Lingeous conjunctivitis with tracheal obstruction. A case report, with light and electron microscopy findings.

A white male infant of 1 year had unilateral membranous conjunctivitis and severe laryngotracheobronchitis which required tracheostomy. Cultures from eye and throat swabs and of fluid suctioned through the tracheostomy grew many organisms, including H. influenzae, adenovirus type 3, and Candida species, but he had no specific immunologic disturbance.

Ultrastructure of airways in children with asthma.

This study describes the histopathology and ultrastructure of bronchial mucosa in lung biopsies from two children with bronchial asthma in remission, and compares them with lung samples from two children who died in status asthmaticus. Light microscopy of all samples showed changes typical of bronchial asthma, e.g.

Identification of neuro-epithelial bodies in rabbit fetal lungs by scanning electron microscopy: a correlative light, transmission and scanning electron microscopic study.

In the present study, neuro-epithelial bodies (NEB) were identified by scanning electron microscopy (SEM) within the bronchial epithelium of near-term rabbit fetuses. The surface features and topography of NEB were correlated by light and transmission electron microscopy. In SEM, the surfaces of NEB were easily visualized in smaller peripheral airways because of paucity of ciliated cells in these regions.

Reye's syndrome: preservation of mitochondrial enzymes in brain and muscle compared with liver.

The activities of five mitochondrial enzymes tested in liver from patients with Reye's syndrome were measured. Citrate synthase, glutamic dehydrogenase, succinic dehydrogenase, pyruvate carboxylase, and pyruvate dehydrogenase were all outside of the range shown by control samples and well below them in activity. The activity of two extramitochondrial enzymes, glucose-6-phosphatase, which is a microsomal enzyme, and fructose-1,6-diphosphatase, which is a soluble enzyme, were in the normal range in samples from Reye's syndrome patients.

Familial enteropathy: a syndrome of protracted diarrhea from birth, failure to thrive, and hypoplastic villus atrophy.

We have studied 5 infants with persistent severe diarrhea from birth and marked abnormalities of absorption associated with failure to thrive leading to death in 4 infants. Three had siblings who died and a sibling of a 4th is ill at present, all with a similar illness; 2 were the products of consanguinous marriages. Exhaustive investigation failed to identify a recognized disease entity in any patient.

Improved ventilation of prematurely delivered primates following tracheal deposition of surfactant.

Twelve rhesus monkeys were delivered prematurely at 129, 130, or 131 days. The first breath was inhibited while tracheotomy was performed and a catheter introduced into the umbilical artery. Into the tracheal tubes of six of the newborn monkeys was instilled 0.

alpha1-Antitrypsin: the presence of excess mannose in the Z variant isolated from liver.

The Z variant of alpha1-antitrypsin was isolated by a new technique from the liver of a patient homozygous for the Z allele of the protease inhibitor locus. The material was homogenous and antigenically competent but had no protease inhibiting capacity. An interesting correlation was found between the subcellular localization and the carbohydrate composition of the Z variant from liver.

Hyaline membrane disease. Effect of surfactant prophylaxis on lung morphology in premature primates.

Neonatal lung morphology was evaluated in 12 rhesus monkeys delivered by caesarean section 1 month before term and cared for as human premature neonates. In 6 monkeys, 0.20 to 0.

Hepatic function following portoenterostomy for extrahepatic biliary atresia.

Liver structure and function in 10 patients with extrahepatic biliary atresia were studied after portoenteric anastomosis (Kasai operation). Bile flow adequate to reduce the serum bilirubin concentration was established in five patients (improved group), three of whom became anicteric. The serum bilirubin concentration did not decrease in the remaining five patients (unimproved group).

Acrodermatitis enteropathica, zinc, and the Paneth cell. A case report with family studies.

An infant with acrodermatitis enteropathica was studied before and after starting zinc therapy. Clinical recovery was rapid, and the plasma zinc, serum and mucosal alkaline phosphatase activities returned to normal. Light microscopy of small intestinal biopsies showed normal mucosa.

Deficient activity of hepatic pyruvate dehydrogenase and pyruvate carboxylase in Reye's syndrome.

The activity of certain hepatic enzymes involved in carbohydrate metabolism was measured in postmortem samples from six cases of Reye's syndrome. The activities of the two exclusively extramitochondrial enzymes, glucose-6-phosphatase and fructose-1,6-diphosphatase, were all within the normal range. Activities of pyruvate carboxylase and pyruvate dehydrogenase, both of which are exclusively mitochondrial enzymes, were below levels, shown by control tissue in every case, the average being 21.

