Development of Focal Segmental Glomerulosclerosis and Thrombotic Microangiopathy in a Liver Transplant Patient on Sorafenib for Hepatocellular Carcinoma: A Case Report.

Transplant patients are at risk for hemodynamic injury and glomerular diseases such as focal segmental glomerulosclerosis (FSGS) and thrombotic microangiopathy (TMA). Calcineurin inhibitors (CNI) can cause various patterns of acute kidney injury (AKI) in transplant patients and their effects must be differentiated from kidney injury due to other agents. Transplant populations are also at risk for atypical infections and malignancies. These conditions and the agents that are used to treat them can then induce their own set of glomerular diseases. We report a patient with hepatitis C who had received an orthotopic liver transplant and then developed recurrent hepatocellular carcinoma, which was treated with the oral tyrosine kinase inhibitor (TKI) sorafenib. In a manner temporally related to the initiation of the TKI, progressive AKI and high-grade rising proteinuria were noted. A biopsy disclosed FSGS and concomitant TMA. Despite the discontinuation of the TKI and high-dose steroid treatment, the patient developed end-stage renal disease and was initiated on hemodialysis. After determining the TKI as the probable culprit, as opposed to CNIs, the patient successfully received a living related renal transplant. CNIs are used to maintain renal and hepatic allografts without the development of hematuria, significant proteinuria, or significant impairment of renal function. It is noted that the pathologic phenotype observed in this case is only the second reported case of concomitant TMA and FSGS in a sorafenib-treated patient.

Case Report: First Reported Combined Heart-Liver Transplant in a Patient With a Congenital Solitary Kidney.

We report a case of successful combined heart liver transplant in a patient with a congenital solitary kidney. The patient had normal renal function before combined heart-liver transplantation and developed acute kidney injury requiring slow continuous dialysis and subsequent intermittent dialysis for almost 8 weeks post transplantation. Her renal function recovered and she remains off dialysis now 7 months post transplantation. She only currently has mild chronic renal insufficiency. We believe this is the first reported case of successful heart liver transplant in a patient with a congenital solitary kidney.

Distinguishing 3 classes of corpus callosal abnormalities in consanguineous families.

OBJECTIVE: We sought to create a classification system for pediatric corpus callosal abnormalities (CCA) based upon midline sagittal brain MRI. We used the term CCA for patients with structural variants of the corpus callosum, excluding patients with interhemispheric cyst variant or pure dysplasia without hypoplasia. Currently, no system exists for nonsyndromic forms of CCA, and attempts to create such a system have been hampered by highly variable morphology in patients with sporadic CCA. We reasoned that any useful strategy should classify affected family members within the same type, and that phenotypic variability should be minimized in patients with recessive disease. METHODS: We focused recruitment toward multiplex consanguineous families, ascertained 30 patients from 19 consanguineous families, and analyzed clinical features together with brain imaging. RESULTS: We identified 3 major CCA classes, including hypoplasia, hypoplasia with dysplasia, and complete agenesis. Affected individuals within a given multiplex family usually displayed the same variant of the class of abnormality and they always displayed the same class of abnormality within each family, or they displayed complete agenesis. The system was validated among a second cohort of 10 sporadic patients with CCA. CONCLUSIONS: The data suggest that complete agenesis may be a common end-phenotype, and implicate multiple overlapping pathways in the etiology of CCA.

Cystosarcoma phyllodes: report of two cases.

Cystosarcoma phyllodes is a rare predominantly benign tumour usually affecting the female breast. In a retrospective review of giant breast masses seen over a period of 20 years, we encountered two cases of this tumour, one benign and the other malignant, the latter exhibiting the very unusual feature of axially lymph node metastases. We present these two cases and briefly review the literature.

Giant fat containing breast masses: report of six cases.

Six patients with giant fat containing breast masses encountered over a 20 year period are presented including a brief review of the literature. These benign tumours especially the giant varieties are rare but are likely to be increasingly diagnosed because of the widespread use of mammography.

Giant fibroadenoma of the breast in an Arab population.

In a retrospective study of giant breast masses over a period of 20 years (1980-2000), we encountered 18 patients with fibroadenomas. Most of them were adolescents and young adults. The bimodal age incidence seen in Caucasians was not observed. The masses ranged from 6 to 15 cm in size and in the youngest patient, they were bilateral. All 18 patients were examined by mammography and 10 of them by ultrasonography (US) as well. The right breast was involved in 12 patients and the left in six. The diagnosis was confirmed pathologically in all patients, by excision biopsy in 17 patients and by fine needle aspiration cytology and excision biopsy in one patient. The radiological findings were the same as those previously described. All patients were treated by simple enuculation. There was only one recurrence over a follow-up period from 2 months to 3 years.

