Nineteen cases of cysts of the external female genitalia were diagnosed over a two-year period, giving an incidence of six per 1,000 female infants. The embryology, morphology, and differential diagnosis are discussed. No treatment was given and follow-up at two months of age revealed complete resolution of the cysts, thereby establishing their benign course in the newborn period.

Clitoris↗

PubMedDate not supplied

Blood-stained maternal milk: prevalence, characteristics and counselling.

Blood-stained maternal milk at delivery and during lactation was prospectively studied in 7774 livebirths over a period of 2 years (1986-1988). Eight mothers had this atypical breast discharge, a prevalence rate of 1:971 livebirths (0.1%). The characteristics of this phenomenon were: early appearance (frequently a short time after delivery), with normal bacteriologic and cytologic investigations, disappearance in 2 to 5 days, without adverse effects on the mothers and their babies, no recurrence after cessation, and occurrence in the previous pregnancies (3 of the 5 multipara). As a result, the best policy is to reassure the parents about the benign and transient course of this condition and to recommend continuation of breast feeding.

Blood↗

PubMedDate not supplied

The outcome of pregnancy in 689 women exposed to therapeutic doses of antidepressants. A collaborative study of the European Network of Teratology Information Services (ENTIS).

The European Network of the Teratology Information Services (ENTIS) has collected and evaluated data on 689 pregnancies in which exposure to tricyclic and nontricyclic antidepressants occurred. Data were collected prospectively from the time of in utero exposure and all the cases were followed up to the first few weeks of postnatal life using standardized procedures. In most cases, no longer term follow-up data were available. Approximately two-thirds of the mothers were on multidrug therapy, and of those, half took a benzodiazepine. About 95% of the patients were exposed during the first trimester. The most striking feature of the pregnancy outcomes is that 97% of live-born babies were morphologically normal. The incidence of spontaneous abortion and late fetal/neonatal deaths were within the normal range. Fourteen live-born babies and one fetus had either major or minor malformations, and six had minor anomalies. However, there was no increase in either a particular type of malformation or a specific pattern of defects. Another 31 infants without malformations had neonatal problems; these were usually associated with chronic multidrug therapy, especially near term. Elective termination of pregnancy occurred more frequently in the multidrug groups (86 out of 488) than in the monotherapy groups (20 out of 201), but data concerning the condition of the fetus are not available in the majority of the cases. Overall, no causal relationship could be established between in utero exposure to antidepressants and adverse pregnancy outcome.

Abnormalities, Drug-Induced↗

PubMedDate not supplied

On the symmetry of limb deficiencies among children with multiple congenital anomalies.

In humans, unpaired organs are placed in a highly ordered pattern along the left-right axis. As indicated by animal studies, a cascade of signaling molecules establish left-right asymmetry in the developing embryo. Some of the same genes are involved also in limb patterning. To provide a better insight into the connection between these processes in humans, we analysed the symmetry of limb deficiencies among infants with multiple congenital anomalies. The study was based on data collected by the International Clearinghouse for Birth Defects Monitoring Systems (ICBDMS). Registries of the ICBDMS provided information on infants who, in addition to a limb deficiency, also had at least one major congenital anomaly in other organ systems. We reviewed 815 such cases of which 149 cases (18.3 %) were syndromic and 666 (81.7 %) were nonsyndromic. The comparisons were made within the associated limb deficiencies, considering the information on symmetry, using a comparison group with malformations associated not involved in the index association. Among the non-syndromic cases, the left-right distribution of limb deficiencies did not differ appreciably between limb deficiency subtypes (e.g., preaxial, transverse, longitudinal). The left-right distribution of limb anomalies did not differ among most types of non-limb anomalies, though a predominance of left-sided limb deficiencies was observed in the presence of severe genital defects - odds ratio [OR], 2.6; 95 % CI, 1.1-6.4). Limb deficiencies (LDs) were more often unilateral than bilateral when accompanied by gastroschisis (OR, 0.1) or axial skeletal defects (OR, 0.5). On the contrary, LDs were more often bilateral than unilateral when associated with cleft lip with or without cleft palate (OR, 3.9) or micrognathia (OR, 2.6). Specifically, we found an association between bilateral preaxial deficiencies and cleft lip, bilateral amelia with gastroschisis and urinary tract anomalies, and bilateral transverse deficiencies and gastroschisis and axial skeleton defects. Of 149 syndromic cases, 62 (41.6 %) were diagnosed as trisomy 18. Out of the 30 cases of trisomy 18 with known laterality, 20 cases were bilateral. In the remainder the right and left sides were equally affected. Also, in most cases (74.4 %) only the upper limbs were involved. In conclusion the left-right distribution of limb deficiencies among some non-limb anomalies may suggest a relationship between the development of the limb and the left-right axis of the embryo.

Abnormalities, Multiple↗

PubMedDate not supplied

Prevalence and new phenotypic and radiologic findings in congenital onychodysplasia of the index finger.

