Axillary lymphadenopathy secondary to cat-scratch disease.
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Adult↗
Biomedical subjects
M R Joyce
Publications and source records attributed to M R Joyce.
Ruptured abdominal aortic aneurysm in a 12-month-old boy.
A rare case of abdominal aortic aneurysm in a 12-month-old boy is reported. The clinical presentation was one of acute catastrophic rupture. The morphologic and histologic findings suggested an idiopathic etiology.
Aortic Aneurysm, Abdominal↗
A case of Fournier gangrene complicating idiopathic nephrotic syndrome of childhood.
A 10-year-old boy presenting with steroid resistant nephrotic syndrome developed Fournier gangrene of the scrotum. Antimicrobial drug therapy, intravenous albumin, excision of necrotic scrotum and left orchidectomy followed by skin grafting 3 weeks later led to an excellent cosmetic and medical result. Six months later he remains nephrotic on diuretic and angiotensin converting enzyme inhibitor medication.
Amoxicillin↗
Giant pharyngeal gland nuclei of Ascaris: unusual cytology suggests a novel pathway for transport of ribosomal proteins to the nucleus.
We describe the unusual giant pharyngeal gland nuclei of the parasitic roundworm Ascaris lumbricoides suum and attempt to reconcile the relationships among its uncommon, and in some instances unique, collection of constituents. The nuclei were studied by light and electron microscopy, Feulgen cytophotometry, nuclear size analysis and histochemical methods, including those for detection of DNA, RNA, acidic and basic proteins, lipids and carbohydrates. A highly active nucleus is revealed: an extensive system of intranuclear annulate lamellae (IAL); membrane-bounded, acidophilic bodies which contain non-basic proteins; an abundance of free nucleoli; and an exceedingly large chromocenter containing a core of DNA surrounded by nucleoli (an apparent nucleolar organizing region, NOR). The relationships among the various nuclear constituents suggest that the acidophilic bodies consist of nucleolus-related proteins imported from the cytoplasm to the NOR, and that the annulate lamellae mediate this transport. Incidental findings include rounded cytoplasmic invaginations into the nucleus, thread-like structures with dense cores and surrounding small granules found among concentrations of nucleoli, and bundles of 12 nm filaments closely associated with these nucleoli. The significance of such huge, morphologically complex and highly polyploid nuclei, derived from chromatin-diminished progenitor cells early in development, and the possible interrelationship of these phenomena, remain obscure.
Animals↗
Mirror image duplication of the hands and feet: report of a sporadic case with multiple congenital anomalies.
Mirror image duplication of the hands and feet is a rare entity. Based on 3 previous reports, findings include nasal abnormalities, dimelia of ulna and fibula, tibial hypoplasia and mirror image duplication of hands and feet. We report on a sporadic case in which mirror image duplication was associated with multiple congenital anomalies. Although these cases may represent variable expression of the same dominantly transmitted complex polysyndactyly syndrome, it is possible that mirror image duplication of the hands and feet is a manifestation common to a number of distinct clinical entities. During limb bud development, duplication and aberrant positioning of the zone of polarizing activity in relation to the apical ectodermal ridge may account for the anatomic abnormalities of the hands and feet in these patients.
Abnormalities, Multiple↗
Microcephalic osteodysplastic dysplasia.
We present two patients with a distinct facial phenotype, short stature, brachydactyly, clubfoot deformities, cataracts, microcephaly, and normal intelligence. Similar radiographic abnormalities of the spine, long bones, hands, and feet were noted. These patients are similar to 2 males previously described by Saul and Wilson [1990: Am J Med Genet 35:388-393]. These 4 patients appear to have a unique skeletal dysplasia characterized by microcephaly, distinct facial phenotype, multisystem abnormalities, and short stature of postnatal onset.
Abnormalities, Multiple↗
Radiographic imaging studies in pediatric chronic sinusitis.
