Mid-childhood outcomes after pre-viable preterm premature rupture of membranes.

OBJECTIVES: Investigate cardiorespiratory outcomes in children surviving previable preterm premature rupture of membranes (PV-PPROM) before 22 weeks' gestational age (GA) with minimum 2 weeks latency. STUDY DESIGN: Single institution, follow-up of retrospectively identified children who were born after PV-PPROM during 2000-2004, and individually matched preterm-born controls. RESULTS: Eleven PV-PPROM and matched control children were included at mean age of 10.5 and 10.7 years. Rupture of membranes occurred at mean GA 182 and 276 weeks and birth at 283 and 286 weeks, respectively. Compared to controls, the PV-PPROM group had significantly poorer lung function, findings on echocardiography indicating mild pulmonary hypertension, and lower peak oxygen consumption. Chart reviews suggested more motor difficulties and a tendency towards more problems with learning and attention. CONCLUSION: The findings highlight a preterm-born sub-group in need of targeted long-term monitoring and possibly interventions regarding future cardiorespiratory and neurodevelopmental function.

Preterm elective caesarean section and early enteral feeding in gastroschisis.

AIMS: To evaluate the effect of elective caesarean section (CS) before term and early enteral nutrition on length of parenteral nutrition and hospital stay in infants with gastroschisis. METHODS: Retrospective review of all infants with gastroschisis treated in a regional level III hospital from 1993 to 2008. During 1993-97, there was no established standard for management of pregnancy or delivery while a protocol on close foetal monitoring and early elective CS was adhered to for 1998-2008. Introduction of human milk on the first day after complete closure of the abdominal wall and rapid increase was the policy during the whole period. RESULTS: With early elective CS, no foetal deaths occurred after 28-week gestational age (GA). Ten infants were born during the first period and 20 during the second period at a median GA (range) of 36.5 (34-40) and 35 (34-37) weeks (p = 0.013). Seven and 20, respectively, were born by CS. Median (range) days before full enteral feeds and hospital stay were 11.5 (7-39) and 13.0 (7-46) (p = 0.85), and 17.5 (12-36) and 22.5 (13-195) (p = 0.67), respectively. One child died of volvulus after discharge. CONCLUSION: Close surveillance of pregnancy, elective preterm caesarean section, early surgery and active approach to primary closure and early enteral feeds appears to be a safe and effective line of management in gastroschisis.

Neonatal mortality and morbidity in extremely preterm small for gestational age infants: a population based study.

AIM: To assess if growth restricted (small for gestational age, SGA) extremely preterm infants have excess neonatal mortality and morbidity. METHODS: This was a cohort study of all infants born alive at 22-27 weeks' post menstrual age in Norway during 1999-2000. Outcomes were compared between those who were SGA, defined as a birth weight less than the fifth percentile for post menstrual age, and those who had weights at or above the fifth percentile. RESULTS: Of 365 infants with a post menstrual age of <28 weeks, 31 (8%) were SGA. Among infants with a post menstrual age of <28 weeks, only chronic lung disease was associated with SGA status (OR 2.7, 95% CI 1.0 to 7.2). SGA infants with a post menstrual age of 26-27 weeks had excess neonatal mortality (OR 3.8, 95% CI 1.3 to 11), chronic lung disease and a significantly higher mean number of days (age) before tolerating full enteral nutrition. SGA infants with a post menstrual age of 22-25 weeks had an excess risk of necrotising enterocolitis. CONCLUSION: Extremely preterm SGA infants had excess neonatal mortality and morbidity in terms of necrotising enterocolitis and chronic lung disease.

Congenital absence of the nose: a case report and literature review.

A case of congenital absence of the nose is presented. The etiology of this rare condition is unknown. A review of the literature reveals that the previously applied terms, e.g. 'arhinia', are unclear. In the reviewed cases there seems to be a pattern of facial anomalies associated with nasal absence. In most cases, one could probably expect a lack of the olfactory bulbs and tracts. We suggest a new terminology and summarize the aims of the radiological evaluation of this condition.

[Treatment of extremely premature infants--which limits and what problems?]. / Behandling av ekstremt premature born--kva grenser og kva problem?

In Norway about 300 babies with a birthweight of 500-999 grams and 200 with a gestational age of 24-27 weeks are born annually. This gives a total of 320-330 babies who come under the definition of extreme prematurity. Obstetric care is important in order to avoid premature birth, or, when premature birth is unavoidable, to find the optimal time and place for delivery. There is ongoing discussion of what constitutes reasonable lower limits for offering life support. The survival rate for babies born before 24 gestational weeks is low and the risk of serious neurological damage high. The risk of severe lung disease is reduced with pre- and postnatal treatment with corticosteroids and postnatal treatment with surfactant, but excessive corticosteroid treatment may possibly increase the risk of neurological damage. Despite major recent progress in the treatment of the preterm baby, significant understanding of why disabilities occur is lacking.

