Nephropathic Cystinosis

Updated: February 27, 2023

Background

Epidemiology

Anatomy

Pathophysiology

Etiology

Genetics

Prognostic Factors

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

cysteamine

Dose Adjustments

Renal impairment- in the case of renal impairment, dose adjustment is not considered meaningful

cysteamine

Starting dose- 1/6th to 1/4th of the target maintenance dose
Gradually increase the dose over 4-6 weeks until the maintenance dosage is achieved that helps reduce the risk of adverse reactions
Maintenance dose for < 12 years: 1.3 gm per day orally every 6 hours; may not increase more than 1.95 g/m2 per day
Maintenance dose for > 12 years: 2 gm per day orally every 6 hours; may not increase more than 1.95 g/m2 per day

Future Trends