Atypical mycobacterial cervical lymphadenitis with extensive local spread: a surgical disease.

Atypical (nontuberculous) mycobacterium is an uncommon cause of cervical lymphadenitis in immunocompetent children. Rarely, this disease progresses to locoregional destruction of the deep structures of the neck including salivary glands. Recent reports suggest medical monotherapy as an effective treatment of this disease. We report three cases of children who experienced progression to locoregional disease while on appropriate antibiotics. We suggest that the patient population to benefit from medical monotherapy has yet to be adequately defined. In our experience, surgical therapy is the only effective treatment for locoregional disease.

Kyphomelic dysplasia: a rare form of semilethal skeletal dysplasia.

Kyphomelic dysplasia is a rare form of generalized skeletal dysplasia with about 15 cases described so far in the literature. We present the clinical, radiological, and pathological findings of an antenatally detected female fetus affected with this disorder. The differential diagnoses of prenatal and perinatal semilethal skeletal dysplasias and salient features of documented cases of kyphomelic dysplasia are presented.

A spectrum of segmental multicystic renal dysplasia.

BACKGROUND: Multicystic renal dysplasia (MCDK) is a common anomaly well described in the literature, but less well described when involving only a portion of a kidney. OBJECTIVE: To present the imaging spectrum, natural history and associated anomalies of six kidneys with segmental MCDK. MATERIALS AND METHODS: Five children with segmental MCDK (one with bilateral segmental MCDK) referred to our hospital between 1989 and 1996 were reviewed. All had at least one ultrasound examination. Four had a voiding cystogram and three had renal scintigraphy. RESULTS: Four children had antenatal diagnosis of cystic renal abnormality. In two, with obvious duplex kidneys and associated ureteroceles, the diagnosis of upper moiety MCDK was obvious either antenatally or immediately postnatally. In the other three there were diagnostic difficulties. One patient had bilateral widespread cysts obscuring the functioning renal portions. Another presented in utero with a large ureterocele and a cystic upper pole that had involuted by birth. The fifth had a nephrectomy at 3 years for a multiloculated cystic mass. Varying degrees of involution occurred in the five kidneys seen early. Reflux was demonstrated into the ipsilateral functioning lower moiety and midpole. CONCLUSION: In these children as in other studies, the commonest presentation of segmental MCDK is in the upper pole of a duplex kidney associated with a ureterocele at the end of the atretic ureter. Atypical segmental MCDK may present a diagnostic dilemma and should be included in the differential diagnosis of multiloculated cystic masses and cystic kidneys.

Nephrocalcinosis in rabbits--correlation of ultrasound, computed tomography, pathology and renal function.

Objective. The purpose of this study was to induce nephrocalcinosis (NC) in rabbits with phosphate, vitamin D, oxalate and furosemide, to determine the effect on renal function and to correlate detection on ultrasound (US) and computed tomography (CT) with pathology. Materials and methods. Seventy-five immature New Zealand white rabbits were divided into five groups of 15. In each group, 5 animals were controls and 10 were given oral phosphate, furosemide, vitamin D or oxalate. Unilateral nephrectomy was performed at 3-6 weeks, and 5 rabbits of each test group were withdrawn from the substance. Weekly US was performed as well as US, CT and measurement of serum creatinine at the time of nephrectomy and prior to planned demise. Results. A total of 140 kidneys in 75 rabbits had both pathological and US correlation, with CT correlation in 126. Forty rabbits developed nephrocalcinosis with early (post nephrectomy at 3-6 weeks) or late (post demise at 12-20 weeks) pathological correlation obtained in 53 kidneys. Forty-one of these kidneys were from test animals: 23 developed NC early, 18 late. Twelve controls developed NC: 4 early, 8 late. Comparing US and CT to pathology, the sensitivity was 96% for US, 64% for CT. Specificity was 85% for US and 96% for CT. In 109 kidneys, information on serum creatinine level was available to correlate with pathology. The mean creatinine level was 138 mmol/l for those with NC and 118 mmol/l for those without NC (P<0.001). Conclusion. In this study, the presence of NC was significantly associated with increasing serum creatinine. Overall, US was more sensitive and CT was more specific in the detection of NC.

Pattern and permanence of phosphate-induced nephrocalcinosis in rabbits.

Objective. The purpose of this study was to observe the pattern and permanence of phosphate-induced nephrocalcinosis (NC) in rabbits. Materials and methods. Thirty immature New Zealand white rabbits were divided into 10 controls and 20 test subjects who were given oral phosphate. Unilateral nephrectomy was performed at 3-6 weeks, and 10 of the test group were withdrawn from the substance. All surviving rabbits were terminated at 6-14 weeks post nephrectomy. Weekly US was performed, as well as US, CT and serum creatinine evaluation at the time of nephrectomy and prior to planned demise. Results. Of the 20 rabbits that received oral phosphate, 16 had NC by 3 weeks and the remainder by 6 weeks. The severity was proportional to intake. NC occurred in the cortex in 69%, the corticomedullary junction (CMJ) in 81% and the medulla in 27% of cases. Only 5 of 10 rabbits that returned to oral water post nephrectomy were suitable for follow-up on pathology. NC improved in 3 of these animals. Conclusion. NC in rabbits occurs maximally at the CMJ but also frequently in the cortex. NC was not permanent or static but improved in 3 of 5 rabbits when phosphate was withdrawn.

Spectrum of phenotypic variability in Niemann-Pick type C disease: A cause of delayed diagnosis.

