Status Of Infants Research Articles

A syndrome of congenital hypoplasia of the alae nasi,situs inversus, and severe hypoproteinemia in two siblings

Patient 1. This boy was the second of four children of healthy parents. We are not aware of any consanguinity, and no familial anomalies are known. The first and third siblings are healthy; the fourth is presented as Patient 2. The 22-year-old mother had an uneventful pregnancy and a normal delivery at term. The birth weight was 3,440 gin, length 49 cm, and head circumference 34 cm. The infant had hypoplastic alae nasi and slightly hypoplastic auricular conchae, together with a midline ectodermal scalp abnormality including an atrophic skin area over and in front of the anterior fontanelle. At 6 days of age he was discharged in clinically good condition. Two weeks later he was hospitalized because of failure to thrive, accompanied by vomiting and poor weight gain. The clinical findings included slight icterus, with a bilirubin concentration of 180 #mol/L (10.5 mg/dl), liver enlargement, and a pansystolic murmur localized mainly to the right of the sternum. Radiologic examination of the chest and lungs revealed situs inversus and cardiac decompensation. Because of his poor general condition, no further investigations could be performed regarding the nature of the suspected cardiac malformation. An ECG showed suspected signs of dextrocardia but no other conclusive signs of heart disease. Gradually increasing hypoproteinemic edema was noted, especially of the hands and feet. The hypoproteinemia could not be attributed to severe impairment of liver function or to renal protein loss. Though laboratory investigations revealed slightly increased levels of aspartate-aminotransferase and alanine-aminotransferase, the prothrombin index was normal. The liver enzyme values returned to normal after treatment for the heart failure. The serum protein concentration of 46 gm/L with an albumin level of only 22 gm/L was not affected by repeated infusions of albumin. The infant's condition was too precarious to allow further studies concerning proiein-losing gastroenteropathy or trypsinogen deficiency disease. Fibrocystic disease

Intestinal stricture after necrotizing enterocolitis

Between 1974 and 1979 inclusive, 20 of 175 neonates with previous necrotizing enterocolitis (NEC) developed obstruction from intestinal stricture 1-20 mo after the diagnosis of NEC. Seven were found in the small bowel, 19 in the colon. Treatment included proximal decompressive enterostomy with subsequent resection in 6 and primary resection in 11. Two died during attempts to restore GI continuity and one died from cardiac disease before any GI surgery. Morbidity occurred in six. Various stages of wound healing from acute inflammation to dense fibrosis were found in pathologic specimens. Fibrosis was most marked in the submucosa and most consistently found in specimens resected 3 mo after the acute episode of NEC. Intestinal stricture develops in 6%-33% of the infants with previous NEC. Eighty percent of these strictures are colonic, 30% are left sided, and 15% are multiple. Multiple lesions are exclusively colonic. Histology varies with age of lesion. The predominant feature of stricture is submucosal fibrosis. Strictures less than 3 mo old still have acute inflammatory disease, older strictures are mature and fibrotic. The best diagnostic tools are a high index of suspicion and a barium enema. An intestinal stricture should be suspected in any infant with a past history of NEC and: (1) symptoms of obstruction or failure to thrive; (2) previous exteriorization or proximal diversion of acute or chronic NEC lesions; or (3) peritoneal drainage under local anesthesia for NEC perforation. Treatment should be tailored to the infant's condition and the time elapsed since the acute NEC episode. Resection of the stricture must be complete otherwise recurrence or leak is unlikely. Intestinal diversion with a 3-6-mo delay before reconstruction appears safer in the child with little margin for error. Earlier restoration of blood flow and cessation of intestinal function during the acute episode of NEC might reduce the incidence of stricture.

The morbidity of total thyroidectomy.

Of 245 total thyroidectomies done over a ten-year period, most were in patients who had previously received irradiation about the head and neck for benign conditions in infancy and childhood. There was no operative mortality, and only one patient had postoperative bleeding requiring reoperation. Of six patients with paralyzed recurrent laryngeal nerves in the immediate postoperative period, vocal cord function returned in all but one over one year. Forty-nine patients required at least one dose of calcium postoperatively, and 41 were taking calcium when discharged from the hospital. After one year, only two patients took calcium regularly; in both, lymph nodes were removed from both paratracheal grooves. In evaluating comparative statistics, increased morbidity of total thyroidectomy needs to be considered on a long-term basis rather than in the immediate postoperative period.

