[Quality evaluation of a neuro-development follow-up program of very low birthweight infants]. / Valoración de la calidad de un programa de seguimiento del neurodesarrollo de prematuros de muy bajo peso al nacimiento.

INTRODUCTION: Advances in perinatology have been associated with improved survival of very low birth weight (VLBW;<1500 g) infants. These children have an increased risk of future neurological and development handicaps. A set of quality indicators have been recommended for the neurodevelopment follow-up programs in this group of patients. AIM: To analyze our neurodevelopment follow-up program according to these quality of care indicators in 5 post-discharge follow-up areas: general care; physical health; vision, hearing, speech and language; developmental and behaviour assessment; and psychosocial issues. PATIENTS AND METHODS: Fifty-one VLWB patients born in 2000 who have completed our 6-year follow-up program have been retrospectively studied. The agreement between our program and the standard of quality is evaluated. RESULTS: A copy of a discharge summary of the patient's neonatal course is present in the medical record in 92% of the cases. The inclusion of clinical data and general medical aspects in the medical summary is heterogeneous. Somatometric evaluation for the first two years of age is always present in a filled-in graphic. The international classification of the retinopathy of the preterm (RDP) is used in 100% of the patients. An ophthalmologic examination for RDP is performed in 72% (37/51) of the infants before hospital discharge; of the other 28%, only in 6 patients the date of the outpatient examination is present. Specific evaluations and interventions are performed within the recommended period of time in most of the children with suspected or neurosensory handicaps. More than ninety per cent of the patients are referred to the neurodevelopment specialist and most of them go to the visit (90.2% between 9 and 15 months and 78.4% between 21 and 30 months). Recommendations for a psycho-educational assessment are followed in the extremely premature (BW<1000 g and/or 28 weeks of gestational age) children. The number of patients who follow the program decreases with time. Psychosocial evaluation does not usually appear in the medical records of the patients. CONCLUSION: We have a good adherence to most of the recommended indicators in our follow-up program. The evaluation of these indicators is a useful tool to analyze the quality of a developmental follow-up program and to improve it.

[Progressive familial intrahepatic cholestasis presenting as liver failure]. / Fallo hepático neonatal como forma de presentación de colestasis intrahepática familiar progresiva.

Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of autosomic-recessive inherited cholestatic disorders that begin in the neonatal period or in the first years of life. There are three types of PFIC defined by different mutations located in the gene responsible for the bile flow through the intrahepatic canalicular transporter system. These disorders usually present in children or young adults and the main clinical manifestations are cholestasis, jaundice and pruritus, and they progress slowly towards liver fibrosis in adult life. PFIC diagnosis is based on clinical suspicion, biochemical findings (that include normal gamma-glutamyl transpeptidase in type 1 and 2, but increased levels in type 3), image techniques that rule-out other disorders, and histological confirmation. Initial treatment consists of symptomatic relief of cholestatic symptoms with choleretic agents (urso-deoxycholic acid). Partial biliary derivation and ileal bypass are intermediate therapeutic options. In case of no response to these treatments, liver transplantation is indicated. We report the case of a neonate with PFIC type 2 presenting as a liver failure.

[Scrotal edema: a rare complication of percutaneous central venous atheters]. / Edema escrotal neonatal: rara complicación de catéter venoso central percutáneo.

The use of percutaneous central venous catheters in neonatal intensive care units is becoming increasingly common. Numerous studies support the safety and effectiveness of the use of these catheters for the infusion of parenteral nutrition or medication. We describe a male patient with a gestational age of 32 weeks who showed swelling of the external genitals during the fifth day of life. The etiology was initially thought to be infectious. A review of X-rays revealed the introduction of the silastic catheter to the spermatic vessels. When the catheter was withdrawn, the genital swelling disappeared in a few hours. The most frequently described complications associated with percutaneous central venous catheters are infectious. Other complications that have been described are thrombosis, embolism, and perforation of the catheter with leakage of fluid to the extravascular space. The case described herein is an unusual complication that has not been previously described and resulted from poor positioning of the catheter tip. Diagnostic delay can cause serious complications.

[Underwater delivery. Consensus of the Spanish Neonatology Society and the Perinatal Section of the Spanish Obstetrics and Gynecology Society]. / Atención del parto en el agua. Consenso de la Sociedad Española de Neonatología y de la Sección de Medicina Perinatal de la Sociedad Española de Obstetricia y Ginecología.

Immersion in water during labor and delivery as an alternative to traditional delivery is a practice that has increased in many countries. This technique is effective in reducing pain and duration of labor. The American Academy of Pediatrics and The American College of Obstetricians and Gynecologists have published a clinical report which indicates the potential maternal benefits during the first stage of labor but, questions the performance of this technique during delivery and birth of the newborn. In this report, the Spanish Society of Neonatology and the Spanish Society of Obstetrics and Gynecology analyze the current scientific evidence on water immersion delivery, and the impact this practice could have in the mother and especially in the wellbeing of newborn.

