Abnormal vascular tone in infants and children with lung hypoplasia: Findings from cardiac catheterization and the response to chronic therapy.

Objective: We describe four cases of chronic pulmonary hypertension in infants and children with chronic lung disease and pulmonary hypoplasia due to severe congenital diaphragmatic hernia (CDH) or congenital cystic adenomatoid malformation (CCAM). We report data from cardiac catheterization under various conditions: baseline respiratory support and room air, hyperoxic and inhaled nitric oxide challenge. We further report cardiac catheterization measures after chronic pulmonary vasodilator therapy with sildenafil alone or a combination of sildenafil and inhaled nitric oxide (three patients).

Idiopathic congenital central hypoventilation syndrome: the next generation.

Idiopathic congenital central hypoventilation syndrome (CCHS) is a rare disorder in which affected children have a decreased sensitivity of their respiratory centers to hypercarbia and hypoxia, as well as evidence for generalized autonomic nervous system dysfunction. A genetic origin has long been hypothesized for CCHS. Previous reports of the syndrome among twins, siblings, and half siblings, as well as an established association with Hirschsprung disease and neural crest tumors support this genetic hypothesis.

Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia.

The etiology of most cases of acute chest syndrome (ACS) in sickle cell disease (SCD) is unknown. Although pulmonary fat embolism (PFE) is frequently found on autopsy, it is rarely considered in the differential diagnosis in pediatric patients. We conducted a study to determine if we could identify PFE in SCD patients with ACS, define the clinical and laboratory course of PFE, and determine if bronchoalveolar lavage is safe and useful in diagnosis of PFE.

Experience with a metered-dose inhaler with a spacer in the pediatric emergency department.

Studies of adults suggest that metered-dose inhalers with spacers are as effective as hand-held nebulizers for bronchodilator delivery. We studied 13 children with acute asthma. They received two puffs every 2 minutes from metered-dose inhalers with spacers (range, 4 to 14 puffs) titrated until improvement stopped.

Calibration of a heated transcutaneous carbon dioxide electrode to reflect arterial carbon dioxide.

The CO2 measured by a heated skin surface electrode is greater than arterial CO2. In this study we (1) determined the magnitude of this difference retrospectively, (2) adjusted the calibration of the heated transcutaneous CO2 electrode to reflect this difference, and (3) tested the validity of the calibration procedure prospectively. The retrospective study consisted of 252 simultaneous arterial and transcutaneous measurements on 38 infants and children (age range, 1 day to 6 yr).

Congenital central alveolar hypoventilation syndrome in six infants.

Six infants with congenital alveolar hypoventilation syndrome (CCHS) were seen and observed over several years. Two had an association of CCHS with Hirchsprung's disease. All infants were treated by tracheostomy and mechanical ventilation.

Continuous transcutaneous oxygen and carbon dioxide monitoring in the pediatric ICU.

The transcutaneous partial pressures of carbon dioxide (PtcCO2) and oxygen (PtcO2) were measured with heated electrodes in 18 hemodynamically stable pediatric ICU patients and these data were compared to simultaneously measured arterial oxygen and carbon dioxide tensions. There was a significant degree of correlation (p less than 0.001) between the skin surface CO2 and O2 and their corresponding arterial tensions.

Transcutaneous measurements of carbon dioxide partial pressure in sick neonates.

The authors measured the partial pressure of carbon dioxide transcutaneously (PtcCO2) in 15 sick newborns and compared the PtcCO2 to simultaneously measured partial pressure of arterial carbon dioxide (PaCO2). The PtcCO2 values reflected changes in the PaCO2 values. A linear regression on 106 paired PtcCO2 and PaCO2 values produced a correlation coefficient of 4 = 0.

Acid-fast actinomyces in a child with pulmonary actinomycosis.

Open-lung biopsy in a child with chronic pneumonia revealed branched gram-positive acid-fast organisms, later identified as Actinomyces israelii. Like Nocardia asteroides, A israelii can be acid fast by the commonly used Putt stain. The pneumonia was cured with 12 weeks of penicillin treatment.

Experimental murine candidiasis: pathological and immune responses in T-lymphocyte-depleted mice.

Mice depleted of T-lymphocytes by thymectomy and irradiation (TXB) and immunologically competent mice were compared for gross and histological pathology as well as immune responses after cutaneous and/or intravenous challenge with Candida albicans. In response to a first cutaneous inoculation with viable Candida, TXB, sham-operated (SXB), and unmanipulated (normal) mice, all developed lesions of comparable size, duration, and histopathology. When challenged a second time cutaneously, normal and SXB mice developed lesions which were greatly increased in size when compared with those produced by a first cutaneous infection, whereas TXB mice developed lesions comparable in size to those initiated by the first infection.

Malignant cystosarcoma phylloides. Treatment and prognosis.

Twenty-six cases of malignant cystosarcoma phylloides treated at The Charity Hospital of Louisiana were reviewed. No metastases to axillary lymph nodes was observed. The lesion appears to metastasize seldom, if ever, to lymphatics, and axillary dissection is seldom required.

Experimental murine candidiasis: pathological and immune responses to cutaneous inoculation with Candida albicans.

Cutaneous infection of mice with Candida albicans elicited a predominantly acute inflammatory response, stimulated the production of precipitating antibodies, and conferred protection against subsequent intravenous challenge with the same organism. The acute inflammatory skin reaction seen after cutaneous infection suggested a predominantly humoral response to Candida. Animals infected cutaneously a second time with viable C.

Benign liver tumors: what is their relationship to oral contraceptives?

A series of eight cases of benign hepatocellular tumors from the New Orleans area is presented. Clinical and histological features of these unusual tumors are discussed. Also, a series of 56 similar cases reported in the literature is reviewed.