An 80-year-old man was admitted for weight loss, abdominal pain and diarrhea for a month.
Family history: parents and eight deceased brothers without digestive pathology of interest.
Personal history: no allergies, smoking (60 cigarettes/day) and benign ethylic acid/day) until 25 years ago, generalized prostatic arthritis, hypertension, chronic obstructive pulmonary disease emphysematous.
She was diagnosed 3 years ago with diverticular colon disease by opaque enema after reporting hypogastric pain.
No significant alterations were detected and the total protein count was normal.
Standard treatment with: tamsulosin, zolpidem tartrate, biotin, candesartán, formosphere fumarate, inhaled ipratropium bromide and budesonide.
Non-steroidal anti-inflammatory drugs or antibiotics were not usually taken.
In the last month, due to the presence of diarrhea, she was treated with loperamide and ciprofloxacin, without improvement.
- Current disease: for one month, progressive asthenia, anorexia, weight loss (10 kg), numerous stools preceded by hypogastric, diurnal and nocturnal pain, with abundant mucus and soft consistency.
No fever, vomiting or other symptoms of interest were reported.
Physical impairment: physical impairment attributable to advanced age and loss of muscle mass.
Height 164 cm. Weight 67 kg. Nicked help for walking.
Absence of mental illness or mental illness
Presence of hyperpigmented macules in the upper limbs.
Rarely corporal hair.
No peripheral lymphadenopathy.
Cardiac arrest was normal.
Heart rate: 80 systoles/minute.
Arterial hypertension: 150/90 mmHg.
Respiratory mucus: diffuse decrease of vesicular murmur with few roncus and wheezing.
Normal auscultation abdomen, hypogastric tenderness and both iliac fossa, with absence of masses or visceromegaly.
Muscle atrophy in extremities, normal pulses and absence of oedemas
- Analytical: hematocrit, hemoglobin, mean corpuscular volume, platelets, glucose, urea, urea, sodium, transaminase levels remained normal, alkaline vitamin B, cholesterol, folic acid, ferritin.
Progressive elevation of white blood cell counts (13,900-30,900, normal 4,600-10,200/mm3), neutrophilia (70-90%), and C-reactive protein (16,9-2,591, normal 0-58,7-dl).
Normal coagulation study.
Proteinogram: hypoalbuminemia (2.49, normal 4.0-4.7 g/dl) and hypogamememia (0.39, normal admission, 2.3 g/dl), total protein levels reached 4.7 g/dl.
Elevated levels of gastrin (263, normal 13-115 pg/ml) and thyrotropin (TSH) (33.9-17.3, normal 0.12-5 uU/ml), normal serum cortisol (0.56-0.56)
TSH and T4 levels were normal at admission.
Thyroid peroxidase antibodies, thyroglobulin, antinuclear antibodies and anti-transglutaminase antibodies were negative.
Normal urinary sediment.
High levels of alpha antigen CA 19.9 (57.8, normal 0-37 U/ml) and CA 19.9 (57.8, normal 0-37 U/ml) antigen were detected; CA-reactive protein levels were normal.
Quantification of fecal fat was not evaluable.
Microbiology: negativity of coprocultives, urocultives, intestinal parasites, Clostridium difficile toxin, Mantoux and serology to Helicobacter pylori, HIV, cytomegalovirus, Salmonella and Yersin
- subsidiary requirements:
• Colonoscopy (practitioned in two occasions): diffuse, mainly from the rectosigmoid junction to the ileocecal valve, numerous nodular lesions or sessile polypoids were observed, almost completely empty colon.
The lesions show a red surface with petechial and ecchymotic lesions.
Biopsies were obtained at different levels and, in a second examination, a rectal macrobiopsy with polypectomy loop.
• Gastroscopy: normal esophagus, fundus and gastric body.
The gastric antrum mucosa, especially prepyloric, and the duodenal mucosa showed, diffusely, small nodules.
• Chest X-ray: emphysematous bullae on the right lung base.
No pleural effusion.
Location of mild pericardial effusion.
• Barium radiology of the esophagus, stomach and small intestine: thickened, irregular antral gastric folds with filling defects.
Thickening of small intestine folds, with nodular pattern, especially in and cyst, and dilution of contrast.
