Weak Sucking Research Articles

Neonatal toxicity and transient neurodevelopmental deficits following prenatal exposure to lithium: Another clinical report and a review of the literature

I report the case of an infant girl who was exposed to lithium during gestation and her follow-up at the age of 1 year. She presented with transient neurodevelopmental deficits including lethargy, hypotonia, and poor oral feeding ability in the neonatal period. She required supportive treatment and made gradual improvement in neurologic functioning. On examination at the age of 1 year, physical findings and psychomotor development were normal. The English literature from 1978 to 2004 is reviewed. A total of 30 patients who were exposed to lithium during gestation with adequate clinical description were identified. A significant number of these babies presented with neurodevelopmental deficits and depressed neurological status including hypotonia, respiratory distress syndrome, cyanosis, lethargy, and weak suck and Moro reflexes in the neonatal period. The majority of these abnormalities resolved and most babies made full recovery. Other abnormalities were structural as well as functional involvement of the cardiovascular system, macrosomia, prematurity, jaundice, diabetes insipidus, and involvement of the thyroid gland. While the use of lithium during pregnancy does not appear to significantly increase the risk of congenital anomalies, it is frequently associated with perinatal complications and reversible neonatal toxicity. Suggested guidelines for appropriate monitoring of infants and breast-feeding of exposed babies are presented. In addition, prenatal surveillance of women with bipolar disorders who are being treated with lithium is briefly discussed.

New syndrome of simplified gyral pattern, micromelia, dysmorphic features and early death.

We report two sisters with a new syndrome of simplified gyral pattern, normal head circumference at birth but with subsequent development of microcephaly, intractable seizures, and early death. Dysmorphic features included coarse face, hypertrichosis, short nose, paranasal widening, long philtrum, short neck, upper limb micromelia, single transverse palmar lines, and clasp thumbs. The proband had repeated convulsions from shortly after birth and she required continuous artificial ventilation. Neurological examination showed absent sucking, rooting, Moro and grasping reflexes. MRI revealed a diffuse simplified gyral pattern with apparent agyria over the frontal lobes. Biochemical screening gave normal results. Her older sister had bilateral renal pelvic dilatation on prenatal ultrasound. She also developed severe convulsions on the first day of life, and she had to be artificially ventilated for 38 days. She had severe developmental retardation and neurological examination showed absence of spontaneous movements and Moro reflex, weak sucking reflex, and hypertonicity. CT scan of the brain showed a simplified gyral pattern. At 3 months, she developed hypocalcemia and hyperphosphatemia with normal levels of vitamin D and alkaline phosphatase, and parathyroid hormone level was low. Other biochemical tests gave normal results. She died at 5 months due to a massive aspiration event. Based on the unique clinical and radiological features found in our patients, we propose that this is a new syndrome.

Early feeding problems in children with cerebral palsy: weight and neurodevelopmental outcomes.

Using prospectively collected data from 13971 births enrolled in a large population-based cohort study (Avon Longitudinal Study of Parents and Children: ALSPAC), the prevalence of feeding difficulties at 4 weeks and 6 months of age in 33 children subsequently diagnosed with cerebral palsy (CP) were investigated. It was also assessed whether early feeding difficulties could be predictive of functional and growth outcomes at age 8 years. Weak sucking at 4 weeks of age was reported in 11 of 23 children with CP and in 2206 of 12299 (18%) of the remaining population (p<0.0001), and great difficulties feeding at 6 months of age was reported in two of 21 (10%) of the group with CP and in 373 of 10941 (3.3%) of control participants (p=0.017). Feeding difficulties at 4 weeks of age were associated with the pattern of functional impairment at age 4 years (p=0.009) and at 8 years (p=0.068), being clinically underweight (p=0.01), and having speech and swallowing difficulties (p=0.005) at 8 years of age. Early, persistent, and severe feeding difficulties are a marker for subsequent poor growth, feeding, and developmental outcomes and can identify children with CP who will benefit from gastrostomy feeding.

