Effects of oral iodine supplementation in very low birth weight preterm infants for the prevention of thyroid function alterations during the neonatal period: results of a randomised assessor-blinded pilot trial and neurodevelopmental outcomes at 24 months.

The trace element iodine (I) is essential for the synthesis of thyroid hormones. Parenteral nutrition solutions, formula milk, and human breast milk contain insufficient iodine to meet recommended intake for preterm infants. Iodine deficiency may affect thyroid function and may be associated with morbidity or neurological outcomes. The primary objective is to assess the evidence that dietary supplementation with iodine affects thyroid function during the neonatal period. The design was a randomised controlled pilot trial. Infants who met the inclusion criteria were enrolled through consecutive sampling and assigned to two different groups. The setting was a Spanish university hospital. Ninety-four patients with very low birth weight (under 1500 g) were included. Intervention group: 30 µg I/kg/day of iodine in oral drops given to 47 infants from their first day of life until hospital discharge. Control group: 47 infants without supplements. Formula and maternal milk samples for the determination of iodine content were collected at 1, 7, 15, 21, 30 days, and at discharge. Blood samples were collected for thyroid hormones. Neurological development was assessed at 2 years of age (Bayley III Test). Infants in the supplemented group reached the recommended levels from the first days of life. The researchers detected the effects of iodine balance on the plasma levels of thyroid hormones measured during the first 12 weeks of age. The trial assessed the impact of the intervention on neurodevelopmental morbidity.Conclusion: Thyroid function is related to iodine intake in preterm infants. Therefore, supplements should be added if iodine intake is found to be inadequate. The analyses found no effects of iodine supplementation on the composite scores for Bayley-III assessments in all major domains. The study results indicate potentially important effects on language development related to low iodine excretion during the first 4 weeks of life What is Known: • Thyroid function is related to iodine intake in preterm infants. • Preterm babies on formula preparations and with exclusive parenteral nutrition are at high risk of iodine deficiency. What is New: • Iodine intake should be monitored during the neonatal period. • Iodine supplements should be added if iodine intake is found to be inadequate.

VIM-Klebsiella oxytoca outbreak in a Neonatal Intensive Care Unit. This time it wasn't the drain.

OBJECTIVE: We describe an outbreak of VIM-carbapenemase Klebsiella oxytoca (VIM-Kox) in a NICU. MATERIALS AND METHODS: Prospective Epidemiological Surveillance:Systematically (weekly screening cultures) or on admission, if the patient had a history of previous colonization by VIM-Kox.Clinical cultures, done if infection was suspected.Other possible microorganism sources were investigated: their mothers (rectal microbiota), milk packages and preparation apparata in the lactodietary section, echocardiagram transductors, cribs, the sinks (faucets and drains), washing bowls, etc.Molecular typing was performed using the DiversiLab (bioMérieux) system on all VIM-Kox isolated from environment or patients (one by neonate). RESULTS: We identified 20 VIM-Kox cases, the most only presented colonization, but 4 showed infection. Three of the ten sinks (drains) in our NICU, were positive for VIM-Kox. Another four drains harbored P.aeruginosa, S. maltophilia and/or Enterobacter sp. Nevertheless the VIM-Kox bacteria in the sinks (drains) were not the same as those in the patients, who showed three different strains. CONCLUSIONS: A VIM-Kox colonization or infection outbreak in a NICU is described. Rather than environment, not even drains, the source of the outbreak was other patients. The outbreak was relatively brief, as a result of the rapidness with which appropriate measures were taken and followed.

Experimental study showed that adding fortifier and extra-hydrolysed proteins to preterm infant mothers' milk increased osmolality.

AIM: Measuring milk osmolality after adjustable fortification is clinically relevant, as values exceeding recommended safety thresholds might result in gastrointestinal consequences. The aim of this study was to evaluate the effect of four fortification levels and storage time on the osmolality of human milk. METHODS: This was an experimental study using 71 spare samples of fresh breastmilk collected from 31 mothers of preterm infants. Osmolality was measured before and after adding commercial human milk fortifier containing dextrinomaltose and hydrolysed proteins at four different concentrations. Measurements were performed at various points during the 23 hours after fortification. RESULTS: The mean basal osmolality of the 71 human milk samples was 296 ± 14 milliosmoles (mOsm)/kg, and these remained stable over a period of 23 hours. Just after fortification, the four fortified formulas showed higher osmolalities than the nonfortified human milk, ranging between 384 ± 14 and 486 ± 15 mOsm/kg, respectively (p < 0.01). This osmolality increased significantly from 20 minutes to 23 hours after fortification (p < 0.05). CONCLUSION: Adding fortifier and extra-hydrolysed proteins to human preterm milk increased osmolality, and these osmolality levels also increased with time. We recommend evaluating the risk of hyperosmolality when a higher fortification level is needed, to avoid gastrointestinal problems.