[Diagnosis and treatment of congenital hyperinsulinism--to Paris at any price?]. / Undersøkelse og behandling av barn med kongenitt hyperinsulinisme--til Paris for enhver pris?

BACKGROUND: Persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) is a hyperfunctional disorder of pancreatic insulin-producing cells with hypertrophic beta-cells present either focally or diffusely. With an estimated frequency of 1:50,000 live births, Norway will on average have one case per year. It is clearly difficult to maintain expertise in diagnostics and treatment with such a low incidence. MATERIAL AND METHODS: We report three Norwegian patients with PHHI who were successfully treated at Hôpital des Enfants Malades in Paris. RESULTS: Two patients were shown to have focal hyperinsulinism treated with partial pancreas resection. After follow-up of three and a half and two years respectively, these patients have normal glucose tolerance and exocrine pancreatic function. One patient with diffuse hyperinsulinism was operated with subtotal (90%) pancreatectomy. At 2.5-years follow-up this patient has slight glucose intolerance whereas her fasting blood glucose is low normal. The exocrine pancreatic function is normal. INTERPRETATION: Patients with PHHI should be referred to a centre where the possibility of focal hyperinsulinism can be thoroughly explored.

Familial non-immune hydrops fetalis and congenital pulmonary lymphangiectasia.

UNLABELLED: We report on three siblings with non-immune hydrops fetalis. Congenital pulmonary lymphangiectasia was diagnosed in two of them. One of these, a girl still alive and suffering from frequent airway infections, has bilateral pleural effusions and distal congenital lymphoedema. CONCLUSION: To our knowledge, this is the first report of non-immune hydrops fetalis and congenital pulmonary lymphangiectasia occurring in siblings.

Evaluation of a protocol for selecting fetuses in breech presentation for vaginal delivery or cesarean section.

OBJECTIVE: Our purpose was to evaluate, with respect to obstetric intervention and neonatal outcome, a protocol for selecting fetuses in breech presentation for vaginal delivery or cesarean section. STUDY DESIGN: A clinical follow-up study was performed between 1984 and 1992 of all term singleton deliveries in breech presentation. Each case selected for vaginal delivery had a matched control in vertex presentation. RESULTS: A total of 1212 infants presented as breech. Vaginal delivery increased from 45% to 57% (p = 0.004), and cesarean section for failure of vaginal delivery declined from 21% to 6% (p < 0.00001). None, however, died or had long-term sequelae because of a complicated or failed vaginal breech delivery. A total of 8.8% of those delivered vaginally in breech versus 5.0% of those in vertex presentation were admitted to the neonatal intensive care unit (p = 0.009). Among those with vaginal delivery, 2.5% in breech presentation were given the clinical diagnosis of birth asphyxia versus none in the vertex position (p = 0.0001). CONCLUSION: Breech presentation at term may be selected for vaginal delivery if properly managed.

Echogenic material in the fetal gallbladder and fetal disease.

The presence of echogenic material within the gallbladder is probably a rare finding in the fetus, and the list of predisposing factors known for postnatal life seems not to be applicable to prenatal diagnosis. In the present study 1656 obstetric scans were performed on referrals to the Unit of Fetal Medicine. No echogenic gallbladder contents were found before 28 weeks. In the subgroup of 523 fetuses who were examined during gestational weeks 28-42, six fetuses were found to have echogenic material in the gallbladder. The echogenicities were found in patients who had the following: extra-amniotic hematoma with intrauterine growth retardation and oligohydramnios, tetralogy of Fallot, trisomy 21 with atrioventricular septal defect and transient ascites, early abnormally distended fetal gallbladder, chromosomal aberration (translocation 10; 11) with bilateral clubfoot, and gastroschisis. Echogenic densities had disappeared at ultrasound scans performed during early postnatal life in four cases, were absent at five weeks in one case, and still present as calculi 8 months after birth in one case. Although no causative conclusions can be drawn from the report, these conditions are suggested as possible predisposing factors for the presence of echogenic material in the fetal gallbladder.

[Diabetes mellitus in pregnancy]. / Diabetes mellitus hos gravide.

The authors highlight some aspects of diabetes mellitus that complicate pregnancy. Several complications, e.g. hypoglycaemia, hyperglycaemia and macrosomia are described briefly. Macrosomia can be diagnosed by ultrasound examination, which should be performed every other week from the 24th week of gestation. Accelerated abdominal circumference (> or = 1.2 cm/week) between 32 and 39 weeks and excess thickness of soft tissue over the proximal humerus of the foetus after the 32nd week (> 13 mm at term) may imply development of macrosomia. The elevated risk related to adiposity and poor metabolic control can be avoided by intensive treatment. Intensive metabolic treatment can also reduce the frequency of preeclampsia and polyhydramnion. Ketoacidosis and intrauterine foetal death may be consequences of poor diabetic control. The authors discuss infectious problems, some aspects of treatment, e.g. risk of preterm delivery, dietary treatment and insulin, indications for delivery and various neonatal problems.