Early feeding problems in children with cerebral palsy: weight and neuro‐developmental outcomes

Using prospectively collected data from 13 971 births enrolled in a large population‐based cohort study (Avon Longitudinal Study of Parents and Children: ALSPAC), the prevalence of feeding difficulties at 4 weeks and 6 months of age in 33 children subsequently diagnosed with cerebral palsy (CP) were investigated. It was also assessed whether early feeding difficulties could be predictive of functional and growth outcomes at age 8 years. Weak sucking at 4 weeks of age was reported in 11 of 23 children with CP and in 2206 of 12299 (18%) of the remaining population (p&lt;0.0001), and great difficulties feeding at 6 months of age was reported in two of 21 (10%) of the group with CP and in 373 of 10941 (3.3%) of control participants (p=0.017). Feeding difficulties at 4 weeks of age were associated with the pattern of functional impairment at age 4 years (p=0.009) and at 8 years (p=0.068), being clinically underweight (p=0.01), and having speech and swallowing difficulties (p=0.005) at 8 years of age. Early, persistent, and severe feeding difficulties are a marker for subsequent poor growth, feeding, and developmental outcomes and can identify children with CP who will benefit from gastrostomy feeding.

Persistent early feeding difficulties and subsequent growth and developmental outcomes

ABSTRACTAim To investigate the prevalence of feeding difficulties in infancy, and to determine whether feeding difficulties in the first 15 months can be predictive of poor growth or developmental impairments in the pre‐school years.Methods Data on infant feeding behaviour and developmental milestones were collected prospectively, using self‐completion questionnaires, from the parents of the 14 138 live births in the Avon Longitudinal Study of Parents and Children.Results Weak sucking (18%), and choking with feeding (55%) were common at 4 weeks of age, and 3.3% of parents described great difficulties with feeding their children at 15 months. Children who had persistent feeding problems at 1, 6, and 15 months had a prevalence of 2.6/1000. This study group had a higher prevalence of delayed motor milestones, with delayed walking at 18 months (p &lt; 0.0001) and inability to run at 30 months (p &lt; 0.0001); delayed language development at 18 months (p = 0.041), and impairment of articulation of speech at 38 months (p = 0.012). They also demonstrated difficult behaviours, with more frequent episodes of temper tantrums (p = 0.004) and meal refusal (p &lt; 0.0001) at 18 months. There were reductions in the mean scores for motor skills (p = 0.001), communication (p = 0.01) and total development (p &lt; 0.0001) at 18 months. At 30 months, 22% of the parents reported concern regarding their child's weight (p = 0.0001), and the mean weight Z scores at 9 and 18 months for this group were lower than the remaining population, although the differences did not reach statistical significance.Conclusions Feeding difficulties were widely reported by parents of infants at 4 weeks of age, and thus did not have a predictive value in identifying children who were subsequently noted to have developmental impairments. The children with persistently difficult feeding throughout their first 15 months had significant impairments in their motor, language and behavioural development, and may benefit from early referral for assessment.

Transient neonatal myasthenia gravis and pyloric stenosis

The case report describes the occurrence of hypertrophic pyloric stenosis in a premature infant with neonatal myasthenia gravis. The infant presented for pyloromyotomy on the nineteenth day of life. Diagnosis of myasthenia gravis was made based on maternal history and clinical findings of poor muscle tone, weak suck, and weak cry. The anesthetic management is discussed with reference to the problems of newborn myasthenia gravis and pyloric stenosis as they relate to anesthetic drugs and techniques.

TRANSIENT MYASTHENIA NEONATORUM (TMN)

TMN, secondary to transplacental passage of antibodies directed to acetylcholine receptors, has been reported in 10 to 12% of infants born to myasthenic mothers. Symptoms may appear at 72 hrs. or up to 7 days due to passage of maternal medication. It is a different entity from Congenital Myasthenia inasmuch as the latter has no maternal history of the disease. The present paper reports data from 6 infants born to myasthenic mothers between 3/88 an 3/93. We retrospectively considered perinatal data, symptoms attributable to TMN (hipotonia, asbsent/decreased reflexes, suction, facial expression, apnea/hypoventilation, and IMV requirements) use of antibiotics, response to medication and length of hospital stay. All mothers had received treatment (Prostigmin (R)) during pregnancy. Two infants had no symptoms out of the rest (2 brothers) all were hypotonic; 3/4 had apnea or weak suck; 3/4 required IMV, and all 3 received aminoglycosides after being placed on the ventilador; 2/4 had decreased reflexes and /or facial expression. All 4 received specific medication (Prostigmin (R)). Mean length of stay was 40 days. These data show that TMN may be more frequent than reported and entiails increased neonatal morbidity. Although controversial, different maternal therapy (IVIG, steroids and/or plasmapheresis) might decreased the incidence of TNM.

