Recent trends in management of common glomerulopathy

Diagnosis of glomerulopathy requires correlation between: (a) clinicopathological syndromes, (b) kidney histology for the abnormalities which are useful for the severity of disease, stage of activity and extent of chronicity.  Subsequently, management dictates the choice of effective medications to avoid renal loss and long-term side effects.  In this review article; we provide our practical experience with drug-therapy in common idiopathic and secondary glomerular diseases. Keywords: glomerulopathy, kidney biopsy, nephrotic syndrome, Rituximab, treatment

The limited role and risky profile of Rituximab in nephritis associated with Henoch-Schönlein purpura

Adult-onset IgA vasculitis or Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA1-dominant deposits.  The symptoms include cutaneous purpura, ankle arthritis, enteritis and nephritis.  HSP nephritis (HSPN) can be severe and refractory to corticosteroids with/without immunosuppressive agents.  The concept of depletion of antibody producing B cell with Rituximab is appealing despite the uncertainity of HSP pathogenesis.  In the present case report; we describe our experience with Rituximab treatment in a patient with this disease.  Our patient had different triggering factors for her relapses and lately Rituximab itself.  Review of the literature indicates that autoantibodies to Gd-IgA1 did not decrease with Rituximab therapy and others indicated an inherited predisposition for higher levels in patients with progressive disease.  Our findings confirm the limited role and risky profile of Rituximab in treatment of HSP.  Keywords: HSP, IgA, Rituximab, vasculitis

Bilateral adrenal hyperplasia; a common cause of drug-refractory hypertension yet amenable to medical treatment

Over the past 4 and 1/2 years, a total of 97 patients had hypertension yet lacked clinical, laboratory and radiological evidence of renal, renovascular and endocrine disease were investigated for A/R ratio.  High A/R was detected in 30 patients.  Five patients had unilateral adrenal adenoma and 1 had cancer while 24 patients (24.7%) had bilateral enlargements indicating bilateral adrenal hyperplasia (BAH).  Our study has shown that BAH is: (a) easily diagnosed with a combination of A/R ratio and CT scan of the adrenal gland, (b) responsible for 24.7% of hypertension cases, (c) associated with moderate to severe hypertension that may require 2-4 antihypertensives, (d) associated with hypokalemia in only in 54% of the cases, (e) not controlled with a single daily dose of Spironolactone (S) and ½ the cases require 50 mg/day.   Moreover, it has shown that S treatment was not associated with significant hyperkalemia yet gynecomastia and erectile dysfunction were common side effects.  Interestingly; and despite normalization of A/R ratio, most patients continue to require antihypertensive drugs though the number and dosage were less.  The latter phenomenon was more evident in those with higher initial A/R ratio and longer duration of hypertension.  Nephroangiosclerosis is the most plausible explanation for it.  In conclusion; BAH is not a rare disease and should be considered in cases of refractory hypertension.  Keywords: Aldosterone, Aldosterone/Renin ratio, hypertension, Spironolactone

Acute Renal Injury: Revisited

Acute kidney injury (AKI) indicates its abrupt deterioration and is defined as an increase in serum creatinine more than the baseline by > 26 umol/L within 48 hours or > 50% within 1 week.  The latter since glomerular failure is the life-threatening one with: (a) uremic intoxication, (b) water and salt retention with fluid overload, and (c) potassium accumulation with cardiac arrest.  The etiology can be pre-renal, post-renal or intrinsic.  Diagnosis is established by history of new insults, physical examination for hydration status, systemic stability and manifestations of autoimmune diseases/infections as well as an initial laboratory testing for renal function (serum creatinine, electrolytes and urine routine) and kidney ultrasound.  Additional specific tests are indicated to assess etiology of AKI and its associated co-morbid conditions that interacts with its management.  Severity of AKI ranges from mild (stage 1) to advanced (stage 5) that requires dialytic support.  Moreover, it depends on the type and duration of the insult.  Prognosis depends on etiology of AKI, its co-morbid conditions and the timely interventions by the supportive medical team.  Keywords: acute, causes, epidemiology, injury, kidney, management

Dietary Restriction: A Major Factor in Prophylaxis against Calcium Oxalate Urolithiasis

Urolithiasis (Ur) Is A Worldwide Problem That Affects All Groups Of Ages.  Nearly 80% Of Renal Stones Are Calcium Oxalate (Cao) And 50% Of The Affected Patients Have Recurrent Disease Within 10 Years.  Our Prospective Study Was Conducted Over 4 And ½ Years And Evaluated The Role Of Dietary Manipulation In Prophylaxis Against Cao Ur.  A Total Of 212 Patients With Recurrent Cao Ur, Who Lacked Anatomical Or Metabolic Derangement, Were Subjected To A Practical And Specific Diet.  The Latter Had: (A) Low Salt, Red-Meat And Green Leafy Vegetable, (B) Moderate Amounts Of Milk, Dairy Products, Poultry And Certain Fish-Items, And (C) High Water Intake (2 Liters/Day).  A Total Of 66/70 (96%), 87/108(88%) And 146/167(87.4%) Patients Were Stone-Free By The End Of 1, 2 And 3 Years Of Follow Up.  The Median Time For Stone-Free Duration Was 33 (28.7-37.3) Months.  Adding A Thiazide And Allopurinol To The 19 Patients Who Had Failed Dietary Prophylaxis Prevented Stone Formation In 16 More Patients Leaving Only 3 True Failures.  Four Patients Could Not Tolerate The Latter 2 Drugs For Allergy.  In Conclusion; Our Practical Dietary Modification Can Aid In Prophylaxis Against Cao Ur.    Keywords: Diet, Calcium Oxalate, Urolithiasis, Urinary Tract Stones, Prophylaxi