Congenital onychodysplasia of the index finger (COIF) is a rare condition characterized by dysplastic changes in the nail with variable phenotypic manifestations. Its prevalence is unknown. We describe three newborn patients with various clinical and radiologic expressions of this entity. The first, born to a mother treated with multiple antidepressant drugs, had bilateral nail dysplasia of the index finger without bony involvement. The second presented with bilateral hypoplastic nail of the index finger and bilateral symphalangism of the fifth finger; aplastic distal phalanges of the second finger was also noted radiologically. The third baby (born to a diabetic mother treated with insulin), had micronychia and brachydactyly of the right index finger and syndactyly of the second and third fingers; the radiologic finding was bilateral brachymesophalangia of the index finger. It would seem that, besides the nail dysplasia of the index finger, COIF may also be associated with bone dysplasia of the same finger.

Female↗

PubMedDate not supplied

Cutis marmorata telangiectatica congenita: clinical findings in 85 patients.

Cutis marmorata telangiectatica congenita (CMTC) is an uncommon, sporadic, congenital cutaneous condition presenting with persistent cutis marmorata, telangiectasia, phlebectasia and possible ulceration of the involved skin, skin atrophy, and undergrowth of the involved extremity. To further the current understanding of this disorder, we analyzed the clinical features of a large series of patients with CMTC. The files of 85 patients with CMTC who were examined in our center over the last 20 years were reviewed. The data recorded included patient sex, age at onset, nature and distribution of the lesions, associated abnormalities, and course of disease. CMTC was characterized by early recognition of the lesions (94% at birth) and equal sex distribution. The lesion was unilateral in 65% of patients and most commonly involved the limbs (69%). Additional capillary lesions, noted in 20% of patients, may well have been an extension of the CMTC itself. Associated anomalies were found in 18.8% of patients. Almost half of the patients showed a definite improvement in the reticular vascular pattern on follow-up. No familial cases of CMTC were noted. CMTC is more prevalent than previously recognized, affects both sexes equally, and has a mostly localized distribution; additional vascular lesions are associated with the disorder, but other anomalies occur less often. Prognosis is generally good.

Adolescent↗

PubMedDate not supplied

Sacral nevus flammeus simplex: the role of imaging.

Neonates with midline lumbar, thoracic, or occipital cutaneous lesions should be suspected of having spinal dysraphism and should undergo an imaging study. The aim of the present study was to evaluate whether sacral nevus flammeus simplex (SNFS) in neonates is associated with spinal dysraphism. During a period of 6 months, we prospectively examined all neonates in our center for the presence of SNFS. Those affected were evaluated for associated anomalies by physical and neurologic examination and ultrasound imaging of the lumbosacral area. Twenty-eight of 3623 neonates (0.77%) were diagnosed as having SNFS. In 64% of the SNFS patients, nevus flammeus simplex (NFS) was noted as well. Physical and neurologic examinations were unremarkable. Ultrasound imaging of the lumbosacral area was performed in 25 patients (89%) and revealed diastematomyelia in one. The results show that SNFS may constitute the only cutaneous sign of spinal dysraphism. In conclusion, we recommend that neonates with SNFS be investigated for occult spinal dysraphism.

Female↗

PubMedDate not supplied

Obesity and the risk of toxaemia of pregnancy.

The purpose of the present study was to examine the relationship between severe pre-eclampsia/eclampsia (toxaemia) and obesity. We collected sociodemographic, anthropometric, medical and pregnancy outcome data from the hospital records of 248 Israeli women diagnosed with either pregnancy-induced or chronic hypertension, and compared these data to a control group of 236 women. Univariate analysis showed that while there exists a statistically significant positive association between obesity and hypertension (both pregnancy-induced and chronic) obesity presents no added risk to the development of toxaemia. Furthermore, we found a significant decrease in the rate of obesity among primigravid versus multigravid mothers with toxaemia superimposed on pregnancy-induced hypertension. On the other hand, primigravid mothers with PIH were at an increased risk of developing toxaemia as compared to multigravid women. These results suggest that obesity is not a significant factor in the development of toxaemia.

Adult↗

PubMedDate not supplied

Effect of neonatal posture on later lower limb rotation and gait in premature infants.

The rotational profile of 265 children age 4-8 years who were born at the gestational age of less than or equal to 30 weeks and were nursed prone for several months after birth showed a marked outtoeing gait up to the age of 6 years. Their positive thigh-foot angles were significantly higher than those of the general population. These angles indicate that their outtoeing gaits were caused by marked external tibial rotation.

Age Factors↗

PubMedDate not supplied

NSAIDs: maternal and fetal considerations.

