Increased renal parenchymal echogenicity in ifosfamide-induced renal fanconi syndrome

Three children who presented with a Fanconi syndrome induced by the chemotherapeutic drug ifosfamide were found to have renal abnormalities on sonogram examinations. Renal echographic changes consisted in hyperechogenicity of the parenchyma with good corticomedullar differentiation. After discontinuation of the chemotherapy, the serum and urine metabolic abnormalities due to proximal tubulopathy were completely or greatly improved. Imaging studies at that time showed a complete resolution of the renal hyperechogenicity. We suggest that in patients exposed to ifosfamide, renal sonogram may be of value to monitor the tubular toxicity of this drug. In these patients, urine and serum monitoring as well as prospective echographic follow-up kidney abnormalities may lead to earlier detection of ifosfamide-induced Fanconi syndrome as well as earlier detection of disease reversibility. © 1995 Wiley-Liss, Inc.     

Pancreatic echogenicity in premature and newborn infants

Little information is available regarding pancreatic echogenicity in premature infants and neonates. We prospectively studied 65 patients (30 premature infants and 35 neonates) and compared pancreatic echogenicity to a control group of 25 infants and 35 older children. Pancreatic echogenicity was graded relative to hepatic echogenicity measured at a similar depth. In the premature infants and neonates the initial ultrasounds were hyperechoic in 71% compared to 5% in both control groups. Follow up ultrasounds were obtained in 73% of the premature infants and 17% of the neonates. The pancreatic echogenicity became isoechoic in 14 of 19 premature infants and 3 of 4 neonates in whom the initial ultrasound was hyperechoic. We conclude that the normal pancreatic echogenicity in premature infants and neonates is usually hyperechoic relative to liver. Pancreatic hyperechogenicity in premature infants and neonates is not necessarily indicative of disease.     

Renal injury in perinatal hypoxia: ultrasonography and changes in renal function

 We studied 12 hypoxaemic neonates (5 mature newborns, birth weight 2850–4200 g, gestational age 37–41 weeks; and 7 premature newborns, birth weight 770–1850 g, gestational age 27–34 weeks;) with repeated urine and blood chemistry on the 1st and 3rd days of life. Nephrosonographical examinations on the 1st, 3rd and 5–7th days of life were also performed. As controls, 12 healthy infants were examined (gestational age 36–42 weeks; birth weight 2450–4200 g). Hypoxic neonates had higher serum creatinine and blood urea nitrogen levels. Tubular markers also demonstrated renal tubular damage. Neonates in both hypoxic groups were hyperuricaemic and hyperuricosuric, and had higher urinary protein concentrations. All these infants exhibited an increased echogenicity of the renal cortex, and 11/12 showed the same finding in the medullary area. These findings disappeared within 1 week in all infants. Among the 12 healthy control infants, no cortical hyperechogenicity was found and only three of these infants displayed transient medullary renal hyperechogenicity. Conclusion Since the hypoxaemic infants demonstrated greatly increased urinary concentrations of uric acid and protein, we suggest that a temporary precipitation of these two agents may be responsible for the ultrasonographic findings. Circulatory redistribution might play a role in the phenomenon of cortical hyperechogenicity. Received: 23 March 2000 / Accepted: 14 March 2001     

Hypothesis: reduced renal mass with glomerular hyperfiltration,a cause of renal hyperechogenicity in children

A group of 10 pediatric patients had renal hyperechogenicity and reduced renal mass. The authors wish to suggest a relationship between renal hyperechogenicity and glomerular hyperfiltration according to Brenner's theory on the progressive nature of kidney disease. Reduced renal mass was related to multicystic dysplastic kidney (3 cases) nephrectomy (3 cases) and to reflux nephropathy (4 cases). The hyperechogenicity was diffuse in 6 cases with the preservation of cortico-medullary differentiation and was localized in all four cases of reflux nephropathy producing a “pseudotumoral” appearance. Hyperfiltration was confirmed by isotope studies in all 3 cases where it was performed. This condition together with secondary glomerulosclerosis could explain hyperechogenicity. The predicitive value of such patterns are still unclear; yet such findings should lead to appropriate radiolocal, functional, clinical and dietary measures. Presented at the ESPR/Meeting Montreux 1988. Selected for Publication by an International Group of the ESPR     

