Spontaneous neonatal renal vein thrombosis, a known pathology without clear management guidelines: An overview.

Renal vein thrombosis (RVT) was first described in 1837 by Rayer. Although tremendous progress has been achieved in the comprehension of its pathophysiology, its management remains controversial over 20 decades later. Therapeutic modalities vary from supportive measures alone to the utilization of thrombolytic agents whose protocols are derived from adult medicine. This review aims to show how difficult the treatment of RVT still is, especially with regard to the prognosis. The majority of affected neonates end with various renal complications (renal atrophy, dysfunction, hypertension, etc.). Like others, we suggest that simple unilateral RVT be managed conservatively, while thrombolytic agents may be attempted in unilateral RVT with extension to VCI and in bilateral RVT. Further studies are needed to reach appropriate consensual guidelines.

Massive recurrent post-tonsillectomy bleedings revealing a transient factor XIII deficiency in a 10-year-old boy. A case report.

A previously healthy 10-year-old boy was hospitalized for a left cervical abscess associated with massive tonsillar hypertrophy. He underwent abscess drainage and bilateral tonsillectomy. At H36 post-surgery, he presented with tonsillar hemorrhage requiring surgical revision. Hemorrhage relapsed 2 days later, with a total of 7 episodes, 5 of which required surgical revisions. Laboratory investigations were normal except for a markedly low factor XIII (FXIII) activity at 7%. After administration of a single dose of 40 IU/kg plasma-derived FXIII (Fibrogammin®) I.V., the bleeding stopped with no further recurrence. FXIII activity gradually normalized (75%) at 6 weeks, confirming the transient character of factor XIII deficiency. Severe congenital FXIII deficiency (FXIIID) (<1%) is very rare (1:2,000,000 births), whereas partial congenital deficiency and/or acquired deficiency may be more frequent but likely underreported. Acquired FXIIID may result from impaired synthesis (liver failure) or increased consumption (surgery, sepsis, leukemia, Henoch-Schönlein, inflammatory bowel disease, stroke, disseminated intravascular coagulation). FXIII replacement in form of fresh frozen plasma (FFP) or plasma-derived FXIII may be necessary for the presence of bleeding.

Unilateral pulmonary agenesis: a report of four cases, two diagnosed antenatally and literature review.

Pulmonary agenesis is a rare congenital malformation of lung development defined as complete absence of lung tissues, bronchi, and pulmonary vessels; it may be uni- or bilateral. The right-sided form carries the poorest prognosis due to severity of co-existent anomalies. Its diagnostic circumstances are variables: first reported cases were diagnosed at autopsy, but early postnatal as well as fortuitous discovery have been reported. In recent years, progress in obstetrical imaging has made antenatal diagnosis possible so that fetal ultrasound and MRI allow early diagnosis and refinement by permitting the elimination of differential diagnoses (diaphragmatic hernia, cystic adenomatoid malformation of the lung, giant lobar emphysema, and situs inversus). This anomaly is compatible with normal life provided co-existent malformations are thoroughly investigated and managed in a multidisciplinary setting. We report four cases of lung agenesis two of which were diagnosed antenatally at 23rd and 30th weeks of gestation respectively. Our aim is to describe the circumstances having led to diagnosis and report both follow-up and outcome of our patients.

Does plasma IGF-BP3 measurement contribute to the diagnosis of growth hormone deficiency in children?

OBJECTIVE: To audit the contribution of plasma IGF-PB3 measurement to the diagnosis of growth hormone deficiency (GHD) in children. POPULATION AND METHODS: Retrospective case study including boys and girls aged 0 to 18 years who attended our paediatric endocrinology clinic for short stature and/or post-irradiation follow-up, and had at least one GH provocative testing. Children with hypothyroidism, Laron or Kowarski syndromes, severe malnutrition, chronic renal failure and liver failure were excluded. RESULTS: Fifty-eight children were enrolled and grouped as GHD [+] (19 cases) and GDH [-] (39 cases). IGF-I and IGF-BP3 assay was carried out in 88% and 62% cases respectively, both groups were comparable for age, sex, BMI, target height, pubertal stage and bone age. There was a significant difference in peak GH between GDH [-] and GHD [+] groups (41.8 mUI/L ± 21.7 versus 11.5 ± 5.9 mUI/L, P<0.00001, respectively). No difference was found between groups with regards to IGF-I Z-scores and IGF-BP3 Z-scores. There was, however, a positive correlation between IGF-I Z-scores and IGF-BP3 Z-scores (r=0.50; P<0.0016). IGF-BP3 measurement could not differentiate between GHD [+] and GHD [-] groups. CONCLUSIONS: Measurement of plasma IGF-BP3 level contributes poorly to the diagnosis of GHD. We do not recommend it in routine use.