BACKGROUND: The diagnosis of chronic sinusitis is dependent on the radiographic evidence of sinus disease. METHODS: We evaluated the performance of radiographs and computed tomographic (CT) scans for the examination of the paranasal sinuses of 91 patients of both sexes, ranging in age from 2 to 17 years, who had chronic upper respiratory tract symptoms for at least 3 months. The CT scan findings were categorized as no disease; minimal disease, and mild, moderate, and severe sinusitis. RESULTS: Fifty-eight patients (63%) had chronic sinusitis: CT scan abnormalities were minimal in 17%, mild in 19%, moderate in 21%, and severe in 43%. There was a statistically significant correlation between rhinorrhea (r = 0.25, p = 0.01), cough (r = 0.27, p = 0.009), and the severity of sinus abnormality as determined by CT scan. Clinical presentation in the mild, moderate, and severe sinusitis groups (p < 0.05) was significantly different from that of the no disease group, whereas the minimal disease group had subclinical presentation (p = 0.11). Clinically significant chronic sinusitis often occurred at multiple sites: 44% of patients had pansinusitis, 50% had disease involvement of at least two sinuses, and 6% had disease in a single sinus. When sinus radiographs were compared with CT scans (n = 70 cases), radiographs could not identify minimal disease. For clinically significant sinusitis, sinus radiographs detected disease in 1 of 5 (20%) frontal sinuses, 0 of 12 (0%) sphenoidal sinuses, and 17 of 31 (54%) ethmoidal sinuses. With the minimal criteria of 40% to 50% opacification or fluid level filling of the maxillary antrum, radiographs detected disease in 37 of 49 (75%) cases. The sensitivity and specificity for a Waters view to confirm clinically significant chronic sinusitis without specifying the sites and severity were acceptable at 76% and 81%, respectively. When limited sinus CT scans were compared with full CT evaluation (n = 49 cases), limited studies detected 5 of 5 (100%) frontal, 9 of 11 (82%) sphenoidal, 14 of 19 (73%) ethmoidal, and 39 of 40 (97%) cases of maxillary sinusitis. The overall agreement was 88%. CONCLUSIONS: A single Waters view is an acceptable part of the initial evaluation of pediatric chronic sinusitis; however, a limited CT scan is a better alternative.
Adolescent↗
Trichothiodystrophy and associated anomalies: a variant of SIBIDS or new symptom complex?
Trichothiodystrophy is characterized by sparse, short, sulfur-deficient hair. Numerous symptom complexes have been described in which the hair abnormality represents a constant feature. We report a boy with trichothiodystrophy, ichthyotic skin changes, onychodystrophy, chronic neutropenia, osteosclerosis, hypothyroidism, nystagmus, growth and mental retardation, and microcephaly, who developed a progressive encephalopathy with ataxia and optic atrophy at 2.5 years of age. In addition to a deficient cystine level identified on a hair sample, a disturbance in the composition of other amino acids was present. Although features were reminiscent of osteosclerosis, ichthyosis, brittle hair due to trichothiodystrophy, impaired intelligence, decreased fertility, and short stature (SIBIDS) and could represent a variant of this disorder, findings in our patient may reflect a new trichothiodystrophy symptom complex that carries a poor prognosis for survival beyond childhood.
Growth Disorders↗
Pregnancy in renal transplant recipients: case report.
Pregnancy in a renal homograft recipient resulted in an emergency cesarean section at 32 weeks' gestation secondary to maternal small bowel obstruction with necrotic bowel and subsequent intestinal perforation. The 1814 gm female product of this pregnancy had a moderate degree of in utero asphyxia, which subsequently, based on radiologic studies, had to be differentiated from the possibility of a congenital viral infection. Several concerns arose regarding the effects on the fetus by maternal immunosuppressive therapy, the risk factors faced by both the fetus and the mother secondary to the mother's renal disease, and the general outcome of the increasing number of pregnancies in this population.
