The position of the water blow-off or safety valve in C.P.A.P.-systems.

After investigating aspects of a single nasal cannula system we conclude that the pressure read-out point should be located as close to the application point as possible; furthermore the water blow-off or safety valve should be positioned before (or at) the pressure application point.

2-Mercaptoethanesulfonate-cysteine disulfide excretion following the administration of 2-mercaptoethanesulfonate--a pitfall in the diagnosis of sulfite oxidase deficiency.

In the urine of a neonate with respiratory insufficiency and convulsions a positive sulfite reaction was found, which is suggestive of sulfite oxidase deficiency. The nitroprusside reaction also was positive. More detailed investigations showed that both tests were positive due to the administration of 2-mercaptoethanesulfonate, a mucolytic drug. The patient's urine contained an acidic amino acid with a column chromatographic behaviour like S-sulfocysteine. The high-voltage electrophoretic mobility was slightly different. This compound was isolated from the urine and identified as the mixed disulfide of 2-mercaptoethanesulfonate and cysteine. Its identity was proven with field desorption mass spectrometry, a technique which is suitable for the analysis of sulfonic acid derivatives.

Retinopathy of prematurity: review of a four-year period.

For a four-year period the development of retinopathy of prematurity (ROP) was determined among neonates considered at risk of acquiring this condition. Fifty-six out of 249 premature infants developed some degree of ROP. Comparison of these infants with a group of 56 controls, admitted to hospital in the same period and matched for sex, birth weight, and gestational age, showed significant differences for sepsis, blood transfusions, and the period of oxygen monitoring in relation to the period of oxygen administration. The most consistent factor associated with the development of ROP was gestational age at the time of birth, though no gestational age group was entirely devoid of ROP. This suggests that screening for ROP should not be restricted to high-risk premature infants only.

Long term follow up of premature infants: detection of strabismus, amblyopia, and refractive errors.

AIM: To establish recommendations for long term ophthalmological follow up of prematurely born infants. METHODS: 130 infants with a gestational age (GA) <37 weeks and born between 1 November 1989 and 31 October 1990 were enrolled in a prospective study about the development of strabismus, amblyopia, and refractive errors. Infants were subdivided in three groups according to GA: A <28 weeks (n=32), B >/=28-32-<37 weeks (n=34). Ophthalmological assessment took place at the postconceptional age of 32 weeks, at term and at 3, 6, 12, and 30 months post term. At the age of 5 years parents received a questionnaire and a majority of the children was examined again (n=99). RESULTS: At the age of 5 years 46 infants were known to have strabismus (n=29) and/or amblyopia (n=22) and/or refractive errors (n=22). Statistical analysis showed that gestational age, duration of supplementary oxygen, and duration of hospitalisation were important predictive variables for the development of strabismus, amblyopia, or refractive errors (SAR) at the age of 5 years (p<0.05). Infants with a GA 32 weeks, who developed an incidence comparable with the normal population. Strabismus developed mainly in the first year of life and at the age of 5 years. Most infants with amblyopia were detected at the age of 2-3 years. Refractive errors were found in the first year of life and at the age of 2.5 and 5 years. CONCLUSION: Infants with a GA <32 weeks should be selected for long term ophthalmological follow up. These infants should be screened at the age of 1 year, in the third year of life (preferably at 30 months), and just before school age (including testing of visual acuity with optotypes).

Early neonatal group B streptococcal disease: degree of colonisation as an important determinant.

During a period of 5 years (1 January 1977 to 1 January 1982) 145 infants colonised with group B streptococci (GBS) were admitted to the neonatal intensive care unit of the University Children's Hospital, Utrecht. In 87 of these infants (60%) vertical transmission of GBS was established; in 43 of these 87 infants (49%) the degree of colonisation was moderate to heavy. Early-onset (EO) GBS disease arose in 21 of 145 infants (attack rate: 14.5%). Of the 43 infants moderately to heavily colonised with GBS, however, 19 suffered from EO GBS disease (attack rate: 44.2%), whereas there were only two cases among the 44, lightly colonised infants (attack rate: 4.5%), a highly significant difference (P less than 0.0005). Similarly, probable sepsis (PS), defined as signs and symptoms of sepsis but without positive blood cultures, was observed significantly more often in moderately to heavily colonised infants (15/43, attack rate: 34.9%) compared with those lightly colonised (4/44, attack rate: 9.1%) (P less than 0.005). Infants moderately to heavily colonised with GBS at birth appear to have a significantly higher risk of developing serious GBS disease (EO or PS) than do infants only lightly colonised.