An epidemiological study on carcinoma of the breast following irradiation for benign conditions in infancy and childhood

An epidemiological study on carcinoma of the breast following irradiation for benign conditions in infancy and childhood

Rumination in infancy: Recent behavioral approaches

The published literature on behavioral treatments of rumination is reviewed, and new case material on two recently reported procedures is presented. Rumination, a medical condition of infancy involving repeated regurgitation of previously ingested food, has historically been treated from a psychodynamic approach involving extended in-patient treatment by mother surrogates. This procedure is both time-consuming and very costly. The initial behavioral approach involved electric shock. Single-case studies reflect the utility of this procedure, but widespread resistance to this form of punishment makes this a difficult procedure to utilize. More recent behavioral procedures reported in single-case studies have included delivering aversive taste stimuli as consequences for ruminating. This procedure has met with mixed success, and has the added drawback of being difficult to implement technically and because of staff resistance. A combination of punishment by scolding, time-out, and reinforcement of retaining the food constitutes a useful alternative. This procedure has the added advantage of being more acceptable to staff and implementable at home. The possibility of individual differences among ruminators that might predict success is also raised.

Management of Patent Ductus Arteriosus in Preterm Babies

There is a higher incidence of delayed closure of the patent ductus arteriosus in premature babies with respiratory distress syndrome. From July, 1975, to December, 1977, 57 small, preterm infants with patent ductus arteriosus were diagnosed at our neonatal intensive care unit. From July, 1975, until September, 1976 (first period), 23 patients were diagnosed, and 11 underwent surgical ligation of a patent ductus arteriosus. There were 3 early deaths. From October, 1976, until December, 1977, out of a total of 34 patients with diagnosed patent ductus, 18 were treated with indomethacin, and only 3 required ligation. Our present policy for patent ductus arteriosus with respiratory distress syndrome in the premature baby is to initiate early treatment with indomethacin. If this treatment fails and the infant's status deteriorates, we perform early surgical ligation of the ductus in order to minimize the time on mechanical ventilation and lessen the chances of the development of bronchopulmonary dysplasia.

Latrogenic Acute Nasal Obstruction in an Obligate Nose Breather

NASAL congestion is a common problem of infants. There are many causes of this condition: congenital abnormalities, trauma, infections, environmental irritants or allergens, foreign bodies, and intranasal drug use. Although nasal congestion is not usually considered an urgent or emergency situation, it may become catastrophic in an obligate nose breather. Recently an infant came to our emergency department with acute nasal obstruction from the use of hypertonic saline nose drops. Report of a Case A 34-day-old girl was brought to the emergency department with nasal congestion and severe respiratory distress. The mother had taken the baby to another hospital for a runny nose and nasal congestion two days previously. The physician at the clinic had instructed her to use a vaporizer and salt solution nose drops every four hours. During the next 36 hours, the infant's condition improved; however, she then became congested again, with the development of intermittent, gasping

Nursing implications in the early recognition of necrotizing enterocolitis.

Necrotizing enterocolitis is “a condition in which there is diffuse or patchy necrosis of the mucosa or submucosa in the small and/or large bowel. The process appears to be localized initially but can spread through the bowel wall to cause perforation, or longitudinally along the wall, leading to the infarction of long segments of the gut.”1 This definition, stressing the likelihood of a rapid spread of the disease, is evidence of the necessity of early recognition of its signs and symptoms. Neonatal nurses, functioning as primary caregivers, are in a position to recognize vital changes in an infant's condition. The continuity of neonatal care enables these nurses to assess accurately an infant's “normal” daily functions. Any deviation from this assessment is immediately evident. In necrotizing enterocolitis, as in many other diseases, early recognition of symptoms, followed by accurate diagnosis and early treatment, greatly enhances the survival rate of affected prematures.

Malformation syndrome associated with small extra chromosome.

We report an infant with a previously apparently unreported lethal malformation syndrome concentrated primarily on the left half of the body, but including midline defects such as meningomyelocele, cleft lip and cleft palate, and imperforate anus. The infant also had a small extra metacentric chromosome in 60% of lymphocytes. It is postulated that the small extra chromosome (whose origin remains unknown) caused the infant's condition, and that its asymmetry is compatible with an uneven distribution of the normal and aneuploid cell lines in the two halves of the body during critical phases of prenatal development.

