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G Kale

Publications and source records attributed to G Kale.

72 records · Page 4Linked to original sources
PubMedDate not supplied

Clinical and histopathological study of merosin-deficient and merosin-positive congenital muscular dystrophy.

The clinical features of merosin-positive congenital muscular dystrophy (CMD) and merosin-deficient CMD are well known, with those of merosin-deficient CMD being more severe. Whether the severity of histopathological findings correlates with these clinical features remains unanswered. In this study, the clinical and histopathological findings of 39 merosin-deficient and 37 merosin-positive CMD patients were compared. Merosin-deficient CMD patients were found to be younger, with earlier onset of symptoms, age of diagnosis, and a more severe clinical state (reflecting maximum motor capacity and contractures). On histopathological evaluation, endomysial fibrosis, perimysial fibrosis, and histopathological state (reflecting fibrosis, adiposis, necrosis, and variation in fiber size) were more severe in merosin-deficient CMD. There was a correlation between clinical and histopathological states only in merosin-deficient CMD.

Adolescent↗
PubMedDate not supplied

Pyogenic granuloma with multiple dissemination in a burn lesion.

Pyogenic granuloma is a common vascular lesion in childhood. The occurrence of pyogenic granulomas after various kinds of trauma to the skin is quite common; however, multiple lesions secondary to a burn are very rare. For this reason, an 18-month-old girl with multiple pyogenic granulomas following a second-degree burn is reported.

Burns↗
PubMedDate not supplied

Pleuropulmonary blastoma in a child presenting with pneumothorax.

Pleuropulmonary blastoma is an uncommon primary malignant tumor of the lung. Surgery is the most important part of the treatment. Despite the use of chemotherapy with or without radiotherapy, the prognosis is poor. Here we report on the case of a 2.5 year-old boy presenting with pneumothorax who turned out to have a right hemithoracic mass. The patient underwent surgical resection of the mass. Pathologic diagnosis was type II pleuropulmonary blastoma. Postoperative thoracic CT showed nodular residual densities so the patient was given adjuvant combination chemotherapy with cisplatin and etoposide. He has been disease free for 12 months.

Child, Preschool↗
PubMedDate not supplied

Follicular thyroid carcinoma with a predominant insular component in a child: a case report.

Thyroid carcinoma is rare in children and differs from its adult counterpart in many aspects, including that childhood thyroid carcinomas are all well differentiated. Here we present a 14-year-old female from a goitrous area with follicular thyroid carcinoma with a predominant insular component. The child was well without recurrence or metastases after 5 years of follow-up.

Adenocarcinoma, Follicular↗
PubMedDate not supplied

Analysis of delta F508 mutation in cystic fibrosis pathology specimens.

Incidence of delta F508, a severe mutation of the CFTR gene is found to be 36.3% in paraffin block cystic fibrosis liver tissues. Samples are histologically grouped according to severity of pancreatic involvement. Two families where delta F508 was detected postmortem and who have no living children, will have the chance for a prenatal diagnosis in the future pregnancies.

Age Factors↗
PubMedDate not supplied

Neuroblastoma presenting as protein-losing enteropathy.

Protein-losing enteropathy is often reported to be associated with malignancies such as Hodgkin's disease, non-Hodgkin's lymphoma, and mesenteric mesenchymoma, but it seldom complicates neuroblastoma. In this report, we describe a case of neuroblastoma presenting as protein-losing enteropathy in which neurohumoral mechanisms were involved.

Female↗
PubMedDate not supplied

Duplication of the rectum resembling a juvenile polyp.

A five-year-old boy with a rectal mass mimicking a rectal polyp, which proved to be a cystic duplication of the rectum, is presented. In a child with painless rectal bleeding, a mass palpated during rectal examination is usually diagnosed initially as being a rectal polyp. However, the case presented revealed the possibility of rectal duplication.

Child, Preschool↗
PubMedDate not supplied

Fatal infectious mononucleosis in a family.

Two male siblings, one aged five and a half months (SB), and the other aged six months (VB), with fatal infectious mononucleosis phenotype of the X-linked lymphoproliferative syndrome, which resulted in the death of both infants, are presented. Both patients had been healthy, one until the age of five and a half months, and the other until the age of six months. Then, they developed a maculopapular rash, hepatosplenomegaly and lymphadenopathy. In one sibling, the serum IgG level was low, the IgM and IgA levels were high, and the proportion of E-rosette forming cells (E-RFC) and in vitro proliferative response to PHA were normal. In the other sibling, however, the serum IgG level was normal, the IgM and IgA levels were high and the stimulation index for proliferative response to PHA was reduced due to increased spontaneous blastogenesis. Anti-EBV antibodies were negative in both siblings, except for the IgM anti-VCA in V.B. A lymph node specimen could be studied in one infant and was found to be positive for the EBV genome. Postmortem histopathological findings included the absence of cortico-medullary differentiation and identifiable Hassal's corpuscles in the thymus and depletion of T-dependent regions of lymph nodes and spleen in V.B. Atypical mononuclear cell infiltration was detected in the portal areas of the postmortem liver biopsy in S.B.