Nonsteroidal anti-inflammatory drugs (NSAIDs) gained popularity in the late 1970s. Inhibition of prostaglandin synthesis with indomethacin has been reported to be effective for prevention of labor and for treatment for symptomatic polyhydramnios. Concern about its possible constrictive effect on the fetal ductus arteriosus has limited its use in pregnancy. Maternal indomethacin therapy has also been associated with reduction in urine production in the fetus and with oligohydramnios. Obstetricians have discouraged pregnant women from taking analgesic doses of aspirin, mainly because of the availability of paracetamol (acetaminophen), which causes less gastric irritation, but also because of fear of maternal and fetal hemorrhage and of possible premature closure of the ductus. These fears largely derive from studies on patients taking large doses and from extrapolation from other NSAIDs. The likelihood that treatment with 60-75 mg/day of aspirin markedly reduces the incidence of preeclampsia and fetal intrauterine growth retardation makes it important to reexamine its use. This review describes the pharmacology and pharmacokinetics of aspirin with particular reference to pregnancy and considers teratogenesis, prolongation of pregnancy and labor, maternal bleeding, fetal and neonatal bleeding, possible effects on the ductus arteriosus and pulmonary circulation, and possible nonspecific effects on intelligence and breast feeding and acute toxicity in the neonate.

Abnormalities, Drug-Induced↗

PubMedDate not supplied

Lipid infusion with different triglyceride cores (long-chain vs medium-chain/long-chain triglycerides): effect on plasma lipids and bilirubin binding in premature infants.

The possible beneficial effects of infusing a lipid emulsion containing 50% by weight of medium-chain triglycerides (MCT) compared with a standard long-chain triglyceride (LCT) emulsion were studied in 18 premature neonates (gestational age less than 34 weeks) requiring parenteral nutrition. The infants were assigned in a double-blind manner to receive one of the two lipid emulsions over 17 hours a day as a supplemental regimen for total parenteral nutrition. A lipid load of 1 g/kg per day was initiated on the third day of life and was increased at the rate of 1 g/kg per day until a maximal dose of 3 g/kg per day was obtained on the fifth day of life and maintained thereafter. Both bound and unbound bilirubin decreased with both infusion regimens during the study period. Despite a marked increase in plasma free fatty acid levels (260% in the MCT/LCT group compared with 210% in the LCT group), the fraction of unbound (free) bilirubin was significantly lower in the MCT/LCT group (34% vs 13%). Free fatty acid levels, corrected to albumin, were positively correlated to the percentage of free bilirubin only for the LCT lipid infusion. The finding of a significant elevation of plasma cholesterol levels only in the MCT/LCT group is now under investigation. Use of the MCT-containing emulsion was not associated with a higher frequency of adverse effects than the commonly used LCT-containing emulsion.(ABSTRACT TRUNCATED AT 250 WORDS)

Bilirubin↗

PubMedDate not supplied

Congenital malformations and developmental changes of the breast: a neonatological view.

Congenital malformations of the breast and nipple-areolar complex are described from the aspect of neonatal dysmorphology. Their classification is based on the accepted nomenclature in the medical literature and on a special method of clinical examination. The embryology and the possible genes involved in breast development are also discussed to clarify the mechanisms underlying the occurrence of breast malformations. Except for amastia, polymastia and polythelia, which have received much attention, the data for many other breast malformations are sparse. Case control studies or even case series are rare, and good evidence is often lacking. In addition, more studies are needed to complete the map of genes involved in breast development. This information will contribute substantially to our understanding and clinical approach to breast malformations in the future.

Breast Diseases↗

PubMedDate not supplied

Normal range of fetal knee movements.

Fetal limb movement abnormalities have been described to date in the literature only in subjective terms. The aim of the present study was to define objectively the normal range of fetal knee motion. The study population consisted of 240 normal fetuses at gestational age 16-39 weeks. The range of active knee motion was determined on routine prenatal ultrasound scans by measuring the angles between the midtibia, midfemur, and midknee on maximum flexion and extension. From 16 to 32 weeks, average knee flexion was 129.4 degrees (range, 120-140 degrees) and average extension was 23.7 degrees (range, 15-40 degrees). From 33 to 39 weeks, there was a gradual decrease in knee extension owing to lack of space in uterus. Knowledge of the normal range of fetal knee motion will assist clinicians in the prenatal diagnosis of neuromusculoskeletal disease.

Female↗

PubMedDate not supplied

Prevalence of congenital anomalies and neonatal complications in the offspring of diabetic mothers in Israel.

The prevalence of diabetes in pregnancy and its fetal and perinatal consequences in a large population of Israeli pregnant women during the last decade are presented. The study population consisted of 878 gestational diabetic women, 132 pre-gestational diabetic women, and 380 healthy pregnant women who served as controls. Minor congenital anomalies ranging between 19.4 and 20.5%, major congenital anomalies between 1.80 and 6.82%, and neonatal complications, such as macrosomia (5.6-25.0%), hypoglycemia (0.9-7.8%), hyperbilirubinemia (8.2-16.7%), hypocalcemia (2.7-5.5%) and polycythemia (3.8-13.3%), were observed in the study population. Despite meticulous maternal glucose control, we could not entirely eliminate fetal and neonatal complications. The definition of the normal and abnormal fetal intrauterine metabolic environment remains to be elucidated.

Congenital Abnormalities↗