[Very preterm birth less than 33 weeks' gestation: how setting-up of a perinatal network does influence the activity of the neonatal tertiary care unit? The experience of the Poitou-Charentes region, France]. / Grande prématurité: impact de la mise en place d'un réseau périnatal sur l'activité du centre de néonatalogie de niveau 3 de la région Poitou-Charentes.

OBJECTIVES: To evaluate the impact of regional perinatal network setting on very preterm neonates (gestational age<33 weeks) referral and activity of regional level 3 NCIU, and short-term outcome of infants cared for. POPULATION AND METHODS: Comparison of data from medical records of hospital days and hospital outcome of very preterm neonates born before and after the setting-up of regional perinatal network (2002-2005). RESULTS: The setting-up of the Poitou-Charentes perinatal network has led to a 45% rise in number of very preterm neonates admitted to the level 3 neonatal care (114 in 2002, 166 in 2005), number of hospitalisation days has also increased by 31% in neonatology unit (2181 days in 2002, 2864 days in 2005) but remained stable in intensive care unit. A transient rise in neonatal mortality was observed, although the incidence of severe ultrasonographic cerebral abnormalities and that of bronchopulmonary dysplasia were lowered. CONCLUSION: Setting-up of perinatal network in Poitou-Charentes (France) has led to improved access to level 3 neonatal care, with rise in very preterm neonates survival and low incidence of short-term sequelae.

Buprenorphine withdrawal syndrome in newborns: a report of 13 cases.

AIMS: To assess neonatal abstinence syndrome (NAS) and neurodevelopmental outcome in infants born to addicted mothers under buprenorphine substitution therapy. SETTING: District general hospital, Angoulême, France. METHODS: Retrospective case records study of infants admitted to the neonatal intensive care unit (NICU) and/or special care baby unit (SCBU) from January 1994 to December 2000 for surveillance and/or treatment of buprenorphine NAS. RESULTS: Thirteen infants were born to addicted mothers under buprenorphine maintenance therapy during the study period. Eight were male and five were female; mean birth term and weight were 39 weeks gestation and 3000 g, respectively. Apgar scores were within normal limits; four infants were small for gestational age, none was dysmorphologic and none was extracted for fetal distress. NAS occurred in 11 cases (85%) and required treatment in 10 cases. Morphine chlorhydrate 0.5 mg/kg/day was administered in divided doses to seven children and gave better results than paregoric alone or in combination with diazepam. Upon follow-up, seven children presented transient lower limbs hypertonia, jerky movements and jitteriness that lasted 3-9 months. The overall milestones acquisitions were within normal limits. CONCLUSION: Buprenorphine substitution seems to be safe during pregnancy, and has had no teratogenic effects reported to date. It induces NAS of variable intensity that is less prolonged in comparison to methadone; the neurodevelopmental outcome of exposed children is normal in the majority of cases, although some presented with transient motor abnormalities that resolved completely in 85% of those recruited to our study.

[Familial Romano-Ward syndrome. Apropos of 2 new observations]. / Syndrome de Romano-Ward familial. A propos de deux nouvelles observations.

BACKGROUND: Congenital long QT syndrome is rare, usually revealed by bouts of syncopal attacks secondary to effort or strong emotions, and more rarely by atypical epileptic crisis. CASE REPORTS: We report a family history of two boys whose mother and grandmother both died suddenly a few days after delivery. The oldest child was 10 years old when admitted to hospital for recurrent loss of consciousness. Neurological examination and biological assays were normal; electrocardiography (ECG) revealed a prolonged QT interval of 0.59 seconds and episodes of torsades de pointe on the 24 hour ECG recording. The inefficacy of beta blocker treatment alone led to the implantation of a pacemaker; no recurrence has occurred since. The family investigation permitted to recognize the same syndrome in his asymptomatic 8-year-old brother for whom a prophylactic treatment was started. CONCLUSION: Both cases remind us of the necessity to carry out systematically an ECG in every child seen for unexplained malaise related or not to stress or for an atypical epileptic crisis. This is the only way for an early diagnosis on which the entire prognosis depends.