[Retinopathy of prematurity: casuistics between 1996 and 2001]. / Retinopatía del prematuro. Casuística de los años 1995-2001.

BACKGROUND: Retinopathy of prematurity (ROP) is a cause of neurosensorial morbidity. OBJECTIVES: To study the incidence, associated risks factors, treatment, and outcome of ROP in premature infants born at less than 32 weeks in our hospital. METHODS: We performed a descriptive study of patients born between the January 1, 1995, and December 31, 2001, in Sant Joan de Déu Hospital in Barcelona (Spain) at <= 32 weeks of gestation who survived until their first month of life. An ocular evaluation was performed between weeks 4 and 6 of life and was repeated every 1-2 weeks until retinal vascularization was complete. Ocular sequelae and visual function were evaluated. Bivariate comparison of groups with and without ROP was performed. RESULTS: Of the 324 patients evaluated, 74 presented ROP (22.8 %), of which 63 patients (21.7 %) were classified as stage 1 or 2 and 11 (3.7 %) as stage 3. An inverse correlation between the incidence of retinopathy and weight and gestational age was found. Threshold disease (3 plus) was detected in 9 patients (16 eyes; 3.1 % of the study sample and 12.1 % of the neonates with retinopathy). All of these neonates were treated with laser therapy. Ocular sequelae were mild in 2.7 % of the patients, moderate in 0.6 % and severe in 0.6 %. The visual function (n 236) of infants with ROP (n 74) was altered in 4 patients (1.7 %). Of these, alterations were severe in 2 patients (0.8 %). Bivariate analysis revealed significant differences (p < 0.001) in low birth weight, gestational age, days of oxygen therapy, days of mechanical ventilation, days of antibiotic therapy, and number of blood transfusions. CONCLUSIONS: In this study the incidence of ROP was similar to that in other centers. Development of ROP was strongly associated with its various risk factors. Severe stages were not seen above 30 weeks of gestational age. The results of laser therapy were optimal, with fewer alterations in ocular examination and visual function than those estimated in patients without treatment.

[Neurological follow-up of very low birth weight newborns at the age of two years (1998-1999)]. / Seguimiento neurológico de recién nacidos menores de 1.500 gramos a los dos años de edad.

BACKGROUND: Because of improved obstetric and neonatal care, there is growing interest in the later outcome of very low birth weight newborns. OBJECTIVES: The aim of this study was to evaluate the survival rate of very low birth weight newborns and to identify disabilities at the age of 2 years. MATERIAL AND METHODS: An observational, follow-up study was performed of neonates with a birth weight of under 1,500 grams born between 1998 and 1999. The follow-up program included pediatric, maturative, neurological, psychological, ophthalmological, and audiological evaluation. Neurosensorial disabilities were classified as mild, moderate, or severe. RESULTS: One hundred thirty-six very low birth weight newborns were admitted. The survival rate was 77.9 % and 83.9 % completed the follow-up to the age of 2 years. The neurosensorial disability rate was 20.2 %; disability was severe in 9 %, moderate in 1.1 %, and mild in 10.1 %. In patients lost to follow-up, birth weight was higher, gestational age was older, and sonographic findings were more frequently normal. CONCLUSION: Survival in very low birth weight newborns has increased with improved neonatal care. The presence of sequelae was similar to that found in other follow-up studies. A substantial number of patients were lost to follow up, which influenced the disability rates.

[Neonatal seizures, our experience]. / Convulsiones neonatales, experiencia en la unidad.

AIM: To study retrospectively all newborns admitted between 1992 and 1998 in our Neonatology Unit with convulsions before 30 days of life. PATIENTS AND METHODS: 77 patients, 63.6% male, with an average gestational age of 36.42 weeks, and an average birth weight of 2,653 g. Apgar score at five minutes was below 4 in 4 newborns. RESULTS: In 35 patients the first convulsion occurred in the first 48 hours of life, 7 patients showed status epilepticus. There was a single type of seizure in 49 newborns (65.3%), specially tonic and clonic (multifocal and focal). Head ultrasound was abnormal in 33 newborn, CT scan and/or MRI abnormal in 24, EEG was pathologic in 55. Phenobarbital was the initial treatment in 81.8%, half of them required a therapeutical change. 34 patients (55.7%) still received treatment at hospital discharge. The etiology was hypoxic ischaemic encephalopathy (39%), though less important than in previous studies, intracranial hemorrhage (13%), central nervous system malformations (9%), among other. 16 patients died, 35 showed an abnormal outcome (45.4%): cognitive delay in 24, epilepsy in 19, motor abnormalities in 12. CONCLUSION: The worst outcome was found in malformations, meningitis, intracranial hemorrhages, metabolic diseases and hypoxic ischaemic encephalopathy, while the best in hypoglycemia and in benign idiopathic neonatal seizures.

