Survival of a very low‐birthweight infant with Potter sequence on long‐term hemodialysis

Recent advances in neonatal cardiorespiratory management and neonatal renal replacement therapy have led to occasional reports of favorable short‐term and long‐term outcomes for Potter sequence, once thought to be fatal. The present patient was a girl born at a gestational age of 34 weeks 4 days with a birthweight of 1398 g. She was diagnosed with Potter sequence complicated by pulmonary hypoplasia due to left renal agenesis and small right kidney. Hemodialysis was started because anuria persisted even after persistent pulmonary hypertension receded and cardiorespiratory status improved. Peritoneal dialysis during the clinical course failed to achieve stability because of pericatheter leakage and frequent obstruction of the peritoneal dialysis catheter; in the meantime, hemodialysis was also performed. Once bodyweight passed 3.5 kg, peritoneal dialysis became more feasible with stability; the patient was discharged at 9 months and at the time of writing was on peritoneal dialysis at home, and renal transplantation was planned.     

Follow-up of infants with bilateral renal disease detected in utero. Growth and renal function

We studied 69 infants who had urinary tract abnormalities detected by antenatal ultrasound examination. There were 21 intrauterine or immediate neonatal deaths; in all 21 infants, severe bilateral renal disease incompatible with life was found at autopsy. Six of the live-born infants with abnormal results of antenatal ultrasound examinations had a normal urinary tract after birth. Of the remaining 42 infants, the prenatal diagnosis was confirmed with renal ultrasound and other studies during the first week of life. Twenty-one of 42 infants had bilateral renal disease. We obtained follow-up data on 19 of 21 of these infants. Twelve of 19 had obstructive uropathy that was treated surgically. After one to 51 (mean, 18) months of follow-up, renal function varied. Ten of 19 patients had a calculated glomerular filtration rate greater than or equal to 79 mL/min/1.73 m2. One infant required long-term ambulatory peritoneal dialysis. Renal function (glomerular filtration rate, 74 +/- 5 mL/min/1.73 m2) and growth (height percentile, 41 +/- 8) were unexpectedly good considering the severity of the urinary tract abnormalities. Prenatal detection of bilateral renal disease followed by careful medical and surgical management results in a favorable outcome with good growth and renal function.     

Renal replacement therapies in pediatric intensive care patients: Experiences of one center in Turkey

BACKGROUND: Despite constant improvements in caring for critically ill neonates and infants with congenital cardiac disease, sepsis, bone marrow and solid organ transplantation, acute renal failure (ARF) is an important problem in these children. ARF, severe fluid overload and inborn errors of metabolism are some of the indications for acute dialysis in infants and children. METHODS: The authors had retrospectively evaluated the medical records of Pediatric Intensive Care Unit, Ankara University School of Medicine, Ankara, Turkey patients who had required acute renal replacement therapy between the dates of January 2002 to February 2005. RESULTS: Medical records of 332 patients were reviewed. Acute renal replacement therapy was performed in 21 patients (6.3%; mean age, 9.6 +/- 7.4 years). Dialysis modalities were peritoneal dialysis in 15 patients (71.4%; mean age, 3.9 +/- 5.6 years) and hemodialysis in six patients (28.6%; mean age, 12.1 +/- 3.2 years). A total of 90% of patients had severe systemic disease leading to ARF. A total of 95% of patients had multiple organ dysfunction syndrome. The most common cause of ARF was refractory shock. At the beginning of renal replacement therapy, 10 patients were anuric, nine patients had volume overload, seven patients had decompensated metabolic acidosis and nine patients had hypotension. The average dialysis period was 4.7 +/- 6.4 days. Mortality rate was 66.7%. Eight patients recovered from ARF and chronic renal failure had developed in one patient. CONCLUSION: In the Pediatric Intensive Care Unit, ARF is frequently seen together with multiple organ dysfunction syndrome and the mortality rate is high. Both peritoneal dialysis and hemodialysis are important renal replacement treatment modalities in patients with ARF. The age and hemodynamic status of the patients are important when choosing treatment modality; generally peritoneal dialysis is preferred in infants and toddler, while hemodialysis is preferred in older children.     