Intra-uterine tachycardia associated with multicystic encephalomalacia (MCE).

Multiple cystic brain lesions in neonates have been described as a result of a variety of causes. All events described thus far in association with multicystic encephalomalacia (MCE) seem to point to hypoxic-ischaemic injury as the common factor for this particular form of central nervous system damage. We describe a neonate in whom repeated, prolonged episodes of intrauterine tachycardia had been documented. Congestive heart failure and fetal hydrops were present at birth and MCE at the age of 13 wk. The obvious relationship between this child's intrauterine tachycardia and his MCE points to the fact that any fetus with prolonged tachycardia should be considered at risk of severe brain damage.

Hydrops fetalis associated with red cell pyruvate kinase deficiency.

A hydrops fetalis and multicystic encephalomalacia were diagnosed in a neonate who was one of twins. The co-twin had died 5 weeks prior to delivery. The most likely explantation for both hydrops and multicystic encephalomalacia was fetal anemia caused by a red cell pyruvate kinase deficiency, and aggravated by an intrauterine disseminated intravascular coagulation.

Prematures with and without regressed retinopathy of prematurity: comparison of long-term (6-10 years) ophthalmological morbidity.

Forty-two ex-prematures having had regressed forms of retinopathy of prematurity (ROP) during the neonatal period were compared with 42 matched non-ROP ex-premature controls at ages 6 to 10 years. The overall incidence of ophthalmologic problems was 55% in the ROP and 36% in the non-ROP group. Strabismus, amblyopia, and refraction anomalies occurred more frequently in the ROP group. In this group, a significantly greater number of children had decreased vision in one or both eyes compared to the non-ROP group. Although the incidence of ophthalmologic problems in the ROP group was considerably higher, the incidence in the non-ROP group still turned out unexpectedly high. this implicates that non-ROP very low birth weight neonates should also be considered to be at a greatly increased risk for later visual problems. Regular ocular examinations, mandatory for the ROP group, thus should be included in follow-up programs of non-ROP very low birthweight neonates as well.

New developments in neonatology: less severe retinopathy of prematurity?

PURPOSE: To determine the effects of surfactant replacement therapy (SRT), high-frequency oscillatory ventilation (HFOV), and general improvements in quality of care on the incidence of severe retinopathy of prematurity (ROP). METHODS: Retrospective comparison of the incidence and severity of ROP in two groups of preterm infants admitted to our neonatal intensive care unit (NICU) in two consecutive 5-year periods (1986-1995) and screened for ROP. During the second study period, natural surfactant was introduced in the treatment of respiratory distress syndrome (RDS) and HFOV was used for treatment of respiratory insufficiency of any origin. The effects of these developments and general improvements on the incidence of severe ROP were analyzed with stepwise logistic regression. RESULTS: The overall incidence of ROP from 1986-1990 was not significantly different from the incidence of ROP from 1991-1995. The incidence of severe ROP (ROP stage 3 or greater) was significantly lower in the second period (15.7% versus 6.4%, P=.015). For infants <1000 g, the incidence of overall ROP was increased significantly during the second study period (47.6% versus 60.1 %, P=.045), although the incidence of severe ROP remained unchanged. Only SRT was associated with a decreased risk for severe ROP; HFOV and general improvements in quality of care had no influence on the outcome. In patients with RDS, the incidence of severe ROP decreased significantly during the second period. CONCLUSION: Of the recent new developments in neonatology, only SRT was associated with a decreased risk for severe ROP.

Screening for retinopathy of prematurity: do former guidelines still apply?

