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There are few data regarding adolescent-onset nephrotic syndrome (NS) and no guidelines for biopsy criteria and treatment protocol. This study was conducted to analyze the clinical spectrum of adolescent-onset NS and evaluate possible biopsy criteria in these children. A prospective analysis was carried out on all patients with idiopathic NS (fulfilling the ISKDC criteria) with onset between 1 and 18 years of age. They were evaluated clinically, followed by biochemical investigations and kidney biopsy. These characteristics of patients with onset between 1 and 12 years (group A) were compared with the same parameters in patients with onset between 12 and 18 years of age (group B) referred to our hospital over the same period. Among all clinical parameters, microhematuria was significantly more prevalent in adolescents (P<0.001). Kidney biopsy was performed in 88% of adolescent patients. Focal segmental glomerulosclerosis (FSGS) was the most-common histopathology in group B (46.3%) compared with minimal change disease (MCD) in group A (42.9%). Group B had a significantly higher frequency of membranoproliferative glomerulonephritis (MPGN) (P<0.005) and a significantly lower frequency of MCD (P<0.001). The biochemical parameters at the onset were similar. On comparing microhematuria, hypertension, and renal insufficiency at presentation, we observed that two or more of these features were present in all patients with MPGN and only in 19.6% of adolescents with MCD, mesangioproliferative glomerulonephritis, and FSGS. The frequency of steroid resistance was significantly higher in group B (P<0.001). In conclusion, adolescent-onset NS differs from the childhood variety in having a significantly higher frequency of hematuria, steroid resistance, and evidence of non-MCD, especially MPGN, on histopathology. Kidney biopsy can be restricted to those adolescents who have at least two abnormal clinical/biochemical features or are steroid non-responders.

Heavy metal toxicity is often caused by occupational exposure. Chronic cadmium toxicity is a significant health concern among workers engaged in zinc smelting, battery production and silver jewellery industries, particularly in developing countries. We report the case of a 48-year-old man who presented with severe osteoporosis, impaired renal function and acquired Fanconi syndrome. He was finally diagnosed with chronic cadmium toxicity resulting from long-term occupational exposure. Cadmium has a long biological half-life and there is no effective treatment for people who are exposed to it. Therefore, an early diagnosis and prevention of further exposure are important. KeywordsDisease, bone disorders, toxicology Accepted: 12th February 2013 Case report A 48-year-old Indian non-smoker male patient presented with progressively worsening skeletal pain for the previous five years. X-rays of the lumbosacral spine and hip showed markedly reduced bone density with prominent trabecular markings. Dual-energy Xray absorptiometry (DEXA) confirmed the presence of severe osteoporosis (T score <À2.5 in the lumbar vertebrae and left femoral neck). He also had impaired renal function, with serum creatinine concentration of 250 mmol/L (reference interval 53-106 mmol/L) and serum urea concentration of 10 mmol/L (reference interval 2.5-7.5 mmol/L). He was referred to our hospital for further evaluation of his impaired renal function. The patient was not diabetic or hypertensive nor did he have any history of corticosteroid therapy. Full blood count showed mild normochromic normocytic anaemia: haemoglobin 10.9 g/dL (reference interval 12.0-15.0 g/dL). Total and differential white blood cell counts were within reference limits. Biochemical investigations showed hypophosphataemia (0.6 mmol/L, reference interval 0.8-1.5 mmol/L), hypouricaemia (100 mmol/L, reference interval 208-416 mmol/L) and increased alkaline phosphatase (412 U/L, reference Routine urinalysis showed trace albumin and 2þ glucose on urine dipstick but plasma glucose was normal. Fractional excretion of uric acid (18%, reference interval 5-11%) and phosphate (31%, reference interval 5-20%) were elevated. Urine protein:creatinine ratio was 310 mg/mmol (reference interval <22 mg/mmol), and urine albumin:creatinine ratio was 5.3 mg/mmol (reference interval <2.5 mg/mmol of creatinine). Urine and serum protein electrophoreses were unremarkable. Subsequently, the patient was re-evaluated. On further questioning, the patient gave an occupational history of working in a silver jewellery industry for the past 15 years.

Incomplete dRTA affects height in children. This observation needs validation in longitudinal studies.

