We experienced a 4-month-old boy presenting with fever and painful scrotal swelling. Diagnostic work-up showed the presence of urinary tract infection and concurrent acute epididymitis. On the voiding cystourethrography, vesicoureteral reflux and urinary reflux through the ejaculatory duct and the seminal vesicle were detected without obvious urethral obstruction. In general, urinary reflux into the seminal vesicle can take place with obstructive lesions of the urethra and may cause epididymitis in infants. We report a case of urinary reflux without urethral obstruction with a brief review of related literatures.
Ejaculatory Ducts ; Epididymitis* ; Fever ; Humans ; Infant ; Male ; Seminal Vesicles* ; Urethra ; Urethral Obstruction ; Urinary Tract Infections ; Vesico-Ureteral Reflux

Scrotal swelling may be acute or chronic, painful or painless. Common causes of scrotal swelling in newborns are hydrocele, inguinal hernia, testicular torsion, testicular tumor, scrotal hematoma, meconium peritonitis and epididymitis. Abrupt onset of a painful scrotal swelling necessitates prompt evaluation. Testicular torsion and incarcerated inguinal hernia require urgent surgical management. We report a case of scrotal swelling caused by a tunica vaginalis abscess in a 20-days-old boy. He was admitted to the hospital due to fever, irritability and left scrotal swelling with local heat, tenderness and redness. Exploratory laparotomy was performed to rule out testicular torsion. On the operative field, congestive erythematous inflammation on the left tunica vaginalis was noted and it was filled with a pus like discharge. The cultured organism was Streptococcus agalactiae(group B). He recovered quickly after debridement and administration of empirical antibiotics.
Abscess* ; Anti-Bacterial Agents ; Debridement ; Epididymitis ; Estrogens, Conjugated (USP) ; Fever ; Hematoma ; Hernia, Inguinal ; Hot Temperature ; Humans ; Infant, Newborn ; Inflammation ; Laparotomy ; Male ; Meconium ; Peritonitis ; Spermatic Cord Torsion ; Streptococcus ; Suppuration

Acute hyperammonemia is a medical emergency in the newborn. Efficient, prompt removal of serum ammonia is essential in preventing irreversible brain damage in order to prevent the profound central nervous system dysfunction due to hyperammonia. We report a case of 2.3 kg, 5-day old girl with methylmalonic acidemia who presented with severe hyperammonemia and was successfully treated with continuous venovenous hemodiafiltration(CVVHDF). CVVHDF is an effective and safe method of ammonia removal in the newborn.
Ammonia ; Brain ; Central Nervous System ; Emergencies ; Female ; Hemodiafiltration* ; Humans ; Hyperammonemia* ; Infant, Newborn

The nephrotic syndrome is characterized by generalized edema, hypoproteinemia(<2 g/dL), proteinuria(>40 mg/m2/hr), and hypercholesterolemia(>200 mg/dL). It is reported that hypoalbuminemia, which is one of the four diagnostic criteria of the nephrotic syndrome, is associated with gallbladder wall thickening. An explanation for the thickened wall in hypoalbuminemic states is the accumulation of fluid in the subserosal layer of the gallbladder wall which contains the most areolar tissue. This report describes a patient who was initially diagnosed with the nephrotic syndrome at the age of 4 and subsequently developed acute acalculous cholecystitis at the age of 5.8 with an albumin level of 1.3 g/dL. The patient responded to fluid therapy, nasogastric suction, and broad spectrum antibiotics.
Acalculous Cholecystitis* ; Anti-Bacterial Agents ; Edema ; Fluid Therapy ; Gallbladder ; Humans ; Hypoalbuminemia ; Nephrotic Syndrome* ; Suction

An 8-month-old male infant presented with persistent, gross, orange-colored crystals in his urine. His physical and neurological development was normal. Laboratory study showed hyperuricemia, hyperuricosuria and urate crystaluria. He was determined to have partial hypoxanthine-guanine phosphoribosyl transferase(HPRT) deficiency. The molecular genetic analysis revealed a missense mutation in the patient's HPRT gene. By sequencing the patient's cDNA, we identified an A-to-G transition at nucleotide 239, resulting in the replacement of Aspartate with Glycine at amino acid 80 in the HPRT. To our knowledge, this mutation has not previously been reported. Our patient is now being placed on allopurinol therapy, and has had no problem since. Partial HPRT deficiency has been known to cause recurrent acute renal failure without the phenotypic features of Lesch-Nyhan syndrome. Therefore, we think that early diagnosis and treatment are very crucial in preventing acute renal failure.
Acute Kidney Injury ; Allopurinol ; Aspartic Acid ; DNA, Complementary ; Early Diagnosis ; Glycine ; Humans ; Hyperuricemia ; Hypoxanthine Phosphoribosyltransferase* ; Infant ; Lesch-Nyhan Syndrome ; Male ; Molecular Biology ; Mutation, Missense* ; Uric Acid

