We report a 2-month-old boy who presented with severe hyponatremia and hyperkalemia secondary to ureteropelvic junction(UPJ) obstruction. By prenatal ultrasonography at 19 weeks of gestation, severe hydronephrosis was found which was confirmed postnatally. Pyeloplasty was done on the 45th day of life, and fifteen days after pyeloplasty, non-bilious vomiting, decreased activity and dehydration developed. Severe hyponatremia and hyperkalemia were observed, as a result of elevated serum aldosterone and plasma renin activity. The anterior posterior pelvic diameter(APPD) and Society for Fetal Urology(SFU) grade measured showed no interval change before and after pyeloplasty. Pseudohypoaldosteronism was diagnosed, and 2M NaCl was administrated orally for 7 days. The electrolyte imbalance was corrected, and 8 weeks later, the elevated levels of aldosterone and plasma renin activity were normalized. The left hydronephrosis was improved at 5 months of age. We hereby report a transient pseudohypoaldosteronism secondary to UPJ obstruction with a review of the literature.
Aldosterone ; Dehydration ; Humans ; Hydronephrosis ; Hyperkalemia ; Hyponatremia ; Infant ; Male ; Plasma ; Pregnancy ; Pseudohypoaldosteronism* ; Renin ; Ultrasonography, Prenatal ; Vomiting

Hemolytic uremic syndrome(HUS) is characterized by acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia and the most common pathogen is Escherichia coli (E. coli) O157:H7. Ischemic colitis, which rarely occurs in children, is due to the reduced local blood flow to the intestine, tissue necrosis and secondary bacterial infection. We describe a patient who was admitted with abdominal pain, vomiting and hematochezia, and diagnosed as ischemic colitis by barium enema. This patient showed hemolytic anemia, thrombocytopenia and progressive renal failure and was subsequently diagnosed as hemolytic uremic syndrome. After hemodialysis, the patient showed improvement of symptoms and resolution of renal failure and ischemic colitis.
Abdominal Pain ; Acute Kidney Injury ; Anemia, Hemolytic ; Bacterial Infections ; Barium ; Child* ; Colitis, Ischemic* ; Enema ; Escherichia coli ; Gastrointestinal Hemorrhage ; Hemolytic-Uremic Syndrome* ; Humans ; Intestines ; Necrosis ; Renal Dialysis ; Renal Insufficiency ; Thrombocytopenia ; Vomiting

The systemic lupus erythematosus(SLE) is a systemic inflammatory disease caused by autoimmune mechanism, involving blood cells, the kidney, the central nervous system, and etc. The heart is one of the frequently involved organs but it is rare as an initial manifestation. Therefore, early suspicion and accurate diagnosis followed by aggressive immunosuppressive therapy including corticosteroid is mandatory for heart-involved patients. We experienced a case of pericardial effusion as an initial manifestation of childhood SLE, which showed immediate response to corticosteroid.
Blood Cells ; Central Nervous System ; Diagnosis ; Heart ; Humans ; Kidney ; Lupus Erythematosus, Systemic* ; Pericardial Effusion*

Orthostatic proteinuria is documented as a benign condition and the most common cause of isolated proteinuria. The etiology and pathogenesis of orthostatic proteinuria is not clear yet. Recently there were a few report that nutcracker syndrome seemed to cause orthostatic proteinuria. We experienced a case of a twelve-year-old female patient with incidently discovered orthostatic proteinuria who was suspected to have nutcracker phenomenon by doppler sonography. We confirmed this patient as nutcracker syndrome by renal venography.
Female ; Humans ; Phlebography ; Proteinuria*