[Pregnancy in diabetes]. / Svangerskapsdiabetes.

The authors review various aspects of gestational diabetes, including definition, screening, diagnostic procedures, complications (hypertension, macrosomia), clinical evaluation (ultrasound, non-stress test), treatment (diet, insulin), indications for delivery and neonatal aspects (hypoglycaemia, hypocalcaemia). Complications can be reduced by intensive dietary treatment and insulin. If the gestational diabetes is regulated well the woman can wait for spontaneous birth at term. In the case of pregnant women with less than optimal regulated diabetes, however, or with complications such as hypertension, macrosomia, previous stillbirth, labour can be induced preterm by local administration of prostaglandin or infusion of oxytocin. Physical training and weight reduction should be instituted to avoid later development of type II diabetes mellitus. There is still some uncertainty about different aspects of gestational diabetes.

[High-frequency oscillation in newborn infants. A promising therapeutic principle when respirator therapy is hazardous]. / Høgfrekvent oscillering av nyfødde. Eit lovande behandlingsprinsipp ved vanskeleg respiratorbehandling.

14 newborn infants were ventilated with oscillation because of severe respiratory distress syndrome (n = 3), pulmonary air leaks (n = 4), pulmonary hypoplasia (n = 4) or sepsis and pneumonia (n = 3). All but four of the infants were more easily stabilized by oscillation than by conventional ventilation. Four infants survived after 1-5 (mean three) days of oscillation, and none developed severe chronic lung disease. New commercial ventilators make this mode of ventilation of newborn infants relatively simple. Small premature babies who require high pressures with conventional ventilation, babies with pulmonary air leaks and babies with hypoplastic lungs may benefit from oscillation.

[Prenatal prevention of respiratory distress syndrome in newborn infants]. / Prenatal forebygging av åndenødssyndrom hos nyfødte.

This article reviews the embryology, pathophysiology, clinical aspects and prophylactic treatment of respiratory distress syndrome (RDS). Prenatal prophylaxis with corticosteroids is indicated for gestational ages between 24 and 32 weeks, even if the effect of prophylaxis is uncertain before the 28th week. Prophylactic treatment is less important between the 32nd and 34th gestational week. Hypertension is not considered a contraindication, but premature rupture of the membranes is a relative contraindication. Corticosteroid treatment may be given, however, in combination with antibiotic prophylaxis.

Ganciclovir therapy of congenital cytomegalovirus disease.

A newborn with cytomegalovirus disease with cerebral involvement was treated with ganciclovir for 21 days. The treatment resulted in only temporary cessation of virus shedding in the urine and probably had no ameliorating effect on the long-term clinical outcome.

[Arterial catheters in newborn infants]. / Arteriekateter hos nyfødde.

The article summarizes one year's experience of arterial lines in at third-level department for newborn children. We successfully placed an arterial line in 69 out of 71 children. 13 out of 84 arterial lines were umbilical catheters, the others were introduced percutaneously in the radial or posterior tibial arteries. Both umbilical arterial and peripheral arterial lines were functioning satisfactorily, with very few complications, and no serious ones. An arterial line is a relatively simple and a safe method for good monitoring of newborn children in need of intensive care.

[Clinical manifestations caused by group A streptococci]. / Kliniske sykdomsbilder forårsaket av gruppe A-streptokokker.

We describe the symptomatology of different disease entities caused by group A beta-hemolytic streptococci (Streptococcus pyogenes, GAS). The case histories of four patients, two of whom died, emphasize the severity of certain clinical manifestations of GAS-infections. A 34 year-old woman was admitted to hospital four days after start of the symptoms. She presented a clinical picture very similar to that observed in fulminant meningococcal septicaemia; i.e. extensive skin haemorrhages, circulatory collapse, and multiple organ failure. She died within 12 hours of admission. GAS were isolated in blood culture. A seven day-old girl died before admission to hospital. GAS were isolated in blood cultures, cerebrospinal fluid and from her nose and throat. An eight year-old, psychomotoric retarded girl developed a severe left-sided pneumonia, empyema and scarlatina. GAS were detected in throat culture. She responded poorly to high doses of benzylpenicillin given intravenously. She recovered rapidly after thoracotomy and decortication of her left lung. Finally, we describe the case of an 11 year-old boy with rheumatic fever without cardiac involvement. The reported cases underline the need for careful diagnosis and penicillin treatment in cases of GAS-infections.