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      Sanjad-Sakati Syndrome and Its Association with Superior Mesenteric Artery Syndrome

      case-report
      Author(s): *
      Publication date (Electronic):
      Journal: Case Reports in Pediatrics
      Publisher: Hindawi Publishing Corporation

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          Abstract

          Sanjad-Sakati syndrome (SSS) is an autosomal recessive disorder found exclusively in people of Arabian origin. It was first reported in the Kingdom of Saudi Arabia in 1988 and confirmed by a definitive report in 1991. The syndrome comprises of congenital hypoparathyroidism, seizures, severe growth and developmental retardation, low IQ, and atypical facial features. Supportive treatment in the form of vitamin D and growth hormone supplementation is often offered to patients suffering from SSS. This case study focuses on the steps taken to help a patient who was found to have very unusual symptoms and was later found to have superior mesenteric artery syndrome.

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          Superior mesenteric artery syndrome: CT and ultrasonography findings.

          Birsen Unal Daphan, Gökhan Kemal, Cagatay Daphan … (2005)
          The purpose of the study was to describe computed tomography (CT) and ultrasonography findings in superior mesenteric artery syndrome (SMAS). The study was performed on 89 CT examinations. Ultrasonography was performed on 32 and barium study was performed on four of these subjects. Group A consisted of cases with one or more of the following complaints: postprandial epigastric pain, weight loss and vomiting. Group B consisted of the remaining cases. Cases who had all of the above-mentioned clinical findings and duodenal dilatation, to-and-fro barium movement and SMA indentation in barium study were diagnosed as having SMAS. Body mass index (BMI, kg/m2) was calculated. The distance between SMA and aorta, at the location where the duodenum passes from, was measured on CT and ultrasonography. The angle between SMA and aorta was measured on ultrasonography images. Group and gender differences were analyzed with t-test, the relationship between clinical and CT findings was analyzed with Mann Whitney U test and the relations between BMI-CT and CT-ultrasonography measurements were analyzed with Pearson coefficients. Of 13 cases in Group A, 3 were diagnosed as SMAS. Eight of the cases showed gastric and/or duodenal dilatation. In 6 cases, antrum had an abnormally high location at portal hilus. In Group A, the SMA-aorta distance was 6.6 +/- 1.5 mm and the SMA-aorta angle was 18.7 +/- 10.7 degrees . In Group B, these values were 16.0 +/- 5.6 mm and 50.9 +/- 25.4 degrees , respectively (p < 0.001). Cut-off values between SMAS and Group B were 8 mm (100% sensitivity and specificity), and 22 degrees (42.8% sensitivity, 100 % specificity). CT and ultrasonography measurements (p < 0.001) and SMA-aorta distance and BMI (p=0.004) were significantly correlated. The SMA-aorta distance was significantly shorter in females (p=0.036). Gastric and/or duodenal dilatation and a diminished SMA-aorta distance have a significant correlation with clinical symptoms of SMAS that include postprandial pain, vomiting and weight loss.
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            A new syndrome of congenital hypoparathyroidism, severe growth failure, and dysmorphic features.

            Andrew R. C. Milner, N Sakati, S. Sanjad … (1991)
            Twelve infants (six boys, six girls) with severe hypocalcaemic tetany or convulsions were seen over a three year period. Nine patients were symptomatic in the newborn period. Their hypocalcaemia was associated with hyperphosphataemia and very low concentrations of immunoreactive parathyroid hormone. None of the babies suffered from congenital cardiac disease. Cell mediated immunity, measured in five patients, was normal. There were no chromosomal abnormalities but all patients shared several dysmorphic features including deep set eyes, microcephaly, thin lips, beaked nose tip, external ear anomalies, micrognathia, and depressed nasal bridge. Mental retardation of varying degree was found in all patients. All had severe intrauterine and postnatal growth retardation. Four patients have died. The remaining eight patients are on treatments with vitamin D and calcium supplements with no change in their growth pattern. We believe that this association of congenital hypoparathyroidism with severe growth failure and dysmorphism represents a new syndrome.
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              Superior mesenteric artery syndrome: an uncommon cause of intestinal obstruction.

              U Baltazar, Valerie Dunn, Ed Schmidt … (2000)
              Superior mesenteric artery (SMA) syndrome is an atypical cause of high intestinal obstruction, most frequently occurring in patients who have had rapid weight loss. Identification of this syndrome can be a diagnostic dilemma and is frequently made by exclusion. The most characteristic symptoms are postprandial epigastric pain, eructation, fullness, and voluminous vomiting. The symptoms are caused by compression of the third portion of the duodenum against the posterior structures by a narrow-angled SMA. When nonsurgical management is not possible or the problem is refractory, surgical intervention is necessary. We report a case of SMA syndrome in a patient without a history of rapid weight loss. The patient complained of early satiety, nausea, and vomiting of partially digested food worsening over 2 years. Diagnostic evaluation revealed compression of the third portion of the duodenum by the SMA with resultant proximal dilatation. The patient successfully had duodenojejunal anastomosis.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Rep Pediatr
                Journal ID (iso-abbrev): Case Rep Pediatr
                Journal ID (publisher-id): CRIPE
                Title: Case Reports in Pediatrics
                Publisher: Hindawi Publishing Corporation
                ISSN (Print): 2090-6803
                ISSN (Electronic): 2090-6811
                Publication date (Print): 2014
                Publication date (Electronic): 9 November 2014
                Volume: 2014
                Electronic Location Identifier: 108051
                Affiliations
                King Faisal Specialist Hospital Research Centre, P.O. Box 280581, Riyadh 11392, Saudi Arabia
                Author notes
                *Osamah Abdullah AlAyed: usamah222@ 123456hotmail.com

                Academic Editor: Doris Fischer

                Article
                DOI: 10.1155/2014/108051
                PMC ID: 4241564
                SO-VID: 53bfdaa8-dda6-41e1-9ef2-1148732c68ea
                Copyright © Copyright © 2014 Osamah Abdullah AlAyed.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 15 May 2014
                Date accepted : 21 October 2014
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Pediatrics
                Data availability:
                ScienceOpen disciplines: Pediatrics

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