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Biomedical subjects

M Berant

Publications and source records attributed to M Berant.

At least 127 records · Page 7Linked to original sources

Pneumococcal meningitis following parenteral alimentation in infants.

Three young infants with protracted diarrhea and malnutrition were successfully treated by means of intravenous nutrition, which included infusions of fat emulsion (Intralipid) and of fresh frozen plasma. Three to five weeks after termination of intravenous feeding, and after full recovery, they developed pneumococcal septicemia and meningitis. One infant died, and postmortem examination showed diffuse deposition of "intravenous fat pigment" in hepatocytes and in reticuloendothelial cells. In the two infants who recovered, follow-up studies did not disclose any primary derangement of immunologic function. We propose that the infused fat may have caused a temporary depression of immunologic defense mechanisms, predisposing these infants to the pneumococcal infection; the delay in onset of the infection might be attributed to a short-lasting protective effect of fresh frozen plasma which was included in the intravenous feeding regimen.

Blood↗

Excretory pattern of bile during phototherapy.

We studied the acute effects of phototherapy (PT) on bile flow and on the biliary excretion of bilirubin pigments and of bile salts in male homozygous Gunn rats (120-150 g). 13 rats received PT and 10 rats were kept in the dark. Bile was collected by cannulation of the common bile duct at hourly intervals from 1 h prior to PT till after 4 h of 'lights on'. Before treatment, all values were similar in both groups. After 4 h of lights on, mean plasma bilirubin fell from 145.3 +/- 4.3 to 99.2 +/- 2.7 mumol/l (p less than 0.01) in the PT rats, but did not change in the controls. During the lights-on period, PT rats had a significantly higher hourly bile volume, and a higher excretion of biliary bilirubin and bile salts than the controls (p less than 0.005). Over the total 4-hour lights-on period, the PT group had a higher mean output of bile than the controls (0.93 +/- 0.17 vs. 1.52 +/- 0.34 ml/4 h; p less than 0.005) and an increased excretion of bilirubin (0.08 +/- 0.016 vs. 0.148 +/- 0.01 mumol/4 h; p less than 0.005) and bile salts (35.1 +/- 3.7 vs. 55.2 +/- 12.5 mumol/4 h; p less than 0.005). The results show that PT of the Gunn rat is associated with a rise in bile flow and with an increased excretion of bile salts, in addition to an increased biliary bilirubin output.

Animals↗

Progressive diaphyseal dysplasia: genetics and clinical and radiologic manifestations.

Progressive diaphyseal dysplasia was found in a three-generation family including 13 affected individuals, the largest family reported to date. Our study confirms that progressive diaphyseal dysplasia, also known as Engelmann's or Camurati-Engelmann disease, is an autosomal dominant disorder with variable osseous and muscular manifestations. Disease distribution among patients, within a given patient, or even in individual bones is unpredictable. The femur is the most commonly and severely affected bone and hence most useful for radiographic screening of possible patients. Radiographs provide a meaningful assessment of disease activity and extent. The severity of symptoms is generally proportionate to severity of involvement shown by roentgenography. Exophthalmos due to osteosclerotic dysplasia of the skull occurred in more than half of the patients with progressive diaphyseal dysplasia. Twelve-year follow-up of this family, with affected individuals ranging in age from 6 months to 12 years, indicates that progressive diaphyseal dysplasia may progress or become quiescent and be remarkably inactive despite advanced osteosclerosis and structural deformity.

Adolescent↗

Vitamin K deficiency presenting with hemarthrosis.

A breast-fed 25-day-old infant was hospitalized because of swelling and tenderness of the left leg, developed after mild rotary motion of the leg by his brother. Radiographic examination showed widening of the left articular hip joint space. On the day of admission, a presumptive diagnosis of septic arthritis was entertained, and antibiotic therapy was instituted. Following profuse bleeding from sites of skin punctures, coagulation studies were performed. Prothrombin time and partial thromboplastin time were prolonged. Administration of phylloquinone (vitamin K1) resulted in rapid normalization of coagulation. Differential diagnosis between hemarthrosis resulting from vitamin K deficiency and septic arthritis with disseminated intravascular coagulation is a matter of great importance in such patients.

Hemarthrosis↗

Primary sclerosing cholangitis associated with immunodeficiency.

An infant, first admitted at the age of 5 months with diarrhea (which was adequately treated with formula), was readmitted at the age of 1 year with poor weight gain, steatorrhea, and hepatomegaly. Liver function test results were compatible with cholestatic jaundice, and hepatobiliary scintigraphy visualized dilated bile ducts and evidence of hepatocellular disease. Exploratory laparotomy, liver biopsy, and cholangiography disclosed pathologic and roentgenographic findings of primary sclerosing cholangitis (PSC). The patient proved to be immunodeficient, pointing to the possible pathogenetic role of immunodeficiency in causing PSC in some patients. It is important to look for the disease in immunodeficient children and in patients with ulcerative colitis, and to consider PSC in the differential diagnosis of cholestatic jaundice.

Bile Ducts↗

Bacterial meningitis. Effect of antibiotic treatment on cerebrospinal fluid.

The effects of full short-term antibiotic treatment on cerebrospinal fluid (CSF) findings were studied retrospectively in 68 children with acute bacterial meningitis. The features of CSF at admission were compared with those of the CSF obtained after 44-68 hours of therapy. Except in one case with H. influenzae and one case with pneumococcal meningitis, all CSF cultures were negative in the repeat specimen. In three of 16 children with meningococcal meningitis, the CSF glucose levels became normal in the second specimen. In all remaining 65 children, however, full intravenous antibiotic treatment for 44-68 hours did not alter the biochemistry and cytology of the CSF, which retained its "bacterial" character. From these findings it may be discerned that partial antibiotic treatment is even less likely to distort a 'bacterial' CSF.

