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Role of slit-lamp biomicroscopy in the diagnosis of cystinosis.

The purpose of presenting this case is to emphasize the importance of the slit lamp examination in patients with complex symptoms and signs of growth retardation, organomegaly, and abnormal kidney function tests. As the clinical expression varies greatly from one patient to another and in spite of extensive and expensive work-up, final diagnosis of cystinosis may be established only by careful ophthalmologic examination.

Child, Preschool↗

Nephrotoxicity of cyclosporin A in liver and kidney transplant patients.

In six of twelve orthotopic liver recipients nephrotoxicity was noted after 13-22 days of treatment with 16.3 + or - 2.9 (SEM) mg/kg per day of cyclosporin A (CyA). With a decrease in the daily CyA dose to 9.2 + or - 2.3 (SEM) mg/kg kidney function returned to normal. No hepatic rejections occurred on this lowered CyA dose. In 4 out of 66 kidney recipients a switch from a CyA dose of 5.2 - 10.7 mg/kg daily to azathioprine was done 4 - 8 months after transplant because of unsatisfactory kidney function, suspected to be due to nephrotoxicity. In three patients, this resulted in an improved graft function. A fourth transplant was lost to an irreversible rejection 13 days later. Thus CyA is nephrotoxic but this toxicity is easily reversed, even after many months of treatment, and the ease with which this complication can be managed suggests that nephrotoxicity should not diminish the high expectations that transplant surgeons have for CyA.

Cyclosporins↗

Long-term ribavirin therapy in hepatitis C virus-positive renal transplant patients: effects on renal function and liver histology.

BACKGROUND: Long-term renal transplant (RT) recipient mortality and graft loss increase significantly in hepatitis C virus positive (HCV-[+]ve) patients. Treatment with alpha-interferon in this population is associated with a high rate of acute rejection. The aims of this study were the evaluation of the efficacy and the safety of ribavirin monotherapy in 16 HCV-(+) RT patients (group A) matched to 32 HCV-(+) RT patients (group B) who did not receive ribavirin. METHODS: Ribavirin was started at a daily dose of 1,000 mg and then adapted to hemoglobin level. The study was scheduled for 1 year. RESULTS: Ribavirin monotherapy was associated with a decrease in liver enzymes and serum creatinine levels. When proteinuria was present, this decreased or disappeared. There were no significant changes in HCV viremia. There was a significant progression in liver fibrosis with no improvement in inflammation scores. Hemoglobin levels fall dramatically, despite an important support by recombinant erythropoeitin (median, 20,000 IU/wk). In 3 cases, ribavirin therapy had to be stopped. In the control group, after 1 year of follow-up, there was a significant increase in serum alanine aminotransferase and creatinine values. Proteinuria decreased in only 2 of 12 patients (P = 0.03 as compared with group A). CONCLUSION: One year of ribavirin monotherapy seems to have, at best, no beneficial effect on liver histology, although it improves liver enzyme levels. Despite its efficiency to dramatically decrease proteinuria, its impact on renal function remains unknown.

Adult↗

New observations in the clinical spectrum of the Budd-Chiari Syndrome.

Some observations on the natural history, surgical management and metabolic phenomena in B.C.S. are presented. In one patient side to side portocaval shunt brought dramatic improvement with asymptomatic followup of 5 years. The patient died of causes unrelated to B.C.S. At postmortem the shunt was patent and normal liver architecture was preserved. The second patient presented with a fulminant course culminating in acute hepatorenal syndrome. There was marked hypofibrinogenemia and extreme elevation of SGOT. Emergency portocaval shunt was followed by marked improvement in liver status. The patient died of respiratory complications on the 8th postoperative day. The third patient is one year on conservative treatment only and well. In this patient excessive renal tubular reabsorption of sodium at a distal site was documented. This may explain the generalised edema formation in patients with B.C.S. even without inferior vena cava obstruction. This is apparently the first reported instance of renal tubular function studied in B.C.S.

Adult↗

Maintenance of unstable kidney donors.

Cerebral death is often associated with haemodynamic changes which include a decrease in cardiac output and peripheral resistance. Brain-death following head injury may also lead to acquired diabetes insipidus with secondary water and electrolyte derangement. It is therefore necessary to prevent and correct these alterations, particularly when long-term maintenance is required, in order to keep kidney function within the normal range. Computerized monitoring of renal function and electrolyte and water derangements has been adopted. In all cases where early data of renal failure or oliguria were present infusions of dopamine and fluids were started. When indicated, the optimal dose of dopamine was calculated using a computerized system to allow drug dosages and the time of haemodynamic derangement to be minimized. When acquired central diabetes insipidus was present and urine output greater than 4 ml kg-1 h-1 desamino-cis-D-arginine vasopressin (DDAVP) was administered.

Acute Kidney Injury↗

Targeting of mannosylated liposome incorporated benzyl derivative of Penicillium nigricans derived compound MT81 to reticuloendothelial systems for the treatment of visceral leishmaniasis.

The antileishmanial property of a Benzyl derivative of a new antibiotic MT81 (Bz2MT81), isolated and purified from a fungal strain of Penicillium nigricans NRRL 917 was tested in free, liposome intercalated and mannose coated liposome intercalated forms in vivo against visceral leishmaniasis in hamsters. Mannose grafted liposome intercalated Bz2MT81 eliminated intracellular amastigotes of Leishmania donovani within splenic macrophages more efficiently than the liposome intercalated Bz2MT81 or free Bz2MT81. At a dose equivalent to 7.5 microg/Kg body weight when injected subcutaneously (s.c) in mannose grafted liposome intercalated form for 15 days in an interval of three days, the splenic parasitic load decreased to the extent of 79.1% of the total parasite present in infected control animals. Whereas, an identical amount (7.5 mug/Kg body weight) of Bz2MT81 in free or liposome intercalated form was found less effective in controlling the parasite in spleen (in free Bz2MT81 form, suppression of parasitic load is 49.8% and in liposome intercalated form, it is 55.1%). Both mannosylated liposomes and Bz2MT81 were noted non-toxic to the host peritoneal macrophages. Histological examinations of spleen and liver, kidney function tests (SGPT, alkaline phosphatase, creatinine and urea in blood plasma) showed that the toxicity of Bz2MT81 was reduced up to normal level when mannose grafted liposomal Bz2MT81 were administered.

Animals↗

Further studies on autoclaved cercarial vaccine against schistosomiasis: safety, longevity and stability.

The autoclaved cercarial vaccine (ACV) which is a special type of killed vaccine has been reported to induce experimental high level of homologous protective immunity. This study was to adjust the dose and to assess vaccine safety, longevity and stability as well as the possibility of transplacental transmission of immune response from pregnant mice to their offspring. The results showed that two doses of the lowest most effective concentration of ACV that achieved the high percentage reduction of worm burden is safe as demonstrated by absence of any local or systemic side effects, normal blood picture and normal liver and kidney function tests. ACV is stable when kept either at 4 degrees C for six months or at -35 degrees C for up to 12 months and it offered considerable duration of longevity. Offspring of vaccinated mothers didn't show any signs of protection against challenge infection.

Animals↗