Familial Hirschsprung's disease and type D brachydactyly: a report of four affected males in two generations.

Hirschsprung's disease usually occurs as an isolated malformation as a result of multifactorial causation. A family in which four males (two brothers and two maternal uncles) had Hirschsprung's disease and absence or hypoplasia of the nails and distal phalanges of the great toe and thumb (type D brachydactyly) is described. Hand abnormalities were not present in any other family members, and the obligate heterozygous females were without gastrointestinal problems. The pattern of inheritance was consistent with X-linked recessive inheritance; however, autosomal dominant inheritance with incomplete penetrance in females or multifactorial causation could not be completely excluded.

Cervical spine anomalies and tumors in Weaver syndrome.

Weaver syndrome is an autosomal dominant disorder comprising accelerated growth rate and rapidly advancing skeletal maturation. Previous reports suggest that the phenotype in adults may be sufficiently subtle to make diagnosis difficult. Half brothers with classical childhood findings of Weaver syndrome and their father with minimal clinical findings showed cervical spine anomalies that likely represent a consistent radiographic finding in this disorder. One of the children represents the third occurrence of neoplasia in Weaver syndrome.

Chondrodysplasia punctata stemming from maternal lupus erythematosus.

The finding of stippled epiphyses on a neonatal radiograph generates a wide differential diagnosis, including genetic and teratogenic causes. We report the case of a male infant with stippled epiphyses evident on neonatal radiographs in whom a typical rash of lupus erythematosus developed. The skin abnormalities in the infant resulted in a diagnosis of systemic lupus erythematosus in his mother. Over a 3-year follow-up period, the child has demonstrated strikingly short stature, midface hypoplasia, anomalous digital development, slow resolution of the stippled epiphyses, and near normal cognitive development. The differential diagnosis of chondrodysplasia punctata and the literature supporting maternal lupus as one cause are reviewed.

Lung tumor incidence after intrabronchial administration of the nonionic contrast agent metrizamide.

RATIONALE AND OBJECTIVES: Metrizamide has been used for examination of the gastrointestinal tract and tracheobronchial tree of infants. Contrast agents may enter the lungs during such examinations. The current study was undertaken to determine whether there would be any later pulmonary effects when metrizamide was administered to the lungs of weanling mice. METHODS: One hundred fifty mice (18-21 days old), divided into groups, received either 75 microL of metrizamide, using the manufacturer's diluent (190 mg iodine [I]/mL), or saline solution administered to the lungs by injection into the trachea. The mice were observed for the duration of their lives. Moribund animals were killed. At death, all animals underwent necropsy. The lungs were fixed in formalin, and histologic sections were examined for pathologic changes. RESULTS: The incidence of lung tumors was increased (P less than .05) in the lungs of mice receiving metrizamide compared with those receiving saline. Eighteen percent of the lung tumors in the metrizamide-treated mice were lymphomas, a histologic type not found in the saline-treated controls. CONCLUSIONS: A hypothesis proposing that metrizamide may be an initiator of carcinogenic transformation rather than a carcinogen was developed.

Radiation dose reduction in the neonatal intensive care unit. Comparison of three gadolinium oxysulfide screen-film combinations.

Infant radiation exposure in the neonatal intensive care unit was quantitated by thermoluminescent dosimetry in 513 mobile unit roentgen examinations of the chest and abdomen. Three combinations of gadolinium oxysulfide intensifying screens and radiographic films were compared. The skin entrance dose in millirads at the mid-chest, mid-abdomen and symphysis pubis levels was measured. Radiation dose reductions of 52% and 68% were achieved using the intermediate and fast combinations relative to the near par speed standard combination. Comparison of the relative line pair resolution of the three combinations under clinical conditions was done and subjective comparison of image detail and image mottle was made by two radiologists. The standard and intermediate speed combinations were considered very acceptable for general neonatal diagnostic interpretation but the fastest combination was not considered acceptable for general purposes. Because of the decreased radiation dose, the intermediate speed combination is now used for all routine neonatal radiography at our institution. The near par speed combination is now used only occasionally for "high-detail" examinations. The fastest combination has a limited role for frequently repeated, less critical studies, such as tube or catheter placement or routine follow-up examinations.