Renal development in the fetus and premature infant

Congenital abnormalities of the kidney and urinary tract (CAKUT) are one of the leading congenital defects to be identified on prenatal ultrasound. CAKUT represent a broad spectrum of abnormalities, from transient hydronephrosis to severe bilateral renal agenesis. CAKUT are a major contributor to chronic and end stage kidney disease (CKD/ESKD) in children. Prenatal imaging is useful to identify CAKUT, but will not detect all defects. Both genetic abnormalities and the fetal environment contribute to CAKUT. Monogenic gene mutations identified in human CAKUT have advanced our understanding of molecular mechanisms of renal development. Low nephron number and solitary kidneys are associated with increased risk of adult onset CKD and ESKD. Premature and low birth weight infants represent a high risk population for low nephron number. Additional research is needed to identify biomarkers and appropriate follow-up of premature and low birth weight infants into adulthood.     

Renal impairment in very low birthweight infants following antenatal indomethacin administration

Three cases of neonatal renal insufficiency in very low birthweight (VLBW) infants following repeated antenatal administration of indomethacin to prevent premature labor are reported. Three pregnant women received indomethacin (total doses of 150–850 mg) for 3–14 days from admission until delivery. The gestational ages and birthweights of the infants ranged from 24 to 28 weeks and 612 to 1432 g, respectively. Oliguria, early onset of hyperkalemia and prolonged renal dysfunction occurred after birth. Renal failure did not improve in one infant. Despite the efficacy of indomethacin for tocolysis in premature labor, VLBW infants born after repeated maternal administration near the time of delivery may have developed impairment of the premature kidney.     

Congenital pulmonary lymphangiectasis

Background  Congenital pulmonary lymphangiectasis (CPL) characterized by dilatation of pulmonary lymphatic vessels occurs as a congenital anomaly. With poor prognosis, neonatal presentation of bilateral CPL is associated with the severe compromise of pulmonary gas exchange and high mortality. Methods  A male infant born at 39 weeks of gestation was found to have CPL. Cyanosis and cardiac arrest occurred a few minutes after birth, and the symptoms remained after artificial ventilation. The infant died of hypoxemic cardiac failure 45 minutes after birth. Autopsy showed neither pleural effusion nor valvular abnormalities. Results  Microscopically dilated vessels with lymphatics were seen in the lung of the infant. Atelectasis, CPL, inhalation of amniotic fluid, partial hydropic degeneration of hepatic cells, and scrotal edema were diagnosed. Conclusion  With regard to treatment and prognosis, CPL must be distinguished from interstitial emphysema and other diseases.     

Exomphalos major: the Northern Ireland experience

Purpose  In exomphalos major (EM), closure of the defect in the abdominal wall presents a challenge. The aim of this study is to evaluate a single centre experience of EM. Materials  A 15-year retrospective case-note review; data presented as median (range). Results  Fourteen infants (7 female) were born with EM: birth weight 2.9 (1.2–3.8) kg, gestational age 38 (31–39) weeks. One infant died in utero and one within the first hour of life. Severe pulmonary hypoplasia was present in 7/13 (54%), and there was a mortality of 6/13 (46%) live births. Infants were treated non-operatively primarily. Two infants underwent early surgery: one infant, born with a ruptured sac, had a surgical silo constructed on day 1 and closure on day 8, while a second infant had partial closure (skin only) on day 11. Ten infants had application of silver sulphadiazine to the sac 2–3 times per week. Enteral feeds were established soon after birth. They were discharged from hospital to allow granulation. Ventral hernia closure was performed on a subsequent admission. Conclusions  Exomphalos major can be successfully treated non-operatively, allowing immediate enteral feeding and early discharge while granulation takes place. In this series, exomphalos major has an incidence of 1 in 26,000, mortality is 46% and severe pulmonary hypoplasia is present in 54% of infants.     

Effects of birth weight,gestational age,and maternal obstetric history on birth prevalence of cerebral palsy.

A register of children with cerebral palsy born to mothers resident in the Mersey region from 1966 to 1977 was compiled from health service records. Frequency distributions and prevalences of birth weight and gestational age differed for those with hemiplegia, diplegia, and quadriplegia. In particular, the children with diplegia showed a bimodal frequency distribution. Children of normal birth weight with diplegia had a higher prevalence of severe mental retardation than those of low birth weight. These differences may be due to survival bias and may not be of aetiological importance. Furthermore, the mothers of diplegic infants had a significantly higher proportion of spontaneous abortions, stillbirths, and low birthweight infants in their obstetric history. This suggests that prenatal factors predominate in the aetiology of diplegia.     

