Saudi Journal of Gastroenterology
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CASE REPORT Table of Contents   
Year : 2001  |  Volume : 7  |  Issue : 3  |  Page : 116-118
Primary intestinal lymphangiectasia, nephrotic syndrome and clomid-induced triplet pregnancy


Department of Pediatrics, King Fahad Specialist Hospital, Braidah, Saudi Arabia

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Date of Submission23-Feb-2000
Date of Acceptance06-May-2001
 

How to cite this article:
Al-Mushaikih AA. Primary intestinal lymphangiectasia, nephrotic syndrome and clomid-induced triplet pregnancy. Saudi J Gastroenterol 2001;7:116-8

How to cite this URL:
Al-Mushaikih AA. Primary intestinal lymphangiectasia, nephrotic syndrome and clomid-induced triplet pregnancy. Saudi J Gastroenterol [serial online] 2001 [cited 2020 Nov 29];7:116-8. Available from: https://www.saudijgastro.com/text.asp?2001/7/3/116/33423


Primary intestinal lymphangiectasia is associated with lymphatic dysfunction resulting in bulk loss of lymph into the bowel lumen and protein losing enteropathy. This is as a result of congenital disorder of the lymphatic system, which is supported by the frequent incidence of associated lymphatic anomalies in the limbs and in many other anatomic locations [1] . It may occur as an isolated abnormality or as part of more generalized syndromes such as Klippel-trenancy syndrome, Turner syndrome and Noonan syndrome [2] . Some cases of primary intestinal lymphangiectasia and nephrotic syndromes are autosomal recessive in nature [3],[4],[5] .

Primary intestinal lymphangiectasia is either diffuse or localized ectasia of the enteric lymphatics. Ectatic lymphatics may be located in the mucosa, submucosa or subserosa [6]. Patients with this disorder are frequently having reduced levels of immunoglobulins and low circulating lymphocytes due to lymph loss [7] .

In this report, we present a case of primary intestinal lymphangiectasia in one member of a triplet (triplet III), the other members triplet I and II have nephrotic syndromes. This triplet is the product of Clomid-induced pregnancy. To our knowledge, the association of primary intestinal lymphangiectasia and nephrotic syndromes in members of clomid-induced triplet pregnancy has not been reported.


   Case Report Top


A 3-year-old Saudi girl (triplet III) was apparently well till the age of 18 months when she presented with a long history of edema of eyelids associated with chronic diarrhea, recurrent abdominal pain and vomiting. She was a product of 35-36 weeks gestation born by lower segment caesarian section, conceived after clomid therapy (50mg for five doses started on the second day of the menstrual cycle), following a 5-year period of secondary infertility. She had a normal neonatal period. Her birth growth parameters were: weight B 1.75kgs, length 45cm and occipito-frontal circumference 31 cm. These birth growth parameters are within the 10 th percentile for this kind of pregnancy. She was breastfed for few weeks, supplemented with Nan-milk formula about 20-30m1 3 hourly for the first five months of life. Similac was then introduced replacing Nan formula, at the same time cerelacs were introduced. She was on normal diet for age on presentation, including rice, bread, meat, vegetables and fruits. In general her nutritional intake was reasonable. The past medical history was unremarkable. She was developing fairly well. The father is 48 years old and the mother is 40. They are first-degree cousins. There is no history of protein losing disorders in the parents or grandparents. Her brother triplet I and sister triplet II have nephrotic syndrome, discovered at the age of 18 months. They presented with long history of puffiness of eyelids without diarrhea or abdominal pain. Her 12-years old sister has a relapsing nephrotic syndrome. The younger brother (one­year-old) is apparently well. Her half six siblings from the second father's wife, are all healthy.

The physical examination showed a malnourished girl, with growth parameters below the fifth centile. The weight was 9.2 kg and the height was 85 cm. Previous growth parameters recorded during her previous admissions indicated that the patient was not thriving. The abdominal examination revealed ascites and the lower limbs showed pitting edema. The remainder of the physical examination was within normal limits.

The laboratory investigations included hematology profile: Hb 10.9g/L, MCV 69.2 (FI) MCH 22.9 (Pg); MCHC 33.1 gm/dl; Platelets (569x10 9 )/L, WBC (10.96x10 9 )/L, differential: neutrophils 15%, lymphocytes 65% equal to 7124/ml 3 , monocytes 0.4% and eosinophils 06%. Urine analysis: albumin normal-trace and the rest of urine analysis was unremarkable. The urine culture grew klebsiella spp. at one occasion and  Escherichia More Details coli at another. The liver function test was normal. Stool analysis: positive occult blood with the presence of red blood cells, pus cells and yeast. The stool culture showed no  Salmonella More Detailse or shigelloe spp. There were no ova, parasites or giardial cysts seen in the stool microscopy. The total serum protein was 31 gm/L (66-87 gm/L), albumin 14 gm/L (38-50 gm/L). Immune profile: IgG 2.5 gm/L (8-19 gm/L), IgA 0.433 gm/L (0.9-1.5 gm/L), IgM 0.372 gm/L (1.30­5.2), and triglycerides 5 mmol/L (0.57-2.26). Abdominal ultrasonography showed normal kidneys, gallbladder and liver. Barium swallow and follow through revealed thickened mucosa and floculation of the barium, a nonspecific radiological finding of malabsorption syndromes. There was no malrotation or any other anatomic abnormalities. Upper gastrointestinal endoscopy showed whitish creamy like mucosa [Figure - 1]. Small bowel histopathology revealed normal villous-crypt ratio and dilatation of the lymphatics in the villi [Figure - 2], resulting in distortion of the villous configuration. There was an excess of lymphocytes and plasma cells in the lamina propria. These features are consistent with lymphangiectasia.