Early detection of retinopathy of prematurity (ROP) in premature and very-low-birth-weight infants is crucial. In this retrospective study, 581 infants either with a birth weight below 1500 g or a gestational age of less than 32 weeks, or who did not fit these criteria but were judged to be at increased risk, were screened for ROP. ROP developed in 159 (27.4%). The incidence of ROP appeared to be inversely proportional to birth weight and gestational age. Infants with a birth weight below 750 g had a significantly higher risk of developing stage 3 and 4 ROP. The mean age at detection was 7.6 +/- 1.6 weeks. Nearly all of the ROP cases and all of the stage 3 and 4 cases were detected between the 5th and 10th week. Because screening should be focused on these vision-threatening stages, ophthalmic examinations should be concentrated in, but not limited to, the period between the 5th and the 10th postnatal week.

Why is there an increase in ROP in recent years?

During a 12 year period (1975-1987) the incidence of blindness and partial sight due to ROP in the Netherlands was constant at approximately 3/100,000 live births. However the incidence appears to be rising since. Probable causes are an increasing number of twin and triplet births associated with assisted reproduction, an increasing mean maternal age, but the largest contribution is formed by the growing number of "relatively low risk" children not referred to university clinics due to screening problems.

[Retinopathy in premature infants]. / Retinopathie van de prematuur geborene.

Retinopathy of prematurity (ROP) is found in about 30% of premature infants with a birth weight < 1500 g and/or a gestational age < 32 weeks. Many risk factors are directly or indirectly involved in the development of ROP. The younger the child and therefore the more immature at birth, the greater the risk of ROP leading to short-sightedness or blindness. As a result of advances in obstetric and neonatal care, more immature and extremely low birth weight infants survive, and thorough ophthalmological screening for ROP is therefore essential. Timely detection (ROP stage 3) enables treatment with cryotherapy or laser therapy. Although the rate of success has increased with these forms of treatment, about 40% of the treated children retain a serious visual handicap. In the Netherlands, this involves about 10 children each year. At a later age, prematurely born children have a higher risk of developing other ophthalmologic problems such as strabismus, amblyopia and refractive errors. Again, timely detection and treatment reduces the risk of permanent visual disability.

[A baby who is too pink]. / Een te roze baby.

A 5 week old preterm boy born by caesarean section on maternal indication at a gestational age of 33 weeks is described, who developed a symptomatic zinc deficiency. He developed diarrhea, dermatological problems and convulsions. IV-zinc supplements and subsequent oral zinc medication induced a complete remission of symptoms. Pathofysiologic sequences as well as disease states (sub-normal intake before and after birth, decreased absorption and increased loss) that can lead to zinc deficiency are discussed. To prevent a deficiency, an intake of 300-450 mcg/kg/day i.v. of elementary zinc in case of total parenteral nutrition and 700 mcg/kg/day p.o. in case of oral feeding is recommended.

[Premature retinopathy: recommendations for a screening policy]. / Prematuren retinopathie: aanbevelingen voor het screeningsbeleid.

Few data exist from which an optimal strategy for retinopathy of prematurity (ROP) screening can be deduced. This strategy should compromise between the dual purpose of screening, to wit monitoring the incidence of ROP and determining an optimal moment for therapeutic intervention. We recorded the timely incidence and course of ROP in all except four premature infants admitted during a 1-year period. The study results indicated that a single screening moment is not likely to detect more than 70% of all cases. When relying, however, on a single screening the investigation should be performed between the 7th and 9th week. The unpredictable course of ROP, varying between early starting, rapidly progressing forms and very late starting, usually benign forms, probably precludes the possibility of 100% ROP detection. Screening during the 4th to 5th, 8th and 11th week probably gives the best chances for determination of an optimal moment for treatment and approximation of the incidence.

[The retinopathy of prematurity]. / Prematuren-retinopathie (retinopathy of prematurity, ROP).