Intrauterine testicular torsion: Not an emergency

A case of intrauterine testicular torsion and infarction is described. Urgent exploration of the testis has been advocated in the management of this condition. However, the risks of early anesthesia in the immediate postdelivery period must be weighed against the chances of a successful outcome of the operation in preserving testicular function. Since this salvage rate is poor, we suggest surgical exploration at a time when the infant's condition is stable, unless there are indications that the event is very recent.

Fatal Haemophilus influenzae Endocarditis Diagnosed by Echocardiography in an Infant

A 1-year-old boy was admitted to the hospital with Haemophilus influenzae meningitis. On the second hospital day a heart murmur heard on admission seemed louder. No signs of congestive heart failure were present. An echocardiogram demonstrated a persistent echo-dense mass behind the anterior leaflet of the mitral valve. A presumptive diagnosis of endocarditis was made. After ten days of antibiotic treatment the child was considerably improved. Treatment was continued for endocarditis. Over the next several days the infant's condition deteriorated. On the 16th hospital day he died. Autopsy examination showed a large vegetation attached to the posterior mitral valve leaflet. Bacterial endocarditis caused by H influenzae is uncommon at any age and rare in children. Echocardiography provided a definitive diagnosis early in the hospital course of this patient.

Critical conditions in infancy: A psychological perspective.

Critical conditions in infancy: A psychological perspective.

Critical conditions in infancy: a psychological perspective.

Critical conditions in infancy: a psychological perspective.

Indicator dilution curves in the diagnosis of D‐transposition of the great arteries in infancy

Paired right and left atrial indicator dilution curves performed in a series of 17 patients with D-transposition of the great arteries (D-TGA) were reviewed. Taken together, the patterns obtained from patients who had trivial mixing between the pulmonary and systemic circulations were distinctive and easily recognizable. In the presence of substantial degrees of intracardiac mixing, however, the paired curves recorded become virtually identical and cannot be distinguished from curves recorded from patients with other forms of cyanotic congenital heart disease characterized by obligatory admixture. Thus paired atrial dye curves demonstrate a high degree of diagnostic specificity for the infant with D-TGA at greatest risk, normally those with a small intracardiac shunt. Such a procedure should facilitate early balloon atrial septostomy and obviate deterioration in the infant's condition due to prolonged catheter manipulation and/or angiography.

Postirradiation neoplasia: A symposium

Summary It is impossible to prove that radiation given for benign conditions in infancy and childhood is an etiologic factor in the subsequent development of carcinoma in the head and neck area. Nevertheless, the suggestive evidence is quite impressive and the information from many sources correlates well. There are some conflicting reports but, in general, it can be said that very high doses of radiation are less likely to be carcinogenic than lesser amounts. Very small doses of radiation are probably not carcinogenic either. In general, the younger the patient at the time of exposure to radiation, the greater is the risk of subsequent development of carcinoma. The breast seems to be an exception, however, as it is more sensitive to radiation carcinogenesis between the ages of 10 and 35. Chronic low doses of radiation seem to be more carcinogenic in humans. The latent interval between irradiation and the appearance of carcinoma in most sites averages about 30 years; leukemia tends to occur 5–8 years after irradiation. The atomic bomb data are confusing. Up to 30% of the Hiroshima bomb radiation consisted of neutrons, whereas the neutron radiation of the Nagasaki bomb was very low. The higher incidence of breast cancer associated with the former type of irradiation may be related to the higher carcinogenicity of neutrons. On the other hand, the lower normal incidence of breast carcinoma in Japanese women may be a modifying factor of RBE of radiation in tumor induction. Irradiation for malignant tumors in all parts of the body, although an exceedingly valuable addition to the cancer therapy armamentarium, probably predisposes to the later development of irradiation carcinoma in the long-term survivors. This becomes increasingly important as more children and young adults with malignant tumors are clinically cured of their original malignant tumor with multimodal therapy including irradiation. The risk of carcinogenesis in radiation-treated patients never disappears. These patients should be followed carefully for life.