Immunohistochemical Diagnosis of Disseminated Intravascular Coagulation in Newborns

Disseminated intravascular coagulation (DIC) and other clotting abnormalities are common in sick newborn infants who have a variety of conditions. To document evidence of DIC at autopsy, immunoperoxidase staining of fibrin-related antigens (FRA) was used to detect intravascular microthrombi in liver, kidney, and lung from 127 newborns. Patients were selected from seven major disease groups: hyaline membrane disease/bronchopulmonary dysplasia, infection, meconium aspiration, necrotizing enterocolitis, congenital heart disease, other congenital anomalies, and extreme prematurity. Staining for FRA in intravascular microthrombi was seen in 40% of cases studied. The liver showed the highest frequency of intravascular microthombri, located predominantly in the sinusoids. Unlike the adult kidney, the newborn kidney seldom had evidence of intravascular coagulation. Extravascular staining of FRA was observed in the renal distal tubular epithelium in 48 cases, many of which also had evidence of intravascular FRA staining. No significant differences in FRA staining patterns were seen among the disease groups except for cases of extreme prematurity in which all tissues showed minimal staining. Control tissues from SIDS patients also showed minimal FRA staining. Hepatic sinusoidal staining was the only tissue finding that correlated with thrombocytopenia, a clinical indicator of DIC. Despite the use of this immunohistochemical staining method, discrepancies between the clinical and autopsy diagnosis of DIC remain.     

Ultrasound differential diagnosis of kidney tumors in childhood

Between 1979 and 1989 21 renal tumors (8 girls and 13 boys) were diagnosed and treated in the Pediatric Hospital of the University of Erlangen. Additionally, there was evidence of nephroblastomatosis in 5 children with Beckwith-Wiedemann Syndrome and hemihypertrophy. One of these infants developed a Wilms' tumor at the age of 3 1/2 years. The most frequent tumor was the Wilms' tumor, which was diagnosed in 14 children. Wilms' tumor are sonographically well delineated, round or oval tumors which often enclose small cysts (72%) but rarely calcifications (5%) and show inhomogenous liver like echogenicity. Metastasis in liver, spleen or abdomen occurred in 2 infants. The most frequent renal tumor in neonates was the mesoblastic nephroma (3 infants). All mesoblastic nephromas were well delineated round tumors with inhomogenous echo-texture and equal or increased echogenicity in comparison to the liver. They often enclosed small cysts but no metastasis or calcifications. Angiomyolipomas of the kidneys could be diagnosed in two children with tuberous sclerosis. These tumors were echogenic nodules spread all over the kidney. We found multilocular nephroblastomas with multiple irregularly delineated cysts in one child. In an other child multiple renal lymphomas simulating solid tumors with liver-like echogenicity could be found.     

The renal lesions of tuberosclerosis (cysts and angiomyolipoma) — screening with sonography and computerized tomography

The two most common sonographic abnormalities in the kidneys of 23 tuberous sclerosis (TS) patients ranging in age from newborn to 30 years are angiomyolipomas (12/23) (AML) and renal cysts (10/23). These usually both occur in the same patient with only 9 cases (39%) having sonographically normal kidneys. Of the 14 affected patients, 2 had cysts without AML and 4 others had AML without cysts. The sonographic appearance of an AML varied from a large 6 cm solid mass with little increased echogenicity (1/12) to subtle small (4 mm) extremely echogenic regions in the periphery of the kidney (11/12). The sonographic appearance of the cysts were anechoic lesions varying in size from 2 mm to 2 cm with thin uniform posterior walls and posterior enhancement. Renal lesions are found more frequently with increasing age. Sonography is the preferred screening procedure for the renal lesions of T.S.     

Increased renal medullary echogenicity in neonates

The prevalence of increased renal medullary echogenicity in healthy neonates was looked for. A group of 178 neonates underwent renal ultrasound on the first and second days of life. On the first day of life 58 % had hypereochoic material in their renal collecting system, whereas on the second day only 33% were found to have ultrasonographically demonstrable increased echogenicity in their kidneys. Urinary protein concentrations in infants with increased renal echogenicity were significantly higher than in those without increased renal echogenicity.

