PubMed Health⌕ Search

Biomedical subjects

R G Azizkhan

Publications and source records attributed to R G Azizkhan.

88 records · Page 5Linked to original sources

Congenital muscular torticollis: a spectrum of pathology.

Congenital muscular torticollis (CMT) is a disorder characterized by a shortening of at least one of the cervical muscles and tilting of the head to the opposite side. The most commonly affected muscle is the sternocleidomastoid (SCM) muscle. An unusual case is presented here: a CMT patient with involvement of the trapezius muscle in addition to the SCM muscle who has recently been treated at North Carolina Memorial Hospital. This patient still has a slight residual deformity despite two surgical procedures, physical therapy, and splinting. A review of 41 CMT patients who presented to this institution from 1952 to the present demonstrates that the disorder is extremely variable in terms of severity of deformity and response to various types of therapy. The condition will resolve spontaneously in the majority of patients by the age of 1 year, and if the condition has not resolved by then, it is unlikely to do so without surgery. We therefore believe that surgery should be postponed until after 1 year of age. Most of the patients requiring surgery are effectively treated by division of the involved muscle in at least one area with or without lengthening of the SCM muscle. Splinting and physical therapy are recommended postoperatively. A few patients such as the unusual one with involvement of the trapezius muscle resist our most aggressive therapeutic efforts.

Female↗

Successful treatment of Candida-infected caval thrombosis in critically ill infants by low-dose streptokinase infusion.

Chronic central venous catheters are an important component in the management of chronically ill infants and children. Sepsis and thrombosis are common complications of these catheters. When the combination of Candida sepsis and caval thrombosis occurs, the prognosis is very poor. Lysis of the thrombus is critical to effective therapy and allows preservation of vascular access. We report the successful treatment of four critically ill infants with Candida-infected caval thrombosis treated with low-dose infusion of streptokinase combined with standard antimicrobial therapy. All four infants survived, and in all cases thrombolysis was complete and Candida sepsis resolved. Each of the infants required continued central venous access, which was made possible by resolution of the caval thrombosis. There were no hemorrhagic or other complications of the therapy.

Anti-Infective Agents↗

Altered intestinal chloride transport in cystic fibrosis.

Sodium ion and chloride transport was studied in vitro in small intestinal and colonic tissue from patients with cystic fibrosis (CF) and from non-CF control subjects matched as to age and sex. Normal histological appearance and substantial response to mucosal glucose (5 mM, ileum) or mucosal amiloride (10(-5) M, colon) indicated normal tissue viability in both control and CF tissues. Electroneutral NaCl absorption was demonstrated in the small intestine of control subjects and CF patients. Small intestinal and colonic tissues of control subjects responded to four secretagogues (theophylline, 5 mM; prostaglandin E2, 10(-6) M; calcium ionophore (A23187), 10(-5) M; bethanechol, 5 x 10(-5) M), with electrogenic chloride secretion. The tissues of CF patients, however, did not respond to any of the test secretagogues. These studies demonstrate that an abnormality in chloride transport is present in the small intestinal and colonic epithelia of CF patients. Unlike airway epithelia, which secrete chloride in response to Ca ionophore, the intestinal epithelia of CF patients do not respond to either cAMP- or Ca-mediated secretagogues. This abnormality in intestinal electrolyte transport may play a role in the pathogenesis of meconium impactions in CF patients.

Adult↗

Malignant rectal carcinoid: a sequential multidisciplinary approach for successful treatment of hepatic metastases.

A malignant rectal carcinoid metastatic to the liver presents a formidable challenge. The uniformly fatal course in patients with liver metastases (average survival of 2 years) justifies an aggressive approach. Although in an occasional patient the tumor is resectable, most are managed by chemotherapy, which generally is of limited effectiveness. Although certain drug combinations such as 5-fluorouracil and streptozotocin have achieved higher response rates, these responses are often brief (3 to 4 months) and poorly documented. Surgical hepatic dearterialization and, more recently, hepatic intraarterial embolization are quite effective in inducing regression in a variety of hepatic neoplasms, including metastatic carcinoids, but these are usually temporary. We have been timely instructed on the value of combined therapy by a patient who is a long-term survivor of a metastatic carcinoid to the liver. She is the only survivor among a group of 14 patients who had an average survival of 17 months. This patient emphasizes the benefit of combined hepatic dearterialization and chemotherapy in patients with metastatic carcinoid to the liver. She initially had intrahepatic infusion of 5-fluorouracil and streptozotocin through the surgically placed hepatic artery and portal vein catheters, but this was curtailed after 2 months because of catheter sepsis. She then had four sequential selective hepatic intraarterial embolizations with Gelfoam over a 16 month period. She also received systemic therapy with 5-fluorouracil and streptozotocin during a major portion of this period (10 months). Significant tumor regression was documented radiologically. Although she had another trial with intrahepatic chemotherapy infusion using surgically placed catheters, this was again discontinued because of catheter sepsis, and systemic chemotherapy was resumed. Currently, the patient is asymptomatic, has excellent performance status, and continues to show objective tumor regression on a program of systemic therapy with fluorodeoxyuridine and doxorubicin. She has survived more than 7 years with liver metastases from a rectal carcinoid.

