Rare Diseases Symptoms Automatic Extraction

[General anesthesia for a boy with Wolf-Hirschhorn syndrome].

[wolf-hirschhorn syndrome]

Wolf-Hirschhorn syndrome (WHS) is a rare chromosomal abnormality in which there is deletion of the short arm of chromosome no. 4. Features of the condition include severe psychomotor retardation, characteristic facies and various congenital midline fusion anomalies. We report the anesthetic management in a 6-year-old boy with WHS, scheduled for renal biopsy under general anesthesia. Anesthesia was induced and maintained with sevoflurane and nitrous oxide in oxygen. Mask ventilation was performed easily. After establishment of mask ventilation, laryngeal mask airway (LMA) was inserted smoothly without muscle relaxant and an adequate airway was established with a LMA. He has episodes of transient deterioration in renal function with physical stress. To decrease renal effects of perioperative stress, transversus abdominis plane (TAP) block and intravenous patient controlled analgesia (IV-PCA) were administered for postoperative analgesia. The operation ended without any complications. Anesthetic emergence was rapid and he had no pain and decline in renal function.

Diseases presenting "pain" symptom

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  • acute rheumatic fever
  • adrenal incidentaloma
  • adrenomyeloneuropathy
  • aniridia
  • aromatase deficiency
  • carcinoma of the gallbladder
  • cholangiocarcinoma
  • coats disease
  • congenital diaphragmatic hernia
  • congenital toxoplasmosis
  • cushing syndrome
  • cutaneous mastocytosis
  • cystinuria
  • dedifferentiated liposarcoma
  • dentin dysplasia
  • dracunculiasis
  • dystrophic epidermolysis bullosa
  • epidermolysis bullosa simplex
  • erdheim-chester disease
  • erythropoietic protoporphyria
  • esophageal adenocarcinoma
  • esophageal carcinoma
  • esophageal squamous cell carcinoma
  • fabry disease
  • familial mediterranean fever
  • focal myositis
  • hirschsprung disease
  • hodgkin lymphoma, classical
  • holt-oram syndrome
  • junctional epidermolysis bullosa
  • kabuki syndrome
  • kindler syndrome
  • lamellar ichthyosis
  • liposarcoma
  • locked-in syndrome
  • lymphangioleiomyomatosis
  • malignant atrophic papulosis
  • neuralgic amyotrophy
  • oligodontia
  • oral submucous fibrosis
  • papillon-lefèvre syndrome
  • phenylketonuria
  • pleomorphic liposarcoma
  • primary hyperoxaluria type 1
  • proteus syndrome
  • pyomyositis
  • scrub typhus
  • sneddon syndrome
  • systemic capillary leak syndrome
  • thoracic outlet syndrome
  • trochlear dysplasia
  • typhoid
  • von hippel-lindau disease
  • waldenström macroglobulinemia
  • well-differentiated liposarcoma
  • wolf-hirschhorn syndrome

This symptom has already been validated