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Biomedical subjects

A J McAdams

Publications and source records attributed to A J McAdams.

At least 19 recordsLinked to original sources
PubMed1992

Long-term follow-up of non-systemic lupus erythematosus glomerulonephritis in patients with hereditary angioedema: report of four cases.

Hereditary angioedema (HAE) is characterized by a deficiency in C1 inhibitor protein (C1 INH) and by clinical symptoms of episodic swelling of subcutaneous or mucosal tissue. It has rarely been reported in association with non-systemic lupus erythematosus (SLE) glomerulonephritis (GN). A recent report of two cases indicates the prognosis to be poor, with both patients progressing to chronic renal failure 8 and 20 years after diagnosis. This report describes the 5-year follow-up of a previously unreported case of an 8-year-old boy with HAE and non-SLE membranoproliferative glomerulonephritis (MPGN). The patient developed macroscopic hematuria, azotemia, and a vasculitic rash. Treatment included prednisone and cyclophosphamide, resulting in clinical improvement. The present report also summarizes the long-term follow-up of three previously reported cases of HAE and non-SLE GN, 25, 16, and 10 years after their initial presentation. Patients monitored for 25 and 16 years had MPGN and normal renal function and received no therapy. The third patient, monitored for 10 years, had segmental MPGN. This patient presented with urinary abnormalities and, after treatment with prednisone, had improvement in her hematuria. None of these four patients developed chronic renal failure. These observations indicate a variable outcome in patients with HAE and non-SLE GN.

Aged↗
PubMed1991

Prolonged reversible renal failure with nephrotic syndrome.

Steroid nonresponsive nephrotic syndrome in a 15-year-old girl with reversible renal failure required dialysis and aggressive nutritional therapy for 1 year. Severe interstitial edema and foot process fusion were the only processes identified to explain the renal failure. Diabetes-like alterations of the glomerular capillary wall basement membrane may have been an outcome of the intense alimentation.

Acute Kidney Injury↗
PubMed1991

The internal carotid artery siphon in children and adolescents.

We observed significant lesions of the carotid artery siphon in two young subjects with fatal stroke. Because stroke in children and adolescents is uncommon and poorly understood, we examined the internal carotid artery in the 'siphon' of the skull in 24 unselected, but nearly consecutive autopsies. The age range was 10 days to 38 years, with 11 males and 13 females, six blacks, and 18 whites. In no case was stroke the cause of death. Intimal lesions of two types were found in the carotid siphon of all cases. (1) The first was focal splitting and/or duplication of the internal elastic lamina with variable proliferation of smooth muscle. The resulting 'fibrous' plaques or cushions, when severe, were usually found at natural bends in the artery. The number and severity of this type of lesion increased with age, but there were no differences in severity or distribution when compared by sex, race, or mode of death. (2) The second was internal elastic calcification which was found in all cases older than 9. This was increasingly severe with age. Although the frequency of the vascular lesions was surprisingly high, the relationship of either type to dissecting aneurysm or other stroke lesion remains unclear.

Adolescent↗
PubMed1990

Multiple endocrine neoplasia type II B with symptoms suggesting Hirschsprung's disease: a case report.

A 3-year-old child was referred with a tentative diagnosis of Hirschsprung's disease because of life-long constipation and "megacolon" demonstrated radiographically. Our rectal biopsy revealed hyperganglionosis suggestive of multiple endocrine neoplasia (MEN) type II B. This, in addition to an elevated serum calcitonin level, prompted surgical removal of her thyroid, which appeared grossly normal but on sectioning, contained a medullary carcinoma in each lobe. She remains disease-free 5 years later. Gastrointestinal symptoms are a significant component of the MEN type II B syndrome, and often antedate the full phenotypic expression of the syndrome and the development of potentially lethal endocrine neoplasms. On the basis of this experience, it is recommended that MEN II B be included in the differential diagnosis of chronic constipation.

Child, Preschool↗
PubMed1989

Congenital midline cervical cleft.

