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Effect of Diet and Water Intake on Aquaporin 2 Function.

Childhood Kidney Diseases.2016;20(1):11-17. doi:10.3339/jkspn.2016.20.1.11

Appropriate control of diet and water intake is important for maintaining normal blood pressure, fluid and electrolyte homeostasis in the body. It is relatively understood that the amount of sodium and potassium intake directly affects blood pressure and regulates ion transporters; Na and K channel functions in the kidney. However, little is known about whether diet and water intake regulates Aquaporin (AQP) function. AQPs, a family of aquaporin proteins with different types being expressed in different tissues, are important for water absorption by the cell. Water reabsorption is a passive process driven by osmotic gradient and water permeability is critical for this process. In most of the nephron, however, water reabsorption is unregulated and coupled to solute reabsorption, such as AQP1 mediated water absorption in the proximal tubule. AQP2 is the only water channel founded so far that can be regulated by hormones in the kidney. AQP2 expressed in the apical membrane of the principal cells in the collecting tubule can be regulated by vasopressin (antidiuretic hormone) controlling the final volume of urine excretion. When vasopressin binds to its receptor on the collecting duct cells, it stimulates the translocation of AQP2 to the membrane, leading to increased water absorption via this AQP2 water channel. However, some studies also indicated that the AQP2 is also been regulated by vasopressin independent mechanism. This review is focused on the regulation of AQP2 by diet and the amount of water intake on salt and water homeostasis.
Absorption ; Aquaporin 2* ; Arginine Vasopressin ; Blood Pressure ; Diet* ; Drinking* ; Homeostasis ; Humans ; Ion Transport ; Kidney ; Membranes ; Nephrons ; Osmolar Concentration ; Permeability ; Potassium ; Sodium ; Vasopressins ; Water*

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Clinical Manifestation Patterns and Trends in Poststreptococcal Glomerulonephritis.

Kee Hyuck KIM

Childhood Kidney Diseases.2016;20(1):6-10. doi:10.3339/jkspn.2016.20.1.6

Poststreptococcal glomerulonephritis (PSGN) is one of the most recognized diseases in pediatric nephrology. Typical clinical features include rapid onset of gross hematuria, edema, and hypertension, and cases are typically preceded by an episode of group A β-hemolytic streptococcus pharyngitis or pyoderma. The most common presenting symptoms of PSGN are the classic triad of glomerulonephritis: gross hematuria, edema, and hypertension . However, patients with PSGN sometimes present with unusual or atypical clinical symptoms that often lead to delayed diagnosis or misdiagnosis of the disease and increased morbidity. Additionally, the epidemiology of postinfectious glomerulonephritis (PIGN), including PSGN, has changed over the past few decades. This paper reviews atypical clinical manifestations of PSGN and discusses the changing demographics of PIGN with a focus on PSGN.
Delayed Diagnosis ; Demography ; Diagnostic Errors ; Edema ; Epidemiology ; Glomerulonephritis* ; Hematuria ; Humans ; Hypertension ; Nephrology ; Pharyngitis ; Pyoderma ; Streptococcus

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Antibiotics and Probiotics Prophylaxis for Recurrent Urinary Tract Infection in Children.

Jung Won LEE

Childhood Kidney Diseases.2016;20(1):1-5. doi:10.3339/jkspn.2016.20.1.1

Since many years, continuous low dose antibiotic prophylaxis (CAP) has been used for children at a risk for recurrent urinary tract infection (UTI), especially those with vesicoureteral reflux (VUR). The incidence of recurrent UTI has been shown to be higher in children with VUR with bladder and bowel dysfunction (BBD) than in those with VUR without BBD. Therefore, CAP has been recommended for children with BBD and VUR because of the increased risk of UTI. However, the use of CAP has become highly controversial because of bacterial resistance developed due to antibiotic over-usage. The preventive effects of probiotics have been proved in various adult urogenital infections, and the antimicrobial activities of lactobacilli against uropathogens have been demonstrated in previous in vitro studies. However, a critical assessment of their efficacy in children with UTI is lacking. The importance of the use of urogenital probiotics is that it is a natural approach that replenishes the depleted normal flora to create a better environment to fight off uropathogens. Probiotics have a great potential, particularly today with the increasing threat of antibiotic-resistant microorganisms.
Adult ; Anti-Bacterial Agents* ; Antibiotic Prophylaxis ; Child* ; Humans ; Incidence ; Probiotics* ; Urinary Bladder ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux

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Two Cases of Toxic Epidermal Necrolysis Associated with Deflazacort Therapy in Nephrotic Syndrome: Successfully Treated with Cyclosporine A.

