Documented latent coeliac disease in a child with insulin-dependent diabetes mellitus.

The histological development of coeliac disease has been documented in a child with insulin-dependent diabetes mellitus (IDDM). Serum antigliadin IgG was temporarily present at the onset of IDDM. It is assumed that IDDM may exert a trigger effect on the development of coeliac disease.

Intestinal lymphangiectasia, lymphedema, mental retardation, and typical face: confirmation of the Hennekam syndrome.

We report on a male with intestinal lymphangiectasia, mild mental retardation, seizures, and a typical face; the syndrome was first delineated by Hennekam et al., Am. J.

Sodium metabolism in offspring of hypertensive parents.

Intracellular sodium concentration and Na+/K(+)-ATPase activity were studied in erythrocytes obtained from members of 14 families with one hypertensive parent and from age-matched control subjects, as part of a study on the genetic and environmental determinants of essential hypertension. We found reduced Na+/K(+)-ATPase activity, increased intracellular Na+ concentration, and reduced urinary Na+ excretion in hypertensive patients as compared with the control subjects. In the offspring of hypertensive parents an increase in intracellular Na+ concentration and a decrease in Na+/K(+)-ATPase activity were observed, with a significant correlation relating such parameters.

Influence of splenectomy on the properties of erythrocytes with hemoglobin Volga disease.

Hemoglobin Volga is a rare unstable hemoglobin in which there is a replacement of an internal alanine residue, beta 27 (B9), by an aspartate. From a clinical point of view it is characterized by a moderately severe Heinz body hemolytic anemia. A possible clue to the cause of the hemolysis is the increased vulnerability to oxidation of Hb Volga, with increased free radical turnover and consequent damage to the red cell membrane.

Clinical application of a simple HPLC method for the sugar intestinal permeability test.

A new high pressure liquid chromatography (HPLC) method has been used to measure urinary sugar levels for the intestinal permeability test with cellobiose and mannitol (C/M test). Urinary specimens have been prepared by simple filtration through a Millipore membrane. The method is highly sensitive (minimal detectable concentration of urinary sugars = 0.

Successful treatment of postpartum shock caused by amniotic fluid embolism with cardiopulmonary bypass and pulmonary artery thromboembolectomy.

We report the successful treatment of a moribund patient as a result of amniotic fluid embolism with cardiopulmonary bypass and open pulmonary artery thromboembolectomy. Review of the literature indicates that this is the first reported case of treatment of amniotic fluid embolism with cardiopulmonary bypass and pulmonary thromboembolectomy.

Parotid gland metastasis from renal carcinoma.

The salivary gland most frequently involved with secondary cancer is the parotid gland. Metastases are responsible for 21-42% of malignant parotid tumors. Malignant melanoma and squamous cell cancer are the two most common tumors to metastasize to the parotid gland.

Surgical resection for colorectal hepatic metastases.

Hepatic resection of metastatic disease due to primary colorectal cancer provides a relatively safe and reliable method to control this otherwise fatal disease. At New York University 45 hepatic resections have been performed in 42 patients over the last fifteen years. Preoperative screening was performed by liver chemistry and intraoperative exploration in synchronous lesions and by liver chemistry, carcinoembryonic antigen, and computed tomography in metachronous lesions.

Experience with selective operative and nonoperative treatment of splenic injuries in 193 patients.

During the past decade splenic salvage procedures rather than splenectomy have been considered the preferred treatment for traumatic splenic injuries. Splenic preservation has been most often accomplished by splenorrhaphy and more recently by a controversial nonoperative approach. This report delineates indications, contraindications, and results with splenectomy, splenorrhaphy, and nonoperative treatment based on an 11-year experience (1978 to 1989) in which 193 consecutive adult patients with splenic injuries were treated.

Alterations of the respiratory burst of polymorphonuclear leukocytes from diabetic children. A chemiluminescence study.

The respiratory burst of polymorphonuclear leukocytes was investigated in 24 children with insulin dependent diabetes mellitus and 24 healthy controls. This oxygen dependent, membrane associated process generates a number of toxic oxygen metabolites which are implicated in the pathogenesis of endothelial damage. The activity of polymorphonuclear leukocytes was studied in terms of luminol amplified chemiluminescence.