     

Renal pyramid echogenicity in ureteropelvic junction obstruction: correlation between altered echogenicity and differential renal function

BACKGROUND: Improvement in resolution and use of high-frequency transducers in US has enabled visualization of previously unreported changes in medullary pyramid echogenicity in children with obstructive hydronephrosis. OBJECTIVE: To determine whether these unreported changes in echogenicity and morphology of the renal pyramids in ureteropelvic junction (UPJ) obstruction correlate with differential renal function (DRF) of the kidney as determined by technetium-99m mercaptoacetyltriglycine ((99m)Tc-MAG3) scan. MATERIALS AND METHODS: Renal sonograms in 60 children with UPJ obstruction were retrospectively reviewed. Children were divided into three groups based on the echogenicity of the pyramids: (1) normal echogenicity of the pyramids, (2) increased echogenicity of the pyramids with maintained corticomedullary differentiation (CMD), and (3) loss of CMD. DRF, as determined by (99m)Tc-MAG3 scan, of the obstructed kidney of >/=45% was considered normal and of 相似文献   

Ultrasound study of congenital mesoblastic nephroma

The mesoblastic nephroma is the most common renal tumor among newborn and young infants. The tumor is benign, and metastatic growth has only been described in individual cases. Three patients suffering from histologically proven mesoblastic nephroma were examined with an ultrasonic device. Sonographically, the mesoblastic nephroma is manifest as solid and homogeneous tumor of a fine to medium coarse basic texture and of a medium echogenity which may show individual small cystic areas. The tumor cannot be delimited sharply from the healthy renal tissue although uneventful tissue which often caps the kidney is proved. A secure differential diagnosis against a Wilms' tumor is not possible if metastases cannot be detected sonographically or by means of an excretory urogram. The echopattern and the relation of the mesoblastic nephroma to the kidney in combination with an excretory urogram should, however, make the delimitation of a neuroblastoma possible.     

Ultrasonographic Findings in Periventricular Leukomalacia in the Newborn: Two Cases Associated with Early Onset Group B Streptococcal Sepsis

The ultrasonographic findings in periventricular leukomalacia (PVL) in the newborn are described, and the relationship between PVL and group B streptococcal (GBS) infection is discussed. Two newborn infants (one preterm and one term) suffered from early onset GBS sepsis with shock; they showed increased echogenicity in the periventricular regions; one of them developed cystic changes. These findings might be due to decreased perfusion of the periventricular end arterial zone. It is suggested that serial ultrasonography should be performed in neonates who suffer hypoxic-ischemic brain injury.     

Renal tubular acidosis type 4 in neonatal unilateral kidney diseases

Three neonates, two with unilateral renal vein thrombosis and one with unilateral dysplastic kidney, developed type 4 renal tubular acidosis, manifested by nonazotemic hyperkalemic metabolic acidosis with alkaline urine pH and reduced potassium excretion. Normal plasma concentrations of sodium, aldosterone, and renin activity, together with normal renal fractional excretion of sodium, supported the diagnosis of renal tubular acidosis type 4, subtype 5. Arginine HCl loading studies showed that despite their ability to bring the urine pH to less than 5.8, net acid excretion was inadequate relative to the corresponding plasma bicarbonate concentration. Treatment with oral bicarbonate resulted in sustained normalization of blood acid-base status and accelerated linear growth in the first two infants, in whom spontaneous recovery occurred by ages 8 and 15 months, respectively. At that time, the affected kidneys were extremely small with distorted collecting systems; the contralateral kidneys showed compensatory hypertrophy. In the third infant, persistent acidosis and growth failure resulted from medical noncompliance; the removal of the dysplastic kidney at 7 months of age was followed by the return to normal blood acid-base status and normalized tubular hydrogen and potassium excretion. We conclude that neonatal unilateral kidney disease can result in renal tubular subtype 5. Spontaneous recovery can be expected, presumably because of " autonephrectomy " of the affected kidney plus the compensatory hypertrophy of the contralateral kidney.     

