Herman Grossman

NECROTIZING ENTEROCOLITIS IN THE PREMATURE INFANT.

Necrotizing enterocolitis as a usually fatal disease of premature infants has been reported with increasing frequency in recent years, primarily in Europe (2, 11, 12, 14, 15, 23). Though the published cases have appeared under diverse titles including neonatal appendicitis (4, 18, 22), peritonitis (12), colitis (21), ileitis (1), pneumatosis intestinalis (20), or portal vein gas in infants (10, 24), we feel all reflect a basically similar disease process. The current report is based on a study of 21 cases of enterocolitis in newborn premature infants seen between 1954 and 1964; 7 were found in a six-month period in 1963. A more detailed study of the clinical and pathologic findings will be reported separately (9). Clinical Features More than 2,000 infants were admitted to the premature nursery of The Babies Hospital from 1954 to 1964. Seventeen died of enterocolitis (0.9 per cent), and 4 survived. Usually weighing under 1,500 grams at birth, the babies showed a similar clinical pattern. They seemed well d...

Angiocardiography in the apical systolic click syndrome. Left ventricular abnormality, mitral insufficiency, late systolic murmur, and inversion of T waves.

Mitral insufficiency is characteristically associated with a high-pitched, apical pansystolic murmur which radiates toward the left axilla and subscapular areas. Murmurs heard in late systole and systolic clicks were considered benign and were thought to originate in extracardiac structures (9), possibly related to pleural and pericardial disease. Recently, however, it has been demonstrated that a number of patients with late apical systolic murmurs, whether initiated by a click or not, have varying degrees of mitral insufficiency. A late apical systolic murmur may be heard in rheumatic mitral insufficiency, subaortic stenosis, coarctation of the aorta, and postmyocardial infarction and with a calcified mitral annulus associated with mitral insufficiency. The cases being reported are all in young females without previous history of congenital or acquired cardiac disease. The term “click” is used to describe a very short, high-pitched systolic clicking sound. This term was introduced by Gallavardin (5) in ...

Extramedullary Hematopoiesis in Patients with Thalassemia Anemia

Abstract A high incidence of thoracic extramedullary hematopoiesis (TEH) was found among patients with thalassemia intermedia. The masses occurred from T2-T11; a few were unilateral in location. The usual clinical course of such patients appears to be related to the physiological requirements for the development of such masses. The pathogenesis of TEH is discussed.

Cholelithiasis in Thalassemia Major

Abstract In a study of patients with thalassemia major by oral cholecystography, the incidence of cholelithiasis is determined. Six of 26 patients aged ten years and older have biliary calculi; in addition, gallstones have been demonstrated during life or at autopsy in 7 of 35 who have died. The association of gallstones with thalassemia, less frequent than in hereditary spherocytosis, has the same degree of magnitude as the relationship in sickle-cell anemia. The authors formulate an explanation for these different frequencies of stone formation in hemolytic disease.

Hyperimmunoglobulinemia E Syndrome: Radiographic Observations

Susceptibility to recurrent staphylococcal cutaneous and respiratory infections beginning in infancy associated with extreme hyperimmunoglobulinemia E is a recently described primary immunodeficiency syndrome. Other clinical features include depressed cellular immunity and deficient antibody formation. Recurrent pneumonia and cyst formation with variable persistence and expansion characterized the radiographic couse in 11 patients. Five cysts resolved with continuous antistaphylococcal therapy; 2 were resected without recurrence; and 4 persisted after surgery and/or antibiotics (2--8 years). The cysts had dense, necrotic surfaces with fibrous walls, eosinophilic and other inflammatory cell infiltrates, and frequent, persistent, bronchial connections. Sinusitis (9/9) and mastoiditis (3/4) were also observed radiographically.

The campomelic syndrome. Congenital bowing of limbs and other skeletal and extraskeletal anomalies.