The hemolytic uremic syndrome(HUS), a heterogenous group of disorders characterized by micorangiopathic hemolytic anemia, thrombocytopenia, and azotemia, is the most frequent cause of acute renal failure in children. The association of the HUS with E. coli O157:H7 has been well-described, but the other intestinal infection have been relatively less reported to date. We report a 18-month-old boy presenting with typical clinical characteristics of HUS associated with amoebic dysentery with a brief review of literatures.
Acute Kidney Injury ; Anemia, Hemolytic ; Azotemia ; Child ; Dysentery, Amebic* ; Hemolytic-Uremic Syndrome* ; Humans ; Infant ; Male ; Thrombocytopenia

Diarrhea-associated hemolytic uremic syndrome is rarely accompanied by complications requiring surgical intervention. We report a case of the diarrhea-associated hemolytic uremic sydrome which developed simultaneously with an acute appendicitis.
Appendicitis* ; Hemolytic-Uremic Syndrome*

A girl aged 21 months with Henoch-Schonlein purpura(HSP) developed heavy proteinuria with hematuria 8 days after the appearance of purpuric rash, swelling and tenderness of both ankle joints. Her clinical and laboratory features demonstrated nephrotic and nephritic syndrome. The percutaneous renal biopsy revealed diffuse mesangial proliferative glomerulonephritis. Unlike usual HSP nephritis, immunoglobulin A deposition was not detected in the mesangium or the capillary of the glomeruli. Instead, numerous subepithelial electron-dense deposits("humps") mimicking acute poststreptococcal glomerulonephritis were found.
Ankle Joint ; Biopsy ; Capillaries ; Exanthema ; Female ; Glomerulonephritis* ; Hematuria ; Humans ; Immunoglobulin A ; Nephritis* ; Proteinuria

Alport syndrome is the most common type of hereditary nephritis, and acute poststreptococcal glomerulonephritis(APSGN) is a common disease in children. We experienced the clinical and pathologic findings of Alport syndrome and APSGN in brothers of one family. Both patients presented with heavy gross hematuria and proteinuria. ASO titer was elevated in both cases, and the C3 level was reduced in one of the cases. In renal pathology, both showed characteristics of Alport syndrome as well as the glomerular changes of APSGN with hump-like subepithelial deposits by electron microscopy. These clinical observation indicated that the patients had APSGN superimposed on Alport syndrome, and that the episode of APSGN might exacerbate the clinical course of Alport syndrome.
Child ; Glomerulonephritis* ; Hematuria ; Humans ; Microscopy, Electron ; Nephritis, Hereditary* ; Pathology ; Proteinuria ; Siblings*

PURPOSE: Neonatal hydronephrosis has been detected with increasing frequency with the widespread use of prenatal ultrasonography, but the consensus about its postnatal management has not yet been reached, especially about surgical intervention. We attempted to determine the guideline of follow-up study and surgical intervention of hydronephrosis by analyzing clinical outcomes of neonates with hydronephrosis. MATERIALS AND METHODS: Between 1994 and 2000, 128 hydronephrotic kidneys were postnatally confirmed. Cases associated with other urologic anomalies were excluded and 90 unilateral hydronephrotic kidneys with a minimum follow-up of 12 months were enrolled in this study. We classified the patients into 6 groups according to the anterior posterior pelvic diameter(APPD) at initial ultrasonography(USG) within 1 month after birth. Renal USG and Tc99m-mercaptoacetyl triglycerine(MAG3) scan were done according to a set protocol, and pyeloplasty was performed when indicated according to our protocol. RESULTS: Most cases whose APPD were below 10 mm improved or resolved. Only few cases with APPD above 20 mm showed spontaneous improvement and most(88%) had undergone operation. Those with initial APPD within 10-19 mm showed variable outcomes. When the risk factors for irreversible renal functional deterioration were analyzed, the age at pyeloplasty and pre-operative functional deficit were significant. CONCLUSION: We concluded that in infants with initial APPD below 10 mm, consideration of surgery is not needed, and in those with initial APPD above 20 mm, early operation is recommended. Our set protocol based on initial USG is useful, but the cut-off value of relative renal function(RRF) for operation might be increased to 40% to improve post operative RRF.
Consensus ; Follow-Up Studies ; Humans ; Hydronephrosis* ; Infant ; Infant, Newborn ; Kidney ; Parturition ; Risk Factors ; Ultrasonography ; Ultrasonography, Prenatal