[A boy with pruritus and blisters]. / Een jongen met jeuk en blaren.

A 4-year-old boy came to the emergency department because of pruritus and multiple tense bullae over his entire body, including his ears, hands, feet and perineum. Biopsy revealed the diagnosis: linear IgA bullous dermatosis.

Changes in neonatology: comparison of two cohorts of very preterm infants (gestational age <32 weeks): the Project On Preterm and Small for Gestational Age Infants 1983 and the Leiden Follow-Up Project on Prematurity 1996-1997.

OBJECTIVE: To determine changes in peri- and neonatal care concerning neonatal mortality and morbidity by comparing 2 cohorts of very prematurely born infants (gestational age [GA] <32 weeks), 1 from the 1980s and 1 from the 1990s. METHODS: The Leiden Follow-Up Project on Prematurity (LFUPP-1996/97), a regional, prospective study, includes all infants who were born alive after a GA <32 weeks in 1996 and 1997 in the Dutch health regions Leiden, The Hague, and Delft. The Project On Preterm and Small for Gestational Age Infants (POPS-1983), a national, prospective study from the presurfactant era, includes all liveborn infants <32 weeks' GA and/or <1500 g from 1983 (n = 1338). For comparison, infants from the POPS-1983 cohort with a GA <32 weeks from the same Dutch health regions were selected (n = 102). RESULTS: The absolute number of preterm births in the study region increased by 30%: 102 in 1983 to on average of 133 in 1996-1997. Centralization of perinatal care improved: the percentage of extrauterinely transported infants decreased from 61% in 1983 to 35% in 1996-1997. A total of 182 (73%) of the LFUPP-1996/97 infants were treated antenatally with glucocorticosteroids compared with 6 (6%) of the POPS-1983 infants. A total of 112 (42%) of the LFUPP-1996/97 infants received surfactant. In-hospital mortality decreased from 30% in the 1980s to 11% in the 1990s. Mortality of the extremely preterm infants (<27 weeks) decreased from 76% to 33%. The incidence of respiratory distress syndrome remained the same: approximately 60% in both groups. Mortality from respiratory distress syndrome, however, decreased from 29% to 8%. The incidence of bronchopulmonary dysplasia increased from 6% to 19%. For the surviving infants, the average length of stay in the hospital and the mean number of NICU days stayed approximately the same ( approximately 67 days total admission time and 44 NICU days in both groups); including the infants who died, the mean NICU admission time increased from 27 days in the 1980s to 41 days in the 1990s. Equal percentages of adverse outcome (dead or an abnormal general condition) at the moment of discharge from hospital were found (+/-40% in both groups). CONCLUSIONS: An increase in the absolute number of very preterm births in this study region was found, leading to a greater burden on the regional NICUs. Improvements in peri- and neonatal care have led to an increased survival of especially extremely preterm infants. However, increased survival has resulted in more morbidity, mainly bronchopulmonary dysplasia, at the moment of discharge from the hospital.

Follow up of children after antenatal treatment for alloimmune thrombocytopenia.

OBJECTIVE: To evaluate the long-term follow-up of surviving offspring after antenatal treatment for fetal or neonatal alloimmune thrombocytopenia (FNAIT). PATIENTS: Fifty children at risk of FNAIT were antenatally treated with maternal intravenous immunoglobulins (IVIG) (n=11), IVIG with intrauterine platelet transfusions (IUPT) (n=26) or IUPT alone (n=9). In four cases (n=4), only fetal blood sampling (FBS) was performed. One child died in the neonatal period and one was lost to follow up. METHODS: The remaining 48 children, aged 1.3-11.6 years (median 5.1 years), were given both general and neurological examinations and assessed on their development and susceptibility for infections or atopic constitution. In addition, immunoglobulin levels were measured in 17 infants, aged 5 years and older. RESULTS: Intracranial hemorrhage (ICH) was not observed. The general health and neurodevelopmental outcome in the children was comparable to a normal Dutch population. Children not exposed to maternal IVIG treatment had significantly more infections and hearing problems than children exposed to IVIG treatment or the normal population. Immunoglobulin G, A and M levels were within the normal range, independent of treatment and severity of FNAIT. A high IgE level was more frequently seen in children exposed to IVIG, but did not result in clinical consequences such as allergy or atopy. CONCLUSIONS: Antenatal treatment of children for FNAIT did not affect general health or neurodevelopmental outcome. In particular, exposure to IVIG in utero showed no adverse effect on the clinical outcome of these children.