Both Gitelman syndrome and Bartter syndrome are autosomal recessively inherited renal tubular disorders characterized by hypokalemic metabolic alkalosis, salt wasting and normal to low blood pressure. Gitelman syndrome is caused by mutations in the thiazide-sensitive Na- Cl cotransporter (NCCT) and distinguished from Bartter syndrome, which is associated with mutations of several genes, by the presence of hypomagnesemia and hypocalciuria. In most of the patients with Gitelman syndrome, the disease manifests with transient episodes of muscular weakness and tetany in the adult period, but, often, is asymptomatic. We report here an 11 years-old female with Gitelman syndrome who presented with aggravation of epileptic seizure. The diagnostic work-up showed typical clinical features of metabolic alkalosis, hypokalemia, hypomagnesemia and hypocalciuria. We also identified a heterozygote mutation(642CGC(Arg)>TGC(Cys)) and an abnormal splicing in the SLC12A3 gene encoding NCCT.
Adult ; Alkalosis ; Bartter Syndrome ; Child ; Epilepsy* ; Female ; Gitelman Syndrome* ; Heterozygote ; Humans ; Hypokalemia ; Hypotension ; Muscle Weakness ; Tetany

A 5-year-old girl was admitted because of an acute onset of weakness in her extremities. She had experienced a similar episode before but had recovered spontaneously. She had previously been diagnosed with distal renal tubular acidosis(RTA) at the age of 2 months. During the period of acute paralysis, her serum potassium level was 1.8 mmol/L and the muscle enzymes were markedly raised suggesting massive rhabdomyolysis. Although hypokalemia is common in renal tubular acidosis, acute paralytic presentation is uncommon and is rarely described in children. We report a case of distal RTA complicated with hypokalemic paralysis with a brief review of related literatures.
Acidosis, Renal Tubular* ; Child ; Child, Preschool ; Extremities ; Female ; Humans ; Hypokalemia ; Paralysis* ; Potassium ; Rhabdomyolysis

We report on two children with a high risk of recurrent focal segmental glomerulosclerosis (FSGS) after renal transplantation that could be effectively prevented by prophylactic administration of cyclosporine combined with preemptive plasmapheresis prior to renal transplantation.
Child ; Cyclosporine ; Glomerulosclerosis, Focal Segmental ; Humans ; Kidney Transplantation ; Plasmapheresis

Obesity-related glomerulopathy (ORG) is a secondary form of focal and segmental glomerulosclerosis (FSGS) manifesting as proteinuria and progressive renal dysfunction that results from maladaptive glomerular response to increasing adiposity. Reports of ORG progressing to end stage renal diseases in rare in the pediatric population. We report a 9-year-old boy with obesity (body mass index 35 kg/m2) who was diagnosed with ORG presenting with proteinuria. He was diagnosed with obesity-related glomerulopathy based on the laboratory, urinary, and kidney biopsy finding. In spite of treatment with angiotensin-converting enzyme (ACE) inhibitor and/or, angiotensin-receptor blocking agent, the degree or amount of proteinuria increased and renal function declined continuously. His BMI did not decrease and eventually progressed to chronic renal failure. Consequently, obese patients should be monitored for proteinuria, which may be the first manifestation of FSGS, a lesion that may be associated with serious renal sequelae.
Adiposity ; Biopsy ; Child ; Glomerulosclerosis, Focal Segmental ; Humans ; Kidney ; Kidney Failure, Chronic ; Obesity ; Proteinuria

Hyponatremia which is a very common electrolyte abnormality in hospitalized patients is defined as a plasma sodium concentration less than 135 mEq/L. Hyponatremia is generally caused by intravascular volume depletion, excessive salt loss and hypotonic fluid overload. It also can be caused by intravascular osmotic agent. Although most cases are mild and asymptomatic, acute severe hyponatremia can cause severe neurologic symptoms, such as seizures and coma. We report a rare case of severe hyponatremia induced by radiographic contrast agent.
Coma ; Humans ; Hyponatremia ; Neurologic Manifestations ; Plasma ; Seizures ; Sodium

Posterior nutcracker phenomenon (PNP) was found to be the cause of significant proteinuria in a 10-year-old female. PNP was documented by Doppler ultrasonography and abdominal 3D CT as a cause of her proteinuria. Despite treatment with ACE inhibitor for several months, her persistent and progressive proteinuria lead us to perform a left renal biopsy, which revealed no significant finding except for focal effacement of foot processes. We speculate that nutcracker phenomenon can induce not only orthostatic proteinuria but also significant proteinuria by focal effacement of foot processes.
Biopsy ; Child ; Female ; Foot ; Humans ; Proteinuria ; Ultrasonography, Doppler