Humans↗
PubMedDate not supplied

Epidermoid cyst of the spleen.

A sixteen-year-old girl treated by total splenectomy for epidermoid cyst of the spleen is presented: Epidermoid cysts of the spleen account for ten percent of non-parasitic cysts. Abdominal ultrasonography and computed tomography are the most reliable studies available in the diagnosis of these cysts. Partial splenectomy is the best mode of treatment for this disease, if feasible.

Adolescent↗
PubMedDate not supplied

Angiomyolipoma located in the lumbar region of a newborn.

An unusual example of angiomyolipoma, which presented as a bulging mass on the right lumbar region of a newborn baby, is presented. The case described is the first report of a newborn with an unusually located angiomyolipoma. The most common predilection sites are the renal parenchyma and the retroperitoneum. However, most of the patients described are adults, and to the best of our knowledge no newborn patient has previously been reported in the literature.

Angiomyolipoma↗
PubMedDate not supplied

Unusual appearance of the liver on ultrasonography and computed tomography in a patient with cystic fibrosis.

A seven-year-old boy with cystic fibrosis (CS) who presented with abdominal pain is reported. Ultrasonographic and computed tomographic studies of the upper abdomen revealed unusual liver findings. An ultrasound scan showed a liver that was exceedingly heterogeneous and a mixed echo pattern with dominant hyperechogenicity. Computed tomography showed large, multiple hypodense cyst-like lesions in the liver. Using the ultrasound scan as a guide, a needle biopsy was performed. The pathological findings were in accord with the findings obtained from ultrasonography and computed tomography, and were consistent with pathological findings seen in CS cases.

Biopsy, Needle↗
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Treatment of X-linked lymphoproliferative disease (Duncan disease) with high-dose methylprednisolone and etoposide (VP-16).

A five-year-old boy in the acute phase of X-linked lymphoproliferative (XLP) syndrome (Duncan disease) with high fever and hepatosplenomegaly was treated successfully with high-dose methylprednisolone and VP-16 for 15 months. He had been alive for four years after diagnosis as of this writing. We recommend high-dose methylprednisolone and VP-16 in patients with XLP who have to wait for a suitable donor before bone marrow transplantation.

Anti-Inflammatory Agents↗
PubMedDate not supplied

Carcinoma of the colon in children.

The symptoms, histology, extent and course of disease in 16 adolescents with colorectal carcinoma who were admitted to Hacettepe University Children's Hospital between 1972 and 1990 are presented. Most patients presented with vague abdominal complaints. Twelve of the 16 patients had mucin-producing adenocarcinoma. Extensive disease at diagnosis and unresponsiveness to medical management were determined. Only one patient survived free of disease four years after diagnosis. Nine of the patients died between one day and one year following the initial surgery. The remaining six patients were very ill when they were discharged from the hospital, after which time no information was received concerning them.

Adenocarcinoma, Mucinous↗
PubMedDate not supplied

Functional significance of dystrophin-positive fibers in Duchenne and Becker muscular dystrophy.

In this study, the ratios of dystrophin-positive (+), partially deficient (+/-), and deficient (-) fibers were investigated immunohistochemically in 28 Duchenne muscular dystrophy (DMD) and 4 Becker muscular dystrophy (BMD) patients using Dys I (midrod), Dys II (COOH-terminal), and Dys III (NH2-terminal) antibodies. In the biopsies of DMD patients, Dys II was negative in all cases; the mean ratio of Dys I (+) fibers was 0.05%, Dys I (+/-) 1.02%, Dys III (+) 0.27%, and Dys III (+/-) 0.75%. There was no correlation between these (+) or (+/-) fibers and the severity of clinical or laboratory findings. In BMD patients, it was shown that amino and carboxyl terminals of dystrophin could be affected in addition to the midportion.

Dystrophin↗
PubMedDate not supplied

Hydatid cyst mimicking pulmonary hematoma in a patient with hemophilia A.

We present a patient of 2.5 years of age with hemophilia A and a pulmonary hydatid cyst. A chest x-ray taken by chance showed a paracardiac opacity resembling an intrapulmonary hematoma which did not reduce in size after infusions of fresh frozen plasma and factor VIII but rather enlarged. Transabdominal ultrasound, colored echocardiography, thoracic computed tomography and magnetic resonance imaging findings were consistent with a cyst that was firmly attached on the border of the right atrium and also indented it; the wall was remarkably thick with no internal echoes. Hydatid cyst was diagnosed after thoracotomy.

Child, Preschool↗