Adult↗
Humero-radio-ulnar synostosis: a new case and review.
We report on a patient with humero-radio-ulnar synostosis and upper limb oligoectrosyndactyly. All cases have been sporadic including discordance in monozygotic twins, and similar findings have occurred in thalidomide embryopathy. Further observations of similarly affected patients are needed to elucidate the nature of this upper limb defect and its cause.
Abnormalities, Multiple↗
Percutaneous stone and stent removal from renal transplants.
Techniques developed for removal of stones from normally sited kidneys can be safely employed in the transplanted kidney. We describe our experience in removing stones, stent material and organised blood clot from renal transplant collecting systems, using modified percutaneous techniques.
Adult↗
Calculus in a congenital recto-urethral fistula: a late presentation.
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Adult↗
Renal function following surgical correction of vesico-ureteric reflux in childhood.
To assess the effectiveness of the surgical correction of vesico-ureteric reflux, current renal function was determined in 56 children with scarred kidneys who had undergone ureteric reimplantation between 1978 and 1983. The children were aged between 2 and 15 years, had no coexisting urological disease and a glomerular filtration rate (GFR) of less than 90 ml/min/1.73 m2. In the 32 patients who had bilateral reimplantation of ureters draining bilaterally scarred kidneys the improvement in GFR following surgery was highly significant (P less than 0.001), with improvement occurring in 75%. The greatest improvement was in patients where the GFR was less than 50 ml/min/1.73 m2 (29%). Individual kidney GFR was estimated in 42 kidneys and 81% improved after surgery. This improvement was highly significant (P less than 0.001).
Adolescent↗
Hypertension and thromboembolism in idiopathic retroperitoneal fibrosis.
Of 36 patients with idiopathic retroperitoneal fibrosis treated surgically, 8 have died and 26 have suffered 67 post-operative complications during 1 to 24 years of follow-up. Four patients died as a consequence of hypertension. Hypertension and thromboembolism accounted for 49% of the post-operative complications in 69% of the patients who developed complications. Attention to these 2 factors is at least as important as prevention of recurrent obstructive uropathy.
Adult↗
Neuropathic vesicourethral dysfunction in children. A trial comparing clean intermittent catheterisation with manual expression combined with drug treatment.
Forty-three children with overt neurological disease and neuropathic vesicourethral dysfunction were entered into a trial comparing clean intermittent catheterisation (CIC) with manual expression combined with drug treatment (non-CIC). The 22 children in the CIC group showed a significantly greater improvement in continence than the 21 children in the non-CIC group (P less than 0.001) without a significant increase in the incidence of urinary tract infections. Neither form of treatment is effective in reducing the risk of deterioration of renal function due to the combination of detrusor-sphincter dyssynergia and vesicoureteric reflux. Neither form of treatment improves continence in the presence of gross sphincter weakness or gross impairment of bladder compliance.
Bethanechol Compounds↗
Dorsiflexory wedge osteotomy of the first ray utilizing internal cancellous screw fixation.
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Bone Screws↗
Improvement in renal function following ureteric reimplantation for vesicoureteric reflux.
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Adolescent↗
Use of 99Tcm-DMSA as a static renal imaging agent.
The use of 99Tcm-labelled DMSA as a static renal imaging agent has been analyzed semi-quantitatively in 366 patients. Study with this agent proved to be of most value in patients with equivocal space-occupying lesions of the kidney, provided useful information in various destructive diseases of the kidney when used for determining divided renal function but was of little value in chronic renal failure. In 33 patients, the uptake of 99Tcm-DMSA at 3 h as a measure of divided renal function was compared with the uptake of 99Tcm-DTPA from 30 to 150 sec following injection and was found to correlate well. In a series of 7 dogs with induced unilateral renal impairment, divided function determined with 99Tcm-DMSA was found to correlate well with results obtained using 51Cr-EDTA.
Animals↗