[The newborn infant at neurologic risk in the year 2000. Recommendations for the follow-up, incorporation of new instruments]. / Recién nacido de riesgo neurológico en el año 2000. Recomendaciones para el seguimiento, incorporación de nuevos instrumentos.

INTRODUCTION: Perinatal mortality has dropped markedly in recent years. However, there is still a considerable prevalence of neurological sequelas. Symptoms may present during the first months of life or later. Thus it is necessary to follow-up children with a clinical history of risk of neurological disorders. DEVELOPMENT: The various programmes for follow-up show slight variations in criteria of inclusion, calendar and methods of evaluation. We report the results of our follow-up of children at risk: long and short term sequelae in children of very low birth-weight and their correlation with neuroimaging findings. We review the use of some investigations (clinical examination, cranial ultrasound, CAT, magnetic resonance, EEG, visual and auditory evoked potentials and different biological, hemodynamic and nerve damage markers. CONCLUSIONS: It is necessary to: 1. Restrict the criteria for inclusion in hospital follow-up programmes; 2. Match a suitable investigative calendar to the pathology of the newborn baby; 3. Coordinate with the pediatricians of primary care areas and centres of early health care; 4. Use specific instruments for detection of mild sequelae at an earlier age; 5. Prolong follow-up at least until the age of 6-7 years old, and 6. To seek new biological markers to allow early detection of brain damage.

[The value of granulocyte elastase in the early diagnosis of infantile meningitis]. / Utilidad de la elastasa granulocitaria en el diagnóstico precoz de las meningitis infantiles.

Elastase-alpha 1-proteinase inhibitor complex (E-alpha 1-PI) was evaluated in blood and cerebrospinal fluid in 176 patients between 1 month and 15 years old. They were divided in three groups: group 1 (n = 61) children without meningitis, group 2 (n = 69) non bacterial meningitis and group 3 (n = 46) bacterial meningitis. The CSF values of E-a 1-PI complex (P50: P2.5; P97.5) (micrograms/L) were (2.85: 0.1; 19.1) in group 1 (31.5: 0.88; 735) in group 2 and (247: 2.6; 10370) in group 3. Significant differences were found in it (p less than 0.001). We didn't find any differences between non bacterial meningitis group and bacterial meningitis group when the granulocytes count were below 100 X mm3. The blood values of E-a 1-PI complex (P50: P2.5; P97.5) (micrograms/L) were (1122: 337; 5005) in group 3, significantly greater (p less than 0.001) than in group 2 (346: 136; 1612). In CSF E-alpha 1-PI complex showed us a sensitivity of 89% and a specificity of 27.7% in the diagnosis of bacterial meningitis, while in blood stream, it was 85.3% and 85.4 respectively.

[Elastase-alpha 1-antiprotease in neonatal sepsis]. / Elastasa-alpha 1-antiproteasa en la sepsis neonatal.

Elastase-alpha 1-proteinase inhibitor complex (E-alpha 1-PI) was evaluated in 682 blood samples from 516 newborn infants. They were divided into three groups: control (group 1; n = 99), non-infectious (group 2; n = 338) and infectious (group 3; n = 82). The plasma values of E-alpha 1-PI complex (median; minimum-maximum, in microgram/L) were 180; 46-296 in group 1, 337; 40-2524 in group 2 and 954.5; 183-8160 in group 3. Significant differences were found between the groups (p < 0.001). E-alpha 1-PI complex in blood showed a sensitivity of 75%, specificity of 81.9%, positive predictive value of 30.6% and negative predictive value of 96.8% for the diagnosis of neonatal infection.

[Congenital diaphragmatic hernia: evaluation of two ways of management]. / Hernia diafragmática congénita: análisis de dos formas de tratamiento.

OBJECTIVE: The aim of this study was to analyze the treatment and evolution of congenital diaphragmatic hernia in the last 16 years, distinguishing two ways of management, and to look for parameters that can predict the evolution. MATERIALS AND METHODS: Between 1978 and 1994, 29 cases of congenital diaphragmatic hernia were treated in our NICU. During the first period (1978-1988) 17 cases (group 1) were treated after birth as a surgical emergency. In the second period (1989-1994), preoperative stabilization was performed before surgery (12 cases, group 2). Two cases of group 2 were excluded because of the association of other malformations that were the cause of death. RESULTS: Both groups were similar in gestational age, birth weight and Apgar score at 5 minutes. Overall mortality was 48.1% (47.0% in group 1 and 50.0% in group 2). No infants with PaCO2 greater than 40 mmHg and OI greater than 40 or VEI over 1,000 survived. CONCLUSION: Despite preoperative stabilization, there is no difference in the mortality rate of the two groups. With congenital diaphragmatic hernia, as with other entities of low incidence, collaborative studies are needed to obtain enough cases to analyze the results more precisely.