Urinary excretion of 5-L-oxoproline (pyroglutamic acid) during early life in term and preterm infants

Urinary 5-L-oxoproline was measured in term and preterm infants from shortly after birth until 6 weeks of postnatal age to determine their ability to synthesise glycine. In term infants the excretion was five to 10 times that seen in normal adults, increasing from 105 µmol/mmol creatinine in the first 72 hours after birth to 170 µmol/mmol creatinine at 6 weeks of age. There was a significant inverse linear correlation between the excretion of 5-L-oxoproline and length of gestation or birthweight. By 6 weeks of age there was no longer a significant difference in 5-L-oxoproline between term and preterm infants. There was no difference in the excretion of 5-L-oxoproline between boys and girls, or between infants fed on human milk or an artificial formula.  If, in part, variability in the excretion of 5-L-oxoproline is determined by the extent to which the endogenous formation of glycine is adequate, then glycine formation may be marginal during early life, more so in preterm than in term infants, providing additional evidence that glycine is a conditionally essential amino acid in the neonate.     

Pretransplant peritoneal dialysis and graft thrombosis following pediatric kidney transplantation: a NAPRTCS report

Graft thrombosis is a common cause of graft failure in pediatric renal transplantation. Several previous studies, including a North American Pediatric Renal Transplant Cooperative Study (NAPRTCS) review of pretransplant dialysis status and graft outcomes, have described a potential correlation of peritoneal dialysis (PD) and graft thrombosis. This issue is of particular concern for pediatric transplant programs as more than 65% of children with end stage renal disease are treated with PD. We reviewed 7247 pediatric renal transplants performed between 1987 and 2001. Thrombosis was the cause of graft loss in 2.7% (199) of all the transplants performed. Among failed transplants, thrombosis was the third most common cause of graft loss in both index (11.6%) and subsequent transplants (14.5%). Thrombosis becomes the most common cause of graft failure (21%, 61/294) if one looks at transplants in the later cohort, from 1996 to 2001. This change is primarily because of a decrease in the incidence of acute rejection. In the PD group, 3.4% of all grafts were lost as a result of thrombosis. This compares with 1.9% in the hemodialysis group, 2.4% in the pre-emptive transplant group, and 4.1% among patients who received both dialysis modalities. There was a statistically significant difference in thrombosis failure risk in the different dialysis groups (p = 0.005) with those who received only peritoneal dialysis having the highest risk. Additional significant risk factors for graft thrombosis included; cadaver donor source (p < 0.001), cold ischemia time >24 h (p < 0.001), history of prior transplant (p < 0.001), donor age <6 yr (p < 0.001), and >5 pretransplant blood transfusions (p = 0.02). Using stepwise proportional hazards modeling, only pretransplant peritoneal dialysis, >24 h cold ischemia time, prior transplant, and donor age <6 yr were simultaneously associated with an increased risk of thrombosis. We conclude that pretransplant PD is associated with an increased risk of graft thrombosis. Special precautions should be undertaken in pediatric renal transplant patients who have received PD, especially infants and young children.     

Retinopathy of prematurity in infants weighing 1000-1499 g at birth

Objectives: To review the incidence and severity of retinopathy of prematurity (ROP) in infants with birthweights 1000-1249 g and 1250-1499 g, to establish whether the upper weight limit for routine ophthalmological examination might safely be lowered. Methodology Prospective cohort study of infants born between 1 January 1977 and 31 December 1992 cared for in the neonatal nurseries at the Royal Women's Hospital, Melbourne. Data were retrieved on 1373 infants who survived their initial hospitalization. They comprised 657 with birthweights 1000-1249g (group 1) and 716 with birthweights 1250-1499g (group 2). There were 76 outborn infants in group 1 and 97 in group 2; the remaining infants were all born at the Royal Women's Hospital. Ocular examinations commenced at 2 weeks of age, when possible, and at 2-weekly intervals after that. Results In group 1, ROP was detected in 14.6% (96/657) and severe ROP (bilateral stage 3-5) in 5.0% (33/657). Five (0.8%) children required surgical intervention (reaching threshold disease); following surgery, one was legally blind, one had severely impaired vision, and the other three had near-normal vision. Another child was blind; he was born at 28 weeks gestational age with a birthweight of 1170g, and was transferred to a Level II hospital at 9 weeks chronological age with no detectable retinopathy. He returned 1 year later totally blind with detached retinae (grade 5 ROP). The prevalence of bilateral blindness in this group was 0.3% (2/657). In group 2, ROP was detected in 6.4% (46/716) and severe ROP in 0.8% (6/716). No children required surgery; three were found to be myopic at follow up but the corrected visual acuity was normal. No children in group 2 were blind. No significant difference was found between the rates of ROP in inborn and outborn infants. Conclusion In neonatal units with similar rates of ROP and visual outcome, routine ophthalmological examination in the neonatal nursery of infants weighing more than 1249g at birth is probably unnecessary.     

