Multicystic dysplasia in one-half of a horseshoe kidney with megaureter and lower ureteric atresia

During evaluation of chronic abdominal pain, a 9-year-old male was found to have a horseshoe kidney (HSK) with multicystic dysplasia (MCD) of the left-sided component. Attached to the MCD was a very large, tortuous ureter occupying almost the whole left side of the abdomen. This ureter on dissection was found to end blindly adjacent to the bladder. MCD of one-half of a HSK is an unusual lesion. Its association with a large megaureter with juxtavesical atresia is a unique event. In HSKs, controversy exists about the need to remove a small dysplastic segment. If this segment is associated with a large ureter, as in our case, removal is mandatory in order to avoid pain and infection.     

Multicystic dysplasia in the upper component of the complete duplex kidney

Four cases of neonatal multicystic dysplasia involving the upper part of a complete duplex kidney are presented. This malformation, suspected on antenatal ultrasound examination, was confirmed by postnatal IVP and ultrasound. This emphasizes the need to perform an IVP when the diagnosis of multicystic kidney is suggested by ultrasound. Conservative surgery may then be achieved, as in three of the four cases.     

Spontaneous remission of a diffuse brainstem lesion in a neonate

We describe here the spontaneous remission of a diffuse brainstem lesion found in an infant at the age of 7 weeks. Clinical and MRI characteristics strongly suggested a diffuse pontine glioma. Repeated MRI studies showed a continuous decrease in the size of the lesion, which was no longer visible by the age of 27 months. Spontaneous remission of diffuse pontine glioma is extremely rare; to our knowledge there are reports of only three similar cases.     

Near-total pancreatectomy for hyperinsulinism. Spontaneous remission of resultant diabetes

Persistent hyperinsulinism in the newborn may warrant surgical intervention to prevent neurologic sequelae. Subtotal pancreatectomy may not be adequate, necessitating near-total pancreatectomy with subsequent development of diabetes mellitus. We report an infant with hyperinsulinemic hypoglycemia who underwent near-total pancreatectomy. The postoperative period was characterized by insulin-dependency and extreme insulin sensitivity. Clinical follow-up and C-peptide determinations showed a return of insulin secretory capacity permitting the discontinuation of insulin therapy after five months. This experience reaffirms the potential for a favorable outcome after near-total pancreatectomy in the newborn period for severe hyperinsulinism.     

Spontaneous remission of congenital leukemia: a case for conservative treatment

A newborn infant with spontaneous remission of congenital leukemia cutis is described and a literature review of this uncommon phenomenon is provided. In view of the unusual and unpredictable behavior of this disease, chemotherapy should be withheld unless there is evidence of an 11q23 translocation or progressive disease. Otherwise, overall survival does not appear to be affected by adopting a conservative approach. Because of occasional late relapses, long-term follow-up is recommended. The biologic basis underlying spontaneous remission of congenital leukemia is unknown; therefore, molecular or molecular cytogenetic analysis of DNA obtained from a skin biopsy is recommended.     

Spontaneous remission of cryptosporidiosis in a child with acute lymphocytic leukemia

Cryptosporidiosis has been documented in pediatric patients with various forms of congenital and acquired immunodeficiency. This is a report of cryptosporidiosis in a child with acute lymphocytic leukemia. Cryptosporidiosis, an uncommon cause of diarrhea, may produce severe diarrhea in children with acute lymphocytic leukemia.     

Fibrous dysplasia of long bones with remission. Apropos of 3 cases]

Three cases of fibrous dysplasia of bone, affecting the long bones of the leg are described. This condition is a clearly defined radiological and clinical entity. The tibial lesion is reminiscent of a congenital pseudarthrosis but the involvement of the fibula is a useful diagnostic clue. The most important feature of the condition is that it heals spontaneously.     

