Varicose bronchiectasis and bronchopulmonary dysplasia.

As a result of improved therapies and technology, including the use of surfactant replacement, the features of bronchopulmonary dysplasia (BPD) have changed, and a "new BPD" is emerging that is substantially different from the classical form of the disease. As the pathogenesis of BPD is evolving, so are other features of the disorder, including radiologic features. We describe varicose bronchiectasis with a bulbous appearance in a 6-year-old child with a complicated course including BPD during the neonatal period.

Adrenal hemorrhage in a newborn.

Sometimes in the course of care in a neonatal intensive care unit, there may be a rush to intervene in cases where limited intervention is actually the correct course. One such example is that of neonatal adrenal hemorrhage. We present the case of a male term neonate with shock, metabolic acidosis, distended abdomen, and falling hematocrit. His prenatal and delivery histories were uneventful except for a nuchal cord. Apgar scores were 9 and 9. Because of his dramatic presentation, certain members of the medical team suggested immediate surgical intervention. However, a calm and careful evaluation revealed the true diagnosis and course of action. Ultrasound of the abdomen showed a mass between the liver and kidney, but the origin was difficult to identify. A computed tomography scan supported the diagnosis of right adrenal hemorrhage. His serum cortisol level was normal. The patient was managed conservatively and discharged home after a 1-week stay in the hospital. Subsequent abdominal ultrasound showed resolving adrenal hemorrhage with minimal calcification. A review of the pertinent literature is presented. Physicians should remember adrenal hemorrhage when evaluating a newborn infant with shock, acidosis, abdominal distention, and falling hematocrit and that conservative management is usually indicated.

Rabson-Mendenhall syndrome: medullary sponge kidney, a new component.

Rabson-Mendenhall syndrome is a rare genetic disorder characterized by severe insulin resistance, extreme hyperinsulinemia, postprandial hyperglycemia, growth retardation, and dysmorphisms. Enlargement of the kidneys and nephrocalcinosis have been described previously. We report a 10-year-old boy who presented with gross hematuria, unilateral hydronephrosis, and the initial diagnosis of bilateral extensive medullary nephrocalcinosis. Medullary sponge kidney (MSK) was included in the differential diagnosis given the ultrasound findings. Further evaluation by intravenous pyelogram confirmed the suspected bilateral MSK. Given the patient's history of hydronephrosis due to an obstructing renal stone and MSK, urine calcium excretion was assessed and found to be markedly increased at 9.5 mg/kg per day. To our knowledge, this is the first report of Rabson-Mendenhall syndrome and an association with MSK. We recommend evaluation for nephrocalcinosis, MSK, and hypercalciuria in all children diagnosed with Rabson-Mendenhall syndrome.

An unusual case of severe therapy-resistant hypertension in a newborn.

Hypertension can occur in up to 2% of neonates, and the spectrum of potential causes is broad. Prompt and thorough evaluation with a main focus on kidney disease is key for appropriate therapy. Here we describe a 2-day-old neonate with feeding intolerance and elevated blood pressure readings. Within 24 hours after birth, the infant's blood pressure increased significantly, with sustained mean arterial pressure >85. Renal Doppler ultrasound showed decreased venous blood flow in the right kidney with an abnormal Doppler wave form suggestive of unilateral renal venous thrombosis. Despite aggressive antihypertensive therapy including hydralazine and enalaprilat, hypertension remained sustained. On day-of-life 4, the infant developed clinical signs of hypertensive encephalopathy and significant cardiac dysfunction. A renal angiography showed complete, likely thrombotic occlusion of the right renal artery. Renal MAG3 imaging showed minimal function of the affected kidney, and a nephrectomy secondary to medically uncontrollable hypertension and worsening cardiac dysfunction was performed. The child is developing normally in all aspects on follow-up evaluations at 6 months and 1 year of age. Reevaluation of the working diagnosis in neonates with hypertension can be necessary to optimize the outcome. The overall prognosis can be excellent even in newborns with profound cardiac and neurologic involvement.

Abdominal manifestations of cystic fibrosis in older children and adults.

OBJECTIVE: Cystic fibrosis (CF) is a disorder of exocrine gland function of which the gene mutation has been existing for thousands of years. With recent medical advances, neonates presently affected have a life expectancy of 40 years. The common gastrointestinal presentations of CF patients, including pancreatic, hepatobiliary, and bowel manifestations, are thus important to recognize. CONCLUSION: Gastrointestinal manifestations of CF are varied yet common and thus increasingly important to recognize. The sonographic, CT, and MRI abdominal findings in older children and adults with CF are presented.

Successful treatment of inflammatory myofibroblastic tumor with malignant transformation by surgical resection and chemotherapy.

Inflammatory myofibroblastic tumor (IMT) is a tumor composed of myofibroblasts and a mixed inflammatory infiltrate that rarely undergoes malignant transformation. The authors present the case of a 7-year-old boy with an abdominal mass diagnosed as IMT with malignant transformation. The tumor recurred twice after attempts at resection and was initially treated with vincristine and etoposide. After a third recurrence and incomplete resection, he was treated with cisplatin, Adriamycin, and methotrexate. He is disease-free after 2 years, representing successful combined surgery and chemotherapy in the treatment of malignant IMT. The use of chemotherapy for aggressive myofibroblastic tumors is reviewed.