Intestinal lymphangiectasia (IL) may vary widely in its manifestations and severity. Fifteen children seen between 1960 and 1974 with histologically proven IL are analyzed by clinical, laboratory, radiologic, and histologic criteria. Remissions occurred in most patients and none died. Exacerbations occurred in five children. Diarrhea was present in 14 patients and in 13 appeared before the age of 3 years. Vomiting occurred in nine patients and growth retardation in seven. Four children had associated peripheral lymphedema and two of these had a family history of lymphedema, both had affected fathers and one had affected siblings and paternal cousins. Seven had hypoproteinemic edema, and, of these, four suffered from hypocalcemic seizures. Chylous effusions were present in five. Hypoproteinemia was present in 12 although five had no hypoalbuminemic edema. Six had lymphopenia which was related to the severity of the disease and was the last abnormality to disappear after clinical remission. Lymphopenia may first appear years after the protein loss begins. Upper gastrointestinal tract series were performed in 13 children and had diagnostic supportive value in seven. Six children had two or more small-intestinal biopsies done. They all showed great variation from one examination to the other, ranging from a normal appearance to severe changes. Lymphatic block may occur at different sites-in the lamina propria only, generalized (lamina propria, submucosa, serosa, and mesentery), or conversely in the mesentery alone with minimal changes in the lamina propria. In three patients intravenous hyperalimentation was necessary. Specific treatment with a high-protein, low-fat diet with added medium-chain triglyceride (MCT) is valuable. Surgical resection was of benefit in one patient, and anastomosis of mesenteric to para-aortic lymph nodes in another.
obscured because these terms a r e often used interchangeably. A l l cases of PC and OC with a positive blood,tissue a s p i r a t e , o r abscess culture seen a t Children's o r Eye and Ear Hospital i n the past 10years were analyzed. Results (including median age): TOTAL H.influenzae B(H1B) S.pneumoniae(SP) Other bacteria -PC 61 57% (15 mos) 18% (12 mos) 25% (6 y r s ) OC 10 0% 0% 100% (11 yrs) PC caused by HIB and SP, characterized by bacteremia, fever and p e r i o r b i t a l swelling, discoloration, induration and pain, is a d i s t i n c t clinicalentityoccurringinyoung chi1dren;sinus radiographs frequently demonstrated "cloudy" sinuses,but interpretation of the radiographswas complicated by the small sizeand i n d i s t i n c t bonymarginsof thesinuses o f i n f a n t s , andby concurrent upper respiratory infection and overlying s o f t t i s s u e swelling. Ophthalmoplegia and proptosis, never present i n cases of PC, always accompanied OCas did radiographic evidence of s i n u s i t i s . OC due t o HIBhasnot been documented i n t h i s study or i n the l i t e r a t u r e . PS secondary t o s i n u s i t i s i s characterized by non-tender, noninduratededema due t o venous congestion, not b a c t e r i a l c e l l u l itis. Careful d i f f e r e n t i a t i o n of PC,OC and PS secondary t o sinus i t i s is necessary f o r the selection of appropriate therapy. Familial Mediterranean fever (FMF) i s characterized by periodic attacks of fever and s e r o s i t i s , particularly p e r i t o n i t i s and synovitis. The disease i s transmitted by an autosomal recessive gene and primarily a f f e c t s Sephardic Jews, Arabs, Turks and Armenians. Duing the period [1970][1971][1972][1973][1974][1975][1976][1977][1978][1979] we observed seven children with FMF who developed HenochShoenlein purpura (HSP). During t h i s period 0.16% of 2140 pediatric admissions per year were due t o HSP. From a pediatric FMF population of 70 patients an average of 23 (33%) were admitted annually and one (4.3%) had HSP each year. Pertinent c l i n i c a l features a t t h e time of HSP -age range 3-13 years (mean 7 years) males: fernales 5:2. All patients had non thrombocytopenic purpura, a r t h r i t i s or arthlagia and fever. Five patients (70%) had diffuse abdominal pain and two boys had t e s t i c u l a r pain and swelling. Four patients (56%) had hematuria and one protei nuria, only. The erythrocyte sedimentation r a t e ranged from 50-150 mn/hr (mean 88) and fibrinogen 208-660 mg/dl (mean 373 mg/dl). The duration of hospitalization ranged from 13-150 days (mean 51 days). The association of FMF and HSP, two diseases of unknown etiology may provide new avenues of study in these diseases.PULMONARY PARAGONIMIASIS I N INDOCHINESE REFUGEE CHIL-691 DREN. 5.T. Shulman, R. Yogev, K. Burton and K . Boyer (spon. by Henry Nadler). Children's Memorial Hosp., Northwestern Univ., and Michael Reese Hosp., Chicago.The high prevalence of tuberculosis i n recent Indochinese refugees of a l l ages i s well documented, with 100-fold grea...
Congenital indifference to pain with anhydrosis (CIPA) is a well-defined entity among a group of sensory deficiency syndromes. Children with this genetic disease are insensitive to pain and temperature and do not sweat and suffer from psychomotor retardation. Self-inflicted trauma may be severe and lead to insoluble orthopedic problems. To date, 11 cases have been reported. We have reviewed the literature and are describing two girls with CIPA, born to consanguine Jewish parents of Moroccan origin. Immunoglobulin deficiency has been reported in CIPA but an immunologic investigation on one of our cases showed only an early and transient deficiency of IgA. The presence in the family of a brother with ataxia telangiectasia and complete absence of IgA would seem to be irrelevant and it seems probable that the parents are heterozygotic for two disparate autosomal recessive syndromes.
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