Dichorionic twin pregnancy discordant for anencephaly: two cases with different management.

BACKGROUND: Prevalence ofanencephaly in dichorionic twins is higher than in singleton pregnancies. The authors report two cases with two different management strategies. CASE 1: Spontaneous dichorionic diamniotic twin pregnancy with the second twin diagnosed with anencephaly at 12 weeks gestation. Selective feticide was performed at the age of 13.2 weeks. Vaginal delivery occurred at 39 weeks, and birth weight was 2,850 g. CASE 2: Dichorionic diamniotic twin pregnancy discordant for anencephaly in the second twin was diagnosed at 13 weeks gestation. An expectant management was decided. Preterm delivery occurred at 35 weeks due to hydramnios of the affected fetus, delivering a healthy newborn weighing 2,300 g and an anencephalic neonate who died immediately after delivery. CONCLUSION: Anencephaly should be diagnosed as soon as possible, idealistically at 11-13+6 weeks ultrasound (US) scan, in order to offer the most appropriate counselling to the parents, ranging from selective feticide or expectant management. This short series suggests that selective early feticide may increase gestational age and birth weight.

Genetic predisposition to fetal and neonatal cancer.

BACKGROUND: Neonatal tumors represent an extremely rare and heterogeneous disease with an unknown etiology. Due to its early onset, it has been proposed that genetic factors could play a critical role; however, germline genetic analysis is not usually performed in neonatal cancer patients PATIENTS AND METHODS: To improve the identification of cancer genetic predisposition syndromes, we retrospectively review clinical characteristics in 45 patients with confirmed tumor diagnosis before 28 days of age, and we carried out germline genetic analysis in 20 patients using next-generation sequencing and directed sequencing. RESULTS: The genetic studies did not find any germline mutation except patients diagnosed with bilateral retinoblastoma who harbored RB1 germline mutations. CONCLUSIONS: Our results suggest that genetic factors have almost no higher impact in most neonatal tumors. However, since the heterogeneity of the tumors and the small sample size analyzed, we recommend complementary and centralized germline studies to discard the early onset as an additional criterion to take into account to improve the identification of cancer genetic predisposition syndromes in neonates.

Successful treatment of intrauterine cytomegalovirus infection with an intraventricular cyst in a dichorionic diamniotic twin gestation using cytomegalovirus immunoglobulin.

Congenital cytomegalovirus (CMV) infection is the leading cause of severe congenital abnormalities. CMV immunoglobulin (CMVIG) may lower risk for symptomatic disease in congenital CMV infection. In a twin pregnancy, only one fetus shows CMV infection, raising a dilemma about intervention since the uninfected fetus would be exposed to treatment unnecessarily. CMVIG (2 × 200 U/kg) was given due to high viral load and development of an intraventricular cyst. The cyst growth plateaued, no other brain damage developed, and at 8 months, the infant was symptom-free. CMVIG appears appropriate to treat intrauterine CMV infection in this setting.

Polyasplenia, caudal deficiency, and agenesis of the corpus callosum.

Fullana et al. [Am J Med Genet (suppl. 2): 23-29, 1986] reported on 2 sibs with an autosomal recessive syndrome of caudal deficiency and polyasplenia anomalies. We report on a similar patient in which agenesis of the corpus callosum (ACC) was also found. Such an association has not been reported previously. This finding of ACC is to be interpreted as another midline anomaly rather than as a causally independent malformation.

Intrapartum chemoprophylaxis of early-onset group B streptococcal disease.

A randomized study in 121 pregnant women carrier of group B streptococci is undertaken in order to assess the administration of 500 mg of intrapartum ampicillin intravenously to interrupt mother-to-fetus group B streptococcal transmission. In the prophylaxis group there was a significant reduction in neonatal colonization (3.7 vs. 42.9%) and in severe neonatal colonization (0 vs. 25%). There was no case of group B streptococcal sepsis in the prophylaxis group compared to 4.6% (3 cases) in the control group (P greater than 0.05). Clinically infected newborns represented 3.3% in the prophylaxis group vs. 13.8% in the control group. When the organism was isolated during delivery in the vagina or amniotic fluid, prophylaxis was quickly followed by second negative cultures. Ampicillin levels in the amniotic fluid were detected early, and they increased significantly till the third hour. Bactericidal levels in the umbilical cord were detected in 60% of newborns. All these findings support the usefulness of ampicillin prophylaxis in the prevention of early-onset group B streptococcal sepsis.

