The omphalomesenteric duct in the embryo is a communication between the yolk sac and primitive gut. The duct closes by six to eight weeks of intrauterine life and disappears soon after. Patent omphalomesenteric duct is a congenital anomaly which occurs when there is incomplete closure of the duct during this period. The authors experienced a cases whereby a 29-day old male baby had patent... |
Lipoid pneumonia is a chronic, interstitial, proliferative inflammation resulting from aspiration of lipoid material; it occurs principally in debilitated infants. Animal oils, owing to their high fatty acid content, are the most damaging. There are no characteristic signs or symptoms; cough is most common and in severe cases there may be dyspnea. We have experenced one case of lipoid pneumonia cause by an aspiration of... |
Congenital nephrotic syndrome is an uncommon disorder, which is caused by a heterogenous group of renal diseases with variable etiology and natural history. It may be inherited, sporadic, acquird or a part of a general malformation syndrome. This disorder has no single or pathological feature for pathognomonic finding, and there is no ultimate curative treatment except renal transplantation. We experienced a case of congenital... |
A clinical and pathologic study was made on 360 cases of renal biopsies in children who were admitted to Severance Hospital, Yonsei University College of Medicine, from Jan. 1979 to Dec. 1984. 1) Among 360 cases, 250 cases were male and 110 cases were female. The sex ratio of male and female was 2.3 : 1. The distribution was from 1 6/12... |
We have experienced 2 cases of glycogen storage disease who were clinically suspected of von Gierke’s disease. Each had a typical clinical findings of marked abdominal distension due to hepatosplenomegaly and also had a typical laboratory findings. Liver biopsy disclosed morphologic details of glycogen storage disease. The authors reported a von Gierke’s disease with a brief review of related literatures. |
The superficial lymph nodes may be enlarged in consequence of biologic or non-biologic antigenic stimuli, lymphoreticular maligna ntneoplasm, metastatic tumor or infiltrative disorders such as histiocytosis-X, and biopsy of the enlarged superficial lymph node is a time-honored diagnostic, therapeutic and follow-up tool. At present it is performed with ease and wideness. But in Korea there is no comprehensive studies about... |
Purpura nephritis, one of the commonly known complications of allergic purpura has variable symptoms ranging from mild transient hematuria to severe nephrotic syndrome leading to renal failure and death. This paper reports on the treatment and course of purpura nephritis with special reference to serum immunoglobulins and immunopathology. These cases were selected among pediatric patients with purpura nephritis admitted to... |
We experienced cases with benign familial hematuria in two siblings and their mother in family. They showed asymptomatic persistent hematuria with episodic gross hematuria of 4 to 6 years duration without clinical or laboratory evidence of impaired renal function. There was a history of deafness in a brother and sister of maternal cousin. Other family members showed normal urinalysis... |
Two cases in which postoperative hepatic necrosis followed by halothane(fulthane) anesthesia are presented. Case 1 was 3 dar-old neonate who was performed corrective surgery for jejunal atresia under the halothane anesthesia. He was placed with hyperalimentation just after operation, and was relatively well. He died on postoperative 11 days. Necropsy matrial was obtained from liver. Histologic finding of liver disclosed... |
Severe segmental renal atrophy with loss of parenchymal elements in small kidney is commonly known as segmental hypoplasia. Since the original description in 1929 by Ask-Upmark, the association of hypertension with congenital renal hypoplasis has been well established and there have been several reports. But there was no reported case in Korea. The diseases commonly recognized after the age of... |
Henoch-Schonlenin purpura is one of the most common cause of purpura of children in Korea at present time, but well summarized report about the incidence in Korea is not submitted yet. Renal involvemnet in Henoch-Schonlein purpura nephritis is one of the important cause of end stage kidney disease in chidhood, accounting for about 15% of children who are on dialysis... |
We successfully transplanted kidney of mother to 13 year old boy who had been suffered from chronic renal failure for 11 months. This is the first case of renal transplantation in childhood in Korea. Patient was treated chronic renal failure with peritoneal dialysis, hemodalysis and transfusion duing 1 month before transplantation. Both kidneys revealed grade 4 vesico-ureteral renal tissue by... |
Recurrent or a second attack of acute poststreptococcal glomerulonephritis have been known to be extremely rare. Acute exacerbation in chronic glomerulonephritis and recurrence of acute poststeptococcal glomerulonephritis would be distinguishable clearly by histopathological, immuno-fluorescent and electron microscopic studies from renal biopsy material. Recently we dealt with two cases of a second attack of APSGN in a 9 year old girl... |
A non-familial type nephrotic syndrome associated with distal renal tubual acidosis, nephrocalcinosis and renal osteodystropy is a very rare disease. A 4 year old boy was treated with prednisone under the impression of minimal lesion nephrotid syndrome but the response was not excellent along with persistant microhematuria in his serial urinalysis. Percutaneous renal biopsy speciman revealed microcalcification around afferent arteriols... |
Lupus nephritis is one of complication of systemic lupus erythematous involving many organ systems. And the incidence of renal involvement is about 80%. Principal site of renal injury is glomerulus, and immune complex and complements are deposited in mesangium, basement membrane capillary loops of the glomerulus in lupus nephritis. Histopathologically, 4 distinct forms of renal injury may be seen such... |
Histiocytic medullary reticulosis was first described as a clinical and pathological entity by Scott and Robb-Smith in 1939. The clinical pictures include progressive pancytopenia, fever, wasting, lymphadenopathy, hepatosplenomegaly, jaundice and purpura. The outstanding morphological features of the disease is proliferation of histiocytes throughout the reticuloendothelial system with frequent leucoerythrophagocytosis. In spite of uniform clinical and fistologcal pictures verified by additional... |
Epidermolysis bullosa hereditaria is an uncommon skin disease which is manifest at birth or in infancy. It is characterized by blister formation occuring spontaneously or after friction or slight trauma. Recently, we experienced a case of epidermolysis bullosa hereditaria in one day old newborn infant, who was developed bullae on the neck, abdomen, both extremities and buttock. A brief review of literature was made. |