Isolated congenital tracheal stenosis in a preterm newborn

Severe tracheal stenosis, resulting in functional atresia of the trachea is a rare congenital malformation with an estimated occurrence of two in 100,000 newborns. If no esophagotracheal fistula is present to allow for spontaneous breathing, this condition is usually fatal. We report on a male infant born at 32 weeks of gestation. The patient presented with respiratory distress immediately after delivery due to severe congenital tracheal stenosis resulting in functional atresia of the trachea. Endotracheal intubation failed and even emergency tracheotomy did not allow ventilation of the patient lungs. The patient finally succumbed to prolonged hypoxia due to functional tracheal atresia. The etiology of tracheal atresia and tracheal stenosis is still unclear, but both conditions are frequently combined with other anomalies of the VACTERL (vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal/radial anomalies and limb defects) and TACRD (tracheal agenesis, cardiac, renal and duodenal malformations) association. Conclusion Successful treatment of severe congenital tracheal stenosis and tracheal atresia depends on either prenatal diagnosis or recognition of this condition immediately after birth to perform tracheotomy without delay. Nevertheless, despite any efforts, the therapeutical results of severe tracheal stenosis and tracheal atresia are still unsatisfactory.     

Contemporary neonatal outcome following rupture of membranes prior to 25 weeks with prolonged oligohydramnios

Background

Prolonged oligohydramnios following early preterm prelabour rupture of membranes (PPROM) is traditionally associated with high neonatal mortality and significant risk of pulmonary hypoplasia. However, recent evidence points to an apparent improvement in outcome.

Aims

To document current neonatal outcomes following rupture of membranes prior to 25 weeks with severe persistent oligohydramnios and a latency to delivery of at least 14 days.

Methods

A retrospective case note analysis over a 28-month period at Saint Luc University Hospital, Brussels.

Results

From 23 pregnancies that were complicated by PPROM prior to 25 weeks, 15 infants were born after 24 weeks with a latency of more than 14 days and persistent oligohydramnios. Nine infants (60%) had severe respiratory failure and clinical signs compatible with pulmonary hypoplasia. Seven of these infants (78%) responded to high frequency ventilation and inhaled nitric oxide therapy with good clinical outcome but two died from severe respiratory failure. Five infants showed no clinical signs of pulmonary hypoplasia and responded to conventional neonatal management. One of these infants died at 77 days of age of necrotising enterocolitis. One infant was not resuscitated and died within minutes of birth, following prior discussion with the perinatal team and the parents. Survivors in this high-risk group (73%) had low morbidity at the time of discharge.

Summary

The favourable neonatal survival and morbidity figures are in keeping with recent published evidence. This study confirms improved outcome even amongst the highest risk infants with documented persistent oligohydramnios.     

Effect of hemodiafiltration therapy in a low‐birthweight infant with congenital sepsis

The clinical course of congenital neonatal sepsis due to Streptococcus pneumoniae progresses rapidly and results in multiorgan failure with high mortality. The swift progression of the disease limits the timeframe for conventional treatment, which often requires waiting for antibiotics to show efficacy. Here, we describe the case of a very low‐birthweight (VLBW) female infant with congenital sepsis due to S. pneumoniae who was treated with continuous hemodiafiltration (CHDF) and polymyxin B‐immobilized fiber column–direct hemoperfusion (PMX‐DHP). The infant was born at 30 weeks’ gestation and diagnosed with hypotension, disseminated intravascular coagulation, and pulmonary hypertension. CHDF and PMX‐DHP were initiated approximately 11 h after birth. Mean blood pressure, oxygenation, and blood interleukin‐6 began to improve after dialysis commencement, and the patient survived with mild sequelae. Combined CHDF and PMX‐DHP may be effective in treating VLBW infants with severe septic shock.     