• Opaque enema: diverticula in the sigmoid and descending colon.
Multiple repletion defects throughout the length of the colon.
• Abdominal computed tomography: cysts in the left kidney.
Probable right adrenal adenoma.
Diverticula in the descending and sigmoid colon.
Diffuse thickening of the colon wall
Prostatic hypertrophy.
• Thyroid ultrasound: thyroid of normal size.
Small cysts in the right lobe.
Left lobe with homogeneous and normal echogenicity.
• Transthoracic echocardiogram: no pericardial effusion, preserved systolic function, mitral and aortic valve insufficiency.
• Histology of colon biopsies and rectal macrobiopsy: mucosa with intense distortion of glandular architecture, glandular dilations microcystic eosinophils and plasmatic neutrophils with abundant mucinous layer with moderate inflammatory infiltrate.
The alterations detected are compatible with the existence of colon and rectal hamartomas.
• Histology of gastric and duodenal biopsies: glandular dilations and intense mixed inflammatory infiltrate are observed, being the diagnosis of hamartomas of the stomach and duodenum.
- clinical manifestations: the patient is diagnosed with Cronkhitis-Canada syndrome, dying two months after hospital admission without any evident improvement in the initial clinical picture.
The patient presented asthenia and anorexia, with a variable number of stools with abundant mucous content, greater weight loss and persistent hypoproteinemia with lower limb edema.
Presence of fever (38 °C) in isolated days with positive blood cultures for E. coli attributable to phlebitis and urinary infections, an episode of hypocalcaemic tetany, pain in lower limbs of probable origin
The development of hypothyroidism and the increase in the numbers of carious fever antigen are noteworthy.
The patient died after presenting, in the last 24 hours, fever, cough with hemoptoic expectoration, dyspnea, data of respiratory failure in the arterial gas and of left base pneumonia in the radiology of the thorax.
Necropsy was performed.
- Treatments: the patient during his prolonged hospital stay received treatment, not simultaneously, with: oral and tube enteral nutrition, serum therapy with potassium chloride, calcium/ systemic calcium, amoxicillin/aspartate oral antibiotics/metalidazole therapy, oral methotrexate 300 mg.
- Autopsy report: the autopsy findings are consistent with the diagnosis of Cronkhite-Canadá syndrome.
- external fixation: reduction of the hair and dark brown macules in the upper limbs, especially on the dorsal side of the right hand and anterior side of the left forearm.
The fingers of both hands present onycholysis with a whitish ungueal coloration.
Bilateral hydrocele.
Oedemas in lower limbs
Internal exam: pericardial, pleural and peritoneal effusion.
Thyroid atrophy.
Pulmonary emphysema and left lung pneumonia
Bilateral adrenal nodular hyperplasia.
Moderate stenosis.
Prostate adenocarcinoma localized in both lobes without extrametastatic involvement.
Testicular atrophy.
No remarkable alterations were detected in liver, biliary tract or pancreas.
Study of the digestive tract: esophagus with no data of interest.
The gastric body shows decreased folds.
The gastric antrum shows a rough surface, "staffed", with nodular thickening of the folds and ecchymosis on the sessile surface, persisting these alterations up to the second portion of the duodenum, where it is present.
No significant alterations were observed in the ileoileum and iliac arteries.
The mucosa of the colon shows, throughout its extension, polypoid morphology, with multiple sessile prominences, ecchymotic surface and friable aspect.
Alterations are less evident in the right colon.
Diverticula in the left colon.
Microscopically, the most striking feature was the involvement of the antrum, duodenum, colon and rectum (in their entirety), which showed a similar histopathology, with the presence of multiple polypoid formations, some of them hamar type.
The lamina propria showed edema with hyperplasia of the fibers of the muscularis mucosa, and mild-to-severe chronic inflammation.
There is a decrease in intestinal villi of both duodenum and colon, and a decrease in gastric folds of fundus and body.
No signs of dysplasia were recognized in any of the sections examined.
No justification was detected for the figures for the increase in carious-elevation antigen.
Catechiae of death: severe malnutrition secondary to Cronkhite syndrome Canada.
Pneumonia in the left lung.
