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Biomedical subjects

R Craver

Publications and source records attributed to R Craver.

At least 19 recordsLinked to original sources

Parathyroid hyperplasia: an unusual cause of neonatal hypercalcemia.

Primary hyperparathyroidism (PHPT) in infants is caused by parathyroid chief cell hyperplasia. Patients present with symptoms of chronic hypercalcemia, such as failure to thrive, irritability, abdominal pain, and anorexia. Medical therapy is inadequate, often resulting in chronic hypercalcemia or death. Partial or total surgical removal of the parathyroid gland is the preferred treatment. We describe a case of a 7-month-old infant with PHPT secondary to hyperplasia successfully treated with a subtotal parathyroidectomy.

Female↗

Renal involvement in juvenile rheumatoid arthritis: report of two cases.

Renal involvement is a rare occurrence in juvenile rheumatoid arthritis (JRA). We report on two JRA patients with kidney disease. The first was a 14-year-old African-American female with a 12-month history of polyarthritis. On presentation she was found to have an ESR of 127 mm/h and a positive ANA, rheumatoid factor (RF), perinuclear antineutrophil cytoplasmic antibodies (pANCA), haematuria, proteinuria with normal BUN and creatinine. Renal biopsy showed focal segmental glomerulosclerosis. Her renal function deteriorated to end-stage renal failure requiring dialysis within a few months, despite aggressive treatment with steorids and monthly i.v. pulses of cyclophosphamide. The second patient presented with a 6-week history of polyarthritis and intermittent fever, and had a salmon-coloured evanescent rash. On presentation his laboratory evaluation was significant for elevated ESR and negative ANA, RF and ANCA tests. Within 8 months the patient had developed a persistent microscopic haematuria. Renal biopsy showed mild mesangial glomerulonephritis. On low-dose methotrexate therapy his JRA went into remission and his renal function remained normal. The haematuria persisted for 1 year and then resolved spontaneously. This is the first time that focal segmental glomerulosclerosis and mesangial glomerulonephritis have been described in JRA. Although the association may be just coincidental, further studies are needed to define the role of JRA in these renal conditions. In patients with JRA, urinalysis and renal function should be routinely monitored.

Adolescent↗

Immunotactoid glomerulopathy in sickle cell anemia.

A 12-year-old African American male with homozygous sickle cell disease (SCD) was admitted with insidious onset of periorbital and scrotal edema. The initial evaluation failed to reveal any underlying monoclonal gammopathy, or cryoglobulinemia, or other systemic causes for the renal disease. A percutaneous renal biopsy was consistent with immunotactoid glomerulopathy (ITG), which is rare in children and is characterized histologically by fibrillar deposits in the glomeruli. Children can present with symptoms of nephrotic syndrome and progress to end stage renal disease. Our patient was treated with an ACE inhibitor and is currently free of edema and with normal renal function on follow-up at 1 year. Immunotactoid glomerulopathy should be considered in the differential diagnosis of nephrotic syndrome in children with sickle cell disease. Renal biopsy is indicated in children with sickle cell disease and nephrotic syndrome and ITG should be considered as potential cause. Although there is no effective treatment for this condition, ACE inhibitors can decrease the protein-uria and possibly delay the progression to end stage renal disease. The side effects related to the use of ACE inhibitors should be monitored. These include renal impairment, hyperkalemia, anemia, neutropenia, and angioedema. Since we have a short follow-up in our patient, the role and safety of ACE inhibitors in the management of ITG need further evaluation.

Anemia, Sickle Cell↗

Detection of interferon regulatory factor-1 in lamina propria mononuclear cells in Crohn's disease.