Natural transmission of group B Streptococcus during delivery.

This is a study of group B Streptococcus during labor of 121 patients in whom group B Streptococcus was isolated in the vagina and/or rectum before delivery. The intrapartum vaginal culture was positive in 55.2% of the antepartum carriers (32/58). When the vaginal culture during delivery was positive, the group B Streptococcus was isolated in the amniotic fluid 2 h after the rupture of membranes in 81% of the cases. The newborns of antepartum carriers, when the labor developed naturally, were colonized by group B Streptococcus in 69.2% of cases when the intrapartum vaginal and/or amniotic fluid cultures were positive (9/13), while only 5.6% of the newborns of antepartum carriers but with negative cultures during delivery were colonized by group B Streptococcus (1/18). The most frequent positive neonatal culture was in the umbilicus (83.3%) followed by the external ear (62.5%).

[Anomalous systemic arterial supply to the lower lobe of the right lung: a rare finding]. / Vascularización anómala sistémica del lóbulo inferior del pulmón derecho: una patología infrecuente.

Vascularization of a segment or lobe of the lung from a systemic artery is a rare anomaly. The lower left lobe is usually involved and clinical presentation typically includes hemoptysis, chest pain, exercise-related dyspnea or murmurs. Some patients are asymptomatic and discovery is fortuitous. Diagnosis depends on images, with angiography and, most recently, helicoid CT scan proving most useful. Treatment is nearly always surgical, with or without prior embolization of the anomalous vessel.We report the case of a 31-year-old man with anomalous systemic arterial supply to the lower right lobe discovered while we examined a pulmonary node that appeared benign. The location was unusual. We discuss diagnostic procedures and assess the relation between this condition and lung sequestration and hypogenetic lung syndrome.

[Chylous fistula following lymphadenectomy in breast cancer]. / Fístula quilosa tras linfadenectomía axilar en cáncer de mama.

Axillary lymph node dissection remains an integral part of surgical treatment in patients with invasive breast cancer and axillary lymph node metastases. Chylous leakage after axillary lymph node dissection is infrequent. We report the case of a patient with an advanced local breast cancer that was subsidiary to surgery treatment after neoadjuvant chemotherapy. She had a postoperative chylous leakage; it was successfully treated conservatively in the postoperative course. Chylous leakage an infrequent complication alters axillary lymph node dissection, which has a conservative management in most published studies.

Genetic predisposition to fetal and neonatal cancer

Background Neonatal tumors represent an extremely rare and heterogeneous disease with an unknown etiology. Due to its early onset, it has been proposed that genetic factors could play a critical role; however, germline genetic analysis is not usually performed in neonatal cancer patients Patients and methods To improve the identification of cancer genetic predisposition syndromes, we retrospectively review clinical characteristics in 45 patients with confirmed tumor diagnosis before 28 days of age, and we carried out germline genetic analysis in 20 patients using next-generation sequencing and directed sequencing. Results The genetic studies did not find any germline mutation except patients diagnosed with bilateral retinoblastoma who harbored RB1 germline mutations. Conclusions Our results suggest that genetic factors have almost no higher impact in most neonatal tumors. However, since the heterogeneity of the tumors and the small sample size analyzed, we recommend complementary and centralized germline studies to discard the early onset as an additional criterion to take into account to improve the identification of cancer genetic predisposition syndromes in neonates (AU)

Identification of risk factors associated with nosocomial infection by rotavirus P4G2, in a neonatal unit of a tertiary-care hospital.