Acute renal failure (ARF) associated with liver disease is a commonly encountered clinical problem of varied etiology and high mortality. We have prospectively analyzed patients with liver disease and ARF to determine the etiology, clinical spectrum, prognosis and factors affecting the outcome. Other than hepatorenal syndrome patients, out of 221 cases, 66 developed ARF secondary to various liver disease like cirrhosis (n = 29, mortality 8, risk factors-older age p < 0.01, grade III/IV encephalopathy p < 0.05), fulminant hepatic failure (n = 25, mortality 15, risk factor-prolonged prothrombin time p < 0.01), and obstructive jaundice (n = 12, mortality 7, risk factor-sepsis p < 0.01). In these three groups the factors leading to ARF were volume depletion (24), gastrointestinal bleed (28), sepsis (34), drugs (27) [aminoglycosides (9) and NSAID (18)] along with hyperbilirubinemia. Various types of ARF with contemporaneous liver injury were malaria (n = 37, mortality 15, risk factors-higher bilirubin p < 0.001, higher creatinine p < 0.05, anuria p < 0.05 and dialysis dependency p < 0.05), sepsis (n = 36, mortality 22, risk factors-age p < 0.001, higher bilirubin p < 0.01, oliguria p < 0.05), hypovolemia with ischemic hepatic injury (n = 14, mortality 5, risk factors-higher creatinine p < 0.05 and SGPT p < 0.01), acute pancreatitis (n = 12, mortality 4, risk factors-higher bilirubin p < 0.001, higher SGPT p < 0.01, dialysis dependency p < 0.05), rifampicin toxicity (n = 10, no mortality), paroxysmal nocturnal hemoglobinuria (n = 3, no mortality), CuSO4 poisoning (n = 3 mortality 2), post abortal (n = 11, mortality 6, risk factors higher creatinine p < 0.05 and SGPT p < 0.01), ARF following delivery including HELLP syndrome (n = 12, mortality 4, risk factors-higher bilirubin p < 0.01 and SGPT p < 0.01), and of uncertain etiology (n= 14 mortality 4). 133 patients (60.2%), required hemodialysis hemodialfiltration or peritoneal dialysis. ARF associated with liver disease is having high mortality (42.5%). Avoidance of dehydration, hypotension, nephrotoxic drugs and sepsis, with promote dialytic support are necessary to reduce mortality and morbidity.

Hereditary renal hypouricaemia (HRH) is a genetic disorder commonly associated with exercise-induced acute kidney injury (AKI). We report the case of a 19-year-old man who was admitted to hospital with exercise-induced AKI and who was subsequently shown to have HRH. We believe this to be the first description of a case of HRH co-presenting with rhabdomyolysis. Case reportA 19-year-old man was admitted to hospital with a history of dysuria, recurrent vomiting, severe muscle pain and weakness. Two days previously he had undertaken a session of rigorous exercise for the first time in the local gymnasium. His urine was slightly dark in colour. He had no history of illegal drug abuse, alcohol consumption, trauma, recent illness or any previous episode of a similar nature. There was no known family history of muscular or metabolic disease.On admission his vital signs were as follows: heart rate 96/min, temperature 37.18C, respiratory rate 20/min and blood pressure 130/80 mmHg. On examination there was muscular tenderness particularly in the calf muscles. There was no evidence of oedema or skin rash in the extremities. Power was 5/5 in both lower and upper limbs. The remaining physical examination was unremarkable. Laboratory investigations showed serum creatinine 736 mmol/L (reference interval 53-106 mmol/L), urea 16.7 mmol/L (reference interval 2.5 -7.5 mmol/L), sodium 138 mmol/L (reference interval 135-145 mmol/L), potassium 6.2 mmol/L (reference interval 3.5 -5.0 mmol/L), uric acid 243 mmol/L (reference interval 208 -416 mmol/L) and creatinine kinase 10,127 U/L (reference interval 10-190 U/ L). Serum C-reactive protein concentration and full blood count were within normal reference intervals. Further investigation revealed mildly increased serum alanine and aspartate aminotransferase activities. Routine urinalysis revealed protein of 1þ with 10 -12 red blood cells per high power field. Abdominal ultrasound scan was normal. A diagnosis of exercise-induced rhabdomyolysis leading to acute kidney injury (AKI) was made and the patient was treated accordingly. Following regular haemodialysis, kidney function improved gradually and he was discharged within a week of hospitalization. At this time his serum creatinine concentration was 172 mmol/L, urea 6.0 mmol/L, sodium 140 mmol/L and potassium 3.9 mmol/L.Two months later on a follow-up visit he had symptoms of myalgia with arthalgia. Laboratory investigations showed: serum creatinine 106 mmol/L, urea 5.7 mmol/L, sodium 137 mmol/L, potassium 3.9 mmol/L and uric acid 17 mmol/L. The uric acid result was rechecked and confirmed as correct on a fresh sample. He was not receiving any uric acid lowering drugs.To investigate the cause of severe hypouricaemia a fractional excretion of uric acid (FE UA ¼ urinary uric acid  serum creatinine  100/serum uric acid  urine creatinine) and 24 h urinary uric acid excretion were measured. The FE UA was 151% (reference interval 5-11%) and 24 h urinary uric acid was 2.59 mmol/24 h (reference interval 1.48-4.43 mmol/24 h). Urinary p...

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