Antineoplastic Combined Chemotherapy Protocols↗

Life-threatening airway obstruction as a complication to the management of mediastinal masses in children.

Life-threatening airway obstruction from large mediastinal masses in children poses a difficult diagnostic and therapeutic dilemma, requiring the close coordination of a pediatric surgeon, anesthesiologist, radiologist, and oncologist. To focus on this problem, the anesthetic and surgical management of 50 consecutive children with mediastinal masses treated between 1978 and 1984 were reviewed. Thirty children presented with respiratory symptoms; nine had life-threatening respiratory compromise with dyspnea, orthopnea, and stridor. Thirteen of these symptomatic children had marked compression of the trachea and/or mainstem bronchi on radiographic studies. The tracheal cross-sectional area which was measured by computed tomography was decreased by 35% to 93% of the normal tracheal dimensions in these children. Nonresectable malignant neoplasms including lymphoma, Hodgkin's disease, rhabdomyosarcoma, and neuroblastoma were the eventual diagnoses in 10 of these patients. The other 3 patients were less than 4 years old and had benign lesions. General anesthesia was judged to be prohibitively risky in 5 of 13 patients. The diagnosis was established by node or needle biopsy under local anesthesia, and general anesthesia was deferred until the compromised airway was alleviated by radiation and chemotherapy. General anesthesia with endotracheal intubation was administered to 8 patients, 5 of whom developed total airway obstruction. Using a variety of maneuvers, ventilation was reestablished in all 5 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗

Surgical complications of neuroblastoma resection.

Locally advanced neuroblastomas in infants and very young children often require intricate dissection to separate the tumors from the anatomic structures that they have enmeshed. The rationale for these procedures is based mainly on the premise that near-total resection is almost as effective as total extirpation, given favorable circumstances of age and stage. The principal reason for not undertaking or aborting such resections has been to avoid the fallacy of intentionally sacrificing vital structures, causing serious disability, in circumstances in which cure is either impossible or equally likely to accrue from a lesser procedure. This report describes five surgical mishaps, two of which resulted in postoperative deaths. The potential for these complications was greatest during resection of locally advanced tumors in small babies. We suspect that this hazard is more prevalent than its scarcity in the literature would suggest and that potential for unintended injury should be a prominent factor in the decision to proceed or desist with resection of a large neuroblastoma.

Child, Preschool↗

Pleuroperitoneal shunts in the management of neonatal chylothorax.

Pleuroperitoneal shunts have been placed in five ventilator-dependent newborns with persistent chylothorax. The etiology of the chylothorax appeared to be secondary to superior vena caval obstruction in three patients and was idiopathic in the remaining two. Despite traditional therapies these infants were on a progressively deteriorating clinical course. Hakim-Cordis low-pressure ventricular-peritoneal shunt catheter systems were used in each infant. Ultrasonography was used to follow the regression of pleural effusions and to determine the need for shunt compression. Shunt patency was confirmed with radionuclide studies. Four of five infants had a complete resolution of their chylothorax and pulmonary insufficiency. Three of these infants were extubated within 28 days following the placement of the shunt. Nutritional and metabolic stability was rapidly achieved. The shunts were removed several weeks later without recurrence of the chylothorax. A fifth infant failed to improve after the placement of the pleuroperitoneal shunt and died of progressive pulmonary insufficiency. The placement of pleuroperitoneal shunts in infants with refractory chylothorax is safe, technically easy to perform, and is associated with few complications.

Catheterization↗

Inhibition of tumor growth, vascularization, and collagenolysis in the rabbit cornea by medroxyprogesterone.