Sonographically detectable cysts in polycystic kidney disease in newborn and young infants

Autosomal dominant (adult type) and autosomal recessive (infantile type) polycystic kidney disease are 2 distinct forms of hereditary cystic renal disease with differing pathologic and clinical features. Glomerulocystic kidney disease is probably a separate entity, whose pathologic features may closely resemble those of autosomal dominant polycystic kidney disease, especially in small infants. An example of each of these conditions in a small infant is presented, all of which had sonographically detectable cysts. Pathologic correlation was available in each case. While there are typical sonographic features of autosomal dominant and autosomal recessive polycystic kidney disease in newborn and young infants, there is no specific appearance of either condition, and glomerulocystic kidney disease can apparently resemble either one. Other investigations, particularly family studies and pathologic verification, are important in order to establish the correct diagnosis.     

Congenital disorders of glycosylation type I: a rare but new cause of hyperechoic kidneys in infants and children due to early microcystic changes

Background There are numerous causes of bilateral hyperechoic kidneys. Congenital disorders of glycosylation (CDGs) are a rapidly growing family of inherited disorders due to defects in the synthesis of the glycans of glycoproteins or other glycoconjugates. Objective To describe renal sonographic abnormalities in CDG type I in infants and children. Material and methods A retrospective study of renal US in 12 infants and children: 8 CDG-Ia (6 multivisceral forms, 2 neurological forms), 2 CDG-Ib, and 2 CDG-Ix, with detailed functional renal tests in 6. Histology of the kidneys of one 35-week fetus with CDG-Ia was available. Results Renal US was normal in the two children with the neurological form of CDG-Ia. All patients with the multivisceral form of CDG-Ia or with CDG-Ib showed increased cortical echogenicity, and/or abnormal pyramids (small +/− hyperechoic). The two patients with CDG-Ix showed predominant involvement of the medulla, with inverted corticomedullary differentiation in one. Kidney size was normal in all but two patients. The fetal kidneys exhibited diffuse microcysts arising from the distal tubules. Conclusions Hyperechoic kidneys are common in CDG-I patients, contrasting with grossly preserved renal function. The US pattern seems to differ slightly according to the type of CDG-I, and is consistent with microcystic changes of the renal parenchyma, which occur prenatally, and may be due to ciliary dysfunction secondary to altered glycosylation of tubular glycoproteins. CDG-I, which remains largely underdiagnosed at present, should be added to the causes of hyperechoic kidneys in children, especially in cases of multivisceral involvement, after ruling out other more frequent causes.     

Radiologic features of “adult type” polycystic kidney disease in the neonate

Two cases are reported of adult type polycystic renal disease (autosomal dominant) presenting in the newborn as a unilateral abdominal mass. The radiographic findings in the involved kidney simulated the ectatic tubules of infantile polycystic disease, yet histologic examination was consistent with the adult variety and both infants had other family members with adult type polycystic kidneys. These cases emphasize some of the ambiguities that exist in the definition and classification of polycystic renal disease.     

Sonographic prediction of chronic lung disease in the premature undergoing mechanical ventilation

The aims of the study are to investigate the possible role of ultrasound (US) of the chest in predicting the development of chronic lung disease (CLD) in patients with hyaline membrane disease (HMD) and to determine the optimal age for the sonographic examination. One hundred and five consecutive prematures undergoing mechanical ventilation were prospectively studied by US of the chest. The US examinations were performed at birth and at least once a week until discharge from the neonatal unit. The sonographic patterns observed behind the diaphragm and their evolutions were recorded and correlated with the clinical and radiological data at day 28, which corresponds to the currently accepted limit for determining the presence of CLD. CLD is currently defined as oxygen dependency on day 28 with radiographic abnormalities. A diffuse retrodiaphragmatic hyperechogenicity was observed in all the patients with HMD. The hyperechogenicity resolved completely in patients with an uncomplicated clinical evolution. In contrast, in patients with CLD the hyperechogenicity resolved only partially, resulting in less diffuse and less extensive hyperechogenicity. Day 18 was the earliest day where the persistence of the abnormal retrodiaphragmatic hyperechogenicity was observed in 100 % of the patients presenting CLD at day 28. At that time, 95.2 % of the patients without abnormal hyperechogenicity showed uncomplicated evolution and no CLD. US can be a useful diagnostic tool to determine the occurrence of CLD and to predict as early as day 18 the prematures at risk for the disease.     