Occult pulmonary abnormalities in asymptomatic asthmatic children.

The pulmonary status of 178 asymptomatic asthmatic children with normal time-volume spirograms was further evaluated using flow-volume loops, body plethysmographic studies, and blood gas tensions in arterialized capillary blood. Residual volume (RV) was abnormal in 26%, total lung capacity (TLC) in 33%, RV/TLC% in 41%, and arterial oxygen pressure in 23% of them. All values for expiratory flow measured relative to observed vital capacity (VC), (ie, the forced expiratory volume in one second [FEV1], the mean forced expiratory flow during the middle half of the forced vital capacity [FEF25-75%; FVC], FEV1/VC, and the instantaneous forced expiratory flow after 75% and after 50% of the FVC has been exhaled) were normal, and VC was subnormal in only five instances, but flow rates measured relative to TLC were abnormal in 26% of the patients.

Dissociation of epithelial cells from rabbit trachea and small intestine with demonstration of APUD endocrine cells.

In this study the entire epithelial lining of tracheas and a 15-cm segments of small intestine were dissociated into individual cell components after 45-minute incubation with 1% pronase. Light and electron microscopy of isolated cells confirmed good morphologic preservation of various epithelial cell types dissociated from the trachea and small intestinal mucosa. Of particular interest was the recovery and preservation of APUD endocrine cells, which are known to be widely dispersed amongst various non-endocrine epithelial cells in both the trachea and small intestine.

Electron microscopy of ancient Egyptian skin.

Sections of skin were examined by electron microscopy from the sole of the foot of a 14-year-old Egyptian, who died 3200 years ago and was preserved naturally by desiccation. Remarkable ultrastructural preservation of the epidermal cells and their components was found.

Membranoproliferative glomerulonephritis in childhood cirrhosis associated with alpha1-antitrypsin deficiency.

Three alpha1-antitrypsin (alpha1AT) deficient, protease inhibitor type ZZ children who died from cirrhosis and its complications had membranoproliferative glomerulonephritis at postmortem examination. During life, all three had clinical and laboratory evidence of renal disease which became apparent when hepatic decompensation developed. Immunofluorescence studies and electron microscopy performed in one patient revealed subendothelial deposits of alpha1AT, complement, and immune globulins along the glomerular basement membrane.

Liver disease associated with alpha1-antitrypsin deficiency in childhood.

Liver disease in children with alpha1-antitrypsin deficiency and protease inhibitor type ZZ does not necessarily carry a bad prognosis. Fourteen of our 18 patients presented with the neonatal hepatitis syndrome and four had hepatomegaly without jaundice. Although four patients have died of cirrhosis and its complications, and three have severe liver disease, most of the 11 others, of whom four are over 13 years of age, have relatively little clinical, biochemical, or histologic evidence of liver disease.

Acute liver disease and encephalopathy mimicking Reye syndrome. A report of three cases.

Three patients are described whose clinical presentation suggested Reye syndrome, and in whom the initial laboratory investigations supported the diagnosis. The subsequent clinical and biochemical evolution of the illness differed from that of Reye syndrome. The liver biopsy of each patient revealed changes in centrilobular hepatocytes rather than the diffuse small droplet fatty change characteristic of Reye syndrome.

Antenatal diagnosis of sphingolipid and mucopolysaccharide storage diseases.

In 4 years of 24 fetuses at risk for various sphingolipid and mucopolysaccharide storage diseases were examined. Amniocentesis at 16 weeks' gestation was followed in most cases by culture of amniotic fluid cells and measurement in the cells of the activity of the enzyme suspected to be deficient. Six fetuses were affected; five were examined morphologically and biochemically after abortion.

Ultrastructure and fluorescence histochemistry of endocrine (APUD-type) cells in tracheal mucosa of human and various animal species.

This study describes distrinctive cells with ultrastructural and histochemical features of APUD-type endocrine cells within the tracheal epithelium of human fetuses, newborns and children as well as different animal species. These cells referred to as Kultschitzky cells (K cells) were found to be argyrophilic, but not argentaffin, and are considered analogous to the same type of cells in lung and gastro-intestinal tract. Fluorescence histochemistry demonstrated the presence of intracellular amine within tracheal K cells, but only after in-vitro or in-vivo administration of amine precursor (L-DOPA).