The work up to rule out a secondary cause for the protein-losing enteropathy, in addition to what has been mentioned above, included the duodenal aspirate which was negative for Giardia lamblia. Lymphangiography was not done due to the lack of strong clinical evidence of lymphatic obstruction. The other clinical entities causing hypoalbuminemia were remote possibilities. Micturating cystourethrography, as part of the work up for urinary tract infection, was normal.

The patient was managed nutritionally by protein­hydrolysate (pregestimil) 100 ml 3 hourly. After few weeks, the patient started to refuse pregestimil, which was replaced by portagen formula for few months. She was then put on fat-free meals supplemented with medium chain triglycerides, fat­soluble vitamins A, D, E and K orally, polycose, elemental iron, and monthly gammaglobulins.

During the course of the illness, the patient presented once with symptoms of acute abdomen with visible peristalsis. Intussusception was suspected clinically and radiologically, laparotomy was done but no intussusception was found. Usually in each admission, the patient comes to the ward directly in cases of recurrences of symptoms and treated symptomatically including albumin and diuretics. During the last admission, the patient presented with weakness being unable to sit or stand, wasted, frequent crying associated with abdominal colic and distension, loose motions and vomiting with carpopedal spasms (tetany). Her last investigations showed: Hb 78gm/L, WBC 15.9x10 9 /L, lymphocytes (48%) 7632/ml -3 , platelets 21000, total protein 23.4g/L, albumin 6.6g/L, Na + 122mmoUL, K + 2.2mmol/L and blood, urine and stool cultures showed no growth.

Despite intensive management, the patient expired due to circulatory collapse most probably secondary to severe hypoalbuminaemia and clinical sepsis.


   Discussion Top


The case presented is not unusual in its clinical features. However, to the best of our knowledge, the association of primary intestinal lymphangiectasia (triplet III) and nephrotic syndromes (triplet I and II) in the members of clomid-induced triplet pregnancy has not been reported [8],[9],[10] . It is possible that this association might be related to clomid as a new side effect alternatively, some cases of nephrotic syndromes and intestinal lymphangiectasia might have the same genetic basis as some of them autosomal recessive in nature [3],[4],[5] . For this reason the role of clomid therapy in inducing these protein­losing disorders is worth raising.

 
   References Top

1.Roy CC, Silverman A, Alagile D (Eds). Pediatric Clinical Gastroenterology, 4` h Edition 1995:362-73.  Back to cited text no. 1    
2.Jones KL. Smith's Recognizable Patterns of Human Malformations, 4 th Edition. Philadelphia WB Saunders. 1988.  Back to cited text no. 2    
3.Hennekam RC, Geerdink RA, Hamel BC, et al. Autosomal recessive Intestinal lymph angiecta-sia and Lymphedema, with facial anomalies and mental retardation, Am J Med Genet 1989, 34:593-600.  Back to cited text no. 3    
4.Hilliard RI., McKendry JBJ, Philips MJ. Congenital abnormalities of the lymphatic system: A new clinical class i fi-cati on, Pediatrics 1990, 85:988-93.  Back to cited text no. 4    
5.Nelson: Textbook of Paediatrics, 14 th Edition, 1992: 882.  Back to cited text no. 5    
6.Waldman TA. Protein-losing Ente-ropathy­Gastroenterology. 1966:422-43.  Back to cited text no. 6    
7.Strober W, Wochner RD, Carbone PP, Waldman TA, Intestinal Lym-phangiectasia A Protein-losing Enteropathy with Hypogammaglobulinaemia, Lympocytopenia and Impaired Homografi Rejection: J. clin Invest. 1967, 46: 1643-56.  Back to cited text no. 7    
8.Seidmon E. Protein-losing enteropathy In: Roy CC, Sideman A, Alagile D (Eds). Pediatric Clinical Gastroenterology, Philadelphia WB Saunders, 1995:367-73.  Back to cited text no. 8    
9.Herait P, Gisselbrecht C, Ferme C, et al. Lymphome non-Hodgkinien survernant au cours d'une lymphangiectasie intestinale de Waldmann, Nouv Rev Fr. Hermatol 1985: 299-302.  Back to cited text no. 9    
10.Bolton RP, Cotter KL, Losowasky MS. Impaired Neutrophil Function in Intestinal Lymphangiectasia, J Clin Pathol. 1986: 876-80.  Back to cited text no. 10    

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Correspondence Address:
Ahmad A Al-Mushaikih
Consultant Pediatrician & Gastroenterologist, King Fahad Specialist Hospital, Braidah, P. 0. Box 2290, AIQasim
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


PMID: 19861780

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