Mortality and neurologic, mental, and psychomotor development at 2 years in infants born less than 27 weeks' gestation: the Leiden follow-up project on prematurity.

OBJECTIVE: To determine the outcome of infants with a gestational age (GA) <27 weeks, born in the mid-1990s. DESIGN: Regional, prospective study; part of the Leiden Follow-Up Project on Prematurity. SETTING: Three health regions in The Netherlands. PATIENTS: A total of 266 live born infants (1996/1997) with GA <32 weeks; 46 infants were <27 weeks. MAIN OUTCOME MEASURES: Neurologic examination (according to Hempel) and assessment of mental and psychomotor development using the Bayley Scales of Infant Development I, at the corrected age of 2 years. RESULTS: Mortality was 35% (16 of 46) <27 weeks, compared with 6% (14 of 220) in infants with GA 27 to 32 weeks; withdrawal of treatment in 60% and 43%, respectively. Below 27 weeks mortality was higher after extra-uterine transport and pregnancy induction. Neonatal morbidity was higher in infants <27 weeks compared with infants 27 to 32 weeks. Below 27 weeks postnatal use of dexamethasone and being hospitalized at term were associated with abnormal neurologic outcome; there was a higher incidence in (mild) mental developmental delay compared with the older infants. Adverse outcome (dead or abnormal neurologic, psychomotor, or mental development) in infants 23 to 24, 25, 26, and 27 to 32 weeks GA was, respectively, 92% (11 of 12), 64% (7 of 11), 35% (8 of 23), and 18% (40 of 220). CONCLUSIONS: Mortality and neonatal morbidity were higher in infants with GA <27 weeks compared with infants born between 27 and 32 weeks. The high adverse outcome of infants <25 weeks suggests that one should carefully weigh whether or not to aggressively resuscitate and treat these extremely premature infants.

Developmental outcome at 18 and 24 months of age in very preterm children: a cohort study from 1996 to 1997.

OBJECTIVE: To determine the effect of prematurity (gestational age (GA) < 32 weeks) on developmental outcome at the corrected age of 18 and 24 months in a regionally defined, prospective cohort study. STUDY DESIGN: The Leiden Follow-Up Project on Prematurity (LFUPP) includes all live-born infants < 32 weeks GA, born in 1996/1997 in three Dutch health regions (n=266). Mental and psychomotor developmental indices (MDI, PDI) were determined with the Bayley Scales of Infant Development I: > or = -1 S.D.: normal, -2 to -1 S.D.: moderate delay and < -2 S.D.: severe delay. RESULTS: At 18 months 168 (71%) and at 24 months, 151 children (64%) of 235 survivors were assessed. Moderate to severely delayed mental and/or psychomotor development occurred in 40% of the children at both ages. Children lost to follow-up were of lower socioeconomic status and more frequently of non-Dutch origin. Since non-Dutch origin negatively affected the outcome at both test ages, availability of the data of these children would probably have worsened the outcome. Postnatal treatment with dexamethasone was associated with an increased risk of delayed development. Other independent predictors of delayed development were bronchopulmonary dysplasia at 18 months and ethnicity, maternal age at birth, birthweight and gender at 24 months. After adjustment for these other predictors of delayed development, the mean PDI of dexamethasone-treated infants was 16.1 points lower than of non-treated infants at 18 months (p=0.03) and 12.7 points lower at 24 months (p=0.04). CONCLUSIONS: At 18 and 24 months corrected age, 40% of the very prematurely born children had both delayed mental and/or psychomotor development. Treatment with dexamethasone postnatally was a major risk factor for delayed (psychomotor) development.