We present a case of congenital midline cervical cleft (CMCC), a sporadic lesion with a predilection for white females. It characteristically occurs in the midline ventral neck and consists of a cephalad skin tag, a mucosal surface, and a caudal sinus. There is an associated submucosal fibrous cord and interwoven bundles of skeletal muscle. This is occasionally tethered to the mandible and/or sternum causing neck contractures. CMCC is usually isolated, but rare cases have been associated with midline clefts of the tongue, lower lip, mandible, and sternum. The embryogenesis is unclear but arguments are given here that CMCC represents ectopic first branchial arch derivatives, including the lower lip and/or tongue.

Branchial Region↗
PubMed1986

The survival of costal cartilage graft in laryngotracheal reconstruction.

Acquired subglottic stenosis in children is a difficult problem. One of the successful procedures used to correct this condition is an autogenous costal cartilage graft to the subglottis and upper trachea. The fate of the cartilage graft is not known, despite the good results of the procedure. Prompted by recovery of viable cartilage graft from two patients previously operated upon by this procedure, we performed autogenous costal cartilage grafting to the subglottic area of fifty young New Zealand rabbits, with inspection of these grafts at 2- and 4-month postoperative intervals. The purpose of this study is to prove the survival of autogenous costal cartilage grafts in the subglottic area. Gross and histologic examination 2 and 4 months postoperatively showed viable cartilage as well as lining of the graft with respiratory epithelium in the majority of cases.

Animals↗
PubMed1985

The effect of prednisone in a high-dose, alternate-day regimen on the natural history of idiopathic membranoproliferative glomerulonephritis.

Experience in 45 children with diffuse proliferative MPGN of all three types has provided evidence that a high-dose, alternate-day regimen of prednisone alters the natural history of the disease. The experience has been gained over a 17-year period and the patients have been followed on the regimen for an average of 6.5 years. Evidence that the regimen has a salutary effect was provided by several observations: Survival was better than that in four other series in which the patients were not treated or treated sporadically. The difference was particularly marked when survival was compared with that in series in which the patients had diffuse proliferative lesions exclusively. Mesangial proliferation was less in biopsies obtained after 2 or more years of the alternate-day regimen. This was quantitated as a significant increase in the estimated percentage of open glomerular capillary lumens and a significant diminution in the prominence of PAS-positive mesangial matrix. In the second biopsy, as compared to the pre-regimen biopsy, no patient had a diminution in the estimated percent of open capillary lumens and 65% had a meaningful increase. Likewise, only 3% had an increase in prominence of PAS positive matrix and 68% had a diminution in matrix prominence. Of 32 patients who were hypoalbuminemic when the regimen started, the level rose into the normal range in 62%. The level became subnormal in none of the 13 who had a normal level at the start of the regimen. While receiving the regimen, renal function, as measured by serum creatinine levels, continued to be normal or improved in 73% and deteriorated in 27%. Hematuria disappeared in 80% of the 41 in whom it was present when the regimen started. Urinalysis became completely normal in 27% and none of these have relapsed while under observation. Comparison of data from 20 patients who did not receive the regimen for an average of 42 months after clinical onset with data for 25 patients who likewise were, on the average, 42 months from clinical onset but who had received the regimen for an average of 38 of those months provided the most convincing evidence that the regimen altered the natural history. In those receiving the regimen, the frequency of hematuria, proteinuria, and hypoalbuminemia was significantly less.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1979

Idiopathic rapidly progressive glomerulonephritis with C3 nephritic factor and hypocomplementemia.

A 7-year-old boy with mild renal failure and signs and symptoms of acute poststreptococcal glomerulonephritis including severe hypocomplementemia had, by renal biopsy, numerous crescents but no deposits in the glomerular capillary loops. Instead, deposits identical in location and composition to those described for children with idiopathic rapidly progressive glomerulonephritis were present. The severe hypocomplementemia was found to be due to high levels of C3 nephritic factor; niether nephritic factor nor hypocomplementemia has been reported in rapidly progressive glomerulonephritis of the idiopathic type. Following prompt therapy with methylprednisolone intravenously, serologic abnormalities disappeared and renal function greatly improved, but a later biopsy showed 50% of the glomeruli obliterated by scarring. The case is of importance not only in indicating that severe hypocomplementemia does not rule out idiopathic rapidly progressive glomerulonephritis but also in adding to the list of diseases in which nephritic factor can be found.