Early clinical symptoms and incidence of aspartylglucosaminuria in Finland.

Early clinical symptoms were analyzed from all known 43 children with aspartylglucosaminuria, born during 1974-1989 in Finland. Pre- and perinatal histories appeared normal for all children, except for muscular hypotonia and weak sucking in some babies. Three infants had abduction stiffness in the hips, needing follow-up. Other abnormalities found in infancy were umbilical or inguinal hernias and unusual susceptibility to respiratory and ear infections. This susceptibility diminished clearly in most patients after six years of age. Episodic diarrhea, described earlier, appeared to be a rather infrequent symptom and a less valuable diagnostic clue. New clinical phenomena were talipes planovalgus or clubfoot, needing surgical treatment, and aggressive behavior, needing, occasionally, child psychiatric consultation or treatment. In addition, angiokeratoma of the skin, not an infrequent phenomenon among adult patients, was found in one child. The main indications for further studies were delayed speech, attention deficit and clumsy or delayed motor functions. The disease is easily misdiagnosed and, universally, probably underdiagnosed. Its incidence in Finland was recalculated and appeared to be at least 1 in 18,500 live-born babies in this country.

INDEX OF SUSPICION

This section of Pediatrics in Review reminds clinicians of those conditions that can present in a misleading fashion and require suspicion for early diagnosis. Emphasis has been placed on conditions in which early diagnosis is important and that the general pediatrician might be expected to encounter, at least once in a while. The reader is encouraged to write possible diagnoses for each case before turning to the discussion, which is on the following page.We invite readers to contribute case presentations and discussions.Case 1 PresentationA 2-month-old breast-fed boy is brought to the office with a 2-day history of constipation, poor feeding, lethargy, wheezing, and a temperature of 101°F (38.3°C). In the office his temperature is recorded at 98.8°F (37.1°C) rectally. The infant does not appear lethargic, and the only significant physical finding is tearing of the left eye. Because of the history of fever, a complete blood count is obtained, which is normal. The infant is discharged home with a diagnosis of conjunctivitis and possible viremia. He returns within 48 hours and is found to be hypotonic with a poor gag reflex, weak suck, and weak cry. Despite his normal temperature a septic evaluation is initiated.Case 2 PresentationA 17-year-old boy complains of bilateral hip pain that has bothered him for the past year.

INDEX OF SUSPICION

This section of Pediatrics in Review reminds clinicians of those conditions that can present in a misleading fashion and require suspicion for early diagnosis. Emphasis has been placed on conditions in which early diagnosis is important and that the general pediatrician might be expected to encounter, at least once in a while. The reader is encouraged to write possible diagnoses for each case before turning to the discussion, which is on the following page. We invite readers to contribute case presentations and discussions. Case 1 Presentation A 2-month-old breast-fed boy is brought to the office with a 2-day history of constipation, poor feeding, lethargy, wheezing, and a temperature of 101°F (38.3°C). In the office his temperature is recorded at 98.8°F (37.1°C) rectally. The infant does not appear lethargic, and the only significant physical finding is tearing of the left eye. Because of the history of fever, a complete blood count is obtained, which is normal. The infant is discharged home with a diagnosis of conjunctivitis and possible viremia. He returns within 48 hours and is found to be hypotonic with a poor gag reflex, weak suck, and weak cry. Despite his normal temperature a septic evaluation is initiated. Case 2 Presentation A 17-year-old boy complains of bilateral hip pain that has bothered him for the past year.

We Were Our Age

DUKE'S BROTHER DIED under the ice in spring, the season I met Duke. The other kid survived?shivering, fat-lipped, the instigator, littler. A miracle, the search team said. Just a hurt front tooth, a gash, a stitch. A boy's likely story. This is what I heard, but not from Duke. Duke said, Go. Duke said, Ladies first, please. Rock, rock, gap, stone-cold to the lighthouse?Duke and I were spiny in the dark. Weeds swam up. Weak sunk stars, a lap, suck and bubble. The usual. Lake and pier. Us and us. Month after month. Duke was always finding things to skip across to who knew where. August. Pinkness hovered.