Throughout a four years period the development of retinopathy of prematurity (ROP) was determined amongst neonates considered 'at risk' for acquiring this condition. Fifty-six out of 249 neonates developed some degree of ROP. Comparison of the ROP-group with a group of 56 controls (admitted in the same period and matched for sex, birth-weight and gestational age) showed significant differences for sepsis, blood-transfusions and the period of O2-monitoring/period of O2-administration. The most consistent factor to which the development of ROP related was gestational age at the time of birth; no gestational age group however turned out to be absolutely devoid of ROP. This suggests that screening for ROP should not be restricted to 'high-risk' neonates only.

[A newborn infant with the xxxxy-syndrome]. / Een pasgeborene met het XXXXY-syndroom.

In a three weeks old infant with dysmorphic features a 49, XXXXY karyotype was demonstrated from chromosome preparations of lymphocytes. In the literature only a few newborn infants have been described with this syndrome. The most frequent symptoms of the syndrome in older patients are mental retardation, dysmorphic signs, hypogonadism and skeletal malformations. In our patient we found a low birth weight, a peculiar facies, in addition to a patent ductus arteriosus, a scoliosis and normal external genitals. The most typical skeletal malformations may develop at a more advanced age.

[A patient with neonatal citrullinemia]. / Een patiënt met neonatale citrullinemie.

In a four days old neonate, showing feeding difficulties, temperature imbalance, convulsions and coma, citrullinaemia was diagnosed. Subsequent treatment, consisting of protein-restriction, exchange transfusions, arginine suppletion and using an alternative pathway for waste nitrogen excretion (through sodium benzoate administration), resulted in a stable clinical and biochemical condition. Aged ten months, our patient shows a normal growth with psychomotor retardation probably linked with the perinatal events. Because it seems possible nowadays to prolong survival in neonates with inborn errors of urea synthesis, prompt and correct diagnosing of these disease entities becomes urgent in view of the long-term prognosis for the children involved.

[Treatment results in children with very low birth weight (less than or equal to 1500 gram)]. / Behandelingsresultaten van kinderen met een zeer laag geboortegewicht (minder dan of gelijk aan 1500 gram).

A retrospective study of morbidity and mortality in the neonatal period was carried out in 170 neonates of low birthweight (less than or equal to 1500 grams), who were admitted to the neonatal intensive care unit of the Wilhelmina Kinderziekenhuis in Utrecht in the years 1976 to 1979; 118 of them survived (69.4%). The survival of children born by caesarean section (n = 32, 84.3%) and of small for gestational age children (n = 18, 83.2%) was higher than the total survival. Respiratory problems were most frequent (n = 151, in 82 of them mechanical ventilation was necessary) and were the most important cause of death. In 92 of the 118 surviving children (ages 1 1/2-3 1/2 years) a follow up study could be done; 42 were considered normal; 3 children had problems due to congenital malformations; 27 children had minor problems (squint and/or emotional disturbances) which were thought to be related to the neonatal period; 11 children had minor handicaps, 9 were severely handicapped.

[Persistent fetal circulation syndrome: long-term clinical and treatment results]. / Het persisterende foetale circulatiesyndroom: kliniek- en behandelingsresultaten op de lange termijn.

The definition, pathophysiology, signs and symptoms, diagnostic and therapeutic procedures of the persistent fetal circulation syndrome are presented. A description of the clinical characteristics of 38 patients, admitted during a 3 years period, is included. The results of a follow-up investigation amongst the 20 surviving children are discouraging, because a maximum of only 5 children appears to be totally normal. At least half of the children is more or less handicapped, the majority of which severely!

[Lung sequestration; a rare congenital lung disorder]. / De longsekwester; een zeldzame congenitale longaandoening.

Pulmonary sequestration is a rare congenital malformation. It is characterized by an abnormal arterial supply and venous drainage and a part of the lung which is not taking part in ventilation. Asymptomatic cases have been reported, but 37% of intralobar and 60% of extralobar sequesters became symptomatic before the age of ten years and need to be removed surgically. In this article one case-report illustrates the clinical course described in most cases whilst the other describes a patient in which the course mimicked an 'untreatable' bronchiolitis.