Medroxyprogesterone, dexamethasone, or cortisone, locally applied in sustained release polymer to rabbit V2 carcinoma implanted in the rabbit cornea, blocked neovascularization and three-dimensional growth of the tumor. These hormones similarly prevented the vascular proliferative response to implants in the rabbit cornea of mouse B-16 melanoma and also the response to implants of polymer containing tumor extract with angiogenesis activity. The inhibitory responses were accompanied by considerable reduction in collagenolytic activity released into culture medium by explants of the two tumors and of the corneal region containing angiogenic hepatoma extract. Morphologic studies revealed extensive three-dimensional disruption of the compact laminated collagenous structure of the cornea by untreated V2 carcinoma. In the presence of hormone the tumor grew slowly as a noninvasive two-dimensional plaque limited to the narrow region of the insertion pocket in the cornea, with no obvious disturbance of structure elsewhere. Cortisone was much les effective than medroxyprogesterone or dexamethasone. Testosterone and estradiol had no effect on the three measured properties. The data suggest that local hormonal interference with neovascularization, collagenase production, and tumor growth can prevent neoplastic invasion and destruction of a dense collagenous connective tissue.

Animals↗

Mast cell heparin stimulates migration of capillary endothelial cells in vitro.

Migration of capillary endothelial cells is an important component of angiogenesis in vivo. Increased numbers of mast cells have been associated with several types of angiogenesis. We have used a quantitative assay in vitro to demonstrate that mast cells release a factor that significantly increases bovine capillary endothelial cell migration. The factor is present in medium conditioned by mast cells as well as lysates of mast cells. The stimulatory effect of mast cells on migration is specific for capillary endothelial cells. Furthermore, mast cells have no mitogenic activity for capillary endothelial cells. Of all the secretory products of mast cells tested, only heparin stimulated capillary endothelial cell migration in vitro. Heparin preparations from a variety of sources stimulated capillary endothelial cell migration to the same degree but did not stimulate migration of several other cell types. The migration activity of heparin and mast cell conditioned medium was blocked by specific antagonists of heparin (protamine and heparinase), but not by chondroitinase ABC. The migration activity of mast cell conditioned medium was resistant to heat (100 degrees C) and incubation with proteolytic enzymes. These results suggest that the role of mast cells in angiogenesis may be to enhance migration of the endothelial cells of growing capillaries.

Animals↗

Achalasia in childhood: a 20-year experience.

The incidence of achalasia in children is lower than that in adults, but the disturbances in growth and development, and the severity of pulmonary symptoms are more profound. We have treated 20 children with the disease over the past 20 yr. All children were first treated with dilatation of the cardioesophageal junction either with filoform and followers, or in older children, pneumatic dilatation under fluoroscopic control. Five children, all older than 9 yr of age and all female, had prolonged relief of symptoms following two dilatations and never required a surgical procedure. Fifteen children responded unsatisfactorily to repeated dilatation and 12 of these accepted a surgical procedure. A modifier Heller procedure was performed in 11 children and one patient was treated with a transthoracic cardioplasty. The operations resulted in dramatic relief of symptoms, satisfactory weight gain, and the disappearance of pulmonary symptoms in every case. We recommend that all children under 9 yr of age should have a modified Heller procedure as primary therapy. In children older than 9 yr of age, dilatation is an appropriate initial therapy but should not be repeated unless significant improvement results.

Adolescent↗

Superficial lymph node infarction.

A case of spontaneous superficial lymph node infarction points up the rarity of the lesion as well as the necessity to include its consideration in the differential diagnosis of groin masses.

Adult↗

Current biologic and therapeutic implications in the surgery of neuroblastoma.

Advances in the understanding of neuroblastoma biology have helped distinguish those patients who require maximum therapy from those who do not. However, management of children with advanced disease remains a significant problem. New more intensive multiagent chemotherapy, ablative chemoradiotherapy with bone marrow transplantation, radiolabelled monoclonal antibody technology and a host of biologic response modifiers are currently being intensively studied. Nevertheless, surgery remains crucial for diagnosis, for providing adequate tissue for biological and histopathologic staging, for primary and secondary resections as well as restaging after therapy. Major surgical questions remain to be addressed in cooperative group studies.

Antineoplastic Combined Chemotherapy Protocols↗

Metastasizing chordoma in early childhood: report of a case at 1.5 T.

We present here the first report of an extraskeletal metastasizing presacral chordoma found in a child. MRI of this large lesion showed heterogeneous signal intensity on both the SE 550/30 and on SE 2440/100 images. MRI demonstrated this mass to be separate from the sacrum and provided nonionizing cross-sectional imaging prior to attempted surgical resection.

Child, Preschool↗