Progress in research, therapy of Prader-Willi syndrome, but numerous challenges remain to be overcome

Infantile hypotonia and feeding difficulty, adolescent hyperphagia and hypogonadism, unremitting behavioral problems, and premature death from complications of obesity: such is the lot of the patient with Prader-Willi syndrome. Infants with this condition eat poorly because of a weak suck reflex. However, by age 5 or 6 years, severe hyperphagia intervenes leading to obesity and such attendant problems as diabetes mellitus and respiratory insufficiency. The eating disorder is thought to be hypothalamic in origin. This is also true of associated endocrine abnormalities, which are discussed in a case report appearing in this issue ofThe Journal(pp 3280-3282). The syndrome, first described by Swiss investigators 30 years ago (Schweiz Med Wochenschr1956; 86:1260-1261), affects one in 10,000 to 25,000 children, with a large preponderance of boys. At least this figure is the best current estimate, according to Suzanne B. Cassidy, MD, assistant professor of pediatrics and director of the division

Clinical characteristics of infant botulism in the United States

We reviewed the clinical features of 99 cases of infant botulism reported to the Centers for Disease Control from states other than California for the period 1976 to 1980. There were no toxin-specific differences in the distribution of ages at onset or sex of the cases. For 76 (76%) patients for whom data were available the most common presenting symptoms were poor feeding (43%) and constipation (24%). Weak suck, poor head control, floppiness, weakness in extremities, difficulty swallowing, altered cry and constipation were reported in over three-fourths of the infants for whom data were available. Loss of facial expression, extraocular muscle paralysis, dilated pupils and depression of deep tendon reflexes occurred significantly more frequently among infants with type B botulism than among those with type A botulism. Ventilatory assistance was required for 61% of infants receiving aminoglycosides after the onset of weakness compared to only 26% of those infants not receiving aminoglycosides (P = 0.01). Infant botulism presents a characteristic clinical picture and should be suspected when an infant presents with weakness.

Infant Botulism Type B in Central Europe

A case of moderately severe botulism was diagnosed in a 4 weeks old white female. Clostridium botulinum toxin was identified repeatedly in the infant's faeces by means of the mouse protection assay. Clostridium botulinum was isolated in pure culture from faecal material. Both the organism and the toxin were type B.The onset of illness was characterized by mild constipation, apathy, weak sucking and difficulty with swallowing. Incipient, probably aspiration, pneumonia was diagnosed at the same time. Further signs of botulism developed during hospitalization, viz. loss of head control, pooled oral secretion, weak cry, mild ptosis, reduced facial expression, generalized muscular weakness and reduced spontaneous activity. A nasogastric feeding tube was needed because the ability to suck and swallow was impaired. Immediately on admission of the infant to hospital emergency treatment was started with Ampicilin, which was followed by penicillin injections. The infant recovered in 60 days. Subsequent medical examinations demonstrated that the recovery was complete and the development normal.The case represents the first instance of infant botulism detected on the European Continent.

Neurologic Activity of Infants following Anesthesia for Cesarean Section

Elective cesarean section was performed in a consecutive series of 30 patients with full-term pregnancies who were not in labor. Epidural (lidocaine, 1.5 per cent, with epinephrine, 1:200,000) and general anesthesia (thiopental, nitrous oxide-oxygen, succinylcholine infusion) was used alternately. Neonatal acid-base values and Apgar scores showed no significant difference between the two anesthetic groups, and most infants were vigorous at birth. The neurologic recoveries of the infants showed no significant difference between the two groups. In the group receiving epidural anesthesia, there was a significant correlation between maternal hypotension and weak rooting and sucking reflexes of the infants during the first two days. All infants of high-risk obstetric patients in the series, independent of anesthetic technique used, had abnormal neurologic activity, as evidenced by either depression of muscle tone and the reflexes or all the tested variables. Neurologic assessment as followed in this series is a sensitive indicator of the effects of fetal stress factors acting during cesarean section.

Neurologic activity of infants following anesthesia for cesarean section.

Elective cesarean section was performed in a consecutive series of 30 patients with full-term pregnancies who were not in labor. Epidural (lidocaine, 1.5 per cent, with epinephrine, 1:200,000) and general anesthesia (thiopental, nitrous oxide-oxygen, succinylcholine infusion) was used alternately. Neonatal acid-base values and Apgar scores showed no significant difference between the two anesthetic groups, and most infants were vigorous at birth. The neurologic recoveries of the infants showed no significant difference between the two groups. In the group receiving epidural anesthesia, there was a significant correlation between maternal hypotension and weak rooting and sucking reflexes of the infants during the first two days. All infants of high-risk obstetric patients in the series, independent of anesthetic technique used, had abnormal neurologic activity, as evidenced by either depression of muscle tone and the reflexes or all the tested variables. Neurologic assessment as followed in this series is a sensitive indicator of the effects of fetal stress factors acting during cesarean section.

