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Biomedical subjects

G B Stickler

Publications and source records attributed to G B Stickler.

At least 73 records · Page 4Linked to original sources
PubMed1977

Acute encephalopathy with liver dysfunction, chylous ascites and cytomegalovirus infection.

A child with acute encephalopathy and liver dysfunction subsequently developed acute chylous ascites. Titers for cytomegalovirus increased from less than 1:2 to 1:32 during the illness, and cytomegalovirus was isolated from the urine. The case is the first one possibly linking cytomegalovirus and acute encephalopathy and liver dysfunction in a child. In our patient, enlargement of the abdominal lymph nodes, as seen on a lymphangiogram, resulted in a severe obstruction of abdominal lymphatic flow, producing a transudation of lymph into the peritoneal cavity. The acute chylous ascites associated with mesenteric lymphadenitis was likely caused by the cytomegalovirus infection.

Brain Diseases↗
PubMed1977

Systemic lupus erythematosus in children: the complex problems of diagnosis and treatment encountered in 101 such patients at the Mayo Clinic.

Between 1945 and 1970, 101 children (86 girls and 15 boys) with systemic lupus erythematosus were evaluated at the Mayo Clinic. Only 9 children were less than 9 years old at the time of diagnosis. The most frequent presenting complaint was arthralgia; fever, fatigue, and a "butterfly" malar rash also were common. Renal involvement, found in more than 76 per cent of patients, was a prognostically poor sign. The overall survival of children with renal involvement is improved by the use of adequate steroid therapy.

Adolescent↗
PubMed1977

Neonatal hepatitis and alpha-1-antitrypsin deficiency. The prognosis in five patients.

Five patients with alpha-1-antitrypsin deficiency (PiZ) are reported. All these patients presented with the neonatal hepatitis syndrome and two fo them had developed cirrhosis at ages 5 and 8 years, respectively. Three patients, ages 1, 9 and 21 years, are asymptomatic. The oldest patient, 21 years of age, has only mild histologic changes in the liver. The prognosis for patients with alpha-1 antitrypsin deficiency (PiZ) presenting with neonatal hepatitis is not necessarily grave, a finding that differs from previous observations.

Adult↗
PubMed1977

Juvenile nephronophthisis and medullary cystic disease.

Juvenile nephronophthisis and medullary cystic disease are morphologically indistinguishable hereditary renal disorders. These diseases have been described independently but very likely are a single disease entity and occur as a juvenile-onset, autosomal recessive form and as an adult-onset, autosomal dominant form. We agree with this hypothesis and present here the clinical, laboratory, and pathologic findings of six cases of the juvenile-onset, autosomal recessive form, along with an analysis of the mode of transmission of these and other published cases of the disorder.

Child↗
PubMed1976

The painful scrotum: torsion vs epididymo-orchitis.

In summary, an acute disorder of the scrotum in a child without previous urologic history must be viewed as torsion until proved otherwise by surgical exploration. No deleterious effects have been reported from such an approach, which requires a minimal period of hospitalization and little discomfort to the child. The shorter the interval between the onset of symptoms and surgical exploration, the greater the likelihood that the testicle will be viable later. The diagnosis of epididymo-orchitis in a healthy child without a history of urologic abnormality or evidence of lower urinary tract infection has no place in the differential diagnosis of the child with the painful scrotum.

Acute Disease↗
PubMed1976

Serum 25-hydroxyvitamin D in infantile rickets.

In small children with nutritional vitamin D deficiency, the serum concentration of 25-hydroxyvitamin D (25-OH-D), the major circulating metabolite of vitamin D, was correlated with the stage of clinical disease. It was low (16 to 20 ng/ml) but within the normal range in the earliest (hypocalcemic) stage of the deficiency syndrome and decreased (less than 15 ng/ml) in the more advanced stages. In patients with familial hypophosphatemia (X-linked dominant), mean serum 25-OH-D concentration was the same as in age-matched normal controls. Evidence is presented that endogenous parathyroid hormone may have a role in the depletion of serum 25-OH0D in deficiency states.