[Assay of lactic acid in the cerebrospinal fluid for the diagnosis of bacterial meningitis. Strategies for the choice of discriminatory threshold]. / Dosage de l'acide lactique dans le liquide céphalorachidien pour le diagnostic des méningites bactériennes. Stratégies pour le choix du seuil discriminant.

OBJECTIVES: In search for a supplementary marker of bacterial meningitis in cases where conventional bacteriology, cytology and chemistry are insufficiently contributive to diagnosis, we assessed the value of cerebrospinal fluid lactate levels in children with bacterial meningitis. PATIENTS AND METHODS: Cerebrospinal fluid lactate levels were measured from all spinal taps performed in a pediatric emergency care unit over a two-year period. Of the 332 usable samples there were 32 cases of bacterial meningitis, 104 cases of viral meningitis and 196 other diagnoses (non meningitis). RESULTS: Average lactate concentration 7 +/- 4 mmol/l in bacterial meningitis versus 2.1 +/- 0.6 mmol/l in viral meningitis (p < 0.0001). The value of lactic acid concentrations in discriminating between bacterial and viral meningitis was found to be superior to that of other chemistry results: protein, glucose, chloride. The discriminatory threshold of cerebrospinal fluid lactate was 3.7 mmol/l with sensitivity of 80% and a specificity of 98%. CONCLUSION: We propose routine assay of cerebrospinal fluid lactate in all cases of suspected meningitis.

[Carbamazepine and vigabatrin in epileptic pregnant woman and side effects in the newborn infant]. / Carbamazépine et vigabatrin chez la femme enceinte épileptique et effets secondaires chez le nouveau-né.

BACKGROUND: Carbamazepine (Tegretol) is frequently prescribed to pregnant epileptic women. Various congenital malformations constitute the most described side-effects in their newborns. CASE REPORTS: Case 1. Esteban was born by caesarean section at 39 weeks of gestation, weighing 3,860 g. His Apgar score was 8, 9, 10 at 1, 3 and 5 minutes. His mother was given phenobarbital until 1.5 months of pregnancy then carbamazepine 400 mg LP x 2/day, raised to 600 mg LP x 2/day at the 25th week of gestation because of epileptic crisis. The newborn was transferred at day 4 for drowsiness, mild jaundice, persistent vomiting and bouts of hypotonia/hypertonia, tremors and hyperexcitability. His plasma Tegretol level was 5.9 micrograms/mL and severe hypocalcemia (1.35 mmol/L) was noted. Hypocalcemia disappeared within 48 hours and gastric and neurologic troubles by day 6. The patient left the hospital at day 14. Case 2. Matheo, Esteban's brother, was born by caesarean section after 39 weeks of gestation, weighing 3,210 g. His Apgar score was 9, 10, 10 at first, third and fifth minutes. The mother's anti-epieptic treatment associated carbamazepine LP 400 mg x 3/day and vigabatrin four tablets of 500 mg/d until the 6th month of pregnancy and five tablets by day thereafter. The newborn presented vomiting from the first feeding; tremors were noted on day 2. His plasma Tegretol level was 5.7 micrograms/mL (N = 4-8 micrograms/mL) and the baby was transferred. Upon arrival, persistent vomiting and succession of hypotonia/hypertonia with intermittent opisthotonos were noted. Blood and urine tests showed: low calcemia (2.19 mmol/L), negative Brand reaction and DNPH test, normal urinary and blood amino acid chromatography. The course was spontaneously favourable and the child went home at day 11. CONCLUSION: Newborns of epileptic mothers treated with carbamazepine and/or vigabatrin during pregnancy should be placed under clinical observation during their first postnatal week. Calcemia monitoring is suggested for infants whose mothers were not supplemented with vitamin D during pregnancy. With a follow up of respectively 26 and 7 months, both brothers have normal milestones, confirming the transitory and benign character of reported side-effects.