having 21-hydroxylase deficiency as a result of two recessive gene defects: 1) a severe 21-hydroxylase gene defect present in the index case with classical CAH (21-OHCAH) and 2) a mild 21-hydroxylase gene defect (21-OHCRYPTIC). Thus, the CAH genotype in the family members with cryptic 21-hydroxylase deficiency is 21-OHCAH/21-OHCRYPTIC. Lod score analysis for linkage between the cryptogenic 21-OH trait and HLA gave a combined Lod score for males and females of theta = 0.00 of 3.409. Close genetic linkage between HLA and 21-OHCRYPTIC was thus established. This study provides support for the previously reported heterogeneity of 21-hydroxylase deficiency which may result from allelic variability at the locus for steroid 21-hydroxylase.