The imaging appearances of hydatid disease at some unusual sites.

On reviewing our cases of hydatid disease seen over a period of 20 years (1978-1998), we encountered a number of unusual radiological appearances and sites, which are demonstrated in this pictorial review. We briefly discuss the pathology of hydatid disease and its complications.

Percutaneous catheter drainage in drug-resistant amoebic liver abscess.

This communication records our experience with the percutaneous catheter drainage (PCD) of 22 amoebic liver abscesses in 19 patients who had failed to respond to amoebicidal therapy. In one patient with a left lobe abscess, imminent rupture was an additional indication for drainage. PCD combined with amoebicidal therapy not only expedited recovery, but was curative in all 19 patients. There were no complications. We conclude that PCD is a most useful adjunct to drug therapy and recommend its routine use in the management of drug-resistant amoebic liver abscesses.

Percutaneous drainage of tuberculous iliopsoas abscesses under image guidance.

Most intra-abdominal and other types of fluid collections are now successfully drained percutaneously under image guidance. The utility of percutaneous drainage of tuberculous abscesses, especially those associated with osseous changes, is, however, less well established. Six patients with tuberculous iliopsoas abscesses were successfully managed by percutaneous drainage combined with antituberculous therapy. The abscesses were bilateral in one patient and unilateral in the other five. Drainage was by needle aspiration under ultrasound (US) guidance in one patient, and by catheter under CT guidance in the other patients. Three patients had associated osseous changes. There were no procedural complications. Tuberculous iliopsoas abscess can be successfully treated by percutaneous drainage and appropriate antituberculous therapy.

Bone tumor radiograph review by pathologists prior to pathologic diagnosis: a receiver operator curve analysis of diagnostic utility.

Radiologic-pathologic correlation has long been considered a prerequisite for the accurate diagnosis of bone lesions. We investigated using receiver-operating characteristic analysis the accuracy of histopathologic diagnoses with and without pathologist review of radiographs. While accuracy of diagnosis did improve when the radiographs were reviewed by the pathologist, this increase did not reach statistical significance (P=0.1311). Potential reasons for this finding including case selection and reviewer expertise are discussed. Based on our study, the review of radiographs did not result in a statistically significant difference in accuracy of the pathologic diagnosis of bone tumors. However, our data suggest that there may be a difference particularly in selected cases and among certain observers.

Extrarenal angiomyolipomas of the perinephric space in tuberose sclerosis.

A case of bilateral angiomyolipomas of the perinephric space in a young Arab woman with tuberose sclerosis is reported. Multiple small angiomyolipomata were present in both kidneys and there was pulmonary involvement. The literature on this very rare occurrence is briefly reviewed.

Aspiration and sclerotherapy of symptomatic simple renal cysts: value of two injections of a sclerosing agent.

OBJECTIVE: The purpose of our study was to determine whether two injections of sclerosant were more effective than a single injection in treating symptomatic simple renal cysts. SUBJECTS AND METHODS: We reviewed our results obtained by simple aspiration, by aspiration and a single sclerosant injection, and by aspiration followed by two injections of sclerosing agent over 48 hr. We used 95% alcohol because of its capacity to safely sclerose the epithelial lining of renal cyst walls without damaging the renal parenchyma. After 1-3 min of contact with the alcohol, the epithelial cells lining the cyst become fixed and nonviable; the cyst capsule is penetrated only after 4-12 hr. Longer contact of alcohol with the epithelial cell lining should theoretically result in the nonviability of more epithelial cells and therefore in a lower recurrence rate of cysts after aspiration and sclerotherapy. Our subjects were 46 patients, 30 men and 16 women, treated over a 13-year period. They ranged in age from 38 to 75 years old (mean age, 47 years). Fifty-two cysts were treated. Twenty patients (20 cysts) had simple aspiration only, 15 patients (19 cysts) had a single injection of sclerosant after aspiration, and 11 patients (13 cysts) had two sclerosant injections after aspiration. RESULTS: Of the 20 patients who had simple aspiration only, 16 cysts had recurred after 2 years. In the single-injection group of patients, we found six recurrences after 2 years. No recurrence has occurred in the group of patients who had two sclerosant injections after aspiration. CONCLUSION: Our findings suggest that the results of treating symptomatic simple renal cysts are better in terms of recurrence with two successive injections of 95% alcohol, probably because of the nonviability of more epithelial cells lining the cyst as a result of a longer period of contact with the alcohol. Our sonographic finding of pseudotumors at the sites of previously aspirated giant cysts in two patients is recorded.

