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Biomedical subjects

G B Stickler

Publications and source records attributed to G B Stickler.

At least 37 records · Page 2Linked to original sources
PubMed1988

Long-term prognosis for children with nephrotic syndrome.

Follow-up survival and health information were obtained, after a median of 27.5 years, from 132 patients who had been seen originally as children with nephrotic syndrome between 1951 and 1967. Ninety seven patients were alive. Recurring edema or proteinuria, or both, persisted in 15 percent of those still alive. Eight of 11 parous women reported relapses during pregnancy. There was no apparent increase in malignancies, atopic diseases, clinical defects in cell-mediated immunity, or cardiovascular diseases. Twenty two patients (17%) died of renal causes between 3 months and 8 years after the onset of nephrotic syndrome. Steroid resistance was the presenting feature universally predictive of a poor outcome; nine of the 11 such patients died and the other two are now receiving hemodialysis. Hematuria was present initially in 41 percent of the patients who died of renal causes, compared with 14 percent of those still alive. Hypertension was noted on the first examination in 22 percent of those who died of renal causes, compared with 10 percent of those alive.

Adolescent↗
PubMed1988

Congenital hepatic fibrosis: evolving morphology.

A clinicopathologic study of congenital hepatic fibrosis in 21 patients confirms a strong association with autosomal recessive renal polycystic disease. The liver specimens were subclassified into two groups according to the severity of fibrosis, showing typical hepatic abnormalities in young infants (mean age 0.3 years) and increased hepatic fibrosis in older patients (mean age 19.6 yr) (p less than 0.02). Apparent progression to perilobular fibrosis with parenchymal nodularity occasionally resembled cirrhosis when the nodules had a regenerative appearance because of rounded contours and inapparent central veins. Progression of fibrosis was observed in second biopsy specimens from 2 cases, but not in that of a 3rd, suggesting that factors other than the heritable disorder itself may be responsible for evolving morphology. Identifiable factors that may have contributed to increased fibrosis included localized intrahepatic biliary obstruction and biliary sepsis with suppuration. A factor possibly contributing to the pathogenesis of biliary sepsis was intrahepatic biliary ectasia, i.e., Caroli's disease, which appears to be one morphologic expression of CHF. This study shows that the hepatic abnormality evolves over time and that it may be altered by secondary complications.

Bile Duct Diseases↗
PubMed1986

Primary lymphatic dysplasia in children: chylothorax, chylous ascites, and generalized lymphatic dysplasia.

Primary lymphatic "dysplasia", a congenital maldevelopment, interferes with function of the lymphatic system and causes effusion of chyle or lymph into the limbs and pleural or peritoneal cavity. Between 1955 and 1982, 38 Mayo Clinic patients were found to have a chylous effusion or dysplasia of the lymphatic system. In 22, the condition was secondary to surgery or other medical problems and in 16 it was primary. These cases were separated into three categories: chylothorax, chylous ascites, and generalized lymphatic dysplasia. Conservative therapy, such as a restricted fat diet or total parenteral nutrition with repeated thoracentesis or paracentesis, was effective in the children with isolated abnormalities of the lymphatic system (75% resolution rate, no deaths). All five children with documented generalized dysplasia reported in the literature had died; of the three reported here, one has died and two have become progressively worse.

Adolescent↗
PubMed1985

The common cold.

The common cold continues to be the major cause of absences from school and work. A better understanding of the epidemiology and the natural history should lead to less anxiety about this disease. Although there is still much misinformation among the public, the time should soon come when better-informed patients with this disease will make fewer visits to the physician and will not expect unnecessary and worthless treatment.

Aged↗
PubMed1985

Primary lymphedema in children and adolescents: a follow-up study and review.

Primary lymphedema, a disorder causing persistent swelling in an extremity, is rare in children and adolescents; it affects 1.15/100,000 persons less than age 20 years. It primarily affects girls near menarche. The records of 125 children and adolescents, aged 0 to 20 years, who were examined at the Mayo Clinic were analyzed; 99 of these patients were contacted to obtain follow-up data. The influences of estrogen and inflammation are thought to be important etiologic factors in primary lymphedema. The diagnosis can be made on the basis of a thorough history and physical examination. Lymphangiograms, venograms, and biopsies add nothing to the diagnosis because of the low incidence of tumor in children and adolescents. Conservative treatment is recommended: a Jobst-type stocking, elevation, and proper foot care. Diuretics are not recommended. Careful psychologic counseling, especially in adolescents, is highly recommended.

Adolescent↗
PubMed1984

Prognosis and management of lesions of the trunk in children with Klippel-Trenaunay syndrome.

The Klippel-Trenaunay syndrome is a congenital vascular anomaly consisting of the triad of soft-tissue and bony hypertrophy of the extremities, hemangiomas and/or lymphangiomas, and varicosities. From 1956 to 1983, 42 patients with the Klippel-Trenaunay syndrome were seen at the Mayo Clinic. Of the 42 patients, 16 had involvement of the trunk (thorax, abdomen and/or pelvis). Twelve of the 16 had evidence of the malformation at birth and 4 had evidence shortly thereafter. Lesions involving the trunk included hemangiomas (75%) and lymphangiomas (50%). Complications included rectal bleeding, hematuria, colonic obstruction, hemothorax, paraparesis secondary to clotting disorder, and compression of the external urethral meatus. One child died of enlargement of diffuse lymphangiomas and hemangiomas of the abdomen and chest. Eight of the 16 patients underwent 13 operative procedures. These included excision of the superficial lymphangiomas, resection of the rectosigmoid colon, and resection of an extremely large retroperitoneal mass of hemangiomatous-lymphangiomatous tissue. While progression requiring major surgery was seen in several patients, most patients had supportive therapy, with minimal surgery being necessary.

Abdomen↗
PubMed1984

Panic attack syndrome.

Panic attack syndrome in four generations of a family and in six additional pediatric patients is reported. The syndrome appears to have an autosomal dominant mode of inheritance. Diagnosis of panic attack syndrome in children has not been reported previously, but the existence of this disorder has been noted for at least 100 years under various synonyms. There is substantial evidence that the syndrome has an endogenous origin and is therefore a biochemical illness. The triggering effect of sodium lactate infusion and the alleviation of symptoms by use of monoamine oxidase inhibitors or beta-blocking agents support this view. Imipramine hydrochloride, propranolol hydrochloride, phenelzine sulfate, and alprazolam are often useful in the treatment of panic attacks, and except for propranolol, any of them may be effective against depression also.

Alprazolam↗
PubMed1984

Problems in the clinical and roentgenographic diagnosis of pneumonia in young children.

The difficulty of diagnosing pneumonia by chest x-ray in children less than 4 years of age was studied by comparing the chest roentgenograms of 34 healthy children with the chest roentgenograms of 34 children previously diagnosed as having pneumonia. Review of these films without knowledge of the previous interpretation and without clinical information disclosed variations regarding the roentgenographic confirmation of pneumonia in 24 percent of the cases and variations regarding the location of the lesion in about 50 percent of the cases.

Child, Preschool↗