Low birthweight in infants born to African HIV-infected women: relationship with maternal body weight during pregnancy: Pregnancy and HIV Study Group (EGE).

The effect of maternal HIV infection on birthweight was estimated. In the prenatal clinic of the Centre Hospitalier de Kigali, HIV screening was proposed to women with a gestational age (GA) of less than 28 weeks. HIV-infected (HIV+) and uninfected (HIV-) women were recruited, when they consented. At inclusion, socioeconomic, obstetrical data, and body weight were collected, a clinical examination was performed, and tests for sexually transmitted diseases (STDs) and malaria were performed. Two prenatal visits were made, at 28-32 and 32-36 weeks, with clinical data and weight measurement. At delivery, birthweight, body length, and head circumference of the infant were documented. At inclusion and at the second follow-up visit, HIV+ women (N = 177) weighed less than HIV- women (N = 194) (p = 0.004). Mean birthweight in infants born to HIV+ women was 2947 g (SD = 429) and 3104 g (SD = 461) in infants born to HIV- women (p = 0.001). Frequencies of low birthweight (LBW, weight < 2500 g), prematurity (GA < 37 weeks, according to Finnstr?m score at birth), and intrauterine growth retardation (defined by LBW and GA > or = 37 weeks) were higher in infants born to HIV+ women than to HIV- women (p = 0.009, 0.01, and 0.053, respectively). In multivariate logistic regression, the association between maternal HIV infection and LBW disappeared (p = 0.61), while low GA (p = 0.01) and low last prenatal weight (p = 0.01) were independant risk factors of LBW. LBW in infants born to HIV+ women could be partly attributable to impaired maternal weight. These results underline the need for nutritional surveillance and dietary counselling, hoping to improve the prognosis of pregnancy in HIV+ women, regardless of other therapeutic interventions.     

Short-term outcome predictors in infants born at 23–24 gestational weeks

Aim: Outcome is uncertain in infants born at 23–24 gestational weeks. The aim of the present study was to identify possible early predictors of outcome in these infants.
Materials and Methods: Data from the Swedish medical birth register (MBR) for live-born infants with gestational ages (GAs) 23 and 24 weeks, born during the time-period 2000–2002, were analysed in relation to short-term outcomes, that is survival and survival without severe brain damage (intraventricular haemorrhage [IVH] grades 3 and 4 and/or periventricular leukomalacia [PVL]).
Results: In 57 infants born at 23 gestational weeks, survival was associated with birthweight (BW) (p = 0.018) and 5-min Apgar score (p = 0.020) on univariate analyses. In 99 infants born at 24 weeks of gestation, survival without severe brain damage correlated with BW (p = 0.039), birth type (singleton/multiple) (p = 0.017) and Apgar score at 1, 5 and 10 min (p = 0.028, 0.014 and 0.030, respectively). The best model for predicting survival without severe brain damage in infants born at 24 gestational weeks was based on 5-min Apgar score and birth type. The small number of live-born infants at 23 weeks of gestation did not allow for multiple logistic regression analyses.
Conclusion: The 5-min Apgar score is associated with short-term outcome in live-born infants at 23–24 gestational weeks. The association is stronger for infants born at 24 weeks of gestation.     

Low‐birthweight infants born to short‐stature mothers are at additional risk of stunting and poor growth velocity: Evidence from secondary data analyses