Secondary hyperparathyroidism and bone disease in infants receiving long-term furosemide therapy

Four preterm infants receiving long-term furosemide therapy were examined for hypercalciuria, hyperparathyroidism, renal calcification, and bone demineralization. All four infants had increased urinary calcium excretion. Three infants had high serum concentrations of parathyroid hormone, and in these three infants, bone mineral content was below the mean of "osteopenic" preterm infants of comparable gestational and postnatal age. In two of these infants, there was ultrasound evidence of renal calcification. In one infant, autopsy disclosed bone changes of hyperparathyroidism, gallstones, and calcification in the heart and kidney.     

PATHOLOGY OF LETHAL FETAL GROWTH RETARDATION SYNDROME WITH AMINOACIDURIA, IRON OVERLOAD, AND LACTIC ACIDOSIS (GRACILE)

Autopsy study of 17 newborn infants with lethal autosomal recessive disease presenting as growth retardation with lactic acidosis, Fanconi aminoaciduria, and hepatic hemosiderosis is reported. The patients succumbed between day 1 and 4 months of life; 9 patients died within the first month. All patients showed severe pathologic changes of liver with cholestasis in all livers. Extensive accumulation of stainable iron of the hepatocytes was present in 9/17 autopsy tissues and in two biopsy specimens. Moderate to abundant iron storage in the Kupffer cells was seen in all liver specimens. The amount of hepatocytic iron was high in livers up to 1 month of age and decreased thereafter. The general features and liver findings of this disorder suggest the name Growth Retardation Aminoaciduria Cholestasis Iron Overload, Lactacidosis and Early Death (GRACILE, OMIM 603358). Calcified concrements were seen in the medulla of 13/16 kidney specimens. Pancreas of 13/14 patients showed interstitial fibrosis and exocrine atrophy. Various pathologic findings such as renal tubular dysgenesis, paucity of hepatic bile ducts and iron storage in the macrophages of spleen and pulmonary alveoli were observed in some cases. Previous extensive clinical genetic and laboratory investigations have revealed that the patients had a previously unrecognized genetic disease. It is inherited as an autosomal recessive trait. The gene locus is 2q 33-37. The basic defect of the disease remains unknown.     

Familial renal abnormalities associated with the oligohydramnios tetrad secondary to renal agenesis and dysgenesis

Abstract Thirty-four families of index cases with the oligohydramnios tetrad secondary to renal agenesis/dysgenesis were screened for renal abnormalities using Real Time ultrasonography. The index cases were separated into two groups. Group 1 consisted of cases of perinatally lethal renal disease and Group 2 of cases of renal dysgenesis secondary to the urethal obstruction malformation. Renal ultrasound screenings of 23 families in Group 1 demonstrated two previously unidentified cases of unilateral renal agenesis in siblings. Screening of 11 families in Group 2 revealed one sibling with a hydronephrotic kidney and one parent with an ectopic pelvic kidney.
There is a recurrence risk of 3.5–5% in families with perinatal lethal renal disease and an increased risk of silent renal anomalies in first degree family members. The recurrence risk is low in families of infants with renal dysgenesis secondary to the urethral obstruction malformation, but immediate family members are at increased risk of structural and functional urinary anomalies. Routine renal ultrasound screening of first degree relatives of infants with lethal renal agenesis and dysgenesis is recommended.     

Neonatal ABO incompatibility. Complicated by hemoglobinuria and acute renal failure

The authors present two infants with isoimmune hemolytic disease due to ABO incompatibility complicated by massive hemoglobinuria and secondary acute renal failure. This represents an incidence of 0.36% of all neonates with ABO hemolytic disease in the author's newborn population. Only two patients have been reported previously to have similar complications. Analysis of data of these four infants revealed the clinical characteristics of this complication of ABO incompatibility: 1) very low frequency; 2) early onset of hemoglobinuria (first voided urine) and of acute renal failure (first 2 days of life); 3) lack of correlation between the clinical presentation of hemolytic disease and appearance and severity of renal failure; 4) complete recovery of renal functions following intravenous fluid administration; and 5) normal renal radiologic investigations.