The radiographic and clinical findings of the campomelic syndrome are described. Radiographically, it is characterized by bony and cartilaginous growth disturbances manifested by bowing of the femurs and tibiae, hypoplasia of the vertebral bodies, scapulae, and pelvis, and dislocation of the hips, knees, and elbows. These findings clearly differentiate this entity from other short-limbed dwarfisms. Clinically, these infants are short-limbed with tibial and femoral bowing, with dimpling over the tibial bow. Generally there is cleft palate, macrocephaly, hypertelorism, micrognathia, hypotonia, and laryngotracheal malacia. Most patients with this syndrome die in infancy, usually of respiratory disorders.

Vertebral Sarcoidosis in Adolescents

Two cases of vertebral sarcoidosis in pediatric patients are presented. All such patients reported have been black, 13-15 years old, and have a history of back pain. Radiographs of the involved vertebrae show primarily lytic destruction with sclerotic borders in some of the lesions. Fungal infections, tuberculosis, pyogenic osteomyelitis, Hodgkins disease and metastatic disease must be considered in every patient with vertebral sarcoidosis.

REVERSIBLE GASTROINTESTINAL SIGNS OF HEMORRHAGE AND EDEMA IN THE PEDIATRIC AGE GROUP.

Reversible changes in the gastrointestinal tract of adults due to bleeding and edema have been described clinically (11, 14) and produced experimentally (11). It is not unexpected that examples should be found in the pediatric age group of diseases of diverse etiology with common roentgen manifestations, relating to hemorrhage, ischemia, edema, and necrosis with return to normal. Schonlein-Henoch purpura, giant hemangioma with platelet trapping, classic hemophilia, and postoperative ischemia of a colonic transplant for reconstructing the esophagus in esophageal atresia have all presented with signs of gastrointestinal bleeding and examples of “thumbprinting” and “pseudotumor” defects on barium studies. Schonlein-Henoch purpura (nonthrombocytopenic purpura) In children with Schonlein-Henoch nonthrombocytopenia or anaphylactoid purpura, swollen painful joints, cutaneous purpura, and abdominal pain with associated bleeding from the intestinal tract are characteristic. The abdominal pain, often colicky, may p...

Wilson's Disease Presenting with Rickets

Wilsons disease is an inherited disorder of copper metabolism. Resultant kidney damage can induce metabolic bone disease, and in children the radiographic manifestations are indistinguishable from those of classical rickets. The patient who is the subject of this review was originally diagnosed as having vitamin D-resistant rickets. He had been well until the age of eight years, when pain and weakness of both knees developed in association with fever. Radiographs of knees and wrists (Fig. 1) taken at another hospital disclosed rachitic changes. Anemia, hypophosphatemia, and hypocalciuria were found. A diagnosis of vitamin D-resistant rickets was made. Treatment with vitamin D at a dose of 100,000 units daily induced symptomatic and radiographic evidence of remission (Fig. 2), but vitamin D intoxication supervened and the drug was discontinued at age ten. Initial evaluation at The New York Hospital in 1966, when the patient was eleven years old, showed return of rachitic changes on radiographs of the wris...

Neonatal Focal Hyperaeration of the Lungs (Wilson-Mikity Syndrome)

AHITHERTO undescribed form of respiratory distress in premature infants was reported by Wilson and Mikity in 1960 (8), associated with roentgenographic visualization of coarse thickening of the interstitial structures of the lungs. Since then, similar instances have been described under the terms “cystic emphysema” (3), “pulmonary dysmaturity” (1), and “a new form of lung fibrosis in premature infants” (4, 5). The onset of symptoms varies between birth and the thirty-fifth day of life, with a mean of eight days. The outstanding symptom is cyanosis requiring oxygen. Auscultation of the lungs is clear in some infants, in others râles, rhonchi, and/or wheezing are revealed. In infants who survive the neonatal period, the chest roentgenogram clears within a period of several months. The purpose of this communication is to discuss the criteria for the x-ray diagnosis of neonatal focal hyperaeration and to demonstrate the sequence of roentgen findings. Material and Methods During the two-year period (January 19...