A rotavirus outbreak in newborns admitted to the 'La Paz' University Hospital, Madrid was detected, followed up and controlled. Uninfected children were selected as control subjects. Samples of faeces were taken once or twice weekly from all the newborns, including those who were asymptomatic and who were admitted to the neonatal unit for early detection of rotavirus and the positive were separated from the rest of the neonates. Contact-related precautions were taken for all patients, and alcohol solutions were used for hand washing. During the months of the outbreak, 1773 children were admitted to the hospital, 131 of whom were affected by the rotavirus infection (7.4%). Of these, 72 (55%) had symptomatic infections. In the first month of the outbreak, nine cases of necrotizing enterocolitis were diagnosed (one patient developed massive intestinal necrosis). The infections (symptomatic and asymptomatic) presented a bimodal distribution caused by a new outbreak of rotavirus type P4G2 after two patients who had acquired the infection outside the hospital were admitted when the first outbreak was subsiding. The characteristics of cases and controls were analysed using bivariate and multivariate methods (non-conditional multivariate logistic regression) to identify four risk factors strongly associated with rotavirus infection: premature birth, infections other than rotavirus, malformation, and changes in glycaemia and/or presence of jaundice.

Fístula quilosa tras linfadenectomía axilar en cáncer de mama / Chylous fistula after lymphadenectomy in breast cancer

La linfadenectomía axilar constituye uno de los pilares en el tratamiento locorregional del cáncer de mama. La fístula quilosa es una complicación infrecuente en el vaciamiento axilar. Se muestra el caso de una paciente con un tumor de mama localmente avanzado, subsidiaria de tratamiento quirúrgico tras la administración de quimioterapia neoadyuvante, que presentó una fístula quilosa en el postoperatorio. La evolución fue satisfactoria con el tratamiento conservador instaurado mediante somatostatina subcutánea y una dieta baja en grasa suplementada con triglicéridos de cadena media. La fístula quilosa es una complicación poco habitual en la linfadenectomía axilar, cuyo manejo es conservador en la gran mayoría de casos descritos (AU)

Axillary lymph node dissection remains an integral part of surgical treatment in patients with invasive breast cancer and axillary lymph node metastases. Chylous leakage after axillary lymph node dissection is infrequent. We report a case patient with an advanced local breast cancer who was treated with neoadjuvant chemotherapy and surgery. She had a postoperative chylous leakage what was successfully treated conservatively in the postoperative course with somatostatin and a fat reduced diet with medium chain triglyceride. Chylous leakage an infrequent complication after axillary lymph node dissection, which has a conservative management in most published studies (AU)

Complete trisomy 9. Two additional cases.

Two infants with complete trisomy of chromosome 9 are described. One patient, died a few minutes after birth and another survived 24 hours. The main clinical findings in this syndrome are: intrauterine growth retardation, characteristic facial dysmorphism, hypoplastic external genitalia and malformations of heart, brain and skeleton.

[Chondrodysplasia punctata associated with Down's syndrome. Presentation of a case and review of the literature]. / Condrodisplasia punctata asociada a síndrome de Down. Presentación de un caso y revisión de la literatura.

A case of chromosomic aberration and chondrodysplasia punctata is presented. Nine similar have been previously reported being this case first described in Spain. Hypothesis arouse from these observations suggest that they are different entities but also permit to include bone alterations among chromosomic aberration signs.

Recto-vaginal colonization and urinary tract infection by group B Streptococcus in the pregnant diabetic patient.

The influence of maternal diabetes on Group B Streptococcus (GBS) colonization and GBS urinary infection was investigated. The population under study comprised 1,050 pregnant women (70 of them diabetics, the remaining 980 non-diabetics). A higher prevalence of GBS colonization was found among diabetics (20% versus 10.9%) (p less than 0.05). The rate of colonization was not correlated to the severity of the diabetes condition. Urinary infection was diagnosed on 8.6% of diabetic patients, versus 7.1% of non-diabetics (p greater than 0.05). Urinary infection by GBS occurred with similar frequency in both groups (0% in diabetics and 1% in non-diabetics). The possible etiological implications are commented on, and vaginal and rectal cultures are recommended for GBS screening in the pregnant diabetic patient.

[Early onset group B streptococcal neonatal sepsis (author's transl)]. / Sepsis precoz por estreptococo del grupo B en el recién nacido. A propósito de 17 observaciones.

Seventy one per cent of neonates had a gestational age longer than 37 weeks. Sixteen showed respiratory distress, and shock occured in twelve cases. The onset of illness happened within the first 12 hours of life, in 15 cases. No differences were observed among preterm and term infants. Differential leukocyte count were altered in all infants, being particularly relevant the fact that the relation between immature neutrophil/total neutrophil, was elevated in 50 per cent of cases. Mortality rate was 52 per cent. Clinical alert and early treatment with penicillin, together with the use of fresh blood exchange transfusion, can be considered useful methods in the treatment of these cases.