Low‐birthweight (LBW) infants are at an increased risk of stunting and poor linear growth. The risk might be additionally higher in these infants when born to short mothers. However, this hypothesis has been less explored. The objective of this secondary data analysis was to determine the risk of linear growth faltering and difference in linear growth velocity in LBW infants born to short mothers (<150 cm) compared to those born to mothers with height ≥150 cm during the first year of life. This analysis uses data from a community‐based randomized controlled trial of 2,052 hospital‐born term infants with birthweight ≤2,500g from urban low–middle socioeconomic neighbourhoods in Delhi, India. Data on maternal height and infant birth length were available from 1,858 (90.5%) of the infants. Infant anthropometry outcomes were measured at birth, 3, 6, 9, and 12 months of age. We found that infants born to short mothers had around twofold higher odds of stunting and lower attained length‐for‐age Z scores compared to infants of mothers with height ≥150 cm, at all ages of assessment. Linear growth velocity was significantly lower in infants of short mothers particularly in the first 6 months of life. We conclude that LBW infants born to short mothers are at a higher risk of stunting and have slower postnatal growth velocity resulting in lower attained length‐for‐age Z scores in infancy. Evidence‐based strategies need to be tested to optimize growth velocity in LBW infants especially those born to short mothers.     

Familial severe congenital diaphragmatic hernia: Left herniation in one sibling and bilateral herniation in another

Familial congenital diaphragmatic hernia (CDH) is extremely rare; it comprises about 2% of all CDH cases. The empirical risk is about 2%, increasing to 10% in a family with two affected children. This report describes severe CDH in two siblings who had been diagnosed prenatally. The female newborn diagnosed with left CDH prenatally was born at 38 weeks of gestation. Despite surgical repair and intensive treatment, she died 10 days after birth. Her younger brother was born at 39 weeks of gestation after being diagnosed with bilateral CDH prenatally, and died 75 min after birth. Both infants had neither other congenital anomaly nor chromosomal abnormalities. Their parents are healthy without consanguinity. Their first daughter and the fourth child have no congenital anomalies.     

Handicaps and health problems in 2 year old children of birth weight 500 to 1500 g

Fifty-nine infants of birthweight 500 to 999 g born in 1977 to 1980 and 132 infants of birthweight 1000 to 1500 g born in 1977 to 1978 were reviewed at two years corrected age. For the whole cohort, cerebral palsy was found in 12.6%, bilateral deafness in 1%, blindness in 1% and severe developmental delay in 12%. There was no significant difference in these disabilities between the groups of larger and smaller infants; 37.7% of the cohort was readmitted to hospital on at least one occasion, 35.6% of children had wheezing episodes and/or lower respiratory tract infections which together accounted for 51% of hospitalizations. The infants of birthweight 500 to 999 g tended to require more frequent and prolonged hospitalizations. Dolicocephalic head shape, chest deformities, iatrogenic sequelae from intensive care, poor growth and cicatricial retrolental fibroplasia were significantly more frequent in children of birthweight 500 to 999 g. Parents reported that 39% of their children had 'colic', 31.6% had sleep disturbance and 25% had multiple behavioural problems. Low frustration tolerance, inability to wait, hypo- or hyperactivity and an inappropriate relationship with the mother as measured by the psychologist all occurred significantly more frequently in children of birthweight of less than 1000 g. This report confirms the belief that a comprehensive follow-up is required for very low birthweight (VLBW) children because significant health problems continue after primary hospitalization.     

Handicaps and health problems in 2 year old children of birth weight 500 to 1500 g

Fifty-nine infants of birthweight 500 to 999 g born in 1977 to 1980 and 132 infants of birthweight 1000 to 1500 g born in 1977 to 1978 were reviewed at two years corrected age. For the whole cohort, cerebral palsy was found in 12.6%, bilateral deafness in 1%, blindness in 1% and severe developmental delay in 12%. There was no significant difference in these disabilities between the groups of larger and smaller infants; 37.7% of the cohort was readmitted to hospital on at least one occasion, 35.6% of children had wheezing episodes and/or lower respiratory tract infections which together accounted for 51% of hospitalizations. The infants of birthweight 500 to 999 g tended to require more frequent and prolonged hospitalizations.
Dolicocephalic head shape, chest deformities, iatrogenic sequelae from intensive care, poor growth and cicatricial retrolental fibroplasia were significantly more frequent in children of birthweight 500 to 999 g. Parents reported that 39% of their children had 'colic', 31.6% had sleep disturbance and 25% had multiple behavioural problems. Low frustration tolerance, inability to wait, hypo- or hyperactivity and an inappropriate relationship with the mother as measured by the psychologist all occurred significantly more frequently in children of birthweight of less than 1000 g. This report confirms the belief that a comprehensive follow-up is required for very low birthweight (VLBW) children because significant health problems continue after primary hospitalization.