Spontaneous remission of chronic hepatitis C in children

The clinical course of 48 children with chronic hepatitis C (33 boys, 15 girls; mean age: 12.2 years) was monitored for more than 3 years to clarify its natural course. All patients were positive for the second-generation antibody to hepatitis C virus (anti-HCV) and for serum hepatitis C virus (HCV) RNA. All but one patient had a history of blood transfusion. Serum levels of alanine aminotransferase (ALT) had been abnormal for more than 1.5 years. Spontaneous remission defined as a biochemical remission lasting more than 1 year in association with the disappearance of serum HCV RNA, occurred in 4 (8.3%), however, in 25%, HCV RNA was still detectable in the liver even after its disappearance from serum. In this patient, the level of antibody to HCV core antigen (anti-HCV core) did not decrease significantly and serum HCV RNA eventually reappeared. The serum titre of HCV RNA in the 4 children with spontaneous remission was lower than in the remaining 44 children. Spontaneous remission may occur in children with chronic hepatitis C in whom the serum titre of HCV RNA is low and serum level of anti-HCV core decreases significantly. Assessment of the intrahepatic HCV RNA is necessary to confirm complete remission. Conclusion A low serum titre of HCV RNA and a significant decrease in the serum titre of anti-HCV core were associated with spontaneous remission in children with chronic hepatitis C. Intrahepatic HCV RNA assessment is necessary to confirm complete remission. Received: 9 April 1996 and in revised form: 8 April 1997 / Accepted: 15 April 1997     

Multicystic kidney dysplasia: a prospective study on the natural history of the affected and the contralateral kidney

In a 6-year period, 41 young infants with multicystic kidney dysplasia were seen in our department. In 30 cases, the diagnosis had already been suspected by prenatal ultrasonography. A prospective protocol was proposed to the parents which comprised ultrasound evaluation every 3 months until the age of 24 months and renal function assessment at the age of 18 months. In 33 patients, the study was completed as scheduled. At the start of the study, the maximal diameter of the multicystic kidney was above the mean length of normal kidneys in all cases where precise measurement was possible. Within 24 months, 7 of the dysplastic kidneys disappeared, 20 regressed in size, 1 remained unchanged and only 5 increased in size. Between the age of 0 to 3 months, renal length of the contralateral kidney was within the normal range in 19 infants and above +2SD in 14 cases. At the age of 18 to 24 months, renal length was, with few exceptions, between 0 and +4SD. Inulin clearance was normal in all 33 individuals with a median value of 112 ml/min per 1.73 m². Conclusion As a rule, multicystic kidneys shrink in the first 2 years of life. In most cases the contralateral kidney maintains a normal renal function as a consequence of progressive compensatory hypertrophy. Received: 19 November 1997 / Accepted in revised form: 31 January 1998     

Pediatric renal artery dysplasia: a morphologic study

Renal arteries from 12 pediatric patients who had stenosis due to renal artery dysplasia (9 surgical resections and 3 autopsy cases) were examined by light microscopy. The patients ranged in age from 15 months to 14 years; the male/female ratio was one. Bilateral renal artery disease was clinically diagnosed in 8 of the 12 patients; 6 of the 8 also had aortic anomalies. All of the lesions were designated as forms of dysplasia, a departure from previous reports in which dysplasia was subclassified as fibroplasia and hyperplasia. The most common lesion was medial fibrous dysplasia (6), followed by perimedial elastic dysplasia (4); intimal fibrous dysplasia (1) and medial muscular dysplasia (1) were the least common. Medial fibrous dysplasia was present as often with bilateral as with unilateral renal artery stenosis, while perimedial elastic dysplasia was seen exclusively in patients with bilateral renal artery disease. Three patients who came to autopsy had extrarenal vascular involvement by a similar dysplastic lesion. The three patients who had neurofibromatosis showed no distinctive histologic type of renal artery dysplasia.     

Ultrasonography of cystic renal dysplasia

Cystic renal dysplasia, classified by Osathanondh and Potter as renal cystic disease types II and IV, is probably the result of urinary tract obstruction in fetal life. Since these kidneys function poorly, sonography plays a more significant role in their diagnosis than excretory urography. Familiarity with the sonographic spectrum of cystic renal dysplasia will help the physician to make an accurate diagnosis, and may obviate the need for unnecessary invasive procedures.     

Spontaneous remission of acute lymphoblastic leukemia with mediastinal mass

Spontaneous remission/regression of cancer is defined as partial or complete disappearance of malignant disease temporarily or permanently in the absence of medical treatment. This event is named as spontaneous regression for solid tumors and spontaneous remission for leukemia. The authors report the case of a girl aged 4 years and 3 months, who presented with mediastinal mass and leukemic findings in the bone marrow both of which reappeared after spontaneous regression and remission, respectively.