Infant Botulism

<i>Clostridium botulinum</i>organisms and toxin were identified in the feces of six infants, aged 5 to 20 weeks, who had illnesses clinically consistent with botulism. Five of the infants lived in California and became ill within a six-month period in 1976; one infant became ill in New Jersey in 1975. Three cases were type A botulism, and three were type B. No source of ingested botulinal toxin could be found in any case. However, one infant with type B botulism had ingested a food containing<i>C botulinum</i>type B organisms, but no toxin was found in it. The clinical findings in these cases include constipation, weak sucking and crying ability, pooled oral secretions, cranial nerve deficits, generalized weakness, and, on occasion, sudden apnea. A characteristic electromyographic pattern termed "brief, small, abundant, motor-unit action potentials" (BSAP) was observed. The sources of<i>C botulinum</i>toxin for these six infants is thought to have been in vivo (gastrointestinal) production following ingestion of<i>C botulinum</i>organisms. Studies are underway to determine the full clinical spectrum, incidence, and potential public health importance of this infectious disease newly recognized in infants. (<i>JAMA</i>237:1946-1951, 1977)

Infant botulism. Epidemiological, clinical, and laboratory aspects

Clostridium botulinum organisms and toxin were identified in the feces of six infants, aged 5 to 20 weeks, who had illnesses clinically consistent with botulism. Five of the infants lived in California and became ill within a six-month period in 1976; one infant became ill in New Jersey in 1975. Three cases were type A botulism, and three were type B. No source of ingested botulinal toxin could be found in any case. However, one infant with type B botulism had ingested a food containing C botulinum type B organisms, and no toxin was found in it. The clinical findings in these cases include constipation, weak sucking and crying ability, pooled oral secretions, cranial nerve deficits, generalized weakness, and, on occasion, sudden apnea. A characteristic electromyographic pattern termed "brief, small, abundant, motor-unit action potentials" (BSAP) was observed. The sources of C botulinum toxin for these six infants is thought to have been in vivo (gastrointestinal) production following ingestion of C botulinum organisms. Studies are underway to determine the full clinical spectrum, incidence, and potential public health importance of this infectious disease newly recognized in infants.

In utero fetal lamb thyroidectomy and thyroid autograft transplantation.

Our fetal surgical model was utilized to perform in utero fetal lamb thyroidectomy and autograft transplantation of thyroid tissue to fetal thigh at 82-93 days gestation. Successful in utero transplantation was possible in two of six experimental animals. In one twin pregnancy with an unoperated control lamb, observations were continued to age six months. The athyrotic lamb with a thigh autograft was larger at birth and had a transient weak sucking reflex and awkward gait. It then grew and developed normally with no stigmata of cretinism or delay in bone maturation. At age six months an increase in thyroid stimulating hormone (oTSH) was the single distinguishing observation in the twin with the transplant. Although oTSH levels were elevated to age six months, the pituitary continued to be responsive to thyrotropin releasing hormone (TRH) stimulation. These findings suggest that in utero transplantation of thyroid tissue is technically feasible and that the previously described development of in utero cretinism following fetal thyroidectomy can be prevented by a functioning autograft. This technique will be useful in attempting allograft transplantation in utero.

RESPIRATORY METABOLISM IN INFANCY AND IN CHILDHOOD

In a recent paper on infant feeding1the statement is made that one of the problems for further study concerns the best method of feeding the premature infant. The lack of unanimity concerning one aspect of the problem, the proper caloric intake, is indicated in two reviews,2which state that various authors recommend from 75 to 200 calories or more per kilogram of body weight per day. This wide variability derives from: (1) the difficulty of clinical assessment because of the weak sucking reflex and inadequate manifestations of hunger; (2) the paucity of quantitative data on the total energy exchange of premature infants,3and (3) the participation of factors other than diet, such as nursing, environmental conditions and infections, in determining the progress of the premature infant. As part of a systematic investigation of the metabolism of premature infants which may ultimately help to define more clearly