Myung Hee LIM ; Hee Jung BAE ; Sun Young PARK ; Sae Yoon KIM ; Yong Hoon PARK

Childhood Kidney Diseases.2016;20(2):97-100. doi:10.3339/jkspn.2016.20.2.97

Toxic epidermal necrolysis (TEN) is a rare, acute, serious, and potentially fatal skin disease, in which cell death causes the epidermis to separate from the dermis. It is thought to be a hypersensitivity complex that affects the skin and mucous membranes, and is caused by certain medications, infections, genetic factors, underlying immunologic disease, or more rarely, cancers. We report two cases of TEN associated with deflazacort (DFZ), a derivative of prednisolone, used in the first episode of nephrotic syndrome (NS). The skin eruption appeared on the 4(th) and 5(th) weeks after DFZ administration, while NS was in remission. The widespread lesions were managed by intensive supportive treatment, discontinuation of DFZ, and oral administration of cyclosporine. Both patients showed a rapid improvement in symptoms of TEN without any complications or relapse of NS.
Administration, Oral ; Cell Death ; Cyclosporine* ; Dermis ; Epidermis ; Humans ; Hypersensitivity ; Immune System Diseases ; Mucous Membrane ; Nephrotic Syndrome* ; Prednisolone ; Recurrence ; Skin ; Skin Diseases ; Stevens-Johnson Syndrome*

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A Case of Streptococcus pneumoniae associated Hemolytic Uremic Syndrome with DIC.

Seong Heon KIM ; Su Young KIM

Childhood Kidney Diseases.2015;19(1):48-52. doi:10.3339/chikd.2015.19.1.48

Streptococcus pneumoniae associated hemolytic uremic syndrome (SpHUS) is one of the causes of atypical hemolytic uremic syndrome, and increasingly reported. They are more severe and leave more long-term sequelae than more prevalent, typical hemolytic uremic syndrome. But it is not so easy to diagnose SpHUS for several reasons (below), and there was no diagnostic criteria of consensus. A 18 month-old-girl with sudden onset of oliguria and generalized edema was admitted through the emergency room. She had pneumonia with pleural effusion and laboratory findings of HUS, DIC, and positive direct Coombs' test. As DIC or SpHUS was suspected, we started to treat her with broad spectrum antibiotics, transfusion of washed RBC and replacement of antithrombin III. On the 3rd day, due to severe hyperkalemia and metabolic acidosis, continuous renal replacement therapy (CRRT) was started. She showed gradual improvement in 4 days on CRRT and discharged in 16 days of hospital care. At the follow up to one year, she has maintained normal renal function without proteinuria and hypertension. We report this case with review of articles including recently suggested diagnostic criteria of SpHUS.
Acidosis ; Anti-Bacterial Agents ; Antithrombin III ; Child ; Consensus ; Coombs Test ; Dacarbazine* ; Disseminated Intravascular Coagulation ; Edema ; Emergency Service, Hospital ; Follow-Up Studies ; Hemolytic-Uremic Syndrome* ; Humans ; Hyperkalemia ; Hypertension ; Oliguria ; Pleural Effusion ; Pneumonia ; Proteinuria ; Renal Replacement Therapy ; Streptococcus pneumoniae*

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A Case of Streptococcus pneumoniae associated Hemolytic Uremic Syndrome with DIC.

Seong Heon KIM ; Su Young KIM

Childhood Kidney Diseases.2015;19(1):48-52. doi:10.3339/chikd.2015.19.1.48

Streptococcus pneumoniae associated hemolytic uremic syndrome (SpHUS) is one of the causes of atypical hemolytic uremic syndrome, and increasingly reported. They are more severe and leave more long-term sequelae than more prevalent, typical hemolytic uremic syndrome. But it is not so easy to diagnose SpHUS for several reasons (below), and there was no diagnostic criteria of consensus. A 18 month-old-girl with sudden onset of oliguria and generalized edema was admitted through the emergency room. She had pneumonia with pleural effusion and laboratory findings of HUS, DIC, and positive direct Coombs' test. As DIC or SpHUS was suspected, we started to treat her with broad spectrum antibiotics, transfusion of washed RBC and replacement of antithrombin III. On the 3rd day, due to severe hyperkalemia and metabolic acidosis, continuous renal replacement therapy (CRRT) was started. She showed gradual improvement in 4 days on CRRT and discharged in 16 days of hospital care. At the follow up to one year, she has maintained normal renal function without proteinuria and hypertension. We report this case with review of articles including recently suggested diagnostic criteria of SpHUS.
Acidosis ; Anti-Bacterial Agents ; Antithrombin III ; Child ; Consensus ; Coombs Test ; Dacarbazine* ; Disseminated Intravascular Coagulation ; Edema ; Emergency Service, Hospital ; Follow-Up Studies ; Hemolytic-Uremic Syndrome* ; Humans ; Hyperkalemia ; Hypertension ; Oliguria ; Pleural Effusion ; Pneumonia ; Proteinuria ; Renal Replacement Therapy ; Streptococcus pneumoniae*

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A 22-month-old Boy with Acute Glomerulonephritis Coexistent with Hemolytic Anemia and Idiopathic Thrombocytopenia.