BACKGROUND: Niemann-Pick type C (NP-C) disease exhibits marked heterogeneity in its phenotype. This can pose diagnostic dilemmas and even delayed recognition of this condition. OBJECTIVE: To highlight the phenotypic variations and distinctive pathological and biochemical findings in this disorder. DESIGN: Descriptive case studies. SETTING: Tertiary care children's hospital and clinic. POPULATION STUDIED: Three cases of NP-C disease where diagnosis was delayed. RESULTS: In each of the three cases the clinical presentation was varied, one as neonatal hepatitis, the second with megaloblastic anemia, chronic hepatitis and short stature, and the third with neonatal hepatitis and chronic respiratory failure. Definitive diagnosis was established in each case by demonstration of defective cholesterol esterification in skin fibroblasts. CONCLUSIONS: In the clinical setting of neonatal hepatitis, hepatosplenomegaly and undiagnosed neurological symptoms, NP-C disease should be considered in the differential diagnosis. Electron microscopic examination of skin biopsy is an effective screening test, although the definitive diagnosis should be made by the cholesterol esterification assay and filipin staining.

Radiological features of a symptomatic splenic hamartoma.

Symptomatic splenic hamartomas are rare in the pediatric age group, with only four previous reports in the literature. Splenic hamartoma has been reported as a solid homogeneous mass without calcification on CT and ultrasound (US), and only one previous report of the findings on MRI has been published. We report a case of a large symptomatic splenic hamartoma in a 14-year-old girl who presented with splenomegaly, pancytopenia and growth retardation. A solid mass with multiple punctate foci resembling calcifications was seen on US. The mass was heterogeneous and better demarcated on enhanced CT. Radiocolloid scintigraphy demonstrated uptake within the lesion, but less than that of normal spleen. The mass was isointense relative to normal splenic tissue on T1-weighted MRI (0.5 T) and of increased intensity with T2 weighting. At splenectomy, a red pulp hamartoma was identified, which contained nodules of hyalinization and necrosis thought to account for the punctate foci seen on US.

Neck pain and headache in an afebrile 15-year-old.

Nuchal rigidity and headache are important signs of bacterial meningitis, although, in the absence of fever other etiologies would be considered. An evaluation of a 15-year-old boy with the above features, focal neurological deficits, and two cerebral contrast enhancing ring lesions is discussed.

Heart-lung protection from ischemic injury during 8 hour hypothermic preservation.

Optimal techniques of heart-lung preservation are yet to be defined. The aim of this study was to develop, in a canine model, a method of heart-lung preservation which would permit distant procurement of the organs. The animals were divided into 2 groups. In the experimental group (N = 6) the method of preservation consisted of cold cardioplegic arrest of the heart with St. Thomas' Hospital solution containing superoxide dismutase, catalase and deferoxamine, followed by cold pulmonary artery flush with modified Euro-Collins solution to which prostaglandin E1, superoxide dismutase, catalase, deferoxamine and Dextran 40 were added. Following harvesting, the heart-lung block was stored for 8 hours in cold (4 degrees C) Euro-Collins solution containing superoxide dismutase, catalase, deferoxamine, lactobionic acid, raffinose, mannitol, Dextran 40, magnesium sulfate, insulin and penicillin. In the control group (n = 6), the heart-lung block underwent the same treatment as the experimental group except that lactobionic acid, raffinose and insulin were omitted from the storage solution, and that oxygen radical scavengers were excluded from the cardioplegic, pneumoplegic and storage solutions. Histologic and electron microscopic examinations of heart-lung specimens taken before and after 8 hours cold storage of the organs suggested that our preservation technique may be effective in preventing ischemia-induced injury.

Nonrenal cystic masses in neonates and children.

With the increasing use of ultrasonography (US) in children, abdominal cystic masses, many of them nonrenal, are being detected more frequently both before and after birth. Between 1986 and 1991, 20 predominantly cystic abdominal or pelvic masses of nonrenal origin were detected by US in patients less than 10 years of age. Ten of the 20 patients were neonates; in 5 of these the masses were detected antenatally. Pathological correlation was obtained for 16 of the patients. Six of the cysts were ovarian; two represented cystic teratoma, two exhibited torsion and hemorrhage, one was a giant simple cyst and one was a simple cyst with herniation. Six of the lesions were duplication cysts; two were found in the antral region, of which one was ulcerated and had perforated. In the remaining group of eight cysts, three were found in newborns; one represented colonic atresia and another was a choledochal cyst. Several of these nonrenal cystic masses were detected incidentally or in patients with nonspecific abdominal pain. US correctly predicted the location of the cyst and the diagnosis in most patients. In many of the patients the masses were complicated by other problems that required diagnosis and treatment.

Toxicity of 3'-deamino-3'-(3-cyano-4-morpholinyl) doxorubicin and doxorubicin in nude mice bearing human tumour xenografts.

The toxicity of the intensely potent anthracycline 3'deamino-3'-(3-cyano-4-morpholinyl)doxorubicin (MRA-CN) has been evaluated in nude mice bearing human colonic cancer xenografts. In addition to dose related toxicity manifested as weight loss and effects on the haematology profile, we obtained evidence of cardiotoxicity with MRA-CN, which has not been reported previously. Even a single dose of 0.012 mg kg-1 could induce significant myocardial changes as seen by electron microscopy. Our results suggest that nude mice bearing human tumour xenografts may offer a very sensitive model for the evaluation of anthracycline induced cardiomyopathy. In view of the potential of MRA-CN in cancer treatment, these results need to be confirmed and extended.

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.

Chlamydia trachomatis pneumonitis: a case study and literature review.

Chlamydia trachomatis was cultured from the lung and eye of an 11-day-old infant who had rapidly deteriorating respiratory symptoms. Chlamydia trachomatis inclusion bodies were identified on thick Epon sections and by electron microscopy. We discuss the unusual presentation and review the published histopathological lung findings.