Antigen-Antibody Complex↗
PubMed1979

Glomerular deposition of complement-control proteins in acute and chronic glomerulonephritis.

Acute poststreptococcal glomerulonephritis (AGN) differed from membranoproliferative glomerulonephritis (MPGN) and lupus nephritis (SLE) in that two of the proteins that control the C3b-dependent convertase, beta 1H and the C3bC4b-inactivator cofactor (C3bC4bICo), were frequently absent from the glomerular deposits. In addition, factor B was distributed with C3 in the capillary walls in hypocomplementemic AGN patients. From this, it can be assumed that C3bBb is in the deposits, uninhibited by control proteins as would be predicted for alternative pathway activation. Factor B could not be found in normocomplementemic AGN, was rarely present in MPGN, but was usually present in SLE, most often in the mesangium. In MPGN and SLE, the control proteins were nearly always present in the glomeruli in a distribution like that of C3; IN MPGN they were particularly abundant. Complement profiles indicated an occasional transient reduction in serum C4 level early in AGN. Thus, although there is occasional evidence of early classical activation in AGN, more characteristic is a long period of alternative activation. Serum levels of control proteins did not deviate greatly from normal except for reduced serum beta 1H levels in MPGN type I.

Biopsy, Needle↗
PubMed1978

Multifocal nephroblastic neoplasia.

Among 23 patients with multifocal metanephric neoplasia were 14 patients with 43 grossly visible subcapsular tumorlets, 0.3-3.5 cm in diameter, that were apparently derived from nodular renal blastema. Tumorlets are defined as circumscribed lesions that are visible on the exterior surface of the kidney, produce either a raised nodule or, in a few instances, a shallow depression, and are independent of the main tumor(s). Most tumorlets displayed homogeneous epithelial maturation. A substantial minority of tumorlets retained a blastemic character and a few were histologically malignant. Tumorlets tended to be multiple and bilateral but were absent in 9 patients with multifocal disease, including 4 of 10 with bilateral tumors. About half the lesions that achieved tumorlet status, including those that gave rise to clinical tumors, underwent malignant transformation. Contralateral tumorlets should not be mistaken for metastasis and do not adversely affect prognosis. The incidence of neoplasia among young, close relatives of patients with multifocal disease appeared to be excessive, but no clear excess of major anomalies, hemihypertrophy, or minor genitourinary tract anomalies was revealed by a retrospective survey of hospital charts.

Child↗
PubMed1978

Observations on the evolution of idiopathic rapidly progressive glomerulonephritis.

Distinctive deposits of C3, C5 and properdin were identified in the minimally proliferative glomerular lesions of a patient with idiopathic rapidly progressive glomerulonephritis. Biopsies of her renal allograft at times of recurrences of her disease and of five other patients with progressive renal failure but less severe crescent formation showed deposits identical composition and position, giving evidence of a common pathogenesis. The deposits were subepithelial and located in that part of the basement membrane in apposition to the mesangium (capillary waist). Breaks in the basement membrane were often exclusively in this area suggesting the deposits were causative. Ancillary observations suggest that the subepithelial deposits become unidentifiable after the scarring of severe extracapillary proliferation develops. Three of the patients had, in addition, intramembranous dense deposits but in other ways their disease was not characteristic of membranoproliferative glomerulonephritis Type II. Instead, the evidence indicated that all six patients were in early or lage stages of idiopathic (non-streptococcal) rapidly progressive glomerulonephritis.

Basement Membrane↗
PubMed1977

Membranoproliferative glomerulonephritis with disruption of the glomerular basement membrane.

Seven patients with a form of membranoproliferative glomerulonephritis distinct in its glomerular ultrastructure from other forms are described. The use of silver impregnated electron micrographs revealed contiguous subepithelial and subendothelial deposits associated with basement membrane disruption, replication and layering of lamina densalike material. By light and fluorescence microscopy the appearance was distinctive but not diagnostic. Immunohistology consistently showed abundant C3 and properdin in a granular pattern while immunoglobulins and Clq were variably present. Low serum C3 concentrations were observed at some time in each patient, often accompanied by low levels of properdin, whereas the concentrations of Clq and C4 were normal. The patients were indistinguishable in their clinical course from those with other types of MPGN.

Basement Membrane↗