Hye Won PARK ; Bo Seon SEO ; Su Jin JUNG ; Jun Ho LEE

Childhood Kidney Diseases.2015;19(1):43-47. doi:10.3339/chikd.2015.19.1.43

Hemolytic anemia and thrombocytopenia are rare clinical manifestations of acute glomerulonephritis. Initially, in all such cases, a diagnosis of hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, and amyloidosis should be ruled out. The presence of hemolytic anemia and thrombocytopenia is rare, but possible, in a case of acute poststreptococcal glomerulonephritis, and may result in delayed diagnosis or misdiagnosis. Correct and timely diagnosis would ensure adequate treatment in such patients. We report of a 22-month-old boy with acute glomerulonephritis coexistent with hemolytic anemia and idiopathic thrombocytopenia.
Amyloidosis ; Anemia, Hemolytic* ; Delayed Diagnosis ; Diagnosis ; Diagnostic Errors ; Glomerulonephritis* ; Hemolytic-Uremic Syndrome ; Humans ; Infant* ; Lupus Erythematosus, Systemic ; Male ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia*

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A 22-month-old Boy with Acute Glomerulonephritis Coexistent with Hemolytic Anemia and Idiopathic Thrombocytopenia.

Hye Won PARK ; Bo Seon SEO ; Su Jin JUNG ; Jun Ho LEE

Childhood Kidney Diseases.2015;19(1):43-47. doi:10.3339/chikd.2015.19.1.43

Hemolytic anemia and thrombocytopenia are rare clinical manifestations of acute glomerulonephritis. Initially, in all such cases, a diagnosis of hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, and amyloidosis should be ruled out. The presence of hemolytic anemia and thrombocytopenia is rare, but possible, in a case of acute poststreptococcal glomerulonephritis, and may result in delayed diagnosis or misdiagnosis. Correct and timely diagnosis would ensure adequate treatment in such patients. We report of a 22-month-old boy with acute glomerulonephritis coexistent with hemolytic anemia and idiopathic thrombocytopenia.
Amyloidosis ; Anemia, Hemolytic* ; Delayed Diagnosis ; Diagnosis ; Diagnostic Errors ; Glomerulonephritis* ; Hemolytic-Uremic Syndrome ; Humans ; Infant* ; Lupus Erythematosus, Systemic ; Male ; Purpura, Thrombotic Thrombocytopenic ; Thrombocytopenia*

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Acute Epstein-Barr Virus Hepatitis in a 32 Month Old Female Manifesting as Henoch-Schonlein Purpura.

Hee Jin KIM ; Su Jin JUNG ; Jun Ho LEE

Childhood Kidney Diseases.2015;19(1):39-42. doi:10.3339/chikd.2015.19.1.39

Henoch-Schonlein purpura can result from exposure to an antigen after infection with several types of organisms. However, Henoch-Schonlein purpura caused by a primary Epstein-Barr virus infection has been rarely reported. Here, we report the case of a 32-month-old female patient who presented with Henoch-Schonlein purpura. Based on abnormal liver function test results and positive results for Epstein-Barr virus infection markers, a diagnosis of Epstein-Barr virus hepatitis manifesting as Henoch-Schonlein purpura was made. Treatment with methylprednisolone and hydration improved the symptoms, and a switch to oral steroids was effective in completely alleviating the purpura. No recurrence was noted and no liver function abnormalities were detected during the follow up period.
Arthritis ; Child, Preschool ; Diagnosis ; Female ; Follow-Up Studies ; Hepatitis* ; Herpesvirus 4, Human* ; Humans ; Liver ; Liver Function Tests ; Methylprednisolone ; Purpura ; Purpura, Schoenlein-Henoch* ; Recurrence ; Steroids

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Acute Epstein-Barr Virus Hepatitis in a 32 Month Old Female Manifesting as Henoch-Schonlein Purpura.

Hee Jin KIM ; Su Jin JUNG ; Jun Ho LEE

Childhood Kidney Diseases.2015;19(1):39-42. doi:10.3339/chikd.2015.19.1.39

Henoch-Schonlein purpura can result from exposure to an antigen after infection with several types of organisms. However, Henoch-Schonlein purpura caused by a primary Epstein-Barr virus infection has been rarely reported. Here, we report the case of a 32-month-old female patient who presented with Henoch-Schonlein purpura. Based on abnormal liver function test results and positive results for Epstein-Barr virus infection markers, a diagnosis of Epstein-Barr virus hepatitis manifesting as Henoch-Schonlein purpura was made. Treatment with methylprednisolone and hydration improved the symptoms, and a switch to oral steroids was effective in completely alleviating the purpura. No recurrence was noted and no liver function abnormalities were detected during the follow up period.
Arthritis ; Child, Preschool ; Diagnosis ; Female ; Follow-Up Studies ; Hepatitis* ; Herpesvirus 4, Human* ; Humans ; Liver ; Liver Function Tests ; Methylprednisolone ; Purpura ; Purpura, Schoenlein-Henoch* ; Recurrence ; Steroids

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