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Biomedical subjects

B Z Garty

Publications and source records attributed to B Z Garty.

106 records · Page 6Linked to original sources

Cholelithiasis in children with immunoglobulin A deficiency: a new gastroenterologic syndrome.

In view of the role of immunoglobulin A (IgA) in the local host defenses of the gut and biliary tract, and the association of IgA deficiency with various intestinal disorders, an ultrasonographic study was performed to determine the prevalence of gallstones in selective IgA deficiency. Eight of 13 children (three girls and five boys) with selective IgA deficiency had abnormal biliary sonograms. Four had cholelithiasis, and four had biliary sludge only. IgA deficiency may predispose to gallbladder infection, and thus to the development of gallstones. Decreased bile acid pool due to intestinal losses, and biliary stasis due to reduced hormonal gallbladder stimulation may also play a role in some cases, but further studies are needed. This association adds a new gastrointestinal abnormality to those known to be associated with selective IgA deficiency.

Adolescent↗

Penile tourniquet syndrome.

A 2 1/2-year-old boy was examined because of swelling of the penis of six weeks' duration. Several strands of hair wrapped around the penis and buried under the epithelium were found to cause a constriction. The penile tourniquet syndrome should be suspected whenever the penis is swollen, especially in children, even if a foreign body is not visible.

Child, Preschool↗

Transient acute cortical blindness associated with hypoglycemia.

A 7-year-old boy with glycogen storage disease type III developed transient acute cortical blindness associated with hypoglycemia on two separate occasions; the patient also demonstrated transient high-voltage slowing on the electroencephalogram over both occipital areas. This patient is the second examined in our pediatric department during a 5 year period with acute cortical blindness associated with hypoglycemia.

Blindness↗

Gerstmann tetrad in leopard syndrome.

We report a 12-year-old boy with multiple lentigines (Leopard) syndrome who was evaluated for learning difficulties and Gerstmann tetrad syndrome (i.e., dyscalculia, left-right disorientation, finger agnosia, and dysgraphia). Cranial computed tomography revealed left ventriculomegaly, more pronounced in the occipital horn suggesting mild atrophy of the left parietal lobe. This is the first report of an association between the Leopard and Gerstmann syndromes and one of the few to demonstrate a computed tomographic abnormality in the latter.

Abnormalities, Multiple↗

Hemophagocytic lymphohistiocytosis associated with visceral leishmaniasis.

A 2-year-old child presented with fever and hepatosplenomegaly. Laboratory findings showed pancytopenia, hypertriglyceridemia, hyperferritinemia, and high levels of soluble-IL2 receptors. Initial bone marrow aspiration and biopsy revealed mild hemophagocytosis. A diagnosis of hemophagocytic lymphohistiocytosis was made and appropriate treatment was begun. Repeated marrow aspiration performed because of lack of clinical response revealed Leishmania amastigotes in macrophages in addition to active hemophagocytosis. Treatment with liposomal amphotericin resulted with rapid recovery. Visceral leishmaniasis should be considered in the differential diagnosis of hemophagocytic syndrome.

Child, Preschool↗

Recurrent dislocations of the hip in a child presenting as nocturnal pains.

Nocturnal pains of the leg were the only complaint of a 2 1/2-year-old girl with recurrent dislocations of the left hip. Unusual movement during sleep probably caused the dislocations. This case represents a benign entity characterized by spontaneous or voluntary posterior dislocations of the femoral head in young children without other significant connective tissue abnormalities.

Child, Preschool↗

Congenital Wells syndrome.

We describe a girl with eosinophilic cellulitis (Wells syndrome) in whom the disease appeared immediately after birth with subcutaneous nodules on the scalp and trunk, followed by the characteristic skin swelling and erythema at the age of 6 months. The lesions disappeared after a few weeks, but recurred several times. The mother had consumed large quantities of medications during the pregnancy, including iron, vitamins, and "natural remedies." Based on time of onset, this may be regarded as a unique case of congenital Wells syndrome. Its relation to the medications taken by the mother remains speculative. Subcutaneous nodules may be the presenting sign of Wells syndrome in children.

Cellulitis↗