Adolescent↗
PubMed1975

[Old and new data on diarrhetic diseases in childhood I. Etiology and pathophysiology].

Etiology and Pathophysiology. The present article is a comprehensive review of recent research results in the field of acute diarrhea. The most important new idea in contrast to older views is that almost all acute diarrheas are associated with a disturbance of intestinal bacterial homeostasis: overgrowth of the small intestine with apathogenic or pathogenic organisms is followed by changes in intestinal metabolism with increase in intestinal water and electrolyte secretion. Anaerobic organisms cause by enzymatic deconugation and dydroxylation of bile acids secretion of fluid into the small intestine and inhibition of fluid absorption from the large intestine. 10-OH-fatty acids, which are formed intraluminally by enzymatic hydroxylation of long-chain unsaturated fatty acids from the diet by similar anaerobic organisms, produce profuse secretion of fluid into the small intestine. The ability of numerous strains of E. coli to produce enterotoxin, which has a qualitatively similar action to cholera toxin, is now considered to be a major cause of infantile diarrhea. The separation of two completely different pathophysiologic mechanisms of E. Coli, the enterotoxic and the enteroinvasive action which are determined by extranuclear chromosomal material, is an important result of recent research. Overgrowth of the small intestine with different bacteria is followed by loss of actiivity of lactase, and later of all disaccharidases in the intestinal mucosa.

Age Factors↗
PubMed1975

[Past and present aspects of diarrheal disease in childhood. Clinical study and treatment (author's transl)].

The etiologic and pathophysiologic findings described in the first part of this paper have important consequences: The recognition of the specific etiology of diarrhea requires new laboratory methods: most of these, however, are technically easy to perform and do not require a large laboratory. A long-ranging consequence of this changed concept is a well-founded modification of therapy. The most important discovery was, that in a well balanced glucose electrolyte solution sodium and glucose enhance their absorption mutually and increase the absorption of water by solvent drag. Since in most acute diarrheas the mechanisms of absorption of glucose and electrolytes are retained this mechanism can be utilized for fast oral rehydration and reinstitution of normal intestinal homeostasis. Prompt institution of a diet consisting of the previously mentioned glucose-electrolyte solution usually prevents severe dehydration and the need for stationary treatment. The elimination of lactose and long chain fatty acids from the diet prevents continuation of the pathologic osmotic and chemical conditions in the intestine. Antibiotics are not indicated in acute diarrhea with the exception of diarrhea caused by enteroinvasive E. Coli or Shigella, in the case of Salmonella-gastroenteritis even contraindicated. Further research concentrates on the development of drugs for neutralisation of E. Coli enterotoxin and the prevention of diarrheas by development of effective vaccines.

Acid-Base Equilibrium↗
PubMed1975

Vitamin D deficiency rickets. Two cases with faulty infant feeding practices.

Two cases of vitamin D deficiency rickets verify the occurrence of deficiency rickets in the United States in 1973. The two cases demonstrate the need for periodic reviews of feeding practices, especically when the possibility of so-called milk allergy is postulated. This may lead to avoidance of milk products. Fortification of various kinds of food with vitamin D does not ensure the protection from nutritional rickets of all children with peculiar feeding habits.

Alkaline Phosphatase↗
PubMed1975

Hypoventilation secondary to chronic upper airway obstruction in childhood.

A 6-year-old child manifested symptoms and signs of alveolar hypoventilation and signs of cor pulmonale caused by obstruction of the upper airway. These symptoms and signs disappeared after tonsillectomy and adenoidectomy. The relationship of upper airway obstruction to hypoventilation and cor pulmonale is discussed in the hope of renewing interest in this problem and alerting physicians to think of this possiblity early in the evaluation of such patients.

Adenoidectomy↗