Case report: a case of solitary Peutz-Jegher's hamartoma in the small bowel with angiographic evaluation.

A case of a solitary small bowel Peutz-Jegher's hamartoma in which angiography revealed the lesion to be vascular is presented. The findings suggest that not all Peutz-Jegher's hamartomas are hypovascular as previously reported and that this type of polyp may mimic other vascular lesions such as haemangiomas and leiomyomas.

MR imaging of prostate cancer with an endorectal surface coil technique: correlation with whole-mount specimens.

PURPOSE: To evaluate the diagnostic accuracy of magnetic resonance (MR) imaging in staging prostate cancer with an endorectal surface coil technique. MATERIALS AND METHODS: The authors prospectively evaluated MR images obtained with an endorectal surface coil from 70 consecutive patients with known prostate cancer. Gadopentetate dimeglumine was administered to 40 patients. Multiple sequences were used, including conventional and fast spin echo, with and without fat suppression. The readers were blinded to the MR findings unless bone or nodal metastasis was present. MR images were compared with whole-mount sections. RESULTS: The prospective staging accuracy for MR imaging was 51% (36 of 70 patients). Stage B disease was present in 27 patients (38%), stage C in 42 (60%), and stage D in one (1%). The retrospective staging accuracy was 67% (47 of 70 patients). Of the 42 patients with stage C disease, positive surgical margins were present in 36 (85%). Gadopentetate dimeglumine did not help detect or stage tumors. CONCLUSION: Further studies must be performed to determine the role of endorectal coil MR imaging in the staging of prostate cancer.

Renal papillary necrosis in Kuwait.

In 2,158 consecutive routine urograms, 85 patients (51 males and 34 females) were found to have typical changes of renal papillary necrosis, an incidence of 3.9% (2.8% in males, 9.4% in females). The changes were bilateral in 60 patients (71.6%) and unilateral in 25 (29.4%). 3 patients were diabetics, 4 had sickle cell anaemia and 7 had obstructive uropathy. 19 patients admitted to analgesic abuse. The remaining 52 patients were idiopathic but a combination of analgesic abuse and dehydration may have been the cause in this group. The possible reasons for the high incidence of RPN especially in females in this hot desert environment are discussed. The literature on the subject is briefly reviewed.

Case report 757: Giant cell tumor of rib.

A case of the rare occurrence of conventional GCT in a rib is presented. Due to its radiological aggressivity, the possibility of a MGCT was entertained clinically, and preoperative chemotherapy and radiation therapy were given. The literature on giant cell tumors of the ribs is reviewed, and their distinction from primary MGCT and other giant-cell-containing lesions is discussed.

Life-threatening post-nephrolithotomy hemorrhage resulting in cessation of kidney function, treated by transcatheter arterial embolization with subsequent restoration of excretory function.

A massive post-nephrolithotomy renal hemorrhage was successfully controlled by selective arterial embolization using Gelfoam particles. Prior to embolization, the kidney showed no excretory function due to complete obstruction of its collecting system by blood clots. Long-term observation revealed restoration of excretory function of the treated kidney and only minimal loss of its parenchyma.

Effect of diuretics on ketamine and sulfanilate elimination in cats.

Rate constants of elimination were used to evaluate potential effects of diuretic agents on the elimination of ketamine from the blood of cats. Furosemide, mannitol, and aminophylline did not significantly alter the ketamine elimination rate constants, although the diuretics had a tendency to prolong elimination. This tendency of furosemide was further substantiated by observing the effect of furosemide on glomerular filtration. The rate of sulfanilate elimination, used as an indicator of glomerular filtration, was significantly decreased in the cats that were administered furosemide. Possible mechanisms for the influence of furosemide on the renal excretion of ketamine are discussed.

Pharmacokinetics of ketamine HCl and metabolite I in the cat: a comparison of i.v., i.m., and rectal administration.

Ketamine HCl [2-(o-chlorophenyl)-2-(methylamino) cyclohexanone HCl] concentrations in whole blood were used to study the pharmacokinetics of i.v., i.m., and rectal administrations, at a dose of 25 mg/kg, in normal domestic cats. Absorption was rapid with both the i.m. and rectal routes. Systemic availability was 51% (SEM 10) for the i.m. dose and 43.5% (SEM 6.1) for the rectal dose. The first-pass effect had a minimal influence on the metabolism of ketamine HCl administered rectally. The elimination rate constant (beta) of the drug was statistically similar in the i.v., i.m., and rectal groups, at a 95% level of significance (P less than 0.05). At the dosage rates studied, ketamine HCl produced an anesthetic effect in the cat following i.v., i.m. and rectal administration.