Adolescent↗

Phototherapy-associated diarrhea. The role of bile salts.

The concentration of fecal bile salts was measured in 14 jaundiced neonates who received phototherapy (PT group) and their 14 nontreated matched controls (C). Before initiation of phototherapy, mean bile salt concentrations in stool specimens from the two groups were similar. At 12 hours of 'lights on', stool specimens from PT babies showed a significantly increased mean bile salt concentration, whereas in the C babies there was no change (3.65 +/- 0.39 vs 2.62 +/- 0.22 mmol/l; p less than 0.01). At 24 hours after 'lights off', stool specimens from the PT infants had a mean bile salt concentration like that before phototherapy, and not different from C. During phototherapy, nine PT babies had a bile salt concentration in their stools of 3.5 mmol/l and above; 6 of these babies had watery stools with a high sodium content. The high concentration of bile salts found in the colonic contents of neonates during phototherapy would appear to be a factor in the pathogenesis of phototherapy-associated diarrhea in the jaundiced neonate.

Bile Acids and Salts↗

Papular acrodermatitis with cytomegalovirus hepatitis.

The syndrome of papular acrodermatitis of childhood with hepatitis (Gianotti-Crosti syndrome) is classically considered to be associated with hepatitis B surface antigen (HBsAg) infection. We report an infant with the syndrome, but with serological evidence of infection by cytomegalovirus.

Acrodermatitis↗

Antibacterial prophylaxis in chronic granulomatous disease. A case report.

The value of long-term prophylactic treatment of chronic granulomatous disease (CGD) in childhood cannot be established with certainty, as controlled studies are not available. We describe a boy, presently 15 years old, who suffered from CGD since early infancy. By the clinical, laboratory and genetic features, this case appeared to be a new variant of CGD, combining elements of the "childhood" type with others that characterize the "adult" type of the syndrome. For years, the patient had been almost continuously ill and needed frequent and prolonged hospitalizations because of severe bacterial infections. At age 13 years, long-term prophylactic treatment with trimethoprim-sulfamethoxazole (TMP-SMX) was instituted. With this regimen, the patient was maintained practically infection-free, relapsing only in those instances when he neglected to comply with the prophylactic regimen for 1 to 2 weeks. Thus, the patient served as his own control in demonstrating the efficacy of antimicrobial prophylaxis in CGD. The rationale for employing TMP-SMX for the prophylactic regimen is discussed.

Adolescent↗

The effect of intrarenal infusion of bile on kidney function in the dog.

1. Obstructive jaundice sensitizes the kidney to anoxic damage. To clarify further this phenomenon the effect of unilateral infusion of bile on kidney function was studied. The contralateral intact kidney served as control. 2. Intrarenal infusion of diluted bile (1:10) resulted in an ipsilateral fourfold increase in mean rate of urinary flow (P less then 0.01), threefold increase in mean fractional excretion of sodium (P less then 0.05) and more than 50% increase in mean rates of potassium excretion (P less then 0.05). Urinary flow rate and electrolyte excretion returned to baseline upon cessation of bile infusion. The mean clearances of inulin and rho-aminohippurate were unchanged during intrarenal bile infusion. 3. Intrarenal infusion of isotonic taurocholate solution (20 mmol/l) mimicked the diuretic, natriuretic and kaliuretic effects of diluted bile, whereas intrarenal infusion of bilirubin did not cause any change in the excretion of electrolytes. 4. It is concluded that increase in circulating bile acids rather than hyperbilirubinaemia may alter kidney function during obstructive jaundice. Acute cholaemia may cause volume depletion by increasing urinary salt loss. This in turn may aggravate the direct nephrotoxicity of circulating bile compounds.

Animals↗

Effect of isolated cholaemia on systemic haemodynamics and kidney function in conscious dogs.

1. Systemic haemodynamics and kidney function were studied in the same dogs before and 14 days after choledochocaval anastomosis. 2. All dogs became deeply jaundiced whereas parenchymal liver function remained unchanged as assessed biochemically. 3. After choledochocaval anastomosis there was a decrease in mean arterial pressure (118 +/- 18 to 98 +/- 13 mmHg, P less than 0.005), and total peripheral resistance (4073.8 +/- 620.0 to 3327.6 +/- 244.9 kPa 1-1 s kg, P less than 0.01), whereas mean cardiac index and plasma volume corrected for body weight did not change. 4. Despite their disturbed systemic haemodynamics the cholaemic dogs and normal mean glomerular filtration rate and renal plasma flow. Maximal ability to concentrate and dilute the urine was, however, impaired during cholaemia. 5. It is concluded that cholaemia per se causes peripheral vasodilatation, hypotension and renal tubular dysfunction. Similar phenomena in jaundiced patients may contribute to their susceptibility to postoperative shock and acute renal failure.

Animals↗

Familial early-onset nephrotic syndrome: diffuse mesangial sclerosis. Clinico-pathological study of a kindred.

Four children from two related families are described with a clinical picture characterized by the onset of asymptomatic proteinuria with subsequent nephrotic syndrome in infancy, and progression to renal failure and death before the age of three years. The clinical picture and the renal pathological studies were consistent with the entity described by Habib as infantile mesangial sclerosis. We propose that this entity possibly represents a genetic disorder which is transmitted as an autosomal recessive trait.

Adolescent↗