Triplication of the esophagus with gastric duplication.

A 2 1/2-year-old girl was seen with an upper abdominal mass. Evaluation by abdominal ultrasonography, esophagography, upper gastrointestinal series, and computerized axial tomography suggested the possibility of duplication of the esophagus, stomach, and duodenum. Surgery revealed a triplication of the esophagus with a gastric duplication, which was resected. The patient recovered and greatly increased her weight-for-age percentile. Of the theories proposed to explain gastrointestinal duplications, the split notochord hypothesis most closely fits this patient's anomalies. It is unusual for a patient with a gastrointestinal duplication to develop symptoms after the first year of life. This is the first report of a triplication of the esophagus with a gastric duplication.

The child with acute abdominal pain and vomiting.

Evaluation of the child who presents with abdominal pain and vomiting is difficult. Close cooperation between pediatricians, surgeons, and radiologists is necessary to identify situations requiring urgent intervention. Close attention to history and physical findings will allow appropriate, judicious use of modern imaging techniques to arrive at an early diagnosis and initiate proper treatment when necessary.

An approach to the asymmetric neonatal chest radiograph.

Conditions causing respiratory distress in the neonate, which may require surgery or an interventional procedure, typically have asymmetric radiographic findings. The findings are conveniently categorized from the plain radiographs as those producing a large lucent-appearing hemithorax, those producing a large cystic-appearing hemithorax, and those producing a large opaque-appearing hemithorax. Additional imaging with ultrasound, CT, MR imaging, or fluoroscopy can be planned based on the initial radiographic appearance.

Cognitive therapy of delusional beliefs.

The present article articulates a cognitive theoretical perspective of delusional beliefs. In contrast to the focus of psychoanalytic therapy (theoretically-derived phenomena beyond personal awareness), the cognitive therapist focuses primarily on the conscious cognitive-experiential level in the treatment of delusional beliefs, thereby emphasizing a 'common-sense' level of analysis or reasoning shared by the delusional patient. Unlike noncognitive behavioral approaches, which focus on topographical 'verbal behavior' as such, cognitive therapy directly targets specific delusional beliefs which theoretically give rise to the disordered verbal behavior. In this article, the adaptation of cognitive therapy to the treatment of delusions is described, with special attention to the following issues: special problems in collaboration; difficulties in obtaining conviction ratings; how to avoid and reduce confrontation through the Socratic method; the collaborative design of homework experiments; distancing strategies; interpersonal relationship issues; and the necessity to identify and explore the emotions associated with the various delusions, especially feelings about the possibility that the delusions may be incorrect.

Follow-up abdominal computed tomography after splenic trauma in children may not be necessary.

Nonoperative management of splenic injuries in children is well accepted. However, the need for follow-up abdominal CT to document splenic healing has not been well studied. We retrospectively reviewed initial and follow-up abdominal CT examinations of pediatric patients admitted to our institution with documented splenic trauma who were managed nonoperatively. Eighty-four patients were admitted to our pediatric surgical service with splenic injury documented by CT from 1994 through 1998. The standard approach for splenic injury was bedrest for 5 to 21 days and limited activity for up to 90 days at the discretion of the attending surgeon. Thirty-five of the 84 had follow-up CTs during outpatient follow-up to evaluate and document splenic healing by CT criteria. The initial and follow-up studies were randomized and read blindly by pediatric radiologists using a modified American Association for the Surgery of Trauma grading system (I-V). The age range of the patients was 6 months to 17 years (mean +/- SE; 11 +/- 1 years). Nineteen (54%) were male and 16 (46%) were female. Causes of splenic trauma included motor vehicle accident (22), fall (seven), assault (four), pedestrian versus vehicle (one), and sports injury (one). Eight children (23%) had grade II injuries, 14 (40%) had grade III injuries, and 13 children (37%) had grade IV injuries on initial CT scan. Seven (88%) of the grade II splenic injuries were healed by 64 +/- 11 days. The remaining grade II injury had healed by 210 days. Thirteen (93%) of the grade III splenic injuries were healed by 76 +/- 7 days. The remaining grade III injury was healed by 140 days. Spleens in 10 (77%) of the 13 patients with grade IV injuries were healed by 81 +/- 8 days. Of the three remaining grade IV injuries two were healed by 173 +/- 14 days. The remaining patient's spleen was radiologically considered to have a grade III defect 91 days from the time of injury, and no further CTs were obtained. Of the 34 patients who underwent follow-up CT imaging until splenic healing was demonstrated the mean time to complete healing was 87 +/- 8 days postinjury (range 11-217 days). These data suggest that routine follow-up abdominal CTs may not be necessary to allow children to resume their normal activities after an appropriate time of restricted activity.