Preliminary study of breastfeeding and bacterial adhesion to uroepithelial cells.

The oligosaccharide content of breast-milk and urine from ten nursing mothers and their babies, collected 30 days after delivery, was analysed by thin-layer and high-performance liquid chromatography. Each woman's milk and urinary oligosaccharide profiles were very similar, and the pattern of oligosaccharides excreted by her infant was also strongly correlated with that of her milk. The babies excreted 300-500 mg/day oligosaccharides and the mothers 500-800 mg/day.

Mild form of Jeune syndrome in two sisters.

Jeune syndrome is characterized by respiratory distress, osseous dysplasia, and short stature. Patients generally die during the first months of life. However, some cases with milder clinical manifestations have been described; these cases show characteristic renal involvement of different severity.

Abdominal mycobacterial infections in immunocompromised patients.

A review of the discharge diagnoses and mycobacterial cultures of patients admitted to a major New York City hospital over an 18-month period revealed 21 patients with abdominal mycobacterial infections (17 male, 4 female) with an average age of 36 years. Acquired immunodeficiency syndrome (AIDS) or an identifiable AIDS risk was present in 14. The disease was manifest by peritoneal (eight patients), ileocecal (seven), and hepatic involvement (three), and psoas abscess (three).

Magnetic resonance imaging in the malformative syndromes with mental retardation.

We reported on thirteen children affected with malformative syndromes associated with mental retardation. MRI examination showed in all of them different types of anomalies; agenesis of corpus callosum, ventricular dilatation and cerebral matter alterations were the most frequent findings. The authors give a brief description of the clinical reports and of the anomalies observed with MRI; they conclude that MRI seems to be a valuable tool especially for the study of the alterations of the cerebral matter and their correlation with mental retardation.

[Study of the SNC using MRI in a case of Lowe's syndrome].

A case of Lowe syndrome is reported. This syndrome is characterized by ocular, cerebral and renal defects. MRI investigation showed severe thickness of the corpus callosum and defective cerebral development.

Giant renal cell carcinoma: CT and MR characteristics.

A case of an unusually large cystic renal cell carcinoma demonstrated on plain film, CT, and magnetic resonance (MR) is presented. Though the origin of huge abdominal tumors can be difficult to determine, the CT and MR findings were able to correctly diagnose the mass as a malignant cystic kidney tumor.

[Progressive pseudorheumatoid arthropathy in childhood (late spondylo-epiphysial dysplasia with progressive arthropathy). Description of a case].

The progressive pseudorheumatoid arthropathy of childhood is a very rare disorder that follows an autosomal recessive mode of inheritance, and which can be framed in the group of the spondyloepiphyseal dysplasia tarda. This affection is characterized by specific radiological anomalies and by clinical signs resembling an inflammatory disease, with the normality of the routine laboratory studies. The authors describe a case recently come to their attention.

Oculo-cerebro-cutaneous syndrome: description of a new case.

A newborn girl is described with the following multiple congenital anomalies: an orbital cyst, cutaneous appendages, focal hypo-aplasia of the skin and multiple cerebral cysts. This case of oculo-cerebro-cutaneous syndrome is compared with four previously published cases.

Factors involved in antibiotic selection in elective colon and rectal surgery.

During a 24-month period, 350 patients were prospectively studied in an effort to determine the perioperative factors in the development of infections after colon and rectal resections. All patients received standard mechanical bowel preparation; perioperative parenteral cefoxitin (group A) or preoperative oral neomycin and erythromycin, in addition to perioperative cefoxitin (Group B), were also given. Both groups were comparable with respect to age, sex, associated diseases, and primary diagnosis.

X/Y translocation in a family with X-linked ichthyosis, chondrodysplasia punctata, and mental retardation: DNA analysis reveals deletion of the steroid sulphatase gene and translocation of its Y pseudogene.

We describe a family with two male members showing an X/Y translocation (karyotype: 46,Y,der(X)t(X;Y)(p22;q11]. At physical examination both patients showed ichthyosis, mental retardation and dysmorphic features. Chondrodysplasia punctata and short stature were present in one case.