BACKGROUND: The transcription factor, interferon regulatory factor (IRF)-1, is stimulated by interferon-gamma and regulates the expression of several genes implicated in the pathogenesis of inflammatory bowel disease, including interleukin-6, major histocompatibility complex class II molecules, and inducible nitric oxide synthase. Interferon regulatory factor-1 also stimulates naive CD4+ T-cells to differentiate into T-helper-1 cells, the T-cell subset that appears to be upregulated in Crohn's disease. The purpose of this study was to examine the expression of IRF-1 in the nuclei of lamina propria mononuclear cells in situ in colonoscopic biopsy specimens from pediatric patients with Crohn's disease, in patients with ulcerative colitis, and in control patients with no histopathologic abnormalities. METHODS: Archival paraffin-embedded tissue sections were obtained from 25 pediatric patients with Crohn's disease, 6 patients with ulcerative colitis, and 12 control patients who had undergone colonoscopy. Tissue sections were stained with polyclonal rabbit anti-human antisera to IRF-1 and horseradish-peroxidase-conjugated, biotinylated, goat anti-rabbit secondary antibody. Slides were scored and scores compared among patient groups using analysis of variance. RESULTS: Patients with Crohn's disease had significantly higher IRF-1 scores (95% confidence interval [CI], 1.70-2.04) than patients with ulcerative colitis (95% CI, 0.92-1.23) or control subjects (95% CI, 1.11-1.52). CONCLUSIONS: Increased expression of IRF-1 in lamina propria mononuclear cells from patients with Crohn's disease may be relevant to the pathogenesis of Crohn's disease.

Adolescent↗

Chondroblastoma.

Explore the source record for details and available documents.

Adolescent↗

Isolated CNS involvement in Ewing's sarcoma.

Ewing's sarcoma, an uncommon malignant neoplasm of bone, represents about 10% of all the malignant primary bone tumors. The assumption that subclinical metastases are already present in patients with apparently localized tumor indicates the need for systemic therapy as an integral part of primary treatment. The usual sites of metastases are the lungs and skeletal system. Central nervous system (CNS) involvement is rare and is usually seen only in disseminated and fairly advanced disease. We report two patients in whom, after aggressive adjuvant chemotherapy, disease developed in the CNS without local recurrence. In one patient, meningeal involvement with malignant cells was identifiable in the cerebrospinal fluid; in the second patient, who presented with a space-occupying lesion, a diagnosis of arachnoid involvement was made histologically.

Adolescent↗

Subacute osteomyelitis.

Subacute osteomyelitis is a chronic low-grade infection of bone characterized by a lack of systemic manifestations. The onset is insidious. Pain is the most common symptom, and has usually been present for several months before the initial evaluation. Swelling and tenderness over the area of involved bone may also be seen. Laboratory evaluation is unrevealing, with a normal white blood cell count and differential. The erythrocyte sedimentation rate may also be normal. The most common organism cultured from a subacute osteomyelitis is a staphylococcus species. Twenty-five percent of subacute bone infections are sterile. The most common manifestation of a subacute osteomyelitis in a child is a geographic lytic metaphyseal lesion (Brodie's abscess). Treatment of a culture positive infection includes surgical debridement and antibiotic therapy. A sterile abscess can be treated conservatively without antibiotics, provided the patient's symptoms are improving and the lesion is regressing radiographically.

Adolescent↗

So-called primitive neuroectodermal tumor in aborted previable fetuses.

We have described the gross anatomic and histopathologic characteristics of 36 tumorlike lesions found in previable fetuses. Although these lesions appear to be invasive, metastasizing, and likely contributing to the death of the fetus, they represent a frequent finding only among severely macerated retained fetuses of 9-16 developmental weeks and have never been found in fresh nonretained fetuses. No family history of neurofibromatosis could be elicited in any of these cases to support the suggestion in the literature that these tumors may represent fetal manifestations of neurofibromatosis. The most likely explanation is that they represent an artifact owing to squeezing of brain tissue into the spinal canal and along the spinal nerves into retroperitoneal and retropleural spaces or the neck area. As this artifact appears to be confined to a specific developmental period (9-16 weeks), it may be related to certain developmental characteristics such as a very soft consistency of brain tissue or incomplete development of vertebral foramina. A higher incidence of these lesions among heteroploid fetuses remains unexplained.

Aneuploidy↗

Congenital obstructive uropathy and nodular renal blastema.

The occurrence of nodular renal blastema and renal dysplasia was determined in a retrospective study of 75 cases of congenital obstructive uropathy. Nodular renal blastema was present in 3 upper pole nephrectomy specimens removed as a consequence of nonfunction owing to ectopic ureterocele; none was dysplastic. A more differentiated type of nodular renal blastema was present in 3 other total nephrectomy specimens, bilateral involvement in a case of posterior urethral valves and unilateral nodular renal blastema associated with ureteral atresia. This subset of differentiated nodular renal blastema was associated with renal dysplasia.

Child↗