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Biomedical subjects

G B Stickler

Publications and source records attributed to G B Stickler.

At least 19 recordsLinked to original sources
PubMed1991

Parents' worries about children compared to actual risks.

To investigate the concerns of parents, questionnaires were given to the "first 100" parents waiting for their child's pediatric appointment in four different medical settings. They were asked to rate whether they worry "frequently," "occasionally," or "not at all" about 17 physical health issues, 16 psychosocial problems, 7 possible injuries, 4 instances of victimization, and 4 questions about parents' ability to provide discipline, affection, values and financial support. The response rate was 94%; 89% were completed adequately for analysis. Parental concerns were compared to data from the National Center for Health Statistics about the statistical likelihood of the risks occurring. Ear infections (65%) were the most common health concern, followed by reaction to immunization (57%), common colds (51%), and cancer (50%). The greatest psychosocial concern was about children watching too much television (53%), followed by concerns about the quality of day care (50%). Three-quarters of parents worry about car accidents and 72% worry about abduction of their children. Parents worry significantly about their own contribution to their child's welfare: appropriate discipline (73%), affection (56%), values and being a role model (55%), and financial support (66%). Parents who had not completed high school worried more than parents who had more than a high school education (P less than 0.03).

Adolescent↗
PubMed1991

Klippel-Trenaunay syndrome: the risks and benefits of vascular interventions.

Our experience with Klippel-Trenaunay syndrome (KTS), a rare congenital malformation, has increased considerably in recent years and now includes 144 patients (65 male and 79 female patients). Hemangioma was present in 137 patients (95.1%), varicosity in 110 (76.4%), and hypertrophy of the soft tissues or bones in 134 (93.1%). In most patients (71.5%) the disease involved one lower extremity. Diagnostic workup included roentgenogram to document limb length discrepancy, noninvasive arterial and venous evaluation, contrast venography, and nuclear magnetic resonance imaging. Most patients did well without treatment or with elastic compression only. Surgical treatment for the vascular malformation in KTS is rarely needed and it continues to be controversial. To evaluate the risks and benefits of vascular interventions, we examined in detail the clinical histories of nine patients who in the last decade underwent operation for a vascular malformation of the lower extremity. In seven patients we removed varicose veins or resected hemangioma of the lower extremity. Although none was cured, all five who underwent resection of varicose veins and one of the two patients who underwent resection of a hemangioma improved. Two additional patients, however, who underwent resection of varicose veins in another institution had worsening of the symptoms. In one patient we performed deep venous reconstruction for atresia of the superficial femoral vein, using the contralateral saphenous vein. Such operation in KTS has not been reported previously. The patient has a patent graft with a competent valve and clinical improvement 6 months after the operation. Although patients with severe chronic venous insufficiency, disturbing cosmetic appearance, or complications of hemangioma may benefit from surgical treatment, detailed preoperative imaging of the extremity and pelvis with magnetic resonance imaging and contrast venography is needed to decrease complications. Rarely, reconstruction for atresia or hypoplasia of the deep veins may be needed.

Adolescent↗
PubMed1991

Anomalies of the fingers and toes associated with Klippel-Trenaunay syndrome.

Klippel-Trenaunay syndrome is a rare congenital malformation characterized by a large angiomatous nevus; hypertrophy of soft tissue or overgrowth of bone, or both; and venous varicosities. The cases of 108 patients who had a diagnosis of this syndrome between 1956 and 1990 were reviewed. One hundred and twenty-six anomalies were found in twenty-nine patients, each of whom had one to thirteen malformations of the fingers or toes, or both. The ratio of female patients to male patients was approximately two to one. Twenty-six patients had macrodactyly affecting one to six digits; nine, syndactyly involving two or more digits; five, metatarsus primus varus; two, clinodacytly; two, polydactyly; one, camptodactyly; and one, a congenital trigger finger. Thirty-three of the 126 anomalies were in extremities that had no nevi, varicose veins, or generalized hypertrophy.

Adolescent↗
PubMed1979

Recurrent abdominal pain.

A long-term follow-up study (minimum of five years) of 161 children with recurrent abdominal pain disclosed that three had organic disease and that was missed--inflammatory bowel disease. Anorexia nervosa developed in one patient. Three fourths of the patients recovered from the initial symptom; most recovered within a few weeks; but some patients continued to have complaints for a number of years. Approximately 20% of patients underwent additional surgical or medical treatments of doubtful necessity. In 18% of patients, other psychosomatic symptoms developed.

Abdomen↗
PubMed1979

Urinary tract infection in children: diagnosis and treatment.

Urinary tract infection (UTI) is a fairly frequent occurrence in children. Diagnosis should be made quickly and accurately by Gram staining and culture of an uncontaminated urine specimen. A first, uncomplicated UTI should be treated with sulfonamides or nitrofurantoin. Careful follow-up is necessary because of the high risk of recurrence. Sensitivity studies and specific antimicrobial therapy are indicated in any patient with recurrence and in patients with anatomic urinary tract abnormalities. Long-term prophylaxis may be needed.

Bacteriuria↗
PubMed1978

Carpal-tarsal osteolysis.

A mother-daughter kindred with carpal-tarsal osteolysis and evidence of renal involvement is presented. There was a similarity between the early clinical manifestations of this syndrome and those of rheumatoid arthritis. The distinction made in previous reports between the dominantly inherited form and the sporadic form with nephropathy is questioned.

Adult↗
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↗