Gadolinium-induced nephrogenic systemic fibrosis in a patient with an acute and transient kidney injury.

Nephrogenic systemic fibrosis (NSF) describes a characteristic fibrosing disorder which typically presents with indurated plaques on the trunk and extremities of patients with advanced renal disease. We present a case of biopsy-confirmed NSF in a patient with severe acute kidney injury with no prior history of renal disease. A 64-year-old man with an acute and severe decrease in glomerular filtration rate underwent magnetic resonance imaging studies with gadolinium contrast (Omniscan) and subsequently developed NSF. His renal disease had normalized at the time his skin disease developed. Skin biopsies revealed findings of NSF and scanning electron microscopy with energy-dispersive X-ray spectroscopy confirmed insoluble gadolinium within lesional tissue.

Dermal inorganic gadolinium concentrations: evidence for in vivo transmetallation and long-term persistence in nephrogenic systemic fibrosis.

BACKGROUND: Gadolinium (Gd)-based magnetic resonance contrast agents (GBMCA), including gadodiamide, have been identified as the probable causative agents of the serious disease, nephrogenic systemic fibrosis (NSF). OBJECTIVES: To investigate retained Gd-containing deposits in skin biopsies from patients with NSF and to determine their relative concentrations over time from administration of GBMCA. METHODS: An investigator-blinded retrospective study, analysing 43 skin biopsies from 20 patients with gadodiamide-related NSF and one NSF-negative gadodiamide-exposed dialysis patient, ranging from 16 days to 1991 days after Gd contrast dose. Utilizing automated quantitative scanning electron microscopy/energy-dispersive X-ray spectroscopy we determined the concentration of Gd and associated elements present as insoluble deposits in situ in the tissues. RESULTS: We detected Gd in skin lesions of all 20 patients with NSF, whereas Gd was undetectable in the NSF-negative patient. Gd concentration increased over time in 60% of patients with multiple sequential biopsies (n=10), decreasing only when the initial sampling time was >23 months after first gadodiamide dose. All Gd-containing deposits contained phosphorus, calcium and sodium. The ratio of Gd to calcium in tissue deposits correlated positively with the gadodiamide dose and with serum ionized calcium at the time of Gd exposure. CONCLUSIONS: These findings demonstrate the in vivo release (through transmetallation) of the toxic free Gd3+ from gadodiamide, and its retention in apatite-like deposits. We suggest that Gd may be mobilized over time from bone stores, explaining variably delayed onset of NSF and increasing skin concentration over time in patients with NSF.

Congenital lobar emphysema and sequestration--treatment by embolization.

A case of congenital lobar emphysema associated with pulmonary sequestration, presenting with respiratory distress in infancy, is reported. The lobar hyperinflation was managed by emergency lobectomy. The ipsilateral lower lobe affected with vascular sequestration was salvaged by therapeutic embolization, thus avoiding the long-term sequelae of pneumonectomy. The case is reported for its rarity.

Congenital lobar emphysema: experience with 21 cases.

A retrospective study of all cases (n = 21) of congenital lobar emphysema (CLE) treated at the Royal Hospital, Muscat, from September 1988 to August 1999 was performed. The presentation, diagnosis, treatment, and outcome are reviewed. All 21 patients were nonwhite. Thirteen had left-upper-lobe, 7 right-middle-lobe, and 1 right-upper-lobe involvement. Of the 14 upperlobe cases, 10 had severe symptoms whereas only 2 of the 7 middle-lobe cases had severe symptoms. Mildly symptomatic cases on conservative treatment invariably needed surgery if infected. It is concluded that CLE is not limited to white infants, upper-lobe disease is more severe than middle-lobe disease, and nonoperative measures are likely to be useful in mildly symptomatic cases involving the middle lobe.

Fetus-in-fetu: a case report and review of the literature.

A unique case of intraperitoneal fetus-in-fetu attached to an ovary is presented. An asymptomatic newborn girl was found to have a mobile cystic mass in right side of her abdomen. Radiological investigations showed fetus-in-fetu. During laparotomy, an intraperitoneal fetus-in-fetu was found attached to right ovary and vascular pedicle to ovarian vessels. Only 79 cases of fetus-in-fetu have been reported, and this is the first such case attached to an ovary.

Concurrent right diaphragmatic hernia and esophageal atresia.

The occurrence of a coexisting congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) with distal tracheoesophageal fistula is extremely rare and is considered highly lethal. Only 19 cases of CDH with EA have been reported in the world literature to date. This is a very challenging clinical problem, and the neonate is likely to deteriorate rapidly. Such a case is reported with a successful outcome, probably the first survivor with a right CDH and EA. Management guidelines for such a case are discussed.