The Beneficial Effect of Paricalcitol (Zemplar) on Secondary hyperparathyroidism Refractory to Calcitriol (Calcijex) and Cinacalcet HCl (Mimpara) in Patients on Maintenance Hemodialysis: A Prospective and Crossover Study

Paricalcitol, 19-nor-1, 25-dihydroxvitamin D2, is a novel 1,25–dihydroxyvitamin D2 analogue, which has greater potential to suppress parathyroid hormone without inducing significant hypercalcemia and hyperphosphatemia.  We therefore investigated its role in treatment of patients with moderate to severe secondary hyperparathyroidism (SHPT) who were refractory to conventional therapy with Calcitriol and Cinacalcet HCl.  Eight patients were recruited in whom conventional therapy was stable over the past 3 months.  After a washout period of 2 weeks, calcijex injections were replaced by Paricalcitol.  Paricalcitol induced more suppression of iPTH compared to the conventional therapy without significant hypercalcemia, hyperphosphatemia or side effects.  The conversion ratio was approximately 1:2.5 (calcitriol:paricalcitol).  In conclusion, injectable Paricalcitol is superior to Calcitriol in treatment of SHPT. Keywords: Cinacalcet, Calciferol, hemodialysis, hyperparathyroidism, Paricalcitol

Diabetes Insipedes in a child disclosing localized Langerhans’ Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is monoclonal neoplastic condition of aberrant bone marrow histiocytes.  The latter are part of the innate immune system and certain exogenous/endogenous stimuli may trigger its expansion.  Hence LCH can present with limited or multiple organ involvement that may include; bones, lung, endocrine, skin, lymph nodes, spleen and bone marrow.  In this case report, we describe a 3-year-old boy who presented with severe polyuria and polydipsia.  Laboratory investigations were consistent with diabetes insipidus (DI).  MRI of the brain; confirmed absence of the bright spot in his pituitary gland did not show evidence of tumor or enlargement by inflammation.  Moreover, MRI revealed 2 skull lesions and their subsequent biopsy confirmed LCH.  Systemic examination and tests including PET scan did not show additional lesions.  Since his disease was localized, he received only Desmopressin acetate 120 ug twice daily for his DI without surgery, radiotherapy or chemotherapy.  One year later, his disease remained limited to DI and the 2 bonny lesions.  Keywords: bone, diabetes insipidus, pituitary, desmopressin, Langerhans cell histiocytosis

Evolocumab in treatment of acute pancreatitis induced by hypertriglyceridemia

Acute pancreatitis (AP) is a common emergency resulting from inflammation of the pancreas. The mechanism involves premature activation of enzyme precursors in the acinar cells triggering a self-digestive inflammatory cascade.  Hypertriglyceridemia is the most common etiology of pancreatitis after gall stones and alcohol.  It usually follows a sudden surge such as diabetic ketoacidosis on top of hereditary hyperlipidemia.  In the present case report; we describe a patient with type-I diabetes mellitus who had developed recurrent attacks of hypertriglyceridemia AP (HTG-AP) despite Fenfibrate-therapy and report on our experience with Evolocumab in his treatment and prophylaxis.  Keywords: diabetes mellitus, hypertriglyceridemia, pancreatitis, prophylaxis, treatment

Rituximab therapy for Severe and Persistent Lichenoid drug-reaction

We report on a patient with severe and fixed lichenoid drug-reaction.  She did not respond to 2 months treatment with high-dose corticosteroids.  Hence, Rituximab was given.  One month later, her lesions improved and remained stable for 14 months of follow up.    Keywords: fixed drug reaction, Prednesone, Rituximab

Long-term maintenance therapy with Cyclosporine A in adults with generalized pustular psoriasis

Generalized pustular psoriasis (GPP) is a rare and serious immune-mediated skin disorder that is characterized by a widespread eruption of sterile and subcorneal pustules.  In the present study we investigated the efficacy of Cyclosporine A (Cy A) in treatment of 9 adults with drug-refractory GPP viz. topical Corticosteroids, retinoids, methotrexate and narrow-band ultraviolet light exposure (UVB).  Initially; they were resuscitated as burn patients. Cy A was administered on day 1 at a dose of 100 mg twice daily either in the form of syrup or Neoral capsules.  In most patients, skin lesions had healed by 6 weeks and the dose of Cy A was reduced to minimum to prevent further recurrence.  Seven patients had required 50 mg twice daily and 2 were controlled with 50 mg am and 25 mg pm.  On follow up, there was no serious relapse, liver and kidney disease.  Minor complications included; hirsutism and dark skin (n: 5) and gingival hyperplasia (n: 2). Trial to replace Cy A with Tacrolimus (Prograf) failed to maintain remission.  In conclusion; Cy A is a safe and effective treatment for GPP. Keywords: Cyclosporin A, treatment, psoriasis, pustular