996 NEONATAL TRANSPORT IN SUB-ZERO AMBIENT CONDITIONS: CLINICAL THERMAL STABILITY WITH RADIANT WARMER

11 sick neonates (weighing 1150 - 2280g) have been studied during transport from sub-zero temperatures (0 to -34°C) in a radiant heat source incubator. This unit has been evaluated ‘in vitro’; our referral centre provided unique opportunities for clinical observation under extreme conditions. One observer monitored the infant's condition, core, skin and incubator air temperatures (4cm below the hood). Environmental temp. and wind speed were obtained. The radiant heater, in a curved transparent canopy generated heat at peak wave length of 8.7 micron over 180C of the infant's surface, servo-controlled from a skin thermistor. Core temperature never fell, and rose to normal in all (6) infants who were hypothermic (mean rise >0.5°C/hour for 3 hours) The greatest fall in skin temperature was <0.75°C (after 10 minutes unavoidable delay at -21°C with the heater control accidentally lowered to 35°C while unloading). Considerable flux in air temperature within the incubator occurred between periods of warmer activity. Once, a broken connection in the servo-control thermistor rendered the heating unit temporarily inoperable. Radiant heat can provide a stable thermal environment under adverse conditions; but data suggest an emergency manual override of thermistor control is mandatory and that heater controls should ‘lock’. Also, the optimum rate of rewarming after hypothermia, and the effects of a marked flux in air temperature must be established for small infants during radiant warming.

Maternal language to prelinguistic infants: Semantic aspects

Transcripts of maternal language to 4-, 6-, and 8-month-old prelinguistic infants were made. Measures of maternal speech included commands to do something, prohibitions, directing attention, invitations to do something, giving information about the infant's condition or about objects, and various types of speech, such as game language. Results showed that mothers of 8-month-olds used more commands and more direct attentions, while mothers of 4-month-olds used more comments about the infant's condition. The data are interpreted to indicate that the older infants elicited these language alterations because of their increasing repertoire of motor behaviors and, more importantly, their developing receptive language skills.

Academic Assumptions Annoy Practitioner

To the Editor: The letters on the danger of Actifed-C (Pediatrics 57:164, January 1976) exemplify an academic assumption of superior knowledge that annoys me no end. Drs. Ivey and Kattwinkel assert that “there is no condition in infancy for which codeine would be indicated.” I infer from this that they have never been called upon to relieve the pain of otitis media or a burn or a fracture-all occasionally known to befall infants, nor have

INCREASED PHYSICIAN EFFICIENCY IN THE INTENSIVE CARE NURSERY WITH A SINGLE ENTRY OF DATA AND COMPUTERIZED PRODUCTION OF FORMS, SUMMARIES AND LETTERS

As the concern for the high-risk infant increases and the disease states multiply, so do the demands for adequate documentation and communication and correspondence to parents, physicians and allied health agencies regarding that infant's status. In order to provide accurate recording and distribution of this information, i.e., transport note, letters, State forms, discharge summary, etc., while preventing an unnecessary duplication of physician paperwork, the following project ensued. A comprehensive four page form for the recording of all available historical and pertinent data about the infant has been devised and repeatedly streamlined. An on-line computer system using COBOL Language and direct entry with light pen has evolved after the initial punch card entry system proved financially feasible and successful. Unconstrained data, e.g., exact temperature, weight and dose rather than group categories are recorded. The test of this system has been willing physician compliance and acceptance by the agencies and departments requesting data about the infant. The reduction of paperwork by > 50% has provided incentive with > 95% physician participation and acceptance. There is immediate availability of data on discharge.

Ductus Arteriosus Dilatation by Prostaglandin E1 in Infants With Pulmonary Atresia

Infants with pulmonary atresia depend on patency of the ductus arteriosus for survival in the immediate postnatal period. Despite continuing hypoxemia after birth the ductus arteriosus usually constricts, thus reducing pulmonary blood flow. This often occurs while awaiting surgical palliation or correction, leading either to marked deterioration in the infant's condition, or death. In ten infants with pulmonary atresia, we infused prostaglandin E1 (PGE1) at a rate of 0.1 mug/kg/min in six and 0.05 mug/kg/min in four into the descending aorta at the orifice of the ductus arteriosus. The ductus arteriosus was effectively dilated; at the narrowest point the diameter, measured in eight infants, almost doubled. In all ten infants arterial blood PO2 increased, averaging 24.6 mm Hg before and 43.7 mm Hg after the infusion was started. Infusion of PGE1 directly into the aorta adjacent to the ductus arteriosus avoided the complications of pyrexia, muscular twitching, and excitability which may be related to the effects of prostaglandins on the central nervous system.