Pleuroperitoneal shunts in the management of neonatal chylothorax.

Pleuroperitoneal shunts have been placed in five ventilator-dependent newborns with persistent chylothorax. The etiology of the chylothorax appeared to be secondary to superior vena caval obstruction in three patients and was idiopathic in the remaining two. Despite traditional therapies these infants were on a progressively deteriorating clinical course. Hakim-Cordis low-pressure ventricular-peritoneal shunt catheter systems were used in each infant. Ultrasonography was used to follow the regression of pleural effusions and to determine the need for shunt compression. Shunt patency was confirmed with radionuclide studies. Four of five infants had a complete resolution of their chylothorax and pulmonary insufficiency. Three of these infants were extubated within 28 days following the placement of the shunt. Nutritional and metabolic stability was rapidly achieved. The shunts were removed several weeks later without recurrence of the chylothorax. A fifth infant failed to improve after the placement of the pleuroperitoneal shunt and died of progressive pulmonary insufficiency. The placement of pleuroperitoneal shunts in infants with refractory chylothorax is safe, technically easy to perform, and is associated with few complications.

Integration of scientific criteria into the psychotherapy integration movement.

Psychotherapy integration has recently received considerable attention among behavior therapists. Although the wisdom of ecumenicism has been questioned, behavior therapy has often been credited with flexibility and modification of practice based on experimental and clinical research. From this perspective, the two central criteria for psychotherapy integration are inclusion of proven therapeutic elements and exclusion of unproven ones. Rationales for the importance of both criteria are discussed, and an analogy to the development of modern medicine is suggested. Formal acceptance of the proposed criteria would place psychotherapy integration in a proactive position to censor fee-for-service clinical practice not grounded in basic clinical or experimental research.

Behavioral design of a positive verbal community: a preliminary experimental analysis.

Behaviorists have theorized (and experimental analyses suggest) the potential clinical application of verbal behavior modification. This study evaluated therapeutic effects of behavioral intervention to modify the intact verbal community. The setting was an adolescent operant treatment center for behavioral disorders. All residents within the center, 16 females and 22 males, participated in the study. A within subjects experimental design compared effects of a positive verbal community (PVC) plus the ongoing operant treatment program to the operant program alone. Conceptually, these were dual-level and single-level operant programs, respectively. Dependent measures included rates of positive goal-relevant verbalizations of residents, and clinical measures of self-control and psychiatric symptoms. Preliminary evidence supported the feasibility of the PVC as a potential novel therapeutic intervention.

Processes of clinical change and resistance. A theoretical synthesis.

Evidence from both experimental laboratory studies and clinical observation supports the behavioral principle that immediate (compared with delayed) consequences are most influential in shaping future actions. This presents the theoretical possibility of conflicts of consequences (e.g., short-term positive vs. long-term negative). As one example, resistance to completing therapeutic homework assignments that instruct clients to approach feared situations may result in short-term positive outcomes, such as freedom from negative emotional experience (emotional avoidance), but is dysfunctional over time. Thus, temporal conflicts of consequences is one theoretic source of resistance in clinical treatment. In this article, the authors articulate how the activation of the metacognitive level theoretically mediates conflicts between short-term (immediate) and long-term (delayed) consequences, thereby facilitating therapeutic change and reducing resistance. This synthesis unifies principles of behaviorism and contemporary clinical cognitive theory.